人工耳蜗植入术治疗重度或极重度耳聋患儿临床价值及与其耳聋基因筛查阳性的相关性分析  

Clinical Value of Cochlear Implantation in the Treatment of Severe or Very Severe Deafness and Its Correlation with Positiveness of Deaf Gene Screening

作　　者：周鲲鹏[1] 刘辉[1] 张学梅[1] 臧雯[1] ZHOU Kunpeng;LIU Hui;ZHANG Xuemei;ZANG Wen(Department of Otorhinolaryngology,the First Hospital of Shijiazhuang,Shijiazhuang 050011,China)

机构地区：[1]石家庄市第一医院耳鼻咽喉科,石家庄050011

出　　处：《解放军预防医学杂志》2018年第11期1448-1450,1468,共4页Journal of Preventive Medicine of Chinese People's Liberation Army

基　　金：河北省重点研发计划项目(No.162777108D)

摘　　要：目的探讨人工耳蜗植入术治疗重度耳聋患儿临床价值及与其耳聋基因筛查阳性的相关性。方法将2016年3月-2017年3月于我院就诊治疗的30例重度或极重度双耳感音神经性耳聋患儿作为研究对象,并对所有患儿临床及随访资料进行回顾性分析,所有患儿均于我院行人工耳蜗植入术治疗,且治疗前均于我院行耳聋基因筛查,治疗后均进行手术效果的评定,根据其筛查和评定结果记录其耳聋基因筛查情况和术后情况,并比较基因不同的患儿间术后效果情况。结果 (1) 30例患儿中基因筛查阳性率为50.00%,其中GJB2突变率最高(53.53%);(2)两组患儿术后不同时间点听觉语言能力、CAP和SIR评分均较术前提高,且随着术后时间的延长,其听觉语言能力、CAP和SIR评分不断提高,比较差异均具有统计学意义(P<0.05);但两组患儿术前及术后不同时间点听觉语言能力、CAP和SIR评分比较均无差异(P>0.05)。结论人工耳蜗植入术可进一步提高感音神经性耳聋患儿听力和语言功能,且耳聋基因筛查有助于减少下一代先天性耳聋的产生,而基因突变对人工耳蜗植入术治疗患者的近期康复效果未存在明显影响。Objective To explore the clinical value of cochlear implantation in the treatment of severe deafness and itscorrelation with the positiveness of deaf gene screening. Methods Thirty children with severe or very severe binaural sensorineuraldeafness treated in our hospital between March 2016 and March 2017 were enrolled in this study. All the clinical data and follow-up were retrospectively analyzed. All these children were treated with cochlear implantation in our hospital, and deafness genescreening was performed in our hospital before treatment. The surgical results were evaluated after treatment. According to the results of screening and evaluation, the results of the deafness gene screening and postoperative conditions were recorded, and thepostoperative effects were compared between these children. Results （1）The positive rate of genetic screening in 30 children was50. 00%, and the mutation rate of GJB2 was the highest （53. 53%）. （2）The auditory language ability, CAP and SIR scores of thetwo groups were improved at different time points after surgery, and continued to increase with time. The difference was of statisticalsignificance （P〈0. 05）. There was no difference in auditory language ability,CAP or SIR scores between the two groups before andafter surgery （P〉0. 05）. Conclusion Artificial cochlear implantation can further improve the hearing and language function of children with sensorineural deafness, and deafness gene screening can help reduce the occurrence of congenital deafness in the nextgeneration. However, gene mutation has no significant effect on the short-term rehabilitation effect of patients treated with cochlearimplantation.

关 键 词：人工耳蜗植入术 重度耳聋 耳聋基因阳性 相关性 

分 类 号：R764.5[医药卫生—耳鼻咽喉科]