Others with Chiari Malformation and/or Unmaskable Fluctuating Tinnitus?

[Slaw]

Sorry for the long post, but overall I'm looking for any suggestions on how to sleep with and not think about loud, fluctuating, 24/7 ringing torture that seems impossible to not think about. I'm also curious if anyone else has experience with Chiari malformation.

I had bilateral idiopathic tinnitus for 3 years, since around my college graduation. I haven't been allowed to enjoy the result of my hard work in college and becoming an independent adult, since the ringing has basically controlled my life since then, and there hasn't been an hour where I wasn't noticing it.

Then I got sudden profound hearing loss in my right ear out of nowhere in June. And the ringing somehow became more intrusive, and my world became a low-pitched static whoosh, a loud twinkling, chirping, pulsing cricket sound, and an ultra-high-pitched hiss.

It is impossible to mask or ignore regardless of the environment, and the sudden hearing loss, reactivity, dysacusis, and hyperacusis make it worse.

I feel if unmaskable tinnitus was more common (which it should be given how many go to concerts), research funding would instantly skyrocket.

I do wonder if Chiari malformation/brainstorm compression could be the cause as it showed in scans and I have other progressive symptoms related to it.

An ENT doesn't think so, though if I had never gone to any ENT I'd be in the same situation. They just tell me to mask my unmaskable tinnitus.

Has anyone had a similar experience or investigated Chiari malformation?

I feel like with hyperacusis/TTS/whatever it is, my left ear will suddenly lose hearing at any moment.

 

[Nick47]

I don't have Chiari malformation but I did this year experience a worsening. I suddenly lost further high frequency hearing. From 4,000-8,000 Hz it dropped to 80-90 dB.

What is your hearing loss frequency and decibels wise? This sometimes gives a clue to the cause.

As for causes, have you been exposed to excessive loud noise, COVID-19 or the vaccine? All are causes!

 

[Greg Sacramento]

Sorry for the long post, but overall I'm looking for any suggestions on how to sleep with and not think about loud, fluctuating, 24/7 ringing torture that seems impossible to not think about. I'm also curious if anyone else has experience with Chiari malformation.

Then I got sudden profound hearing loss in my right ear out of nowhere in June. And the ringing somehow became more intrusive, and my world became a low-pitched static whoosh, a loud twinkling, chirping, pulsing cricket sound, and an ultra-high-pitched hiss.

You mentioned this in your introduction post:

"When I breathe or move my arm I hear weird sounds like rubbing a violin. This happened during the hearing test, too - the white noise played in my good ear reflected in my deaf one."

"anyone talks there is a robotic crunch."

"It feels like a buzzing and pulsing pressure suddenly appeared in the entire right side of my head."​

I would seek out Interventional Radiology - Interventional Radiologist.

Subclavian artery stenosis - a blood pressure difference between the arms or on ultrasound testing of patients with carotid artery problems.

The pathophysiology involves blood flow diversion from the brain territories to the arm, causing symptoms of vertebrobasilar insufficiency, especially during the vigorous exercise of the arm or sudden sharp turning of the head in the direction of the affected side. The symptoms arise due to two types of mechanisms by which the arm "steals" blood flow from the vertebrobasilar territory; 1) a lack of blood supply because of subclavian artery stenosis or 2) rarely malformation disease.

Arm paresthesias, blurring of vision, syncope, vertigo, disequilibrium, ataxia, tinnitus, pulsatile tinnitus and hearing loss.

A difference of at least 15 mmHg presents between the affected armand the normal arm. Examination of bilateral carotid arteries using palpation and ausculation for detecting occusive condition. Subclavian arteries should be examined in the supraclavicular fossa using palpation.

Duplex ultrasound is one of the less invasive bedside investigations to start with if the symptoms are consistent with subclavian steal syndrome, and it is always indicated as the first-line of tests. It can readily diagnose and also quantify proximal subclavian artery stenoses. Significant subclavian artery stenosis is predicted by a subclavian artery peak systolic velocity greater than 240 cm/second. However, a Doppler ultrasound cannot properly evaluate the origin of the vertebral artery. It helps in identifying the extracranial occlusive disease.

Magnetic resonance angiography is an accurate diagnostic modality for patients with suspected SSS. It can also aid in the evaluation of intracranial cerebrovascular circulation as well as the extracranial vessels.

