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| ID | Type | Description | Link |
|---|---|---|---|
| 000414-CH |
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Early termination due to low enrollment
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Background:
CLN3 involves vision loss observed around the preschool years, with eventual progression to blindness within 1-3 years. Researchers want to test an assistive device that may help children with CLN3 or with non-CLN3 related blindness.
Objective:
To learn if it is safe, easy, and useful for children with CLN3 or with non-CLN3 related blindness to use the OrCam.
Eligibility:
People aged 6-18 years who have either CLN3-related disease or non-CLN3 related blindness.
Design:
Participants will be screened with the following:
Medical history
Physical exam
Family history
Eye exam and vision tests. They will get eyedrops to dilate their eyes.
Psychological and neurocognitive tests. They will be asked questions and observed for how they do various tasks, such as talking, playing, writing, drawing, and solving problems.
Hearing tests. They may wear headphones or earplugs. Electrodes may be taped to their head.
Blood samples
Skin biopsy, if needed
Cheek cell, saliva, or urine samples
The OrCam is the size of and weighs about half as much as a pack of gum. It is attached to eyeglass frames by magnets. Participants will do tasks before and after they have been trained on the OrCam. They will do these tasks without or with using the OrCam.
Participants will be given an OrCam to use for 1 week or 1 month. They will have check-in sessions with the study team.
Participants and/or their caregivers will be asked about abilities, behaviors, social skills, learning methods, intelligence, and health-related quality of life.
Participants samples may be used for genetic testing and/or to make a type of stem cell.
Participation will last for 1-5 weeks.
Study Description:
This is a pilot study of an augmentative visual device, OrCam MyEye 2 (OrCam), in pediatric individuals with CLN3-related conditions or low vision. We hypothesize that, given the relatively simple design and operating procedure of the device, the use of the OrCam by study participants will be safe and feasible. We also hypothesize that the device will enhance their ability to obtain visually based information.
Objectives:
Primary: Assess the safety and feasibility of OrCam use by children with CLN3 or low vision.
Endpoints:
Primary:
The assessment periods will be 1 week at study site.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Children with CLN3 disease | Experimental | Children with CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device. |
|
| Children with Low vision without CLN3 | Experimental | Children with low vision not related to CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| OrCam MyEye 2 | Device | The OrCam MyEye 2 is a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs. Its advertised functions include text reading, facial recognition, and product identification. |
| Measure | Description | Time Frame |
|---|---|---|
| Participants With Adverse Events | Number of participants with adverse events related to the use of OrCam MyEye 2. Adverse events were collected from parental report. | 1 week |
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INCLUSION CRITERIA:
To participate in the screening portion of this study, an individual must meet all of the following criteria:
Have a diagnosis or suspected diagnosis of any genetically based condition causing low vision to the level specified in criteria 2.
If the genetic condition is CLN3-related, the individual must have one of the following:
a. Two CLN3 pathogenic variants, OR
b. One CLN3 pathogenic variant AND
Have an estimated visual acuity in the better seeing eye < 20/200, without the use of an assistive or augmentative device.
Is between 6 to 18 years of age.
To participate in the intervention/device use portion of this study, an individual must meet the above screening criteria and the following criteria:
EXCLUSION CRITERIA:
An individual who meets any of the following criteria will be excluded from participation in this study:
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| Name | Affiliation | Role |
|---|---|---|
| An N Dang Do, M.D. | Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| National Institutes of Health Clinical Center | Bethesda | Maryland | 20892 | United States |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 41933359 | Derived | Nguyen TT, Munoz A, Jenkins K, Agbede I, Zein WM, Huryn LA, Brooks BP, Chisholm J, Christensen J, Zalewski C, Magone MT, Chlebowski C, Thurm A, Dang Do AN. Prospective pilot safety, feasibility study of an optic-to-audio device for children with CLN3 disease. Orphanet J Rare Dis. 2026 Apr 3;21(1):199. doi: 10.1186/s13023-026-04319-0. |
| Label | URL |
|---|---|
| NIH Clinical Center Detailed Web Page | View source |
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De-identified individual participant data (IPD) that underlie results in a publication will be shared.
