David S. Klein, M.D.

Eagle's Syndrome

NOTE- Look below for a new, minimally invasive treatment option.  I am currently evaluating a treatment technique that can be performed at home, by the patient, using a new, single-use electrode.  See the bottome of page.

Non-invasive option #2:  Transdermal ketoprofen/gabapentin admixture.

Stay tuned........ should have on line soon.  dsk

Synonyms: Hyoid syndrome, styloid elongation syndrome, stylohoid syndrome, styloid syndrome, styloid processs syndrome, styloid process-carotid artery syndrome, styloid-stylohoid syndrome.

Eagle Syndrome is caused by irritation of the eleventh cranial nerve (CN XI) caused by trauma to the region near the attachment of the Sternocleidomastoid Muscle at the Mastoid Process.  Predisposition to the development of this problem can result from either the overgrowth of the styloid process itself or the ossification of the stylohyoid ligament, first described by Watt Eagle, an otolaryngologist in Duke University in 1937.

Eagle described two distinct group of patients: the  post-tonsillectomy patients for whom the palpation of the styloid process/ossified stylohyoid ligament reproduced the symptoms (and he believed that the post-surgical changes caused incorporation of the glossopharyngeal nerve by the scar tissue/ossification), and patients with no history of prior tonsillectomy who complained of pain along the distribution of the carotid artery.   

Trauma most likely to result in Eagle's Syndrome involves rotational forces of the head, whereby the development of Eagle's Syndrome occurs on the side opposite of rotation, for example, the right neck following rapid rotation to the left side. 

Symptoms:

The symptoms of Eagle's syndrome are a foreign-body sensation in the throat, dysphagia, and intermittent facial pain related to an elongated styloid process and a calcified stylohyoid ligament.

Eagle described a group of patients who had symptoms of intermittent and nagging pain in the pharynx that radiated to the mastoid region, a foreign-body sensation in the throat, dysphagia, and taste disturbance.  His original patients had a history of tonsillectomy that resulted in scar tissue in the tonsillar fossa.

Eagle believed that the scar tissue incorporated branches of the glossopharyngeal nerve. Radiographs demonstrated an ossified stylohyoid ligament.   The diagnosis of Eagle's syndrome is made by radiographic imaging and by physical examination when an elongated and calcified stylohyoid ligament can be palpated in the tonsillar area. In the case illustrated here, the heavily calcified and thickened stylohyoid ligament was clearly identified on digital scout radiography  prior to CT, as well as on axial images.

Although approximately 4% of the general population is thought to have an elongated styloid process and a calcified stylohyoid ligament, only a small percentage of this group (4 to 10%) is symptomatic. Nearly all of these calcified stylohyoid ligaments are considered to be anatomic variants that are of no clinical concern.  The stylohyoid apparatus has four distinct segments: tympanohyal, stylohyal, ceratohyal, and hypohyal. The ligamentous part of that apparatus originates in the ceratohyal cartilage and extends from the stylohyoid to the lesser cornu of the hyoid bone.  More important than the elongation of the styloid process and the calcification of the stylohyoid ligament is the thickening or ossification of those structures. This ossification should be differentiated from an incidental calcification of the stylohyoid ligament in asymptomatic individuals. The cause of stylohyoid calcification is not well understood, but it might be related to congenital factors such as persistence of a cartilag inous analog or an embryonic precursor to the styloid process.

Eagle's syndrome is a relatively common disorder that is frequently misdiagnosed. It occurs more frequently in women. Presenting symptoms generally include posterior oropharyngeal pain, intermittent glossitis, and phantom foreign body discomfort of the pharynx. Surgical removal of elongated styloid processes generally provides relief of symptoms. The elongation of the styloid process and stylohyoid ligament calcification. The tissues in the throat rub on the styloid process, which is a spike-like projection sticking off the base of the skull, during the act of swallowing with resulting pain along the glossopharyngeal nerve. There is also pain upon turning the head or extending the tongue. Other symptoms may include voice alteration, cough, dizziness, sinusitis or bloodshot eyes.

