Eagle Syndrome Treatment – Surgical and non-surgical approaches
Ross Hauser, MD
If you have landed on this page, it is very likely that you have been on a medical journey of discovery for many years. What you are obviously trying to discover is the mysterious and seemingly untreatable group of symptoms you have. As you will see in some of our sample stories below, you are not alone in this. It is extremely rare that someone will be given the first diagnosis of Eagle Syndrome or Stylo-Jugular Venous Compression Syndrome or stylohyoid complex syndrome, it is usually a diagnosis made after, in some cases, the realization of “what else could it be?” sinks in, and more “rare disorders” are sought out. Finally, for many, probably like yourself as you are reading up about Eagle Syndrome here, the diagnosis came. It must be Eagle syndrome or its close companion Ernest Syndrome.
Article outline and discussion points:
- Eagle syndrome and the diagnosis of the stylohyoid complex syndrome.
- My doctors are confused by my symptoms.
- If there is an injury involved, whiplash, fall, or accident, your doctor should be more aware of the possibility of Eagle Syndrome.
- Is it dentistry? There is controversy about the role of dental work in the development of Eagle Syndrome.
- Controversy: “There is not enough literature linking extractive dental events to Eagle syndrome.”
- The patient’s pain began 16 years prior following a root canal of a mandibular tooth on the left side.
- The dental student who was misdiagnosed: “even with knowledge of the condition and unusual direct access to several oral and maxillofacial specialists, it took 4 years and multiple misdiagnoses to reach the final diagnosis.”
- The patient who had no symptoms or evidence of abnormalities on scans has a problem when he turns his head to the left.
- Ligament calcification and elongation of the styloid process.
- Vascular Eagle syndrome – Cervical Spine instability and length of styloid process elongation?
- Case histories on treatment and surgical recommendations.
- Styloidectomy and sometimes tonsillectomy.
- Eagle Syndrome as Neuropathy.
- A case of facial palsy.
- Overview of diagnosis and treatment for Ernest Syndrome and Eagle Syndrome.
- The problems of nerve and artery compression: Elongated styloid process and carotid artery syndrome.
- Discussing arterial compression or carotid artery syndrome.
- The compression of the jugular vein – Stylo-jugular venous compression syndrome.
- The internal jugular vein gets compressed by anterior subluxation of C1 and C0-C1 instability
- Prolotherapy Treatment of Eagle syndrome
Eagle syndrome and the diagnosis of a stylohyoid complex syndrome
Here are some of the things we hear that helped people get to a diagnosis of Eagle syndrome.
A note of explanation: Eagle syndrome and the diagnosis of the stylohyoid complex syndrome are very similar. Researchers over the years have written of the main distinction being Eagle Syndrome is acquired after a neck injury, a stylohyoid complex syndrome that develops in childhood, adolescence, or earlier in life. There is no history of previous injury, surgery, or trauma. The symptoms can be the same, the treatments can be the same.
My doctors are confused by my symptoms
I have been diagnosed with Eagle Syndrome, my doctors are confused by my symptoms. My symptoms are all isolated on the right side of my head and neck.
I was in a car accident as a teenager, I had a whiplash-type injury. I have had chronic neck and back pain since. Over the years I have felt a grinding and crunching in my neck. My most recent MRI has revealed that the grinding was my C5-C6. They are grinding against each other and have caused developing bone spurs in that joint. I had been seeing a chiropractor and was prescribed physical therapy for problems in my neck that I thought were more related to C0-C1. It feels like my C1 is banging against the back of my skull.
I also developed severe and terrible ear pain. None of my doctors could figure it out. The ENT sent me to an ear specialist, I had lots of tests, but no answers. I was put on medication for Meniere’s disease because there seemed to be nothing else to do. I have so many symptoms and so many problems. I have been searching online to see if I can possibly help myself and my doctors. One doctor suggested “Eagle Syndrome,” and I found some articles.
After all these years I think I may have found “ground zero,” to my problems. My vertigo, tinnitus, and numbness in my face. The constant pain at the base of my skull and countless neurologic symptoms had my doctors looking at ALS and MS. I am trying to get all my doctors on board. The problem is that each specialist is so focused on their part of the body. My spinal specialist may as well be a million miles away from my ENT as much as they communicate. But I am going to concentrate on my neck and going to focus on that.
I was finally diagnosed with Eagle syndrome. My ENT initially suspected cancer. I was sent for a barrage of tests. Once it was NOT cancer, my ENT felt more free to explore other possibilities. He suggested Eagle syndrome. Cancer entered into this because no one could figure out why I had so much throat inflammation.
Novocaine and the dentist
I have been on a long medical journey. It started over 10 years ago with some dental work. I had numerous novocaine injections into my jaw at the TMJ. After the dental work, the symptoms then progressed over the years. Burning pain and numbness in my face and neck. Then, ear pain and fullness, balance problems, dizziness and vertigo, difficulty swallowing food stuck in the throat, throat inflammation, vision problems, pain at the back of my head, and the sensation when I woke up that I was punched at the base of my skull.
My neurologist figured that all this was coming from nerve compression in my neck and I had a C5-C7 fusion. Symptoms continued. The result of the surgery was an increase in my medications. The Diazepam does help with multiple symptoms including muscle pain and this odd sweating I have. I also have to take Vicodin when the pain is severe.
I took it upon myself to see what more I can do for myself besides relying on these medications. My interest was peaked in understanding the stylomandibular ligament, Ernest Syndrome, Eagles syndrome, and Barre Liéou syndrome as your description of these symptoms is actually describing me.
My ENT then suggested Trigeminal Neuralgia
My doctors and I have been searching for almost 20 years to try to figure out what is wrong with me. All my pain is limited to the left side of my body. Each specialist I see limits how much I can be helped by their specialty. I have been putting off a neck surgery because I already had some surgical attempts to fix the pain in my shoulders and arms. I am not really sure how much the neck surgery will help me when these other surgeries did not. My ENT then suggested Trigeminal Neuralgia. So I went home and started researching that. Things started to make sense because the Trigeminal Neuralgia possibility lead me to Ernest and Eagles Syndromes and the burning and tingling I feel in my mouth and face, the ear pain and fullness, the swallowing difficulties, sinus pressure, and neck pain, everything started to come together for me.
