Teenage Boy With Dwarfism Battles Rare Cholesteatoma Ear Cyst

3

Cholesteatoma Ear Cyst           

When my 13-year-old son, Samuel, rubbed his ear a few months ago, I didn’t think anything of it. He had not been prone to ear infections. When he was four or five years old, he experienced a double ear infection but no other occurrences.  At the time, we took him to his pediatrician and received treatment. Within a week, he was all better, and I never thought of it again until this year.

Earlier this spring Samuel not only rubbed his ear but had clear ear drainage which I found concerning. After three rounds of antibiotics which did not help the situation, we made an appointment with his Ear, Nose, Throat doctor to take a closer look.

At the first meeting, he said he could not see inside Samuel’s ear clearly and wanted to do a debridement. This procedure required my son to be sedated while the doctor removed a small amount of tissue for testing. The doctor said he wanted to know if he was dealing with just the tip of the iceberg or the whole iceberg.

Teenager Faces Ear Cyst
*Samuel’s primary diagnosis is Thanatophoric Dwarfism.

Further Investigation

The results of the test came back clear. Samuel’s doctor ordered a CT scan, just to be sure. I didn’t think anything of it. Since the debridement came back with no issues, I didn’t think there was anything to worry about. That was until after the doctor read the CT films. It turns out Samuel would need either a Canal Wall Down or a Canal Wall Up procedure both considered radical ear surgeries.

The surgery would remove ear tissue called Cholesteatoma. What surprised me in my research was this condition is not limited to special needs children, children with dwarfism or any other condition. Children without medical challenges can have symptoms. Even adults can receive this diagnosis.

WebMD states: Cholesteatomas aren’t common — only 9 out of every 100,000 adults in the U.S. get them. They can show up at any age, and men are more likely to get them than women.

Definition of Cholesteatoma

Cholesteatoma is an abnormal skin growth or skin cyst trapped behind the eardrum, or the bone behind the ear. Cholesteatomas begin as a build-up of ear wax and skin, which causes either a lump on the eardrum or an eardrum retraction pocket. Over time, the skin collects and eventually causes problems like infection, drainage, and hearing loss. The skin may take a long time to accumulate and can spread to the area behind the eardrum (the middle ear space) or to the bone behind the ear, called the mastoid bone. https://www.enthealth.org/conditions/cholesteatoma/

 

Diagram of Ear Cyst
Diagram of Ear Cyst: Cholesteatoma

List of Symptoms

What are the symptoms of Cholesteatoma? For my son, it was drainage which did not clear up with the treatment of antibiotics. Symptoms typically appear in one ear but can also appear in both ears. Per WebMD, symptoms can be the following:

  • Constant sound inside your ear (tinnitus)
  • Dizziness (or vertigo)
  • Ear infection
  • Earache
  • Feeling of “fullness” in one ear
  • Fluid that smells bad and leaks from your ears
  • Trouble hearing in one ear
  • Weakness in half your face

Treatment

To remove the Cholesteatoma requires surgery. The question then becomes which procedure is best. An Ear, Nose, Throat doctor renders their opinion based on each child’s individual case. Options include Canal Wall Up or Canal Wall Down surgery. Because the Cholesteatoma had grown extensively in my son’s case, the approach taken was a canal wall down procedure.

Click here read more about the Canal Wall Up verses the Canal Wall Down debate: http://theotorhinolaryngologist.co.uk/new/images/pdf/v6_n3/The%20pros%20and%20cons.pdf

Prognosis

One month out, Samuel is doing great. I worried for weeks before surgery but had great hope knowing another boy who had the procedure last year is doing great. That one story gave me so much hope in the face of my son’s surgery and recovery. Next steps include looking into hearing aid options. The story continues. Feel free to follow Samuel’s journey on my personal blog.

If you have heard of this condition and would like to share your own story of hope with other moms, comment below. Knowing you are not alone in the journey helps so much. 

 

3 COMMENTS

  1. Dear Evelyn,
    So sorry your son had to go through this! I fully understand. I have a daughter who has had numerous surgeries fighting this disease.
    Mother to mother…
    Stay up on it. Get his ears checked every 3 to 6 months for new growths and pray without ceasing!
    Thinking of you today,
    Glenda
    Charlevoix, Michigan

    • Hi Glenda,
      Thanks for reaching out. Right now we are going monthly with a plan to go every three months to clean the ear. The ear is still in the healing phase right now. Thank you for your encouragement to be diligent and pray. We certainly will. How is your daughter doing?

    • Hi Glenda,
      Thanks for reaching out. Right now we are going monthly with a plan to go every three months to clean the ear. The ear is still in the healing phase right now. Thank you for your encouragement to be diligent and pray. We certainly will. How is your daughter doing?

LEAVE A REPLY

Please enter your comment!
Please enter your name here