Glossopharyngeal Neuralgia

What happens in glossopharyngeal neuralgia

The glossopharyngeal nerve (the ninth cranial nerve) carries sensation from the back of the tongue, the tonsils, the throat and part of the ear, and helps with swallowing. When the nerve's insulating coating (myelin) is damaged, often where a nearby artery presses on it as it leaves the brainstem, the nerve can misfire and send bursts of intense pain. This is similar to the mechanism of trigeminal neuralgia, which affects the face and is far more common.

Because the glossopharyngeal nerve has close links with the vagus nerve, which slows the heart, attacks occasionally cause a sudden drop in heart rate and blood pressure, leading to fainting.

Symptoms

• sudden, severe, stabbing, shooting or electric pain on one side
• felt deep in the throat, base of the tongue, tonsil area and ear, sometimes spreading below the jaw angle
• attacks lasting a few seconds to 2 minutes, sometimes in clusters
• triggered by swallowing, chewing, talking, coughing, yawning, or cold liquids
• pain-free periods between attacks, sometimes lasting weeks to months
• in some people, a dull ache between attacks
• rarely, fainting, slow heartbeat, coughing or hoarseness during attacks

People often start avoiding eating and talking, which can lead to weight loss.

Causes

• Classic (primary): compression of the nerve by a blood vessel, most commonly the posterior inferior cerebellar artery.
• Secondary: tumors at the skull base or in the throat (including oropharyngeal cancer), multiple sclerosis, infections, an elongated styloid process (Eagle syndrome), or injury.

It is most common after age 40.

Diagnosis

• History: the typical pattern of brief, triggered, one-sided throat and ear pain is the key.
• Examination: of the mouth, throat and neck, and cranial nerves.
• Topical anesthetic test: numbing the tonsil and throat area with a spray temporarily stops attacks, supporting the diagnosis.
• MRI with special sequences of the brainstem and skull base to look for vessel compression, tumors or MS.
• CT to look for an elongated styloid process.
• ENT examination with a scope to rule out throat cancer, especially in smokers or people with persistent pain.
• ECG or heart monitoring if fainting occurs.

Treatment

Medicines

• Carbamazepine and oxcarbazepine: first-line anti-seizure medicines that calm nerve firing.
• Gabapentin, pregabalin, baclofen, lamotrigine or phenytoin as alternatives or add-ons.
• Local anesthetic sprays can help temporarily, for example before meals.

These medicines need dose adjustment and blood monitoring for side effects such as dizziness, low sodium and liver or blood count changes.

Surgery and procedures

• Microvascular decompression: a neurosurgeon moves the compressing vessel away from the nerve and places a small cushion. It has good long-term success for classic cases.
• Nerve section (rhizotomy): cutting the glossopharyngeal nerve and upper vagus rootlets, with risk of numbness and swallowing changes.
• Stereotactic radiosurgery or nerve blocks in selected people.

Treating secondary causes

Tumors, MS, infections or Eagle syndrome are treated directly.

Outlook

The condition can go into remission for long periods, then return. Most people get good control with medicines, and surgery provides lasting relief for many who do not.

Frequently asked questions

What does glossopharyngeal neuralgia feel like?

It feels like a sudden, sharp, stabbing or electric-shock pain deep in one side of the throat, base of the tongue and ear. Attacks last seconds to a couple of minutes and are often triggered by swallowing, talking or coughing.

What causes glossopharyngeal neuralgia?

It is usually caused by a blood vessel pressing on the glossopharyngeal nerve near the brainstem. Less often, it is caused by tumors, multiple sclerosis, infection, injury or an elongated styloid bone.

Is glossopharyngeal neuralgia the same as trigeminal neuralgia?

They are similar conditions caused by different nerves. Trigeminal neuralgia causes shock-like pain in the face and jaw. Glossopharyngeal neuralgia causes it in the throat, tongue base and ear. Both are treated with similar medicines and surgery.

Can glossopharyngeal neuralgia make you faint?

Rarely, yes. Pain attacks can trigger a reflex that slows the heart and drops blood pressure, causing fainting. This needs urgent medical assessment and sometimes a pacemaker or surgery.