I cannot recall a time when I didn’t feel that something was stuck in my throat
A recent CT scan revealed an elongated styloid process. I cannot recall a time when I didn’t feel that something was stuck in my throat where the tonsils used to be. My tonsils were removed and I read that in some cases a tonsillectomy can promote an elongated styloid.
My symptoms are pain in the ear, pain on the side of my face, eye pain, Adam’s apple shifted, and clicking or snapping in my throat/neck when I lift my head. When I look up and talk for a few minutes, my ears get clogged and my hearing diminishes significantly. I’ve had an esophagram and the results were fine. Physical therapy was prescribed but I held off pending the results of the CT scan. Without a full understanding of what I’m dealing it, it seems dangerous to start a therapy program. So no treatment has taken place yet.
My feeling of doom
My symptoms are primarily passing out, dizziness, intense stomach pain, dull neck pain, fatigue, and quite literally “doom” or “feeling like I was dying.” I eventually received a pacemaker to deal with the symptoms related to syncope. Five years later symptoms are worsening.
No consensus on treatment for Eagle Syndrome
Part of the problems and challenges these patients faced was that their doctors cannot fall back on a consensus treatment for Eagle Syndrome.
A March 2022 paper in the European Archives of Otorhinolaryngology (19) writes: “Eagle’s syndrome is not uncommon in clinical work. Because of its atypical symptoms, (Eagle’s syndrome), it is easy to be misdiagnosed as other diseases, further leading to misdiagnosis and mistreatment. At present, there are no expert consensus or treatment guidelines for the disease.” More on this paper below.
If there is an injury involved, whiplash, fall, or accident, your doctor should be more aware of the possibility of Eagle Syndrome
Doctors at The New York Eye and Ear Infirmary of Mount Sinai Hospital have published their recommendations for Eagle Syndrome in the journal Clinical Neurology and Neurosurgery. (1) Here are the study highlights:
- A comprehensive literature review was conducted on peer-reviewed publications on Eagle Syndrome across multiple disciplines in order to gain a thorough understanding of the presentation, diagnosis, and management of this disorder.
- Diagnoses of Eagle Syndrome have increased, in part due to the awareness of physicians of patient symptomatology.
- While cervical pain and dysphagia (swallowing difficulties) are among the typical symptoms, patients can present with a wide spectrum of benign and dangerous symptoms.
- Treatment strategies include medical management (analgesics, corticosteroids, antidepressants, and anticonvulsants) and varied surgical approaches.
- Increased understanding by providers treating patients with Eagle Syndrome allows for a more comprehensive treatment plan. With a variety of medical regimens and more definitive surgical approaches, Eagle Syndrome can be treated safely and effectively.
Most physicians have not heard of Eagle Syndrome and do not know where the stylomandibular ligament is located. For this reason, many people receiving the treatments mentioned above do not obtain relief from their pain as in the symptoms listed below.
Is it dentistry? There is controversy about the role of dental work in the development of Eagle Syndrome
There is controversy about the role of dental work in the development of Eagle Syndrome. An August 2018 case history in The Neuroradiology Journal (2) reported on one such case of “Eagle syndrome that became symptomatic after a dental procedure (wisdom teeth removal).” This was considered rare as the authors noted further: “A literature review (the looked at previously published research to find a similar case history) performed with focus on various etiologies (origins) of Eagle syndrome diagnosis found a previously published case of Eagle syndrome presenting as the pain of dental origin. However, no case reports of symptoms arising in a patient post-dental procedure were found in our search.”
In this case, a 36-year-old African American male went to the emergency room with odynophagia (painful swallowing) for the previous five days. The pain started approximately two hours following tooth extraction at a dentist’s office. The pain was described as 10/10 in intensity, continuous, sharp, non-radiating, associated with globus sensation (a lump or something stuck in the throat), cyclical vomiting, and dysphagia (difficulty swallowing). There was a worsening of the pain upon swallowing, yawning, and chewing. On CT scan the styloid processes of the patient were noted to be elongated. Additionally, multiple calcifications were seen at the tip of the left styloid process with an unusual configuration at the superior surface of the left hyoid, suggesting a pseudo articulation (the elongation was so long it created a joint-like structure in the process). The patient was admitted, empirically treated for symptoms with oral pain medication, and discharged with recommended outpatient follow-up. However, the patient was subsequently lost to follow-up. No one knows what happened to him.
“There is not enough literature linking extractive dental events to Eagle syndrome”
At our center, we see many people who had cervical instability exasperated and their symptomology made worse by a long stay in the dental chair. To suggest to these people that the dental work had nothing to do with their current situation would be to suggest to them something they know is not true. Medical research and case histories are important elements in understanding diseases and disorders. There is a famous expression in science, “Absence of evidence is not evidence of absence.” Meaning that evidence for dental work causing symptomology has not yet been documented sufficiently.
Articles confirm the correlation between traumatic events and the onset of typical symptoms of Eagle syndrome
A June 2021 study in the medical journal Healthcare (3) examined thirteen previously published medical papers articles that showed correlations between the onset of symptoms in Eagle syndrome and traumatic events and highlighted two possibilities: traumatic events could fracture the already elongated styloid process or calcified stylohyoid ligament; trauma itself triggers the pathophysiological mechanisms that lead to a lengthening of styloid process or calcification of stylohyoid ligament and therefore the typical symptoms. The two case reports concerning Eagle syndrome symptoms after extractive dental events describe the onset of the classic type. The conclusion: “The analyzed articles confirm the correlation between traumatic event and onset of typical symptoms of Eagle syndrome. There is not enough literature linking extractive dental events to Eagle syndrome.”
The two case reports: The first case report was the one above published in The Neuroradiology Journal of the 36-year-old man that was lost to follow up. In the second case (4) “the patient presented for observation with acute pain on the right side of the face which radiated to the temporal and neck ipsilateral area, also causing a decrease in rotational movements of the neck and head in that direction. He also reported stress and insomnia for about three months, a period during which he underwent lower right jaw third molar extraction.”
Yet there were other case histories mounting connecting dental work with Eagle’s syndrome.
