Glad to have found this community. My name is Xander, and I’m a 20 year old male. Based on the accounts I’ve read here, I am 100% certain I have this condition. I have been treated for conditions that I believe were just results of ES (namely extensive orthodontic treatment for TMJ) and have had symptoms brushed under the rug for the last two years.
Cognitive decline (brain fog, memory issues difficulty articulating my thoughts)
Feeling of having a tumor in my throat (right on my parotid gland)
Limited neck mobility - symptoms worsen when turning head sharply
Severe ear pain
Vision anomalies (mainly eye floaters. I’m only 20 and my vision is basically filled with floaters).
Neck instability (my head feels too big for my body)
TESTS I’VE HAD:
Brain & Neck MRI (normal except for mega cisterna manga which they said was congenital etiology that required no further investigation)
Neck ultrasound (they also evaluated carotid blood flow here, normal)
Thyroid tests (TSH, T3, T4 all normal)
Abdominal CT scan (normal)
Kidney panel (normal)
Liver panel (normal)
Visual acuity test (normal)
24 hr holter monitor (normal)
The only two other possible conditions that could possibly be causing my symptoms are either ES or Idiopathic Intracranial Hypertension. Oddly enough, I saw a few members on here with both so I’m wondering if there is a correlation between the two.
My family thinks I am a hypochondriac, as does my PCP at this point. I, however, know something is off in my body and will continue to push for a diagnosis. My next test will be an extensive eye exam where they actually dilate my pupils to rule out papilledemas that may be a result of IIH.
That being said, I do believe ES is my true condition as the one symptom I do NOT have is headaches. Headaches are the presenting symptom in almost every IIH case I’ve read, and assuming my eye exam checks out, I will be focusing my efforts on pushing for an ES diagnosis.
If any of you have advice on getting a diagnosis I would greatly appreciate it. I am running low on funds due to countless copays so I want to make sure I am investing in the proper tests. Also, if you guys have any insights as to what my symptoms could be then I’m all ears because doctors have not been any help unfortunately.
Hi Xander & welcome to the site!
The best test for getting diagnosed with ES is a CT scan of the head & neck, down to the hyoid bone. It’s best to request that it’s evaluated for ES, & the the length, width & angle of the styloids are noted. As well as elongated styloid processes, the stylo-hyoid ligaments can get calcified & cause symptoms , so that needs checking.
If you suspect that you have IH, it can be caused by the styloid processes compressing the jugular veins, so a CT with contrast would be an idea, it might confirm it (although sometimes this only happens with your head in a certain position )
There are a few discussions at the moment by members with IH; if you search for jugular compression it’s probably come up with discussions you might find helpful.
Also there’s info in the Newbies Guide Section about Vascular ES; here’s a link:
So try to get a CT done, & then take it from there- if you can get copies of the images & the report, as it’ll be helpful to send to any doctors if you do get a diagnosis, plus if you want to you can always post them on here.
Hope that you get some answers soon…
Thank you so much for your prompt reply. It definitely seems like a neck CT scan sent to a doctor who specializes in ES is the way to go…
However, as @vdm pointed out, it looks like panoramic x-rays are also capable of spotting ES? I had my braces removed a year ago and I vividly remember getting a panoramic x-ray! Wondering if requesting a copy of it would be smart?
Then perhaps I can send it to one of the listed doctors. I’ve had lots of tests done in the last 2 years - way too much radiation and would like to avoid any more if possible.
The type of doctor that usually treats ES is a skull-based ENT surgeon, neurosurgeon or maxillofacial surgeon. These doctors are often cancer specialists. Understanding the type & thoroughness of a doctor’s surgical approach is important. We recommend the external (vs. intraoral) approach as the styloid can be removed back to the skull base & the stylohyoid ligaments, if calcified, can be removed from styloid tips to hyoid bone which provides the best long-term recovery from ES symptoms. Nerves & vascular tissues can also be better monitored via the external approach post op infection rate is lower.
A CT scan is the best way to diagnose ES. As Jules said, your CT needs to show the area between your hyoid bone & skull base focusing on the styloid processes & stylohyoid ligaments. Taking a look at your panoramic x-ray first to see if a CT is warranted for official diagnosis is a good idea. A CT w/ contrast will show vascular constriction when your head is in the provocative position (the position that brings on the dizziness, etc), but some doctors prefer a CT w/o contrast as it allows them to better visualize the styloids & stylohyoid ligaments. Calcification on your stylohyoid ligaments, will show up in a CT & presumably a pano x-ray, but the ligaments themselves will be invisible - only the calcified sections can be seen.
The symptoms of ES are caused by irritation of up to 6 cranial nerves that “live” in your neck in the same area as the styloid processes & stylohyoid ligaments. Elongation of the styloid(s) &/or calcification of the stylohyoid ligaments can cause irritation to these nerves. Alone each nerve when “upset” can create painful & sometimes scary symptoms. When more than one is irritated the symptoms caused can be downright debilitating. Additionally, vascular compression of the internal carotid artery &/or jugular vein can occur which creates other miserable & concerning symptoms. It does sound like you have vascular symptoms based on the symptoms you get when you turn your head. Obviously, the solution for recovery is to remove the offending bones/ligaments so the nerves &/or vascular tissues can recover. There is a series of YouTube videos called Two Minute Neuroscience . Each cranial nerve is featured in a two-minute video w/ explanation as to location, function & dysfunction of the nerve. It would be good for you to watch them. These are the nerves affected by ES: vagus, facial, glossopharyngeal, hypoglossal, trigeminal & accessory. Hopefully the information you receive will help you understand the source of some/most of your symptoms.
