Double Masked Period: Photopic Negative Response Electroretinography (PhNR-ERG) A-wave Amplitude
Change from baseline in photopic negative response electroretinography (PhNR-ERG) a-wave amplitude by Visit. Photopic negative response electroretinography (PhNR-ERG) assesses retinal cell function. A decrease in the amplitude of the a-wave is associated with worse outcomes. Change from baseline in a-wave amplitude: a more positive number equals a decrease in amplitude, and therefore a worse outcome, a more negative number mean a better outcome.
Open Label Extension Period: Photopic Negative Response Electroretinography (PhNR-ERG) A-wave Amplitude
Change from baseline in photopic negative response electroretinography (PhNR-ERG) a-wave Amplitude by Visit. Photopic negative response electroretinography (PhNR-ERG) assesses retinal cell function. A decrease in the amplitude of the a-wave is associated with worse outcomes. Change from baseline in a-wave amplitude: a more positive number equals a decrease in amplitude, and therefore a worse outcome, a more negative number mean a better outcome.
Double Masked Period: Photopic Negative Response Electroretinography (PhNR-ERG) B-wave Amplitude
Change from baseline in photopic negative response electroretinography (PhNR-ERG) b-wave amplitude by visit. Photopic negative response electroretinography (PhNR -ERG) assesses retinal cell function. A decrease in the amplitude of the b-wave is associated with worse outcomes. Change from baseline in b-wave amplitude: a more negative number equals a decrease in amplitude, and therefore a worse outcome, a more positive number mean a better outcome.
Open Label Extension Period: PhNR-ERG B-wave Amplitude
Change from baseline in photopic negative response electroretinography (PhNR-ERG) b-wave Amplitude by Visit. Photopic negative response electroretinography (PhNR -ERG) assesses retinal cell function. A decrease in the amplitude of the b-wave is associated with worse outcomes. Change from baseline in b-wave amplitude: a more negative number equals a decrease in amplitude, and therefore a worse outcome, a more positive number means a better outcome.
Double Masked Period: PhNR Amplitude
Double Masked Period: Change from baseline in PhNR Amplitude by Visit. Photopic negative response electroretinography (PhNR -ERG) assesses retinal cell function. A decrease in PhNR amplitude means worse outcome. Change from baseline: A more negative number means a better outcome, a more positive number means a worse outcome.
Open Label Extension Period: PhNR Amplitude
Open Label Extension Period: Change from baseline in PhNR Amplitude by Visit. Photopic negative response electroretinography (PhNR -ERG) assesses retinal cell function. A decrease in PhNR amplitude means worse outcome. Change from baseline: A more negative number means a better outcome, a more positive number means a worse outcome.
Double Masked Period: PhNR/B-wave Amplitude Ratio
Double Masked Period: Change from baseline in PhNR/b-wave amplitude ratio by Visit. Photopic negative response electroretinography (PhNR assesses retinal cell function. A decrease in PhNR amplitude means worse outcome.
Open Label Extension Period: Change From Baseline by Visit PhNR/b Wave Amplitude Ratio
Open Label Extension Period: Change from baseline by visit PhNR/b Wave Amplitude Ratio by visit. A reduced amplitude ratio equals worse outcome.
Double Masked Period: Change From Baseline by Visit PhNR Peak to Trough Adjusted for A-wave Amplitude
Double Masked Period: Change from baseline by visit in PhNR Peak to Trough Adjusted for a-wave Amplitude by Visit. Calculated as (b-wave amplitude - PhNR amplitude)/a-wave amplitude. A reduced amplitude ratio equals worse outcome.
Open Label Extension Period: Change From Baseline by Visit PhNR Peak to Trough Adjusted for A-wave Amplitude
Open Label Extension Period: Change from baseline by visit in PhNR Peak to Trough Adjusted for a-wave Amplitude by Visit. [Calculated by b-wave amplitude - PhNR amplitude)/a-wave amplitude.] Photopic negative response electroretinography (PhNR-ERG) assesses retinal cell function. A decrease in the amplitude of the a-wave is associated with worse outcomes. A reduced amplitude ratio equals worse outcome.
Double Masked Period: PhNR Peak to Trough Amplitude (Unadjusted) From Baseline
Double Masked Period: Change from baseline in PhNR Peak to Trough Amplitude (Unadjusted) by Visit. Calculated as b-wave amplitude - PhNR amplitude. Photopic negative response electroretinography (PhNR-ERG) assesses retinal cell function. A decrease in amplitude is associated with worse outcomes.
Open Label Extension Period: PhNR Peak to Trough Amplitude (Unadjusted)
Open Label Extension Period: Change from baseline in PhNR Peak to Trough Amplitude (Unadjusted) by Visit. b-wave amplitude - PhNR amplitude. Photopic negative response electroretinography (PhNR-ERG) assesses retinal cell function. A decrease in the amplitude of the a-wave is associated with worse outcomes.
