ENT (Otorhinolaryngology)

The External Ear

• The external ear comprises the auricle (pinna) and the external auditory canal, ending at the tympanic membrane.

The Middle Ear

• The middle ear (tympanic cavity) is an air-containing space in the petrous temporal bone, lined by mucosa and connected to the nasopharynx by the eustachian tube and to the mastoid air cells by the aditus ad antrum
• It is described as a biconcave disc or a matchbox on edge, and is divided into three parts by lines drawn through the upper and lower margins of the tympanic membrane: the epitympanum (attic), above; the mesotympanum, opposite; and the hypotympanum, below
• The attic is the site of cholesteatoma, and the protympanum lies anteriorly, leading to the Eustachian tube

• The ossicles — the malleus (head, neck, handle, and the lateral and anterior processes); the incus (body, short process and long process ending in the lenticular process); and the stapes (head, two crura and footplate), which is the smallest bone in the body
• The long process of the incus is the commonest ossicle to necrose, because its blood supply is tenuous and end-arterial — hence incus erosion in chronic otitis media and cholesteatoma
• The intratympanic muscles — tensor tympani, inserted into the malleus and supplied by the mandibular nerve (V3); and stapedius, the smallest skeletal muscle in the body, inserted into the neck of the stapes and supplied by the facial nerve (VII). They contract reflexly to loud sound, stiffening the ossicular chain and attenuating transmission — the basis of the acoustic (stapedial) reflex, and the reason its loss causes hyperacusis in facial palsy
• The chorda tympani crosses the middle ear between the long process of the incus and the handle of the malleus, carrying taste from the anterior two-thirds of the tongue and secretomotor fibres to the submandibular and sublingual glands. It is at risk in middle ear surgery, and its injury causes metallic taste and dryness
• Blood supply — chiefly from branches of the maxillary artery, principally the anterior tympanic, with contributions from the stylomastoid, middle meningeal and ascending pharyngeal arteries
• Nerve supply of the mucosa — the tympanic plexus on the promontory, formed mainly by jacobson’S nerve, the tympanic branch of the glossopharyngeal (IX) — which is why tonsillitis and tonsillectomy cause referred otalgia
• Auricular cartilage has no blood supply of its own, which is the whole reason a haematoma must be drained without delay
• Pull the pinna up and back in an adult and back and down in an infant, whose canal is short, straight and largely cartilaginous
• The isthmus is where foreign bodies impact, at the junction of the cartilaginous and bony parts
• Local anaesthetic cannot be infiltrated in the bony canal.
• The canal cleans itself by outward epithelial migration, which is why cotton buds cause impaction by pushing wax against the flow
• Cerumen is acidic and lysozyme-rich, so removing it predisposes to otitis externa rather than preventing it

The Bony and Membranous Labyrinth

• The inner ear (labyrinth) lies within the petrous temporal bone and comprises a bony labyrinth containing perilymph, within which floats a membranous labyrinth containing endolymph.
• It houses the organs of both hearing and balance.
• The bony labyrinth has three parts — the vestibule centrally; the cochlea anteriorly; and the three semicircular canals posteriorly
• The cochlea is a spiral of two and a half to two and three-quarter turns about a central bony modiolus.
• The cochlea has three compartments: the scala vestibuli above and the scala tympani below, both containing perilymph and communicating at the apex through the helicotrema; and between them the scala media (cochlear duct), containing endolymph and housing the organ of CORTI
• The scala media is bounded by reissner’S membrane above, the basilar membrane below, and the stria vascularis laterally
• The stria vascularis secretes the endolymph and generates the endocochlear potential of +80 mV. It is the only epithelium in the body with an intraepithelial capillary bed, and it is highly susceptible to ototoxic drugs and to ischaemia
• The fluids differ IN composition like intra- and extracellular fluid, and this IS the key TO the physiology: perilymph resembles extracellular fluid — high sodium, low potassium; whereas endolymph resembles intracellular fluid — high potassium (150 mmol/L), low sodium. It is the only extracellular fluid in the body with this composition
• The consequence is a large electrochemical gradient between the endolymph (+80 mV) and the interior of the hair cell (about −60 mV) — a potential difference of some 140 mV, which drives potassium into the hair cell when its channels open and powers transduction
• Perilymph communicates with the CSF through the cochlear aqueduct; endolymph drains through the endolymphatic duct and sac.

The Organ of Corti and the Vestibular Apparatus

• The organ of CORTI rests on the basilar membrane and is the end organ of hearing. It contains one row of inner hair cells (3,500) and three rows of outer hair cells (12,000 to 20,000), separated by the tunnel of Corti and supported by the pillar cells and the cells of deiters, with the tectorial membrane overlying the stereocilia
• The division OF labour between the two types OF hair cell IS the most important point, and is frequently stated the wrong way round:
• Inner hair cells are the true sensory receptors. Although far fewer, they receive 95% of the afferent fibres of the cochlear nerve, and they are what actually send hearing to the brain
• Outer hair cells are the cochlear amplifier. They receive mainly efferent innervation (from the olivocochlear bundle), and they contract and elongate in response to sound (electromotility, mediated by the protein prestin), actively amplifying and sharpening the basilar membrane response by some 40 to 60 dB
• Two clinical consequences follow directly: otoacoustic emissions are generated by the outer hair cells.
• The vestibular apparatus comprises the three semicircular canals.
• The canals lie in three mutually perpendicular planes, and each is coplanar with its opposite-side partner — the two laterals together, and each anterior with the contralateral posterior. The lateral canal is inclined 30 degrees to the horizontal, which is why the head is tilted 30 degrees forward when it is being tested by caloric stimulation

Conduction of Sound to the Cochlea

• Hearing is the conversion of airborne sound into nerve impulses.
• Sound is conducted mechanically through the external and middle ear, transduced in the cochlea, and analysed in the central auditory pathway.
• The central problem the middle ear exists to solve is impedance mismatch. Sound arrives in air but must be transmitted into the fluid of the cochlea. Fluid has a far higher acoustic impedance, so 99.9% of sound energy striking a water surface is reflected, a loss of some 30 dB

Cochlear Transduction

• 1.
• Stapes footplate moves at the oval window, displacing perilymph in the scala vestibuli → 2.
• A travelling wave is set up along the basilar membrane (von Bekesy), moving from base to apex → 3.
• Tonotopic localisation — the wave peaks at a position determined by frequency.
• The base is narrow and stiff and responds to high frequencies; the apex is wide and floppy and responds to low frequencies.
• This place principle is preserved all the way to the cortex, and is what a cochlear implant electrode array exploits → 4.
• Shearing between the basilar and tectorial membranes deflects the stereocilia → 5.
• Deflection toward the tallest stereocilium opens mechanically gated channels through the tip links → 6.
• Potassium floods IN from the high-potassium endolymph, driven by the 140 mV gradient, depolarising the hair cell → 7.
• Depolarisation opens calcium channels and releases glutamate at the base of the inner hair cell → 8.
• The cochlear nerve fires, and the signal passes to the spiral ganglion and centrally → 9.
• Outer hair cells simultaneously amplify the travelling wave through prestin-mediated electromotility, sharpening the peak and adding 40 to 60 dB of gain
• The central auditory pathway — cochlear nerve to the cochlear nuclei in the pons; then to the superior olivary complex (largely crossed, but bilateral from this point onward); the lateral lemniscus; the inferior colliculus; the medial geniculate body; and the auditory cortex in the superior temporal gyrus (areas 41 and 42)
• Because the pathway is bilateral above the cochlear nuclei, a unilateral central lesion does not cause significant deafness — which is why unilateral deafness always indicates a lesion of the cochlea or the cochlear nerve, and never of the cortex
• The superior olive is where the two ears are first compared, and is therefore the basis of sound localisation, using interaural time differences for low frequencies and interaural intensity differences for high frequencies
• The audible range is 20 to 20,000 Hz, with greatest sensitivity between 500 and 4,000 Hz — the speech frequencies, which is what audiometry concentrates on
• The decibel is a logarithmic ratio, not an absolute unit, which is why a 10 dB change represents a tenfold change in intensity and why hearing loss cannot be averaged naively
• The acoustic (stapedial) reflex contracts stapedius bilaterally to sound above about 70 to 100 dB, stiffening the chain and protecting the cochlea from sustained loud sound. Its latency is too long to protect against impulse noise such as a blast or gunshot — which is precisely why such injuries are so damaging
• The efferent olivocochlear bundle modulates outer hair cell activity, improving the detection of signals in noise

The Vestibular End Organs

• Balance is maintained by three inputs integrated centrally — the vestibular apparatus, vision, and proprioception from the neck and limbs. Any two can compensate for the loss of the third.
• The semicircular canals detect angular (rotational) acceleration. When the head rotates, endolymph lags behind by inertia, deflecting the cupula and the stereocilia within it
• The cupula has the same specific gravity as endolymph, so it is not deflected by gravity — which is why the canals respond to rotation but not to head position. When otoconia become attached to it (cupulolithiasis), it becomes gravity-sensitive, and that is one mechanism of positional vertigo

Reflexes and Clinical Application

• The vestibulo-ocular reflex (VOR) is the most important vestibular reflex: head movement produces an equal and opposite eye movement, stabilising the image on the retina. It is the fastest reflex in the body (latency 10 ms) — far faster than visual tracking.
• Its failure causes oscillopsia — the world appearing to bounce with each step — which is the disabling symptom of bilateral vestibular loss, as after gentamicin
• The head impulse (Halmagyi) test tests it directly: a rapid, small, unpredictable head turn toward the affected side produces a corrective saccade because the reflex fails. It is central to the hints examination distinguishing peripheral from central vertigo
• The vestibulospinal reflexes maintain posture and tone, and are what is tested by romberg, sharpened Romberg, unterberger stepping and gait testing
• Nystagmus has a slow phase generated by the vestibular system and a fast corrective phase generated centrally; by convention it is named by the direction of the fast phase, although the slow phase is the pathological one
• Caloric testing applies this physiology — with the head raised 30 degrees to bring the lateral canal vertical, warm water induces nystagmus toward the same side and cold toward the opposite side, remembered as COWS: Cold Opposite, Warm Same. It is the only test that examines each labyrinth separately
• Central compensation is the reason most vestibular disease recovers: the cerebellum and brainstem re-balance the resting tone over days to weeks. It is promoted by early mobilisation and vestibular exercises and delayed by prolonged bed rest and by vestibular sedatives — which is why prochlorperazine should be stopped within a few days and is one of the commonest errors in managing vertigo
• Compensation is also impaired in the elderly, in visual impairment, in cerebellar disease and by anxiety
• Motion sickness arises from sensory conflict between vestibular, visual and proprioceptive inputs — classically reading in a moving car, where the vestibular system signals motion and vision does not
• The cupula has the same specific gravity as endolymph, so gravity does not deflect it and the canals sense rotation only
• Otoconia attached to the cupula make it gravity-sensitive, which is the cupulolithiasis mechanism of positional vertigo
• Ampullopetal flow excites the lateral canal and ampullofugal flow excites the vertical canals.
• Nystagmus occurs in the plane of the canal stimulated, which is how the offending canal is identified in positional testing
• The canals work in push-pull pairs, so the brain reads a difference and gains both sensitivity and noise rejection
• Acute loss makes the intact side appear maximally excited, giving violent vertigo with nystagmus beating away from the affected ear

Course of the Facial Nerve

• It is a mixed nerve with four components: motor to the muscles of facial expression, stapedius, stylohyoid and the posterior belly of digastric; secretomotor (parasympathetic) to the lacrimal, submandibular, sublingual and nasal glands; special sensory (taste) from the anterior two-thirds of the tongue; and general sensory from the concha and a small area behind the ear
• The sensory and secretomotor fibres travel as the nervus intermedius, a separate root between the motor root and the eighth nerve

Topodiagnosis and Applied Aspects

• The branches allow the level OF A lesion TO BE deduced from the signs, and this is the classical application — each branch lost places the lesion above its origin:
• Lacrimation reduced (Schirmer test) — lesion at or above the geniculate ganglion, since the greater superficial petrosal nerve leaves there
• Stapedial reflex absent, with hyperacusis — lesion above the nerve to stapedius
• Taste lost and salivary flow reduced — lesion above the chorda tympani
• All of these intact with facial weakness — lesion below the chorda, in the mastoid segment or extratemporal
• The most important clinical distinction IS upper versus lower motor neurone, and it turns on the forehead: the upper face has bilateral cortical representation, so an upper motor neurone (supranuclear) lesion spares the forehead and the patient can still wrinkle the brow and close the eye; a lower motor neurone lesion involves the whole half of the face
• Two refinements are worth knowing: an upper motor neurone lesion often shows emotional sparing, the face moving normally on spontaneous laughter though not to command, because emotional pathways are separate; and it is accompanied by other neurological signs
• Causes relevant to ENT — bell’S palsy, much the commonest, a diagnosis of exclusion; RAMSAY hunt syndrome (herpes zoster oticus, with vesicles in the concha and canal, severe pain and a worse prognosis); acute and chronic suppurative otitis media, and cholesteatoma, in which facial palsy is an indication for urgent surgery; malignant otitis externa; temporal bone fracture; iatrogenic injury at mastoid or parotid surgery; parotid malignancy, in which facial palsy strongly suggests malignancy rather than a benign tumour; and acoustic neuroma
• Temporal bone fractures — a longitudinal fracture (80%) more often causes conductive loss with delayed, incomplete palsy which recovers; a transverse fracture causes sensorineural loss and immediate, complete palsy, implying transection, which warrants exploration
• The timing distinction is what matters: immediate and complete palsy suggests transection and needs surgical consideration; delayed or incomplete palsy suggests oedema and is managed conservatively
• Grading is by the house-brackmann scale from I (normal) to VI (total paralysis), and should be recorded at every visit so that progress can be judged
• The eye IS the urgent responsibility in any facial palsy: lubricants, ointment and taping at night, and tarsorrhaphy where the cornea is threatened — particularly if corneal sensation is also impaired
• The labyrinthine segment is the narrowest, which is why oedema compresses the nerve there and why decompression targets it
• The tympanic segment is dehiscent in ten to fifteen per cent, and is the segment most often injured in middle ear surgery
• Acoustic neuroma spares the facial nerve until late despite stretching it, which is a diagnostic peculiarity worth knowing

Predisposing Factors and Classification

• Otitis externa is inflammation of the skin of the external auditory canal, with or without involvement of the pinna and tympanic membrane.
• It is among the commonest conditions in ENT practice and is essentially a dermatological disease occurring in an awkward place.
• The canal IS normally protected BY four mechanisms, and otitis externa follows when they are breached — which is why the predisposing factors matter more than the organism:

Diffuse Otitis Externa

• Diffuse otitis externa is generalised inflammation of the canal skin, and is the commonest form
• Organisms — pseudomonas aeruginosa is the commonest, followed by Staphylococcus aureus, proteus and other Gram-negative organisms; and often a mixed growth. Fungi in 10%
• Symptoms — pain, which is severe and out of proportion to the visible signs because the skin is tightly bound to periosteum in the bony canal with no room to swell; itching, which frequently precedes the pain and leads to the scratching that started it; discharge, scanty and watery at first; a blocked ear with conductive deafness if the canal occludes; and tenderness on chewing
• Signs — tragal tenderness and pain on moving the pinna, which are the cardinal signs and are absent in otitis media — this single distinction settles the diagnosis at the bedside; a red, oedematous, narrowed canal filled with debris; regional lymphadenopathy; and, in severe cases, spread to the pinna and face with cellulitis
• The drum must BE seen, even if this requires review after treatment, because otitis externa may be secondary to a discharging middle ear and the management is entirely different
• Treatment — the principles are clean, medicate, and treat the cause:
• Aural toilet is the single most important measure, and is frequently omitted. Debris must be removed under vision by suction or dry mopping.
• Topical therapy — antibiotic with steroid drops (ciprofloxacin, gentamicin, neomycin with hydrocortisone), or acidifying agents (2% acetic acid, aluminium acetate).
• A wick or ribbon gauze soaked in the medication should be inserted where the canal is too swollen for drops to enter, and changed daily. This is what converts a failing treatment into a working one
• Analgesia is essential and is under-prescribed; the pain is severe and patients frequently need more than paracetamol
• Systemic antibiotics are not routinely required, and are reserved for spreading cellulitis, fever, lymphadenopathy, diabetes or immunosuppression
• Keep the ear dry — no swimming, cotton wool with petroleum jelly while bathing, and nothing to be put into the ear
• Treat the underlying dermatosis, and stop any drop causing contact sensitivity.
• Tragal tenderness settles the diagnosis at the bedside, being present in otitis externa and absent in otitis media
• Pain is out of proportion to the signs because the skin is bound to periosteum in the bony canal and cannot swell
• Self-cleaning is the commonest precipitant, and removing the wax removes the protection that prevented the infection
• Pseudomonas is the commonest organism, followed by staphylococcus, and mixed growths are frequent
• Acidifying drops restore the natural pH, are cheap and effective, and do not select for resistance
• Insert a wick when the canal is too swollen for drops to enter, and change it daily until the swelling subsides
• Never syringe an inflamed canal; it adds the moisture and trauma that caused the condition in the first place
• Prescribe adequate analgesia; the pain is severe, is routinely underestimated and frequently needs more than paracetamol
• Systemic antibiotics are not routinely needed, and are reserved for cellulitis, fever, diabetes or immunosuppression
• See the drum before you finish, since a discharging middle ear may be the cause and needs entirely different treatment
• Suspect contact allergy if it worsens on treatment, and neomycin in ear drops is the commonest culprit
• Look for an underlying dermatosis; eczema, seborrhoeic dermatitis and psoriasis all present in the canal and need treating

Nature and Presentation

• Malignant (necrotising) otitis externa is a progressive osteomyelitis of the temporal bone and skull base, beginning as an otitis externa.
• It is not a malignancy — the name reflects its relentless course and former high mortality.
• IT IS A disease OF the elderly diabetic, and this is the single most important association: the great majority occur in diabetics over 60, the remainder in the immunosuppressed — HIV, chemotherapy, transplantation and long-term steroid

Investigation and Management

• Diagnosis rests on the clinical picture in a diabetic, supported by imaging and inflammatory markers:
• Biopsy of the granulation tissue is mandatory — not to prove the diagnosis but to exclude carcinoma of the external canal.
• Microbiology — deep swab or tissue for culture and sensitivity, including fungal culture, ideally before antibiotics are started
• Blood — ESR is characteristically very high and is the best marker for monitoring the response; CRP; blood glucose and HbA1c; and a search for immunosuppression where the patient is not diabetic
• CT of the temporal bone shows bony erosion and the extent of disease, but requires about a third of the bone to be lost before it is visible, so it is insensitive early and changes persist long after cure, making it useless for follow-up
• MRI is superior for soft tissue and intracranial extension and for marrow involvement
• Technetium-99m bone scan is highly sensitive and positive early, but is non-specific and remains positive after cure
• Gallium-67 scan reflects active inflammation and normalises with resolution — so it is the investigation used to decide when treatment may be stopped.
• Treatment:
• Prolonged systemic antipseudomonal antibiotics are the mainstay — ciprofloxacin, oral or intravenous, is the standard, with ceftazidime, piperacillin-tazobactam or meropenem, often with an aminoglycoside. Treatment must continue for 6 to 8 weeks at least, and sometimes for months
• The commonest management error is stopping too early, when the pain settles; relapse then occurs and is harder to treat. Treatment is guided by symptoms, ESR and gallium scanning, not by the CT appearance
• Strict glycaemic control is essential and is as important as the antibiotic; these patients need insulin and admission initially
• Local measures — regular aural toilet, removal of granulations, and topical antipseudomonal drops
• Surgery has a limited role — it is not curative, since the disease is an osteomyelitis rather than a collection. It is used for biopsy, debridement of sequestra and drainage of abscess
• Hyperbaric oxygen is used as an adjunct in refractory disease, with limited evidence
• Follow up long term, since relapse may occur months later
• It is not a malignancy; the name reflects the relentless course and the former high mortality of the condition
• Suspect it in any elderly diabetic whose otitis externa has not settled after two weeks of appropriate topical treatment
• Pain worse at night out of proportion to signs is the cardinal symptom, and is what should trigger the suspicion
• Look for granulations on the canal floor at the bony-cartilaginous junction; they must be sought specifically
• Biopsy the granulations to exclude carcinoma, which presents identically and is the error that must not be made
• Pseudomonas causes over ninety per cent, and aspergillus is the important alternative in the severely immunosuppressed
• Spread occurs through the fissures of Santorini in the cartilaginous canal floor and along the skull base
• Facial palsy is the first cranial nerve affected and is a grave sign indicating skull base involvement
• Nerves nine, ten and eleven go together at the jugular foramen, giving dysphagia, hoarseness and shoulder weakness
• The drum and hearing are often normal, which falsely reassures the unwary examiner
• ESR is very high and is the monitoring marker, whereas CT changes persist long after cure and cannot be used
• CT needs a third of the bone lost before erosion is visible, so it is insensitive early in the disease

