CIST and SSHL

Today I was woken up at 5AM by incredibly loud ringing in my right ear and headache. It’s weird that ringing can be so loud it wakes me up from sleep, even when I know it’s just in my head. It’s not a real sound.

It’s been there all day (now 4pm). Around 900kHz and again at 1800kHz. What does that mean?I’m not sure. Is this a side effect of hearing improving or reducing. I have no idea. But My ear is again extremely sensitive today so I’ll wait out the day and see what tomorrow brings.

I have absolutely no hearing of notes between D6 (1175Hz) and A6 (1790). Anything between around 1100 and 1800 I just hear it as one tone at 1800. And if I try to turn it up in my right ear enough to hear it, I only hear it in my left ear.

This is the deafest that ear has been since this started. I can’t listen to music at all as I hear it in the wrong ear.

I am trying the best I can do with the CIST, listening of a bit of music trying the frequency training, but it’s hard.

I have been doing my new strict CIST Protocol for 3 days.

Well mostly.

I managed 8 hrs audio the first day but 6 the following 2 days.

And I’ve forgotten to do the ‘change habits’ and I still haven’t worked out what positive emotions means exactly, but working on it!

Here’s my hearing graph.

6 Jan was day 1 of new strict CIST Protocol. A very gradual increase since then…

To put things into perspective, here is my complete graph since day 1 of SSHL.

So I am a long way down but at least the direction is upwards once again!

It’s a new year and I’ve decided to really tackle this hearing thing. I’ve been reading a lot and today I wrote 2 papers which I will post on the front page.

One is about the Stages of SSHL to give me language to use, the other is is called CIST Protocol and I’ll be sticking to this as closely as I can in the New Year!!

Today my hearing has improved ever so slightly. Midnight Oil and Cold Chisel still sound off. Stryper is kind of OK. Crowded house is good.

When my hearing is bad it’s hard to find any music to listen to, it all sounds off.

Some is worse than others. it may have something to do with the frequencies in the music, but I think it’s got more to do with how deep in my brain it is. Stick with me!!

I thought I would really enjoy Sons of Korah, but it’s way off. I’ve only known of them for 15 years.

Midnight oil is OK. Crowded house too. And Stryper. they are all bands I listened to in the 80’s.

But since before Christmas when my hearing crashed nothing sounds any good. So yesterday I tried something from my childhood. Yes Spotify actually had the Sesame St Ernie and Bert sing along. And yes, it actually sounded OK. ‘Old Mc Donald had a Farm’ etc

My guess, it’s from when I was 5-10 years old, it’s so deep in my brain it can’t hear it wrong.

I have listened to U2 ‘Where the streets have no name’ many more times. But even that sounds a bit off.

So if you find me listening to Harry Belafonte ‘Banana Boat’ don’t panic. I’m just having a bad hearing day.

Today I had the slightest increase in hearing. I can’t perceive it but the graph is slightly up.

It looks like the latest up and down wobble bit is over, which is nice because the wobble bit after a crash seems to be when my ear is most painful.

It feels like the pre Christmas crash from Dec 15 is finally over and I am on the mend again. If so, that took 2 weeks.

My ears are not ringing for the first time in 10 days which is nice!

And I can almost hear the pure tone in a tone sweep all the way from 30Hz to 1kHz. There’s just a tiny place around 800Hz where I can’t make out the tone at all.

Bono sounds almost OK but Crowded House not so good.

In 1 week I will have had SSHL for 6 months. I had no idea that my hearing 6 months would be just as bad as the day it all started. I had no idea that a month ago my hearing would be almost back to normal and then drop again.

All the literature and my own ENT specialist talk about improving, but none of them talk about going backwards.

So the last couple of days I wrote this summary of my thinking and the way my understanding has grown about this problem that I have.

 ”A new approach to SSHL needed? (pdf)

Here’s the text of the article:

Brain Plasticity and SSHL – a new approach needed.

Wayne Connor Dec 2023

Brain Plasticity and SSHL – a new approach needed.

Wayne Connor Dec 2023

I have had Sudden Sensorineural Hearing Loss (SSHL) for just under 6 months. SSHL is defined as greater than 30dB of hearing loss in greater than 3 frequencies in under 3 days. It’s a sudden deafness in one ear. 

The longer it goes on the more I’m realising there’s a problem in the way the medical world approaches this condition. Currently all kinds of hearing disorders are lumped together under the heading of ‘SSHL’.  

The cause can be anything from a viral infection (including COVID) to a blood flow problem, or trauma, autoimmune disorders, stress, or the side effect of other medications.  Moreover the location of the cause of the SSHL can vary: the cochlea hair cells, or the nerves that connect the cochlea to the brain, or the brain itself, including the higher level auditory cortex.  Not to mention each of those areas can be broken down into more detail.

