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Review Article

Eagle syndrome: A review of current diagnostic criteria


and evaluation strategies
Vishlesh Arora, Arvind Shetti, Vaishali Keluskar
Department of Oral Medicine and Radiology, Institute of Dental Sciences, Belgaum, Karnataka, India

ABSTRACT
The mineralized and elongated styloid process and Eagle’s syndrome are similar processes of elongation in which mineralization of
the stylohyoid ligament leads to styloid process of the temporal bone. The mineralized and elongated styloid process and Eagle’s
syndrome differ significantly in terms of the symptoms displayed and the treatment modalities that are sought. The mineralized and
elongated styloid process refers to unilateral or bilateral elongation of the styloid process that does not result in any significant pain,
discomfort, or limitation of neck movement. It often remains asymptomatic until it is discovered on extraoral radiographs. Eagle’s
syndrome refers to pain and discomfort in the cervicofacial region resulting specifically from the elongated styloid process. Surgical
shortening may be the only treatment that will alleviate the patient’s symptoms.This article reviews the entire process of elongation
pertaining to the styloid process and discusses the associated syndromes, including current knowledge of the theories of elongation,
diagnostic criteria and treatment strategies.

Key words: Eagle’s syndrome, elongation and styloid process

INTRODUCTION process, although it has been reported as starting at the


lower end of the stylohyoid ligament, at the lesser cornu of
The styloid process of the temporal bone lies anterior to the the hyoid bone, and, less commonly, in the mid-portion.[4]
stylomastoid foramen. It is attached by cartilaginous tissue. Subjects with elongated styloid processes at least 40 mm
The process normally measures 25 mm in length, although long had the highest incidence of discomfort on swallowing.
it varies in length from person to person and even from side The majority of patient with styloids less than 30 mm were
to side of the same person.[1-3] symptom-free.

Although researchers agree that elongation is common, ANATOMIC AND EMBRYOLOGICAL CONSIDERATIONS
symptoms associated with elongation are uncommon
unless the calcification has progressed through the stylohyal The styloid process normally projects down, forward and
complex to in duce symptoms. Diagnosis is a clinical slightly medially. The tip of the process is situated between
challenge for general dentists when the symptoms appear, the internal and external carotid arteries. It lies posterior
as symptoms may emerge in a variety of manifestations. to the tonsillar fossa and lateral to the pharyngeal wall;
Common symptoms include foreign body sensation in the muscular and ligamentous structures are attached at various
throat, dysphagia, and intermittent facial pain related to the locations on the process. Three muscles are attached to the
mineralized and elongated styloid process (MESP). Various process, the stylopharyngeus (arising from the base and
studies have reported incidence of the MESP ranging from innervated by the glossopharyngeal nerve), the stylohyoid
1.4-30%. (attached to the middle portion and innervated by the facial
nerve), and the styloglossus (originating from the extremity
Elongation usually results from ossification within the of the process and innervated by hypoglossal nerve).
stylohyoid ligament. The ossification usually occurs in the
upper end of the ligament and is in keeping with the styloid The two ligaments in relation to the process are the
stylomandibular and the stylohyoid. The stylomandibular
Correspondence Dr. Vishlesh Arora, Department of Oral Medicine and
Correspondence:
Radiology, Institute of Dental Sciences, Belgaum, Karnataka, India. ligament is inserted at the apex of the process and attaches
E-mail: vishlesharora@gmail.com at the angle of the mandible. The stylohyoid ligament

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Arora, et al.: Eagle syndrome

