New member from NZ

[BraveKat]

Hi all! Thank you for accepting me into this incredible community and resource. Disclaimer: this is long and my brain fog/inability to focus has probably led to many typing mistakes.
I am an almost 40 year old who has been experiencing pain on my left upper body for over 10 years as well as a host of what I had thought were odd unrelated symptoms. Prior to this I have had ongoing reflux and oesophageal motility since I was born, as well as frequent ear infections during childhood, and ongoing left ear sensitivity. Since I can remember, my throat makes a noise when I swallow (small or large gulps) which is quite embarrassing in a quiet room. I’ve learnt to tilt my head down to try to do it more quietly. I also have prominent visible cartilage of the throat and a very visible female Adam’s apple - again something I’ve been incredibly self conscious of since a young tween. I have a tight feel on the join of the jaw to the neck on my left side, like I have a noose around my neck on one side, but I can’t remember if this was experienced following removal of my wisdom teeth about 11 years ago or before that. I have a cracking sound in my ears when I swallow too, and have struggled to put on weight my entire life thus far. I sit between 41.5 and 43kg and even while pregnant with my son, only got to around 52kg. I am short in statue so it kind of works but also something I’ve been self-conscious of as a young teen/older adult as I imagine most would assume I have body image issues. I eat normally but have never enjoyed the experience much, as food gets stuck mid way down my oesophagus when I eat, and takes numerous swallows to continue on its journey. Over the years I’ve learnt to avoid wheat as it sticks more than other things.
Anyway fast forward to 10 years ago when I started to get left rib and shoulder pain, as well as an inability to open my jaw, feeling like I can’t clear my throat properly and a sudden inability to have hot showers or sleep on my stomach. Followed by constant ache in my left temple, left ear, left jaw, bottom teeth, inside mouth under tongue on left side, left eye strain (particularly when turning my head), left cheek and area between eye and nose on left, muscles and ligaments down left front and side of neck, left back neck, and a general feeling of the back of my head and face pushing toward each other and crushing anything in their path. I have brain fog, my voice is strained after only like use, I sweat profusely when I am cold or the slightest bit anxiety (eg. I have an appointment to get ready for), and feel like I am so limited with what my body can withstand these days. My heart rate is constantly elevated ~80-90bpm at rest, even when sleeping, and then higher when moving. I went to the hospital this week as I was woken by it at a very high rate, admitted into the emergency department with it at 150bpm. I was later discharged because my ecg and bloods were normal and blood pressure only slightly low.
I am at my wits end. I have seen respiratory, heart, oral, spine, muscular skeletal and ent specialists. All imaging (and there is a lot) are unremarkable except for an elongated styloid process on the left side at 34mm. I was told I don’t have eagles because my tonsil doesn’t hurt when palpated. I’ve been driving my health journey because no one else is. I’m constantly blown off and made to feel like I’m just anxious - one female doctor even said I needed to see a psychiatrist when I explained all of my symptoms to her at once, and that was the end of that consult.
A few years ago I have had an episode where food, water, pills were getting stuck in my throat, gastroscopy was clear so I decided to go on a soft food diet for a week and then alkaline diet for 5 months which helped. I also feel like I have TOS at times and have found some TOS exercises to help with pain. But MRI of brachial plexus was clear.

I feel stuck and very alone - despite having loving family and friends.

I managed to get a consult with a neurologist at the end of my recent hospital visit this week who said she didn’t think my symptoms are neurological but that she’d be happy to be a point of care for me and has organised a “ MRI brain and cervical spine and neck with contrast and neck and intracranial angiography and venography”. I have already had a MRI brain and MRI full spine as well as CT of the neck, the latter showing the “elongated styloid process with calcified stylohyoid ligament suggestive of eagles syndrome”. And have had about four other mris and one other ct in the last 10 years. Do I really need these images and to be exposed to more harmful waves? Or should I just push for surgery with someone who recognises ES based on symptoms and elongated styloid?

I also have a consult with a vascular specialist in 5 weeks but I’m struggling to use my head/neck at the moment due to so much pain (being in bed is best right now). Again do I need the vascular input or is it better to push for surgery instead?

I am very fortunate to have private medical insurance and so my consult and imaging to find the elongated styloid was through self-referral and my query about Eagles or Ernst Syndrome. I just don’t have any medical knowledge other than the internet so it’s hard to know what direction to take next. I believed the ENT specialist when he said I didn’t have ES because of my non-existent tonsil pain. So 2 years on, im still in the same place.