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Data may be requested 5 years after the completion of the primary endpoint
Contact the principal investigator or National Institute of Child Health and Human Development (NICHD)
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Of 11 participants consented for the study, one participant was a screen failure
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| ID | Title | Description |
|---|---|---|
| FG000 | Children With CLN3 Disease | Children with CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device. |
| FG001 | Children With Low Vision Without CLN3 | Children with low vision not related to CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device. |
| Title | Milestones | Reasons Not Completed | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Overall Study |
|
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| ID | Title | Description |
|---|---|---|
| BG000 | Children With CLN3 Disease | Children with CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device. |
| BG001 |
| Units | Counts |
|---|---|
| Participants |
|
| Title | Description | Population Description | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Denominator Units Selected | Denominators | Classes |
|---|---|---|---|---|---|---|---|---|---|
| Age, Categorical | Count of Participants |
| Type | Title | Description | Population Description | Reporting Status | Anticipated Posting Date | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Time Frame | Units Analyzed | Denominator Units Selected | Arm/Group Information | Denominators | Classes | Analyses | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Primary | Participants With Adverse Events | Number of participants with adverse events related to the use of OrCam MyEye 2. Adverse events were collected from parental report. | All participants who completed week one of the study. | Posted | Count of Participants | Participants | 1 week |
|
One week and up to one month depending on length of participation in study
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| ID | Title | Description | Deaths (Affected) | Deaths (At Risk) | Serious Events (Affected) | Serious Events (At Risk) | Other Events (Affected) | Other Events (At Risk) |
|---|---|---|---|---|---|---|---|---|
| EG000 | Children With CLN3 Disease | Children with CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device. |
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| Term | Organ System | Source Vocabulary | Assessment Type | Notes | Statistical Information |
|---|---|---|---|---|---|
| Pruritus | Skin and subcutaneous tissue disorders | Systematic Assessment |
Early termination due to low subject enrollment
| Title | Organization | Phone | Extension | |
|---|---|---|---|---|
| Dr. An Ngoc Dang Do | National Institute of Child Health and Human Development (NICHD) | 301-496-8849 | an.dangdo@nih.gov |
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| Type | Includes Protocol | Includes SAP | Includes ICF | Document Label | Document Date | Document Uploaded Date | Document File Name |
|---|---|---|---|---|---|---|---|
| Prot_SAP | Yes | Yes | No | Study Protocol and Statistical Analysis Plan | Oct 25, 2022 | Sep 24, 2024 | Prot_SAP_000.pdf |
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| ID | Term |
|---|---|
| D001766 | Blindness |
| ID | Term |
|---|---|
| D014786 | Vision Disorders |
| D012678 | Sensation Disorders |
| D009461 | Neurologic Manifestations |
| D009422 | Nervous System Diseases |
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| Children With Low Vision Without CLN3 |
Children with low vision not related to CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device. |
| BG002 | Total | Total of all reporting groups |
| Participants |
|
| Sex: Female, Male | Count of Participants | Participants |
|
| Ethnicity (NIH/OMB) | Count of Participants | Participants |
|
| Race (NIH/OMB) | Count of Participants | Participants |
|
| Region of Enrollment | Number | participants |
|
Children with low vision not related to CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device.
|
|
| 0 |
| 9 |
| 0 |
| 9 |
| 1 |
| 9 |
| EG001 | Children With Low Vision Without CLN3 | Children with low vision not related to CLN3 use the OrCam MyEye 2, a 22.5-g portable, eyeglass-mounted device that converts camera-captured inputs to auditory outputs for text reading, facial recognition, and product identification. The study participants and parents/guardians were trained on how to use the device. | 0 | 1 | 0 | 1 | 0 | 1 |
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| D005128 |
| Eye Diseases |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |