![]() The qualifier pigmentosa reflects the fact that clumps of pigment may be visible by an ophthalmoscope in advanced stages of degeneration. The gradual vision loss of this syndrome is associated with retinitis pigmentosa, which is a degeneration of the retinal cells. People with Usher syndrome III are not born deaf, but experience a gradual loss of their hearing and vision they may or may not have balance difficulties. People with Usher II are also born deaf, but do not seem to have noticeable problems with balance they also begin to lose their vision later (in the second decade of life) and may preserve some vision even into middle age. They also exhibit balance difficulties and learn to walk slowly as children, due to problems in their vestibular system. People with Usher I are born profoundly deaf, and begin to lose their vision in the first decade of life. Usher syndrome has three clinical subtypes, denoted as I, II and III in decreasing order of severity. Usher syndrome is incurable at present, but preliminary experiments with gene therapy in mice are promising. The word syndrome means that multiple symptoms occur together, in this case, deafness and blindness. Other names for Usher syndrome include Hallgren syndrome, Usher-Hallgren syndrome, rp-dysacusis syndrome and dystrophia retinae dysacusis syndrome. ![]() Usher syndrome (sometimes referred to as " Usher's syndrome") is a relatively rare inherited condition that is a leading cause of deaf-blindness. ![]()
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