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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。
Objective: To investigate the clinical and auditory functional characteristics of auditory neuropathy. Methods: The clinical data, audiological tests and electrophysiological examination of 54 patients with auditory neuropathy were summarized and analyzed. Results: Pure tone audiogram showed ascending 70 ears, tuberose 25 ears, flat 5 ears, descending 4 ears. The mean threshold of low frequency, middle frequency and high frequency were (67.63 ± 15.30,43.61 ± 16.28,32.25 ± 14.80) dB HL. Acoustic conductivity tympanogram all normal, 77 ear ossicle acoustic reflex disappeared, 31 acoustic reflex threshold increased. Auditory brainstem response (ABR) did not lead to all. Distortion product otoacoustic emissions (DPOAE) normal lead, 26 patients contralateral acoustic suppression was not affected. 16 cases of speech recognition rate is poor, and tone threshold is out of proportion. 23 cases of temporal bone CT or MRI showed no abnormalities. 10 cases with peripheral neuropathy. CONCLUSIONS: ABR is absent from wave Ⅰ and DPOAE is normally elicited. The difference of speech resolution is not proportional to the threshold of pure tone, and the tone of stapedius muscle and OAE cross-inhibitory abnormality. The pure tone audiogram mostly shows ascending type with low frequency loss, Listen to the important features of the function. Tip lesions mainly located in the cochlear auditory nerve fibers. Differentiation should be with the general sensorineural deafness and central deafness.