共同腔畸形患儿缝状开窗定制电极耳蜗植入术及其效果评估

Transmastoidslotted labyrinthotomy approach cochlear implantation with customized electrode for patients with common cavity deformity

目的采用缝状开窗经迷路入路对内耳共同腔畸形患儿进行定制电极人工耳蜗植入术并评估术后效果。方法回顾性分析2016年4月至2017年12月就诊于首都医科大学附属北京同仁医院进行人工耳蜗植入术的10例共同腔畸形的双耳重度或极重度感音神经性聋患儿临床资料(采用缝状开窗经迷路入路及定制电极),作为试验组,进行术中监测,并随访其术后效果;同时收集10例年龄及性别匹配的内耳结构正常的双耳重度或极重度感音神经性聋患儿作为对照组,收集两组患儿进行术前及开机6个月、1年的四种问卷评分,不同评估时间点间的比较采用Wilcoxon符号秩和检验,试验组与对照组的比较采用两独立样本的秩和检验。结果所有患儿手术过程顺利,试验组患儿均未出现面瘫、脑脊液耳漏等并发症;试验组有8例患儿的平均阻抗在5kΩ以下;试验组患儿术中所有刺激电极的听觉神经反应遥测(ART)总引出率为55%(33/60),不同位置电极的电诱发复合动作电位(ECAP)反应幅值范围50.69~170.3μV;试验组患儿的手术平均时间46.4 min,与传统面隐窝入路植入非定制电极相比较短。试验组患儿的四项问卷评分在术前与开机6个月、开机1年比较,差异均有统计学意义;开机6个月与开机1年评分比较:听觉行为分级标准量表(CAP):2.0(2.0,3.0)分比4.0(3.8,4.0)分,Z=-3.109,P=0.002;言语可懂度分级标准量表(SIR):2.0(2.0,2.3)分比3.0(2.8,4.0)分,Z=-2.952,P=0.003;有意义使用言语量表(MUSS):4.0(3.3,6.0)分比9.0(6.0,11.8)分,Z=-3.421,P=0.001;有意义听觉整合量表或婴幼儿有意义听觉整合量表(MAIS或IT-MAIS):5.5(3.8,9.0)分比15.5(10.8,18.5)分,Z=-3.522,P=0.000)。开机6个月和1年后,试验组四项问卷评分均差于对照组(P值均<0.01);术后1周及术后1年高分辨率颞骨CT显示电极贴壁良好,无移位。结论采用缝状开窗经迷路入路对共同腔畸形患儿进行人工耳蜗植入术安全有效,术中操作难度较低,并发症发生率低,手术时间短;可用术中ART监测辅助判断听觉通路完整性,共同腔畸形患儿术后听觉康复周期较长,言语康复效果个体差异大,明显差于结构正常患儿。

Objective To discuss the benefit of using transmastoid slotted labyrinthotomy approach (TSLA) and customized electrode for common cavity deformity (CCD) patients, and to evaluate the audiological outcomes. Methods A retrospective analysis of the intraoperative monitoring data and postoperative auditory outcomes of 10 CCD cases who received cochlear implantation in Beijing Tongren Hospital,Capital Medical University from April 2016 to December 2017, was conducted using TSLA and customized electrod as a test group.At the same time, 10 cases of age and gender matched children with severe or severe sensorineural hearing loss and normal inner ear structures were recorded as a control group. Four questionnaires were collected from the two groupspre-operative, 6 months and 1 year after start-up. The Wilcoxon signed rank sum test was used to compare the scores of different time points. Rank sum test of two independent samples was used to compare the scores between different groups. Results None of the patients exhibited CSF leakage or facial paralysis after operation. The average impedance of 8 CCD children in the experimental group was below 5 kΩ. The total extraction rate of auditory neural response telemetry (ART) of all stimulating electrodes was 55%(33/60), and the amplitude of the electricity induced complex action potential (ECAP) of electrodes at different positions ranged from 50.69 to 170.3 μV.The average surgical time of the TSLA group was 46.4 min, shorter than the traditional approach.There was significant difference between the scores of pre-operative, 6 months and 1 year after start-up for the TSLA group(categories of auditory performance,CAP: 2.0 (2.0,3.0) vs. 4.0 (3.8,4.0), Z=-3.109, P=0.002;speech intelligibility rating,SIR: 2.0 (2.0,2.3) vs. 3.0 (2.8,4.0), Z=-2.952, P=0.003;meaningful use of speech scale,MUSS: 4.0 (3.3,6.0) vs. 9.0 (6.0,11.8), Z=-3.421, P=0.001;meaningful auditory integration scale, MAIS or infant-toddler and meaningful auditory integration scale, IT-MAIS: 5.5 (3.8,9.0) vs. 15.5 (10.8,18.5), Z=-3.522, P=0.000 for the latter two).In addition, The scores of the TSLA group were significantly worse than the control group at 6 months and 1 year after start-up.The high-resolution CT scan showed good adhesion of the electrodes without displacementone week and one year after surgery. Conclusions For CCD patients, TSLA and customized electrode is recommended due to lower surgical difficulty and post-operative risk, shortened surgical time;Intraoperative ART monitoring can be used to determine the integrity of the auditory pathway. Children with common cavity deformity have a longer period of auditory rehabilitation, and the individual differences in speech rehabilitation are significantly different, which is significantly worse than those with normal structure.