HEARING DEFICIT DUE TO AUDITORY NEUROPATHY

描述：拟议的研究将确定听力学， 神经，神经生理，知觉和遗传特征的新的 第八颅神经病变继发的听力损害类型 神经。 这些患者有轻度至中度纯音损害 阈值，言语理解明显受损，听觉缺失 脑干电位，但保留耳蜗外毛细胞的测量 功能（完整耳声发射）。 该项目将量化 听觉感知障碍（时间、频率和强度）， 与耳蜗纯音听力障碍患者的结果进行比较 起源 电生理学方法将确定听觉通路 活动，并提供客观的措施，以本地化的障碍， 第八根神经。 患者将接受神经学研究（临床 检查，神经传导测量，肌电图，脑神经 测试），以确定神经性疾病的程度和分布。 一 由于许多疾病的遗传基础， 这些病人现在被发现有遗传性障碍， 外周神经功能 基因鉴定的可能性 这种疾病可能导致治疗该疾病的分子方法。

DESCRIPTION: The proposed studies will identify the audiological, neurological, neurophysiological, perceptual, and genetic features of a new type of hearing impairment secondary to the neuropathy of the VIIIth Cranial Nerve. These patients have light-to-moderate impairment of pure tone thresholds, marked impairment of speech comprehension, absence of auditory brain stem potentials, but preserved measures of cochlea outer hair cell function (intact otoacoustic emissions). This project will quantify the disturbances of auditory perception (timing, frequency, and intensity) and compare the findings to patients with pure tone hearing deficits of cochlear origin. The electrophysiological methods will define auditory pathway activity and provide objective measures to localize the disorder with the VIIIth nerve. The patients will have neurological studies (clinical examinations, nerve conduction measures, electromyography, cranial nerve tests) to define the extent and distribution of the neuropathic disorder. A search for the genetic basis of the disorder will be made since a number of these patients have now been found to have a hereditary disturbance of peripheral nerve function. The possibility of a genetic identification for this disorder may lead to molecular methods of treatment for the disorder.