不同致聋基因突变的患儿人工耳蜗植入术后听觉言语康复效果观察  

Effects of cochlear implantation on hearing and speech ability of children with different deafness gene mutations

作　　者：李兴程 张金慧[1] 陈蓓[1] LI Xingcheng;ZHANG Jinhui;CHEN Bei(Department of Otology,Otorhinolaryngology Hospital,the First Affiliated Hospital,Zhengzhou University,Zhengzhou 450052)

机构地区：[1]郑州大学第一附属医院耳鼻咽喉医院耳科,郑州450052

出　　处：《郑州大学学报（医学版）》2022年第1期111-115,共5页Journal of Zhengzhou University(Medical Sciences)

基　　金：国家自然科学基金项目(81700905)。

摘　　要：目的:观察主要常见致聋基因突变患儿人工耳蜗植入术后听觉言语康复效果。方法:回顾性分析郑州大学第一附属医院耳鼻咽喉医院耳科65例行人工耳蜗植入术患儿的临床资料,检测其主要遗传性致聋基因突变位点,采用全序列分析杂合突变的相关基因。术后6个月,通过声场扬声器对患儿术后助听听阈进行检测;采用婴幼儿有意义听觉整合量表、听觉行为分级标准和言语可懂度分级标准评价患儿术后12个月听觉和言语能力的康复效果。结果:65例患儿中,SLC26A4基因突变7例(A组),GJB2基因突变13例(B组),线粒体DNA 12SrRNA基因突变5例(C组),未发现上述基因突变40例(D组)。各组术后助听听阈值的比较差异无统计学意义(P>0.05)。不同致聋基因突变的患儿术后12个月听觉和言语能力较术前均有所提升(P<0.05);与D组相比,B组术后12个月听觉整合量表(IT-MAIS)、听觉行为分级标准(CAP)分级、言语可懂度分级标准(SIR)分级评分均提高(P<0.05)。A组和C组IT-MAIS、CAP分级、SIR分级与D组比较,差异无统计学意义(P>0.05)。结论:主要常见致聋基因突变患儿人工耳蜗植入术后听觉和言语能力在一定程度上有所提升,其中GJB2基因突变患儿术后听觉和言语能力的康复效果相对较好。Aim:To observe the effects of cochlear implantation on hearing and speech ability of children with common deafness gene mutation.Methods:The clinical data of 65 children undergoing cochlear implantation in our hospital were retrospectively analyzed,and the main genetic deafness gene mutation sites were detected.Six months after the operation,the hearing threshold was detected by sound field loudspeaker.The rehabilitation effect of hearing and speech ability 12 months after surgery was evaluated by infant-toddler meaningful auditory integration scale(IT-MAIS),categories of auditory performance(CAP)and speech intelligibility rating(SIR).Results:There were 7 cases with SLC26A4 gene mutation(group A),13 cases with GJB2 gene mutation(group B),5 cases with mtDNA 12SrRNA gene mutation(group C),and 40 cases without the above gene mutation(group D).There was no significant difference in the hearing threshold value of each group(P>0.05).The hearing and speech ability of children with different deafness gene mutations were significantly improved 12 months after operation(P<0.05).Compared with group D,the scores of IT-MAIS,CAP and SIR in group B were significantly improved 12 months after operation(P<0.05).There were no significant differences in IT-MAIS,CAP or SIR between group A and group C and group D(P>0.05).Conclusion:The hearing and speech ability of children with common deafness gene mutation after cochlear implantation are improved to a certain extent,and the rehabilitation effect of hearing and speech ability of children with GJB2 gene mutation after cochlear implantation is quite good.

关 键 词：人工耳蜗植入术 耳聋 基因突变 听觉 言语能力 

分 类 号：R764[医药卫生—耳鼻咽喉科]