Introduction: Usher syndrome is an autosomal recessive disease characterized by sensorineural hearing loss and pigmentary retinitis. There is an association of inherited sensorineural deficiencies with total or partial hearing loss and progressive reduction of vision due to degeneration of retinal photoreceptor cells, termed retinitis pigmentosa.

Objective: To present a systematic review of Usher Syndrome over the last ten years.

Data Synthesis: Usher syndrome is a rare condition, with an incidence of 3 / 100,000 people and leads to deaf-blindness, being the most common cause in adults. Among patients with retinitis pigmentosa, it is the most common cause, present in 6-10%. It was classified by Merin and colleagues into four types, according to the age of onset of the deficiencies and their severity. I is characterized by severe and congenital loss of hearing, absence of vestibular function and appearance of retinitis pigmentosa in the first decade of life; II presents moderate congenital loss of hearing, preserved vestibular function and onset of pigmentary retinitis in the second decade of life; III presents late progressive hearing loss, vestibular ataxia, onset of pigmentary retinitis at puberty, and may have psychosis; IV, the rarest , with severe hearing loss, retinitis pigmentosa and mental retardation. Early diagnosis is essential for the otologic therapeutic plan, which may include auditory protection with audiologic rehabilitation.

Conclusion: As a rare condition with different presentations, it can have a delayed diagnosis, compromising the patient's early therapy, thus influencing his quality of life.