Eagle Syndrome - Rare misunderstood cause of nerve pain
updated April 2021
What is Eagle Syndrome? In the most basic terms, it is a syndrome whereby there is calcified bone and ligament in the neck area. It is not uncommon for calcified bone and ligaments to form in the feet resulting in heel spurs and plantar fasciitis. It is less common to find this in the neck. More specifically, the calcified bone is called the styloid process and it extends from the base of the skull under the ear and extends down into the neck along the throat and jaw line. If this bone calcifies and elongates, it may get entangled in the nerves in the neck. This can be extremely painful and is now referred to as Eagle syndrome, named after the doctor who first discovered this connection.
This is my favorite picture to illustrate the relationship of the nerves in the neck to this bone (the styloid process) in the simplest terms. It was posted from an article called An uncommonly common: Glossopharyngeal neuralgia (Singh P M, Kaur M, Trikha A. An uncommonly common: Glossopharyngeal neuralgia. Ann Indian Acad Neurol 2013;16:1-8).
I can't emphasize enough how misunderstood this relationship is between the nerves and the styloid process. Even after I learned that this could cause all kinds of neuralgia, no one believed it was possible in my child. I have learned that the length of the bone is not always correlated to the damage and pain it can cause. It can depend on many factors such as angle of the bone, placement of the nerves in relation to that bone, whether or not there is calcified ligament, etc. The bad news is that there is no real test to definitively state that a person is suffering from this disorder. There is CT scan of the neck that can show the length of the styloid process. Most radiologists will not automatically look for this or measure the bone unless specifically asked to rule out Eagle syndrome, or specifically asked to measure the length of the styloid process. And despite that information, it is still almost impossible to have a doctor agree with any level of certainty that this bone is causing nerve pain. Unfortunately, the only way to find out is to have the bone removed surgically. This surgery is called a styloidectomy. And there are only a handful of doctors world-wide with experience performing this type of surgery.
This entanglement of the nerves can happen at any age, at any time, and with a styloid of any length. It can happen on one side only or on both sides (bilateral). My son had bilateral Eagle syndrome. His styloids weren't long enough by adult research standards, but after two delicate surgeries, he was completely pain-free. After each styloidectomy his palatal myoclonus subsided to almost nothing (unless he got sick—then the nerve pain would come back temporarily). In addition, after the styloidectomies, Jacob's myoclonus of his diaphragm disappeared altogether. The jerking in his arms and pain in his legs disappeared as well. Post-surgeries, he could eat without the throat pain and ear pain that would send him away from each meal screaming in pain. This was another huge win in our favor and our son was well on his way to healing.
Unfortunately, for some, symptoms of Eagle syndrome may recur, even after surgery. It is not always clear why. My son's symptoms of GPN returned approximately one year later due to new bone growth. When the pain recurred, the clicking from his PM also returned. This was a devastating turn of events. His pain was life altering, and prevented him from being able to function normally—again. Subsequent revision styloid surgeries confirmed new bone growth, and removal of those bones successfully eliminated the nerve pain. A pain-free life is possible after Eagle syndrome. For more information about my son's case, see my second book Connecting the Dots. This book includes symptoms year by year, medications and dosing, alternative treatment therapies, and surgeries along with surgical pictures.
CONNECTION TO PALATAL MYOCLONUS
As previously stated, the connection to palatal myoclonus may never be fully clear, however, it is clear that there is a connection between the nerves and the signals that set off the rhythmic contractions of the soft palate. Eagle syndrome, or the calcification and elongation of the styloid process in the neck, can cause nerve pain as that bone can become entangled or pull on the nerves that are present in the neck. The glossopharyngeal nerve can impact the soft palate.
Continue to read on the next tab, Lyme Disease
This is my favorite picture to illustrate the relationship of the nerves in the neck to this bone (the styloid process) in the simplest terms. It was posted from an article called An uncommonly common: Glossopharyngeal neuralgia (Singh P M, Kaur M, Trikha A. An uncommonly common: Glossopharyngeal neuralgia. Ann Indian Acad Neurol 2013;16:1-8).
