8 otosclerosis

Otosclerosis Otosclerosis

Hongyan Jiang MD&PhD

Otorhinolaryngology Hospital,The First Affiliated Hospital of Sun Yat-sen University

Background Background Definition Definition

primary metabolic bone disease of the otic capsule primary metabolic bone disease of the otic capsule and ossiclesand ossicles

otosclerosis is recognized as an alteration in bony metaboliotosclerosis is recognized as an alteration in bony metabolism of the endochondral bone of the otic capsule.sm of the endochondral bone of the otic capsule.

It causes fixation of the ossicles (stapes)It causes fixation of the ossicles (stapes) It results in conductive or mixed hearing loss. It results in conductive or mixed hearing loss. It is genetically-mediated via autosomal dominant It is genetically-mediated via autosomal dominant

transmissiontransmission

The term otosclerosis is derived from the Greek words for “hardening of the ear.”

Politzer first recognized otosclerosis in 1893

PREVALENCE

RaceRace prevalence rate

CaucasianCaucasian 8.3% 8.3%

AsianAsian 1%1%

African AmericanAfrican American 0.5% 0.5%

Native AmericanNative American 0% 0%

In postmortem examinations of temporal bones

PREVALENCE

Sex variation (M:F=1:2.5)Sex variation (M:F=1:2.5) Women more commonly seek medical attenWomen more commonly seek medical atten

tion for hearing loss secondary to otosclerotion for hearing loss secondary to otosclerosis, sis,

histologic studies prevalence of otosclerosis histologic studies prevalence of otosclerosis show no difference in men versus women. show no difference in men versus women.

PREVALENCE

Age Age The incidence of otosclerosis increases with The incidence of otosclerosis increases with

age. age. The most common age group presenting wiThe most common age group presenting wi

th hearing loss from otosclerosis is 15-45 yeth hearing loss from otosclerosis is 15-45 years, ars,

however it has been reported to manifest as however it has been reported to manifest as early as 7 years and as late as the mid 50s. early as 7 years and as late as the mid 50s.

Etiology Etiology

Many theories have been proposed such as Many theories have been proposed such as hereditary, 54% of patients present with family history hereditary, 54% of patients present with family history && endocrine, women with pregnancy worse her hearingendocrine, women with pregnancy worse her hearing metabolic, enzyme abnormal was pathogenmetabolic, enzyme abnormal was pathogen infectious, virus was identified in the lesion infectious, virus was identified in the lesion ((measles virus) vascular,vascular, autoimmune, autoimmune,

none have be proven. Hormonal factors have been sunone have be proven. Hormonal factors have been suggested to play a role in otosclerosis based on the obsggested to play a role in otosclerosis based on the observation that pregnancy sometimes accelerates the prervation that pregnancy sometimes accelerates the progression of the disease. ogression of the disease.

PathophysiologyPathophysiologyThe otosclerotic process is divided into two phases

histologically. the early phase-otospongiosis(spongy-like appearance)

----Bone resorption and increased vascularity. ““Schwartze's sign”: The Schwartze's sign”: The increased vascularity is similar to h

yperemia, it can be seen grossly as red hue behind the TMcan be seen grossly as red hue behind the TM

the late stage-otosclerosis ----The reabsorbed bone is replaced with dense sclerotic

bone.

PathophysiologyPathophysiology If only the footplate is involved, it is sometimes referred to If only the footplate is involved, it is sometimes referred to

as a “as a “stapedial otosclerosisstapedial otosclerosis”. ”. When the entire footplate and annular ligament are involveWhen the entire footplate and annular ligament are involve

d it is known as an “ d it is known as an “ obliterated footplate obliterated footplate ” or “ ” or “ obliterativobliterative otosclerosis e otosclerosis ”. ”.

IfIf the cochlea or the labyrinthine is involved is named “is involved is named “cochlea or labyrinthine otosclerosis otosclerosis ””

The round window is involved in approximately 30% to 50The round window is involved in approximately 30% to 50% of cases% of cases

Symptoms Symptoms

Hearing lossHearing loss: Slowly progressive, bilateral (80%), asymmetric, conductive hearing loss.: Slowly progressive, bilateral (80%), asymmetric, conductive hearing loss.

