Hern J D, Hollis L J, Mochloulis G, Montgomery P Q, Tolley N S
Department of Otolaryngology, St Mary's Hospital, London, UK.
J Laryngol Otol. 1996 May;110(5):459-61. doi: 10.1017/s0022215100133973.
Wegener's granulomatosis is a multi-system disease characterized by granuloma formation and a necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory and renal systems. However locoregional disease is common and may include otological manifestations. Facial nerve palsy has been reported during the course of the disease process but it is extremely rare for it to be the presenting feature. Previously reported cases have involved a protracted diagnostic process including exploratory tympanotomy, mastoidectomy and facial nerve decompression. We report a case of Wegener's granulomatosis which presented with a facial nerve palsy. An early diagnosis was achieved by measurement of the erythrocyte sedimentation rate (ESR), followed by serological assay of cytoplasmic anti-neutrophil cytoplasmic antibody (cANCA). Histological examination of nasal mucosal biopsies confirmed granuloma formation consistent with a diagnosis of Wegener's granulomatosis. This allowed early treatment with immunosuppressive therapy and avoided unnecessary and potentially hazardous middle ear surgery.
韦格纳肉芽肿病是一种多系统疾病，其特征为肉芽肿形成和坏死性血管炎。典型表现为上、下呼吸道及肾脏系统受累。然而，局部区域疾病也很常见，可能包括耳部表现。面神经麻痹在疾病过程中已有报道，但极为罕见地作为首发特征出现。既往报道的病例诊断过程漫长，包括探查性鼓室切开术、乳突切除术及面神经减压术。我们报告一例以面神经麻痹为表现的韦格纳肉芽肿病病例。通过检测红细胞沉降率（ESR），随后进行抗中性粒细胞胞浆抗体（cANCA）的血清学检测实现了早期诊断。鼻黏膜活检的组织学检查证实了肉芽肿形成，符合韦格纳肉芽肿病的诊断。这使得能够早期进行免疫抑制治疗，避免了不必要且可能有风险的中耳手术。
