ES Information: Common Symptoms And Possible Explanations For Their Causes:
Disclaimer: These answers to common questions were put together for new members to gain a head start in finding information about Eagle Syndrome; to give you confidence to discuss issues with your medical team and to encourage you to research issues further for yourselves. It was compiled by a volunteer Moderator, who does not claim to be a medical professional, merely an informed observer and patient!
The sources used are personal experiences, LivingWithTheEagle members’ experiences, and research from professional publications (some of the articles can’t be read fully unless subscribed to). Many thanks to heidemt for her research and contribution, and for her example of being your own advocate and not giving up. Members are encouraged to seek medical opinion and these pages are not intended to replace that. Members are also encouraged to research more for themselves- there is more research available but with the limitation of time and neck pain, this was the best that I could do! Past discussions are useful sources of info as well; search whatever the subject is, and you’ll often find someone who’s been through it too!
The symptoms most doctors are aware of are
*the sensation of a foreign body in the throat, and
*difficulty swallowing or painful swallowing (dysphagia/ odynophagia).
But it’s quite possible to have ES without these symptoms. The symptoms can also vary depending on whether you have Classic or Vascular ES, and in my opinion it is possible to have both, so you might get a double dose!
Other common symptoms are
- lateral neck and oropharyngeal pain (in the angle of the mandible, submandibular space and upper neck), made worse by head and tongue movements; with speaking, swallowing, chewing, yawning, head turning and other oral and cervical movements.
*Also swelling in the submandibular area,
*otalgia (earache or pain),
*reduced hearing or hypersensitive hearing,
*pulsatile tinnitus (hearing your heartbeat, often a whooshing sound), and
Other less common symptoms I have found reference to include:
- hypersalivation and
*changes in vocal quality (Management of Dysphagia Pre and Postoperatively In a Case Of Eagle’s Syndrome by Lewis, Hoffman, Spector In 2015).
Also in Fusco, Asteraki, and Spetzler’s (Eagle’s Syndrome Embryology, Anatomy, and Clinical management, 2012) study, they mentioned
*sore throats, and
*also rarely alteration in taste or
MusicGeek also posted an article by a Professor who had to leave her job as she was unable to talk properly, due to ES: Survey Results
A study by Monsour and Young found that:
*subjects with elongated styloid processes had the highest incidences of dysphagia (difficulty swallowing),
*whereas subjects with calcified ligaments had higher incidences of discomfort turning their head side to side.
Member Ang1 put together a small survey back in 2013. Unfortunately with the changeover of the site, the final results do seem to have disappeared. I have a record of the first survey results, which is only 20 respondents, but still an interesting read. Here’s a link: Survey Results
The additional symptoms members attributed to ES either in the study or on the forum include
*limited movement in shoulder and/or neck, *palpitations,
*pressure in head/ ears,
*Thoracic Outlet Syndrome (TOC),
*episodes of feeling unwell,
*Burning Mouth Syndrome (BMS),
*Trigeminal, Geniculate or Glossopharyngeal Neuralgia,
*partial facial paralysis.
Having symptoms worsen in different positions is common, and in different weathers. For more info it’s worth using the search function for discussions on particular symptoms.
Symptoms common with Vascular ES are
The styloid process is located between the External and Internal Carotid arteries, so if it is angulated, it can compress either of these. The stylohyoid ligament, if compressed, can aIso compress the arteries.
It is possible that compression of the External or Internal Carotid arteries could temporarily cut off part of the blood supply to the brain, leading to a temporary loss of consciousness. Many people have found turning or moving their head into a certain position causes this, as this can move the styloid process or calcified ligament and so compresses the artery.
In addition, pressure on these arteries can irritate the sympathetic nerve fibres in the artery walls, and this can send pain signals all along the artery. The ICA branches to the Ophthalmic artery, so if blood flow is reduced to this, there will be eye pain, and vision problems. Research states that if the ECA is compressed, pain is in the infraorbital (below the eye and to the side of the nose), temporal, and mastoid regions (below and behind the ear). If the ICA is compressed, then pain is in the ophthalmic area (E.Beder, Ozgursoy, Karatayli: Current Diagnosis and Transoral Surgical Treatment Of Eagle’s Syndrome).
And also ‘Hence, if the external carotid artery is affected, the patient may complain of pain in the neck on turning the head, or pain radiation to the eye, ear, angle of the mandible, soft palate and nose.
