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40 The ear

GRANT J.E.M. BATES Surgical anatomy of the ear The external ear consists of the pinna and the ear canal. The pinna is made of yello elastic cartilage co!ered "y tightly adherent s#in. The external and middle ear de!elop from the first t o "ranchial arches. The external ear canal is $ cm in length% the outer t o&thirds is cartilage and the inner third is "ony. The s#in on the lateral surface of the tympanic mem"rane and the inner t o&thirds of the ear canal is highly specialised. 't does not simply shed li#e the s#in from the rest of the "ody. 't migrates out ards from the tympanic mem"rane and along the ear canal. As a result of this migration most people(s ears are self&cleaning. )isorders of s#in migration can result in ear disease *e.g. cholesteatoma+. The external canal is richly inner!ated and the s#in is tightly "ound do n to the perichondrium so that oedema in this region results in se!ere pain. The lymphatics of the external ear drain to the retro auricular% parotid% retropharyngeal and deep upper cer!ical lymph nodes. The middle ear contains the ossicles. ,aterally it is "ounded "y the tympanic mem"rane% medially "y the cochlea% anteriorly "y the eustachian tu"e and posteriorly it communicates ith the mastoid air cells *-ig. ./.0+. Ent ined in this tiny space is the facial ner!e hich pursues a tortuous course through the middle ear and exits the s#ull "ase at the stylomastoid foramen. 1no ledge of the anatomy of the middle ear is important "ecause infection can spread through it to the cranial ca!ity hich lies millimetres a ay. The tympanic mem"rane has three layers2 an inner mucosal layer% a dense fi"rous middle layer and the outer stratified s3uamous epithelium *s#in+. The upper portion that lies a"o!e the lateral process of the malleus is called the pars flaccida. The lo er portion% ma#ing up the ma4ority of the drum% is called the pars tensa *-ig. ./.5+. The tympanic mem"rane and ossicles act as a transformer system con!erting !i"rations in the air to !i"rations ithin the fluid&filled inner ear *perilymph+.The e!olution of the middle ear is interesting. -ish do not ha!e one% hereas amphi"ians *e.g. salamanders+ ha!e a single strut for an ossicle. At an air6 ater interface there is a $/ deci"els loss of sound energy. The mammalian middle ear o!ercomes !irtually all of this potential loss of sound energy. The inner ear comprises the cochlea and !esti"ular la"yrinth *saccule% utricle and semicircular canals+. These structures are em"edded in dense "one called the otic capsule. The cochlea is a minute spiral of t o and three&3uarter turns. 7ithin this spiral% perilymph and endolymph are partitioned "y the thinnest of mem"ranes *Reissner(s mem"rane+. The endolymph has a high concentration of potassium similar to intracellular fluid% and the perilymph has a high sodium concentration similar to extracellular fluid. Maintenance of the ionic gradients is an acti!e process and is essential for neuronal acti!ity. There are approximately 08 /// hair cells in the human cochlea. They are arranged in ro s of inner and outer hair cells. The inner hair cells act as mechanicoelectric transducers% con!erting the acoustic signal into an electric impulse. The outer hair cells contain contractile proteins andha!e an efferent ner!e supply from the "rain. They ser!e to tune the "asilar mem"rane on hich they are positioned. Each inner hair cell responds to a particular fre3uency and hen stimulated it depolarises and passes an impulse to the cochlea nuclei in the "rainstem.

