The beginning... cholesteatoma

Common questions I am asked 'was he born with it?", "was there something wrong in your pregnancy?". To answer this we must start at the beginning. I was 26 when I was pregnant with Braydon. He did attempt to come into the world a bit early (30 weeks) but I was prescribed tributaline and bedrest (strict bedrest). He made it to 37 weeks.

From the start he was a screaming baby. Hard to nurse. Did not enjoy contact or life for that matter. I finally got him nursing and learned that if I wore him in a baby carrier on my chest with him forward facing and straight up he would finally stop screaming. He hated car rides, the swing, laying down, you get the jist.

His first eardrum burst at 3 months old. By 12 months old he was only saying dada and throwing horrible temper tantrums. His walking was not on schedule. We saw our first ENT (Ear Nose & Throat doctor). This resulted in a referral to a speach therapist and an occupational therapist. He had 7 sets of tubes in the next 2 years and his adnoids removed. He did begin walking and some talking but clearly things were not right.

By the age of 3 his eardrums were just gone. He had large amounts of green and yellow goo draining down from his ears. We asked for another opinion. We were directed to Riley Children's Hospital in Indianapolis, IN. where we met Dr. Micheal Fritsch.

Our first meeting with Dr. Fritsch he announced that Braydon had pollups (begnine bumps) in his ear canal. He did surgery and removed them. Within 4 months the snotty drainage was back. His poor ears were raw from the constant drainage.

At age 4 Dr. Fritsch went into surgery to remove those pesky pollups again and found that each ear (bi-lateral meaning both sides) had a cholesteatoma. This was upsetting for everyone. He scheduled surgery for the 1st removal the next week.

A cholesteatoma is usually caused when the eardrum sucks into the middle ear and then bursts. The tissue (skin) develops an enzyme that can eat through bone. The normal ear has 3 bones (ossicles) that are used for hearing. The eardrum transmits the sound to the ossicles which vibrate and send the sound on. In Braydon's case the cholesteatoma wrapped around 2 of the 3 bones in both ears.

I must tell you that having a cholesteatoma is very rare. Having bi-lateral cholesteatoma's is unbelievable. To remove Braydon's cholesteatoma an incision was made behind the ear. The ear was pulled forward exposing the middle ear. The cholesteatoma and damaged bones and tissue were removed. The ear was sewn back in place.

An attempt was made in the left ear to replicate the ossicle chain. This worked for a small time which allowed the doctor to remove the cholesteatoma from the right ear. Since each removal rendered him nearly deaf in each ear the goal was to restore hearing in one ear before moving onto the next ear. Of course the entire time the cholesteatoma was left in, it continued its path of destruction.

At the age of 6 in May 2006 a new nightmare began. It started in the form of a fever and an earache. I thought nothing of it. Called the pediatrician asking for an antibiotic (it was the Friday before the Memorial Day weekend). My request was denied. I resigned and took Braydon to the Riley Children's Hospital Emergency room. My thought was we would be done in 2 hours with our antibiotic.

Unfortunately, that was not our situation. Braydon had what is called a mastoid infection. Your mastoid is the bone behind your ear that has little to no hair on it. Braydon was given 2 cat-scan's and admitted. He was started on 1 IV antibiotic. After 12 hours his fever was at 104.8 and a 2nd antibiotic was added. I noticed his lymph nodes on his neck were the size of golf balls. I had one very sick child.

He was rushed into surgery where an incision was made into his ear drum and the infection was suctioned out. We stayed in the hospital another 4 days until his fever finally broke. Turns out we would repeat that infection hospital stay and surgical intervention within 2 months. At that our surgeon said we had no choice but to have a 'mastoidectomy'. What this means is the surgeon made the incision behind the ear again. He then shaved the inside portion of that bone down to smooth to eliminate the possibility of another infection.

Wouldn't you guess that within a very short time the other ear began having these exact same infections. Each time we were in the hospital for about a week, IV antibiotics and emergency surgeries to reduce infection.
Ultimately, a mastoidectomy was needed on the left side as well.
So, by 2nd grade Braydon had each ear removed 2 times. He had a cholesteatoma removed from each ear and a complete mastoidectomy bi-laterally.

Right now you are asking "what are the signs?". The first sign is a high fever, then pain behind the ear in the mastoid area, it may be hot to the touch, it will become swollen, it may even turn red, swollen lymph nodes. If you have swelling in the mastoid area for any reason please rush to the ER. This can be a very fast spreading infection.

2012 started the next part of Braydon's journey.. fungus. The problem started out with me noticing that Braydon's ear drum had a hole. Then while watching the hole over a period of about 2 months the hole was getting noticeably larger. Before long the entire ear drum was GONE! Then it started. The black fungus. The never ending pain.

At this point we began seeing Dr. Charles Yates as our new ENT. He is the top specialist at Riley Children's Hospital. He is very kind, patient and intelligent. I can only explain what an intelligent doctor he is by continuing with Braydon's journey.

The pain behind his ear was so intrusive and intense he began seeing a neurologist and a psychologist at the Riley Pain Clinic. They determined that the repeated need for cuts behind Braydon's ears had caused nerve damage. This nerve damage causes Braydon to have constant pain. We are slowly learning things that make the pain worse but yet to find anything to relieve the pain.

