CASE REPORT Brown–Vialetto–Van Laere syn

The Brown–Vialetto–Van Laere syndrome (BVVL) is a rare disorder characterized by sensorineural deafness followed or hypoglossal nerves. It was first described by Brown in 1894,3 and later by Vialetto in 19364 and Van Laere in have been reported in just over a century. Around half of all 8 X-linked inheritance1 have been suggested in a few families. C20orf54, by studying a consanguineous family with multiple affected individuals and subsequently demonstrated that muta-tions in this gene were the cause of disease in other, unrelated families. We reported the clinical and neurophysiological features of a patient with deafness, with cranial nerve involvement, and respiratory difficulties. This clinical picture and neurosensorial deafness suggest the diagnosis of pontobulbar palsy with sen-sorineural deafness or Brown–Vialetto–Van Laere syndrome. brain stem auditory evoked potential; MRI, magnetic resonanc