Murata Junko, Horii Arata, Tamura Manabu, Mitani Kenji, Mizuki Masao, Kubo Takeshi
Department of Otolaryngology, Osaka University School of Medicine, Osaka, Japan.
Acta Otolaryngol. 2006 May;126(5):548-52. doi: 10.1080/00016480500437369.
Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant -SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP -- including the inner ear dysfunction -- were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.
复发性多软骨炎（RP）的特征是软骨炎症及随后的退变。我们报告一名61岁患有RP并伴有听-前庭表现的女性。她还被诊断为伴有髓样化生的骨髓纤维化（MMM）。通过耳蜗电图（ECochG）的优势-SP/AP证实存在双侧内淋巴积水（EH）。当使用沙利度胺和泼尼松龙治疗MMM时，RP的症状——包括内耳功能障碍——得到改善。异山梨醇是日本常用于治疗梅尼埃病（MD）的渗透性利尿剂之一，对防止她出现内耳功能障碍也有效。这是首次证实伴有听-前庭表现的RP患者存在EH的报告。我们推测，MMM导致的免疫失衡，结合特定的免疫遗传背景，可能在该患者RP的发病机制及EH的形成中发挥了作用。
