Hello, I am pretty new to ES but not new to understanding the symptoms and terminology. Here’s my situation and I’m looking for advice on where to begin and what type of doctor you’d recommend on the Western side of the US. I live in North Idaho but have family in So Cal, CO, and WA, but I will go where I need to.
My styloids are not considered long enough for a proper ES diagnosis, but my CTA showed at least the tip of my right styloid compressing my IJV, and I have bilateral symptoms that match up with ES, so I’m wondering if docs will even take me seriously if my styloids aren’t long enough? They’re only 1.7 and 1.8 cm, but the radiologist did tell my dr that due to their angles and my anatomy, they could be causing my issues, but he didn’t write that in the report, of course! But my Dr said it’s partially c1 compressing IJVs and partially the styloids, so he also thinks I may need part of c1 removed. Where to even begin? He doesn’t have any Dr recommendations for me at the moment, as this is not his area of expertise, he just happened to be able to see what was going on.
Symptoms:
Bilateral severe neck pain at base of skull behind ears.
Sharp pain below jaw in front of neck and nerve twang when I turn my neck too fast either way (pain on whichever side I’m turning towards).
Clicking in sides of neck in the front, an inch or so below jaw.
Intracranial hypertension.
Tinnitus.
Hearing pulse in ears.
Vertigo.
Swallowing diffculties.
Swollen submandibular and parotid glands (for years).
Lump in throat feeling.
Nerve tingling in left cheeckbone.
A host of vagus nerve symptoms and dysautonomia.
And here’s the biggie: I cant lie down at all anymore ever because it feels like I’m being strangled with intense head pressure and violent vertigo, and I feel like I will black out. It has been 6 months of sleeping sitting FULLY upright and I am at the end of my rope already and can barely hang on. My doctor just did CTA and discovered the jugular compression.
You’ll see my scan shows bilateral IJV compression on my back, and when my head is rotated to the right it shows my left IJV compressed. Also, what else do you see in the images?
@BlacknBlueSaint - I’m really sorry for the many symptoms you have & for your sleeping difficulty. Many of our members w/ vES have found they need to sleep w/ head elevation. Another thing that will help you is to get an Rx for a blood thinner such as Plavix. Taking a blood thinner while awaiting surgery has been very helpful for a number of our vES members in reducing symptoms.
I upgraded your status so you can post your images now. I explained in a private message that our system, in order to protect us from spammers, won’t allow a new member to post images at the get go, but as moderators, Jules & I will in some circumstances, do the status upgrade for a new member.
You’re not alone in having IJV compression w/o elongated styloids but nonetheless caused by the styloids due to the angle they’re growing. Though elongated styloids are a something most doctors look for in order to diagnose ES, elongation isn’t the end all. Thickness, angle of growth, how pointed or twisted the styloids are even w/o elongation can allow them to cause symptoms.
The clicking at the sides/front of your neck could indicate that you have calcified stylohyoid ligaments &/or the greater horns of your hyoid bone could be elongated. Hyoid Bone Syndrome has many symptoms in common w/ ES but it’s something most radiologists don’t look for when evaluating styloids. Did your radiologist make any note of your stylohyoid ligaments?
If you need C-1 to be shaved, the only doctors on our list who do that are Drs. Costantino & Lo in NY who work as a team & Dr. Nakaji & Dr. Lawton in Arizona. There may be others in the U.S. who do this, but those are the few we know of at this time. Dr. Hepworth in Denver is masterful at working w/ IJV compression, & it has been mentioned that he’s moved the IJV away from C-1 in at least one case to avoid the need for vertebral shaving. You would need to confirm this w/ him if you do make an appt w/ him. Even though he’s not currently taking new patients, it would be worthwhile calling his office (an exercise in patience trying to get through to them as they’re short-staffed at the moment), & getting on a wait list for when he’s taking patients again.
Thank you sooooo much for helping me and giving me all the info you have tonight! I so appreciate it! I am going to start with contacting the Dr.s in AZ, as that would be closest! I will edit my post to include the images in a little bit. Also, I have Isaiah 40:31 printed out and framed right next to me! Haha. I love that one and it is the hope I am holding onto!
Also, no mention of stylohyoid ligaments in report, unfortunately.
Hi & welcome!
So frustrating that the points the radiologist mentioned to your doctor aren’t included in the report! But doctors quite often look at your scans themselves anyway, so hopefully this’ll happen and they’ll see the issues! (Can’t comment on those as I’m not that good at reading the CTs, but hopefully some of our very skilled members can look for you!)
I had bilateral IJV compression but no C1 involvement, so I was lucky & just having the styloids out made a massive improvement to my symptoms. I was feeling pretty rough before the surgery though, so I can sympathise with you on that front… @Isaiah_40_31 has given you info about the doctors, I hope that you can get somewhere with them & get surgery soon.
Based on the images you shared, I would say the jugulars show a mild compression. Based on your symptoms (pulse in ears and can’t lay down) I would say we need to look higher up in the skull at transverse sinuses or superior sagittal sinus.
If possible please upload your scan to https://www.dicomlibrary.com/ which will anonymize it. Then post the anonymized link here. There are some really well trained members on this forum that can read these scans better than most radiologists and at least give you a baseline opinion or point out areas of interest to focus on.
Also could be lower or a compressed artery, but we won’t know without seeing the full scan.
Thank you for the info and help! I am only able to access my images through the patient portal for now, but they are supposed to be mailing me a CD of the scan, so I can upload it then and post the link here when I have it. Thanks again!
I’ll just add in to this we’re not doctors on here, but some of our members have studied anatomy so that they can read their own scans & advocate for themselves, and have been really good at spotting things that aren’t mentioned on radiology reports!
