以突發性感音性聽力喪失爲最初表現的Wegener氏肉芽腫

Wegener氏肉芽腫(Wegener's granulomatosis)是一種以肉芽腫和壞死性血管炎侵犯全身的自體免疫疾病，目前致病機轉仍不明。在臨床土，約71-90%的患者會有頭頸部的症狀。其中約19-38%的患者會有耳部的表現，包括傳導性聽力障礙、感音性聽力障礙、暈眩和顏面神經麻痺。其中以漿液性中耳炎表現的傳導性聽力障礙最常見，而單純的感音性聽力障礙則較少見。此案例以左側急性感音性聽力喪失爲最初表現。患者陸續出現發燒、呼吸困難、四肢關節疼痛和皮膚出現出血點。免疫學檢查嗜中性白血球細胞質抗體(Anti-neutrophil cytoplasmatic antibodies, C-ANCA)和抗proteinase 3 (anti-proteinase 3, PR3)抗體呈現陽性，讓我們早期診斷出Wegener氏肉芽腫，並開始予以免疫抑制藥物cyclophophamide及類固醇口服治療。肺部組織切片的病理表現爲慢性肉芽腫性的發炎，確定診斷爲Wegener氏肉芽腫。以急性感音性聽力喪失爲最初症狀的Wegener氏肉芽腫，在本土文獻並未有類似案例，特提出報告，提醒耳鼻喉科醫師提高封此疾病的認識，對以耳部症狀表現的疑似患者進行系統性的檢查，早期診斷，及時治療，避免不可逆的聽力喪失和減少全身的傷害。

關鍵字

Wegener氏肉芽腫；急性感音性聽力喪失

並列摘要

Wegener's granulomatosis is an organ-threatening autoimmune disease of as yet unknown etiology characterized by granulomatous lesions and a necrotizing vasculitis. Head and neck manifestations account for the initial presenting symptoms in 71-90% of patients who are ultimately diagnosed with Wegener's granulomatosis. Approximately 19-38% of Wegener's granulomatosis patients have otologic findings, including conductive hearing and sensorineural hearing loss (SNHL). The reported incidence of SNHL in Wegener's granulomatosis is rare. Here, we report a case of Wegner's granulomatosis in a patient who first presented with acute onset of left side sensorineural hearing loss. Physical examination showed an intact ear drum. Shortly after hospitalization, shortness of breath, arthlagia, purpura of limbs developed. Anti-neutrophil cytoplasmatic antibodies (C-ANCA) anti-proteinase 3 which were highly specific for Wegener's granulomatois showed positive. Biopsy of lung confirmed the diagnosis of Wegener's granulomatosis. This case report presents on unusual clinical manifestation of Wegener's granulomatosis with acute sensorineural hearing loss.

並列關鍵字

Wegener's granulomatosis ； acute sensorineural hearing loss

國際替代計量

以突發性感音性聽力喪失爲最初表現的Wegener氏肉芽腫

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