I can't emphasize enough how misunderstood this relationship is between the nerves and the styloid process. Even after I learned that this could cause all kinds of neuralgia, no one believed it was possible in my child. I have learned that the length of the bone is not always correlated to the damage and pain it can cause. It can depend on many factors such as angle of the bone, placement of the nerves in relation to that bone, whether or not there is calcified ligament, etc. The bad news is that there is no real test to definitively state that a person is suffering from this disorder. There is CT scan of the neck that can show the length of the styloid process. Most radiologists will not automatically look for this or measure the bone unless specifically asked to rule out Eagle syndrome, or specifically asked to measure the length of the styloid process. And despite that information, it is still almost impossible to have a doctor agree with any level of certainty that this bone is causing nerve pain. Unfortunately, the only way to find out is to have the bone removed surgically. This surgery is called a styloidectomy. And there are only a handful of doctors world-wide with experience performing this type of surgery.
This entanglement of the nerves can happen at any age, at any time, and with a styloid of any length. It can happen on one side only or on both sides (bilateral). My son had bilateral Eagle syndrome. His styloids weren't long enough by adult research standards, but after two delicate surgeries, he was completely pain-free. After each styloidectomy his palatal myoclonus subsided to almost nothing (unless he got sick—then the nerve pain would come back temporarily). In addition, after the styloidectomies, Jacob's myoclonus of his diaphragm disappeared altogether. The jerking in his arms and pain in his legs disappeared as well. Post-surgeries, he could eat without the throat pain and ear pain that would send him away from each meal screaming in pain. This was another huge win in our favor and our son was well on his way to healing.
Unfortunately, for some, symptoms of Eagle syndrome may recur, even after surgery. It is not always clear why. My son's symptoms of GPN returned approximately one year later due to new bone growth. When the pain recurred, the clicking from his PM also returned. This was a devastating turn of events. His pain was life altering, and prevented him from being able to function normally—again. Subsequent revision styloid surgeries confirmed new bone growth, and removal of those bones successfully eliminated the nerve pain. A pain-free life is possible after Eagle syndrome. For more information about my son's case, see my second book Connecting the Dots. This book includes symptoms year by year, medications and dosing, alternative treatment therapies, and surgeries along with surgical pictures.
CONNECTION TO PALATAL MYOCLONUS
As previously stated, the connection to palatal myoclonus may never be fully clear, however, it is clear that there is a connection between the nerves and the signals that set off the rhythmic contractions of the soft palate. Eagle syndrome, or the calcification and elongation of the styloid process in the neck, can cause nerve pain as that bone can become entangled or pull on the nerves that are present in the neck. The glossopharyngeal nerve can impact the soft palate.
Continue to read on the next tab, Lyme Disease
Links to other sites:
Link to Eagle's Syndrome defined
Link to the Ben's Friends Living with Eagle's Syndrome Site
1. Radiologic Case of the Month. A pediatric case of Eagle’s syndrome. (Am J Dis Child. AJDC–Vol 145(3): 339–40, March 1991) A 5 year old ends up in the ER with foreign object sensation in the throat. http://www.ncbi.nlm.nih.gov/pubmed/2003486 (no abstract available)
Items 2–5 were found on Pub Med Central Canada:
2. Significance of styloidectomy in Eagle’s syndrome (Indian Journal of Otolaryngology and Head & Neck Surgery 2010;61(4)262–265) 28 cases of Eagle’s syndrome, unilateral and bilateral, are examined and followed over time after styloidectomy. Total ear and throat pain relief for 27 out of 28 with styloid processes measuring anywhere from 12–40mm. Patients measuring between 12 & 20 mm also had the glossopharyngeal nerve cut during removal of the styloid process.
3. Stylalgia and its surgical management (Indian Journal of Otolaryngology and Head & Neck Surgery 2003;55(2):87–90) Summary of 332 cases stating unilateral versus bilateral statistics. Two patients as young as 15 were cited in the article.
4. Symptomatic Elongated Styloid Process (Journal of Dental Research, Dental Clinics, Dental Prospects (JODDD) 2008;2(3)102–105 ) Summary of symptoms associated with Eagle’s syndrome and a case analysis.