I totally understand, and I’m definitely going to see a doctor, but in my experience doctors miss so much because they don’t take the time to really look around, so hopefully some of the group members will see my post and chime in with their thoughts.
I don’t think it would relate to ES if the compression was higher up and isn’t caused by a styloid. My right styloid is for sure part of the IJV compression I have, and the C1 is the other part. My images might not show the extent of compression I get, which is positional, so they might not show much compression in neutral head position, but when I turn my head all the way or lay on my side, I am most likely getting much more compression because I almost black out.
Like @BlacknBlueSaint said, it doesn’t have anything to do with Eagle’s because that implies the involvement of the styloid. But a compression of a major vein or artery can occur in multiple locations throughout the head and the symptoms are similar with regards to head pain with positional changes, like lying down.
In the supine position blood flow increases to the head. If there is a major venous compression/blockage there will be increased CSF pressure and buildup, leading to IIH and the associated symptoms.
Pulsatile tinnitus is commonly a sign of venous stenosis near the ear canal which would be at the sigmoid sinus or transverse sinus. Because the compression happens near the ear you literally are hearing the blockage trying to force blood through a narrowed vein timed to your heartbeat. The dizziness/vertigo side effect from lying down may be increased blood flow/pressure to the blockage, which puts pressure on the ear. But it could also be increased CSF in general, there are a lot of possibilities.
This is one possible scenario based on some of the symptoms. A lot of the nerve based symptoms described by BlacknBlueSaint indicate there is also some sort of nerve interaction with the movement of her head, which may or may not be related to the head pressure/vertigo. That is definitely not something I have researched enough to comment on.
Here is an example of IJV stenosis vs transverse sinus stenosis. Very similar just in slightly different part of the venous flow from the head.
Wow, that is very thought provoking info! I had also wondered about how CSF was affected by this, because I have heard it twice now. Once, when this first got super bad but I was still able to lie down some of the time, so I had been lying down asleep for a couple hours, then I woke up and heard in my brain what sounded like crackly fluid rushing down a drain, as if a drain plug was pulled.
And one other time I tried lying on my back for a while to see if I could tolerate it and I started to literally hear a loud constant dripping noise in the top of my neck, like a leaky faucet. So freaky!
I have told multiple doctors this and they have no idea what caused it. But if you’re saying the intracranial pressure can block CSF, then thay must be why it happened.
I have sent all my tests and records to Dr. Lawton and just waiting to see if he can help me. I’m starting to feel like no doctor will accept my case and I don’t know where to turn! Can’t stop crying all day. How do I find someone who can get to the bottom of this for me? I keep being blown off!
This is what concerns me the most. Prior to my surgery, I would almost black out if I looked down to long, say 15 minutes. But your inability to lie down with vertigo makes things much more serious in your case.
Most patient portals do allow you to download the dicom images needed to make these 2D and 3D reconstructions. Sometimes you just have to click around, but a few do not offer the feature.
Also, if I have severe hypoplasia in my right vertebral artery, can it cause all that? Sorry for the crude markup… that was to help my mom understand the picture. I have had this my whole life, and been symptomatic for a lot of it, but only recently did I become this severe.
As a kid:
Migraines
Heat intolerance
Mini-seizures that only lasted 1 to 3 seconds
Super clumsy
Tons of vagus nerve and dysautonomia type problems, like chronic nausea, waking up with trembling episodes in the night followed by a bowel movement, heart palpitations, motility issues, temperature regulation issues.
I’m also very hypermobile so that could explain lots of that, too, so it’s confusing!
Unfortunately, my portal won’t let me export any of the images or scans. I even spoke with their IT person, who confirmed that. I am trying to see if the disc is ready and I will go pick it up today or tomorrow and upoad it to DICOM library. Also, they did make some 3D models so I have attached screenshots from those here in case they show anything important.
Like so many people on this forum and others it is very confusing and there are many layers to the symptoms they suffer from. Unfortunately for you, hypermobile people do tend be more prone to vascular issues. It’s usually the first question a vascular specialist will ask.
First, these images are amazing and show the blood vessels very clearly. I see the hypoplastic vertebral artery. It’s not completely uncommon, but I did find a study that suggest there is a correlation with vertigo. I wouldn’t say that this is for sure what has caused your lifelong issues as many people have this genetic anomaly and don’t have issues, but it’s worth investigating further.
The other thing that is suspicious is this big indent into the left transverse sinus (blue circle). Because if it was always narrow like this from birth it shouldn’t balloon open further along the sinus (green circle).
The left side looks to be your non dominant side for blood flow. Although that could be because of this compression (blue circle). The right side has more blood flow and is your dominant side, but also shows compression in the same area just not as much (yellow circle). I would need to see from more angles to confirm how severe the compression is on the right side. But regardless of my opinion, you need be properly worked up by vascular specialist to asses these areas and possibly other things I have missed.
Personally, I can only recommend Dr. Patsalides as he known as one of the leading experts in this field. Your symptoms and anatomy fit the type of profile of the patients he treats. He does offer online telehealth appointments, but he is usually booked out about 2-3 months. There is a Facebook group called the Whooshers, which is reference to the pulsatile noise you are hearing that maybe able to offer other specialists to review your case sooner.
Do you hear the pulsatile tinnitus in both ears or a specific one?
Thank you for all of that information! I really appreciate you taking the time to look at those and write me back a few times! I will look into the doctor you recommended. My husband is picking up my disc with the CTA scan now, so I hope to upload it to DICOM library tonight and share the link here.
I think the pulsatile tinnitus is only in my right ear. I’m trying to remember if it’s ever been in the left, but I don’t think so.
I had pulsatile tinnitus in both ears, but worst on the left where there was more compression of the IJV, it was purely styloid compression which caused mine. The whooshers site is good!
