听神经病临床表现特征分析

Analysis of Clinical Features of Auditory Neuropathy

目的探讨听神经病患者的临床表现特点。方法总结分析18例(36耳)听神经病患者的一般情况、听力学及电生理检查结果。结果听神经病患者均为双耳发病,青少年多见,平均发病年龄17.94岁;纯音测听表现为双耳基本对称、上升型感音神经性听力损失,以低频250Hz、500Hz损失为主;鼓室压力图多为A型,声反射均消失;畸变产物耳声发射(DPOAE)正常,对侧声抑制消失;听性脑干反应(ABR)异常,阈值均≥90dBnHL。结论 ABR严重异常、声反射消失、低频纯音听阈升高而DPOAE正常,多见于青少年、双耳发病等是听神经病的临床特征。

Objective To explore the clinical features of auditory neuropathy. Methods General state of health, hearing findings and electrophysiologic results were analyzed in 18 cases of auditory neuropathy. Results The patients were predominantly youngster with bilateral hearing loss and the average age of onset was 17.94 years old. The pure tone audiogram showed binaural symmetric upward - sloping audiogram with sensorineurat hearing loss at low frequencies of 250Hz and 500Hz. Tympanogram were A style mostly with absent stapedial reflex. DPOAE which cann＇t be suppressed by contralateral white noise was normal. ABR was abnormal with higher threshold o- ver 90dBnHL. Conclusion Patients with auditory neuropathy were predominantely juveniles with bilateral hearing loss at low frequencies, meanwhile their stapedial reflex and ABR were absent or threshold elevated besides of normal DPOAE.