TinnitusTinnitus: is associated with 75% patients: is associated with 75% patients

Vestibular symptoms:are uncommon.

Paracusis of Willis:Some patients report improved speech understanding in a noisy environm

ent.

The age of onset of hearing loss is youngThe age of onset of hearing loss is young History of significant ear infections makes the diagnosis of otosclerosis less likely. History of significant ear infections makes the diagnosis of otosclerosis less likely. 25% of patients present with some vestibular complaints 25% of patients present with some vestibular complaints

ExaminationPhysical examination A normal appearance of the external auditory canal TM appears normal in the majority of patients. Schwartze’s sign: a reddish hue over the promontory caused by increased vascularit

y of the bone immediately under the periosteum.

----may be seen in the early stages of the disease ---- is not present in all patients.

Examination

TestsTests Rinne test: negative Rinne test: negative

Early in the disease, low frequency CHL will predominate resultEarly in the disease, low frequency CHL will predominate resulting in a negative Rinne test with the 256-Hz only. ing in a negative Rinne test with the 256-Hz only.

As progression occurs, the 512 and then the 1,024-Hz TF will beAs progression occurs, the 512 and then the 1,024-Hz TF will become negative.come negative.

Weber test: laterization to poor HLWeber test: laterization to poor HL Schwabach test: prolonged bone conductionSchwabach test: prolonged bone conduction Gelle test: negativeGelle test: negative

ExaminationTestsTests Pure tone audiometryPure tone audiometry

Early stage: a decrease in air conduction in the low frequency, especialEarly stage: a decrease in air conduction in the low frequency, especially below 1000 Hz. ly below 1000 Hz.

As the disease progresses, the air line flattens. because the otosclerotic As the disease progresses, the air line flattens. because the otosclerotic focus has a mass affect on the entire system, focus has a mass affect on the entire system, carhart notch is noted. carhart notch is noted. &&

Further progression of otosclerosis to involve the cochlea may result in Further progression of otosclerosis to involve the cochlea may result in increased bone conduction thresholds in high frequency, A-B gap existincreased bone conduction thresholds in high frequency, A-B gap exists in low frequency. s in low frequency.

More isolated cochlear otosclerosis may sometimes result in a mixed hMore isolated cochlear otosclerosis may sometimes result in a mixed hearing loss with a “cookie-bite” pattern with both air and bone lines.earing loss with a “cookie-bite” pattern with both air and bone lines.

TestsTests Type As (s-stiffness curve) tympanogram Type As (s-stiffness curve) tympanogram

and is characteristic of advanced otoscland is characteristic of advanced otosclerosis but more commonly, malleus fixaerosis but more commonly, malleus fixation. tion.

Examination

Image study Image study

CT can characterize the extent of the otosclerotic CT can characterize the extent of the otosclerotic focus at the oval window focus at the oval window

CT scan can exclude capsular involvement when CT scan can exclude capsular involvement when patients have significant mixed hearing loss patients have significant mixed hearing loss

An enlarged cochlear aqueduct may be seen whicAn enlarged cochlear aqueduct may be seen which potential causes perilymph gusher during footph potential causes perilymph gusher during footplate fenestration or removal. late fenestration or removal.

It reveal normal round window and normal mastIt reveal normal round window and normal mastoid pneumatization.oid pneumatization.

DiagnosisDiagnosis

According to Symptoms and examination,it is According to Symptoms and examination,it is easy to make the clinical diagnosis.easy to make the clinical diagnosis.

Other assisted events:Other assisted events: low-volume speech.low-volume speech.

conductive nature of their hearing loss, they conductive nature of their hearing loss, they perceive there voice as louder than it actually is. perceive there voice as louder than it actually is.

Two-thirds of patients will report a Two-thirds of patients will report a family family history of hearing loss. history of hearing loss.

