Otosclerosis Otosclerosis DR Kamlesh Dubey DR Kamlesh Dubey AIIMS, New AIIMS, New Delhi Delhi
OtosclerosisOtosclerosis
DR Kamlesh DubeyDR Kamlesh Dubey
AIIMS, New DelhiAIIMS, New Delhi
IntroductionIntroduction
Otosclerosis Otosclerosis Primary metabolic bone disease of the otic capsule Primary metabolic bone disease of the otic capsule
and ossiclesand ossicles Results in fixation of the ossicles and conductive Results in fixation of the ossicles and conductive
hearing losshearing loss May have sensorineural component if the cochlea May have sensorineural component if the cochlea
is involvedis involved Genetically mediatedGenetically mediated
Autosomal dominant with incomplete penetrance (40%) Autosomal dominant with incomplete penetrance (40%) and variable expressivityand variable expressivity
History of Otosclerosis and Stapes History of Otosclerosis and Stapes SurgerySurgery
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”
History of Otosclerosis and Stapes History of Otosclerosis and Stapes SurgerySurgery
Gunnar HolmgrenGunnar Holmgren 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
History of Otosclerosis and Stapes History of Otosclerosis and Stapes SurgerySurgery
Julius LempertJulius Lempert Popularized the single Popularized the single
staged fenestration staged fenestration procedureprocedure
John HouseJohn House Further refined the Further refined the
procedureprocedure Popularized blue lining Popularized blue lining
the horizontal canalthe horizontal canal
History of Otosclerosis and Stapes History of Otosclerosis and Stapes SurgerySurgery
Fenestration procedure for otosclerosisFenestration procedure for otosclerosis Fenestration in the horizontal canal with a tissue Fenestration in the horizontal canal with a tissue
graft coveringgraft covering >2% profound SNHL>2% profound SNHL Rarely complete closure of the ABGRarely complete closure of the ABG
History of Otosclerosis and Stapes History of Otosclerosis and Stapes SurgerySurgery
Samuel RosenSamuel Rosen 1953 – first suggest 1953 – first suggest
mobilization of the mobilization of the stapesstapes
Immediate improved Immediate improved hearinghearing
Re-fixationRe-fixation
History of Otosclerosis and Stapes History of Otosclerosis and Stapes SurgerySurgery
John SheaJohn Shea 1956 – first to perform 1956 – first to perform
stapedectomystapedectomy Oval window vein graftOval window vein graft Nylon prosthesis from Nylon prosthesis from
incus to oval windowincus to oval window
EpidemiologyEpidemiology
10% overall prevalence of histologic 10% overall prevalence of histologic otosclerosisotosclerosis
1% overall prevalence of clinically significant 1% overall prevalence of clinically significant otosclerosisotosclerosis
EpidemiologyEpidemiology
% incidence of% incidence of
Race otosclerosisRace otosclerosis
CaucasianCaucasian 10%10%
AsianAsian 5%5%
African AmericanAfrican American 1%1%
Native AmericanNative American 0%0%
EpidemiologyEpidemiology
Gender Gender Histologic otosclerosis – 1:1 ratio Histologic otosclerosis – 1:1 ratio Clinical otosclerosis – 2:1 (W:M)Clinical otosclerosis – 2:1 (W:M)
Increase progression during pregnancy (10%-17%)Increase progression during pregnancy (10%-17%) Bilaterality more common (89% vs. 65%)Bilaterality more common (89% vs. 65%)
EpidemiologyEpidemiology
AgeAge 15-45 most common age range of presentation15-45 most common age range of presentation Youngest presentation7 years Youngest presentation7 years Oldest presentation 50sOldest presentation 50s 0.6% of individuals <5 years old have foci of 0.6% of individuals <5 years old have foci of
otosclerosisotosclerosis
PathophysiologyPathophysiology
Osseous dyscrasiaOsseous dyscrasia Resorption and formation of new boneResorption and formation of new bone Limited to the temporal bone and ossiclesLimited to the temporal bone and ossicles Inciting event unknownInciting event unknown
Hereditary, endocrine, metabolic, infectious, vascular, Hereditary, endocrine, metabolic, infectious, vascular, autoimmune, hormonalautoimmune, hormonal
PathophysiologyPathophysiology
Siebenmann – first to describe the microscopic Siebenmann – first to describe the microscopic appearanceappearance SpongySpongy Usually limited to the anterior footplateUsually limited to the anterior footplate
