PDF(Pubmed)
Abstract：
Relapsing polychondritis is an autoimmune disorder causing inflammation of cartilaginous structures, sensory epithelium, and cardiovascular system. Hearing loss is a rare and dreadful complication of this pathology. We report a case of relapsing polychondritis in a 38-year-old female who developed gradually progressive bilateral profound hearing loss. She did not have any improvement with medical management. Cochlear implantation was performed to rehabilitate her hearing. As the scala tympani was obliterated, a scala vestibuli insertion was performed. A complete insertion was possible with a compressed electrode, and she had good evoked compound action potential scores. Her categories of auditory performance scores were 6 at the end of one year. Patients with relapsing polychondritis can progress to profound hearing loss in rare cases and should be carefully followed up to identify early labyrinthine ossification. A scala vestibuli insertion can be performed with good outcomes in cases with ossification involving scala tympani. The surgeon should be ready for a middle-turn cochleostomy or a drill-out procedure in patients with advanced ossification.
摘要：
复发性多软骨炎是一种引起软骨结构炎症的自身免疫性疾病，感觉上皮,和心血管系统。听力损失是这种病理的一种罕见且可怕的并发症。我们报告了一名38岁女性复发性多软骨炎的病例，该女性逐渐发展为双侧深度听力损失。她在医疗管理方面没有任何改善。进行人工耳蜗植入以恢复她的听力。当鼓阶被切除时，进行了前庭骨的插入。使用压缩电极可以完全插入，她有很好的诱发复合动作电位分数。在一年结束时，她的听觉成绩类别为6。在极少数情况下，复发性多软骨炎患者可发展为严重的听力损失，应仔细随访以识别早期迷路骨化。在涉及鼓室骨骨化的情况下，前庭骨插入可以取得良好的效果。对于晚期骨化患者，外科医生应准备进行中转耳蜗造口术或钻孔手术。
