Abstract：
Rudimentary otocyst (RO) is characterized by an otic capsule without an internal auditory canal, which is considered a contraindication to cochlear implantation (CI). In this study, we were the first to report two patients with ROs who underwent CI.
Two patients (18 months old and 2 years old) presenting with bilateral congenital hearing loss were diagnosed with ROs.
CI was performed. The transmastoid slotted labyrinthotomy approach was used with customized MED-EL electrode arrays.
Categorical auditory performance, infant-toddler meaningful auditory integration of sound, the speech intelligibility rating, and meaningful use of speech scale.
Both children could understand common phrases and had intelligible, connected speech 2 years after CI.
With proper indication, surgical approach and postoperative training, a child with an RO may benefit from CI.
Two patients (18 months old and 2 years old) presenting with bilateral congenital hearing loss were diagnosed with ROs.
CI was performed. The transmastoid slotted labyrinthotomy approach was used with customized MED-EL electrode arrays.
Categorical auditory performance, infant-toddler meaningful auditory integration of sound, the speech intelligibility rating, and meaningful use of speech scale.
Both children could understand common phrases and had intelligible, connected speech 2 years after CI.
With proper indication, surgical approach and postoperative training, a child with an RO may benefit from CI.
摘要：
目的：先天性耳囊（RO）的特征是没有内耳道的耳囊，这被认为是耳蜗植入（CI）的禁忌症。在这项研究中,我们是第一个报告两名患有CI的RO患者。
方法：2例（18个月和2岁）双侧先天性耳聋患者被诊断为ROs。
方法：进行CI。经乳突开槽迷宫切开术与定制的MED-EL电极阵列一起使用。
方法：分类听觉表现，婴儿-幼儿有意义的声音听觉整合，语音清晰度等级，和有意义的使用语音量表。
结果：两个孩子都能听懂常用短语，CI后2年的连接语音。
结论：有适当的适应症，手术方法和术后训练，有RO的孩子可能会从CI中受益。
方法：2例（18个月和2岁）双侧先天性耳聋患者被诊断为ROs。
方法：进行CI。经乳突开槽迷宫切开术与定制的MED-EL电极阵列一起使用。
方法：分类听觉表现，婴儿-幼儿有意义的声音听觉整合，语音清晰度等级，和有意义的使用语音量表。
结果：两个孩子都能听懂常用短语，CI后2年的连接语音。
结论：有适当的适应症，手术方法和术后训练，有RO的孩子可能会从CI中受益。
