Cochlear implantation in children with syndromic deafness
Cochlear implantation in children with syndromic deafness
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DOI：
10.1016/j.ijporl.2013.05.022
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发表时间：
2013-08-01
影响因子：
1.5
通讯作者：
Green, Kevin M. J.
中科院分区：
文献类型：
--
作者：
Broomfield, Stephen J.;Bruce, Iain A.;Green, Kevin M. J.
目的：研究综合征性耳聋儿童人工耳蜗植入术的结果，这些儿童越来越多地被考虑进行人工耳蜗植入术，并且对人工耳蜗团队构成了独特的挑战。方法：在这个回顾性的病例系列在三级转诊人工耳蜗中心，我们描述了一系列38名儿童临床综合征导致耳聋谁接受了人工耳蜗植入。结果测量是Bench-Kowal-Bamford （BKB）语音接收评分（范围0-100%）和使用Geers和Moog语音接收评分（SRS）的语音感知能力（范围从0；无语音感知，到6；单词的开放集识别）。结果：鉴定出的综合征为Waardenburg综合征（n = 10）、Usher综合征（n = 9）、Pendred综合征（n = 7）、Jervell和Lange-Nielsen综合征（n = 5）、CHARGE综合征（n = 2）， Stickler综合征、CINCA综合征、Butter综合征、Down综合征、donnaibarrow综合征各1例。植入后至少19个月，38名患者中有20名患者的BKB可测量，在安静状态下，BKB从46%到100%不等（中位87%，平均81%）。18名儿童（55%）在6级达到了SRS，另有8名儿童（24%）达到了5级。两组间及组内的结果有显著差异。结论：在考虑儿童人工耳蜗植入术时，经常会遇到额外的残疾，并且可能是公认综合征的一部分。结果通常很好，但即使在同一综合征组中也可能变化，因此对这些儿童进行个体评估，以确保现实的期望。2013爱思唯尔爱尔兰有限公司版权所有。
Objective: To examine the outcome of cochlear implantation in children with syndromic deafness, who are increasingly being considered for cochlear implants and who represent a unique challenge to the cochlear implant team.Method: In this retrospective case series in a tertiary referral cochlear implant centre, we describe a series of 38 children with a clinical syndrome causing deafness who have undergone cochlear implantation. The outcome measures are Bench-Kowal-Bamford (BKB) speech reception score (range 0-100%) and speech perception ability using the Geers and Moog Speech Reception Score (SRS) (range from 0; no speech perception, to 6; open set recognition of words).Results: The syndromes identified were Waardenburg syndrome (n = 10), Usher syndrome (n = 9), Pendred syndrome (n = 7), Jervell and Lange-Nielsen syndrome (n = 5), CHARGE syndrome (n = 2), and 1 each of Stickler, CINCA (Chronic Infantile Neurological Cutaneous and Articular), Butter, Down, and Donnai-Barrow syndromes. After a minimum of 19 months following implantation, BKB was measurable in 20 of 38 patients, and ranged from 46 to 100% in quiet (median 87%, mean 81%). Eighteen children (55%) achieved a SRS at level six, and a further 8(24%) achieved level five. There was significant variation of outcome between and within syndrome groups.Conclusions: Additional disabilities are frequently encountered when considering children for cochlear implantation, and may be part of a recognised syndrome. Outcome is often excellent but can be variable even within the same syndrome group, and such children are therefore assessed on an individual basis to ensure a realistic expectation. (C) 2013 Elsevier Ireland Ltd. All rights reserved.