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What is microtia and atresia?
Microtia and atresia are two rare congenital conditions which affect the formation of the ear and ear canal. Microtia refers to a condition where the external ear is incomplete or underdeveloped. It may affect one ear only (unilateral) or both ears (bilateral). It occurs in one in 6,000 to 12,000 births worldwide.1,2
Microtia is often accompanied by aural atresia because the baby’s outer ear and the ear canal develop at the same time during pregnancy. Atresia is the absence of an external ear canal. In some instances, the ear drum and tiny ear bones (ossicles) can also be affected. Atresia will impact sound transmission through the external hearing system as there is no canal for sound to travel through, resulting in a conductive hearing loss.
Microtia and atresia can affect one ear (unilateral) or both ears (bilateral). It occurs unilaterally in 90 per cent of cases, and in these cases, it occurs more often on the right side than the left.
How is microtia diagnosed?
If your child has a small, underdeveloped or absent ear, microtia will be quite obvious at the time of birth. As microtia is not very common, it will depend on your medical team’s experience and knowledge as to whether you are given the diagnosis of ‘microtia’. In some cases, families discover the name of their child’s condition months later or stumble upon it themselves through research.
What is the cause of microtia and atresia?
The exact cause of microtia and atresia is often unknown. Some evidence suggests environmental and genetic factors may play a role1, however, a medical specialist should always be consulted in regard to this question. There is no evidence that anything you do during pregnancy causes microtia or atresia.
What are the different grades of microtia?
Microtia is graded according to severity. There are four different grades of microtia:
- Grade one: a smaller version of a typical ear
- Grade two: a partially formed ear that is missing some visible features
- Grade three: only a small nodule of misshaped tissue (often a peanut-shaped structure)
- Grade four: the complete absence of the entire ear (also known as anotia)
How will microtia and atresia affect my child?
Babies born with microtia and atresia do not have a typical pathway for sound waves to travel to their inner ear. If the ear canal is narrow, malformed or absent3, sound is not able to pass through the hearing system in the normal way, which will result in a hearing loss in the affected ear/s.
The middle ear (which contains the ear drum and tiny ear bones called ossicles) may also be affected. Most children with microtia and atresia have a normal inner ear. A hearing assessment with an audiologist will determine your child’s hearing levels.
Specialist’s children may see if they have microtia and atresia
If your baby has microtia and/or atresia, a multidisciplinary team of professionals will be needed to support you and your child. Your team will likely include an audiologist, Ear Nose and Throat (ENT) specialist, plastic surgeon, geneticist and listening and spoken language specialists (speech pathologists, teachers of the deaf or audiologists with specialist training in working with children with hearing loss). Other specialists may be needed depending on your child’s individual needs.
"The team answered a lot of questions I had about future options for surgery and hearing aids, and invited me to the Hear and Say centre in Brisbane. There I learnt about listening and spoken language therapy lessons and [Group Social Skills] playgroups that we could attend.”
Clem, mum to 18-month-old, Violet
Current treatment options for microtia
There are currently four treatment options available for microtia including:
- Do nothing – leave the ear as it is
- Reconstruction using Rib Graft (cartilage is taken from the ribs)
- Reconstruction using a porous polyethylene implant (a synthetic material – commonly called Medpor, Supor or Starpore)
- Have a prosthetic ear made Each option has advantages and disadvantages. No option is “perfect”, and there are no right or wrong options.
You need to investigate each option and choose which is right for you and your child.
Research projects are currently investigating future technologies including 3D printing and biofabrication as possible future options.
Current treatment options for atresia
The treatment options currently available for atresia include:
- Bone conduction hearing devices worn on a soft band
- Implanted bone conduction devices
- Implantable middle ear devices
- Atresia repair surgery (for suitable candidates only)
“Sophie especially enjoys going along to LEAP, and all the kids are so friendly and happy to be there. Sophie loves music and singing songs with [clinical facilitators] Liz and Kirsty. Singing has really helped Sophie to use spoken language, and she’s always singing in the car on the way home."
Kate, Sophie's mum
Microtia and atresia FAQs
The exact cause of microtia and atresia is actually not known. Some evidence suggests environmental and genetic factors may play a role1, however, a medical specialist should always be consulted in regard to this question.
Generally, children who are born with atresia have some degree of hearing loss due to the absence of the ear canal.
Most children with microtia and atresia will have some hearing on the affected side/s, although sound will be much softer and muffled. If the ear canal is narrow, malformed or absent3, sound is not able to pass through the hearing system resulting in a hearing loss in the affected ear/s.
If your baby has microtia and/or atresia they will see a team of specialists, known as a multidisciplinary team. The team may include an audiologist, Ear Nose and Throat (ENT) specialist, plastic surgeon, geneticist and listening and spoken language specialists. Other specialists may be needed depending on your child’s individual needs.
Currently there are four treatment options for microtia including leaving the ear as is, reconstruction using rib graft, reconstruction using porous polyethylene implant or having a prosthetic ear made.
You are not alone! While microtia and atresia are not very common conditions, there are many other families and organisations that can help you with information and support. Hear and Say can help connect you with information, support, services and other families.
Ear infections, such as otitis media can occur on the side where there is microtia and atresia. It is recommended to seek medical advice as diagnosing an ear infection on the microtia/atresia side can be difficult as there is generally no ear canal through which the middle ear can be viewed.
There are many abnormalities that can be detected on an ultrasound. Ultrasound technicians usually focus on the major organs unless specifically instructed otherwise. External ears are not routinely checked during an ultrasound and may be difficult to assess.
Microtia can appear on its own or as alongside other conditions such as Hemifacial Microsomia, Goldenhar Syndrome or Treacher Collins Syndrome. Your medical professional will be able to recognise these conditions upon examining your baby and will refer for further testing if required. If microtia is not related to any of these conditions and occurs in isolation, it should not cause any ongoing health problems for your child.
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