SoundSpace Online

Explore Causes of Hearing Loss in more detail :

Syndromes

The term “syndrome” refers to a combination of different observable clinical findings which are thought to have the same underlying cause. Sometimes a child is born with hearing loss in combination with other difficulties but a specific cause is not found-in which case the term used is “association”. An important point is that syndromes can be associated with different degrees of hearing loss (see SoundSpace Online - Hearing Loss represented on an audiogram for explanation of severity) and also different types (see SoundSpace Online - Types of hearing loss), as well as different degrees of risk of hearing loss. Syndromes are not always genetic.

Types of inheritance and syndromic hearing loss

As with genetic causes of hearing loss more generally, syndromes can be classified as due to “recessive”, “dominant” and “other genetic” causes. For an explanation of these terms, and the distinction between them, please go to section SoundSpace Online - Genetic hearing loss.

Some of the relatively common dominant syndromes are Treacher-Collins syndrome (in which the person has a conductive hearing loss and particular facial features, such as underdeveloped cheek bones) and Waardenburg syndrome (this includes prematurely greying hair, a white forelock and typically sensorineural hearing loss). Recessive syndromes associated with hearing loss include Pendred’s syndrome (different types of hearing loss, sometimes abnormalities of the cochlea, which exist alongside goiter) and Usher syndrome (a combined visual/hearing loss which develops over time).

“Chromosomal” syndromes are those that are associated with a disorder in a particular part of the chromosome, small structures which sit within the nucleus of our cells. Down’s syndrome is the most common such syndrome that can lead to hearing loss. Although it is not associated with an increased risk of congenital sensorineural hearing loss, it is still important in the context of hearing loss because children with Down’s are likely to have more persistent glue ear (or otitis media with effusion-see nhs.uk/conditions/otitis-media). Also, glue ear in people with Down’s syndrome is thought to be associated with greater degrees of hearing loss than is the case with non-Down’s children, and also a much higher rate of ear infections and other factors affecting the middle ear and associated with persistent conductive hearing loss. Also, individuals with Down’s syndrome tend to develop age-related hearing loss much earlier than people without Down’s. Please visit the Down's Syndrome Association website for more information on Down's syndrome. 

More information about syndromes and hearing loss

Overall, there are a large number of identified syndromes that have some association with increased risk of hearing loss. A good overview can be found in ([203]) - see reference list for different options. Please also visit this IN.gov website page which provides a list of the most common syndromes and genetic conditions associated with hearing loss.