Vital Signs: An Unwelcome Ringing — Magazine Article About How Doctors Cured a Guy's Ear Pain Spasms

This might mean the spasms are the pain itself. A microscope and ear speculum was used to see his ear drum twitching and to see which muscle it was.

''The Boston doctor made a small opening in Stephen’s left eardrum and inserted a microscopic telescope attached to a video camera to visualize the middle ear muscles. All of this was done under topical anesthesia while Stephen was awake. The results matched my diagnosis but added an important new piece of information: The offending muscle was the tensor tympani muscle, not the stapedius.''​

This is probably ear spasm, a certain kind of ear pain, and there's a chance- hopefully for me- that it's the same pain that happens with those of us that have startle sound sensitivity and those of you who might think you have standard hyperacusis when this is what you really have.

Vital Signs An Unwelcome Ringing

The young man’s ear pain was constant and debilitating, but doctor after doctor could not find the cure.

by Christopher Linstrom

From the April 2010 issue; published online June 10, 2010

By the time Stephen came to my office complaining of painful spasms and ringing in both ears, he had already been to several physicians about his problem. He had been given an array of diagnoses; most doctors said he had acute outer or middle ear infections and prescribed oral antibiotics or ear drops. These measures had not helped, though. He was 21 years old and had suffered with this pain for nearly a year.

Like many people with ear or head sounds—which we lump into one word, tinnitus—Stephen was distraught. Knowing that there is no magic drug for ringing, I mentally prepared my usual sermonette: Avoid noise, aspirin, and methylxanthines (stimulants of the central nervous system, such as caffeine). And embrace the tried-and-true helper, sound substitution, or “masking.” This technique, in my experience, is a remedy for almost every patient with tinnitus.

Then Stephen delivered the bombshell. “I’ve been treated twice with extended courses of intravenous antibiotics for Lyme disease,” he told me. “In fact, I have an intravenous line in my arm right now and am just finishing an eight-week course of Rocephin [an antibiotic] for my second bout of Lyme. I’ve had to drop out of college because the pulses in my ears are so bad I can’t concentrate on anything. Could it be related to Lyme?” The spasms were occurring every few seconds in both of his ears throughout his entire waking day, he continued. The associated pain was so severe that his whole life revolved around dealing with it. Clearly this was not the typical case of tinnitus.

Stephen was first exposed to Lyme disease, a tickborne infection, as a child while playing in the woods near his home in Westchester County, New York. He was reinfected as a college student in 2007. Severe cases of Lyme disease can involve cardiac or neurological symptoms and are often treated with the intravenous antibiotic ceftriaxone. But I was unaware of any link between Lyme disease and pulsatile tinnitus. Otherwise, Stephen was in stable health with no other contributory medical or surgical problems.

I examined his right ear with a microscope and an ear speculum. There it was, apparent right away in an otherwise normal eardrum: The drum pulsed and appeared to go into spasm in an irregular way. I observed the exact same thing in the left ear. There was no mass behind either drum, and the middle ear clefts on each side were normal. My exam did find spasms of his soft palate (palatal myoclonus) and twitching around his eyes (blepharospasm), but these were not as bothersome to him as the pulsing. I had never before seen a patient like this.

It was time to put Stephen through some proper hearing tests to find out what was going on. The middle ear has two muscles, the tensor tympani and the stapedius. The stapedius and, to a lesser extent, the tensor tympani engage in the acoustic reflex (or the middle ear muscle reflex), a contraction in response to loud sounds. A reflex is an involuntary motor response, so it is present both while a person is awake and while unconscious. All reflexes follow a looping nerve response: an arc toward the central nervous system, called the afferent limb, and an arc away from the central nervous system to the end organ, termed the efferent limb. For the middle ear muscle reflex, the afferent limb is the auditory nerve; the efferent limb is the facial nerve for the stapedius muscle and the trigeminal nerve for the tensor tympani muscle. The reflex itself occurs in the brain stem, one of the deepest and most basic parts of the brain.

The stapedius responds by tightening the mobility of the stapes (or “stirrup”), and the tensor tympani tightens the eardrum and pulls the malleus (“hammer”). The stapes and malleus are tiny bones that transmit hearing through the inner ear. By restricting the motion of the stapes, the stapedius may be able to dampen loud sounds coming toward the ear, but that response does not happen quickly enough to prevent acoustic trauma from fast-acting sounds, such as a gun blast. The acoustic reflex is also thought to play a role in preventing us from hearing our own voices as we speak. Although we know that the reflex is present in healthy individuals and we evaluate it as part of every complete hearing test, its ultimate function is not entirely understood.

