Aural toilet is the key to treatment. If severe, the meatus is narrowed. A thin Pope wick c an be inserted and hydrated with eardrops, eg Sofradex (framy- cetin + dexametasone). An alternative is a strip of ribbon gauze soaked in ich- thammol glycerin (very soothing) or a luminum acetate (astringent). After a few days, the meatus will open up enough for either m icrosuction or careful cleansing with cotton wool. Commercial Qtips or cotton buds should n \200\231t be used: they are too large; instead thin out or make one yourself by wrapping a small piece of cotton wool gently around an orange stick. Use drops only short-term, as t roublesome fungal infections can arise. Non-specialists should not syringe the ear. s e s a e s i d T N E \200\207Beware persistent unilateral otitis externa in diabetics/immunosup- pressed/ the e lderly: the risk is malignant/necrotizing otitis externa (see BOX). \200\207OE which is re sistant to treatment can be a sign of malignancy. Do biopsy. Furunculosis This is a very pa inful staphylococcal abscess arising in a hair follicle. Pathologically it is identical to a boil anywhere else; if there is cellulitis consider \202 ucloxacillin. Diabetes is an i mportant predisposing factor. Bullous myringitis These are very painful haemorrhagic bliste rs on deep meatal skin and on the drum ( serosanginous \202 uid behind it). Classically as- sociated with in \202 uenza infection, but Mycoplasma pneumoniae has also been implic ated as have a variety of other organisms. It may simply represent a variant of acute otiti s media. Sensorineural hearing loss is much more frequent than previously thought. Treatmen t is generally supportive only, eg pain relief; oral antibiotics can be considered in cases with middle ear eff usions. Barotrauma (aerotitis) If the Eustachian tube is occluded, mid dle ear pres- sure cannot be equalized during descent in an aircraft or diving, so causing damage. Risk factors: Conditions inhibiting function of the Eustachian tube eg in \202 amm ation/infection. Symptoms: Severe pain as the drum becomes indrawn, eg from transudation or bleeding into middle ear. Barotrauma to the inner ear causes vertigo, tinnitus, and deafne ss. Prevention: Not \202 ying with a URTI, de- congestants into the nose (eg xylometazoli ne every 20min), repeated yawns, swallows/jaw movements. In \202 ating an Otovent device is eff ective (it is recom- mended to air passengers with problems clearing the ears.)29 P ositive pressure (higher than Eustachian tube resistance) through mask to the nasopharynx c an also help.30 \200\224: Supportive if simple barotrauma; eff usions usually clear sponta ne- ously, and most perforations heal. Temporomandibular joint (TMJ) dysfunction Symptoms: Earache, facial pain, and joint clicking/popping related to malocclusion, teeth-grinding (b rux- ism) or joint derangement, and, importantly, stress, making this a biopsycho- social d isorder which may become a chronic pain syndrome (p636).31,32 Signs: Joint tenderness exace rbated by lateral movement of the open jaw, or trigger points in the pterygoids. Imaging: M RI. Associations: Depression;33 Ehlers \200\223 Danlos, p642.3 4 \200\224: NSAIDs (PO or t opical, eg diclofenac);35 stabilizing orthodontic occlusal prostheses;36 cognitive therapy (p374); physiotherapy; biofeedback; surgery (eg reconstruction),37 acupuncture.32 543 Performing aural toilet (learn by watching an expert at work) \200 Tools: otoscope, cotto n wool, Jobson Horne probe, Crocodile forceps, which must be used under a direct headlight. Pull the pinna back and up to straighten the canal, and under direct vision, dry mop with J obson Horne probe with a small piece of cotton wool on its serrated end. \200 Clean the a uditory meatus with a gentle rotary action; don \200\231t touch the drum. \200 Replace th e cotton wool as becomes soiled. Attend to the anterior-inferior recess, which often harbors debris. Intermittently re-examine the meatus, using the otoscope, during cleaning. \200 Patients who have mastoid cavities (a surgical widened canal done to treat infec- tion) should be fo llowed up in the ENT department for irrigation and drying. Cavi- ties repeatedly infected m ay need surgical repair of the posterior wall. s e s a e s i d T N E Take swabs as needed and advise on ear care. Referred otalgia: when the cause is not in the ear Referred (secondary) pain can arise from disease processes in the territories of the sensory nerves supplying the ear: \200 V: Th e auriculotemporal nerve (a branch of the trigeminal nerve which sup- plies lateral upper h alf of pinna) may refer pain from dental disease and