Addressing My Son’s Mild Hearing Loss With New Ear Tubes

Hearing loss and ear infections can be a common symptom of infantile-onset Pompe disease

Keara Engle avatar

by Keara Engle |

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At my 4-year-old son Cayden’s last appointment with the otolaryngologist (ears, nose, and throat doctor, or ENT), the doctor and I decided that we should look into getting a new set of tubes for Cayden’s ears.

Cayden, who has infantile-onset Pompe disease, showed signs of mild hearing loss during a test and had fluid in his ears. I decided that getting new tubes was the right decision for him.

He recently had the surgery, which went as expected, for the most part. Getting the ear tubes in place wasn’t an issue, but we did have problems with Cayden’s port around the same time. Because we couldn’t access his port, the anesthesia team had to place an IV prior to surgery. Once that was placed, things proceeded as expected.

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Cayden has had ear tubes before due to recurring ear infections and mild hearing loss, a common symptom in children with infantile-onset Pompe disease. While we are familiar with the tubes, the procedure was performed by a new doctor, as we had switched ENTs.

I was a little nervous about someone new performing the surgery, yet at the same time, I knew that it’s a common procedure, which helped to ease some of the stress. Plus, I ended up really liking the new doctor.

After the tubes were placed, my grandma, who accompanied us, and I had a long conversation with the doctor, who said that Cayden had a ton of thick fluid in his ears that wouldn’t have drained without the tubes.

We’re still learning about Pompe

He also told us that one of Cayden’s eardrums was retracted, or “sucked in.” Apparently, this is common in children with glycogen storage diseases, and can be caused by the facial structure that is a characteristic of Pompe disease. Children with Pompe may sometimes have cheekbones that sink in or are pushed back, which can cause problems for the ears. This can eventually lead to permanent hearing loss.

I was unaware this could occur. However, it makes sense the more I think about it. I’m glad the new doctor mentioned it. Even though we are more than four years into this Pompe disease journey, I’m still learning as time goes on.

The last thing Cayden’s ENT doctor mentioned is that ear tubes may be a more permanent thing for Cayden, due to the retracted eardrum and the chronic fluid buildup that makes it hard for him to hear. We may look into something that lasts longer, such as a pair of long-term ear tubes. There are pros and cons to these types of tubes, but that’s a discussion for another day.

Overall, I think the procedure was worth it. I have noticed that Cayden can hear a lot better now. He doesn’t ask, “Huh?” or “What’d you say?” as much anymore. I’m anxious to see how he does at an upcoming hearing test at the end of the month, where I’m confident we will receive good news.

Note: Pompe Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pompe Disease News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to Pompe disease.


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