Glossopharyngeal Neuralgia

What is Glossopharyngeal Neuralgia?

Glossopharyngeal neuralgia is a disorder that causes severe pain, often described as a stabbing pain, in the ninth cranial nerve, which is also known as the glossopharyngeal nerve. This guide will inform you about glossopharyngeal neuralgia and outline the causes, symptoms and treatment options.

What exactly is glossopharyngeal neuralgia?

Neuralgia is a term used to describe serious pain linked to nerve damage. This may result from an injury or arise as a complication of a chronic or underlying condition. Glossopharyngeal neuralgia is pain that affects the glossopharyngeal nerve. This nerve is located at the back of the throat and it is attached to the brainstem. It enables you to feel sensations in your throat, ears and tongue and carries messages from the throat to the brain.

Glossopharyngeal neuralgia occurs when the nerve becomes irritated and the most common symptom is shooting pain, which is often compared to that caused by an electric shock. This disorder affects people in different ways; some may suffer short attacks of pain and experience episodes of remission (when there are no symptoms), while others may endure long periods of pain and increasingly frequent bouts of discomfort. Pain is usually experienced in the throat and tonsils and on one side of the tongue. It can also radiate to the ears and some parts of the jaw.

Who gets glossopharyngeal neuralgia?

This disorder is most commonly diagnosed in people aged over 40 years old and it is more common in females than males. It is relatively rare, however the exact number of cases in the UK is unknown as it can be difficult to diagnose. This disorder is similar to trigeminal neuralgia, which occurs when the trigeminal nerve is compressed and causes severe facial pain, usually on one side of the face. Like glossopharyngeal neuralgia, trigeminal neuralgia is most commonly found in women aged between 40 and 60 years old.

What causes glossopharyngeal neuralgia?

In many cases, it is difficult to establish an exact cause of glossopharyngeal neuralgia. However, it is believed to be linked to damage to the glossopharyngeal nerve, which may be associated with ageing or result from deterioration of the nerve as this affects its ability to carry nerve signals and interrupts the normal process of delivering messages to and from the brain. The most common cause of episodic pain is compression of the nerve by a nearby blood vessel. Damage may also occur as a result of an injury, underlying health condition such as oral cancer, infection around the nerve or multiple sclerosis, although this is relatively uncommon.

In rare cases, the nerve may also be compressed as a result of an abnormally long bone located in the neck, in a condition known as the styloid process. This is referred to as eagle syndrome.

What are the symptoms of glossopharyngeal neuralgia?

The most notable symptom of this condition is severe shooting pain, which is commonly described as an electric shock or an attack of pain. Episodes may last just a few seconds or up to 2-3 minutes and the pain can be felt in the tonsils, throat, tongue and ears. In the vast majority of cases, pain is only felt on one side. However, it is possible to develop pain on both sides of the face as a result of glossopharyngeal neuralgia.

Many people find that there is a trigger for a pain attack, which could involve chewing food, biting down, swallowing, coughing or talking. However, it is possible for an episode to come on without any obvious trigger. Sometimes, you can have frequent attacks more than once a day while in other cases people go for days or even weeks or months without experiencing symptoms.

Some people find that attacks become more frequent as they get older. In rare cases (around 10% of individuals), attacks can be life-threatening and trigger heart problems, which result from the involvement of the vagus nerve, which is also known as the 10th cranial nerve and is located nearby. In this case, additional symptoms to pain may include dizziness and feeling faint, fainting, a sudden drop in blood pressure and seizures.

How is glossopharyngeal neuralgia diagnosed?

There is no specific single test used to confirm or rule out glossopharyngeal neuralgia and in most cases, doctors will analyse and evaluate symptoms before ordering tests to make a diagnosis. Usually, a doctor first suspects this condition when a patient presents with stabbing pain on one side of the throat and additional pain in the ear and tongue. If the symptoms suggest glossopharyngeal neuralgia, for example the location of the pain, the pattern of episodes and intense pain on contact with the soft palate or tonsils, tests may be recommended. A swab test may also be carried out. This involves trying to prompt pain by touching the back of the throat and then applying anaesthetic to the throat and attempting to trigger another attack. If there is no pain while the throat is numb, this would indicate glossopharyngeal neuralgia.

 Imaging scans such as CT and MRI scans may help to identify compression of the nerve and any lesions or tumours located around the ninth cranial nerve. A scan may also detect eagle syndrome, which occurs when the styloid process, a bony structure in the neck, is longer than normal.

Glossopharyngeal neuralgia is similar to trigeminal neuralgia and a diagnosis is usually differentiated by the exact location of the pain. In cases where pain affects one side of the ear and throat, a diagnosis of glossopharyngeal neuralgia is usually given.

How is glossopharyngeal neuralgia treated?

In most cases, the first treatment option is medication. Over the counter painkillers are not effective in dealing with neuralgia and prescription medications, such as anticonvulsants, are commonly used to prevent pain. Examples of medications used include carbamazepine, gabapentin and oxcarabazepine. Antidepressants may also be used independently or in conjunction with anticonvulsants. Sometimes, unpleasant side-effects can occur and it may be necessary to try a combination or series of different drugs to find a medication that suits the individual. Possible side effects include:

• feeling drowsy and tired
• skin irritation and rashes
• nausea

It may be also be possible to use lidocaine spray to ease pain temporarily. This can provide quick relief and enable you to carry on with actions such as swallowing.

Regular blood tests are often recommended for patients undergoing treatment for glossopharyngeal neuralgia. Blood tests are used to monitor the efficacy of treatment and determine optimum dosages and drug combinations.

Surgery

In cases where the nerve is compressed and medication is unsuccessful, surgery may be recommended. A surgical procedure known as microvascular decompression (MVD) is carried out to divert the blood vessel, reducing pressure on the nerve. The procedure is conducted under general anaesthetic and usually involves at least one night’s stay in hospital.

MVD involves making a small inch-long incision in the skull. The incision is made just behind the ear and this process is known as craniotomy. The aim of the process is to expose the ninth cranial nerve at the point where it connects to the brainstem and redirect the blood vessel to prevent it from compressing the nerve. After the procedure, a small sponge is applied to the nerve to offer protection and this remains in place. MVD is successful in around 85 percent of cases. The risks are relatively small in contrast to the benefits, but side-effects may include numbness, infection, excessive bleeding, a feeling of weakness in the facial muscles and problems related to your vision.

In cases where eagle syndrome occurs, a surgical procedure is carried out to resize the styloid process. This can usually be done by minimally invasive means.

In cases where other treatment options are ineffective, high cervical spinal cord stimulation may be recommended.