中华耳科学杂志Issue(2)：163-167,5.
GJB2及SLC26A4基因突变患儿耳蜗植入效果分析
Treatment outcomes of cochlear implantation in children with GJB2 and SLC26A4 mutations
摘要
Abstract
Objective To report treatment outcomes in pediatric cochlear implant (CI) patients with mutations in GJB2 or SLC26A4. Methods The case series included 40 children who received unilateral cochlear implants. Fifteen of these children had GJB2-related deafness, 8 had SLC26A4-related deafness, and 17 had neither GJB2 nor SLC26A4 mutations. Evaluation included Meaningful Auditory Integration Scale (MAIS), Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR) and Babbling Spurt, and was conducted before and after implantation (up to 1 year). Results The MAIS and CAP performance was better in the GJB2 group than other two groups (P< 0.05). A trend towards better performance for the GJB2 group (vs. The other groups combined) was also observed regarding SIR and the time of Babbling spurt, although statistically insigni?cant. The SLC26A4 group performed better than no-mutation group, although again without statistically significant difference. Conclusion The results of the study seem to support a better performance in children with GJB2-related deafness after cochlear implant, possibly due to the cochlear origin of the defect. The performance of SLC26A 4 patients needs to be further confirmed关键词
GJB2/SL C26A4/耳聋/耳蜗植入/术后效果Key words
GJB2 gene/SLC26A4 gene/Deafness/Cochlear implantation(CI)/Outcome分类
医药卫生引用本文复制引用
颜玉君,李蕴,杨涛,黄琦,吴皓..GJB2及SLC26A4基因突变患儿耳蜗植入效果分析[J].中华耳科学杂志,2011,(2):163-167,5.基金项目
上海市科委重大项目(No.08DZ1980100) （No.08DZ1980100）
