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Microtia is a congenital deformity where the external ear (auricle) is underdeveloped, ranging from a slightly small ear to complete absence (anotia). It occurs in about 1 in 5,000–10,000 births, often unilaterally (90% of cases), and may affect hearing due to an absent or narrow ear canal.
The exact cause of Microtia is often unknown, but it may result from genetic factors, environmental triggers (e.g., Accutane use during pregnancy), or prenatal issues like reduced blood supply or oxygen levels in the first trimester. It’s rarely hereditary (5% familial cases) and not caused by parental actions during pregnancy.
Microtia is classified into four grades: Grade I (slightly small ear with most features), Grade II (partially developed ear, often with a closed ear canal), Grade III (small, peanut-shaped remnant, no canal—most common), and Grade IV (anotia, complete absence of the ear). Severity impacts treatment options, from hearing aids to surgical reconstruction.
Microtia is classified into four grades: Grade I (slightly small ear with most features), Grade II (partially developed ear, often with a closed ear canal), Grade III (small, peanut-shaped remnant, no canal—most common), and Grade IV (anotia, complete absence of the ear). Severity impacts treatment options, from hearing aids to surgical reconstruction.
Treatments include surgical reconstruction using rib cartilage (ages 8–10) or porous polyethylene (MEDPOR, ages 3–5), prosthetic ears (silicone, adhesive, or bone-anchored), and hearing aids like bone-anchored hearing aids (BAHA). The choice depends on severity, age, and patient preference, with surgery aiming to improve appearance and hearing.
Surgery, such as rib cartilage reconstruction, is typically recommended at ages 8–10 when sufficient cartilage is available and the child can consent, while polyethylene implants may be used earlier (ages 3–5). Timing balances physical development, psychological readiness, and postoperative care.
Microtia can occur alone or with syndromes like hemifacial microsomia (facial asymmetry), Treacher Collins (affecting ears, eyes, jaw), Goldenhar syndrome (ear, face, and neck issues), or Townes-Brocks syndrome. About 20–60% of cases involve associated anomalies, requiring comprehensive medical evaluation.
Microtia occurs in approximately 1 in 5,000–10,000 births globally, with higher prevalence in Hispanic, Asian, Native American, and Andean populations. It’s usually unilateral (90%), more often affecting the right ear, and more common in males.
The gold-standard or most common microtia surgery technique in the world is using the patient's own cartilage from three small pieces of rib. There are other techniques using a polyethylene plastic ear which can have good definition but is known to be a very hard material and can have a risk of exposure or fracture for the rest of the patient's life. A prosthetic us usually a last resort option.
Microtia is rarely detected via prenatal ultrasound due to its low incidence and small ear size, but 4D ultrasound may occasionally identify it. Diagnosis is typically confirmed post-birth through physical examination, followed by hearing tests and imaging like CT scans for severe cases.
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