Abstract
We report an Egyptian case with Brown–Vialetto–Van Laere syndrome. A five-year-old girl with progressive hearing loss was documented by brainstem auditory evoked potentials (BAEP) as sensorineural deafness, bilateral facial palsy and bilateral partial vocal cord paralysis in the paramedian position. There was no evidence of involvement of the upper motor neurons or spinal motor nerves. There is no similar case in the family.