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Overview

New parents delight in gazing at their babies, taking note of the sweet curled toes, observing dimples in the cheeks, elbows and knees, and analyzing whether these long, graceful fingers presage a talent for playing piano. But you may also notice problems like a scaly scalp, baby acne or, perhaps, that one or both of your newborn’s ears look a little unusual. Maybe the ear sticks out a bit or its top looks a little pointy. Though rare, sometimes babies are born with part of an ear missing entirely, which might cause hearing problems. It’s natural, then, to worry about whether that misshapen ear could make your child a target for teasing or if hearing loss could affect learning and speech abilities.

Fortunately, craniofacial surgeons have many ways to treat these kinds of ear deformities and the hearing loss that sometimes accompanies them. Options range from noninvasive ear molding to correct the shape of a malformed ear to surgery, typically not recommended until a child reaches school age. At the right time, however, surgical treatments can reshape and even entirely reconstruct abnormal ears, and in some cases, help restore hearing.

Congenital deformities of the outer ear—that is, deformities of the visible ear and ear canal that are present from birth—are common. Approximately 1 in every 6,000 newborns has an outer ear deformity. In general, treatments result in good outcomes, and if they are undertaken when your child is still young, they may help avoid social stigmatization altogether.

“Ear deformity correction offers a dramatic and permanent improvement to the ear appearance with an over 90% success rate if initiated on time,” says plastic surgeon Michael Alperovich, MD.  

What is a congenital ear deformity?

The ear is made up of three main sections—the outer, middle, and inner ear. The outer ear represents the external, visible ear—called the auricle or pinna—and the external auditory canal. It is more complex than you might guess, containing many structures, curves, and folds—all of which work in concert to collect sound waves and direct them to the middle and inner ear, which work together with the brain to allow us to hear sound.

As with all body parts and organs, the ear develops as a baby grows in the uterus. Sometimes, though, this growth and development does not proceed properly. When this happens, it can result in what are known as congenital deformities in the outer ear present at or before birth.

Congenital deformities of the outer ear can occur in any of the many structures of the outer ear. They can affect one ear or both and tend not to affect hearing function. Still, outer ear deformities can have long-term social and psychological implications as children grow up.

What are the types of outer ear congenital deformities?

There are many varieties of congenital deformities of the outer ear, including:

  • Protruding ear: Also known as “prominent ear,” this is a normal-looking ear that sticks out from the head at an angle greater than 40 degrees.
  • Constricted ear: Often called “cup ear,” this deformity involves the top rim of the ear, known as the helical rim, which is abnormally tight.
  • Stahl’s ear: This is characterized by an extra fold of cartilage in the upper ear, which produces a pointed ear appearance.    Cryptotia: Sometimes called “hidden ear,” cryptotia occurs when the upper portion of the ear is buried underneath a fold of scalp skin on the side of the head. 

What causes congenital ear deformities?

Because outer ear deformities are present from birth, a number of factors may contribute to their development:  

  • Environment. Congenital ear deformities can occur when a developing baby is exposed to certain conditions in the uterus. Prenatal exposure to particular drugs, including isotretinoin (Accutane, for example), thalidomide, mycophenolate, and alcohol have been linked to the development of outer ear deformities.
  • Fetus positioning. In some cases, deformities arise due to how a baby is positioned in the uterus or during birth. For instance, a reduction in blood supply to the outer ear can lead to abnormal development. 

How are congenital ear deformities diagnosed?

Congenital deformities of the outer ear are typically diagnosed at birth during a physical exam. Your doctor will closely examine your baby to assess symmetry and proportionality, as well as to check for facial and jaw defects. They will also look into the ears using an otoscope, a device that allows for the visual examination of ear canal and tympanic membrane, or ear drum. 

What is the treatment and prognosis for congenital ear deformities?

Many people with mild ear deformities do not require treatment. For people with more severe forms of ear deformity, surgical and nonsurgical treatments are available. These treatments aim to correct the shape of the ear and restore hearing, if necessary.

Deformities where all of the elements of the ear are present, though abnormally shaped, sized, or positioned, may be treated through a nonsurgical technique called ear molding.

  • Ear Molding. During the first several weeks of life, the cartilage that makes up the ears is particularly pliable. It can be reshaped using ear molding, a treatment in which a custom-fit, soft plastic mold—in a “corrected” shape—is temporarily attached to the infant’s ear using adhesive strips and compounds. Over time, the ear conforms to this corrected shape. Ear molding works well in the treatment of protruding ears, Stahl’s ears, constricted ears, lop ears, and cryptotia. Usually this treatment takes between two and four weeks.
  • Surgical Treatment. Surgery may be an option for people whose congenital outer ear deformity cannot be or was not treated with ear molding. However, some ear deformities can only be addressed using surgical correction. Doctors recommend waiting until a child reaches at least school age before having surgery to correct a congenital ear deformity.