Cerumen and its Removal

• Cerumen (wax) is the normal secretion of the ceruminous and sebaceous glands of the cartilaginous canal, mixed with desquamated epithelium.
• It is protective, not dirt, being acidic, water-repellent and rich in lysozyme and immunoglobulins.
• Two genetically determined types — wet (sticky, brown), dominant and usual in Europeans, Africans and most Indians; and dry (flaky, grey), recessive and usual in East Asians. The difference lies in a single gene affecting apocrine secretion

Foreign Bodies in the Ear

• Foreign bodies are commonest in children, and are self-inserted and asymptomatic, discovered incidentally or after a clumsy removal attempt. In adults, cotton wool, insects and broken cotton buds predominate
• The classification governs the management:
• Non-living, non-vegetable (inorganic) — beads, pebbles, plastic toys, buttons. They are inert, may be left safely until proper facilities are available, and syringing is permissible
• Non-living, vegetable (organic) — peas, beans, seeds, grain. These swell with water and become impacted, so syringing IS absolutely contraindicated and they must be removed instrumentally
• Living (insects) — which cause intense distress, noise and pain from movement against the drum. The insect must be killed first, by instilling oil, spirit or lignocaine, before any attempt at removal
• Button (disc) batteries are a special emergency — they leak alkali and cause liquefactive necrosis within hours, destroying the canal skin, drum and ossicles. They require immediate removal without irrigation
• Principles OF removal, and they are mostly about restraint:
• The first attempt IS the best attempt. Repeated blind attempts push the object past the isthmus, cause bleeding and oedema, and turn a simple problem into a general anaesthetic
• Remove under direct vision, with adequate light, magnification and appropriate instruments — never blindly with forceps
• Do not grasp a smooth, round object with forceps.
• Syringing is suitable for small inert objects but not for vegetable matter, batteries or a suspected perforation
• General anaesthesia is indicated for an uncooperative child, a deeply impacted object, a failed first attempt, or where the object is against the drum — and choosing it early is a sign of good judgement, not of failure
• Examine the other ear and the nose, since children frequently insert more than one
• Always examine the drum after removal and document its state
• Wax is protective, not dirt, and explaining this is what prevents the patient returning with the same problem
• Impaction means the self-cleaning mechanism has failed, and cotton buds pushing wax inward are much the commonest reason
• Soften for several days before removal; it eases every method and converts a difficult procedure into a simple one
• Syringe at body temperature; water too hot or too cold causes caloric vertigo that is unpleasant and entirely avoidable
• Direct the stream posterosuperiorly so that it passes behind the wax and floats it outward rather than compacting it
• Never syringe a perforated or previously operated ear, nor an only hearing ear, nor where vegetable matter is present
• Microsuction is safer and is now preferred, being done under direct vision and without wetting the ear
• Syringing generates disproportionate medicolegal claims, so consent and documentation of the drum afterwards both matter
• Sudden deafness after bathing is the classic history of wax swelling and occluding a canal that was already narrowed
• Wax rarely causes more than thirty to forty decibels of loss, so a greater loss needs explaining after the wax is removed
• Kill an insect before attempting removal with oil, spirit or lignocaine; a moving insect against the drum is intolerable
• A button battery is an emergency, leaking alkali and causing liquefactive necrosis within hours
• Never irrigate a button battery; it accelerates the leak and spreads the alkali along the canal
• Pass a hook beyond a round object rather than grasping it with forceps.

Benign Tumours and Tumour-like Lesions

• Tumours of the external ear are dominated by skin cancers of the pinna.

Malignant Tumours

• Malignancy OF the pinna — squamous cell carcinoma is the commonest on the pinna, unlike the face where basal cell carcinoma predominates, and arises on the helix in elderly men with chronic sun exposure. It metastasises to the parotid and cervical nodes
• Basal cell carcinoma occurs more often on the postauricular and preauricular skin, and is locally invasive without metastasis
• Melanoma is uncommon but carries a worse prognosis on the ear than elsewhere
• Predisposing factors — chronic sun exposure, fair skin, actinic keratosis, chronic irritation, previous radiotherapy and immunosuppression
• Malignancy OF the external canal is rare, and its importance lies entirely in the delay in diagnosis. It presents with pain, blood-stained discharge, granulations and a canal that fails to respond to treatment — that is, it is indistinguishable from chronic otitis externa or malignant otitis externa
• The rule that follows IS the most important IN this answer: biopsy any granulation tissue or non-healing lesion in the canal, and biopsy any "otitis externa" that has not responded to adequate treatment. Delay is the reason these tumours present late and do badly
• A long history of chronic suppurative otitis media is a recognised risk factor for carcinoma of the middle ear and canal, so a change in the character of the discharge to blood-stained, or the onset of pain in a previously painless discharging ear, demands biopsy
• Spread is early and difficult to assess, into the parotid, temporomandibular joint, mastoid, facial nerve and skull base; facial palsy indicates advanced disease
• Investigation — biopsy; high-resolution CT for bone and MRI for soft tissue and dural involvement; and assessment of the neck and parotid
• Management — surgical excision with clear margins is the mainstay: wedge excision or partial pinnectomy for small pinna lesions, with reconstruction; and, for canal tumours, lateral or subtotal temporal bone resection with parotidectomy and neck dissection as indicated, followed by postoperative radiotherapy
• Prognosis is good for small pinna lesions and poor for canal and temporal bone tumours, chiefly because of late presentation and the difficulty of clearing margins in that anatomy
• Squamous carcinoma predominates on the pinna, unlike the face where basal cell carcinoma is commoner
• Biopsy any granulation in the canal; carcinoma is indistinguishable from chronic otitis externa without histology
• Blood-stained discharge in a chronic ear demands biopsy, as does the onset of pain in a previously painless discharging ear

Injuries of the Pinna

• The pinna is exposed and projecting, so it is injured readily; its rich blood supply from both the posterior auricular and superficial temporal arteries means it heals remarkably well and needs minimal debridement
• Haematoma auris is the injury that must be understood. Blunt trauma — classically in wrestling, BOXING and rugby — shears the perichondrium off the cartilage, and blood collects in the subperichondrial plane
• The danger follows from a fact of anatomy: the auricular cartilage has NO blood supply of its own and is nourished entirely by the overlying perichondrium. A haematoma therefore separates the cartilage from its only source of nutrition

Injuries of the Canal and Drum

• Canal injuries arise from cotton buds, hairpins and instrumentation, from syringing, from a blow to the jaw driving the condyle backward through the anterior canal wall, and from temporal bone fracture
• Bleeding from the canal after head injury raises three possibilities — a simple canal laceration, a ruptured tympanic membrane, or a temporal bone fracture; and CSF otorrhoea must be excluded, which is suggested by clear fluid, a halo sign on tissue, and confirmed by beta-2 transferrin assay
• Traumatic perforation of the tympanic membrane follows a slap or blow to the ear, which is much the commonest cause in India and frequently follows assault; a blast; diving or a fall onto water; instrumentation; and lightning strike
• Features — sudden pain, bleeding, conductive deafness and tinnitus; the perforation is characteristically irregular with everted, blood-stained margins, which distinguishes it from a chronic perforation
• Vertigo or sensorineural loss suggests inner ear injury — a perilymph fistula or ossicular dislocation — and is a warning sign requiring specialist assessment rather than reassurance
• Management of a traumatic perforation is conservative, and this is the key point: the great majority — around 80 to 90% — heal spontaneously within 6 to 8 weeks
• The essential instruction is to keep the ear dry — no swimming, no drops, no syringing, cotton wool and petroleum jelly while bathing. Topical drops and syringing are contraindicated, since they introduce infection into the middle ear and convert a healing perforation into chronic suppuration
• Do not prescribe antibiotic drops routinely; oral antibiotics are given only if the ear was contaminated (as after a dirty injury or water entry)
• Review at 6 to 8 weeks with audiometry; myringoplasty is offered only if the perforation persists beyond 3 months or the hearing loss is disproportionate.
• Barotrauma and blast injury may also cause haemotympanum, ossicular disruption and sensorineural loss
• Document carefully in every traumatic perforation — the mechanism in the patient’s words, the appearance and the audiogram — since these frequently follow assault and generate legal proceedings
• Cartilage is fed only by perichondrium, which is the whole reason a haematoma of the pinna is an urgent problem
• Evacuation is the easy half of the treatment; preventing re-accumulation is what determines whether the ear deforms
• Use a moulded pressure dressing or bolster sutures and review daily, since re-accumulation is common and silent

Aetiology and Pathology

• It is predominantly a disease of children, with a peak between 6 and 24 months and a second peak at school entry. The great majority of children have at least one episode
• The underlying event IS eustachian tube dysfunction, and the infant tube explains the age distribution: it is shorter, wider and more horizontal than the adult tube, so nasopharyngeal organisms and secretions reflux readily into the middle ear

Management

• Diagnosis is clinical, and the essentials are a red, bulging, immobile drum with acute onset of pain and fever. Redness alone is not enough — a crying child, fever or recent instrumentation reddens a normal drum.
• Pneumatic otoscopy showing reduced mobility is what confirms it, and is the most useful bedside addition
• In an infant, the signs are non-specific — irritability, ear-tugging, fever, vomiting, diarrhoea and refusal of feeds — so the ears must be examined in every febrile infant
• Treatment of the acute episode:
• Analgesia and antipyretics are the first and most important measure — paracetamol or ibuprofen — and are frequently neglected in favour of the antibiotic, although the pain is what distresses the child
• Antibiotics — amoxicillin is first line, in high dose (80 to 90 mg/kg/day) for 5 to 10 days; amoxicillin-clavulanate where there is recent antibiotic use, failure at 48 to 72 hours, or associated conjunctivitis (suggesting Haemophilus); and a macrolide or cephalosporin in penicillin allergy
• "watchful waiting" for 48 to 72 hours with analgesia alone is appropriate in a child over 2 with mild unilateral disease who is not systemically unwell, since most episodes are viral or self-limiting. Antibiotics are given without delay in the child under 6 months, in bilateral disease under 2, where there is otorrhoea, in severe illness, and where follow-up is uncertain — which in Indian practice is frequently the case
• Nasal decongestants and antihistamines are of NO proven benefit and are widely over-prescribed
• Myringotomy is indicated for a bulging drum with severe unrelieved pain, failure to respond after 48 to 72 hours, a complication such as facial palsy or incipient mastoiditis, and in a very young or immunocompromised child where pus is needed for culture. It is performed in the anteroinferior quadrant
• Aural toilet by dry mopping where there is discharge, and keeping the ear dry
• Follow UP until the drum and hearing are normal.
• Sequelae — persistent effusion; persistent perforation; adhesive otitis media and tympanosclerosis; ossicular necrosis; recurrent ASOM; and progression to chronic suppurative otitis media
• Prevention — breastfeeding; avoiding bottle-feeding lying flat; avoiding passive smoking; treatment of nasal and adenoid disease; and pneumococcal conjugate and influenza vaccination.
• The infant tube explains the age distribution, being shorter, wider and more horizontal so that nasopharyngeal contents reflux
• Pain and fever settle the moment the drum ruptures, which is the classic history and makes the diagnosis retrospectively
• Redness alone is not otitis media; a crying child or recent instrumentation reddens a normal drum
• Reduced mobility on pneumatic otoscopy confirms it, and is the most useful bedside addition to plain inspection
• Examine the ears of every febrile infant; the signs are non-specific and ear-tugging may be the only clue

Nature and Aetiology

• Otitis media with effusion (OME, "glue ear", serous or secretory otitis media) is the presence of fluid in the middle ear without signs of acute infection, behind an intact tympanic membrane.
• It is the commonest cause of acquired hearing loss in children.
• The peak incidence is at 2 and again at 5 years, and it is characteristically seasonal, being commoner in winter

Management

• The foundation OF management IS that most resolve spontaneously: 50% within 3 months and 90% within a year. This is why watchful waiting for 3 months with two audiological assessments is the correct first step, and why early surgery is inappropriate
• What does not work, and should not be prescribed — antibiotics (no lasting benefit); antihistamines and decongestants (no benefit, with side effects); mucolytics; and topical or systemic steroids, whose effect is transient. These are among the most over-prescribed drugs in paediatric practice
• Autoinflation (Otovent balloon, Valsalva) has modest evidence and is harmless, and is worth teaching to a cooperative child
• Treat the associated conditions — allergic rhinitis, sinusitis and adenoid disease; and eliminate passive smoking
• Indications for surgery — persistence beyond 3 months bilaterally with a hearing loss of 25 to 30 dB or worse in the better ear; speech, language or developmental delay; significant behavioural or educational problems; structural change in the drum — a retraction pocket, atelectasis or ossicular erosion.
• The threshold is lower in a child with down syndrome, cleft palate, visual impairment, existing developmental delay or autism, in whom the hearing loss compounds an existing disadvantage
• Grommet (ventilation tube) insertion is the standard operation, restoring hearing immediately
• Adenoidectomy should be added where there is nasal obstruction or recurrent disease, and particularly at a second set of grommets, since it reduces the recurrence rate independently of adenoid size — which is a point regularly examined and reflects its role as a reservoir of infection rather than a mechanical obstruction alone
• Hearing AIDS are a legitimate alternative to grommets, particularly where surgery is refused, anaesthesia is risky, or in a child with cleft palate or Down syndrome in whom grommets do less well
• Alongside any treatment — inform the school, seat the child at the front, speak face to face, and reduce background noise. These simple measures address the actual disability while the effusion is being managed
• It is painless, which is why it presents late and is noticed first by a teacher rather than a doctor
• Half resolve in three months and ninety per cent in a year.
• Antibiotics, antihistamines and decongestants do not work, and prescribing them delays proper assessment
• Bacterial biofilms explain antibiotic failure, and are found in a substantial proportion of effusions
• A flat type B trace with normal canal volume means effusion, whereas a large volume means a perforation or patent grommet
• Air bubbles or a fluid level are diagnostic, and are worth looking for specifically before relying on colour alone
• Test hearing twice over three months before offering surgery, since the effusion and the loss both fluctuate
• Adenoidectomy reduces recurrence independently of adenoid size, acting as a reservoir of infection rather than a mechanical block
• Add adenoidectomy at a second set of grommets, which is where the evidence for it is strongest

Nature and Pathology

• Chronic suppurative otitis media (CSOM) is a long-standing infection of the middle ear cleft with a permanent perforation of the tympanic membrane.
• The tubotympanic (mucosal, "safe") type involves the anteroinferior part of the cleft and the Eustachian tube, and does not produce cholesteatoma.
• CSOM remains a major cause of preventable hearing loss and of death from intracranial sepsis in India, and is far commoner here than in high-income countries because of overcrowding, poor nutrition, untreated upper respiratory infection and limited access to care

Clinical Features and Management

• The two cardinal symptoms are discharge and deafness:
• Discharge (otorrhoea) that is profuse, mucoid or mucopurulent, odourless and non-blood-stained, and characteristically intermittent — worsening with upper respiratory infection or water entry and settling between. The mucoid character is the point: mucus can only come from middle ear mucosa, so it indicates mucosal rather than bony disease
• Deafness — conductive, mild to moderate (20 to 40 dB), and depending on the size and site of the perforation and the state of the ossicles. A loss exceeding 40 to 50 dB suggests ossicular involvement
• Paradoxically, some patients hear better when the ear is discharging — the "round window shielding" effect, in which fluid bridges an ossicular gap and restores some conduction. Its presence suggests ossicular discontinuity
• Pain, vertigo, facial weakness or headache are not features of safe disease, and their appearance means either an acute exacerbation, otitis externa, or a complication — and should always prompt reassessment
• Examination — a central perforation with pale, healthy or oedematous middle ear mucosa visible through it; mucoid discharge; and NO attic involvement, NO granulations, NO cholesteatoma. The attic must be inspected specifically.
• Tuning fork tests and pure tone audiometry confirm a conductive loss; a sensorineural component may develop with long-standing disease from toxin diffusion through the round window
• Swab for culture and sensitivity; and examine the nose, throat and postnasal space.
• Medical treatment — the aim is to obtain a dry ear:
• Aural toilet is the single most important measure, by dry mopping or suction under vision, repeated as often as necessary. Drops cannot work in an ear full of discharge
• Topical antibiotic drops — ciprofloxacin or ofloxacin are preferred because they are not ototoxic. Aminoglycoside drops (neomycin, gentamicin) should be avoided or used only briefly in the presence of a perforation.
• Systemic antibiotics for acute exacerbations, guided by culture
• Keep the ear dry — no swimming, cotton wool and petroleum jelly while bathing. This instruction does more than any drop
• Treat the nose and throat — allergic rhinitis, sinusitis, adenoids and tonsils.
• Surgical treatment — myringoplasty or tympanoplasty once the ear has been dry for at least 6 weeks to 3 months, to close the perforation, restore hearing and prevent reinfection; with ossicular reconstruction where needed. Cortical mastoidectomy is added where there is persistent discharge from mastoid disease
• Safe does not mean harmless; tubotympanic disease still causes significant deafness and can complicate during an exacerbation
• Mucus can only come from middle ear mucosa, so mucoid discharge indicates mucosal rather than bony disease
• A central perforation has a rim all round, so the annulus is intact, which is the definition rather than a description of position
• Pain, vertigo or facial weakness are not features of safe disease, and their appearance demands reassessment
• Better hearing when discharging suggests ossicular discontinuity, the fluid bridging the gap and restoring some conduction
• A loss beyond forty to fifty decibels suggests ossicular damage rather than the perforation alone
• Use ciprofloxacin or ofloxacin drops, which are not ototoxic, rather than aminoglycosides through a perforation
• Aminoglycoside drops risk sensorineural loss when the drum is perforated, and should be avoided or used only briefly
• Aural toilet does more than any drop, and must be repeated as often as the discharge recurs

Nature and Pathogenesis

• Cholesteatoma is the presence of keratinising stratified squamous epithelium in the middle ear cleft, with progressive accumulation of keratin and destruction of surrounding bone.
• The atticoantral (squamosal, "unsafe") type of chronic otitis media involves the attic, antrum and posterosuperior region.
• The name IS A triple misnomer, and saying so shows understanding: it contains NO cholesterol, it is not a fatty tumour, and it is not a neoplasm. It is skin in the wrong place — and Gray’s description of it as "skin in the wrong place" conveys its nature exactly

Clinical Features and Management

• The clinical picture IS the mirror image OF safe disease, and the contrast IS the answer:
• Discharge that is scanty, purulent and foul-smelling — the odour comes from bone destruction and anaerobes, and is what should raise the alarm. It is often continuous rather than intermittent
• Deafness, which may be severe; though hearing may be deceptively good where the cholesteatoma itself bridges the ossicular gap and conducts sound — so good hearing does not exclude extensive disease
• An attic or marginal perforation, or a retraction pocket, with white keratin flakes visible within it
• Granulation tissue or an aural polyp.
• The alarm symptoms, each OF which means A complication: pain (which safe disease does not cause); vertigo or a positive fistula test (labyrinthine fistula); facial weakness; severe headache, fever, vomiting or altered consciousness (intracranial); and bleeding
• Investigation — pure tone audiometry; high-resolution CT of the temporal bone.
• The management IS surgical.
• The aims of surgery, in strict order of priority: 1. To eradicate the disease and give a safe ear; 2. To achieve a dry ear; and 3. To preserve or restore hearing — which is always subordinate to safety
• Canal wall down (modified radical) mastoidectomy removes the posterior canal wall, exteriorising the disease into a single open cavity. It gives the lowest recurrence rate and the best exposure, but leaves a cavity requiring lifelong cleaning and permanent water precautions
• Canal wall UP (intact canal wall) mastoidectomy preserves the canal wall, giving a normal ear anatomically with better hearing potential and no cavity — but a higher rate of residual and recurrent disease, so it commits the patient to a second-look procedure or diffusion-weighted MRI surveillance
• The choice depends on the extent of disease, the state of the other ear, the reliability of follow-up, the presence of a fistula or complication, and the surgeon’s experience. Where follow-up is uncertain, canal wall down is safer — a genuine consideration in much of India
• Radical mastoidectomy, which exteriorises the middle ear entirely and sacrifices hearing, is now reserved for extensive disease or malignancy
• All patients need long-term follow-up, for residual disease, recurrence and cavity problems
• It is not cholesterol, not fat and not a tumour; it is skin in the wrong place, and saying so shows understanding
• Bone destruction is enzymatic, not merely pressure, with collagenase and osteoclast activation from the perimatrix
• Infection accelerates bone erosion markedly, which is why a discharging cholesteatoma advances faster than a dry one
• The pars flaccida gives way first because it lacks the fibrous middle layer.

