HEARING DEFICIT DUE TO AUDITORY NEUROPATHY

听觉神经病变导致的听力缺陷

• 批准号: 2770227
• 负责人: Arnold Starr
• 金额: $ 25.42万
• 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 2002-02-28
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/2770227
• 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

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，