otosclerosis slides 061018 (1)

OtosclerosisOtosclerosis

Alan L. Cowan, MDAlan L. Cowan, MDTomoko Makishima, MD, PhDTomoko Makishima, MD, PhD

Department of OtolaryngologyDepartment of OtolaryngologyUniversity of Texas Medical BranchUniversity of Texas Medical Branch

Galveston, TXGalveston, TX

October 18, 2006October 18, 2006

IntroductionIntroduction

Otosclerosis Otosclerosis Primary metabolic bone disease of the Primary metabolic bone disease of the

otic capsule and ossiclesotic capsule and ossicles Results in fixation of the ossicles and Results in fixation of the ossicles and

conductive hearing lossconductive hearing loss May have sensorineural component if May have sensorineural component if

the cochlea is involvedthe cochlea is involved Genetically mediatedGenetically mediated

Autosomal dominant with incomplete Autosomal dominant with incomplete penetrance (40%) and variable expressivitypenetrance (40%) and variable expressivity

History of Otosclerosis and History of Otosclerosis and Stapes SurgeryStapes Surgery

1704 – Valsalva first described stapes fixation1704 – Valsalva first described stapes fixation 1857 – Toynbee linked stapes fixation to1857 – Toynbee linked stapes fixation to hearing losshearing loss 1890 – Katz was first to find microscopic1890 – Katz was first to find microscopic evidence of otosclerosisevidence of otosclerosis 1893 – Politzer described the clinical entity of 1893 – Politzer described the clinical entity of “ “otosclerosis”otosclerosis” 1890 – Bacon describes medical therapy for 1890 – Bacon describes medical therapy for

the condition, and supports the common view the condition, and supports the common view that “surgery should not be considered for a that “surgery should not be considered for a moment.“moment.“


Gunnar Holmgren Gunnar Holmgren (1923)(1923) Father of fenestration Father of fenestration

surgerysurgery Single stage techniqueSingle stage technique

SourdilleSourdille Holmgren’s studentHolmgren’s student 3 stage procedure3 stage procedure 64% satisfactory results64% satisfactory results


Julius LempertJulius Lempert Popularized the Popularized the

single staged single staged fenestration fenestration procedureprocedure

John HouseJohn House Further refined the Further refined the

procedureprocedure Popularized blue Popularized blue

lining the horizontal lining the horizontal canalcanal


Fenestration procedure for Fenestration procedure for otosclerosisotosclerosis Fenestration in the horizontal canal Fenestration in the horizontal canal

with a tissue graft coveringwith a tissue graft covering >2% profound SNHL>2% profound SNHL Rarely complete closure of the ABGRarely complete closure of the ABG May exhibit vestibular disturbancesMay exhibit vestibular disturbances


Samuel RosenSamuel Rosen 1953 – first suggest 1953 – first suggest

mobilization of the mobilization of the stapesstapes

Immediate improved Immediate improved hearinghearing

Re-fixationRe-fixation


John SheaJohn Shea 1956 – first to 1956 – first to

perform perform stapedectomystapedectomy

Oval window vein Oval window vein graftgraft

Nylon prosthesis Nylon prosthesis from incus to oval from incus to oval windowwindow

EpidemiologyEpidemiology

10% overall prevalence of histologic 10% overall prevalence of histologic otosclerosisotosclerosis

1% overall prevalence of clinically 1% overall prevalence of clinically significant otosclerosissignificant otosclerosis


Race Race Incidence of Incidence of otosclerosisotosclerosis

CaucasianCaucasian 10%10%

AsianAsian 5%5%

African AmericanAfrican American 1%1%

Native AmericanNative American 0%0%


Gender Gender Histologic otosclerosis – 1:1 ratio Histologic otosclerosis – 1:1 ratio Clinical otosclerosis – 2:1 (W:M)Clinical otosclerosis – 2:1 (W:M)

Possible progression during pregnancy (10%-Possible progression during pregnancy (10%-17%)17%)

Studies which have demonstrated changes during Studies which have demonstrated changes during pregnancy are often retrospective or lack pregnancy are often retrospective or lack audiometric data.audiometric data.