The !esti"ular la"yrinth consists of the semicircular canals% the utricle and saccule% and their central connections. The three semicircular canals are arranged in the three planes of space at right angles to each other. As in the auditory system% hair cells are present. 'n the lateral canals the hair cells are em"edded in a gelatinous cupula% and shearing forces% caused "y angular mo!ements of the head% produce hair cell mo!ements and generate action potentials. 'n the utricle and saccule the hair cells are em"edded in an otoconial mem"rane hich contains particles of calcium car"onate. These respond to changes in linear acceleration and the pull of gra!ity. 'mpulses are carried centrally "y the !esti"ular ner!e% and connections are made to the spinal cord% cere"ellum and external ocular muscles. The sensory ner!e supply of the ear is complex. The external ear is supplied "y the auriculotemporal "ranch of the trigeminal ner!e *9+% and this supplies most of the anterior half of the pinna and the external auditory meatus. The greater auricular ner!e *:5%$+% together ith "ranches of the lesser occipital ner!e *:5+% supply the posterior part of the pinna. The 9''th% ';th and ;th cranial ner!es also supply small sensory "ranches to the external ear< this explains hy the !esicles of herpes =oster affecting the 9''th ner!e appear in the concha *see -ig. ./.5> later+. The middle ear is supplied "y the glossopharyngeal ner!e *';+. This complicated and rich sensory inner!ation means that referred otalgia is common and may originate from the normal area of distri"ution of any of the a"o!e ner!es. A classic example is the referred otalgia caused "y a malignancy in the pyriform fossa of the pharynx or a cancer of the larynx. Anatomy of the ear ?Referred otalgia has many causes *e.g. cancer of the larynx+ ?Middle ear is intimately related to the cranial ca!ity ?The 9llth ner!e has a tortuous course through the ear :onditions of the external ear :ongenital anomalies :ongenital anomalies can range from total a"sence of the ear through to mild cosmetic deformities such as tiny accessory auricles or s#in tags. External ear anomalies can "e isolated or may "e associated ith middle ear deformity. The external and middle ear originate from the first and second "ranchial arches% hereas the cochlea is of neuroectodermal origin. This means that an indi!idual may ha!e no pinna or ear canal "ut a normal cochlea may ell "e present. 'n these circumstances% sound can "e transmitted from a hearing aid connected to an osteo integrated peg that is scre ed into the mastoid "one. *Ta" ./.0+ :hildren ho ha!e a significant deformity of the pinna *microtia+ can "e helped ith osteointegrated implants to hich a prosthetic ear is connected *-ig. ./.$+. The ear can "e unclipped prior to playing !iolent sport *e.g. rug"y+ and this unsettles the opposition. @reauricular sinuses are a common congenital a"normality and occasionally need excising "ecause of recurrent infections and discharge. The sinus usually ends near the external canal "ut occasionally the trac# is !ery extensi!e and is closely related to the facial ner!e% hich ma#es life exciting. @rominent ears are a common deformity hich usually results from the a"sence of the antihelix cur!e. 9arious cartilage scoring methods are a!aila"le to correct this deformity. Trauma Trauma often affects the external ear. A haematoma of the pinna occurs hen "lood collects "et een the penchondrium and the cartilage. The cartilage recei!es its "lood

supply from the perichondrial layer and ill die if the haematoma is not e!acuated *cauliflo er ear+. An extensi!e excision% under general anaesthetic% ith a pressure dressing and anti"iotic co!er is recommended *see -ig. ./..+. -oreign "odies in the ear canal need to "e treated ith the greatest respect. 'f an o"4ect is not simply remo!ed at the first attempt% it is "etter to do it ith the aid of a microscope and general anaesthesia. An acti!e 5&year&old ith a "ead in the ear can "e a formida"le opponent *-ig. ./.8+. 'nflammatory disorders 'nflammatory disorders of the external ear are extremely common. Atitis externa fre3uently presents to general practice and to ear% nose and throat *ENT+ surgeons. There is generalised inflammation of the s#in of the external auditory meatus. 't can occur as an acute episode or can run a more chronic course. The cause is often multifactorial "ut includes general s#in disorders% such as psoriasis and ec=ema% and trauma. :ommon pathogens are pseudomonas and staphylococcus "acteria% and amongst fungi% candida and aspergillus. Ance the s#in of the ear canal "ecomes soggy and oedematous% s#in migration stops and de"ris collects in the ear canal hich acts as a su"strate for the pathogens. The hallmar# of acute otitis externa is se!ere pain *e!idently on a par ith child"irth+. Bnli#e otitis media% mo!ement of the pinna elicits pain. The condition is often "ilateral. The initial treatment is ith topical anti"iotics and steroid ear drops% together ith analgesia. 