Auditory brainstem responses (ABR) have been obtained in 22 patients, 15 with familial and 7 with sporadic olivopontocerebellar atrophy; 8 of them were pairs of siblings. ABR abnormalities have been found in 72.7%, with a significant higher percentage in familial versus sporadic patients. Similar ABR patterns have been obtained in affected members of the same family. No correlations have been found between ABR results and the following parameters: age, gender, disease duration, degree of clinical involvement and fashion of inheritance. ABR pattern did not show any modification over a follow-up period of 15–24 months. Our findings suggest that the involvement of the auditory pathways may be under genetic contro