Glossopharyngeal neuralgia

Glossopharyngeal neuralgia causes repeated episodes of severe pain in areas connected to the ninth cranial nerve, also known as the glossopharyngeal nerve. These areas include the back of the nose and throat, back of the tongue, ear, tonsil area, and voice box. Some episodes may also be associated with coughing or hoarseness. Episodes may last for seconds or a few minutes, and they may occur many times throughout the day and night. Episodes may be triggered by coughing, sneezing, swallowing, talking, laughing, or chewing.^[1][2][3]

Symptoms of glossopharyngeal neuralgia typically occur in individuals over 40 or 50-years-old.^[1][2] Pain usually begins at the back of the tongue or throat, and it sometimes spreads  to the ear or the back of the jaw. The pain can cause difficulty speaking and swallowing. In rare cases, the disorder can cause a slow heart rate (bradycardia), hypotension, no heart beat due to cardiac arrest (asystole), or fainting (syncope).^[1][2][4]

Glossopharyngeal neuralgia is thought to be caused by irritation of the glossopharyngeal nerve. In many cases, the source of the irritation is not found.^[1][2] In some cases, the source of the irritation is found to be increased pressure on the glossopharyngeal nerve, such as an abnormally positioned artery, growths at the base of the skull, an infection, an injury, or tumors of the throat, mouth, or brain.^[1][2] Rarely, the disorder may be caused by an aneurysm in the carotid artery, an abnormally long bone below the ear (styloid process), or multiple sclerosis.^[2]

In most cases, glossopharyngeal neuralgia is caused by irritation of the glossopharyngeal nerve and is not inherited (passed down from parent to child). In most cases, a person with glossopharyngeal neuralgia is the only person with the disorder in the family. However, if the underlying cause of the disorder runs in families, there may be more than one family member with the disorder.^[2] 

A diagnosis of glossopharyngeal neuralgia is suspected when a person presents to the doctor with symptoms of the disorder. A test can then be completed in which a cotton swab is touched to the back of the throat. This typically causes pain in people with glossopharyngeal neuralgia, but the pain is relieved when a local anesthetic is applied.^[2]

After glossopharyngeal neuralgia is diagnosed, doctors may try to determine the underlying cause of the pain. Tests such as a blood test, CT scan, MRI, or X-rays may be ordered. If it is suspected that there may be problems with the blood vessels, an MR-angiogram (MRA) may also be ordered.^[1][2]

Treatment for glossopharyngeal neuralgia is aimed at controlling the pain associated with the disorder. Over-the-counter pain medications are generally not very effective at controlling the pain. However, anti-seizure medications and anti-depressants may help relieve pain for some individuals.^[1][2] The application of local anesthetics to the affected region may also help control pain, but typically only for a short time.^[3] If an underlying cause for the disorder is identified, treatment is generally aimed at treating the underlying problem.^[1]

In severe cases, surgery may be necessary to remove pressure from the glossopharyngeal nerve. This may involve removing the cause of the pressure on the nerve or severing the nerve so that it no longer causes pain.^[1]

The long-term outlook for people affected by glossopharyngeal neuralgia may depend on the underlying cause of the disorder and the response to treatment. Some people with the disorder respond well to medications that help control the pain such as anti-seizure medications and anti-depressants. If medications are not successful, surgery is typically able to relieve the pain. In some cases, side effects of surgery may include loss of sensation in the mouth and throat.^[5]

Some people with glossopharyngeal neuralgia have just one episode of pain, others may have episodes that occur in clusters with periods in between without any episodes of pain. For some individuals, the episodes of pain may occur daily.^[5] People who have glossopharyngeal neuralgia and experience frequent pain that medications are not able to control are encouraged to speak with their doctors about surgical options.