HEARING DEFICIT DUE TO AUDITORY NEUROPATHY

描述：拟议的研究将确定听力学、 一种新的神经学、神经生理学、知觉和遗传学特征 第七颅神经病继发的听力损害类型 很有胆量。这些患者对纯音有轻到中度的损害。 阈值，言语理解明显障碍，听觉缺失 脑干电位，但耳蜗外毛细胞的保存措施 功能(完整的耳声发射)。这个项目将量化 听觉的干扰(定时、频率和强度)和 与纯音听障患者的对比研究 起源。电生理学方法将定义听觉通路 活动，并提供客观措施，以定位的障碍与 第七神经。患者将进行神经学研究(临床 检查、神经传导测量、肌电图术、脑神经 测试)，以确定神经性疾病的范围和分布。一个 将对这种疾病的遗传基础进行研究，因为有一些 这些患者现在被发现有一种遗传性疾病 周围神经功能。进行遗传鉴定的可能性 这种疾病可能会导致治疗这种疾病的分子方法。

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.