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Article
June 1995

Prevalence of Foveal Lesions in Type 1 and Type 2 Usher's Syndrome

Author Affiliations

From the Department of Ophthalmology and Visual Sciences, UIC Eye Center, University of Illinois at Chicago College of Medicine (Dr Fishman and Ms Derlacki); the Division of Epidemiology-Biostatistics, University of Illinois at Chicago School of Public Health (Dr Anderson); and the Department of Ophthalmology, University of Arkansas for Medical Sciences, Little Rock (Dr Lam).

Arch Ophthalmol. 1995;113(6):770-773. doi:10.1001/archopht.1995.01100060096040
Abstract

Purpose:  To evaluate possible differences in the prevalence of clinically detectable foveal lesions between patients with type 1 and type 2 Usher's syndrome.

Methods:  Records of 48 patients with type 1 and 98 patients with type 2 Usher's syndrome were retrospectively evaluated for the presence of a foveal lesion. The age, gender, and racial distribution of patients were similar in the two subtypes. Two investigators reviewed fundus photographs from all patients and, when available, fluorescein angiograms.

Results:  In the 48 patients with type 1 Usher's syndrome, 30 (62%) showed a clinically apparent atrophic- or cystic-appearing foveal lesion, whereas in the 98 patients with type 2 Usher's syndrome, 33 (34%) had either an atrophic- or a cystic-appearing foveal lesion. Logistic regression analysis showed that the probability of exhibiting a foveal lesion in both type 1 and type 2 Usher's syndrome increases with age and that patients with type 1 Usher's syndrome are more likely to have a foveal lesion than are patients with type 2 Usher's syndrome.

Conclusions:  Patients with type 1 Usher's syndrome show a greater probability of having either an atrophicor cystic-appearing foveal lesion than do patients with type 2 Usher's syndrome. This higher prevalence of foveal lesions is consistent with a previous observation that the severity of visual acuity impairment with age is greater for patients with type 1 than type 2 Usher's syndrome. These data are useful in counseling such patients as to their prognosis for central visual function.

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