Hearing Deficit Due to Auditory Neuropathy

听觉神经病变导致的听力缺陷

• 批准号: 7243368
• 负责人: Arnold Starr
• 金额: $ 49.51万
• 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 2010-06-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7243368
• 关键词: Accounting; Acoustic Nerve; Acoustics; Address; Adult; Affect; Attention; Auditory; Auditory Perception; Auditory Perceptual Disorders; Axon; Brain Stem; Cell physiology; Childhood; Chromosome Pairing; Classification; Clinical; Cochlear Implants; Cognitive; Comprehension; Cues; Diagnosis; Disease; Experimental Animal Model; Frequencies; Functional disorder; Goals; Growth; Hair Cells; Hearing; Hearing problem; Inner Hair Cells; Measures; Methods; Nerve; Neuropathy; Newborn Infant; Noise; Numbers; Outer Hair Cells; Peripheral Nervous System Diseases; Physiological; Procedures; Process; Psychophysiology; Publications; Research; Role; Sensorineural Hearing Loss; Sensory; Site; Speech; Stimulus; Synapses; Techniques; Therapeutic Intervention; auditory pathway; base; computerized data processing; deafness; ganglion cell; hearing impairment; implantation; improved; interest; receptor; relating to nervous system; research study; response; speech recognition

DESCRIPTION (provided by applicant): This proposal will study a recently recognized form of hearing disorder called auditory neuropathy (AN) that is due to a disorder of auditory nerve functions in the presence of normal cochlear receptor activities. AN subjects have normal measures of cochlear outer hair cell activities but abnormal measures of the central auditory pathway functions beginning with auditory nerve. The hearing disorder typically affects speech comprehension out-of-proportion to the pure tone loss, particularly speech recognition in noise. AN is not rare and accounts for 10 percent of newborns identified as having hearing loss and also develops in childhood and in adults. In adults the disorder of auditory nerve is commonly associated with a peripheral neuropathy. In addition, AN and sensory hearing loss occur together. Loss of neural synchrony and decreased auditory nerve input are proposed as cardinal mechanisms underlying the hearing disorder. The sites of abnormality along auditory nerve have recently been identified at auditory ganglion cells and their axons (Type I AN) or distally in the auditory periphery where auditory nerve terminals, inner hair cells, and the synapses between inner hair cells with nerve terminals are in close approximation (Type II AN). Our long-term goals are to understand the underlying mechanisms of AN and the accompanying hearing impairments to provide a scientific basis for alleviating the hearing deficit in AN subjects. We propose four experiments using both psychophysical and electrophysiological techniques that will help us achieve these goals. We will characterize (1) the reorganization of central auditory pathway occurring in response to altered auditory nerve input; (2) the contributions of dysynchronization and decreased auditory input as pathophysiological mechanisms underlying AN; (3) the objective physiological and psychophysical measures of improved auditory temporal processes in cochlear implanted AN subjects, and (4) the improvement of speech recognition using new auditory processing techniques. The results of our studies could have major impact on the diagnosis, classification, understanding, and treatment of auditory neuropathy.

描述（由申请人提供）：本提案将研究一种最近认识到的称为听神经病（AN）的听力障碍形式，其是由于在存在正常耳蜗受体活动的情况下听神经功能障碍所致。AN受试者耳蜗外毛细胞活动正常，但从听神经开始的中枢听觉通路功能异常。听力障碍通常会影响言语理解，尤其是噪音中的言语识别。AN并不罕见，占新生儿听力损失的10%，并且在儿童和成人中也会发展。在成年人中，听神经障碍通常与周围神经病变有关。此外，AN和感觉性听力损失一起发生。神经同步性丧失和听觉神经输入减少被认为是听力障碍的主要机制。近来，沿着听神经的异常部位沿着被鉴定为在听神经节细胞及其轴突（I型AN）或在听神经末梢、内毛细胞和内毛细胞与神经末梢之间的突触紧密接近的听觉外周中的远端（II型AN）。我们的长期目标是了解AN及其伴随的听力障碍的潜在机制，为减轻AN受试者的听力障碍提供科学依据。我们提出了四个实验，同时使用心理物理和电生理技术，这将有助于我们实现这些目标。我们将描述（1）中枢听觉通路的重组发生在对改变的听觉神经输入的反应中;（2）作为AN基础的病理生理机制的同步失调和听觉输入减少的贡献;（3）人工耳蜗植入后听觉时间过程改善的客观生理和心理物理指标，以及（4）使用新的听觉处理技术来改进语音识别。我们的研究结果可能对听神经病的诊断、分类、理解和治疗产生重大影响。