'f this fails meticulous remo!al of the de"ris ith the aid of an operating microscope is re3uired. Regular cleaning of the canal% together ith topical steroids% needs to "e continued until normal s#in migration resumes. 'f fungal infection is present it can easily "e recognised "y the presence of hyphae and spores ithin the canal *-ig. ./.C+. -ungal infection causes irritation and itch% and the treatment is meticulous remo!al of the fungus and any de"ris% as ell as stopping any concurrent anti"iotics. Systemic anti"iotics are rarely re3uired for otitis externa "ut should "e used if cellulitis of the pinna occurs *-ig. ./.D+. Necrotising otitis externa is a rare "ut !ery important condition. 't presents as a se!ere% persistent% unilateral otitis externa in an immunocompromised indi!idual% for example it is important to thin# of the diagnosis in an elderly dia"etic. Asteomyelitis of the s#ull "ase occurs and usually the infecting organism is @seudomonas aeruginosa. Se!eral cranial ner!es *9''% '; and ;+ may "e destroyed "y the progressing infection. 'ntensi!e systemic anti"iotic treatment is re3uired and the disease process is monitored "y high&resolution imaging. A furuncle of the external ear is an infection of a hair follicle and is due to a staphylococcal infection. Mo!ing the pinna causes extreme pain. ,ocal treatment of the ear canal *oto& ic# and steroid drops+ together ith systemic anti"iotic therapy is re3uired. Neoplasms Benign neoplasms Benign neoplasms of the external ear are common if osteomas are included. These arise from the "one of the ear canal in indi!iduals ho ha!e done a lot of s imming in cold ater. No treatment is re3uired unless they o"struct the migration of s#in out of the canal. Ather "enign tumours include papillomas and adenomas. Malignant primary tumours Malignant primary tumours of the external ear are either "asal cell or s3uamous cell carcinomas *-ig. ./.E+. Both may present as ulcerating or crusting lesions hich gro slo ly and may "e ignored "y elderly patients. S3uamous cell carcinomas may

metastasise to the parotid andFor nec# nodes and need radical surgical clearance. The ear canal may "e in!aded "y tumours from the parotid and postnasal space carcinoma hich Gcreep( up the eustachian tu"e. All resecta"le malignant tumours of the ear are treated primarily ith surgery ith or ithout the addition of radiation therapy. The external ear ? Atitis externa responds to topical medication ? Bnilateral otitis externa in a dia"etic may "e fatal ?Auricular haematoma needs a ro"ust incision% drainage and pressure dressing ?Thin# osteo integration for congenital malformations :onditions of the middle ear *Ta" ./.5+ :ongenital anomalies :ongenital anomalies of the middle ear may "e isolated or may "e associated ith other ear or general congenital deformities. There is a num"er of "ranchial arch syndromes 6 for example @ierre Ro"in(s syndrome% craniofacial dysostosis% )o n(s syndrome and Treacher :ollins( syndrome. 'f there is an external ear a"normality% it should raise suspicion of an underlying middle ear deformity. Middle ear deformity can "e assessed "y high&resolution computerised tomography *:T+ scanning and% if the inner ear is normal% reconstructi!e surgery of the middle ear can "e !ery successful. Trauma Trauma to the middle ear can result in a perforated tympanic mem"rane *-ig. ./.>a+. Such perforations usually heal spontaneously *-ig. ./.>"+. Trauma can result is ossicular discontinuity and typically it is the incus that is displaced. 9arious operations termed Gtympanoplasties( are a!aila"le to reconstruct the damaged ossicular chain and repair the tympanic mem"rane if necessary. 'nflammatory disorders The most common inflammatory condition of the middle ear is acute suppurati!e otitis media. 't is extremely common in childhood and is characterised "y purulent fluid in the middle ear. Mastoiditis may "e associated ith otitis media "ecause the mastoid air cells connect freely ith the middle ear space. The tympanic mem"rane is hyperemic and "ulges o ing to pressure from the pus in the middle ear *-ig. ./.0/+. The child suffers extreme pain until the tympanic mem"rane "ursts. The most common infecting organisms are Streptococcus pneumoniae and Haemophilus influen=ae. Appropriate systemic anti"iotics should "e gi!en for 0/ days. The incidence of acute mastoiditis has diminished ith the idespread use of anti"iotics for otitis media. Sometimes% ho e!er% a child ill ha!e had a num"er of courses of anti"iotics% none of hich completely resol!es the middle ear infection. 'n such cases the pain and s elling "ehind the ear may not "e 3uite so apparent as in -ig. ./.00. 7hen mastoiditis is present% if the tympanic mem"rane can "e seen% there is al ays a sag in the posterior superior part of the drum. *:on!ersely% a normal tympanic mem"rane excludes mastoiditis.+ Treatment re3uires hospital admission and intensi!e parenteral anti"iotics. 'f this does not resol!e the infection 3uic#ly a cortical mastoidectomy is re3uired% together ith a myringotomy. Mastoiditis ? Se3uelae of acute otitis media ? May "e mas#ed "y anti"iotics ?Re3uires intensi!e anti"iotics andFor drainage

