Ears - Hearing loss in children with Down syndrome

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Hearing loss is common in children with Down syndrome.  Children with Down syndrome have issues with speech and language development.   If there is also a hearing loss, then a very severe language delay can occur.  All children with Down syndrome should be regularly seen by both the family doctor and a paediatrician.

Do all Down syndrome children have hearing loss?

Many children and adults with Down syndrome can have temporary, permanent or even fluctuating hearing loss.  There are a number of causes of this hearing loss. 

How severe is the hearing loss?

Hearing loss may vary from being a mild or even a one sided hearing loss right up to profound permanent hearing loss in both ears.  It may also be a fluctuating hearing loss ie, reasonably good hearing at times and poor hearing at other times. 

How will a hearing loss effect my child with Down syndrome?

As almost all children with Down syndrome have some degree of intellectual disability, they will have more difficulty developing speech.  If a child with Down syndrome does not have a hearing loss the child’s ability to develop speech is dependent on the level of intellectual ability (ability to learn).  If the child has only a mild intellectual disability (and no hearing loss) he or she usually develops a great deal of speech and can both read and have a simple conversation with family or friends.  If a child with Down syndrome has a very severe degree of intellectual disability, they are likely to have a great deal of difficulty developing spoken language, and may need to use signs and/or pictures (augmentative communication) to communicate.  However, if a child with Down syndrome has hearing loss as well as intellectual disability, then there will be more difficulty with speech development, unless we treat this problem very quickly.  Constantly watching for hearing loss in any child or adult with Down syndrome is essential.  Even adults with Down syndrome can develop hearing loss for the first time in adult life.

How do we know if a child with Down syndrome has hearing loss?

A permanent hearing loss in a child with Down syndrome is often picked up at the newborn screen of hearing (SWISH).  Unfortunately, many children with Down syndrome acquire hearing losses after birth and these may be picked up by behavioural testing (the “puppet” test) or play audiometry performed by an audiologist.  If the child is not able to do behavioural testing, hearing can be tested (if needed) by special electrical computer-assisted electrophysiological testing (brainstem auditory evoked responses or electrocochleography). This is performed under sedation or an anaesthetic.

What types of hearing loss occur in Down syndrome and how do we treat them?

Some children with Down syndrome may have a permanent sensorineural loss and may need hearing aids from the newborn period onward.  Many other children with Down syndrome have poor eustachian tube function and small nasal passages.  These children become congested, often for long periods and fluid accumulates in the middle ear (behind the ear drum).  All children with allergic rhinitis (“hay fever” or “sinus”) are at particular risk.  When the child with Down syndrome has to listen through this layer of fluid (which may be thick and sticky ie, glue ear) they often have quite a marked hearing conductive loss (as much as 50 decibels).  Ear, Nose and Throat Surgeons may treat this hearing loss by inserting ventilating tubes (“grommets”) (sometimes needed repeatedly).  Antibiotics may also be needed when there is an acute infection.  In some cases when the child repeatedly accumulates middle ear fluid, the Ear, Nose and Throat Surgeon may request the child’s audiologist to fit hearing aids.  Obviously, if hearing aids are fitted because of chronic middle ear fluid, the hearing should be tested frequently (at least once every four months) to check if the middle ear fluid is still present.  An Ear, Nose and Throat  Surgeon will also need to check the condition of the eardrums frequently.  If the child has repeated or persistent discharge from the ear or if the eardrum collapses inward over the middle ear structures (atelectasis) then surgery might be needed to correct this problem in order to prevent further ear disease developing. 

Can a child with Down syndrome have permanent/long term conductive hearing loss?

Despite careful management, many children with Down syndrome seem to have some degree of conductive hearing loss each time they are tested ie, hearing loss due to problems in the middle ear.  This may go on for many years and occasionally it may -be permanent.  Rarely, there may be structural abnormalities of the middle ear in children with Down syndrome, but usually permanent or long term conductive hearing loss occurs because the mucous membrane lining the middle ear is always slightly congested or swollen, or the eardrum has become scarred or stiffer than normal.  If long term or permanent conductive hearing loss occurs in children with Down syndrome hearing aids are usually very helpful.

Surgical advances now mean that implantable hearing devices, which give a clearer, sharper and more reliable sound than hearing aids, are available for consideration in certain situations..

What are “mixed” hearing losses in children with Down syndrome?

A ‘mixed’ hearing loss means that the electrical (sensorineural) and the mechanical (conductive) parts of the ear are both not working well. The sensorineural loss is permanent and the conductive loss may be temporary, permanent or fluctuate. The sensorineural loss needs a hearing aid and often this can be adjusted up to help the conductive loss. Fluctuating hearing loss is difficult to handle because at times the hearing loss will naturally improve and the amount of power that the hearing aid provides will be too much.

Children with Down syndrome who have a mixed hearing loss have some permanent hearing loss due to abnormalities in the inner ear ie, the cochlea. Together with this permanent hearing loss, they have an extra hearing loss due to problems with the middle ear.  In these children, we aim to treat the middle ear problems if possible, but the hearing aids should be set to cater for the extra degree of hearing loss.  A fluctuating hearing loss often seems to make language development particularly difficult for a child with Down syndrome.  If speech is not developing steadily in a child with mixed hearing loss, alternative means of communication should be introduced along with spoken words. 

A speech pathologist or teacher of the deaf may also be very helpful and may recommend any of Makaton signs, photos or picture systems to assist the development of communication with Down syndrome.

Close collaboration between the audiologist, speech pathologist, family doctor, paediatrician, ear nose and throat surgeon and carers will help make the best treatment decisions.

 

Remember:

  • Children with Down syndrome frequently have temporary, fluctuating or permanent hearing loss.  This may lead to very severe speech delay if left untreated.
  • Children with Down syndrome who have middle ear disease need frequent hearing tests and supervision by both an ENT surgeon and a paediatrician.
  • Fitting hearing aids in the child with Down syndrome is often very helpful in maximising the child’s opportunity to hear well and to develop good speech.  For information and fitting of hearing aids contact Australian Hearing 131 797.
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The Children's Hospital at Westmead
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Sydney Children's Hospital, Randwick

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