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49 Headache or Facial Pain Attributed toInammation oftheStylohyoid Ligament
Fig. 49.1 Panoramic
radiography suggests Eagle
Syndrome, revealing
calcication of the right
stylohyoid ligament
469
Fig. 49.2 Cone beam CT with 3D reconstruction conrms calcication and elongation of the right
stylohyoid ligament (50 mm). The panels are labeled in Portuguese: Lado Esquerdo Vista Esquerda
(Top Left), Lado Direito Vista Direita (Top Right), Lado Direito Vista Direita (Bottom Left), and
Lado Direito Vista Esq. Obliqua (Bottom Right)
with intraoperative conrmation of the previously suspected fracture (Fig.49.3).
There was immediate resolution of the syndrome’s symptoms after the procedure.
The postoperative course includes peripheral facial paralysis on the right, showing
signicant improvement in 3 months.
470
Fig. 49.3 The gure
shows the styloid process
after extraction and placed
next to surgical forceps to
aid visual comparison
E. Sarmento
49.4 Clinical Presentation
The clinical presentation of headache or facial pain attributed to inammation of the
stylohyoid ligament is variable, depending on the underlying cause, the extent of
inammation, and the adjacent structures involved. According to the ICHD-3 [1],
this condition typically manifests as a unilateral headache, with pain in the neck,
pharynx, and/or face.
Pain associated with inammation of the stylohyoid ligament is typically localized to the lateral region of the neck, throat, face, ear, or skull. According to the
ICHD-3 classication, while the pain is generally perceived in the oropharynx,
neck, or face, some patients may report a more diffuse headache pattern. The quality
of pain varies and is often described as sharp, lancinating, throbbing, burning, or
dull. Movements of the head and neck, swallowing, speaking, or palpation over the
stylohyoid ligament area may aggravate it. Patients commonly experience dysphagia, manifesting as difculty in swallowing solids or liquids and a persistent sensation of a foreign body in the throat. Odynophagia, or pain during swallowing, is also
frequently reported. Other associated symptoms may include tinnitus [3], vertigo,
headache, facial pain, and restricted movement of the head and neck. Signicantly,
clinical presentations vary: while some patients may report only localized pain,
49 Headache or Facial Pain Attributed toInammation oftheStylohyoid Ligament
471
others may experience a broader constellation of symptoms reecting the extent of
inammation or mechanical irritation in the region.
49.5 Diagnosis
Diagnosing headache or facial pain attributed to inammation of the stylohyoid
ligament necessitates a high index of suspicion, particularly due to its rarity and
frequent misclassication as temporomandibular joint disorders, dental pathologies,
or idiopathic cervical pain. A systematic diagnostic strategy combining clinical
assessment and imaging is essential for accurate identication.
A thorough clinical history should elicit the nature, location, and triggers of the
pain, along with any history of trauma, infection, or head and neck surgeries.
Patients typically report unilateral pain localized to the neck, submandibular region,
or posterior tonsillar area, often exacerbated by head rotation or digital palpation.
Associated symptoms may include dysphagia, odynophagia, otalgia, vertigo, and
temporomandibular discomfort.
Physical examination should include targeted palpation along the styloid process
and adjacent tissues to assess tenderness or replication of symptoms. A positive
response here can be diagnostically suggestive.
The ICHD-3 diagnostic criteria for 11.8 Headache or facial pain attributed to
inammation of the stylohyoid ligament (Headache Classication Committee of the
International Headache Society, 2018) require:
A. Headache, neck, pharynx, and/or facial pain meeting criterion C.
B. Radiological evidence of a calcied or elongated stylohyoid ligament.
C. Evidence of causality by at least two of the following:
1. Pain induced by digital palpation of the ligament.
2. Pain exacerbated by head movement.
3. Pain relief with local anesthetic injection or styloidectomy.
4. Ipsilateral pain to the involved ligament.
D. Not better explained by another ICHD-3 diagnosis.
Imaging plays a critical role in conrming the diagnosis. While panoramic radiographs can reveal elongation or calcication of the styloid process, CT with 3D
reconstruction offers superior anatomical detail, identifying the extent of calcication, elongation, or compression on adjacent neurovascular structures [4, 5]. MRI is
valuable for assessing soft tissue inammation and ruling out other pathologies. In
select cases, cervical angiotomography may be warranted to evaluate vascular
impingement. Additionally, a diagnostic anesthetic block into the stylohyoid ligament region can offer both therapeutic relief and conrm the source of pain [2, 5].
