Down's syndrome
Many children with Down’s syndrome have some level of hearing loss. It’s important to find out if your child has hearing loss as early as possible so that they can get the right support to develop social, language and communication skills.
The Down Syndrome Medical Interest Group (DSMIG) offers guidance for professionals supporting children and adults with Down’s syndrome and hearing loss.
As a general rule, most people understand that his Down’s syndrome makes him who he is, but his deafness does get pushed to the side. I don’t think people quite realise how much it impacts him. There’s so much involved in it.”
Clarissa is mum to Griffin (3) who’s severely deaf and wears hearing aids.
How Down's syndrome causes deafness
Children with Down’s syndrome often have differences in the shape of their middle and outer ears. They are likely to have:
- small pinna (outer ear)
- ears which naturally cup forwards
- narrow ear canals
- small eustachian tubes (the tube that connects the throat and middle ear)
These differences can mean they’re more likely to experience some forms of hearing loss. Most children with Down’s syndrome will experience conductive hearing loss (when sound cannot pass properly to the inner ear). This can be temporary.
Around 6% of children with Down’s syndrome have sensorineural hearing loss, which is permanent. This increases to 20% of adolescents and young adults.
Glue ear
Glue ear (also known as otitis media with effusion or OME) is a common childhood condition which causes conductive hearing loss. It happens when the middle ear becomes filled with sticky fluid.
Children with Down’s syndrome often have glue ear for longer than hearing children because they have smaller eustachian tubes. Children with Down’s syndrome often begin experiencing glue ear before the age of 1 and continue to experience it until they are 10. In some cases, glue ear can continue into adulthood.
Earwax
Wax is a common problem for children and adults with Down’s syndrome because it easily blocks the narrow ear canals and can cause hearing difficulty. For children who use hearing aids, wax can cause feedback (a whistling noise). Eardrops may not help because when the ear canal is very narrow, it can easily become even more blocked by the drops and wax combining.
If you’re concerned about how earwax is affecting your child, ask your GP or audiologist to check your child’s ears and arrange for wax to be removed by your ear, nose and throat (ENT) doctor if necessary.
If you’re experiencing long waits for ENT treatment, you may be able to have your child’s earwax removed privately. It's important to make sure the person removing your child’s earwax is a trained, registered health professional such as an audiologist, ENT nurse or doctor, or pharmacist.
Hyperacusis (sensitivity to noise)
Studies have shown that a few children with Down’s syndrome are particularly sensitive to noise. These children may be distressed in some situations, especially when there is a lot of background noise. If this seems to be a problem, discuss this with your child’s audiologist.
Visit Tinnitus UK for more information about hyperacusis.
Hearing tests
All children with Down’s syndrome should be referred to audiology after diagnosis. If they pass their hearing tests, they should be seen again at:
8 to 10 months old
every 6 months until the age of 2
at least once a year until they leave school
If you feel your child’s hearing may have changed between appointments, ask for an earlier appointment.
Order or download our free guide 'Understanding your child’s hearing tests' (Shopify) to learn about the different types of hearing tests.
Managing hearing tests
Children with Down’s syndrome typically need extra time to complete hearing tests. It may take longer to understand how the audiologist wants them to respond, and be more difficult to keep them interested and engaged in the tests.
There are some things you can do to make hearing tests easier for your child.
- Ask your audiology service if it’s possible to book a double appointment as a reasonable adjustment for your child’s needs.
- Ask if you can have an appointment at a quiet time when your child will not have to wait too long.
- If you know your child will sleep at a particular time of day, or be more alert at certain times, ask if the appointment can be scheduled at a time when your child is at their best.
- Bring books and toys to keep your child occupied in the waiting room.
- Use videos and social stories to prepare your child for their hearing test.
Evalina London have produced videos to help parents and children prepare for hearing tests.
Some children will need different types of hearing tests, over multiple appointments, to build an accurate picture of their hearing.
Order or download our free comic 'Going to the hearing clinic' (Shopify) to remind older children what will happen at a hearing test.
Hearing aids and implants
Lots of children with Down’s syndrome benefit from using hearing aids. However, the shape of the outer ear can make it difficult to keep a behind-the-ear hearing aid in place. You can use headbands, surgical tape (like Micropore) or wig tape to hold the hearing aid in place.
Read our tips for encouraging babies and children to keep their hearing aids on.
Some children find it difficult to wear behind-the-ear hearing aids because they get a lot of ‘feedback’. This is caused by sound from the hearing aid bouncing back off built-up earwax or the wall of the ear canal, making a whistling sound. Narrow ear canals can also make it difficult for earmoulds to fit properly. These children may benefit from other types of hearing devices.
Bone conduction hearing aids
Bone conduction hearing aids use a vibrating pad that allows sound to be conducted through the bone rather than through the middle ear. They’re worn on a headband with the vibrator behind the ear, resting on the mastoid bone (part of the skull behind the ear).
Children with permanent conductive hearing loss who find a bone conduction hearing aid useful may also be suitable for a bone anchored hearing device.
Find out about bone anchored hearing devices.
Cochlear implants
Cochlear implants can be useful for children who have severe to profound sensorineural deafness and cannot hear the full range of speech sounds with hearing aids.
Grommet surgery
Grommets are a surgical treatment for glue ear. Grommets are tiny plastic tubes, inserted into the ear drum, which help air to circulate in the middle ear to prevent fluid from building up.
Grommet operations can be very difficult in young children with Down’s syndrome because the ear canal can be too narrow for the surgeon to operate.
Older children may be offered grommet surgery once their ear canals have grown bigger, but the grommets may not work for very long. The grommet itself may become blocked, or it may slip out of the eardrum sooner than it should.
If your child has already had grommet surgery and their glue ear has come back, it may be possible to have the procedure done again. However, repeated grommet surgeries increase the risk of complications.
Order or download our free comic, 'Harvey gets grommets' (Shopify), to prepare your child for grommet surgery.
Full references for this webpage are available by emailing
informationteam@ndcs.org.uk