HEARING DEFICIT DUE TO AUDITORY NEUROPATHY

听觉神经病变导致的听力缺陷

• 批准号: 2518083
• 负责人: Arnold Starr
• 金额: $ 28.46万
• 依托单位: UNIVERSITY OF CALIFORNIA-IRVINE
• 依托单位国家: 美国
• 财政年份: 1996
• 资助国家: 美国
• 起止时间: 1996-09-01 至 1999-08-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/2518083
• 关键词: acoustic nerve; audiometry; auditory nuclei; auditory threshold; binaural hearing; clinical research; electromyography; evoked potentials; family genetics; human subject; neural conduction; perception disorder; perceptual maskings; psychoacoustics; psychophysiology; sensorineural hearing loss; sound frequency; acoustic nerve; audiometry; auditory nuclei; auditory threshold; binaural hearing; clinical research; electromyography; evoked potentials; family genetics; human subject; neural conduction; perception disorder; perceptual maskings; psychoacoustics; psychophysiology; sensorineural hearing loss; sound frequency

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.

描述：拟议的研究将确定听力学 新的神经，神经生理学，感知和遗传特征 听力障碍的类型是颅骨神经病的继发性障碍 神经。 这些患者的纯音障碍障碍 阈值，明显的语音理解障碍，缺乏听觉 脑干电位，但保留了耳蜗外毛细胞的测量 功能（完整的耳声排放）。 该项目将量化 听觉感知的干扰（时机，频率和强度）和 将这些发现与人工耳蜗纯净听力缺陷的患者进行比较 起源。 电生理方法将定义听觉途径 活动并提供客观的措施，使该疾病与 神经。 患者将进行神经学研究（临床 检查，神经传导措施，肌电图，颅神经 测试）定义神经性疾病的程度和分布。 一个 搜索该疾病的遗传基础，因为许多 现在发现这些患者对 周围神经功能。 遗传鉴定的可能性 这种疾病可能导致该疾病的分子治疗方法。