In the presented case, the patient met all ICHD-3 criteria. Imaging revealed a
50mm elongated and calcied right stylohyoid ligament, with CT and angiotomography showing close contact with the external carotid artery. These ndings, along
472
with symptom provocation during palpation and movement, conrmed the diagnosis. Intraoperatively, a fracture of the styloid process was identied, likely caused by
digital self-manipulation. Symptom resolution following styloidectomy provided
strong evidence for the etiological role of the ligament pathology [3].
E. Sarmento
49.6 Treatment
The treatment of headache or facial pain attributed to inammation of the stylohyoid ligament aims to relieve pain, reduce inammation, and treat the underlying
cause. Treatment options vary depending on the severity of symptoms and the cause
of the inammation.
The treatment of stylohyoid ligament-related pain can be approached conservatively or surgically, depending on the severity of symptoms and the patient’s
response to initial therapies. Conservative treatment typically begins with the use of
analgesic medications, such as paracetamol, nonsteroidal anti-inammatory drugs
(NSAIDs), and, in more severe cases, opioids to manage pain. Muscle relaxants
may also be prescribed to alleviate cervical muscle tension. Physical therapy plays
a supportive role by incorporating stretching and strengthening exercises aimed at
improving neck mobility and reducing discomfort. For temporary pain relief and
diagnostic conrmation, anesthetic blocks involving the injection of local anesthetic
into the stylohyoid ligament region may be utilized. If conservative measures fail to
provide adequate relief or if there is signicant compression of neural or vascular
structures, surgical treatment in the form of a styloidectomy may be indicated. This
procedure involves the removal of the elongated or calcied styloid process and can
be performed using either an intraoral or extraoral surgical approach [6]. While
surgery is often effective in alleviating symptoms, it carries potential risks, including bleeding, infection, nerve injury, and recurrence of pain.
49.7 Conclusion
Headache or facial pain attributed to inammation of the stylohyoid ligament is a
rare but important condition that can cause signicant morbidity. Early recognition
and proper management, following the diagnostic criteria of the ICHD-3 (Headache
Classication Committee of the International Headache Society, 2018), are essential to improve patient outcomes. This chapter provided a case that gives a comprehensive overview of the condition and made a literature review of the topic including
anatomy, pathophysiology, clinical manifestations, diagnostic approach, and treatment options. We hope that this information helps clinicians to better diagnose and
treat this often-neglected cause of craniofacial pain.
49 Headache or Facial Pain Attributed toInammation oftheStylohyoid Ligament
473
References
1. Headache Classication Committee of the International Headache Society (IHS). The
international classication of headache disorders, 3rd edition (ICHD-3). Cephalalgia.
2018;38(1):1–211.
2. Badhey A, Jategaonkar A, Anglin Kovacs AJ, Kadakia S, De Deyn PP, Ducic Y, Schantz S, Shin
E.Eagle syndrome: a comprehensive review. Clin Neurol Neurosurg. 2017;159:34–8. https://
doi.org/10.1016/j.clineuro.2017.04.021.
3. Pagano S, Ricciuti V, Mancini F, Barbieri FR, Chegai F, Marini A, Marruzzo D, Paracino R,
Ricciuti RA.Eagle syndrome: an updated review. Surg Neurol Int. 2023;14:389. https://doi.
org/10.25259/SNI_666_2023.
4. Murtagh RD, Caracciolo JT, Fernandez G.CT ndings associated with Eagle syndrome. AJNR
Am J Neuroradiol. 2001;22(7):1401–2.
5. Demirtaş N, Erdem T, Korkmaz H.Three-dimensional computed tomography in the diagnosis
of Eagle’s syndrome. Eur Arch Otorrinolaringol. 2008;265(10):1219–25.
6. Fusco DJ, Asteraki S, Spetzler RF.Eagle’s syndrome: embryology, anatomy, and clinical man-
agement. Acta Neurochir. 2012;154(7):1119–26.
Part IX
Painful Lesions of the Cranial Nerves
and Other Facial Pain
Chapter 50
Glossopharyngeal Neuralgia
MarceloValença
50.1 Introduction
Glossopharyngeal neuralgia (GPN) is a rare craniofacial pain syndrome characterized by paroxysmal, severe, unilateral pain in areas innervated by the glossopharyngeal nerve (cranial nerve [CN] IX) [1, 2]. The pain is typically described as
lancinating, electric shock-like, and brief, often affecting the oropharynx, tonsillar
fossa, posterior tongue, and sometimes radiating to the ear. It is frequently triggered
by activities such as swallowing, chewing, coughing, talking, or yawning [3].
