Waardenburg综合征患者人工耳蜗植入后效果分析

The Outcomes Measures of the Cochlear Implantation in Patients with Waardenburg Syndrome

目的评价伴有Waardenburg综合征患者人工耳蜗植入术后的效果。方法用青少年儿童及婴幼儿听说能力评估问卷家长观察记录表(parients′evaluation of aural/oral performance of children,PEACH)对16例在北京协和医院行人工耳蜗植入术的Waardenburg综合征患者进行术后效果调查,并与非综合征且无明显病因的人工耳蜗植入患者比较。结果经统计学分析,Waardenburg综合征患者组PEACH安静下得分、噪声下得分、总得分以及电话交流能力与对照组相应得分比较,差异均无统计学意义(P>0.05),两组患者安静下得分明显高于噪声下得分(P<0.05)。结论伴有重度或极重度感音神经性聋的Waardenburg综合征患者人工耳蜗植入术后的听觉感知及言语效果与正常耳蜗的重度或极重度感音神经性聋患者相同,安静下听觉感知及言语效果好于噪声下听觉感知及言语效果。

Objective To measure the outcomes of the cochlear implantation for patients with Warrensburg syndrome in comparison with non-syndromic patients with sensorineural hearing loss.Methods 16 of 1 300 Waardenburg syndrome patients who received cochlear implants at PUMC hospital were reviewed in this papers.The authors will discuss the effects between Warrensburg syndrome and non-syndromic patients with sensorineural hearing loss by means of the Parents Evaluation of Aural-Oral Performance of Children（PEACH）.Results There were no statistical difference in quiet,in noise,in overall results and in conversation on the phone between Warrensburg syndrome and non-syndromic patients.There was statistical difference between in quiet and in noise in every group.Conclusion The auditory perception and speech recognition between Warrensburg syndrome and non-syndromic patients with sensorineural hearing loss is same,and the results in quiet is better than that in noise.