Atitis media ith effusion *glue ear+ is !ery common ith the ma4ority of children experiencing at least one episode of it during de!elopment. Many factors ha!e "een implicated% although it is primarily thought to "e due to poor eustachian tu"e function. Axygen is continually "eing a"sor"ed "y the middle ear mucosa and this results in a negati!e middle ear pressure unless the eustachian tu"e opens to replenish the air. This negati!e middle ear pressure initially results in transudation of fluid into the middle ear space *-ig. ./.05+. 'f the hypoxia continues% a mucoid exudate is produced "y the glands ithin the middle ear mucosa. This stic#y exudate is referred to as Gglue ear(. The follo ing symptoms may "e associated ith glue ear2 ?hearing impairment hich often fluctuates< ?delayed speech< ?"eha!ioral pro"lems< ?recurrent ear infections 6 this occurs "ecause the exudate is an ideal culture medium for microorganisms< ?reading and learning difficulties at school. 'f these symptoms are present for a short time only% it is li#ely that no long&term se3uelae ill de!elop. Ho e!er% if symptoms persist% particularly a long&term "ilateral conducti!e hearing loss% the child ill miss out on educational opportunities and may not fulfil his or her academic potential. The otoscopic findings of exudati!e glue ear are of a dull drum that is immo"ile on pneumatic otoscopy *-ig. ./.0$+. The tympanic mem"rane is retracted and radial "lood !essels may "e present *-ig. ./.0.+. Af children first presenting ith "ilateral glue ear% 8/ per cent of the effusions ill resol!e spontaneously ithin C ee#s of onset. 'nitially% a G ait and atch( policy is therefore appropriate. 'f the "ilateral glue ear persists ith a significant hearing loss then treatment is re3uired. There is no su"stantial e!idence for medical treatment. Bse of the Ato!ent de!ice *-ig. ./.08+ may impro!e eustachian tu"e function and is orth trying hile aiting for resolution of the effusion. Ho e!er% surgical inter!ention is the only effecti!e ay of curing glue ear. Both !entilation tu"e *groin&mets+ and adenoidectomy are effecti!e. The contro!ersy is not hether surgery or#s "ut hen to inter!ene.insertion of !entilation tu"es andFor an adenoidectomy re3uire a general anaesthetic. The !entilation tu"e is placed in the anterior inferior portion of the tympanic mem"rane "ecause there is no important cloc# or# "ehind this part of the drum. The !entilation tu"es stay in position for approximately C60E months and are then extruded "ecause of the migratory "eha!iour of the tympanic mem"rane. There is no reason hy children ith !entilation tu"es should not "e allo ed to s im *-ig. ./.0C+. Atitis media ith effusion *glue ear+ ?9ery common% pea#s at 0E months and 8 years ? Ma4ority of children need no treatment ?@rolonged hearing loss treated ith !entilation tu"es andFor adenoidectomy A middle effusion in adults is relati!ely rare and hen it occurs does not usually last long. The condition is often associated ith an upper respiratory tract infection. A persistent unilateral effusion in an adult should al ays "e !ie ed ith suspicion. A nasopharangeal carcinoma may cause the effusion "y "loc#ing the opening of the eustachian tu"e in the postnasal space. This is the most common carcinoma in males in southern :hina. :hronic suppurati!e otitis media

:hronic suppurati!e otitis media *:SAM+ is classified into t o types2 tu"otympanic disease% in hich there is a perforation of the pars tensa< and atticoantral disease% in hich a retraction poc#et de!elops from the pars flaccida. :SAM of the tu"otympanic type. :SAM of the tu"otympanic type can result from trauma or infection. 7hen perforated the tympanic mem"rane usually repairs itself% "ut occasionally the outer layer of the tympanic mem"rane fuses ith inner mucosa and a chronic perforation results *-ig. ./.0D+. 7ith this type of disease the patient(s main symptoms are of an intermittent or chronic mucoid discharge associated ith a mild conducti!e hearing loss. 't is rare for this type of disease to "e associated ith intracranial complications. A diagnosis is made on otoscopy and the tuning for#s usually suggest a conducti!e hearing impairment. The first&line treatment is topical anti"iotic and steroid drops% and on occasion microsuction. 'f medical treatment fails% the patient may re3uest an operation to graft the tympanic mem"rane in order to gi!e a dry ear. This operation is termed a myringoplasty *type ' tympanoplasty+. The edges of the perforation are freshened and a small piece of temporalis fascia is inserted under the tympanic mem"rane to graft the drum. The ra epithelial edges then gro across the graft to repair the tympanic mem"rane. :SAM of the atticotympanic type. :SAM of the atticoantral type is important "ecause of the complications associated ith it. :holesteatoma is the alternati!e name and means a cyst or sac of s3uamous epithelium that is present in the attic part of the middle ear. The exact aetiology of cholesteatoma is not #no n% although poor eustachian tu"e function is implicated *e.g. patients ith cleft palates ha!e relati!ely poor eustachian tu"e function and ha!e a higher incidence of cholesteatoma+. A retraction poc#et de!elops in the pars flaccida and% if the s3uamous epithelium cannot migrate out of this poc#et% a cholesteatoma results. The expanding "all of s#in causes a lo &grade osteomyelitis hich results in the release of fatty acids from the "one. This gi!es the discharge its characteristic faecal smell. 