Double Masked: VFQ-39 Composite
Double Masked: Change from baseline in Visual Function Questionnaire (VFQ-39) Composite score by visit. National Eye Institute VFQ-39 measures health-related quality of life in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, peripheral vision, and composite score. For each domain: the lowest and highest possible scores are 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 Composite Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Composite score.National Eye Institute VFQ-39 measures health-related quality of life in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, peripheral vision, and composite score. For each domain: the lowest and highest possible scores are 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 General Health Score
Change from baseline in Visual Function Questionnaire (VFQ-39) General Health score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked: VFQ-39 General Vision Score
Change from baseline in VFQ-39 General Vision Score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculate
Open Label Extension Period: VFQ-39 General Vision Score
Change from baseline in VFQ-39 General Vision score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculate
Double Masked: VFQ-39 Ocular Pain Score
Change from baseline in VFQ-39 Ocular Pain score. National Eye Institute VFQ-39 measures health-related quality of life in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, peripheral vision, and composite score. For each domain: the lowest and highest possible scores are 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 Ocular Pain Score
Change from baseline in VFQ-39 Ocular Pain score by visit. National Eye Institute VFQ-39 measures health-related quality of life in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, peripheral vision, and composite score. For each domain: the lowest and highest possible scores are 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked: VFQ-39 Near Activities Score
Change from Baseline in VFQ-39 Near Activities Score from Baseline. National Eye Institute VFQ-39 measures health-related quality of life in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, peripheral vision, and composite score. For each domain: the lowest and highest possible scores are 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 Near Activities
Change from Baseline in Visual Function Questionnaire (VFQ-39) Near Activities score.. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; score ranges from 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked Period: Mean Retinal Nerve Fiber (RNFL) Layer Thickness
Change from baseline in retinal nerve fiber layer thickness by spectral domain optical coherence tomography (SD-OCT). RNFL measures the loss of retinal ganglion cell axons. RNFL thickness decreases as disease progresses. A positive number, or, absence of change from baseline reflects a good clinical outcome, a negative number reflects loss of thickness, or a bad outcome.
Open Label Extension Period: Retinal Nerve Fiber Layer Thickness
Open Label Extension Period: Change from baseline in Retinal Nerve Fiber Layer Thickness by SD-OCT by visit. Change from baseline in retinal nerve fiber layer thickness by spectral domain optical coherence tomography (SD-OCT). RNFL measures the loss of retinal ganglion cell axons. RNFL thickness decreases as disease progresses. A positive number, or, absence of change from baseline reflects a good clinical outcome, a negative number reflects loss of thickness, or a bad outcome.
Double Masked Period: Visual Field Mean Deviation as Measured by Humphrey Automated Visual Field Testing Stimulus III
Change from Baseline in visual field Mean Deviation(MD) as measured by Humphrey automated visual field testing stimulus III by visit. The Humphrey visual field test measures the entire area of peripheral vision that can be seen while the eye is focused on a central point. Mean deviation (MD) is the mean deviation in the patient's results compared to those expected from the age-matched normative database. Lower/More negative scores mean worse outcome, higher/more positive score means better outcome.
Open Label Extension Period: Visual Field Mean Deviation (dB) Measured by Humphrey From Baseline
Change from Baseline in visual field Mean Deviation(MD) as measured by Humphrey automated visual field testing stimulus III by visit. The Humphrey visual field test measures the entire area of peripheral vision that can be seen while the eye is focused on a central point. Mean deviation (MD) is the mean deviation in the patient's results compared to those expected from the age-matched normative database. Lower/More negative scores mean worse outcome, higher/more positive score means better outcome.
Double Masked Period: Color Discrimination - Number of Plates
Double Masked Period: Change from Baseline in Color Discrimination - Number of Plates from Baseline by Visit by Ishihara Test. Score range: 0-38. Higher number equals better color discrimination equals better outcome. Lower number equals worse color discrimination equals worse outcome.
Open Label Extension Period: Change From Baseline in Color Discrimination - Number of Plates by Visit
Open Label Extension Period: Change from Baseline in Color Discrimination - Number of Plates from Baseline by Visit by Ishihara Test. Score range: 0-38. Higher number equals better color discrimination equals better outcome. Lower number equals worse color discrimination equals worse outcome.
Double Masked Period: Change From Baseline in Contrast Sensitivity by Pelli-Robson Contrast Sensitivity Chart by Visit
Letters are arranged on a 60 x 85 cm chart in sets of triplets which contain the same contrast, decreasing in log10 contrast from top to bottom and left to right as participant progresses from one triplet to the other. Each group of three letters is decreased in contrast by a factor 0.71 (1/√2) (log contrast 0.15) of the proceeding set. Participant reads letters, starting with highest contrast, until unable to read letters in a single group. Score is based on the contrast of the last group read correctly.The size of the letters on the chart subtend 0.5 degrees at 3m, The test is scored in LogCS units, where each set of triplets advances in steps of 0.15 log units ranging from LogCS of 0.00 (approx. 100% contrast) to LogCS 2.25 (approx. 0.56% contrast). Change from baseline: the more negative the number means poor outcome, the more positive the number means better outcome.
