Microtia, meatal atresia and conductive deafness

Microtia (meaning 'small ear') is a congenital deformity of the pinna (outer ear). It can be unilateral (one side only) or bilateral (affecting both sides). It occurs in 1 out of about 8,000-10,000 births. In unilateral microtia, the right ear is most typically affected. There are four grades of microtia : more...

• Grade I: A slightly small ear with identifiable structures and a small but present external ear canal
• Grade II: A partial or hemi-ear with a closed off or stenotic external ear canal producing a conductive hearing loss
• Grade III: Absence of the external ear with a small peanut vestige structure and an absence of the external ear canal and ear drum
• Grade IV: Absence of the total ear or anotia.

Grade III is most common, and can be corrected by surgery. Typically, testing is first done to determine if the inner ear is intact and hearing is normal. If hearing is normal, the next step (if a canal is not visible externally) will be to determine if a canal exists, by MRI. For younger patients, this is done under sedation. Surgery may not necessarily be attempted until at a later age (generally about age 8, when the ear is full adult size). Cosmetic surgery (building a new outer ear) is done first to ensure position in relation to the other side; the patient's own cartilage is used (taken from ribs or elsewhere). Surgery to build a canal and/or canal opening can then proceed.

Options

There are two separate issues in microtia surgery:

a) Cosmetic reconstruction to restore the visual appearance of the ear

b) Repair of atresia or application of a bone-anchored hearing aid BAHA to enable hearing. Unilateral deafness is not a serious disability, especially when the person is able to adjust to it from birth. In some jurisdictions the benefits of intervention to enable hearing in the microtic ear are not considered to outweigh the risks, except in bilateral microtia.

For cosmetic reconstruction, there are four different options:

1) Do a rib graft reconstruction (age 5 1/2 is the earliest you can start). This is a four-stage surgery discussed above. The foremost surgeon in the United States in rib graft microtia reconstruction is Dr. Burt Brent, www.earsurgery.com

2) Reconstruct the ear using a Medpor polyethelene plastic implant. Surgery for this can start around age 4. Note that for patients with both Microtia and Atresia, that when using Medpor for the outer ear reconstruction the atresia repair must be done FIRST (as mentioned above with the rib graft reconstruction, the atresia repair is done AFTER). The foremost surgeon in the United States for medport microtia reconstruction is Dr. Jack Reinisch, www.hopeforkids.com

3) Do nothing.

4) Get a prosthetic

Prosthetics are available in several varieties ranging from glue-on, to those which fit over the existing ear, to titanium abutments which are embedded in the skull to which the prosthetic is screwed on. Perhaps the most remarkable difference between surgery and prosthetic is that cartiledge taken from the costal rib area and grown becomes a sensitive ear - this means that it will blush, it will tan, feel wind, and kisses etc;

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