'n!aria"ly the discharge is accompanied "y hearing loss and mild discomfort. The patient may simply put up ith the symptoms until a se!ere complication occurs. The hearing loss that is caused "y cholesteatoma may "e conducti!e due to erosion of the incus or sensori neural due to direct erosion of the cochlea or migrations of toxins into the inner ear. 9esti"ular symptoms may occur "ecause of erosion of the semicircular canals or the migration of toxins into the !esti"ule. @ressure or erosion of the facial ner!e is relati!ely unusual. The close proximity of the middle ear and mastoid to the middle and posterior cranial fossae means that intracranial sepsis can result from chronic ear disease. The infection spreads to the dura !ia emissary !eins hich connect the middle ear mucosa to the dura or "y direct extension of the disease through the "one. Meningitis% extradural% su"dural or intracere"ral a"scess% or a com"ination of these may occur. The main causes of intracranial sepsis in the B1 are chronic ear disease and chronic sinus disease. )iagnosis should "e suspected on otoscopy *-ig. ./.0E+. @us% crusts% granulations or a hitish de"ris in the attic are hallmar#s of the disease. Examination under the microscope% audiometry and% sometimes% :T scanning are indicated. The treatment is surgical and follo s the principle of exposing the disease% excising the disease and then exteriorising the affected area. T o commonly applied operations for this disease are an atticotomy and a modified radical mastoidectomy. An atticotomy or modified radical mastoidectomy is performed "y ma#ing an incision "ehind the ear *post auricular+% or "et een the tragus and the pinna *end aural+. The

attic part of the "ony ear can is drilled a ay and the retraction poc#et is follo ed "ac# into the mastoid until the end of the disease is found. An attempt is made to excise totally the poc#et and then the resulting ca!ity is usually lined ith temporalis fascia. A mastoid ca!ity heals ith normal s#in that does not migrate% and for this reason patients ith a mastoid ca!ity need to "e seen in an out&patients( clinic on a regular "asis. Any s#in that collects in the mastoid ca!ity needs to "e remo!ed ith the aid of a suc#er and a microscope *-ig. ./.0>+. :hronic suppurati!e otitis media *:SAM+ ?'n tu"otympanic :SAM there is a perforated tympanic mem"rane and fre3uently a mucoid discharge ?Atticoantral :SAM2 6 's s#in in an attic retractionI J cholesteatoma @resents ith hearing loss and smelly discharge 6 's a common cause of intracranial sepsis Tu"erculous otitis media is an important cause of suppuration in some countries. The diagnosis should al ays "e considered in any ear hich fails to respond to standard therapy. A s a" for appropriate culture studies% coupled ith chest radiography% ill usually confirm the diagnosis. Atosclerosis is a condition in hich ne a"normal spongy "one is laid do n in the dense otic capsule. Af particular importance is the "one that is laid do n around the footplate of the stapes hich impedes the mo"ility of the stapes and results in a conducti!e hearing loss *-ig. ./.5/+. Toxins released from the ne "one formation may also cause a gradual sensorineural hearing loss. Atosclerosis is more common in omen% and in 8/ per cent of patients there is a family history. The typical presentation is of a conducti!e hearing loss in a young oman ith the condition "eing exacer"ated "y the hormonal flux of pregnancy. A similar type of stapes fixation occurs in osteogenesis imperfecta and is #no n as 9an der Hoe!e(s syndrome. Atosclerosis is often "ilateral. A diagnosis should "e suspected in any patient ith a conducti!e hearing loss and a normal tympanic mem"rane. The treatment options are simple reassurance% a hearing aid or a stapedotomy operation. 'n the stapedotomy operation% the stapes crura are remo!ed and a small hole is drilled in the fixed stapes footplate. A !ein graft is then inserted o!er the hole and a piston lin#ing the incus to the !ein graft is delicately placed in position *-ig. ./.50+. 'n >/ per cent of cases the operation is highly successful% "ut rare complica& tions include se!ere sensorineural hearing loss and "alance distur"ance. Atosclerosis ?Ne "one formation in otic capsule ?Stapes fixation ?Aptions2 6 Reassurance< 6 Hearing aid< 6 Stapedotomy Neoplasms Neoplasms of the middle ear are rare% the most common "eing a glomus tumour *-ig. ./.55+. Glomus tumours arise from nonchromaffin paraganglionic tissue. The carotid "ody tumour arising in the nec# is an example of this type of tumour. 'n the temporal "one three types of glomus tumour are recognised and classification depends on the location2 glomus tympanicum *arising in the middle ear+% glomus 4ugularae *arising next to the 4ugular "ul"+ and glomus !agali *s#ull "ase+.

