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Diffuse cochlear disease in both ears; anyone know what this means?
Results from my audiology & vestibular testing were there when I picked up the mail today. I tried to google the results, but I'm confused. My appointment with my oto-neurologist is June 23, so I don't have long to wait to hear what he has to say, but I'm curious now. :o)
The report summary is as follows:
"This patient has normal hearing and middle ear pressure and compliance are within normal limits. Reflex decay patterns are abnormal and suggest diffuse cochlear disease in both ears. Auditory evoked potential testing does not support the presence of elevated endolymphatic pressure or any unilateral N.VIII disease at this time."
Is this some sort of cochlear Meniere's? Will it get worse? The dizziness episodes have gotten worse, and more frequent since my neurological symptoms started my trip through limboland in Feb. '08.
Any ideas? I'd really appreciate any input.
Thanks,
Kathy
The report summary is as follows:
"This patient has normal hearing and middle ear pressure and compliance are within normal limits. Reflex decay patterns are abnormal and suggest diffuse cochlear disease in both ears. Auditory evoked potential testing does not support the presence of elevated endolymphatic pressure or any unilateral N.VIII disease at this time."
Is this some sort of cochlear Meniere's? Will it get worse? The dizziness episodes have gotten worse, and more frequent since my neurological symptoms started my trip through limboland in Feb. '08.
Any ideas? I'd really appreciate any input.
Thanks,
Kathy
I've been lucky in never having trouble with air travel--it doesn't make me dizzier. I think it depends on the cause of one's vertigo/dizziness.
I was suspected of having a perilymph fistula simply because of my history of sudden hearing loss when I sneezed (PLF can be caused by sneezing, rarely); I even had the repair surgery, but a PLF was not seen upon surgery and I did not improve, thus I almost certainly didn't have one. Most people with PLF have pretty bad dizziness (worse than what I had). There is a lot of controversy surrounding PLF (whether it occurs spontaneously, how often it occurs, etc.)--a few doctors diagnose it a lot, but most think it's rare, especially without a clear history of trauma.
Dr. Hain's site is indeed fantastic. I do think his site is intended for patients as well. It contains technical info and references, but an educated layperson can learn a great deal from it. Dr. Hain is a neurologist, specializing in dizziness and highly respected in the dizzy world.
Nancy
I was suspected of having a perilymph fistula simply because of my history of sudden hearing loss when I sneezed (PLF can be caused by sneezing, rarely); I even had the repair surgery, but a PLF was not seen upon surgery and I did not improve, thus I almost certainly didn't have one. Most people with PLF have pretty bad dizziness (worse than what I had). There is a lot of controversy surrounding PLF (whether it occurs spontaneously, how often it occurs, etc.)--a few doctors diagnose it a lot, but most think it's rare, especially without a clear history of trauma.
Dr. Hain's site is indeed fantastic. I do think his site is intended for patients as well. It contains technical info and references, but an educated layperson can learn a great deal from it. Dr. Hain is a neurologist, specializing in dizziness and highly respected in the dizzy world.
Nancy
Thank you so much for your reply, and for trying to explain my test results. You did a great job of making it more understandable.
I just saw this, as I haven't been getting any notifications.
I see Dr. B., my oto-neurologist, tomorrow, and am curious as to what he'll say; I'd like to print out and memorize your post. Then I'll have some intelligent questions to ask; I can always say "I have a family friend that's a doctor, and she suggested the possibility of ..." or something like that.
I'm so looking forward to seeing Dr. M.!
When I flew into Orlando, my dizziness wasn't bad at all; I had several pretty good days. Then I had the time on the beach and the dizziness got worse; maybe the flight home cemented the dizziness with the pressure changes; changed planes in Denver, so it was up down up down.
I suppose my old mountain climbing days were too slow of an ascent and descent to cause a trauma, and that volleyball I got slammed in my left ear in 6th grade was too long ago.
Doggone, I have short flights to Sacramento coming up in July and August. Plan on going to Seattle, too, but at least those trips will be on Amtrak!
