Among headache disorders, Glossopharyngeal Neuralgia, which is rarely seen but extremely distressing, is a severe pain syndrome that occurs in the throat, tongue base, and ear region as a result of the ninth cranial nerve being affected. In Glossopharyngeal Neuralgia, the pain can seriously impair patients’ quality of daily life by affecting basic vital functions such as swallowing, speaking, and even breathing. This condition, which can be controlled with correct diagnosis and appropriate treatment, requires specialized medical approach.

What is Glossopharyngeal Neuralgia?

Glossopharyngeal Neuralgia is a rare neuropathic pain syndrome that develops as a result of irritation or dysfunction of the glossopharyngeal nerve, which is the ninth cranial nerve. This nerve provides sensory sensation to the tongue base, pharynx, tonsillar region, and middle ear, and is also responsible for carrying out the parasympathetic functions of the parotid gland.

Anatomically, the glossopharyngeal nerve exits from the medulla oblongata, leaves the skull through the jugular foramen, and innervates structures in the throat region. This nerve transmits sensory information from the posterior 1/3 of the tongue, soft palate, tonsillar region, pharynx, and middle ear mucosa to the brainstem.

Who Gets Glossopharyngeal Neuralgia?

Glossopharyngeal neuralgia is a rare head-neck pain disorder that generally appears after age 40. It is seen slightly more frequently in women compared to men.

This disease is the most common type of nerve-related face and throat pain after trigeminal neuralgia. However, it is quite rare; approximately 0.7-0.8 cases per 100,000 people are seen annually. This means it is approximately 100 times less common compared to trigeminal neuralgia.

Glossopharyngeal neuralgia generally follows a unilateral course and manifests with sudden onset, brief but very severe pain attacks. The pain is mostly felt in the throat, tongue base, and ear region. Thanks to these characteristic features, it can be distinguished from other head and neck pains.

What Causes Glossopharyngeal Neuralgia?

Various factors can play a role in the development of Glossopharyngeal Neuralgia. These causes can be evaluated in two main categories: primary (idiopathic) and secondary causes.

Idiopathic: Used for diseases that arise spontaneously without any identifiable underlying cause. That is, the cause of the disease is not exactly known.

Secondary: Used for conditions that develop due to another disease, structural problem, or external factor. For example, it can occur following tumor, vascular compression, infection, or trauma.

Although the cause of Glossopharyngeal Neuralgia is not fully understood, it is thought that mechanisms of abnormal electrical activity development in nerve fibers and central sensitization play a role. Approximately 80% of Glossopharyngeal Neuralgia cases have no identifiable underlying cause and these patients are classified as idiopathic. Degenerative changes occurring in nerve tissue with the aging process, thinning of the myelin sheath, and impairments in nerve conduction can play a role in the development of Glossopharyngeal Neuralgia.

Vascular compressions constitute the most important group of secondary causes. Compression created on the glossopharyngeal nerve by abnormal course of the posterior inferior cerebellar artery (PICA), anterior inferior cerebellar artery (AICA), and vertebral artery branches can cause pain syndrome by disrupting nerve functions. This compression generally occurs at the cerebellopontine angle.

Tumoral causes constitute a significant portion of Glossopharyngeal Neuralgia cases. Cerebellopontine angle tumors, especially acoustic neuroma, meningioma, and epidermoid cysts can create pressure on the nerve. Additionally, spread of nasopharyngeal carcinoma, tonsillar tumors, and other head-neck cancers can also cause Glossopharyngeal Neuralgia.

Infectious causes include viral and bacterial infections. Herpes zoster (Shingles), Epstein-Barr virus, cytomegalovirus, and other viral agents can cause damage to nerve tissue. Additionally, chronic tonsillitis, pharyngitis, and other upper respiratory tract infections can contribute to the development of Glossopharyngeal Neuralgia.

Traumatic causes can develop as a result of surgical interventions and injuries. Tonsillectomy, pharyngeal surgery, dental interventions, and neck trauma can damage the glossopharyngeal nerve. Trauma occurring during intubation can also play a role in disease development.

