EM of the ears - best doctor and medication?

General
#1

Hello everyone :blush:

I have recently joined the Living with EM forum. I wanted to ask people’s advice about my situation, which is bothersome EM of the ears in particular. Without medication they flush for three hours at a time; with medication they can flush for around an hour and go very warm. There are also broken blood vessels on them from the regular flushing. I find the condition embarrassing, irritating and (without medication) painful.

Currently I take moxonodine and hydroxychloroquine – the most effective combination to date for me. Botox injections also help. I have tried beta blockers and pregabalin and neither have been effective. I have not, however, taken gaberpentin yet. I am in the UK and wondered if anyone has managed to get mexiletine here (as I know it requires a special license to import it unlike in some other countries)?

I’m not sure if my current medications are the best or sustainable to take in the long term, so I am interested if anyone has found particular medications helpful for the ears?

Even with medication they are still very sensitive to heat, wind, etc.

I wondered if anyone had found an EM specialist in the UK who had a particular understanding of how to treat EM of the ears (I know the focus is more usually on the feet and hands)? I would be happy to travel to another country for treatment if there was someone who really knew how to treat EM of the ears as well.

I have been diagnosed with EM of the ears by a dermatologist with an interest in the condition but he did not have that many suggestions for treatment, so I am keen to find a specialist with more experience of treating this if anyone has suggestions.

I have read through the threads on ears on this site.

I look forward to any advice and recommendations you have – thank you so much for taking the time to read my experiences.

#2

Is it just in your ears? If so, red ears can be many things, EM being one of them. However, red hot ears is fairly common in allergic reactions. Maybe try watching your food intake and/or try an antihistamine to see if it eases the redness. EM is a rare disorder so ruling out much more common causes of red hot ears would be a good step in my opinion.

Finding a local doctor that is willing to trial and error medications is the recommended way to get to the bottom of your EM due to no doctor having a magic test or pill for EM that hides behind blood tests and skin biopsy. It can take awhile to find what works but calcium channel blockers are recommended to be tried carefully due to their effectiveness for some types or EM (they can temporarily worsen EM if you don’t have the vasoconstrictive form so that’s why carefully is emphasized by Dr. Cohen). With beta blockers not helping it may be worth a try. You will find something that works, it just might take time. I have had my EM for 9 months and it took 7 months to find a medication that helps and since then I have found another that helps. Sometimes throwing the kitchen sink at the problem and then tapering off some medications is the best way to do it, at least that’s what has worked for me.

1 Like
#3

Hey there

some links i had bookmarked -

https://entokey.com/refractory-erythromelalgia-of-the-ears-response-to-mexiletine/ case study of ears responding to mexiletine

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3850925/ - comparing red ear syndrome to EM, saying the pathophysiology might be similar.

If you respond to plaquenil, I hope that you have seen a rheum and got thorough testing of autoimmune stuff - my observation is that when people are responsive to plaquenil for EM, they have autoimmune conditions like lupus behind their EM.

There are also other non steroidal anti-inflammatory drugs maybe a rheum could talk to you about, like methotrexate, IVIG (IVIG is sometimes tried for “apparently autoimmune” SFN / EM) to squeeze out more benefit from an anti-inflammatory approach.

I’d have to read the second link again, but I think the authors thought that EM symptoms of the ears could be due to either angry C fibers (sensory nerves) and/or a lack of sympathetic vasoconstriction (ears don’t really have the parasympathetic dilation going on normally). In the case of the latter, I wonder if something like effexor or something else with norepinephrine reuptake inhibition mechanism, might be helpful (the norepinephrine might help to boost the sympathetic constriction).

I’m just brainstorming, of course talk to your doc.

If mex isn’t available where you are, there are other sodium channel blockers like ranexa, flecainide, and anti-seizures like zonegran, topamax, carbamazepine. I have noticed flecainide to be helpful for my heat intolerant symptoms which include ears.

best wishes to you. I am glad you have found some medication so far to help you – that is huge! Usually EM and its variants are refractory.

Edit - If you want someone to think about the ear aspect specifically, then perhaps a pain doctor, who might be able to talk to you about certain nerve blocks - greater auricular nerve block, stellate ganglion nerve block, etc. Some of these can be diagnostic too in thinking about how your pain is originating. Other than that, no matter what you call your symptoms, it sort of devolves into trial and error with the same group of medications anyway as the other comments suggested, so the best docs for me have been the ones I can communicate with frequently to expedite the process.

#4

Assuming for the moment the diagnosis is correct, finding an intellectually curious doctor willing to trial and error with you may be more realistic. It will be difficult to find an expert on auricular EM. There isn’t any sort of established treatment protocol for erythromelalgia in general, much less for auricular EM specifically. Finding an effective treatment will likely require trial and error regardless who you see

#5

Thank you very much for everyone’s replies so far - most appreciated.

Joe - My specialist has diagnosed EM. I have some irritation of the feet but this is controlled by medications. My main problem is the ears, which is why I’m particularly interested what medications people have found helpful in reducing the flushing of ears. I am definitely interested in which calcium channel blockers may be most helpful to trial in my case, yes.

Cat - Thank you very much for your suggestions. I agree a neurologist may be helpful. I am thinking of going to see one of the EM experts at the National Neurology Hospital in London. I’d be interested if anyone has any feedback on any of these doctors.

Carter - I agree EM is to be treated through trial and error. As you say, you need a doctor who is curious to work with you on your main concerns which for me is my ears. So I’m very interested if anyone can recommend such a doctor in the UK who has helped them (obviously particularly with the ears, but also in general with EM).

#6

Is there no one in the NHS able to seek out such a doctor on your behalf? No administrative officials or social workers who can assist patients with rare illnesses? Because here is the math you’re facing: Erythromelalgia is estimated to affect 1 in every 100,000 people. Auricular EM is itself an uncommon subset. Its prevalence among individuals with EM isn’t known, but lets estimate it at 1 out of every 25. There are 66 million people in the United Kingdom. That means there are around 660 people nationwide in the UK with erythromelalgia and perhaps just 26 with EM affecting the ear. Those are tough numbers when you’re looking for a doctor on your own. There just aren’t many EM doctors because there aren’t many EM patients.

The Erythromelalgia Association does have a physician directory. They list a couple dozen doctors within 100 miles of London and a handful within 100 miles of Manchester. There are also some in Scotland. If there is no one in the NHS able to assist you in finding a doctor, I would just try calling all of the British doctors in that directory.

#7