Is Deafness Hereditary? The Role of Genetics in Hearing Loss

Hearing loss affects around 430 million people worldwide.¹ Genetic factors play a major role in about 50% of cases.² With hundreds of genes identified as contributing factors, hereditary hearing loss is a complex issue.³

Read on to understand the differences between environmental and hereditary causes of deafness and how genes contribute to hearing ability. You'll also learn about breakthroughs in genetic testing that support early diagnosis and treatment and ways to connect with helpful community resources for individuals and families affected by hearing loss.

Understanding Deafness

Deafness involves partial or complete hearing loss and occurs when a component of your ear or auditory system malfunctions.¹

The severity of deafness varies based on the intensity or loudness required for sounds to be audible. You may experience uneven sound perception, hearing certain sounds more effectively than others. For instance, someone might be partially deaf, retaining some auditory ability, or profoundly deaf, with minimal to no hearing.¹

Different Types of Hearing Loss

There are three main types of hearing loss. A hearing test performed by a qualified audiologist or otolaryngologist (ear, nose and throat doctor) is the most reliable method to determine the type of hearing loss someone has. This is important because even though each has different causes, some types can have overlapping symptoms.³

Sensorineural hearing loss

This is the most common type of hearing loss. 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 curable, but hearing aids are usually beneficial.⁴

Cookie-bite hearing loss, also known as mid-frequency sensorineural hearing loss, is primarily caused by genetic factors. A family history of this condition significantly increases the chance of developing it. It can manifest at birth or emerge later in life due to specific genetic predispositions.³

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 (ringing in the ear). About half of the 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.⁵

Conductive hearing loss

Conductive hearing loss 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 a damaged eardrum. Sometimes, medical or surgical intervention can reverse conductive hearing loss. It's also possible to have a combination of sensorineural and conductive hearing loss.⁴

Environmental vs. Hereditary Deafness

Hearing loss is often a complex issue that may stem from either genetic or environmental factors. Genetic hearing loss is caused by inherited abnormalities or genetic changes. Hearing loss can be congenital (present at birth) or later-onset. Environmental hearing loss arises from exposure to loud noises, toxins, medications or infections, potentially causing permanent damage. Lifestyle factors like smoking and poor nutrition also contribute.¹

The Role of Genetics in Deafness: How Hearing Ability Is Inherited

Deafness is inherited when genetic mutations are passed from parents to children. Genetic hearing loss can be either syndromic (co-occurring with other symptoms) or non-syndromic (hearing loss is the only symptom). Inheritance patterns typically fall into five main categories:²

Autosomal recessive inheritance

Autosomal recessive inheritance is the most common cause of genetic hearing loss, at 75% to 80% of cases.² It's inherited from both parents.² A child with deafness resulting from a recessive gene may not have any family history of hearing loss.³

Autosomal dominant inheritance

Autosomal dominant hearing loss comes from one parent and occurs in nearly 20% of cases. The parent passing along the dominant gene has some degree of hearing loss as well.³

X-linked inheritance

This is a rare type of inherited deafness.³ It occurs due to changes in genes on the X chromosome.² Since males have one X chromosome and females have two, the condition usually affects males more severely.² Mothers can be carriers of the condition without having hearing loss themselves, or they may have milder symptoms.³

Mitochondrial inheritance

Mitochondrial hearing loss is another rare type of inherited deafness, with genetic information inherited solely from the mother.² Since mitochondrial DNA comes only from the egg cell, both sons and daughters can inherit these hearing-related traits, but only daughters can pass them on to future generations.³

Genetic syndromes

A genetic syndrome is a group of medical signs and symptoms that consistently occur together and form a recognizable pattern.² Certain genetic syndromes can affect hearing at birth (congenital) while others develop later in life (late-onset).³

Deafness and hearing loss may be caused by abnormalities in the development or function of the inner ear, auditory nerve or sound processing pathways.² While some individuals experience hearing loss as their first noticeable symptom, others may experience it gradually after developing other characteristics.³