Although GPN is far less common than trigeminal neuralgia, it can cause signicant disability. A particularly rare and dangerous subtype of GPN is the syncopal or
vagal form, in which pain episodes are associated with autonomic symptoms, most
notably bradycardia, hypotension, or even syncope. These events are thought to
result from stimulation of the adjacent vagus nerve (cranial nerve X), which shares
a close anatomical relationship with the glossopharyngeal nerve at the brainstem
and jugular foramen. In such cases, early recognition and management are critical,
as syncopal events may lead to life-threatening consequences.
The etiology of GPN includes idiopathic cases as well as secondary causes such
as neurovascular compression, neoplastic lesions, demyelinating diseases, and postsurgical or post-infectious changes. Neurovascular compression at the root entry
zone (REZ) of the glossopharyngeal or vagus nerves is often implicated, especially
in refractory or atypical cases. It may be conrmed via high-resolution magnetic
resonance imaging (MRI) with angiographic sequences.
M. Valença (*)
Federal University of Pernambuco, Recife, Pernambuco, Brazil
Center of Excellence in the Treatment of Migraine and Other Headache Disorders, Hospital
Esperança, Recife, Pernambuco, Brazil
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_50
477© The Author(s), under exclusive license to Springer Nature
478
M. Valença
This presents a case of glossopharyngeal neuralgia with associated bradycardia—representing the syncopal variant—occurring in an elderly patient, with partial
response to pharmacological therapy and radiological evidence of bilateral neurovascular contact.
50.2 Pathophysiology
The glossopharyngeal and vagus nerves both emerge from the medulla and exit the
skull via the jugular foramen, making them anatomically susceptible to simultaneous neurovascular compression. When compressed by arterial loops—typically
posterior inferior cerebellar artery (PICA) or vertebral artery branches—these
nerves may develop hyperexcitability or ephaptic transmission, resulting in pain
paroxysms and, in the case of vagal stimulation, bradycardia or syncope.
The pathophysiology of glossopharyngeal neuralgia (GPN) involves paroxysmal
activation of the glossopharyngeal nerve (cranial nerve IX), resulting in severe, episodic pain typically perceived in the posterior oropharynx, tonsillar region, base of
the tongue, and sometimes radiating to the ear. These pain episodes are thought to
arise from hyperexcitability or ephaptic transmission along the affected nerve bers,
often triggered by sensory stimuli such as swallowing, talking, coughing, or
yawning [4].
A predominant underlying mechanism is neurovascular compression, particularly at the root entry zone (REZ) of the glossopharyngeal nerve, where it emerges
from the medulla oblongata. The REZ is especially vulnerable to compression due
to its transition from central (oligodendrocyte-myelinated) to peripheral (Schwann
cell-myelinated) bers. This histological interface is known to predispose to demyelination and aberrant synaptic activity. In many cases, the compressing vessel is a
loop of the posterior inferior cerebellar artery (PICA) or another nearby artery,
which exerts pulsatile mechanical pressure on the nerve, contributing to both structural irritation and functional sensitization.
In the vagal or syncopal variant of GPN, pathophysiological complexity increases
due to involvement of the adjacent vagus nerve (cranial nerve X), which lies in close
anatomical proximity to the glossopharyngeal nerve in the jugular foramen and
medullary region. Noxious stimuli conducted through CN IX may cross-excite
parasympathetic bers of CN X, leading to cardioinhibitory reexes such as bradycardia, hypotension, and in some cases, syncope. These autonomic manifestations
are mediated via reex arcs involving the nucleus tractus solitarius, dorsal motor
nucleus of the vagus, and cardiac parasympathetic output pathways [5].
Secondary etiologies of GPN may include neoplastic compression, demyelinating disease (e.g., multiple sclerosis), inammatory neuropathies, or iatrogenic
injury following oropharyngeal or skull base procedures. However, in idiopathic
or classic cases, neurovascular contact remains the most frequently identied
structural abnormality—especially when detected via high-resolution MRI with
angiographic sequences, which allow visualization of the nerve-vessel interface.
