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目的 :介绍一种特殊的感音神经性听力疾患—听神经病 ,探讨其临床特征及听力学特点。方法 :报道 5例听神经病患者 ,2例为成人 ;3例为儿童。记录患者的临床资料 ,并对患者进行纯音测听、脑干电反应测听、耳声发射、耳蜗电图及语言辨别率等听力学检查。结果 :5例均主诉听力下降 ,听力学检查纯音听阈为轻、中度感音神经性聋 ,与纯音测听不相符的语言辨别率明显下降 ,不能引出脑干诱发电位 (ABR) ,耳蜗电图基本正常 ,畸变产物耳声发射 (DPOAE)基本正常。提示外毛细胞功能正常 ,病变可能在听神经。结论 :听神经病是一种主诉听力下降 ,纯音听阈为轻、中度感音神经性聋 ,不能引出脑干诱发电位 (ABR) ,畸变产物耳声发射正常的听力疾患 ,临床上应与其他感音神经性聋区别
Objective: To introduce a special sensorineural hearing disorders - auditory neuropathy, to explore its clinical features and audiological features. Methods: Five patients with auditory neuropathy were reported, two were adults and three were children. The clinical data of the patients were recorded and the patients were tested with pure tone audiometry, auditory brainstem response, otoacoustic emissions, electrocochleogram and language discrimination. Results: All of the 5 patients complained of hearing loss. The audiologic thresholds of audiometry were mild and moderate sensorineural deafness. The speech discrimination rate was significantly lower than that of pure tone audiometry. It could not lead to brain stem evoked potential (ABR), cochlear electricity Figure is normal, distortion product otoacoustic emissions (DPOAE) was normal. Tip outer hair cells function properly, the lesion may be in the auditory nerve. Conclusion: Auditory neuropathy is a chief complaint of hearing loss, pure tone threshold for mild to moderate sensorineural deafness, can not lead to brainstem response potential (ABR), distortion product otoacoustic emission of normal hearing disorders, clinical and other sensations should be Nervous deafness difference