Principles and Reconstructive Surgery

• Surgery for chronic otitis media has two distinct purposes, and every operation is a combination of them: eradication of disease, and reconstruction of the sound-conducting mechanism. Eradication always takes precedence
• Myringoplasty is repair of the tympanic membrane alone, with a normal ossicular chain. Tympanoplasty is repair of the drum with reconstruction of the ossicular chain
• Indications for myringoplasty — a dry central perforation to prevent recurrent infection, permit water exposure, improve hearing, and as a preliminary to fitting a hearing aid

Mastoid Surgery

• The choice between canal wall up and down depends on the extent of disease, the state of the only or better hearing ear, the presence of a labyrinthine fistula or complication, the degree of mastoid pneumatisation, the reliability of follow-UP, and the surgeon’s experience
• Complications of mastoid surgery.
• Postoperative care — packing removal, aural toilet, water precautions, and audiometry at 3 months
• Follow up for life after cholesteatoma surgery; this is not a condition that can be discharged
• Eradication always takes precedence over reconstruction, and a hearing result is never bought at the cost of leaving disease
• Myringoplasty repairs the drum and tympanoplasty the chain as well.
• Operate only on a dry ear; grafting a wet ear fails and wastes both the anaesthetic and the opportunity
• Assess Eustachian function before grafting; without ventilation the graft retracts and the operation fails slowly
• Check the cochlear reserve; there is no point restoring conduction to an ear whose cochlea no longer works
• Consider the opposite ear before operating, since the risk that is acceptable depends entirely on what the patient has to lose
• Temporalis fascia is the standard graft, being available through the same incision and of similar thickness to the drum
• Cartilage resists retraction better, and is preferred in revision surgery, poor tube function, atelectasis and subtotal perforations

Classification

• A complication of suppurative otitis media is spread of infection beyond the confines of the middle ear cleft and its pneumatised extensions.
• Complications are classified as intratemporal (extracranial) and intracranial.
• Intratemporal (extracranial) complications — acute mastoiditis and its abscesses (postauricular, BEZOLD, Citelli, Luc, zygomatic); petrositis; facial nerve palsy; labyrinthitis and labyrinthine fistula; and masked mastoiditis

Predisposing Factors and Pathways of Spread

• Predisposing factors — cholesteatoma, which is much the most important because it erodes bone; a virulent organism; inadequate or partial antibiotic treatment, which suppresses signs while disease advances; poor host resistance — malnutrition, diabetes, immunosuppression, extremes of age; obstruction to drainage, such as a blocked aditus or a perforation that closes over; a well-pneumatised mastoid; and previous surgery or trauma creating a defect

Recognising a Complication

• The single most useful principle IS that A chronically discharging ear does not hurt. Uncomplicated chronic suppurative otitis media is painless. So the appearance of pain in a chronically discharging ear is a warning sign and must never be dismissed as an exacerbation
• The alarm features, each of which points to a specific complication:
• Pain, especially deep or nocturnal headache — extradural abscess, brain abscess, petrositis
• Fever with rigors in a discharging ear — lateral sinus thrombophlebitis until proved otherwise
• Vertigo, or a positive fistula sign — labyrinthine fistula or labyrinthitis
• Facial weakness — facial canal erosion; an indication for urgent surgery
• Sudden profuse discharge followed by relief — drainage of a collection into the ear
• Persistent headache, vomiting, drowsiness or altered behaviour — raised intracranial pressure; brain abscess
• Neck stiffness and photophobia — meningitis
• Diplopia — a sixth nerve palsy, from petrositis (Gradenigo) or raised intracranial pressure
• Blurred vision with papilloedema and few other signs — otitic hydrocephalus
• Seizures, dysphasia or hemiparesis — temporal lobe abscess
• Ataxia, nystagmus, past-pointing or dysdiadochokinesia — cerebellar abscess
• A patient who is simply "not right" or unusually drowsy — which in practice is often the earliest change noticed by the family
• Examination must therefore extend beyond the ear — temperature chart; conscious level; fundoscopy for papilloedema; all cranial nerves; meningism; cerebellar signs; and the neck for tenderness along the jugular vein
• Principles OF management, which apply to all of them:
• Admit, and treat as an emergency
• High-dose intravenous antibiotics that cross the blood-brain barrier — a third-generation cephalosporin with metronidazole for anaerobic cover, adjusted by culture
• Urgent imaging — contrast-enhanced CT of the brain and high-resolution CT of the temporal bone, with MRI where available.
• Neurosurgical involvement where there is an intracranial collection

Otogenic Meningitis

• Otogenic meningitis is inflammation of the leptomeninges secondary to middle ear or mastoid infection.
• It is the commonest intracranial complication of otitis media, and is the commonest cause of death from ear disease in children.
• Routes — in acute otitis media, chiefly through preformed pathways (the oval and round windows, the cochlear aqueduct, congenital dehiscences and the petrosquamous suture) and by haematogenous spread; in chronic disease, by bone erosion from cholesteatoma, by extension from an extradural or brain abscess, or through a labyrinthine fistula

Extradural and Subdural Abscess

• Extradural (epidural) abscess is a collection of pus between the bone and the dura, and is the commonest intracranial complication after meningitis
• It arises by direct bone erosion, of the tegmen (giving a middle fossa collection) or of the sinus plate (a perisinus abscess)
• The difficulty IS that IT IS frequently silent, and is most often discovered incidentally at mastoid surgery when granulation tissue and pus are found on exposed dura
• When it does declare itself — persistent deep-seated headache on the affected side; low-grade fever; malaise; and, characteristically, profuse pulsatile discharge from the ear that relieves the headache as the abscess drains
• Investigation — contrast CT or MRI, showing a lentiform collection with dural enhancement, and bony erosion on temporal bone CT
• Treatment — intravenous antibiotics and surgical drainage.
• Subdural abscess (empyema) lies between the dura and the arachnoid, and is far more dangerous because there is no anatomical barrier to its spread over the cerebral convexity
• It is rarer but has a much higher mortality, and is more often sinogenic than otogenic
• Features — rapid deterioration, with high fever, severe headache, meningism, seizures (which are common and often focal), rapidly progressive focal neurological deficit and falling conscious level — the combination of meningeal and focal signs progressing quickly is characteristic
• Investigation — urgent contrast CT or, preferably, MRI.
• Treatment — urgent neurosurgical drainage by burr holes or craniotomy, high-dose intravenous antibiotics, anticonvulsants, and management of raised intracranial pressure; with mastoid surgery to the source once stabilised
• Scan before you tap; a coexisting abscess or raised pressure turns a lumbar puncture into coning
• Neck stiffness is absent in infants, who show a bulging fontanelle, high-pitched cry and refusal of feeds instead
• Give dexamethasone with or before the first antibiotic in pneumococcal meningitis, which reduces deafness and neurological sequelae
• Test hearing early after meningitis; labyrinthine ossification begins within weeks and can make implantation impossible
• Recurrent meningitis means a persistent defect — a CSF fistula, a Mondini malformation or an old temporal bone fracture
• Low CSF glucose distinguishes bacterial disease, below about forty per cent of the blood level, with raised polymorphs and protein
• Operate on the ear once the patient is stable; leaving the source means the meningitis returns
• Chronic disease grows Gram-negatives and anaerobes, quite unlike the pneumococcus and haemophilus of acute otitis media
• Extradural abscess is often silent, and is most commonly discovered incidentally during mastoid surgery
• Pulsatile discharge that relieves headache suggests an extradural abscess draining into the ear
• Expose healthy dura on all sides when draining an extradural abscess, and do not incise the dura itself
• Subdural empyema spreads unchecked over the convexity, having no anatomical barrier.
• Meningeal plus rapidly progressive focal signs suggest subdural empyema rather than meningitis alone
• MRI beats CT for a thin subdural collection, which plain CT may miss entirely at the convexity
• Subdural empyema is more often sinogenic than otogenic, so the paranasal sinuses must be assessed as well
• Start antibiotics before the culture returns; delay while awaiting results costs lives in meningitis
• Projectile vomiting without nausea indicates raised intracranial pressure rather than a gastrointestinal cause
• Seizures in subdural empyema are common and focal, and anticonvulsant cover is given prophylactically

Nature and Pathology

• An otogenic brain abscess is a focal collection of pus within the brain substance arising from middle ear or mastoid infection.
• It carries the highest mortality of all otitic complications, and remains a significant cause of death in young people in India.
• The two sites are the temporal lobe and the cerebellum.

Investigation and Management

• Contrast-enhanced CT of the brain is the standard investigation, showing a hypodense centre with ring enhancement and surrounding oedema, with mass effect and midline shift
• MRI is superior, particularly for early cerebritis and posterior fossa lesions.
• High-resolution CT of the temporal bone defines the ear disease and the bony defect
• Lumbar puncture IS absolutely contraindicated — it risks coning and death, and it will not establish the diagnosis
• Blood cultures, inflammatory markers, and culture of the aspirated pus including anaerobic culture
• Management is a neurosurgical and otological emergency, and it is joint:
• High-dose intravenous antibiotics crossing the blood-brain barrier — a third-generation cephalosporin with metronidazole, with vancomycin where staphylococcus is likely — continued for 6 to 8 weeks
• Control of raised intracranial pressure with mannitol and dexamethasone, and anticonvulsants
• Neurosurgical drainage — repeated aspiration through a burr hole, which is preferred; or excision of the capsule for a multiloculated, thick-walled or recurrent abscess
• Mastoid surgery to eradicate the ear disease, which is essential: a brain abscess whose source is left will recur. The timing is decided jointly — the abscess is addressed first, and the ear as soon as the patient is stable, sometimes at the same sitting
• Prognosis — mortality has fallen greatly with imaging and antibiotics but remains significant, and depends chiefly on the conscious level at presentation. Rupture into the ventricle is fatal
• Survivors may have persistent epilepsy and neurological deficit, and need long-term follow-up
• The latent stage is why the diagnosis is missed; the patient seems well for days or weeks between cerebritis and manifest disease
• Absence of fever does not exclude a brain abscess, and the temperature is frequently normal in the manifest stage
• Ask the patient to name a pen and a watch; nominal aphasia is an early sign of dominant temporal lobe abscess and costs nothing
• Temporal lobe abscess is twice as common as cerebellar, and follows erosion of the tegmen
• Cerebellar abscess often accompanies sinus thrombosis, both arising through the sinus plate and Trautmann triangle
• Falling toward the side of the lesion is characteristic of a cerebellar abscess, along with ipsilateral incoordination
• Metronidazole is essential, since otogenic abscesses are frequently anaerobic or mixed
• Never do a lumbar puncture; it risks coning and will not establish the diagnosis
• Diffusion-weighted MRI separates abscess from tumour, an abscess restricting diffusion where a cystic tumour does not
• MRI is superior in the posterior fossa, where bone artefact degrades the CT and a cerebellar abscess may be missed

Nature and Presentation

• Lateral (sigmoid) sinus thrombophlebitis is inflammation and thrombosis of the lateral venous sinus secondary to middle ear or mastoid infection.
• It is important because it is a treatable cause of a septicaemic illness that is readily attributed to something else.
• Pathogenesis proceeds in an orderly sequence, and knowing it explains every sign: infection erodes or spreads through the sinus plate → a perisinus abscess forms → the sinus wall becomes inflamed (phlebitis) → a mural thrombus forms on the intima → the thrombus propagates and occludes the lumen → it breaks down and becomes infected, releasing septic emboli into the circulation

Investigation and Treatment

• Blood culture taken during a rigor gives the highest yield.
• Full blood count showing polymorphonuclear leucocytosis and anaemia, which is characteristic of the prolonged septic course
• Contrast-enhanced CT shows the delta (empty triangle) sign — enhancement of the dural wall around a non-enhancing intraluminal clot — and shows the bone and any coexisting abscess
• MR venography (or CT venography) is the investigation of choice, demonstrating absent flow in the sinus and the extent of propagation into the jugular vein and other sinuses
• High-resolution CT of the temporal bone for the ear disease
• Treatment:
• High-dose intravenous antibiotics, broad-spectrum with anaerobic cover, continued for several weeks and guided by culture
• Surgery on the ear — mastoidectomy (canal wall down) to eradicate the disease, with exposure of the sinus plate and removal of the perisinus abscess and granulations until healthy sinus wall is seen on either side
• Whether to open the sinus and evacuate the clot is debated. The modern view is that the sinus need not be opened routinely, since antibiotics and drainage of the perisinus infection suffice; it is reserved for a patient who remains septic despite adequate treatment, when the thrombus is aspirated or the sinus incised and the infected clot removed
• Ligation of the internal jugular vein, once routine, is now rarely performed, and is reserved for continuing septic emboli despite treatment
• Anticoagulation remains controversial — it may limit propagation of thrombus and reduce embolisation, particularly with extension into the cavernous or sagittal sinus, but risks haemorrhage. It is used selectively, and the decision is individualised
• Supportive care — correction of anaemia and dehydration, and nutrition.
• Prognosis is good with prompt treatment; the sinus recanalises in many, and the collateral venous drainage is adequate even if it does not
• Look for coexisting complications — particularly cerebellar abscess and otitic hydrocephalus, both of which are associated with lateral sinus thrombosis and are easily overlooked once the first diagnosis is made
• Take the blood culture during the rigor; bacteraemia is intermittent and this single timing change alters the yield
• The picket-fence pattern matters more than the height of the fever, rising with a rigor and falling by crisis with sweating
• Griesinger sign is oedema behind the mastoid from emissary vein thrombosis, and should be looked for deliberately
• The delta sign on contrast CT shows an enhancing dural wall around a non-enhancing clot
• MR venography is the investigation of choice, showing absent flow and the extent of propagation into the jugular vein
• Antibiotics mask the classical picture, so a patient merely failing to improve deserves venography rather than reassurance
• Anaemia is characteristic of the prolonged septic course and should be corrected alongside the antibiotic
• The sinus need not be opened routinely; drainage of the perisinus infection with antibiotics suffices
• Jugular ligation is now rarely performed, and is reserved for continuing septic emboli despite adequate treatment
• Anticoagulation remains controversial and is used selectively, balancing propagation against haemorrhage
• Septic emboli lodge in the lungs first, causing abscesses and empyema, so a chest film is part of the assessment
• Palpate the neck for a tender cord along the internal jugular vein.

Labyrinthine Fistula

• A labyrinthine fistula is an abnormal communication between the middle ear and the labyrinth, created by erosion of the bony capsule.
• The lateral (horizontal) semicircular canal is by far the commonest site, because its prominence lies in the medial wall of the attic where cholesteatoma sits.
• Cause — cholesteatoma in the great majority; also chronic osteitis, trauma, previous surgery, tumour and syphilis

Labyrinthitis

• Suppurative labyrinthitis is a medical and surgical emergency, not because the ear can be saved — it cannot — but because the labyrinth is the gateway to the meninges through the internal acoustic meatus and the cochlear aqueduct. Labyrinthitis is therefore a warning of impending meningitis
• Management of suppurative labyrinthitis — admit; high-dose intravenous antibiotics crossing the blood-brain barrier; vestibular sedatives briefly and fluids for vomiting; urgent mastoid surgery to eradicate the source; and labyrinthectomy only where infection persists or to drain the labyrinth in the presence of meningitis
• Do not continue vestibular sedatives beyond a few days.
• Encourage early mobilisation and vestibular exercises.
• Test hearing early and consider cochlear implantation urgently where the loss is profound, before ossification
• The lateral semicircular canal is the commonest site, its prominence lying exactly where attic cholesteatoma sits
• Ask about vertigo on nose-blowing or straining; pressure-provoked vertigo is the symptom that suggests a fistula
• A negative fistula test may mean a dead labyrinth, which is not reassurance but the worst possible result
• Hennebert sign is a false positive, occurring in congenital syphilis and Meniere disease without any fistula
• Get a CT before operating on a suspected fistula, since the surgical plan depends on its size and site
• The matrix over a fistula may be left and exteriorised, particularly in an only hearing ear or where the fistula is large
• Remove the matrix last if at all, gently and under irrigation, and seal the fistula immediately with fascia or bone pate
• Nystagmus toward the diseased ear is irritative and suggests serous labyrinthitis that may still be reversible
• Nystagmus away from the diseased ear is paralytic and means the labyrinth has been destroyed
• Labyrinthitis is a warning of meningitis, the labyrinth being the gateway through the internal meatus and cochlear aqueduct
• Stop vestibular sedatives after a few days; they delay the central compensation that is the patient only route to recovery
• Reassure that vertigo settles even with a dead labyrinth.
• Encourage early mobilisation and exercises, which accelerate compensation and shorten the disability
• Ossification begins within weeks after suppurative labyrinthitis, which is why implantation cannot wait for convalescence
• Serous labyrinthitis is toxin diffusion through the round window without organisms entering the labyrinth itself

Types of Hearing Loss

• Hearing loss is classified by the site of the lesion into conductive, sensorineural, mixed, central and functional (non-organic).
• Establishing the type precedes establishing the cause, and it can be done with a tuning fork.
• Conductive loss arises anywhere from the pinna to the stapes footplate — the sound-conducting apparatus. The cochlea is normal, so bone conduction is normal and there is an air-bone gap

Causes

Clinical Approach

• The history should establish — which ear and whether both; the onset (sudden, rapid or gradual); the course (progressive, fluctuating or stable); and associated symptoms
• The pattern of onset is highly discriminating: sudden loss suggests vascular, viral, trauma or a perilymph fistula; fluctuating loss suggests meniere disease or a large vestibular aqueduct; slowly progressive suggests presbycusis, otosclerosis or a schwannoma
• Ask about discharge, pain, tinnitus, vertigo and facial weakness; about noise exposure at work and in recreation; about ototoxic drugs by name (aminoglycosides, furosemide, cisplatin, high-dose aspirin).
• Two questions are worth asking specifically: "Can you use the telephone on that side?", which uncovers a unilateral loss the patient has not noticed; and "Is it worse in a noisy room?", which suggests a cochlear or central problem rather than a conductive one
• Paracusis willisii — hearing better in noisy surroundings — is characteristic of otosclerosis and of conductive loss generally.
• Examination — otoscopy after clearing the canal; tuning fork tests; the facial nerve; the nose, postnasal space and throat; and cranial nerves and cerebellar signs where a schwannoma is possible
• Investigation — pure tone audiometry with masking; tympanometry and acoustic reflexes; speech audiometry; otoacoustic emissions and BERA; and imaging — CT for bone and middle ear disease, and MRI with gadolinium of the internal acoustic meatus for any asymmetric sensorineural loss
• The rule that must not BE forgotten: an asymmetric or unilateral sensorineural loss, or unilateral tinnitus, requires MRI to exclude a vestibular schwannoma — however plausible the alternative explanation seems
• Assess the disability, not only the audiogram — the effect on work, on family life and on safety.
• The maximum conductive loss is 60 decibels, so a greater loss proves a sensorineural component whatever the drum looks like
• A unilateral sensorineural loss is never central, because the pathway is bilateral above the cochlear nuclei
• Any asymmetric sensorineural loss needs an MRI of the internal acoustic meatus, however plausible the alternative explanation
• Otitis media with effusion is the commonest cause in children, and presbycusis much the commonest overall
• Otosclerosis is the commonest progressive conductive loss in a young adult with a normal drum and no history of ear disease

