Auditory neuropathy
Auditory neuropathy
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DOI：
10.1093/brain/119.3.741
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发表时间：
1996-06-01
期刊：
影响因子：
14.5
通讯作者：
Berlin, CI
中科院分区：
文献类型：
--
作者：
Starr, A;Picton, TW;Berlin, CI
十名患者表现为患有听力障碍的儿童或年轻人，通过行为和生理测试，这些患者与第八脑神经听觉部分的疾病相一致。所有患者的耳声发射和耳蜗麦克风的保存都提供了正常耳蜗外毛细胞功能的证据。听觉脑干电位显示从第 VIII 神经开始的听觉通路功能异常的证据：九名患者的电位缺失，一名患者的电位严重扭曲。所有接受测试的患者均不存在听觉脑干反射（中耳肌肉；耳声发射的交叉抑制）。行为听力测试显示九名患者的纯音阈值轻度至中度升高。如果听力损失的程度是由于耳蜗受体损伤造成的，则不应导致听觉脑干电位的丧失。纯音损失的形状各不相同，五名患者的纯音损失主要为低频，三名患者的所有频率均平坦，两名患者的纯音损失主要为高频。对八名患者的言语清晰度进行了测试，其中六名患者的言语清晰度受到的影响与如果纯音损失是耳蜗来源的情况所预期的不成比例。当听力障碍首次出现时，患者的其他神经功能均正常。随后，其中八名患者出现周围神经病变的证据。神经病有 3 例是遗传性的，5 例是散发性的。我们认为，这种类型的听力障碍是由于听神经功能障碍引起的，并且可能有听神经神经病变作为其原因之一，该神经病变可以单独发生，也可以作为全身神经病变过程的一部分。
Ten patients presented as children or young adults with hearing impairments that, by behavioural and physiological testing, were compatible with a disorder of the auditory portion of the VIII cranial nerve. Evidence of normal cochlear outer hair cell function was provided by preservation of otoacoustic emissions and cochlear microphonics in all of the patients. Auditory brainstem potentials showed evidence of abnormal auditory pathway function beginning with the VIII nerve: the potentials were absent in nine patients and severely distorted in one patient. Auditory brainstem reflexes (middle ear muscles; crossed suppression of otoacoustic emissions) were absent in all of the tested patients. Behavioural audiometric testing showed a mild to moderate elevation of pure tone threshold in nine patients. The extent of the hearing loss, if due to cochlear receptor damage, should not have resulted in the loss of auditory brainstem potentials. The shape of the pure tone loss varied, being predominantly low frequency in five patients, flat across all frequencies in three patients and predominantly high frequency in two patients. Speech intelligibility was tested in eight patients, and in six was affected out of proportion to what would have been expected if the pure tone loss were of cochlear origin. The patients were otherwise neurologically normal when the hearing impairment was first manifest. Subsequently, eight of these patients developed evidence for a peripheral neuropathy. The neuropathy was hereditary in three and sporadic in five. We suggest that this type of hearing impairment is due to a disorder of auditory nerve function and may have, as one of its causes, a neuropathy of the auditory nerve, occurring either in isolation or as part of a generalized neuropathic process.