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 than normal Deposition of new bone (sclerotic and less dense than normal
bone)bone)
““Blue mantles of Manasseh”Blue mantles of Manasseh”
PathophysiologyPathophysiology
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
Fissula ante and post fenestrumFissula ante and post fenestrum
Fissula ante fenestrumFissula ante fenestrum
Non-clinical foci of otosclerosisNon-clinical foci of otosclerosis
Annular ligament involvementAnnular ligament involvement
Footplate InvolvementFootplate Involvement
Anterior footplate involvementAnterior footplate involvement
Bipolar involvement of the footplateBipolar involvement of the footplate
Round WindowRound Window
Labyrinthine OtosclerosisLabyrinthine Otosclerosis
1912 – Siebenmann described labyrinthine 1912 – Siebenmann described labyrinthine otosclerosisotosclerosis Suggested otosclerosis may cause SNHLSuggested otosclerosis may cause SNHL
Toxic metabolitesToxic metabolites Decreased blood supplyDecreased blood supply Direct extensionDirect extension
IACIAC
Hyalinization of the spiral ligamentHyalinization of the spiral ligament
Erosion into inner earErosion into inner ear
Organ of CortiOrgan of Corti
DiagnosisDiagnosis
HistoryHistory
Most common presentationMost common presentation Women in her 20s or 30sWomen in her 20s or 30s 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
Vestibular symptomsVestibular symptoms 25%25% Most commonly dysequilibriumMost commonly dysequilibrium Occasionally attacks of vertigo with rotatory Occasionally attacks of vertigo with rotatory
nystagmusnystagmus Prior otologic surgeryPrior otologic surgery History of ear infectionsHistory of ear infections
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
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
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 frequencies to Hearing loss progresses form low frequencies to
high frequencieshigh frequencies 256, 512, and 1024 Hz TF should be used256, 512, and 1024 Hz TF should be used
RinneRinne 256 Hz – negative test indicates at least a 20 dB ABG256 Hz – negative test indicates at least a 20 dB ABG 512 Hz – negative test indicates at least a 25 dB ABG512 Hz – negative test indicates at least a 25 dB ABG
Differential DiagnosisDifferential Diagnosis
Ossicular discontinuityOssicular discontinuity Congenital stapes fixationCongenital stapes fixation Malleus head fixationMalleus head fixation Paget’s diseasePaget’s disease Osteogenesis imperfectaOsteogenesis imperfecta
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 ear Result from a change in the middle ear compliance in response to a sound stimuluscompliance in response to a sound 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 vestibuleReduces the sound transmission to the vestibule
Acoustic ReflexesAcoustic Reflexes
Otosclerosis has a predictable pattern of Otosclerosis has a predictable pattern of abnormal reflexes over timeabnormal reflexes over time Diphasic reflex patternDiphasic reflex pattern Reduced reflex amplitudeReduced reflex amplitude Elevation of ipsilateral thresholdsElevation of ipsilateral thresholds Elevation of contralateral thresholdsElevation of contralateral thresholds Absence of reflexesAbsence of reflexes
Acoustic ReflexesAcoustic Reflexes
Pure Tone AudiometryPure Tone Audiometry
Most useful audiometric test for otosclerosisMost useful audiometric test for otosclerosis Characterizes the severity of diseaseCharacterizes the severity of disease Frequency specificFrequency specific
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
Pure Tone AudiometryPure Tone Audiometry
With disease With disease progressionprogression Air line flattensAir line flattens
Secondary to mass effectSecondary to mass effect
Pure Tone AudiometryPure Tone Audiometry
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
Carhart’s notchCarhart’s notch Proposed theoryProposed theory
Stapes fixation disrupts the normal ossicular resonance Stapes fixation disrupts the normal ossicular resonance (2000 Hz)(2000 Hz)
Normal compressional mode of bone conduction is Normal compressional mode of bone conduction is disturbed because of relative perilymph immobilitydisturbed because of relative perilymph immobility
