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Geniculate ganglionitis or geniculate neuralgia (GN), also called nervus intermedius neuralgia, Ramsay Hunt syndrome, or Hunt's neuralgia, is a rare disorder characterized by severe paroxysmal neuralgic pain deep in the ear,[1] that may spread to the ear canal, outer ear, mastoid or eye regions. GN may also occur in combination with trigeminal or glossopharyngeal neuralgia.[2]
| Geniculate neuralgia | |
|---|---|
| Specialty | Neurology  |
The pain of GN is sharp, shooting or burning and can last for hours. Painful attacks can be triggered by cold, noise, swallowing or touch, but triggers are usually unique to the sufferer. Other related symptoms that may be experienced include increased salivation, bitter taste, tinnitus and vertigo.
GN is rare, and only limited data is available regarding the incidence, prevalence, and risk factors associated with this condition. Middle-aged adults, however, seem to be predominantly affected, women more than men.[3]
Cause
editGN may be caused by compression of somatic sensory branch of cranial nerve VII which goes through the nervus intermedius. In sufferers of GN, signals sent along these nerves are altered and interpreted by the geniculate ganglion (a structure in the brain) as GN pain. GN may also develop following herpes zoster oticus (Ramsay Hunt syndrome), where cold sores occur on the ear drum or ear. This may also be associated with facial paresis (weakness), tinnitus, vertigo and deafness. Disorders of lacrimation, salivation and/or taste sometimes accompany the pain. There is a common association with herpes zoster.[4]
Diagnosis
editDiagnostic criteria:[4]
A. Pain paroxysms of intermittent occurrence, lasting for seconds or minutes, in the depth of the ear
B. Presence of a trigger area in the posterior wall of the auditory canal
C. Not attributed to another disorder
Treatment
editPharmacological
editA trial of the anticonvulsant drug carbamazepine is common for patients diagnosed with GN.[5] For patients who do not tolerate or respond to carbamazepine, alternative drugs include oxcarbazepine, gabapentin, phenytoin, lamotrigine, and baclofen. In addition, tricyclics (e.g., amitriptyline) and pregabalin are useful in other types of neuropathic pain.
Surgical
editA variety of surgeries have been performed including microvascular decompression (MVD) of the fifth, ninth, and tenth nerves;[2][6] as well as partial cutting of the nervus intermedius,[7] geniculate ganglion, chorda tympani and/or the ninth and tenth cranial nerves.
References
edit- ^ Pulec, J. L. (1976-07-01). "Geniculate neuralgia: diagnosis and surgical management". The Laryngoscope. 86 (7): 955–964. doi:10.1288/00005537-197607000-00008. ISSN 0023-852X. PMID 933690.
- ^ a b Lovely, T. J.; Jannetta, P. J. (1997-07-01). "Surgical management of geniculate neuralgia". The American Journal of Otology. 18 (4): 512–517. ISSN 0192-9763. PMID 9233495.
- ^ Bruyn, G. W. (1984-03-01). "Nervus intermedius neuralgia (Hunt)". Cephalalgia: An International Journal of Headache. 4 (1): 71–78. doi:10.1046/j.1468-2982.1984.0401071.x. ISSN 0333-1024. PMID 6370459. S2CID 39954111.
- ^ a b The International Classification of Headache Disorders, 2nd Edition, 1st revision (May, 2005). Headache Classification Subcommittee of the International Headache Society (IHS). 2005. p. 194.
- ^ "Nervus intermedius neuralgia: a case report". CRANIO: The Journal of Craniomandibular Practice. 25. 2007.
- ^ Saers, S. J. F.; Han, K. S.; Ru, J. A. de (2011-05-01). "Microvascular decompression may be an effective treatment for nervus intermedius neuralgia". The Journal of Laryngology & Otology. 125 (5): 520–522. doi:10.1017/S0022215110002677. ISSN 1748-5460. PMID 21223630. S2CID 22457191.
- ^ Pulec, JL (1976). "Geniculate neuralgia: diagnosis and surgical management". Laryngoscope. 86 (7): 955–64. doi:10.1288/00005537-197607000-00008. PMID 933690.
Use of these new techniques, sometimes in combination with selective section of the Vth cranial nerve, has been successful in relieving the pain of geniculate neuralgia.