Evidence supports the hypothesis that autoimmune mechanisms are operat
ional in the etiopathogenesis of certain neurologic paraneoplastic syn
dromes (PNSs), including paraneoplastic encephalomyelitis (PEM) and pa
raneoplastic cerebellar degeneration (PCD). The antibodies (Anti-Hu an
d Anti-Yo), the antigens (Hu and Yo), and complementary DNA clones enc
oding Hu and Yo, central to PEM and PCD, respectively, have been isola
ted. In contrast, the antigens, and antibodies if any, involved in aut
oimmune cochleovestibular dysfunction remain unknown. The temporal bon
e histopathology and neuropathology of 2 patients, 1 with PEM and 1 wi
th PCD, who developed signs and symptoms of cochleovestibular dysfunct
ion, are reviewed and contrasted to the literature. It is concluded th
at both auditory and vestibular symptomatology and pathologic alterati
ons can be seen in association with neurologic PNSs and that studies u
sing the antigens and antibodies involved in neurologic PNSs may provi
de a new perspective on the investigation of autoimmune cochleovestibu
lar dysfunction.