The patient’s pain began 16 years prior following a root canal of a mandibular tooth on the left side
Here a case history was presented in the Journal of the Korean Association of Oral and Maxillofacial Surgeons. (5) In this case doctors wrote of a 53-year-old woman with a complaint of pain in her throat and the left side of the head and face. Her pain began 16 years prior following a root canal of a mandibular tooth on the left side. She at times experienced severe pain attacks lasting approximately 10 minutes with a frequency of 10 times per day, which extended from the external auditory canal to the left side of the throat.
Additionally, the cough was a trigger for these pain attacks. Certain odors or nervousness were also aggravating factors of her pain, while pressure on the ear and diphenhydramine (antihistamine use) were mitigating factors. The patient had a past medical history significant for hypertension and favism (a condition where exposure to fava beans or certain medications causes the breakdown of red blood cells).
The patient’s general health as well as her head and neck were thoroughly examined. Her skull, cranial nerves, eyes, ears, nose, sinuses, thyroid, and dentition were unremarkable. No cervical, axillary, epitrochlear, or inguinal adenopathy (swelling of the lymph nodes) was detected. On oral and pharyngeal examination, severe tenderness was present on a bony prominence in the tonsillar cavity. Head and neck rotation was painful at the end of the active range of motion. As a result, lateral skull and neck x-rays were taken that showed elongation of the styloid process, thereby confirming the diagnosis of Eagle syndrome.
The dental student who was misdiagnosed: “even with knowledge of the condition and unusual direct access to several oral and maxillofacial specialists, it took 4 years and multiple misdiagnoses to reach the final diagnosis.”
Here is an April 2021 case report presented in The American Journal of Case Reports. (6) It was presented by doctors at the Department of Dental Clinical Sciences, Faculty of Dentistry, Dalhousie University, Halifax, Nova Scotia, Canada.
Eagle syndrome symptoms are non-specific and include severe throat, facial, and ear pain, or headaches. They are usually exacerbated by head rotation, swallowing, yawning, or chewing, but atypical presentations exist. It is a difficult pathology to diagnose and it can take several years before a proper diagnosis is made.
This report describes the case of a dental student presenting with an atypical presentation of Eagle syndrome. His styloid processes were 75 mm long and he was affected with severe pain in his throat, the anterior part of his ears, his submandibular area, and his molar teeth.
The pain was exacerbated during maximal mouth opening, yawning, mandibular protrusion, and downward head tilts, but not during the classically described movements of head rotation, swallowing, yawning, or chewing. Due to the absence of the classic symptoms, even with knowledge of the condition and unusual direct access to several oral and maxillofacial specialists, it took 4 years and multiple misdiagnoses to reach the final diagnosis.
The patient who had no symptoms or evidence of abnormalities on scans has a problem when he turns his head to the left
The confusion and misunderstanding about Eagle’s Syndrome may continue to prolong the belief that this is a very rare condition. Therefore a doctor not knowing what to look for will miss it as did the dental surgeons in the above study.
Here is a case history reported and published by the Department of Radiology, Suleyman Demirel University Hospital, Faculty of Medicine, in Turkey. (7)
No distinctive feature was found during the physical examination of the patient. The patient did complain that he suffered from vertigo when he turned his head to the left, which indicated that the vertigo was caused by cervical rotation, and the patient’s complaints disappeared completely in the neutral position.
The patient stated that he had been suffering from this complaint for about two years. The patient had no complaints of neck pain, foreign body sensation, dysphagia, or visual impairment. No finding was observed on the patient’s two-way cervical x-ray.
A CT angiography scan was performed. No intraluminal-extraluminal pathologies were detected on the common carotid artery, internal carotid artery, external carotid artery, or intra-cerebral vascular structures during the CT angiography examination.
No pathologies were found on soft tissues in the extra-vascular region. However, the styloid processes were found to be longer than normal when bone structures were examined. Also, it was discovered that the left process was adjacent to the internal carotid artery and caused a slight compression of the internal carotid artery.
The CT angiography scan was re-performed focusing on the left cervical rotation position considering that the elongated styloid processes might be the cause of vertigo secondary to vascular compression when taking the patient’s complaints and CT angiography findings together. As a result, it was observed that the compression of the internal carotid artery became evident. Based on the existing finding, the patient was diagnosed with Eagle syndrome aggravating with cervical rotation and was referred for surgery.
What are we seeing in this image? Compression on rotation
In this image we see a CT venogram 3-D reconstruction showing enlarged styloid bones. The image is divided in two with the A image to the left showing an anterior-posterior view. To the right on the B side with a lateral view.
In this patient, the enlarged styloid did not obstruct the internal jugular vein during the scan when she was supine, but when she was scanned using doppler ultrasound while upright and moving her neck, they both encroached on the carotid sheath.
Ligament calcification and elongation of the styloid process
Above I described the problems of diagnosis and treatment of suspected Eagle Syndrome. I also described the medical literature calling Eagle Syndrome a “rare” disorder. Yet in recent medical publications, this rare disorder is getting its fair share of attention as doctors share their case histories with each other.
Here we have a report on five people with suspected Eagle’s Syndrome. It was published in the July-August 2021 issue of the medical journal Neurologia. (8) The doctors of this paper describe the clinical characteristics and response to treatment of five patients who suffered from headaches and came for headache treatments and were then later diagnosed with Eagle syndrome.
Here is a summary report:
The patients were three men and two women between the ages of 24 and 51, presenting dull, intense pain, predominantly in the inner ear and the ipsilateral tonsillar fossa. All patients had chronic, continuous pain in the temporal region, with exacerbations triggered by swallowing.
Four patients had previously consulted several specialists at otorhinolaryngology departments; one had been prescribed antibiotics for suspected Eustachian tube inflammation.
In all cases, the palpation of the tonsillar fossa was painful. Computed tomography scans revealed elongation of the styloid process and/or calcification of the stylohyoid ligament in three patients. Four patients improved with neuromodulatory therapy (duloxetine, gabapentin, pregabalin) and only one required surgery for the elongated styloid process.
Vascular Eagle syndrome – Cervical Spine instability and length of styloid process elongation?
