Brown-Vialetto-Van Laere syndrome, or pontobulbar palsy with deafness, is a rare disorder characterized by bilateral sensorineural deafness and a variety of cranial nerve disorders usually involving the motor components of the lower cranial nerves. Less commonly, spinal motor nerves and upper motor neurons are involved. Familial and sporadic cases have been reported. Based on evidences, this syndrome has been related to autosomal recessive, autosomal dominant and X-linked inheritance. Autoimmune origin has been considered as well. In this paper, we report the case of a 38-year-old female patient who primarily presented with bilateral sensorineural hearing loss and then progressively developed Xth and VIIth cranial nerve paralysis. Brown-Vialetto-Van Laere syndrome was diagnosed with this symptom complex of sensorineural hearing loss and pontobulbar palsy.