Kale S U, Donaldson I, West R J, Shehu A
Department of Ear, Nose, and Throat, University Hospital, Birmingham, UK.
Otol Neurotol. 2003 Jan;24(1):90-5. doi: 10.1097/00129492-200301000-00019.
To study the otolaryngologic disorders in patients with superficial siderosis of the meninges.
Superficial siderosis of the central nervous system is a rare disorder characterized by progressive bilateral hearing loss and ataxia caused by recurrent bleeding into the subarachnoid space. The cerebellum, eighth cranial nerve, and olfactory nerve are particularly susceptible to the deposition of hemosiderin, which is responsible for the symptoms. The diagnosis is confirmed by magnetic resonance imaging.
The clinical notes of five patients with superficial siderosis of the meninges were reviewed with the intent of reporting the otolaryngologic symptoms and signs, the clinical investigations, and treatments.
Four of the five patients had sensorineural deafness, two had smell disturbances, and three had gait abnormalities. Magnetic resonance imaging was the most important investigation used to identify the condition.
Superficial siderosis of the meninges is an important differential diagnosis in patients with progressive sensorineural deafness.
研究脑膜表面铁沉积症患者的耳鼻喉科疾病。
中枢神经系统表面铁沉积症是一种罕见疾病，其特征为蛛网膜下腔反复出血导致进行性双侧听力丧失和共济失调。小脑、第八对脑神经和嗅神经特别容易受到含铁血黄素沉积的影响，而含铁血黄素是导致这些症状的原因。通过磁共振成像可确诊。
回顾了5例脑膜表面铁沉积症患者的临床记录，旨在报告耳鼻喉科症状和体征、临床检查及治疗情况。
5例患者中有4例患有感音神经性耳聋，2例有嗅觉障碍，3例有步态异常。磁共振成像是用于识别该病症的最重要检查。
脑膜表面铁沉积症是进行性感音神经性耳聋患者的重要鉴别诊断疾病。
