中文摘要：

目的：探讨蜗轴缺如畸形患者人工耳蜗植入术后电诱发听性脑干反应（EABR）的特点,明确蜗轴缺如畸形患者EABR阈值与耳蜗发育正常且合并极重度感音神经性聋患者之间的差异。方法：回顾性分析2004-09-2015-08期间行人工耳蜗植入的14例蜗轴缺如畸形患者（A组）,并筛选耳蜗发育正常且合并极重度感音神经性聋的14例患者（B组）作为对照。2组患者人工耳蜗植入术后分别选取2、6、11电极行EABR检测,比较EABR引出率及阈值的差异。结果：A组EABR引出率为71.4%,B组EABR引出率为100%。A组患者EABR阈值明显高于B组,差异有统计学意义（P〈0.05）。A组患者3个电极间EABR阈值差异无统计学意义（P〉0.05）;B组患者EABR阈值在2、11电极间差异有统计学意义（P〈0.05）,2电极EABR阈值低于11电极阈值。结论：蜗轴缺如畸形患者EABR阈值明显高于耳蜗发育正常患者,2、6、11电极间EABR阈值差异无统计学意义。EABR作为客观的检测方法,可以应用于蜗轴缺如畸形患者人工耳蜗植入术后的客观听觉效果评价。

英文摘要：

Objective：The purpose of this study was analyzing the characteristic of the electrically evoked auditory brainstem responses（EABR）of cochlear implantation（CI）patients with absence of cochlear modiolus（ACM）and comparing the EABR results in deaf patients with normal anatomic structures.Also we assessed the auditory rehabilitation of ACM patients after cochlear implantation.Method：Twenty-eight profoundly deaf patients were contained,14 of them were ACM（group A）and the rest were normal anatomic structures（group B）.They all received Austria cochlear implants from 2004 to 2015.Postoperative EABR were measured and recorded with No.2,6,11 electrodes in patients receving cochlear implantation.The lead-out rate and threshold of EABR between groups were compared and analyzed.Result：The lead-out rates of EABR in group A was 71.4% and group B was100%.The thresholds of EABR in group A were higher than group B（P〈0.05）.In group A,there were no statistical difference in the thresholds of EABR among electrodes No.2,6and 11.However,in group B,there were statistical difference in the thresholds of EABR between electrodes No.2 and 11（P〈0.05）,and the thresholds of No.2was lower than No.11.Conclusions：EABR could be used in assessing the objective auditory rehabilitation in ACM patients after cochlear implantation.