Abstract
We describe a case of the Brown-Vialetto-Van Laere syndrome, which is a rare disorder characterized pontobulbar palsy associated with sensorineural deafness. More than 30 cases have been reported since the first case by progressive was described in 1894. We review the literature of this condition, comparing our case with those reported in the literature and emphasizing important features to improve our understanding of this syndrome. (ALS 2000; 1: 277-281)