How Rare Is Severe Hyperacusis?

Hello all. Apologies for my many posts of late. I just wanted to find some opinion on the prevalence of severe hyperacusis.

In the following thread about research @Juan made a comment:

Where Does Hyperacusis Stand in Terms of Ongoing Research/Treatments?

I have been panicking a fair bit lately about worsening of my hyperacusis. About it becoming very bad later on. Essentially at present all that I experience in terms of pain is with tinny speakers and artificial audio. I used to find cutlery and plates etc problematic but no longer. But I’m zeroing in on this statement as I’m hoping that it is true and that worsening of the condition is hopefully not very likely if I’m careful (as I can be)?

 

Hi FrontRoomFanatic,

I understand that you may be very worried that your condition may get much worse, I can imagine how scary it must be to read stories like some of ours on here of how ours progressed and worsened. I get how real that can feel. Now, I want you to take a step back from that and understand that we can only fight through our battles one day at a time. As someone who is suffering from very severe symptoms, I can tell you it is scary and it is hard - but I also have a happy life. I decided a while back that I couldn’t live with my condition my whole life, so I’m not; I’m living with it today, and tomorrow I’ll live with it then. That’s all we can do. Sometimes I wake up and my first sensation is stabbing, horrible pain, but sometimes I wake up and my tinnitus is a quiet hum and my H a low low throb and I pick up my guitar and play until I can’t play anymore. So if you’re scared, we’ll be there with you, but have hope with that scared. The bad days sometimes out number the good, but the good days make all the difference. But after all the nice pretty words are said, invest in a good set of earplugs and be careful.

 

It may be rare across the population at large, but it seems quite common in this forum.

Good news: It gets better for many people. If it gets worse, learn quickly from it and avoid the situations that made it worse if you can, or limit your exposure.

Mine got worse quickly over a period of two months, but in the 10 months since then it's steadily improved.

 

What's yours like now? It definitely seems like for many people it does improve.

 

Hi there!

I wrote that sentence because for me hyperacusis at its worst meant feeling sound cut through my brain even wearing earplugs and earmuffs on top, and that's apparently very rare. Actually several ENTs said they had seen only another patient like me in 40 years of practice. Twice I felt also electricity going up from ears to my brain... kind of a "frying" sensation, pretty bad.

There are statistics on the Internet that say 1 person in 100.000 have hyperacusis. That's why I wrote that true hyperacusis is very rare, and my case can be even less common than statistics show.

The problem with sound, as I perceived it, is that a single and very brief (seconds) noise exposure can make your hearing feel muffled, distorted, not balanced, coupled with ear pressure, facial pain, massive headaches and ear pain. This starts subsiding very slowly over a period of 4-6 weeks, if the noise was bad enough. After 6 weeks the hearing does not usually return to baseline, it keeps changing, and may deteriorate, so there is a down trend and "good days" are just days without much pain, pressure or symptoms in general... but the underlying problem (noise trauma, really loud noise) that caused hyperacusis in the first place cannot be solved or cured, since it is mainly cochlear damage, and damage to synapses, and maybe some deterioration of the pathways that drive sound to the brain.

This "cycles" of noise exposure and recovery basically go one after another because it is almost impossible to avoid loud sound when ears are so sensitive that double protection won't be enough. So just going out to buy a loaf of bread may mean running into noise.

True hyperacusis means that wearing double protection you can turn your back to people and hear them talking at normal volume. So that's why I wrote this is very rare, and most people, even in this forum, who say they have hyperacusis don't have it to this degree. They have just some sort of noise sensitivity.

After a few years with so much hyperacusis I lost some hearing due to just the odd noises (just a bunch of one-off brief episodes of running into noise), so now I am not troubled by sound so much, but it does not mean I am cured, it was mean that my body traded a certain degree of extreme hyperacusis for hearing loss and tinnitus.

So basically my advice is to put things in perspective, and do what is in your hands to feel better, check with ENT, neurologist, TMJ doctor, check your jaw, check with your dentist, go to physiotherapy, eat healthy, do some exercise... it's all we can do. Maybe doctors find some obvious cause for your hyperacusis, but this is unlikely if it comes from noise trauma, for instance, and this is really loud noise, not just any noise. There are clinical definitions for all this..

So after doing all the stuff and medical visits that are in your hands.. then there's not much else to do but accepting this condition, as people do with any other chronic health problem... that's what I did.

I hope you improve, good luck!

 

My advice: Take common sense measures and don't apologize for them. This condition is rare enough to have a healthy "trust yourself" aspect to it. Don't overprotect, but lean hard on the side of caution.

