简介：ObjectiveToreportoutcomesofcochlearimplantation(CI)inachildwithauditoryneuropathyspectrumdisorder(ANSD)andtoprovidepreliminaryclinicalevidenceoftheefficacyofCIinANSDpatients.MethodsA4-year-oldboywithdiagnosedauditoryneuropathyspectrumdisorder(ANSD)receivedimplantationofaNucleusCI24Rafteranunsatisfactorytrialofamplification.Post-implantationperformanceinbothhearingsensitivityandspeechrecognitionwasassessedindifferentsessions.Aidedhearingthresholdsweretestedbybehavioralaudiometry.MandarinEarlySpeechPerceptionTest(MESP),MeaningfulAuditoryIntegrationScale(MAIS),categoryofauditoryperformance(CAP)andSpeechIntelligibilityRating(SIR)wereusedtoassessthebenefitsinauditoryskillsorspeechrecognitiontheboyobtainedfromCI.Thetestswereadministeredbeforesurgeryandat3monthsand7monthsafteropening.ResultsTheboydemonstratedimprovedauditorysensitivitybyusingCI.Concerningspeechrecognitionandcommunication,bothspeechaudiometryandquestionnairesshowedanobviousbenefitfromCI.ConclusionsCIhasworkedefficientlyinthisANSDboy.ButbecauseoflimitedunderstandingofANSDandrehabilitationeffectbycochlearimplantationinthiscondition,theclinicaldecisiontoimplantshouldbecautiousandonlyafterathoroughevaluation.Meanwhile,wellcontrolledandlongtermstudiesareneededtoconfirmtheefficacyofcochlearimplantationinpatientswithANSD.

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