Studies comparing multigravid vs. nulligravid Studies comparing multigravid vs. nulligravid women with otosclerosis have failed to show women with otosclerosis have failed to show audiometric differences. audiometric differences.

Bilaterality more common (89% vs. 65%)Bilaterality more common (89% vs. 65%)


AgeAge 15-45 most common age range of 15-45 most common age range of

presentationpresentation Youngest presentation 7 years Youngest presentation 7 years Oldest presentation 50sOldest presentation 50s 0.6% of individuals <5 years old have 0.6% of individuals <5 years old have

foci of otosclerosisfoci of otosclerosis

PathophysiologyPathophysiology

Osseous dyscrasiaOsseous dyscrasia Resorption and formation of new boneResorption and formation of new bone Limited to the temporal bone and Limited to the temporal bone and

ossiclesossicles Inciting event unknownInciting event unknown

Hereditary, endocrine, metabolic, Hereditary, endocrine, metabolic, infectious, vascular, autoimmune, hormonalinfectious, vascular, autoimmune, hormonal

PathologyPathology

Two phases of diseaseTwo phases of disease Active (otospongiosis phase)Active (otospongiosis phase)

Osteocytes, histiocytes, osteoblastsOsteocytes, histiocytes, osteoblasts Active resorption of boneActive resorption of bone Dilation of vesselsDilation of vessels

Schwartze’s signSchwartze’s sign Mature (sclerotic phase)Mature (sclerotic phase)

Deposition of new bone (sclerotic and less dense Deposition of new bone (sclerotic and less dense than normal bone)than normal bone)

PathologyPathology

Most common sites of involvementMost common sites of involvement Fissula ante fenestrumFissula ante fenestrum Round window niche (30%-50% of cases)Round window niche (30%-50% of cases) Anterior wall of the IACAnterior wall of the IAC

Non-clinical foci of Non-clinical foci of otosclerosisotosclerosis

Anterior footplate Anterior footplate involvementinvolvement

Annular ligament Annular ligament involvementinvolvement

Bipolar involvement of the Bipolar involvement of the footplatefootplate

Round WindowRound Window

Labyrinthine Labyrinthine OtosclerosisOtosclerosis

1912 – Siebenmann described 1912 – Siebenmann described labyrinthine otosclerosislabyrinthine otosclerosis Suggested otosclerosis may cause Suggested otosclerosis may cause

SNHL viaSNHL via Toxic metabolitesToxic metabolites Decreased blood supplyDecreased blood supply Direct extensionDirect extension Disruption of membranesDisruption of membranes

Hyalinization of the spiral Hyalinization of the spiral ligamentligament

Erosion into inner earErosion into inner ear

Organ of CortiOrgan of Corti

Cochlear OtosclerosisCochlear Otosclerosis Audiometric studiesAudiometric studies

Some studies have shown that in cases of unilateral otosclerosis ~ Some studies have shown that in cases of unilateral otosclerosis ~ 60% may have decreased sensory thresholds even after stapes surgery60% may have decreased sensory thresholds even after stapes surgery

Histiologic studiesHistiologic studies Cases of documented otosclerosis and a large sensory loss have shown Cases of documented otosclerosis and a large sensory loss have shown

large foci of otosclerosis in the otic capsule.large foci of otosclerosis in the otic capsule. Many cases of large otic capsule foci without sensory loss or of Many cases of large otic capsule foci without sensory loss or of

sensory loss without foci have also been described.sensory loss without foci have also been described.

Biochemical studiesBiochemical studies Some authors have noted increased levels of perilymph protein during Some authors have noted increased levels of perilymph protein during

stapedotomy in patients with radiographic evidence of otic capsule stapedotomy in patients with radiographic evidence of otic capsule foci and sensory hearing loss.foci and sensory hearing loss.

