I found the most thorough study on Ernest Syndrome. It’s from the year 1987 and not free-to-view, but I decided to purchase and share it on this forum as it contains vital information about Ernest Syndrome, which is closely related to Eagle Syndrome. You’ll find the link down below, I’ll summarise the most important findings in this topic.
Note: Research on this rare condition is scarce, so don’t take all the information for granted - especially the possible symptoms, as there can be much more, as most Eagle patients will know. Surveys on people suffering from Eagle syndrome revealed many other symptoms resulting directly from Eagle syndrome which are not mentioned in the respective literature. This might be the case with Ernest as well.
Just like Eagle Syndrome, Ernest Syndrome also affects the styloid bone, but the main root of pain is the stylomandibular ligament rather than an elongated styloid process and/or stylohyoid ligament. It was discovered by Dr. Ernest in 1982.
In this study, 68 patients were diagnosed with Ernest according to following criteria: (1) Pain in and around TMJ, ear, temple, body of the mandible, pain in the eye, pain in the throat, pain in the shoulder, (2) pain on palpation of the insertion of the stylomandibular ligament, (3) relief of pain after an injection into the ligament.
Until those patients finally got diagnosed and received adequate treatment, they had been having pain for 4.37 years on average. You can therefore infer that Ernest syndrome is very frequently overlooked. A survey on 20 people suffering from Eagle Syndrome showed that it took them 2.95 years on average to get diagnosed. This is another parallel between Eagle and Ernest syndrome. Here’s the link to the survey on Eagle Syndrome that was conducted in this forum four years ago: ES Survey Results.pdf (120.7 KB)
Ernest Syndrome frequently occurs after some sort of neck/head trauma. 32% were involved in a car accident, 16% got a blow on the mandible. Back injuries, traction, dental treatment and intubation are mentioned as well. However, in 41% of the cases the real cause for Ernest was unknown, yet many listed in the category “unknown” stated to have had numerous traumatic blows on the mandible, but they couldn’t attribute the onset of their pain to a specific incident, therefore the cause was considered “unknown”.
Not a single patient had a normal range of motion of the mandible, 40% had myofascial pain dysfunction, 38% had internal joint derangements, 17% had temporal tendonitis and 10% occipital neuralgia.
43% of the cases involved both stylomandibular ligaments, 32% the right ligament only and 25% the left ligament only.
Treatment of Ernest Syndrome was defined as elimination of pain and restoring normal range of motion of the mandible. 78% got treated with a local injection and a placement on a soft diet. The local injection had to be executed up to 4 times with a two-week interval to successfully treat the condition. 22% got treated surgically with a radiofrequency thermoneurolosis. Whether this surgical approach has been successful and whether these patients had undergone the conservative approach beforehand, is not clearly mentioned in the study. However, I’d assume these patients had unsuccessfully undergone conservative treatment and got complete relief of their pain after the surgical approach was conducted.
Keep in mind: I’m just a medical layman, I only summarise and cummulate information I find in scientific literature. Feel free to read about Ernest syndrome yourself, I’ll put more links in a new comment below.
Here’s another study on five anterior throat pain syndromes: Ernest Syndrome, Eagle Syndrome, Hyoid Bone Syndrome, Carotid Artery Syndrome and Superior Pharyngeal Constrictor Syndrome. Fortunately, I could download it for free via my student log-in. I have yet to read it myself, but it should contain important infos: Anterior Throat Pain Syndromes Causes for Undiagnosed Craniofacial Pain.pdf (8.6 MB)
This is the only YouTube video I found about Ernest Syndrome. The differences between TMJ, Ernest, Eagle and Barre-Lieou are discussed. I’d recommend giving it a watch, it’s a short video anyway: https://www.youtube.com/watch?v=gsZ60Y0Uqaw
No worries, good luck! You have to be adamant, try to convince the neurologist that you’ve been having pain for a long time already, that this condition matches all your symptoms and that most patients are forced to self-diagnose.
Again that you, I want to the Neurologist at Baylor Scott & White Temple, TX they ordered MRI angiogram, MRI brain Head, Neck & brain. She thinks it’s a partial not full Trigeminal Neuralgia. She said my Styloid was so long and involved pushing the Hyoid bone forward, a lot of nerves were involved and may just need time to heal. But I did have head & neck injury with concussion syndrome, she wants to make sure all is ok. She wanted me to take Lyrica low dose 25mg at bedtime to start, if pain stops, then wing off. I was pleased. I think the other side should not be as bad, it’s much smaller. I am glad I requested a neuro consult.