TMJ dysfunction (temporomandibular joi nt dysfunction, opposite). VII: A sensory branch of the facial nerve (supplies lateral surface of drum) refers pain in geniculate herpes (Ramsay Hunt syndrome, p652). IX: Primary glossopharyngeal neuralgia is a rare cause of pain often in- duced by talking or swallowing. \200 IX & X: The tympanic branch of the glossopharyngeal nerve and the au- ricular branch of the vagus (supplies medial surface of drum) refer pain from the cancer of the posterior \205\223 of the tongue, pyriform fossa, or larynx, or fro m the throat to the ear, eg in tonsillitis or quinsy. Otalgia is common post-tonsillectomy (esp. in adults), so it is worth warning all patients. \200 c2, 3: The great auricular ne rve (C2, 3, supplying lower of pinna) refers pain from soft tissue injury in the neck an d from cervical spondylosis/arthritis. Use of eardrops in otitis externa \200\207Take a swab \201 rst, and do aural toilet. Is there chronic otitis externa that needs specialist attention? If not and no perforation, tr y local drops: \200 Betamethasone 0.1% cheap and good for non-infected eczema. \200 Fra mycetin + dexametasone = Sofradex . \200 Polymyxin B + neomycin+hydrocortisone=Otosporin . \200 Gentamicin 0.3%+hydrocortisone 1% = Gentisone HC drops; more costly than betam ethasone but can be good: advised by some only as a 2nd-line agent, eg in Pseudomonas infec tions. To minimize risks of gentamicin oto- toxicity, use for <2wks, and after baseline aud iometry (if possible); explain risks and bene \201 ts of gentamicin esp. if tympanic perfo ration. 38 \200 Clioquinol 1% + \202 umetasone=Locorten-Vioform , not if iodine sensit ive). \200 Sodium bicarbonate 5% drops are useful if wax is an additional problem. \200 \200\207Over-treating may lead to fungal infection: if suspected, try clotrimazole 1% sol ution/8 \200\22312h, continued for \211 2weeks after disappearance of infection. Malignant/necrotizing otitis externa This is aggressive, life-threatening infection of the external ear that can lead to temporal bone destruction and base-of-skull osteomyelitis. 90 % are diabetic. Immunosuppression (and probably aural irrigation)39 are other risk factors. The cause is usually Pseudomonas aeruginosa (also Proteus and Klebsiella). Treatment is by surgical debridement, systemic antibiotics, speci \201 c immuno- globulins, and, possibly , hyperbaric oxygen therapy.40 Discharging ears and otitis media 544 \200 The character of the discharge provides clues Causes of discharge to consider \200 Otitis externa/media. \200 Cholesteatoma to its source and cause: \200 External ear: In \202 ammation, ie otitis externa (OE) pro duces a scanty watery discharge, as there are no mucinous glands \200\224see p542. Blood ca n result from trauma to the canal. Liquid wax can sometimes \200\230leak \200\231 out. (rare but imp ortant). NB: crust in the attic/postero-superior quadr- ant often hides patho logy, so remove it. Middle ear: Mucous discharges are almost always due to middle ear disease. Serosanguinous d ischarge suggests a granular mucosa of chronic otitis media (COM). An off ensive discharge suggests cholesteatoma. CSF otorrhoea: CSF leaks may follow trauma: suspect if you see a halo sign on \201 lter p aper, or its glucose is \200\200, or \200 2 (tau) transferrin is present. Acute otitis me dia (OM) entails middle ear in \202 ammation; it presents with rapid onset of pain, fever irritability, anorexia, or vomiting often after a viral upper respiratory infection. Chr onic OM is in \202 ammation with middle ear \202 uid of several months \200\231 duration . Common organisms: Pneumococcus, Haemophil- us, Moraxella, other streps and staphs. In acu te OM drum bulging causes pain, then purulent discharge if it perforates (often settles in 48h). \201\227: Give analgesia. Amoxicillin ( clavulanate) for \200\2057 days is 1st li ne. 41 Anti biotics are not always needed if well, and follow-up is simple;42 but in one NE JM study (2011 N=319)43 early use of co-amoxiclav did prevented signi \201 cant morbidity. 