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目的:探讨Waardenburg综合征(WS)患者人工耳蜗植入方法及手术前后听力评估和术后言语康复效果。方法:回顾性分析2000-2008年确诊的12例WSⅡ型行人工耳蜗植入患儿的临床资料,所有患儿均经乳突面隐窝进路行人工耳蜗植入,将患儿植入后听力情况与12例内耳发育正常的人工耳蜗植入者(对照组)进行比较。结果:12例WS患儿术中电极植入鼓阶顺利,术后无面瘫及脑脊液漏现象发生,术后听阈与对照组无明显区别。经过超过0.5年的言语康复训练,患儿的听力及言语能力均有不同程度提高。结论:人工耳蜗适用于WS患儿,术前应进行全面的听力学及影像学评估。
Objective: To investigate the methods of cochlear implant in patients with Waardenburg syndrome (WS) and the assessment of hearing before and after operation and speech rehabilitation after operation. Methods: The clinical data of 12 children with WS Ⅱ type cochlear implants diagnosed from 2000 to 2008 were retrospectively analyzed. All the children underwent cochlear implants via the mastoid process, and the children were implanted Hearing was compared with that of 12 cochlear implants (control group) with normal inner ear development. Results: In 12 cases of WS children, the electroplating was smooth and the facial paralysis and cerebrospinal fluid leakage did not occur. There was no significant difference between the control group and the threshold after operation. After more than 0.5 years of speech rehabilitation training, children’s hearing and speech skills have improved to varying degrees. Conclusion: Cochlear implants are suitable for children with WS. Comprehensive preoperative audiology and radiological assessment should be performed.