50 Glossopharyngeal Neuralgia
479
50.3 Case Presentation
A 78-year-old woman presented with a ve-year history of paroxysmal right facial
pain, described as acute, stabbing, electric shock-like, and unilateral. The pain
lasted from a few seconds to up to 1 minute and occurred multiple times a day, both
spontaneously and in response to specic triggers such as swallowing, speaking, or
coughing. These episodes were localized to the right posterior oropharyngeal
region, radiating occasionally to the ear.
Notably, during some of these episodes, the patient reported accompanying sensations of dizziness and lightheadedness. A 24-hour Holter monitor was performed
and revealed episodes of signicant bradycardia temporally associated with the pain
paroxysms. These ndings were consistent with the so-called vagal or syncopal
variant of glossopharyngeal neuralgia. There were no episodes of syncope, but transient cardiovascular symptoms raised clinical concern.
Approximately 1 year after symptom onset, the patient experienced periods of
spontaneous remission lasting between 6 and 12months. During these intervals, she
remained entirely asymptomatic without the need for pharmacological treatment.
However, over the past 6 months, the pain recurred with the same intensity, quality,
and frequency as previously reported. Treatment with carbamazepine (initiated at
200mg/day and titrated up to 600mg/day) provided only partial relief. The intensity and frequency of attacks decreased, but breakthrough pain continued to impact
her activities. The drug was tolerated without signicant side effects.
A brain MRI with angiographic sequences (angio-MRI) was performed, which
demonstrated evidence of vascular contact with the lower cranial nerves bilaterally,
more pronounced on the right side. The vessels involved appeared to be loops of the
posterior inferior cerebellar artery (PICA), in close apposition to the
glossopharyngeal- vagal complex at the root entry zone in the medulla oblongata.
No mass lesions, demyelination, or inammatory signs were detected. These ndings supported the diagnosis of neurovascular compression as the underlying
etiology.
50.4 Case Discussion
This case illustrates several key features of glossopharyngeal neuralgia, particularly
the syncopal variant. While classic GPN is rare, the syncopal form is even more
uncommon and may initially be misattributed to cardiac or psychiatric causes. In
this patient, the presence of reproducible pain triggered by typical stimuli (swallowing, speaking) and the involvement of autonomic symptoms—demonstrated objectively by Holter monitoring—strongly indicated the vagal involvement associated
with the glossopharyngeal nerve.
480
M. Valença
50.5 Clinical Characteristics
Glossopharyngeal neuralgia (GPN) is clinically characterized by paroxysmal,
severe, and unilateral pain in regions innervated by the glossopharyngeal nerve (cranial nerve IX). The pain is classically described as sharp, lancinating, or electric
shock-like, with each episode typically lasting a few seconds to 1 minute. Attacks
can occur spontaneously or be triggered by innocuous stimuli, most commonly
swallowing, chewing, talking, coughing, or yawning [6].
The pain localization includes the posterior oropharynx, tonsillar fossa, base of
the tongue, and may radiate to the ipsilateral ear—a distribution that reects the
sensory territory of CN IX.Patients often report pain on one side only, and the episodes can occur several times per day, often without any warning [4].
A particularly important subset of patients presents with the syncopal (vagal)
variant of GPN.In these individuals, paroxysmal pain is accompanied by autonomic
symptoms, including:
• Bradycardia
• Hypotension
• Presyncope or syncope
• Dizziness or lightheadedness
These symptoms are attributed to concomitant activation or irritation of the adjacent vagus nerve (cranial nerve X), which shares close anatomical proximity with
CN IX, especially at the jugular foramen and root entry zone in the medulla. This
co-activation may lead to exaggerated cardioinhibitory reexes, potentially resulting in life-threatening events if not recognized early.
GPN may also demonstrate periods of spontaneous remission, lasting weeks to
months, followed by recurrent symptom ares. Between attacks, patients are typically asymptomatic and may not exhibit any neurological decits. However, during
active phases, the condition can be profoundly disabling and may signicantly
impair oral intake, speech, and quality of life.
In drug-responsive cases, medications such as carbamazepine or oxcarbazepine
can reduce the frequency and severity of attacks, although partial or refractory
responses are not uncommon. The presence of autonomic features often necessitates further cardiac evaluation and may inuence treatment strategies, including
the need for cardiac pacing in select cases or surgical decompression when neurovascular compression is conrmed.
50.6 Diagnosis
The diagnosis of glossopharyngeal neuralgia (GPN) is primarily clinical but requires
a systematic approach to distinguish it from other craniofacial pain syndromes and
to identify potentially treatable structural causes, especially in cases presenting with
autonomic involvement.