Abstract：
A primary intracochlear schwannoma (ICS) is a unique type of vestibular schwannoma (VS); the tumor originates from the terminal branches of the cochlear nerve and is confined to the cochlea. An ICS is the most common subtype of schwannoma in the inner ear. As an ICS is clinically rare, diagnosis and treatment remain challenging. We report a rare case of cochlear implantation (CI) in a patient with neurofibromatosis type 2 and an ICS. The patient exhibited bilateral, profound, sensorineural hearing loss. The tumor on one side was a common VS treated via tumor and acoustic nerve resection and that on the other side an ICS. To ensure auditory rehabilitation via CI, we performed CI while removing part of the ICS via an enlarged round window. Auditory rehabilitation was satisfactory. Thus, ICS patients, especially those who urgently require auditory rehabilitation, can undergo simultaneous CI and (total or partial) tumor removal. However, the long-term results require close observation.
摘要：
原发性耳蜗内神经鞘瘤（ICS）是前庭神经鞘瘤（VS）的一种独特类型；肿瘤起源于耳蜗神经的末端分支，仅限于耳蜗。ICS是内耳中最常见的神经鞘瘤亚型。由于ICS在临床上很罕见，诊断和治疗仍然具有挑战性。我们报告了2型神经纤维瘤病和ICS患者的耳蜗植入（CI）的罕见病例。患者表现为双侧，深刻的,感觉神经性听力损失.一侧的肿瘤是通过肿瘤和听神经切除术治疗的常见VS，另一侧的肿瘤是ICS。为了确保通过听觉康复，我们执行CI，同时通过扩大的圆窗去除部分ICS。听觉康复令人满意。因此，ICS患者，尤其是那些迫切需要听觉康复的人，可以同时进行CI和（全部或部分）肿瘤切除。然而,长期结果需要密切观察。
