Cobalt exerts well-known and documented toxic effects on the thyroid, heart and the haematopoietic system, in addition to the occupational lung disease, allergic manifestations and a probably carcinogenic action. Cobalt neurotoxicity is reported in isolated cases, and it has never been systematically treated. Bilateral optic atrophy and retinopathy, bilateral nerve deafness and sensory-motor polyneuropathy have been described long ago as a result of chronic occupational exposure to cobal powder or during long-term treatment of anaemia with cobalt chloride. Recently, some patients with high levels of cobalt released from metal prosthesis have been referred as presenting with tinnitus, deafness, vertigo, visual changes, optic atrophy, tremor and peripheral neuropathy. The aim of this work is to group these cases and to identify a possible mechanism of cobalt neurotoxicity, focusing on hypothetic individual susceptibility such as altered metal-binding proteins, altered transport processes in target cells or polymorphic variation of genetic background.