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.
Hearing Loss
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.
Natural History of Autosomal Dominant Hearing Loss
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National Institutes of Health Clinical Center, Bethesda, Maryland, United States, 20892
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.
For general information about clinical research, read Learn About Studies.
3 Years to 99 Years
ALL
Yes
National Institute on Deafness and Other Communication Disorders (NIDCD),
Joshua Levy, M.D., PRINCIPAL_INVESTIGATOR, National Institute on Deafness and Other Communication Disorders (NIDCD)
2029-08-21