When the internal carotid artery is involved, pain over the entire head and larynx may be involved.’ (Correll RW, Jensen JL, et al. Mineralization of the stylohyoid-stylomandibular ligament complex. Oral surg Oral med Oral path 1979.)
Pressure on the ECA can also contribute to jaw pain. Dizziness could be caused by compression of the Hering Nerve, which is a branch of the Glossopharyngeal Nerve, and connects to the carotid sinus to help regulate blood pressure. Also if there is compression or irritation of the carotid sinus- the area just before the carotid artery splits into the ECA and the ECA- this can affect the vagus nerve, and through the parasympathetic nervous system can affect blood pressure and heart rate.
The symptoms if the Jugular veins are compressed are slightly different. Because the veins take the blood flow from the brain, any compression of these interrupts the blood flow coming from the brain, and so therefore can increase the pressure in the brain (Intracranial Pressure- ICP). ). Over a long period sometimes other veins can compensate and take the blood flow away (‘venous collateral drainage’- Callahan et al). If the pressure in the brain increases, this is known as Intracranial Hypertension. Symptoms of this are also dizziness, plus headaches, tiredness, slow or confused thinking (brain fog), feeling of pressure or pulsing in the head and neck, feeling of pressure in the ears, pulsatile tinnitus (hearing heartbeat, often a whooshing sound), feeling generally ‘out of it’ or off-balance. It can also cause other neurological symptoms such as feelings of falling, or feelings of pressure on the head ‘like you’re wearing a hat’.
The impact of elongated Styloid processes compressing the jugular veins is the subject of a research paper- ‘New Eagle’s Syndrome Variant Complicating Management Of Intracranial Hypertension After Traumatic brain Injury’, by Callahan, Kang, Dudekula, Eusterman and Rabb- where there authors conclude ‘We believe that this demonstration of venous compression constitutes a new variant of Eagles Syndrome… This variant of Eagle’s Syndrome may represent a rare cause of such venous insufficiency that should be in the differential diagnosis of unexplained or disproportionately elevated ICP.’ The authors stated that the patient’s styloid processes were ‘elongated and posteriorly positioned’.
Another interesting research article is Styloidogenic Jugular Venous Compression Syndrome: Daignosis and Treatment, A Case Report by Dashti et al, 2012, Neurosurgery: http://www.ncbi.nlm.nih.gov/pubmed/21866063
The area where the styloid process and the stylohyoid ligaments lie is a very cramped space, full of blood vessels and Cranial Nerves. Any elongation, angulation or calcification could cause inflammatory changes, which can impinge on the surrounding blood vessels and nerves.
To give you an idea of how crowded an area it is:
‘The temporal styloid process is a slender bone lying just below the ear, anterior to the mastoid process and between the internal and external carotid arteries, with the internal jugular vein and the glossopharyngeal, vagus, hypoglossal, and accessory nerves lying medial’. (Strauss M, Zohar Y, Laurian N. Elongated styloid process syndrome: intraoral versus external approach for styloid surgery.)
‘The styloid process is a bony outgrowth of the temporal bone located between the internal and external carotid arteries and juxtaposed near cranial nerves VII, IX, X, XI (accessory), and XII.’(Kim E, Hansen K, Frizzi J.Eagle Syndrome: Case report and review of literature.) Eagle’s syndrome: A case of symptomatic calcification of the stylohyoid ligaments, by Victor B Feldman.
‘Symptoms are divided into two groups. The first group of symptoms, are characterized by pain located in the areas where the fifth, seventh, eighth, ninth and tenth cranial nerves are distributed and occurs in most of the cases after tonsillectomy which may have been performed many years earlier.’ (Dolan, Mullen, Papyoanou).
‘Pain following tonsillectomy is presumably created by stretching or compressing the nerve or nerve endings of cranial nerves V, VII, VIII, IX, or X in the tonsillar fossa either during healing (scar tissue) or shortly thereafter.’ (Langlais RP, Miles DA, Van Dis ML. Elongated and mineralized stylohyoid ligament complex: A proposed classification and report of a case of Eagle’s syndrome, 1986).
The attachments to the styloid process are 3 muscles and 2 ligaments:
*the stylopharyngeus muscle which helps to control the larynx and pharynx;
*the styloglossus muscle which goes from the styloid process to the hypoglossus muscle, which connects to the tongue, and is part of the floor of the submandibular triangle;
*the stylohyoid muscle/ ligament which links the styloid process to the hyoid bone, and serves to move the hyoid bone and lift the tongue;
*and the stylomandibular ligament which limits excessive jaw opening.