Any thoughts would be so greatly appreciated.

Apologies for the length of this…x

[Isaiah_40_31]

Welcome to our forum @BraveKat! Your screen name says it all - You are brave for going through what you have & continuing to persevere to the point of getting the help you need. Also, long posts are fine here. Sometimes it takes a few lines for a person to share his/her story.

The symptoms you’ve listed sound very neurological to me except brain fog which is often caused by vascular compression by the styloid of either the internal jugular vein (IJV) or internal carotid artery (ICA). The most common scenario w/ the IJV is it gets squashed between the transverse process of C1 & the styloid though there can be other tissues responsible such as muscle, nerve, scar, lymph & even vascular which contribute to the problem. This is also true w/ the ICA but most commonly it’s the styloid itself that causes the problems there.

Your larger Adam’s apple could point to something off w/ your thyroid cartilage (i.e. excess of it) which can cause clicking sounds & noisy swallowing. The pain in your left temple, ear, jaw, space between eye & nose, & bottom teeth sound like trigeminal nerve irritation; under tongue is likely glossopharyngeal nerve, & eye strain can be facial or trigeminal nerve but also indicative of vascular compression. The pains in your neck are probably being caused by your accessory nerve and the crushing sensation you feel between the front & back of your head plus brain fog sound like vascular compression symptoms. Vocal strain, profuse sweating, anxiety, digestive issues, & crazy heart rate can all be related to vagus nerve irritation. The nerves I’ve listed are among those most often irritated by elongated styloids so I suggest that the ENT who said you don’t have ES because your tonsil doesn’t hurt is incorrect. Not everyone gets a sore throat or sore tonsils as a result of ES. Symptoms have to do w/ the angle, curve, thickness, how pointed or twisted the styloid is as to which nerves are affected & which symptoms a person gets. Styloid “architecture” is often not taken into account by doctors as they only look at the length, & length alone often doesn’t tell the whole story.

@BraveKat

one female doctor even said I needed to see a psychiatrist when I explained all of my symptoms to her at once, and that was the end of that consult.

You aren’t alone in this scenario. I’m sorry this happened to you!

@BraveKat

I managed to get a consult with a neurologist at the end of my recent hospital visit this week who said she didn’t think my symptoms are neurological but that she’d be happy to be a point of care for me and has organised a “ MRI brain and cervical spine and neck with contrast and neck and intracranial angiography and venography”.

I’m glad you’ve found a doctor who is willing to be a point of care for you. HOORAY for a medical advocate!! Of the tests she’s suggested, I think the most diagnostic would be the angio/venogram, but it needs to be done with manometry i.e. blood flow pressure gradients need to be measured along the catheter’s course in each test as this is telling for compression or blockages. It’s also best if both the angio & venograms are done through the groin.

The neck CT w/ contrast could be helpful in identifying whether you have abnormal/excessive thyroid cartilage &/or hyoid bone syndrome which is elongation of the greater horns of the hyoid bone. Since you’ve already had spine & brain MRIs perhaps the neurologist could review the scans you’ve had to see if she sees something the radiologist who previously read them missed.

@BraveKat

Again do I need the vascular input or is it better to push for surgery instead?

I think the vascular input is important in light of some of your symptoms, however, the vascular doctor may be a dead end as well so be prepared for that.

ES skull based surgeons are often the doctors who do ES surgeries, & in cases w/ vascular compression, they will have a vascular or neurosurgeon assist. You may need to look for this combination to get the best surgical results.

We have many papers in our list of research papers that talk about ES with vascular compression (IJV or ICA). Perhaps printing one or two that have information relative to your situation to present to your neurologist could be helpful in getting you pointed to the right specialists for treatment.

Research Papers

[Blodyn]

Hi BraveKat, just want to say really feel for you with the terrible time you’ve had all this time, but glad you found this forum as it’s excellent and at least you’ll have more info and hear others’ experiences and hopefully not feel so alone with it. Wishing you all the best with your investigations and conclusions & the right course of action. Take care x

[Jules]

