简介：<正>OtosclerosiswasfirstdiscoveredbyValsalvain1704[1],andthetermwasfirstusedbyAntonvonTroltschin1872todifferentiatefromtympanosclerosis[2].Normallytheoticcystinhumanremainsstationaryafterfulldevelopmentandcontainsnovisibleactivatedosteoblastsor

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简介：MutationsinGJB2genearethemostfrequentlyfoundmutationsinpatientswithnonsyndromichearingimpairment.However,thespectrumandprevalenceofmutationsinthisgenevaryamongdifferentethnicgroups.InChina,30,000infantsarebornwithcongenitalhearingimpairmentannually.Inordertoprovideappropriategenetictestingandcounselingtothefamilies,weinvestigatedthemolecularetiologyofnonsyndromicdeafnessin103unrelatedschoolchildrenattendingNantongSchoolfortheDeafandMuteinJiangsuProvince,China.ThecodingexonoftheGJB2genewasPCRamplifiedandsequenced.SixtytwoGJB2mutantalleleswereidentifiedin35.9%(37/103)ofthepatients.Twentyfivepatientscarriedtwopathogenicmutationsand12patientscarriedonemutantallele.The235delCwasthemostcommonmutationaccountingfor69.4%(43/62)ofGJB2mutantalleles.TheGJB2mutantallelesaccountedfor30.1%(62/206)ofallchromosomesresponsiblefornonsyndromichearingimpairment.Testingofthe3mostprevalentdeleteriousframeshiftmutationsinthiscohortdetected100%ofallGJB2mutantalleles.TheseresultsdemonstratethataneffectivegenetictestingofGJB2geneforpatientsandfamilieswithnonsyndromichearingimpairmentispossible.

简介：Hearinglossandtinnitusareamongthemostcommonconsequencesoflongtermnoiseexposureandre-mainanunder-addressedheathissueinmostdevelopingnationsincludingChina.Therapidindustrializa-tionandlifestylechangesinChinaincreasetheconcernovernoiseexposureandnoiseinducedhearingloss(NIHL).ResearchonNIHLinChinaislimited.ThecurrentpaperreviewsstudiespublishedinEnglishandChineselanguageliteraturesregardingnoiseexposureandNIHLinChina.TheirimplicationontheChi-nesepopulationisdiscussed.ThepossibleutilityofaresearchmodelsuchastheDangerousDecibels?asameanstoincreaseunderstandingofthescopeofNIHLamongtheChinesepopulation,toeducatethegener-alpublicinChina(especiallytheyoung)aboutNIHLanditsprevention,andtostudyeffectsoflanguageandculturalfactorsoninternationalinformationdisseminationandbehavioralinterventionsisproposed.

简介：Thestudyreportsacaseofa5-year-oldChinesegirldiagnosedwithKabukimake-upSyndrome(KMS).ThepatientshowedclassicKMSappearance:widelyseparatedeyes,ectropionoflateralone-thirdlowereyelids,flatnasaltip,andprominentears.Auditoryfeaturesinthisindividualincludedbilateralseveresensorineuralhearinglossandlackof40HzAERPresponsesidentifiedatIyearofage.Theindividualreceivedcochlearimplant(CI)intheleftearwhen5yearsold.andrehabilitationafterCItreatmentwere3inspeechintelligibilityand5inauditoryperformance.Thus,ourfindingssuggestthatcochlearimplantmaybehelpfultorestorehearingforindividualswithKabukisyndrome.

简介：Sincethe1970s,outcomestudiesforchildrenwithhearinglossexpandedfromfocusingonassessingauditoryawarenessandspeechperceptionskillstoevaluatinglanguageandspeechdevelopment.Sincetheearly2000s,themulti-centerlargescaleresearchsystematicallystudiedoutcomesintheareasofauditoryawareness,speech-perception,languagedevelopment,speechdevelopment,educationalachievements,cognitivedevelopment,andpsychosocialdevelopment.Thesestudiesadvocatedtheestablishmentofbaselineandregularfollow-upevaluationswithacomprehensiveframeworkcenteredonlanguagedevelopment.Recentresearchinterestsalsoincludeunderstandingthevastdifferencesinoutcomesforchildrenwithhearingloss,understandingtherelationshipsbetweenneurocognitivedevelopmentandlanguageacquisitioninchildrenwithhearingloss,andusingoutcomestudiestoguideevidence-basedclinicalpractice.AftertheestablishmentofstandardizedMandarinlanguageassessments,outcomesresearchinMainlandChinahasthepotentialtoexpandbeyondauditoryawarenessandspeechperceptionstudies.