Relationship Between Foveal Cone Structure and Visual Acuity Measured With Adaptive Optics Scanning Laser Ophthalmoscopy in Retinal Degeneration

PURPOSE: To evaluate foveal function in patients with inherited retinal degenerations (IRD) by measuring visual acuity (VA) after correction of higher-order aberrations. METHODS: Adaptive optics scanning laser ophthalmoscopy (AOSLO) was used to image cones in 4 healthy subjects and 15 patients with IRD. The 840-nm scanning laser delivered an “E” optotype to measure AOSLO-mediated VA (AOSLO-VA). Cone spacing was measured at the preferred retinal locus by two independent graders and the percentage of cones below the average density of 47 age-similar healthy subjects was computed. Cone spacing was correlated with best-corrected VA measured with the Early Treatment of Diabetic Retinopathy Study protocol (ETDRS-VA), AOSLO-VA, and foveal sensitivity. RESULTS: ETDRS-VA significantly correlated with AOSLO-VA (ρ = 0.79, 95% confidence interval [CI] 0.5–0.9). Cone spacing correlated with AOSLO-VA (ρ = 0.54, 95% CI 0.02–0.7), and negatively correlated with ETDRS letters read (ρ = −0.64, 95% CI −0.8 to −0.2). AOSLO-VA remained ≥20/20 until cones decreased to 40.2% (CI 31.1–45.5) below normal. Similarly, ETDRS-VA remained ≥20/20 until cones were 42.0% (95% CI 36.5–46.1) below normal. Cone spacing z scores negatively correlated with foveal sensitivity (ρ = −0.79, 95% CI −0.9 to −0.4) and foveal sensitivity was ≥35 dB until cones were 43.1% (95% CI 39.3–46.6) below average. CONCLUSIONS: VA and foveal cone spacing were weakly correlated until cones were reduced by 40% to 43% below normal. The relationship suggests that VA is an insensitive measure of foveal cone survival; cone spacing may be a more sensitive measure of cone loss.