I think @Isaiah_40_31 has said pretty much all I’d be able to contribute…I do agree with you that you have had plenty of testing & obviously your CT shows the styloid & calcified ligament! MRIs don’t use radiation so that’s not an issue, but ones with contrast usually use gadolinium as the contrast medium which can cause side effects, so might be an idea to read up on this before you make a decision- we’ve had some info posted on here by @vdm in this discussion:
MRI contrast risks / Gadolinium toxicity - General - Living with Eagle
A CT does expose you to more radiation, but uses a different contrast medium & doesn’t cause issues…It would be helpful to see if there is any vascular compression, although this doesn’t always show depending on whether you get worse symptoms with your head in a certain position. Surgery is really the only ‘cure’, so in that sense, yes you would need to consider pushing for that.
It would be wise to to get your hyoid looked at in imaging, as we’ve had several members who have had clicking in their throat caused by elongated hyoid bone processes rather than the styloids, so technically hyoid bone syndrome. It can also be caused if the stylo-hyoid liament is calcified near the hyoid bone end (it’s more common to have calcifications at the top/ styloid end) , or it could be that yours is calcified the whole length.
Do you know of any doctors in your private healthcare group who treat ES? Are you able to see one of the doctors on the list? They both seem to be head & neck surgeons so good for both styloids & the hyoid bone if needed…otherwise I did find this research paper online:
saskia_boogaard_-_eagle_syndrome.pdf
I hope that you can get some treatment soon

[BraveKat]

Hi again, wow one day on here and I’ve learnt so much. Following your detailed responses I managed a bit of deep dive learning on how to read brain ct scans - thank you youtube. From this i think i managed to find the ct images showing my elongated styloid, which I’ll attach below if i can. I’m pretty sure I can see that the angle at which the left styloid has calcified is compressing the IJV (as you suggested @Isaiah_40_31 ) against my C1 TP. The right side by comparison is on a completely different angle. The left IJV seems very dilated compared to right. Even above and below the compression point.

Do you think I am reading these correctly?

Thanks so much

Also it appears that my face through this area is extremely asymmetrical. This is how if feels for me. Like I don’t have a centre point, haha. Do you know if swelling is common as a response to the compression?

So very appreciative of all of your support, teachings and best wishes - @Isaiah_40_31 @Blodyn @Jules

@Jules, in NZ luckily most specialists are affiliated with my insurance company so to be honest, it’s quite open as to who is ‘available’ to see. I think for me here, it’s more about who has the most experience and has an interest for this particular issue. I have come across a Craniofacial Plastic Surgeon in our capital city (plane ride from Auckland) who has done a ES surgery in the past and is very happy to help me, so I may pursue this contact along with the ones you provided. Feeling a bit happier now I feel like I have more of an understanding as to what is going on and thoughts on how I would like it to move forward. Until now, I had felt like I didn’t know what to do next. What were the best next steps as it still seemed elusive.

[Chrickychricky]

@BraveKat It looks to me that both sides are flattening out against c1 transverse process. On one side you can see the bright spot of the styloid but not on the other side. It could be the particular slices you posted or it could be that something other than the styloid is causing compression such as digastric muscle.

[Jules]

I’m not an expert, & am rubbish with tech so can’t annotate your images, but looking at them I think it looks like the C1 process which is compressing the IJV more than the styloid perhaps. It does look compressed, but not as much as some images we’ve seen on here, so removing the styloid could give it enough space…it’s certainly something to discuss with whichever doctor you see. Also good to ask if they remove the styloid right up to skull base, the higher it’s removed the better the chances of freeing the IJV & giving you good results.
We have info in the Newbies Guide Section about surgery & suggestions of questions to ask a doctor, here’s a link:
ES Information- Treatment: Surgery - Welcome / Newbies Guide to Eagle Syndrome - Living with Eagle
I’m glad that you can chose a doctor, if there are more than one to chose from!

[Isaiah_40_31]

Good work, @BraveKat! You did an admirable job in seeing things in your images.

I agree w/ @Chrickychricky that it looks like both IJVs may have some compression. I’ve slightly annotated your 3rd image with arrows.
Red point to the IJVs - you can see they’re flattened & not round as they should be.’
Green point to what I think are the styloids
Orange point to the transverse processes of C1

I’m glad you’ve already found a doctor who is willing to work with you. That’s a great beginning!

[BraveKat]

Thank you all for taking a look at my imaging and providing your thoughts. Yes I also saw the compression of the right IJV but decided to turn a blind eye haha, oh boy. I’ve just had 5 days in the hospital here due to increased pain, head pressure, high heart rate etc, and finally got to talk to an ENT specialist on day 4. He did a consult, asked questions etc and then said he was unfamiliar with ES and thinks most of the doctors in the public system in my district will be too. And I’ve exhausted my options in private too. Luckily I still have that one specialist (the craniofacial plastic surgeon) in Wellington who has opened an urgent appointment for me after-hours tomorrow. He also looked over the ct images from above and verbally confirmed that he also saw compression of the IJV. I’m interested to hear more in the consult, especially to get a feeling on his understanding of the syndrome and how it is best treated. Fingers crossed he knows more than me at this point.