One audiologist may say ‘be careful with loud noises’ because if the damage is to the cochlea hairs, of course you want to protect them from further damage. But if the problem is that the auditory cortex is confused, it may actually need stimulation to repair itself.  So the generic term SSHL at that point is not helpful.  

Diagnosing someone with SSHL is about as useful as saying ‘your car won’t start’. One car might be fixed with a new battery. Another by changing the spark plugs, and another by filling the car with fuel.  Sure, the WD40 might help wherever you spray it, but after you’ve sprayed it everywhere, what next?

The standard treatment (the WD40 of SSHL) is steroids. Prednisolone apparently targets all the areas potentially involved in SSHL. It can assist if the hair cells are damaged, reduce inflammation anywhere along the pathway from the chochlea to the brain, and it suppress the immune system which may be damaging the hair cells or nerves. But what if the steroids don’t work, or only partially restore the hearing. Here lies the big problem, with such a broad diagnosis (SSHL) how can you begin to treat it? 

In Australia the problem is exacerbated by the specialising of doctors into areas. So there may be an Ear, Nose and Throat specialist, an audiologist, or a neurologist, but who of those best deals with SSHL? It’s like my car won’t start, but do I go to an auto electrician, or a carburettor specialist?

As my SSHL has progressed I suspect it’s become, or always was, neurological. By that I mean the problem has moved from the inner ear (if it as ever there, but I suspect that it was) to my brain.

If you take a look at my hearing graph on my blog, it is very erratic. My ENT said SSHL doesn’t go backwards, and my audiologist said my hearing shouldn’t swing so far daily like that.  Which leads me to conclude that perhaps it’s the brain causing the problem, not the ear. 

The most recent theories out there are that when the brain stops receiving signals from one ear, it re-wires itself somewhere in the signal chain to listen to the other ear – your good ear. The signals from each ear travel into the brain and are combined at several points, in the brain stem but finally in the auditory cortex. The way this happens is very complex. When your brain receives no input from one ear, it can re-wire itself to stop listening to the bad ear. Studies show this happening within 72 hours of hearing loss.  When 3 or 4 days later, your ear heals, it’s possible that your brain is so re-wired that it’s stopped listening to that ear. It’s like being put on hold on a phone for so long that you hang up.  The brain has ‘hung up’ on your ear. 

My suspicion is this is why they say that SSHL is a medical emergency. It needs to be treated in the first 72 hours. It’s possible that the physical condition needs treatment in the first 72 hours. But it’s also possible that 72 hours is how long it takes the brain to re-wire itself. So the 72 hour window might be critical to fix the physical condition before the brain re-wires. This explains why some people can regain hearing after 72 hours. The brain can re-wire itself. It’s just harder. It also explains why the more severe the hearing loss, the more likely it is to be permanent. If the loss of hearing in the deaf ear is greater than the cross-hearing (50dB), then any sound into the bad ear will in reinforce the ‘bad’ wiring. 

One proposed treatment for this is called CIST.  To encourage the brain to re-wire itself back again you stimulate the bad ear with music.

The idea was introduced in 2014

“Constraint-induced sound therapy for sudden sensorineural hearing loss – behavioral and neurophysiological outcomes” 

Okamoto,Fukushima et al. 2014

This paper from 2016 explains it in more detail:

“Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss”

Sekiya, Fukushima

These papers found no improvement from CIST:

Neuroplastic Effect of Constraint-Induced Music Therapy on Hearing Recovery in Patients with Sudden Sensorineural Hearing Loss. (2019)

Effect of Constraint-Induced Music Therapy in Idiopathic Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-Analysis (2022)

These studies found improvements using CIST:

Effect of Combining Sound Therapy with Pharmacotherapy on the Recovery of Hearing Abilities in the Case of Sudden Sensorineural Hearing Loss: A Prospective Study (2022)

Sound therapy in sudden deafness (in Spanish) M.A. López-González (2017)

So it’s a bit hard to know is CIST helps, but it’s certain there is a brain plasticity issue.

What is CIST? (Constraint-induced sound therapy)

CIST is a therapy that combines standard medical treatment (like corticosteroids) with auditory stimulation. The idea is to force the brain to pay attention to the affected ear by plugging the healthy ear and listening to music or other sounds through headphones on the deaf ear for several hours a day. This increased stimulation is believed to promote neuroplasticity, the brain’s ability to reorganize and adapt, which could help to recover hearing and prevent auditory deprivation.

“Scientists are testing a new way to treat sudden hearing loss. It’s like how they help stroke patients regain movement: by forcing the brain to use the affected part. Here’s how it works for hearing:

1. Plug the good ear. This makes the brain focus on the ear that’s not working well.
2. Listen to music in the bad ear for 6 hours a day. This gives the brain lots of sound to work with, kind of like exercising the ear.

