Superficial siderosis of the CNS and otoneurological manifestations

Superficial siderosis is an uncommon condition occurring in central nervous system (CNS) due to deposition of hemosiderin in the subpial meningeal layer causing progressive neurological dysfunction. The classical clinical manifestation is a triad of chronic ataxia, bipyramidal signs and progressive bilateral symmetrical sensorineural hearing loss (SNHL). It has rarely been reported in Indian literature. We report an unusual case of superficial siderosis in a 60-year-old farmer who presented with the above triad along with involvement of olfactory nerve. We present this case to highlight the fact that progressive SNHL can be an important sign for the early awareness of this rare disorder. The literature on superficial siderosis is reviewed and the pathogenesis is discussed.