As many of you are already aware from your own symptomology of fainting, passing out, blackouts, increased intracranial pressure, and transient ischemic attack, the diagnosis of Vascular Eagle Syndrome is made when the elongated styloid process compresses the internal carotid artery in the upper cervical spine below the skull.
A May 2021 paper in the journal Neurological Sciences (9) suggests that cervical spine instability may play a greater role in internal carotid artery compression than the length of the styloid process elongation.
“Vascular Eagle syndrome, due to impingement of the extracranial internal carotid artery by the styloid process, is an uncommon and not yet widely recognized cause of internal carotid artery dissection. Up to now, this diagnosis is still presumptive, based mainly on the length of the styloid process. However, given the discrepancy between the much higher prevalence of an elongated styloid process in the population compared to the reported rate of Eagle syndrome, other anatomical factors beyond the length itself of this bony structure seem to be involved.
Results: Our study showed that in patients with styloid process-related dissection the styloid process angulation on the coronal plane tends to be more acute and that styloid process-C1 distance is significantly shorter at the side of the dissection. This data reinforces the idea that internal carotid artery dissection risk in the vascular Eagle syndrome has probably multifactorial pathogenesis.”
Post-styloidectomy surgery update
Case histories on treatment and surgical recommendations
In Spain, doctors published their observations on five patients diagnosed with Eagle Syndrome and how their treatment progressed. Of these five patients, one was sent to surgery. Here is their presentation published in the Spanish medical journal Neurología. (10)
(Of the five patients) three were men and two were women between the ages of 24 and 51. All five presented dull, intense pain, predominantly in the inner ear and the ipsilateral (on the same side of the ear pain) tonsillar fossa. All patients had chronic, continuous pain in the temporal region, with exacerbations triggered by swallowing.
Four patients had previously consulted several specialists at otorhinolaryngology departments; one had been prescribed antibiotics for suspected Eustachian tube inflammation. In all cases, the palpation of the tonsillar fossa was painful. Computed tomography scans revealed elongation of the styloid process and/or calcification of the stylohyoid ligament in 3 patients.
Four patients improved with neuromodulatory therapy (duloxetine, gabapentin, pregabalin) and only one required surgical excision of the styloid process.
Styloidectomy and sometimes tonsillectomy
Above I cited a March 2022 paper (20) that wrote “At present, there are no expert consensus or treatment guidelines for the disease.” That paper sought to help doctors better understand the treatment options, specifically the surgical options in treating Eagle Syndrome.
In this paper the doctors examined the postoperative effectiveness of 103 patients who had one of three surgical methods:
- styloid process resection through the external cervical approach (the styloid process could not be touched through the mouth or could be touched under the jaw or when the CT scan showed that the inclination angle was not large),
- tonsillectomy + styloidectomy,
- and preservation of the tonsil for styloidectomy (the styloid process bone could be touched directly during intraoral palpation or in whom the distal part of the styloid process could not be directly touched, but the CT scan showed that the bone inclined toward the oropharynx and its distal part was relatively close to the oropharynx cavity; whether tonsillectomy was performed depending on whether the patient’s tonsil was too large to affect the surgical incision).
The patients were followed up for 12-36 months (an average of 15 months).
- Of the 103 patients,
- 21 underwent styloid process resection through the external cervical approach,
- 49 underwent tonsillectomy and styloidectomy, and
- 33 underwent styloidectomy with preservation of the tonsil.
- The surgery cured 48 (46%) cases, was effective in 35 (34%), and was ineffective in 20 (20%).
Conclusions: Operation is an effective method for treating Eagle’s syndrome. There were no significant differences between the effects of the intraoral and external cervical approaches.
The transcervical approach for styloidectomy
The transcervical approach is from the side of the neck. A paper from the Department of Otorhinolaryngology-Head & Neck Surgery, University of Pennsylvania Hospital System published in February 2022 in the journal Head and Neck (23) described the case histories of fifteen patients who were recommended for surgical management of Eagle syndrome. The patients had styloidectomy via a transcervical approach. The procedure was well-tolerated without complications, and all patients were discharged on a postoperative day one. Thirteen patients (87%) said they had significant improvement or complete resolution of their presenting complaint(s), most commonly throat and neck pain/discomfort (53%), otalgia (47%), and/or tinnitus (40%). The surgeon/authors concluded: “The transcervical approach for styloidectomy provides an alternative for operative access that overcomes the limitations associated with the transoral approach. It enables better exposure to the operative field, a more efficient procedure, and, in the senior author’s experience, results in decreased postoperative pain, trismus, and length of hospital stay.”
Eagle Syndrome as Neuropathy
We are not the only center to see strange or confusing cases of neurologic-like symptoms. Surgical centers report confusion as to why patients had continued progressive symptoms following a procedure that should have helped them. This is a February 2022 case report published in the journal BMJ Case Reports. (18)
Findings at a glaucoma clinic.
“Eagle syndrome is a rare entity that occurs when an elongated styloid process compresses the neck vasculonervous structures. A 47-year-old female patient was referred to our glaucoma clinic for a second opinion concerning bilateral visual field progression despite maximal tolerated medical therapy (the use of the most drugs that the patient could tolerate).
After aggressive intraocular pressure lowering and resorting to surgical and optimized medical treatment, the visual field kept stable in the right eye but was still showing progression in the left eye.
As optic disc was paler in the left eye (blood flow back and forth to the eye was suspected of being blocked) and after full appreciation of systemic complaints, including left neck, ear, and eye pain, a multidisciplinary approach with otorhinolaryngology was prompted.
Plain radiographs of the skull showed an elongated styloid process on the left side, compatible with Eagle syndrome. Surgical resection of the styloid process provided definitive relief of the patient’s eye, ear, and neck pain. Since this intervention, there was no further deterioration of visual field defect in the left eye.”
