Clinical trials to arrest, slow, prevent, or reverse blinding retinal degenerative diseases are on the horizon and are already increasing in number. Given the possible high-risk nature of some therapies, legally blind subjects with advanced eye disease are likely to be the participants in early phases of such trials; thus, these trials require reliable and well-validated measures of visual function that are particularly well suited to quantify low levels of vision.1–3 Furthermore, evaluating remaining functional vision such as visual acuity (VA) and contrast sensitivity (CS) in persons with severe vision loss is central for determining rehabilitative strategies. The ability of a person with severe vision impairment to resolve details in daily living (e.g., reading) is assessed by measures of VA, whereas the ability of a person to detect objects relative to their background (e.g., walking down steps) is measured by CS.4 Accurately evaluating visual function in these subjects requires outcome measures of VA and CS that can accommodate subjects with vision at or beyond the limits of charts established for use in clinical practice and previous clinical trials.5 The Early Treatment of Diabetic Retinopathy Study (ETDRS) chart used to measure VA and the Pelli-Robson (PR) chart used to measure CS are both standardized and validated in clinical practice, yet are suitable for monitoring VA and CS changes only in patients with VA >20/1600 (at 0.5 m) and >20/700 (at 1 m), respectively. Beyond this range, clinicians typically default to estimates such as “counting fingers” and “hand motion”; outcome measures in clinical trials require quantification, however. The range of functional deficits that accompany ocular disease may introduce other limitations for the use of letter charts. For patients with severely compromised visual fields or patients with large central or paracentral scotomas, the letters to be resolved might fall outside of the field of view or preferred retinal locus. For patients with poor acuity, resolving letters may be subject to the influence of nearby letters or high-contrast edges, and eye movements, collectively known as the “crowding effect,”6 and this effect is greater when letters are viewed in the periphery.7 The PR chart uses a single optotype size that may be at or beyond the patient’s level of VA in cases of severe vision loss, whereas it is possible for computer-based programs evaluating CS to adjust the test target size so that it is well below the patient’s threshold. Letter discrimination is dependent upon recognition of several features of a complex optotype, whereas an alternative to quantify vision beyond the range of letter charts that only requires the recognition of a single feature is a grating-based test to assess very low levels of VA.8 Discrimination of very large letter optotypes at a close test distance (e.g., at 50 cm) requires patients to effectively scan across a large area, which may be a difficult task for those with severely constricted visual fields or large central scotomas, and therefore gratings may be a better alternative. For patients with cataracts or other opacities, the use of gratings has been demonstrated to provide a better estimate of resolution.9 VA measured with gratings is less affected by optical defocus, which might be found in patients with various ocular diseases.10–12 Commercially available grating acuity tests, in particular the Teller acuity charts, have been extensively used among pediatric populations to quantify and monitor vision13 and as a vision screening test for cognitively impaired elderly in nursing homes.14 However, the use of such non-automated tests in clinical trials for low vision may be limited by interexaminer variability that could potentially influence acuity measures. The Berkeley Rudimentary Vision Test15 is a set of test cards that have been developed for subjects with more severe vision [using a single tumbling E up to 2.6 logMAR (20/8000) or gratings up to 2.9 logMAR (20/16,000) at 25 cm]. However, these tests depend on operator skill to vary the test stimuli according to patients’ responses (e.g., changing from optotype to gratings, changing or maintaining test distance, randomizing stimulus orientation order, and manual record keeping), and therefore do not lend themselves to standards of objectivity and rigorous statistics required in clinical research. Computer-based high-contrast square-wave grating tests presented on a liquid crystal display (LCD) monitor have been previously3 and are currently being used12 to quantify and monitor VA in clinical trials of retinal prostheses in patients with advanced retinitis pigmentosa (RP),2 and a grating CS test displayed on a video monitor has also been used to test a retinal degeneration patient1; however, these types of grating tests have not been independently validated outside of the clinical trials. The reliability of another newly developed VA test for patients with severe vision loss has been previously studied. The Freiburg Visual Acuity Test (FrACT) is a computerized VA test that presents Landolt C optotypes on a LCD screen using an adaptive staircase procedure and can quantify VA in the “hand motions” range between 20/2000 and 20/6060.16 However, the test-retest variability of this test in terms of a 95% confidence interval (CI) has been reported as being more variable than the ETDRS letter charts.17 The Frankfurt-Freiburg Contrast and Acuity Test is based on the FrACT and may have the potential to monitor CS in those with advanced vision loss, but this test’s capability has not yet been reported or validated. Variability of CS testing with the Frankfurt-Freiburg Contrast and Acuity Test in subjects without retinal disease has been reported to be about 1.5 to 2 times as high as with the PR letter charts.18 A computerized logMAR VA measurement system (COMPlog) was recently validated by Laidlaw et al.19 in amblyopic children and a group of ocular disease patients; however, this automated letter test only reaches a 1.68 logMAR (~20/1000) limit at 3 m and has not been validated to measure worse VA at closer test distances. Beyond this range, the program relies on scoring in terms of counting fingers or hand movements. The Basic Assessment of Light and Motion, a computerized test battery developed to evaluate temporal resolution, light perception, and localization of light and motion, was designed to measure improvements in visual function with a visual prosthesis and does not target VA or CS specifically.20 Furthermore, as VA and CS are complementary measures,8 validating tests for both VA and CS concurrently would be beneficial because ocular disease affects both measures to varying degrees. It is well known that both VA and CS are important predictors for the ability of low-vision patients to perform activities of daily living21 and mobility,22 thus validating both VA and CS tests together at more extreme ranges may be more clinically relevant. Previously described studies generally focus on validating one or the other. Clearly, a need still exists to establish reliable tests of VA and CS to monitor patients with severe vision loss. The Grating Acuity Test (GAT) and Grating Contrast Sensitivity (GCS) tests were developed to quantify varying levels of severe vision loss for use during a multicenter clinical trial of the Optobionics’ Artificial Silicon Retina for patients with RP.3 The GCS was designed to assess CS in subjects who were unable to provide meaningful results with the PR standardized test of CS, as a result of severely advanced disease resulting in reduced VA and CS. Our aim was to determine whether the grating tests may be used to follow VA and CS stability, and thus the clinical course of progressive deterioration or improvement (e.g., with effective treatment) of overall vision in legally blind subjects. To accomplish this goal, we assessed validity by comparing results with the new tests to results obtained with established VA and CS tests and the ETDRS and PR letter charts. Reliability was assessed by repeating measurements over time, both within and across sessions separated by 6 to 50 days.