Pinna Abnormalities And Low-Set Ears


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Definition

Pinna abnormalities and low-set ears refer to abnormalities in the shape or position of the outer ear (pinna or auricle).


Alternative Names

Low-set ears; Microtia; "Lop" ear


Considerations

The outer ear or "pinna" forms when the baby is growing in the mother's womb. The growth of this ear part takes place at a time when many other important organs are developing (such as the kidneys). Abnormalities in the shape or position of the pinna may be a sign that the baby also has other related problems.

Common abnormalities include cysts in the pinna or skin tags.

Many children are born with ears that stick out (prominent or protruding ears). Although people may comment on the ear shape, this condition is a variation of normal and is not associated with other disorders.

However, the following problems may be related to medical conditions:

  • Abnormal folds or location of the pinna
  • Low-set ears
  • No opening to the ear canal
  • No pinna
  • No pinna and ear canal (anotia)

Common Causes

The following common conditions can cause low-set and unusually formed ears:

  • Down syndrome
  • Turner syndrome

Rare conditions that can cause low-set and malformed ears include:

  • Beckwith-Wiedemann syndrome
  • Potter syndrome
  • Rubinstein-Taybi syndrome
  • Smith-Lemli-Opitz syndrome
  • Treacher Collins syndrome
  • Trisomy 13
  • Trisomy 18

References

Haddad J Jr. Congenital malformations. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 630.


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Review Date: 11/7/2011
Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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Last Updated 2/21/2012
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