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Hereditary Hearing Loss: What is it, what are the signs, treatments and how to manage it?

Kimberley Bradshaw - Head of Marketing
Written By:
Kimberley Bradshaw

Head of Online Medical Content

Paul Harrison - Audiology Expert at Hearing Aid UK
Audiologically Reviewed By:
Paul Harrison

Audiology Expert

Updated: 24th February 2026
Updated and medically reviewed: 24th February 2026 in: Hearing Loss Awareness
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What is Hereditary Hearing Loss?

What you need to know about the types, signs, treatments, and how to manage them

 

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

 

Last Hearing Aid UK Update:  24

Overview

Many people lose their hearing as they get older, but some people lose it earlier in life because of their genes. Hereditary hearing loss is a condition that runs in families. It happens when changes in certain genes cause hearing ability to slowly or suddenly get worse.

It's important to know about the genetic factors at play if you've noticed that someone in your family has trouble hearing or if you've been told you have hearing loss.

 

What is hereditary hearing loss?

Genetic mutations that affect the inner ear or auditory nerve cause hereditary hearing loss. It may be congenital, meaning it is present at birth, or progressive, meaning it gets worse over time.  It may be connected to certain syndromes in some instances, but it frequently occurs in the absence of any related health conditions.

The most common genetic cause of hereditary hearing loss is a mutation in the GJB2 gene, also known as connexin 26. This gene plays a key role in how the inner ear processes sound, and when it doesn't work as it should, hearing loss can occur from birth or develop in early childhood.

 

There are two types of hereditary hearing loss:

  • Non-syndromic hearing loss:  This is the most common type and occurs on its own, without other health issues.
  • Syndromic hearing loss:  This is less common and is linked to other medical conditions, such as vision or balance issues.

 

How is hereditary hearing loss inherited?

Depending on the genes involved, there are a few ways that hereditary hearing loss can be passed down:

  • Autosomal dominant inheritance: If one parent has the gene that causes hearing loss, there is a 50% chance that the child will get it. Most of the time, people lose their hearing later in life, usually when they are adults.
  • Autosomal recessive inheritance: Both parents must have the defective gene for the child to be affected. In this case, the person may be born deaf or lose their hearing early in life.
  • X-linked inheritance: This is much less common and means that the gene is on the X chromosome. It usually has a bigger effect on men than on women.
  • Mitochondrial inheritance: This is the rarest pattern and is passed down through the mother's side only. It affects both men and women, but only mothers can pass it on to their children.

 

Signs of hereditary hearing loss

Hereditary hearing loss can be pinpointed in different ways, depending on when and how it develops. Some common red flags to look out for include:

 

Signs Of Hereditary Hearing Loss

 

When to see a doctor

If you have a family history of hearing loss, you should keep an eye on your own hearing and see a doctor if you notice any changes.

Hearing loss that runs in families usually happens over time, but if you notice any sudden or big changes in your hearing, you should see a doctor right away.

Finding and diagnosing hearing loss early is important for treating it. An audiologist can help you figure out what is wrong with your hearing and how bad it is. Your doctor will send you to them for a hearing test.

Genetic testing may be recommended in some cases to find out which genes are causing the problem and how they work. This can help you find treatment options and, if you need them, get the support services that work for you.

Related reading:  What is an audiologist?

 

Treatment for hereditary hearing loss

There is no cure for hereditary hearing loss, but there are many treatments that can help you deal with the problem and make your life better:

  • Hearing aids make sounds louder and make it easier to hear in many different places.
  • People with severe or profound hearing loss may be told to get cochlear implants. They go around parts of the ear that are broken and stimulate the auditory nerve.
  • Speech therapy can help people who lose their hearing at a young age communicate better.

 

Living with hereditary hearing loss

Living with hereditary hearing loss can feel challenging at times, but there are many ways to make life easier. Support groups, online communities, and support from your audiologist are beneficial resources for those who need help adjusting.

Modern technology, such as hearing aids and smartphones, can also make communication much more accessible.

In the UK, organisations like RNID offer great access to support and advice for people and families affected by hereditary hearing loss. 

 

Summary

Many people have hereditary hearing loss, which is a genetic condition that runs in families over generations. It can feel overwhelming, but knowing how it is passed down and getting help early can make a big difference in your quality of life.