When did you have the surgery to shorten your elongated styloid? It can take up to a year or a bit more for nerves to heal & sadly in some cases they don’t heal completely so pain, numbness, tingling, etc., can continue but often at a reduced level than before surgery. I hope the Lyrica helps you!
Thank you for sharing this information. I was diagnosed with Eagle’s Syndrome last fall and had my left styloid process removed. I was pain free until 4 weeks ago. I yawned too wide while laying down and my pain returned. It felt only slightly different, most likely because the elongated styloid was removed. I came across Ernest Syndrome again yesterday, and it fits my symptoms and experiences to a T. I’m working with a new doctor who ordered a CT for me. I messaged him asking to look into Ernest Syndrome. I’m hoping we can see something on the images and get a diagnosis!
I’m so sorry your pain has returned. Please let us know what you find out. Have you considered getting a follow-up CT scan to see if your styloid has regrown or if the other side has become elongated? That can happen though it’s rare.
Interesting, SML is something I’ve wondered about. I’ve had a very hard time finding it on my CT. The standard anatomical diagram doesn’t seem to fit my anatomy. My styloid process is further forward, it kind of runs along the length of my mandible, to the inside.
In the axial plane, if you measure an angle off the SP the SML would project out at a 100 degree angle, if 0 degrees was pointed toward the front of my face.
In a standard CT i.e. a CT w/o contrast, the ligaments are not visible unless they are calcified. If your SML or SHL have no calcification on them then you won’t be able to see them unless your CT was w/ contrast. My SHLs looked like dashed lines on my CT scan because there was intermittent calcification on both of them. That’s a little atypical, but it did make them visible on my contrastless CT.
Isaiah_40_31, makes sense, couldn’t find it on mri either, I imagine its very thin. Oddly enough the SP on my CT is not solid bone. It has some soft tissue signal running through the middle of it, do you know if the SHL is intact inside of the bone? Could be that is has fractured a few times.
Jules, I hope it all is assessed together. I’ve had so many scans and stopped taking the radiologist’s report as gospel. Funny you mention the hyoid bone cornu size, I had to have a CTA last week because of carotid pain when my neck was turned. I have my own 3D rendering software and noticed the bone is poking the lingual and carotid artery. The 3D picture really helps to understand how things are jammed together.
Nice image, sigmet25. I agree w/ Jules that the greater cornu of your hyoid looks long especially in the 3D image. Definitely something to address at your consult. I would imagine your doctor could shorten that as well when he’s in there but better to ask so you know for sure. Is your ES bilateral?
You’re smart to not take radiology reports as gospel. Radiologists are “only human” & thus subject to expressing their opinions of what they see based on their experience & education. Obviously no two radiologists will be equal in those arenas so opinions will differ which can be confusing to the patient.
sigmet25,
Interesting thought about the SHL being “encapsulated” inside the calcification. I’m sure that’s possible, but I think more often, the calcification consumes the ligament making it into an inflexible bony structure which in turn immobilizes (or partially so) the hyoid bone thus causing additional symptoms.
Michael, Thanks so much for this valuable information! I will take some time to read the studies. When this was brought up a week or so ago, It got my attention. Given I was just in yesterday with neurologist getting botox mix into my massetter and temporalis areas and occipital areas I especially am taking notice. The end of April, I had ES surgery on this side since it was the most painful. I am still struggling with ongoing pain and nerve pain. The areas I had injections are so locked up and I am having almost weekly massage, acupucture, etc. It is getting better but in my opinion, not significantly. If I dont have the massage or other interventions for a few weeks, it gets worse again. I mentioned to neurologist that it is so nice to not have pain after injections…which goes away after a couple hours when the buvicane wears off. He explained that the hope is with these injections, that with each application it takes you one step closer to final relief and gives the nerves a break. It will take a week or two for botox to kick in. I did get a higher dosage this time around and massage person said the muscles were still pretty tight after the last round 3 months ago. I did ask about radiofrequency. He thinks this is an issue with the trigeminal nerve bundle and that there are concerns about using it there and spoke about its use above and below the areas on nerves that lead into it. Ive had major tooth sensitivity and about 8 root canals. After struggling for so long, you do get to a point where you just want to severe the nerve causing the problems.