ConclusionConclusion Many experts believe that extensive involvement of the cochlea will Many experts believe that extensive involvement of the cochlea will

produce sensorineural hearing deficits, although it is not known how produce sensorineural hearing deficits, although it is not known how this occurs or why it only occurs in a subset of patients with cochlear this occurs or why it only occurs in a subset of patients with cochlear foci.foci.

Diagnosis Diagnosis of Otosclerosisof Otosclerosis

HistoryHistory

Most common presentationMost common presentation Women age 20 - 30Women age 20 - 30 Conductive or Mixed hearing lossConductive or Mixed hearing loss

Slowly progressive, Slowly progressive, Bilateral (80%)Bilateral (80%) Asymmetric Asymmetric

Tinnitus (75%)Tinnitus (75%)

HistoryHistory

Age of onset of hearing lossAge of onset of hearing loss ProgressionProgression LateralityLaterality Associated symptomsAssociated symptoms

DizzinessDizziness OtalgiaOtalgia OtorrheaOtorrhea TinnitusTinnitus

HistoryHistory Family historyFamily history

2/3 have a significant family history2/3 have a significant family history Particularly helpful in patients with severe or Particularly helpful in patients with severe or

profound mixed hearing lossprofound mixed hearing loss Prior otologic surgeryPrior otologic surgery History of ear infectionsHistory of ear infections Vestibular symptomsVestibular symptoms

25%25% Most commonly dysequilibriumMost commonly dysequilibrium Occasionally attacks of vertigo with rotatory Occasionally attacks of vertigo with rotatory

nystagmusnystagmus

Physical ExamPhysical Exam OtomicroscopyOtomicroscopy

Most helpful in ruling out other disordersMost helpful in ruling out other disorders Middle ear effusionsMiddle ear effusions TympanosclerosisTympanosclerosis Tympanic membrane perforationsTympanic membrane perforations Cholesteatoma or retraction pocketsCholesteatoma or retraction pockets Superior semicircular canal dehiscenceSuperior semicircular canal dehiscence

Schwartze’s signSchwartze’s sign Red hue in oval window niche areaRed hue in oval window niche area 10% of cases10% of cases

Pneumatic otoscopyPneumatic otoscopy Distinguish from malleus fixationDistinguish from malleus fixation

Physical ExamPhysical Exam

Tuning forksTuning forks Hearing loss progresses form low Hearing loss progresses form low

frequencies to high frequenciesfrequencies to high frequencies 256, 512, and 1024 Hz TF should be 256, 512, and 1024 Hz TF should be

usedused RinneRinne

256 Hz – negative test indicates at least a 20 dB 256 Hz – negative test indicates at least a 20 dB ABGABG

512 Hz – negative test indicates at least a 25 dB 512 Hz – negative test indicates at least a 25 dB ABGABG

Differential DiagnosisDifferential Diagnosis

Ossicular discontinuityOssicular discontinuity Congenital stapes fixationCongenital stapes fixation Malleus head fixationMalleus head fixation Paget’s diseasePaget’s disease Osteogenesis imperfectaOsteogenesis imperfecta Superior semicircular canal Superior semicircular canal

dehiscencedehiscence

AudiometryAudiometry

TympanometryTympanometry Impedance testingImpedance testing

Acoustic reflexesAcoustic reflexes Pure tonesPure tones

TympanometryTympanometry

Jerger (1970) – classification of Jerger (1970) – classification of tympanogramstympanograms Type AType A

Type AType A Type AsType As Type AdType Ad

Type BType B Type CType C

Acoustic ReflexesAcoustic Reflexes

Result from a change in the middle Result from a change in the middle ear compliance in response to a ear compliance in response to a sound stimulussound stimulus

Change in compliance Change in compliance Stapedius muscle contractionStapedius muscle contraction Stiffening of the ossicular chainStiffening of the ossicular chain Reduces the sound transmission to the Reduces the sound transmission to the

vestibulevestibule

Acoustic ReflexesAcoustic Reflexes

Otosclerosis has a predictable Otosclerosis has a predictable pattern of abnormal reflexes over pattern of abnormal reflexes over timetime Reduced reflex amplitudeReduced reflex amplitude Elevation of ipsilateral thresholdsElevation of ipsilateral thresholds Elevation of contralateral thresholdsElevation of contralateral thresholds Absence of reflexesAbsence of reflexes

