(February 21, 2008 - Insidermedicine) Welcome to Insidermedicine's If I Had, where we get a chance to ask an expert what they would do if they had a medical condition.
On a recent trip to Boston, we caught up with Dr. Roland Eavey, MD, a Pediatric Otologist at Massachusetts Eye and Ear Infirmary and Professor of Otology and Laryngology at Harvard Medical School. Surgically, Dr. Eavey specializes in both microtia and atresia in children. He is also an active researcher who focusses both on tissue engineering and on genetics.
We asked Dr. Eavey about what he would do if he had a child born with microtia, a congenital deformity of the outer ear.
Dr. Eavey: If I had a child born with microtia and aural atresia, I would probably not be able to find out much information from the local caregivers. And that’s not to say something disparaging about the local caregivers, but this is a very rare condition and most people don’t have enough experience with it to make comments about it. If I was a typical parent and my child came out with ten fingers and ten toes, which are usually things you’re counting, and had one ear, the first thing that would run through my mind is “Can my child hear?” And so I’d want to know that.
It is possible to test the hearing on infants. Typically, what you find out is that the ear on one side, and this can be a bilateral condition, but it’s usually unilateral. The side that looks normal tends to have normal hearing, which gives reassurance that speech and language skills should develop normally because of that one normal hearing ear. The side with the microtia and atresia will have a maximum conductive hearing loss, which is the equivalent of you putting your finger in your ear canal – you can hear, but not very well. The parents then are fairly well reassured that at least the hearing is fine, and they go to phase two, which is “Whew, my child can hear. What can you do about this ear? Can you make it look like the other ear?” That’s not an emergency, that’s going to take time.
It’s going to be a few years before a method is selected because there is a limited variety of methods to correct the ear, and it depends on how the ear happens to look – if it’s severely malformed or moderately malformed. And it depends if there is both microtia, the malformation, as well as aural atresia, which means you can’t hear. So somewhere in the first few years of life, and I’m talking about 3 to 7 depending again on what the malformation looks like and how much of it is microtia and how much of it is atresia. Surgery can be performed on the external ear and also for aural atresia if a CAT scan had been obtained which showed that the internal anatomy in the middle ear and mastoid area actually looks adequate to make that approach.
If I had a child with microtia and aural atresia…
Dr. Eavey: If I had child with microtia and aural atresia, I first would have a hearing test, an Auditory Brain Stem Evoked Response hearing test, to let me know what the hearing levels are. If it was unilateral, the hearing would be adequate for speech and language development. If it was in both ears, my child would need a hearing aid device. Typically, this is a bone conduction hearing aid and it’s held on an infant by a headband. This would help normal speech and language development. If I had a child then who was going for treatment, it would depend on how the ear looked to know what type of surgical reconstruction—this is not cosmetic surgery, this is reconstructive surgery—that would be able to be performed, and there’s a small variety of options. And if my child had aural atresia I would want to have a CAT scan. I would not want a CAT scan near birth; I would not want a CAT scan in the first couple years of life. I would want the CAT scan at about the time we would be potentially doing surgery, which is several years of age. Typically the external ear is operated on first, because aural atresia and microtia nearly always occur together with certain exceptions. Usually the external ear is operated on first, to fix that with whatever technique was used, and then the aural atresia surgery is performed subsequently.