inserts to the apex of the process and attaches at its far An extensive review concerning the association between
end to the lesser cornu of the hyoid bone. The stylohyoid cervicopharyngeal pain and stylohyoid ossification placed
chain consists of the styloid process, the lesser cornu of patients in three broad categories based on their symptoms
the hyoid bone, and its connecting ligament (usually the (the stylohyoid, pseudostylohyoid, or Eagle’s syndromes).[11]
stylohyoid ligament). The chain is derived from the sec- The same study reported that Eagle’s syndrome typically
ond branchial or hyoid arch known as Reichert’s cartilage. involves patients with a recent history of neck trauma or
In many mammals, this cartilage gives rise to a series of surgical procedures (for example, a tonsillectomy) who
four bony parts: the tympanohyal, stylohyal, epihyal and show radiographic evidence of an elongated styloid process.
ceratohyal.[5] In humans, it is believed that the tympanohyal Clinical palpation of such elongation or calcification usually
fuses with the stylohyal and the petrous part of the temporal can be performed chairside. Patients may complain of
bone to form the styloid process. Normally, the epihyal sensation of a foreign body in the throat, pain on swal-
cartilage degenerates but its fibrous sheath remains; this lowing, or neurologic pain in the head and neck region; they
sheath is the stylohyoid ligament.[6] The ceratohyal or also may have significant pain in the temporomandibular
hypohyal cartilage persists and becomes the lesser cornu joint (TMJ) region, radiating to the sub-auricular and
of the hyoid bone.[7] Variation in the ossification and fusion submandibular regions. The term stylohyoid syndrome
of these various parts can result in marked variation in the applies to patients of any age, although most usually are
chain’s appearance. older than 40 at the time of diagnosis; while these patients
may not have significant symptoms, the ossification can
In 1964, Lengele and Dhem proposed the developmental be demonstrated on routine extraoral radiographs.[12] Such
theory about the elongated styloid process, based on the ossification also may be palpable clinically.
morphogenesis of Reichert’s cartilage.[8] To establish the
mechanisms involved in the morphogenesis of the so-called Pseudostylohyoid syndrome is reserved for patients older
elongated styloid process, a comparative microradiographic than 40 who have no history of trauma and no radiographic
and histological study was performed on 19 long and short or clinical evidence of stylohyoid chain ossification. These
patients have symptoms identical to those of stylohyoid
processes. Some morphological differences between short
syndrome patients but do not have radiographic or clinical
and long processes were noticed. Numerous partially calci-
findings of ossification within the ligament. It is proposed
fied cartilaginous islets were observed within the trabecular
that these people have tendonitis at the junction of the
bone of very long styloid processes or covering their tip.
stylohyoid ligament and the lesser horn of the hyoid bone
Although calcified fibrous tissue or calcified fibrocartilage
resulting from the degenerative and inflammatory changes
sometimes contributed to the thickening of an enlarged
in the tendinous portions of the stylohyoid insertion.[4]
styloid process, the authors reported that the growth of
the process did not seem to result from calcification or
Langlais et al. reported on a subgroup of patients with
ossification of the stylohyoid ligament. Mechanical stresses
constant parietal headaches; these patients were theorized
stretch the second branchial arch during fetal development
as having a “stylo-carotid syndrome.”[4] The differential
and the morphogenesis of the styloid process may induce a
diagnosis depends on the symptoms. The most common
variable involvement from the different parts of Reichert’s inclusions are glossopharyngeal neuralgia, sphenopalatine
cartilage. Lengele and Dhem concluded that the elongated neuralgia (also known as Sluder’s syndrome), histamine
styloid process should he congenital; however, they agreed cephalgia (cluster headaches), migraine headaches,
that further growth still was possible through the car- carotidynia, myofascial pain dysfunction, impacted third
tilaginous cap of the tip of the styloid process.[8] molars, and other conditions associated with dysphagia,
otalgia and tinnitus.[4]
CLINICAL FEATURES AND EXAMINATION
The effects of hyperflexion/hyperex tension (that is,
According to studies performed on the elongated styloid whiplash) injuries are exacerbated by the presence of this
process between 1964 and 1986, symptomatic patients unexpected calcified structure. Researchers have correlated
usually were over the age of 40.[9,10] There was no sex the ligamentous ossification of the stylohyal complex
predilection in the majority of the studies. When pain and and osteophytes of the cervical spine, concluding that
discomfort upon swallowing, foreign body sensation in the a significant correlation exists between cervical diffuse
throat, or a limitation of mandibular movements appear to idiopathic skeletal hyperostosis (DISH) and various styloid
be associated with the radiographically detectable MESP, process-stylohyoid ligament complex abnormalities.[13]
the condition known as Eagle’s syndrome (also known as
elongated styloid process syndrome) is diagnosed. The examination of the styloid region can be performed by

