脳表ヘモジデリン沈着症による進行性感音難聴の１例

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  • A Case of Progressive Sensorineural Hearing Loss Caused by Superficial Siderosis of Central Nervous System.

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Superficial siderosis of the central nervous system (SSCN) characterized by sensorineural hearing loss, ataxia, and pyramidal tract signs is a rare. The symptoms are caused by intracellular and extracellular hemosiderin deposition in the leptomeninges, subpial tissue, spinal cord, and cranial nerves as a result of chronic bleeding in the subarachnoid space, In the previous reports, the diagnosis of SSCN was often made at autopsy or during a neurosurgical procedure. Recently, MRI makes the non-invasive diagnosis of SSCN possible.<br>A 43-year-old male with SSCN presented with the chief complaint of bilateral hearing loss and gait disturbance. The diagnosis of SSCN was made by T-2 weighted images on MRI of the central nervous system showing diffuse marginal hypointensity of the cerebrum, brain stem and cerebellum. Bleeding source into the subarachnoid space could not be detected on the detailed examinations, and the hearing loss was rapidly progressed to total deafness in both ears.<br>SSCN should be considered in the differential diagnosis of unexplainable progressive sensorineural hearing loss. MRI is useful as noninvasive method for accurate diagnosis of SSCN.

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