Inflammatory processes are also important in the etiology of Glossopharyngeal Neuralgia. Multiple sclerosis, sarcoidosis, and other demyelinating diseases can affect the glossopharyngeal nerve. Additionally, vasculitis and autoimmune diseases can also disrupt nerve functions.

Anatomical variations can create predisposition to the disease in some individuals. Anomalies in bone structures at the skull base, narrowing of the jugular foramen, or increased length of the styloid process can increase the risk of Glossopharyngeal Neuralgia. The condition of elongated styloid process known as Eagle syndrome can cause pain by pressing on the glossopharyngeal nerve.

Anatomical Factors (Structural Features) can play a role in the development of Glossopharyngeal Neuralgia. Bone structure differences that some people are born with or develop later can create predisposition to glossopharyngeal neuralgia. Especially narrow canals at the skull base or projections being longer than normal can lead to pain by pressing on the nerve. The most well-known example is Eagle Syndrome. In this condition, the styloid projection located deep in the neck becomes longer than normal and can trigger pain attacks by pressing on the glossopharyngeal nerve.

What are the Symptoms of Glossopharyngeal Neuralgia?

The symptoms of Glossopharyngeal Neuralgia are quite characteristic and can be recognized by an experienced physician based on clinical findings. The symptom profile of this disease shows different features from other causes of head-neck pain.

Pain characteristics are the most prominent and defining feature of the disease. Glossopharyngeal Neuralgia pain is generally severe, sharp, electric shock-like or stabbing pain felt deep in the throat, at the tongue base, and inside the ear. This pain is paroxysmal in character and starts and ends suddenly. Patients often describe this condition as “electric shock in my throat” or “sudden stabbing in the pharyngeal area.”

Pain localization shows a typical anatomical distribution. Pain generally concentrates in the tongue base, tonsillar region, soft palate, posterior pharyngeal wall, and deep in the ear. Sometimes pain can radiate toward the submandibular area, lateral neck, and behind the ear. Glossopharyngeal Neuralgia pain is typically unilateral and rarely seen bilaterally.

Triggering factors constitute an important and characteristic feature of the disease. Swallowing, speaking, coughing, yawning, chewing, and even tongue movements can trigger severe pain attacks. Especially drinking hot or cold liquids, consuming acidic foods, and touching the throat area can trigger Glossopharyngeal Neuralgia attacks. This condition seriously affects patients’ eating habits.

Duration and frequency of pain attacks show variability. Each attack generally lasts from a few seconds to a few minutes. While some patients experience attacks several times a day, others may have less frequent but more severe attacks. There are generally completely pain-free periods between attacks.

Cardiac symptoms are a special complication of Glossopharyngeal Neuralgia. As a result of the parasympathetic fibers of the glossopharyngeal nerve being affected, bradycardia, hypotension, and even asystole can develop. This condition is seen in 5-10% of patients and is potentially life-threatening. Cardiac symptoms generally occur simultaneously with the pain attack.

Difficulty swallowing (dysphagia) and speech problems are frequently accompanying findings. Patients with Glossopharyngeal Neuralgia may avoid swallowing due to fear of pain, and this situation can lead to dehydration and nutritional disorders. Additionally, due to pain triggered during speech, patients may tend toward social isolation.

Dry mouth can develop as a result of parotid gland innervation being affected. In patients with Glossopharyngeal Neuralgia, reduction in saliva production and consequently dry mouth can be seen. This condition can lead to oral hygiene problems and dental complications.

Hearing problems may accompany in some cases. As a result of ear involvement, tinnitus, fullness sensation, and mild hearing loss can be seen. These findings especially suggest the presence of underlying tumoral pathology. Advanced examination should be performed.

