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Eagle syndrome has been a hot topic among some patients lately. Why? Many have symptoms that are similar to those with this syndrome. So what is Eagle syndrome? What can be done to treat it? Let’s dig in.

What Is Eagle Syndrome?

Eagle syndrome was first described in 1949. It can happen when a bone at the base of the skull (the styloid process) grows too long and impinges on important nerves and/or blood vessels. The symptoms include throat pain (which can range from dull and nagging to severe) or trouble swallowing. There can also be neck and/or facial pain. This can also be felt in the jaw or ear. Other symptoms can also include tinnitus or a ringing sound in the ear or increased symptoms with head movements, such as turning the head or chewing.

This problem impacts women more than men. This is a RARE condition with an incidence of 4-8 per 10,000 people.

Anatomy

The styloid process is the key piece of anatomy to understand Eagle syndrome. This is a tooth-like projection from the side at the bottom of the skull just in front of and down from the ear (shown in yellow here). It connects to the stylohyoid muscle (red) which connects to the hyoid bone (blue). The styloid process also has muscles that go from there to the tongue (styloglossus) and back of the throat (stylopharyngeus).

There is also a ligament connecting the styloid process to the hyoid bone called the stylohyoid ligament and one that goes to the jaw bone (stylomandibular ligament). Confused yet? You should be, this is a complex area. But here’s the summary so far – this styloid process is connected to lots of critical stuff!

What else is in this vicinity? There are vessels and nerves in this neighborhood as well. The internal jugular vein, internal carotid artery, and glossopharyngeal nerve (CN IX), vagus nerve (CN X), and accessory nerve (CN XI) run inside of the styloid process. The occipital artery, hypoglossal nerve (CN XII), (CN VII) run to it’s outside. The trigeminal nerve (CN V) is also in this general vicinity.

Why the Symptoms?

The idea here is that the elongated bone aggravates nerves in the area. Here’s what we believe happens:

• Tounge spasm and throat pain-irritation of the glossopharyngeal nerve which supplies the tongue and the back of the throat and the hypoglossal nerve which moves the tongue.
• Neck pain-irritation of the accessory nerve which supplies the trapezius muscle
• Facial Pain-irritation of a part of the facial nerve or the trigeminal nerve

How Is This Diagnosis Made?

The first thing that’s usually noted is a long styloid process on x-ray. A normal one is about an inch long (25mm). A styloid process that’s longer than 30mm is considered abnormal. However, this is where you as a patient need to be VERY CAREFUL!  Why? 4-7% of everyone walking around out there have a long styloid process, Only 4% of patients with elongation of the styloid process show symptoms!

Isn’t there a highly accurate test that doctors perform to tell if I’m one of those 4% of patients with a long styloid bone that is causing my symptoms? NO. Most patients are operated on based on the x-ray (or CT scan) and the symptoms.

There is an ultrasound or x-ray guided diagnostic numbing injection that can be performed, but nobody is sure if that’s a 100% accurate way to chose patients who will respond to surgery. However, you should consider getting this done by THE RARE QUALIFIED EXPERT who understands how to perform this injection. If it’s a positive block, your pain and symptoms should go away for several hours.

Why Is My Styloid Bone Long?

Nobody is 100% sure why this happens. However, looking at the diagrams above, note that the styloid process is attached to the jaw through ligaments and muscles. Hence, chronic TMJ or neck issues causing too much force on the TMJ can pull on the bone causing it to get beat up and lengthen. This wear and tear can also cause the normal pliable ligament to turn to bone (ossify).

Do Other Things Cause These Symptoms?

YES. Many other conditions cause these same symptoms. Meaning that this 4% of patients with a long styloid bone and symptoms may actually have something else causing their pain. While I know that this may be a disconcerting message to hear when you believe you have finally found the cause of your symptoms, it’s a very critical one to absorb.

One of the biggest overlaps we see is Craniocervical Instability (CCI). All of these same cranial nerves can get irritated not by a long styloid bone, but by loose ligaments that hold the head causing too much movement of the skull on the spine. This can cause irritation of these nerves where they exit the skull. Hence operating on the neck and taking out a piece of this bone will do nothing to relieve these symptoms.

We have also been tracking a number of patients who got this diagnosis and ended up with surgery. Why? As a clinic, we have seen a rash of patients getting their styloid bones removed who are no better or worse after the procedure.

What Else Could Be Causing My Symptoms?

Many things cause neck pain that are MUCH more common than Eagle syndrome, including damaged neck joints, pinched neck nerves, loose ligaments, irritated muscles, etc… Other things cause facial pain such as TMJ syndrome and trigeminal neuralgia. Other things cause throat pain including local lesions in the throat.

