期刊
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE
卷 55, 期 2, 页码 832-840出版社
ASSOC RESEARCH VISION OPHTHALMOLOGY INC
DOI: 10.1167/iovs.13-12919
关键词
supernormal rod ERG; cone dystrophy; cone-rod dystrophies; flicker sensitivity; critical flicker fusion; temporal acuity; temporal processing; KCNV2 gene
资金
- Fight for Sight, Biotechnology and Biological Sciences Research Council
- Engineering and Physical Sciences Research Council
- National Institute for Health Research Biomedical Research Centre at Moorfields Eye Hospital National Health Service Foundation Trust
- University College London Institute of Ophthalmology
- Foundation for Fighting Blindness Research Center
- Foundation Fighting Blindness Career Development Award
- Biotechnology and Biological Sciences Research Council [BB/I003444/1] Funding Source: researchfish
- Engineering and Physical Sciences Research Council [EP/J005193/1] Funding Source: researchfish
- National Institute for Health Research [NF-SI-0507-10204] Funding Source: researchfish
- BBSRC [BB/I003444/1] Funding Source: UKRI
- EPSRC [EP/J005193/1] Funding Source: UKRI
PURPOSE. We report a psychophysical investigation of 5 observers with the retinal disorder cone dystrophy with supernormal rod ERG, caused by mutations in the gene KCNV2 that encodes a voltage-gated potassium channel found in rod and cone photoreceptors. We compared losses for rod-and for cone-mediated vision to further investigate the disorder and to assess whether the supernormal ERG is associated with any visual benefit. METHODS. L-cone, S-cone, and rod temporal acuity (critical flicker fusion frequency) were measured as a function of target irradiance; L-cone temporal contrast sensitivity was measured as a function of temporal frequency. RESULTS. Temporal acuity measures revealed that losses for vision mediated by rods, S-cones, and L-cones are roughly equivalent. Further, the gain in rod function implied by the supernormal ERG provides no apparent benefit to near-threshold rod-mediated visual performance. The L-cone temporal contrast sensitivity function in affected observers was similar in shape to the mean normal function but only after the mean function was compressed by halving the logarithmic sensitivities. CONCLUSIONS. The name of this disorder is potentially misleading because the comparable losses found across rod and cone vision suggest that the disorder is a generalized cone-rod dystrophy. Temporal acuity and temporal contrast sensitivity measures are broadly consistent with the defect in the voltage-gated potassium channel producing a nonlinear distortion of the photoreceptor response but after otherwise normal transduction processes.
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