A March 2022 paper in the publication BMJ Case Reports (21) discusses the case of a teenage boy, diagnosed with right internal carotid artery dissection and middle cerebral artery infarction, with no cause initially identified. Here is what the doctors wrote:
“Classically, (Eagle’s syndrome) presents as unilateral throat pain or rarely, as acute neurological symptoms secondary to compression of the internal carotid artery: so-called ‘stylocarotid syndrome’. Significant neurological events in teenagers, secondary to Eagle syndrome have not been reported. (In the case of the teenage boy’s case): Following further admission with a transient neurological episode, he was noted to have elongated styloid processes with the right abutting the site of carotid dissection (a tear in one of your carotid arteries). He underwent styloidectomy and has since remained symptom-free. This case highlights the importance of considering anatomical variants when assessing young patients with neurological symptoms, and the potential morbidity and mortality benefit that early surgical intervention may have.”
A case of facial palsy
In February 2022, doctors presented a case history in the journal Cureus (22) about a patient with facial palsy. They wrote:
Facial palsy is a condition that affects the facial nerve and results in weakness or total paralysis of the facial muscles that control expression. Here, (the attending doctors) describe a rare presentation of Eagle Syndrome presenting as facial palsy. (They) present the case of a 62-year-old female who was admitted to the emergency department with right peripheral facial palsy. A computed tomography (CT) scan of the neck confirmed the diagnosis. The patient underwent conservative management and physical therapy, which resulted in good evolution with an improvement of symptoms. She was referred to the otorhinolaryngologist for surgical evaluation.”
The treatment plan this patient received was:
- A five-day course of prednisolone 60 mg/day, then tapered 10v mg/day, for a total treatment time of 10 days.
- She also started physical therapy.
Ross Hauser, MD presents an overview of diagnosis and treatment for Ernest Syndrome and Eagle Syndrome.
Eagle Syndrome is medically known as the elongation of the styloid process and stylohyoid ligament calcification. This typically occurs with aging and often results in sharp, intermittent pain along the glossopharyngeal nerve that is located in the hypopharynx and at the base of the tongue. This is a stylohyoid ligament problem and is further explained in our accompanying article on Ernest Syndrome.
Summary video transcript
- When we see patients with unresolved head, neck, cranial, and TMJ pain we think of injury to the stylomandibular ligament. This injury can cause symptoms like those problems mentioned above because of the impact this injury creates on the cranial nerves, specifically the glossopharyngeal nerve, the vagus nerve, and the Hypoglossal nerve (Cranial nerve XII).
Focus on the stylomandibular ligament and development of bone spurs at the styloid process
- (3:18) Bone spurs. When the stylomandibular ligament is under constant pressure from the instability of the mandible or pressure on the mandible exhibited in the head-down position, it distributes this stress into the styloid process. This stress causes the development of bone spurs. The formation of bone spurs at the styloid process can impinge on the vagus nerve, the glossopharyngeal nerve, and the hypoglossal nerve. This impingement can be a leading culprit in the myriad of symptoms the patient suffers from.
The problems of nerve and artery compression: Elongated styloid process and carotid artery syndrome
In our sample stories above and the many problems they describe we see that at the base of these people’s problems is a lack of diagnosis and understanding of what their symptoms are. This is not unique to Eagle Syndrome people. It is a common characteristic in many of the patients we see with upper cervical spine instability.
This is what your doctors know: Eagle syndrome is medically known as the elongation of the styloid process and stylohyoid ligament calcification. This typically occurs with aging and often results in sharp, intermittent pain along the glossopharyngeal nerve that is located in the hypopharynx and at the base of the tongue.
What are we seeing in this image?
The image is from a medical paper, published in the Journal of Occupational Medicine and Toxicology. (11)
- This is an image of the neck of a 42-year-old secretary. She complained of a long history of neck pain and limited movement of the cervical spine. To her doctors’ surprise, a radiologic examination revealed a bilateral ossification of the stylohyoid ligament complex.
- Her symptoms did not resolve nor did her condition improve with conservative treatment consisting of anti-inflammatory medication as well as physical therapy. The patient was admitted for surgical resection of the ossified stylohyoid ligament complex. Afterward, she was free of any complaints and went back to work.
- Our note: Surgery was the only tool in this patient’s case. For some people, surgery may be the only tool. For others, non-surgical options can work as well and this will be discussed below.
What is the ossification of the stylohyoid ligament complex?
Simply, the ligament is taking on the characteristic of bone. It is calcifying, thickening, hardening, and losing its natural elasticity. When the cervical spine ligaments lose their natural elasticity, they can lead to a “fusion,” where the neck does not move, or to hypermobility, where the vertebrae move too much and this can cause pinching and compression of the nerves and arteries and the myriad of symptoms associated with this problem.
Do you also see something in this image that we see? Lots of fillings in the back teeth.
The styloid process is a piece of bone that starts at the base of the skull and attaches to a number of muscles and ligaments that are connected to the throat and tongue. Elongation of the stolid process, as well as calcification of the stylohyoid ligament, can result in Eagle Syndrome. The stylohyoid ligament is located between the styloid process and the hyoid bone, a bone in the front of the throat, to which a number of throat muscles are attached.
What are we seeing in this image?
Here we have a rotational motion x-ray showing a patient’s styloid process.
- In patient A, the styloid is near the lateral mass of the C1 meaning that it is causing compression of the carotid sheath and a host of symptoms, like those described in this article, this patient.
- In Patient B, the styloid is not near the lateral mass of the C1 producing no symptoms.
Discussing arterial compression or carotid artery syndrome
A 2018 paper (12) discussed five case histories of patients with “rare” neurological symptoms of unknown origin: Here are the learning points observed in these five patients:
- In addition to symptoms of recurrent throat pain, neck pain, dysphagia (swallowing difficulties), or facial pain due to an elongated styloid process or calcified stylohyoid ligament, clinical findings related to lower cranial nerve compression have also been reported.
- Explanatory note on the lower cranial nerves: This comes from a paper titled: “Disorders of the lower cranial nerves.”(13)
- Lower cranial nerves include the paired 9th (glossopharyngeal nerve), 10th (vagal nerve), 11th (accessory nerve), and 12th (hypoglossal nerve) cranial nerves
- The 9th (glossopharyngeal nerve), 10th (vagal nerve), and 12th (hypoglossal nerve) are involved in swallowing, tasting, speech, heart rate and blood pressure control, and peristalsis (the muscles of the intestines that push food). Please see our article: SIBO: Small intestinal bacterial overgrowth and the Vagus nerve.
