Electrically-evoked neural responses in children with auditory neuropathy

描述(由申请人提供)：人工耳蜗植入(CI)已被用作治疗听神经病谱系障碍(ANSD)儿童的一种选择，这些儿童无法从传统助听器中受益。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，为个别ANSD患者选择的编程设置可能不是最佳的，因为许多ANSD儿童由于年龄较小和/或合并疾病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，有时甚至不确定。这项研究计划的长期目标是开发可用于临床环境的客观工具，以准确预测植入后的言语感知能力，并优化ANSD儿童个体的编程设置。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儿童的神经的时间反应特性来评估电刺激由听神经传递的情况。此外，该项目还旨在探索在外周和更中央水平记录的神经反应相互关联的程度，以及它们是否与ANSD患者的言语感知能力相关。具体目的1通过测量不同刺激率下的电诱发听觉复合动作电位(ECAP)来表征儿童CI使用者听神经的时间反应特性。ANSD儿童和感音神经性听力损失儿童都将接受测试。这些实验的结果将为更好地理解ANSD儿童植入后言语感知能力的跨患者差异的来源提供更好的理解。具体目的2评估植入ANSD儿童的听神经时间反应特性、间隙检测阈值和言语感知能力之间的关联。这些研究的结果应该会扩大人们对中央听觉皮质中的听觉处理如何受到听神经输入的影响的理解。总体而言，这个项目的结果可能会导致发展 一种客观的工具，用于识别ANSD患者亚群，这些患者不太可能在植入后的早期阶段从他们的设备中获得实质性好处。此外，这些结果还可能导致特定信号处理改进的开发，这可能会改善个别植入的ANSD患者的表现。因此，这个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.