As for how rare is hyperacusis, I think, like Juan said, much of it depends on severity. You may hear people say "true" hyperacusis because there's a massive difference between small sound sensitivities and hyperacusis. You do not and can not live a normal life with hyperacusis. Trust me, I've tried. I've drank the "just live with it" kool-aid. It's a total joke. I'm literally struggling to type this message. When I do too much exposure (and I'm not talking about loud noises) my condition worsens. If I talk too much, it worsens.

Anyways, true hyperacusis is so rare that even my TRT doctor (months ago) was dumbfounded. The ENT director of a nationally recognized hospital didn't have even the slightest clue on what I had. Another ENT, with a pretty big name, thought I was crazy for not getting an MRI.

So you figure, for a top level ENT to be more clueless than my cat on what hyperacusis is, it can't be that common. I would guess that moderate noise sensitivities (can't go to concerts, but can live a normal life) probably affects 1 in thousands. True hyperacusis, where teams of doctors are dumbfounded, is very, very rare. A low estimate is probably 1 in 300,000, but honestly, 1 in a million wouldn't surprise me.

It's rare enough that I never even heard of it before getting it. It's so rare that I had it and didn't even know what it was for a while. I just assumed that if it was a medical problem, I would have heard of it. Then it got worse and I realized how life-altering it would be.

 

@Juan,

It sounds like you have experience with severe hyperacusis. I'm wondering if, for you, it was ever bad to the point where you couldn't drive?

Driving seems to be the biggest trigger for me, which is distressing because it keeps me trapped inside this house.

Please let me know how you recovered.

Thanks.

 

Hyperacusis itself is very rare. While studies seem to indicate that only 1 in 50,000 people get it, the numbers could be higher than that, but the cases might be mild and that might explain why they’re not getting more attention.

Severe hyperacusis is extremely rare; infinitesimally rare almost. I live in Columbus, Ohio. In the groups I am and, that we know of, it’s only me and 1 other person in a city of 1 million people who have severe hyperacusis. I’ve paid attention to the general numbers, too. I honestly think that severe hyperacusis only happens for 1 to 3 people for every million. It’s literally like the lottery, but the worst kind of lottery ever. It’s so rare that people have a higher chance of getting struck by lightning in their life. The National Weather Service says there’s a 1 in 15,000 chance of getting struck by it. Well, with hyperacusis, there’s a 20 out of a million chance of getting struck by it, and only a 2 out of a million chance of it being severe. It’s so rare that you wonder why anyone has to get it. Like, what is going on in those people’s bodies that isn’t in everyone else’s? Why does anyone need to get it when it’s so exceedingly rare?

 

I do not think this 1 in 50,000 number has any basis in fact. Apparently this figure was stated on an old hyperacusis patient support site (but with no basis in fact) and then it got repeated over and over, and now is mentioned on many websites (never with any reference to the source).

I think your 1 to 3 out of a million assessment is in the right ballpark. I have come to a similar estimate on my own, thinking about it the same way you are thinking about it.

 

How do we define severe hyperacusis? Does it imply pain? I think using noxacusis is preferable to distinguish these conditions.

Mine is quite certainly not severe, mild perhaps even. Certain sounds (trucks, motorbikes) appear very loud and very distressing. Earmuffs or foam earplugs make them manageable even at fairly close distance.

And I can drive my fairly loud convertible (with top up) with 26 dB musician's earplugs (foam earplugs are probably better for longer drives).

 

With hyperacusis and its subsets, what makes it even worse is knowing that only a few people for every million get it severe, and only 10 or 20 for every million get it at all. It makes you realize that it’s not profitable for the world to care about. Like I’ve said before, there’s a greater chance of someone getting struck by lightning at some point in their life than ever getting hyperacusis — 1 in 15,000 according to the National Weather Service. And hyperacusis is a handful of people for every million, much rarer. So when I think about this stuff, I sometimes wonder if our quest for change or making a difference is ever gonna work. Truth is, most people can be as reckless as they want with their ears and never get this crap; generally, they’ll only get mild tinnitus or some hearing loss, which isn’t that debilitating in most cases. The general public doesn’t worry about lightning or thunderstorms, so why would they worry about ears, especially when the chances of catastrophe are astronomically low? The world doesn’t show much compassion when it comes to disabled minorities with rare diseases. Part of that is because the conditions are rare and often invisible, and another part — which also ties into their rarity — is because there’s no money to be made off them. That’s the systematic flaw, where the government, big pharma, and the donors themselves don’t see any benefits for them. After all, the world runs on money. It’s a business. It’s a scheme. But it’s profoundly evil because these same governments make it next-to-impossible to get disability rights for a disease they don’t believe in — or even care about — and they also won’t fund the research to treat it. So what do they expect these people to do when they’re facing the impossible — surviving with with a disease that makes them incompatible with life? They almost can’t survive. I think our best hope is if they somehow find a treatment or cure for tinnitus, which is a way more common ailment (though in a mild form usually), and if so, it might also help hyperacusis since they’re closely linked.

