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：ObjectiveToevaluateefficacyofsurgicaltreatmentintraumaticfacialparalysis.Methods:Thirty-threecaseswerereviewed,includingtemporalbonefractureandiatrogenicfacialnerveinjury.Allthepatientsweretreatedwithvarioussurgicalmethodsaccordingtotheirpathogeny.ResultsThemeanpercentagefacialfunctionimprovement(House-BrackmannGradeⅠ-Ⅱ)was86%intemporalbonefractureandfunctionwasimprovedafterproperoperationtoiatrogenicfacialnerveinjury.ConclusionsPatientswithtraumaticfacialparalysisreceiveprovedoutcomesitreaedwithpropersurgicalmethodsaccordingtotheirparticularconditionofnerveinjury.

简介：ObjectiveToinvestigateTcellactivationfollowingfacialnerveaxotomizationandlatentneuroimmunologicmechanismsintraumaticfacialparalysis.MethodsAmurinemodeloffacialnervetransactionwasused.LymphocytesfromcervicalandmesentericlymphnodesinBABL/cmiceatspecifictimeswerecollectedandexpressionratesofCD69onTcellswereassessedbyflowcytometry.ResultsInfiltratingTcellsweredetectedaroundthefacialneuronsinthefacialnervenucleusinmicewhosefacialnervewastransected.ImmunofluorescentstainingshowedrecruitmentofactivatedTcells.Threedayspost-facialnervetransection,theexpressionrateofCD69onTcellsfromcervicaldraininglymphoidnodes(CDLNs)wassignificantlydifferentfromthatonTcellsfrommesentericlymphnodes(MLNs)(P=0.0457),whereasthelatterwassimilartothatinanimalsundergoingshamsurgeriesandthatinblankcontrolanimals(p=0.2817and0.2724,respectively).Twoweekspost-nervetransection,theTcellCD69expressionratefromCDLNsremainedatahigherlevelandthanthatinthesham-operationanimals(p=0.0007).Attwoweeks,CD69expressionrateonTcellsfromMLNswasalsoup-regulatedanddifferentcomparedwiththesham-operationanimalsandwithitselfatthreedayspost-operation(p=0.0082and0.0133,respectively).ConclusionTcellsappeartobeactivatedandup-regulatedinCDLNsfollowingfacialnervetransection.ThereisevenevidenceofTcellactivationinMLNsat2weekspost-nervetransection.Thissuggestesanalterationofimmuneresponsefromlocaltogeneralimmunityintheacutestageoffacialnervetrauma,whichmayhelpcoordinatingandcontrollingthescalesandorientationoftheneuroimmuneresponseduringthepathogenesisandprogressionoffacialnervetrauma.

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

简介：Cochlearimplantationisauniquemethodtore-constructauditionforpatientswithseveretoprofoundhearingloss.Withincreasingclinicalapplicationofthistechnique,itsrehabilitativeeffectsinpatientshasbecomeanimportanttopicofhearingandspeechrehabilitationresearch.Thisarticleisanoverviewofhearingandspeechrehabilitativetraining,rehabilitativeefficacyevaluationandfactorsaffectingrehabilitationefficacyfollowingcochlearimplantation.

简介：TheGermanwaltzingguineapigisastrainofanimalsexpressingdeafnessandseverebalancedisordersatbirth.ThemutationarosespontaneouslyinabreedingfacilityinGermanyandastheaffectedanimalsshowacharacteristicwaltzingbehavior,thestrainisnamedtheGermanwaltzingguineapig.ThestrainispresentlybredonlyatKarolinskaInstitutet.Thehereditaryinnerearimpairmenthasarecessivemodeofinheritanceandthestrainthusproducesnotonlyaffectedhomozygotesbutalsosymptom-freeheterozygotesandfullynormaloffspring.Theoutcomedependssolelyonthegenotypeoftheparents.Theheterozygotes,whichhaveobtainedthe'waltzing'genefromoneparentonly,havenormalhearingandnobalancedysfunction.Theheterozygousanimalsappearnormalbutwill,inturn,carrythegeneticdefecttothenextgeneration.Thepresentthesisisfocusedontheseanimals.Noiseandototoxicdrugsarewellknownstressfactorsthatinterferenegativelywiththehearingorganinbothhumansandanimals,causinghearingimpairment.However,theinter-individualvariabilityinsusceptibilitytoauditorystressfactorsissurprisinglylarge,mostlikelyduetodifferentgeneticpredisposition.Inthisstudy,heterozygousanimalsoftheGermanwaltzingguineapig,animalscarryingageneticdefectknowntocauseseverehearingimpairment,wereusedtostudyhowanunexploredgenefordeafnessinteractswithauditorystressagents,i.e.noiseexposureandtheototoxicdrugsgentamicinandcisplatin.AnimalswereexposedtobothnarrowbandaswellasbroadbandnoiseatdifferentagesandhearingthresholdsweremeasuredusingABRs.HeterozygotesoftheGermanwaltzingguineapigshowedlessthresholdshiftscomparedtocontrolstrains.Olderanimalswerelessaffectedbythenoisetraumathanyoungeranimals.Totestthehypothesisthattheefferentsystemcontributestoprotectionoftheinnerearagainstnoisetrauma,measurementsusinganewmethodofpostonsetadaptationofDPOAEsandmaximumadaptationmagnitudewereconducted.T