Hearing loss in children with Down syndrome

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).

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. 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.

• 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.