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中樞神經性表面血鐵質沉積症引起聽力損失

Hearing Loss Caused by Superficial Siderosis of Central Nervous System

Abstracts


中樞神經性表面血鐵質沉積症極為罕見,導因於持續性或反覆性的蛛網膜下腔出血,使血鐵質不斷地沉積在腦部或脊髓表面,引起典型的兩側感覺神經性聽力障礙、小腦性運動失調及脊髓病變之三合一症候群。一55歲男性,24年前曾發生一場嚴重的車禍,因昏迷住院了兩個月。5年前開始步態不穩,1年後開始感到雙側耳鳴,當時純音聽力檢查顯示兩側平均聽閾(500~4,000 Hz)均為38.8 dBHL,磁振造影之梯度迴訊時序T2*權重成像顯示整個腦膜均有散在性之血鐵質的沉積,包括上段頸部脊髓、腦幹、小腦、大腦及腦室,確診是中樞神經性表面血鐵質沉積症,但患者對於診斷遲疑,不願接受治療。在往後的4年,兩側聽力障礙不斷持續地惡化,最近一次的純音聽力檢查顯示平均聽閾為右側80 dBHL,左側83.8 dBHL,患者甫同意開始接受口服鐵質螯合劑deferipone治療。當我們在診治雙側進行性感覺神經性聽力障礙時,如果患者同時有步態不穩,並且先前亦有嚴重的外傷病史時,要想到中樞神經性血鐵質沉積症的可能性,儘早提供治療,否則,聽力會持續惡化。

Parallel abstracts


Superficial siderosis of central nervous system (SSCNS) is quite rare. It is caused by the deposition of hemosiderin iron on the brain or spinal cord surface, and could be attributable to continuous or repeated bleeding into the subarachnoid space. The classical triad manifestations included bilateral sensorineural hearing loss (SNHL), cerebellar ataxia and myelopathy. A 55-year-old man suffered from a traffic accident 24 years ago. He became comatose so was hospitalized over the following two months. However, his gait became unsteady 5 years ago, and he had bilateral tinnitus one year later. The average hearing thresholds in 500-4,000 Hz of the right and left ears were measured showing both 33.8 dBHL. Gradient-echo T2*-weighted magnetic images demonstrated diffuse hemosiderin deposition along the leptomeninges, including upper cervical spinal cord, brainstem, cerebellum, basal cerebrum and all ventricles. He was diagnosed with SSCNS. Because he distrusted our diagnosis, he has not received any medication. Over the following four years, bilateral SNHL has exacerbated. The latest average hearing thresholds of the right and left ears were measured showing 80 and 83.8 dBHL, respectively. He finally agreed to daily take 1,000 mg deferipone. SSCNS should be considered if a patient with bilateral progressive SNHL has unstable gait after a major trauma history. An appropriate treatment should be recommended as early as possible, or the SNHL will exacerbate.

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