Helmchen C, Jäger L, Büttner U, Reiser M, Brandt T
Department of Neurology, Ludwig-Maximillians-Universität, Munich, Germany.
J Vestib Res. 1998 Mar-Apr;8(2):155-67.
In the chronic-relapsing form of Cogan's syndrome, it can be difficult to evaluate the activity of the disease. In contrast to the initial stage, routine diagnostic techniques sometimes fail to indicate progression in the chronic stage. To determine whether high-resolution magnetic resonance imaging (HR-MRI) can be used to differentiate between active and inactive stages, we examined three patients with Cogan's syndrome (one during an acute relapse, two with chronic audiovestibular deficits), all of whom had antibodies to inner ear tissue (cochlea, vestibular labyrinth). Unenhanced T1-, T2, gadolinium-enhanced T1-weighted, and three-dimensional constructive interference in steady stage (CISS) images were used. Abnormal MRI signals of the inner ear were related to the activity of the disease. The patient studied during an acute exacerbation showed abnormal MRI signals in the vestibule, semicircular canals, vestibular nerve, and cochlea, which disappeared after the relapse. These abnormalities included high signal in the membranous labyrinth, the vestibule, and cochlea, with enhancement on T1-weighted images, indicating gadolinium leakage through the abnormal labyrinthine membrane into the perilymphatic spaces. In contrast, the other two patients with chronic audiovestibular deficits but no clinical signs of an acute relapse, had narrowing or occlusion of semicircular canals of the cochlea on the 3D-CISS images, but no high signal lesions (T1) and no enhancement. We conclude that sequential gadolinium-enhanced MRI can identify the active stage of Cogan's syndrome. The combination of HR-MRI and antibodies to inner ear antigens are helpful in the diagnosis of acute, sequential, bilateral audiovestibular impairment of autoimmune origin.
在科根综合征的慢性复发性形式中，评估疾病活动度可能较为困难。与疾病初期不同，常规诊断技术有时无法显示慢性期的病情进展。为了确定高分辨率磁共振成像（HR-MRI）是否可用于区分活动期和非活动期，我们检查了3例科根综合征患者（1例处于急性复发期，2例有慢性听觉前庭功能缺损），所有患者均有内耳组织（耳蜗、前庭迷路）抗体。使用了未增强的T1加权、T2加权、钆增强T1加权以及稳态三维建设性干扰（CISS）图像。内耳的MRI异常信号与疾病活动度相关。在急性加重期接受研究的患者，其前庭、半规管、前庭神经和耳蜗出现MRI异常信号，复发后这些信号消失。这些异常包括膜迷路、前庭和耳蜗内的高信号，T1加权图像上有强化，表明钆通过异常的迷路膜漏入外淋巴间隙。相比之下，另外2例有慢性听觉前庭功能缺损但无急性复发临床体征的患者，在三维CISS图像上显示耳蜗半规管变窄或闭塞，但无高信号病变（T1）且无强化。我们得出结论，序贯钆增强MRI可识别科根综合征的活动期。HR-MRI与内耳抗原抗体相结合有助于诊断自身免疫性起源的急性、序贯性、双侧听觉前庭损害。
