Thirteen patients affected by either dominant or recessive and/or sporadic olivopontocerebellar atrophy were studied. All patients were subjected to auditory evoked potential recordings including early and long latency components, CT scans, vestibular and EMG-ENG examinations. In nine patients somatosensory evoked potentials were also recorded. Clear-cut abnormalities in brainstem auditory evoked potentials were observed in only two patients while a slight reduction of the IV-V/I amplitude ratio was found in seven cases. N85 was increased in two patients. The main feature of somatosensory evoked potentials abnormalities was a delayed N20 in association with prolonged N13-N20 central conduction time (five patients). For all patients the CT scan varying degrees of cerebellar and brainstem atrophy. There was no clear correlation between the abnormalities revealed by neurophysiological and neuroradiological investigations and the severity and duration of the illness. It is noteworthy that auditory and/or somatosensory evoked potential changes were found in all dominant olivopontocerebellar atrophy patients.