44 SE: diarrhea; resistance/ \200\230super-bugs \200\231etc. Decongestants don \200\231t h elp much.45 s e s a e s i d T N E Continuing discharge may indicate complications: mastoiditis which is rare (see below), and even rarer are petrositis; labyrinthitis; facial palsy; meningi- tis; intracranial abscess es. Mucopus may continue to drain when there is no mastoiditis, especially if grommets are in place. Treat with appropriate oral or topical antibiotics according to swab results. Do aural toilet to remove in- fected material from meatus. \200\207If discharge continues, ge t expert help. Chronic otitis media This is mucosal or squamous, and each may be inactive c lassi \201 cation There is discharge, \200\201hearing, but little or with active in \202 ammation.Browning \200\231s pain. For glue ear, see p546. Resolution is the rule (eg with antibiotic therapy, p543), but if secretions cannot drain, diff erentiation into squamous e pithelium occurs more serious complications, eg a retraction pocket of the pars tensa or \202 accida ( \201 g 1, p539). ie active Cholesteatoma squamous chronic otitis media) h as serious rare complications (menin- gitis/cerebral abscess). Incidence: 1 : 10,000. Peak age: 5 \200\22315yrs. It is a misnomer as it is neither cholesterol nor a tumor (it is loca lly destruc- tive around and beyond the pars \202 accida). \201\231 : Foul discharge deaf- ness; headache, pain, facial paralysis, and vertigo indicate impending CNS complicati ons. \201\227: Mastoid surgery is needed to make a safe dry ear by removing the disease; i mproving hearing is secondary. Mastoiditis: Middle ear in \202 ammation leads to destructi on of air cells in the mastoid bone abscess formation. Beware intracranial extension. Ri sk fac- tors: Withholding antibiotics in otitis media.46 Signs: T \200\200; tender mastoi d; pro- truding auricle. Imaging: CT. \201\227 : Hospitalization, IV antibiotics, myringot omy de \201 nitive mastoidectomy. ( \201 g 1; Fig 1. Attic cholesteatoma; like cottage cheese with mucopus. Rory Herdman FRCS 545 Hearing loss from middle ear causes: when to refer Refer to community audiology service (if available) for:47 \200 Determination of severity and cause of hearing loss. Is it conduc tive? \200 Is the hearing loss leading to a disability? Refer direct to an ENT consultant if: \200 Deaf for over 3 months \200\224or diffi culties with speech or learning \200 \224or \200 Recurrent and persisting earache over a 3-month period \200\224or \200 Othe r disabilities making correction of deafness urgent. Possibilities while the patient is wai ting to be seen: \200 Discourage passive smoking. What \200\231s it like at the childmind er \200\231s? \200 Encourage drinking from a cup, not a bottle (better Eustachian functio n). \200 Encourage interlocutors to sit at the child \200\231s level, and to keep instruc tions s e s a e s i d T N E short and simple. Explain the problem to all. Turn off the TV (less background noise, and this makes it more likely that the patient will look at the person speaking to him). Tell the teacher which side hears best (for class seating plan). \200 Nasal steroids for 1 month \200\224or \200 Co-amoxiclav for 14 days (may be of little real bene \201 t) \200\224or \200 Oral steroids for 5 days (eg prednisolone 1mg/kg/day PO). Tympanomastoid surgery for chronic otitis media Canal wall down (CWD) and canal wall up (CW U) mas- toidectomy are 2 surgical ways to address chronic middle ear cleft pathology. CWD p robably results in worse hearing outcomes and a mastoid cavity which may require lifelong f ollow-up or later surgi- cal obliteration. CWU surgery requires a second-look procedure at 1yr, but advances in MRI may obviate the need for this in the near future. NB: endoscopes a re improving visualization in CWU techniques. 48 Debriding an infected mastoid cavity \200\207Aim to remove all in- fected epithelium. \200 Culture secretions \200 Clean with hydrogen peroxide Remove debris/patch- es of granulation using suction and curette \200 Silver nitrate cautery to polyps (avoid use anywhere near cranial nerve VII) Post-op complications include failure of the self-cleaning mechanism of the canal with eith er a skin-lined healed cavity (dry), or chronic (or URTI-associated) discharge blood and pain. Here, repeat visits are needed (eg \211\2103/yr) to the ENT ward for removal of wax and skin debris, debridement (see MINIBOX) and endoscopy to identify further infection or cholesteatoma. Control bleeding with cotton balls (eg adrenaline-soaked). Risk factors for otitis media \200 URTI (and autumn/winter) \200 Bottle-feeding \200 Passive smoking \200 Dummy/paci \201 er49 Presence of adenoids \200 Asthma \200 Malformations (eg cleft palate) \200 Gastro-oes ophageal re \202 ux/BMI \200\200 in adults Fluid in the middle ear 546 NICE 2008 \200 This is detected by otoscopy ( \202 uid level or bubbles behind the drum) or indi- rectly, by tympanometry ( \201 g 3). 