International Classification of Headache Disorders (ICHD-3) Diagnostic Criteria- 13.2.1 Glossopharyngeal Neuralgia

1. Recurrent attacks of unilateral, severe, brief, electric shock-like or stabbing pain felt along the glossopharyngeal nerve distribution (i.e., in the tongue base, tonsillar region, posterior pharynx, deep in the ear, or submandibular region).
2. Pain has all of the following characteristics:
3. Paroxysmal (in attacks) pain lasting from seconds to 2 minutes.
4. Pain is severe, sharp, piercing, stabbing in nature.
5. Occurs in one or more of the following areas:
   • Tongue base
   • Tonsil or posterior pharyngeal wall
   • Deep in ear (especially ear canal or middle ear sensation)
   • Angle of lower jaw
6. Can be triggered by one of the following factors:
   • Swallowing
   • Speaking
   • Coughing
   • Yawning
   • Throat clearing
   • Cold air or contact
   • Neck movement

1. The underlying cause of pain may be related to irritation, compression, or vascular contact of the glossopharyngeal nerve.
2. There is no significant deficit other than pain in neurological examination.
3. Cannot be explained by another diagnosis.

How is Glossopharyngeal Neuralgia Diagnosed?

The diagnosis of Glossopharyngeal Neuralgia requires careful evaluation by an experienced neurology specialist due to the disease being rarely seen and its symptoms being confusable with other conditions. The diagnostic process is carried out with a systematic approach.

The history-taking process has critical importance in diagnosis. The physician queries in detail the patient’s pain description, onset manner, duration, localization, triggering factors, and accompanying symptoms. The characteristic features of Glossopharyngeal Neuralgia such as pain being in the throat region, triggering with swallowing, and coming in attacks provide important clues in distinguishing it from other pain syndromes.

Physical examination includes comprehensive neurological evaluation. Cranial nerves are systematically examined and especially the functions of the ninth, tenth, and eleventh cranial nerves are assessed. During examination, triggering of pain by mild stimulation of the tonsillar region and tongue base is a valuable finding for diagnosis.

Provocation tests can be helpful in diagnosing Glossopharyngeal Neuralgia. Light touching of the tongue base and tonsillar region with a cotton swab, gargling with cold water, or local anesthetic application have diagnostic value. These tests should be carefully applied by an experienced physician.

The differential diagnosis process is a complex process because Glossopharyngeal Neuralgia symptoms can be confused with many different conditions. It needs to be distinguished from conditions such as trigeminal neuralgia, temporomandibular joint disorders, atypical facial pain, Eagle syndrome, tonsillitis, pharyngitis, and even myocardial infarction.

Imaging methods are used to support diagnosis and investigate underlying pathology. High-resolution MRI is the gold standard for evaluating the cerebellopontine angle and jugular foramen region. Contrast-enhanced MRI is useful in showing tumoral lesions and vascular compression. The anatomy of the glossopharyngeal nerve can be examined in detail with FIESTA and CISS sequences.

Computed tomography (CT) is important in evaluating bone structures. Especially in determining jugular foramen pathologies, styloid process anomalies, and skull base lesions, CT imaging is valuable. Use of contrast material is useful in detecting tumoral pathologies.

Electrophysiological tests can be used to objectively evaluate nerve functions. Blink reflex study, masseter reflex test, and pharyngeal reflex evaluation can provide supportive findings for Glossopharyngeal Neuralgia diagnosis. These tests are particularly useful in determining the degree of underlying nerve damage.

Diagnostic block application is a valuable method used for definitive diagnosis. Temporary relief of pain with local anesthetic application to the tonsillar region and glossopharyngeal nerve tract supports the diagnosis of Glossopharyngeal Neuralgia. This procedure can be used for both diagnostic and therapeutic purposes.

Cardiological evaluation is necessary especially in patients with cardiac symptoms. Cardiac arrhythmias should be investigated with ECG, echocardiography, and Holter monitoring when necessary. Determining cardiac complications due to Glossopharyngeal Neuralgia is important in creating the treatment plan.

Laboratory Tests and Glossopharyngeal Neuralgia
In diagnosing Glossopharyngeal Neuralgia, laboratory tests are used to investigate other underlying causes that may lead to the disease.

