Usher syndrome is a rare autosomal recessive genetic disorder characterized by the association of sensorineural deafness with retinitis pigmentosa and progressive vision loss.  Prevalence is estimated at 1 in 6,000 to 20,000 worldwide.  About 50% of all people who are deaf/blind have Usher syndrome.  Clinically, Usher syndrome has three clinical subtypes, namely USH1, USH2 and USH3.  Type 1 accounts for about 40% of cases, in which hearing loss is congenital, profound, nonprogressive, and typically associated with delayed development in motor skills, vestibular problems, and early onset of vision loss due to retinitis pigmentosa.There are seven distinct subtypes for USH1 from IA to IG.  Clinical diagnosis for type 1 is based on findings of bilateral sensorineural hearing loss symmetric, congenital and profound.