Each of these reproduces the characteristic early-onset hearing loss and vestibular defects as found in USH1 patients; however, none of these mouse models shows retinal degeneration, with the exception of the Ush1c knock-in mouse, generated to study the c.216G>A mutation, which displays hearing loss, vestibular defects and retinal degeneration [167]. This evidence concerns the gene USH1C and retinal degeneration.