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Genetic Hearing Loss & Testing

Genetics of hearing loss and genetic testing for hearing loss can be a tricky and overwhelming subject. Here are some resources to help families better understand developments in these areas.

This information is presented in partnership with:

Why is Genetic Hearing Loss and Testing Important?

For every child who is deaf or hard of hearing (DHH), there is one or more reasons for their hearing loss. According to the CDC and other sources*, genetic factors are responsible for around 50-60% of childhood hearing loss.

It’s also good for families/individuals to be aware that there are genes that cause genetic hearing loss later in life. They may want to be aware of those factors sooner rather than later. OAEs/ABRs cannot detect hearing loss caused by genetic factors. With this information, families and their health care providers can be more vigilant in watching for progressive hearing loss. 

This means that a significant portion of children who are born with or acquire a permanent hearing loss have a genetic basis for their condition.

Key concepts to consider about genetic hearing loss:

  • Majority of Cases:

    Over half of all childhood hearing loss is considered to be genetic in origin.

  • Inheritance Patterns:

    Genetic hearing loss can be inherited in several ways, such as autosomal dominant, autosomal recessive, X-linked genes, or mitochondrial inheritance patterns.

  • Syndromic vs. non-Syndromic:

    While many children with genetic hearing loss have no other associated medical issues (non-syndromic), some may have additional symptoms as part of a syndrome like Usher syndrome, Pendred syndrome, or Waardenburg syndrome.

Key concepts to consider about genetic hearing loss:

  • Majority of Cases:

    Over half of all childhood hearing loss is considered to be genetic in origin.

  • Inheritance Patterns:

    Genetic hearing loss can be inherited in several ways, such as autosomal dominant, autosomal recessive, X-linked genes, or mitochondrial inheritance patterns.

  • Syndromic vs. non-Syndromic:

    While many children with genetic hearing loss have no other associated medical issues (non-syndromic), some may have additional symptoms as part of a syndrome like Usher syndrome, Pendred syndrome, or Waardenburg syndrome.

Resources

The following resources can help you navigate the ins and outs of genetic hearing loss, genetic testings, and gene therapy to determine if it is the right path for you and your child.

Please note that none of this should be considered medical advice. If you think your child is a good candidate to participate in a gene therapy clinical trial, please consult with your audiologist, ENT, and/or family doctor.

Data Statistics About Hearing Loss in Children

Centers for Disease Control (CDC)

Causes of Hearing Loss in Children

American Speech-Language-Hearing Association (ASHA)

Genetics and Hearing Loss

Mott Children’s Hospital, University of Michigan Health

The Epidemiology of Deafness

Genetic Hearing Loss Overview

American Society for Genes and Cell Therapy (ASGCT): Gene Therapy for Genetic Hearing Loss

Akouos, Inc., a wholly owned subsidiary of Eli Lilly and Company, is a precision genetic medicine company focused on developing gene therapies with the potential to restore, improve and preserve high-acuity physiologic hearing for people worldwide who live with disabling hearing loss.

https://akouos.com/

Additional Resources:

Regeneron is a leading biotechnology company that invents, develops and commercializes treatments using science and data-powered insights for people worldwide who live with serious conditions or diseases.

www.regeneron.com

FDA Approves Otarmeni™ as First and Only Gene Therapy for Genetic Hearing Loss (Full Press Release)

Additional Resources:

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Stay in the Loop with AG Bell!

Be the first to know about webinars, events, symposiums, and more.

  • Timely Announcements

    Stay informed about upcoming conferences, webinars, and public events.

  • Professional Insights

    Get updates on thought leadership in hearing health, education, and innovation.

  • Free Resources

    Access toolkits, expert tips, and opportunities to engage.

  • Resource Library

    Get answers with access to our information library

  • Volta Voices

    A bilingual magazine with in-depth stories about growing up with hearing loss

  • Community Connections

    Learn how you can support or collaborate with the hearing loss community.

Whether you're an educator, audiologist, medical provider, business, or advocate—there's something for you.

Get updates delivered straight to your inbox.

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