GJB2相关非综合征性感音神经性聋人工耳蜗植入后的疗效观察

Assessment of the curative effective of cochlear implantation in childer with GJB2-associated NSSNHL

目的:评价携带GJB2基因突变的儿童人工耳蜗植入后的疗效。方法:对40例携带GJB2基因突变的人工耳蜗植入患儿进行疗效评估,测试内容包括:声场助听听阈测试、(婴幼儿)有意义听觉整合量表、听觉行为分级标准评分、言语可懂度分级标准评分及普通话早期言语感知测试。以80例未检出已知热点突变者为对照组。结果:携带GJB2基因突变的患儿术后声场助听听阈优于对照组,其余测试与对照组比较差异无统计学意义(P>0.05)。结论:携带GJB2基因突变的非综合征性感音神经性聋患儿适于行人工耳蜗植入手术,术后效果满意。

Objective：To analyze the curative effect of CI in children with GJB2-associated NSSNHL.Method：The evaluations of curative effect with CI include auditory threshold,IT-MAIS/MAIS,CAP,SIR,MESP.The outcomes of 40cases with GJB2-associated NSSNHL were compared 80patients with negative results of screening of gene mutation（control group）.Result：In comparison with control group,the auditory threshold in children with GJB2-associated NSSNH is better,however had no significant difference in other tests（P〉0.05）.Conclusion：CI could be performed on children with GJB2-associated NSSNHL.Postoperative outcomes of hearing and speech were satisfied.