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.

简介：Cochlearimplantationisoneofthebestamongstthevariousmanagementoptionsavailableforchildrenandadultswithseveretoprofoundsensorineuralhearingloss.Innerearandinternalauditorycanal(IAC)malformationsaccountstoapproximately25%ofcongenitalsensorineuralhearinglossinchildren.Theprimarygoalofthisreportwastoevaluatethecommunicationoutcomesaftercochlearimplantationinachildwithcysticcochleovestibularanomaly(CCVA).Thechildwasevaluatedthroughvariousstandardizedoutcomemeasuresatregularintervalstotracktheprogressintermsofauditoryandspokenlanguageskills.ThescoresonCategoriesofAuditoryPerception(CAP),MeaningfulAuditoryIntegrationScale(MAIS),SpeechIntelligibilityRating(SIR),MeaningfulUseofSpeechScale(MUSS),andlisteningandspokenlanguageskillsshowedasignificantleapin12monthsdurationpostimplantation.Thereportthushighlightsandcorrelatesthesignificantprogressinauditoryandspokenlanguageskillsofthechildwithcongenitalmalformationstoappropriateauditoryrehabilitationandintensiveparentaltraining.

简介：Infantswithcongenitaldeafnesscausedbyseverebilateralinnerearmalformationsfrequentlysufferfromseverehearinglossandpoorbalance.Unfortunately,theuseofhearingaidsisusuallyineffectiveinrecoveringhearing,necessitatingcochlearimplants.Wereportacaseofa6-year-oldboywithcongenitaldeafnessandbilateralinnerearmalformations(rightside,incompletepartitiontypeI[IP-I];leftside,commoncavitydeformity).Hearingaidshadaremarkableeffectinthispatient,enablingsufficientandfavorablehearingrecoverysuchastoallowthepatienttoengageindailyconversations.Per-rotatorynystagmuswasrecordedonanelectronystagmogramforbothrightandleftrotationsinadampedrotationalchairtest.Itisrarefordeafchildrenwithseverebilateralinnerearmalformationtodemonstratefavorabledevelopmentinhearingandgoodequilibriumfunction.OurfindingssuggestthatauditoryevestibularhaircellsinthispatientmayhavebeenpartiallypreserveddespiteIP-Iintherightearandcommoncavitydeformityoftheleftear.