But in the meantime, when trying to crack the mysteries of hyperacusis (for the few researchers there are in the world, which you can almost count on 1 or 2 hands), this is something the community needs to focus on more: the fact that it’s infinitesimally rare. Like it or not, if you’re looking at hyperacusis as an exposure-based illness, the reality is that those who have it are no different than anyone else; meaning, it only manifests when contact is made with the aggressors responsible. But I don’t believe that. It doesn’t make sense, as most people claim they didn’t do anything different than their neighbor or friend. In fact, most will say that they did far less than other people they know who abuse sound or medications routinely, or way more than they ever did. The victims just got unlucky or had some preconceived genetic component which allowed hyperacusis to fester. That’s something that the hyperacusis community ignores more than they should. They’re always so focused on noise and medications as being the culprit, and while that’s true that they do bring it on, I think they’re only able to because the person had a predisposition to hyperacusis. In other words, person 1 can be as reckless as they want and never get hyperacusis, but person 2 has the gene or whatever which makes them vulnerable. They had weak ears to begin with. That can’t be stated enough. And researchers need to hone-in on that as the numbers speak for themselves. Why is it that so many are way more abusive with their ears and never get this crap? Obviously, there’s some genetic component at work.

This modern world with louder noise and more medications is more toxic than the century before it. Perhaps 100 years ago, we would’ve been okay and never got hyperacusis. But our ears couldn’t handle these present day stressors because they were weak from conception. Nevertheless, a labyrinth of mysteries is unavoidable, as the researchers face financial obstacles, too. They’re working with pennies on the dollar; even lower than that. That’s why I always say this stuff needs huge amounts of money to truly crack; not even millions — billions. Medical breakthroughs happen when billions are thrown around. In the USA, cancer gets $26 billion a year in funding. AIDs gets $28 billion; Parkinson’s $52 billion. They have decent or good treatments for those ailments already, yet they still keep funding them solely and don’t focus on the ones that don’t have treatments yet, that get almost no funding at all. Personally, I don’t believe the world will save us from hyperacusis anytime soon. They don’t care and don’t need to. Hyperacusis is too rare. Those other ailments cited above are a lot more common. I know how this world works and it’s not pretty. I’ve seen the worst of it, even before all this, in my years working in the media with NBC. But perhaps there’s a chance for medication breakthroughs sooner rather than later, where the pain can be controlled or managed better, but a cure seems unlikely, as the prevalence of hyperacusis doesn’t warrant the urgency to develop one. It’s just the unfortunate truth.

 

It's really unfortunate, eh? I've only had it for a little over a month now and I just can't stand it. I can't stand the thought that I'll lose everything from a condition that I could have never gotten if I just treated my ears better.

I really don't know how anyone survives with this, I feel like the world would rather be blind to our suffering and pretend like we don't exist rather than fork over the funding for research that could save people's lives. It's insulting, really.

The only hope I have is that we'll start to see more and more people end up with hearing conditions due to our increasingly noisy environment. As bad as that sounds, maybe more people would pay attention to shit like this if there was a real hearing epidemic in the next decade.

 

I don't think you can find anything more common than hearing loss but still there is no cure!

There is a good chance that hearing loss cure will also cure tinnitus and hyperacusis.

But, there are still no good treatments for hearing loss. No treatments, no MF treatments!

 

Maybe. But I had an extended audiogram that went up to 20,000 Hz, covering all frequencies, and no hearing loss was detected at all, yet I have catastrophic noxacusis and severe tinnitus. I know those tests are flawed, though.

 

I know what you mean. According to my audiologist, my extended audiometry is also normal, my ABR to detect hidden hearing loss is also normal, but here I am! As you said, we don't know if the tests are accurate enough or not. Maybe we don't have any hearing loss but something in our brain has been activated due to some reason like noise exposure. Who knows what's going on! Even hearing loss doesn't necessarily cause tinnitus and hyperacusis. I know people who have hearing loss with no hyperacusis.

 

That's assuming it's only the ear apparatus (middle ear, cochlea etc) that gets damaged from loud noise. Is it possible part of the brain can be damaged from loud noise and that causes tinnitus as well?

