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Congenital Cholesteatoma
Boots Plott

Like thousands of families from every county in Alabama, the Plott family of Tuscaloosa depends on the Children’s Hospital of Alabama for the specialized pediatric care their son, Boots, can receive nowhere else in our state. A persistent ear infection resulted in Boots being seen at Children’s by Dr. Audie Woolley, a renowned otolaryngologist. The news was not good. Boots was one of only a handful of children in the world to have congenital cholesteatomas in both ears. Cholesteatomas are benign but fast-growing cysts that can result in hearing loss and even harm the brain.

Here is Boots’ story, as told by his mother, Blair Plott.

Dr. Woolley and the rest of his staff were amazing from the onset of our visit. After reviewing Boots’ CT scan, Dr. Woolley believed him to have fluid in his right ear and a small congenital cholesteatoma in his left ear. He wanted to schedule surgery immediately.

Forty-eight hours later, we were squeezed into a schedule already packed with a very long day of intense surgeries. The original plan was to release the fluid in the right ear, put in a tube and then remove the cholesteatoma in his left ear. But soon after Boots was taken back to surgery, Dr. Woolley appeared in our room with bad news. Boots had extremely rare bilateral congenital cholesteatomas. Later we would find out that only a handful of cases of bilateral congenital cholesteatomas have been reported in the world.

Instead of doing the major operation on the left ear, Dr. Woolley elected to postpone that surgery in order to remove the very large cholesteatoma that was in Boots’ right ear. The right ear would require three hours of drilling, and the mass would need to be untangled from the facial nerves and ear bones. Because of its size, it had obstructed the CT scan and appeared as fluid rather than the large mass.

After hours of drilling, Dr. Woolley came back with news. The cholesteatoma was out and Boots was doing great. His facial nerves were intact, but his inca and malleus (ear bones) were completely corroded because of the cholesteatoma. Dr. Woolley reconstructed the eardrum and hoped to put in titanium implants to replace the two lost bones at a later date. We knew there would be a loss of hearing in this ear, but prayed the left ear would be okay after surgery and Boots’ hearing would be intact in the left ear.

Eight weeks later, we went in for surgery on the small mass in Boots’ left ear. Again, Dr. Woolley came out with a report soon after starting surgery. The mass was not as small as originally suspected, and it too was entangled in the facial nerves and ear bones. Three hours later we received the news that surgery had gone very well; Boots was in great shape, but once again an ear bone had to be removed and his eardrum reconstructed.

On Boots’ follow-up visits, hearing tests were performed and it was confirmed that he had some hearing loss in both ears. For the next year, he used the loaner hearing aids graciously provided by the HEAR Center at Children’s Hospital.

A year later (earlier this summer), Boots had surgery on his right ear to search for and remove any re-growth of the cholesteatomas and to place titanium implants that would act as the lost inca and malleus bones. If even the tiniest piece of the cholesteatoma cyst was missed during the previous surgery, this procedure could again be a very long one since cholesteatomas can grow back very rapidly.

This time, however, we received very, very good news. Dr. Woolley had been successful in removing all traces of the cholesteatoma during the first surgery on the right ear, so there was no cholesteatoma re-growth – truly an amazing result! In addition, Dr. Woolley was able to insert the new, titanium ear bones.

For the next three weeks, Boots had to be totally sedentary. It was imperative for him to remain calm in order for the implants to take hold. The most ‘activity’ he allowed was to get in the car to go to a movie.

Six weeks after his latest surgery, we returned for his hearing tests and, while he does still have some hearing loss in his right ear, much of the hearing was recovered through the tiny implants. We are thrilled with the success of his surgeries to date. Boots will have surgery on his left ear in November and we expect he will then have functional hearing in both ears.
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