简介：Objective:Congenitalauditoryneuropathy(AN)affectshearingandspeechdevelopment.ThedegreeofhearingdifficultyincongenitalANvariesasafunctionofpathologyattheinnerearhaircell(IHC)synapsesortheauditorynerve.WereportacaseofaChinesegirlwithANrevealedbyOTOF(otoferlin)genemutationanalysiswhohadonlyamildhearingloss.Patient:A13-year-oldChinesegirlwasdiagnosedashavingcongenitalANonthebasisofOTOFgenemutationanalysis.Shemanifestamildsensorineuralhearinglosswith50%maximummonosyllablespeechdiscriminationrate,normalDPOAEs(distortionproductotoacousticemissions)beyondambientnoiselevels,onlySPs(summatingpotentials)evokedduringECoG(electrocochleography)andabsentABRs(auditoryevokedbrainstemresponses)bilaterallytoclickspresentedat100dBnHL.Shewasabletoeffectivelycommunicatewithothersbyspeechreadingowingtohermildhearingloss.Moreover,bilateralhearingaidshelpedhertocommunicate.Conclusions:OurpatientwasdemonstratedtohaveamutationontheOTOFgene.Nevertheless,shewasabletocommunicateusingauditoryvisualspeechreadinginspiteofamildauditorythresholdelevationprobablyduetopartialpathologyattheIHCsynapsesorintheauditorynerve.

简介：Objective:ToevaluateHighResolutionComputerTomography(HRCT)inthediagnosisofexternalearcanalcholesteatoma.Methods:Inthisretrospectivestudy,HRCTsof27patientswithexternalearcanalcholesteatomawerereviewed.Thechangesintheexternalearcanal,tympanicmembrane(TM),scutum,tympanumandmastoidweremeasuredandcategorized.Results:Fourteenpatientsshowednoormilddestructionintheexternalearcanal(stageIgroup).Eightpatientshadobviousenlargementintheexternalearcanal(stageIIgroup)butshowedlimiteddestructionsofthemastoidboneandnodamageofthetympanums.Fivepatientshadseriousdestructionofthemastoidboneanddamageofthetympanum(stageIIIgroup).AllpatientsinthestageIIIgroupshowedacompressionofmanubriumsandTMs,with3havingdamagesonossicularchain.BonedestructionoftheverticalsectionoffacialnervecanalwasdiscoveredinonecaseinthestageIIIgroup.Conclusion:HRCTcanprovidedetailinformationabouttheextentofexternalearcanalcholesteatoma.Suchinformationcanbeusedtoidentifyspecialsituationswithseriouscomplicationsandtodifferentiateexternalearcanalcholesteatomafrommiddleearcholesteatoma.

简介：ObjectiveToassesstheutilityoflow-andhigh-frequencytympanometryinthediagnosisofmiddleeardysfunctioninChineseinfants.MethodsTympanogramswereobtainedwith226Hz,678Hzand1000Hzprobetonesfrominfantsaged5-25weekswithnormalauditorybrainstemresponses(ABRs)(15infants,30ears)andwithprolongedwaveIlatencies(17infants,20ears),suggestingmiddleeardysfunction,usingaGSITympstarmiddleearanalyzerVersionII.ResultsThesingle-peakedtympanogramwasthemostcharacteristictypeinbothgroupsandseenin25ears(83.3%)inthenormalABRgroupandin18ears(90%)inthedelayedwaveIgroup,respectively.Thepeakpressure,peakcompensatedstaticacousticadmittanceandgradientof226Hztympanometrywereofnosignificantdifferencesbetweenthetwogroups.The678Hztympanogramsofadmittance,susceptanceandconductancedemonstratednon-peak,single-,double-andtri-peakedpatternsinbothgroups.TheagreementbetweenABRsand678Hztympanogramsofadmittance,susceptanceandconductancewere70.0%,58.0%and64.0%(kappa=0.324,0.234and0.118)respectively.For1000Hzprobetone,admittance,susceptanceandconductancetympanogramsshowedsinglepeakedpatternsin28(93.3%),25(83.3%)and26(86.7%)ofthe30normalears.Admittance,susceptanceandconductancetympanogramsusingthe1000Hzprobetonewereflatin15(75%),17(85%)and13(65%)oftheearsininfantswithprolongedwaveIlatencies.For1000Hzadmittance,susceptanceandconductanceTympanograms,theagreementbetweentympanometryandABRresultswere90.0%,92.0%and86.0%withkappaat0.783,0.831and0.690,respectively.Conclusion1000Hzprobetonetympanometryisapromisingmiddleearfunctiontestforinfantsof1-6monthsage,while226Hzand678Hzprobetonesarelessefficientindetectingmiddleeardysfunctionininfants.

