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

Low-set ears; Microtia; "Lop" ear

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)

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

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

The doctor will:

• Examine and test the child for other physical abnormalities of the kidneys, bones of the face, skull, and facial nerve
• Ask if you have a family history of abnormal-shaped ears

To determine whether the pinna is abnormal, the doctor will take a series of measurements with a tape measure. Other parts of the body will also be measured, including the eyes, hands, and feet.

All newborns should have a hearing test. A child with pinna abnormalities should also have a hearing test. Examinations for any changes in mental development may be performed as the child grows. Genetic testing may also be done.

TREATMENT

Generally, no treatment is needed for pinna abnormalities because they do not affect hearing. However, sometimes cosmetic surgery is recommended.

• Skin tags may be tied off, unless there is cartilage in them. In that case, surgery is needed to remove them.
• Ears that are prominent or that stick out may be treated for cosmetic reasons. During the newborn period, a small framework may be attached using tape or Steri-Strips. The child wears this framework for several months. Surgery to correct the ears cannot be done until the child is 5 years old.

More severe abnormalities may require surgery for appearance or cosmetic reasons, as well as for function. They usually are done in several stages to create and attach a new ear.

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.