Abstract: Superficial siderosis of the central nervous system (SSCNS) is a rare disease with impaired cerebellum and brain stem as main clinical manifestations,  and with iron depositions on the surfaces of central nervous system tissues as MR neuroimaging characteristics. This paper reported the clinical diagnosis and MR features of one SSCNS female who had recurrent headaches,  progressive sensorineural hearing loss,  cerebellar ataxia,  slight pyramidal signs and no history of cerebral hemorrhage. Combined with literatures review,  its possible pathological mechanisms and comprehensive methods for early diagnosis and treatment will be explored.

Key words: superficial siderosis of the central nervous system, cerebellar ataxia, sensorineural hearing loss, susceptibility weighted imaging