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Arora, et al.: Eagle syndrome

seating the patient in a chair with firm back support. The unilateral or bilateral presence and elongation of the styloid
palpation of the styloid region of the neck can be conducted process. The mineralized stylohyoid ligament is easy to
bimanually and digitally by standing behind the patient. recognize when it extends below the mandibular border or
An intraoral examination method includes visualizing the when its attachment to the mandibular cornu of the hyoid
tonsillar area with a tongue blade or a mouth mirror and bone can be seen on the radiographs.
digital palpation of the area. Patients whose referred pain is
in the tonsillar area will be able to respond to the palpatory Radiographic features alone are not enough to distinguish
stimulus. the symptomatic from the asymptomatic elongated styloid
processes. When the posteroanterior radiographs show
RADIOGRAPHIC FEATURES AND CLASSIFICATION extreme medial deviation, the carotid artery subtype may
be considered.[4] Langlais et al. proposed a radiographic
Elongation and mineralization of the stylohyoid ligament classification of the elongated and mineralized stylohyoid
complex can be observed readily on panoramic radiographs ligament complex22. Their classification included three types
of abnormal radiographic appearances and four patterns of
[Figure 1]. However, because the panoramic radiographs
calcification/mineralization (Figure 3) (see Tables 1 and 2).
are obtained primarily for the visualization of teeth and
associated bony structures within the jaws, observing the
mineralized stylohyoid complex within the pantomograms is DIAGNOSIS AND MANAGEMENT
inconsistent and often distorted. This is due to the fact that
An elongated styloid process may be one cause of
the styloid process frequently lies outside of the panoramic
aggravated maxillofacial or craniocervical pain. Diagnosis
focal trough depending on the patient positioning. The
requires a thorough clinical examination of the head and
images become distorted due to the unreliable magnification
neck and should be confirmed radiographically. Panoramic
within the posterior focal trough of the panoramic machines.
radiographs or bilateral lateral oblique radiographs should
Lateral skull view, reverse Towne view [Figure 2] and
be taken for patients who are examined in a dental office
posterior-anterior views of the skull also demonstrate the
setting for oropharyngeal discomfort, chronic cervi-
cofacial pains, or chronic headaches. Radiographically, the
mineralized styloid process falls under any of the aforemen-
tioned types of elongation or the patterns of mineralization.
The outcomes of treatment have no bearing on the type of

Table 1: The three types of elongation


Characteristics
Elongated Characterized by uninterrupted elongation (>25-
Figure 1: Panoramic radiograph showing the mineralized, elongated 28 mm)
and pseudoarticulated styloid process bilaterally, with a calcified outline Pseudoarticulated Less frequent than elongation; the styloid
pattern. Orange arrow represent pseudo-articulation process is joined to the mineralized
stylomandibular or stylohyoid ligament by a
single pseudo-articulation, usually located
superior to the inferior border of the mandible
Segmented Consists of short or long noncontinuous portions
of the styloid process or interrupted segments of
mineralized ligament

Table 2: Patterns of calcification


Characteristics
CalciÞed outline Reminiscent of the radiographic appearance
of a long bone with a thin radiopaque cortex
and a central lucency that constitutes most
of the process
Partially calciÞed Thicker radiopaque outline, with almost
complete opaciÞcation as well as a small
and occasionally discontinuous radiolucent
core
Nodular complex This pattern has a scalloped outline and
may be partially or completely calciÞed with
Figure 2: A reverse Towne view of the skull, demonstrating the calci- varying degrees of central lucency
fied styloid processes bilaterally and showing pseudoarticulation on Completely calciÞed This pattern is totally radiopaque with no
the left side (orange arrow) evidence of a radiolucent inner core

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Arora, et al.: Eagle syndrome

Figure 3: A representation of the various types of elongation commonly observed in the stylohyoid complex. Bottom:
The patterns of mineralization that are commonly observed in the stylohyoid complex

elongation or pattern of mineralization. The classification is Surgical shortening is the universally accepted treatment for
designed to help dentists understand the variation among confirmed elongation of the styloid process. Multidisciplinary
the different elongation patterns and may even offer clues management yields optimal results; some studies have
as to the etiology of the elongation. recommended an intraoral approach (that is, trans-tonsillar
fossa excision). Other authors have preferred an extraoral
Eagle’s syndrome can be a secondary cause of cervical approach, believing that surgical visualization was
glossopharyngeal neuralgia. Glossopharyngeal neuralgia optimal and the risk of deep cervical infection was minimal.
may be a life-threatening condition as a result of associated Trans-tonsillar fossa excisions is a simple technique that does
cardiovascular complications. Even when life-threatening not result in greater morbidity than a routine tonsillectomy,
complications are absent, glossopharyngeal neuralgia can although the trans-tonsillar approach has been criticized for
be a severely debilitating disease, leading to depression, inadequate surgical shortening of the styloid process due to
suicidal tendencies, fear of swallowing, weight loss and poor visualization of the surgical field. Dentists must weigh
malnutrition. Due to the elongated styloid process, Eagle’s the advantages and disadvantages of each technique and
syndrome is considered an important etiological factor for select the procedure that is appropriate for the patient.
precipitation of secondary glossopharyngeal neuralgia.[11]
A stylectomy is effective for Eagle’s syndrome and should Cases in which a MESP alone is present do not require any
be considered before embarking on any neurosurgical immediate treatment, although patients should be warned
procedure. about the elongation of the styloid process.