Tuning Fork Tests

• Tuning fork tests compare air and bone conduction and the two ears, and so establish the type of hearing loss at the bedside.
• A 512 Hz fork is used: lower frequencies are felt as vibration, and higher ones decay too quickly.
• The fork is struck on a firm but yielding surface — the elbow or knee — at about one-third from the free end, and struck gently, since a hard strike produces overtones that confuse the test
• For air conduction the vibrating prongs are held parallel to the canal 2 cm from it; for bone conduction the foot is pressed firmly on the mastoid

Assessment in Children and Limitations

• Tuning fork tests require a cooperative patient, and are unreliable below 4 to 5 years
• In the preverbal child — otoacoustic emissions and automated BERA for newborn screening; behavioural observation audiometry in the first months; distraction testing at 6 to 9 months; visual reinforcement audiometry from 6 months to 2 years, which is the most useful; and play audiometry from 2 to 4 years
• Never rely on a parent’s reassurance that the child hears: children with significant loss respond to visual cues, vibration and routine, and families are frequently confident about a deaf child
• The limitations of tuning fork tests — they are qualitative rather than quantitative; they miss losses below 15 to 20 dB; they test one frequency; they are subject to the false negative Rinne; and they depend on patient cooperation and on correct technique
• They therefore supplement rather than replace audiometry — but their great value is that they are immediate, free, and available where no audiometer exists.
• A discrepancy between the tuning fork tests and the audiogram should always be resolved rather than ignored: it means a technical error, incorrect masking, or a non-organic loss
• Strike the fork gently and a third from the free end; a hard strike produces overtones that confuse the result
• Use a 512 hertz fork; lower frequencies are felt as vibration and higher ones decay too quickly to be useful
• The Rinne becomes negative at about fifteen to twenty decibels, so it misses milder conductive losses entirely
• A false negative Rinne means a dead ear, the fork being heard through the skull by the opposite cochlea
• Mask the opposite ear with a Barany box whenever the Rinne and Weber disagree.
• Weber to the worse ear means conductive and to the better ear means sensorineural.
• Block your own ear during the Weber test; the sound moves to that side and demonstrates the mechanism in seconds
• Interpret Rinne and Weber together, never separately; their agreement is what makes either of them reliable
• Absolute bone conduction assumes a normal examiner, which is an obvious but frequently unstated limitation
• A negative Gelle indicates ossicular fixation, and is the classical though now historical test for otosclerosis
• The Stenger test detects feigned unilateral deafness, and is worth knowing where compensation is at issue
• Tuning fork tests are qualitative, test one frequency and miss mild losses, so they supplement rather than replace audiometry
• Their value is that they need no equipment, which matters greatly in district practice where no audiometer exists

Pure Tone Audiometry

• Pure tone audiometry (PTA) measures the threshold of hearing — the quietest sound heard at least half the time — for pure tones at each frequency, by air and by bone conduction.
• It is the standard quantitative test of hearing.
• The frequencies tested are 250, 500, 1000, 2000, 4000 and 8000 Hz, with 3000 and 6000 Hz added where noise damage is suspected
• The result is plotted in dB HL (hearing level), a scale on which 0 dB is the average threshold of a normal young adult, not silence — which is why a person may score better than 0 dB

Impedance Audiometry

• Impedance (acoustic immittance) audiometry measures the compliance of the tympano-ossicular system as air pressure in the canal is varied.
• It is objective, needs no response from the patient, and takes seconds — which makes it invaluable in children.

• The acoustic (stapedial) reflex is measured at the same time: a loud sound (about 70 to 100 dB above threshold) causes bilateral contraction of stapedius, stiffening the chain and producing a measurable change in compliance
• Its pathway explains everything it can detect — cochlea, eighth nerve, cochlear nucleus, superior olive, facial motor nucleus, seventh nerve, stapedius. A lesion anywhere abolishes it
• The reflex is absent in — any conductive loss, even a small one.
• Reflex decay — the reflex normally holds for 10 seconds at 500 and 1000 Hz. Decay to less than half in 5 seconds suggests a retrocochlear (nerve) lesion, and is a classical screening sign for vestibular schwannoma
• The reflex also provides an objective cross-check: a present reflex in an ear said to be profoundly deaf suggests a non-organic loss
• Eustachian tube function can be tested by recording tympanograms before and after swallowing or Valsalva, and with a perforation by measuring the pressure required to open the tube
• Zero decibels hearing level is a normal young adult, not silence, which is why a good ear can score better than zero
• Right is red circles and left is blue crosses, and confusing them reverses the diagnosis entirely
• Interaural attenuation is about forty decibels by air and close to nothing by bone.
• A shadow curve makes a dead ear look useful, and is the characteristic error of an unmasked audiogram
• An air-bone gap over ten to fifteen decibels is taken as significant, and defines the conductive component
• The pure tone average uses 500, 1000 and 2000 hertz, the frequencies that carry speech
• A notch at 4000 hertz with recovery at 8000 is essentially diagnostic of noise-induced hearing loss
• The Carhart notch is an artefact of fixation, not true cochlear loss, and disappears after successful stapes surgery
• A rising low-frequency loss suggests early Meniere disease or a middle ear effusion
• Add 3000 and 6000 hertz where noise is suspected, since the notch may sit between the standard frequencies
• Tympanometry needs no response from the patient, which makes it invaluable in an uncooperative child

Nature and Pathology

• Otosclerosis is a primary disease of the otic capsule in which normal enchondral bone is replaced by spongy, highly vascular new bone, which later becomes sclerotic.
• Fixation of the stapes footplate produces a progressive conductive hearing loss in an ear that looks entirely normal.
• The name is a misnomer: the active disease is otospongiosis, the sclerotic phase coming later. It is confined to the otic capsule, which is the only bone in the body that normally undergoes no remodelling

Clinical Features and Diagnosis

• The classical presentation is a young adult, often a woman, with progressive deafness and a normal-looking drum — and it is the combination of a significant conductive loss with a normal ear that makes the diagnosis
• Hearing loss — bilateral in 70 to 80%, though often asymmetrical; slowly progressive over years; and conductive at first, becoming mixed as cochlear involvement supervenes
• Paracusis willisii — hearing better in noisy surroundings, which is characteristic and is explained by others raising their voices while the background noise is filtered out

Management

• Three options must be offered and discussed: observation, a hearing aid, or surgery. A hearing aid is a legitimate first choice and works well in otosclerosis.
• Stapedotomy (or stapedectomy) is the definitive operation: through a permeatal approach, the stapes superstructure is removed, a small fenestra made in the footplate, and a prosthesis placed from the incus to the fenestra. Stapedotomy with a small fenestra has replaced total stapedectomy, giving better results and fewer complications
• Results are excellent — closure of the air-bone gap to within 10 dB in 90%
• The rules of case selection matter — operate on the worse ear first; require an air-bone gap of at least 20 to 30 dB and adequate cochlear reserve; and never operate on an only hearing ear, since there is a dead-ear risk of 1%
• Other contraindications — active otosclerosis with a marked Schwartze sign; active middle ear infection; a patient whose occupation involves flying, diving or extremes of pressure; poor general health; and the very elderly, in whom an aid is simpler
• Complications — sensorineural loss or a dead ear (1%); vertigo, transient; perilymph fistula; reparative granuloma; prosthesis displacement or incus necrosis causing late recurrence; a floating or depressed footplate.
• Sodium fluoride may be used in cochlear otosclerosis with a progressive sensorineural loss, on the basis that it promotes maturation of the active focus; the evidence is limited, and it does not help the conductive component
• Cochlear implantation for far-advanced otosclerosis with profound loss, noting that facial nerve stimulation is commoner in these implanted ears because current spreads through demineralised bone
• Counsel about pregnancy, which may accelerate progression, and about the hereditary pattern
• The disease is otospongiosis before it is sclerosis, the active vascular phase preceding the dense one
• The otic capsule is the only bone that does not remodel, which is why a focus of remodelling there is pathological
• Pregnancy accelerates it, and the history should be asked for directly in any young woman with progressive conductive loss
• Penetrance is incomplete at about forty per cent, so the absence of a family history means very little
• Measles RNA has been found in otosclerotic foci, and the incidence has fallen since vaccination became widespread
• The fissula ante fenestram is the usual site, which is why stapes fixation is the usual consequence
• A soft voice suggests conductive loss and a loud voice sensorineural.
• The Schwartze sign indicates active vascular disease and is a relative contraindication to immediate surgery
• Superior canal dehiscence mimics otosclerosis but has present acoustic reflexes and sound-induced vertigo
• Ask about the Tullio phenomenon; vertigo provoked by loud sound points away from otosclerosis and toward dehiscence
• Offer the hearing aid honestly; the cochlea is normal so an aid works very well, and surgery is not the only option

Causes and Screening

• Deafness IN childhood matters out of proportion to its prevalence because hearing is the substrate for speech and language.
• A child who does not hear in the first years does not learn to speak, and the consequences are educational, social and lifelong.
• The critical period is the whole point: the auditory cortex requires sound input to develop, and the first 2 to 3 years are decisive for language. Habilitation delayed beyond this is far less effective, however good the technology — which is why the entire subject is about early detection

Assessment and Habilitation

• Age-appropriate testing — otoacoustic emissions and BERA in the newborn; behavioural observation in the first months; distraction testing at 6 to 9 months; visual reinforcement audiometry from 6 months to 2 years; play audiometry from 2 to 4 years; and conventional audiometry thereafter — with tympanometry throughout
• Aetiological work-up — a careful pregnancy, birth and family history including consanguinity; examination for syndromic features; ophthalmology referral (for Usher syndrome, and because a deaf child depends on vision); an ECG for the prolonged QT syndrome; urinalysis for Alport; CMV testing, which must be done on a sample taken within the first 3 weeks or on the stored newborn blood spot, since later testing cannot distinguish congenital from acquired infection; genetic testing including connexin 26; and MRI and CT of the temporal bones
• Habilitation must begin as soon as the diagnosis is made, and is a team effort involving audiologist, speech and language therapist, teacher of the deaf, and the family
• Hearing AIDS — fitted as early as a few weeks of age, bilaterally, with regular re-assessment as the ear grows and the thresholds are refined
• Cochlear implantation for severe to profound bilateral loss with inadequate benefit from aids, ideally before 12 months and certainly before 2 to 3 years; and urgently after meningitis, before the cochlea ossifies
• Speech and language therapy and a chosen communication approach — auditory-verbal, total communication or sign language. The family’s choice must be respected, and there is a genuine Deaf-community perspective that values sign language as a language rather than as a failure of speech
• Educational support — increasingly inclusive schooling with a resource teacher, FM systems and preferential seating, rather than segregated schooling
• Treat the treatable — a coexisting middle ear effusion must be dealt with, since it adds a further conductive loss on top of a sensorineural one and is easily forgotten
• Support the family — the diagnosis is a bereavement for many parents, and counselling, parent groups and clear information determine whether the habilitation is actually carried out
• Under the rights OF persons with disabilities act 2016, hearing disability is certified and confers entitlements to education, aids and reservation, and families should be helped to obtain the certificate
• The first two to three years are decisive for language, which is why the whole subject is about early detection
• Targeted screening misses about half of affected babies, who have no risk factor at all
• Screen by one month, diagnose by three, intervene by six, which is the standard to state and the one that determines outcome
• Emissions alone miss auditory neuropathy, so high-risk babies need automated brainstem response as well
• Connexin 26 is the commonest single genetic cause, and most non-syndromic congenital deafness is autosomal recessive
• Consanguinity substantially raises the risk, which matters particularly in parts of India and should be asked about
• Every deaf child needs an eye examination, for Usher syndrome and because a deaf child depends on vision
• Do an ECG in profound congenital deafness; the prolonged QT of Jervell and Lange-Nielsen causes sudden death
• Test for cytomegalovirus within three weeks or on the stored blood spot; later testing cannot prove congenital infection
• Congenital cytomegalovirus may be silent at birth and cause late-onset progressive loss, so it must be actively sought

Defining the Complaint

• Vertigo is an illusion of movement — of the patient or of the surroundings — arising from a mismatch between the vestibular, visual and proprioceptive inputs.
• It is not the same as dizziness, and the distinction is the whole of the initial assessment.
• "dizziness" IS A useless word until IT IS taken apart. The single most productive question is "describe it without using the word dizzy", and the answer sorts patients into four groups with entirely different causes
• 1. Vertigo — spinning, tilting or rocking; a true illusion of movement. Indicates vestibular disease, peripheral or central
• 2. Presyncope — light-headedness, feeling faint, greying of vision, especially on standing. Indicates cardiovascular causes — postural hypotension, arrhythmia, anaemia, hypoglycaemia, drugs
• 3. Disequilibrium — unsteadiness only when walking or standing, with no sensation in the head. Indicates neurological or multisensory causes — peripheral neuropathy, cerebellar disease, parkinsonism, visual impairment, or all of these together in the elderly
• 4. Non-specific light-headedness — floating, dissociation, or a vague swimming sensation. Frequently anxiety, hyperventilation or depression, and frequently coexisting with genuine vestibular disease
• The time course IS the second discriminator, and IT IS almost diagnostic. Ask how long a single attack lasts, not how long the illness has lasted — a distinction patients confuse and clinicians rarely clarify
• Seconds to under a minute, positional — benign paroxysmal positional vertigo
• Minutes to hours (20 minutes to 12 hours), episodic, with hearing symptoms — meniere disease
• Minutes to an hour without hearing symptoms, with headache or aura — vestibular migraine
• Days, single prolonged attack, then gradual improvement — vestibular neuritis (no hearing loss) or labyrinthitis (with hearing loss)
• Sudden onset, persistent, with other neurological signs — brainstem or cerebellar stroke
• Continuous, progressive over months, with unilateral hearing loss — acoustic neuroma
• Provoked by pressure, straining, sneezing or loud sound — perilymph fistula or a superior canal dehiscence
• The third discriminator IS the associated symptoms, and specifically whether there are auditory symptoms — hearing loss, tinnitus, aural fullness. Their presence localises to the labyrinth or the eighth nerve; their absence widens the differential considerably
• Ask about neurological symptoms — the five Ds: diplopia, dysarthria, dysphagia, dysmetria and drop attacks, with numbness, weakness and ataxia. Any of these makes the cause central until proved otherwise
• Ask about the drug history — aminoglycosides, loop diuretics, antihypertensives, antidepressants, anticonvulsants, alcohol and vestibular sedatives themselves
• Ask about head injury, ear surgery, migraine and cardiovascular risk

Examination and the Peripheral-central Distinction

• Examination should be directed rather than exhaustive, and these are the elements that change the diagnosis:
• The ear — otoscopy for perforation, cholesteatoma or effusion; tuning fork tests; and the fistula test
• Spontaneous nystagmus, observed with fixation and, crucially, without fixation using Frenzel glasses or an ophthalmoscope on the fundus — because peripheral nystagmus is suppressed by visual fixation and central nystagmus is not
• The head impulse (Halmagyi) test.
• The DIX-hallpike manoeuvre, in any patient with positional symptoms
• Cranial nerves, cerebellar signs, gait and Romberg
• Lying and standing blood pressure, which is omitted far too often in a patient whose real problem is postural hypotension

Nature and Pathogenesis

• Benign paroxysmal positional vertigo (BPPV) is brief vertigo provoked by a change in head position, caused by displaced otoconia from the utricle entering a semicircular canal.
• It is the commonest cause of vertigo of all, and is curable in one visit.
• The mechanism follows from one fact: the cupula normally has the same specific gravity as endolymph, so it is not affected by gravity and the canals respond only to rotation. Otoconia are denser — so once they enter a canal, gravity begins to move the endolymph and the canal becomes gravity-sensitive

Diagnosis and Treatment

• The DIX-hallpike manoeuvre is the diagnostic test for posterior canal BPPV, and it must be performed properly:
• Sit the patient upright, turn the head 45 degrees toward the side being tested, then lie them rapidly supine with the head extended 20 to 30 degrees below the horizontal, maintaining the head turn, and observe the eyes for at least 30 seconds
• The positive response has five characteristics, all of which should be present: a latency of 5 to 20 seconds; upbeating and torsional (geotropic) nystagmus, the upper pole of the eye beating toward the lower (affected) ear; a duration under a minute; reversal of the nystagmus on sitting up; and fatigue on repetition
• The vertigo accompanies the nystagmus, and the patient should be warned beforehand that the test will provoke it, or they will resist
• Contraindications to Dix-Hallpike — cervical spine instability, severe cervical spondylosis, recent neck injury, carotid disease and severe cardiac disease; the side-lying (Semont) test is an alternative
• The supine roll (Pagnini-McClure) test diagnoses lateral canal BPPV, in which the nystagmus is horizontal and lasts longer: geotropic (beating toward the ground) indicates canalolithiasis, and apogeotropic indicates cupulolithiasis, with the affected side being that with the more intense response in geotropic disease
• Treatment IS a particle repositioning manoeuvre, and it is curative in a single session in the majority — which makes BPPV one of the most satisfying conditions in medicine to treat
• The EPLEY manoeuvre for the posterior canal moves the otoconia around the canal and back into the utricle through a sequence of four head positions, each held until the nystagmus settles
• The SEMONT (liberatory) manoeuvre is an alternative, using a rapid movement from one side-lying position to the other
• The barbecue (Lempert) roll for the lateral canal, rolling the patient through 360 degrees toward the unaffected side
• BRANDT-DAROFF exercises are performed by the patient at home, and are used for residual symptoms or where manoeuvres have failed; they are less effective than a properly performed Epley
• Drugs DO not treat BPPV.
• Post-manoeuvre restrictions (sleeping upright, avoiding head movement) were formerly advised but are not supported by evidence and are no longer recommended
• Prognosis — excellent, but recurrence occurs in up to half within a few years, so the patient should be told this and taught to recognise it; and self-treatment can be taught to those with frequent recurrences
• When BPPV does not respond, reconsider the diagnosis — central positional vertigo from a posterior fossa lesion produces downbeating nystagmus with NO latency and NO fatigue, and requires imaging
• Turning over in bed is close to pathognomonic, and is the single most useful line in the history
• Attacks last seconds, never minutes, though patients feel unwell for longer afterwards and report the whole period
• Auditory symptoms suggest another diagnosis; BPPV causes no hearing loss, tinnitus or fullness
• The posterior canal is the most dependent, which is why it is affected in the great majority of cases
• Head injury often causes bilateral BPPV, and is the commonest identifiable cause
• Warn the patient before Dix-Hallpike that it will provoke the vertigo, or they will resist and the test will fail
• Observe for at least thirty seconds, since the latency may be up to twenty and a brief look will miss the response

Nature and Pathology

• The pathological hallmark is endolymphatic hydrops — distension of the endolymphatic system, demonstrated at post-mortem as ballooning of Reissner membrane and the saccule
• The mechanism is failure of endolymph homeostasis: either over-production by the stria vascularis or, more probably, under-absorption by the endolymphatic sac.