Pure Tone AudiometryPure Tone Audiometry Most useful audiometric test for Most useful audiometric test for

otosclerosisotosclerosis Characterizes the severity of diseaseCharacterizes the severity of disease Frequency specificFrequency specific

Carhart’s notchCarhart’s notch Hallmark audiologic sign of otosclerosisHallmark audiologic sign of otosclerosis Decrease in bone conduction thresholdsDecrease in bone conduction thresholds

5 dB at 500 Hz5 dB at 500 Hz 10 dB at 1000 Hz10 dB at 1000 Hz 15 dB at 2000 Hz15 dB at 2000 Hz 5 dB at 4000 Hz5 dB at 4000 Hz

Pure Tone AudiometryPure Tone Audiometry Low frequencies Low frequencies

affected firstaffected first Below 1000 HzBelow 1000 Hz

Rising air lineRising air line ““Stiffness tilt” Stiffness tilt” Secondary to stapes Secondary to stapes

fixationfixation

With disease With disease progressionprogression Air line flattensAir line flattens

Pure Tone AudiometryPure Tone Audiometry

Carhart’s notchCarhart’s notch Proposed theoryProposed theory

Stapes fixation disrupts the normal Stapes fixation disrupts the normal ossicular resonance (2000 Hz)ossicular resonance (2000 Hz)

Normal compressional mode of bone Normal compressional mode of bone conduction is disturbed because of relative conduction is disturbed because of relative perilymph immobilityperilymph immobility

Mechanical artifactMechanical artifact Reverses with stapes mobilizationReverses with stapes mobilization

Pure Tone AudiometryPure Tone Audiometry Committee on Hearing and BalanceCommittee on Hearing and Balance

Set standards for reporting results in cases of Set standards for reporting results in cases of otosclerosis procedures.otosclerosis procedures.

Operative hearing results should be reported using post-Operative hearing results should be reported using post-operative data, specifically, the post-operative air-bone gap.operative data, specifically, the post-operative air-bone gap.

This prevents exaggeration of surgical results and This prevents exaggeration of surgical results and “overclosure.” “overclosure.”

Adopted by the AAOHNS in 1994Adopted by the AAOHNS in 1994 Important in reviewing literature regarding surgical Important in reviewing literature regarding surgical

outcomesoutcomes Studies prior to this time often use pre-op bone lines and post-Studies prior to this time often use pre-op bone lines and post-

op air conduction measurements which may exaggerate op air conduction measurements which may exaggerate results.results.

This convention is not uniform in all parts of the world, so the This convention is not uniform in all parts of the world, so the methods is very important in determining the consistency of methods is very important in determining the consistency of data.data.

ImagingImaging

Computed tomography (CT) of the Computed tomography (CT) of the temporal bonetemporal bone Proponents of CT for evaluation of Proponents of CT for evaluation of

otosclerosisotosclerosis Pre-opPre-op

Characterize the extent of otosclerosisCharacterize the extent of otosclerosis Severe or profound mixed hearing lossSevere or profound mixed hearing loss Evaluate for enlarge cochlear aqueductEvaluate for enlarge cochlear aqueduct

Post-opPost-op Recurrent CHLRecurrent CHL

Re-obliteration vs. prosthesis dislocationRe-obliteration vs. prosthesis dislocation VertigoVertigo

““Halo sign”Halo sign”

Paget’s diseasePaget’s disease

Osteogenesis ImperfectaOsteogenesis Imperfecta

Management OptionsManagement Options

Medical Medical AmplificationAmplification SurgerySurgery CombinationsCombinations

Patient SelectionPatient Selection

FactorsFactors Result of tuning fork tests and Result of tuning fork tests and

audiometryaudiometry Skill of the surgeonSkill of the surgeon FacilitiesFacilities Medical condition of the patientMedical condition of the patient Patient wishesPatient wishes