Mechanical artifactMechanical artifact Reverses with stapes mobilizationReverses with stapes mobilization
ImagingImaging
Computed tomography (CT) of the temporal Computed tomography (CT) of the temporal bonebone Proponents of CT for evaluation of otosclerosisProponents of CT for evaluation of otosclerosis
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
ImagingImaging
CTCT Axial cutsAxial cuts
Patient position – canthomeatal line perpendicular to the Patient position – canthomeatal line perpendicular to the table toptable top
1 mm cuts1 mm cuts Top of sup. SCC to bottom of the cochleaTop of sup. SCC to bottom of the cochlea
CoronalCoronal Patient position – supine w/ head overextendedPatient position – supine w/ head overextended
face turned 20 degrees face turned 20 degrees ipsilateralipsilateral
““Halo sign”Halo sign”
Paget’s diseasePaget’s disease
Osteogenesis ImperfectaOsteogenesis Imperfecta
Natural history of otosclerosisNatural history of otosclerosis
90% of all cases are never clinically apparent90% of all cases are never clinically apparent Foci begins in childhoodFoci begins in childhood Most commonly becomes symptomatic in the 3Most commonly becomes symptomatic in the 3rdrd and and
44thth decades decades After clinical presentationAfter clinical presentation
Conductive hearing loss progressiveConductive hearing loss progressive Periods of quiescence and deteriorationPeriods of quiescence and deterioration Worsening tinnitusWorsening tinnitus Associated SNHL (rarely purely SN)Associated SNHL (rarely purely SN)
Matures by age 50-70 with max. CHL of 50 dBMatures by age 50-70 with max. CHL of 50 dB
ManagementManagement
Medical – Sodium FluorideMedical – Sodium Fluoride AmplificationAmplification SurgerySurgery CombinationsCombinations
Patient SelectionPatient Selection
FactorsFactors Result of TF tests and audiometryResult of TF tests and audiometry Skill of the surgeonSkill of the surgeon FacilitiesFacilities Medical condition of the patientMedical condition of the patient Patient wishesPatient wishes
Patient CounselingPatient Counseling
Options for treatmentOptions for treatment Advantages and disadvantages of eachAdvantages and disadvantages of each
Repeat clinic visitRepeat clinic visit
SurgerySurgery
Best surgical candidateBest surgical candidate Previously un-operated earPreviously un-operated ear Good healthGood health Unacceptable ABGUnacceptable ABG
25 to 40 dB, bilateral ABG recommended by different 25 to 40 dB, bilateral ABG recommended by different authoritiesauthorities
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 rate)Congenital stapes fixation (44% success 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
Endolymphatic HydropsEndolymphatic Hydrops
Surgical StepsSurgical Steps
Subtleties of technique and styleSubtleties of technique and style Local Local vsvs. general anesthesia. general anesthesia Stapedectomy vs. partial stapedectomy vs. Stapedectomy vs. partial stapedectomy vs.
stapedotomystapedotomy Laser vs. drill vs. cold instrumentationLaser vs. drill vs. cold instrumentation Oval window sealsOval window seals ProsthesisProsthesis
Pre-opPre-op Confirm the correct ear (largest ABG)Confirm the correct ear (largest ABG)
With the patientWith the patient AudiogramAudiogram History and physical examHistory and physical exam
Place CT and audiogram in a visible location in the Place CT and audiogram in a visible location in the OR for easy intra-operative evaluationOR for easy intra-operative evaluation
Canal InjectionCanal Injection
2-3 cc of 1% lidocaine 2-3 cc of 1% lidocaine with 1:50,000 or with 1:50,000 or 1:100,000 epinephrine1:100,000 epinephrine
4 quadrants4 quadrants
Bony cartilaginous Bony cartilaginous junctionjunction
Raise Tympanomeatal FlapRaise Tympanomeatal Flap
6 and 12 o’clock 6 and 12 o’clock positionspositions
6-8 mm lateral to the 6-8 mm lateral to the annulusannulus
Take into account Take into account curettage of the scutumcurettage of the scutum
Separation of chorda tympani nerve Separation of chorda tympani nerve from malleusfrom malleus
Separate the chorda Separate the chorda from the medial from the medial surface of the malleus surface of the malleus to gain slackto gain slack
Avoid stretching the n.Avoid stretching the n.
