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Usher syndrome is a group of autosomal recessive disorders involving progressive degeneration of the retina that leads to severe visual impairment, deafness, and variable degrees of vestibular dysfunction. These disorders are divided into three clinical classes and are differentiated by the severity and progression of both the hearing loss and visual impairment and by the absence or presence of vestibular symptoms.
Usher syndrome type 1: It is characterized by profound congenital deafness, pre-pubertal onset of retinitis pigmentosa, and vestibular dysfunction.
Usher syndrome type 2: It is characterized by congenital moderate to severe deafness, early onset of retinitis pigmentosa in the first to second decade of life, and no vestibular dysfunction.
Usher syndrome type 3: It is characterized by variable onset of deafness and of retinitis pigmentosa, and variable impairment of vestibular function.