Isaiah Amal, Lee Daniel, Lenes-Voit Felicity, Sweeney Melissa, Kutz Walter, Isaacson Brandon, Roland Peter, Lee Kenneth H
Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Albany Medical College, Albany, NY, USA.
Int J Pediatr Otorhinolaryngol. 2017 Feb;93:1-6. doi: 10.1016/j.ijporl.2016.12.001. Epub 2016 Dec 5.
A significant proportion of children with congenital hearing loss who are candidates for cochlear implants (CIs) may have inner ear malformations (IEMs). Surgical and speech outcomes following CI in these children have not been widely reported.
The charts of children who were evaluated for a CI between 1/1/1986 and 12/31/2014 at a university-based tertiary level pediatric cochlear implant center were reviewed. Principal inclusion criteria included (i) age 1-18 years, (ii) history of bilateral severe to profound sensorineural hearing loss, and (iii) limited benefit from binaural amplification. Exclusion criteria included (i) underlying diagnosis of neurodevelopmental disorder and (ii) lack of follow up for speech assessment if a CI was performed. The following outcome measures were reviewed: (i) imaging findings with magnetic resonance imaging or high resolution computed tomography, (ii) intraoperative complications, and (iii) speech perception categorized as the ability to perceive closed set, open set, or none.
The prevalence of IEMs was 27% (102 of 381), of which 79% were bilateral. Cochlear dysplasia accounted for 30% (40 of 136) of the anomalies. Seventy-eight of the 102 patients received a CI (78%). Surgery was noted to be challenging in 24% (19 of 78), with a perilymphatic gusher being the most common intraoperative finding. Cochlear dysplasia, vestibular dysplasia and cochlear nerve hypoplasia were associated with poor speech perception (open OR closed set speech recognition scores, 0-23%), although the outcomes in children with enlarged vestibular aqueduct were similar to those of children with normal inner ear anatomy (65%).
Cochlear implantation is safe in children with IEMs. However, the speech perception outcomes are notably below those of patients with normal anatomy, with the exception of when an enlarged vestibular aqueduct is present.
很大一部分适合接受人工耳蜗植入（CI）的先天性听力损失儿童可能存在内耳畸形（IEM）。这些儿童接受CI后的手术和言语结果尚未得到广泛报道。
回顾了1986年1月1日至2014年12月31日期间在一家大学附属三级儿科人工耳蜗植入中心接受CI评估的儿童病历。主要纳入标准包括：（i）年龄1至18岁，（ii）双侧重度至极重度感音神经性听力损失病史，以及（iii）双耳放大效果有限。排除标准包括：（i）潜在的神经发育障碍诊断，以及（ii）如果进行了CI但缺乏言语评估随访。回顾了以下结果指标：（i）磁共振成像或高分辨率计算机断层扫描的影像学结果，（ii）术中并发症，以及（iii）言语感知，分为能够感知封闭集、开放集或无感知能力。
IEM的患病率为27%（381例中的102例），其中79%为双侧。耳蜗发育异常占畸形的30%（136例中的40例）。102例患者中有78例接受了CI（78%）。24%（78例中的19例）的手术具有挑战性，术中最常见的发现是外淋巴瘘。耳蜗发育异常、前庭发育异常和蜗神经发育不全与言语感知不良相关（开放集或封闭集言语识别分数为0 - 23%），尽管前庭导水管扩大的儿童的结果与内耳解剖结构正常的儿童相似（65%）。
IEM儿童接受人工耳蜗植入是安全的。然而，除了存在前庭导水管扩大的情况外，言语感知结果明显低于解剖结构正常的患者。