If the styloid process is elongated or the ligaments are calcified and causing irritation in this area then there can be pain and limited movement.
A new diagnostic classification of Stylohyoid Complex Syndrome (SHCS) was suggested by Candice C. Colby, MD; John M. Del Gaudio, MD in their paper ‘Stylohyoid Complex Syndrome- A New Diagnostic Classification’ (JAMA Network), and they suggested that an elongated styloid process, ossified stylohyoid ligament or elongated hyoid bone could result in tension and reduced distensibility of the SHC, resulting in irritation of the surrounding cervical structures with movement of the complex.
I’ve found a few variations on which of the cranial nerves can be affected by the styloid processes or calcified ligaments, and what effects there can be with compression/ damage to those nerves, so this summary is the best I could do!
As the styloid process lies close to the Facial Nerve (Cranial Nerve VII), which supplies the External Auditory Canal, compression of this could cause ear pain.
CN VII is also responsible for facial muscles and expressions (some members have found facial expressions are affected); taste, so explaining possible altered taste; and also for eye and salivary gland function, which might possibly explain the dry eyes and mouth or hyper-salivation some members have experienced.
I have also seen hyperacusis mentioned- hypersensitivity to some sounds; again this is something which members have noticed.
(http://calder.med.miami.edu/pointis/tbiprov/MEDICINE/sense1.html1) The Vestibulocochlear Nerve, Cranial Nerve VIII, if damaged can cause positional vertigo, hearing loss, and tinnitus. Again, I haven’t found much research about this symptom/ damage to this nerve, but found in one research paper ‘The first group of symptoms, [concerning classic symptoms as compared to vascular symptoms] are characterized by pain located in the areas where the fifth, seventh, eighth, ninth and tenth cranial nerves are distributed and occurs in most of the cases after tonsillectomy which may have been performed many years earlier’. (Source Dolan EA, Mullen JB, Papayoanou J. Styloid-Stylohyoid Syndrome in the Differential Diagnosis of Atypical Facial Pain. Surg Neurol 1984, in the paper: Eagle’s syndrome: a case of symptomatic calcification of the stylohyoid ligaments, by Victor B Feldman).
If the Glossopharyngeal Nerve (Cranial Nerve IX) is pulled across the Styloid Process, then it can cause sharp, stabbing pain to the Temporomandibular Joint region, the ear, the temple, throat, tongue and neck (Anniker Isberg).
The stylohyoid ligament lies next to the Glossopharyngeal Nerve, which also supplies the External Auditory Canal, so again could cause ear pain, and as the GP Nerve is responsible for pain sensation in the pharynx, this explains the lancing pain in the throat, and why swallowing can be painful. It also innervates the stylopharyngeus muscle, part of the tongue, explaining tongue pain, and the parotid glands, which could explain swelling/ tenderness some members have experienced, plus damage can cause difficulty swallowing, feeling of food stuck in throat, choking and drooling. The feeling of something stuck in the throat can be a symptom of this nerve being irritated, not necessarily that the styloid itself is poking into the throat.
(Eagle’s Syndrome is listed as one of the possible causes of Glossopharyngeal Neuralgia, or GPN. GPN causes intense, shooting pains in the back of the tongue and throat, tonsillar areas, and middle ear. There is a Ben’s Friends page for GPN which is worth looking at for more information on this if you think you’re affected).
In the research paper ‘The glossopharyngeal nerve, glossopharyngeal neuralgia and the Eagle’s syndrome–current concepts and management’, the author K.B. Soh states ‘Eagle’s syndrome due to an elongated styloid process is the most important cause of secondary glossopharyngeal neuralgia’. (One symptom of GPN can be ‘globus’- the sensation of something poking/ stuck in the throat, but this ‘foreign body’ sensation can also often be caused by GERD, which explains why often members are diagnosed and treated for this before ES is found).
The pharynx is also innervated by the Vagus Nerve (CN X), and part of the Cranial/ Spinal Accessory Nerve (CN XI), which exit the skull in this region, so if these are compressed it could be another explanation of why swallowing can be painful.
Motor branches of CN X are responsible for phonation, so compression of this part of the nerve could explain hoarseness or vocal changes.
