What is microtia?
Microtia can affect the development of one or both ears. Children with microtia have an underdeveloped ear or ears and affects how the outer ear looks. This condition develops before babies are born, beginning during the first two months of pregnancy.
Ears affected by microtia can be:
- Slightly smaller than normal
- Significantly smaller than normal with different parts of the ear underdeveloped
- Completely absent, which is called anotia
What is atresia of the ear canal?
A child with atresia is born without an external ear canal. Atresia can be complete, where there is no ear opening, or there can be a very thin opening of the external ear canal (ear canal stenosis). Most children with ear canal stenosis or atresia have significant hearing loss on that side.
Can kids have both microtia and atresia?
Yes. Children with microtia and atresia have both a small or absent outer ear, and they are missing an ear canal.
A child with microtia and atresia may have additional health challenges, including conditions that affect the face, such as hemifacial microsomia or oculo-auriculo-vertebral syndrome, also known as Goldenhar syndrome.
When someone has microtia or microtia and atresia and no other developmental conditions, it's called isolated microtia.
What causes microtia and atresia?
Microtia happens because of a mix of genetic and environmental factors. For most patients, the exact cause is not clear. Sometimes, the condition is passed to a child from a parent.
Microtia and atresia have not been linked to anything that happens during pregnancy, except in very rare cases. The prescription drugs isotretinoin (Accutane) and thalidomide, for example, can increase the chances of microtia (and other conditions) when used during early pregnancy. Both drugs are not allowed to be used when you are pregnant.
Who gets microtia and atresia?
Babies of any ethnicity can get microtia and atresia. However, it's more common in Hispanic and Native American ethnic groups. Researchers have developed a rate of occurrence based on ethnicity:
- Navajo, Inuit, Andean: 1 in 1,200 live births
- Native Americans: 1 in 1,800 live births
- Hispanic: 1 in 10,000 live births
- Asian: 1 in 10,000 live births
- Caucasian: 1 in 20,000 live births
- African: 1 in 40,000 live births
Why choose Children's Colorado to treat your child's microtia and atresia?
Children's Colorado provides patients and their families the unique opportunity to get treatment and rehabilitation for microtia and atresia from an internationally recognized team that supports each child’s specific needs. Our Microtia Clinic provides specialized experts for your child’s care needs. This multidisciplinary approach brings experts from around the hospital to address every aspect of your child’s treatment.
To help our patients and families choose the best treatment option for them, we provide current and accurate information from a surgeon, audiologist, family consultant, psychologist, speech language pathologist, anaplastologist and medical photographer. A clinical nurse helps guide families through the process so patients and families feel confident and supported in their treatment plans. Additional support, such as parent-to-parent and patient-to-patient social work resources are also available.
What to expect at the Microtia Clinic
The Microtia Clinic is held once a month at Children’s Hospital Colorado, Anschutz Medical Campus, Aurora. Appointments generally last 60 to 90 minutes.
Families meet with an ear, nose and throat specialist, a family consultant and an anaplastologist (who makes prosthetic devices) at the beginning of the visit. They also meet with a developmental psychologist and speech language pathologist to evaluate development. They meet with the audiologist as needed for hearing tests, amplification consultations and to schedule future appointments.
Families are also invited to stay longer to meet other families and patients that join the clinic.
What are the signs and symptoms of microtia and atresia?
Microtia is only rarely diagnosed and seen on a 20-week ultrasound. It is not routinely evaluated during a typical pregnancy and is most commonly diagnosed at birth.
Once a baby is born, parents and healthcare specialists will notice:
- Unusual ear size
- Unusual shape of the outer ear
- A closed or very small ear canal opening
What is the risk of future children having microtia and atresia?
The risk of having a child with microtia and atresia is low. The risk of having more than one child with microtia and atresia is also low unless your ethnicity has a higher rate of occurrence. Still, it is rare to have parents and children or two siblings with microtia and atresia.
What tests do we use to diagnose microtia and atresia?
Newborn testing for microtia and atresia
All newborns with microtia and/or atresia should receive a diagnostic test called an auditory evoked potentials (AEP) or an auditory brainstem response (ABR) evaluation within three months of birth. The test can be done as early as any routine newborn hearing screening.
An AEP/ABR evaluation is done by an audiologist. We highly recommend that newborns diagnosed with hearing loss find a pediatric audiologist for care. Experts at our Audiology, Speech and Learning Center are highly qualified to perform these evaluations.
Testing for older children
All children should receive a hearing screening when starting public school and are referred for a diagnostic hearing test if needed. However, hearing loss can be missed in newborns if screening isn't done before leaving the hospital after birth or if your pediatrician doesn’t recommend a follow-up appointment. This can happen if microtia and atresia is only in one ear, because hearing loss in one ear is harder to detect.
How do specialists at Children's Colorado diagnose microtia and atresia?
In order to diagnose microtia and atresia, your child should see a microtia specialist. Specialists at our Microtia Clinic can perform very accurate evaluations for your child.
During the evaluation, the specialist evaluates the outer ear and ear canal (if there is one) and tests hearing in both ears, even if the canals are not closed.
An otolaryngologist (ear, nose and throat specialist) and an audiologist who specialize in caring for children with microtia do the evaluation and provide a diagnosis.
Ear canal atresia treatment
Hearing loss caused by atresia or stenosis can be treated with hearing devices and/or surgery:
- A wearable, bone-anchored hearing aid (commonly known as BAHA)
- An implanted hearing device — usually an active transcutaneous bone-conduction hearing device (BCHD)
- An ear canal opening procedure
If the hearing loss is sensorineural (nerve deafness), your child’s doctor may recommend hearing aids, preferential seating in the classroom and speech therapy.
Microtia treatment
Depending on the child's and parent's wishes, the ear can be left as it is or can be reconstructed.
Reconstruction of the outer ear (ear surgery) can be done with a prosthesis or rib cartilage grafting. Reconstruction usually begins when the child is 9 to 10 years old but can happen at any later age.
Microtia and atresia treatment timelines
Below is an example of a timeline of care that can be adjusted for each patient's needs.
Birth to 3 years old
- Establish care with an audiologist (hearing specialist)
- Consider a trial with sound amplification and daily use of a BAHA or bone aid
- Early intervention services
- Visit the Microtia Clinic every one to two years
3 to 6 years old
- Hearing (audiology) visits per recommendations by a specialist or as needed for sound amplification
- Visit the Microtia Clinic annually
- Consider surgery for implanted hearing device
6 years and older
- Visit the Microtia Clinic for ear surgery and prosthetic options based on how the ear looks and the patient’s wishes
- Hearing (audiology) care annually or as needed for sound amplification
Microtia surgery for ear reconstruction
Colorado Families for Hands & Voices
Parent-run organization that supports families with children who are deaf or hard of hearing without a bias around communication modes or methodology
Colorado Hearing Resource Coordinator
Assists families with children birth to 3 years old in getting access to funding, community resources, coordination of services and providing expertise about hearing loss.
Colorado School for the Deaf and Blind
Hard of hearing and deaf adult role models trained to provide awareness experiences and increase understanding about the needs of students who are hard of hearing/deaf and to improve this child's sense of identity and self-esteem
Ear Community
Online support group for people with microtia and atresia
Phonak Guide to Access Planning
Provides information that is important to effectively and independently advocate and be responsible for your communication access supports
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