Visual acuity loss in patients with Usher's syndrome

L. Piazza, G. A. Fishman, M. Farber, D. Derlacki and R. J. Anderson

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.

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Usher syndromes due to MYO7A, PCDH15, USH2A or GPR98 mutations share retinal disease mechanism
Jacobson et al.
Hum Mol Genet 2008;17:2405-2415.
ABSTRACT | FULL TEXT  

Retinal Disease in Usher Syndrome III Caused by Mutations in the Clarin-1 Gene
Herrera et al.
IOVS 2008;49:2651-2660.
ABSTRACT | FULL TEXT  

Disease Expression in Usher Syndrome Caused by VLGR1 Gene Mutation (USH2C) and Comparison with USH2A Phenotype
Schwartz et al.
IOVS 2005;46:734-743.
ABSTRACT | FULL TEXT  

Kinetics of Visual Field Loss in Usher Syndrome Type II
Iannaccone et al.
IOVS 2004;45:784-792.
ABSTRACT | FULL TEXT  

Usher syndrome in the city of Birmingham---prevalence and clinical classification
Hope et al.
Br. J. Ophthalmol. 1997;81:46-53.
ABSTRACT | FULL TEXT