CT angiography is another diagnostic modality that can help in the diagnosis and grading of subclavian artery stenosis. It is indicated in patients with abnormal findings on duplex ultrasound.

A confirmatory test is usually needed to decide on the intervention strategy, and color doppler ultrasound must always be complemented with either contrast-enhanced magnetic resonance angiography or computed tomography angiography before making any decisions about treatment. Digital subtraction angiography (DSA) is generally the choice if stenting has been decided as the treatment of choice and helps better in depicting the details of the anatomical hurdle.

Besides stenting, Antiplatelet therapy and oral anticoagulation can be tried.

An echocardiogram is definitive in diagnosing aortic stenosis, but not a concern for you at 25 years of age. Need to be over 50 years of age.

Most patients (over 95%) have sustained resolution and a substantial tinnitus decrease even with profound hearing loss.

I hope you come back to Tinnitus Talk and see this post. See a Neuro Interventional Radiologist for confirmation.

 

[Steph1710]

I'm also curious if anyone else has experience with Chiari malformation.

Yes! I do! However, it is only a contributor to my pulsatile tinnitus - not my "normal tinnitus".

 

[Steph1710]

@Greg Sacramento, you're a beacon of knowledge on this subject. I am always impressed by the information you supply.

Thank you.

 

[shrimp]

Has anyone had a similar experience or investigated Chiari malformation?

I have a slight Chiari malformation, however I am not under any more investigations or care for it, it was just visible on an MRI. I do have an inkling myself it's the reason for my frequent neck pains and cervicogenic migraines/headaches I've had my entire life.

But I've also got SCDS on both ears, so that's probably the reason for my tinnitus and tendency to getting vertigo.

On top of all this I've also got a pituitary adenoma, non functioning, so it's impossible to know what symptoms are from what. My head is just a big ol' mess

It was quite a surprise after my first MRI a few years back to find there were three different issues

 

[Slaw]

You mentioned this in your introduction post:

"When I breathe or move my arm I hear weird sounds like rubbing a violin. This happened during the hearing test, too - the white noise played in my good ear reflected in my deaf one."

"anyone talks there is a robotic crunch."

"It feels like a buzzing and pulsing pressure suddenly appeared in the entire right side of my head."​

I would seek out Interventional Radiology - Interventional Radiologist.

Subclavian artery stenosis - a blood pressure difference between the arms or on ultrasound testing of patients with carotid artery problems.

The pathophysiology involves blood flow diversion from the brain territories to the arm, causing symptoms of vertebrobasilar insufficiency, especially during the vigorous exercise of the arm or sudden sharp turning of the head in the direction of the affected side. The symptoms arise due to two types of mechanisms by which the arm "steals" blood flow from the vertebrobasilar territory; 1) a lack of blood supply because of subclavian artery stenosis or 2) rarely malformation disease.

Arm paresthesias, blurring of vision, syncope, vertigo, disequilibrium, ataxia, tinnitus, pulsatile tinnitus and hearing loss.

A difference of at least 15 mmHg presents between the affected armand the normal arm. Examination of bilateral carotid arteries using palpation and ausculation for detecting occusive condition. Subclavian arteries should be examined in the supraclavicular fossa using palpation.

Duplex ultrasound is one of the less invasive bedside investigations to start with if the symptoms are consistent with subclavian steal syndrome, and it is always indicated as the first-line of tests. It can readily diagnose and also quantify proximal subclavian artery stenoses. Significant subclavian artery stenosis is predicted by a subclavian artery peak systolic velocity greater than 240 cm/second. However, a Doppler ultrasound cannot properly evaluate the origin of the vertebral artery. It helps in identifying the extracranial occlusive disease.

Magnetic resonance angiography is an accurate diagnostic modality for patients with suspected SSS. It can also aid in the evaluation of intracranial cerebrovascular circulation as well as the extracranial vessels.

CT angiography is another diagnostic modality that can help in the diagnosis and grading of subclavian artery stenosis. It is indicated in patients with abnormal findings on duplex ultrasound.

