Hearing Loss and Genetics

Penelope Woods with customer | A Better Ear

Your genes contain all the information needed to build and maintain your cells and pass on that information to your children. Your genes can directly cause some health conditions and make you more susceptible to others.

So, can hearing loss be genetic? Yes, it can.

Many people are surprised to learn that there’s a genetic component to hearing loss. There are numerous genes involved in hearing and sometimes they don’t form as they should. That’s known as a mutation.

The cochlear, which changes soundwaves into nerve signals, is a highly specialised organ that relies on many genes to instruct its development and function. Any mutation here can lead to hearing loss.

Some mutations arise spontaneously while others run in families (known as familial hearing loss). You may inherit a gene mutation that causes a malformation in your outer, middle or inner ear. Some people have no ear canal, for example, or other changes to their ear anatomy.

Those gene mutations may be passed down by one or both parents and can affect one or both ears. Genetic hearing loss may be present from birth (that’s why we have the newborn hearing screen) or develop later in life, becoming progressively worse as time goes by. It may be mild or profound.

Genetic hearing loss in babies

Estimates suggest that 50-60% of hearing loss in babies is due to genetic causes.

That’s why Australia offers newborn babies a hearing screening test soon after birth. The goal is to identify the 1-2 babies in every 1,000 who are born with significant hearing loss. Identifying those babies enables early intervention and treatment.

Genetic hearing loss syndromes

Some genetic hearing loss, about 30% in fact, is related to a syndrome that’s affecting your other organs too.

Genetic causes of hearing loss relating to a syndrome include:

  • Usher syndrome, a rare genetic disorder that causes deafness because the inner ear and auditory nerve can’t transmit sound to the brain (known as sensorineural hearing loss). It also causes progressive blindness.
  • Down syndrome, which can cause temporary or fluctuating hearing loss due to poor Eustachian tube function or small nasal passages, or permanent hearing loss due to a problem with the cochlear or auditory nerve (sensorineural hearing loss).
  • Pendred syndrome, which causes severe to profound hearing loss either from birth or childhood, alongside a thyroid disorder.
  • Jervell and Lange-Nielsen syndrome, which causes deafness from birth alongside abnormalities with the heart’s electrical system.

Non-syndromic genetic hearing loss

Hearing loss can also be caused by mutations that are not part of a broader syndrome but only affect your auditory system. About 70% of genetic hearing loss falls into this category.

A genetic mutation may not directly cause hearing loss. But it can make you more vulnerable to hearing loss from other causes, such as noise exposure, ototoxic medications or illnesses.

How A Better Ear can help

If you’re not sure whether you have a genetic risk but are concerned about your hearing, then please visit A Better Ear for a hearing assessment. That’s especially important if your hearing is declining rapidly.

At A Better Ear, we will run a thorough hearing test and investigate possible causes of hearing loss to rule out other causes.

If you know you have a genetic predisposition to hearing loss, then it’s very important to protect your hearing throughout life, for example by wearing protective earphones in loud environments, turning down loud music and avoiding medicines that are toxic to your ears.

You should have regular hearing checkups to pick up any changes in your hearing. If you wear a hearing aid, then it’s also important to have it regularly tuned so it’s working at its best. We can help you with all that. Please get in touch to protect your hearing.

All information is general in nature. Patients should consider their own personal circumstances and seek a second opinion.