Hearing Loss

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.

About Hearing Loss

There are two types of hearing loss:  
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 http://www.nice.org.uk/guidance/cg60. This guidance was last reviewed in Feb 2014. A summary of the guidance with commentary by Patrick Sheehan was published in DSA Journal 118 ‘Management of glue ear’.
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 difficulties in children
Further information about hearing difficulties in children with Down’s syndrome can be found in the booklet ‘Down’s syndrome and childhood deafness’. This can be downloaded, after free registration, on the National Deaf Children’s Society website
Hearing obviously plays an important part in children’s development and learning, especially in speech & 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: Summary of Early Hearing Loss and Language Abilities in Children with Down’s Syndrome Research by Glynis Laws and Amanda Hall
If your child needs to wear a hearing aid, you may find this article by Patrick Sheehan useful ‘Hearing Aid Advice’ (this article was published in DSA Journal 118).
Resource
Early Support Information on Deafness and Hearing Loss (Council for Disabled Children)

Older children

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.

Adults

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
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.  The Hearing and Learning Disabilities (HaLD) Special Interest Group (SIG) is a useful source of advice for professionals and carers concerned with people with learning disabilities and hearing difficulties
There is information for your GP about ear and hearing issues on our website: Annual Health Check Information for GPs
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.
Please do not hesitate to contact the DSA helpline if you have any further questions: 0333 1212 300