大前庭水管综合征患者的听力学及前庭诱发肌源性电位检测的特点  

Clinical characterization of audiometrics and vestibular evoked myogenic potentials in patients with large vestibular aqueduct syndrome

作　　者：吴军[1,2] 王锦玲[1] 谢娟[1] 韩丽萍[1] 高磊[1] 

机构地区：[1]第四军医大学西京医院耳鼻咽喉头颈外科,西安710032 [2]总政治部机关门诊部,北京100011

出　　处：《临床耳鼻咽喉头颈外科杂志》2010年第1期25-27,共3页Journal of Clinical Otorhinolaryngology Head And Neck Surgery

摘　　要：目的:探讨大前庭水管综合征(LVAS)患者的听力学、前庭诱发肌源性电位(VEMP)检测的特点及诊断意义。方法:对30例(60耳)LVAS患者行纯音测听、声导抗、ABR、畸变产物耳声发射(DPOAE)、VEMP及冷热实验检测,分析其诊断意义。结果:30例(60耳)LVAS患者均呈进行性、波动性听力下降,16例在听力波动性下降时伴眩晕。纯音测听主要呈感音神经性聋,但47耳(94.0%)在中耳功能正常的情况下于低频250、500Hz分别出现(43±17)dBHL及(33±18)dBHL骨气导差,呈混合性聋。ABR检测18耳(64.3%)于(3.06±0.52)ms引出声诱发短潜伏期负反应。42耳VEMP振幅均值为(147.10±107.55)μV,19耳反应阈为75dBnHL,7耳反应阈为65dBnHL。结论:根据进行性波动性听力减退、感音神经性聋在中耳功能正常的情况下出现低频骨气导差,ABR测试引出声诱发短潜伏期负反应及VEMP呈现高振幅低阈值的特征性提示,有助于临床医生得出LVAS的初步印象,可进一步行影像学检查以确诊。Objective：To investigate the clinical and diagnostic characteristics of audiometric findings and vestibular-evoked myogenic potentials in patients with large vestibular aqueduct syndrome（LVAS）.Method：Thirty LVAS subjects（60 ears）recruited received pure tone audiometry,acoustic immittance,auditory brain stem responses（ABRs）,distortion-product otoacoustic emission（DPOAE）,Vestibular evoked myogenic potentials（VEMP）and caloric test,and the diagnostic significance of the results was analyzed.Result：All 30 cases（60 ears）showed progressive and fluctuating hearing loss,while 16 cases experienced dizziness when hearing fluctuated.Most of our cases showed sensorineural hearing loss,and 47 ears（94.0%）showed air-bone gap in the low frequencies,with mean gaps of（43±17）dB HL at 250 Hz,（33±18）dB HL at 500 Hz,in which the middle ear function showed normal.The acoustically evoked short latency negative response（ASNR）with medium latency（3.06±0.52）ms was elicited from 18 ears（64.3%）.The mean amplitude of vestibular evoked myogenic potentials（VEMP）of 42 ears was（147.10± 107.55）μv,and the threshold of VEMP of 19 ears was 75 dB nHL,of 7 ears was 65 dB nHL.Conclusion：Characteristics of hearing performance,such as progressive and fluctuating hearing loss,air-bone gap at the low frequencies with normal middle ears,the ASNR,and increased amplitude and decreased threshold of the VEMPs,will help clinicians make initial diagnosis of LVAS,and provide a reference for further imaging examination.

关 键 词：大前庭水管综合征 聋 感音神经性 低频骨气导差 短潜伏期负反应 前庭诱发肌源性电位 

分 类 号：R746[医药卫生—神经病学与精神病学] R318.18[医药卫生—临床医学]