• Using a life-table analysis, visual acuity loss with age was determined for a population of 106 patients with type 1 (N = 35) and type 2 (N = 71) Usher's syndrome. The cumulative percentage of patients maintaining visual acuity of 6/12 (20/40) or better in at least one eye by age 29 years was 69% for type 1 patients and 94% for type 2 patients. The cumulative percentage maintaining visual acuity of 6/24 (20/80) or better at this age was 89% for type 1 and 98% for type 2 patients. Within each subtype, the cumulative percentage maintaining 6/60 (20/ 200) or better was similar to the percentage maintaining 6/24 (20/80) or better. Knowledge of these data provides an objective basis for counseling patients with Usher's syndrome about their probability of developing loss of central visual acuity with age.
Piazza L, Fishman GA, Farber M, Derlacki D, Anderson RJ. Visual Acuity Loss in Patients With Usher's Syndrome. Arch Ophthalmol. 1986;104(9):1336-1339. doi:10.1001/archopht.1986.01050210090031