OTOSCLEROSISOTOSCLEROSIS

Selina SiwakotiSelina SiwakotiRadhika Upreti Radhika Upreti

Anita GurungAnita Gurung

IntroductionIntroduction

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

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

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

the cochlea is involvedthe cochlea is involvedGenetically mediatedGenetically mediated

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

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

5% prevalence in Asia.5% prevalence in Asia.

EpidemiologyEpidemiology

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

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

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

EpidemiologyEpidemiology

AgeAge15-45 most common age range of 15-45 most common age range of

presentationpresentationYoungest presentation 7 years Youngest presentation 7 years Oldest presentation 50sOldest presentation 50s0.6% of individuals <5 years old have 0.6% of individuals <5 years old have

foci of otosclerosisfoci of otosclerosis

PathophysiologyPathophysiology

Osseous dyscrasiaOsseous dyscrasiaResorption and formation of new boneResorption and formation of new boneLimited to the temporal bone and Limited to the temporal bone and

ossiclesossiclesInciting event unknownInciting event unknown

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

ETIOLOGYETIOLOGY

Exact cause not known.Exact cause not known.Anatomical basis.Anatomical basis.HeredityHeredityRaceRaceSexSexAgeAgeOthersOthers

TYPES OF OTOSCLEROSISTYPES OF OTOSCLEROSIS

Stapedial otosclerosis : most Stapedial otosclerosis : most commoncommon

Cochlear otosclerosisCochlear otosclerosisHistologic otosclerosisHistologic otosclerosis

SYMPTOMSSYMPTOMS

Hearing loss : conductive HL in Hearing loss : conductive HL in stapedial and SNHL in cochlearstapedial and SNHL in cochlear

TinnitusTinnitusVertigoVertigoParacusis willisiiParacusis willisiiMonotonous and soft speechMonotonous and soft speech

Diagnosis Diagnosis of Otosclerosisof Otosclerosis

HistoryHistory

Most common presentationMost common presentationWomen age 20 - 30Women age 20 - 30Conductive 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 lossProgressionProgressionLateralityLateralityAssociated symptomsAssociated symptoms

DizzinessDizzinessOtalgiaOtalgiaOtorrheaOtorrheaTinnitusTinnitus

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

Pneumatic otoscopyPneumatic otoscopy Distinguish from malleus fixationDistinguish from malleus fixation

Physical ExamPhysical Exam

Tuning forksTuning forksHearing loss progresses form low Hearing loss progresses form low

frequencies to high frequenciesfrequencies to high frequencies256, 512, and 1024 Hz TF should be 256, 512, and 1024 Hz TF should be

usedused

Differential DiagnosisDifferential Diagnosis

Ossicular discontinuityOssicular discontinuityCongenital stapes fixationCongenital stapes fixationMalleus head fixationMalleus head fixationPaget’s diseasePaget’s diseaseOsteogenesis imperfectaOsteogenesis imperfectaSuperior semicircular canal Superior semicircular canal

dehiscencedehiscence

ImagingImaging

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

otosclerosisotosclerosisPre-opPre-op

Characterize the extent of otosclerosisCharacterize the extent of otosclerosisSevere or profound mixed hearing lossSevere or profound mixed hearing lossEvaluate for enlarge cochlear aqueductEvaluate for enlarge cochlear aqueduct

Post-opPost-opRecurrent CHLRecurrent CHL

Re-obliteration vs. prosthesis dislocationRe-obliteration vs. prosthesis dislocationVertigoVertigo

Management OptionsManagement Options

Medical Medical AmplificationAmplificationSurgerySurgeryCombinationsCombinations

Patient SelectionPatient Selection

FactorsFactorsResult of tuning fork tests and Result of tuning fork tests and

audiometryaudiometrySkill of the surgeonSkill of the surgeonFacilitiesFacilitiesMedical condition of the patientMedical condition of the patientPatient wishesPatient wishes

SurgerySurgery

Best surgical candidateBest surgical candidatePreviously un-operated earPreviously un-operated earGood healthGood healthUnacceptable ABGUnacceptable ABG

