Ovadia S, Dror I, Zubkov T, Tanay A, Levy D, Zandman-Goddard G
Department of Medicine C, Sackler Faculty of Medicine, Tel-Aviv University, Wolfson Medical Center, Halochamim 62, Holon, Israel, 58100.
Clin Rheumatol. 2009 Jun;28 Suppl 1:S35-8. doi: 10.1007/s10067-009-1119-x. Epub 2009 Feb 19.
Churg-Strauss syndrome (CSS) is a rare multisystem autoimmune disease characterized by diffuse eosinophilic infiltration and necrotizing vasculitis. There are typical manifestations of longstanding rhinosinusitis and polyposis but otological manifestations are rare and characterized by dense aural discharge, granulomatous eosinophilic infiltrate in the mastoid and middle ear with conductive hearing loss, and progressive sensory neural hearing loss--all of which are not responsive to conventional treatment. We describe the case of a 59-year-old man with a rare presentation of CSS that included chronic bilateral otitis media with hearing loss and life-threatening pericardial tamponade. Treatment with pericardiocentesis, prednisone and cyclophosphamide was beneficial and resulted in an improvement of the pericardial and otological manifestations. Early recognition and treatment of otological involvement in CSS is extremely important because of the dramatic response to corticosteroids which may prevent progression of hearing loss.
变应性肉芽肿性血管炎（CSS）是一种罕见的多系统自身免疫性疾病，其特征为弥漫性嗜酸性粒细胞浸润和坏死性血管炎。该病有长期鼻窦炎和鼻息肉的典型表现，但耳部表现罕见，其特征为耳内大量脓性分泌物、乳突和中耳的肉芽肿性嗜酸性粒细胞浸润伴传导性听力损失，以及进行性感觉神经性听力损失——所有这些对传统治疗均无反应。我们报告一例59岁男性，其CSS表现罕见，包括慢性双侧中耳炎伴听力损失及危及生命的心包填塞。心包穿刺、泼尼松和环磷酰胺治疗有效，使心包和耳部表现均得到改善。由于对皮质类固醇有显著反应，早期识别和治疗CSS的耳部受累极为重要，这可能会防止听力损失的进展。
