Visual acuity loss in patients with Usher's syndrome.
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.
D000206:Actuarial Analysis; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002648:Child; D003638:Deafness / Q000150:complications / Q000151:congenital* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012174:Retinitis Pigmentosa / Q000150:complications / Q000503:physiopathology*; D013577:Syndrome; D014786:Vision Disorders / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D014792:Visual Acuity