Definition

Low-set ears and pinna abnormalities refer to an abnormal shape or position of the outer ear (pinna or auricle).

Alternative Names

Low-set ears; Microtia; "Lop" ear; Pinna abnormalities; Genetic defect - pinna; Congenital defect - pinna

Considerations

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

Common abnormal findings include cysts in the pinna or skin tags.

Many children are born with ears that stick out. Although people may comment on the ear shape, this condition is a variation of normal and is not linked 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)

Causes

Common conditions that can cause low-set and unusually formed ears include:

• 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

When to Contact a Medical Professional

In most cases, a health care provider finds pinna abnormalities during the first well-baby exam. This exam is most often done at the hospital at the time of delivery.

What to Expect at Your Office Visit

The provider 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 provider will take 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. Exams for any changes in mental development may be performed as the child grows. Genetic testing may also be done.

TREATMENT

Most of the time, 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 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 cosmetic reasons as well as for function. Surgery to create and attach a new ear is often done in stages.

References

Haddad J, Dodhia SN. Congenital malformations of the ear. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 656.

Madan-Khetarpal S, Arnold G, Ortiz D. Genetic disorders and dysmorphic conditions. In: Zitelli BJ, McIntire SC, Nowalk AJ, Garrison J, eds. Zitelli and Davis' Atlas of Pediatric Physical Diagnosis. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 1.

Mitchell AL. Congenital anomalies. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 30.