©1994-2010ChinaAcademicJournalElectronicPublishingHouse.Allrightsreserved.http://www.cnki.net·听神经病·听神经病听力学特征及病损部位分析王锦玲1石力1薛飞1,2孙伟1,3高磊1谢娟1韩丽萍1【摘要】目的总结286例听神经病(auditoryneuropathy,AN)患者的临床表现和听力学特征,分析听力学检测在AN诊断的意义并探讨其病损部位。方法回顾性分析5年来诊治的286例AN患者的临床表现及听力学特征,分析患者年龄、病程和听力损失程度与纯音听阈、畸变产物耳声发射(DPOAE)之间的关系;讨论AV的诊断和病损部位。结果我院诊治的AN发病率占听力减退患者的1.37%,占感音神经性聋患者的2.49%。大多来自农村和山区。286例AN中单发性148例,并发于周围性、遗传性神经病138例,其中并发前庭神经病124例。男女性别之比为1:1.4。就诊者多为青少年(55.5%),平均20.2岁;发病年龄平均16.9岁;病程平均3.3年。其中277例(96.8%)表现为双耳渐进性听力减退,9例(3.1%)表现为单耳听力减退(单侧听神经病,UAN)。突出主诉为辨不清言语,尤其在嘈杂环境中;部分伴耳鸣、轻度头晕。并发性AN依病因不同可出现下肢无力、行走困难等多种症状。16例有家族遗传史。纯音听力图型以低频减退为主(76.2%),听力损失程度与患者年龄和病程具有相关性;言语识别率不成比例的差于纯音听阈;镫骨肌声反射多引不出,223耳(41%)单个或多个频率可引出阈值升高的非交叉和交叉声反射;530耳(92.8%)ABR自波Ⅰ起未引出,35耳引出Ⅴ波、6耳引出Ⅰ波,部分波幅低或潜伏期延长;DPOAE全部可引出,各频率DP-gram幅值与患者纯音听阈损失程度和病程具有相关性;DPOAE对侧声抑制减弱或消失。9例UAN患者的听功能特征与双侧的基本相同,但单侧6耳的听力图为下降型,以重度及极重度聋多见,DP-gram幅值全频降低,DPOAE健耳对侧声抑制存在,患耳对侧声抑制减弱或消失。结论AN多单独发病,也可并发于外周性、遗传性神经病。多发病于青少年。以辨不清言语,尤其在嘈杂环境中为突出主诉。本组听力学检测符合第Ⅷ颅神经的听支受损引起的听力学特征。对诊断标准的统一认识,主要需对“ABR严重异常”作进一步分析和判断。推测本病的病损部位主要在耳蜗神经传入通路和耳蜗传出神经周围通路。神经脱髓鞘病变可能为其主要的病理基础。【关键词】听神经病;听力学检测;病变部位;听觉脑干反射弧【中图分类号】R764.4【文献标识码】A【文章编号】1006-7299(2007)02-0089-09DifferentialAudiologicalAssessmentofPatientswithAuditoryNeuropathyWangJinling,ShiLi,XueFei,etal.(DepartmentofOtolaryngology,XijingHospital,FourthMilitaryMedicalUniversity,Xi’an710032,China)【Abstract】ObjectiveToconductdifferentialaudiologicalassessmentof286auditoryneuropathy(AN)patientsforthepurposeoftheevaluationofthediagnosisofANandthesiteoflesion.MethodsInretrospective,theaudiologicaldataof286ANpatientswerecarefullyreviewedandvariousparametersanalyzedfortheidentificationofimportantcharacteristicsinrelationtoAN.Theapplicationoftheclinicalbatteryofauditorythreshold,acousticimmittance,ABRandDPOAEwasstudiedforitsroleinthediagnosisofthesiteoflesion.ResultsIncidenceofANpatientswas1.37%inhearingimpairmentpatientsand2.49%insen2sorineuralhearinglosspatientsfromourhospital.138patientswerenotedtohaveperipheralandhereditarynervedisorder,and124patientswereaccompaniedwithvestibularneuropathyin286patientsidentifiedtohaveauditoryneuropathy.Inallthepa2tients,theratioofbothgenderswas1:1.40.Mostofthemwereyounger(55.5%).277patients(96.9%)complainedofbilateralgradualhearinglossand9patients(3.1%)complainedofunilateralhearingloss(unilateralauditoryneuropathy,UAN).Patientscouldnotdiscriminatespeechcorrectly,especiallyintheenvironmentofnoise.Accordingtoetiologicalfactor,thepatientsaccom2paniedwithothernervoussystemdiseaseshowedvariedsymptoms.Thevariousfindingofthereviewofthedatewerepresentedinthispaper.Concl...