- Explanatory note on the lower cranial nerves: This comes from a paper titled: “Disorders of the lower cranial nerves.”(13)
- In some cases, it is reported that carotid artery compression or dissection can be seen due to the elongated styloid process and this is called carotid artery syndrome. Carotid artery compression causes flow reduction and carotidynia (also called Fay Syndrome – face and neck pain of unknown origin) or neurological symptoms can be seen. Dural sinuses and the jugular vein can be compressed. Eagle syndrome with neurological symptoms has been rarely reported.
The five patients:
- The 5 patients were between 22 and 68 years old.
- They all had various neurological symptoms, each patient underwent computed tomography (CT) that revealed a long styloid process.
- An elongated styloid process caused neurological symptoms.
- Two patients had venous compression by the styloid process.
- The other patients had transient ischemic attacks due to internal carotid artery compression by the styloid process.
- Only one patient underwent surgical removal.
- In conservative care patients, outcomes were good after a treatment and no symptoms remained.
The compression of the jugular vein – Stylo-jugular venous compression syndrome
A case history presented in The American Journal of Case Reports (14) by doctors at Catania University, in Catania, Italy discussed a 36-year-old woman with recurrent episodes of drug-resistant headache and recent memory disturbances. Here is what the doctors wrote:
“Stylo-jugular venous compression syndrome is a subtype of Eagle syndrome and is caused by compression of the internal jugular vein. Treatment varies according to the symptoms and the severity of the compression, and it can be pharmacological or surgical, with vascular stenting and/or removal of the styloid process. We describe a rare case of left cerebral venous sinus thrombosis (a blood clot in the brain’s cerebral venous sinus) and ipsilateral (on that side of the head) internal jugular vein stenosis sustained by excessive length of the left styloid process.”
What makes this case unique enough to publish the findings?
What makes this case unique enough to publish the findings is that the woman was not initially tested for compression of the jugular vein by the styloid process because it is considered “rare.” As her drug-resistant headaches continued and her memory lapses increased, she had cerebral and neck multidetector computed tomography-angiography and Doppler ultrasound of the epiaortic (images of the aortic root and arch) vessels that respectively revealed thrombosis of the left cerebral venous sinus and left internal jugular vein stenosis due to a very long styloid process.
“the patient was treated with anticoagulant drugs and experienced a gradual remission of symptoms.”
The doctors reported that “the patient was treated with anticoagulant drugs and experienced a gradual remission of symptoms.” the case history presented the suggestion to other doctors that “compression of the jugular vein by the styloid process is a rare entity, and it often goes undiagnosed when it is asymptomatic. Doppler ultrasound is a sensitive method for identifying jugular vein stenosis and can provide an estimated degree of stenosis, which is useful for treatment planning. Doppler ultrasound should be combined with multidetector computed tomography-angiography to rule out compression of other vascular structures and other causes of compression. Failure to treat these patients could have serious health consequences for them.”
Please see our article for more information on Using Transcranial Doppler & Extracranial Doppler Ultrasound Testing at the Hauser Neck Center.
In January 2020 a team of researchers publishing in the journal CNS Neuroscience and Therapeutics (15) offered a series of findings in the patients that they were seeing that allowed the researchers to come up with a diagnosis label called cervical spondylotic internal jugular venous compression syndrome. Let me explain this briefly because this syndrome is what we are seeing in our patients and can be applied to many diagnoses including Eagle Syndrome.
The possible compression of the jugular vein in Eagle Syndrome: The learning points of the research:
- Cervical spondylosis is a commonly encountered degenerative disorder of the cervical spine. Abnormal structures, repetitive movements, and aging degeneration can exacerbate bone hyperplasia (overgrowth) and losses of intervertebral disc contents (the disc flattens) and subsequently cause the disc to bulge outward, which in turn hasten the damage to the disc and surrounding structures gradually.
- Cervical spondylosis may contribute to many symptoms such as cervical radiculopathy, cervical myelopathy, axial neck or shoulder pain, and vertebral artery insufficiency.
- The researchers noticed in their clinical practice, that a group of patients with unexplained nonfocal (not a nerve problem) neurological dysfunctions display cerebral venous outflow disturbance in relation to the atlas (C1) compression of the superior segment of the internal jugular vein.
Before we finish looking at this research, a brief note: We have published extensive articles on the problems we are going to mention below. The links are provided.
- Internal jugular vein stenosis has been associated with several central nervous system disorders, including transient monocular blindness, Ménière disease, Alzheimer’s disease, and idiopathic intracranial hypertension (please see our article Cervical Spine Instability, Vein blockage, fluid build up, and intracranial hypertension), and multiple sclerosis.
- Decreased cerebral perfusion (blood flow to the brain – see our article Dynamic Analysis of Blood Flow Measurements to the Brain, Brainstem, Cervical Spinal Cord, and Cranial Nerves), cerebral microvascular structures impairment (This is the network where arteries and veins connect by way of the arterioles (the part of the artery that leads to the capillaries), capillaries (that connect the arterioles to the venules), and venules (which bring the connection to the vein), impaired cerebrospinal fluid (CSF) dynamics, and elevated intracranial pressure may be the underlining mechanisms of internal jugular vein‐induced brain structural and functional disorders.
- This study saw many of the things we see: Some nonfocal neurological symptoms like headache, head noise, tinnitus, and visual impairment are tightly correlated to unilateral or bilateral internal jugular vein syndrome.
The internal jugular vein gets compressed by anterior subluxation of C1 and C0-C1 instability
In December 2019, a paper appeared in the European Archives of Otorhinolaryngology. (16) The paper was the combined effort of a university hospital’s department of radiology, computer engineering department, and the anatomy department. The goal was to help doctors better identify stylohyoid complex syndrome by offering standardization of measurements of how long the styloid process should be and at what angle should it jut from the temporal bone (base of the skull). Deviations in these measurements can help with a diagnosis of Eagle syndrome.
While this would be helpful, Dr. Enrico Nastro Siniscalchi, maxillofacial surgery and assistant professor at the University of Messina took it one step further and is more in agreement with what we have seen in our patients. Basically, the styloid process does not have to be that elongated or even display the type of angulation or measurement deviations discussed in the previously mentioned study. A normal size styloid process can cause arterial compression if there is cervical spine instability.
