I’ve noted a definite trend over the past year: my postings on this blog have migrated largely into the realm of poetry. It was not a conscious migration. I think that when I am in the midst of such uncertainty, as we’ve been with Josh’s right ear, I need to use the sort of vision-scope that only comes through the lyrical. I am touching down into tender places; touching lightly so as not to upset any of the delicate balance we might have found on a given day (or week or month).
But this blog has, in the past, gone into great detail about Josh, my third child’s hearing loss journey. If you’re here because of your own (or your child’s) hearing loss, you may want to check out past entries to get all caught up. Still, I haven’t posted in a long time, so I need to update the timeline.
Josh is now six and has started Kindergarten. He still wears a hearing aid in his left ear, which shows moderate conductive loss (so it has slipped through the years from mild-moderate loss–we are not sure why he’s losing ground because conductive loss typically remains stable). This year, Josh is using an FM system at school and loves it–he loves hearing his teacher so clearly in his left ear.
In May, 2012 (at age 2.5), Josh had a tympanoplasty and an ossicular chain reconstruction, with a PORP (titanium rod) replacing his first two middle ear bones. He needed the tympanoplasty to repair his eardrum, although we would have waited until he was older if he hadn’t needed major dental surgery. The decision to replace his first two middle ear bones was made in the middle of his surgery. You can read more about this surgery on this blog if you look under “tympanoplasty.”
The surgery restored Josh’s hearing to (at one point) 10 db across all frequencies. It was pretty miraculous. We’d often forget to use Josh’s left hearing aid because he was hearing so well in his right ear, we wouldn’t even notice when his left ear went unaided. Still, within 5-6 months of the surgery, that ear started to steadily discharge an amber greasy matter. We saw our former ENT many times in the following two years; occasionally the discharge was cultured and never churned up anything. Perhaps his ear just couldn’t rid itself of ear wax, we were told.
In December of 2013, Josh’s hearing in his right ear started to decline–slowly, but steadily. We’d chip away at 5 db every 3-6 months. It was worse, for sure, when he had a cold. His former ENT raised the possibility of cholesteatoma and ordered a CT exam. The CT exam showed excess fluid in the ear and so Josh was put on three-week course of strong oral antibiotics (at age 4, this was Josh’s first time taking antibiotics–now it’s become routine) and it was hoped that all was resolved. It was summer then, so Josh seemed to improve (although his hearing never went back to 10 db–we did get to about 20 db).
During the 2014-2015 school year, Josh had more trouble again with his molars (all molars had been capped in his surgery at 2.5). Two of the roots became infected and he lost 2 molars–with spacers inserted in their place. Are the teeth issues tied to the middle ear issues? No one can tell us. We’ve been told Josh’s issues are syndromic, but after repeated testing, Josh’s genetic doctor has not found an exact cause or a similar case to Josh’s.
In March of 2015, Josh had terrible ear pain in his right ear (9 on a scale of 10–and Josh is pretty experienced with rating pain). On the way to the ER, the eardrum ruptured, relieving his pain–and he was placed on more oral antibiotics, instead of drops (because it was the ER, after all). The next morning, Josh awoke in a panic; he had absolutely no hearing in his right ear. He also ran a fever for about a week, in spite of the antibiotics. His infection eventually healed, but his hearing stayed at about 25-30 db. Three weeks later, the same thing happened; fever and loss of hearing his ear. His ENT refused to engage in a conversation about what was going on and said Josh was unlikely to have cholesteatoma, because the CT scan a year earlier hadn’t shown it.
We went to the Mayo Clinic for a second opinion. Our new ENT suggested sedating Josh to go in and see what was going on. He told us that CT scans and MRI’s don’t always catch cholesteatoma (and it can hide out in the mastoids). On June 19, 2015, Josh was sedated at a hospital in Rochester, MN. The ENT came out from the OR to tell us that he was 1000% sure Josh had cholesteatoma–it was coming out of his ear and had wiped out some of the eardrum. He proceeded that afternoon with a five-hour surgery to remove the cholesteatoma in a wall up procedure (retaining Josh’s ear wall); tympanomastoidectomy.
Our ENT told us that he was confidant he got all of the cholesteatoma (although one can never be 100% sure because if a small speck is left, it comes back). Cholesteatoma is basically extra skin that gets in the ear and grows much like a tumor but is almost always benign. Where cholestetoma can wreak havoc is it can erode the ear bones, the facial nerve, and worst case, can grow near the brain and cause meningitis. Thus, it has to be dealt with if present.
After removal, cholesteatoma returns in 30% of the cases (I’ve seen the odds at 50% for children because they often have eustachian tube disfunction, which can create a pressure that pulls in the eardrum, creating a good environment for cholesteatoma). Our ENT also was able to retain Josh’s previous PORP because it wasn’t in the zone of the cholesteatoma.
By October of 2015, Josh’s hearing on the right had returned to 25 db across the frequencies. We tried a hearing aid, but Josh complained it was “echoey” and that the ear mold rubbed against the still-angry scar where they took cartilage for his new eardrum. So, we gave up on aiding the right side, for now.
Our ENT is monitoring Josh but did not propose a “second look” surgery at the six to eight month point, which is done when an ENT is not sure they got all the cholesteatoma and they want to go back in before any returning cysts get too large. While the cysts are yet small, an ENT has a better chance of eliminating all of the growth.
Our ENT told us if he did have to go back into Josh’s ear a second time, he’d likely do a “wall down” procedure, that would involve removing Josh’s ear wall and basically eliminate the outer ear, creating a large cavity that couldn’t ever get water in it and has to be cleaned out every six months. I’m not sure how Josh could have much hearing left after a “wall down” procedure. It’s so hard to think of Josh never being able to swim or play near water.
Well, Josh was almost at the five month point past surgery and we were feeling pretty good, especially since it seemed this tympanoplasty actually took and Josh’s new eardrum is holding up really well. Josh had a bad cold last week and then awoke on Thursday morning, as my poem references, with a fever and ear pain in his right ear. He was placed on antibiotics over the phone since his ENT at the Mayo Clinic cannot see him until this coming Thursday (the 19th). Josh’s hearing has been knocked out again too. This makes us quite worried about the return of cholesteatoma because colds causing ear infections were rare in Josh before cholesteatoma and they never knocked out his hearing.
We are still out here hoping and praying for the best possible result; and the ENT will unlikely be unable to “see” anything on Thursday, as cholesteatoma exists mostly within the middle ear. I’m sure Josh will be monitored more closely now, however. I’m not sure if this event is enough to have the ENT schedule a “second look” procedure. We aren’t eager for more surgery; we fear Josh having a lifetime of surgeries. We may even seek a third opinion if a wall-down procedure is proposed–because this is, after all, Josh’s entire lifetime ahead that will be impacted.