大前庭水管综合征人工耳蜗植入者的听力特点与效果分析

Cochlear Implantation in Patients with LVAS:Audiological Characteristics and Outcomes

目的分析接受人工耳蜗植入的大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患者听力特点以及确诊时间与人工耳蜗植入后听觉言语发育的关系,探讨LVAS早期确诊的临床价值。方法选择2000-2008年间在首都医科大学附属北京同仁医院接受人工耳蜗植入的112例LVAS患者资料并进行分析,依据年龄分别进行婴幼儿日常听觉综合能力量表(the Infant-Toddle Meaningful Auditory Integration Scale,IT-MAIS)或有意义听觉整合量表(Meaningful Auditory Integration Scale,MAIS)以及Nijmegen人工耳蜗植入量表(Nijmegen Cochlear Implantation Questionnaire,NCIQ)效果评估。结果①112例患者发现耳聋的平均年龄为(2.1±1.76)岁,确诊LVAS平均年龄为(7.0±7.34)岁,耳聋发现年龄与LVAS确诊年龄间有较大差距;②术前检查时确诊组50例(44.6%)患者,耳聋发现中位年龄2岁,确诊中位年龄9岁,手术中位年龄9岁;非术前确诊组62例(55.4%)患者,耳聋发现中位年龄为1.5岁,确诊中位年龄为2.3岁,手术中位年龄为4岁;③112例患者均为极重度感音神经性耳聋,术前助听言语交流能力2组间差异无统计学意义(P=0.859);④对18例成人患者术后NCIQ数据分析,术前诊断组和非术前诊断组的术后言语恢复能力差异具有统计学意义(P=0.029)。对16例7岁以下植入者术后听觉能力评估发现术前诊断组和非术前诊断组在开机后2个月内对声音的察觉能力和理解能力有差异但无统计学意义。结论大前庭水管综合征的确诊时间明显滞后于耳聋发现时间;LVAS的早期诊断有助于儿童残余听力的保护并有利于人工耳蜗术后早期听觉言语康复训练。

Objective To analyze the audiological characteristics of the cochlear implanted patients with large vestibular aqueduct syndrome（LVAS） and investigate the effects of the age of final diagnosis of LVAS on the postoperative results and the value of early diagnosis. Methods One hundred and twelve patients with LVAS who received cochlear implants between the year of 2000 and 2008 at the Cochlear Implant Center of Beijing Tongren Hospital were investigated in this study. The audiological results were assessed by using Infant-toddle Meaningful Auditory Integration Scale（IT-MAIS） or Meaningful Auditory Integration Scale（MAIS） questionnaire or Nijmegen Cochlear Implantation Questionnaire（NCIQ） depending on the age of the patients. Results ① The average age of onset of hearing loss（HL） was（2.1±1.76） years old. The average age of final diagnosis of LVAS was（7.0±7.34） years old. There is a significant delay from the onset to final diagnosis of LVAS. ② In the preoperatively diagnosed group（group A） of 50（44.6%） patients,the average age of HL onset was 2.0 years old,and both the average age of final diagnosis of LVAS and the average age of operation were 9.0 years old. In the non-preoperatively diagnosed group（group B） of 62 patients（55.4%）,the average age of HL onset was 1.5 years old,and the average age of final diagnosis of LVAS and the average age of implantation were 2.3 and 4.0 years old,respectively. ③ One hundred and twelve patients suffered from profound sensorineural hearing losses. There was no significant difference in the speech communication ability between group A and B. ④ For 18 adult patients,the scores of NCIQ were significantly different between the group A and B（P=0.029）; For the 16 patients under the age of 7 years old,there was no significant difference in the ability of MAIS questionnaire between the two groups during the first two months following implantation. Conclusion The final diagnosis of LVAS was much delayed from the onset of HL. Early diagnosis of LVAS may contribute to the protection of the residual hearing and benefit for auditory rehabilitation after cochlear implantation.