Brown-Vialetto-Van Laere Syndrome 1 (BVVLS1) is a neurological condition characterised by sensorineural deafness and progressive ponto-bulbar palsy. Age of onset is variable and the patients develop numerous neurological symptoms within a relatively short period of time that result from the involvement of different cranial and spinal nerves.

More than 65 cases of BVVLS1 have been reported worldwide with a female to male prevalence ratio of 3:1. Differential diagnosis includes amyotrophic lateral sclerosis (ALS), Fazio-Londe disease, Nathalie syndrome, Poretti-Boltshauser syndrome, and Madras motor neuron disease.

BVVLS1 has been known to show familial as well as sporadic occurrence. Majority of the familial cases follow an autosomal recessive mode of inheritance. However, at least one report each points to an autosomal dominant or X-linked mode of inheritance for the syndrome.

Brown-Vialetto-Van Laere Syndrome 1 is associated with homozygous or compound heterozygous mutations in SLC52A3 gene. SLC52A3 encodes riboflavin transporter protein involved in intestinal absorption of riboflavin.