Hearing impairment and Down syndrome
It is thought that between 40 per cent and 80 per cent of people with Down syndrome have a hearing impairment.
Better developmental outcomes for children with hearing loss result when interventions of support and resources are put in place as early as possible.
Types of hearing loss in children with Down syndrome
Children with Down syndrome can have either a conductive hearing loss or sensori-neural hearing loss.
Conductive hearing loss is more common in these children, but both types are more common than in the general population. It is also possible to have a combination of the two. Possible treatments will depend on which kind of hearing loss a child has.
Conductive hearing loss occurs when sounds are unable to pass from the child’s outer ear to their inner ear, as the result of a blockage or a disorder of the hearing bones. In Down syndrome this is commonly as a result of otitis media with effusion, also known as glue ear. This is defined as inflammation of the middle ear, accompanied by the accumulation of fluid in the middle-ear cleft.
Practitioners or parents who suspect the presence of glue ear should seek an initial assessment from a GP, or formal assessment from a paediatric audiologist.
Features that suggest that a child may have glue ear include:
- Repeated ear infections or earache
- Indistinct speech or delayed language development
- A history of recurrent upper respiratory tract infections or frequent nasal obstruction
- Behavioural problems, particularly lack of concentration or attention, or being withdrawn
- Hearing difficulty (for example, mishearing when not looking directly at the speaker, difficulty in a group, asking for things to be repeated)
- Poor educational progress
Treatment of glue ear
In most instances of uncomplicated glue ear, no intervention is required - because the fluid clears spontaneously and the hearing recovers. Where the condition persists it is recommended that there is a three month period of observation to determine whether the condition passes spontaneously or needs further investigation.
After this, children with persistent glue ear in both ears causing a significant hearing loss should be considered for the surgical insertion of ventilation tubes, known as grommets. A grommet is a very small tube that is inserted into a child's ear through a small cut in their eardrum. It drains away fluid in the middle ear and helps to maintain the air pressure in the middle ear cavity.
Hearing aids should be offered to children with persistent glue ear as an alternative to grommets where surgery is not considered acceptable.
It should be noted that because many children with Down syndrome have smaller ears and narrower ear canals than the general population, particular care should be given to the fitting of hearing aids.
Sensori-neural hearing loss occurs whenthe sensitive hair cells, either inside the cochlea or the auditory nerve, are damaged. It is a permanent hearing loss.
Children are screened for hearing loss in the first few weeks of life under the NHS Newborn Hearing Screening Programme.The possibility of sensori-neural hearing loss can be identified at this time and will lead to a referral to an audiologist for more detailed tests.
Treatment of sensori-neural hearing loss
Finding out that a baby has a hearing loss early means that parents will get advice and support right from the start. If a sensori-neural loss is confirmed after testing, the audiologist will explain what the results mean in detail.
The audiologist will be able to explain to parents which sounds their baby can hear and which sounds they may have difficulty hearing.
Children with a sensori-neural hearing loss may benefit from the use of hearing aids in one or both ears. They may benefit the child by magnifying sound vibrations entering the ear. Surviving hair cells inside the cochlea or auditory nerve detect the larger vibrations and convert them into neural signals that are passed along to the brain.
Cochlear implants may be recommended for children with severe to profound hearing loss if they do not get enough benefit from hearing aids after trying them for three months. A cochlear implant team should carry out an assessment to find out if an implant would help before they consider proceeding.
Sense Children’s Specialist Services
Sense's Children's Specialist Services are a team of specialist advisory teachers, children’s therapists, and Children and Family Support Workers.
The team provides advice and information to multi-sensory-impaired children and young people, to their families or carers, and to the professionals who work with them. They also provide support in the home, at school or at their centres of excellence.
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Reviewed: May 2015
Review due: May 2017
First published: Thursday 20 June 2013
Updated: Tuesday 22 December 2015