To investigate Stephen’s ear spasms, I ordered a complete hearing test. Any neurological process involving two or more cranial nerves almost always points the finger of diagnostic suspicion to the brain stem, which is the anatomical takeoff point of the cranial nerves. So I also ordered an MRI of my patient’s brain, with and without contrast enhancement, to get a good look at that area.

The tests showed that Stephen’s hearing was completely normal, but the acoustic reflexes could not be tested because of the ongoing spasms in each ear. The MRI of his brain was entirely normal as well. At this point, my most likely diagnosis was myoclonus (muscle spasm) of either or both the stapedius and tensor tympani muscles. The painful eardrum spasms were the result of the contractions of one or both of the middle ear muscles.

All physicians are trained to begin by thinking broadly and then apply reasonable medical and surgical measures to the most likely cause of the clinical problem. Physicians are also trained to treat medically first and to reserve surgery for cases that fail medical management. Because Stephen had already been treated for Lyme with intravenous antibiotics, I suggested a consultation with a neurologist and perhaps a short trial of common medications used for either seizure or peripheral neuropathy, such as tegretol or gabapentin. Tegretol decreases the spread of seizure; it is also used to treat bipolar disorder and trigeminal neuralgia, a condition causing severe facial pain. Gabapentin is a molecule related to gamma-aminobutyric acid, or GABA, a common neurotransmitter. It has well-studied analgesic and anticonvulsant effects, although its exact mechanism of action is unknown. Gabapentin is used for a variety of neurological disorders, including spasm and extremity pain.

Stephen began with a short course of tegretol, prescribed by his neurologist. It did not lessen the symptoms or block the spasms, so I started Stephen on gabapentin. After a few weeks, he reported that it had no beneficial effect either.

Understandably anxious, Stephen had sought a few other consultations, with conflicting recommendations. I suggested he see a colleague of mine in Boston who had a great deal of experience with eustachian tube dysfunction. The Boston doctor made a small opening in Stephen’s left eardrum and inserted a microscopic telescope attached to a video camera to visualize the middle ear muscles. All of this was done under topical anesthesia while Stephen was awake. The results matched my diagnosis but added an important new piece of information: The offending muscle was the tensor tympani muscle, not the stapedius.

Now that we had identified the culprit, how to treat it? There were two possibilities: injecting a neuromuscular-blocking medication, such as botulinum toxin (Botox), into the tensor tympani muscle in the middle ear, or cutting the muscle surgically. The former would be a temporary trial that would wear off in about 6 to 12 weeks. The second option would be permanent. There were potential risks associated with either choice. First, either temporarily or permanently blocking the muscle might not solve the problem. Second, loud sounds coming toward the treated ear might seem even louder than before. Stephen considered his options and, after speaking with his parents, decided to have the muscle cut. He had been suffering with the spasms for so long that he did not want to try a temporary solution.

I started with the left ear because it was the more bothersome. The surgery was all done through the outer ear channel; it involved making small incisions around the eardrum so it could be turned aside like the page of a book. The eardrum was then dissected off the hammer bone to allow me to see the tensor tympani muscle as it approaches the hammer bone at its narrow neck. The nerve to Stephen’s face runs in a bony channel near the tensor tympani muscle. We monitored his face with a electromyography system, which allowed me to electrically stimulate the facial nerve and make sure it was not being injured by the surgery. I then found and divided the tensor tympani muscle, first partially with a small knife and then completely with a scalpel-like laser. I placed a small amount of packing under the eardrum, turned it back into its usual place, put some packing material over the eardrum, and bandaged the ear. Stephen was awakened and transferred to the recovery room.

About an hour later, my patient was fully awake and ready to go home. I went to see him in the patient recovery area and he exclaimed, “It’s gone! I think it’s really gone!” His parents were as overjoyed as he was. It appeared that his long nightmare might finally be ending. Fast-forward several weeks: The surgery did in fact abolish the spasm in Stephen’s left ear. His hearing remained normal, and the spasms around his left eye subsided.
About six weeks after the left side, I operated on the right ear in a similar fashion. This side also healed, and the spasms went away with no significant side effects. Today Stephen has resumed his university studies and is getting his life back on track.