Does Surgery Work?

The real answer is we really don’t know for sure, as there are NO gold-standard randomized controlled trials comparing surgery to no surgery. However, there are some lower-level studies that suggest that surgery may help. We do know that the surgery, because of all of the nerves and vessels near this area has potential complications. These include:

• A localized infection requiring IV antibiotics and/or additional surgery
• Trigeminal neuralgia (damage to this nerve that causes more face pain)
• Vascular injury leading to severe bleeding and requiring microsurgical repair of the vasculature
• Facial paralysis due to injury of the facial nerve

Are There Other Ways to Get Me Out of Pain?

USUALLY. Meaning in our experience, most patients who have been told they need surgery for Eagle Syndrome actually have other causes of their symptoms. Many different neck treatments can be applied to help these symptoms if the actual cause is a neck problem in the spine and not the long styloid process. Again, realize that 96% of patients with a long styloid process do not have Eagle syndrome.

A Surgeon Told Me that I NEEDED to Have this Removed

This messaging of severe problems like a stroke or other issue that could happen if the styloid bone is not cut out are surgical sales techniques. While for some patients with severe disease there may be additional risks, in our clinical experience, for the vast majority of patients who are told they need surgery, the risk of removing the bone is greater than the risk of stroke or puncture of vessels due to the elongated bone.

The upshot? We’re seeing a rash of patients who are being told they need to have surgery because they have Eagle Syndrome. The problem is that based on our analysis, while these patients have a long styloid process, it’s more likely that there are other causes of their neck pain. Hence, please seek out expert advice and multiple opinions before you sign up for this invasive surgery.

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References:

(1) Ceylan A, Köybaşioğlu A, Celenk F, Yilmaz O, Uslu S. Surgical treatment of elongated styloid process: experience of 61 cases. Skull Base. 2008;18(5):289-295. doi:10.1055/s-0028-1086057

(2) Anuradha V, Sachidananda R, Pugazhendi SK, Satish P, Navaneetham R. Bilateral Atypical Facial Pain Caused by Eagle’s Syndrome. Case Rep Dent. 2020 Feb 25;2020:3013029. doi: 10.1155/2020/3013029. PMID: 32181021; PMCID: PMC7063204.

(3) Saccomanno S, Greco F, DE Corso E, et al. Eagle’s Syndrome, from clinical presentation to diagnosis and surgical treatment: a case report. Acta Otorhinolaryngol Ital. 2018;38(2):166-169. doi:10.14639/0392-100X-1479

(4) Gokce C, Sisman Y, Sipahioglu M. Styloid Process Elongation or Eagle’s Syndrome: Is There Any Role for Ectopic Calcification?. Eur J Dent. 2008;2(3):224-228.

(5) K. C. Prasad, M. P. Kamath, K. J. M. Reddy, K. Raju, and S. Agarwal, “Elongated styloid process (Eagle’s syndrome): a clinical study,” Journal of Oral and Maxillofacial Surgery, vol. 60, no. 2, pp. 171–175, 2002.

(6) M. Ilgüy, D. Ilgüy, N. Güler, and G. Bayirli, “Incidence of the type and calcification patterns in patients with elongated styloid process,” Journal of International Medical Research, vol. 33, no. 1, pp. 96–102, 2005.

(7) T. Jung, H. Tschernitschek, H. Hippen, B. Schneider, and L. Borchers, “Elongated styloid process: when is it really elongated?” Dentomaxillofacial Radiology, vol. 33, no. 2, pp. 119–124, 2004.

(8) Sundaram S, Punj J. Randomized Controlled Trial Comparing Landmark and Ultrasound-Guided Glossopharyngeal Nerve in Eagle Syndrome. Pain Med. 2020 Jun 1;21(6):1208-1215. doi: 10.1093/pm/pnz370. PMID: 32167550.

(9) Blackett JW, Ferraro DJ, Stephens JJ, Dowling JL, Jaboin JJ. Trigeminal neuralgia post-styloidectomy in Eagle syndrome: a case report. J Med Case Rep. 2012 Oct 2;6:333. doi: 10.1186/1752-1947-6-333. PMID: 23031688; PMCID: PMC3492092.

(10) Pigache P, Fontaine C, Ferri J, Raoul G. Transcervical styloidectomy in Eagle’s syndrome. Eur Ann Otorhinolaryngol Head Neck Dis. 2018 Dec;135(6):433-436. doi: 10.1016/j.anorl.2018.05.001. Epub 2018 Aug 31. PMID: 30174260.