Cochlear Implantation in Children with Asymmetric Hearing Loss or Single Sided Deafness Clinical Trial

摘要 使听觉环境，例如，教室、学校的体育馆、操场， 孩子一天的一部分。双耳听力有助于在这些环境中的言语理解， 和语音重叠，源自多个源，并且在级别上变化。双耳听觉线索对于 在声学复杂的环境中理解语音并定位说话者;然而，双耳提示被降级或 不对称性听力损失（阿勒，定义为一耳重度至极重度听力损失）儿童 另一方轻度至中度听力损失）或单侧耳聋（SSD，定义为重度至极重度 一只耳朵听力损失，另一只耳朵听力正常或接近正常）。人工耳蜗植入体（CI）是唯一 治疗可以为患有严重至极重度听力损失的耳朵提供听力，因此， 用于双耳听觉。然而，患有阿勒/SSD的儿童并没有常规地植入贫穷的耳朵，因为越好， 耳朵受益于放大（阿勒）或具有正常听力（SSD）。最近，FDA批准了 植入阿勒/SSD儿童子集;然而，批准是限制性的，因为它仅限于以下儿童： 极重度听力损失和弱耳中≤ 5%的单词识别。此外，在以下方面没有达成共识： CI候选人标准，评估工具，或随着时间的推移，这些孩子的表现结果。纵向， 迫切需要采用FDA批准的方案进行前瞻性多中心临床试验，以解决缺陷 阿勒/SSD儿童的研究，并探索CI治疗。我们提出的连续两阶段研究允许 使用当前助听器（HA）技术植入前与植入后的性能增长比较 和一个线人目的1：获得在弱耳接受CI的阿勒/SSD儿童的初步疗效数据。 将单独使用CI的植入后性能与使用HA的植入前性能进行比较。措施 包括单词识别和声场检测水平。目的2评估双模态听力的有效性 定义为弱耳CI和好耳HA（阿勒）或弱耳CI和听力正常， 更好的耳朵（SSD）。通过比较以下各项来测量双峰疗效：1）植入后双峰评分与植入前 最佳辅助评分，2）植入后，双峰评分改善为更好的单耳评分，以及3）双峰评分的变化 随着时间的推移，性能（CI阶段）的最佳辅助性能（HA阶段）的变化。措施 包括噪声和软电平下的语音识别、定位和生活质量指标。基本 与患者选择、测试措施和方法学相关的信息将告知临床管理这些 服务不足的人群。总之，研究结果将提供关于以下方面的关键循证数据： 听力差耳接受CI的阿勒或SSD儿童的双耳听力发展， 这是建立标准化治疗不可或缺的一部分。

ABSTRACT Challenging auditory environments, e.g., classrooms, school cafeterias, playgrounds, comprise a significant portion of a child's day. Binaural hearing facilitates speech understanding in these environments where sounds and speech overlap, originate from multiple sources, and vary in level. Binaural auditory cues are necessary to understand speech and locate talkers in acoustically complex settings; however, binaural cues are degraded or eliminated for children with asymmetric hearing loss (AHL, defined as severe to profound hearing loss in one ear and mild to moderate hearing loss in the other) or single-sided deafness (SSD, defined as severe to profound hearing loss in one ear and normal or near normal hearing in the other). A cochlear implant (CI) is the only treatment that can provide hearing to an ear with severe to profound hearing loss and thus, the only opportunity for binaural hearing. Yet, children with AHL/SSD are not routinely implanted in the poor ear because the better ear benefits from amplification (AHL) or has normal hearing (SSD). Recently, the FDA granted approval to implant a subset of children with AHL/SSD; however, the approval is restrictive as it is limited to children with profound hearing loss and ≤ 5% word recognition in the poor ear. Furthermore, there is no consensus regarding CI candidacy criteria, assessment tools, or performance outcomes over time for these children. A longitudinal, prospective, multi-center clinical trial employing an FDA-approved protocol is critically needed to address deficits of children with AHL/SSD and explore treatment with a CI. Our proposed sequential, two-phase study allows for comparison of performance growth pre-implant with current hearing aid (HA) technology versus post-implant with a CI. Aim 1 obtains preliminary efficacy data in children with AHL/SSD who receive a CI in the poor ear. Post-implant performance with a CI alone is compared with pre-implant performance with a HA. Measures include word recognition and sound field detection levels. Aim 2 evaluates the effectiveness of bimodal hearing defined as a CI in the poor ear and a HA in the better ear (AHL) or a CI in the poor ear and normal hearing in the better ear (SSD). Bimodal efficacy is measured by comparing: 1) post-implant bimodal scores to pre-implant best-aided scores, 2) post-implant, bimodal scores to better ear alone scores, and 3) change in bimodal performance across time (CI phase) to change in best-aided performance over time (HA phase). Measures encompass speech recognition in noise and at soft levels, localization, and Quality of Life metrics. Essential information related to patient selection, test measures, and methodology will inform clinical management of these underserved populations. In summary, study outcomes will provide crucial evidence-based data regarding the development of binaural hearing abilities in children with AHL or SSD who receive a CI in the poor hearing ear, which is integral to the establishment of standardized treatment.