Women with pregnancy worse her hearingWomen with pregnancy worse her hearing

Differential diagnosisDifferential diagnosis

Ossicular discontinuityOssicular discontinuity conductive loss of 60 db usually without senconductive loss of 60 db usually without sen

sorineural componentsorineural component flaccid tympanic membrane on pneumatic flaccid tympanic membrane on pneumatic

otoscopyotoscopy type Ad tympanogramtype Ad tympanogram


Congenital stapes fixationCongenital stapes fixation Family history less likely (10%)Family history less likely (10%) usually detected in the first decade of lifeusually detected in the first decade of life 25% incidence of other congenital anomalie25% incidence of other congenital anomalie

s (3% for juvenile otosclerosis)s (3% for juvenile otosclerosis) non-progressive CHLnon-progressive CHL


Malleus head fixationMalleus head fixation when when congenitalcongenital, associated with other stig, associated with other stig

mata (aural atresia)mata (aural atresia) presence of presence of tympanosclerosistympanosclerosis pneumatic otoscopypneumatic otoscopy almost always associated with type As tympalmost always associated with type As tymp

anogram (only in advanced otosclerosis)anogram (only in advanced otosclerosis)


Paget’s diseasePaget’s disease - diffuse involvement of the bony skeleton- diffuse involvement of the bony skeleton - elevated alkaline phosphatase- elevated alkaline phosphatase - CT - diffuse, bilateral, petrous bone involvement - CT - diffuse, bilateral, petrous bone involvement

with extensive with extensive -de-mineralization-de-mineralization - More commonly crowds the ossicles in the epitym- More commonly crowds the ossicles in the epitym

panum, partially fixing panum, partially fixing the ossicular chainthe ossicular chain


Osteogenesis imperfectaOsteogenesis imperfecta presence of blue sclerapresence of blue sclera multiple bone fracturesmultiple bone fractures CT – more common involves the otic capsulCT – more common involves the otic capsul

e and to a greater extente and to a greater extent

Surgical interventionsSurgical interventions

The best surgical candidate The best surgical candidate good health with a socially unacceptable ABG,good health with a socially unacceptable ABG,

a negative Rinne test, a negative Rinne test, excellent discrimination, excellent discrimination, the desire for surgery after an appropriate perthe desire for surgery after an appropriate per

iod of time for deliberation.iod of time for deliberation. Younger patients are more likely to develop rYounger patients are more likely to develop r

e-ossification of the stapes footplate over theie-ossification of the stapes footplate over their lifetime. r lifetime.


Most authors discourage performing staMost authors discourage performing stapes surgery in patients with Meniere's dipes surgery in patients with Meniere's disease, especially when it is active. sease, especially when it is active.


StapedotomyStapedotomy Less trauma to the oval wiLess trauma to the oval wi

ndowndow Less possibility of damagiLess possibility of damagi

ng to the inner earng to the inner ear In addition, revision surgeIn addition, revision surge

ry, if required, is easier dury, if required, is easier due to preserved anatomye to preserved anatomy

stapedectomystapedectomy

Non-surgical Non-surgical interventionsinterventions

Amplification:Amplification: hearing aide hearing aide Patients who do not want to undergo surgerPatients who do not want to undergo surger

y for otosclerosisy for otosclerosis patients who are not fit for surgery. patients who are not fit for surgery.


Medical treatment:Medical treatment: Usual dose is about 20-120mg of fluoride a dayUsual dose is about 20-120mg of fluoride a day Efficacy of the treatment can be evaluated 2 years lEfficacy of the treatment can be evaluated 2 years l

ater. ater. Schwartze’s sign, and the degree of tinnitus and imbalanSchwartze’s sign, and the degree of tinnitus and imbalan

ce are reassessed, and a CT scan is repeated. ce are reassessed, and a CT scan is repeated. Once the disease was stable, the patient is placed oOnce the disease was stable, the patient is placed o

n a life-ling maintenance dose of about 25mg of flun a life-ling maintenance dose of about 25mg of fluoride a day. oride a day.

50% of patients have stabilization of their disease, 50% of patients have stabilization of their disease, 30% improve, and the rest continue to progress. 30% improve, and the rest continue to progress.


