Objective To study the auditory perception and speech ability outcomes after cochlear implantation in prelingually deaf children with extremely severe neurosensory hearing loss and comorbid leukoencephalopathy.Methods Our study included 14 prelingually deaf children with leukoencephalopathy (confirmed by preoperative MRI scanning) who were treated with cochlear implantation in the Department of Otorhinolaryngology Head and Neck Surgery of Hainan Provincial People''s Hospital, including 8 males and 6 females of 1~6 yr with a mean age of 3.8 yr.Sixteen synchronous prelingually deaf children without central nervous system (CNS) diseases were also included as the control group, including 11 males and 5 females of 1~6 yr with a mean age of 4.4 yr.All the patients underwent pre-surgical assessments of audiology, radiology, speech ability and intelligence before cochlear implantation via transmastoid facial nerve recess approach was done, after which rehabilitation was provided at the Hainan Rehabilitation Center for Deaf Children.Categories of auditory performance (CAP) and speech intelligibility rate (SIR) were employed as the assessment criteria for the outcome of surgery, the scores of which at different timepoints after surgery were statistically compared through paired t-test between the two groups.Results All the patients gained post-surgical hearing and speech abilities to different degrees, whereas CAP and SIR scores both chronologically improved in the both groups.No statistical difference was revealed either in CAP or in SIR scores between the two groups at 6, 12 and 24 months after surgery (P>0.05).Conclusion Children with extremely severe neurosensory hearing loss can be reated with cochlear implantation even if they have comorbid leukoencephalopathy.The effects of auditory and speech rehabilitation for the same age patients with leukoencephalopathy are similar to those without leukoencephalopathy in 2 years after surgery.%目的 探讨伴脑白质病的语前极重度感音神经性聋患儿人工耳蜗植入的可行性及术后听觉言语康复效果.方法 海南省人民医院耳鼻咽喉科2013年9~11月行人工耳蜗植入的14例伴脑白质病语前聋患儿为研究组(年龄1~6岁,平均3.79±1.93岁),同期无中枢神经系统病变的语前聋患儿16例为对照组(年龄1~6岁,平均4.38±1.93岁),术前均行临床听力学、影像学检查及语言能力、智力水平等评估,经乳突后鼓室面神经隐窝入路一期行人工耳蜗植入术,术后均到海南省聋儿康复中心进行言语康复训练;采用听觉行为分级标准(categories of auditory performance,CAP)和言语可懂度分级标准(speech intelligibility rate,SIR)对两组术后康复效果进行评估,比较两组患儿术后不同阶段的CAP和SIR分级.结果 所有患儿人工耳蜗植入术后均获得听觉反应和不同程度的言语交流能力,随着康复时间延长两组患儿CAP及SIR分级均呈上升趋势;研究组术后6、12和24个月CAP平均分级分别为2.571±0.416、3.714±0.496、5.000±0.492级,SIR平均分级分别为1.357±0.133、2.143±0.275、3.071±0.245级,与对照组CAP(分别为2.688±0.313、3.875±0.364、5.000±0.354级)及SIR(分别为1.500±0.129、2.313±0.176、2.875±0.221级)比较,差异均无统计学意义(P>0.05).结论 伴脑白质病的语前极重度感音性聋患儿经过术前充分评估后可以实施人工耳蜗植入,术后2年内听觉言语康复效果与不伴脑白质病的同龄患儿相当.
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