Electrically-evoked neural responses in children with auditory neuropathy

描述（由申请人提供）：耳蜗植入（CI）已被用作听力神经病变谱系障碍（ANSD）儿童的治疗选择，这些儿童不能从传统的助听器中获益。到目前为止，还没有可靠的指标来预测这些患者的CI结果。此外，为个体ANSD患者选择的程序设置可能不是最佳的，因为许多ANSD儿童由于年龄小和/或合并症而无法提供可靠的行为反应。这些情况使得植入式ANSD儿童的听力学管理极具挑战性，并且在许多时候不确定。该研究项目的长期目标是开发客观的工具，用于临床环境，以准确预测植入后的语音感知表现，并优化个体ANSD儿童的编程设置。作为实现这一长期目标的第一步，该项目旨在通过评估植入的ANSD儿童听神经的时间反应特性来评估电刺激在听神经上的传导情况。此外，本项目还旨在探讨在外围和更中枢水平记录的神经反应之间的相互关联程度，以及它们是否与ANSD患者的言语感知表现相关。特异性目的1通过测量不同刺激率下的电诱发听觉复合动作电位（ECAP）来表征儿科CI使用者听神经的时间反应特性。患有ANSD的儿童和患有感音神经性听力损失的儿童都将接受测试。这些实验的结果将提供更好的理解跨患者差异的来源植入后的语音感知表现的ANSD儿童。特异性目的2评估植入性ANSD儿童听神经颞叶反应特性、间隙检测阈值与言语感知表现之间的关系。这些研究的结果应该扩大对听觉中枢皮层的听觉处理如何受到听神经输入的影响的理解。总的来说，这个项目的结果可能会导致发展

DESCRIPTION (provided by applicant): Cochlear implantation (CI) has been used as a treatment option for children with auditory neuropathy spectrum disorder (ANSD) who do not benefit from conventional hearing aids. Up to date, there is no robust indicator for predicting CI outcomes in these patients. In addition, programming settings selected for individual ANSD patients might not be optimal since many ANSD children are not able to provide reliable behavioral responses due to their young ages and/or comorbid conditions. These conditions make the audiologic management of implanted ANSD children extremely challenging and at many times uncertain. The long-term goal of this program of research is to develop objective tools that can be used in clinical settings for accurately predicting post-implant speech perception performance and optimizing programming settings for individual ANSD children. As the first step towards fulfilling this long-term goal, this project aims to evaluate how well electrical stimulation is transmitted by the auditory nerve by evaluating temporal response properties of the nerve in implanted ANSD children. In addition, this project also aims to explore the extent to which neural responses recorded at a peripheral and more central level are correlated with each other and whether they are correlated with speech perception performance in ANSD patients. Specific Aim 1 characterizes temporal response properties of the auditory nerve in pediatric CI users by measuring the electrically evoked auditory compound action potential (ECAP) at different stimulation rates. Both children with ANSD and children with sensorineural hearing loss will be tested. Results from these experiments will provide a better understanding of sources of across-patient variations in post-implant speech perception performance among ANSD children. Specific Aim 2 evaluates the association among temporal response properties of the auditory nerve, gap detection threshold and speech perception performance in implanted ANSD children. Results of these studies should expand understandings of how auditory processing in the central auditory cortex is affected by the input from the auditory nerve. Overall, results of this project could potentially lead to the development of an objective tool for identifying a subgroup of ANSD patients who are unlikely to receive substantial benefit from their devices at an early post-implant stage. In addition, these results could also potentially lead to the development of specific signal processing modifications that might result in improved performance for individual implanted ANSD patients. Therefore, this project has tremendous translational significance.