@ulsatile tinnitus is a classic symptom of these tumours. Hearing loss occurs and may "e either conducti!e or sensorineural% and paralysis of the 9''th% ';th% ;th% ;'th andFor ;''th ner!es may occur. The classic sign is a cherry&red mass lying "ehind the tympanic mem"rane. An audi"le "ruit may "e heard ith a stethoscope o!er the temporal "one. The treatment of choice is preoperati!e ein"olisation follo ed "y surgical excision. Radiotherapy is also effecti!e. S3uamous cell carcinoma may also occur ithin the middle ear. 't usually presents ith deep seated pain and a "loodstained discharge. The facial ner!e may "e paralysed. S3uamous carcinomas usually arise in a chronically discharging ear and can certainly arise in a chronically infected mastoid ca!ity. Radical surgical excision ith or ithout radiotherapy pro!ides the only chance of cure. :onditions of the 'nner ear*Ta" ./.$+ :ongenital :ongenital inner ear disorders may "e associated ith external or middle ear a"normalities or exist on their o n. The most common anomaly is dysplasia of the mem"ranous la"yrinth% although dysplasia of the "ony la"yrinth and e!en total aplasia of the ear may occur. 'ntrauterine infections% including ru"ella% toxoplasmosis and cytomegalo!irus% can cause inner ear damage. @erinatal hypoxia% 4aundice and pre& maturity are also ris# factors for a hearing loss. After "irth% meningitis may cause profound sensorineural hearing loss. 'f a child(s parents suspect a hearing impairment it is important to "elie!e them% especially hen glue ear has "een excluded. 'n children in hom there is a suspicion of sensorineural hearing loss% "rainstem e!o#ed audiometry is used to esta"lish hearing thresholds *-ig. ./.5$+. 'f some hearing is present% the early fitment of hearing aids can maximise the neural plasticity that is present in the de!eloping "rain. 'f a child has a profound hearing loss% then early inter!ention ith a cochlea implant may he appropriate *-ig. ./.5.+. Most cases of profound sensorineural hearing loss are due to loss of cochlear hair cells so that an implant inserted through the round indo can selecti!ely stimulate the cochlear neurons hich usually remain intact. )egenerati!e @res"yacusis% a degenerati!e disorder% is a term used to descri"e the hearing loss of old age. 't is characterised "y a gradual loss of hearing in "oth ears% ith or ithout tinnitus. The hearing loss usually affects the higher fre3uencies and a classical audiogram is sho n in -ig. ./.58. The consonants of speech lie ithin the high&fre3uency range hich ma#es speech discrimination difficult. Examination of an elderly person s cochlea sho s loss of hair cells% particularly at the "asal turn of the cochlea. 7ith ageing the dynamic range of hearing is also reduced so that elderly people often find loud noises uncomforta"le. This phenomenon is #no n as Grecruitment(. Many patients ith pres"ycusis are concerned that they may lose their hearing completely and need reassurance. Hearing aid technology has impro!ed dramatically and most patients no "enefit *-ig. ./.5C+. :are and attention to detail hen fitting the hearing aid are essential% together ith monitoring the patient(s progress. 'f this does not occur the hearing aid ends up in the "edroom dra er. Tinnitus descri"es an a"normal noise that appears to come from the ear or ithin the head. 't may ha!e an extrinsic cause% for example the pulsatile tinnitus of a glomus 4ugularae tumour. Bsually% ho e!er% the tinnitus is generated ithin the cochlea% and most people ill experience tinnitus at some time in their life.

