Microtia: Born Without an Ear

Microtia (my-kro-shuh) is a problem that affects the outer part of the ear and/or inner part of the ear. Microtia happens when the precursor ear parts fail to form into a normal ear in the fetus. Specifically, these parts of the ear are not completely formed or they may be completely absent. The earlier the problem occurs while the baby is in the womb, the more severe the deformity. Plastic surgeons can surgically correct these ear deformities.

Microtia is present at birth. It occurs in one to two babies per 10,000 births. It affects males more than females and is more common in Asians and Hispanics than Caucasians and African-Americans. The highest occurrence is among the Navajo tribe Native Americans. The right ear is affected more often than the left ear or both ears.

Microtia can take on different forms. Sometimes a bump of skin may be present where the ear should be. Sometimes parts of the lower ear are present, but the upper ear is absent. The most severe form is when the ear is completely missing. It is known as anotia (an-o-shuh). There may be a lack of an opening into the ear canal.

No one knows the exact reason microtia occurs. Certain drugs, decreased blood flow to the fetus, and genetics are thought to be the causes of microtia.

Even though it is the outer ear that has the noticeable deformity in microtia, other parts of the ear system are affected. The inside of the ear, specifically the ear canal and the middle ear, often have defects as well. Sometimes the ear canal does not develop. In these individuals there is no visible hole that leads to the ear drum and the middle ear. These abnormalities can lead to hearing loss.

People with bilateral microtia (microtia in both ears) are in a totally different situation than people with a unilateral microtia. Those with unilateral microtia have normal hearing in the normal ear, whereas those with bilateral microtia may be functionally deaf.

The main goal of restoring the outer ear is to improve hearing. The outer ear catches sounds and directs it into the ear canal and towards the eardrum and the hearing apparatus. Improved hearing helps with speech. A hearing aid may still be needed. Of course, an improvement in appearance is an additional benefit that can boost self-esteem and social acceptance. Reconstruction of the ear canal is performed by an otologist (oh-tall-uh-jist).

The ear reaches the majority of its growth -- 85 percent -- by the age of four. The ear continues to increase in width until age ten. The two factors that influence when ear reconstruction occur are:

• Availability of enough rib cartilage to make the structure of the ear: It is not until age five or six that a child’s rib cage is large enough to provide adequate donor cartilage for an ear framework.
• Psychological effect of having abnormal/no ear(s): Reconstruction is done around school age in order to minimize/avoid teasing from peers.

The best results occur when the reconstruction is delayed until after the age of ten.

The outer ear can be reconstructed with synthetic (man-made) materials or from rib cartilage. There are advantages and disadvantages to both options:

Synthetic Materials

• Advantages:

No scar on chest from harvesting rib cartilage

No pain from harvesting rib cartilage

No foreign body

Excellent cosmetic appearance

Can be done in one procedure

• Disadvantages:

Susceptible to minor trauma

Susceptible to infection

Spontaneous rejection/extrusion

Cartilage Graft

• Advantages:

Best long-term option

Uses person’s own materials

• Disadvantages:

More than one surgery needed

Pain from retrieving rib cartilage

Scar on chest

In cases where there is not enough skin to cover either a synthetic framework or a cartilage framework due to trauma, burns, radiation, etc., a prosthetic ear can be used. A prosthetic ear has its advantages and shortcomings as well. While a prosthetic ear can look very much like a real ear, the quality of the device depends on the skills of the maker and available technology. Additionally, a prosthetic ear can be quite expensive. How long the device lasts depends on the care rendered by the owner.

In half of microtia cases, there is enough of the middle ear that can be reconstructed to improve or restore hearing.

As with any surgical procedure, there are potential complications when it comes to ear reconstruction. Infection is a potential complication with either the synthetic or cartilage framework. Hematoma, otherwise known as a blood collection, can also compromise the cartilage framework. Skin loss in the ear area is also a possibility. Additionally, in cases where rib cartilage is used, lung problems, chest wall contour deformities, and unsightly scarring can occur. Complications involving the framework can result in needing to perform the procedure again.

_Sources:

_{LeBlanc DM. Microtia. In: Janis JE, ed. Essentials of Plastic Surgery. St. Louis: Quality Medical Publishing, Inc., 2007.}

_{Thorne CH. The External Ear. In: McCarthy JG, RD Galiano, Boutros SG, eds. Current Therapy in Plastic Surgery, 1st Ed. Philadelphia: Elsevier, Inc, 2006.}

_{Thorne CH. Otoplasty and Ear Reconstruction. In: Thorne CH, Beasely RW, Aston SJ, Bartlett SP, Gurtner GC, Spear S, eds. Grabb and Smith’s Plastic Surgery, 6th ed. Philadelphia: Lippincott, 2007.}