Approximately 400 medical syndromes include some degree of hearing loss. Some of the most common include:²

• Alport syndrome
• Crouzon syndrome
• Jervell and Lange-Nielsen syndrome
• Stickler syndrome
• Usher syndrome
• Waardenburg syndrome

Genetic Testing for Diagnosis and Treatment

Diagnostic methods for hearing loss include newborn hearing screenings, audiometric evaluations and imaging studies like CT or MRI scans to detect structural abnormalities. But it isn't always clear whether a person has genetic deafness or other factors cause the hearing loss. Your family history of hearing loss will provide some insight into whether your child's hearing loss is genetically based. Your medical team may suggest genetic testing to determine if you or your children are genetically predisposed to hearing loss.³

How genetic testing for hearing loss works

Genetic testing for hearing loss is a process that examines a person’s DNA to identify specific changes or mutations known to cause hearing loss. It can be performed using a sample of blood, a cheek swab or saliva, and helps test for both syndromic and non-syndromic forms of hearing loss.²

However, genetic testing isn't without limitations. Sometimes, more invasive testing is needed to confirm a diagnosis, and testing requires careful interpretation by specialists to ensure families fully understand the results and their implications.³

Genetic testing to guide treatment options

Genetic testing helps provide clarity on the underlying reasons for hearing impairment and helps guide treatment options, including hearing aids or cochlear implants.

Gene therapy treatments. Recent medical breakthroughs may offer hope for people with inherited hearing loss. Scientists have made exciting progress using gene therapy — a technique that can fix the faulty genes that cause deafness.⁶ Think of it as being able to deliver a tiny repair kit directly to your DNA. Researchers are able to send healthy genes into the inner ear to replace ones that aren't working properly.⁶ Early trials are underway.⁶

Stem cell treatments. Another promising area involves stem cells, which could potentially be used to grow new hearing cells to replace ones that have been damaged or lost. While many of these treatments are still being tested, they represent a dramatic shift from simply managing hearing loss to potentially curing it at its genetic source.⁷

Living Well With Hearing Loss

While research into genetic treatments continues, families today have many options to help children with hearing loss thrive. The key is early intervention and building a strong support network.⁸

Several proven approaches can help children communicate effectively, including:³

• Cochlear implants for severe cases of hearing loss
• Hearing aids for various degrees of hearing loss
• Sign language and lipreading skills

Medical and professional support

Working with a healthcare team is essential. Members typically include:³

• An audiologist for hearing tests and device fitting
• An otolaryngologist for medical care
• A speech therapist to support communication development

Community connection

One of the most valuable resources is connecting with the Deaf community. Being part of this vibrant culture can:⁹

• Create opportunities for children to meet peers with similar experiences
• Help families learn about Deaf culture and identity
• Offer emotional support and shared experiences
• Provide role models and mentorship

Why Testing and Early Intervention Matters

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.³

Test Your Hearing Online

Find Solutions by Understanding Where Hearing Loss Comes From

Hereditary hearing loss and deafness are complex conditions with many facets. So, there's no one-size-fits-all approach to treatment and care. Some families choose to focus on spoken language with hearing devices, others embrace sign language and many opt for a combination of approaches. The most important thing is making informed choices that work best for your child and family while maintaining connections with both hearing and Deaf communities.

Financing Ear Care With the CareCredit Credit Card

The CareCredit credit card can help you pay for hearing care, including regular hearing tests, screenings and more.* Use our Acceptance Locator to find a hearing specialist near you that accepts CareCredit. Continue to invest in your wellness journey by downloading the CareCredit Mobile App to manage your account, find a provider on the go and easily access the Well U blog for more great articles, podcasts and videos.

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Author Bio

Pamela Cagle, R.N., has extensive experience in a range of clinical settings, including ER, surgical and cardiovascular. For the past decade, she has leveraged her nursing experience in writing for health and technology publications such as AARP, VKTR, National Council on Aging and others. She is passionate about blending her medical and storytelling expertise to bring authenticity to health and wellness topics.