Concord Hospital, Sydney, New South Wales, Australia.
Int J Rheum Dis. 2022 Oct;25(10):1203-1207. doi: 10.1111/1756-185X.14401. Epub 2022 Jul 26.
Giant cell arteritis (GCA) is a chronic vasculitic disorder predominantly affecting medium to large sized arteries, prevalent in the 50 plus age group. This case illustrates an atypical presentation of this disease in the form of bilateral sensorineural hearing loss (SNHL). Apart from the presence of constitutional and vertiginous symptoms, there were essentially no classical features of GCA. Differentials were broad including infection, malignancy and medication toxicity as well as brain, eye and ear syndromes such as Cogan's syndrome, all of which were eventually excluded. Her diagnosis was ultimately confirmed on positron emission tomography, which highlights the diagnostic importance of this modality. She was managed with corticosteroids then tocilizumab and is making a gradual recovery. Literature review demonstrates that SNHL is more prevalent than previously suggested in GCA, although this does not have widespread recognition. Mechanisms of SNHL in GCA include vascular occlusion, immunological mechanisms including cross reactivity with viral antigens and direct viral infection. SNHL does appear to improve with corticosteroids. This case emphasizes the importance of considering GCA as an important differential in SNHL.
巨细胞动脉炎（GCA）是一种主要影响中到大动脉的慢性血管炎疾病，在 50 岁以上人群中较为常见。本例以双侧感觉神经性听力损失（SNHL）的形式呈现出该疾病的非典型表现。除了存在全身症状和眩晕外，基本上没有 GCA 的典型特征。鉴别诊断包括感染、恶性肿瘤和药物毒性以及脑、眼和耳部综合征，如 Cogan 综合征，最终都被排除了。她的诊断最终通过正电子发射断层扫描得到确认，这凸显了该诊断方法的重要性。她接受了皮质类固醇治疗，然后是托珠单抗治疗，目前正在逐渐康复。文献回顾表明，SNHL 在 GCA 中的发病率比以前认为的要高，尽管这并没有得到广泛认可。GCA 中 SNHL 的发生机制包括血管闭塞、免疫机制，包括与病毒抗原的交叉反应和直接病毒感染。SNHL 似乎确实可以通过皮质类固醇改善。本例强调了在 SNHL 中考虑 GCA 作为重要鉴别诊断的重要性。