Indications for medical treatment Indications for medical treatment Not surgical candidates, Not surgical candidates, Decide against surgery, Decide against surgery, Patient with SNHL or vestibular symptoms Patient with SNHL or vestibular symptoms positive Schwartze’s sign may be given fluoride trpositive Schwartze’s sign may be given fluoride tr

eatments for 6-12 months prior to surgery to induceatments for 6-12 months prior to surgery to induce the focus to mature and potentially prevent the pe the focus to mature and potentially prevent the progression of disease after surgery.rogression of disease after surgery.

determined to be active during surgery, determined to be active during surgery, postoperative treatment can be initiated. postoperative treatment can be initiated.

Etiology Etiology

hereditaryhereditary A genetic component has long been recognized Transmission has generally been accepted to be autoso

mal dominant with incomplete penetrance. The gene for otosclerosis has not been clearly identified ----authors in one study narrowed its location to chromosome 15q25-26 ----Others have related otosclerosis to the COL1A1 gene that encodes for type 1 collagen. &

Tests Tests Carhart notchCarhart notch

Carhart’s notch is the hallmark audiologic sign of otosclerois the hallmark audiologic sign of otosclerosis. sis.

Carhart’s notch is characteristic of otosclerosis and appears as a sensorineural hearing loss at 2 kHz that is spurious since the bone conduction in the mid-frequency range is not reliable. &

Meniere’s DiseaseMeniere’s Disease



What is Meniere’s Disease?What is Meniere’s Disease? In 1861 Prosper Meniere described a syndromIn 1861 Prosper Meniere described a syndrom

e characterized by e characterized by deafness, tinnitus, and episdeafness, tinnitus, and episodic vertigoodic vertigo. He linked this condition to a disor. He linked this condition to a disorder of the inner ear.der of the inner ear.

In 1938 Hallpike and Cairns described the undIn 1938 Hallpike and Cairns described the underlying pathology of Meniere’s disease as beierlying pathology of Meniere’s disease as being ng endolymphatic hydropsendolymphatic hydrops but the precise eti but the precise etiology still remains elusive. ology still remains elusive.

Incidence is 4/100000 in Incidence is 4/100000 in Japan. 15/100000 in US, Japan. 15/100000 in US, 46/100000 in Sweden, 1046/100000 in Sweden, 100/100000 in UK.0/100000 in UK.

The large differences are The large differences are due to geographic, genetdue to geographic, genetic, ethnic or environmentic, ethnic or environmental factors, or different diaal factors, or different diagnostic criteria.gnostic criteria.

PrevalencePrevalence

Women>MenWomen>Men

Anatomical-abnormalitiesAnatomical-abnormalities Radial circulationRadial circulation Longitudinal flowLongitudinal flow

Immunological-immune complex depositionImmunological-immune complex deposition Vascular-associated with migrainesVascular-associated with migraines Genetic-autosomal dominant Genetic-autosomal dominant Viral-serum IgE to herpes simples virus types I Viral-serum IgE to herpes simples virus types I

and II, Epstein-Barr virus and CMVand II, Epstein-Barr virus and CMV Metabolic-potassium intoxicationMetabolic-potassium intoxication

Possible CausesPossible Causes

Normal membranous labyrinth Dilated membranous labyrinth in Meniere's disease (Hydrops)

Periodic episodes of Periodic episodes of rotatory rotatory vertigovertigo or or didizzinesszziness

Fluctuating, progressFluctuating, progressive, low-frequency ive, low-frequency hhearing lossearing loss

TinnitusTinnitus Fullness/pressureFullness/pressure

SymptomsSymptoms

DiagnosisDiagnosis

The diagnosis of Meniere disease is made baseThe diagnosis of Meniere disease is made based on a careful history and physical exam.d on a careful history and physical exam.

If the work-up is normal and the classic symptomIf the work-up is normal and the classic symptoms continue, the diagnosis of Meniere disease is s continue, the diagnosis of Meniere disease is made.made.

HistoryHistory

Most important part of the diagnosis Most important part of the diagnosis Pattern of symptoms Pattern of symptoms Association between hearing loss, tinnitus, Association between hearing loss, tinnitus,

and vertigo and vertigo

Physical ExaminationPhysical Examination

Examination results vary, depending upon the phase Examination results vary, depending upon the phase of disease. of disease. During remissionDuring remission, physical examination fin, physical examination findings may be completely normal, particularly if the padings may be completely normal, particularly if the patient is symptom free.tient is symptom free.

