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1、儿童大前庭导水管综合征听力特点分析(附6例报告)【摘要】目的探讨儿童大前庭导水管综合征的听力学特点、。方法对6例2.5-S.5岁患有大前庭导水管综合征的病儿在初次发病时进行听性脑干诱发反应(ABR)>40Hz诱发反应、多频稳态诱发反应(ASSR)检查，并在初次发病及最近一次发病时行瞬态诱发性耳声发射(TEOAE)、声导抗及纯音听阈(或行为测听)检查。结果6例12耳初诊及最近一次的声导抗检查均为A型鼓室图，磴骨肌反射均未引出。TEOAE检查均未通过。AER、40Hz诱发反应、ASSR、纯音听阈检查
2、结果均为中度、重度甚至极重度听力损失，且98dBnHLclick刺激声时ABR检查结果示在潜伏期2.77ms左右均有显著负反应波(ASNR)出现。纯音听阈结果示在250、500、1000Hz时均有气骨导差且均大于15dEnHL。结论大前庭导水管综合征病儿初次发病时一般为双侧中重度以上感音神经性听力损失，随后波动性听力逐渐下降直至全聋oASNR的出现可以作为大前庭导水管综合征听力学诊断的参考指标，但确诊仍依靠影像学检查。大前庭导水管综合征纯音听阈可以有低范围的气骨导差，但原因不明。本病目前没有有效
3、的治疗方法，但及时助听语训，对病儿的言语语言发育是有效的。重视日常防护及发病时尽早治疗，大前庭导水管综合征是可以有效预防或延迟发作的。【关键词】大前庭导水管综合征;听力检查;声诱发短潜伏期负反应;儿童[ABSTRACT]ObjectiveToinvestigatetheaudiologicalcharacteristicsofchildrenwithlargevestibularaqueductsyndrome(LVAS).MethodsExaminationsofABR,40Hz,andASS
4、Rwerecarriedoutinsixchildrenages2.5-8.5yearswithLVASatthefirstepisode・Transientevokedoto-acousticemission(TEOAE),acousticimmitance,andaudibilitythresholdsforapuretone(ATPT)werenvestigatedatthefirstandlatestepisodes.ResultsAllthe12earsofthesixchildren
5、weretypeAtympanogramatboththefirstandthelatestexamination.Stapedialacousticreflexwasnotproduced,andtheyallfailedinTEOAE.TheresultsofABR,40Hz,ASSRandATPTweremoderate,severeorextremelyseverehearingloss.TheABRshowedthatsignificantASNRappearedat2.77msoft
6、helatencieswhenstimulatedwith98dBnHLclicks・ATPTtestshowedthatairbonegapexistedat250,500,and1000Hz,andexcelled15dBnHL.ConclusionGenerally,moderateorsevereneurosensoryhearinglossofbothearsoccursattheinitialepisodeofLVASandaggravatesgraduallytototaldeaf
7、ness・TheappearanceofASNRcanberegardedasareferenceforthediagnosisofLVAS,butthefinaldiagnosisdependingonimageology・Anair-bonegapmightexistinATPTofLVASatlowfrequencies,whiletheunderlyingcauseremainsunclear.Todate,therearenoeffectivetherapiesavailable,bu
8、tprompttraininginhearingandspeakingiseffectiveinthelanguagespeakingdevelopmentofthesickchildren.Thinkinghighlyofusualprotectionandtreatmentassoonaspossiblecaneffectivelypreventordelaytheonsetofthedisease・[KEYWORDS]largevestibularaqueductsyndrome;hear
