Rate of Progression in USH2A-related Retinal Degeneration

Inclusion Criteria: * Willing and able to complete the informed consent process * Ability to return for all study visits over 48 months if in the natural history study * Age ≥ 8 years * At least 2 pathogenic or likely pathogenic mutations in USH2A gene from a clinically certified lab report Ocular Inclusion Criteria Both eyes must meet all of the following: * Clinical diagnosis of a rod-cone degeneration * Clear ocular media and adequate pupil dilation to permit good quality photographic imaging * Ability to perform kinetic and static perimetry reliably Exclusion Criteria: * Mutations in genes that cause autosomal dominant RP, X-linked RP, or presence of biallelic mutations in autosomal recessive RP/retinal dystrophy genes other than USH2A * Expected to enter experimental treatment trial at any time during this study * History of more than 1 year of cumulative treatment, at any time, with an agent associated with pigmentary retinopathy (including hydroxychloroquine, chloroquine, thioridazine, and deferoxamine) Ocular Exclusion Criteria If either eye has any of the following, the patient is not eligible: * Current vitreous hemorrhage * Current or any history of rhegmatogenous retinal detachment * Current or any history of (e.g., prior to cataract or refractive surgery) spherical equivalent of the refractive error worse than -8 Diopters of myopia * History of intraocular surgery (e.g., cataract surgery, vitrectomy, penetrating keratoplasty, or LASIK) within the last 3 …