I did have another ct with contrast in the hospital of head and neck two days ago which apparently (as per the radiology report) found nothing remarkable but I’m intrigued to see the imaging. I imagine the compression is far worse than it was in my 2022 scans. My symptoms seem to relate to ICA compression too now

Also as an update, Dr Mark Izzard from the NZ specialist list has retired so no longer an option. But perhaps we can add this new specialist if it all works out,

[Isaiah_40_31]

I’m sorry you needed to be hospitalized @BraveKat, but I hope the treatment you received helped calm your symptoms down.

Scary that you may also have ICA compression. It’s rare to have both IJV & ICA but not unheard of.

I’ll take Dr. Izzard off the Doctors List. Thank you for the update. Please do let us know how your appointment goes tomorrow. I’m really glad you’re seeing a doctor who seems to have some understanding of ES & has acknowledged your vascular compression.

[Jules]

I’m sorry that Dr Izzard has retired, it seems sometimes we lose doctors off the list faster than we find them! And sorry for you that you’ve been in hospital, I hope the doctor in Wellington can help you, let us know how you get on

[BraveKat]

Just a quick update for anyone interested now or in the future, that I met with the specialist in Wellington, Dr Charles Davis (craniofacial plastic surgeon) and he agreed it’s time to remove this thing (my calcified ligament). He said he’d remove it and part of the styloid process leaving only part of the SP. He won’t be using any large instruments so would snap it off (with the ligament) near the base. Which sounds in line with recommendations on here. My first thought was that I hope it’s not jagged or sharp but he explained the close proximity of the SP to the pathway of the nerves coming through the skull from the brain there - so didn’t feel I needed to question him on this. He also said he’d only do a small incision at the tip of the calcified ligament near the left jaw and then just follow it up to the skull base, carefully working past all the other important structures around it. In his mind, the surgery is relatively simple compared to other surgeries he has to do, like facial reconstructions and tumour removals where the jaw needs to be broken etc. He said he’s done two ES surgeries in the past and both patients recovered fully. He said my symptoms were a bit different to theirs but the compression of the veins and possibly artery means they would be. He said he can’t guarantee all of my symptoms will resolve but that it needs to be done to find out. I’m so happy to find someone who is unfazed by working in this area (previous ent specialist who said the it was unlikely I had it also tried to scare me off the surgery due to the risks and the large incision it would require to operate). He also said he was happy to try to fit me in as soon as possible. Either 2 or 3 weeks from now. I’m aware that there can be complications like all surgeries but feeling so grateful to have finally found someone in NZ who is interested to help me and comfortable with this kind of surgery.

I was conscious going into the consultation that it was important that I let him lead the conversation and also feel out his personality as we talked through his questions to me and suggestions, to see if it was a good fit. I did enquire about whether my right side might also be an issue, but his feeling is to just start with the one side first. He explained the anatomy of the area and the possible nerves that could be affected by the calcified ligament and how these relate to my symptoms which was great and of course again in line with the thoughts/feedback I’ve received on here. All in all, a good outcome at this stage. Just waiting for paperwork to be sent to insurance - fingers crossed for cover.

I do have an independent consult with a vascular specialist in mid-December. Would love to get feedback on whether I should pursue with this. Currently thinking I could push it back til 6 weeks or so after surgery with the desired outcome for the Vascular specialist to ultrasound the veins/arteries and check flow rates post surgery. Keen to hear thoughts on this and whether it is necessary. Thank you

[BraveKat]

Oh also, he obtained my ct scan with contrast imaging from my recent hospital visit last week, and lo and behold, they didn’t even do a neck scan to look at the dilated and impinged veins like they said they would do to check for dissection. They just did the head. In the hospital, I questioned the Gen Med. doctors overseeing me when they said everything came back fine. I said did you look at the imaging because I find it difficult to belief nothing has changed over the past two years despite symptoms intensifying and being added to. I asked how the impingement now compared to my 2022 scan. To which they replied, they don’t read the scans (and don’t know how) and rely on the radiologist report. I am so baffled by this. I just want to relay this experience as it really opened my eyes to the process. It’s very important to get hold of your health information and look into it further if you feel something doesn’t add up/may have been overlooked. Listen to your gut and get second opinions when you feel it’s needed. Or seek out a specialist who knows more and can read imaging.

