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Protocol Details

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

This study is NOT currently recruiting participants.

Summary | Eligibility | Citations | Contacts

Summary

Number

000414-CH

Sponsoring Institute

National Institute of Child Health and Human Development (NICHD)

Recruitment Detail

Type: Completed Study; data analyses ongoing
Gender: Male & Female
Min Age: 6 Years
Max Age: 18 Years

Referral Letter Required

No

Population Exclusion(s)

Neonates

Keywords

OrCam;
Legal blindness;
Safety;
Feasibility;
Efficacy

Recruitment Keyword(s)

None

Condition(s)

CLN3-related disorders;
Low vision

Investigational Drug(s)

None

Investigational Device(s)

None

Intervention(s)

Device: OrCam MyEye 2

Supporting Site

National Institute of Child Health and Human Development

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.

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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.


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Citations:

Not Provided

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Contacts:

Principal Investigator

Referral Contact

For more information:

An N. Dang Do, M.D.
National Institute of Child Health and Human Development (NICHD)
NIHBC 10 - CRC BG RM 1-3342
10 CENTER DR
BETHESDA MD 20892
(301) 496-8849
an.dangdo@nih.gov

An N. Dang Do, M.D.
National Institute of Child Health and Human Development (NICHD)
NIHBC 10 - CRC BG RM 1-3342
10 CENTER DR
BETHESDA MD 20892
(301) 496-8849
an.dangdo@nih.gov

Office of Patient Recruitment
National Institutes of Health Clinical Center (CC)
Building 61, 10 Cloister Court
Bethesda, Maryland 20892
Toll Free: 1-800-411-1222
Local Phone: 301-451-4383
TTY: TTY Users Dial 7-1-1
ccopr@nih.gov

Clinical Trials Number:

NCT04974307

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