简介：IntroductionHyperbilirubinemiaiscommoninthenewbornowingtotheirspecialmetabolismcharacteristics.Physiologicbilirubinlevelsareprotectivebecauseoftheirantioxidanteffects[1],whilepathologicaljaundiceisharmfultothehealthoftheneonate.Previously,mostpathologicalstudiesonthejaundiceofneonateshaveconcentrat-

简介：Objective:Toevaluatetheauditoryfunctionofanindividualwithgeneticallyconfirmedhemochromatosis.Methods:A57yearoldmalewithmildlyimpairedsounddetectionthresholdsunderwentarangeofbehavioural,electroacousticandelectrophysiologicassessments.Theseincludedtherecordingofotoacousticemissionsandauditorybrainstemresponses,measurementofmonauraltemporalresolutionandevaluationofbinauralspeechprocessing.Findingsforthispatientweresubsequentlycomparedwiththoseof80healthycontrolswithsimilaraudiometricthresholds.Results:Thepatientshowedthethreecardinalfeaturesofauditoryneuropathy,presentingwithevidenceofnormalcochlearouterhaircellfunction,disruptedneuralactivityintheauditorynerve/brainstemandimpairedtemporalprocessing.Hisfunctionalhearingability(speechperception)wassignificantlyaffectedandsuggestedareducedcapacitytouselocalizationcuestosegregatesignalsinthepresenceofbackgroundnoise.Conclusion:Wepresentthefirstcaseofanindividualwithhemochromatosisandauditoryneuropathy.Thefindingsforthispatienthighlighttheneedforcarefulevaluationofauditoryfunctioninindividualswiththedisorder.

简介：Objectives:Auditoryneuropathy(AN)isasensorineuralhearingdisordercharacterizedbyabsentorabnormalauditorybrainstemresponses(ABRs)andnormalcochlearouterhaircellfunctionasmeasuredbyotoacousticemissions(OAEs).Manyriskfactorsarethoughttobeinvolvedinitsetiologyandpathophysiology.ThreeChinesepedigreeswithfamilialANarepresentedhereintodemonstrateinvolvementofgeneticfactorsinANetiology.Methods:Probandsoftheabove-mentionedpedigrees,whohadbeendiagnosedwithAN,wereevaluatedandfollowedupintheDepartmentofOtolaryngologyHeadandNeckSurgery,ChinaPLAGeneralHospital.Theirfamilymemberswerestudiedandthepedigreediagramswereestablished.Historyofillness,physicalexamination,puretoneaudiometry,acousticreflex,ABRsandtransientevokedanddistortion-productotoacousticemissions(TEOAEsandDPOAEs)wereobtainedfrommembersofthesefamilies.DPOAEchangesundertheinfluenceofcontralateralsoundstimuliwereobservedbypresentingasetofcontinuouswhitenoisetothenon-recordingeartoexamthefunctionofauditoryefferentsystem.Somesubjectsreceivedvestibularcalorictest,computedtomography(CT)scanofthetemporalboneandelectrocardiography(ECG)toexcludeotherpossibleneuropathydisorders.Results:Inmostaffectedsubjects,hearinglossofvariousdegreesandspeechdiscriminationdifficultiesstartedat10to16yearsofage.TheiraudiologicalevaluationshowedabsenceofacousticreflexandABRs.AsexpectedinAN,thesesubjectsexhibitednearnormalcochlearouterhaircellfunctionasshowninTEOAE&DPOAErecordings.Pure-toneaudiometryrevealedhearinglossrangingfrommildtosevereinthesepatients.Autosomalrecessiveinheritancepatternswereobservedinthethreefamilies.InPedigreeⅠandⅡ,twoaffectedbrotherswerefoundrespectively,whileinpedigreeⅢ,2sisterswereaffected.Allthepatientswereotherwisenormalwithoutevidenceofperipheralneuropathyatthetimeofth

简介：ObjectiveThemainpurposeofthepresentstudywastodescribethevestibularfunctioninpatientswithauditoryneuropathy(AN),andtoassesstheirabilitytomaintainbalance.MethodsVestibularfunctiontestswereperformedon32patientswithANand36normalsubjectsincludingelectronystagmopraphy(ENG)andstaticpostrography(SPG).Theresultsfromthetwogroupswerecompared.ResultsEquilibriumfunctioninpatientswithAN,wasabnormal,comparedtonormalsubjects.ConclusionVestibularfunctiontests,espe-ciallystaticpostrography,shouldbeperformedonpatientswithAN.