SurgerySurgery

Best surgical candidateBest surgical candidate Previously un-operated earPreviously un-operated ear Good healthGood health Unacceptable ABGUnacceptable ABG

25 to 40 dB25 to 40 dB Negative Rinne testNegative Rinne test

Excellent discriminationExcellent discrimination Desire for surgery Desire for surgery

SurgerySurgery

Other factorsOther factors Age of the patientAge of the patient

ElderlyElderly Poorer results in the high frequenciesPoorer results in the high frequencies

Congenital stapes fixation (44% success Congenital stapes fixation (44% success rate)rate)

Juvenile otosclerosis (82% success rate)Juvenile otosclerosis (82% success rate) OccupationOccupation

DiverDiver PilotPilot Airline steward/stewardessAirline steward/stewardess

SurgerySurgery

Other factorsOther factors Vestibular symptomsVestibular symptoms

Meniere's diseaseMeniere's disease Concomitant otologic diseaseConcomitant otologic disease

CholesteatomaCholesteatoma Tympanic membrane perforationTympanic membrane perforation

Surgical StepsSurgical Steps

Subtleties of technique and styleSubtleties of technique and style Local Local vsvs. general anesthesia. general anesthesia Stapedectomy vs. partial stapedectomy Stapedectomy vs. partial stapedectomy

vs. stapedotomyvs. stapedotomy Laser vs. drill vs. cold instrumentationLaser vs. drill vs. cold instrumentation Oval window sealsOval window seals ProsthesisProsthesis

Canal InjectionCanal Injection

2-3 cc of 1% 2-3 cc of 1% lidocaine with lidocaine with 1:50,000 or 1:50,000 or 1:100,000 1:100,000 epinephrineepinephrine

4 quadrants4 quadrants

Bony cartilaginous Bony cartilaginous junctionjunction

Raise Tympanomeatal Raise Tympanomeatal FlapFlap

6 and 12 o’clock 6 and 12 o’clock positionspositions

6-8 mm lateral to 6-8 mm lateral to the annulusthe annulus

Take into account Take into account curettage of the curettage of the scutumscutum

Separation of chorda Separation of chorda tympani nerve from malleustympani nerve from malleus Separate the Separate the

chorda from the chorda from the medial surface of medial surface of the malleus to the malleus to gain slackgain slack

Avoid stretching Avoid stretching the nervethe nerve

Cut the nerve Cut the nerve rather than rather than stretch itstretch it

Curettage of ScutumCurettage of Scutum

Curettage a trough Curettage a trough lateral to the lateral to the scutum, thinning itscutum, thinning it

Then remove the Then remove the scutum (incus to scutum (incus to the round window)the round window)

Curettage of ScutumCurettage of Scutum Exposure Exposure

Vertical:Vertical: Facial nerve Facial nerve

to round to round windowwindow

Horizontal:Horizontal: Pyramidal Pyramidal

process to process to malleusmalleus

Preservation Preservation of bone over of bone over incusincus

Middle ear examinationMiddle ear examination

Mobility of ossiclesMobility of ossicles Confirm stapes fixationConfirm stapes fixation Evaluate for malleus or incus fixationEvaluate for malleus or incus fixation

Abnormal anatomyAbnormal anatomy Dehiscent facial nerveDehiscent facial nerve Overhanging facial nerveOverhanging facial nerve Deep narrow oval window nicheDeep narrow oval window niche Ossicular abnormalitiesOssicular abnormalities

Measurement for Measurement for prosthesisprosthesis

MeasurementMeasurement Lateral aspect Lateral aspect

of the long of the long process of the process of the incus to the incus to the footplatefootplate

Total StapedectomyTotal Stapedectomy

UsesUses Extensive fixation of the footplateExtensive fixation of the footplate Floating footplateFloating footplate

