Rare congenital malformation of the external ear.

Findings range from minor changes such as preauricular tags to anotia, which is the complete absence of the external ear.

Prevalence ranges from 0.66 to 17.4 per 10,000 .

Strong genetic contribution.

Shared genotype in monozygotic twins increased the risk of auricular malformations by a factor of 30.

May be as a result of somatic mutations or epigenetic events that occur early in embryogenesis.

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