中文摘要：

目的：评价脑白质发育异常的极重度感音神经性聋患儿人工耳蜗植入术后的听觉言语康复效果与脑白质病变程度的关系。方法：回顾性分析35例行人工耳蜗植入术的脑白质发育异常患儿（实验组）,并选取35例植入年龄匹配、耳蜗正常的极重度感音神经性聋患儿作为对照组。根据患儿术前MRI表现进行脑白质病变评测（Scheltens量表评分）,术后6、12、24个月采用听觉行为（CAP）和言语可懂度（SIR）分级标准对两组患儿的听力及言语恢复水平分别评分。结果：经统计学分析,实验组术后6个月的CAP得分、SIR得分均低于对照组（P〈0.05）,术后12、24个月的CAP得分与对照组无明显差异,SIR得分低于对照组（P〈0.05）。其中Scheltens分级越高的患儿,CAP和SIR得分越低。结论：脑白质发育异常的语前聋患儿人工耳蜗植入术后听觉感知及言语康复效果与脑白质病变程度有关,病变累及范围越广,听觉和言语康复水平越低,言语康复效果受脑白质病变程度影响更为显著。

英文摘要：

Objective：To investigatethe effection of white matter abnormality to auditory and speech rehabili- tation after cochlear implantation in prelingual deafness children. Method： Thirty-five children with white matter abnormality were included in this study. The degree of leukoaraiosis was evaluated by Scheltens scale based on MRI. The hearing and speechrecovery level was rated by auditory behavior grading standards（CAP） and speech in- telligibility grading standards（SIR） at 6 months, 12 months, and 24 months post operation. Result： The CAP scores and SIR scores of the children with white matter abnormality were lower than those of the control group at 6 months after operation （P〈0.05）. The SIR scores of the children with white matter abnormality at 12 months and 24 months post operation were significantly lower than those of the control group. There was no statistically significant difference between the CAP scores of the two groups at 12 and 24 months after operation（P〉0.05）. Schelten classification had a greater impact on SIR scores than on CAP scores. Conclusion：The effect of white mat- ter abnormality on auditory and speech rehabilitation after cochlear implantation was related to the degree of leu- koencephalopathy. When the lesion of white matter abnormality was larger, the level of hearing and verbal reha- bilitation was lower, and the speech rehabilitation was more significantly impacted by white matter lesions degree.