DisadvantagesDisadvantages Increased post-op vestibular symptomsIncreased post-op vestibular symptoms More technically difficultMore technically difficult Increased potential for prosthesis Increased potential for prosthesis

migrationmigration

Stapedotomy/Small Stapedotomy/Small FenestraFenestra

Originally for obliterated or solid Originally for obliterated or solid footplatesfootplates EuropeEurope 1970-801970-80

First laser stapedotomy performed by First laser stapedotomy performed by Perkins in 1978Perkins in 1978 Less trauma to the vestibuleLess trauma to the vestibule Less incidence of prosthesis migrationLess incidence of prosthesis migration Less fixation of prosthesis by scar tissueLess fixation of prosthesis by scar tissue

Drill FenestrationDrill Fenestration

0.7mm diamond 0.7mm diamond burrburr Motion of the burr Motion of the burr

removes bone dustremoves bone dust Avoids smoke Avoids smoke

productionproduction Avoids surrounding Avoids surrounding

heat productionheat production

Laser FenestrationLaser Fenestration LaserLaser

Avoids manipulation of the footplateAvoids manipulation of the footplate Argon and Potassium titanyl phosphate Argon and Potassium titanyl phosphate

(KTP/532)(KTP/532) Wave length 500 nmWave length 500 nm Visible lightVisible light Absorbed by hemoglobin Absorbed by hemoglobin Surgical and aiming beamSurgical and aiming beam

Carbon dioxide (CO2)Carbon dioxide (CO2) 10,000 nm10,000 nm Not in visible light rangeNot in visible light range Surgical beam onlySurgical beam only

Requires separate laser for an aiming beam (red helium-Requires separate laser for an aiming beam (red helium-neon)neon)

Ill defined fuzzy beamIll defined fuzzy beam

Oval window sealOval window seal

Tragal perichondriumTragal perichondrium Vein (hand or wrist)Vein (hand or wrist) Temporalis fasciaTemporalis fascia BloodBlood FatFat Gelfoam (now discouraged)Gelfoam (now discouraged)

Reconstructing the annular Reconstructing the annular ligamentligament

Placement of the Placement of the ProsthesisProsthesis

Prosthesis is Prosthesis is chosen and length chosen and length pickedpicked

Some prefer Some prefer bucket handle to bucket handle to incorporate the incorporate the lenticular process lenticular process of the incusof the incus

Stapedectomy Stapedectomy vs.vs. StapedotomyStapedotomy

ABG closure < 10dB (PTA)ABG closure < 10dB (PTA)

Special Special Considerations and Considerations and Complications in Complications in Stapes SurgeryStapes Surgery

Overhanging Facial Overhanging Facial NerveNerve

Usually dehiscentUsually dehiscent Consider aborting the procedureConsider aborting the procedure Facial nerve displacement (Perkins, 2001)Facial nerve displacement (Perkins, 2001)

Facial nerve is compressed superiorly with No. Facial nerve is compressed superiorly with No. 24 suction (5 second periods)24 suction (5 second periods)

10-15 sec delay between compressions10-15 sec delay between compressions Perkins describes laser stapedotomy while Perkins describes laser stapedotomy while

nerve is compressednerve is compressed Wire piston usedWire piston used

Add 0.5 to 0.75 mm to accommodate curve Add 0.5 to 0.75 mm to accommodate curve around the nervearound the nerve

Floating FootplateFloating Footplate Footplate dislodges from the Footplate dislodges from the

surrounding OW nichesurrounding OW niche Incidental findingIncidental finding More commonly iatrogenicMore commonly iatrogenic

PreventionPrevention LaserLaser Footplate control holeFootplate control hole

ManagementManagement AbortAbort H. House favors promontory H. House favors promontory

fenestration and total stapedectomyfenestration and total stapedectomy Perkins favors laser fenestrationPerkins favors laser fenestration

Diffuse Obliterative Diffuse Obliterative OtosclerosisOtosclerosis

Occurs when the Occurs when the footplate, annular footplate, annular ligament, and oval ligament, and oval window niche are window niche are involved involved

Closure of air-bone Closure of air-bone gap < 10 dB less gap < 10 dB less common.common.

