Microtia is the medical word to describe a small or absent ear. If you have microtia, you will have had it from birth. It is treated at Great Ormond Street Hospital (GOSH) with cosmetic procedures.
Microtia is congenital (present from birth) and can appear on its own or alongside other symptoms as part of a syndrome. Some syndromes that have microtia as a feature include:
- Goldenhar syndrome
- hemifacial microsomia
- Treacher-Collins syndrome
Microtia can affect one ear only (unilateral) or both ears (bilateral). Around ten per cent of people with microtia are affected in both ears.
Microtia is a rare condition affecting about one in 7,000 babies. It seems to affect more boys than girls, and affects right ears more than left ears, although we don’t yet understand why this should be the case. It also seems to be more common in Asian people than people of other races, but again we don’t yet know why.
Microtia and deafness
Most people with microtia will have some degree of hearing loss on the affected side. This is because the middle ear, which contains the eardrum and tiny ear bones, is affected as well as the outer ear. In some people, the ear canal is blocked or absent, so sound waves can’t pass through the ear in the normal way. Generally, although the outer and middle ear is affected, the inner ear is healthy so some options for restoring hearing are available. We don’t recommend operations to repair the hearing tube and middle ear as they don’t offer very good success rates and, in some circumstances, could damage hearing further.
People with unilateral microtia usually have normal hearing in their other ear, although this must be confirmed with hearing tests at a young age. Having normal hearing in only one ear will not usually cause any speech and language delay, and there are ways to make the most of your hearing while at school, for instance. It is unusual for a person with unilateral microtia to need a hearing aid.
People with bilaterial microtia will usually need some form of hearing aid at a young age to enable them to develop speech. This is often a ‘bone conduction hearing aid’ that transmits sound waves to the inner ear through the bones of the skull. In very young children, these hearing aids are usually on a headband, but as a child grows older a ‘bone anchored hearing aid’ might be better.
It’s obvious at birth if you have a small or absent ear. Hearing tests will have been used to work out whether you have any hearing in the affected ear, and if so, to what degree. Hearing in the unaffected ear will also be confirmed. This is helpful for planning your education.
If your microtia was suspected as part of a syndrome, you probably had other tests to confirm or rule out the diagnosis. Some syndromes are inherited, in which case you may be offered genetic counselling.
All the treatment options available are cosmetic. That is, they improve the look of the ear but can’t improve its function. There are three options for treatment available:
- no surgical treatment
- ear reconstruction using a rib graft
- false (prosthetic) ear
More information about each option follows. The team will discuss each option with you, and the final decision about which option to take will be made jointly between you, your family or carers and the microtia team.
No surgical treatment
While you’re young, you may decide to leave the ear as it is. Some people camouflage their ear by growing their hair long, while others deal with comments and questions more easily. The chances of successful surgery or prosthesis improve with age, so if you change your mind in the future, these options will still be open to you.
Ear reconstruction using a rib graft
This involves making a new ear from your own tissue. The framework or ‘scaffolding’ is made from cartilage taken from your ribcage. The reconstruction is carried out in two stages. Most people are offered this option when they are teenagers because their cartilage is mature enough to use at that stage.
False (prosthetic) ear
This involves attaching implants and fixtures to your head onto which a false ear is attached. It usually takes two operations: one to insert the implants and another to attach the fixtures. Once the operations are complete, the false ear is attached to the fixtures. The false ear itself is made from soft, silicone material and is sculpted to match your other ear. It will need to be replaced every two years and the fixtures need to be cleaned every day.
When should treatment start?
As a teenager, you can have treatment at any time.
Living with microtia
Here are some ways of coping with microtia.
- You will find it easier to hear if people stand or sit on the side of your hearing ear. This is especially important if there is background noise from the television or brothers and sisters.
- If you suspect you have an ear infection in the hearing ear, visit your family doctor (GP) as soon as possible.
Ask if you can sit towards the front, with your hearing ear near the teacher.
- Learning to read may be more difficult if certain sounds are difficult to hear over classroom noise. Ask if quiet reading time is possible on the timetable.
- Check your eyesight regularly so that you can read instructions easily.
- Talk to the Special Educational Needs Coordinator (SENCo) about any other aids that might help you.
As discussed in the treatments section, operations to correct microtia will only make the ear look better. It will not improve how well it works. The results from surgery are usually very realistic and will rarely need repeating in later life. Most young people adapt to any hearing loss.