Liu Riyuan, Liao Simin, Zhao Yurong, Zhu Jian, Yang Shiming, Zhao Hui
College of Otolaryngology Head and Neck Surgery，the Sixth Medical Center of Chinese PLA General Hospital.
Department of Rheumatology and Immunology，the First Medical Center of Chinese PLA General Hospital.
Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2024 Mar;38(3):183-187. doi: 10.13201/j.issn.2096-7993.2024.03.001.
To analyze the clinical feature, diagnosis and treatment of Anca-associated vasculitis with ear symptoms. In this retrospective study, we summarized the clinical and laboratory examination, pure tone audiometry, aural immittance measurement, CT scan of temporal bone and treatment of 40 patients in the First Medical Center of the PLA General Hospital. A total of 11 cases（27.5%） had the initial symptom in the ear. The most common symptoms were hearing loss, and the other symptoms included a sense of ear fullness, otorrhea and tinnitus. There were 35 cases with hearing loss: 19 cases with conductive hearing loss（47.5%）, 9 cases with sensorineural hearing loss（22.5%）, and 7 cases with mixed hearing loss（17.5%）. 5 cases had a sense of ear fullness or tinnitus, and the results of the hearing test were normal（12.5%）. All of the 40 patients had multi-system involvement, and respiratory system accounted for the most. All patients had a positive result of Anti-neutrophil cytoplasmic antibody（ANCA）. Treatment included systemic hormonal, immunosuppressive, or biologic therapy. There were 3 cases recovered（7.5%）, 22 cases with alleviated ear symptoms（55.0%）, 6 cases with recurrent hearing loss（15%） and 9 cases had no significant improvement（22.5%）. Conductive deafness（secretory otitis media） can be the first manifestation in the early stage of otitis media with AAV（OMAAV）, later it may turn to binaural mixed deafness. Otolaryngologists need to consider OMAAV diagnosis when diagnosing and treating patients with recurrent secretory otitis media. Multi-system symptom consultation and ANCA examination can help identify. Early systemic medication and the application of immunosuppressants or biological agents can help relieve the ear symptoms.
分析伴有耳部症状的抗中性粒细胞胞浆抗体相关性血管炎的临床特征、诊断及治疗。在这项回顾性研究中，我们总结了解放军总医院第一医学中心40例患者的临床及实验室检查、纯音听力测试、耳声导抗测量、颞骨CT扫描及治疗情况。共有11例（27.5%）患者首发症状出现在耳部。最常见的症状是听力减退，其他症状包括耳闷胀感、耳漏和耳鸣。有35例存在听力减退：19例为传导性听力减退（47.5%），9例为感音神经性听力减退（22.5%），7例为混合性听力减退（17.5%）。5例有耳闷胀感或耳鸣，听力测试结果正常（12.5%）。40例患者均有多系统受累，其中呼吸系统受累最为常见。所有患者抗中性粒细胞胞浆抗体（ANCA）检测均为阳性。治疗包括全身激素、免疫抑制或生物治疗。3例康复（7.5%），22例耳部症状缓解（55.0%），6例听力减退复发（15%），9例无明显改善（22.5%）。传导性耳聋（分泌性中耳炎）可为抗中性粒细胞胞浆抗体相关性血管炎伴中耳炎（OMAAV）早期的首发表现，后期可能转为双耳混合性耳聋。耳鼻咽喉科医生在诊治复发性分泌性中耳炎患者时需考虑OMAAV的诊断。多系统症状会诊及ANCA检查有助于明确诊断。早期全身用药及应用免疫抑制剂或生物制剂有助于缓解耳部症状。
