Hearing loss is more common in people with Down syndrome (DS) of all ages. Hearing loss is typically divided into three categories:
Conductive. This involves the outer and middle ear and may include things such as cerumen (ear wax) blocking the ear canal or serous otitis (fluid behind the ear drum). Both conditions seem to be more common in people with DS.
Sensorineural. This involves the inner ear. Aging and chronic exposure to loud noise are possible causes of this type of hearing loss. In our experience, presbycusis (hearing loss associated with aging) occurs at a younger age in people with DS.
Mixed. This is a combination of conductive and sensorineural.
Sometimes, causes are reversible, such as ear wax blocking the canal or fluid behind the ear drum. It can also be due to permanent causes such as inner ear problems or damage. Other problems are not necessarily permanent but can be more challenging to treat. These include perforation in the tympanic membrane (ear drum) or damage to the small bones in the middle ear.
Hearing problems may be difficult to detect in adults with DS. If the person does not or cannot complain of discomfort in their ears or of their difficulties hearing, behavior changes or skill loss related to hearing loss may be attributed to psychological or other causes. We have evaluated many people who have had a behavioral change or seem to be losing skills in which hearing loss contributed to the symptoms.
Because of the high prevalence of hearing problems in people with DS, we recommend that adults with Down syndrome have their hearing checked every one to two years.
Audiologists are the professionals who assess hearing. An otolaryngologist (ear-nose-throat/ENT doctor) may also be part of the assessment of underlying problems. During a hearing test, sounds are played (typically through headphones but sometimes in a sound booth) and the individual is asked to respond when the sound plays. For those who are unable to do that, alternative testing is available.
When hearing loss is found, treatment includes addressing reversible causes (such as removing ear wax from the canals) or providing hearing augmentation (hearing aids) when there is not an otherwise reversible cause. While some people with DS find wearing hearing aids very difficult, many are able to use them successfully.
Information about hearing in children and adolescents with Down syndrome can be found in Health Supervision for Children and Adolescents with Down Syndrome (the pediatric medical care guidelines).