Here are his learning points in his published response. (17)
- Even in the presence of a styloid process of normal length, lateral (side) and medial (middle) deviations can occur, in fact, a normal styloid process can cause compression of vessels and nerves.
- (Dr. Siniscalchi’s) the recent experience focuses the attention on the “dynamic” relationship between the stylohyoid process and near structures. (In other words, the relationship is in motion). In particular, above all in the vascular variant, neurological (symptoms) seem to be often influenced by the position of the head and neck, varying in the same patient from one position to another.
- Note: What Dr. Siniscalchi is saying is what we see in our digital motion x-ray images. A digital motion x-ray is an x-ray movie. It reveals the “dynamic” elements of neck instability. That is, what is happening to the neck in movement, and does moving the neck into different positions cause neurological symptoms? For example, does turning your head to the right make you pass out or get dizzy. Does looking up “clog” your ears. Does looking down create brain fog, etc.
- Dr. Siniscalchi suggests that as a consequence of this strong influence on head and neck position and symptomology, in the case of a suspected Eagle Syndrome, a standard “static” tool could not be sufficient to better clarify the relationship between styloid and other structures. In other words, in the case of suspected Eagle Syndrome, an imaging tool like MRI and ultrasonography of the head and neck region should be taken in different positions of the head, being the structures involved in such syndrome are conditioned by the different positions of the entire head and neck region.
- Dr. Siniscalchi suggests taking an image in five positions of the head: rest position, maximum extension, maximum flexion of the head, maximum right, and maximum left rotation.
Digital motion X-ray showing C1-C2
This is one of our videos, it gives a clearer view of the DMX demonstrating C1-C2 instability in a patient.
- Digital Motion X-ray is a great tool to show instability at the C1-C2 Facet Joints
- The amount of misalignment or “overhang” between the C1-C2 demonstrates the degree of instability in the upper cervical spine.
- This is treated with Prolotherapy injections (explained below) to the posterior ligaments that can cause instability.
- At 0:40 of this video, a repeat DMX is shown to demonstrate the correction of this problem.
Prolotherapy Treatment of Eagle syndrome
In this section, we are going to discuss possible treatments. That treatment is Prolotherapy. In simple terms, Prolotherapy repairs ligaments. In the case of Eagle syndrome. The cervical spine ligaments.
In the research and clinical observations explained above, we have been able to demonstrate that some neurological symptoms are related to the compression of the cervical nerves, arteries, and even the jugular vein, In Eagle Syndrome, this compression may be caused by a weakened or damaged set of cervical ligaments, here focused on the stylohyoid ligament complex. Prolotherapy injections at the stylomandibular ligament bony attachments will start the repair process. Once the stylomandibular ligament is strengthened, the chronic ear-mouth pain, tinnitus, dizziness, vertigo, and other pain complaints subside.
Research on cervical instability and Prolotherapy
Caring Medical has published dozens of papers on Prolotherapy injections as a treatment for difficult to treat musculoskeletal disorders. Here are some.
In our paper Chronic Neck Pain: Making the Connection Between Capsular Ligament Laxity and Cervical Instability we wrote:
“To date, there is no consensus on the diagnosis of cervical spine instability or on traditional treatments that relieve chronic neck instability issues like those mentioned above. In such cases, patients often seek out alternative treatments for pain and symptom relief. Prolotherapy is one such treatment that is intended for acute and chronic musculoskeletal injuries, including those causing chronic neck pain related to underlying joint instability and ligament laxity. While these symptom classifications should be obvious signs of a patient in distress, the cause of the problems is not so obvious. Further and unfortunately, there is often no correlation between the hypermobility or subluxation of the vertebrae, clinical signs or symptoms, or neurological signs or symptoms. Sometimes there are no symptoms at all which further broadens the already very wide spectrum of possible diagnoses for cervical instability.”
In other words, Prolotherapy may treat very difficult to treat.
Caring Medical research
- Chronic Neck Pain: Making the Connection Between Capsular Ligament Laxity and Cervical Instability
- This paper was published in the European Journal of Preventive Medicine
- Steilen D, Hauser R, Woldin B, Sawyer S. Chronic neck pain: making the connection between capsular ligament laxity and cervical instability. The Open Orthopaedics Journal. 2014;8:326-345. [Google Scholar]
- The Biology of Prolotherapy and Its Application in Clinical Cervical Spine Instability and Chronic Neck Pain: A Retrospective Study
- This paper was published in the European Journal of Preventive Medicine
- Ross Hauser, MD, Steilen-Matias D, Gordin K. The biology of prolotherapy and its application in clinical cervical spine instability and chronic neck pain: a retrospective study. European Journal of Preventive Medicine. 2015;3(4):85-102. [Google Scholar]
- Non-Operative Treatment of Cervical Radiculopathy: A Three-Part Article from the Approach of a Physiatrist, Chiropractor, and Physical Therapists
- This paper was published in the Journal of Prolotherapy
- Ross Hauser, MD, Batson G, Ferrigno C. Non-operative treatment of cervical radiculopathy: a three-part article from the approach of a physiatrist, chiropractor, and physical therapists. Journal of Prolotherapy. 2009;1(4):217-231.
- Dextrose Prolotherapy for Unresolved Neck Pain
- This paper was published in Practical Pain Management
- Hauser R, Hauser M, Blakemore K. Dextrose Prolotherapy for unresolved neck pain. Practical Pain Management. 2007;7(8):58-69.
- Cervical Instability as a Cause of Barré-Liéou Syndrome and Definitive Treatment with Prolotherapy: A Case Series
- This paper was published in the European Journal of Preventive Medicine
- Hauser R, Steilen-Matias D, Sprague IS. Cervical instability as a cause of Barré-Liéou syndrome and definitive treatment with prolotherapy: a case series. European Journal of Preventive Medicine. 2015;3(5):155-166. [Google Scholar]
Summary and contact us. Can we help you? How do I know if I’m a good candidate?
