Zorzi Silvia, Testa Gabriele, Tomasoni Michele, Taboni Stefano, Nassif Nader, Gregorini Gina Alessandra, Sorrentino Tommaso, Piazza Cesare, Redaelli de Zinis Luca Oscar
Unit of Otorhinolaryngology - Head and Neck Surgery, ASST Spedali Civili di Brescia, Brescia, Italy.
Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, School of Medicine, Brescia, Italy.
Acta Otorhinolaryngol Ital. 2025 Jun;45(3):217-230. doi: 10.14639/0392-100X-N3008.
To describe the clinical characteristics and outcomes of patients affected by granulomatosis with polyangiitis (GPA) presenting with isolated ear involvement.
A retrospective review of patients affected by GPA and treated at the University of Brescia, Italy, from 2002 to 2023 was conducted. Only patients with exclusive otologic manifestation as first presentation were included.
Among 610 patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) diagnosed and followed at our Institution, 6 (0.8%) presented with exclusive ear involvement as first presentation, all affected by GPA. Most frequently patients presented with otitis media with effusion, sensorineural or mixed hearing loss, and dizziness. Two patients developed systemic symptoms. All patients experienced at least a partial recovery of middle ear function after starting immunosuppressive therapy.
AAVs rarely show initial presentation as isolated ear involvement, and more commonly present as otitis media with hearing loss that is unresponsive to conventional therapy. Once an AAV is suspected, surgery should be avoided since further damage can be caused by local iatrogenic inflammation sustained by the underlying condition. Local improvement is generally seen after the start of immunosuppressive therapy.
描述以耳部孤立受累为表现的肉芽肿性多血管炎（GPA）患者的临床特征及预后。
对2002年至2023年在意大利布雷西亚大学接受治疗的GPA患者进行回顾性研究。仅纳入以耳部症状为首发表现的患者。
在我院诊断并随访的610例抗中性粒细胞胞浆抗体相关性血管炎（AAV）患者中，6例（0.8%）以耳部孤立受累为首发表现，均为GPA。患者最常见的表现为中耳积液性中耳炎、感音神经性或混合性听力损失以及头晕。2例患者出现全身症状。所有患者在开始免疫抑制治疗后中耳功能至少部分恢复。
AAV很少以耳部孤立受累为首发表现，更常见的表现为对传统治疗无反应的伴有听力损失的中耳炎。一旦怀疑为AAV，应避免手术，因为潜在疾病导致的局部医源性炎症可能会造成进一步损害。免疫抑制治疗开始后通常可见局部症状改善。
