Usher syndrome (USH) is a genetic disorder associated with a dual sensory disability (auditory and visual) called deaf blindness. We report the observation of a 20-year-old patient from a consanguineous marriage with congenital deafness with normal
vestibular function and pigmentary retinopathy responsible for a bilateral decrease in visual acuity that occurred at age 9 years. This association forms type 2 of Usher syndrome. USH is inherited in an autosomal recessive pattern.