What are microtia and atresia?
Microtia is the term used to describe malformation of the external part of the ear (the pinna). Microtia varies in severity from minor changes, the ear may be smaller than expected, to complete absence of the pinna. Absence of the ear canal (external auditory meatus) is called atresia.
Miocrotia is frequently associated with atresia because a baby’s outer ear and ear canal develop together during pregnancy. It is something that babies are born with i.e. it is congenital. It can affect one ear (unilateral) or both ears (bilateral) less commonly. It can occur by itself, or with other craniofacial anomalies or as part of a syndrome. Common syndromes which may involve microtia are goldenhaar, hemifacial microsomia or treacher-collins.
Image © Scott Maskell
What does this mean for my child?
Children with microtia and atresia do not have a normal pathway for sound to reach the inner ear on the affected side. When there is no outer ear canal or a very narrow ear canal, the ear drum and middle ear can be affected too. Usually sound is transmitted through the middle ear by the small bones of hearing but in microtia and atresia this may not be possible. However, even though the outer and middle ear can be affected by the condition, often the inner ear is healthy. If the situation is favourable the hearing can be restored by bypassing the parts which aren’t present or working properly and taking sound directly to the inner ear.
The other important consideration is the appearance of the outer ear. There are two main possible options for improving this; one is a surgical reconstruction and the other is to have a prosthetic ear. Our team has specialists with the skills and equipment to provide a suitable long-term solution for your child.
What if one or both ears are affected?
If one ear is affected, often the other ear can hear normally. This will usually be confirmed with hearing tests at a young age. These children still benefit from stimulation to the affected side and so we advocate aiding on that side as soon as possible.
If both ears are affected, children will definitely need a form of hearing aid to help them develop their speech and language. This is initially a bone conduction hearing aid which bypasses the blocked outer and middle ear, conducting sound directly to the inner ear. Small children can simply have this held in place with a headband. Older children can have other more permanent alternatives.
At St Thomas’ hearing implant centre, we can offer many types of more permanent solutions such as implantable bone conduction hearing aids and middle ear implants. The options will depend upon the parts of your child’s hearing pathway that are affected. The hearing rehabilitation options will be discussed within our team of experts to offer your child the most appropriate hearing solution tailored to their specific needs. This field is developing all the time and our specialists have access to the newest available solutions. For children who go ahead with hearing implant surgery, a full rehabilitation package is offered.
The Specialist Microtia and Atresia Clinic
Our clinic occurs 4-5 times per year. Before the clinic we hold an information session for patients, parents and families so we can explain more about the condition and the treatment options. The clinic involves meeting all the specialists together which enables us to optimally coordinate care for children with this complex problem.
A number of appointments are likely to be necessary to assess, discuss and plan management. It also may require appointments for further tests such as hearing and language tests or scans of the ear. As a general rule implantable hearing solutions are considered from the age of 3 and a half whereas ear reconstruction is not possible until children are older – usually between 8 and 13.
- ENT surgeons: Professor Dan Jiang, Ms Irumee Pai, Mr Harry Powell
- Plastic surgeon: Miss Norma Timoney
- Maxillofacial prosthetic rehabilitation specialist: Dr Trevor Coward
- Audiological scientists: Marsha Jenkins, Terry Nunn, Tisa Thomas
- Speech and language therapists: Kathryn Webb, Caroline Bartrop
With access to psychological support and genetic counselling.
Please address referrals to Professor Jiang, Mr H Powell or Marsha Jenkins.
St Thomas' Hearing Implant Centre
Guy's and St Thomas' NHS Foundation Trust
2nd Floor, Lambeth wing
St Thomas’ Hospital
Westminster Bridge Road,
London, SE1 7EH