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PURPOSE: To assess the relationship between objective tests of visual function and vision-related quality of life in patients with Bothnia dystrophy (BD), a retinal dystrophy of retinitis pigmentosa type with progressive maculopathy.

METHODS: Forty-nine patients were tested. Weighted distance logMAR visual acuity (WVA), weighted logMAR low contrast VA (WCS), and binocular visual field (VF) areas were calculated. Vision-related quality of life (VRQL) was assessed using the 25-item National Eye Institute-Visual Function Questionnaire (NEI-VFQ-25). Correlation statistics were used and adjusted analyses of the relationship between the composite score and the objective visual function tests were performed with multiple linear regression.

RESULTS: VRQL was significantly correlated with age, WVA, WCS, and binocular VF areas (P < 0.001). Calculation of partial correlation coefficients showed age to be significantly correlated only with VF (V-4-e) area (P < 0.0001). Multiple linear regression analyses revealed age and WVA to be significantly associated with the NEI-VFQ-25 composite score (P < 0.02 and P = 0.001, respectively). WVA alone was the strongest predictor of self-reported experience of total visual function in BD patients (r 2= 0.69).

CONCLUSIONS: A strong relationship between objective tests of visual function and patient perceived VRQL as assessed by a questionnaire was found. WVA was the strongest predictor and together with age explained almost 70% of the variability of the composite score of the questionnaire.