目的 探讨电刺激听觉脑干诱发电位(electrical evoked auditory brainstem responses,EABR)辅助Mondini畸形患者人工耳蜗植入手术的方法及术后效果分析.方法 我院自2001年开始为Mondini畸形患者植入人工耳蜗,收集2012年至2015年在我院行人工耳蜗手术的Mondini畸形患者31例,回顾性分析Mondini畸形患者术前听力学特征,术前EABR特点,术中手术特点、术后NRT测试结果及开机调试结果,随访术后听觉言语康复能力CAP(categories of auditory performance)和SIR(speech intelligibility rating)评分.另选择相似耳聋年龄的耳蜗结构正常耳蜗植入患者20例作为正常对照组,测试结果进行对比分析.结果31例Mondini畸形患者术前听力均为极重度感音神经性聋,其中14例无残余听力,对照组7例无残余听力,术前行EABR测试,引出率100%,有反应者植入不同类型电极的人工耳蜗;术中电极全部植入耳蜗内,其中4例出现脑脊液井喷,颞肌封堵,术后无面瘫、脑脊液漏、脑膜炎等手术并发症;所有Mondini畸形患者术后均获得听觉,开机测试神经反射遥测(neural response telemetry,NRT),引出率41.94%低于对照组95%;开机6月平均T值Mondini组171.37±13.14CL;对照组145.34±17.43CL;动态范围Mondini组33.24±10.73CL,对照组43.55±15.36CL,经统计学分析P<0.05,有显著差异;随访6月~4年,Mondini组CAP平均得分6.13±1.15,SIR平均得分3.05±0.62;对照组CAP平均得分7.12±0.80,SIR平均得分4.03±0.91,经两样本比较t检验,P<0.05有显著性差异.结论 Mondini畸形的双侧极重度感音神经性聋患者可以根据残余听力情况在EABR辅助下行人工耳蜗植入,术后听力言语康复较正常耳蜗结构患者效果稍差,但明显好于术前听觉和言语能力.%Objective To investigate the hearing and speech rehabilitation outcomes in the cases with Mondi-ni malformation underwent cochlear implantation with preoperative evaluation using electrically evoked auditory brainstem responses (EABR). Methods Thirty-one cases with Mondini malformation underwent cochlear implanta-tion in our hospital from the year 2012 to 2015. The audiologic testing, preoperative EABR, surgical technique, and postoperative neural response telemetry (NRT) results in these cases were analyzed retrospectively. The hearing and speech rehabilitation ability were evaluated using categories of auditory performance (CAP) and speech intelligibili-ty rating (SIR) scores. Another 20 cases of cochlear implantation with normal cochlea served as the normal control group. Results Thirty-one cases with Mondini malformation presented with profound sensorineural hearing loss, 14 cases of which showing no residual hearing. Seven cases in control group showed no residual hearing. All cases demonstrated presence of EABR response preoperatively. The patients with no residual hearing were implanted with different types of electrodes. Four cases had cerebrospinal fluid (CSF) gusher during procedure, which was controlled by plugging with temporaries muscle, and no postoperative complications occurred. All Mondini malfor-mation patients gained hearing postoperatively. The inducible rate of NRT on the boot day was 41.94%which was lower than that in the control group (which was 95%). About 6 month postoperatively the average T value was 171.37 ± 13.14CL in Mondini group versus 145.34 ± 17.43CL in control group (P<0.05); the dynamic range was 33.24 ± 10.73CL in Mondini group versus 43.55 ± 15.36CL in control group (P<0.05). Postoperative follow-up from 6 months to 4 years show that the Mondini group has the average CAP scores of 6.13±1.15 (7.12±0.80 in con-trol) and SIR scores of 3.05±0.62 (4.03±0.91 in control),with significant difference (P<0.05) compared to the con-trol group. Conclusions Cochlea implantation can be performed in patients with bilateral profound hearing loss in Mondini malformation, and even for those without residual hearing, if preoperative EABR is present. Significantly improved hearing and speech were achieved, though the postoperative hearing and speech rehabilitation outcomes were poorer in Mondini malformation than that with normal cochlear structure.
展开▼