During an During an acute attackacute attack, the patient has severe vertigo., the patient has severe vertigo. Spontaneous nystagmusSpontaneous nystagmus directed toward affected ear directed toward affected ear

is typical during an acute attack. is typical during an acute attack.

Physical Examination (con’t)Physical Examination (con’t)

The The Romberg testRomberg test generally shows significant instability generally shows significant instability and worsening when the eyes are closed.and worsening when the eyes are closed.

The Weber tuning fork test usually lateralizes away from The Weber tuning fork test usually lateralizes away from the affected ear.the affected ear.

The Rinne test usually indicates that air conduction remaThe Rinne test usually indicates that air conduction remains better than bone conduction.ins better than bone conduction.

Complete neurologic evaluation is important. New-onset Complete neurologic evaluation is important. New-onset vertigo might be an early sign of stroke, migraine, or braivertigo might be an early sign of stroke, migraine, or brainstem compression that may require emergent evaluationstem compression that may require emergent evaluation and care.n and care.

Lab studiesLab studies No lab studies are specific for Meniere disease.No lab studies are specific for Meniere disease. CT scansCT scans reveal dehiscent superior semicircular reveal dehiscent superior semicircular

canals and/or widened cochlear and vestibular acanals and/or widened cochlear and vestibular aqueductsqueducts

AudiometryAudiometry is particularly helpful to document pr is particularly helpful to document present hearing acuity and to detect future change.esent hearing acuity and to detect future change.

----Typically, the lower frequencies are affected ----Typically, the lower frequencies are affected more severely.more severely.

Lab studiesLab studies Electrocochleography (ECOG)Electrocochleography (ECOG)

ECOG measures the ECOG measures the ratioratio of the of the summating potesummating potentialntial (probably from the movement of the basilar (probably from the movement of the basilar membrane) and the nerve membrane) and the nerve action potentialaction potential in res in response to auditory stimuli. Hydrops is suggested ponse to auditory stimuli. Hydrops is suggested when when this ratio is greater than 35-40%.this ratio is greater than 35-40%.

Possible Meniere’s disease Episodic vertigo without documented hearing

loss Sensorineural hearing loss, fluctuating or fixed

Probable Meniere’s disease One definite episode of vertigo Audiometrically documented hearing loss on a

t least one occasion Tinnitus and aural fullness

Diagnostic Scale for Meniere’s Disease of the American Academy of Otolaryngology-Head and Neck Surgery

Definitive Meniere’s disease Two or more episodes of vertigo of at least 20 min Audiometrically documented hearing loss on at lea

st one occasion Tinnitus and aural fullness

Certain Meniere’s disease Definitive Meniere’s disease, plus histopathologic confirmation

Diagnostic Scale for Meniere’s Disease of the American Academy of Otolaryngology-Head and Neck Surgery

In all scales, other causes must be excluded using any technical

methods (eg, imaging, laboratory, etc).

Differential DiagnosisDifferential Diagnosis

The differential diagnosis is broad and includeThe differential diagnosis is broad and includes:s:perilymph fistula, recurrent labyrinthitis, otoscperilymph fistula, recurrent labyrinthitis, otosclerosis, migraine , congenital ear malformatiolerosis, migraine , congenital ear malformations of many kinds,viral meningitis, viral encephns of many kinds,viral meningitis, viral encephalitis, neurosyphilis, stroke, tumors, trauma, aalitis, neurosyphilis, stroke, tumors, trauma, autoimmune disorders, MS, etc.utoimmune disorders, MS, etc.

Differential DiagnosisDifferential DiagnosisDifferential Diagnosis of Vertigo Based on Time Frame of Vertigo and Presence or Absence of Hearing Loss

TreatmentTreatment

Salt restrictionSalt restriction SedativeSedative

The key is to use sedative brieflyThe key is to use sedative briefly Prolonged use of sedative impair compensaProlonged use of sedative impair compensa

tion, prolongs symptoms and produces a sution, prolongs symptoms and produces a suboptimal result.boptimal result.