简介：<正>IntroductionDeafnessisoneofthemostcommonotologicdiseasesandamajordiseasethatgreatlyimpactstheChinesepopulation.FromtheSecondNationalSampleSurveyofDisabledPersons,itisestimatedthatthereare27,800,000hearingdisabledpersonsinChina,about2.14%of

简介：Auditoryneuropathy(AN)isahearingdisordercharacterizedbyabsenceofauditorybrainstemresponsesdespitepreservationofouterhaircellfunction,andhasattractedattentionsfromresearchersandaudiologistssincereportedbyStarretal[1].ClinicalmanifestationsinANpatientsincludeabnormalauditorybrainstemresponsesnormalotoacousticemissions,lackofacousticreflexeslargecochlearmicrophonics,speechperceptiondeteriora

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简介：我们评估了与Toxocara眼的神经病临床上并且血清学地诊断的12个病人的13只眼睛。十一个病人有单方的参与，一个病人有双边的眼的神经病。八个病人(66.7%)让可能的感染采购原料到Toxocara。六个病人(50%)有无痛苦的尖锐眼的神经病。十只眼睛与peripapillary渗入有不对称的、裂的眼的圆盘浮肿，三只眼睛有弥漫的眼的圆盘浮肿。嗜曙红血球过多在五个病人(41.7%)被注意，眼的神经改进与可得到的轨道在十一只眼睛(72.7%)中的八个被观察磁性的回声成像(MRI)。吝啬的视觉尖酸显著地改进了后面的治疗[分辨率(logMAR)的最小的角度对数的平均数0.94

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：目的：探讨艾灸治疗糖尿病周围神经病变（DiabeticPeripheralNeuropathy，DPN）的周围神经保护机制。方法：以链脲佐菌素（Streptozotocin，STZ）腹腔注射诱导形成DPN模型，艾灸大鼠胰俞、足三里穴，每日1次，每穴15min，连续治疗56d。通过血糖、尿糖、体重、饮食量等的监测，结合神经电生理方法检测神经传导速度，评价艾灸对DPN的治疗效应；HE染色、光镜观察坐骨神经组织结构变化；酶联免疫吸附法检测坐骨神经NGF含量。结果：治疗后，艾灸组大鼠血糖水平明显低于模型组（P〈0．01）；艾灸组感觉神经传导速度明显快于模型组（P〈0．01）；艾灸纽病理形态改变较模型纽明显；艾灸组NGF含量也明显高于模型组（P〈0．01）结论：艾灸能有效改善DPN模型大鼠周围神经病变症状、体征。艾灸改善DPN模型大鼠周围神经症状的效应可能与提高NGF含量，促进周围神经保护有关。

简介：目的：观察温针治疗糖尿病周围神经病患者的临床疗效及对胫神经和腓总神经传导速度的影响。方法：把52例符合纳入标准的患者随机分为治疗组和对照组，每组26例．两组均在基础治疗上，治疗组采用温针疗法。每日1次，每星期治疗6次，共治疗4星期。对照组肌注弥可保（甲钴胺针）500μg，每日1次，共治疗4星期。检查并记录治疗前后两组患者临床症状及胫神经、腓总神经传导速度，并进行比较。结果：治疗4星期后，治疗组总有效率为88．5％，对照组为61．5％，两组，临床疗效比较治疗组优于对照组（P〈0．05）；神经传导速度比较两组治疗后均优于治疗前，差异有统计学意5Z．（P〈0．05）；治疗后比较治疗组胫神经感觉神经传导速度及腓总神经运动传导速度均优于对照组（P〈0．05）。结论：温针疗法治疗糖尿病周围神经病具有良好的临床疗效，且可以改善神经传导速度，是治疗糖尿病周围神经病变的有效方法之一。

简介：Objective:Toestablishananimalmodeloflike-auditoryneuropathyinneonatalrat.MethodsTheani-malswereinjectedwithphenylhydrazinehydrochlorideorsalineat7-dayofage.ABRandDPOAEwereperformedtoassesstheauditoryfunction.Thecochleabasilarmembranestretchedpreparationandcochlearfrozensectionswerepreparedforimmunohistochemicalstainingtoexaminethemorphologicalchangeofhaircellsandspiralganglioncells(SGNs).ResultsAt7-dayagetheABRwaveI,III,V,latenciesandI-III,I-VIWIsintheexperimentalgroupweresignificantlyprolongedcomparedwiththoseinthecontrolgroup.TheABRthresholdswerealsoelevatedintheexperimentalgroup.Wefoundthereisnosignificantdiffer-enceinDPOAEinphenylhydrazinehydrochlorideexposuregroupcomparetocontrolgroup.Thecochlearhaircellsshowednosignsoflossinbothgroup,butthetotalnumberofneurofilamentspositivecellsinSGNsweresignificantlyreducedinthephenylhydrazinetreatedanimals.ConclusionOurstudysuggeststhatphenylhydrazinehydrochloridecanchangetheauditoryfunctionandinduceperipheralnervepathologybytargetedmainlytheSGNsinneonatalrat.

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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：Auditoryneuropathy(AN)wasreported30yearsagoin1979whenDavisandHirshpresentedthefirstcasewithnormalornearnormalhearingthresholdbutabsentauditorybrainstemresponses.Manynameshavebeengivensincethenincludingparadoxicalhearingloss,brainstemauditoryprocessingsyndrome,centralauditorydysfunction,neuralsynchronydisorderorneuraldyssynchrony.ThetermauditoryneuropathywasfirstgivenbySiningerandcolleaguesin1995.MoreandmoreANarticleshavebeenpublishedinrecentyears.ThepresentshortreviewandcasereportfocusonthemostimportantcharacteristicsfromaclinicalpointofviewinordertoletyoungphysiciansknowAN,andconsequentlymakecorrectdiagnosis.