The overall goal of this research program is to develop novel methodologies with enhanced sensitivity for use in evaluating visual function, assessing the efficacy of therapeutic interventions, and providing new insight into the underlying causes of vision loss in ocular diseases. The goal of this proposal is to identify the mechanisms that form the basis for the spatial contrast sensitivity deficits observed in patients with retinitis pigmentosa (RP), a group of hereditaty retinal degenerations that are that are the most common genetic cause of blindness in adults. This goal Will be achieved by completing two specific aims:
Aim 1 is to develop a computerimplemented vlsual-riolse-based testing strategy. Visual-noise-based techniques can provide more information than standard clinical tests because perfomnance is factored into the components that govern contrast sensitivity. However, several issues regarding visual-noise-based approaches have been identified in pilot work that must be resolved.
Aim 2 will apply the novel testing strategy to individuals with RP to test three distinct, non-mutually exclusive hypotheses regarding tiie factors underlying their contrast sensitivity losses: patients with RP have elevated levels of additive noise within the visual pathway, patients with RP have elevated levels of multiplicative noise within the visual pathway, or patients with RP have an Inability to extract signal information from the noise. In addition to providing a better understanding ofthe source of contrast sensitivity losses In patients with RP, this research will yield a sensitive technique that will be useful for evaluating the efficacy of future therapeutic interventions in a variety of ocular disorders. The development of more sensitive metfiods of assessing visual function, such as that proposed here, is becoming increasingly important as gene-directed therapies and retinal cell transplantation therapies begin to enter clinical trials.
(RELEVANCE) This study will develop a test that will permit the factors underlying contrast sensitivity deficits in patients with retinitis pigmentosa to be defined. This research addresses the need for Improved testing strategies with enhanced sensitivity for evaluating ocular disease progression, subtyping patients for inclusion In treatment trails, and In assessing the outcomes of future therapeutic interventions. Novel tests with enhanced sensitivity are becoming increasingly needed as gene-directed therapies and retinal cell transplantation therapies begin to enter clinical trials.
|Patangay, Shresta; Derafshi, Zahra; Vajaranant, Thasarat S et al. (2018) Three Dimensional Stimulus Source for Pattern Electroretinography in Mid- and Far-peripheral Retina. Transl Vis Sci Technol 7:8|
|Collison, Frederick T; Park, Jason C; Fishman, Gerald A et al. (2016) Two-color pupillometry in enhanced S-cone syndrome caused by NR2E3 mutations. Doc Ophthalmol 132:157-66|
|McAnany, J Jason; Park, Jason C; Collison, Frederick T et al. (2016) Abnormal 8-Hz flicker electroretinograms in carriers of X-linked retinoschisis. Doc Ophthalmol 133:61-70|
|Park, Jason C; Moss, Heather E; McAnany, J Jason (2016) The Pupillary Light Reflex in Idiopathic Intracranial Hypertension. Invest Ophthalmol Vis Sci 57:23-9|
|Collison, Frederick T; Park, Jason C; Fishman, Gerald A et al. (2015) Full-Field Pupillary Light Responses, Luminance Thresholds, and Light Discomfort Thresholds in CEP290 Leber Congenital Amaurosis Patients. Invest Ophthalmol Vis Sci 56:7130-6|
|McAnany, J Jason; Park, Jason C; Cao, Dingcai (2015) Rod- and cone-isolated flicker electroretinograms and their response summation characteristics. Vis Neurosci 32:E018|
|Hall, Cierra; Wang, Shu; McAnany, J Jason (2015) Individual Letter Contrast Thresholds: Effect of Object Frequency and Noise. Optom Vis Sci 92:1125-32|
|Park, Jason C; McAnany, J Jason (2015) Effect of stimulus size and luminance on the rod-, cone-, and melanopsin-mediated pupillary light reflex. J Vis 15:|
|Moss, Heather E; Park, Jason C; McAnany, J Jason (2015) The Photopic Negative Response in Idiopathic Intracranial Hypertension. Invest Ophthalmol Vis Sci 56:3709-14|
|Park, Jason C; Cao, Dingcai; Collison, Frederick T et al. (2015) Rod and cone contributions to the dark-adapted 15-Hz flicker electroretinogram. Doc Ophthalmol 130:111-9|
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