SUPERFICIAL SIDEROSIS OF THE CENTRAL-NERVOUS-SYSTEM - AN UNDERESTIMATED CAUSE OF HEARING-LOSS

Superficial siderosis of the central nervous system (CNS) is a rare di sease resulting in the accumulation of haemosiderin in the meninges, t he brain surface, the spinal cord and the cranial nerves. The pigment is deposited as a result of chronic bleeding in the subarachnoid space . This produces a clinical picture of deafness, ataxia, cranial nerve deficits and in the latest stages dementia. In some cases the source o f bleeding can be identified, whilst in others it can not. Despite its rarity the disease should be considered in the differential diagnosis of sensorineural deafness, particularly as it is a progressive and in some cases curable disease which is easily diagnosed by magnetic reso nance imaging (MRI). In this case report the haemosiderin was derived from an ependymoma of the fourth ventricle with extension into the cer ebello-pontine angle. The first symptom was a worsening sensorineural hearing loss.