Clinical Features

• The classical tetrad — and the diagnosis requires the combination, not any single element:
• 1. Vertigo — episodic, rotatory, lasting 20 minutes to several hours (by definition at least 20 minutes and less than 12 hours), with severe nausea and vomiting and prostration. Attacks under 20 minutes suggest something else, and attacks lasting days are not Meniere
• 2. Fluctuating sensorineural hearing loss — initially low-frequency and reversible, recovering between attacks, which is highly characteristic; later becoming flat and permanent as the disease progresses

Investigation and Management

• Pure tone audiometry shows a low-frequency (rising) sensorineural loss early, becoming flat later; and serial audiograms document the fluctuation.
• Other tests — electrocochleography, with a raised SP/AP ratio supporting hydrops; caloric testing, showing reduced response on the affected side; glycerol dehydration test, historically used, in which hearing improves after an osmotic agent; and MRI with delayed gadolinium, which can now demonstrate hydrops directly
• MRI of the internal acoustic meatus is mandatory to exclude acoustic neuroma.
• Exclude syphilis, autoimmune disease and thyroid dysfunction where the picture is atypical or bilateral
• Management of the acute attack — bed rest in a quiet darkened room; vestibular sedatives (prochlorperazine, cinnarizine) and antiemetics, given parenterally if vomiting; and reassurance.
• Long-term medical management — salt restriction (under 2 g sodium daily) with avoidance of caffeine, alcohol and tobacco, which is the foundation and is frequently the only treatment needed; betahistine, widely used, which is thought to improve cochlear microcirculation, though the evidence is contested; and diuretics (hydrochlorothiazide with amiloride, or acetazolamide)
• Treat coexisting migraine, which is common and may be driving much of the symptom burden
• Vestibular rehabilitation between attacks, and a hearing aid for the established loss
• Intratympanic therapy where medical treatment fails:
• Intratympanic steroid (dexamethasone) — non-destructive and hearing-preserving, and therefore the first intratympanic step, particularly where hearing is still useful
• Intratympanic gentamicin — a chemical labyrinthectomy.
• Surgery — endolymphatic sac decompression or shunt.
• The principle governing all destructive treatment is that Meniere disease becomes bilateral in up to a third — so destroying one labyrinth may leave the patient without vestibular function at all if the other ear later becomes involved. This must be weighed and discussed
• Counsel about driving, working at heights and swimming, and about the unpredictability of attacks, which is often the most disabling aspect of the disease
• Attacks under twenty minutes are not Meniere, and attacks lasting days are not either; the duration is part of the definition
• The low-frequency loss recovers early on, which is what makes serial audiograms the most useful investigation
• Aural fullness gives the patient warning, and many learn to recognise an attack building an hour beforehand
• Tumarkin drop attacks cause serious injury and are an indication for definitive treatment rather than further medical trials
• MRI is mandatory to exclude acoustic neuroma, which can present with an identical picture
• Salt restriction is the foundation and is frequently the only treatment needed, though compliance is the difficulty
• Treat coexisting migraine, which is strongly associated and may be driving much of the symptom burden
• Intratympanic steroid is hearing-preserving and is therefore the first intratympanic step where hearing remains useful
• Gentamicin is more vestibulotoxic than cochleotoxic, which is the principle exploited by chemical labyrinthectomy
• Labyrinthectomy destroys hearing and is reserved for an ear with no useful hearing left to lose

Vestibular Neuritis and Labyrinthitis

• Vestibular neuritis is acute, spontaneous, prolonged vertigo from inflammation of the vestibular nerve, without hearing loss.
• When the cochlea is also involved, producing hearing loss, the condition is called labyrinthitis.
• The distinction is purely on the presence of auditory symptoms.
• Aetiology — most probably reactivation of latent herpes simplex type 1 in the vestibular ganglion, supported by the finding of viral DNA in vestibular ganglia; also post-viral inflammation following an upper respiratory infection, which precedes many cases; and vascular occlusion of the vestibular artery in the elderly vasculopath
• The superior vestibular nerve is affected more often than the inferior, because it runs in a longer and narrower bony canal and is more susceptible to compression by swelling — which also explains why BPPV of the posterior canal (supplied by the inferior nerve) commonly follows neuritis: the otoconia are released but the canal that receives them still works
• Presentation — sudden, severe, continuous rotatory vertigo with intense nausea and vomiting, lasting hours to days; the patient lies still with eyes closed.
• Examination — spontaneous horizontal-torsional nystagmus with the fast phase beating away from the affected ear (a paralytic response), suppressed by fixation; an abnormal head impulse test toward the affected side; falling toward the affected side on Romberg and Unterberger; and NO other neurological signs
• The essential differential IS cerebellar or brainstem stroke.
• Additional red flags — severe truncal ataxia with inability to sit unaided; headache or neck pain (suggesting vertebral dissection); vascular risk factors; and any focal deficit
• Management of the acute phase — vestibular sedatives and antiemetics for the first 24 to 72 hours only, with intravenous fluids for vomiting
• The most important instruction IS TO stop the sedatives early and mobilise. Vestibular sedatives suppress the central compensation that is the patient’s only route to recovery, so continuing them beyond a few days converts a self-limiting illness into chronic dizziness. This is the commonest management error in the whole subject
• Corticosteroids improve the recovery of vestibular function on caloric testing, though the effect on symptoms is debated; antivirals have not been shown to help, alone or with steroid
• Vestibular rehabilitation exercises started early are the mainstay of recovery, and should be prescribed at the first visit rather than after weeks of failure
• Prognosis — the vertigo settles over days to weeks and most recover fully; but a proportion have persistent unsteadiness, particularly the elderly, the anxious and those who remained on sedatives
• Complications and sequelae — secondary BPPV, which is common; persistent postural-perceptual dizziness (PPPD), a chronic functional disorder that follows an acute vestibular event and is treated with rehabilitation and antidepressants; and recurrent episodes

Perilymph Fistula and Superior Canal Dehiscence

• Perilymph fistula is an abnormal communication between the perilymphatic space and the middle ear, at the oval or round window
• Causes — barotrauma from diving, flying or forceful Valsalva; head injury and temporal bone fracture; stapes surgery, which is a recognised complication; cholesteatoma eroding the windows; heavy lifting or straining; and congenital malformation
• Features — vertigo and hearing loss provoked by pressure change, straining, sneezing, nose-blowing or loud sound; often with a pop or click at the moment of onset; and fluctuating sensorineural hearing loss with tinnitus and fullness
• A positive fistula test supports it, and the diagnosis is frequently only confirmed at exploratory tympanotomy.
• Management — conservative first: bed rest with the head elevated, avoidance of straining, stool softeners, for a week or two; and surgical repair by tympanotomy with grafting of the windows where symptoms persist or hearing deteriorates
• Superior semicircular canal dehiscence is a defect in the bone overlying the superior canal, creating a third mobile window into the labyrinth — which is the concept that explains all its features
• TULLIO phenomenon — vertigo and oscillopsia provoked by loud sound; and hennebert sign, vertigo provoked by pressure

Nature and Presentation

• The name IS doubly misleading, and saying so shows understanding: it is not acoustic, arising from the vestibular (superior vestibular) division; and it is not a neuroma, being a schwannoma. Vestibular schwannoma is the correct term
• It is the commonest tumour of the cerebellopontine angle, accounting for 80% of lesions there

Investigation and Management

• Pure tone audiometry shows an asymmetric sensorineural loss, characteristically worse in the high frequencies
• Speech audiometry shows disproportionately poor discrimination for the pure tone thresholds — a roll-over phenomenon in which discrimination worsens as the sound is made louder. This is the classical retrocochlear pattern and distinguishes nerve from cochlear disease
• Other retrocochlear signs — absent stapedial reflexes or reflex decay; absent or reduced otoacoustic emissions with a normal cochlea is not expected — in a retrocochlear lesion the OAEs may be present despite a poor audiogram.
• BERA (auditory brainstem response) shows prolonged wave V latency and an increased interaural latency difference; it was formerly the screening test but is insensitive for small intracanalicular tumours
• Caloric testing shows reduced or absent response on the affected side
• Gadolinium-enhanced MRI IS the investigation OF choice and the gold standard, detecting tumours of only a few millimetres. A normal MRI excludes the diagnosis, and no other test does
• CT is inadequate for small tumours and is used only where MRI is contraindicated
• Three management options, and the choice depends on the tumour, the hearing and the patient:
• 1. Observation ("watch, wait and rescan") with serial MRI — appropriate for small tumours, elderly or unfit patients, and where hearing is still useful, given that many do not grow. This is now the commonest initial management
• 2. Stereotactic radiosurgery (gamma knife) — for small to medium tumours (under 3 cm), achieving growth control in a high proportion with better facial nerve preservation than surgery; hearing preservation is variable and declines with time, and there is a small risk of malignant transformation
• 3. Microsurgical excision — for large tumours, brainstem compression, hydrocephalus, or documented growth in a young patient
• The three surgical approaches, chosen by tumour size and whether hearing is worth preserving:
• Translabyrinthine — sacrifices hearing completely, but gives excellent early identification of the facial nerve at the fundus and requires no cerebellar retraction. Used where hearing is already poor
• Retrosigmoid (suboccipital) — hearing may be preserved; suits tumours of any size, but requires cerebellar retraction and access to the lateral end of the canal is limited
• Middle fossa — hearing may be preserved; best for small, mainly intracanalicular tumours, but involves temporal lobe retraction and the facial nerve lies superficial to the tumour, so it is more at risk
• Facial nerve monitoring is used throughout, and facial nerve preservation is now the principal measure of surgical success alongside complete excision
• Complications — facial palsy; total hearing loss; CSF leak and meningitis; headache; cerebellar injury; lower cranial nerve palsies; and persistent imbalance, which is common and frequently the patient’s chief long-term complaint

The External Nose and Nasal Vestibule

The Lateral Wall

• The lateral wall IS the most important single area IN rhinology, because everything drains onto it and all endoscopic surgery is performed there
• It bears three (sometimes four) turbinates (conchae), each overhanging a meatus into which structures open
• The inferior turbinate is a separate bone; the middle, superior and supreme turbinates are part of the ethmoid
• The inferior meatus receives only the nasolacrimal duct, guarded by the valve of HASNER, 3 cm behind the anterior end of the inferior turbinate. This is the site of antral puncture and of dacryocystorhinostomy
• The middle meatus is the drainage site of the anterior group of sinuses — the maxillary, anterior ethmoid and frontal — and is therefore the key area in sinus disease and in endoscopic sinus surgery
• The superior meatus receives the posterior ethmoid cells
• The sphenoethmoidal recess, above and behind the superior turbinate, receives the sphenoid sinus
• The simple rule that organises all OF this: everything drains into the middle meatus except the nasolacrimal duct (inferior meatus), the posterior ethmoids (superior meatus) and the sphenoid (sphenoethmoidal recess)
• The structures OF the middle meatus.
• The uncinate process — a sickle-shaped bony leaflet, and the first structure removed in endoscopic sinus surgery (uncinectomy)
• The hiatus semilunaris — the two-dimensional cleft between the uncinate process and the ethmoid bulla
• The ethmoid bulla — the largest and most constant anterior ethmoid air cell
• The infundibulum — the three-dimensional channel lateral to the uncinate, into which the maxillary and anterior ethmoid sinuses drain
• The frontal recess — the drainage pathway of the frontal sinus, anterosuperiorly
• The middle turbinate has a three-part attachment that is the key to safe surgery: anteriorly vertical to the cribriform plate, middle oblique (the basal or ground lamella) to the lamina papyracea, and posteriorly horizontal to the lamina papyracea
• The ground lamella is the surgical landmark dividing the anterior from the posterior ethmoid cells
• The vertical attachment reaches the cribriform plate — which is why excessive medial traction on the middle turbinate can fracture the cribriform plate and cause a CSF leak
• Anatomical variants that predispose to sinus disease — concha bullosa (a pneumatised middle turbinate); paradoxical middle turbinate, curving the wrong way; HALLER cells (infraorbital ethmoid cells narrowing the maxillary ostium); ONODI cells (posterior ethmoid cells extending over the sphenoid, intimately related to the optic nerve and a genuine hazard); and AGGER nasi cells, the most anterior ethmoid cells.
• Accessory maxillary ostia occur in a substantial proportion and may cause recirculation of mucus, which is a recognised cause of persistent symptoms after surgery
• The nasal valve is the narrowest part of the airway and contributes about half the total resistance, so small changes there matter greatly
• The vestibule is lined by skin, not mucosa, which is why furuncles and vestibulitis occur there and nowhere else in the nose
• The inferior turbinate is a separate bone; the middle, superior and supreme turbinates belong to the ethmoid
• The nasolacrimal duct is the only thing in the inferior meatus, which is why antral puncture and dacryocystorhinostomy are done there

The Sinuses Individually

• The paranasal sinuses are air-filled cavities within the facial and skull bones, lined by respiratory epithelium and communicating with the nasal cavity through ostia.
• They comprise the maxillary, ethmoid, frontal and sphenoid sinuses.
• Their functions are debated and none is proven, but those proposed are: lightening the skull; voice resonance; humidifying and warming inspired air; a crumple zone protecting the brain and orbit in facial trauma; thermal insulation of the skull base; and contributing to facial growth and shape

The Osteomeatal Complex

• The osteomeatal complex (OMC) is the functional unit that determines the health of the anterior sinuses, and understanding it is the basis of modern rhinology
• It is not a single structure but a region, comprising the maxillary ostium, the infundibulum, the uncinate process, the hiatus semilunaris, the ethmoid bulla, the anterior ethmoid cells and the frontal recess, together with the middle meatus into which they all open
• The central concept is that obstruction of this narrow common channel causes disease in all the sinuses that drain through it, even though the sinuses themselves are healthy
• The sequence is: mucosal oedema (from infection, allergy or a variant) → ostial obstruction → reduced ventilation, oxygen absorption and negative pressure → impaired mucociliary clearance and retained secretion → bacterial growth → further oedema — a self-perpetuating vicious circle
• The therapeutic consequence IS the whole rationale OF functional endoscopic sinus surgery (FESS): relieving the obstruction at the OMC allows the diseased sinus mucosa to recover by itself. This replaced the older radical operations.
• Hence the word functional — the surgery restores ventilation and natural drainage rather than removing tissue or creating an artificial opening
• It also explains why an antral washout or an inferior meatal antrostomy often fails: the cilia continue to sweep mucus toward the natural ostium in the middle meatus regardless of any artificial window made elsewhere, so an opening in the wrong place is bypassed
• Factors narrowing the OMC — anatomical variants (concha bullosa, Haller cells, a paradoxical or medialised middle turbinate, a large ethmoid bulla, septal deviation with a spur); mucosal oedema from allergy or infection; polyps; and previous surgery with scarring
• CT in the coronal plane is the imaging of choice.
• The essential preoperative CT checklist — the height and symmetry of the skull base; the KEROS classification of olfactory fossa depth (a deep type III fossa carries the greatest risk of CSF leak); dehiscence of the lamina papyracea; the position of the anterior ethmoidal artery; ONODI cells and the optic nerve; and dehiscence of the carotid canal in the sphenoid
• The maxillary ostium drains against gravity, which is why the sinus depends entirely on mucociliary action and is the commonest infected
• The molar roots may project into the antral floor, so dental infection causes sinusitis and extraction may leave a fistula
• The lamina papyracea is paper-thin, and is the route by which ethmoiditis becomes orbital cellulitis in a child
• The cribriform plate is lower and thinner than the fovea ethmoidalis.

Arterial Supply

• The nose has a remarkably rich blood supply, derived from both the internal and the external carotid systems, which anastomose freely.
• This explains both the excellent healing of nasal tissue and the severity of epistaxis.
• From the internal carotid, via the ophthalmic artery — the anterior and posterior ethmoidal arteries.

Nerve Supply and Lymphatics

• The nerve supply divides cleanly into three functional groups, and organising the answer this way makes it far clearer than a list:
• 1. Special sensory (olfaction) — the olfactory (first) nerve. Its receptors lie in the olfactory epithelium of the roof, the upper septum and the superior turbinate, and their unmyelinated axons pass through the cribriform plate to the olfactory bulb
• Because these fibres pass through bone with a sheath of dura and arachnoid, the cribriform plate is a route for CSF leak and for intracranial spread of infection
• 2. General sensory — the trigeminal nerve: the anterior ethmoidal nerve (from the ophthalmic division, V1) supplies the anterosuperior part; and branches of the maxillary division (V2) — the nasopalatine, greater palatine and posterior lateral nasal nerves, from the pterygopalatine ganglion — supply the posterior and inferior part
• The anterior ethmoidal nerve also supplies the external nasal skin at the tip via its external nasal branch.
• The sneeze reflex is mediated by trigeminal afferents, which is why irritation of the nasal mucosa provokes it; and the nasociliary nerve explains the PHOTIC sneeze (ACHOO) reflex
• 3. Autonomic — the group that governs nasal physiology: parasympathetic secretomotor fibres arise in the superior salivatory nucleus and travel in the nervus intermedius → greater superficial petrosal nerve → nerve of the pterygoid canal (vidian nerve) → pterygopalatine ganglion, and cause vasodilatation and secretion
• Sympathetic fibres arise from the superior cervical ganglion, travel with the deep petrosal nerve (which joins the greater petrosal to form the vidian nerve), and cause vasoconstriction and decongestion
• The balance between them explains most OF nasal physiology and pharmacology — the nasal cycle, the blockage of vasomotor rhinitis, the action of topical sympathomimetic decongestants, and the rationale of vidian neurectomy, formerly performed for intractable rhinorrhoea
• Lymphatic drainage — the anterior part drains to the submandibular nodes; the posterior part and the sinuses to the retropharyngeal and upper deep cervical nodes
• The retropharyngeal drainage is clinically important.
• Four arteries meet at Little area, which is why the antero-inferior septum accounts for about ninety per cent of nosebleeds
• The sphenopalatine is the principal artery of the nose, entering behind the posterior end of the middle turbinate
• The ethmoidal arteries come from the internal carotid, which is why external carotid ligation alone may fail to stop bleeding
• Endoscopic sphenopalatine ligation has replaced proximal ligation, being closer to the bleeding point and more reliable
• Woodruff plexus is venous and posterior, and explains the heavier posterior epistaxis of the elderly
• Children essentially always bleed from Little area, so a posterior bleed in a child should prompt a search for another cause
• The ophthalmic veins are valveless, which is the anatomical basis of the danger area of the face

The Functions of the Nose

• The nose is far more than an airway.
• Its functions are respiration and air-conditioning, protection and defence, olfaction, vocal resonance, and reflex regulation — and every one of them is lost or impaired by the mouth-breathing that follows nasal obstruction.
• 1. Respiration and air-conditioning
• The nose accounts for about half the total resistance of the entire respiratory tract, most of it at the nasal valve. This resistance is not a fault but a function: it slows and directs the airflow, creating the back-pressure needed for normal alveolar ventilation and for the elastic recoil of the lung

The Nasal Cycle and the Mucosa

• The nasal cycle is a normal physiological phenomenon present in a majority of people: alternating congestion and decongestion of the two sides, with a period of 2 to 7 hours, under hypothalamic autonomic control
• The total airway resistance stays constant.
• Its clinical importance is threefold: a patient with a mildly deviated septum notices obstruction only when the cycle congests the narrow side, giving alternating blockage; it confounds rhinomanometry and acoustic rhinometry unless the nose is decongested first; and it explains why a patient may report the obstruction moving from side to side.
• The turbinates contain venous sinusoids (cavernous tissue) whose filling and emptying, under autonomic control, produce these changes rapidly
• Posture affects them — the dependent side congests on lying on one side, and both sides congest on lying supine, which is why nasal obstruction is characteristically worse at night
• Other influences — exercise decongests (through sympathetic activity); alcohol, hot drinks and emotion congest; and pregnancy and hormonal changes cause congestion — rhinitis of pregnancy
• Mucociliary clearance is considered separately, but the essential physiology is that cilia beat 10 to 20 times per second in a double layer of mucus, moving secretion toward the nasopharynx at 1 cm per minute, clearing the nose in about 20 to 30 minutes
• Ciliary function is impaired by — drying, which is the commonest and most correctable; extremes of temperature and of pH; smoking; infection, particularly viral; topical decongestants and preservatives (benzalkonium); and anaesthetic agents. Optimal function requires a pH near 7 and adequate hydration
• The practical consequence for treatment is that saline irrigation, humidification and stopping smoking support the nose’s own defences.
• Nasal resistance is a function, not a fault, providing the back-pressure needed for normal alveolar ventilation
• Half the total airway resistance is nasal, and most of that is at the nasal valve
• Mouth breathing delivers cold dry unfiltered air to the lower airway.
• The nose uses about a litre of water daily to humidify inspired air.
• Particles above ten micrometres are filtered out almost entirely, while smaller ones reach the lower airway
• Secretory IgA is the principal immunoglobulin of nasal secretion, alongside lysozyme, lactoferrin and interferon
• The sinuses produce nitric oxide, which is bacteriostatic and improves ventilation-perfusion matching when inhaled
• Only a small fraction of air reaches the olfactory cleft in quiet breathing, which is why sniffing is needed to smell
• Rhinolalia clausa is a blocked voice and rhinolalia aperta an excessively nasal one, and the two indicate opposite problems
• The nasal cycle keeps total resistance constant, which is why a healthy person is unaware of it
• Alternating blockage is reassuring, indicating the cycle acting on a mild deviation rather than a progressive lesion
• Decongest before rhinomanometry, or the nasal cycle makes the measurement uninterpretable
• The dependent side congests on lying, which is why obstruction is characteristically worse at night and on one side
• Exercise decongests the nose through sympathetic activity, and alcohol and hot drinks congest it
• Rhinitis of pregnancy is hormonal, resolves after delivery, and should not be treated with prolonged decongestants
• Cilia clear the nose in twenty to thirty minutes, moving mucus at about a centimetre a minute toward the nasopharynx
• Drying is the commonest impairment of cilia and the most easily corrected.