Oh, one question; should I consider faxing those results to Dr. M., or ask that my oto-neurologist fax the results and his impressions to Dr. M.? I guess I could call Dr. M's office and ask if he would like to see the results before he sees me in September.
I'll now go google perilymph fistula and check out Dr. Timothy Hains.
Thanks again, Quix.
Kathy
PS I hope you're hanging in there with all you have going on. Thinking about you often and wishing you the best.
I just saw this, as I haven't been getting any notifications.
I see Dr. B., my oto-neurologist, tomorrow, and am curious as to what he'll say; I'd like to print out and memorize your post. Then I'll have some intelligent questions to ask; I can always say "I have a family friend that's a doctor, and she suggested the possibility of ..." or something like that.
I'm so looking forward to seeing Dr. M.!
When I flew into Orlando, my dizziness wasn't bad at all; I had several pretty good days. Then I had the time on the beach and the dizziness got worse; maybe the flight home cemented the dizziness with the pressure changes; changed planes in Denver, so it was up down up down.
I suppose my old mountain climbing days were too slow of an ascent and descent to cause a trauma, and that volleyball I got slammed in my left ear in 6th grade was too long ago.
Doggone, I have short flights to Sacramento coming up in July and August. Plan on going to Seattle, too, but at least those trips will be on Amtrak!
Oh, one question; should I consider faxing those results to Dr. M., or ask that my oto-neurologist fax the results and his impressions to Dr. M.? I guess I could call Dr. M's office and ask if he would like to see the results before he sees me in September.
I'll now go google perilymph fistula and check out Dr. Timothy Hains.
Thanks again, Quix.
Kathy
PS I hope you're hanging in there with all you have going on. Thinking about you often and wishing you the best.
I have been trying to sort all this out and I think it is probably folly to try to do this as laywomen or non-specialist.
Your ABR is an evoked potential. In the context of MS we would be looking for a normal, well-preserved wave form that had a significantly increased latency. Literally meaning that it was delayed in arriving at the electrodes. This would indicate cranial nerve VIII demyelination. They look for a difference between the two ears in interpreting the results. You had a delay (with normal waveform) at 500Hz in both ears and at 1000Hz on the right. The report "hedges" on the interpretation. It basically ignores this delay and says there is no evidence for "unilateral" cranial nerve VIII disease. The literature says that "bilateral" involvement of the ears in MS is very rare. So, this interpetation is doing a little bit of editorializing.
Okay. Then you had an ElectroCochleagram, or ECog. This test is to ascertain whether the cochlea has excessive fluid built up within it. The entire inner part of the cochlea is bathed with a fluid called "endolymph." This fluid is made and reabsorbed just like the CSF. If, for some reason too much fluid is made or too little reabsorbed, the pressure in the cochlea will be high. This is felt to be the basis for Meniere's Disease.
The ECog attempts to "read" the pressure in the cochlea. It is a test that is hard to do and often difficult to interpret. Your pressures were normal - enough. Since Meniere's always has elevated fluid pressure in the cochlea, called "endolymphatic hydrops" then you DO NOT have any form of Meniere's.
So, the only abnormalities were the AER - Audio Evoded Response (Potential). Since the problem is bilateral they called it "cochlear disease." This is stating that the problem is within the inner ear and not a problem with the CNS. I don't see how they can say this. I have come to believe that, just because they haven't reported something in MS, that doesn't mean that it can't happen. The optic nerve (CN II) can be affected bilaterally, The eye muscle nerves (CN III, IV, and VI) can all be affected bilaterally, We ALL know the Trigeminal Nerve (CN VIII) can have bilateral MS plaques, as can the Facial Nerve (VII). Where is the rule that some other cranial nerve can't have bilateral involvement?
Can you have this kind of cochlear disease and have normal hearing. Absolutely yes. Remember, the signal is arriving at it's destination. It's just some milliseconds late. Can You tell that it is a minor fraction of a second late? No, we are not that sensitive. Your brain puts it all together into normal hearing.
