中文摘要：

目的探讨韦格纳肉芽肿在耳部的临床表现，增强耳鼻喉科医师对该疾病的认识。方法回顾性分析2000～2015年以中耳炎为首诊的5例韦格纳肉芽肿患者的临床表现、治疗及预后。结果首发症状表现为听力下降3例、耳闷2例、耳痛1例、耳间断流脓1例，5例患者均伴有不同程度的发热（最高达40℃），伴咽痛2例，鼻堵2例，流涕2例，咳嗽、咳痰1例。耳科体征表现为鼓膜内陷3例、鼓膜充血1例、鼓膜瘢痕性增生1例。5例患者中抗中性粒胞浆抗体（antimeutro phileytoplasmic anyibocties, ANCA）阳性2例，阴性3例。3例患者行组织活检，形成肉芽肿结节者2例，血管内膜炎、管壁坏死者1例。采用糖皮质激素加免疫抑制剂联合冶疗，4例患者症状明显缓解，1例失访。结论以中耳炎作为首诊韦格纳肉芽肿患者的临床表现缺乏特异性。ANCA检测和组织活检对本病的诊断具有重要价值。糖皮质激素与免疫抑制剂联合治疗对稳定病情、缓解症状具有明显作用。

英文摘要：

Objective To discuss the otological manifestations of Wegener＇s granulomatosis（WG）, and strengthen the understanding of this disease. Methods This was a retrospective analysis of five Wegener＇s granulomatosis cases. All were in-hospital patients at our hospital seeking treatment for otitis media during 2000-2015. Results The initial symptoms included hearing loss in 3 cases, fullness in 2cases, pain and discharging in 1 case. All the 5 patients were accompanied with different degrees of fever （up to 40~C）, with pharyngalgia in 2 cases, rhinocleisis in 2 cases, running nose in 2 cases and cough phlegm in 1 case. Otological examinations included otopiesis in 3 cases, tympanic membrane congestion in 1 case and tympanic membrane scarhyperplasia in 1 case. ANCA test results showed 2 positive and 3 negative in 5 patients. 3 cases underwent biopsy： granulomatous nodule formation in 2 cases; endangiitis and angionecrosis in 1 case. After the treatment combined with glucocorticoid and immunosuppressants, patients＇ otological symptoms were relieved in 4 cases, and 1 case lost. Conclusion The patients who suffered from otitis media as the first visit of WG usually showed a lack of clinical specificity. ANCA test and biopsy results played an important role in the diagnosis. The combined treatment of glucocorticoid and immunosuppressantsis obviously contributed to control the disease and relieved the symptoms.