5. Transoral surgical resection of bilateral styloid processes elongation (ACTA Informatica Medica 2012;20(2):133–135) Summary of a bilateral case of Eagle’s operated on transorally—3D pictures included.
6. Another case report from Pub Med Central Canada
7. Stylohyoid chain ossification Part I (Oral Surg Oral Med Oral Pathol 1989;67:508–14) Calcification versus ossification of the styloid and causes discussed.
8. Stylohyoid chain ossification (Part II): A discussion of etiology. (Oral Surgery, Oral Medicine, Oral Pathology Volume 67, Issue 5, May 1989, Pages 515–520 Article regarding mean length of styloid processes in children of 10mm with a mean age of 11 years old. Link to the abstract
9. Entrapment of the glossopharyngeal nerve in patients with Eagle Syndrome: surgical technique and outcomes in a series of 5 patients. ( J Neurosurg 111:1226–1230, 2009) Article explains in detail how the styloid process can irritate or entangle in the glossopharyngeal and other nerves. Multiple cases are discussed including successful surgical removal of styloid processes.
These are other helpful links already on the Ben's Friends, Living with Eagle’s website buried in discussions:
Link to Eagle's Syndrome defined
Link to the Ben's Friends Living with Eagle's Syndrome Site
1. Radiologic Case of the Month. A pediatric case of Eagle’s syndrome. (Am J Dis Child. AJDC–Vol 145(3): 339–40, March 1991) A 5 year old ends up in the ER with foreign object sensation in the throat. http://www.ncbi.nlm.nih.gov/pubmed/2003486 (no abstract available)
Items 2–5 were found on Pub Med Central Canada:
2. Significance of styloidectomy in Eagle’s syndrome (Indian Journal of Otolaryngology and Head & Neck Surgery 2010;61(4)262–265) 28 cases of Eagle’s syndrome, unilateral and bilateral, are examined and followed over time after styloidectomy. Total ear and throat pain relief for 27 out of 28 with styloid processes measuring anywhere from 12–40mm. Patients measuring between 12 & 20 mm also had the glossopharyngeal nerve cut during removal of the styloid process.
3. Stylalgia and its surgical management (Indian Journal of Otolaryngology and Head & Neck Surgery 2003;55(2):87–90) Summary of 332 cases stating unilateral versus bilateral statistics. Two patients as young as 15 were cited in the article.
4. Symptomatic Elongated Styloid Process (Journal of Dental Research, Dental Clinics, Dental Prospects (JODDD) 2008;2(3)102–105 ) Summary of symptoms associated with Eagle’s syndrome and a case analysis.
5. Transoral surgical resection of bilateral styloid processes elongation (ACTA Informatica Medica 2012;20(2):133–135) Summary of a bilateral case of Eagle’s operated on transorally—3D pictures included.
6. Another case report from Pub Med Central Canada
7. Stylohyoid chain ossification Part I (Oral Surg Oral Med Oral Pathol 1989;67:508–14) Calcification versus ossification of the styloid and causes discussed.
8. Stylohyoid chain ossification (Part II): A discussion of etiology. (Oral Surgery, Oral Medicine, Oral Pathology Volume 67, Issue 5, May 1989, Pages 515–520 Article regarding mean length of styloid processes in children of 10mm with a mean age of 11 years old. Link to the abstract
9. Entrapment of the glossopharyngeal nerve in patients with Eagle Syndrome: surgical technique and outcomes in a series of 5 patients. ( J Neurosurg 111:1226–1230, 2009) Article explains in detail how the styloid process can irritate or entangle in the glossopharyngeal and other nerves. Multiple cases are discussed including successful surgical removal of styloid processes.
These are other helpful links already on the Ben's Friends, Living with Eagle’s website buried in discussions:
- The Glossopharyngeal Nerve, Glossopharyngeal Neuralgia and the Eagle’s Syndrome—Current Concepts and Management by K B K Soh printed in the Singapore Medical Journal (Singapore Med J 1999; Vol 40(10))
- Elongated styloid process and Eagle's syndrome.Montalbetti L1, Ferrandi D, Pergami P, Savoldi F. printed in Pub Med US Library of Medicine National Institute of Health