I have one final thought - well two (or more actually. We'll just have to see when I stop typing,) The first is the potential of a perilymph fistula. Perilymph is the fluid that surrounds the structures of the vestibular portion of the inner ear. It's counterpart in the cochlea is the endolymph. Sometimes a tear can form in the sac of the vestibular labyrinth that allows fluid to leak out of the inner ear to the middle ear. This situation causes intermittent severe and ongoing less severe vertigo. The most common cause of a PLF is "barotrauma" or damage done by changing pressures - typically like with SCUBA diving or airplanes, but it can also be caused by head trauma and severe audio trauma.
Anyway, I had a severe perilymph fistula that was causing a great deal of my vertigo problems and it had to be repaired surgically. I thought of this as a (probably unlikely) possible cause of your vertigo because the ECog might not be able to "read" the pressure of the endolymph because of the continual leak of fluid into the middle ear which changes its volume. Your ECog had trouble with stable values in one ear.
Also, the worst thing for a person with a PLF is air travel. The marked change in barometric pressure of taking off and, especially, landing will really make it act up.
And air travel is bad for ANYONE who has vertigo. You seem to fly a lot and this could be adding to your troubles, no matter what the cause of your vertigo.
You can Google perilymph fistula. Dr. Timothy Hains' site is great for all topics on vertigo - though he writes for health professionals.
Dr. M is not only an MS Neuro, but a Neuro-Otologist (and a Neuro-Ophthalmologist) also. He can tell you if any of these results are significant.
The rest of my thoughts just skipped town.
Quix
Your ABR is an evoked potential. In the context of MS we would be looking for a normal, well-preserved wave form that had a significantly increased latency. Literally meaning that it was delayed in arriving at the electrodes. This would indicate cranial nerve VIII demyelination. They look for a difference between the two ears in interpreting the results. You had a delay (with normal waveform) at 500Hz in both ears and at 1000Hz on the right. The report "hedges" on the interpretation. It basically ignores this delay and says there is no evidence for "unilateral" cranial nerve VIII disease. The literature says that "bilateral" involvement of the ears in MS is very rare. So, this interpetation is doing a little bit of editorializing.
Okay. Then you had an ElectroCochleagram, or ECog. This test is to ascertain whether the cochlea has excessive fluid built up within it. The entire inner part of the cochlea is bathed with a fluid called "endolymph." This fluid is made and reabsorbed just like the CSF. If, for some reason too much fluid is made or too little reabsorbed, the pressure in the cochlea will be high. This is felt to be the basis for Meniere's Disease.
The ECog attempts to "read" the pressure in the cochlea. It is a test that is hard to do and often difficult to interpret. Your pressures were normal - enough. Since Meniere's always has elevated fluid pressure in the cochlea, called "endolymphatic hydrops" then you DO NOT have any form of Meniere's.
So, the only abnormalities were the AER - Audio Evoded Response (Potential). Since the problem is bilateral they called it "cochlear disease." This is stating that the problem is within the inner ear and not a problem with the CNS. I don't see how they can say this. I have come to believe that, just because they haven't reported something in MS, that doesn't mean that it can't happen. The optic nerve (CN II) can be affected bilaterally, The eye muscle nerves (CN III, IV, and VI) can all be affected bilaterally, We ALL know the Trigeminal Nerve (CN VIII) can have bilateral MS plaques, as can the Facial Nerve (VII). Where is the rule that some other cranial nerve can't have bilateral involvement?
Can you have this kind of cochlear disease and have normal hearing. Absolutely yes. Remember, the signal is arriving at it's destination. It's just some milliseconds late. Can You tell that it is a minor fraction of a second late? No, we are not that sensitive. Your brain puts it all together into normal hearing.
