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目的 探讨听觉神经病的临床特点、听力学及电生理特征。方法 总结了 17例听觉神经病患者的病史、纯音及语言听力、镫骨肌反射、听性脑干反应 (auditorybrainstemresponse ,ABR)、耳蜗电图、鼓岬电刺激试验、耳声发射及对侧声抑制试验的特点。结果 该病多在青春期后起病 ,女性较多发 ,表现为渐进性双耳对称性听力下降 ,纯音听力以低频损失为主 ,语言接受阈升高 ,分辨率下降 ,镫骨肌反射及ABR引不出或阈值明显升高与纯音听力不成比例 ,耳蜗微音器电位正常 ,听神经动作电位引不出 ,耳声发射波幅高、时程长且不为对侧声刺激所抑制。结论 听觉神经病的诊断应在除外各种已知原因的感音神经性聋后 ,根据其纯音听力与客观测听的不一致及电生理学上的耳蜗活动与中枢系统反应分离的特征作出 ,对该病的认识尚需进一步深入。
Objective To investigate the clinical features, audiology and electrophysiological features of auditory neuropathy. Methods A total of 17 patients with auditory neuropathy were reviewed. Their medical history, pure tone and verbal hearing, auditory brainstem response (ABR), auditory brainstem response (ABR), electrocochleography, promontory stimulation, otoacoustic emissions and contralateral acoustic suppression The characteristics of the experiment. Results The disease mostly occurred after puberty. The women had multiple onset, manifested as progressive binaural symmetry hearing loss, pure tone hearing loss with low frequency loss, increased language threshold, decreased resolution, stapedius reflex and ABR No significant increase threshold or pure tone hearing loss, normal cochlear microphones potential, auditory nerve action potential can not be cited, otoacoustic emissions amplitude, duration and not contralateral acoustic stimulation inhibited. Conclusions The diagnosis of auditory neuropathy should be based on the exclusion of various known causes of sensorineural deafness based on the discrepancy between pure tone hearing and objective audiometry and the characteristics of electrophysiological cochlear activity and central nervous system response. Understanding needs to be further deepened.