Hereditary Hearing Loss: Causes, Signs and What Can Help

What is Hereditary Hearing Loss?

What you need to know about the types, signs and what can help

Overview     |    Types of hereditary hearing loss    |    How is hearing loss inherited?    |    What are the signs?    |    When to seek help    |     What can help    |    Living with hereditary hearing loss    |    Summary

Last Hearing Aid UK Update:  19/10/2026

Overview

Most people lose some hearing as they get older, but for some, it happens much earlier, simply because of their genes. Hereditary hearing loss runs in families. It’s caused by gene changes passed down from one or both parents. Some people are born with it, while for others it develops later, usually gradually, though occasionally more quickly.

If a relative has trouble hearing, or you’ve been told you have hearing loss yourself, understanding the genetic side can help you know what to look out for.

What is hereditary hearing loss?

Hereditary hearing loss comes down to a genetic change that affects the inner ear or the auditory nerve. Sometimes it's there from birth. Other times it develops gradually. It can occasionally be linked to a wider syndrome, but in most cases, it shows up on its own with no other health conditions involved.

One of the most common causes, particularly of hearing loss that’s present from birth, is a small change in the GJB2 gene, also known as connexin 26, which normally helps the inner ear make sense of sound. When it doesn't quite do its job, hearing loss can be there from the very beginning, or emerge quietly in the early years of childhood.

There are two types of hereditary hearing loss:

• Non-syndromic hearing loss: The most common form, appearing on its own with no other health issues alongside it.
• Syndromic hearing loss: This is less common. Here, hearing loss is one part of a wider condition, and it can come alongside other things, like changes to vision or balance.

How is it inherited?

Autosomal dominant inheritance simply means one parent is enough. If either your mum or dad carries the gene change, it can be passed on to you. That means each child has a 50% chance of inheriting it. This type of hearing loss often doesn’t show itself straight away.

Autosomal recessive works differently: both parents need to carry a copy of the gene change for a child to be affected, and when that happens, hearing loss can be present from birth or develop early on. Parents who carry it often have no hearing loss themselves, which is why it can seem to appear out of nowhere.

The other two patterns are much rarer. X-linked inheritance is carried on the X chromosome and tends to affect males more than females. Mitochondrial inheritance, the rarest of all, is passed down only through the mother, though it can affect sons and daughters alike.

Signs of hereditary hearing loss

• Difficulty following speech, especially in noisy environments.
• Frequently asking people to repeat themselves.
• Ringing or buzzing in the ears (tinnitus).
• Struggling with high-pitched sounds, like a phone ringing or a doorbell (though not all hereditary hearing loss starts in the high frequencies).

When to seek help

If hearing loss runs in your family, it's worth keeping half an eye on your own hearing over the years, and having a word with your GP if anything changes.

Hearing loss that runs in families tends to develop gradually, but if you notice any sudden or significant changes, it's best to see a doctor without delay. The NHS advises seeing a GP if you think your hearing is getting worse, and getting urgent advice if it changes suddenly.

Getting your hearing checked early makes it easier to find what can help. An audiologist can help you figure out what's happening with your hearing and how significant it is. Your GP can refer you for an NHS hearing test, or you can book a free hearing test with one of Hearing Aid UK’s HCPC-registered audiologists directly, without a referral.

Genetic testing may be recommended by your GP or a genetics specialist in some cases, to find out which genes are involved. This can help identify what might help, and, if you need them, the support services that are right for you. Genetic testing is arranged through the NHS or a specialist genetics service, not through Hearing Aid UK.

What can help with hereditary hearing loss?

Hereditary hearing loss is usually permanent, but there's a lot that can make everyday life easier:

• Hearing aids make sounds louder and easier to follow in many different settings, and are fitted and fine-tuned in person by a qualified audiologist.
• People with severe or profound hearing loss may be offered cochlear implants, which work around the damaged parts of the ear to stimulate the auditory nerve directly. These are assessed and provided through a specialist NHS audiology or ENT team.
• Speech and language therapy can help people who lose their hearing at a young age communicate more easily. This is usually arranged through your GP or the NHS.

"Hereditary hearing loss is often misunderstood because people assume it only ever shows up in childhood. In reality, some gene changes don't cause noticeable changes until adulthood.

If hearing loss runs in your family, it's worth getting your own hearing checked even if you feel fine, rather than waiting until it becomes obvious."

says Paul Harrison, Audiology Expert & Founder, Hearing Aid UK

Living with hereditary hearing loss

Living with hereditary hearing loss has its challenges, but there's a lot that can make things easier. Support groups, online communities and your audiologist can all be a real source of help while you find your feet.

Everyday technology, from hearing aids to smartphones, can also make a big difference to how easily you communicate. In the UK, organisations like RNID offer helpful support and advice for people and families affected by hereditary hearing loss.

Summary

Hereditary hearing loss can show up anywhere along the family tree, not just in the generation you'd expect. Understanding how it's passed down takes away a lot of the mystery, and getting your hearing checked sooner rather than later just makes everything that follows a bit easier.

With the right support, many people with hereditary hearing loss get on really well, helped by better hearing aids, cochlear implants, and other audiologist-led care.

If you think you might have hereditary hearing loss, speak to your GP if your hearing has changed suddenly, or if you’re worried about a child. Otherwise, a free hearing test with an HCPC-registered audiologist is a good next step, and it’ll give you clear, independent advice on what can help.

Thinking hereditary hearing loss might run in your family?

If hearing loss runs in your family, or you've noticed changes in your own hearing, a hearing test is a good first step. If anything has changed suddenly, see your GP first.

You don’t need a referral to see us. Hearing Aid UK connects you with over 200 independent, HCPC-registered audiologists who offer a free hearing test, either at one of our 93+ clinics or during a free home visit, whichever suits you best. There's no obligation and no pressure, just clear, independent advice on what can help.

We're also Which? recommended, and trusted by over 31,000 customers across the UK. Call our team free on 0800 567 7621, or book a free hearing test online.