OBJECTIVE To review the paraneoplastic neurologic syndromes associated with otoneurophthalmologic manifestations. METHODS Paraneoplastic otoneurophthalmologic syndromes usually result from encephalitis of the brainstem and/or cerebellar degeneration. They can develop in association with various immunological responses against onconeuronal proteins (e.g. anti-Hu, anti-Ri, anti-Yo, anti-Ma, anti-Ta and anti-Tr) or with immunological mechanisms against unknown antigens. The eye movement abnormalities may have a supranuclear, nuclear or internuclear origin. There is no ocular movement abnormality which can be considered pathognomonic of a paraneoplastic disorder, but opsoclonus-myoclonus of infancy is often associated with neuroblastoma. The association of hearing loss and paraneoplastic sensory neuronopathy suggests that in some patients the neurosensory deafness result from involvement of the ganglia of Corti and/or the cochlear nuclei in the brain stem. The management of these syndromes depends on their rapid identification as paraneoplastic disorders and on the early diagnosis and treatment of the cancer. Patients with anti-Ta (or anti Ma-2) antibodies may improve with treatment of the cancer, usually a germ-cell tumor of the testis. Paraneoplastic opsoclonus-myoclonus of infancy usually improves with treatment that combines chemotherapy, steroids, and intravenous immunoglobulins, although neurological sequelae (psychomotor and language retardation) are frequent. CONCLUSIONS There are several paraneoplastic neurologic syndromes that may present with otoneurophthalmologic symptoms. Detection of antineuronal antibodies facilitates the early identification of some of these syndromes and associated tumors. In general, the management of these syndromes is based on treatment of the associated cancer.