Low-set ears

Alternative names
Pinna abnormalities and low-set ears

This condition describes the abnormalities of the shape or position of the external ear (pinna or auricle).


During fetal development, the outer ear or “pinna” forms at a time when many other critical organs are developing (such as the kidneys). Abnormalities in the shape or positioning of the pinna may be an indication that there are other associated abnormalities present.

Common abnormalities include abnormal folds in the pinna, prominence of the ears, low-set positioning, abnormal rotation of the pinna, and even absence of the pinna.

Many children are born with ears that stick out (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, low-set ears, absent pinna, and abnormal folds may be associated with various medical conditions.

Common Causes

The following common conditions are associated with low-set and malformed ears:

Rare conditions associated with low-set and malformed ears include:

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

Call your health care provider if

Call if you notice abnormally shaped or positioned ears.

In most cases, pinna abnormalities are found by a health care provider during the first well baby exam. This exam is usually performed at the hospital, if that is where the baby is delivered.

What to expect at your health care provider’s office

The doctor will ask medical history questions, such as:

  • Are you aware of any other physical abnormalities?  
  • Is there any family history of having abnormal-shaped ears?  
  • Is there a family history of any of the disorders associated with pinna abnormalities and low set ears?

To determine if the pinna is abnormal, the doctor will conduct a series of measurements with a tape measure. Other parts of the body will be measured, including the eyes, hands, and feet, to determine if other abnormalities are present.

All newborns should have a hearing test. A child with pinna abnormalities should certainly have a hearing test, if he or she hasn’t already. Examination for any mental development changes may be performed as the child grows. Diagnostic tests that may be performed include a chromosome analysis.

No treatment is needed for pinna abnormalities, as they do not affect the hearing. However, sometimes cosmetic surgery is recommended.

Johns Hopkins patient information

Last revised: December 6, 2012
by Dave R. Roger, M.D.

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All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.