Del Poggio Anna, Cangiano Iacopo, Baldissera Elena, Farina Nicola, Dagna Lorenzo, Mapelli Roberto, Falini Andrea, Teggi Roberto
Department of Neuroradiology and CERMAC, IRCCS San Raffaele Scientific Institute and Vita- Salute San Raffaele University, Via Olgettina 60, Milan, 20132, Italy.
Department of Otolaryngology, IRCCS San Raffaele Scientific Institute, San Raffaele Hospital, via Olgettina 60, Milan, 20132, Italy.
Eur Arch Otorhinolaryngol. 2025 May;282(5):2753-2760. doi: 10.1007/s00405-024-09191-x. Epub 2025 Feb 1.
Autoimmune inner ear disorder (AIED) diagnosis is based on the onset of bilateral sensorineural hearing loss (SNHL) that progresses over 3 to 90 days and responds to steroid treatment. Among autoimmune conditions, Cogan's syndrome (CS) is a rare disorder characterized by the onset of inflammation in both eyes and in the inner ear, leading to progressive sensorineural hearing loss, particularly at high frequencies, often with vestibular symptoms. If left untreated, CS can cause profound bilateral hearing loss. The disease is believed to have an autoimmune etiology, although a common biomarker has not been clearly identified. Early-stage inner ear involvement in CS can mimic Menière's Disease (MD), although key differences between phenotypes, such as the pattern and speed of progression, characterized by a pousseè evolution of bilateral high frequencies hearing loss rather than low frequencies fluctuating, help distinguish the two conditions. Few is known about imaging of the inner ear in patients with CS, and most of the insight into the radiological manifestation in the inner ear of this rare pathology came from the '90s. Recent advances in imaging, particularly with 3D-FLAIR MRI sequences after gadolinium administration, have shown promise in detecting endolymphatic hydrops (EH) in MD patients. Our study reports findings in five CS patients on whom we performed a 3 Tesla MRI, revealing EH in three of them. This is the first report of such a correlation in vivo, contributing valuable new insights into the radiological manifestations of CS.
自身免疫性内耳疾病（AIED）的诊断基于双侧感音神经性听力损失（SNHL）的发作，这种听力损失在3至90天内进展，并对类固醇治疗有反应。在自身免疫性疾病中，科根综合征（CS）是一种罕见的疾病，其特征是双眼和内耳出现炎症，导致进行性感音神经性听力损失，尤其是高频听力损失，常伴有前庭症状。如果不进行治疗，CS可导致严重的双侧听力损失。尽管尚未明确鉴定出常见的生物标志物，但该疾病被认为具有自身免疫病因。CS早期内耳受累可模仿梅尼埃病（MD），尽管表型之间的关键差异，如进展模式和速度，以双侧高频听力损失的突然演进而非低频波动为特征，有助于区分这两种疾病。关于CS患者内耳成像的了解很少，对这种罕见病理内耳放射学表现的大多数见解来自20世纪90年代。成像技术的最新进展，特别是钆增强后的3D-FLAIR MRI序列，在检测MD患者的内淋巴积水（EH）方面显示出前景。我们的研究报告了对五名CS患者进行3特斯拉MRI检查的结果，其中三名患者显示有EH。这是体内这种相关性的首次报告，为CS的放射学表现提供了有价值的新见解。
