» Hearing

Hearing loss ‘problems’ of CdLS children

Despite the fact that research and anecdotal evidence points to the fact that many - if not most - CdLS children suffer some degree of hearing loss, little information is available about the degree of the problem in the Syndrome.

Professor Valerie Newton heads-up Manchester University’s audiology department. She began by explaining that although the hearing problems experienced by CdLS children are no different to those in other children, diagnosis and treatment can be more complicated.

She said: “The importance of hearing may be obvious to us but it’s a good thing to go back to basics and define precisely what we use hearing for. Hearing is used for communication, for safety and for enjoyment and CdLS children are no different to others in this respect... although the results can be more dramatic.

“For instance, hearing problems can cause speech and language delays, behavioural problems and difficulties with mobility and balance. As these symptoms are frequently present in CdLS children anyway, hearing loss can accentuate them.”

CdLS children often have their ears positioned low on the head meaning that the ear canals may be narrow and malformed and this - combined with signs indicating possible hearing loss - should be investigated as early as possible, she said.

If a parent notices that their CdLS baby has little or no reaction to loud noises or that their infant doesn’t respond to voice or noise-making toys, then further investigation is essential, especially if the symptoms are associated with frequent colds and ear infections...or maybe a cleft palate.

“All babies are tested for hearing loss,” she said, “but it’s important that the parents of CdLS children realise the prevalence of hearing problems in the syndrome and that they continue to be vigilant for both existing hearing loss and for new problems arising.”

Fluid in the inner ear - following an infection for instance - is sometimes slow to clear in CdLS children. The Syndrome frequently results in a malformation of the eustachian tube which links the inner ear and the throat in order to equalise pressure and prevent the ear drum from being over-stressed. It’s this tube which causes ears to ‘pop’ in aircraft or even when travelling up or down steep hills in cars and if it is narrowed or blocked, it can prevent a build-up of fluid in the inner ear from dispersing naturally.

Normally, this fluid disperses spontaneously but if it becomes sticky and glue-like (as in glue ear) it may be necessary to insert grommets into the eardrum and/or remove the child’s adenoids. This is one symptom that is also successfully treated with hearing aids although the use of grommets is still a contentious subject, provoking a considerable degree of disagreement between specialists.

Finally, Professor Newton said that the diagnosis of hearing loss in CdLS children could - for obvious reasons - be difficult. And it’s here that some of the latest electrophysiological tests, that don’t require the infant or child to participate, can help enormously.
 
Reaching Out newsletter
October 2000