People with hereditary hearing loss can still live full lives with the right help, thanks to better hearing aids, cochlear implants, and other treatments.

If you think you might have hereditary hearing loss, make an appointment with your GP. They will then send you to an audiologist for a full hearing test and personalised advice.

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Do you think you might be losing your hearing?

If you think you or someone you care about might be losing their hearing because of genetics, don't wait.

Getting professional help and learning about the condition early can make a big difference in how well you can deal with it and how good your life is.

Make an appointment with an audiologist today to get a full hearing test and talk about the best treatment options for you.

With the right help, you can keep living life to the fullest.

Don't let your hearing loss get in the way. Get started on improving your hearing right away!

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Written by Kimberley Bradshaw

Meet Kimberley Bradshaw, Head of Online Medical Content

Kimberley Bradshaw started her love of content creation, as a freelancer for many well-established medical brands.  She has written about hearing healthcare for several UK and US online health and wellness publications since.  Connect with Kimberley on LinkedIn.

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Watch the hereditary hearing loss video below

FAQs

What is the best hearing aid model for me?

In general, any audiologist will always recommend to you the hearing aid model that best suits your needs. Here is a useful checklist to make sure that is the case.

  • Audiologist's level of knowledge: The audiologist you have seen will hopefully have a wide knowledge of all available hearing aids; however, some will only be familiar with a small number of brands and, therefore, may not really be in a position to know which model is the best for you. It is OK to challenge their recommendation and ask them to justify why this particular brand is the one for you.
  • Do research: Read about the hearing aid that was recommended. Does it seem like it will suit your lifestyle? Does it have more or fewer features than you need? 
  • Be aware of sales targets: Many high street retailers have specific tie-ins to a particular manufacturer/brand. The hearing aid they have suggested may still be the correct one for you, but do your research so that you know why they might have recommended it.
Do I need one hearing aid or two?

If you have significant hearing loss in both ears, you should be wearing two hearing aids. Here are the audiological reasons why:

Localisation: The brain decodes information from both ears and compares and contrasts them. By analysing the minuscule time delays as well as the difference in the loudness of each sound reaching the ears, the person is able to accurately locate a sound source. 

Simply put, if you have better hearing on one side than the other, you can't accurately tell what direction sounds are coming from.

Less amplification is required: A phenomenon known as “binaural summation” means that the hearing aids can be set at a lower and more natural volume setting than if you wore only one hearing aid.

Head shadow effect: High frequencies, the part of your hearing that gives clarity and meaning to speech sounds, cannot bend around your head. Only low frequencies can. Therefore, if someone is talking on your unaided side, you are likely to hear that they are speaking, but be unable to tell what they have said.

Noise reduction: The brain has its own built-in noise reduction, which is only really effective when it is receiving information from both ears. If only one ear is aided, even with the best hearing aid in the world, it will be difficult for you to hear in background noise as your brain is trying to retain all of the sounds (including background noise) rather than filtering them out.

Sound quality: We are designed to hear in stereo. Only hearing from one side sounds a lot less natural to us.

Fancy some further reading on this topic?  You can read about why two hearing aids are better than one in our article, hearing aids for Both Ears, here

What are the benefits of rechargeable hearing aids?

For most people, the main benefit of a rechargeable hearing aid is simple convenience. We are used to plugging in our phones and other devices overnight for them to charge up.  Here are some other pros and cons:

For anybody with poor dexterity or issues with their fingers, having a rechargeable aid makes a huge difference, as normal hearing aid batteries are quite small and some people find them fiddly to change.

One downside is that if you forget to charge your hearing aid, then it is a problem that can't be instantly fixed. For most, a 30-minute charge will get you at least two or three hours of hearing, but if you are the type of person who is likely to forget to plug them in regularly, then you're probably better off with standard batteries.

Rechargeable aids are also a little bit bigger and are only available in Behind-the-Ear models.

Finally, just like with a mobile phone, the amount of charge you get on day one is not going to be the same as you get a few years down the line. Be sure to ask what the policy is with the manufacturer's warranty when it comes to replacing the battery.

Are Behind-the-Ear hearing aids better than In-the-Ear hearing aids?

For most people, the answer is yes. But it's never that simple.

The majority of hearing problems affect the high frequencies a lot more than the low ones. Therefore, open fitting hearing aids sound a lot more natural and ones that block your ears up can make your own voice sound like you are talking with your head in a bucket. Therefore, in-ear aids tend to be less natural.

