Evaluation of photoreceptor function in inherited retinal diseases using rod- and cone-enhanced flicker stimuli

Purpose: Clinical assessment of rod and cone photoreceptor sensitivity often involves the use of extended dark adaptation times to minimise cone involvement or the use of bright adapting backgrounds to saturate rods. In this study we examine a new rod/cone sensitivity test, which requires minimal dark adaptation. The aim was to establish whether rod/cone sensitivity losses could be measured reliably in patients with retinal diseases that selectively affect rods or cones when compared to age-matched subjects with normal vision.

Methods: Flicker modulation thresholds (FMTs) were measured psychophysically, using cone- and rod-enhanced stimuli located centrally, and in four quadrants, at 5° retinal eccentricity in 20 patients (age range: 10-41 years) with cone-dominated (Stargardt's disease or macular dystrophy; n = 13) and rod-dominated (retinitis pigmentosa; n = 7) disease. These data were compared against age-matched normals tested with identical stimuli.

Results: Across all retinal locations, cone FMTs in cone-dominated diseases (Median ± IQR: 32.32 ± 28.15% for central location) were greater than a majority (83%; 49/59) of corresponding rod FMTs (18.7 ± 3.29%; p = 0.05) and cone FMTs of controls (4.24 ± 2.00%). Similarly, rod FMTs in rod-dominant disease (14.99 ± 22.58%) were greater than a majority (88%; 29/39) of the corresponding cone FMTs (9.09 ± 10.33%) (p = 0.13) and rod FMT of controls (6.80 ± 2.60 %).

Conclusions: Cone-specific deficits were larger than rod-specific deficits in cone-dominated diseases, and vice versa in rod-dominated disease. These results suggest that the new method of assessing photoreceptor sensitivity has potential application in detecting specific rod/cone losses without the need for dark adaptation.