DiureticDiuretic

TreatmentTreatment Short course of steroid is reasonableShort course of steroid is reasonable

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Intratympanic gentamicin injectinIntratympanic gentamicin injectin Medical therapy failsMedical therapy fails Intratympanic injection is recommendedIntratympanic injection is recommended Less than 5% patients progress to the stage where Less than 5% patients progress to the stage where

destructive treatment is indicated.destructive treatment is indicated.

Surgical interventions Surgical interventions Endolymphatic sac Endolymphatic sac decodeco

mpression or shuntmpression or shunt Vestibular neurectomyVestibular neurectomy LabyrinthectomyLabyrinthectomy

PrognosisPrognosis

Prognosis is variablePrognosis is variable, since the disease pattern , since the disease pattern of exacerbation and remission makes evaluation of exacerbation and remission makes evaluation of treatment and prognosis difficult to predict.of treatment and prognosis difficult to predict. In general, Ménière symptoms tend to stabilize spontaIn general, Ménière symptoms tend to stabilize sponta

neously with time. With regard to vertigo, about half of neously with time. With regard to vertigo, about half of patients stabilize over several years.patients stabilize over several years.

Patients tend to "burn out" over time and with residual Patients tend to "burn out" over time and with residual poor balance and hearing.poor balance and hearing.

Prognosis Prognosis Cont’dCont’d

Ménière disease can be classified into several stMénière disease can be classified into several stages of progression. Early stages involve cochleages of progression. Early stages involve cochlear hydrops, which proceeds to affect the vestibular hydrops, which proceeds to affect the vestibular system.ar system. Ménière disease is most bothersome during these earMénière disease is most bothersome during these ear

ly stages.ly stages. As patients progress to later stages, the hydrops fills tAs patients progress to later stages, the hydrops fills t

he vestibule so completely that no further room is avaihe vestibule so completely that no further room is available for pressure fluctuation and the vertigo spells dislable for pressure fluctuation and the vertigo spells disappear.appear.

The acute attacks are replaced by constant imbalance The acute attacks are replaced by constant imbalance and progressive hearing loss.and progressive hearing loss.

House institution experiencesHouse institution experiences

ESS is the first line of surgical treatment ESS is the first line of surgical treatment for MD unresponsive to medical treatmefor MD unresponsive to medical treatment ( diuretic and vasodilator therapy)nt ( diuretic and vasodilator therapy)

In case with disable vertigo unresponsivIn case with disable vertigo unresponsive to medical treatment or failing ESS, VNe to medical treatment or failing ESS, VNS is recommended and often combine wS is recommended and often combine with primary or revision ESS ith primary or revision ESS

House institution experiencesHouse institution experiences

Concurrent ESS and VNS does not imprConcurrent ESS and VNS does not improvement hearing or tinnitus outcome ovovement hearing or tinnitus outcome over vestibular nerve section aloneer vestibular nerve section alone

Karolinska hospital policyKarolinska hospital policy Patients with MD still having serviceable heariPatients with MD still having serviceable heari

ng were primarily offered ELSng were primarily offered ELS In total loss of cochlear function or persisting sIn total loss of cochlear function or persisting s

ymptoms after a previous ESS, intratympanic ymptoms after a previous ESS, intratympanic gentamicin injection was chosen. gentamicin injection was chosen.

In patients with normal cochear function and In patients with normal cochear function and non Menieriform peripheral vestibular dysfuncnon Menieriform peripheral vestibular dysfunction, vestibular neurectomy was recommendetion, vestibular neurectomy was recommended.d.

Karolinska hospital policyKarolinska hospital policy As compared with destructive procedures, sucAs compared with destructive procedures, suc

h as labyrinthectomy and neurectomy that resh as labyrinthectomy and neurectomy that result in severe vertigo postoperatively.ult in severe vertigo postoperatively.