• Infection tests: Show whether there is a bacterial or viral infection.
• Autoimmune panel: Done to understand whether the immune system is mistakenly attacking its own nerve tissue.
• Viral serology: Investigates the effects of viruses such as herpes zoster or Epstein-Barr virus.
• Tumor markers: Provide clues about the presence of tumors in the head-neck region or that could press on the nerve.

Thanks to these tests, secondary causes can be revealed and the correct treatment plan can be made.

Glossopharyngeal Neuralgia Treatment

Glossopharyngeal Neuralgia treatment requires a specialized approach due to the disease being rarely seen and its potential complications. The treatment strategy is planned considering the severity of the patient’s pain, accompanying cardiac findings, underlying cause, and general health status.

Medical treatment is preferred as first-line treatment in most patients. Antiepileptic drugs are the most effective options in neuropathic pain treatment. Carbamazepine is accepted as the gold standard drug in Glossopharyngeal Neuralgia treatment and is effective in 70-80% of cases. The starting dose is kept low and gradually increased to achieve balance between effectiveness and side effects.

In patients who do not respond to carbamazepine or who develop side effects, there are other options for pain control. Drugs such as gabapentin, pregabalin, lamotrigine, and phenytoin can be considered as second-choice options in Glossopharyngeal Neuralgia treatment. These drugs are generally used in combination therapy form.

Baclofen with its muscle relaxant effect can be useful in Glossopharyngeal Neuralgia treatment. Especially when used in combination with antiepileptic drugs, it can show synergistic effect. Dosage should be gradually increased and should not be stopped suddenly.

Tricyclic antidepressants can play a supportive role in neuropathic pain treatment. Drugs such as amitriptyline, nortriptyline, and imipramine can be used both in pain control and in treating accompanying depression symptoms.

Invasive treatment options are considered when medical treatment is insufficient. Glossopharyngeal nerve block is an effective procedure performed with local anesthetic and steroid combination. This procedure can be performed through the tonsillar region or near the stylomastoid foramen.

Radiofrequency ablation therapy is an advanced technique used in resistant Glossopharyngeal Neuralgia cases. The pain-transmitting parts of nerve fibers are selectively damaged by controlled heat application. This method is minimally invasive, does not require general anesthesia, and has an 80-90% success rate.

Gamma knife radiosurgery is an alternative treatment option in selected patients. Especially in patients with high surgical risk, high-dose radiation focused on the glossopharyngeal nerve root is applied with stereotactic radiosurgery. Success rates of 70-80% have been reported with this method.

Surgical treatment options come up in very special situations. Microvascular decompression surgery is the gold standard surgical method in cases caused by vascular compression. With this operation, vascular pressure on the nerve is removed and the nerve is protected by placing teflon pads. The success rate is 85-95%.

Glossopharyngeal nerve section is a surgical option considered as a last resort. Pain is eliminated by complete cutting of the nerve, but undesirable consequences such as permanent dysphagia (difficulty swallowing) and loss of taste can develop. Therefore, it is only applied in elderly patients and when all other treatment options have failed.

Treatment of cardiac complications is an important component of Glossopharyngeal Neuralgia management. Pacemaker implantation may be necessary in patients with bradycardia and asystole episodes.

Physical therapy and rehabilitation programs are applied as supportive treatment. Exercises that reduce neck and jaw muscle tension, swallowing rehabilitation, and speech therapy can provide benefit in Glossopharyngeal Neuralgia patients. TENS devices can help in pain control.

Nutritional support in patients with difficulty swallowing, soft foods, liquid supplements, and enteral feeding routes should be evaluated when necessary. A nutrition plan should be created with dietitian consultation.

Lifestyle adjustments are an integral part of treatment. Avoiding triggering factors, stress management, regular sleep pattern, and quitting smoking-alcohol use are important in Glossopharyngeal Neuralgia management.

Psychosocial support is necessary to support the psychological state of patients who have to live with chronic pain. Psychiatry consultation and antidepressant treatment should be planned when necessary.

