听神经病的临床与听功能特征

目的:探讨听神经病的临床与听功能特征。方法:总结分析54例听神经病患者的临床资料、听力学测试及电生理检查情况。结果:纯音听力图呈上升型70耳,覆盆型25耳,平坦型5耳,下降型4耳;低频、中频及高频平均阈值为(67.63±15.30,43.61±16.28,32.25±14.80)dB HL。声导抗鼓室图全部正常,77耳镫骨肌声反射消失,31耳声反射阈部分增高。听性脑干反应(ABR)全部未引出。畸变产物耳声发射(DPOAE)正常引出,26例行对侧声抑制未受影响。16例言语识别率差,与纯音听阈不成比例。23例颞骨CT或MRI未见异常。10例伴有周围神经病。结论:ABR自波Ⅰ起缺失而DPOAE正常引出,言语分辨力差与纯音听阈不成比例,镫骨肌声反射及OAE交叉抑制异常,纯音听力图多呈上升型以低频损失为主,是听神经病听功能的重要特征。提示病损主要位于耳蜗内听神经纤维。应与一般的感音神经性聋和中枢性聋相鉴别。

Auditory neuropathy

OBJECTIVE: To explore the clinical and auditory functional feature of auditory neuropathy(AN). METHOD: The clinic signs, audiometric test and electrophysiology were analyzed on 54 cases of AN. RESULT: 70 ears had low frequency pure tone hearing loss. 24 ears had cover basic hearing threshold. 6 ears had flat hearing threshold. 4 ears showed high frequency hearing loss. The average low-frequency, median-frequency and high-frequency hearing threshold were 67.63 +/- 15.30, 43.61 +/- 16.28 and 32.25 +/- 14.80 dB HL respectively. The tympanograms are normal. 77 ears lost acoustic reflex. 31 ears increased acoustic reflex threshold. All of them induced no ABR and had normal DPOAE with 26 cases' contralateral acoustic suppression uninfluenced. 16 cases had controversial pure tone threshold compared with speech discrimination. 23 had normal temporal bone CT image or MRI. 10 cases accompanied with peripheral neuropathy. CONCLUSION: Abnormal ABR, normal DPOAE, unparallel pure tone threshold to speech audiometry, loss of stapedial acoustic reflex and OAE contralateral acoustic suppression, loss of the pure tone threshold primarily in low-frequency SNHL are the important features of AN, which shows that the lesioned site may lie at the intracochlear acoustic nerve. It is necessary to differentiate it from the common SNHL and central nerve hearing loss.