A confirmatory test is usually needed to decide on the intervention strategy, and color doppler ultrasound must always be complemented with either contrast-enhanced magnetic resonance angiography or computed tomography angiography before making any decisions about treatment. Digital subtraction angiography (DSA) is generally the choice if stenting has been decided as the treatment of choice and helps better in depicting the details of the anatomical hurdle.

Besides stenting, Antiplatelet therapy and oral anticoagulation can be tried.

An echocardiogram is definitive in diagnosing aortic stenosis, but not a concern for you at 25 years of age. Need to be over 50 years of age.

Most patients (over 95%) have sustained resolution and a substantial tinnitus decrease even with profound hearing loss.

I hope you come back to Tinnitus Talk and see this post. See a Neuro Interventional Radiologist for confirmation.

Thank you so much for the info and advice.

I actually did have an MRA with contrast and MRV (1.5T) recently, though only of the head. The MRA report mentioned:

• Tortuosity of distal cervical segments of internal carotid arteries bilaterally.
• Junctional prominence of the right side of the anterior communicating artery junction with right anterior cerebral artery, triangular configuration may represent infundibular origin, without definite saccular aneurysm visualized (stable since 2015 MRA).
• Right mastoid mucosal disease

I'm not sure how to interpret it, but I will be going to a neuro institute this month so perhaps I can also get a session with an neuro-interventional radiologist. I also am trying to get more blood tests (had positive ANA and had Lyme in 2015) just in case.

My extra symptoms vary day to day though I'd be ok with everything else if the catastrophic tinnitus would subside as everyone says it should.

Oscillopsia during exercise, nausea, numb tingling foot, new sin wave tinnitus, static shocks in left ear upon touching it, left ear fluttering in response to any noise.

 

[Slaw]

I have a slight Chiari malformation, however I am not under any more investigations or care for it, it was just visible on an MRI. I do have an inkling myself it's the reason for my frequent neck pains and cervicogenic migraines/headaches I've had my entire life.

But I've also got SCDS on both ears, so that's probably the reason for my tinnitus and tendency to getting vertigo.

On top of all this I've also got a pituitary adenoma, non functioning, so it's impossible to know what symptoms are from what. My head is just a big ol' mess

It was quite a surprise after my first MRI a few years back to find there were three different issues

@shrimp, would you mind sharing how you knew it was SCDS?

I'm not sure if it shows on MRI, but the ENT refuses to do a temporal bone CT because my hearing loss is "nearly all sensorineural". I think I have the symptoms like oscillopsia and sensitivity.

 

[shrimp]

@shrimp, would you mind sharing how you knew it was SCDS?

I'm not sure if it shows on MRI, but the ENT refuses to do a temporal bone CT because my hearing loss is "nearly all sensorineural". I think I have the symptoms like oscillopsia and sensitivity.

I didn't actually suffer any hearing loss, most with SCDS have it but not all of us. For me it started with vertigo. I was hit with a bout of vertigo that lasted more than 3 months straight. At first they thought I had BPPV and tried Epley manoeuvres etc. I couldn't walk straight and it was just pure horror with 24/7 vertigo, sometimes when lying in bed I would feel like I was falling out of the bed even though I was lying completely still.

Since it didn't clear up for so many months which is unusual, an ENT did an MRI with contrast and on the results it says I have thinning, possibly openings on both sides on my superior canals. I know it doesn't always show on MRIs, I'm in a few SCDS support groups and some have multiple scans of different kinds before it's found.

I also have bone conducting noises which is typical. As well as pulsatile tinnitus on one ear depending on how my neck is positioned (the one that doesn't have normal tinnitus of course, let's mess things up as much as possible) and when I do stuff with my right hand like stirring my coffee cup or opening a bag of something, knitting etc. I hear loud thuds in my head/inner ear and I can hear my joints moving if the room is silent. It's quite unnerving, but I'm glad I don't have the thudding from walking or just blinking like some get (yet).

I'm not under any care for it for now, since operations are only performed for those that are almost totally crippled by the condition (and are super risky) and there isn't much else they can do.

 

[Greg Sacramento]

Hi @Slaw, tortuosity of distal cervical segments of internal carotid arteries bilaterally relates to what I described.

Infundibular origin without definite saccular aneurysm is very common and often is shown on radiological exams.

Both right mastoid mucosal disease and tortuosity of distal cervical segments of internal carotid arteries bilaterally can cause tinnitus.