 

Driving was very challenging, very complicated for me when hyperacusis was at its worst. It felt really uncomfortable, and the noise of trucks passing by, motorbikes, car horns, sirens etc was just unbearable and painful (pain sometimes would linger for weeks, for instance from a car behind mine honking very loud, that's why it was key to avoid the very loud sounds) even when wearing hearing protection (of course, with windows always closed).

I drove with earmuffs for some time, which I do not recommend since it is apparently illegal, and I got fined for it. I used earmuffs because the bone conduction of sound (the car wheels running on tarmac) seemed less bad than using earplugs. With earplugs I noticed more sound delivered through bone conduction. Afterwards, I started driving with earplugs.

At that time, I would very rarely drive long distances. When I did so, it seemed best to drive more slowly, as the noise inside the car would be lower. I also took frequent breaks, where I would park in a quiet place and remove my earplugs to rest my ears, so a normal 6 hour drive by car would take at least 2 or 3 hours more.

I remember parking my car on the street just in front of my house, in blazing sun, instead of leaving it at a garage space I own nearby, just because sometimes there were motorbikes running around and if I left the car at the garage, I would "run into noise" in the 5 minute walk back home... so that can give you an idea of how bad my situation was.

Forgot to mention: of course, drive very carefully to avoid being honked at, and try to strategically navigate around traffic to avoid the loudest spots (i.e. if I spotted construction on one side of the street, I would change lanes to be as far as possible from the noise source).

Driving with bad hyperacusis is very stressful.

 

Good question but no one can answer you! We don't know. Maybe. But I believe that it's brain plasticity which is changed as a result of loud noise (maybe hearing damage). Just my 2 cents.

 

And where was that? That's so fucked up. It's not illegal to drive for deaf people.

 

Spain. This country has become a tax hell, and on top of that your property can be seized by the government as they like, and one can be fined unfairly even over a debilitating health condition.

 

Thanks Juan.

Benzos (Clonazepam) completely destroyed my life beyond all recognition and I have very severe hyperacusis. I only have one permanent tone it seems, which is in my right ear, and it is definitely the most annoying.

This is a stupid question, but I'm new to this. I'm leaving Colorado soon and going to drive somewhere. I don't know where yet. I know the hyperacusis is going to wreck me. But staying here with a chance of offing myself is more dangerous than doing what I want with the pain.

If I choose to make the decision to drive a far distance, does the tinnitus have a limit? A better way of asking this is, will a drive across the state permanently increase my tinnitus if I have severe hyperacusis?

 

This is state specific in the U.S. regarding headphones (also earbuds and earplugs). It's illegal in my state to wear them over both ears while driving. There are some exceptions where use is allowed, such as one piece headsets / Bluetooth receivers.

 

You were smart for wearing earmuffs. In my case, airbags turned moderate loudness hyperacusis into the worst case of noxacusis ever. I'm surprised you got fined but that is better than making your ears worse.

 

If you had an ABR, you don't have hyperacusis or noxacusis.

 

So deaf people can't drive? I'd figure this would be a similar situation.

 

Severe hyperacusis is more rare than the death penalty, yet it’s worse. It leaves you alive to bear witness to the devastation of its wrath, and nothing can come between you and it, except pain and sorrow, and memories of a bygone time that’s nowhere to be found. It’s horrific. It robs you of your ability to do everything in life, as sound is everywhere, especially when it’s coupled with severe, reactive tinnitus. I have both, unfortunately. My quality-of-life went from 99 percent to 1 percent, and continues to dwindle. I’m scared for my life. If these conditions killed us once they got to a certain point, that’d be nature’s way of cleaning up the mess it threw upon us. But alas, we get no such luxury. Now, we’re facing a lifetime of disaster. Like, can I even do this? I’m genuinely scared. It’s horrifyingly evil.

 

I don't know if I can have an ABR test again or not because that was immediately after the onset. Anyway, you can't say so! My problem is not with loudness, the sharpness and the pitch is the problem for me.

 

I assume most adult healthy deaf people can drive.

But while driving, it would be illegal for them to be wearing a pair of headphones over both ears in this state. I don't believe there is an exception that allows deaf people to wear headphones, but I haven't read the official law. They could use a one piece headset. In some neighboring states it is legal.

 

It's very confusing considering deaf drivers are absolutely a thing, so the distinction with headsets is weird unless it's because it's actively generating noise. I ask because at least here it seems legal to drive with earmuffs which I kind of have to.

 

Sure I guess, ABR is also like 90+ dB of clicking non-stop for what, half an hour? I almost did it and the tech, who got tinnitus from the fucking test herself, convinced me to drop it.