International Journal of Contemporary Pediatrics | 2019
Brown Vialetto Van Laere syndrome, a fatal disease with a simple solution: a case series
Abstract
Brown-Vialleto-Van Laere Syndrome (BVVL) also called progressive ponto bulbar palsy with deafness or bulbar hereditary neuropathy type I is a rare disorder characterized by rapid onset progressive sensorineural hearing loss (SNHL) with lower motor cranial nerve disorders and respiratory difficulties. It was first described by Brown in 1894 and later by Vialetto in 19364 and Van Laere in 1966. Less than 60 cases have been described in literature till date and majority of them have been reported to be sporadic.