The effects of hyperfiexion/hyperextension/whiplash injuries SUMMARY


can be exacerbated by the presence of the calcified styloid.
Additional symptoms may include neck or throat pain Although the elongated styloid process is not an entirely new
radiating to the ipsilateral ear. Diagnosis usually can be made entity, studies of symptomatology and diagnostic strategies
by physical examination. Digital palpation of the styloid involving different population groups have produced new
process in the tonsillar fossa exacerbates the pain. This information about the various types of elongation, patterns
diagnosis should be considered if symptoms can be .relieved of calcification, and symptomatology. When evaluating
by injecting an anesthetic solution in to the tonsillar fossa. the chronic orofacial or cervicofacial pain, elongation of

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Arora, et al.: Eagle syndrome

the styloid process should always be considered. Proper 5. Kingsley IS. The vertebrate skeleton from the developmental
standpoint. Philadelphia: P. Blakiston’s Son and Company; 1925.
selective radiographic techniques and examination of p. 337.
the tonsillar and cervical areas may reveal elongation or 6. Hamilton WJ, Boyd JD, Mossman HW. Human embryology: Prenatal
mineralization in the stylohyoid complex, which may be development of form and function. Baltimore: Williams and Wilkins
Co.; 1945. p. 366.
the cause of the elongated styloid process. The styloid 7. Gibilisco IA, editor. Stafne’s oral radiographic diagnosis. 5th ed.
elongation should be included in the list of differential Philadelphia: W.B. Sansders; 1985. p. 13-7.
diagnoses for evaluating headaches, tonsillar discomfort, 8. Lengele B, Dhem A. Microradiographic and histological study
of the styloid process of the temporal bone. Acta Anat (Basel)
pain referred to the jaw region, difficulty in swallowing and
1989;135:193-9.
the inability to move the jaw from side to side during lateral 9. Gossman JR Jr, Tarsitano JJ. The styloidstylohyoid syndrome. J Oral
excursions. Symptomatic cases of stylohyoid elongations Surg 1977;35:55-60.
10. Keur JJ, Campbell JP, McCarthy JF, Ralph WJ. The clinical
should be referred to as Eagle’s syndrome. When complex
significance of the elongated styloid process. Oral Surg Oral Med
patterns of stylohyoid chain mineralization appear on plain Oral Pathol 1986;61:399-404.
radiographs and tomograms with no signs of disabling 11. Soh KB. The glossopharyngeal nerve, glossopharyngeal neuralgia
and the Eagle’s syndrome-Current concepts and management.
symptoms, mineralized and elongated styloid process may
Singapore Med 1999;40:659-65.
be the more appropriate diagnosis. 12. Camarda A, Deschamps C, Forest D 2nd. Stylohyoid chain
ossification: A discussion of etiology. Oral Surg Oral Med Oral Pathol
1989;67:515-20.
REFERENCES 13. Guo B, Jaovisidha S, Sartoris DJ, Ryu KN, Berthiaume Ml, Clopton P,
et al. Correlation between ossification of the stylohyoid ligament and
1. Worth HM. Principles and practice of oral radiologic interpretation. osteophytes of the cervical spine. J Rheumatol 1997;24:1575-81.
Chicago: Year Book Medical Publishers; 1963. p. 327.
2. Eagle WW. Elongated styloid process: Report of two cases. Arch
Otolaryngol 1937;25:584-7.
3. Eagle WW. Elongated styloid process: Symptoms and treatment. Arch
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4. Langlais RP, Langlmd OE, Nortje CJ. Diagnostic imaging of the jaws.
Source of Support: Nil, Conflict of Interest: Nil
Philadelphia: Lea and Febiger; 1995. p. 617-23.

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