Back to the fungus. We tried surgically removing it. It grew back. We tried creams and concoctions only to have it grow back. Finally, insurance approved us to go to Cincinnatic Children's Hospital. Dr. Arjmand had a new idea. A skin graft would be taken and placed where the bad tissue was.

Back in Indiana we went for surgery in September of 2013. In Braydon's left ear the mucosalized tissue was removed. It went all the way to the bone.

What is mucosalized tissue? Well besides most likely being misspelled it is wet tissue. In our normal ear the skin is dry like the skin on your arm. Behind the ear drum it is wet like in your nose or mouth. It is a mucosa tissue. When Braydon's ear had no eardrum for over a year there was no boundary for his skin to separate the middle ear from the ear canal.
In a 5 hour surgery the mucosalized tissue was removed and a graft was taken from his leg.

After this surgery his air sacks were not absorbing the oxygen. He was not panting or gasping. Everytime he fell asleep his oxygen level would drop dramatically. It was very scary. We stayed in the hospital for 48 hours. A respiratory therapist worked with him to get he air sacks to open back up. Finally, his lungs were working! We went home to heal. We have seen the surgeon every 2 weeks since the surgery in September and it appears as though we have finally rid ourselves of the fungus!

Braydon has a condition where he creates a fluid in his middle ear that is thicker than what a normal person creates. In my ear and your ear the fluid drains down our estuation tube. His tubes were ruined by the cholesteatoma. So his fluid has no where to go. That is why his ear drums burst. The simple answer is put a tube in the drum. However, because his mucous is so thick the tubes only last about 6 months and he still suffers from frequent infections.

If your child suffers from this problem we have found that (doctor approved and recommended) 1/2 water and 1/2 white vinegar is a great natural fix. You want to ensure that the mixture is room temperature or your child might actually throw up. The temperature change actually triggers vomiting. You can use this twice a day when the ear is clogged or when the drainage from an active cholesteatoma is bad. We have reached a point where we use this as maintenance. It keeps the mucous thin and helps us to prevent infections. We are still having problems keeping tubes where they belong.
We have tried regular tubes, butterfly tubes and T-tubes. Currently, we are using a T-tube in the right ear.

Back to the left ear. When the skin graft was constructed a new ear drum was also constructed from tissue under Braydon's scalp. A silicone filled ball was placed in the middle ear space to effectively create a middle ear space and to hopefully prevent the new eardrum from sucking back into the middle ear and creating yet another cholesteatoma. So far.. it has worked!

We currently are working on hearing reconstruction. Here is what we have tried so far. First, Braydon had an ossicular chain reconstruction. "A what" you say. This is a combination of titanium and cadaver bone constructed to mimic the ossicles. This is actually a great fix. However, due to Braydon's thick mucous this did not last long.

We have tried hearing aids. This seems to trap moisture in the ear canal and create.. you guessed it..fungus.

Now what? When the bones are gone you have what is called a conductive hearing loss. There is a great device known as a BAHA (Bone Anchored Hearing Aid) that will fix this. The older version had what is called an abutment that is implanted into the skull and sticks through the skin, behind the ear, and the BAHA device is attached to that. The BAHA device is the processor and translates the sound. The abutment just conducts it through the bone. Essentially fixing the problem. You can get the BAHA and not have an implant (if the abutment is not for you) but wear it on a special headband.

This has been Braydon's preferred method of several months now. We have been waiting for Cochlear to come forward with a magnet that would allow the BAHA to magnetically stick to the head and conduct sound. There is another company (Sophono) who offers this however we have personally decided that they were not the best company/product for our son. Our son chose not to have an abutment so he wears the band. It is my personal belief that if it is not a life saving surgery I will allow Braydon to have input. This is his life, after all.

Today, Braydon is 10 years old. He attends mainstream 4th grade school in Indiana. He has had 31 ear surgeries to date.

We have just received word that the FDA has approved Cochlea's magnetic implant for the BAHA. Braydon's 32nd ear surgery is scheduled for February 25. During this surgery we have a lot going on. The right ear will have the current tube removed, a patch placed over it's current location and a new tube inserted in another place in the eardrum.

In the left ear, the silicone ball will be removed. If there is enough room possible the surgeon would like to try another titanium implant. While I don't personally have a lot of hope in that process I respect what the doctor hopes to accomplish.

Now for the exciting part... Braydon will be the first child to be implanted at Riley Children's hospital with the magnetic BAHA option. It will take 30 days before he can 'go live'. That means attach his BAHA to his new magnet implant. 90 days after the first magnet is implanted Braydon will be eligible for his implant on the other side.

We have been seeing Dr. Charles Yates since 2011. He will be performing the upcoming surgery in February. We are equally excited to see how things turn out!

So now you know the beginning of Braydon's journey. Stay tuned and I will be posting ideas that our family has been implementing to help make your hearing impaired child's life a little easier.

Quick example: Your child finds Closed Captioning to hard to follow/read. We bought a karaoke game and the words scroll across the screen just like CC. Guess whose son learned to watch TV with CC in less than a week? This is something that your school is obligated to provide CC for any shows watched in class.

Companies that I recommend for help:
Hands and Voices
Guide By Your Side
Hear Indiana
Alexander Graham Bell Association

I can't wait to hear your feedback, questions and suggestions.
Sincerely,
Braydon's Mom