Waardenburg综合征患者人工耳蜗术后效果评价:系统评价以及meta分析

Rehabilitation Outcomes after Cochlear Implantation in Waardenburg Syndrome:A Systematic Review and Meta-Analysis

目的 评价Waardenburg综合征患者人工耳蜗植入后听觉功能改善及言语表达能力变化,以及耳蜗植入时间与术后改善情况之间的关系。方法 通过检索2000年-2021年间Pubmed,Web of Science,EMbase以及CNKI,万方数据库中发表的英文以及中文研究中,依据纳入以及排除标准,筛选文献共15篇,进行系统性评价,同时选取具有可比性的研究纳入meta分析,进一步分析比较WS患者人工耳蜗术后言语康复效果与其他非综合征感音神经性聋患者相比是否存在显著差异。结果 共有15篇文献纳入系统评价,其中从CAP,SIR,PEACH,MAIS,MUSS以及PTA等方面分别进行评价,并分别将3篇文献,共计34名WS患者纳入CAP以及SIR的meta分析中,结果提示WS以及对照组之间的差异,无统计学意义。将2篇文献纳入PEACH评分的meta分析,结果提示二者之间的差异不具有统计学意义。结论 Waardenburg综合征在行人工耳蜗植入后可以获得与非综合征性耳聋患者相似的术后康复效果。

Objective To review outcomes of auditory function and speech intelligibility after cochlear implantation in patients with Waardenburg syndrome.Methods Pubmed,Web of Science,EMbase and CNKI,Wanfang database were searched for English and Chinese articles published between 2000 and 2021.A total of 15 articles were included,based on the inclusion and exclusion criteria,for systematic evaluation,of which 5 were also used in meta-analysis to determine any difference in postoperative auditory outcomes between WS and non-syndromic sensorineural hearing loss patients.Meta-analysis was performed using random or fixed effects models according to the heterogeneity of data.Results In the 15 articles included in systematic review,the Categories of Auditory Performance(CAP),Speech Intelligibility Rating(SIR),Parents Evaluation of Aural/Oral Performance of Children(PEACH),Meaningful Auditory Integration Scales(MAIS),Meaningful Use of Speech Scale(MUSS),and pure tone audiometry(PTA) were evaluated separately.Meta-analysis of CAP and SIR involving 34 WS patients from 3 articles suggested no statistically significant difference between WS and the controls.Meta-analysis of PEACH in 2 articles also showed no statistically significant difference.Conclusion Cochlear implantation in Waardenburg syndrome can result in postoperative rehabilitation outcomes similar to that in patients with non-syndromic sensorineural hearing loss.