Intratympanic gentamicin injection seldom forIntratympanic gentamicin injection seldom force the patient to require bed rest and physical ce the patient to require bed rest and physical inactivity, due to slowly declining vestibular iinactivity, due to slowly declining vestibular impairment, patients only feel slightly unsteadmpairment, patients only feel slightly unsteadness.ness.

LabyrinthectomyLabyrinthectomy Transcanal labyrinthectomyTranscanal labyrinthectomy

Semicircular canals remain intact.Semicircular canals remain intact. Gentamicin us usually placed in the ear as wellGentamicin us usually placed in the ear as well

Transmastoid labytinthectomyTransmastoid labytinthectomy Drilling the semicircular canalsDrilling the semicircular canals Opening the vestibule and destroying the saccule aOpening the vestibule and destroying the saccule a

nd utricle.nd utricle. Gentamicin usually placed in the ear as wellGentamicin usually placed in the ear as well

Deafness and its Deafness and its rehabilitationrehabilitation



Background Background

Speech frequency 500-3000HzSpeech frequency 500-3000Hz Normal hearing Normal hearing

Sound conductionSound conduction Sound perception (cochlea) Sound perception (cochlea) Sound analysis (retro-cochlea)Sound analysis (retro-cochlea)

Deafmutism: Deafmutism: Profound hearing loss. Profound hearing loss. No language speech perceptionNo language speech perception Language development quit (6M-6Y)Language development quit (6M-6Y)

Postlingual deafnessPostlingual deafness deafness present following language developeddeafness present following language developed

MorbidityMorbidity

1/1000 in new born baby1/1000 in new born baby 1/100 in younger1/100 in younger 14/100 in middle age14/100 in middle age 30/100 in 65-75 year old (presbycusis)30/100 in 65-75 year old (presbycusis) 50/100 in >75 year old50/100 in >75 year old

Classification Classification

Conductive deafnessConductive deafness Sensorineural deafnessSensorineural deafness

Sensory deafness Sensory deafness Lesion located in cochlea Lesion located in cochlea

( cochlear deafness)( cochlear deafness) Nervous deafnessNervous deafness

Lesion located in retrocoLesion located in retrocochlea (retrocochlea deafchlea (retrocochlea deafness)ness)

Mixed deafnessMixed deafness

ClassificationClassification

Congenital deafness Congenital deafness Hereditary deafnessHereditary deafness Non hereditary deafnessNon hereditary deafness

Acquired deafnessAcquired deafness Prelingual deafnessPrelingual deafness Postlingual deafnessPostlingual deafness

Degree of hearing lossDegree of hearing loss

Based on PTA in 500, 1k, 2k Hz (WHO Based on PTA in 500, 1k, 2k Hz (WHO 1980 criteria)1980 criteria) Mild HL (<40 dB)Mild HL (<40 dB) Middle HL (41-55dB)Middle HL (41-55dB) Middle-severe HL (56-70 dB)Middle-severe HL (56-70 dB) Severe HL (71-90 dB)Severe HL (71-90 dB) Profound HL (>90 dB)Profound HL (>90 dB)

Conductive hearing lossConductive hearing loss

PathogenPathogen Infection Infection TraumaTrauma EAC Obstruction (foreign EAC Obstruction (foreign

body, cerumen, tumor)body, cerumen, tumor) Deformation (aural atresiDeformation (aural atresi

a, malformation of ossicua, malformation of ossicular chain, dysplasia of ovlar chain, dysplasia of oval or round window.al or round window.


Location Location Deformation of auricle (3dB)Deformation of auricle (3dB) Stenosis and autrsia of EAC (45-Stenosis and autrsia of EAC (45-

60 dB)60 dB) TM lesion (30-45 dB)TM lesion (30-45 dB) Dysfunction of ossicular chain Dysfunction of ossicular chain

(50dB)(50dB) Dysfunction of Eustachian tube Dysfunction of Eustachian tube

(60dB)(60dB) Lymphatic fluid dysfunctionLymphatic fluid dysfunction


Diagnosis Diagnosis Tuning forkTuning fork

RT: negativeRT: negative WT: lesion sideWT: lesion side ST: prolongationST: prolongation