History and Clinical Examination

• The history should establish six symptoms, and each points in a different direction:
• Nasal obstruction — is it unilateral or bilateral, constant or alternating, and is it progressive? Alternating obstruction is reassuring (the nasal cycle on a mild deviation); persistent unilateral obstruction in an adult is the pattern that demands a tumour be excluded
• Discharge (rhinorrhoea) — watery (allergic, vasomotor, CSF); mucoid; purulent (infective); unilateral foul discharge in a child is a foreign body until proved otherwise; and blood-stained unilateral discharge in an adult is a malignancy until proved otherwise
• Epistaxis — side, frequency, severity, and precipitants
• Sneezing and itching — which point strongly to allergy, particularly with itching of the eyes and palate

Investigation

• Nasal endoscopy — rigid (0 and 30 degree) or flexible, after decongestion and topical anaesthesia; performed in three passes along the floor, the middle meatus, and the sphenoethmoidal recess
• Imaging — plain radiographs are now obsolete for sinus disease, being insensitive and non-specific, and should not be requested
• CT is the imaging of choice, in the coronal plane, showing the osteomeatal complex, the extent of disease, anatomical variants and the relations to the orbit and skull base
• The essential caveat about CT: incidental mucosal thickening is present in a large proportion of asymptomatic people, so CT must be interpreted with the symptoms and the endoscopy, never alone. Treating a scan rather than a patient is a common error
• CT is best performed after a course of medical treatment, so that it shows persistent rather than reversible disease
• MRI is superior for soft tissue, and is used to distinguish tumour from retained secretion, and to assess orbital, dural and intracranial extension and perineural spread. CT and MRI are complementary in malignancy, not alternatives
• Allergy testing — skin prick tests, which are quick and cheap but require antihistamines to be stopped; and specific IgE (RAST), which is unaffected by drugs and is used where skin testing is impractical or there is dermographism
• Nasal smear for eosinophils, supporting an allergic or eosinophilic cause
• Objective airway measurement — peak nasal inspiratory flow.
• Smell testing — the UPSIT and Sniffin’ Sticks
• Mucociliary function — the saccharin test as a screen, with nasal nitric oxide (characteristically very low in primary ciliary dyskinesia) and ciliary biopsy with electron microscopy where it is suspected
• Biopsy of any mass — but never biopsy a nasal mass in a child, or any pulsatile or highly vascular mass, before imaging.
• Blood tests where relevant — ANCA for granulomatosis with polyangiitis; ACE for sarcoidosis; immunoglobulins where there is recurrent infection; and clotting studies in recurrent epistaxis
• Sweat chloride and genetic testing for cystic fibrosis in a child with nasal polyps, which is the rule that must not be forgotten
• Persistent unilateral obstruction in an adult demands that a tumour be excluded, whatever else is found
• Unilateral foul discharge in a child is a foreign body until proved otherwise, and the smell is the giveaway
• Blood-stained unilateral discharge in an adult is malignancy until proved otherwise and needs endoscopy
• Alternating obstruction is reassuring, indicating the nasal cycle acting on a mild deviation
• Facial pain alone is rarely sinusitis, and is far more often midfacial segment pain or migraine
• Ask about aspirin sensitivity and asthma; the Samter triad with polyps changes both prognosis and management
• Ask about hardwood dust and nickel, which are occupational causes of sinonasal adenocarcinoma
• Pale boggy mucosa suggests allergy and red mucosa suggests infection.
• Always examine again after decongestion; it separates fixed from mucosal obstruction and is routinely skipped
• The Cottle test suggests valve compromise, and identifies patients whose obstruction will not be relieved by septal surgery alone
• Look for the allergic salute and nasal crease in a child, which make the diagnosis across the room

Sites and Causes

• Epistaxis is bleeding from the nose.
• It is a symptom, not a diagnosis, and is the commonest ENT emergency.
• It is classified by site into anterior and posterior, a distinction that governs both severity and management.

Management

• 1.
• Resuscitate first if the bleeding is heavy — assess airway, breathing and circulation; obtain intravenous access, take blood for full blood count, group and save or cross-match, and clotting; and give fluids.
• A patient can exsanguinate from the nose → 2.
• Position and first aid — sit the patient upright and leaning forward, so blood is not swallowed (which causes vomiting) or aspirated; pinch the soft part of the nose, not the bony bridge, for 10 to 15 minutes continuously; and ask the patient to spit blood out.
• Apply ice to the neck or forehead → 3.
• Remove clot by blowing or suction and inspect with good light and a speculum.
• Nothing can be treated until the nose has been cleared → 4.
• Topical vasoconstrictor and anaesthetic on a pledget — lignocaine with adrenaline or phenylephrine — left for several minutes, which frequently stops the bleeding by itself and always makes what follows tolerable → 5.
• Identify the bleeding point and cauterise — silver nitrate chemically or electrocautery.
• Cauterise around the vessel first, then the point itself, and never cauterise both sides of the septum at the same sitting, which risks perforation → 6.
• If bleeding continues, pack the nose — a nasal tampon (Merocel), inflatable balloon pack, or ribbon gauze impregnated with bismuth iodoform paraffin paste (BIPP).
• Pack along the floor of the nose, horizontally, not upward → 7.
• Posterior bleeding — a postnasal pack, a FOLEY catheter inflated in the nasopharynx and drawn forward, or a double-balloon device.
• These patients must be admitted → 8.
• Surgical or radiological intervention where packing fails — endoscopic sphenopalatine artery ligation or clipping, which is now the procedure of choice; anterior ethmoidal artery ligation where bleeding is high in the nose; and angiographic embolisation
• Why endoscopic sphenopalatine ligation has replaced the older operations — it is close to the bleeding point, so collateral flow cannot maintain the haemorrhage. Maxillary artery ligation in the antrum and external carotid ligation are more proximal, have a higher failure rate, and external carotid ligation cannot control bleeding from the ethmoidal arteries.
• Points about packing — give antibiotic cover for packs left beyond 24 to 48 hours, because of sinusitis and the risk of toxic shock syndrome; remove the pack at 24 to 48 hours; and be aware that packing may cause hypoxia through the nasopulmonary reflex, particularly in the elderly and in those with respiratory disease, who need oxygen saturation monitoring
• Treat the cause, not just the bleed — control hypertension; review and where possible correct anticoagulation, in discussion with the physician; correct coagulopathy; and treat rhinitis or crusting
• DO not reflexly stop anticoagulation in a patient with a mechanical valve or recent thrombosis; the decision is a balance and should be made jointly
• Prevention and advice on discharge — NO nose-picking, NO forceful blowing, NO hot drinks or hot baths for a few days; petroleum jelly or an antiseptic cream to Little’s area.

Nature and Classification

• Allergic rhinitis is an IgE-mediated type I hypersensitivity inflammation of the nasal mucosa following exposure to an allergen in a sensitised individual.
• It is extremely common, affecting perhaps 20 to 30% of the population, and is a disease of the whole airway rather than of the nose alone.
• Pathophysiology proceeds in two phases, and the distinction explains why different drugs work at different times:

Clinical Features and Management

• The cardinal symptoms are the four — sneezing (paroxysmal, often in bouts), itching (of the nose, eyes, palate and throat), watery rhinorrhoea, and nasal blockage
• Itching is the symptom that best distinguishes allergic from other rhinitis, and should be asked about specifically
• Associated features — allergic conjunctivitis with itching, watering and redness; postnasal drip and cough; loss of smell; and fatigue and impaired concentration from disturbed sleep.
• Signs — a pale, bluish, boggy, oedematous inferior turbinate with clear secretion; allergic shiners (dark infraorbital discoloration from venous stasis); the DENNIE-MORGAN fold beneath the lower lid; the allergic salute, an upward rub of the nose with the palm, producing a transverse nasal crease; and mouth breathing
• Associations — the atopic triad of rhinitis, asthma and eczema. A large majority of asthmatics have rhinitis, and treating the nose improves asthma control — the "united airway" concept.
• Investigation — the diagnosis is clinical; skin prick testing, which is quick, cheap and sensitive but requires antihistamines to be stopped and carries a small risk of anaphylaxis; specific IgE (RAST), unaffected by drugs; nasal smear for eosinophils; and total IgE.
• Management proceeds in four steps:
• 1. Allergen avoidance — sensible but frequently impractical. For house dust mite: mattress and pillow covers, hot washing of bedding, removal of carpets and soft toys, and reducing humidity; for pollen: keeping windows shut, avoiding the outdoors at peak times, and washing on returning indoors; and removing a pet, which patients rarely accept
• 2. Pharmacotherapy:
• Intranasal corticosteroids are the most effective single treatment for all four symptoms including blockage — fluticasone, mometasone, budesonide. They take days to reach full effect and must be used regularly, not as required, and most treatment failure is failure of technique or adherence
• The correct technique must be taught: head slightly forward, spray with the opposite hand aiming outward toward the ipsilateral ear, and do not sniff hard. Aiming at the septum causes bleeding, crusting and perforation
• Oral antihistamines — second-generation, non-sedating agents (cetirizine, loratadine, fexofenadine) for sneezing, itching and rhinorrhoea; first-generation agents (chlorpheniramine) should be avoided because of sedation, impaired performance and anticholinergic effects
• Topical antihistamines (azelastine), which act faster; leukotriene receptor antagonists (montelukast), particularly with coexisting asthma; topical anticholinergic (ipratropium) for watery rhinorrhoea; sodium cromoglicate, safe in children and pregnancy but requiring frequent dosing; and saline irrigation, which is cheap and genuinely useful
• Short courses of oral steroid for severe exacerbations; depot injections should not be used
• Topical decongestants may be used for a few days only, to open the nose so that a steroid spray can reach the mucosa — and never beyond 5 to 7 days.
• 3. Immunotherapy — subcutaneous or sublingual desensitisation, which is the only treatment that alters the natural history of the disease. It is indicated where symptoms are severe, a single or few allergens are demonstrated, and medical treatment has failed; requires 3 to 5 years; and carries a small risk of anaphylaxis, so subcutaneous therapy is given only where resuscitation facilities are available
• 4. Surgery has a limited and specific role — it does not treat the allergy, and is used only to correct anatomical obstruction that prevents medical treatment from working: turbinate reduction for irreversible hypertrophy, and septoplasty for a significant deviation
• Itching is what distinguishes allergic rhinitis from other forms, and should be asked about specifically
• The early phase is histamine and the late phase is cellular, which is why blockage needs a steroid rather than an antihistamine

Classification and the Major Types

• Non-allergic rhinitis is inflammation or dysfunction of the nasal mucosa producing rhinitic symptoms without demonstrable IgE-mediated allergy.
• It is a diagnosis of exclusion, made when allergy testing is negative, and accounts for a substantial minority of all rhinitis.
• The clinical picture differs from allergic rhinitis in ways that are worth stating: itching and sneezing are less prominent; blockage and rhinorrhoea dominate; there is NO seasonal pattern and no personal or family history of atopy; onset is in adult life; and eye symptoms are absent
• Vasomotor (idiopathic) rhinitis is the commonest type — an imbalance of autonomic control of the nasal vasculature, with relative parasympathetic overactivity producing vasodilatation and secretion

Assessment and Management

• The history IS the diagnosis, and four questions do most of the work: what provokes it (physical triggers versus allergens); is there itching (suggesting allergy); what drugs are used, including sprays bought over the counter; and is it better away from work
• Examine for the appearance of the mucosa, septal deviation, polyps and crusting; and exclude the mimics
• The conditions that masquerade AS rhinitis and must BE excluded — chronic rhinosinusitis and nasal polyps; CSF rhinorrhoea in a unilateral clear watery discharge, particularly after trauma or surgery; a nasal tumour where symptoms are unilateral; a foreign body in a child with unilateral foul discharge; granulomatous disease where there is crusting and destruction; and primary ciliary dyskinesia or cystic fibrosis in a child
• Any unilateral rhinitic symptom deserves endoscopy.
• Investigation — allergy testing to exclude allergy, which is what defines the group; nasal smear for eosinophils; endoscopy; and CT where sinus disease is suspected
• Management:
• Identify and avoid the triggers where possible — which for vasomotor rhinitis means practical measures against cold air, smoke and strong smells rather than allergen avoidance
• Stop or substitute the offending drug, in discussion with the prescriber.
• Saline irrigation, which is safe, cheap and useful in all forms
• Topical intranasal steroid, which is the most effective drug for blockage and works in most non-allergic forms, particularly nares
• Topical anticholinergic (ipratropium) is the treatment of choice where watery rhinorrhoea is the dominant symptom — in vasomotor, gustatory and elderly rhinitis — because it blocks the parasympathetic secretomotor drive directly
• Topical antihistamine (azelastine) has an effect in vasomotor rhinitis beyond its antihistamine action, whereas oral antihistamines are of little value in non-allergic disease
• Capsaicin for intractable vasomotor rhinitis, acting by defunctionalising sensory C-fibres
• Surgery — turbinate reduction for irreversible hypertrophy, and septoplasty for obstruction; with vidian neurectomy, historically performed for intractable rhinorrhoea and now largely abandoned because of the risk of a dry eye from interrupting the lacrimal secretomotor supply
• Ask directly about over-the-counter sprays; patients do not volunteer them because a decongestant does not feel like a medicine
• Rhinitis medicamentosa is self-inflicted and curable, and the whole treatment is to stop the drug
• Warn that the nose worsens for a week or two after stopping, or the patient simply restarts the spray
• Stopping one side at a time is a practical strategy that many patients tolerate far better than stopping both
• Itching and sneezing suggest allergy, whereas blockage and watery discharge dominate non-allergic disease
• Physical triggers point to vasomotor rhinitis — cold air, strong smells, alcohol, emotion and spicy food
• Ipratropium is the drug for watery rhinorrhoea, blocking the parasympathetic secretomotor drive directly
• Oral antihistamines are of little value in non-allergic rhinitis, though topical azelastine has an effect beyond antihistamine action
• Improvement at weekends suggests occupational rhinitis.
• Recognising occupational rhinitis matters beyond the nose.
• Rhinitis of pregnancy resolves after delivery and should be managed with saline and posture rather than decongestants
• Check thyroid function where rhinitis is unexplained; hypothyroidism causes mucosal congestion
• Review antihypertensives as a cause; beta-blockers, ACE inhibitors and methyldopa all cause nasal congestion

Nasal Fractures

• The nasal bones are the most commonly fractured bones of the face, because the nose projects and its upper third is bone with a thin, unsupported lower part.
• Assessment is clinical rather than radiological.
• Mechanism determines the deformity — a lateral blow (the commonest) causes deviation to the opposite side, often with depression of the near side; a frontal blow causes flattening and splaying of the nasal bones, and if severe telescoping with a saddle deformity and telecanthus

Other Nasal and Facial Trauma

• Septal fracture and dislocation frequently accompany a nasal fracture and are the commonest reason a manipulated nose remains obstructed — the bones are straightened but the septum is not.
• Soft tissue injuries — irrigate, debride minimally given the excellent blood supply, and close in layers ensuring cartilage is covered; give tetanus prophylaxis and antibiotics for contaminated wounds
• Maxillofacial fractures classified by LE FORT — I: a horizontal fracture above the teeth, giving a mobile upper dental arch ("floating palate"); II: a pyramidal fracture through the nasal bones, orbital floors and maxilla, giving a mobile midface with the nose; and III: craniofacial dysjunction, separating the facial skeleton from the skull base — all of which may be associated with CSF leak and airway compromise
• Naso-orbito-ethmoid (NOE) fractures are the serious ones for the nose: they cause telecanthus (an increased intercanthal distance from disruption of the medial canthal tendon), a flattened nasal bridge, and frequently CSF leak and lacrimal injury; and require open reduction and canthal fixation
• Orbital blow-out fracture — giving diplopia on upgaze, enophthalmos and infraorbital numbness; the white-eyed trapdoor fracture in a child is a surgical emergency because the trapped muscle becomes ischaemic
• Warn against nose-blowing after any midfacial fracture.
• Late deformities — a saddle nose (from septal cartilage loss after haematoma, abscess or over-resection); a deviated external nose; persistent obstruction; and synechiae. These are corrected by septorhinoplasty with cartilage or bone grafting, ideally deferred at least 6 months and, in children, until facial growth is complete
• Consider non-accidental injury in a child, and domestic violence in an adult with recurrent or inconsistent facial injury — and ask
• Do not order plain nasal radiographs; they are frequently wrong in both directions and change nothing
• The decision to manipulate rests on appearance and airway, judged clinically rather than radiologically
• Palpate the septum in every broken nose; a haematoma is soft and bilateral and is missed by inspection alone
• Assess within hours or wait a week; a grossly swollen nose at forty-eight hours cannot be judged at all
• Manipulate within ten to fourteen days in an adult and seven to ten in a child, before the fracture unites
• A lateral blow deviates and a frontal blow flattens, which is why the mechanism predicts the deformity
• The septum is why a manipulated nose stays blocked, so it must be assessed and addressed separately from the bones
• Exclude CSF rhinorrhoea after any midfacial injury, and confirm with beta-2 transferrin rather than a glucose stick
• Assess the eye and conscious level first; the associated injuries matter more than the nasal fracture
• Warn against nose-blowing after a midfacial fracture, which forces air and organisms into the orbit and cranial cavity
• Telecanthus indicates a naso-orbito-ethmoid fracture, from disruption of the medial canthal tendon, and needs open fixation
• A white-eyed trapdoor fracture in a child is a surgical emergency despite the unimpressive appearance
• Photograph the injury and document the mechanism; assault is a common cause and litigation frequently follows
• Warn that a perfect result is not guaranteed before manipulating; a proportion need later septorhinoplasty
• Defer septorhinoplasty at least six months, and until facial growth is complete in a child
• Ask about domestic violence and non-accidental injury where facial injuries are recurrent or the history is inconsistent

Approach and the Infective Granulomas

• Granulomatous diseases OF the nose are conditions producing chronic granulomatous inflammation of the nasal mucosa, with crusting, nasal obstruction, discharge and tissue destruction.
• They are important in India, where the infective causes remain common.
• The presentation IS remarkably similar whatever the cause.

