Turner Syndrome Awareness Month: TS and Hearing Loss

Among the many health problems that can come with Turner Syndrome is hearing loss, for which there are many reasons. Girls with TS, myself included, tend to have lower-set ears and a wider, shorter ear canal. This leads to frequent ear infections during childhood and into adolescence as well as fluid build up in the middle ear which can result in perforation of the tympanic membrane, aka the eardrum, and lead to conductive hearing loss. Girls with TS can also experience what is called sensorineural hearing loss which happens when there are problems with the inner ear. This type of hearing loss tends to occur in young adulthood but can begin in childhood and progress over time. Most girls with TS have a combination of both conductive and sensorineural hearing loss and more than 25% end up with hearing aids by the time they are in their forties which is why it is extremely important that they keep up with their hearing tests and receive early intervention.

So how has this part of TS affected me? Like most girls with TS, as a baby I was extremely prone to ear infections and would often scream non-stop from the pain, giving my parents quite the time. As I got a little older, I remember looking forward to the banana flavored penicillin that I would get every time I had an infection, however, that was about the only perk. The shape and placement of my ear canal also caused me to build up a lot of ear wax which would impact my hearing and I remember having the visit the ENT a few times a year to get it cleaned out. I hated the feel of the machine they would use but always looked forward to my annual hearing tests afterwards because of the fun puppets that would move around and play different sounds. Many girls with TS have to get tubes placed in their ears to help alleviate the fluid and wax build up and subsequent infections and hearing loss but thankfully I never needed this and by the end of elementary school I was no longer really getting infections or needing to see the ENT and audiologist.

In my late teens and into my twenties, I started noticing more trouble with my hearing again to the point that I was frequently having to ask people to repeat themselves multiple times which got very embarrassing and frustrating. After a subsequent trip to the audiologist ended in a failed hearing test and very painful cleaning out of a large buildup of impacted wax from my ears (an experience I hope never to repeat), as well as getting a few ear infections, one of which was so bad I experienced my first ruptured eardrum (yes, it’s as painful as it sounds), I finally got reconnected with an ENT for regular ear cleanings. While I do have some permanent conductive and sensorineural hearing loss, especially at certain angles and frequencies, and while it definitely gets worse when I get wax build up during cold season, it is still mild enough that it doesn’t impact my daily life or require hearing aids for which I am extremely grateful. That being said, I am very aware that this could change at any time and am therefore cognizant of protecting my hearing, especially since I love to sing and have a job that requires me to be on the phone with patients a lot. This includes not listening to music or the tv too loud and wearing earplugs at concerts or the movie theatre. I don’t know what the future holds in regards to my hearing but there are so many more options available now for people experiencing hearing loss that I am no longer so terrified of the prospect and am confident that I will be ok if or when the time comes to look into aids.

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