Department of Otolaryngology, Helsinki University Central Hospital, Helsinki, Finland.
Otol Neurotol. 2013 Dec;34(9):1736-8. doi: 10.1097/MAO.0b013e3182953154.
To show that both hearing and vestibular loss can be reversible and quantified in Cogan's syndrome.
Immunosuppressive medication was continued for more than 6 years in a young woman with Cogan's syndrome. Standard pure tone audiometry (PTA) and speech discrimination score (SDS) for hearing, motorized head impulse test (MHIT) for horizontal, angular vestibulo-ocular reflex, and erythrocyte sedimentation rate were followed serially.
The PTA before medication was 59/74 dB, and it stabilized to 13/46 dB in 2 years. The initial SDS of 92/72% improved later to 100/100%, respectively. The gain of the vestibulo-ocular reflex was asymmetric (32%) and decreased to 0.48/0.25 at the beginning. Two years after the treatment, both the asymmetry (7%) and the gain (0.95/0.82) were recovered within normal limits. Erythrocyte sedimentation rate improved from 40 to 5 mm/h.
Our case report of Cogan's syndrome demonstrates objective, simultaneous deterioration of hearing and vestibular function, which improved and stabilized after the introduction of immunosuppressive medication. The efficacy of immunosuppressive medication on vestibular function may be followed repetitively using MHIT in patients with Cogan's syndrome.
展示在 Cogan 综合征中，听力和前庭损失均可逆转并可量化。
一位年轻的 Cogan 综合征女性患者接受了超过 6 年的免疫抑制治疗。我们对其进行了标准纯音听阈测试（PTA）和言语辨别率（SDS）以评估听力、水平和角前庭眼反射的电动头脉冲试验（MHIT）以及红细胞沉降率，对其进行了连续监测。
用药前 PTA 为 59/74dB，2 年内稳定在 13/46dB。最初的 SDS 为 92/72%，后来分别提高到 100/100%。前庭眼反射的增益呈不对称性（32%），最初为 0.48/0.25。治疗 2 年后，不对称性（7%）和增益（0.95/0.82）均恢复至正常范围内。红细胞沉降率从 40 毫米/小时提高到 5 毫米/小时。
我们的 Cogan 综合征病例报告表明，听力和前庭功能同时出现客观恶化，在免疫抑制治疗后得到改善和稳定。在 Cogan 综合征患者中，使用 MHIT 可以重复监测免疫抑制治疗对前庭功能的疗效。
