上海交通大学耳鼻咽喉科学英文版课件 disease of inner ear

DiseaseofinnerearCategory

OtotoxicdeafnessInfecteddeafnessSuddendeafnessAutoimmuneinnereardiseasePresbycusisOtotoxicdeafnessDefinition&commondrugPathomechanismClinicalmanifestationTherapyOtotoxicdeafness

Definition&commondrugDefinition:adeafnesscausebycertainsdrugswhodamagetoauditoryreceptororauditorypathway,orbylong-termexposureofcertainschemicaldrugsCommondrug：

Aminoglycosides：streptomycin,kanamycin,gentamicin,micronomicin,adriamycin

Antimalarials：quinine,chloroquine

Antitumordrug：vincristine,nitroimidazole,cis-diamminedichloroplatin

Salicylicacids

Loopdiuretic：EtacrynicAcid

Heavymetals

Thalidomide

Chemicalmaterials,alcohol，misttobaccoOtotoxicdeafness

PathomechanismRouteofintoxication：General/Localapplication①→systemiccirculation→innerear/auditorypathways②→CSF/fenestratedmembrane→innerear/auditorypathways③→placenta→foetusMechanism：aminoglycosidesDrugs→haircell’smembranestructure→membraneproteinandphospholipidsprotein→destroymembranepermeability→Na+influx→destroymitochondrion→metabolicdisorder→cellulardegenerationandnecrosisMetabolicpathway：KidneymetabolismOtotoxicdeafness

ClinicalmanifestationBilateralhearinglossdamagetohighfrequencyfirstlyTinnitus,vestibularfunctiondecrease,vertigo,instabilityofgaitDelayonset:aminoglycosideantibioticsCompensatedvestibularsymptomOtotoxicdeafness

TherapyPrevention：observation-earlydiagnosisearlytreatmentearlydrugdiscontinuanceTherapeuticprinciple：

Neurotrophicandhaircellsdrug:traditionalChinesemedicine？ImprovemicrocirculationCalciumNeurotrophicfactorsCelluloseMicroelementGlucocorticoidHearingaidorcochlearimplant

InfecteddeafnessDefinition:deafnesswasresultedfromauditorynervesystemaffectedbypathogenicmicroorganism,includingbacterial,virus,rickettsia,protozoan,etc.buteliminatingotitismediaorsecondarylabyrinthitis.Typing：accordingtoinfectedtime:congenital,acquiredaccordingtopathogenicmicroorganism:bacterial,virus,rickettsiaaccordingtopathogeneticcondition:varyingdegreeofdeafnessInfecteddeafness

RubellaSourceofinfection：RubellavirusIncidence：themostfrequentdeafnessinpregnancyinfection<3monthsofpregnancy,rateofdeafness=68%infection=4-6months,rateofdeafness=40%Routeofinfection：virus→placenta→lymphaticsystemininnerearoffoetus→Degenerationandadhesionsofendolymphaticlabyrinth,Scheibe’sabnomality，bilateralseveresensoneuralhearinglossPrevention：preventinfectionduringpregnancy,strengthenexaminationinperinatalperiodInfecteddeafness

EpidemicmeningitisSourceofinfection:MeningococcusIncidence:24.9%ofinfecteddeafnessRouteofinfection:①→nervetrunk

②→peri-neurovascularspaceofinneracousticmeatus,cochlearaqueduct

③→blood→striavascularis→innerearPathology:perilymphcellularinfiltration→serocelluloseexudation→degeneration&disintegrationofspiralorganofcorti&spiralganglioncell→after2-4

weeks→granulationinSpatiumperilymphaceum→fibration→occlusionincochearductPrevention:vaccinesTherapy:sulfadiazine,penicillinCochlearossification:earlycochlearimplantationInfecteddeafness

EpidemicparotitisSourceofinfection:mumpsvirusRouteofinfection:①→nervetrunk

②→endo&perilymph③→CSF,blood

→innerear①→striavascularisincochlear

②→atrophyoforganofCorti

③→spiralnervefibersandganglioncellreduction

④→destructionofvestibularmacula,parsampullarisClinicalfeature:swellingofparotidgland,rapidprogression,unilateralpermanentseveresensorineuraldeafnessPrevention:earlymumpsvaccineInfecteddeafness

MeaslesSourceofinfection:measlesvirusIncidence:10%ofmeaslesvirusRouteofinfection:→CSF,Blood→innerearClinicalfeature：oftenbilateral,differentdegreePrevention:measlesvaccineTherapy:treatotitismedia,antiinflammatory,drainageInfecteddeafness

