Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, SC; Case Western Reserve University School of Medicine, Cleveland, OH.
Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, SC.
J Pediatr. 2018 Feb;193:27-33.e2. doi: 10.1016/j.jpeds.2017.09.053. Epub 2017 Nov 22.
To evaluate hearing impairment in children with Down syndrome, and to describe the factors that influence the severity of hearing loss or changes in hearing over time.
Using the Audiological and Genetic Database (AudGenDB), audiograms of children with Down syndrome were analyzed retrospectively for type, severity, and laterality of hearing loss, as well as changes over time. Medical charts and imaging results were reviewed to identify factors influencing hearing loss.
Among the 1088 patients with Down syndrome included in the study, 921 had hearing loss in at least 1 ear, 91.1% had bilateral hearing loss, and 8.9% had unilateral hearing loss (1760 total ears with hearing loss). Of the ears with hearing loss, 18.8% (n = 180) had moderate or worse hearing loss. "Undefined" hearing loss and pure conductive hearing loss (CHL) were the most common types, followed by mixed hearing loss and sensorineural hearing loss (SNHL). Three-quarters (75.4%) of the children had experienced chronic otitis media or more than 2 episodes of acute otitis media. Patients with bilateral, mixed hearing loss or a history of seizures were at risk for more severe hearing loss. CHL, absence of cholesteatoma, and placement of first ear tubes before age 2 years were associated with greater improvement in hearing over time, whereas SNHL and mixed hearing loss were associated with progressive decline.
Children with Down syndrome who have bilateral, mixed hearing loss or a history of seizures are at risk for more severe hearing loss. SNHL and mixed hearing loss should not be overlooked in patients with CHL. All patients with Down syndrome will benefit from serial audiograms, especially those children with SNHL or mixed hearing loss, which is likely to worsen over time.
评估唐氏综合征儿童的听力障碍，并描述影响听力损失严重程度或随时间变化的因素。
使用听力学和遗传学数据库（AudGenDB），对唐氏综合征儿童的听力图进行回顾性分析，以评估听力损失的类型、严重程度和侧别，以及随时间的变化。查阅病历和影像学结果，以确定影响听力损失的因素。
在研究中纳入的 1088 例唐氏综合征患者中，921 例至少 1 耳有听力损失，91.1%为双侧听力损失，8.9%为单侧听力损失（共有 1760 只听力损失耳）。听力损失耳中，18.8%（n=180）为中度或更严重听力损失。“未定义”听力损失和单纯传导性听力损失（CHL）最为常见，其次是混合性听力损失和感音神经性听力损失（SNHL）。四分之三（75.4%）的儿童患有慢性中耳炎或 2 次以上急性中耳炎。双侧混合性听力损失或有癫痫发作史的患者有发生更严重听力损失的风险。CHL、无胆脂瘤及 2 岁前首次置管与随时间听力改善相关，而 SNHL 和混合性听力损失与听力逐渐下降相关。
双侧混合性听力损失或有癫痫发作史的唐氏综合征儿童有发生更严重听力损失的风险。CHL 患者不应忽视 SNHL 和混合性听力损失。所有唐氏综合征患者均应进行连续听力测试，尤其是那些有 SNHL 或混合性听力损失的儿童，因为这些患者的听力可能随时间恶化。
