Possible Otosclerosis — I'm Not Convinced!

I've had a complicated history with hearing loss and tinnitus stretching back 10+ years. I'm now 30, almost 31. I could probably talk all day about symptoms and what's possibly causing them. It would be an understatement to say I've researched quite a lot because I feel every ENT has either misdiagnosed me or failed to investigate the issues properly. I'll try to keep things brief but informative enough because I'm interested in other people's opinions on this.

Back in 2008 I caught a flu and experienced hearing loss in my left ear that never recovered. There was lots of squeaks and crackles coming from that ear and issues with ear pressure so ETD was likely the issue. I also had seasonal allergies which didn't help the either. Years later I tried a grommet but although on paper my audiogram was slightly better in the low frequencies it never felt as loud and clear as my right ear. I managed pretty well eventually, although the biggest hurdle was the loud intrusive tinnitus that came with it. I managed that eventually too. At this point my loss was conductive.

2 years later I suffered a blow to my right ear that caused a 30% perforation. That healed nicely. But I noticed a slight dip in the clarity of high pitched noises, even compared to my left ear which now seemed clearer in that department. It wasn't louder so it was barely a noticeable difference.

Fast forward 5 or so years later and I woke up with the strangest hearing issue in my RIGHT ear this time. This was after a night drinking alcohol. Every noise internal and external had a strange metallic echo, and high pitched noises felt like they were piercing my ears. Almost like a flutter in my ear drum and it gave me the chills like claws scraping a blackboard.

This issue fluctuated for months. Coming and going usually within the space of a day or 2 before returning to normal. It eventually subsided for almost 2 years before I had 1 more episode (2017). This time it took around 5 days to completely clear. Again though, everything went back to normal. Or close enough.

I've managed fine up until the start of this year (April) when the hearing in my right ear deteriorated pretty fast. 4 days and everything sounded muffled more and more as the days passed. My left ear started to sound much clearer in comparison. I was sure the left ear was now the better one, although multiple hearing tests told me my right is louder in every department. Tinnitus has spiked a lot during this period... becoming very intrusive and making it even more difficult hearing people, or anything over the noise it makes. Everything from pulsatile tinnitus, low hum, high hiss and random beeps like Morse code.

This is when my mentality changed from I can manage this, to this is becoming a serious issue. I'm suspecting an SSHL at this point in time. And also that each episode of the strange issues I had in my right ear years back were early warning signs of a more serious issue.

The thing is. I've produced music since I was a teenager. So I'm very attuned to frequencies and how everything should sound. An audiophile at heart so I was able to explain to an ENT in depth the issues I've had. According to my history and audiogram he thinks otosclerosis.

I'm not convinced. Ever since that drop in my right ear I've had some degree of improvement, and then other times it feels even worse because of how muffled things sound. Like a party next door type of muffled sound. From my research otosclerosis doesn't usually happen suddenly over a few days and usually progresses slowly whilst also not affecting the clarity until late into the disease stage when it progresses to the inner ear.

It now also seems to be having a fluctuating affect on my left ear were sometimes its muffled and other times its decent enough to hear speech. Just not to the degree of my right ear.

I was in the car the other day driving for an hour (mostly downhill) and when I got back my right ear was so much more muffled. Even though through the years of ETD issues I've learned techniques to pop my ears and relieve any pressure. It never seemed to make any difference and the constant pressure changing has affected my hearing clarity a lot.

This leads me to my final point. I'm flying to Paris next Tuesday. I've never flew before and the flight is only just over an hour. I'm really concerned about it negatively affecting my right ear because of the pressure changes from flying.

I've had a CT scan done to confirm the diagnosis but wont get the results till December. NHS is terrible for wait times.

I'm especially concerned because I've been researching about enlarged vestibular aqueduct recently and it seems to match my recent symptoms to a tee...

Sudden hearing loss that is mixed and progressive, fluctuates, bilateral and can also appear in adulthood. Also it mimics otosclerosis audiograms. I also have mild dizziness from time to time. BPPV is something that fits that issue and is also associated with EVA. The big issue is that it can be made worse by barotrauma or head trauma. I've suffered both and continue to have ongoing issues with ETD, more so on my right side where I have the most problems. Hence my now fear of flying and aggravating it even further.

