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Microtia is a congenital deformity where the auricle (external ear) is underdeveloped. A completely undeveloped pinna is referred to as anotia. Because microtia and anotia have the same origin, it can be referred to as microtia-anotia. Microtia can be unilateral (one side only) or bilateral (affecting both sides). Microtia occurs in 1 out of about 8,000–10,000 births. In unilateral microtia, the right ear is most commonly affected. It may occur as a complication of taking Accutane (isotretinoin) during pregnancy.

Microtia, the etiology of microtia in children remains uncertain, but there are some cases that associate the cause of microtia with genetic defects in multiple or single genes, altitude, and gestational diabetes.

According to the Altman-classification, there are four grades of microtia:
Grade I: A less than complete development of the external ear with identifiable structures and a small but present external ear canal.
Grade II: A partially developed ear (usually the top portion is underdeveloped) with a closed stenotic external ear canal producing a conductive hearing loss.
Grade III: Absence of the external ear with a small peanut-like vestige structure and an absence of the external ear canal and ear drum.
Grade III microtia is the most common form of microtia.
Grade IV: Absence of the total ear or anotia.

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