背景：要报告演示文稿，诊断过程,诊断为Bjornstad综合征伴深度感音神经性听力损失（SNHL）的患者人工耳蜗植入的治疗和结果。
■回顾性报道，两名患有Bjornstad综合征的兄弟姐妹患有严重的SNHL，对接受双侧同步人工耳蜗植入治疗的常规助听器无反应。
方法：三级转诊中心。
结果：对患有严重SNHL和双侧内耳异常（不完全分区类型1）的两个兄弟姐妹（四耳）进行人工耳蜗植入手术，无并发症。术后听力测量显示纯音阈值和单词识别得分显着提高。在文献综述中,以前没有报道过人工耳蜗植入治疗的Bjornstad综合征病例.
结论：人工耳蜗植入是一种有效的，安全,和Bjornstad综合征的最终治疗选择，严重的SNHL对助听器无反应。
BACKGROUND: To report the presentation, diagnostic process, management and results of cochlear implantation of patients diagnosed with Bjornstad syndrome with profound sensorineural hearing loss (SNHL).
UNASSIGNED: A retrospective report of two siblings with Bjornstad syndrome suffering profound SNHL unresponsive to conventional hearing aids treated with bilateral simultaneous cochlear implantation.
METHODS: Tertiary-referral center.
RESULTS: Cochlear implant surgeries of two siblings (four ears) with profound SNHL and bilateral inner ear anomaly (incomplete partition type 1) were performed without complications. Postoperative audiometric measurements showed a significiant improvement in pure-tone threshold and a word recognition score. In the literature review, no previous case of Bjornstad syndrome treated with cochlear implantation has been reported.
CONCLUSIONS: Cochlear implantation is an effective, safe, and ultimate treatment option for Bjornstad syndrome with profound SNHL not responding to hearing aids.