However, the true answer is we can't tell until we have had a look in your ears to assess the size of your ear canal, and until we have tested your hearing to see which frequencies are being affected.

People with wider ear canals tend to have more flexibility, also there are open fitting modular CIC hearing aids now that do not block your ears.

There is also the age-old rule to consider, that a hearing aid will not help you if it's sat in the drawer gathering dust. If the only hearing aid you would be happy wearing is one that people can't see, then that's what you should get.

Most people can adapt to any type of hearing aid, as long as they know what to expect. Have an honest conversation with your audiologist as to what your needs are.

What are channels, and how many do I need?

Generally speaking, six or more. Unless it's none at all.  The number of channels a hearing aid has is often a simplistic way an audiologist will use to explain why one hearing aid is better than another, but channels are complex, and it is really not that straightforward.  Here are some reasons why:

Hearing aids amplify sounds of different frequencies by different amounts. Most people have lost more high frequencies than low, and therefore need more amplification in the high frequencies. The range of sounds you hear is split into frequency bands or channels, and the hearing aids are set to provide the right amount of hearing at each frequency level.

Less than six channels, and this cannot be done with much accuracy, so six is the magic number. However, a six-channel aid is typically very basic with few other features and is suitable only for hearing a single speaker in a quiet room. The number of channels is not what you should be looking at; it's more the rest of the technology that comes with them.

As a final note, different manufacturers have different approaches. One method is not necessarily better than any other. For example, some manufacturers have as many as 64 channels in their top aids. Most tend to have between 17 and 20. One manufacturer has no channels at all.

What's covered in a manufacturer's warranty?​

Manufacturer's warranties typically last between 2-5 years, depending on the brand and model, and cover defects in materials and workmanship. This includes repairs for component failures, electronic malfunctions, and manufacturing defects, but excludes damage from misuse, accidents, or normal wear. Most manufacturers also include loss and damage insurance for the first year.

We handle all warranty claims on your behalf, liaising with manufacturers and ensuring you get replacement devices quickly when needed. This comprehensive warranty coverage, combined with our lifetime aftercare, gives you complete peace of mind.

How much does the hearing test cost?

Our hearing tests are completely free, whether at our clinics or in your home. Unlike other providers who charge £30-£100 for home visits, we believe hearing healthcare should be accessible without financial barriers. Our comprehensive assessments include examination by a registered audiologist, audiogram results, and personalised recommendations.

All testing, future adjustments, and ongoing support are included at no extra cost. While NHS tests are also free, typical 6-week waiting periods often lead people to seek immediate private testing. We provide prompt, professional assessments that fit your schedule and budget.

Do you offer home visits, and are they included in the price?

Yes, we offer completely free home visits throughout the UK, and this service is included in our prices with no additional charges. Home visits are particularly valuable for people with mobility issues, busy schedules, or those who simply prefer the comfort and convenience of their own environment.

Our audiologists can conduct full hearing tests, fit hearing aids, and provide ongoing support in your home.  This service sets us apart from many providers who either don't offer home visits or charge extra for them.

Why are your hearing aids cheaper than what I'd pay on the high street?

We can offer prices up to 40% lower than high street retailers because of our business model. As a network of 200+ independent audiologists, we don't have the massive overheads of large retail chains - no expensive high street premises, no sales targets pushing audiologists to sell the most expensive options, and no costly marketing campaigns.

However, we maintain the same buying power as the big chains because we purchase on behalf of our entire nationwide network. This means you get access to the same premium hearing aids with professional service, but at genuinely competitive prices.

How long do I have to try the hearing aids before committing to keep them?

We offer a comprehensive 60-day money-back guarantee, which gives you twice the industry standard time to properly assess whether your hearing aids are right for you. This extended period recognises that adjusting to hearing aids takes time, and your brain needs several weeks to adapt to the amplified sounds.

Unlike many providers who offer just 30 days, we believe 60 days gives you the confidence to test your hearing aids in all the situations that matter to you - from quiet conversations at home to busy restaurants and outdoor activities.

Other pages you might find useful

Audiology Home Visits - For free in the UK
View Audiology Home Visits - For free in the UK
Best hearing aids available in the UK for 2026
View Best hearing aids available in the UK for 2026
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View Hearing Tests at Home in the UK for free
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