Microtia and atresia

What are microtia and atresia?

Microtia describes when an outer ear is not fully formed. It can range from an ear that is just slightly smaller than the normal one to an ear that is substantially different.

Atresia is the absence of a complete ear canal.

The two conditions will often but not always occur together. 80% of microtic ears also have an atresia.

Which professionals will help my child with their ears and hearing?

Children with microtia and atresia should be seen by a wide range of professionals who will each have a different role in their care. They will cover three main aspects which are cosmetic, audiological and psychological.

What is the role of the ENT Consultant?

The ear, nose and throat (ENT) consultant will examine the ear, discuss possible causes, outline options and may refer on to other professions such as plastic surgery, genetics or psychology.

They will be able to give advice and suggest treatment for the hearing loss including any fluctuating conductive loss which might occur in the better hearing ear.

Routine checks including blood and urine tests, kidney scan, vision test and a CT scan of the inner ear will need to be carried out. Your ENT consultant should arrange for these at the appropriate time. Sometimes a paediatrician will be the doctor to request these tests.

Who will provide cosmetic treatment and when will this be possible?

The ENT doctor will discuss the options for cosmetic treatment quite soon after diagnosis so that parents know what is available. Treatment itself is carried out in specialised centres. Where your child will be seen depends on the type of treatment chosen. Procedures are not usually carried out before the age of 9. There is a lot of emphasis on the child deciding whether and when to have surgery and what type to choose.

What is the role of the audiologist?

Your child will be seen by an audiologist soon after birth or soon after diagnosis of the condition. They will check their hearing and discuss the types of hearing aid that could be helpful. They will discuss all options including not doing anything at all just yet.

Hearing levels will be checked regularly so that you have information necessary to make choices as time goes on. If a hearing device is chosen we will see regularly to check that it is appropriately fitted and giving benefit. Usually children have normal hearing in the unaffected ear but whether the decision is made to use a hearing device or not they will need to be seen regularly to ensure that the hearing remains good in the better ear.

What types of deafness are associated with microtia and atresia?

Usually the affected ear will have a conductive hearing loss. This is when the inner ear works well but the sound cannot reach it effectively.

Sometimes there can be a ‘sensorineural’ hearing loss. This is when it is the inner ear that is affected. There can also be a combination of the two, which is known as a mixed loss.

In 90 percent of children only one ear is affected.

What type of hearing aid might be helpful?

There are a number of different options. The audiologist can tell you about these.

Usually a bone conduction hearing device, known as a ‘BCHD’ is chosen. They are useful when a conventional hearing aid can’t be used because they enable sound to pass directly through the skin to the inner ear. They can be worn on a band or can be fitted after a surgical procedure. Different types are available.

For some children a middle ear implant becomes a possibility when they are older.

What other groups might offer support and advice?

There are many groups who can help and support your child.

The National Deaf Children’s Society (NDCS) will provide information for relating to any level and type of hearing loss in children.

The Sheffield Deaf Children’s Society (SDCS) is the local group who provide support and social activities for Sheffield children.

There are groups set up specifically to help children who have a physical difference such as Changing Faces and Microtia UK.

Sometimes microtia and atresia are part of a group of signs known as a syndrome. Examples of such syndromes are Treacher Collins and Goldenhar. Your doctor will have told you whether your child has one of these syndromes and more information will then be available.