Tinnitus fre3uently accompanies pres"ycusis% as ell as any condition that damages the inner ear structures. Most indi!iduals adapt to the presence of tinnitus "ut in some patients it pro!es intrusi!e. Reassurance and relaxation therapy are highly effecti!e% as is a hearing aid for patients ho also ha!e pres"ycusis. An ENT surgeon ho as a #een fisherman found that he could not hear his tinnitus hen fishing next to a aterfall. -rom this o"ser!ation tinnitus mas#ers ha!e "een de!eloped *-ig. ./.5D+. A mas#er pro!ides a similar noise to the tinnitus and G"lan#s it Aut. Trauma Trauma to the inner ear can "e caused "y noise or direct in4ury. Hair cells ithin the cochlea are damaged "y sudden acoustic trauma *"last in4ury or gun fire+ or "y prolonged exposure to excessi!e noise. The sensorineural hearing loss that results is greatest at high fre3uencies *particularly ./// H=+ and is often accompanied "y tinnitus *-ig. ./.5E+. The la in the B1 re3uires that or#ers are protected from noise% "ut in a disco an indi!idual relies on common senseK The otic capsule is the hardest "one in the "ody "ut if trauma to the head is se!ere temporal "one fractures may occur. These tend to "e either longitudinal *E/ per cent+ or trans!erse *5/ per cent+. Trans!erse fractures usually in!ol!e the la"yrinth and lead to a sensorineural hearing loss hich is permanent. @rofound !ertigo occurs initially follo ed "y gradual compensation. 'n a"out 8/ per cent of cases there is an associated facial ner!e paralysis.7hen assessing a se!erely in4ured patient% it is important to record the facial ner!e function and% in particular% hether any facial ea#ness is partial or total. Total facial paralysis immediately follo ing a head trauma suggests a ma4or in4ury to the ner!e and under certain circumstances exploration of the facial ner!e to decompress it or repair it may "e appropriate. ,ongitudinal fractures usually spare the la"yrinth "ut fre3uently in!ol!e the external meatus and roof of the middle ear.'mportant physical findings that may accompany a s#ull "ase fracture include a haematoma o!er the mastoid "one *Battle(s sign+% "lood in the external ear or a laceration along the roof of the external canal. :ere"rospinal fluid *:S-+ otorrhoea or :S- rhinorrhoea *if the tympanic mem"rane is intact+ may occur. A conducti!e hearing loss may "e present "ecause of fluid in the middle ear or disruption of the ossicular chair. A high&resolution :T scan is re3uired to assess s#ull "ase fractures. -acial paralysis ?Thin# complete or partial ?@rotect the eye ?Atoneurological examination to find cause ?Early treatment ith steroids andFor anti!iral therapy dependent on aetiology Barotrauma is a rare cause of a sudden sensorineural hearing loss *SNH,+ or acute !esti"ular distur"ance. Rapid changes in pressure across the la"yrinthine mem"ranes may occur ith di!ing or flying and may allo air to "e forced into the cochlea. Any indi!idual ith a sudden sensorineural hearing loss re3uires urgent hospital admission% and in those ith a history of "arotrauma it may "e appropriate to raise the tympanic mem"rane and to search for a lea# of pen&lymph in the region of the o!al or round indo . )rug ototoxicity is a form of trauma that may damage the inner ear. Some drugs differentially affect the cochlea causing hearing loss and tinnitus hile others pic# out the !esti"ular system causing !ertigo. Aminoglycocides are ell #no n to "e ototoxic% as is cisplatinum. Recognition of ris# factors% such as poor renal function in patients "eing treated ith aminoglycocides% is most important. Although many topical ear drops contain aminoglycocides% there is little

e!idence that such topical treatment causes sensorineural hearing loss if used for short periods. Benign paroxysmal positional !ertigo *B@@9+ may follo head or nec# trauma. 9ertigo is an illusion of mo!ement and B@@9 is characterised "y intermittent attac#s of !ertigo that occur hen the head is mo!ed in a certain position. Typically the !ertigo only lasts for a fe seconds and is not associated ith other otological symptoms. @ositional testing can e!o#e nystagmus and helps in the diagnosis of this condition. The condition is usually self&limiting and special manoeu!res descri"ed "y Epley help the ma4ority of patients *-ig. ./.5>+. 9ascular occlusion A reduction in la"yninthine "lood flo ith associated hypoxia is the most li#ely cause for most cases of sudden onset of se!ere sensorineural hearing loss. All patients ith a sudden sensorineural hearing loss should "e referred immediately for specialist treatment. The treatment consists of "ed rest% steroids and% in some centres% the administration of :ar"ogen *an oxygen and car"on dioxide mixture+. -i!e per cent of acoustic neuromas present ith a sudden sensorineural hearing loss and therefore radiological in!estigation% prefera"ly ith magnetic resonance imaging *MR'+% is re3uired to exclude this tumour. 