A 24-Year-Old Man With Ear Pain, Dizziness, and a Rash

D. Brady Pregerson, MD

Disclosures

December 11, 2019

Discussion

Ramsay Hunt syndrome, also known as herpes zoster oticus, is a vesicular herpetiform eruption that occurs through reactivation of latent varicella-zoster virus (VZV) within the geniculate ganglia of the seventh cranial nerve (CN VII). The syndrome typically presents with unilateral ear pain, followed by a vesicular rash within the ear canal and/or pinna. Subsequently, facial weakness emerges as a result of CN VII involvement. Involvement of the adjacent nerve, CN VIII, may produce vestibulocochlear dysfunction resulting in tinnitus, ipsilateral hearing loss, vertigo, and nystagmus. Taste and lacrimation may be less commonly affected.

The geniculate ganglion is located near the petrous pyramid area of the temporal bone, in close proximity to the inner ear apparatus, which includes the cochlea (the auditory portion of the inner ear with the organ of Corti, the sensory organ of hearing) and the vestibular ganglion (Scarpa ganglion) responsible for equilibrium. The facial paresis is caused by inflammation of the facial nerve that courses through the inner and middle ear. Classically, the syndrome has been associated with VZV infection.[1]

Pain is typically the first symptom of the syndrome; it is usually followed by a vesicular rash within 1-9 days, although some patients never develop a rash (zoster sine herpete). The rash typically crusts within 7-10 days, but it may persist for 1 month or longer. Initially, the vesicular rash may not be overtly apparent. It may instead be seen within the ear canal; on the tympanic membrane itself; or on the tongue, oral cavity, soft palate, or posterior hard palate. Looking carefully for such vesicles in all cases of facial nerve weakness is important because herpes zoster tends to cause a more severe paralysis than idiopathic Bell palsy and early treatment may reduce morbidity.

A careful history must be obtained. Most patients usually present with paroxysmal pain deep within the ear, without evidence of lesions. The pain often radiates outward to the pinna of the ear.[1,2]

The primary physical findings in classic Ramsay Hunt syndrome include peripheral facial nerve paresis with associated rash or herpetic blisters in the distribution of the nervus intermedius. The location of the accompanying rash varies from patient to patient, as does the area innervated by the nervus intermedius. The affected area may include the anterior two thirds of the tongue, the soft palate, the external auditory canal, and the pinna. The herpetic blisters of the skin of the ear canal, auricle, or both may become infected secondarily, causing cellulitis.

As mentioned previously, the patient may have associated ipsilateral hearing loss and balance problems. Ideally, a neuro-otologic and audiometric assessment should be performed on patients with the syndrome.[1,2]

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