[Jules]

It’s always a difficult decision having surgery & which doctor to have surgery with…it’s good that you feel confident with him, & I appreciate that it’s been hard for you to find a doctor in NZ, I just wonder if you would be better to hold off if you can & see the vascular specialist…if you do have IJV compression, and possibly ICA compression, then this is a trickier surgery than classic ES surgery, and to be done safely I would want the doctor to have as good a visibility as possible (which is why research suggests that external surgery is usually safer). For a surgeon to feel up inside your neck & to then snap the styloid off sounds a little concerning given how close the styloid is to major blood vessels and nerves, your thought about the end being jagged or sharp is a valid one, that’s one of the questions we suggest that people ask- if they smooth the tip off as we have had members still have symptoms from a broken end.
If you didn’t potentially have vascular ES I’d be less concerned, I don’t want to put you off but it is a tricky surgery & you don’t want to rush into it

[BraveKat]

Thank you @Jules for your thoughts - it’s so very helpful. Taking on board your advice, I managed to move the vascular specialist appointment forward and had it yesterday. Only to be told as soon as I walked in the door that he had reviewed my imaging and nothing is wrong. Hmmm, I obviously took out my 2d ct scans and showed him the compression of my left IJV and pointed out my calcified ligament and he just put up his hands signally defeat. I asked with the compression in mind if he thought I’d benefit from the peak flow analysis and again he said you know more than me on this. I’m blown away, again another eye opening moment for me about the general knowledge of health professionals. I really didn’t realise the specific degree of specialisation there is within each department of the health care structure, and how this leaves so many gaps in the care of individuals health issues. I had thought it would be general knowledge to understand what a compression of a vein looks like in the vascular specialist field. Quite mind blowing.
Anyway he said I’d have to go to Germany or such for more information if I wanted it.
It was good to hear it though, and know that right now my best option for treatment in Nz is with Dr Davis. The vascular surgeon also responded to some other questions I posed, and made me feel comfortable that aside from the obvious risk of working with sharp objects around the veins, there shouldn’t be any worsening symptoms from removing the ligament compressing it/them, even if it was dissected currently.
Maybe he was giving me the kiwi ‘she’ll be right’ attitude, but for now, I’m satisfied that my vein related queries have been answered.

[Jules]

Oh, wow, how could a vascular specialist not be able to see the compression & know what testing is available I’m glad that you feel reassured with questions answered to go forward with the other doctor you’ve seen, Dr Davis…surgery would be quite soon I think you said?

[BraveKat]

Hi @Jules, thanks for remembering. Yes surgery tomorrow.

My only concern at this stage is how the removal of the ligament will affect my overall head stability post-surgery. Through all the imaging and tests I’ve had to date (full spine mri, Ct of head and neck, brachial plexus mri, brain and jaw mri, neck X-ray), nothing suggests c1/c2 rotation or instability but I’m assuming the calcification of the ligament as a need to help provide further stabilisation of the skull to jaw connection, is due to some weakness in my neck (or spine generally). So removal may further weaken the joint at the head to neck. Of course, I can only push forward and see, while continuing to uncover where the other weakness is stemming from and working on correcting this. I cant live with the constant pain, tachycardia, pressure, dizziness etc so am happy to have this surgery.
Will update post-surgery

[Isaiah_40_31]

I had a fair bit of ligament calcification, too, @BraveKat & have not noticed any change in my cervical stability now that it’s gone. I am also in your situation w/ no c1/c2 instability as far as I know. I believe my ligaments calcified due to a whiplash injury when I was 23. My ES symptoms didn’t show up until I was 57.

[BraveKat]

Oh that’s good to hear.

I do think mine, as the simplest reasoning, could have been caused by my wisdom teeth removal surgery - I haven’t been able to open my mouth very wide since then and my other symptoms have all started since then - so will wait to see what happens. Fingers crossed it’s that simple - just trauma from the surgery.

Though my cervical spine is also unhappy around c5-t1 (some disc bulging and frequent cracking of bones in this area when I move my arms etc.) hmmm

Hopefully surgery is the ‘magic pill’

[Jules]

Praying for a good outcome for you @BraveKat