简介：Cisplatin,awidelyusedanticancerdrug,damageshaircellsincochlearorganotypicculturesatlowdoses,butparadoxicallycauseslittledamageathighdosesresultinginaU-shapeddose-responsefunction.Todetermineifthecisplatindose-responsefunctionforvestibularhaircellsfollowsasimilarpattern,wetreatedvestibularorganotypiccultureswithdosesofcisplatinrangingfrom10to1000μM.Vestibularhaircelllesionsprogressivelyincreasedasthedoseofcisplatinincreasedwithmaximumdamageoccurringaround50–100μM,butthelesionsprogressivelydecreasedathigherdosesresultinginlittlehaircelllossat1000μM.TheU-shapeddoseresponsefunctionforcisplatin-treatedvestibularhaircellsincultureappearstoberegulatedbycoppertransporters,Ctr1,ATP7AandATP7B,thatdose-dependentlyregulatetheuptake,sequestrationandextrusionofcisplatin.

简介：Hearingloss(HL)isthemostcommonsensorydisorder,affectingallagegroups,ethnicities,andgen-ders.AccordingtoWorldHealthOrganization(WHO)estimatesin2005,278millionpeopleworldwidehavemoderatetoprofoundHLinbothears.Resultsofthe2002NationalHealthInterviewSurveyindicatethatnearly31millionofallnon-institutionalizedadults(aged18andover)intheUnitedStateshavetroublehearing.Epidemiologicalstudieshaveestimatedthatapproximately50%ofprofoundHLcanbeattributedtogeneticcauses.Withover60genesimplicatedinnonsyndromichearingloss,itisalsoanextremelyhet-erogeneoustrait.Recentprogressinidentifyinggenesresponsibleforhearinglossenablesotolaryngologistsandotherclinicianstoapplymoleculardiagnosisbygenetictesting.Theadventofthe$1000genomehasthepotentialtorevolutionizetheidentificationofgenesandtheirmutationsunderlyinggeneticdisorders.ThisisespeciallytrueforextremelyheterogeneousMendelianconditionssuchasdeafness,wherethemuta-tion,andindeedthegene,maybeprivate.Therecenttechnologicaladvancesintarget-enrichmentmethodsandnextgenerationsequencingofferauniqueopportunitytobreakthroughthebarriersoflimitationsim-posedbygenearrays.Theseapproachesnowallowforthecompleteanalysisofallknowndeafness-causinggenesandwillresultinanewwaveofdiscoveriesoftheremaininggenesforMendeliandisorders.Thisre-viewfocusesondescribinggenotype-phenotypecorrelationsofthemostfrequentgenesincludingGJB2,whichisresponsibleformorethanhalfofcases,followedbyothercommongenesandondiscussingtheim-pactofgenomicadvancesforcomprehensivegenetictestingandgenediscoveryinhereditaryhearingloss.

简介：ThisstudywasperformedtoassesswhetherthereisanassociationbetweenelevatedFastingBloodGlucose(FBG)andhearingimpairmentinBangladeshipopulation.Atotalof142subjects(72withelevatedFBG;70control)wereincludedinthestudy.Themeanauditorythresholdsofthecontrolsubjectsat1,4,8and12kHzfrequencieswere6.35±0.35,10.07±0.91,27.57±1.82,51.28±3.01dBSPL(decibelsoundpressurelevel),respectivelyandthatofthesubjectswithelevatedFBGwere8.33±0.66,14.37±1.14,38.96±2.23,and71.11±2.96dB,respectively.TheauditorythresholdsofthesubjectswithelevatedFBGweresignificantly(p<0.05)higherthanthecontrolsubjectsatalltheabovefrequencies,althoughhearingimpairmentwasmostevidentlyobservedatanextra-high(12kHz)frequency.Subjectswithalongdurationofdiabetes(>10years)showedsignificantly(p<0.05)higherlevelofauditorythresholdsat8and12kHz,butnotat1and4kHzfrequencies,comparedtosubjectswithshorterdurationofdiabetes(
10years).Inaddition,basedonthedataofoddsratio,moreacuteimpairmentofhearingattheextra-highfrequencywasobservedindiabeticsubjectsofbotholder(>40years)andyounger(
40years)agegroupscomparedtotherespectivecontrols.Thebinarylogisticregressionanalysisshoweda5.79-foldincreaseintheoddsofextra-highfrequencyhearingimpairmentindiabeticsubjectsafteradjustmentforage,genderandBMI.Thisstudyprovidesconclusiveevidencethatauditorythresholdatanextra-highfrequencycouldbeasensitivemarkerforhearingimpairmentindiabeticsubjects.

