Microtia

what to do

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Microtia

what to do

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This deformity of the infant’s ear is characterized by an underdeveloped and malformed outer ear. Microtia, which means “little ear,” occurs in about one in 6,000 births and is more common in people of Asian, Hispanic and Native American descent. Males are also affected twice as often as females. The ear deformity usually affects only one ear (unilateral microtia), but in rarer cases it can occur on both sides (about 20% of microtia patients). In most cases of microtia, the earlobe is present, but other parts of the ear may be absent or malformed. While microtia is a description of the external ear, it often occurs in conjunction with absence of the ear canal (canal or aural atresia) or narrowing of the ear canal (canal stenosis).

What causes microtia?

Microtia is rarely genetically inherited (no family history in 95% of cases). However, the exact cause is often unknown, and it frequently occurs in association with certain syndromes (e.g., hemifacial microsomia, Goldenhar syndrome, or Treacher-Collins syndrome). Contributing factors include maternal diabetes, prenatal alcohol consumption, or prenatal exposure to the acne drug Acccutane, thalidomide, or mycophenolate. However, maternal nutrition also plays a role: too little folic acid or carbohydrates can trigger microtia. Similarly, decreased oxygen or blood supply to the fetus during the first trimester is associated with microtia.

How can microtia be treated?

Unfortunately, in the case of microtia, treatment with the EarWell® system is not possible. In all cases, an otolaryngologist and/or pediatric audiologist is consulted to evaluate the inner ear and determine if hearing loss is present. The recommended treatment for microtia is usually a collaborative effort among a team of pediatric experts (in addition to the ENT physician, an audiologist, geneticist and plastic surgeon may also be involved). Basically, it depends on the severity of the condition.

For patients with hearing loss, traditional hearing aids may be used, or a bone anchored hearing aid (BAHA) may be surgically implanted, which transmits sound through the existing bone. Cochlear implants are also an option to improve hearing loss. These can be of great benefit for speech development. For patients with conductive hearing loss, surgery may also be performed to improve hearing.

In grade 1 microtia cases, where there is no hearing loss, no treatment may be needed. In most cases of microtia, surgical ear reconstruction is the recommended treatment option for the outer ear deformity. Surgical reconstruction is usually not performed until the child is between four and ten years old, meaning the unaffected ear has reached maturity. The most severe grade 4 (known as anotia) is characterized by the complete absence of the outer ear. You can read more about anotia here.