SyphilisSourceofinfection:spirochaetapallidaRouteofinfection:①→foetus

②→bloodDiagnosis:SerologicaltestingTherapy:penicillinInfecteddeafness

TyphoidfeverSourceofinfection:bacteriumtyphosumRouteofinfection:→digestivetract→mesenterylymphatictissue→blood→reticuloendothelialtissue→2ndweekafterinfection→inflammationofacousticnerve→involveganglioncellTherapy:supportingtherapy:hydroelectrolyte,vitaminsanti-infectivetherapy:chloramphenicol,macrolidescorticoidInfecteddeafness

MalariaSourceofinfection:plasmodiumRouteofinfection:①→mosquitos

②→transfusionPathology:plasmodiuminvadeintoerythrocytes→malariapigmentum→hyperplasiaofreticuloendothelialcell→blockageofbloodvesselsofinnerear→serofibrinousinflammation→epitheliumatrophy→ofsensorynervecellsandneurondegenerationTherapy：quinine:ototoxicityartemisinin:slightototoxicityInfecteddeafness

InfluenzaSourceofinfection:influenzavirusesClinicalmanifestation:

①→bullousmyringitis,otitismedia→conductivedeafness

②→hyperemiaandexudationininnerearandacousticnerve→sensorineuraldeafnessTherapy:

antivirus,vasodilatation,neurotrophy,glucocorticoidspreventcomplicationSuddendeafness

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IdiopathicsuddendeafnessSuddendeafnessIdiopathicsuddendeafnessSuddendeafnessDefinition:asensorineuralhearinglossthatdevelopsoveraperiodofhoursorafewdays.Theseverityofthehearinglossmayvaryfrommildtototallossofperceptionofthemostintensesound.Thelossofhearingmaybepermanent,orthehearingmayspontaneouslyreturntonormalornearnormal.Itisusuallyunilateralbutitcanbebilateralinabout4%ofthecases.Incidence:5~20/100,000Etiology:infection,tumor,craniocerebraltrauma,ruptureoffenestratedmembrane,drugintoxication,qutoimmuneresponse,insufficientofbloodininnerear,congenitaldysplasia,idiopathicdisease,menieredisease,multiplesclerosis,sarcoidosis,psychologicfactorDiagnosticcriteria

ofsuddendeafnessabruptonset,within72hours

non-undulatorysensorineuralhearingloss,atleast20dBat2consecutivefrequenciesEtiologyunknowMayaccompanywithtinnitusMayaccompanywithvertigo,nausea,vomitingOnlythesymptomofthe8thcraniannerveTherapeuticprinciple

ofsuddendeafnessvasodilatadors

neurotrophy

anticoagulantion

corticosteroidsvitamins,sedation,bedrest,hyperbaricoxygenation.Curativeeffectclassification

ofsudden

deafnessRecovery:NormalauditorythresholdatdamagedfrequencyExcellence:Averageauditionimprovesatleast30dBatdamagedfrequencyEffectiveness:Averageauditionimproves15to30dBatdamagedfrequencyInefficiency:Averageauditionimproveslessthan15dBatdamagedfrequencyIdiopathicsuddendeafnessDefinition：AgnogenicsuddendeafnessEtiology:

Virus,innerearblood-supplydisturbanceTherapy:sameassuddendeafnessAutoimmunediseaseofinnerearClassificationofautoimmunediseaseofinnerearPartialmanifestationofgeneralautoimmunedisease:Cogan

syndrome,Wegenergranuloma,SLE,polyarthritisdestruensAutoimmuneinnereardisease(AIED)AIED

ClinicalmanifestationMiddle-agedwomenprogressiveSNHL,weekstomonthsdizziness,auralfullnessbilateral79%novestibularsymptomssystemicautoimmunediseasein29%Mayaccompanywithrheumatoidarthritis,SLE,Cogansyndrome,WegenergranulomaLaboratoryexaminationofAIEDESRCRPC1qbindingassayanti-cardiolipinANCAsyphilistestingLymetitersCompletebloodcell(CBC)chemistriesthyroidfunctionsimagingTherapyofAIEDSteroidsCyclophosphamidePlasmapheresisMethotrexate--dihydrofolatereductaseinhibitorPresbycusisIncidence:，72%ofaged>65

yearsoldEtiology:AmbientnoiseandenviromentalpollutionInternalenvironmentmodificationDegenerationofauditorysystemCochlearmitochondrialDN