HEARING DEFICIT DUE TO AUDITORY NEUROPATHY

描述（由申请人提供）： 这项提案将研究一种最近被认可的听力障碍形式， 听神经病（AN）。与耳蜗损害不同，AN的特点是 通过耳蜗外毛细胞的正常测量， 从听神经开始的中枢听觉通路。听力障碍 典型地影响与纯音损失不成比例的语音理解， 尤其是噪声中的语音识别。AN并不罕见，占10 新生儿被确定为听力损失的百分比。这种疾病发生在 儿童和成人。在成年人中，这种疾病通常与 周围神经病变神经同步性的丧失被认为是一个重要的 听力障碍的潜在机制。AN的异常部位可能 包括听神经和/或内毛细胞及其与听觉突触 神经树突我们的长期目标是了解AN机制， 为缓解听力障碍提供科学依据 A.受试者的缺陷。我们提出了三个实验，使用心理物理学和 和电生理技术的特点：（1）基本和复杂的 听觉过程，以解释他们的语音识别 （2）在噪音方面;（3）在噪音方面;（4）在噪音方面。 听觉神经或内毛细胞/突触复合体的病症;以及 (3)为植入人工耳蜗的AN患者优化信号处理。的 我们的研究结果可能对诊断，分类， 和治疗听神经病。

DESCRIPTION (provided by applicant): This proposal will study a recently recognized form of hearing disorder called auditory neuropathy (AN). Different from cochlear damage, AN is characterized by normal measures of cochlear outer hair cells but abnormal measures of the central auditory pathway 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. The disorder occurs in children and adults. In adults the disorder is commonly associated with a peripheral neuropathy. Loss of neural synchrony is proposed as a cardinal mechanism underlying the hearing disorder. The sites of abnormality in AN could include auditory nerve and/or inner hair cells and their synapses with auditory nerve dendrites. Our long-term goals are to understand the AN mechanisms and functions in order to provide a scientific basis for alleviating the hearing deficit in AN subjects. We propose three experiments using both psychophysical and elecrophysiological techniques to characterize: (1) fundamental and complex auditory processes in AN subjects in order to explain their speech recognition difficulty, particularly in noise; (2) distinguish between the site of the disorder as being at auditory nerve or at inner hair cell/synapse complex; and (3) optimize signal processing for AN subjects with cochlear implants. The resultsof our studies could have major impact on the diagnosis, classification, and treatment of auditory neuropathy.