The Non-infective Granulomas and Mimics

• Granulomatosis with polyangiitis (formerly wegener) is the most important non-infective cause, and the nose is frequently where it first declares itself
• It is a necrotising vasculitis with granulomas, affecting the upper airway, lungs and kidneys — the classical triad
• Nasal features — persistent crusting and blood-stained discharge that fails to respond to treatment; septal perforation and saddle deformity; obstruction; and friable, granular mucosa that bleeds on touch
• Systemic features to seek — haemoptysis and pulmonary infiltrates or cavities; glomerulonephritis with haematuria and a rising creatinine.
• Diagnosis — c-ANCA with PR3 specificity.
• Treatment is immunosuppression — corticosteroid with cyclophosphamide or rituximab for induction, then maintenance with azathioprine or methotrexate; with co-trimoxazole.
• Nasal care — douching and crust removal, and surgery only when the disease is quiescent.
• Sarcoidosis — non-caseating granulomas; nasal crusting and obstruction with submucosal nodules giving a "strawberry skin" appearance on the inferior turbinate; with lupus pernio, a violaceous plaque on the nose and cheeks. Raised serum ACE and calcium, hilar lymphadenopathy on chest imaging; treated with steroid
• NK/T-cell lymphoma, nasal type (formerly "lethal midline granuloma") — an EBV-associated malignancy causing relentless midfacial destruction. It is commoner in Asian and South American populations, and must not be treated as an infection. Diagnosed by repeated deep biopsy with immunohistochemistry (CD56 positive) and EBV testing; treated with radiotherapy and chemotherapy
• Rhinoscleroma and rhinosporidiosis are considered separately, but both belong in this differential
• The mimics that must not be forgotten — cocaine and inhaled drug abuse.
• Ask about cocaine use directly and non-judgementally in any midfacial destructive lesion; patients rarely volunteer it and the diagnosis is otherwise repeatedly missed
• They all look the same, so biopsy decides; appearance alone cannot separate infection, vasculitis, lymphoma and carcinoma
• Take a deep and generous biopsy; superficial specimens show only non-specific inflammation and waste the procedure
• Repeat a negative biopsy where suspicion remains, particularly in vasculitis and lymphoma
• Send for fungal and mycobacterial culture as well as histology, or the treatable infections will be missed
• Tuberculosis and leprosy perforate cartilage, whereas syphilis destroys the bony septum, which is a useful discriminator
• The nose is an early site in lepromatous leprosy and a major route of transmission.
• Nasal tuberculosis is secondary to pulmonary disease, so the chest must be imaged in every case
• Check urinalysis and creatinine in every suspected vasculitis; the kidney determines the prognosis and is silent early
• C-ANCA may be negative in limited disease, so a negative result does not exclude granulomatosis with polyangiitis
• Operate only when vasculitis is quiescent; surgery on active disease fails and may worsen the destruction
• Co-trimoxazole reduces relapse in limited upper airway disease, and is a cheap addition to immunosuppression
• Strawberry skin on the turbinate suggests sarcoidosis, with lupus pernio on the nose and cheeks
• Relentless midfacial destruction suggests lymphoma, which must not be treated as an infection while it advances
• NK/T-cell lymphoma is EBV-associated and commoner in Asian populations, and needs immunohistochemistry to diagnose
• Ask about cocaine directly and without judgement; it mimics vasculitis exactly and may even give a positive ANCA
• Chronic steroid spray misuse perforates the septum, and is a mimic that is corrected simply by changing the technique
• Carcinoma is the reason biopsy is mandatory, since it may present as a crusting non-healing lesion like any of these
• Mucormycosis is an angioinvasive emergency, and a black eschar in a diabetic must be acted on the same day
• Lupus vulgaris shows apple-jelly nodules on diascopy, and is the indolent cutaneous form of tuberculosis

Nature and Pathogenesis

• Acute rhinosinusitis is inflammation of the nose and paranasal sinuses of less than 12 weeks duration, with complete resolution of symptoms.
• The term rhinosinusitis is preferred to "sinusitis", because the nasal and sinus mucosa are continuous and are always involved together.
• The pathogenesis IS A sequence that should BE recited.

Clinical Features and Management

• Diagnosis is clinical, and requires at least two symptoms, one of which must be nasal blockage or discharge:
• Nasal blockage or obstruction
• Nasal discharge, anterior or posterior, characteristically purulent
• Facial pain or pressure
• Reduction or loss of smell
• The site of pain suggests the sinus — maxillary: cheek pain, referred to the upper teeth; frontal: forehead pain, classically worse in the morning and easing by afternoon; ethmoid: pain between and behind the eyes, with periorbital oedema; sphenoid: deep-seated vertex or occipital pain.
• Examination — tenderness over the affected sinus; pus in the middle meatus on anterior rhinoscopy or endoscopy.
• Investigation IS not routinely required.
• Plain radiographs should not be used — they are insensitive, non-specific, and mucosal thickening is present in many normal people and in almost anyone with a cold
• CT is indicated only where there is suspected complication, failure to respond to treatment, immunocompromise, or planned surgery — not for diagnosis
• Management of viral and mild disease — symptomatic treatment alone: analgesia, saline irrigation, steam inhalation, adequate fluids and rest, and topical decongestant for NO more than 5 to 7 days
• Intranasal corticosteroid has good evidence in acute rhinosinusitis, reducing symptoms and speeding recovery, and is appropriate as first-line treatment in moderate disease
• Watchful waiting with symptomatic treatment is appropriate for the first 7 to 10 days in an otherwise well patient
• Antibiotics are indicated for — severe symptoms (high fever, severe pain); symptoms persisting beyond 10 days without improvement; double sickening; suspected complication; and immunocompromise or significant comorbidity
• First-line antibiotic is amoxicillin, or amoxicillin-clavulanate where there is recent antibiotic use, treatment failure or a high local resistance rate; with doxycycline or a macrolide in penicillin allergy, guided by local sensitivity. A course of 5 to 10 days is usual
• Adjuncts — mucolytics, whose evidence is weak; and antihistamines only where there is underlying allergy.
• Surgery is rarely needed in acute disease, and is reserved for a complication, or an unresolving collection — antral washout or endoscopic drainage
• The red flags demanding urgent assessment and imaging — periorbital swelling, redness or displacement of the eye; diplopia, reduced visual acuity or an afferent pupillary defect; severe unilateral or bilateral frontal headache; frontal swelling; signs of meningitis; and any neurological sign or reduced conscious level
• These features mean the patient is referred the same day, because orbital and intracranial complications progress in hours
• Double sickening is the most useful single sign, meaning improvement followed by deterioration after five or six days
• Most acute rhinosinusitis is viral and needs no antibiotic.
• Do not order plain sinus radiographs; mucosal thickening appears in almost anyone with a cold and changes nothing
• Pus in the middle meatus is the most useful sign, and is seen on anterior rhinoscopy or endoscopy after decongestion
• Two symptoms are required, one being blockage or discharge.
• The maxillary sinus is most often affected, because its ostium sits high and drainage depends entirely on cilia
• Sphenoid pain is vertex or occipital and deep-seated, and is the presentation most often misattributed
• Frontal pain is classically worse in the morning and eases through the afternoon as the sinus drains
• Maxillary pain refers to the upper teeth, which sends many patients to a dentist before an ENT surgeon
• Topical steroid has good evidence in acute disease, reducing symptoms and speeding recovery without an antibiotic
• Use decongestants for five to seven days at most, or rhinitis medicamentosa is created while treating the sinusitis

Definition and Phenotypes

• Chronic rhinosinusitis (CRS) is inflammation of the nose and paranasal sinuses lasting 12 weeks or more, with at least two symptoms — one being nasal blockage or discharge — together with objective evidence on endoscopy or CT.
• The objective requirement matters.
• The four cardinal symptoms are nasal blockage, nasal discharge (anterior or postnasal), facial pain or pressure, and reduction or loss of smell — with at least one of blockage or discharge required

Assessment and Management

• Assessment — a full history with duration, the four symptoms, and their impact; and specific enquiry about asthma, aspirin sensitivity, allergy, smoking and previous surgery
• Nasal endoscopy in every case, which is what converts a symptom complex into a diagnosis
• CT of the sinuses in the coronal plane, but performed after a course of maximal medical treatment, so it shows persistent rather than reversible disease — and interpreted with the symptoms, never alone
• Investigate the contributing factors — allergy testing; immunoglobulins and vaccine responses where infections are recurrent; sweat chloride and ciliary studies in a child or where suggested; ANCA where there is crusting or destruction; and dental assessment in unilateral maxillary disease
• Medical treatment IS the foundation, and surgery IS for its failure
• 1. Intranasal corticosteroid is the mainstay, used long-term and regularly. Technique and adherence determine success, and should be checked before the drug is changed
• 2. Saline irrigation, in high volume.
• Steroid can be added to the irrigation fluid in refractory disease, delivering far more drug to the sinus mucosa than a spray
• 3. A course of oral corticosteroid for severe polyposis or a marked exacerbation — highly effective, but limited by systemic effects and not for repeated long-term use
• 4. Antibiotics — a short course for an acute exacerbation, guided by culture; and low-dose long-term macrolide (such as clarithromycin for 12 weeks) in CRSsNP with normal IgE, acting through an anti-inflammatory and immunomodulatory rather than antibacterial effect
• 5. Treat the contributors — allergy, asthma, reflux, dental disease; and stop smoking, without which little else works
• 6. Leukotriene antagonists in aspirin-exacerbated disease; and aspirin desensitisation in selected patients with AERD.
• 7. Biologics — the major recent advance: dupilumab (anti-IL-4/13), omalizumab (anti-IgE) and mepolizumab (anti-IL-5) for severe type-2 CRSwNP.
• Surgery — functional endoscopic sinus surgery (FESS) is indicated where maximal medical treatment for an adequate period has failed, and for complications, mucocele, fungal ball and suspected neoplasm
• Its principle is to restore ventilation and natural drainage by relieving obstruction at the osteomeatal complex, allowing the diseased mucosa to recover — hence mucosa is preserved, not stripped
• Surgery does not cure the underlying inflammation: medical treatment must continue afterwards, and a patient who stops their steroid spray after surgery will relapse — which must be explained before the operation, not after
• Postoperative care determines the result — douching, debridement of crusts, and topical steroid
• Counsel realistically — smell recovers least reliably, and polyps recur in a substantial proportion, particularly with asthma and aspirin sensitivity
• Twelve weeks is the dividing line between acute and chronic disease, and it should be stated in the definition
• Objective findings are required, on endoscopy or CT.
• Facial pain alone is not sinusitis, and is midfacial segment pain or migraine
• Polyps mean blockage and anosmia, whereas disease without polyps gives pain and discharge
• Type-2 eosinophilic disease responds to steroid and to biologics, whereas neutrophilic disease responds to surgery and macrolides
• Ask every patient about asthma and aspirin; the triad changes the prognosis and the treatment entirely
• Scan after medical treatment, not before, so the CT shows persistent rather than reversible disease
• Read the scan with the symptoms; incidental mucosal thickening is common and treating a scan helps nobody
• High-volume saline irrigation genuinely works and should be done before the steroid spray, not after
• Check spray technique before changing the drug; most apparent failure is technique or adherence

Nature and Pathology

• Nasal polyps are benign, pedunculated, oedematous masses of prolapsed, inflamed sinonasal mucosa.
• They are not tumours and not a disease in themselves, but the end result of chronic mucosal inflammation — which is why removing them without treating the inflammation guarantees recurrence.
• They arise chiefly from the ethmoid air cells and the middle meatus, prolapsing into the nasal cavity through the osteomeatal complex

Clinical Features and Management

• Symptoms — progressive bilateral nasal obstruction.
• Massive polyposis may cause broadening of the nasal bridge and hypertelorism — the so-called frog-face deformity — and polyps may become visible at the nostril
• The critical clinical discrimination IS bilateral versus unilateral:
• Bilateral polyps are usually inflammatory and benign
• A unilateral nasal mass is not a simple polyp until proved otherwise, and must be imaged and biopsied — the differential being antrochoanal polyp, inverted papilloma, angiofibroma, malignancy, encephalocele and a fungal ball
• The features that should alarm — unilateral disease; bleeding or a friable, fleshy appearance; pain; orbital signs, proptosis or diplopia; cranial nerve involvement or facial numbness; and bone erosion on imaging
• Investigation — nasal endoscopy; CT of the sinuses to assess extent, the osteomeatal complex, the skull base and any bone erosion; MRI where a tumour, encephalocele or intracranial extension is suspected; allergy testing; and sweat chloride in a child
• Histology of everything removed, without exception
• Medical treatment first:
• Topical intranasal corticosteroid, long-term, which is the mainstay and shrinks polyps, improves obstruction and delays recurrence. Drops reach the ethmoids better than sprays and are used with the head-down position in significant polyposis
• A short course of oral corticosteroid ("medical polypectomy").
• High-volume saline irrigation; treatment of allergy and asthma; leukotriene antagonists in aspirin-exacerbated disease; and antibiotics only for acute infective exacerbations
• Biologics — dupilumab, omalizumab and mepolizumab for severe, recurrent type-2 polyposis.
• Surgery — endoscopic polypectomy with FESS, indicated for failure of medical treatment, complete obstruction, complications, and diagnostic uncertainty
• The operation must address the ethmoids, not merely remove the visible polyps — simple polypectomy alone has a very high recurrence rate.
• Medical treatment must continue after surgery, indefinitely; surgery removes polyps but does not treat the inflammation that makes them
• Counsel about recurrence explicitly — it is common, particularly with asthma, aspirin sensitivity and eosinophilic disease, and a patient who was promised a cure will be justifiably dissatisfied
• Polyps in a child mean cystic fibrosis until the sweat test says otherwise; they are rare in childhood
• A unilateral polyp is a tumour until histology says otherwise, and must be imaged and biopsied rather than simply removed
• Polyps are pale, insensitive and do not bleed, which distinguishes them from every dangerous differential
• They are a symptom of inflammation, not a disease, which is why removing them without continuing steroid guarantees recurrence
• Samter triad is polyps, asthma and aspirin sensitivity, and these patients have the most aggressive and recurrent disease
• Aspirin must be avoided absolutely in that group, since a single dose can precipitate severe bronchospasm
• Leukotriene antagonists suit aspirin-exacerbated disease, which follows directly from the shunting of arachidonic acid
• Eosinophils in the usual type and neutrophils in cystic fibrosis.
• Anosmia is the least reversible symptom and frequently the most distressing, and should be discussed honestly
• Steroid drops reach the ethmoids better than sprays, used with the head down, in significant polyposis
• Oral steroid is a medical polypectomy and may restore smell, but cannot be repeated indefinitely
• Biologics have changed the outlook for the worst-affected, shrinking polyps and restoring smell where surgery could not
• Address the ethmoids, not just the visible polyps; simple polypectomy alone recurs at a very high rate
• Continue medical treatment indefinitely after surgery, and say so before operating rather than afterwards
• Send everything removed for histology, without exception, whatever the operative appearance suggested

Orbital Complications

• Complications of sinusitis arise when infection spreads beyond the sinus, by direct extension through thin or dehiscent bone, by retrograde thrombophlebitis through valveless veins, or through preformed pathways.
• They are classified as orbital, intracranial and osseous.
• Orbital complications are the commonest, and arise chiefly from the ethmoid sinuses through the paper-thin lamina papyracea — which is why they are predominantly a disease of children, in whom the ethmoids are the developed sinus
• Chandler’S classification describes five progressive stages, and the essential division within it is between the first stage and all the rest:

Intracranial and Osseous Complications

• Intracranial complications arise chiefly from the frontal and sphenoid sinuses, and are predominantly a disease of adolescent males — an epidemiological pattern that is characteristic and worth stating, related to the vascularity of the developing frontal sinus
• They spread by retrograde thrombophlebitis through the valveless diploic veins of breschet in the posterior wall of the frontal sinus.
• Meningitis — the commonest, particularly from sphenoid and ethmoid disease
• Extradural, subdural (empyema) and intracerebral abscess — frontal lobe abscess being characteristic of frontal sinusitis, and notoriously silent, presenting with headache, personality change, drowsiness and vomiting rather than focal signs
• Subdural empyema is more often sinogenic than otogenic, spreads rapidly over the convexity, and has a high mortality
• Cavernous sinus thrombosis — from sphenoid or ethmoid disease, with proptosis, chemosis, ophthalmoplegia, a sixth nerve palsy earliest, and spread to the opposite eye.
• Superior sagittal sinus thrombosis
• Osseous complications — osteomyelitis of the frontal bone, giving POTT’S puffy tumour — a doughy, tender, fluctuant swelling of the forehead from a subperiosteal abscess over frontal osteomyelitis
• Pott’s puffy tumour is not a tumour and not a trivial swelling: it indicates osteomyelitis, and is associated with intracranial complications in a high proportion.
• Osteomyelitis of the maxilla occurs in infants
• Mucocele — considered separately, but belongs among the complications
• Investigation — contrast-enhanced CT of the sinuses, orbits and brain; with MRI.
• Lumbar puncture only after imaging, and never where there is a space-occupying collection
• Management — high-dose intravenous antibiotics crossing the blood-brain barrier (a third-generation cephalosporin with metronidazole), for weeks; neurosurgical drainage of any intracranial collection; and surgical drainage of the sinus.
• The organisms are frequently anaerobic or mixed, particularly Streptococcus anginosus (milleri) group, which has a particular tendency to form abscesses — hence the metronidazole
• The reason these complications are still seen is delayed presentation and partially treated infection, in which antibiotics blunt the clinical picture while the disease advances
• Visual acuity, eye movements and proptosis are the three findings that separate preseptal from postseptal disease
• A relative afferent pupillary defect demands surgery now, as does loss of red colour vision.
• Orbital complications come from the ethmoids through the lamina papyracea.
• Intracranial complications come from the frontal sinus and affect adolescent males.
• Infection reaches the brain through intact bone by retrograde thrombophlebitis, so a normal bony wall is no reassurance
• Frontal lobe abscess is notoriously silent, presenting with personality change and drowsiness rather than focal signs
• Pott puffy tumour means osteomyelitis, and the brain must be imaged because intracranial disease accompanies it more often than not
• Admit any child with periorbital swelling and fever, and review the vision hourly rather than daily
• Operate if there is no improvement in forty-eight hours on intravenous antibiotics, or sooner if vision deteriorates
• A small medial abscess in a young child may be observed, which is a recognised exception but demands close monitoring
• Drain the sinus as well as the collection; the sinus is the source and the complication recurs if it is left
• Give metronidazole, since the organisms are frequently anaerobic or mixed and the milleri group forms abscesses
• Image before any lumbar puncture, and never tap where there is a space-occupying collection

Benign Tumours

• The single most important principle: unilateral nasal obstruction, unilateral blood-stained discharge, or a unilateral mass in an adult is a tumour until proved otherwise — and must be endoscoped, imaged and biopsied
• Osteoma — the commonest benign bony tumour, most often in the frontal sinus. an incidental finding needing no treatment; removed if it obstructs drainage, causes a mucocele, or extends into the orbit or cranium. Gardner syndrome — multiple osteomas with colonic polyposis and soft tissue tumours — must be excluded where osteomas are multiple.

