Hearing loss will affect many people with Down’s syndrome at some point in their lives. This may be mild temporary hearing loss or a longer term problem with hearing. Here are some of the reasons that may cause higher levels of hearing loss in people with Down’s syndrome:

• increased incidence of chronic ear diseases
• differences in the structure of the ear
• weaker immune systems.

Types of hearing loss

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Conductive hearing loss – This is usually a temporary hearing difficulty where sounds cannot pass freely into the inner ear.  In children this often happens because they have a build-up of fluid in the space behind the eardrum (glue ear, sometimes referred to as Otitis Media with Effusion OME).  It can also occur as a result of a build-up of ear wax or an ear infection.

Because this is a common problem, guidance from the National Institute for Clinical Excellence (NICE) about the management of glue ear has a specific section on the management of glue ear in children with Down’s syndrome. This guidance was last reviewed in Feb 2014.

Patrick Sheehan, ENT consultant surgeon has written a summary of the NICE guidelines on the management of glue ear in children with Down’s syndrome

Sensorineural hearing loss – This is a permanent hearing difficulty that happens where there is damage in the inner ear. The hearing nerve can sometimes also be affected. Some children with Down’s syndrome will be born with this type of hearing loss and some may develop it as they get older.

It is possible to have both conductive and sensorineural hearing loss.

Hearing loss in children

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Hearing obviously plays an important part in children’s development and learning, especially speech and language and social skills. Therefore it’s vital that all children with Down’s syndrome have their hearing tested regularly. Hearing tests will be carried out by an audiologist at your local audiology services. You can ask your child’s paediatrician, GP or health visitor to make a referral to the audiology service.

The UK Down Syndrome Medical Interest Group recommends that :

• all new babies have universal newborn hearing screening
• there is a full audiological review at 10 months including a hearing test and impedance check followed by yearly audiological reviews until a child starts school.
• When a child starts school, an audiological review every two years or more frequently if recommended.

Recent research highlights the need for  audiology and speech and language therapy services to work together as soon as a child is diagnosed with severe, ongoing hearing difficulties.

Resources 

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Down’s Syndrome And Childhood Deafness

Patrick Sheehan, ENT Consultant Surgeon gives advice and tips on hearing aids for children with Down’s syndrome.

Early Support Information on Deafness and Hearing Loss

Hearing tests for all by Dr Lynzee McShea, Senior Clinical Scientist Audiology

The importance of good hearing for well-being by Siobhán Brennan, Audiological Scientist

GPs and carers may also find the following posts useful:

Supporting adults to have a hearing test – tips for GPs and Supporting adults to have a hearing test – tips for carers both by Dr Lynzee McShea, Senior Clinical Scientist Audiology

Hearing, teenagers and Annual Health Checks  

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14 to 17 year olds are now entitled to free Annual Health Checks with their GP. Information about Annual Health Checks for people with Down’s syndrome can be found here. Your GP should look at your ears as part of the Annual Health Check.

There is information for your GP about hearing issues here

Hearing, adults and Annual Health Checks

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As with children, adults with Down’s syndrome may experience hearing loss due to glue ear. Some other common causes of hearing loss in adults are:

• a build-up of impacted wax in the ear canal
• changes in the structure of the ear as a person ages
• middle ear infections

Adults with Down’s syndrome should have:

• an audiological assessment every year (including auditory thresholds, impedance testing)
• inspection of their ears at their Annual Health Check

Information about Annual Health Checks for people with Down’s syndrome can be found here

There is information for your GP about hearing issues here

Anyone with sudden unexplained hearing loss should be referred for an audiological assessment. If possible, hearing tests should be carried out by an audiologist with experience in seeing people with a learning disability.

If an older adult is displaying signs of confusion or changes in behaviour, it is always a good idea to check for an underlying medical cause such as a loss of hearing.  Don’t always suspect the worst. We have come across a few cases over the years where too much ear wax, which affects the person’s ability to hear, has been misinterpreted as Alzheimer’s disease.

Useful organisations

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National Deaf Children’s Society (NDCS)

NDCS offer free information and support to families with a deaf child via their Helpline, website and web forums 

Hearing and Learning Disabilities Special Interest Group  

The Hearing and Learning Disabilities Special Interest Group is for professionals and carers concerned with people with learning disabilities and hearing problems.

All members of the Group work with people with learning disabilities. They come from a variety of professional backgrounds including, Speech and Language Therapy, Audiology, Hearing Therapy, Audiological Medicine, Community nursing and Social Care. Group members support and learn from each other thereby improving hearing services for people with learning disabilities across the NHS.