This is sheer worst case scenario at this point, but it's the only condition I've found that matches most of my symptoms, if not all of them. I'm not looking for a diagnosis or anything, just maybe some personal insight from people with similar issues and how things are for them. I'm at my wits end with it all if I'm honest and definitely frightened by the fact my hearing seems to be rapidly declining in volume and clarity.

I'll press my ENT on these issues next I meet but until then I'm stuck with these thoughts. If you've made it this far thanks for taking the time to read, even if you cant help me with anything.

Guessing this wasn't so brief after all lol. Almost 6 in the morning here and I really should be sleeping.. just had to get this off my chest really!

Latest audiogram in the picture for anyone interested in seeing it. I have another 2 from 2009 and 2015 if they would help!

Edit: added some extra details and fixed some typos. Will go through again if I think of anything else.

 

That is true, but there are odd cases... I'm one of them. My hearing loss appeared overnight - and within 6 weeks tinnitus appeared. Otosclerosis, confirmed by surgery. Nobody in my family has it. Nobody can explain why it went so fast (instead of the years-long slow-progressing normal process), or why it immediately attacked the inner ear.

 

Very strange indeed. There is no history in my family of it either. Has yours affected clarity also and is it progressive/fluctuating?

I've been offered surgery if otosclerosis is confirmed, but if its misdiagnosed and it's really EVA, it could obliterate any hearing I have left. I don't have much AB gap anyway so doubt surgery would make much, if any difference for me.

 

Yes mine affects clarity. I did surgery 3 years ago and I am taking heavy doses of fluoride. Since then my hearing has been stable.

Leading to my surgery, a 55 dB air bone gap opened in just 6-8 weeks.

The surgery gave me back some hearing by closing the air-bone gap, but since I had sensorineural losses as well, those stayed of course (which explains the bad T I imagine).

You have about a 1% risk of total loss after a stapedotomy, IIRC. I asked my surgeon about his personal stats and they were much better than that.

Your surgeon is not going to move forward with the stapedotomy if s/he doesn't confirm otosclerosis "on site" (ie by actually visually confirming the site after retracting your ear drum), so you are very unlikely to lose your hearing from simply retracting the ear drum and putting it back, if it turns out you don't have otosclerosis.

Your AB gap isn't huge indeed, but it's not small either. I think you'd notice a change. Do you wear hearing aids?

It sounds like you also have hyperacusis (from your original post). This is one of the best things about surgery: it obliterating my debilitating hyperacusis.

Too bad you have to wait that long for your CT results. Note that it's only one out of many clues you should be looking at, and quite often, doctors do not see it on a CT. I had to show mine to about half a dozen doctors before the last one saw something.

Other symptoms to look at: lateralization (Weber/Rinne fork tests), stapedial reflexes (missing/weak), Carhart notch, Schwartz sign, AB gap, and others I'm probably forgetting.

Good luck!

 

Here in the UK I'm not sure they even know about fluoride treatment but if by chance they do find any abnormalities in my CT then I'll be sure to mention that.

Air bone gap in my left ear is far more noticable I guess so maybe there I could see a change. My right I'm not so sure. And really that's the ear I'm having most issue with at the moment unfortunately !

And yes I have hyperacusis but I wouldn't say its particularly bothersome for the most part. I'd even go as far to say that the more high pitches noises sound dull in my right ear the less hyperacusis there seems to be.

The fork test laterized to my left ear. I knew that would be the case as my voice is more pronounced on that side and a lot clearer in my head due to the conductive loss. The ENT mentioned bone conduction tests arent reliable for otosclerosis but if it works for my left and that is supposed to be oto then I'd imagine the same would happen for my right ear. It just doesnt though unfortunalty.

Also because of scar tissue on my right ear drum due to the perforation there isn't a clear window to see through. So visually it would be hard to spot the Schwartz sign.

There is however a clear carhart notch at 2k on both ears hence his reasoning for the diagnosis.

I'm glad yours has been stable and hopefully with some luck mine can be stabilised. The thought of going deaf scares the shit out of me. And the rate I'm going it wouldn't take long to get there.

I'm not wearing hearing aids at the moment but I've been referred for them. Again the wait times are abysmal so God knows when that will be. They put me on a 40 week wait list after my sudden hearing loss because they thought it was conductive. I already knew it wasnt as it felt so different to my ear that actually had conductive loss for 10 years. So I got tested at a private audiologist who basically referred me straight to an NHS ent because it came back sensorineural loss. Even then I didn't get to see them for a month.