PTAPTA Bone threshold: Bone threshold:

normal normal Air threshold: 25-60 Air threshold: 25-60

dBdB Image studyImage study


InterventionsInterventions Surgery (based on pathogen)Surgery (based on pathogen)

Ventilation tube placementVentilation tube placement MyringoplastyMyringoplasty TympanoplastyTympanoplasty Stapes surgeryStapes surgery

Hearing aidHearing aid

Sensorineuronal hearing lossSensorineuronal hearing loss Definition: damage oDefinition: damage o

f hair cell, stria vaculf hair cell, stria vacular, spinal ganglion nar, spinal ganglion neuron, auditory nerveuron, auditory nerve and central auditore and central auditory system. Pathogeny system. Pathogen

Sensorineuronal hearing lossSensorineuronal hearing loss

PathogenPathogen Congenital hearing lossCongenital hearing loss

Hereditary hearing lossHereditary hearing loss Non-syndrome hearing lossNon-syndrome hearing loss Syndrome hearing lossSyndrome hearing loss

Nonhereditary hearing lossNonhereditary hearing loss

Sensorineural hearing lossSensorineural hearing loss Acquired hearing losAcquired hearing los

ss PresbycusisPresbycusis

Sensorineural hearing lossSensorineural hearing loss Virus or bacteria Virus or bacteria

induced hearing induced hearing lossloss

Drug-induced Drug-induced hearing losshearing loss

Trauma induced Trauma induced hearing losshearing loss

Noise-induced Noise-induced hearing losshearing loss

Sensorineural hearing lossSensorineural hearing loss Idiopathic sudden seIdiopathic sudden se

nsorineural hearing lnsorineural hearing lossoss

Autoimmune inner eaAutoimmune inner ear diseaser disease

Sensorineural hearing lossSensorineural hearing loss

InterventionsInterventions Medical therapyMedical therapy HypobaroxygenHypobaroxygen Cochlear implant or auditory brainstem impCochlear implant or auditory brainstem imp

lantlant Hearing aidHearing aid Auditory speech trainingAuditory speech training Prevention Prevention

Peak, OSPL 90Peak, OSPL 90

Full on Gain, PeakFull on Gain, Peak

Frequency Range Frequency Range ((2cc.2cc.))

Battery sizeBattery size

Operating currentOperating current

Battery lifeBattery life

Behind-The-Ear InstrumentsBehind-The-Ear Instruments

11 3131 dB dB

6161 dB dB

100-6100-60000 Hz00 Hz

11 33 0.90.9

289289 hours hours

SXT 100

11 3535 dB dB

668 dB8 dB

100-100-595900 Hz00 Hz

11 33 11..00

260260 hours hours

SXT 110

11 335 dB5 dB

6767 dB dB

100-100-595900 Hz00 Hz

1313 1.1.11

SXT 115

236236 hours hours

Battery sizeBattery size

Peak, OSPL 90Peak, OSPL 90

Full on Gain, PeakFull on Gain, Peak

Frequency Frequency RangeRange ((2cc.2cc.))

Operating currentOperating current

Battery lifeBattery life

11 2121 dB dB

5757 dB dB

100-6100-65500 Hz00 Hz

312312

11..00

SXT 320

141455 hours hours

121266 dB dB

6262 dB dB

100-6800 Hz100-6800 Hz

1313

11..00

SXT 200

22 6060 hours hours

In-The-Ear InstrumentsIn-The-Ear Instruments

111177 dB dB

4646 dB dB

100-6300 Hz100-6300 Hz

1010

0.90.9

8989 hours hours

SXT 410

111177 dB dB

4545 dB dB

100-6100-65500 Hz00 Hz

1010

0.90.9

8989 hours hours

SXT 400

-10

0

10

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100

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4 Channel Instrument4 Channel Instrument

16 Channel Instrument16 Channel Instrument

Multi-Channel Multi-Channel Amplification ErrorAmplification Error

-10

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ChannelFreeInterpolates

For accuracy

ChannelFreeInterpolates

For accuracy

ChannelFreeChannelFreeTMTM PrecisionPrecision

人工耳蜗植入示意图

插入电极

植入完成

Education

8 otosclerosis