Neurologic paraneoplastic syndromes with neurotologic manifestations

Evidence supports the hypothesis that autoimmune mechanisms are operational in the etiopathogenesis of certain neurologic paraneoplastic syndromes (PNSs), including paraneoplastic encephalomyelitis (PEM) and paraneoplastic cerebellar degeneration (PCD). The antibodies (Anti-Hu and Anti-Yo), the antigens (Hu and Yo), and complementary DNA clones encoding Hu and Yo, central to PEM and PCD, respectively, have been isolated. In contrast, the antigens, and antibodies if any, involved in autoimmune cochleovestibular dysfunction remain unknown. The temporal bone histopathology and neuropathology of 2 patients, 1 with PEM and 1 with PCD, who developed signs and symptoms of cochleovestibular dysfunction, are reviewed and contrasted to the literature. It is concluded that both auditory and vestibular symptomatology and pathologic alterations can be seen in association with neurologic PNSs and that studies using the antigens and antibodies involved in neurologic PNSs may provide a new perspective on the investigation of autoimmune cochleovestibular dysfunction.