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Sudden hearing loss - acoustic neuroma?
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Sudden hearing loss - acoustic neuroma?

I lost most of my hearing in my left ear overnight in november 2006. I have been diagnosed with Idiopathic sudden sensorineural hearing loss. I had oral steroids, aciclovir, carbogen and diuretics in the first month and some hearing recovery. I had an MRI but it was not enhanced (it was normal). I have spasmodic earache, screaming tinnitus (3 sounds) and regular left sided headache around my ear, I have an average 38dB loss across all frequencies but this goes up to 70dB at high frequencies. Specialists say that I just have to live with this. I take betahistine and co-codamol for the pain. I have another hearing test next week as either my aid is not working so well or more likely I have lost some more hearing.I also have strange sensations on the left side of my face from my ear to my nose but no pain or weakness. Could I have an acoustic neuroma? Should I have a gadolinium enhanced MRI scan?
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Avatar_n_tn
Yes & Yes

Acoustic N. rarely manifest in a sudden HL. BUT they can.  I would be PO'd to no end for an MRI without contrast. That should have been done in the first one. Waste of time and money.

In this case I doubt an ABR would be of any use due to the amount of hearing loss that you have.

Any how, the next step is to rule our an acoustic neuroma or any other neurological pathology.
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152264_tn?1280358257
I totally agree with W/a/J. It was unconscionable to do an MRI without contrast in looking for acoustic neuroma. MRI with gadolnium contrast is unquestionably the gold standard for acoustic neuroma, because with contrast it can see any small tumor that might be there that a non-enhanced scan might not pick up.

Especially if you are having odd facial sensations on that side, they really should rule out an AN conclusively. I would DEMAND that the imaging center re-do your scan at no charge, because they goofed up the first time not doing it with contrast.

I had a sudden unilateral hearing loss in 1999 (75 dB loss starting at 750 Hz and dropping to 90-110 in the higher frequencies) when I sneezed hard. I did not get as much initial treatment as you did (prednisone a month later, and much later exploratory surgery to rule out a fistula) but the ENT did order an MRI with contrast after seeing that the steroid did not work. I never regained any hearing.

Good luck--it's a good sign if you regained SOME hearing, but it sounds like something is still going on. Definitely insist on contrast, and I really would make them pay for it (whoever is responsible for your getting the first scan without contrast).

Nancy T.
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Avatar_n_tn
Nancy,

I am not so sure who goofed up.  The docs I have worked with write the type of MRI they want on a script pad....

The techies do as told....

If the techies should know better, out of training.... I don't know that answer.

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Avatar_f_tn
Thank you for your respose but what is an ABR? I'm in the UK and while the National Health Service is great there are some limitations. I have asked for another scan with Gadolinium but have been told that this is not necessary though everything I have read suggests otherwise. I also saw a leading specialist in London and though he agreed that people with SSHL don't have headaches or ear pain he could not offer any more help.
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Avatar_n_tn
An ABR is an archaic way of testing for an AN. The equipment is still sold and manufactured today and in fact it is still in use. I perform ABR's weekly. It is basically a form of an EKG (a rather simplistic explanation)

None the less, MRIs, in my opinion are more useful in determining neurological pathologies. Simply put, it is better technology. An ABR machine costs around 30K USD, an MRI machin over a 1,000,000 USD, and there is a reason for that.
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152264_tn?1280358257
W/a/J, I am always seeing on an MS board where I read that the radiologist (or technician? but I PRESUME the radiologist lurking behind the scenes) often decides to use, or NOT use, contrast regardless of the doctor's orders, because of what they initially see or don't see.

Can you imagine that an MRI place would NOT use gadolinium if instructed to rule out acoustic neuroma? Even if the referring doctor forgot, or didn't know, to include it? I think there are standard protocols for ruling out certain things, so the MRI place should KNOW the protocol for acoustic neuroma, even if the referring doctor didn't.

To Jewel, I would tell them about your new symptoms and request an MRI with gadolinium anyway. Acoustic neuromas can be very small but grow to cause other symptoms. In fact, even with a properly done MRI with gadolinium, it's possible for there to be very tiny hidden ANs, although that is very rare.

As for ABR, my otoneurologist--a highly trained neurologist specializing in hearing/balance with a Ph.D. in auditory neuroscience as well as an MD--ordered one for me even after I'd had the MRI to rule out acoustic neuroma. I think he was looking for any nerve damage to explain my bilateral symptoms including some possible MS symptoms. In fact my ABR was highly abnormal despite the normal MRI--and not just in my hearing-loss ear, but in the normal-hearing ear as well. ABR is a type of evoked potential which is one of three types (the others are visual and somatosensory) which are sometimes used to look for nerve damage in MS or in other circumstances.

Nancy T.
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Avatar_n_tn
Nerve damage would have shown up in an Audiogram. Nerve damage would have been evident long before an ABR would need to be ordered. An OAE would be nice to help localize the pathology. But even then one theoritically can have a cochlear problem and a retro-cochlear pathology (AN). The AN presses down on to the blood supply to the cochlea, starving it, and killing it.  So this would lead to a poor OAE results. But in this case if one does an ABR, the cochlea damage, would probably prevent the patient from hearing the stimulus. Thus rindering the ABR study, useless.

Any ENT would weigh the results of an MRI heavier than the results of an ABR.  Even if an individual has a normal ABR with symptoms, MRIs are usually ordered. Or if the ABR is ordered and deemed abnormal an MRI is then ordered....

So where is the utility for ABR studies for an Acoustic Neuroma?