I have one final thought - well two (or more actually. We'll just have to see when I stop typing,) The first is the potential of a perilymph fistula. Perilymph is the fluid that surrounds the structures of the vestibular portion of the inner ear. It's counterpart in the cochlea is the endolymph. Sometimes a tear can form in the sac of the vestibular labyrinth that allows fluid to leak out of the inner ear to the middle ear. This situation causes intermittent severe and ongoing less severe vertigo. The most common cause of a PLF is "barotrauma" or damage done by changing pressures - typically like with SCUBA diving or airplanes, but it can also be caused by head trauma and severe audio trauma.
Anyway, I had a severe perilymph fistula that was causing a great deal of my vertigo problems and it had to be repaired surgically. I thought of this as a (probably unlikely) possible cause of your vertigo because the ECog might not be able to "read" the pressure of the endolymph because of the continual leak of fluid into the middle ear which changes its volume. Your ECog had trouble with stable values in one ear.
Also, the worst thing for a person with a PLF is air travel. The marked change in barometric pressure of taking off and, especially, landing will really make it act up.
And air travel is bad for ANYONE who has vertigo. You seem to fly a lot and this could be adding to your troubles, no matter what the cause of your vertigo.
You can Google perilymph fistula. Dr. Timothy Hains' site is great for all topics on vertigo - though he writes for health professionals.
Dr. M is not only an MS Neuro, but a Neuro-Otologist (and a Neuro-Ophthalmologist) also. He can tell you if any of these results are significant.
The rest of my thoughts just skipped town.
Quix
All my ABR report said was "Waveforms are of good amplitude, morphology and repeatability for both ears. Absoluure and interwave latencies fall within normal limits for both ears. The expected Wave V latency shift for the high rate AR waveforms falls within normal limits for both ears" In other words, way normal!
The latency difference numbers in my last post were still about the ECoG. It really seems that endolymphatic hydrops is not an issue with me.
I normally eat a low-salt diet, for the most part I don't add salt when I cook, buy low-salt canned beans, tomatoes, sauce, etc. I did try a diuretic once when my blood pressure was slightly elevated, and it didn't help anything. I drank some dandelion root tea for a few weeks (natural diuretic) and haven't had high blood pressure since.
I could try some dandelion root tea again, and watch my salt intake even more closely; see if that makes a difference with my dizziness. There doesn't seem to be much rhyme or reason as to when it gets worse. Well, it got worse Friday after I'd spent an hour on the beach on Honeymoon Island in Florida, then had to walk the hour back. I'd been collecting shells.
I've been dizzy since. I don't know if the long flight home on Saturday contributed, or the heat, or what. The tests were a hunt for clues, the vestibular rehab is an attempt to decrease the dizziness. I'll be glad to try reducing my salt intake. Can't hurt, might help!
Hmm, I'll check with my PCP; since my blood pressure has been low, she told me that I could try eating more salt to raise it a little. My sodium levels have been the bottom of the normal range. I'll be seeing her a week after I see Dr. B.
Kathy
The latency difference numbers in my last post were still about the ECoG. It really seems that endolymphatic hydrops is not an issue with me.
I normally eat a low-salt diet, for the most part I don't add salt when I cook, buy low-salt canned beans, tomatoes, sauce, etc. I did try a diuretic once when my blood pressure was slightly elevated, and it didn't help anything. I drank some dandelion root tea for a few weeks (natural diuretic) and haven't had high blood pressure since.
I could try some dandelion root tea again, and watch my salt intake even more closely; see if that makes a difference with my dizziness. There doesn't seem to be much rhyme or reason as to when it gets worse. Well, it got worse Friday after I'd spent an hour on the beach on Honeymoon Island in Florida, then had to walk the hour back. I'd been collecting shells.
I've been dizzy since. I don't know if the long flight home on Saturday contributed, or the heat, or what. The tests were a hunt for clues, the vestibular rehab is an attempt to decrease the dizziness. I'll be glad to try reducing my salt intake. Can't hurt, might help!