Refixation Refixation commonly occurscommonly occurs

Perilymphatic GusherPerilymphatic Gusher

Associated with patent cochlear aqueductAssociated with patent cochlear aqueduct More common on the leftMore common on the left Increased incidence with congenital stapes Increased incidence with congenital stapes

fixationfixation Increases risk of SNHLIncreases risk of SNHL ManagementManagement

Rough up the footplateRough up the footplate Rapid placement of the OW seal then the Rapid placement of the OW seal then the

prosthesisprosthesis HOB elevated, stool softeners, bed rest, avoid HOB elevated, stool softeners, bed rest, avoid

Valsalva, +/- lumbar drainValsalva, +/- lumbar drain

Round Window ClosureRound Window Closure 20%-50% of 20%-50% of

casescases 1% completely 1% completely

closedclosed

No effect on No effect on hearing unless hearing unless 100% closed100% closed

Opening has a Opening has a high rate of high rate of SNHLSNHL

SNHLSNHL 1%-3% incidence of profound permanent SNHL1%-3% incidence of profound permanent SNHL

Surgeon experienceSurgeon experience Extent of diseaseExtent of disease

CochlearCochlear Prior stapes surgeryPrior stapes surgery

TemporaryTemporary Serous labyrinthitisSerous labyrinthitis Reparative granulomaReparative granuloma

PermanentPermanent Suppurative labyrinthitisSuppurative labyrinthitis Extensive drillingExtensive drilling Basilar membrane breaksBasilar membrane breaks Vascular compromiseVascular compromise Sudden drop in perilymph pressureSudden drop in perilymph pressure

Reparative GranulomaReparative Granuloma Granuloma formation around the prosthesis and Granuloma formation around the prosthesis and

incusincus 2 -3 weeks postop2 -3 weeks postop Initial good hearing results followed by an Initial good hearing results followed by an

increase in the high frequency bone line increase in the high frequency bone line thresholdsthresholds

Associated tinnitus and vertigoAssociated tinnitus and vertigo Exam – reddish discoloration of the posterior TMExam – reddish discoloration of the posterior TM TreatmentTreatment

ME explorationME exploration Removal of granulomaRemoval of granuloma

Prognosis – return of hearing with early excisionPrognosis – return of hearing with early excision Associated with use of GelfoamAssociated with use of Gelfoam

VertigoVertigo

Most commonly short lived (2-3 days)Most commonly short lived (2-3 days) More prolonged after stapedectomy More prolonged after stapedectomy

compared to stapedotomycompared to stapedotomy Due to serous labyrinthitisDue to serous labyrinthitis

Medialization of the prosthesis into Medialization of the prosthesis into the vestibulethe vestibule With or without perilymphatic fistulaWith or without perilymphatic fistula

Reparative granulomaReparative granuloma

Recurrent Conductive Recurrent Conductive Hearing LossHearing Loss

Slippage or displacement of the prosthesisSlippage or displacement of the prosthesis Most common cause of failureMost common cause of failure ImmediateImmediate

TechniqueTechnique Trauma Trauma

DelayedDelayed Slippage from incus narrowing or erosionSlippage from incus narrowing or erosion Adherence to edge of OW nicheAdherence to edge of OW niche Stapes re-fixationStapes re-fixation Progression of disease with re-obliteration of OWProgression of disease with re-obliteration of OW Malleus or incus ankylosisMalleus or incus ankylosis

AmplificationAmplification

Excellent alternative Excellent alternative Non-surgical candidatesNon-surgical candidates Patients who do not desire surgeryPatients who do not desire surgery

Patient satisfaction rate lower than Patient satisfaction rate lower than that of successful surgerythat of successful surgery Canal occlusion effectCanal occlusion effect Amplification not used at nightAmplification not used at night

MedicalMedical

Sodium FluorideSodium Fluoride 1923 - Escot suggested using calcium 1923 - Escot suggested using calcium

fluoridefluoride 1965 – Shambaugh popularized its use1965 – Shambaugh popularized its use MechanismMechanism