The goal is to:

• Stop the brain from giving up on the bad ear. When hearing loss happens, the brain can start to ignore the bad ear and focus on the good one. This new therapy tries to prevent that.
• Help the brain rebuild connections to the bad ear. This could help people hear better again.

[Constraint-induced sound therapy for sudden sensorineural hearing loss – behavioural and neurophysiological outcomes 2014]

The following articles explain all this in a bit more depth:  I’ve used Google Bard to help summarise some of these papers. T

Brain rewiring in SSHL

Normally, sound triggers stronger brain activity in the opposite side of the brain. When one ear loses hearing, this still happens for the deaf ear. But for the good ear, the brain acts differently: it relies more on the same side instead of the opposite.

 [“Healthy-side dominance of cortical neuromagnetic responses in sudden hearing loss” (An-Suey Shiao)]

Brain rewiring in SSHL

The brain reorganises itself after hearing loss in order to compensate for the loss of hearing. The ipsilateral dominance observed in this study is a new finding and could have implications for understanding and treating hearing loss.

 [“Healthy-side dominance of middle- and long-latency neuromagnetic fields in idiopathic sudden sensorineural hearing loss “(2006)]

Higher level brain rewiring in SSHL

Sudden hearing loss can affect the brain, not just the ears. The brain may try to adapt by changing its activity patterns. More research is needed to understand these changes and develop better treatments.   

“The advancement of imaging technology has revealed that individuals  with SSHL exhibit not only alterations in brain cortical and subcortical  structures but also  associated with changes in brain functional activities involving various  brain networks such as the default mode, auditory, executive control, and visual networks.”

[Altered static and dynamic intrinsic brain activity in unilateral sudden sensorineural hearing loss – August 2023 Frontiers in Neuroscience 17]

SSHL and the Auditory Cortex

“SSHL affects neural activity not only in the cochlea, but also in the auditory cortex.  Several neuroimaging studies suggested that SSHL induced cortical plasticity in the human auditory cortex within a few days of its onset. Moreover, the degree of cortical reorganization represented by the loss of contralateral hemispheric dominancy of the auditory evoked fields appeared to negatively correlate with recovery rates from hearing loss.  The cortical plasticity induced by the onset of SSHL may be considered as maladaptive for the hearing capability of the affected ear. Therefore, the prevention of this maladaptive cortical reorganization associated with SSHL may represent a new treatment strategy.”

 [Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss 2016]

Brain learns not to hear.

Maladaptive cortical reorganization refers to changes in the auditory cortex that can occur after SSHL. These changes are thought to be harmful and may contribute to the difficulties some people experience in recovering their hearing. The exact cause of maladaptive cortical reorganization is unknown, but it is thought to be related to changes in brain activity after the inner ear is damaged. Maladaptive cortical reorganization can lead to the “learned non-use” of the affected ear, meaning the brain stops paying attention to sounds coming from that ear. This can make it harder to recover hearing and can also lead to tinnitus.

[Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss January 2016 Journal of Visualized Experiments 2016(107)]

Can we reversing the Brian re-wiring?

Cortical structures in the adult human brain were previously considered to be non-elastic; however, recent studies have revealed that the adult brain can be reorganized based on its surrounding environment and behavior. Reorganization in the human auditory cortex generally occurs in a direction that is preferable for fulfilling demands; however, it may also induce pathological phenomena in the auditory system such as tinnitus. Therefore, it appears to be important to develop suitable neurorehabilitation approaches to treat maladaptive reorganization in the auditory system. 

 [“Investigating the maladaptive cortical reorganization in the human auditory cortex using magnetoencephalography”  2020]

Spanish Trial

The study by López-González et al. (2017) investigated the efficacy of a new treatment for sudden sensorineural hearing loss (SSNHL). The treatment consisted of acoustic stimulation with sounds delivered through headphones for one month. The results showed that the treatment group had a significantly better hearing recovery than the control group. At the end of the study, the mean hearing improvement in the treatment group was 20 decibels (dB), compared to 4 dB in the control group. The authors concluded that acoustic stimulation is an effective treatment for SSNHL. The treatment is especially effective when it is started early.

Summary in English of the study by M.A. López-González et al. (2017)

Google Bard can you give me a simple explanation of ‘Maladaptive cortical reorganization’ (Note this is just a summary of the information available to Google Bard, it’s not intelligent at all)

Maladaptive cortical reorganization is a fancy way of saying that the brain rewires itself in a way that actually causes problems rather than helping. 

Imagine your brain is like a city, and the streets are connections between different parts. When you suddenly lose hearing, it’s like a major road closure – the sounds that used to travel to your auditory area (the hearing neighborhood) can’t get through anymore. Now, the city doesn’t like empty streets, so it starts rerouting things.  When the ear tries to send a signal again, it hits a traffic jam of a dead end. 