Every ABR report study I submit I attach a disclaimer to them....  for a reason.
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Avatar_n_tn
and on that note, I have 2 ABRs scheduled for tomorrow....
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152264_tn?1280358257
Yes, I know ABRs aren't much use when you can get an MRI, which is the last word on ANs. The ENT who diagnosed my hearing loss ordered an MRI right off the bat (after the steroid did not restore my hearing), no mucking around with an ABR.

I wasn't suggesting that an ABR is useful for diagnosing AN in the MRI era, only that ABR DOES have other uses, of course. In my case to see if there was something abnormal other than AN. Even if you discount the results in the lost frequencies in my left ear, there was still a very large abnormality in the normal frequencies (up to 500 Hz) in that ear and also in my normal-hearing ear. (Ordered by a neurologist a year after the MRI ruled out AN.)

Nancy T.
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Avatar_f_tn
Thanks for you help. I don't really have any new symptoms except I may have lost a bit more hearing. I think I will get this checked and then go back to my GP. The process is long unfortunately because he then has to refer to ENT and I will have to argue the case again for an enhanced MRI. Are the one sided headaches which sometimes seem to radiate into my neck, a possible sympton of AN too.
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152264_tn?1280358257
I don't know whether headaches are a symptom of AN--but I don't think so.

One-sided headaches are most likely to be due to migraine.

Anytime you have lost more hearing I would insist on getting a new audiogram and reassessment of your situation. Sudden hearing loss is a very urgent situation and your ENT should treat it as such.

Regardless of whether you have an AN or not, new hearing loss should be given immediate attention and evaluated anew, taking into account your other symptoms such as the ear pain and headache! They can't just rule out AN and then say, we have nothing more to look at here.

Best of luck,
Nancy T.
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Avatar_f_tn
Thanks. I suppose they could be migraines but they started at the same time as my initial hearing loss.

I do rarely get migraines but not with headache ie visual disturbances, speech problems and mild confusion but I have not had one of these for the past couple of years.

I should have insisted on further investigation sooner but even the epert in London said this was not necessary. Why would they do that?
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Avatar_n_tn
Yes headaches are a symptom of AN

ABRs basically have 2 functions. One to determine if ther is an AN, two to determing objective hearing thresholds.

So as to what Nancie's ENT was looking for I have no earthly idea. The normative data for AN type ABRs is based on click stimuli with a band with for 2K Hz - 4K Hz. So I have no idea why they were messing around with a 500 Hz tone pip or tone burst stimuli. That type of stimuli is used in order to determine hearing thresholds, and even then the normative data is vague. Most facilities use a click stimuli for thresholds, and most threshold testing is done on children.

Threshold testing on adults is usually done when one suspects someone is fakeing their hearing loss, or do not have the mental capacity to participate in a subjective hearing test.

Theshold ABR testing is now old technology. Today we use ASSR, and it offers a lot more information than what a click ABR can give.

Any how, have got to get two ABR reports out of the way.

One was ordered because a conductive asymetrical hearing loss. (a needless ABR)

The second was ordered on a mild asymetric SNHL. The guy had a gun go off near his ear 3 months ago.  No other symptoms, no dizziness, vertigo, facial paralyssis, head aches, loss of taste, rapid change in vission etc.

OAE test resutls show cochlear damage.....  another needless ABR.

But hey...  I just do as told.
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152264_tn?1280358257
"So as to what Nancie's ENT was looking for I have no earthly idea."

Evidence of MS, I believe (though he didn't say so--but he talked about MS my whole first appointment). See http://www.nationalmssociety.org/site/PageServer?pagename=HOM_LIB_sourcebook_evoked.  Later when I got his report and saw he referred to it as "cranial neuropathy" he also mentioned sarcoidosis as a cause, but that I didn't have other evidence of this. Apparently there are numerous things other than AN that can cause cranial nerve damage.

Jewel: I don't know why the expert that you saw would say no further investigation was needed. If they were paying attention ONLY to your hearing loss--well, once an acoustic neuroma or suchlike is ruled out, and they've tried their arsenal of treatments for the hearing loss, it's true that you're stuck with it. (Although I don't know how they can rule it out definitively without a contrast MRI!)

But if you're having other symptoms, you should be followed up for those.

Migraines can take different forms at different times of a person's life. At one period you might have headaches. At another time you might have just visual aura, with no headache. At another time you might have just nausea, or a tingling numbness marching up your arm. I had headaches in my youth, but didn't even know I was a migraineur until getting an ocular migraine at age 42.

Oddly, I got my first ocular migraine (plus a raft of other odd symptoms, including facial shocks, off-balance, widespread paresthesias, etc.) all within weeks of my hearing loss. But in my case I think it was most likely a coincidence, as the hearing loss happened when I sneezed, so was probably a traumatic event.

If you are having other symptoms or further hearing loss, they REALLY should follow up with you! Make it clear to your doctor that it's not just the initial hearing loss.

Nancy T.
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152264_tn?1280358257
Correction: this was not my ENT who ordered the ABR--it was the NEUROLOGIST.
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Avatar_n_tn
When one starts dealing with late latency responses, thing really get out of hand...  If the patient just has a "thought" like, Hmm I like ice cream, or I am hungry, the results are skewed.

Imgain trying to formulate normative data with that.
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152264_tn?1280358257
Guess I was thinking too much during the test... ice cream would have been a likely subject! :)

I read the book "Women Who Think Too Much," but it probably didn't help. :)
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Avatar_m_tn
Headaches are noted in about 50% of patients with acoustic neuromas although they are seldom the complaint that brings them to the physician. That is not to say many people with headaches have acoustic neuromas. But the two are associated.

Most common symptom of acoustic neuroma is asymmetric sensorineural hearing loss. MRI with gadolinium enhancement is the gold standard test for acoustic neuromas. MRI without enhancement can miss small tumors aound 2-4mm which are still plenty large enough to cause symptoms if in the right place.
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