不完全分隔Ⅲ型耳蜗畸形患儿行人工耳蜗植入的临床特点及早期效果分析

Cochlear implantation and early outcomes in children with incompletely partition type Ⅲ malformation

目的:分析伴有不完全分隔Ⅲ型(IP-Ⅲ)耳蜗畸形的患儿人工耳蜗植入手术的特点及其术后早期效果。方法:回顾性分析10例IP-Ⅲ患儿术前听力学特征及言语水平,术中手术特点,术后耳蜗助听听阈,术后听觉言语康复能力CAP及SIR评分及术后言语识别率结果。另外选取10例耳蜗发育正常的人工耳蜗植入患儿作为对照,对照组在术前残余听力及言语发育水平、植入年龄、耳蜗使用品牌、耳蜗使用时间等与IP-Ⅲ组相匹配。采用SPSS 20.0软件分析IP-Ⅲ组及对照组人工耳蜗植入的术后效果。结果:所有患儿术前CT显示耳蜗呈特殊IP-Ⅲ型表现,术中均发生较为严重的脑脊液涌出("井喷"),电极均位于耳蜗内且完全植入。术后无并发症发生。术后3、6、9、12个月,IP-Ⅲ组耳蜗助听听阈分别为(40.8±8.5)dB HL、(36.1±9.1)dB HL、(32.5±6.8)dB HL、(33.0±7.3)dB HL,与对照组相比差异无统计学意义(P>0.05);术后3、6、9、12个月,IP-Ⅲ组CAP评分低于对照组,但差异无统计学意义(P>0.05);IP-Ⅲ组SIR评分低于对照组,且在术后6、9、12个月差异有统计学意义(P<0.05)。结论:人工耳蜗植入是IP-Ⅲ耳蜗畸形患儿重建听力的有效办法。该类畸形患儿行人工耳蜗植入具有一定难度,但处理得当一般不会引发严重并发症。人工耳蜗对IP-Ⅲ耳蜗畸形患儿有效,但术后听觉言语能力发展速度较耳蜗发育正常患儿缓慢。

Objective：To analyze the early effect of the cochlear implantation（CI）in children with cochlear incompletely partition type Ⅲ malformation（IP-Ⅲ）.Method：Ten children with IP-Ⅲ malformation who underwent CI were recruited in this study.The hearing characteristics,preoperative speech performance and surgery were analyzed retrospectively.The aided hearing threshold with CI,the categories of auditory performance（CAP）score,speech intelligibility rating（SIR）score and speech perception were designed to access the benefits of CI.Ten children with normal cochlea were also enrolled as the control group.Demographic information of children in the control group including hearing loss and speech level before implantation,age at implantation,hearing aids using history,duration with CI were matched with those in the IP-Ⅲ group.The hearing threshold,CAP score and SIR score in the IP-Ⅲ group were compared with the control group using the SPSS 20.0 software.Result：The computed tomography of temporal bones showed typical IP-Ⅲ malformation in all patients.The electrode arrays were properly and totally implanted in all children.Cerebrospinal fluid gusher occurred intra-operatively,and no other complications in all patients.The pure tone average（PTA）threshold at the 3 rd,6 th,9 th and 12 th month after implantation were（40.8±8.5）dB HL,（36.1±9.1）dB HL,（32.5±6.8）dB HL and（33.0±7.3）dB HL,respectively.The PTA thresholds in the IP-Ⅲ group were similar to those in the control group at all tested time points（P0.05）.At the 3 rd,6 th,9 th and 12 th month after implantation,the CAP scores in the IP-Ⅲ group were lower than those in the control group,but there was no significantly difference（P0.05）.Furthermore,the SIR scores were lower than those in the control group,and there were significantly difference at the 6 th,9 th and 12 th month after implantation（P0.05）.Conclusion：CI was an effective treatment for children with IP-Ⅲmalformation.Surgery on IP-Ⅲ was challenging,however,seldom complication would occur with excellent surgical skills.Though the CI was benefit for IP-Ⅲ,the development of hearing and speech ability were slower than children with normal cochlea.