Hmm, I'll check with my PCP; since my blood pressure has been low, she told me that I could try eating more salt to raise it a little. My sodium levels have been the bottom of the normal range. I'll be seeing her a week after I see Dr. B.
Kathy
Kathy, have you ever tried an experiment of using a diuretic plus low-salt diet, which constitute the standard treatment (or trial) for hydrops? It's a pretty easy and cheap thing to try. I've been on a diuretic three times in the past 10 years, once for blood pressure and twice to see if it helped the dizziness. (It didn't.) The first trial, three weeks long (under Dr. B), also included the low-salt diet. He didn't necessarily think I had hydrops, but it's such an easy and dirt-cheap thing to try that one might as well--barring any medical problems that might contraindicate the diuretic and low salt. The other two times, I was on the diuretic for months or years at a time but didn't feel any worse when I quit taking it.
My ABR numbers were WAY higher--like 5 to 10 msec delays.
Nancy
My ABR numbers were WAY higher--like 5 to 10 msec delays.
Nancy
Hiya! At the end of that section, it says that my latency difference does not point towards hydrops, though the number for the left ear is higher, it's not high enough to be called abnormal (.25ms, greater than .38ms suggests endolymphatic hydrops, and my right ear is .04ms).
Something weird going on! I hope Dr. B. can sort it all out.
Thanks for all your input!
Kathy
Something weird going on! I hope Dr. B. can sort it all out.
Thanks for all your input!
Kathy
Hi Kathy. All I know is that ECoG is a test done for hydrops (although it may have other uses, I don't know; and I don't know whether it's a definitive test for hydrops) and that "morphology" has to do with how the wave is formed. So, it sounds like something may be going on in your left ear, particularly since the test results were clearly different than in the right ear.
Nancy
Nancy
I found another abnormality in my test results, under Electrocochleography. It says:
"Waveforms obtained are clear and repeatable in the right ear. Morphology was deemed fair to poor in the left ear".
Any idea what that might mean as far as my left ear is concerned?
"Waveforms obtained are clear and repeatable in the right ear. Morphology was deemed fair to poor in the left ear".
Any idea what that might mean as far as my left ear is concerned?
Hi Kathy. Yes, it will be interesting to see what he thinks. I thought that cochlear Meniere's meant just hearing loss without vestibular symptoms, but then I have not really researched this.
The report says (under tympanometry and acoustic reflex studies) "Contralateral acoustic reflex decay patterns are elevated at 500 Hz in both ears and at 1000 Hz in the right ear suggestive of diffuse cochlear pathology".
I did read something about cochlear Meniere's, which has somewhat different symptoms than Meniere's. I remember Dr. B. telling me that he thought I had something like a form of Meniere's; maybe the guy really knows his stuff, if he guessed that before the testing!
We'll see what he says. Quix's plate is full, and I wouldn't want her to use her energy on answering my curiosity. Unless she found this interesting, of course!
Hope you're doing well,
Kathy
I did read something about cochlear Meniere's, which has somewhat different symptoms than Meniere's. I remember Dr. B. telling me that he thought I had something like a form of Meniere's; maybe the guy really knows his stuff, if he guessed that before the testing!
We'll see what he says. Quix's plate is full, and I wouldn't want her to use her energy on answering my curiosity. Unless she found this interesting, of course!
Hope you're doing well,
Kathy
Hi Kathy. Good to hear that your results are mostly normal. Of course, normal results don't mean there's no reason for your dizziness, only that it's not showing up on the tests.
I don't know what "cochlear disease" means, but if your hearing is fine, I'm betting it's not going to be anything to worry about. The cochlea, of course, is the organ of hearing--the spiral inside your inner ear containing the hair cells by which you hear.
I think reflex decay has to do with how your ear responds to loud sounds. (You had a test that involved a sudden loud sound?) I had a highly abnormal acoustic reflex decay on the left indicating a retrocochlear problem (that is, "behind" or "beyond" the cochlea, thus having to do with the nerve or brain, instead of the cochlea itself). Still unknown!