Open Label Extension Period: Change From Baseline in Contrast Sensitivity by Visit
Letters are arranged on a 60 x 85 cm chart in sets of triplets which contain the same contrast, decreasing in log10 contrast from top to bottom and left to right as participant progresses from one triplet to the other. Each group of three letters is decreased in contrast by a factor 0.71 (1/√2) (log contrast 0.15) of the proceeding set. Participant reads letters, starting with highest contrast, until unable to read letters in a single group. Score is based on the contrast of the last group read correctly. The size of the letters on the chart subtend 0.5 degrees at 3m, The test is scored in LogCS units, where each set of triplets advances in steps of 0.15 log units ranging from LogCS of 0.00 (approx. 100% contrast) to LogCS 2.25 (approx. 0.56% contrast). Change from baseline: the more negative the number means poor outcome, the more positive the number means better outcome.
Double Masked Change From Baseline in Retinal Ganglion Cell Layer Thickness
Change from baseline in Retinal Ganglion Cell Layer Thickness by SD-OCT From Baseline by Visit
Open Label Extension Period: Change From Baseline in Retinal Ganglion Cell Layer Thickness
Open Label Extension Period: Change from baseline in Retinal Ganglion Cell Layer Thickness by SD-OCT by SD-OCT by visit
Double Masked: VFQ-39 Distance Activities Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Distance Activities score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 Distance Activities Score
Change in Visual Function Questionnaire (VFQ-39) Distance Activities Score.National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 Social Functioning Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Social Functioning Score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked: VFQ-39 Social Functioning Score.
Change from baseline in Visual Function Questionnaire (VFQ-39) Social Functioning score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked: VFQ-39 Mental Health Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Mental Health score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 Mental Health Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Mental Health Score . National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked: VFQ-39 Role Difficulties Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Role Difficulties score National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 Role Difficulties Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Role Difficulties Score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period:VFQ-39 Dependency Score
Change from baseline in Visual Function Questionnaire (VFQ-39 ) Dependency Score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked: VFQ-39 Dependency Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Dependency score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked: VFQ-39 Color Vision Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Color Vision score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: VFQ-39 Color Vision Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Color Vision score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked: VFQ-39 Peripheral Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Peripheral Score . National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Open Label Extension Period: Visual Function Questionnaire (VFQ-39) Peripheral Score
Change from baseline in Visual Function Questionnaire (VFQ-39) Peripheral Score . National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.
Double Masked Period: Change in Intraocular Pressure (IOP)
Double Masked Period: Change in intraocular Pressure (IOP) in mmHg from Baseline by Visit
Open Label Extension Period: Intraocular Pressure (IOP)
Change from baseline in Intraocular Pressure (IOP) in mmHg by Visit.
OLE: Slit Lamp Examination (SLE)-Vehicle Eye
Open Label Extension: Shift in Slit Lamp Examination from Baseline by Visit: number of eyes that changed from normal or abnormal not clinically significant, to abnormal clinically significant for Vehicle Eyes
OLE: Slit Lamp Examination (SLE)-Elamipretide Eye
Open Label Extension: Shift in Slit Lamp Examination from Baseline by Visit: number of eyes that changed from normal or abnormal not clinically significant, to abnormal clinically significant for Elamipretide Eyes
Double Masked: Slit Lamp Changes From Baseline: Vehicle Eye and Elamipretide Eye
Incidence of Change from normal or Abnormal, clinically insignificant to normal clinically significant
Double Masked: Dilated Fundus Changes From Baseline: Vehicle Eye and Elamipretide Eyes
Double Masked: Dilated Fundus Changes from Baseline: Vehicle Eye and Elamipretide Eyes Incidence of Change from normal or Abnormal-clinically insignificant, to normal clinically significant
Open Label Extension Period: Dilated Fundus Changes From Baseline: Vehicle Eye and Elamipretide Eyes
Incidence of Change from normal or Abnormal-clinically insignificant, to normal clinically significant
Double Masked: VFQ-39 General Health Score
Change from baseline in Visual Function Questionnaire (VFQ-39) General Health score. National Eye Institute VFQ-39 score measures health-related quality of life of subjects with visual impairment, in 12 domains: general vision, ocular pain, near activities, distance activities, vision-specific social functioning, vision-specific mental health, vision-specific role difficulties, vision-specific dependency, driving, color vision, and peripheral vision and 1 composite score. Each domain is converted to a 0 to 100 scale; the lowest and highest possible scores are set at 0 and 100 points, respectively. Higher score means higher functioning. Scores represent the achieved percentage of the total possible score, e.g. a score of 50 represents 50% of the highest possible score. For the composite score, the vision-targeted sub-scale scores are averaged, excluding the general health questions. Domain scores from each cohort are averaged and the change from baseline per domain is calculated.