Optic-to-Audio Device in a Pediatric Cohort With CLN3-related Conditions or Low Vision

Low Vision Clinical Trial

Official title:

Pilot Study of an Optic-to-Audio Device in a Pediatric Cohort With CLN3-related Conditions or Low Vision

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT04974307
• Other study ID #: 10000414
• Secondary ID: 000414-CH
• Status: Completed
• Phase: N/A
• Start date: September 30, 2021
• Est. completion date: October 7, 2023

Study information

• Verified date: February 23, 2024
• Source: National Institutes of Health Clinical Center (CC)
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

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 blindness.

Objective:

To learn if it is safe, easy, and useful for children with CLN3 or blindness to use the OrCam.

Eligibility:

People aged 6-18 years who have either CLN3-related disease or 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.

Description:

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.

Secondary:

Assess the effectiveness of OrCam use by children with CLN3 or low vision.

Exploratory:

Assess the feasibility and effectiveness of OrCam use in CLN3 versus non-CLN3 groups. Optimize methods for assessing efficacy of visual accommodation/assistive devices for the CLN3/NCL population

Endpoints:

Primary:

1) Adverse events during the use of OrCam. 2) Feasibility test. 3) Feasibility questionnaire. 4) Device use diary. The assessment periods will be 1 week at study site and 1 month at home.

Secondary:

1) Function test of using OrCam. 2) Ability questionnaire.

Exploratory:

1) Function test. 2) Feasibility, Ability, Applicability questionnaires.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 11
• Est. completion date: October 7, 2023
• Est. primary completion date: October 6, 2023
• Accepts healthy volunteers: No
• Gender: All
• Age group: 6 Years to 18 Years

Eligibility

• INCLUSION CRITERIA:

To participate in the screening portion of this study, an individual must meet all of the following criteria:

-Has 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:

• Two CLN3 pathogenic variants,

• One CLN3 pathogenic variant AND

• clinical presentation suggestive of CLN3, OR

• characteristic electron microscopy (EM) findings (such as curvilinear body, fingerprint profile, granular osmiophilic deposits).

• Has 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:

• Has an appropriate cognitive developmental ability to participate based on Investigators screening assessment.

EXCLUSION CRITERIA:

An individual who meets any of the following criteria will be excluded from participation in this study:

• Has any of the following auditory dysfunctions: non-reversible or non-correctable hearing loss, tinnitus that is chronic or occurring daily, auditory hallucinations that occur daily.

• Uses an optic-to-audio assistive device at the time or within 3 months of screening and enrollment.

• Is unable to travel to the NIH because of medical condition for required in-person portions of the study.

• Is unable to comply with or have medical conditions that would potentially increase the risk of participation.

Related Conditions & MeSH terms

• Low Vision
• Vision, Low

Intervention

Device:
• OrCam MyEye 2
The study uses the OrCam MyEye 2, 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. The study participants and parents/guardians will be trained on how to use the device. Evaluations for primary and secondary endpoints will be done following 1 week and 1 month of device use.

Locations

Country	Name	City	State
United States	National Institutes of Health Clinical Center	Bethesda	Maryland

Sponsors (1)

Lead Sponsor	Collaborator
Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)

Country where clinical trial is conducted

United States, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	To assess: a) safety and b) feasibility of using OrCam MyEye 2 by children with CLN3 or low vision	a) Adverse events; b) Feasibility test, Feasibility questionnaire, and Device use diary	1 week, 1 month
Secondary	To assess the efficacy of using the OrCam by children with CLN3 or low vision	1) Function Test - Efficacy Scores; 2) Ability questionnaire	1 week, 1 month

External Links

•   NIH Clinical Center Detailed Web Page