Cochlear implants are for people who are totally deaf. If you had Meniere's, I would think you would have at least some hearing loss. From what I remember of your symptoms, it doesn't sound like you've had attacks suggestive of Meniere's. Quix would probably have some good comments about your results.
Glad you had an enjoyable trip!
Nancy
I don't know what "cochlear disease" means, but if your hearing is fine, I'm betting it's not going to be anything to worry about. The cochlea, of course, is the organ of hearing--the spiral inside your inner ear containing the hair cells by which you hear.
I think reflex decay has to do with how your ear responds to loud sounds. (You had a test that involved a sudden loud sound?) I had a highly abnormal acoustic reflex decay on the left indicating a retrocochlear problem (that is, "behind" or "beyond" the cochlea, thus having to do with the nerve or brain, instead of the cochlea itself). Still unknown!
Cochlear implants are for people who are totally deaf. If you had Meniere's, I would think you would have at least some hearing loss. From what I remember of your symptoms, it doesn't sound like you've had attacks suggestive of Meniere's. Quix would probably have some good comments about your results.
Glad you had an enjoyable trip!
Nancy
Lulu, cochlear implants of course were the first thing that came to mind for me, too. I just hope that my excellent hearing means its something relatively benign. I don't even know if that is what is contributing to my dizziness.
I also hope it's something benign, and there's some way to minimize the dizziness and balance issues. Tee hee, could I have an ear fungus? Just some drops or pills and I'm well? Ha ha ha!
Nancy, I did get my VNG results; normal
1) The oculomotor examination is normal
2) There is no significant gaze evoked, positional or headshake evoked nystagmust.
3) There is no objective evidence of benign paroxysmal positional vertigo.
4) Horizontal semicircular canal responses to caloric stimulation are normal.
5) Vestibular evoked myogenic potentials are normal bilaterally consistent with normal saccular and inferior vestibular nerve function.
I find it interesting that since my two hours on the beach in the hot sun (with hat, sunscreen, cold water, etc.), my dizziness has come back and today it's really bad, combined with fatigue. I'm even slightly slurring my words.
Tomorrow is another day; I hope I'm just feeling the effects of a pseudo-exacerbation combined with jet lag.
Kathy
I also hope it's something benign, and there's some way to minimize the dizziness and balance issues. Tee hee, could I have an ear fungus? Just some drops or pills and I'm well? Ha ha ha!
Nancy, I did get my VNG results; normal
1) The oculomotor examination is normal
2) There is no significant gaze evoked, positional or headshake evoked nystagmust.
3) There is no objective evidence of benign paroxysmal positional vertigo.
4) Horizontal semicircular canal responses to caloric stimulation are normal.
5) Vestibular evoked myogenic potentials are normal bilaterally consistent with normal saccular and inferior vestibular nerve function.
I find it interesting that since my two hours on the beach in the hot sun (with hat, sunscreen, cold water, etc.), my dizziness has come back and today it's really bad, combined with fatigue. I'm even slightly slurring my words.
Tomorrow is another day; I hope I'm just feeling the effects of a pseudo-exacerbation combined with jet lag.
Kathy
*cochlear* makes me think cochlear implants which are done to restore hearing - like I said its just a guess. Perhaps its next to nothing - you know the word *disease* is attached pretty randomly to things that are abnormal in our body. For example, I have a diseased great toe - sounds pretty bad but its really just a case of fungus that's been there forever. Hopefully your ear is something similarly benign.
L
L
Well, I hope not! My hearing is great;
"normal hearing sensitivity for both ears. ...word recognition in quiet was good in both ears at 100% for the right ear and 96% correct for the left"
I left out the more involved stuff with 10dBHL something or other.
I'll let you know what Dr. B. says!
Kathy
"normal hearing sensitivity for both ears. ...word recognition in quiet was good in both ears at 100% for the right ear and 96% correct for the left"
I left out the more involved stuff with 10dBHL something or other.
I'll let you know what Dr. B. says!
Kathy
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