50% of 3-yr-olds have \211 1 ear eff usion/yr.50 In adults, exclude a postnasal space tumor as the cause of middle ear \202 uid. Glue ear/OM E (otitis media with eff usion; serous/secretory otitis media). \200\207Hearing impairment noticed by parents is the mode of presentation in 80%. The fundamental problem lies with d ysfunction of the Eustachian tubes. The exact cause is unclear, but there are associations with upper respiratory tract infections, oversized adenoids and narrow nasopharygneal dimen sions. Bacterial bio \201 lms present on the adenoids can be a source of infection, and ar e probably more important indicators of trouble than adenoid size. OME is com- moner in boy s, Down \200\231s syndrome, winter season, atopy, children of smokers and primary ciliary d yskinesia.1 OME is the chief cause of hearing loss in young children, and can cause disastr ous learning problems (rare). OME may cause no pain, so its presence may not be suspected. History: Focus on poor listening, poor speech, language delay, inattention, poor behavior, hear- ing \202 uctuation, ear infections/URTI, balance and, school work. Signs: Variable, eg retracted or bulging drum. It can look dull, gray, or yellow. There may be bub- bles or a \202 uid level, or super \201 cial radial vessels and \200\201drum mobility when tes ted with a Siegle specu- lum or with a pneumatic attachment. Tests: Audiograms: Look for co nductive def ects. Impedance audiometry: Look for \202 at tympano- gram ( \201 g 3; help s distinguish OME from Eustachi- an malfunction and otosclerosis). \201\227: It usually re solves over time. Explanation/ reas surance + 3-monthly review may be enough. Topical and s ystemic methods: NICE doesn \200\231t rec- ommend antibiotics, diet change, antihistamines, decongestants, steroids \200\224or acupuncture.etc Hearing aids: Reserve for persistent bi la teral OME and hearing loss if surgery is not accepted. Surgery: If persistent bilateral OME + hearing level in better ear of <25 \200\22330dBHL(=decibel hearing loss) con \201 rm ed over 3 months then myrin- gotomy + suction of \202 uid, and insertion of air-conductin g grommets adenoid- ectomy2 (if hearing loss).51 NICE also says surgery is an option if hearing loss is less severe but learning diffi culties are to the fore. Grommets are prefer red to T-tubes (tympanostomy tube), which cause too many complications52 (eg re- sidual per foration \211\210 50%). The main complications of grommets are infections and tympanoscler osis. Treat infections with aural toilet and antibiotic/steroid ear drops; grommet removal may be needed. Tonsillectomy doesn \200\231t help.53 Post-op: 90,000 operations/yr are done for OME in the UK (1 in 200 children). It is OK to swim with grommets, but avoid forcing w ater into the middle ear by diving. Some form of ear plug is wise, eg cotton wool and Vasel ine (esp. when shampooing as soap reduces the surface tension of water). Grommets extrude after 3 \200\22312 months; recheck the hearing then; 25% need re-insertion. Prognosis: L anguage development sequelae, eg \200\201reading skills, may persist.54 s e s a e s i d T N E Fig 1. Glue ear with retr a- cted drum & dull drumhead. R Herdman FRCS. Fig 2. A grommet. R Herdman FRCS. 1 PCD/Kartagener \200\231s syndrome (p646) often causes otitis media and OEM throughout chi ldhood (owing to loss of ciliary function in the Eustachian tube and middle ear), despite f airly continuous antibiotics. After 18yrs of age, the ear improves somewhat. Grommet placem ent do not improve middle ear function.55 2 Unless loss of hearing is a problem, adenoidect omy off ers little bene \201 t over watchful waiting in terms of URTI-associated stuffi ne ss, mouth breathing and quality of life, as these symptoms get less with age.51 547 Grommets and hearing gain: what do meta-analyzes say? Use of grommets to ventilate the midd le ear is common but controversial. In one careful review (Browning 2010) of research in ch ildren with OME the eff ect of grommets on hearing, as measured by standard tests, was smal l ( 12dB) and diminished after 6 months (4dB) by which time natural resolution had led to i mproved hearing in non-surgically treated children. 56 No eff ect was found on language or speech development or behavior, or cognitive or quality-of-life outcomes. Tympanosclerosis ( \201 g 7, p539) was seen in \205\223 of ears receiving grommets. No eff ect was found on other child outcomes but data on these were sparse. No good study has been done in child ren with established speech, language, learning or developmental problems so no conclusions can be made regarding treatment of such children. Note that adenoidectomy add- ed to gromm ets often gives the best results. 57 Target s e s a e s i d T N E trial Type C Types A and AD (red) Type B 1.5 1.5 1.5 s e r t i l i l i 1.0 1.0 1.0 m n 0.5 0.5 0.5 i e c n a 0 0 0 i l p m o C \200\223400 \200\223200 \200\223400 \200\223200 200 200 \200\223400 \200\223200 0 0 200 Ear canal air pressure \200\223as set by the tympanometer in decaPascals (daPa; 0=atmos pheric pressure) 0 Fig 3. Acoustic impedance audiometry (tympanometry). Courtesy of Jane Jones, Oxford Radcliff e Hospitals NHS Trust. Acoustic impedance audiometry (tympanometry) ( \201 g 3) Principles: In normal ears, compl iance of the drum (the amount of displacement in mL for a given sound) \200\224hence transm ission of sound \200\224peaks when middle ear pres- sure = ear canal pressure. So the peak of the tympanometry curve re \202 ects middle ear pressure. Operation: The tympanometer ha s various sized probes that snugly \201 t most ears. As it continuously changes the press ure in the ear ca- nal, a transmitter sends a sound to the drum. The wave that is re \202 ected from the drum is then picked up by a microphone in the probe. The tympanometer measur es the energy of the re \202 ected sound. Results: If the middle ear space is \201 lled with air, and the ossicles are intact, energy is absorbed by the drum, the ossicles, and in ner ear structures, and the tracing will show a normal peak with normal compliance (TYPE A, PURPLE). If there is disruption of the ossicles, or if part of the drum is \202 accid, a large amo unt of energy will be absorbed into the drum which is free to move a lot \200\224and it wil l move most when canal pressure=middle ear pressure (high compliance; type AD). Fluid in th e middle ear makes the drum stiff , so most of the sound is re \202 ected back to the prob e \200\224and changing the pressure in the canal has little eff ect \200\224hence a low, \202 at result (low compliance, TYPE B). This \201 nding must be related to ear canal vol ume (also measured by the tympa- nometer): if normal \211\210 otitis media; if low \211 \210 wax occlusion; if high \211\210 grommets or perforation. TYPE C: Shift in peak of the curve to the left found with nega- tive middle ear pressure, eg as in developing or resolv ing otitis media.58 Childhood deafness \200\207If you or a parent are worried, refer to a local community audi ology unit. Congenital causes Central (rare): Hyperbilirubinaemia/kernicterus, hypoxia, IVH . Conductive: Anomalies of pinna, external ear canal, drum, or ossicles, congeni- tal chole steatoma, Treacher \200\223Collins \200\231 (p655), Pierre Robin (p650). Sensorineural hear ing loss \200\224SNHL (sometimes no cause is found): \200 Autosomal dominant: Waardenburg (SNHL, heterochromia, \201 g 1, p425 & tele- canthus, ie \200\200distance between media l canthi), Klippel \200\223Feil, Alport (p648, 638). \200 Autosomal recessive: Pendred (S NHL with goiter), Usher \200\231s syndrome (SNHL, retinitis pigmentosa), Jervell and Lange- Nielsen (JSNL1 \200\224SNHL, long QT interval). 548 \200 X-linked: Alport \200\231s syndrome, Turner \200\231s syndrome. \200 Infections: CMV, HSV , syphilis, rubella, toxoplasmosis, group B strep sepsis. \200 Ototoxic drugs. Perinatal and postnatal causes Anoxia, birth trauma, cerebral palsy, ker- nicterus, meningitis, ototo xic drugs, lead, skull fracture. Universal newborn hearing screening (UNHS) Screening withi n hours of birth is the best way to ensure that deafness is diagnosed and managed before 6 months old. \200\207This does improve language.59 Tests: Otoacoustic emissions (OAE): A mi crophone in the external meatus detects tiny cochlear sounds produced by movement in its ba silar membrane in response to an auditory stimulus. OAE evalu- ates function of the periphe ral auditory system, primarily the cochlea, the area most often involved in SNHL.60 This is abnormal or equivocal in 3 \200\2238%.61 Most of these \200\230failures \200\231 (84%) ha ve external ear canal obstruction (collapsed ear canal or debris).62 In these patients, ABR (see below) should be measured.63,64 Audiologi- cal brainstem responses (ABR): The ears ar e covered with earphones that emit a series of soft clicks. Electrodes on the infant \200 \231s forehead and neck measure brain wave activity in response to the clicks. ABR tests th e auditory pathway from the external ear to the lower brainstem. Prevalence of deafness fou nd at UNHS: 0.9 \200\223 3.24 : 1000 for permanent bilateral hearing loss of >35dB; 5.95:10 00 when unilateral and moderate hearing loss infants are counted.65 No test performs perfec tly!66 Subjective hearing tests in the older child \200 7 months: Distraction testing (su itable if aged 6 \200\22318 months, though not a very accurate technique): As the child sit s on parent \200\231s lap, an assistant in front attracts the child \200\231s attention whi le a tester attempts to distract by making noises behind and beside child, eg with a rattle , conversational voice. \200 2 \200\2234 years: Conditioned response audiometry (at 24 \200\22360 months): The child is trained to put pegs into holes or give toys to a parent on a particular audi- tory cue. This method can also obtain PTAs (pure tone audiograms) in an older child. Speech discrimination: (24 \200\22360 months): the child touches selected ob- jects cued by acoustically similar phrases, eg key/tree. s e s a e s i d T N E 5 years: Pure tone audiogram. Management Aim to provide as good hearing as possible to help language and education. Teachers of the deaf make arrangements for \201 tting hearing ai ds and help monitor progress. Children usually need higher gain from their aids than adults . Encourage parents to talk as much as possible to their deaf child. Children may be educat ed at ordinary schools with visits from teachers of the deaf, or, for the partially hearing , in special units in ordinary schools, or in schools for the deaf, depending on need. A co chlear implant may be suit- able (BOX). NB: the shorter the duration of deafness, the bette r the outcome, so funding decisions must not be delayed. Preventing post-implantation men- ingitis: \200\207Give pneumococcal vaccine \211 2wks before implant (or vaccinate the unv accinated if implant already in situ). \200\207Treat any otitis media promptly; if recurre nt, insert grommets. \200\207Give IV antibiotics if mastoiditis suspected.67 549 Cochlear implant issues1 Cochlear implant image: p534 They take 2h to \201 t (under GA); an external device processes sound and transmits it across the skin to the subcutaneous re ceiver coil \200\224to an elec- trode placed in the cochlea via the round window and direct ly stimulates the auditory nerve. The signal is not normal sound; rehabilitation is needed to understand the new sounds. Bene \201 ts: better lip-reading; recognition of environment al sounds, relief of isolation. Cost: \200\205 35,000. Pre-op assessment is vital. Give p neumococcal vaccine. Selection criteria: best if language has been acquired. Age is no limi t. Commitment is vital. Exclude central deafness. Implants may be damaged by direct trauma, MRI, surgical diathermy, dental pulp testers and \200\230therapeutic \200\231 (short-wave and microwave) diathermy used in physiotherapy departments. www.bcig.org tel.: 0207 915 13 01 Economic and ethical issues with cochlear implants These have been ad- dressed by NICE. Guidance now states that they may be considered in profound sensorineural deafness whether a pre-language child or a deaf adult. Beware statements such as \200\234after cochlear imp lant patients can lead normal lives \200\235 (this may only mean that other people don \200 \231t notice the hearing problem). Post- op training is vital, and the user is by no means an inactive recipient. NB: quality is now suffi cient for previously deaf people to be able to use mobile phones. QALYS For multichannel cochlear implants, average cost per QALY (qua lity-ad- justed life-year) for prelingually very deaf children is 13,413/QALY68 which is in the middle of quoted estimates for costed NHS therapies (for a list, see OHCM p10). Imp lantation of those at least up to 70yrs old makes economic sense. NB: multiple disabilities are no bar to bene \201 t from a device, but it is not clear that they merit extra consid eration (they certainly merit full assessment). Does deafness always count as a disability? Some deaf families partic- ularly welcome the arrival of a deaf child, and some deaf coupl es selecting donors for arti \201 cial insemination choose a deaf father with a \200\230g ood \200\231 pedigree of deafness.69 Such off spring will not grow up in a deaf world \200 \224so it is very debatable whether this is in the child \200\231s best interests, especial ly as in some areas sign language is dying out owing to the absence of congenital rubella. s e s a e s i d T N E BAHA: the bone-anchored hearing aid This hearing aid augments bone conduction. It is best s uited to conductive hearing loss, unilateral hearing loss, and children and adults with hea ring loss who do not get on with \200\230in ear \200\231 or \200\230behind ear \200\231 a ids. A trial of a bone con- ductor is traditionally used to determine whether BAHA will wor k well. They have a special bene \201 ts in some children with complex disorders because t he children do not physically feel the presence of the hearing aid. On quality of life meas ures BAHA do very well. Complications are reported in 6% (eg skin