Spinal ependymoma complicated by superficial siderosis.

To cite: Grech R, Galvin L, Looby S, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013201036 DESCRIPTION A 64-year-old woman presented with progressive hearing loss. A pure-tone audiography confirmed bilateral high-frequency sensorineural hearing loss. Her medical and surgical history was unremarkable except for long-standing back pain. Neurological examination revealed mild dysmetria and gait ataxia. There was no leg weakness or sphincteric dysfunction. MRI revealed superficial siderosis lining the cerebellum and brainstem, which prompted further imaging of the entire neuraxis in order to exclude an underlying cause. A myxopapillary ependymoma seen at the level of the conus medullaris was felt to be responsible for recurrent haemorrhage within the subarachnoid space. Excision of the spinal ependymoma was performed and the patient regained near-normal hearing following a cochlear implant. DISCUSSION Superficial siderosis presents with slowly progressive sensorineural hearing loss, gait ataxia and cerebellar dysarthria. Involvement of other cranial nerves has also been described. Clinical history and examination often suggest a neurodegenerative disorder. Superficial siderosis results from haemosiderin deposition in the subpial layers of the neuraxis, and complicates chronic haemorrhage within the subarachnoid space. The posterior fossa is preferentially affected, which may in part be explained by the presence of Bergmann glia in the cerebellum that display increased ferritin synthesis. The long glial segment of the vestibulocochlear nerve makes it more vulnerable to axonal damage by iron deposition. Bilateral sensorineural hearing loss is present in 95% of affected individuals.