I still had not addressed Stephen’s original question: Was Lyme disease responsible for his ear agonies? I would say yes. The disease certainly has many far-reaching neurological symptoms. As Sir William Osler, regarded by many as the father of modern medicine, said many years ago, “He who knows syphilis knows medicine.” Of course, syphilis was not Stephen’s problem, but the cause of Lyme disease, Borrelia burgdorferi, is in the same family as the causative agent of syphilis, Treponema pallidum. They are both spirochetes, bacteria that cause an insidious range of health issues.

For me, Stephen’s case reinforced the notion that the medical history, as detailed by the patient himself or herself, is the most important part of any medical or surgical encounter. Patients who have difficult medical conditions are usually good historians: They live with the problem all day, every day, and can reflect upon its origins and its clinical course. The patient almost always provides all of the diagnostic clues to the solution, guiding the physician in where to look and how to treat.

Christopher Linstrom is an otologist/neurotologist and professor of otolaryngology at the New York Eye and Ear Infirmary. The cases described in Vital Signs are real, but names and certain details have been changed.​

taken from

http://discovermagazine.com/2010/apr/15-vital-signs/?searchterm=myoclonus

 

Great sleuthing.

 

The real problem is, there are no such doctors. Nobody will deal with Tinnitus in such way. There is no doctor in my country (and I imagine many more) that will treat such a condition. They may know it exists, but they will just do what they always do: Suggest there is nothing to be done, give a TRT suggestion or something like that and leave the patient in agony... They simply do not do their job because there is a simpler case following the tinnitus one, (like a flu) and choose to discard with the T patient. It is as simple as that. They deal with us as we are not human beings.

Are we to imagine that this case was the only one that the T was not a normal hair cell-suggested unsolvable problem? Or that T is in fact caused by hearing loss and not some somatic condition such as this? There are other conditions too, vascular compression, neck problems etc. Have they examined anything? No. But they do have the nerve to tell the patient there are these cases too! And when the patent asks to be examined further (like with with a stethoscope) they do not proceed!

So sad indeed...

 

These doctors do exist. They are always fully booked and do not take new patients though.

 

U are right, there are numerous cases of t. that are not caused by hearing loss. I believe more 50%, but there are no doctors that want (or have a knowledge) to deal with it.

 

Do you have personal experience? How can somebody now that one is a real doctor and not an imitation? How many doctors do you have to pay until you find one that really wants to help and is able to?

 

A good doctor will try to get every bit of info out of you that may be relevant. Doctors patients may not be telling everything. A good Doctor is curious and inquisitive about the patient's symptoms.

It's also important to manage expectations as well. We all know there is no cure. The doctor will not generate an answer to your tinnitus on the spot. What the doctor can do though, is try to find out the physical cause of the problem, like it was presented in this thread's article.

If you have tinnitus as a result of acoustic exposure then trying to answers with doctors is unfortunately beating a dead horse as far as getting rid the noise goes.

 

And how can a doctor find out positively that the sound exposure is responsible and not other abnormalities like for example microvascular compression? And what happens if you have both? Shouldn't one try to fix what can be fixed?
A good doctor is a doctor that does what can be done to cure your problem. Who is to tell they tried everything when in fact no doctor rules out for example objective tinnitus? Which is treatable and detectable. IF they look for it!

 

I just haven't met one yet.

 

If you have both then the obvious thing to try is to fix what can be fixed. The doctor should be made aware of the whole story in a consultation.

If he thinks attempting to fix the MVD will fix the issue then he will likely want to try it. If not then he is putting you at risk for nothing for all he's concerned.

 

it was taken from another forum

in which a british doctor is mentioned, albeit not for doing these kinds of explorations, but for possibly being open to doing them. In europe there is Sismanis, who used to be in the US, but is now apparently in Greece. As usual, though, these avant-garde or dodgy procedures are done in the U.S., or weird countries like Israel, China or South Korea.

This particular case only links up the spasms with ear pain, its one example that may possibly leave the idea that spasms are not linked to pain. So its a step forward, but we still need more definitive testimonies to start to talk about whether sound-induced spasms are the same thing, and that's why I'm reading Lib from the healthboard forums, or Joe and Astrid from the chat-hyperacusis forums.

I also suppose that before going through this possibility, one has to try the more conventional TRT method.

 

For a patient, there's a world of a difference between going to doctors before or after dedicating hundreds and hundreds of hours to researching the condition online. If the patient is active and (politely) aggressive, then a better result can be extracted from the doctor, especially if its not a doc that's about to retire, is tenured and has it all made. The rest maybe depends on the medical model in each country, if it has a for-profit or a insurance/social security model.

For example, is myoclonus a rare condition, or an undiagnosed condition, like it says here.

