The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome.
The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome.
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DOI：
10.1155/2016/2854736
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发表时间：
2016
影响因子：
--
通讯作者：
Yamasoba T
中科院分区：
文献类型：
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作者：
Koyama H;Kashio A;Sakata A;Tsutsumiuchi K;Matsumoto Y;Karino S;Kakigi A;Iwasaki S;Yamasoba T
目标。本研究旨在探讨人工耳蜗术治疗Waardenburg综合征感音神经性耳聋的可行性。方法。对在东京大学医院接受人工耳蜗术的患者进行了回顾性图表回顾。评估临床分类、基因突变、临床病程、术前听力阈值、高分辨率计算机断层扫描和术后听力结果。结果。5例Waardenburg综合征患儿接受人工耳蜗植入术。植入时平均年龄2岁11个月(1岁9个月~6岁3个月)。4例为先天性重度听力损失，1例为进行性听力损失。2例内耳畸形为耳蜗2型不完全分隔型，无手术并发症或困难。所有患者术后听力均良好。结论。人工耳蜗术可能是治疗Waardenburg综合征的一种很好的选择。
Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.