We hope you found this article informative and it helped answer many of the questions you may have surrounding the problems of cervical spine instability and Eagle Syndrome. Just like you, we want to make sure you are a good fit for our clinic prior to accepting your case. While our mission is to help as many people with chronic pain as we can, sadly, we cannot accept all cases. We have a multi-step process so our team can really get to know you and your case to ensure that it sounds like you are a good fit for the unique testing and treatments that we offer here.
Brian Hutcheson, DC | Ross Hauser, MD | Danielle Steilen-Matias, PA-C
1 Badhey A, Jategaonkar A, Kovacs AJ, Kadakia S, De Deyn PP, Ducic Y, Schantz S, Shin E. Eagle syndrome: A comprehensive review. Clinical Neurology and Neurosurgery. 2017 May 6. [Google Scholar]
2 Li S, Blatt N, Jacob J, Gupta N, Kumar Y, Smith S. Provoked Eagle syndrome after dental procedure: A review of the literature. The neuroradiology journal. 2018 Aug;31(4):426-9. [Google Scholar]
3 Saccomanno S, Quinzi V, D’Andrea N, Albani A, Coceani Paskay L, Marzo G. Traumatic Events and Eagle Syndrome: Is There Any Correlation? A Systematic Review. InHealthcare 2021 Jul (Vol. 9, No. 7, p. 825). Multidisciplinary Digital Publishing Institute. [Google Scholar]
4 Sowmya GV, Singh MP, Manjunatha BS, Nahar P, Astekar M. A case of unilateral atypical orofacial pain with Eagle’s syndrome. Journal of cancer research and therapeutics. 2016 Oct 1;12(4):1323. [Google Scholar]
5 Taheri A, Firouzi-Marani S, Khoshbin M. Nonsurgical treatment of stylohyoid (Eagle) syndrome: a case report. Journal of the Korean Association of Oral and Maxillofacial Surgeons. 2014 Oct 1;40(5):246-9. [Google Scholar]
6 Michaud PL, Gebril M. A Prolonged Time to Diagnosis Due to Misdiagnoses: A Case Report of an Atypical Presentation of Eagle Syndrome. The American Journal of Case Reports. 2021;22:e929816-1. [Google Scholar]
7 Demirtaş H, Kayan M, Koyuncuoğlu HR, Çelik AO, Kara M, Şengeze N. Eagle syndrome causing vascular compression with cervical rotation: case report. Polish journal of radiology. 2016;81:277. [Google Scholar]
8 González-García N, Porta-Etessam J, García-Azorín D. Eagle syndrome: Toward a clinical delimitation. Neurología (English Edition). 2020 Feb 13. [Google Scholar]
9 Tardivo V, Castaldi A, Baldino G, Siri G, Bruzzo M, Del Sette M, Romano N. Internal carotid artery dissection related to abnormalities of styloid process: is it only a matter of length?. Neurological Sciences. 2021 May 31:1-7. [Google Scholar]
10 González-García N, Porta-Etessam J, García-Azorín D. Eagle syndrome: Toward a clinical delimitation. Neurología (English Edition). 2021 Jul 1;36(6):412-7. [Google Scholar]
11 Kirchhoff G, Kirchhoff C, Buhmann S, Kanz KG, Lenz M, Vogel T, Kichhoff RM. A rare differential diagnosis to occupational neck pain: bilateral stylohyoid syndrome. Journal of Occupational Medicine and Toxicology. 2006 Dec;1(1):1-5. [Google Scholar]
12 Aydin E, Quliyev H, Cinar C, Bozkaya H, Oran I. Eagle syndrome presenting with neurological symptoms. Turk Neurosurg. 2018 Jan 1;28(2):219-25. [Google Scholar]
13 Finsterer J, Grisold W. Disorders of the lower cranial nerves. Journal of neurosciences in rural practice. 2015 Jul;6(3):377. [Google Scholar]
14 Farina R, Foti PV, Pennisi I, Conti A, Meli GA, Vasile T, Gozzo C, Tallamona E, Inì C, Palmucci S, Venturini M. Stylo-Jugular Venous Compression Syndrome: Lessons Based on a Case Report. The American Journal of Case Reports. 2021;22:e932035-1. [Google Scholar]
15 Ding JY, Zhou D, Pan LQ, Ya JY, Liu C, Yan F, Fan CQ, Ding YC, Ji XM, Meng R. Cervical spondylotic internal jugular venous compression syndrome. CNS neuroscience & therapeutics. 2020 Jan;26(1):47-54. [Google Scholar]
16 Ayyildiz VA, Senel FA, Dursun A, Ozturk K. Morphometric examination of the styloid process by 3D-CT in patients with Eagle syndrome. European Archives of Oto-Rhino-Laryngology. 2019 Dec 1;276(12):3453-9. [Google Scholar]
17 Siniscalchi EN. Dynamic imaging in suspected Eagle syndrome. European Archives of Oto-Rhino-Laryngology. 2020 Jan 1;277(1):307-. [Google Scholar]
18 Rodrigues CO, Loureiro T, Abegão Pinto L, Tavares Ferreira J. Eagle syndrome presenting as anterior ischaemic neuropathy: a multidisciplinary approach. BMJ Case Rep. 2022 Feb 2;15(2):e246862. doi: 10.1136/bcr-2021-246862. PMID: 35110286. [Google Scholar]
19, 20 Wang J, Liu Y, Wang ZB, Yan KS. Intraoral and extraoral approach for surgical treatment of Eagle’s syndrome: a retrospective study. European Archives of Oto-Rhino-Laryngology. 2022 Mar;279(3):1481-7. [Google Scholar]
21 Selvadurai S, Williamson A, Virk JS, Clarke P. Eagle syndrome and carotid artery dissection: a rare skull base cause of stroke. BMJ Case Reports CP. 2022 Mar 1;15(3):e247954. [Google Scholar]
22 Nunes F, Fernandes MJ, Silva M, Porteiro B, Dutschmann R. Eagle’s Syndrome Presenting as Peripheral Facial Palsy. Cureus. 2022 Feb 22;14(2). [Google Scholar].
23 De Ravin E, Frost AS, Mady LJ, Newman JG. Transcervical styloidectomy for Eagle syndrome. Head & neck. [Google Scholar].
This article was updated May 3, 2022