Electrically-evoked neural responses in children with auditory neuropathy

描述（由申请人提供）：耳蜗植入（CI）已被用作无法从传统助听器中获益的听神经病谱系障碍（ANSD）儿童的治疗选择。到目前为止，还没有可靠的指标来预测这些患者的CI结局。此外，为个别ANSD患者选择的程控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。该研究计划的长期目标是开发可用于临床环境的客观工具，用于准确预测植入后的语音感知性能，并优化个别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ANSD儿童神经的时间响应特性来评估电刺激通过听觉神经传输的情况。此外，本项目还旨在探索在外周和更中央水平记录的神经反应彼此相关的程度，以及它们是否与ANSD患者的言语感知表现相关。具体目标1通过测量不同刺激速率下的电诱发听觉复合动作电位（ECAP）来表征儿科CI用户的听觉神经的时间响应特性。ANSD儿童和感音神经性听力损失儿童都将接受测试。从这些实验的结果将提供一个更好的理解源植入后的言语感知表现ANSD儿童之间的跨患者的变化。特定目标2评估了植入ANSD儿童的听神经的时间反应特性、间隙检测阈值和言语感知性能之间的关联。这些研究的结果应该扩大理解如何在中央听觉皮层的听觉处理是由来自听觉神经的输入的影响。总的来说，该项目的结果可能会导致开发 用于识别不太可能在植入后早期从其器械中获得实质性获益的ANSD患者亚组的客观工具。此外，这些结果还可能导致特定信号处理修改的开发，这可能导致个体植入ANSD患者的性能改善。因此，该项目具有巨大的翻译意义。

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.