 

You can be surprised to what diagnosis you get sometimes by those that are considered specialists... It will make you laugh. And more can be said about their attitude! I will leave it to that. Perhaps there are REAL doctors out there that try to help. I surely haven't visited any of them till now. And I doubt there are any of them in Greece.

 

Interessting I noticed that I have an eye spasm as well.

 

I'm not saying we should think about this without giving TRT a fair chance, and I know Greece sounds uber dodgy but if you look in the healthboard forum, in the myoclonus proboards forum, and the chat-hyperacusis forum, you will see that while in the US, it was Sismanis who cured Lib, a user with over a thousand posts detailing how a decade ago it solved her myoclonus, and possibly her ear pain and maybe certain sound sensitivities she sometimes commented on having. I contacted her recently and she was convinced I had myoclonus and that would solve me, but I am not so sure and want to be extra cautious so I'm still researching, and you don't have to look further than chat-hyperacusis to see another user named Astrid who was unhappy with the operation, even though Lib to this day is pretty sure Astrid's operation might have been incomplete. I could even contact the doctors on the research papers. Obviously some country's medical establishments are going to be more conservative than others, but maybe if we go to doctors with knowledge we can get contacts for ones more addicted to risk.

 

I think that many won't go down this path until TRT has been tried and has failed over a reasonable time frame. What that is I don't know, maybe a year? As to what constitutes failure? I seriously pondered the idea of a para-suicide attempt just to force the system in this country to take me seriously, but public systems aren't always the panacea for everything. I probably would have just got a short Mental Health admission, toxic anti-depressants and a foot up the arse out the door.

I do know of someone from my town who had a sudden hearing loss and super-tinnitus who was so distressed that he found his way to Sydney where someone there did a cochlear implant. I don't know if it helped him, but from what little I do know, anything was preferable as far as he was concerned.

 

You'd be done in for ''malingering'', which is often the kind of research they're really interested in. When I say ''give TRT a fair chance'' I don't know what the hell I mean. We've seen it happen with laser, with its half-assed at the edge of science explanations and piss-poor differential diagnoses, why wouldn't it also be happening with TRT? This thing is a condition where, in the words of an Israeli doctor that attended IDF army patients, 85% of the patients don't even return for a second appointment without any treatment whatsoever. Why wouldn't those ridiculously high numbers seep into the success statistics of TRT. Why would any of those involved have the incentive to do differential diagnosis. We've seen how TRT ''specialists'' have spread from country to country and I'm not impressed when cbBen, a prominent user on chat-hyperacusis with thousand messages, disappears when he says he's going to a TRT doctor, as if TRT docs were shrinks from born-again jesus camps telling him not to think negative thoughts and not to surround himself with negative influences. And the tinnitus handicap inventory on which it bases its success, apparently, what a joke. And the time-frame, 1 or 2 years with a background noise and if you think negative thoughts you're not doing the treatment so you can't be considered for entry into the TRT success stats. At that rate, it would be a paradigm that never fails. I'm also not impressed with the halo of mysticism its specialists seem to have, if all they really do offer is here.

I don't need to get ''inspired to meet goals'', or be part of a ''team'', if I needed a shrink I'd be dead long ago. I can listen to their TRT sound no end, but I won't listen to the nature sounds, nor will I sit around listening to sounds above the TRT, despite how apparently they, or the bunch of studies floating near to them, can't seem to tell the difference. So if it turns out that a decade ago, Lib and JoeM had the same thing, tht Lib had my kind of sound sensitivity, that this article has my kind of muscle pain, and that Astrid's weird experience was the thing that jammed progress, and that TRT served to halt it further, I will be pretty pissed off. But I want to give TRT a prolonged fair exploration, and that means researching it, seeing what people are really saying about it, and doing it. But 2 or 20 years of doing it, no sir.

 

That's one of the things about TRT and long timeframes. Does TRT jump on the back of a certain amount of natural healing/evolution of the condition/change that happens to be for the better that was occurring anyway, and then claim "I did that"?

 

Not only that, but according to a certain TRT clinician if the ''therapy'' failed for you it was either because

1) Because you didn't want to get better or 2) because it was not administered properly in the first place.

Screw this TRT garbage.

 

True Nucleo. The smart practitioner always has an exit strategy.

 

I have hearing loss and a microvascular compression (as well as lyme disease as described in the OP's article)
I am attempting to get a pre-surgical diagnosis is particularly difficult both either MVD or TTTS.