简介：Objective:Totestthefeasibilityofmeasuringfinetemporalbonestructuresusinganewlyestablishedcone-beamcomputedtomography(CBCT)system.Materialsandmethods:Sixformalin-fixedhumancadavertemporalboneswereimagedusingahigh-resolutionCBCTsystemthathas900framesandcoppertaluminumfiltration.Finetemporalbonestructures,includingthoseofthefacialnervecanalandvestibularstructures,wereidentifiedandmeasured.Results:Thefinestructuresofthemiddleear,includingthetympanicmembrane,tendonofthetensortympani,cochleariformprocessofthesemicanalofthetensortympani,pyramidaleminence,footplateofthestapes,fullpathofthefacialnervewithinthetemporalbone,supralabyrinthinespace,semicircularcanals,pathwayofthesubarcuatecanal,andfullpathofthevestibularaqueduct,wereclearlydemonstrated.Thevestibularaqueducthasamidpointwidthof0.4±0.0mmandopercularwidthof0.5±0.1mm(mean±SD).Thelengthoftheinternalacousticmeatuswas10.6±1.2mm(mean±SD),andthediameteroftheinternalacousticmeatuswas3.7±0.3mm(mean±SD).Conclusion:Thisnovelhigh-resolutionCBCTsystemhaspotentiallybroadapplicationsinthediagnosisofinnereardiseaseandinmonitoringassociatedpathologicalchanges,surgicalplanning,navigationfortheearsurgery,andtemporalbonetraining.

简介：Togaininsightsintotheototoxiceffectsofaminoglycosideantibiotics(AmAn)anddelayedperipheralganglionneurondeathintheinnerear.experimentalanimalmodelswerewidelyusedwithseveraldifferentapproachesincludingAmAnsystemicinjections,combinationtreatmentofAmAnanddiuretics,orlocalapplicationofAmAn.Intheseapproaches,systemicAmAntreatmentaloneusuallycausesincompletedamagetohaircellsintheinnerear.Co-administrationofdiureticandAmAncancompletelydestroythecochlearhaircells,butitisimpossibletodamagethevestibularsystem.OnlytheapproachofAmAnlocalapplicationcanselectivelyeliminatemostsensoryhaircellsintheinnerear.Therefore,AmAnlocalapplicationismoresuitableforstudiesforcompletehaircelldestructionsincochlearandvestibularsystemandthefollowingdelayedperipheralganglionneurondeath.Incurrentstudies,guineapigswereunilaterallytreatedwithahighconcentrationofgentamicin(GM,40nig/ml)throughthetympanicmembraneintothemiddleearcavity.AuditoryfunctionsandvestibularfunctionsweremeasuredbeforeandafterGMtreatment.Thelossofhaircellsanddelayeddegenerationofganglionneuronsinbothcochlearandvestibularsystemwerequantified30daysor60daysaftertreatment.TheresultsshowedthatbothauditoryandvestibularfunctionswerecompletelyabolishedafterGMtreatment.Thesensoryhaircellsweretotallymissinginthecochlea,andseverelydestroyedinvestibularend-organs.Thedelayedspiralganglionneurondeath60daysafterthedeafeningprocedurewasover50%.However,noobviouspathologicalchangeswereobservedinvestibularganglionneurons60dayspost-treatment.Theseresultsindicatedthatahighconcentrationofgentamycindeliveredtothemiddleearcavitycandestroymostsensoryhaircellsintheinnerearthatsubsequentlycausesthedelayedspiralganglionneurondegeneration.Thismodelmightbeusefulforstudiesofhaircellregenerations,delayeddegenerationofperipheralauditoryne

简介：Noise-inducedhearinglossisacommoncauseofacquiredhearinglossintheadultpopulation.Acousticoverstimulationcausescochleardamagethroughmechanicalstresstothetissue.Consequently,complexmolec-ularchangesareinitiated,andthesechangesleadtomorphologicalandbiologicalalterationsinthecochlea,whichinturncompromisethecochlearfunctionandcausehearingloss.Inthepast10years,therehavebeensignificantadvancesinourunderstandingofthemolecularmechanismsofnoise-inducedhearingloss.Theseadvancesareattributed,inpart,tothedevelopmentofhigh-throughputtechnologiesfortheglobalanalysesofmolecularchanges.Inthisreview,webrieflydescribethenewlydevelopedmethodsforinvestigatingthemo-lecularresponsesofthecochleatoacoustictraumaandtheknowledgegeneratedfromthesestudies.Wealsodiscussthestrengthsandlimitationsofeachtechniqueandthemajorchallengestoinvestigatecochleardegen-erationfollowingacousticinjury.