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Longterm visual prognosis in Usher syndrome types 1 and 2
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2006 (English)In: Acta Ophthalmologica Scandinavica, ISSN 1395-3907, E-ISSN 1600-0420, Vol. 84, no 4, 537-544 p.Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
Copenhagen: Blackwell Munksgaard , 2006. Vol. 84, no 4, 537-544 p.
National Category
Medical and Health Sciences
Research subject
Medicine
Identifiers
URN: urn:nbn:se:oru:diva-10359DOI: 10.1111/j.1600-0420.2006.00675.xOAI: oai:DiVA.org:oru-10359DiVA: diva2:310228
Available from: 2010-04-13 Created: 2010-04-13 Last updated: 2016-12-16Bibliographically approved

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Möller, Claes
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