Ito Y, Shinogi J, Yuta A, Okada E, Taki M, Matsukage H
Department of Otorhinolaryngology, Tsu National Hospital, Hisai, Japan.
Auris Nasus Larynx. 1991;18(3):281-9. doi: 10.1016/s0385-8146(12)80264-1.
A 12-year-old girl had a 1-week history of left otalgia with hearing loss in May, 1989. Audiometry showed a conductive hearing loss of 60 dB. The conventional medical treatment for acute otitis media was initiated, but the symptoms and signs failed to respond this treatment. Four weeks after initial presentation, a diagnostic mastoidectomy was performed. The histological findings of the granulomatous tissue in the mastoid cavity showed nonspecific granulomatous inflammation, infiltrated with multinucleated giant cells. Initial laboratory studies revealed elevated ESR, positive CRP and RA, inverted A/G ratio, and increased Ig level. She had no other sites of involvement, such as the nose, paranasal sinus, pharynx, and larynx, and also no pulmonary or renal involvement was indicated. The diagnosis of a limited form of Wegener's granulomatosis was made on the above clinical and histological findings and laboratory examinations. The patient was then treated with cyclophosphamide, 80 mg/day (2 mg/kg) and prednisolone, 60 mg/day. After 1 week her symptoms and general condition were dramatically improved. Out-patient follow-up 1 year later showed her hearing level was improved to 28 dB, and she continues to do well without evidence of recurrence of the disease.
1989年5月，一名12岁女孩有1周的左耳疼痛伴听力丧失病史。听力测定显示传导性听力损失60分贝。开始了针对急性中耳炎的常规药物治疗，但症状和体征对该治疗无反应。初次就诊四周后，进行了诊断性乳突切除术。乳突腔肉芽肿组织的组织学检查结果显示为非特异性肉芽肿性炎症，有多核巨细胞浸润。最初的实验室检查显示血沉升高、C反应蛋白和类风湿因子阳性、白蛋白/球蛋白比例倒置以及免疫球蛋白水平升高。她没有其他受累部位，如鼻、鼻窦、咽和喉，也未提示有肺部或肾脏受累。根据上述临床、组织学检查结果和实验室检查，诊断为局限性韦格纳肉芽肿。然后该患者接受环磷酰胺80毫克/天（2毫克/千克）和泼尼松龙60毫克/天的治疗。1周后，她的症状和一般状况显著改善。1年后门诊随访显示她的听力水平提高到28分贝，并且她病情持续良好，无疾病复发迹象。
