Pediatric Ear Malformations

Causes of various types of ear malformations are not fully understood, but some possible factors include:

• A lack of blood supply to the baby’s ear during fetal development
• Genetic mutations or inherited conditions
• Exposure to certain toxins or medications
• Viral infections such as the flu or rubella (German measles)

There are many types of congenital ear deformities, including:

• Microtia: Underdeveloped outer ear
• Anotia: Missing one or both ears
• Protruding ears: More than 2 cm from the head
• Constricted ears (or lop or cup ears): Flattened or rolled outer ears
• Cryptotia: Upper ear underneath the scalp skin
• Stahl's ear: Pointed outer ear
• Ear tag (accessory tragus): Cartilage bump in front of the ear
• Congenital earlobe deformities: Duplicate earlobes or earlobes with clefts or skin tags
• Ear hemangiomas: Small to large benign tumors

Congenital ear deformities are usually visibly noticeable at birth, although milder forms may be less apparent. Other, less noticeable symptoms of ear deformities include:

• Frequent ear infections
• Facial deformities including small cheekbones and a small or crooked lower jaw, sometimes on only one side
• Crowded, crooked teeth
• Abnormally shaped mouth, either wide or small

Many congenital ear malformations can be easily corrected if they are diagnosed very early, within a few weeks of birth. At Children's National Hospital, our pediatric team can usually detect ear deformities through a physical exam. Other tests our physicians may recommend include:

• Basic or advanced hearing tests to detect any hearing loss
• Diagnostic imaging, including CT scans, to look for related facial, jaw or kidney deformities

Children who have minor ear malformations may not need treatment if their hearing is normal. For more severe cases, the treatment goals focus on correcting the shape of the ear and restoring any hearing loss. Children need good hearing ability for proper speech and language development.

At Children's National, our treatment options include:

• Ear molding for newborns with protruding, constricted or Stahl's ears, ideally at 1 to 3 weeks of age
• Surgery (usually after age 6, when the ear has grown to almost adult size) to correct problems with the outer ear or earlobes
• Hearing aids or cochlear implants (surgically implanted hearing device) to improve hearing