Fact Sheet - Kids Health @ CHW

Fact Sheet
Hearing loss in children with Down syndrome Page 1
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Hearing loss is common in children
with Down syndrome and if no steps
are taken to minimise it hearing loss
may lead to very severe language
delay in these children.
Do all Down syndrome
children have hearing
loss?
Unfortunately a very large fraction of
patients with Down syndrome have
hearing loss either permanently or
temporarily at some stages in
childhood or in adult years. There are
a number of causes of this hearing
loss.
How severe is the hearing
loss?
It 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
affect my child with Down
syndrome?
As almost all children with Down
syndrome have some degree of
intellectual disability, we expect them
to have more difficulty developing
speech than do normal children. If
the child with Down syndrome does
not have a hearing loss we expect the
child’s ability to develop speech to be
somewhat dependent on the level of
intellectual ability. Therefore, 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 have a simple
conversation with family or friends. If
the child has a very severe degree of
intellectual disability we expect the
Down syndrome child to have a great
deal of difficulty developing spoken
language, and we may use signs
and/or pictures (augmentative
communication) to help us
communicate with that child.
However, if a child with Down
syndrome has hearing loss as well as
intellectual disability, unfortunately
we must expect even more difficulty
in developing speech, unless we treat
this problem very quickly. We must
constantly watch for hearing loss in
any child or adult with Down
syndrome. 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?
As mentioned, a child with Down
syndrome may have any degree of
hearing loss from none at all to a
profound loss. Permanent hearing
losses in children with Down
syndrome are often picked up at birth
when the child has the newborn
screen of hearing. However,
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 electrophysiological
testing under sedation or anaesthetic
(brainstem auditory evoked
responses or electrocochleography).

Fact Sheet
Hearing loss in children with Down syndrome Page 2
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What types of hearing
loss occur in Down
syndrome and how do we
treat them?
Some children with Down syndrome
may have 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).
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 at least 50
decibels of conductive hearing loss.
Ear, Nose and Throat Specialists may
treat this hearing loss by inserting
grommet tubes (often needed
repeatedly). Antibiotics may also be
needed. In some cases when the
child repeatedly accumulates middle
ear fluid, the Ear, Nose and Throat
Specialist may simply 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 Specialist will also need to
check the condition of the eardrums
frequently. If the child has
experienced severe ear infections or
if the eardrum collapses inward over
the middle ear structures
(atelectasis) then the ENT specialist
may need to operate to correct this
problem.
Can a child with Down
syndrome have
permanent/long term
conductive hearing loss?
Even despite careful management,
many children with Down syndrome
always seem to have some degree of
conductive hearing loss when tested
ie, hearing loss due to problems in
the middle ear. This may go on for
many years and occasionally it may
appear to 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.
What are “mixed” hearing
losses in children with
Down syndrome?
• Children with Down syndrome
who have mixed hearing losses
have some permanent hearing
loss due to abnormalities in the
inner ear ie, the cochlea, and as
well as this permanent hearing
loss, they have 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.

Fact Sheet
Hearing loss in children with Down syndrome Page 3
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A fluctuating ie, variable degree of
extra hearing loss often seems to
make language development
particularly difficult for the child with
Down syndrome. If oral speech is not
developing steadily in a child with
mixed hearing loss, it is wise to
introduce alternative means of
communication to be used along with
spoken words. Makaton signs,
photos or picture systems may be
very useful supplements to assist the
development of communication. A
speech pathologist or teacher of the
deaf may also be very helpful to
families of children with Down
syndrome.
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 an ENT specialist.
• 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. Ring
Australian Hearing 131 797.
This fact sheet is for education purposes only.
Please consult with your doctor or other health professional
to make sure this information is right for your child. This document was reviewed on 28 th May 2010.
www.chw.edu.au www.sch.edu.au www.kaleidoscope.org.au
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