The Vagus nerve also is part of the parasympathetic nervous system (regulation of the body’s unconscious/ involuntary actions, like heart rate, and digestion), and innervates the heart, lungs, and some of the digestive tract. If this nerve is affected by an elongated SP or calcified ligaments, it would explain heart palpitations etc.
The Spinal Accessory Nerve, CNXI, has motor function of the shoulder muscles- the Sternocleidomastoid and the Trapezius muscle, explaining problems with this area.
The Hypoglossal nerve (CN XII) innervates the stylohyoid muscle, so calcification could cause compression and this may explain tongue weakness and the inability to stick the tongue right out, which again is something some people have experienced, and also swallowing difficulties. Monsour and Young (1986) thought that ossification of the Stylohyoid ligament complex could cause contraction of the stylopharyngeal muscle, which could stretch CN XII.
Another sensory nerve branch, upper branches of the cervical plexus, travel with CN XII, so could contribute to neck pain if they are compressed. Trigeminal Nerve branches (CN V) are also in this region- Eagles Syndrome is listed as a possible cause of Trigeminal Neuralgia (TN)/ Non-Neuropathic Facial Pain. TN causes two types of symptoms- Type1 is sudden intense, electric- shock like, stabbing, or burning pains which can also cause muscle spasms, and Type 2 is a constant aching, boring or burning pain.
The Trigeminal Nerve has 3 branches, which carry sensations of pain and touch. The branches go along the lower jaw, into the lower teeth and the temple region; the upper jaw, teeth, cheek, part of the nose and just below the eye; and across the scalp, forehead, and the eye. The motor branches of the Trigeminal Nerve also innervate muscles for chewing, and sensory branches go to the mucous membranes of the eyes and nose, and symptoms of damage can be dry eyes and mouth. (http://calder.med.miami.edu/pointis/tbiprov/MEDICINE/sense1.html1).
I have seen research showing that it is the lower branch (Mandibular) of the Trigeminal Nerve which is most commonly affected by Eagle’s Syndrome. In one documented case, the TN pain the patient was suffering was eased by a partial removal of the styloid, but then returned. The patient later had a full styloidectomy, and again the pain eased, before returning years later, and with more severe TN. The researchers state ‘This suggests that trigeminal neuralgia often exists in a prodromal state characterized by dull constant jaw pain, particularly if there is a distal structural defect to exacerbate the pain. The underlying abnormality that eventually causes classical trigeminal neuralgia may be present in an early form. This early form might not be capable of causing facial pain on its own, but might sensitize distal trigeminal nerve branches to local compression or stretching. It might sensitize the alveolar nerves to pain in the jaw from dental problems. (or in our case from an elongated styloid process and scar tissue.) Eagle syndrome… may also be associated with pain in a trigeminal V3 distribution.
Although the glossopharyngeal nerve is most commonly implicated in Eagle syndrome, involvement of the mandibular nerve is possible. Eagle syndrome should be considered a possible etiology of dull pain along the jaw line or temple. Pain in this distribution is an uncommon but possible symptom of Eagle syndrome that is easily confused with other sources of facial discomfort, such as temporomandibular joint disorder or dental pain.’ (Trigeminal neuralgia post-styloidectomy in Eagle syndrome: a case report by JW Blackett, DJ Ferraro, JJ Stephens, JL Dowling, JJ Jaboin.)
Forgive me for going into more detail about the TN angle- but I have heard of at least one member who may have had an unnecessary MVD procedure for TN! Again, there is a Ben’s Friends site for TN, and one of the Moderators on that site has researched and written extremely useful face pain information, so it’s well worth looking at their site if you think you have TN.
Also on the Ben’s Friends TN site is a link to an interesting talk by Dr. Ken Casey, where he discusses nerve pain, and the effects of long term nerve pain: https://vimeo.com/10284243
Because of the motor function of this nerve, damage can cause weakness or spasms in the jaw, and as it is the most important sensory nerve of the head and face, and a common cause of headaches, damage to this nerve can therefore cause headaches. Also parasympathetic nerves which are not part of the CNV travel alongside it. These supply the lacrimal glands in the eye, nasal glands and also some salivary glands. This is just speculation on my part, but maybe damage to this nerve could cause dryness in the eyes, nose or mouth; I haven’t found any research on this. (These symptoms should be looked into though, as there is an Autoimmune disease called Sjogrens Syndrome which causes the same symptoms, and untreated dryness in the eyes can cause corneal abrasions and ulcers.)