Wang Jibao, Duan Jiade, Li Qingtian, Huang Xiang, Chen Haihua, Jin Jing, Gong Shusheng, Kong Weijia
Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.
Zhonghua Er Bi Yan Hou Ke Za Zhi. 2002 Aug;37(4):252-5.
To study the auditory electrophysiological characteristics of the auditory neuropathy.
Ten patients were diagnosed as auditory neuropathy. The history, pure tone audiometry, stapedial reflex, auditory brainstem response, electrocochleogram, distortion product otoacoustic emission and his contralateral suppression of white noise, middle latency response and slow cortical response were assessed. Five patients were investigated with brain CT or MRI.
The patients were of both genders. The average age was 20.3 years old and most of them were teenagers. Nine patients complained of bilaterally hearing impairment, while one patient who did not complain of hearing loss had a tinnitus of both ears. The pure tone audiogram showed a middle or moderate sensorineural hearing loss at low frequencies 0.5 kHz and/or 0.25 kHz in nineteen ears but a normal audiogram in one ear. Stapedial reflex was absent in nineteen ears, and threshold elevated in one ear while his pure tone audiogram revealed an ascending curve. Auditory brainstem response cannot be recorded or only wave V or/and wave I presented. All of the patients were evoked a distortion product OAE, which cannot be suppressed by contralateral white noise. Electrocochleogram demonstrated AP absent or very small amplitude in most ear and the--SP amplitude were 0.595 microV, while they had a normal slow cortical response but except one patient presented normal middle latency response. The findings of CT or MRI were normal.
The pure tone audiogram could preserve unchanged at early stage of auditory neuropathy, but their stapedial reflex and auditory brainstem response are absent or threshold elevated. The precise pathologic site of this disorder is yet to be defined, but it may locate in the auditory system below brainstem.
研究听神经病的听觉电生理特征。
10例患者被诊断为听神经病。对其病史、纯音听力测定、镫骨肌反射、听性脑干反应、耳蜗电图、畸变产物耳声发射及其对侧白噪声抑制、中潜伏期反应和慢皮层反应进行评估。5例患者进行了头颅CT或MRI检查。
患者男女均有。平均年龄20.3岁,以青少年居多。9例患者诉双侧听力减退,1例未诉听力下降的患者双耳耳鸣。纯音听力图显示,19耳在0.5kHz和/或0.25kHz低频处呈中度或重度感音神经性听力损失,1耳听力图正常。19耳镫骨肌反射消失,1耳阈值升高,其纯音听力图呈上升型曲线。听性脑干反应无法引出或仅引出V波或/和I波。所有患者均能引出畸变产物耳声发射,且不能被对侧白噪声抑制。耳蜗电图显示多数耳AP消失或波幅极小,-SP波幅为0.595μV,慢皮层反应正常,但1例患者中潜伏期反应正常。CT或MRI检查结果正常。
听神经病早期纯音听力图可无变化,但其镫骨肌反射和听性脑干反应消失或阈值升高。该疾病的确切病理部位尚待明确,但可能位于脑干以下的听觉系统。