regrowth around the titan ium screw and non-osseointegration).70 \200\22372 Indications: Intolerance of conventional hearing aids (draining ear; mas- toid cavity; topical sensitivity); congenital malformation s (eg microtia; atre- sia); single-sided deafness. Contraindications: Average bone threshol d worse than 45dB; non-compliance; poor hygiene; lack of bone volume.73 It is not the actual sound itself that matters, but the reverberations that it makes as it travels through our mind. These are often to be found far away, strangely tr ansformed; but it is only by gathering up and putting together these echoes and fragments t hat we arrive at the true nature of our experience. Virginia Woolf, 1926. Note that deepeni ng the emotional content of music, for example, by association melody with concrete events in our lives depends on dealings in ancient sub-neocortical limbic regions such as the hipp ocampus, amygdala,74 and anterior cingulate cortex, which form the hub of all our emotions, passions, and delights. Deafness in adults Many cope well with mild hearing loss if given comprehensive rehabilitat ion. Management Classify the deafness (BOX); \201 nd treatable causes. Exclude the danger - ous (esp. if asymmetric): acoustic neuroma, cholesteatoma, eff usion from nasopharyn- gea l cancer. Palliative: eg \201 tting a suitable aid. De \201 nitive: eg surgery for perfo rations or otosclerosis. Severe sensorineural deafness (>100 \200\223120dB) may be helped b y a cochlear implant ( \201 g 1 p534 & 549 BOX 1) to stimulate residual neural tissue. Sud den hearing loss \200 If sensorineural: \200\207Immediate specialist referral, see BOX 3 . De \201 nition: Loss of \211 30dB in 3 contiguous pure tone frequencies over \211 72h. Incidence: 5 \200\22320 per 100,000/yr. \200 : \200 \201\2261: 1. Partial or complete sp onta- neous recovery occurs in 30 \200\22365%. Detailed evaluation reveals underlying disea ses (eg noise exposure; gentamicin toxicity; mumps; acoustic neuroma; MS; vasculopathy; TB; rarities1) in 10%. Tests: BOX 3. Start rehabilitation early. Prognosis: Better if early pr esentation. Negative prognostic factors: Age <15yrs or >65yrs, \200\200ESR, vertigo, heari ng loss in the opposite ear, severe hear- ing loss. 75 There is evidence that idiopathic ca uses may respond to steroids hyperbaric O2 therapy, if given promptly. 76 Anticoagulants , acic lovir, and carbamazepine are often given, but are of dubious effi cacy. \200 Condu ctive: A cause is \200\230always \200\231 found: infection, occlusion, trauma, fracture. O tosclerosis Prevalence 0.5 \200\2232% clinically, 10% subclinically. Cause: Auto- somal dom inant with incomplete penetrance; 50% have a family history. 85% are bilateral; \200 : \200 \211\210 2 : 1. Pathology: Vascular spongy bone replaces normal la- mellar bone of o tic capsule origin particularly around the oval window which \201 xes the stapes footplat e. Symptoms usually appear in early adult life and are made worse by pregnancy, menstruatio n, and the menopause. There is con- ductive deafness (hearing is often better with backgrou nd noise), 75% have tinnitus; mild, transient vertigo is common too. 10% have Schwartz \200 \231s sign \200\224a pink tinge to the drum; audiometry with masked bone conduction shows a dip at 2kHz (Cahart \200\231s notch). Treatment: Historically by fenestration. Fluo- ride may inhibit sclerotic progression. 77 Hearing aid or surgery to replace the adherent stapes with an implant2 helps 90%. Microdrill and CO2 laser staped- otomy give similar results. 7 8 Many prefer surgery to wearing an aid79 (1 \200\2234% risk of a dead ear). Cochlear impla nt is another option (if severe). 80 Presby(a)cusis (Age-related hearing loss from accumula ted environmental noise toxicity.) Loss of high-frequency sounds starts before 30yrs; rate of loss is progressive thereafter. Deafness (loss of hair cells: see \201 g 3, p535) is g radual and we do not usually notice it until hearing of speech is aff ected with loss of hi gh-frequency sounds (consonants at 3 \200\2234kHz are needed for speech dis- crimination). Hearing is most aff ected in the presence of background noise. Hearing aids are the usual treatment. See pure tone audiograms on p541 ( \201 g 2). Tips on communicating with those who are hard of hearing \200 Decrease background noise. Use of an induction loop system ( eg a wire around the TV room) achieves this for those with suitably adapted aids (with a sw itch, ie the T setting; NB: some new aids are too small for these). 