This article includes discussion of herpes zoster oticus, aural herpes zoster, aural zoster, cephalic herpes zoster, cephalic zoster, geniculate ganglion syndrome, geniculate ganglionitis, geniculate herpes, geniculate herpes zoster, geniculate zoster, herpes zoster auricularis, herpes zoster auris, herpes zoster cephalicus, herpes zoster oticus, herpetic geniculate ganglionitis, Ramsay-Hunt syndrome, and zoster sine herpete. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
The author explains the clinical presentation, pathophysiology, diagnostic work-up, and management of herpes zoster oticus, ie, a form of herpes zoster with peripheral facial nerve impairment due to varicella-zoster virus. In 2005, a live zoster vaccine was shown to markedly reduce the morbidity from both herpes zoster and postherpetic neuralgia among older adults. In 2006, the U.S. Food and Drug Administration licensed this vaccine for use in people aged 60 years and older. Multiple cranial nerve involvement is rare in herpes zoster oticus, but when present, appears to worsen prognosis for recovery of facial paresis and hearing loss.
• Herpes zoster oticus can be considered as peripheral facial nerve impairment (motor and/or sensory) due to varicella-zoster virus, with or without associated rash, and is associated with otologic manifestations or other neurologic complications, including cranial polyneuropathy or meningitis.
• Herpes zoster oticus is a specific form of herpes zoster that often presents with pre-eruptive (“pre-herpetic”) pain, allodynia, burning, or itching generally localized to the ear and mastoid region. Facial palsy may precede, occur simultaneously with, or follow erythematous maculopapular rash in herpes zoster oticus.
• A small proportion of cases of facial palsy associated with varicella-zoster infection do not have an associated rash (or at least no rash in the expected location in the internal auditory canal or on the tympanic membrane), a condition referred to as “zoster sine herpete.”
• Impaired cell-mediated immunity is an important factor in reactivation of varicella-zoster virus and development of clinical herpes zoster.
• Secondary prevention of herpes zoster is now possible for older adults previously infected with chickenpox. The United States Food and Drug Administration licensed a live zoster vaccine (Zostavax) in 2006 for use in people aged 60 years and older.
• Multiple cranial nerve involvement is rare in herpes zoster oticus, but when present, appears to worsen prognosis for recovery of facial paresis and hearing loss.
Historical note and terminology
Clinical observations in the late 19th century suggested that both chickenpox and herpes zoster were caused by the same process (von Bokay 1909). This concept was finally proven in the 1950s by isolation of the same virus from lesions of both conditions (Weller and Stoddard 1952; Weller et al 1958).
In a series of papers from 1907 to 1937, American neurologist James Ramsay Hunt (1872-1937) called attention to a form of cranial herpes zoster that he termed “geniculate neuralgia” (Hunt 1907a; Hunt 1907b; Hunt 1908; Hunt 1909; Hunt 1910; 1915; 1937; Louis and Williams 2003; Louis 2004; Lanska and Hunt 2014.) He proposed a concept of viral inflammation of the geniculate ganglion with associated neurologic manifestations, particularly skin eruptions affecting the auricle or external auditory canal and facial nerve palsy.
In 1907 Hunt outlined 3 groups of cases of herpes zoster (of which he had 4 personal cases, combined with the 56 he collected from the literature, for a total of 60) (Hunt 1907a; Brody and Wilkins 1968; Lanska and Hunt 2014).
Group 1. Herpes zoster of the auricle and external canal with periauricular pain (Hunt had no cases in this group).
Group 2. Herpes zoster with facial paralysis and periauricular pain, usually with skin lesions of the auricle and external canal, or alternatively of the face, the occiput, or the neck. Hunt attributed this to “pressure of the inflamed (geniculate) ganglion, or in some cases, by a direct extension of the inflammation to the (facial) nerve” (Hunt 1907a).
Group 3. Herpes zoster with facial paralysis, periauricular pain, and auditory/vestibular symptoms, again usually with skin lesions of the auricle and external canal, or alternatively of the face, occiput, or neck. Hunt assumed in these cases “that the inflammatory process has extended to the auditory nerve which is enveloped in the same sheath, and courses in the same canal as the facial nerve” (Hunt 1907a).
Several problems are apparent with this classification (Lanska and Hunt 2014).
(1) What exactly is “Ramsay Hunt syndrome” given that there were 3 groups of cases and no defining theme? Most subsequent authors have simply considered Ramsay Hunt as herpes zoster of the auricle and external canal with facial paralysis, with or without auditory/vestibular symptoms, ie, a subset of groups 2 and 3 in which the cutaneous lesions are restricted to the auricle or external auditory canal.
(2) If one tries to define Ramsay Hunt syndrome as “herpes zoster oticus” and by that include all cases with herpes zoster of the auricle or external auditory canal, one excludes some of what Hunt included, eg, cases of facial paralysis without skin lesions of the auricle or external canal, but with instead skin lesions of the face, occiput, or neck.
(3) Other groups of cases have subsequently been recognized that do not fit in Hunt's scheme, but seem as if they should be included, ie, (a) facial nerve paralysis due to varicella-zoster virus but without any apparent skin lesions; and (b) cranial polyneuropathy (including facial nerve paralysis) due to zoster, extending to nerves other than VII and VIII.
(4) Some authors apply Hunt's classification of cases even when the cases do not strictly meet the characteristics of the groups he described (Zhu and Pyatkevich 2014).
A modified scheme is presented below for cranial zoster, in which all of Ramsay Hunt's collected cases could be grouped, as well as subsequently identified groups such as zoster sine herpete and zoster cranial polyneuropathy that includes facial paresis.
Group 1. Herpes zoster skin lesions without neurologic manifestations, involving the head, subcategorized by dermatomal territory (or territories) involved.
Group 2. Herpes zoster cranial neuropathy (or polyneuropathy) without skin lesions, subcategorized by the cranial nerves involved.
Group 3. Herpes zoster cranial neuropathy (or polyneuropathy) with skin lesions, subcategorized by the cranial nerves and dermatomal territories involved.
However, for purposes of simplicity and for consistency with previous publications, in this article herpes zoster oticus will be considered as peripheral facial nerve impairment (motor and/or sensory) due to varicella-zoster virus, with or without associated rash, with or without associated otologic manifestations, and with or without other neurologic complications (including cranial polyneuropathy or meningitis).
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