Perilymph, cerebrospinal fluid (CSF) and plasma from 19 patients with acoustic neuromas were examined for albumin, alpha 2-macroglobulin and IgG. Patients with otosclerosis were used as controls. A highly significant increase of the tested proteins was found in perilymph from patients with acoustic neuromas. Determination of the tau-transferrin, which is a specific CNS protein, could neither be demonstrated in plasma nor in perilymph. This is in favour of the assumption that perilymph is plasma-derived and not concentrated CSF. Various causes for the high protein concentrations are discussed, and it is suggested that the increase is caused by a blockage of the neuroaxonal transport mechanisms.