Case Western Reserve University School of Medicine, Cleveland, OH, United States of America.
Cleveland Clinic Foundation Head & Neck Institute, Cleveland, OH, United States of America.
Am J Otolaryngol. 2021 May-Jun;42(3):102923. doi: 10.1016/j.amjoto.2021.102923. Epub 2021 Jan 13.
INTRODUCTION & OBJECTIVE: Children with cognitive delay often experience challenges with obtaining hearing thresholds through behavioral audiometry (BA). This necessitates sedated Auditory Brainstem Response (sABR) testing. This study aimed to evaluate diagnostic and hearing patterns in children with Down Syndrome (DS), Autism Spectrum Disorder (ASD), Global Developmental delay (GDD), and Cerebral Palsy (CP) who were unable to complete reliable BA testing due to severe cognitive delay.
Retrospective chart review on a cohort of children aged 0.5-18 years with a diagnosis of DS, ASD, GDD, or CP who underwent sABR due to unsuccessful BA testing. This was performed at a tertiary care institution from 2014 to 2019. Testing patterns and audiometric data were collected.
Across 15 DS, 39 ASD, 10 GDD, and 11 CP patients, the average time from first nondiagnostic BA to sABR ranged from 8.6 months (in GDD) to 21.8 months (in DS). The average number of BAs performed before sABR ranged from 1.6 (in ASD and GDD) to 2.7 (in DS). Hearing loss (HL) was diagnosed in 10%, 13%, 36% and 46% of patients with GDD, ASD, CP and DS respectively. Up to 75% of the HL was sensorineural (in CP patients).
In children with significant cognitive delays, a high incidence of HL (especially SNHL) was identified, therefore high suspicion for HL should be held in these patients. Multiple unsuccessful BAs contribute to prolonged time to diagnosis and treatment, thus prompt sABR should be performed in patients whose severe cognitive delay inhibits reliable testing with BA.
认知延迟的儿童在进行行为测听（BA）获取听力阈值时常常会遇到困难。这就需要进行镇静听觉脑干反应（sABR）测试。本研究旨在评估因严重认知延迟而无法完成可靠 BA 测试的唐氏综合征（DS）、自闭症谱系障碍（ASD）、全面发育迟缓（GDD）和脑瘫（CP）儿童的诊断和听力模式。
回顾性分析了 2014 年至 2019 年在一家三级医疗机构因 BA 测试失败而接受 sABR 的 0.5-18 岁患有 DS、ASD、GDD 或 CP 的儿童队列的病历。收集了测试模式和听力数据。
在 15 例 DS、39 例 ASD、10 例 GDD 和 11 例 CP 患者中，从首次非诊断性 BA 到 sABR 的平均时间从 GDD 的 8.6 个月到 DS 的 21.8 个月不等。在进行 sABR 之前进行的 BA 数量平均从 ASD 和 GDD 的 1.6 次到 DS 的 2.7 次不等。GDD、ASD、CP 和 DS 患者的听力损失（HL）诊断率分别为 10%、13%、36%和 46%。高达 75%的 HL 为感音神经性（在 CP 患者中）。
在认知能力严重延迟的儿童中，发现 HL（尤其是 SNHL）的发生率很高，因此应高度怀疑这些患者存在 HL。多次不成功的 BA 会导致诊断和治疗时间延长，因此对于因严重认知延迟而无法进行可靠 BA 测试的患者，应及时进行 sABR。
