Pinna abnormalities and low-set ears

Alternative names:

low-set ears

Definition:

Abnormalities of the shape or position of the external ear (pinna or auricle).

Considerations:

The pinna or auricle forms (during fetal development) at a time when many other critical organs are forming (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, and absence of the pinna.

Many children are born with ears that stick out (protruding ears). Although people may comment on the ear shape, and other children may call the child names, this condition is a variation of normal and is not associated with other disorders. However, low-set ears, absent pinna, and abnormal folds CAN be associated with various conditions.

Common causes:

The following conditions include both low-set and malformed ears.

Common conditions:

• Down syndrome
• Turner Syndrome

Rare conditions:

• Treacher Collins syndrome
• trisomy 18
• Beckwith-Wiedemann syndrome
• Turner's syndrome
• trisomy 13
• Rubinstein-Taybi syndrome
• Smith-Lemli-Opitz syndrome
• Aniridia-Wilms' tumor association

Note: There may be other causes of abnormal ear shapes. This list is not all inclusive, and the causes are not presented in order of likelihood. The causes of this symptom can include unlikely diseases and medications. Furthermore, the causes may vary based on age and gender of the affected person, as well as on the specific characteristics of the symptom such as quality and associated complaints. Use the Symptom Analysis option to explore the possible explanations for pinna abnormalities and low-set ears, occurring alone or in combination with other problems.

Update Date: 02/09/00
Updated by: J. Gordon Lambert, MD, Associate Medical Director, Utah Health Informatics and adam.com editorial