Fluoride ion replaces hydroxyl group in bone Fluoride ion replaces hydroxyl group in bone forming fluorapatiteforming fluorapatite

Resistant to resorptionResistant to resorption Increases calcification of new boneIncreases calcification of new bone Causes maturation of active foci of otosclerosisCauses maturation of active foci of otosclerosis

MedicalMedical

Sodium FluorideSodium Fluoride Reduces tinnitus, reverses Schwartze’s sign, Reduces tinnitus, reverses Schwartze’s sign,

resolution of otospongiosis seen on CT resolution of otospongiosis seen on CT OTC – FloricalOTC – Florical Dose – 20-120mgDose – 20-120mg IndicationsIndications

Non-surgical candidatesNon-surgical candidates Patients who do not want surgeryPatients who do not want surgery Surgical candidates with + Schwartze’s signSurgical candidates with + Schwartze’s sign

Treat for 6 mo pre-op Treat for 6 mo pre-op Postop if otospongiosis detected intra-opPostop if otospongiosis detected intra-op

MedicalMedical

Sodium fluorideSodium fluoride Hearing resultsHearing results

50% stabilize50% stabilize 30% improve30% improve

Re-evaluate q 2 yrs with CT and for Re-evaluate q 2 yrs with CT and for Schwartze’s sign to resolveSchwartze’s sign to resolve

If fluoride are stopped – expect re-If fluoride are stopped – expect re-activation within 2-3 yearsactivation within 2-3 years

MedicalMedical

BisphosphonatesBisphosphonates Class of medications that inhibits bone resorption Class of medications that inhibits bone resorption

by inhibiting osteoclastic activityby inhibiting osteoclastic activity Dosing not standardDosing not standard Often supplement with Vitamin D and CalciumOften supplement with Vitamin D and Calcium Studies conducted on otosclerosis patients with Studies conducted on otosclerosis patients with

neurotologic symptoms report the majority of neurotologic symptoms report the majority of patients with subjective improvement or patients with subjective improvement or resolution.resolution.

Future application of this treatment unclear, Future application of this treatment unclear, especially with new reports of bisphosphonate especially with new reports of bisphosphonate related osteonecrosis.related osteonecrosis.

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1996;115(1):78-811996;115(1):78-81 Minor L. Clinical Manifestiations of Superior Semicircular Canal Dehiscence. Minor L. Clinical Manifestiations of Superior Semicircular Canal Dehiscence. The LaryngoscopeThe Laryngoscope. 2005. 115: 1717-1727.. 2005. 115: 1717-1727. Muller, C. Gadre, A. Otosclerosis. Quinn’s online textbook of Otolaryngology. Muller, C. Gadre, A. Otosclerosis. Quinn’s online textbook of Otolaryngology. http://http://

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LaryngoscopeLaryngoscope 1955;65:224-269 1955;65:224-269 Shea J Jr. Fenestration of the oval window. Shea J Jr. Fenestration of the oval window. Ann Otol Rhinol LaryngolAnn Otol Rhinol Laryngol 1958;67:932-951 1958;67:932-951 Shambaugh G. Clinical diagnosis of cochlear (labyrinthine) otosclerosis. Shambaugh G. Clinical diagnosis of cochlear (labyrinthine) otosclerosis. LaryngoscopeLaryngoscope 1965;75:1558-1562 1965;75:1558-1562 Shambaugh GE, Jr. and Glasscock ME, III. Shambaugh GE, Jr. and Glasscock ME, III. Surgery of the earSurgery of the ear, 3rd ed. Philadelphia, W. B. Saunders, 1980;455-516, 3rd ed. Philadelphia, W. B. Saunders, 1980;455-516 Toynbee, Joseph. The Diseases of the Ear: Their Nature, Diagnosis, and Treatment. With a Supplement by James Hinton. Toynbee, Joseph. The Diseases of the Ear: Their Nature, Diagnosis, and Treatment. With a Supplement by James Hinton.

London, 1868.London, 1868.

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