This deformity of the infant ear is characterized by an underdeveloped and malformed outer ear. Microtia, which means “little ear,” occurs in about 1 in 6,000 births and is more common in people of Asian, Hispanic, and Native American descent. Men are also affected twice as often as women. The ear deformity usually affects only one ear (unilateral microtia), but in rarer cases it can also 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 missing or malformed. While microtia is a description of the outer ear, it often occurs in conjunction with an absence of the ear canal (canal or aural atresia) or a narrowing of the ear canal (canal stenosis).

What causes microtia?

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

How can microtia be treated?

Unfortunately, treatment with the EarWell® system is not possible in the case of microtia. Usually an otolaryngologist and/or a pediatric audiologist is consulted to evaluate the inner ear and determine if there is a hearing loss. The recommended treatment of microtia is most often a collaborative effort of a team of pediatric experts (in addition to the ENT doctor, an audiologist, a geneticist and a plastic surgeon may also be involved). Basically, it depends on the severity of the disease. For patients with hearing loss, traditional hearing aids can be used, or a bone-anchored hearing aid (BAHA) can be surgically implanted, which transmits sound through existing bone. Cochlear implants are also an option to improve hearing loss. These can be of great use for language development. Surgery to improve hearing may also be done in patients with conductive hearing loss. Grade 1 microtia cases in which there is no hearing loss may not require treatment. In most cases of microtia, surgical ear reconstruction is the recommended treatment option for the deformity of the outer ear. Surgical reconstruction is usually only carried out when the child is between four and ten years old, i.e. 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 find more about Anotie here .
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