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What is congenital hearing loss? This refers to hearing loss that’s present at birth. Early detection is essential for preventing speech and language delays, as well as promoting social, emotional, and overall development.
Read on to explore the causes, types, and treatments from the experts at Amplifon Hearing Health Care.


*Disclosure: This article will be discussing deafness, which is a medical term for severe to profound hearing loss in a person whose primary communication is aural/oral and who will utilize hearing aids or cochlear implants to treat this hearing loss. It is not to be confused with Deaf, which refers to a community of people who share a common language, culture, and experiences related to their hearing loss.

Congenital Hearing Loss Causes

Hearing loss that’s present at birth can happen because of genetic or environmental factors (like illness or injury to the mother of the baby). Here are some of the most common causes, broken down

  1. Genetic Causes: Is deafness hereditary? It definitely can be. Some children are born with hearing loss because of genes passed down from their parents. In fact, about 50–60% of hearing loss at birth is contributed to genes. Genetic changes can affect the ear's structure or how the brain processes sounds.
  2. Environmental Causes: External factors like infections (e.g., cytomegalovirus or meningitis) during pregnancy or childhood can cause hearing loss. Babies born prematurely might also be at higher risk.
  3. Combination of Genetic and Environmental Factors: Sometimes, hearing loss happens because of both genetic changes and outside events, like exposure to certain medications that can cause hearing loss when combined with a specific genetic predisposition.

Types of Genetic Hearing Loss

Genes can be inherited in various ways, and these inheritance patterns play a crucial role in whether a person develops hearing loss.
Is hereditary hearing loss dominant or recessive? As you’ll read below, the answer can be both. Take a look at the following types of genetic hearing loss:

Autosomal Dominant Inheritance

If one parent carries a mutated gene, there's a 50% chance the child will inherit it. In this case, only one mutated gene is enough to cause hearing loss.

Autosomal Recessive Inheritance

For hearing loss to occur, a child must inherit two copies of the mutated gene—one from each parent. Even if both parents are carriers (having one mutated gene), their child still has a 25% chance of developing hearing loss.

X-linked Inheritance

These disorders are linked to the X chromosome. Since males only have one X chromosome, they are more likely to be affected by X-linked gene problems. Females, with two X chromosomes, can be carriers without showing symptoms.

Mitochondrial Inheritance

Mitochondria, the "powerhouses" of our cells, have their own DNA, which is passed down from the mother. Certain types of genetic hearing loss, such as sensitivity to specific antibiotics, can be inherited through this mitochondrial DNA.

Genetic Testing for Hearing Loss

Hearing loss in babies is often detected through a newborn hearing screening, which is quick, easy, and non-invasive. This can be done while the baby sleeps, usually taking just a few minutes. By one month of age, all babies should be screened.

There are two common screening methods:

  • Otoacoustic Emissions (OAE): This checks the inner ear’s response to sound.
  • Auditory Brainstem Response (ABR): This checks the brain’s response to sound.

If a baby doesn’t pass the screening, they should get a full hearing test by three months of age to confirm whether or not there is hearing loss.

Preventing Hearing Loss

Though congenital hearing loss isn’t always preventable, early detection through neonatal screenings is key to improving outcomes.
Here are a few preventative measures to take:

  • Vaccination against rubella
  • Minimizing the risk of Cytomegalovirus (CMV) during pregnancy. This can be done by reducing contact with saliva (spit) and urine from babies and young children if you are pregnant.
  • Avoiding ototoxic medications while pregnant
  • Regular monitoring and follow-up care

These measures are important, especially for those at higher risk of progressive hearing loss.

Is Hearing Loss Genetic?: How Doctors Figure Out the Cause of Hearing Loss

Healthcare providers start by gathering information about the person's family history and any other medical details. Based on this, they’ll look at the type of hearing loss and may ask for further tests, including genetic testing, to determine the exact cause.

If you're worried about your child's hearing, early detection and intervention are key, so be sure to get regular screenings and seek help if you notice any signs of hearing issues.

Congenital Hearing Loss Treatments

Though there isn’t currently a cure for congenital hearing loss, there are some ways to make this condition more manageable and allow individuals to hear the best that they can. A few tools utilized are:

Hearing Aids

Early fitting of hearing aids is critical for language development. The most common style for children is behind-the-ear (BTE), which supports binaural hearing (using both ears) and helps with speech in noisy environments.

Take a closer look at the differences between RIC (receiver-in-canal) and BTE hearing aids.

Cochlear Implants

For severe or profound hearing loss, cochlear implants are effective, especially when implanted before the age of 2. These devices directly stimulate the auditory nerve and improve speech perception.

Auditory Brainstem Implants

Used for individuals who can't benefit from cochlear implants, often in cases of cochlear nerve malformations.

Congenital Hearing Loss in Adults

Congenital hearing loss can significantly impact a child’s development, but early identification and intervention can lead to better outcomes as children age into adults. Advances in genetic testing and newborn screenings provide crucial information for managing and preventing hearing loss well into adulthood. With the right treatment and support, individuals with hearing loss can grow to thrive in all areas of life as they age.

If your child has been diagnosed with congenital hearing loss and you’re wondering where to start, Amplifon Hearing Healthcare can help. Take a look at our blog for some continuing education on hearing aids, or find a clinic near you to start tackling your child’s hearing health head-on. 

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Talk to a Professional About Hearing

With a diagnosis like congenital deafness, finding hearing aids can seem overwhelming. Connect with trusted experts who will compassionately guide you on this journey. Find a clinic near you to start the process today.

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