The ear canal is always affected with Aural Atresia if the outer ear malformation is graded 2-4. Grade 4: Also referred to as anotia, no ear remnants or canal present. Grade 3: Most common form of microtia, characterized by small, undeveloped ear remnants and no canal. ![]() The bottom 1/3 of the ear is usually normal, no ear canal or a narrow canal. Grade 2: Characterized by some ear anatomy, including a developed tragus. Grade 1: Small ear with mostly normal ear anatomy, the ear canal may or may not be affected. Microtia is graded on a scale of from 1-4: ![]() Microtia refers to deformity of the external portion of the ear and is present from birth. The ear can be broken down into 3 components: internal, middle and external portions. Microtia is Latin for the words micro and otia, meaning “little ear.” Microtia is usually isolated, but may occur in conjunction with hemifacial microsomia, Goldenhar Syndrome or Treacher-Collins Syndrome. Microtia can be unilateral (one side only) or bilateral (affecting both sides). Because microtia and anotia have the same origin, it can be referred to as microtia-anotia. A completely undeveloped pinna is referred to as anotia. Microtia is a congenital deformity where the pinna (external ear) is underdeveloped.
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