Cut the nerve rather Cut the nerve rather than stretch itthan stretch it
Curettage of ScutumCurettage of Scutum
Curettage a trough Curettage a trough lateral to the scutum, lateral to the scutum, thinning itthinning it
Then remove the Then remove the scutum (incus to the scutum (incus to the round window)round window)
Visualize the pyramidal Visualize the pyramidal process and facial n.process and facial n.
Curettage of ScutumCurettage of Scutum
Exposure of Exposure of pyramidal pyramidal process and process and facial n.facial n.
Preservation of Preservation of bone over incusbone over incus
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
Measurement for prosthesisMeasurement for prosthesis
MeasurementMeasurement Lateral aspect of Lateral aspect of
the long process of the long process of the incus to the the incus to the footplatefootplate
Average 4.5 mmAverage 4.5 mm
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 migrationIncreased potential for prosthesis migration
Stapedotomy/Small FenestraStapedotomy/Small Fenestra
Originally for obliterated or solid footplatesOriginally for obliterated or solid footplates EuropeEurope 1970-801970-80
First laser stapedotomy performed by Perkins First laser stapedotomy performed by Perkins in 1978in 1978
AdvantagesAdvantages 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 burr0.7mm diamond burr
Motion of the burr Motion of the burr removes bone dustremoves bone dust
Avoids smoke Avoids smoke productionproduction
Avoids surrounding heat Avoids surrounding heat productionproduction
Laser FenestrationLaser Fenestration LaserLaser
Avoids manipulation of the footplateAvoids manipulation of the footplate Argon and Potassium titanyl phosphate (KTP/532)Argon and Potassium titanyl phosphate (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-neon)Requires separate laser for an aiming beam (red helium-neon) Ill defined fuzzy beamIll defined fuzzy beam
FenestrationFenestration
Causse et al. (1993)Causse et al. (1993) Recommends posteriorly placed fenestration to Recommends posteriorly placed fenestration to
better recreate the natural physiologic dynamics of better recreate the natural physiologic dynamics of the footplatethe footplate
Pivoting stapesPivoting stapes
Energy transmission to the stapesEnergy transmission to the stapes
Posterior FenestrationPosterior Fenestration
Posteriorly Posteriorly placed placed fenestration fenestration with the laserwith the laser
Causse also Causse also recommends recommends following the following the laser with the laser with the diamond burr diamond burr to remove charto remove char
Oval window sealOval window seal
Tragal perichondriumTragal perichondrium Vein (hand or wrist)Vein (hand or wrist) Temporalis fasciaTemporalis fascia BloodBlood FatFat
Vein graft Vein graft
Reconstructing the annular ligamentReconstructing the annular ligament
Placement of the ProsthesisPlacement of the Prosthesis
Prosthesis is chosen and Prosthesis is chosen and length pickedlength picked
Some prefer bucket Some prefer bucket handle to incorporate handle to incorporate the lenticular process of the lenticular process of the incusthe incus
Stapedectomy Stapedectomy vs.vs. Stapedotomy Stapedotomy
ABG closure < 10dB (PTA)ABG closure < 10dB (PTA)
Stapedectomy Stapedectomy vs.vs. Stapedotomy Stapedotomy
ABG closure at 4 kHzABG closure at 4 kHz
Special Considerations and Special Considerations and Complications in Stapes Complications in Stapes
SurgerySurgery
Overhanging Facial NerveOverhanging Facial Nerve
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. 24 suction Facial nerve is compressed superiorly with No. 24 suction (5 second periods)(5 second periods)
10-15 sec delay between compressions10-15 sec delay between compressions Perkins describes laser stapedotomy while nerve is Perkins describes laser stapedotomy while nerve is
compressedcompressed Wire piston usedWire piston used
Add 0.5 to 0.75 mm to accommodate curve around the Add 0.5 to 0.75 mm to accommodate curve around the nervenerve
Floating FootplateFloating Footplate Footplate dislodges from the surrounding Footplate dislodges from the surrounding