Follow-up and monitoring process has critical importance in Glossopharyngeal Neuralgia management. Patients should be checked at regular intervals, treatment response should be evaluated, and side effects should be monitored. The most effective results are obtained with a multidisciplinary team approach.

Prognosis generally varies depending on treatment response. With early diagnosis and appropriate treatment, significant improvement can be achieved in the vast majority of patients. Glossopharyngeal Neuralgia is a chronic disease and may require long-term follow-up, but normal quality of life can be achieved with appropriate treatment.

Frequently Asked Questions

1. How Rare is Glossopharyngeal Neuralgia?

Glossopharyngeal Neuralgia is a quite rare disease that constitutes approximately 1% of all cranial neuralgia cases. It has an incidence of 0.7-0.8 cases per 100,000 people annually, which means it is 100 times rarer compared to trigeminal neuralgia. Glossopharyngeal Neuralgia generally appears after age 40 and is seen slightly more frequently in women compared to men. Due to this rare condition, many physicians have limited experience with this disease, so it is important to seek help from experienced neurology or pain specialists. The rarity of the disease can also cause prolonged diagnostic process and sometimes misdiagnosis.

2. Can This Disease Cause Heart Problems?

Yes, Glossopharyngeal Neuralgia can cause serious cardiac complications in some patients. As a result of the parasympathetic fibers of the glossopharyngeal nerve being affected, bradycardia (slowing of heart rate), hypotension (low blood pressure), and even asystole (cardiac arrest) can develop. These complications are seen in approximately 5-10% of Glossopharyngeal Neuralgia patients and are potentially life-threatening. Cardiac symptoms generally occur simultaneously with the pain attack and can especially be triggered during swallowing. Therefore, patients diagnosed with Glossopharyngeal Neuralgia should also be evaluated cardiologically and even pacemaker placement can be considered when necessary.

3. How Does the Disease Affect Swallowing and Speaking?

Glossopharyngeal Neuralgia can seriously affect swallowing and speaking functions. Due to pain being triggered by swallowing and speaking, patients tend to avoid these activities. This condition manifests as dysphagia (difficulty swallowing) and dysarthria (speech disorder). Patients have more difficulty especially when swallowing liquids because liquids have more contact with throat walls. Patients with Glossopharyngeal Neuralgia may experience nutritional disorders, dehydration, and weight loss. Additionally, social isolation may develop due to fear of speaking. Therefore, it is important for patients to receive swallowing rehabilitation and speech therapy, and for nutritional support to be provided.

4. How Effective is Treatment and What are the Side Effects?

Glossopharyngeal Neuralgia treatment is generally quite effective. With medical treatment, successful results are obtained in 70-80% of patients. Carbamazepine is the most effective drug and provides pain control in the vast majority of patients. However, it can have side effects such as dizziness, fatigue, double vision, and liver function disorder. Invasive treatment methods have an 80-95% success rate. Radiofrequency ablation is a minimally invasive method and provides effective pain control with low complication risk. Surgical treatments have the highest success rates but carry risks of dysphagia, hoarseness, and infection. Early intervention is important in Glossopharyngeal Neuralgia treatment because delayed treatment can lead to chronicity and treatment resistance.

5. Can Disease Recurrence be Prevented and How is Quality of Life Affected?

Glossopharyngeal Neuralgia relapses can be partially prevented. Long-term remission can be achieved in approximately 80-90% of patients receiving appropriate treatment. To prevent relapse, continuing regular drug use, avoiding triggering factors, and stress management are important. Especially cold beverages, acidic foods, and hard foods should be avoided, and soft and warm foods should be preferred. Glossopharyngeal Neuralgia, when untreated, can seriously affect quality of life. Patients may experience nutritional problems, social isolation, and depression. However, with appropriate treatment, normal quality of life can be regained in 85-90% of patients. With a multidisciplinary approach involving neurology, otolaryngology, cardiology, and physical therapy specialists working together, the best results are achieved. With regular check-ups and early intervention, Glossopharyngeal Neuralgia can be successfully managed.