Wow, so much great information here. Thank you for your input as well @vdm - I will be requesting copies of my most recent panoramic x-ray first thing Monday.
What you said about jugular compression is interesting, Isaiah. However, I was under the impression that the neck ultrasound ruled out jugular compression because it showed healthy blood flow? Perhaps I may be wrong but I’d definitely love to hear your follow up on that.
Symptom-wise I feel like I might have a combination of both going on but the jugular compression/anything vascular petrifies me. My biggest worry is sudden death from stroke/aneurysm.
Two things, 1) Either the internal jugular vein OR internal carotid artery can be compressed by an elongated styloid. Vascular symptoms will be similar between the two. A neck ultrasound is a fine diagnostic tool, but if your head is not in the “provocative” position when you get the ultrasound your blood flow will appear to be normal. When your head is in the position where you note symptoms getting worse, then the ultrasound may show decreased blood flow. 2) If your vascular tissues were compromised enough to cause a stroke, your vascular symptoms would most likely be debilitating at this point. Our blood vessels are pretty resilient. Additionally, the compression caused by the styloids is often intermittent (based on head position) which means pressure is not always on the vessels in question thus they get respite periods to recover.
I’d suggest to request all the pano xrays you can, not only the latest. Some of them might be wider angle than the others and show more around the areas you are interested in. Also sometimes the xray can be too dark to display thin calcifications clearly or separate them from the other adjacent bones, depending on the xray exposure time. So having multiple pics would obviously increase your chances of finding anything if there is anything to find.
A stroke is very rare, I hope that is reassuring…
JustBreathe is very knowledgeable about IH/ Jugular compression, she posted this in another discussion:
Here is a bit more information to start.
Velocity of greater than 50 are considered abnormal. Diameter readings are usually compared from the jugular foramen, C1, and a few points below to the omohyoid muscle looking for stenosis. There is little evidence describing normal active data.
Keep in mind that this testing is very limited for a few reasons:
it can vary greatly depending on the sonographer’s technique and pressure applied to the probe
more importantly the readings are taken in supine which does not correlate to the functional position of being upright. Gravity influences the IJV greatly.
While it does have value it is not usually the primary form of imaging but a backup. CTV, MRA/MRV, and venogram are more reliable sources of IJV information but again are all done in supine.
Hope this is helpful!’
It’s in this discussion if you want to find out more:
I requested my old pano after a doc suggested the eagles as a cause of my pain. I specifically asked for the styloid area to be reviewed. Low and behold it did show some calcifications and elongated styloid. The oral health guy was dismissive. This gem was ignored 5 years earlier. It wasnt the best pix but was enough to support my request for CT scan that confirmed the diagnosis of ES.
Xander, I have or had many of the same symptoms as you. TMJ, neck instability, etc. Have you ever been told you were hyper-mobile? Many of us with neck instability have what is called Ehlers Danlos Syndrome Type 3 - hypermobility. It has been the cause of my neck / jaw issues from a very young age. Also had MRI’s, x-rays, you name it with no findings so dont let others make you think all in your head.
Thank you all for providing all of this information! I feel like this forum alone has been more helpful than my own doctors, unfortunately. The orthodontic practice I got my x-rays done at is closed (due to covid I’m assuming). That does put a major monkey ranch in my plan to get a diagnosis but I will be persistent.
Snapple, I have read up on EDS. I have all symptoms except for the increased flexibility.
One thing I didn’t note was my slightly translucent skin. I can see my veins very clearly around my eyes and especially my eyelids.
thin translucent skin is associated with EDS. Another thing associated with EDS is POTS (Postural Orthostatic Tachyhcardia Syndrome / Dysautonomia), Not so common in males. 50% with POTS have EDS. My daughter has it so I know something about it. Ive lost my flexibility as I have aged.
You may be able to leave a message or talk to ortho service to get your records even if they are closed. They should have an emergency # to call?
“A heart rate increase from horizontal to standing (or as tested on a tilt table) of at least 30 beats per minute in adults, or at least 40 beats per minute in adolescents, measured during the first 10 minutes of standing”
“The symptoms of POTS include but are not limited to lightheadedness (occasionally with fainting), difficulty thinking and concentrating (brain fog), fatigue, intolerance of exercise, headache, blurry vision, palpitations, tremor and nausea”
Some people with EDS have tendencies towards something called Chiari-malformation which can cause unusual pressure on skull. That would most likely be picked up on MRI of skull.
My daughter started passing out at age 7. Around 9 she developed migraines and was exercise and heat intolerant. Her eyes are hyper-sensitive to the sun. She has the milky skin you talk of. It took a geneticist to give an official diagnosis of the EDS although there is no true blood test for it. Its based on “Breighton” scores. It is a gray area for diagnosis.
A CT scan will confirm if you have Eagles. If you have that kind of heart rate jump while standing, I would suggest you get an evaluation by a doctor (usually neurologist) who specializes in dysautonomia. Just like with Eagles you want to seek out experts or those with experience. I hope this helps.