OW nicheOW niche Incidental findingIncidental finding More commonly iatrogenicMore commonly iatrogenic
PreventionPrevention LaserLaser Footplate control holeFootplate control hole
ManagementManagement AbortAbort H. House favors promontory fenestration and H. House favors promontory fenestration and
total stapedectomytotal stapedectomy Perkins favors laser fenestrationPerkins favors laser fenestration
Floating FootplateFloating Footplate
Hearing resultsHearing results Thin or blue footplate – 97% ABG closure Thin or blue footplate – 97% ABG closure
(<10dB)(<10dB) White or “biscuit” footplate – 52% ABG closureWhite or “biscuit” footplate – 52% ABG closure
Diffuse Obliterative OtosclerosisDiffuse Obliterative Otosclerosis
Occurs when the Occurs when the footplate, annular footplate, annular ligament, and oval ligament, and oval window niche are window niche are involved involved Bone is thinned with a Bone is thinned with a
small cutting burrsmall cutting burr Blue lined at Blue lined at
anteroposterior edges anteroposterior edges firstfirst
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 fixationIncreased incidence with congenital stapes fixation Increases risk of SNHLIncreases risk of SNHL ManagementManagement
Ruff up the footplateRuff up the footplate Rapid placement of the OW seal then the prosthesisRapid placement of the OW seal then the prosthesis HOB elevated, stool softeners, bed rest, avoid Valsalva, +/- HOB elevated, stool softeners, bed rest, avoid Valsalva, +/-
lumbar drainlumbar drain
Round Window ClosureRound Window Closure
20%-50% of cases20%-50% of cases 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 SNHLhigh rate of SNHL
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 incusGranuloma formation around the prosthesis and incus 2 -3 weeks postop2 -3 weeks postop Initial good hearing results followed by an increase in Initial good hearing results followed by an increase in
the high frequency bone line thresholdsthe high frequency bone line thresholds 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
VertigoVertigo
Most commonly short lived (2-3 days)Most commonly short lived (2-3 days) More prolonged after stapedectomy compared More prolonged after stapedectomy compared
to stapedotomyto stapedotomy Due to serous labyrinthitisDue to serous labyrinthitis
Medialization of the prosthesis into the Medialization of the prosthesis into the vestibulevestibule With or without perilymphatic fistulaWith or without perilymphatic fistula
Reparative granulomaReparative granuloma
Recurrent Conductive Hearing LossRecurrent Conductive Hearing 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
AmplificationAmplification Excellent alternative Excellent alternative
Non-surgical candidatesNon-surgical candidates Patients who do not desire surgeryPatients who do not desire surgery
Satisfaction rate less than with successful SxSatisfaction rate less than with successful Sx Canal occlusion effectCanal occlusion effect Amplification not used at nightAmplification not used at night
MedicalMedical
Sodium FluorideSodium Fluoride 1923 - Escot suggested using calcium fluoride1923 - Escot suggested using calcium fluoride 1965 – Shambaugh popularized its use1965 – Shambaugh popularized its use MechanismMechanism
Fluoride ion replaces hydroxyl group in bone forming Fluoride ion replaces hydroxyl group in bone forming fluorapatitefluorapatite
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
Sodium FluorideSodium Fluoride Reduces tinnitus, reverses Schwartze’s sign, resolution of Reduces tinnitus, reverses Schwartze’s sign, resolution of
otospongiosis seen on CT 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
Sodium fluorideSodium fluoride Hearing resultsHearing results
50% stabilize50% stabilize 30% improve30% improve
Re-evaluate q 2 yrs with CT and for Schwartze’s Re-evaluate q 2 yrs with CT and for Schwartze’s sign to resolvesign to resolve
If fluoride are stopped – expect re-activation If fluoride are stopped – expect re-activation within 2-3 yearswithin 2-3 years