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发现的听力障碍形式，称为 听神经病(AN)。与耳蜗性损伤不同的是，听神经炎的特点是 正常测量耳蜗外毛细胞而异常测量耳蜗外毛细胞 中枢听觉通路始于听神经。听力障碍 通常与纯音损失不成比例地影响语音理解， 尤其是噪声中的语音识别。An并不罕见，占10% 被确认为听力损失的新生儿的百分比。这种疾病发生在 无论是孩子还是成年人。在成年人中，这种障碍通常与 周围神经病。神经同步性的丧失被认为是一个基本因素 听力障碍的潜在机制。可能出现异常的位置 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机制和 功能，为缓解听力障碍提供科学依据 受试者有缺陷。我们提出了三个实验，分别使用心理物理和 和电生理技术来表征：(1)基本和复杂 受试者的听觉过程以解释他们的语音识别 难度大，特别是在噪音方面；(2)区分场地的 听神经或内毛细胞/突触复合体的紊乱；以及 (3)优化人工耳蜗术后患者的信号处理。这个 我们的研究结果可能会对诊断、分类、 以及听神经病的治疗。

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.