'nflammatory disorders 'nflammation caused "y a !iral infection is thought to account for acute !esti"ular failure *!esti"ular neuroritis+. This condition is characterised "y a sudden onset of !ertigo. The !ertigo is so se!ere that the patient often ta#es to his or her "ed for "et een 5 and 8days. :entral compensation then occurs% although recurring episodes of !ertigo for up to 0E months can occur. This is thought to "e due to incomplete compensation for the original !esti"ular damage. The aetiology of Mnires disease is not #no n. The condition is characterised "y a triad of symptoms. 'ntermittent attac#s of !ertigo% a fluctuating sensorineural hearing loss and tinnitus. The patient often has a sensation of pressure in the affected ear "efore an attac#. The hearing loss typically affects the lo er fre3uencies and is !irtually the only type of sensorineural hearing loss that fluctuates. The time&course of the !ertigo characteristically lasts "et een $/ minutes and C hours. 't is often accompanied "y nausea and !omiting. Although the cause of the condition is un#no n% the pathology is ell documented. There is an excessi!e accumulation of endolymphatic fluid *hydrops+ and it is thought that the distension of the endolymphatic compartment may rupture Reissner(s mem"rane hich leads to mixing of endolymph and perilymph. This is the "asis for the cochlear6!esti"ular failure hich characterises the condition. The in!estigations include puretone audiometry% electrocochleography and MR' scan if a!aila"le. The latter is re3uired to exclude an acoustic neuroma hich may mimic the symptoms of MLniMre(s disease. 9iral infections that in!ol!e the facial ner!e are possi"ly one of the commonest causes of facial ea#ness *E/ per cent+. Bell(s palsy results from a !iral infection of the facial ner!e. The ner!e s ells and is compressed in its la"yninthine portion as it passes from the internal auditory meatus to ards the middle ear. 'f the patient presents ithin the first .E hours% treatment ith high&dose steroids is appropriate. Not all facial ner!e palsies are due to !iral infection and a thorough otoneurological examination is re3uired. The facial ner!e can "e damaged ithin the "rainstem at the cere"ellopontine angle% ithin the internal auditory meatus% ithin the middle ear% at the s#ull "ase and ithin the parotid. 't is essential to consider these potential sites of facial ner!e damage in any patient ith 9''th ner!e paralysis.

Ramsey Hunt(s syndrome is caused "y herpes =oster !irus. 't is characterised "y a facial palsy and is often associated ith facial pain and the appearance of !esicles on the ear drum% ear canal and pinna *-ig. ./.$/+. 9ertigo and sensorineural hearing loss *9'''th ner!e+ accompany it. Treatment ith aciclo!ir is effecti!e if gi!en early. Meta"olic causes The meta"olic causes of inner ear damage include dia"etes mellitus and thyroid disease% "oth of hich may cause sensorineural hearing loss. Neoplasms Tumours of the inner ear are uncommon "ut can present ith sensorineural hearing loss% tinnitus and !ertigo. Acoustic neuromas% hich are actually Sch annomas of the !esti"ular di!ision of the 9'''th ner!e% are the most common% follo ed "y meningiomas. Acoustic neuromas gro slo ly and some hat unpredicta"ly and as they expand can cause cranial ner!e palsies% "rainstem compression and raised intracranial pressure. The early symptoms are a unilateral sensorineural hearing loss or unilateral tinnitus% or "oth. 't is important to diagnose these tumours early and remo!e them hen they are small. The mor"idity and mortality from surgery is directly related to tumour si=e. 'f the tumour is remo!ed hen it is small% there is an extremely good chance of preser!ing facial ner!e function. The in!estigation of choice for detecting acoustic neuromas is MR' *-ig. ./.$0+. 'n many centres patients ith any unilateral otological symptoms are screened using MR'. 'f MR' is not a!aila"le% :T scanning is the next "est diagnostic tool. Accasionally% in an elderly patient% Ga ait and see( policy may "e adopted. 'n such patients repeat MR's can "e used to monitor the tumour. There are three main surgical approaches for resecting acoustic neuromas. A temporal craniotomy and a direct middle fossa approach is used for small tumours only. The transla"yrinthine approach is entirely through the ear and does not "reach the dura. The neurosurgeon and ENT surgeon or# together. There is minimal distur"ance to the patient% although the hearing is completely destroyed. A su"occipital approach is performed "y a neurosurgeon doing a craniotomy ith the ENT surgeon remo!ing "one at the internal auditory meatus. Traction on the cere"ellum is re3uired. This increases the mor"idity. 'n some cases% ho e!er% hat remains of the hearing can "e preser!ed using this approach. The physiological monitoring of the facial ner!e and auditory ner!e during surgery has impro!ed acoustic ner!e surgery results The inner ear ?@res"ycusis2 "ilateral high&fre3uency loss ? Bnilateral tinnitus or sudden sensory neural hearing loss needs to "e in!estigated for possi"le acoustic neuroma ?Sudden sensory neural hearing loss needs immediate treatment -urther reading Booth% J. *ed.+ *0>>D+ Scott&Bro n(s Atolaryngology% Cth edn% 9ol. $% Butter orth& Heinemann% Axford. ,udman% H. and 7right% A. *eds+ *0>>E+ Ma son(s )iseases of the Ear% Cth edn% Arnold% ,ondon. 9an Hassell% A.% Milford% :.A. and Bleach% N. *eds+ *0>>D+ Aperati!e Atolaryngology% Blac# ell Scientific% Axford.