RECRUITING

Natural History of Autosomal Dominant Hearing Loss

Study Overview

This clinical trial focuses on testing the efficacy of different digital interventions to promote re-engagement in cancer-related long-term follow-up care for adolescent and young adult (AYA) survivors of childhood cancer.

Description

Background: Hereditary hearing loss is one of the most common sensory disabilities affecting newborns. The main options for people with hereditary hearing loss are hearing aids and cochlear implants. Both options have their limitations and do not restore biological hearing. Researchers want to learn if gene editing might be a treatment option. Objective: To understand the genes that cause non-syndromic autosomal dominant hearing loss (DFNA) in people with DFNA as well as their family members. Eligibility: People age 3 99 who have DFNA, affected family members of enrolled participants with DFNA, and unaffected family members of enrolled participants Design: Participants will be screened with a medical and hearing history. Their medical records will be reviewed. Participants will have hearing tests. They will wear headphones or earplugs. They will listen to tones, sounds, and words and may be asked to describe what they hear. Participants will have balance tests. For these, they will wear googles as they watch moving lights or as cold or warm air is blown into their ears. They will sit in a spinning chair in a quiet, dark booth. From a reclined position, they will raise their head while listening to clicking sounds. Participants will have blood drawn through a needle in the arm. Some blood will be used for gene testing. Some participants will have 2 skin biopsies. The skin will be washed, and a numbing medicine will be injected. Two small pieces of skin will be removed. Participants may have a physical exam. Participation will last for up to 20 years. Participants may give medical updates once a year.

Official Title

Natural History of Autosomal Dominant Hearing Loss

Quick Facts

Study Start:2021-02-09
Study Completion:2029-08-21
Study Type:Not specified
Phase:Not Applicable
Enrollment:Not specified
Status:RECRUITING

Study ID

NCT04501081

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Ages Eligible for Study:3 Years to 99 Years
Sexes Eligible for Study:ALL
Accepts Healthy Volunteers:Yes
Standard Ages:CHILD, ADULT, OLDER_ADULT
Inclusion CriteriaExclusion Criteria
  1. * Affected persons with autosomal dominant hereditary sensorineural hearing loss, preferably confirmed by prior genetic testing
  2. * Affected family members of enrolled participants with known autosomal dominant hereditary hearing loss
  3. * Unaffected Family Members (Healthy Volunteers) of enrolled participant
  4. * Adults must be able to provide informed consent
  5. * Minors must have a parent or guardian able to provide informed consent
  6. * Subjects must be 3-99 years of age
  1. * Persons with sensorineural hearing loss (SNHL) and/or peripheral vestibular dysfunction associated with a non-genetic etiology such as infection, metabolic or immunologic disorders, or exposure to ototoxic agents such as cisplatin, or aminoglycoside antibiotics will not be included in this protocol.
  2. * Persons with sensorineural hearing loss known to be associated with surgical intervention (e.g. acoustic neuroma removal, failed stapedectomy).

Contacts and Locations

Study Contact

Marcia L Mulquin, R.N.
CONTACT
(240) 858-3752
mmulquin@mail.nih.gov
Joshua Levy, M.D.
CONTACT
(240) 935-8305
joshua.levy@nih.gov

Principal Investigator

Joshua Levy, M.D.
PRINCIPAL_INVESTIGATOR
National Institute on Deafness and Other Communication Disorders (NIDCD)

Study Locations (Sites)

National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
United States

Collaborators and Investigators

Sponsor: National Institute on Deafness and Other Communication Disorders (NIDCD)

  • Joshua Levy, M.D., PRINCIPAL_INVESTIGATOR, National Institute on Deafness and Other Communication Disorders (NIDCD)

Study Record Dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Registration Dates

Study Start Date2021-02-09
Study Completion Date2029-08-21

Study Record Updates

Study Start Date2021-02-09
Study Completion Date2029-08-21

Terms related to this study

Keywords Provided by Researchers

  • DFNA
  • Genome Editing
  • Natural History

Additional Relevant MeSH Terms

  • Hearing Loss