目的对大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患者的听力学特点进行分析,为其早期诊断和干预提供依据。方法对2010年5月~2013年10月经颞骨CT检查确诊为大前庭水管综合征的49例(96耳)患者均进行声导抗、耳声发射、听性脑干反应(ABR)及听性稳态反应(ASSR)检查,其中23例46耳(6岁以上者,均为双侧LVAS)同时行纯音听阈测试。结果 23例(46耳)6岁以上患者中33耳(71.74%,33/46)纯音听阈表现为低中频(2kHz以下)存在明显气骨导差而高频下降型的混合性听力损失,13耳(28.26%,13/46)表现为无气骨导差的感音神经性听力损失;49例96耳鼓室导抗图均为A型,其中5耳可引出声反射;36例68耳(70.83%,68/96)ABR测试在3~4ms处记录到特征性的负相波(ANSR);46例96耳DPOAE均未通过,ASSR检查多为中、重度听力损失。结论 LVAS患者中耳功能正常,纯音听阈可表现为明显的低频气骨导差而高频下降的混合性听力损失,ABR检查可引出ASNR负相波。
Objective To study the audiological characteristics of large vestibular aqueduct syndrome (LVAS)and provide evidence for early diagnosis and prevention.Methods Tympanometry,Otoacoustic emission , auditory brainstem response (ABR),auditory steady-state response(ASSR)were performed on the 49 cases of LVAS which were diagnosed by CT scanning from May,2010 to October,2013.Among them,23 cases(46ears)were examined by pure tone andiometry at the same time.ResuIts Pure tone andiometry showed that 33 ears were mixed hearing loss in the 23 cases(46 ears),the air-bone gap was larger at low frequencies than that of at high frequen-cies,15 ears were senserineural hearing loss with no air-bone gap;96 ears were type A tympanogram.Acoustic re-flex were present in 5 ears ;34 cases (68 ears)of LVAS group were detected with ASNR in 3 -4 ms by the ABR testing,the positive rate was 70.8%.ConcIusion Our study indicates that for confirmed LAVS,if the pure tone andiometry shows significant air-bone