The Genetics of Hearing Loss: Is Hearing Loss Hereditary?

The World Health Organization estimates that by 2050, nearly 2.5 billion people will have some degree of hearing loss, and at least 700 million of them will require hearing rehabilitation.¹ While hearing loss can happen to anyone at any age due to various causes,¹ genetics is to blame for 50 to 60 percent of babies born with hearing loss.² Inherited genetic factors can also play a role in hearing loss that develops later in life for some people.³

Here we look at how hereditary hearing loss happens, how it can be treated and if genetic deafness can be cured.

Hereditary Hearing Loss: Is Deafness Genetic?

Genes play a role in everything in the body, from our appearance to our organs and our health. We inherit half our genes from one parent and half from the other parent. This includes any defective genes.

Changes or mutations in the genes related to hearing structures can lead to abnormalities that prevent components in your ear, such as the sensory hair cells or cochlea, from working properly. This can lead to hearing problems.³

Hearing loss and deafness can be inherited. The chances of developing genetic deafness depend on whether the gene is recessive or dominant.⁴

Autosomal recessive hearing loss

Autosomal recessive hearing loss is inherited from both parents. This is the most common cause of genetic hearing loss. A person with autosomal recessive hearing loss may not have any family members with hearing loss.⁴

Autosomal dominant inheritance

Autosomal dominant inheritance is inherited from one parent. The parent with the mutation often has hearing loss too.⁴

Genetic syndromes and external factors

Genetic hearing loss can also be part of a genetic syndrome, such as Down syndrome.⁵

In some cases, genetics combine with external factors to cause hearing loss. For instance, some medications may lead to hearing loss, but only in people with a certain gene mutation.²

Non-Syndromic Hearing Loss vs. Syndromic Hearing Loss

Inherited hearing loss may be non-syndromic or syndromic. Non-syndromic hearing loss isn't associated with or caused by any other health issues. It accounts for about 70 percent of genetic hearing loss cases.²

Syndromic hearing loss occurs in conjunction with another disorder, such as vision problems, or may actually be part of another genetic condition. Approximately 400 syndromes include hearing loss.³

Different Types of Hearing Loss: Inherited vs. Not

There are different types of hearing loss based on the part of the ear that's affected. These can be inherited or not.

They include:

Sensorineural hearing loss - This is the most common type. It occurs when the inner ear or hearing nerve becomes damaged, often because of damage to the hair cells in the cochlea. It can be caused by an inherited condition, as well as exposure to loud noise, disease, injury, or certain medications. It's not medically treatable, but hearing aids are usually beneficial.⁶

Sudden sensorineural hearing loss - Sudden hearing loss occurs at once or over a few days. It usually affects only one ear. It may also cause a feeling of fullness in the affected ear, dizziness and tinnitus, which is a ringing in the ear. About half of people with the condition will spontaneously recover some or all of their hearing. Getting immediate treatment is important as it significantly increases the chance of recovering at least some hearing. ⁷

Conductive hearing loss - This occurs in the outer or middle ear when sound waves are blocked from getting through to the inner ear. This can be caused by a buildup of wax or a foreign object in the ear canal, or if the middle ear is blocked by fluid or infection. It can also be caused by a bone abnormality or damaged eardrum. Sometimes, medical or surgical intervention can reverse conductive hearing loss.⁶

It's also possible to have a combination of sensorineural and conductive hearing loss.⁶

Hearing loss can also be caused by auditory neuropathy spectrum disorder, which occurs when the sounds enter the ear normally but aren't organized in a way that's understood by the brain.⁸

The Different Degrees of Hearing Loss

Hearing loss can range from mild to profound. Here's how it presents:⁸

Mild: A person with mild hearing loss may be able to make out most speech but has trouble hearing soft sounds.

Moderate: A person with moderate hearing loss may not be able to hear most speech when another person is talking at a normal volume.

Severe: A person with severe hearing loss can only hear some loud sounds and no speech when someone is talking at a normal volume.

Profound: A person with profound hearing loss can't hear any speech, only very loud sounds.

How to Tell If You Have Hereditary Deafness

It isn't always clear whether a person has genetic deafness or whether the hearing loss is caused by other factors. Your family history of hearing loss may provide some insight into whether your child's hearing loss is genetically based. Some medical professionals may also offer genetic testing to determine if you or your children are genetically at risk for hearing loss.

Whether or not you or your loved one has a genetic predisposition for hearing loss, it's important to have regular hearing screenings and follow healthy hearing care practices to ensure good hearing health for life. With babies and young children, in particular, early intervention can help prevent developmental problems, such as speech disorders.⁹

Living With Hearing Loss

Though most types of genetic hearing loss can't be cured, there are treatments and interventions that can help. Working closely with an audiologist, otolaryngologist and speech therapist can ensure your child gets the help they need.

Depending on the child's age and degree of hearing loss, treatment can include:¹⁰

• Hearing aids
• Cochlear implants
• Learning sign language and lip reading

Hearing loss is considered a disability and a person may qualify for financial assistance as long as the individual meets certain criteria.¹¹ Also, depending on the state you live in, hearing aids and cochlear implants for children and adults may be covered. Check with your insurance provider.

Here are some tips to support your child with hearing loss:

• Stand close to your child and away from other noises before speaking.
• If hearing is better in one ear, make sure it's closer to you before speaking.
• Close windows and doors to make it easier to hear indoors.
• Use carpet and rugs, acoustic tiles and heavy drapes to create a quieter environment.
• Face your child and stay still while speaking to them.
• Speak slowly and clearly.
• Encourage your child to let you and others know when they don't hear or understand.

CareCredit Financing for Hearing Tests and More

Remember that the CareCredit credit card is an easy way to pay for hearing tests, audiology appointments and other hearing care services and products not covered by insurance.* Use our Acceptance Locator or the CareCredit Mobile App to find a hearing care professional near you that accepts the CareCredit credit card.

Author Bio

Adrienne Santos-Longhurst is a writer who has been covering health and lifestyle for almost two decades. She's written extensively on physical and mental health conditions and treatments, as well as fitness, beauty, and relationships. She's best known for her empathetic and informative pieces for Healthline, Insider, Medical News Today, and more.