Ferri Emanuele, Armato Enrico, Capuzzo Paolo, Cavaleri Stefano, Ianniello Ferdinando
Department of Otorhinolaryngology, General Hospital of Dolo, Riviera XXIX Aprile, 2, 30031 DOLO, Venice, Italy.
Auris Nasus Larynx. 2007 Sep;34(3):379-82. doi: 10.1016/j.anl.2007.01.005. Epub 2007 Mar 9.
Wegener's granulomatosis (WG) is a multi-system disease characterized by granuloma formation and widespread necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory tracts and renal system. Facial nerve palsy and otologic manifestations have been reported during the course of the disease but it is extremely rare as the presenting features. In Literature only one case of bilateral facial palsy as presenting sign of WG is reported. The testing of anticytoplasmic antibodies versus neutrophil polymorphonucleate granules (c-ANCA) are highly specific for the diagnosis of WG, being positive in 97% of the cases. The early diagnosis and the timely medical treatment lead to high rates of remission of an otherwise lethal disease. A case of WG presenting with bilateral facial paralysis and bilateral serous otitis media is reported.
韦格纳肉芽肿病(WG)是一种多系统疾病,其特征为肉芽肿形成和广泛的坏死性血管炎。典型表现为上、下呼吸道及肾脏系统受累。在该疾病过程中曾有面神经麻痹和耳部表现的报道,但极为罕见作为首发特征。文献中仅报道过1例以双侧面神经麻痹为WG首发体征的病例。抗中性粒细胞胞浆抗体(c-ANCA)检测对WG诊断具有高度特异性,97%的病例呈阳性。早期诊断和及时治疗可使这种原本致命的疾病获得较高的缓解率。本文报道1例以双侧面瘫和双侧浆液性中耳炎为表现的WG病例。