This rewiring can also lead to problems like:

• Phantom sounds: Hearing sounds that aren’t actually there, like ringing or buzzing in the ears (tinnitus).
• Speech in noise problems: Having difficulty understanding speech in noisy environments.

Here are some things that can be done to help prevent or reverse maladaptive cortical reorganization:

• Early intervention: The sooner you start treatment for hearing loss, the less likely it is that your brain will have time to rewire itself in a maladaptive way.
• Sound therapy: Listening to sounds through the affected ear, like music or white noise, can help to keep the auditory cortex active and prevent it from being taken over by other sensory areas.
• Brain training: There are some new brain-training programs that are being developed to help people with hearing loss learn to use their brains more effectively to process sound.

No I didn’t do the mowing! But we had the mower going yesterday to mow the lawns and I was around (with headphones on) doing gardening.

I woke up this morning with bad ringing and finding it hard to hear. I just did my hearing test which confirmed my suspicions. After 3 good days I’ve gone almost deaf again! So what happened? Yesterday morning we did some gardening and the kids were using the lawn mower and whipper snipper.

I had headphones on but I could still hear the mower sounds mildly.

My hearing was pretty good so I had lunch and dinner with the family.

Last night we were up at the church setting up some fairy lights.

So that included a car trip, and there were some conversation going on while we were setting up the lights.

On reflecting, that was a big day for my ears. Too big. I need to be more careful when my ears are good to prevent them crashing again.

This means today I’ll need to be by myself, eat by myself, and try not to have any conversations.

As you can see from this graph, I’ve had a very bad week with my hearing. The worst it’s ever been.

For the last 5 days Bono sounds like a Dalek, Cold Chisel sound out of tune, Midnight Oil is missing all it’s melody, and Crowded House sound like they are underwater. 

But today Bono’s voice is back to normal! So things are looking up.

Well the graph says it all. My new plan started day 96. (The borrow left of the nice looking mound on the right of the graph.) I decided to be very careful about my hearing. Listening to music 4-6 hours a day. Limiting myself to 1 hour of conversation a day. It seemed to be working very well.

On 30th November (Day 119) about the peak of that nice smooth hill I went for my 3 monthly checkup to the ENT.

Needless to say he was very happy with my progress.

I had 3 questions for him.

1. How can I stop my hearing from dropping again?
2. How careful should I be about noisy situations?
3. What could be causing my hearing to drop?

I guess I was most interested in questions 3. In once sense I know what causes it to drop – going to a meeting, going to church, going to a coffee shop.

But what about those situations causes my hearing to drop?

Is it the multiple conversations?

Is it my talking?

Is it the background noise?

Is it just the noise level?

Why does a conversation cause my hearing to drop but listening to music doesn’t?

The ENT had no answers at all. He was sceptical of my graph. He said with what I have, my hearing doesn’t go backwards! My hearing was now better. I didn’t need to be careful at all.

This sounds strange, but I wanted to believe his words. So over the next couple of weeks I started being less cautious, having more conversations. Tentatively at first, but it gained momentum. I wore my headphones less, and I stopped listing to music as much. I tried to live more ‘normally’.

It was hard to see at the time, but looking back on the graph now it’s obvious. After my visit to the ENT my hearing went from improving to declining. The curve tells the story. It’s like I was coming down a hill I’d just climbed. But I failed to notice it at the time.

The big crash came on day 135-138.

On day 133 I had a practice for our church carols. (This is where the hill turns into a cliff on the graph at the top right,) I think in hindsight if this was all that I did my hearing may have bounced back. But this was followed up on day 135 with a wedding. I was feeling confident after what the ENT had told me and was ‘pushing’ things to see if my ear would hold out.

On day 136 I could tell things were not good. My ear was feeling bad and blocked again. But the damage was done. And it takes a day or two for the effect to appear. The next 2 days I stayed at home to give my ear some rest, but it continued to crash.

And now it’s as bad as it ever was. My ear is in pain. It’s hard to describe how aggravating the ear is. I can’t be in a conversation again or it hurts. I feel like I want to poke a pencil through my eardrum to stop the ringing. (Only I know that won’t stop it!) The ringing is so loud I can’t sleep. If you have SSHL you’ll know what I mean.

I am looking back at my graph thinking why did I do this?

Why did I listen to the specialist when I had a gut reaction he was wrong?

I don’t think the ENT has much of an idea of what’s going on. But also a professional can’t trust a person’s home measurements. If he did he’d be in trouble. He can only go on the professional hearing test results. And they didn’t show my hearing drops as the tests were done when I was at the top of the hill.

I’m going to have to put in a bit more work reading up on how to manage this!