耳硬化症课件

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1、Otosclerosis2015-7-31OtosclerosisOtosclerosisisadisorderofthebonylabyrinthandstapesaffectingonlyhumans.In1735,theItaliananatomistandsurgeonAntonioMariaValsalvaofBolognawasthefirsttoreportonthelesionIntroductionAutosomaldominant：incompletepenetranceMale:Female=1:2Peak

2、incidenceinthesecondorthirddecade60%withfamilyhistory10%ofCaucasianswithhistologicfindings，12%haveclinicalsymptoms.Oneearisaffected,theotherisinvolved80%Primarilyconductivehearingloss，maybeasensorineuralormixedhearinglossEmbryologyAt8weeks,thecartilaginousframeworkis

3、begun.At16weeks,endochondralbonyreplacementofthisframework.Thefissulaantefenestram,anteriortoovalwindowisthelastareaofendochondralboneformation.Thisregionisaffectedin80~90%ofpatientsHistopathologyA:Active(GradeI)otosclerosis.Hypercellularity,intensebasophilicstaining

4、andpseudo-vascularspacesfilledbymultinucleatedosteoclastsandhyaline(whitearrow)canbeseen.HistopathologyB:Otosclerosiswithmoderateactivity(GradeII）.Thepseudo-vascularspacesarewidenedandempty(whitearrow).Thereisamarkedthickeningofthestapesfootplate.Theosteoidsubstances

5、howsmarkedbasophiliawithhypercellularity.HistopathologyC:Inactive(GradeIII)otosclerosis.Osteoidsubstanceshowseosinophilicstainingandsparselylocatedosteocytes.HistopathologyD:Burnedout(GradeIV)otoscleroticfocuswithintenseeosinophilicstainingandacellularosteoidsubstanc

6、e(whitearrow).Otosclerosis-associatedgenesAlthoughtheselocihavebeenmapped,wehavelittleinformationregardingtheroleofthesegenesindiseasedevelopmentImmunohistologicresearchThelesionsofotosclerosismaybeisolatedtotheovalwindowormayinvolvethelabyrinthmoreextensivelyOtologi

7、stsrefertoeitherfenestralorretrofenestral(cochlear)otosclerosis.PredilectionforOtosclerosisBorderofroundwindowApicalmedialwallofcochlearAreaposteriortocochlearaqueductStapesfootplateDiagnosisofOtosclerosisHistorySlowlyprogressivehearinglossHearingspeechmoreeasilyinno

8、isysituationParacusisofWillisTinnituspresentin75%FamilyhistoryofOSPE&AudiologicTestTympanicmembraneSchwartzesignWebertestBezold'str