Microtia and atresia: When ears don't develop fully
Microtia is a rare birth defect in which the external ear is not completely developed, occurring in one of every 5,000 to 7,000 births. It usually affects hearing and generally requires ongoing medical care from a pediatric ear-nose-throat specialist as well as an audiologist. It's more common in males than females.
Microtia is almost always accompanied by atresia, also known as aural atresia, a condition in which the auditory ear canal is either absent or closed.
What causes microtia and atresia?
Medical professionals aren’t sure exactly what causes microtia. It's theorized that it occurs during the first trimester of pregnancy when the soft tissues of the outer ear and middle ear are being formed. It's also linked to using Retin-A (tretinoin), a common drug for skin conditions like acne and aging, during pregnancy.
There are four grades of microtia:
Because this condition affects the outer ear, it is often accompanied by moderate to severe conductive hearing loss. Affected children are also susceptible to ear infections and may become self-conscious about their condition as they grow older. Fortunately, microtia and atresia can usually be repaired, and the hearing loss treated.
One of the biggest risks for surgery is damage to the facial nerve, although modern surgical techniques have substantially lowered this risk. Other complications can include stenosis (stiffening), drainage from the ear and infection. Sensorineural hearing loss occurs in approximately 5% of patients. Medical professionals recommend parents wait until children are 5-7 years old to ensure compliance with postoperative care instructions.
Advocacy and awareness
Colorado native Melissa Tumblin was inspired to learn more about microtia when her daughter Ally was born with the condition. Through advocating for Ally's unique needs, she learned a great deal and wanted to find new ways to educate and support other parents. Tumblin subsequently founded Ear Community, an online support group and caring place for families affected by microtia and atresia.
November 9 is National Microtia and Atresia Awareness Day, an observance created by Tumblin in the hopes of bringing public awareness to the rare congenital birth defect of the ear.
Detecting hearing loss in babies and kids
Newborn hearing screenings can help detect hearing loss in infants even before parents bring their babies home from the hospital. When abnormalities such as microtia and atresia are apparent at birth, or when other hearing problems are detected during the screenings, medical professionals can help you decide which treatment options are best, and direct you to the resources needed to make the right decisions.
Children with hearing loss caused by ear abnormalities or other causes are best served through a team approach led by their pediatrician or ENT specialist. Pediatric audiologists have the equipment and education to evaluate hearing loss in babies and children. They will work with your pediatrician and medical specialists to implement the best treatment options so your little one has every social, educational and developmental advantage.