Congenital Ear Malformation

Drs. Hwang, Patel, and Skoner discussing a patient's case.

Although there is no identifiable cause, some children are born with misshapen or absent ears. 

Microtia

Microtia is a congenital issue where there is an underdeveloped, malformed, or missing outer ear. The ear canal may also be narrowed or missing. Children with microtia may have a partial loss of their hearing due to the undersized or missing ear canal. The inner ear, the portion responsible for hearing, is typically not affected. Almost 90% of children born with microtia have only one ear affected (unilateral microtia). For this reason, all children with microtia or misshapen ears should have their hearing evaluated. Microtia is more common in males than females and affects the right ear more than the left. Microtia occurs in 1 out of every 8,000 to 10,000 births. Research has also shown decreased self-esteem associated with microtia.

Grades of microtia

Grade 1 microtia

Grade One – affected ear is smaller than normal, but normal features of the ear are present

Grade 2 microtia

Grade Two – some of the normal features of the outer ear are missing

Grade 3 microtia

Grade Three – affected ear is a small piece of skin and cartilage, ear canal and ear drum commonly missing (most common form)

Surgical Repair

There are different options for how to treat microtia. The family and child may opt to have it reconstructed from the child’s natural tissues or the child can have a prosthetic ear created. The decision on how to reconstruct the ear is an important one and intimately involves the decision making of the child and family.

Autologous reconstruction is performed by a multi-stage surgery, using your child’s rib cartilage sculpted into the form of the other ear and placed under the skin where the ear would be. This is then refined and structured to recreate a normal ear. The procedure is normally done at 6-10 years of age. The surgery is usually done in three stages that are spaced 3-6 months apart to allow healing between each stage. This surgery allows the child to have an ear that is created from their own tissue, creating a very natural feeling ear that will heal just as a natural ear does. The reconstruction allows for wearing glasses or sunglasses, boosts self-esteem, and improves quality of life.

Grade 2 microtia before and after repair

Grade two microtia before and after repair

Grade 3 microtia before and after repair

Grade three microtia before and after repair

Another option to be discussed during your visit is a custom made ear prosthesis which is a type of artificial ear, constructed from synthetic material that can be secured to the natural location of the ear with either the use of skin glue or magnetic metal posts. We work closely with our prosthetics division for the placement and design of the fabricated ears.

Ear prosthesis

Prosthetic ear secured with magnetized titanium implants

We will want to test your baby’s ability to hear, this is done using either an audiogram or an auditory brainstem response evaluation (ABR). These tests are performed by our colleagues who specialize in ear and hearing disorders. If hearing loss is present your child may be a candidate for a special type of hearing implant or reconstruction of the middle ear and ear canal, usually performed after the external ear reconstruction.

Otoplasty

Otoplasty is a procedure to improve the appearance or position of the external ear. Otoplasty is a good option for protruding ears or a mild malformation of the ears. The goal of the surgery is to move the ears closer to the head and to give them a more normal appearing shape. Studies suggest that children with prominent or misshaped ears are more susceptible to teasing in social settings. Surgery helps reduce this risk. The surgery is a one staged procedure typically performed on both sides if needed.

Before and after left ear otoplasty

Left ear before and after otoplasty

Before and after right ear otoplasty

Right ear before and after otoplasty

Ear molding

If there is a mild ear deformity and it is recognized immediately after birth, we can perform ear molding to try to shape the ear with tape and molds that do not require surgery. In newborns, cartilage in the ear is very malleable and it can be manipulated to take on a new shape. If molding is started immediately and performed for the first 6 weeks of life, the cartilage will sometimes retain the new shape and save the child from needing surgery later in life. With our prosthodontist we also provide molding with the Earwell™ Infant Ear Correction System.