Ernest or Eagle's syndrome, a problem similar to temporomandibular joint pain, involves the stylomandibular ligament, a structure that connects the styloid process at base of the skull with the hyoid bone. Symptoms of Ernest syndrome, in decreasing order of occurrence, are: temporomandibular joint and temporal pain, ear and mandibular pain, posterior tooth sensitivity, eye pain, and throat pain (especially when swallowing). Treatment of Ernest syndrome, which is successful about 80 % of the time, includes a soft diet, use of an intra-oral splint, physiotherapy, medication and injections of local anesthetics at the insertion of the ligament.

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.

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 a 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. Elongated styloid process syndrome, which is synonymous with Eagle's syndrome, is a rare disease. Although an elongated styloid process is not always symptomatic, if it compresses the cranial nerves and/or carotid artery, a patient can present with sore throat, dysphasia, or dysphoria of the throat. We present two cases of elongated styloid process syndrome, focusing on the imaging findings. The elongated styloid processes were visualized by panoramic photograms. However, CT images, especially 3DCT images, were useful in visualizing the elongated styloid process itself as well as the relationship between the styloid process and surrounding soft tissue.

What are the symptoms of Eagle syndrome?

People with Eagle syndrome may experience a wide range of symptoms, including pain in both the throat and the ear, vertigo, voice alteration, cough, dizziness, sinusitis or bloodshot eyes. It may feel like something is stuck in one's throat, and swallowing may be difficult. It may also be painful to turn one's head. Pain during swallowing, opening the mouth or turning the head may also be experienced.

Bibliography:

W. W. Eagle: Elongated styloid process.  Archives of Otolaryngology, Chicago, 1937, 25: 584-587

Elongated styloid processes: report of two cases. Archives of Otolaryngology, Chicago, 1937, 25: 584–587.

Elongated styloid process: further observations and a new syndrome. Archives of Otolaryngology, Chicago, 1948, 47: 630–640.

Symptomatic elongated styloid proces: report of two cases of styloid process-carotid artery syndrome with operation.  Archives of Otolaryngology, Chicago, 1949; 49: 490-503.

Elongated styloid process: symptoms and treatment. Archives of Otolaryngology, Chicago, 1958, 64: 172-176

The symptoms, diagnosis and treatment of the elongated styloid process. The American Surgeon, Philadelphia, 1962, 28: 1-5.

Shankland WE 2nd. Ernest syndrome as a consequence of stylomandibular ligament injury: A report of 68 patients. J Prosthet Dent. 1987;57(4):501-506.

Wilk SJ. Surgical management of refractory craniomandibular pain using radiofrequency thermolysis: A report of thirty patients. Cranio. 1994;12(2):93-99.

Brown CR. Ernest syndrome: Insertion tendinosis of the stylomandibular ligament. Pract Periodontics Aesthet Dent. 1996;8(8):762.

DuPont JS Jr. Panoramic imaging of the stylohyoid complex in patients with suspected Ernest or Eagle's syndrome. Cranio. 1998;16(1):60-63.

Slavin KV. Eagle syndrome: entrapment of the glossopharyngeal nerve? Case report and review of the literature. J Neurosurg. 2002 Jul;97(1):216-8.

New approach:

Empi Corporation offers their Iontophoresis 'Action patch' which is a self-contained iontophoresis system that permits the application of medications into the patch for delivery through the skin to the desired target tissues.  The physician can custom-configure the medication mixture, and have the patient apply the medication at home, thereby saving tremendously in time lost, and in unnecessary physician or Physical therapy office visits.

So far, it seems to be working.  After 4 treatments, pain relief is moderate, but activity tolerance has improved dramatically.  Will go for 4 more treatments. 

David S. Klein, MD, FACA, FACPM, FACMIMS
Director, Pain Center of Orlando
www.suffernomore.com