History
A careful history must be obtained in patients with suspected Ramsay Hunt syndrome.
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 onset of pain usually precedes the rash by several hours and even days.
Classic Ramsay Hunt syndrome can be associated with the following:
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Vesicular rash of the ear or mouth (as many as 80% of cases)
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The rash might precede the onset of facial paresis/palsy (involvement of the seventh cranial nerve [CN VII])
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Ipsilateral lower motor neuron facial paresis/palsy (CN VII)
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Vertigo and ipsilateral hearing loss (CN VIII)
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Tinnitus
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Otalgia
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Headaches
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Dysarthria
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Gait ataxia
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Fever
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Cervical adenopathy
Facial weakness usually reaches maximum severity by 1 week after the onset of symptoms.
Other cranial neuropathies might be present and may involve cranial nerves (CNs) VIII, IX, X, V, and VI. [7, 8]
Ipsilateral hearing loss has been reported in as many as 50% of cases. Vertigo is usually present in those with hearing loss. [9]
Blisters of the skin of the ear canal, auricle, or both may become secondarily infected, causing cellulitis.
Poor prognostic factors for good functional recovery include the following:
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Age older than 50 years
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Complete facial paralysis
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Lack of CN VII nerve excitability
Physical
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. [10]
The location of the accompanying rash varies from patient to patient, as does the area innervated by the nervus intermedius. It may include the following:
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Anterior two thirds of the tongue
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Soft palate
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External auditory canal
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Pinna
The patient may have associated ipsilateral hearing loss and/or vertigo. Hearing impairment is usually more severe in patients with vertigo than in those without vertigo. [11]
A thorough physical examination must be performed, including neuro-otologic and audiometric assessment.
Causes
Classic Ramsay Hunt syndrome is ascribed to infection of the geniculate ganglion by herpesvirus 3 (varicella-zoster virus [VZV]).
Complications
Rare complications include:
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Herpes zoster oticus, day 6. Image courtesy of Manolette Roque, MD, ROQUE Eye Clinic.