Something at your young age is causing either vertebrobasilar, cerebellar artery, internal carotid arteries blood flow insufficiency. Direct issues from blood pressure hypertension - most likely not the single contributing source factor at your age.

Oscillopsia during exercise, nausea, numb tingling foot and arm relates to my other post, this post and all our disscussions.

Distal cervical segments of internal carotid arteries bilaterally can cause tinnitus.

With your all your symptoms, including mastoid mucosal disease, medical history and MRA notiations - high blood sugar, but maybe not diabetes is the most common noted association cause for all your combined medical time events.

Lyme disease can also cause tinnitus. Lyme disease can cause high blood sugar - very much so.

Is it as simple as this - maybe not - and high blood sugar and/or Lyme may not have postulation.

Please stay in touch.

 

[Slaw]

I had positive ANA so I found a rheumatologist and took another blood test (demanded Lyme PCR) and I'm waiting for results. The rheumatologist mentioned Cogan's which I was aware of. However, steroids did nothing to help hearing loss or the increased screaming.

I got a referral to an otologist (not sure if they will say anything besides "mask it even though it's unmaskable.")

My 2015 MRA looks consistent with 2022 but the tinnitus started in 2019 (both ears, same time) so I'm curious how that works if the arteries are a reason.

I'm not sure who to ask for an ultrasound Doppler test of my neck/head, I guess the Neurosurgeon. They might order a CSF flow scan. I'll ask for highest resolution in case it can show vascular compression issues.

Is there a certain diabetes test blood tests don't cover? It's hard to find exact information. I'm not confident glucose/etc has been tested lately.

Any of these possible reasons don't seem to be reversible. I cannot stop being aware of the piercing screaming and chiming, especially in the severe loss ear.

 

[Greg Sacramento]

I had positive ANA so I found a rheumatologist and took another blood test

The other blood test may be a titer.

Seeing a Rheumatologist and an Otologist is wise.

Rheumatoid arthritis may surely be one problem, but there could be a causative factor.

Hearing impairment in rheumatoid arthritis can be a multifactorial condition.

Hearing loss can be treated with rheumatoid arthritis - there are several medications including Prednisone.

Place any rheumatoid arthritis medication into internet search alongside of hearing loss and tinnitus. Some rheumatoid arthritis medications do cause hearing loss and tinnitus, while others don't.

 

[Slaw]

@Greg Sacramento, I got blood test results and they are hard to interpret, but the doctor said lupus is possible (though I have no apparent liver/kidney/rash issues) but vasculitis seems most likely due to detection of P-ANCA and C-ANCA.

I met with a neuro and they said the MRV shows stenosis on both sides, which could lead to Chiari, and referred me to an interventional neuroradiologist (I see that person has a 2nd opinion form on their page, so unless it's new I'm kicking myself for not seeing that months ago).

I had summarized my history and how when I lie down, the visual snow and tinnitus somehow get worse.

I'm curious how stenosis can trigger bilateral tinnitus, though, especially pulsatile. My tinnitus is occasionally clearly pulsing, esp. on right side, but I'm not sure.

It seems to take a long time to get any appt or scan, which is frustrating.

 

[Greg Sacramento]

I met with a neuro and they said the MRV shows stenosis on both sides, which could lead to Chiari

Yes, it could lead to Chiari, but often need to show signs of fibromyalgia first.

I considered over 100 conditions and diseases. I don't think it is Lupus, but many blood tests are needed.

What is your blood pressure and heart rate?

Does your pulsatile tinnitus sound like a marching band?

What are your white blood cell called eosinophils results?

Protein in the urine can also be detected on urine dipstick, and can sometimes make the urine look foamy or bubbly. Red blood cells and protein can leak into the urine. Sometimes blood can be seen in the urine, making it look brown like tea or Coke, but sometimes it can be seen on a urine dipstick.

I'm curious how stenosis can trigger bilateral tinnitus, though, especially pulsatile

Fast heart rate, hypertension blood pressure - sometimes from a brief hypertension crisis, visual snow, abdominal pain, aortic valve not always opening (with this, pulsatile tinnitus is not always present), aortic stenosis and protein in blood are all markers. Takayasu's arteritis (but carotid arteries may be non-problematic).

Your MRV would had noted other stenosis causes.