Longterm visual prognosis in Usher syndrome types 1 and 2

André M. Sadeghi, Kristina Eriksson, William J. Kimberling, Anders Sjöström, Claes Möller

Research output: Contribution to journalArticle

24 Scopus citations

Abstract

Purpose: To estimate the age at diagnosis of retinitis pigmentosa and to determine visual acuity deterioration, visual field impairment and the frequency of cataracts in Usher syndrome types 1 and 2. Methods: We carried out a retrospective study of 328 affected subjects with Usher syndrome types 1 and 2. Study subjects were divided into seven different age groups by decade. Data were analysed using descriptive statistics, general linear model anova and survival analysis. Results: Retinitis pigmentosa was diagnosed significantly earlier in subjects with Usher syndrome type 1 than in those with type 2. Visual acuity was significantly more impaired in affected subjects with Usher syndrome type 1 than in those with type 2 from 50 years of age onwards. Survival analysis revealed a significant difference in visual field loss (≤ 10 degrees) between the two groups, with type 2 subjects tending to be more impaired, while comparison indicated no significant differences between the groups in any of the other visual field categories. Cataract was found to be generally more common in Usher syndrome type 1 than type 2. Conclusions: Progressive loss of visual acuity and visual field begins to be substantial between the second and third decades of life in both Usher types. The rate of degeneration varies between individuals in both groups. The data are useful for the counselling of affected subjects with Usher syndrome types 1 and 2.

Original languageEnglish (US)
Pages (from-to)537-544
Number of pages8
JournalActa Ophthalmologica Scandinavica
Volume84
Issue number4
DOIs
StatePublished - Aug 2006

Keywords

  • Cataract
  • Retinitis pigmentosa
  • Usher syndrome
  • Visual acuity loss
  • Visual field loss

ASJC Scopus subject areas

  • Ophthalmology

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    Sadeghi, A. M., Eriksson, K., Kimberling, W. J., Sjöström, A., & Möller, C. (2006). Longterm visual prognosis in Usher syndrome types 1 and 2. Acta Ophthalmologica Scandinavica, 84(4), 537-544. https://doi.org/10.1111/j.1600-0420.2006.00675.x