550 UK prevalence: 10% \200 Is your hearing reduced? \200 Do people \200\230mumble \200\231? \200 Do you keep sayi ng \200\234What? \200\235 \200 Do you misunderstand names? \200 Is your TV volume \200\230too loud \200\231? \200 Are noisy rooms a problem? s e s a e s i d T N E Use short sentences. Be on the same level as the person you are addressing. \200 Ensure t hat light falls on your face. Unilateral sensorineural deafness Think of acoustic neuroma. 1 Stroke; sarcoidosis; 81 Takayasu \200\231s arteritis; Chlamydia pneumoniae; immune comple xes; autoantibodies to inner ear proteins; anticardiolipin (aCL) antibodies; cellular immun e defects. 82 2 Middle ear implants: Surgical implantation of auditory devices is a rapidly growing area in the rehabilita- tion of deafness. They should be viewed as an alternative to conventional hearing aids for individuals who are either unable to wear hearing aids or reject them for any reason. 83 551 Classi \201 cation of deafness Conductive deafness There is impaired sound transmission vi a the exter- nal canal and middle ear ossicles to the foot of the stapes through a va- riet y of causes. External canal obstruction (wax, pus, debris, foreign body, developmental anom alies); drum perforation (trauma, barotrauma, infec- tion); problems with the ossicular cha in (otosclerosis, infection, trauma); and inadequate Eustachian tube ventilation of the mid dle ear (eg with eff usion secondary to nasopharyngeal carcinoma) all result in conductive deafness. Sensorineural deafness results from defects central to the oval window in the coc hlea (sensory), cochlear nerve (neural) or, rarely, more central path- ways. Ototoxic drugs (eg streptomycin, vancomycin, gentamicin, chloroquine and hydroxychloroquine, vinca alkalo ids), postinfective (meningitis, measles, mumps, \202 u, herpes, syphilis), cochlear vasc ular disease, M ni re \200\231s (p554), trauma, and presbyacusis are all sensorineural. R are causes: Acoustic neu- roma, B12 de \201 ciency, multiple sclerosis, secondary carcinom a in the brain. s e s a e s i d T N E Choosing an aid: head-to-head speech vs cross-talk (amid noise) Amplifying speech is a majo r factor to take into account when choosing an aid: get help from independent experts as di gital technologies are constant- ly improving. We know that speech is best heard when liste ning binaurally. The bene \201 ts of directional microphones in hearing aids are also well docu- mented in lab settings. If it is necessary to \201 t 2 aids, should they both be d irectional (symmetrical directional \201 tting)? Yes, if the speech comes from in front o f the listener (head-to-head) and the noise sources are located to the side of or surround the listener. When speech is located to the side of the listener, use of directional proces sing on the ear adjacent to the speaker is likely to reduce speech audibility and thus degr ade speech understand- ing. 84 NB: hearing aids with directional microphones cannot be \201 tted entirely within the ear canal, and so are more noticeable that smaller aids. Also , aids that \201 t in the canal may give better results with the telephone. \200 Managin g sudden sensorineural deafness: a summary \200 Get expert ENT help. \200 Look for causes : WR; ANA; INR; TSH; blood glucose; cholesterol; ESR; FBC; LFT; viral titres; audiology; ev oked response audiometry CXR; Mantoux; MRI; pANCA; lymph node and nasopharyngeal biopsy for malignancy/TB culture. \200 Imaging: gadolinium MRI; CT. \200 Audiometry and auditory brainstem evoked responses . \200 High-dose steroids are no more eff ective when combined with antivirals. One starti ng regimen is prednisolone 80mg/24h PO for 4 days tapered over 8 days.85 Intratympanic dexa methasone may have a salvage role. The bounds of \200\230normal \200\231 hearing Both frequency (Hz) and sound pressure (deci bels, dB) are important for the detection of sound by the human ear, though the relationshi p between the two is also important. Sound frequencies between 500 and 4000Hz are the most important for speech interpretation. Severe sensorineural deafness is de \201 ned as >100 \200\223120dB loss; signi \201 cant hearing loss is considered as >35dB loss. Remember tha t decibels are a logarithmic scale, and that the range from 0 to 120dBs actually represents a million times relative increase in sound pressure. Another unit for the sound pressure ( \211\210 \200\230volume \200\231 in common par- lance) is the dyne/cm2, which is not a log arithmic scale. At high intensity ( \200\206130dB), sound can also be a painful stimulus, showing an intere sting (and variable) threshold relationship between useful information from special senses and painful stimuli (also present in the eye).