Definition

Geniculate Neuralgia (GN), also known as Ramsay Hunt Syndrome, nervus intermedius neuralgia, and Hunts neuralgia, is a nerve disorder affecting the geniculate ganglion of 7th and 8th cranial nerves. Relatively little is known about GN making it a very infrequently diagnosed disorder.

GN is often misdiagnosed as migraine headaches, cluster headaches, or trigeminal neuralgia. It is rarely diagnosed independently of an existing diagnosis of TN. There is no estimate of the number of people with GN.

The response to medical treatment for this condition varies between individuals. The long-term outcomes of surgery remain unknown because of limited data.

Symptoms

GN results in severe, deep ear pain which is usually sharp—often described as an “ice pick in the ear”—but may also be dull and burning, or a continual, severe ache. Geniculate neuralgia ear pain can also be accompanied by facial pain. This helps to explain the frequent misdiagnosis of geniculate neuralgia, either as migraine headaches or trigeminal neuralgia

This pain can be triggered by stimulation of the ear canal, or can follow swallowing or talking. GN patients often report hypersensitivity to light and sound. Loud noises or bright light can trigger prolonged, painful episodes.

Geniculate Ganglion
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Causes
Geniculate neuralgia is caused by the nervus intermedius being compressed by a blood vessel.  The nervus intermedius is the part of the facial nerve (cranial nerve VII) located between the motor component of the facial nerve and the vestibulocochlear nerve (cranial nerve VIII). It contains the sensory and parasympathetic fibers of the facial nerve. Upon reaching the facial canal, it joins with the motor root of the facial nerve at the geniculate ganglion.

The exact cause of this compression in an otherwise healthy individual is unknown.


 

Further Exploration:
Journal Articles:
Tang IP, Freeman SR, Kontorinis G, Tang MY, Rutherford SA, King AT, Lloyd SK; Geniculate neuralgia: a systematic review, 2014. https://www.ncbi.nlm.nih.gov/pubmed/24819337
Pulec JL; Geniculate neuralgia: long-term results of surgical treatment, 2002. https://www.ncbi.nlm.nih.gov/pubmed/11816385
Lovely TJ, Jannetta P; Surgical management of geniculate neuralgia, 1997. https://www.ncbi.nlm.nih.gov/pubmed/9233495
Last updated 11/17