Malignant Tumours

• Squamous cell carcinoma accounts for the majority, arising most often in the maxillary sinus
• Adenocarcinoma arises characteristically in the ethmoid, and is strongly associated with hardwood dust exposure in furniture and woodworkers — a classical occupational association which must be asked about
• Other occupational associations — nickel refining (squamous carcinoma); chromium; leather work; and radium
• Other histologies — adenoid cystic carcinoma, notable for perineural spread, late distant metastasis and very long-term recurrence; olfactory neuroblastoma (esthesioneuroblastoma), arising from olfactory epithelium high in the nose, with a bimodal age distribution; mucosal melanoma.
• Presentation is late, because the tumour grows silently in an air-filled cavity until it breaches a wall — which is the fundamental reason for the poor prognosis
• Early symptoms mimic sinusitis — unilateral obstruction, blood-stained discharge and facial pain
• The features OF spread indicate the direction and are worth listing by wall: medial → nasal obstruction, epistaxis, mass; superior (orbital floor) → proptosis, diplopia, epiphora, visual loss; anterior → cheek swelling and infraorbital numbness.
• Unexplained loosening of teeth, an ill-fitting denture, infraorbital numbness or new trismus in an adult are the signs that should prompt imaging, and each is commonly attributed to something benign for months
• Ohngren’S line — a theoretical plane from the medial canthus to the angle of the mandible, dividing the maxilla into a superoposterior (suprastructure) and an anteroinferior (infrastructure) part. Tumours above the line have a worse prognosis, being close to the orbit, skull base and pterygoid region and presenting later
• Lymph node metastasis is uncommon at presentation.
• Investigation — endoscopy with biopsy.
• Do not biopsy before imaging where an angiofibroma or an encephalocele is possible
• Treatment is surgery with postoperative radiotherapy, decided by a multidisciplinary team
• Surgical options — endoscopic resection for selected tumours, which has grown considerably; medial maxillectomy, partial or total maxillectomy, with orbital exenteration where the orbit is invaded; and craniofacial resection for tumours involving the anterior skull base
• Reconstruction and rehabilitation matter greatly — an obturator or prosthesis after maxillectomy, free flaps, and dental, speech and swallowing rehabilitation
• Prognosis remains poor, with five-year survival for maxillary carcinoma of the order of 30 to 50%, chiefly because of late presentation
• The practical message for the general clinician is that the diagnosis is made early only by taking unilateral nasal symptoms seriously and looking with an endoscope
• Unilateral symptoms in an adult mean a tumour until endoscopy, imaging and biopsy have said otherwise
• Infraorbital numbness is an early sign of maxillary malignancy and is attributed to something benign for months
• Loosening teeth and an ill-fitting denture in an adult should prompt imaging rather than another dental adjustment
• New trismus indicates posterior spread into the pterygoid region, and is never a benign finding
• Inverted papilloma is fleshy and mulberry-like, quite unlike the pale glistening inflammatory polyp
• Drill the bone at the attachment site, since recurrence of inverted papilloma arises exactly there
• Five to ten per cent become squamous carcinoma, which is why inverted papilloma needs long-term endoscopic follow-up
• A cerebriform pattern on MRI suggests inverted papilloma, and helps separate it from carcinoma and retained secretion

Divisions and Structure

• The pharynx is a musculomembranous tube extending from the skull base to the lower border of the cricoid cartilage (the level of C6), where it becomes the oesophagus.
• It is 12 to 14 cm long and is divided into nasopharynx, oropharynx and hypopharynx.
• The nasopharynx extends from the skull base to the level of the soft palate; it is a respiratory passage, lined largely by ciliated columnar epithelium, and contains the adenoid, the eustachian tube openings and the fossa of rosenmuller
• The oropharynx extends from the soft palate to the level of the hyoid (the tip of the epiglottis); it is a shared food and air passage, lined by stratified squamous epithelium, and contains the palatine tonsils, the base of tongue with the lingual tonsil, the soft palate and the posterior pharyngeal wall
• The hypopharynx (laryngopharynx) extends from the hyoid to the lower border of the cricoid, and has three subsites — the pyriform fossae, the postcricoid region and the posterior pharyngeal wall
• The wall has four layers — mucosa, a fibrous pharyngobasilar fascia, a muscular layer, and the buccopharyngeal fascia
• The muscles are the three constrictors (superior, middle and inferior).
• The longitudinal muscles are the stylopharyngeus, salpingopharyngeus and palatopharyngeus.
• The inferior constrictor has two parts.
• Cricopharyngeus is the upper oesophageal sphincter — tonically contracted at rest and relaxing during swallowing
• Killian’S dehiscence is the potential gap between thyropharyngeus and cricopharyngeus on the posterior wall — an area of relative weakness through which a pharyngeal pouch herniates. It is the "gateway of tears" for the endoscopist, being the commonest site of perforation during rigid oesophagoscopy
• Nerve supply — the pharyngeal plexus, formed by the glossopharyngeal (IX), vagus (X) and sympathetic contributions
• Motor supply is from the vagus (through the cranial accessory) to all muscles except stylopharyngeus.
• Sensory supply — the nasopharynx by the maxillary nerve (V2); the oropharynx by the glossopharyngeal; and the hypopharynx by the vagus (internal laryngeal branch)
• The gag reflex has a glossopharyngeal afferent and a vagal efferent
• Blood supply is from branches of the external carotid — the ascending pharyngeal, ascending palatine and tonsillar branches of the facial, the greater palatine, and the dorsal lingual arteries

Waldeyer Ring, Swallowing and Applied Anatomy

• Waldeyer’S ring is the ring of subepithelial lymphoid tissue guarding the entrance to the aerodigestive tract, comprising the adenoid (nasopharyngeal tonsil) above, the tubal tonsils, the palatine tonsils laterally, the lingual tonsil below, and lateral pharyngeal bands
• It is part of mucosa-associated lymphoid tissue (malt), sampling inhaled and ingested antigen and producing secretory IgA; it is most active between 4 and 10 years and involutes thereafter
• The palatine tonsil is worth describing in detail, since it is the commonest object of surgery in the chapter
• It lies in the tonsillar fossa, bounded by the palatoglossal arch anteriorly, the palatopharyngeal arch posteriorly, and the superior constrictor laterally
• It has a capsule of condensed pharyngobasilar fascia, and a loose peritonsillar space between capsule and superior constrictor — which is the plane of surgical dissection and the space in which a quinsy forms
• It has 12 to 15 crypts.
• Its blood supply is chiefly from the tonsillar branch of the facial artery, entering at the lower pole, with the ascending pharyngeal, ascending palatine, dorsal lingual and greater palatine arteries. The lower pole is the site of the main bleeding at tonsillectomy
• Venous drainage is to the paratonsillar vein, a further source of bleeding
• Nerve supply is by the glossopharyngeal nerve and the lesser palatine branches — which explains referred otalgia after tonsillectomy and in tonsillar disease, through the tympanic branch of IX (jacobson’S nerve)
• Lymphatic drainage is to the jugulodigastric (tonsillar) node, at the angle of the mandible

Acute Tonsillitis

• Acute tonsillitis is acute inflammation of the palatine tonsils.
• It is predominantly a disease of children and young adults, and the central clinical task is to distinguish the viral majority from the bacterial minority.
• Organisms — the majority are viral (rhinovirus, adenovirus, influenza, epstein-BARR virus); the most important bacterial cause is group A beta-haemolytic streptococcus (Streptococcus pyogenes), which accounts for perhaps 15 to 30% in children and fewer in adults
• Types — acute catarrhal (part of a generalised pharyngitis); acute follicular, with yellowish exudate at the crypt openings, which is the classical appearance; acute parenchymatous, with a uniformly enlarged, congested tonsil; and acute membranous, in which the exudate coalesces into a membrane
• Features — sore throat and odynophagia; fever and malaise; referred otalgia via the glossopharyngeal nerve; halitosis; and, in children, abdominal pain and vomiting from mesenteric adenitis, which may dominate the presentation
• Signs — enlarged, congested tonsils with exudate; and tender, enlarged jugulodigastric nodes
• The CENTOR (or McISAAC) criteria estimate the probability of streptococcal infection, and are worth knowing precisely — tonsillar exudate; tender anterior cervical lymphadenopathy; fever (history of); and absence of cough — with a point added for age 3 to 14 and subtracted for age over 45 in the McIsaac modification
• The absence OF cough IS the point most often forgotten: a prominent cough, coryza, hoarseness or conjunctivitis points away from streptococcus and toward a virus
• Fewer criteria mean symptomatic treatment; more criteria justify a throat swab or empirical antibiotic, according to local policy
• Management — adequate analgesia, which is the most important and most neglected part of treatment; paracetamol and ibuprofen; fluids, with admission for dehydration or inability to swallow; rest; and salt-water gargles
• Antibiotic where streptococcal infection is likely — penicillin V or amoxicillin for 10 days, with a macrolide in penicillin allergy
• The full 10-day course matters, because the purpose is not merely symptom relief but eradication of the organism to prevent rheumatic fever — a point of real importance in India, where rheumatic heart disease remains common
• Avoid ampicillin and amoxicillin where infectious mononucleosis is possible.
• A short course of corticosteroid may be used for severe odynophagia or airway concern
• Complications:
• Suppurative (local) — peritonsillar abscess (quinsy); parapharyngeal and retropharyngeal abscess; acute otitis media; cervical adenitis and abscess; and lemierre’S syndrome — septic thrombophlebitis of the internal jugular vein with septic pulmonary emboli, caused by fusobacterium necrophorum, in a young adult with a sore throat who becomes systemically very unwell
• Non-suppurative (systemic, following streptococcal infection) — acute rheumatic fever.
• Also — scarlet fever, and PANDAS (paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection).

Chronic Tonsillitis and the Differential Diagnosis

• Chronic tonsillitis follows repeated acute attacks, or persistent low-grade infection within the crypts, with fibrosis and crypt obstruction
• Features — recurrent sore throat; persistent halitosis and a foul taste; tonsilloliths (crypt debris); chronic cervical lymphadenopathy; and irritable cough
• Signs — tonsils that may be large or small and fibrosed; persistent congestion of the anterior pillars.
• The size of the tonsils has NO relation to the frequency of infection — large tonsils in a healthy child are normal, and fibrosed, small tonsils may be the most infected. This point is consistently misunderstood by families
• The differential diagnosis of a membrane or exudate on the tonsil is the most examinable part of this topic:
• Infectious mononucleosis (glandular fever) — epstein-BARR virus in an adolescent or young adult, with marked malaise and fatigue, a thick grey-white membrane, generalised lymphadenopathy (especially posterior cervical), splenomegaly and hepatomegaly. Diagnosed by atypical lymphocytes on the film, a positive monospot or PAUL-bunnell test, and EBV serology
• Its management is supportive, with steroid for airway obstruction; NO ampicillin; and avoidance of contact sport for at least 4 to 6 weeks because of the risk of splenic rupture

Indications and Contraindications

• Tonsillectomy is surgical removal of the palatine tonsils with their capsule, dissecting in the peritonsillar plane between capsule and superior constrictor.
• It is one of the commonest operations in ENT and one of the most frequently performed without adequate indication.
• The indications divide into absolute and relative, and the distinction should lead the answer

Technique, Complications and Aftercare

• Preoperative assessment — document the indication and the episodes; check haemoglobin and clotting where indicated; take a family and personal bleeding history; examine and palpate the palate for a submucous cleft; and assess for sleep apnoea.
• Position and exposure — supine with the rose position (head extended, shoulders raised), a BOYLE-DAVIS gag on suspension, and a cuffed oral tube
• Techniques — cold steel dissection with a snare and ligation or bipolar haemostasis.
• The guillotine (Sluder) method is of historical interest
• Complications — haemorrhage is the most important, and its classification by timing governs management:
• Primary haemorrhage — during the operation; controlled by pressure, ligation or bipolar diathermy
• Reactionary (immediate secondary) haemorrhage — within the first 24 hours.
• Secondary haemorrhage — after 5 to 10 days, caused by infection and slough separation. It is managed with admission, antibiotics and observation, though a proportion need surgery
• The management OF A post-tonsillectomy bleed IS AN exam favourite and a real emergency: admit and resuscitate; large-bore intravenous access, blood for full blood count and cross-match; keep nil BY mouth; sit the patient up and encourage them to spit rather than swallow; ice and hydrogen peroxide gargles; antibiotics; and return TO theatre if bleeding is active or continues
• Children compensate well and then decompensate suddenly — tachycardia, pallor and repeated swallowing are the early signs, and a large volume may be swallowed before any blood is seen. Do not be reassured by a normal blood pressure in a child
• Other complications — pain and referred otalgia.
• Aftercare determines the experience — regular analgesia, prescribed by the clock rather than as required; paracetamol with ibuprofen.
• Encourage eating and drinking normally — a normal diet cleans the fossae and reduces infection, and the old advice to eat only soft food is wrong
• Warn the family that the throat will look white and sloughy, which is normal healing and not infection; that pain often peaks around day 5 to 7; and to attend immediately if there is any bleeding
• Know the Paradise numbers — seven in one year, five a year for two, or three a year for three — which is what makes an answer specific
• Episodes must be documented, not merely recalled, and each needs a sore throat plus fever, exudate, nodes or a positive culture
• Tonsillectomy is the biopsy where malignancy is suspected, and a unilateral tonsil is removed rather than sampled
• The benefit in mild disease is modest, two or three fewer sore throats in the first year.
• Watchful waiting with documentation is often the right first step.
• Palpate the palate before operating, since a submucous cleft means permanent velopharyngeal insufficiency after surgery
• Cold steel has the lowest secondary haemorrhage rate, while diathermy is faster but more painful afterwards
• Intracapsular tonsillotomy suits obstruction, not infection.
• Reactionary bleeding is within twenty-four hours and needs theatre, as vessels in spasm reopen
• Secondary bleeding is at five to ten days from slough separation, and is managed with antibiotics and observation
• Sit the patient up and let them spit; swallowed blood causes vomiting and hides the true volume lost
• Children compensate and then crash, so watch the pulse and swallowing rather than the blood pressure
• Cross-match early in a post-tonsillectomy bleed rather than waiting to see whether it settles
• Prescribe analgesia by the clock, not as required.
• Ibuprofen is safe and does not increase bleeding, and combining it with paracetamol is better than either alone
• Codeine is contraindicated in children after adenotonsillectomy for sleep apnoea.

Peritonsillar Abscess (quinsy)

• Deep neck space infections are collections of pus within the potential spaces bounded by the layers of deep cervical fascia.
• They matter because these spaces communicate with each other and with the mediastinum, so infection can spread rapidly and threaten the airway and life.
• A peritonsillar abscess (quinsy) is a collection of pus in the peritonsillar space, between the tonsillar capsule and the superior constrictor, at the superior pole
• It is the commonest deep neck infection, in young adults, and follows acute tonsillitis or arises from infection in WEBER’S glands in the supratonsillar region
• Organisms are mixed — group A streptococcus with anaerobes, particularly Fusobacterium
• The classical features, each of which should be named — severe unilateral sore throat and odynophagia; trismus, from irritation of the pterygoid muscles.
• Signs — unilateral bulging of the soft palate and anterior pillar; the tonsil displaced downward and medially; the uvula deviated to the opposite side; and ipsilateral cervical lymphadenopathy
• The differential — severe unilateral tonsillitis without abscess (peritonsillitis); a parapharyngeal abscess; infectious mononucleosis; a tonsillar or supratonsillar malignancy.
• Management — needle aspiration or incision and drainage under local anaesthesia, at the point of maximum bulge, or at the junction of a line from the base of the uvula to the last molar tooth
• Aspirate first with a needle, which confirms pus and excludes a vascular lesion, and guard the needle or blade so that it cannot pass more than 1 cm — because the internal carotid lies 2.5 cm laterally
• Plus — intravenous antibiotics covering streptococci and anaerobes (benzylpenicillin with metronidazole, or co-amoxiclav); analgesia; fluids; and a single dose of corticosteroid, which reduces pain and trismus and speeds recovery
• Interval tonsillectomy at 6 weeks is considered, particularly for a second quinsy, a history of recurrent tonsillitis, or airway compromise; a first quinsy in a patient with no prior history does not automatically require it.
• "hot" (immediate) tonsillectomy is practised in some centres, draining the abscess and removing the tonsil at once

Parapharyngeal, Retropharyngeal and Other Spaces

• The retropharyngeal space lies between the buccopharyngeal fascia and the prevertebral fascia, from the skull base to the mediastinum
• Acute retropharyngeal abscess is a disease of children under 5 years, arising from suppuration of the retropharyngeal lymph nodes (nodes of Rouvière).
• Features — fever and irritability, refusal of feeds, drooling, a muffled cry, neck stiffness and hyperextension of the neck (an important sign), stridor and torticollis
• A soft, unilateral bulge of the posterior pharyngeal wall is seen; a lateral soft-tissue neck radiograph shows widening of the prevertebral soft tissue (taken in inspiration with the neck extended to avoid a false positive), with contrast CT as the definitive investigation
• Chronic retropharyngeal abscess arises from tuberculosis of the cervical spine, is midline, and is drained externally, never through the mouth, with antitubercular treatment
• The parapharyngeal (lateral pharyngeal) space is an inverted cone from the skull base to the hyoid, divided by the styloid process into an anterior (prestyloid, muscular) and a posterior (poststyloid, neurovascular) compartment
• The posterior compartment contains the carotid sheath and cranial nerves IX to XII and the sympathetic chain — so infection there gives HORNER syndrome, cranial nerve palsies, carotid ERosion and internal jugular thrombosis
• Sources — tonsil, pharynx, teeth, parotid, and spread from other spaces
• Features — trismus, a tender swelling below the angle of the mandible, medial displacement of the lateral pharyngeal wall and tonsil, fever and toxicity
• LUDWIG’S angina is considered separately
• The complications that make these infections dangerous — airway obstruction.
• Management principles common to all — secure the airway first.
• The most dangerous error IS attempting intubation OR instrumentation IN A compromised airway without preparation — a partially obstructed airway can be lost completely, and the equipment for a surgical airway must be open and ready before induction
• Trismus separates quinsy from tonsillitis, and is the single most useful sign at the bedside

Nature and Consequences

• The mechanism — during sleep the pharyngeal dilator muscles (chiefly genioglossus) lose tone, and the pharynx, having NO rigid skeleton, collapses under the negative inspiratory pressure. Obstruction is worst in REM sleep, when muscle atonia is greatest, and when supine
• The cycle repeats: obstruction → hypoxia and hypercapnia → increasing respiratory effort → arousal (not remembered) → airway reopens → sleep resumes → obstruction — often hundreds of times a night

Assessment and Management

• History — from the patient and, crucially, from the partner.
• From the patient — unrefreshing sleep, morning headache, dry mouth, nocturia, poor concentration, irritability, reduced libido, and daytime sleepiness
• Ask about driving and occupation — this is a legal and safety obligation, not an optional question. Patients must be advised not to drive when sleepy and, where relevant, to inform the licensing authority
• The epworth sleepiness scale quantifies subjective sleepiness (8 situations, scored 0 to 3, maximum 24; above 10 is abnormal) — but it correlates poorly with severity and is a screening aid, not a diagnostic test
• The stop-BANG questionnaire is a useful screening tool — Snoring, Tiredness, Observed apnoea, blood Pressure, BMI, Age, Neck circumference, Gender
• Examination — BMI and neck circumference; blood pressure; nasal examination; the modified mallampati grade; tonsil size; retrognathia and dental occlusion; tongue size; and flexible nasendoscopy with the MULLER manoeuvre to assess the level of collapse
• Drug-induced sleep endoscopy (DISE) assesses the site and pattern of collapse under sedation, and is used to select patients for surgery
• Polysomnography is the gold standard, giving the apnoea-hypopnoea index (AHI) — the number of events per hour: mild 5 to 15, moderate 15 to 30, severe above 30; with oxygen desaturation indices and sleep architecture
• Home sleep apnoea testing is adequate for many uncomplicated adults and is far more accessible
• Check TSH, and consider acromegaly where the features suggest it
• Management — conservative measures apply to everyone: weight loss.
• Continuous positive airway pressure (CPAP) is the treatment of choice for moderate and severe disease — it acts as a pneumatic splint holding the pharynx open
• It is highly effective, improving sleepiness, blood pressure and accident risk; but adherence is the limiting factor, and depends on mask fit, humidification, treating nasal obstruction, and proper follow-up and support — which is where most CPAP failure actually originates
• Mandibular advancement devices — for mild to moderate disease, simple snoring, and CPAP intolerance; they advance the mandible and tongue base, and require adequate dentition
• Surgery is for selected patients, chiefly where there is a correctable anatomical obstruction or CPAP has genuinely failed:
• Adenotonsillectomy, which is the first-line treatment in children and is highly effective; and is useful in adults with large tonsils
• Nasal surgery (septoplasty, turbinate reduction), which rarely cures OSA but improves CPAP tolerance — an important and honest distinction
• Uvulopalatopharyngoplasty (UPPP) and modern palatal procedures, whose results are variable and depend heavily on patient selection
• Tongue base procedures, maxillomandibular advancement (which is highly effective but major surgery), and hypoglossal nerve stimulation, a newer option for selected patients
• Tracheostomy, which is curative by bypassing the obstruction and is reserved for life-threatening disease where nothing else is tolerated
• Warn about anaesthetic risk — patients with OSA are sensitive to opioids and sedatives and need careful postoperative monitoring.
• Ask the partner, not just the patient; witnessed apnoeas and choking arousals cannot be self-reported
• Ask about driving and occupation, which is a safety and legal obligation rather than an optional question
• Respiratory effort is preserved, which is what distinguishes obstructive from central apnoea
• The pharynx has no rigid skeleton, which is why it collapses when dilator tone is lost in sleep