This syndrome also is known as geniculate neuralgia or nervus intermedius neuralgia. Besides deep otalgia on the affected side, the classic Ramsay Hunt syndrome, which always develops after a herpetic infection, also can be associated with vertigo, ipsilateral hearing loss, tinnitus, and facial paresis. These symptoms are due to involvement of the geniculate ganglion, which is located near the petrous pyramid portion of the temporal bone where the ear apparatus is located, and to that of the ganglia of Corti and Scarpa, which are located in the inner ear and involved with hearing and balance. This area may include the anterior two thirds of the tongue, the soft palate, the external auditory canal, and the pinna. A careful history must be obtained.
Patients usually present with paroxysmal pain deep within the ear.
The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain. The patient also complains of rash or blisters in the distribution of the nervus intermedius. These herpetic blisters of the skin of the ear canal, auricle, or both may become infected secondarily, causing cellulitis. 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.