25 to 40 dB25 to 40 dBNegative Rinne testNegative Rinne test

Excellent discriminationExcellent discriminationDesire for surgery Desire for surgery

SurgerySurgery

Other factorsOther factorsAge of the patientAge of the patient

ElderlyElderlyPoorer 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

DiverDiverPilotPilotAirline steward/stewardessAirline steward/stewardess

SurgerySurgery

Other factorsOther factorsVestibular symptomsVestibular symptoms

Meniere's diseaseMeniere's diseaseConcomitant otologic diseaseConcomitant otologic disease

CholesteatomaCholesteatomaTympanic membrane perforationTympanic membrane perforation

Surgical StepsSurgical Steps

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

vs. stapedotomyvs. stapedotomyLaser vs. drill vs. cold instrumentationLaser vs. drill vs. cold instrumentationOval window sealsOval window sealsProsthesisProsthesis

Total StapedectomyTotal Stapedectomy

UsesUsesExtensive fixation of the footplateExtensive fixation of the footplateFloating footplateFloating footplate

DisadvantagesDisadvantagesIncreased post-op vestibular symptomsIncreased post-op vestibular symptomsMore technically difficultMore technically difficultIncreased potential for prosthesis Increased potential for prosthesis

migrationmigration

Stapedotomy/Small FenestraStapedotomy/Small Fenestra

Less trauma to the vestibuleLess trauma to the vestibuleLess incidence of prosthesis migrationLess incidence of prosthesis migrationLess 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

Placement of the ProsthesisPlacement of the Prosthesis

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

CONTRAINDICATION OF CONTRAINDICATION OF SURGERYSURGERY

The only hearing earThe only hearing earHistory of Meniere’s History of Meniere’s

diseasediseaseYoung childrenYoung childrenProfessional Professional

atheletes, high atheletes, high constructive workers, constructive workers, drivers, frequent air drivers, frequent air travellerstravellers

PregnancyPregnancy

COMPLICATIONS OF SURGERYCOMPLICATIONS OF SURGERY

Overhanging Overhanging facial nerve facial nerve

Floating footplateFloating footplateDiffuse Diffuse

obliterative obliterative otosclorosisotosclorosis

Perilymphatics Perilymphatics GuscherGuscher

SNHLSNHL

Round window Round window closureclosure

Recurrent CHLRecurrent CHLRegenerative Regenerative

granulomagranulomaVertigoVertigo

AmplificationAmplification

Excellent alternative Excellent alternative Non-surgical candidatesNon-surgical candidatesPatients 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 surgeryCanal occlusion effectCanal occlusion effectAmplification not used at nightAmplification not used at night

MedicalMedical

Sodium fluorideSodium fluorideMechanismMechanism

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

Resistant to resorptionResistant to resorptionIncreases calcification of new boneIncreases calcification of new boneCauses maturation of active foci of Causes maturation of active foci of

otosclerosisotosclerosis

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 candidatesPatients who do not want surgeryPatients who do not want surgerySurgical 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 fluorideHearing resultsHearing results

50% stabilize50% stabilize30% 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.

SELF CARE AT HOME(POST-SELF CARE AT HOME(POST-OPERATIVE)OPERATIVE)

Take medicine as prescribed.Take medicine as prescribed. Blow nose gently.Blow nose gently. Sneeze and cough with mouth open for few Sneeze and cough with mouth open for few

weeks after surgery.weeks after surgery. Avoid heavy lifting, straining and bending.Avoid heavy lifting, straining and bending. Popping and crackling sensation are normal for Popping and crackling sensation are normal for

3-5 weeks after surgery.3-5 weeks after surgery. Temporary hearing loss is normal in operative Temporary hearing loss is normal in operative

ear.ear. Change cotton ball in the ear as needed.Change cotton ball in the ear as needed. Avoid getting in water for 2 weeks after surgery.Avoid getting in water for 2 weeks after surgery.