I feel as if I have to flip a coin between brain surgery and TTS surgery.
I think that working from the outside in might be a smarter (safer) bet?
Start with the ear first to see if symptoms go away?

I have an MRI that shows 'suspected compression of the glossopharyngeal nerve', suspected being the operative word. I was also told by the ordering neurosurgeon that the MRI isn't diagnostic. Someone with simular anatomy could be symptom free.

The stakes are pretty high for MVD surgery.

How are ENT's diagnosing TTTS?
Only by visualization as did Dr Poe in the above article?
Upon contacting Dr. Poe's office I was told that 'he doesn't do this anymore' and that there are other means to diagnose my this issue (without saying how of coarse)
I understand the 'visualization' procedure that Poe did in order to observe the TTS was in 2010.
Has TTTS diagnosis moved that rapidly in 6 years? I find it unlikely.

Being in the NY area I attempted to contact Dr Lindstrom author of the article suddenly passed in Feb 2016




I know this thread is old but I'm bumping anyway as it is extraordinarily pertinent to my current situation

 

I'm not sure. Maybe officially they dx the TT by eardrum inspection and the ST by acoustic reflex testing. Then entering the ear with the video would be superfluous. But then there's a handful of doctors either because they know more or because they're irresponsible who don't care anything, like Sismanis (retired) or Harold Kim (an ENT from Portland) who don't believe those tests are conclusive and go by patient description of thumps or something like that.

 

Just thought I'd point out that cbBen was banned from the hyperacusis network several years ago. I speak to him and his wife from time to time. He continues to suffer from chronic ear pain, and has not improved using TRT. He is very open about this.

 

Another possibility is that the ear is just tense and the vibrations can't be seen because they become tonic. Another that the vibrations are too small to be seen with the human eye.

Seems like that's a ritual we all have to go through these days, a badge of honour...

 

Didn't he get better for a while using lidocaine? What happened to that?

 

Not that I'm aware of.

 

So apparently looking inside the ear through tympanotomy isn't superfluous at all. Sismanis wrote in an article in the book Clinical Otology that a definitive dx can only be done by looking under the drum. And I'm quite sure that in person he told me that even that wasn't good enough as anaesthetic could leak into the muscle...

 

Doctors will probably not find or deal with your problem. The reason is obvious: There is little they can do. Personaly, I had contact with many people about this, nobody got cured by doctors, especially those that had mvd surgery. So, my advice is to stop looking for a solution by doctors. Assuming they have ruled out any urgent problem (for example ear tumors) or something else that needs and can be corrected, I suggest you WAIT and be patient. Anything else is an arbitrary attempt with many risks and no real propability of releaf. As you can see, many people with this problem have been at the forum for about 2 years and then dissapear. This should give you REAL hope that tinnitus can actually go away, contrary to what others, even doctors, may tell you.

Sorry for my late responce, I don't hang arround here for some time now.

 

Or they just give up.

 

Thanks for this information. However T is the lowest priority for me and pales in comparison to my trigeminal neuralgia pain which is a progressive disease. In my case, doing nothing as you say could be the worst possible choice as they don't call trigeminal neuralgia the 'suicide disease' for nothing. The facial pain and ear pain is unmeasurable. On a scale of 1 to 10 it's probably a 20. Yes it's that bad, it feels like your face is burning off and that you have a knife lodged in your ear.
Considering the progressive nature of trigeminal neuralgia doing nothing isn't an option.
Furthermore, I wanted to share the article as it is an example of TTS being resolved surgically.

If possible, I'd like to get in touch with those that you know that have had the mvd surgery. Perhaps they are on this board? Please advise if you would..

edit:
@skoupidis:
I did come across some posts that you discuss MVD, wondering if you went through with it, did you have trigeminal neuralgia, or were you pursuing MVD just for your T?
I'd be very interested on if you went through with it and your experiences. I happen to have several compression including the ACIA (as I believe you have) impinging on the GN (Glossopharengeal Nerve) (ear throat tonque) as well a the basilar artery pushing up on my TN, third a compression on the VIII 'bundle'. I've seen Linksy (CA), Brown (NY), and Horowitz (Pa) the high volume MVD guys.
BUT, I'm pretty apprehensive as anyone should be. Sometimes I starting thinking what came first, the chicken or the egg?;
The BIG QUESTION IS:
Does the H and T and TTS irritate the nerves?, OR is it the nerve dysfunction causing the pain, T H ?

MVD vs TTS surgery
Should I work from the inside out with, or outside in, the big question..
That's where I'm at right now.