Novel mobility test to assess functional vision in patients with inherited retinal dystrophies.

ABSTRACT

IMPORTANCE This novel endpoint tracks functional vision changes in patients with inherited retinal dystrophies (IRDs) over time. BACKGROUND: The aims of the study were to determine whether a multi-luminance mobility test (MLMT) can detect functional vision changes over time in subjects with IRDs and to assess natural history and potential effects of investigational agents. DESIGN: This is a prospective, observational study. PARTICIPANTS: Sixty-two subjects were enrolled. Sixty (29 normal sighted and 31 visually impaired) were eligible; 54 (28 visually impaired and 26 normal-sighted) completed all testing visits. METHODS: Subjects navigated MLMT courses three times over 1 year. At each visit, subjects completed testing using individual eyes, and both eyes, at up to nine standardized, increasing luminance levels (range 1 to 400 lux). Accuracy and speed were evaluated and compared with visual acuity (VA), visual field (VF) and a visual function questionnaire. MAIN OUTCOME MEASURES: Accuracy and speed of normal and visually impaired subjects on MLMT, and reliability and content validity of MLMT were the main outcome measures. RESULTS: MLMT distinguished normal-sighted from visually impaired subjects. All control subjects passed all MLMT attempts at all tested light levels. Visually impaired subjects' performance varied widely; some declined over 1 year. Performance declined markedly below certain VA and VF thresholds. Concordance on performance on two baseline visits was high correlations for accuracy were 94% and 98% for lowest common and highest common lux levels. CONCLUSIONS AND RELEVANCE MLMT differentiated visually impaired from control populations and, in visually impaired subjects, identified a range of performances; and tracked performance declines over time, consistent with these progressive conditions.