目的：探讨脑白质病对先天性耳聋患儿人工耳蜗术后听觉言语恢复的影响，为聋儿提供术前评价及术后恢复情况的评估。方法回顾性分析86例行人工耳蜗植入术的先天性耳聋患儿，年龄10月～6岁。术前行头颅常规 MRI 和内耳水成像，根据常规 MRI 表现分为脑白质病组和无脑白质病组，观察两组内耳畸形情况。术后半年采用听觉行为分级标准（CAP）和言语可懂度分级标准（SIR）对两组患儿的听力及言语恢复水平分别评分，并进行统计学分析比较。结果86例患者中22例存在脑白质病变，其中2例伴有内耳畸形；64例无脑白质病变，其中26例伴有内耳畸形。术后半年，脑白质病组 CAP 和 SIR 评分分别为（3．95±1．00）和（2．05±0．72），无脑白质病组则分别为（5．72±1．31）和（3．08±0．74），两组间差异显著。结论脑白质病是先天性耳聋患儿人工耳蜗植入后听觉感知及言语效果恢复的不利因素。%Objective To investigate the influence of leukoncephalopathy on the auditory and speech rehabilitation in patients with con-genital hearing loss after cochlear implantation,so as to define preoperative assessment and postoperative follow-up.Methods Eighty-six patients with congenital hearing loss performed with brain conventional MR imaging and inner ear hydrography before cochlear im-plantation,ages form ten months to six years.The patients were assigned into leukoncephalopathy group and non-leukoncephalopathy group according to the appearance on conventional MR images,and the malformation of inner ear was recorded.The hearing and speech recovery level was rated by using auditory behavior grading standards (CAP)and speech intelligibility grading standards (SIR)postop-eration half a year,which were compared between two groups.Results Twenty-two patients had leukoncephalopathy,among whom two cases had inner ear malformation.Sixty-four patients hadn’t leukoncephalopathy,among whom twenty-six cases had inner ear malforma-tion.Half a year after operation,the CAP and SIR scores were (3.95 ±1.00)and(2.05 ±0.72)in leukoncephalopathy group,(5.72 ±1.31)and(3.08 ±0.74)in non-leukoncephalopathy group,respectively.The difference was statistically significant between two groups.Conclusions Leukoncephalophathy is an adverse factor to auditory and speech rehabilitation in patients with congenital hear-ing loss after cochlear implantation.
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