Fig. 3. a Audiogram of zoster-induced hearing loss (sensory-neural) of the left ear. b Almost complete recovery after 4 weeks (treatment with infusions plus acyclovir and cortisone).
The clinical picture may vary in severity from case to case. General characteristics are periauricular pain, herpetiform eruptions and neural dysfunctions, predominantly involving the VII and VIII cranial nerve [3, 19].
A prodromal stage with fatigue and feeling sick (approximately 7-14 days) is followed by a phase of erupting herpetiform lesions. Erythematous macu-lopapules around or on the auricle (most often in the concha or the superficial part of the outer ear canal) soon vesiculate and sometimes turn to ulcers. In rare cases, vesicles can (also) be observed in the buccal mucosa of the correspond ing side or in the oropharynx. Body temperature may slightly be raised, usually there is pain - mostly moderate to severe - in the ear and its environment. After approximately 1 week, the lesions turn dry and crust. Regional lymphadenopa-thy is rather common. One of the leading symptoms is local pain (deep earache till pain around the auricle), which may persist even after the crust fell off ('postherpetic neuralgia').
Cutaneous zoster eruptions are in no way obligatory: 8-15% of the patients present without any rash or of vesicles ('zoster sine herpete' ).
Special attention must be turned to neural disorders: about 70% of the zoster oticus patients show some kind of involvement of the facial nerve, the incidence of inner ear affections (deafness, vertigo) seems to range within equal dimensions.
Signs of facial palsy mostly emerge later than the rash, typically proceeding rapidly from mild facial weakness to a complete unilateral peripheral paralysis.
Some patients complain of dysgeusia due to loss of chorda tympani-related functions. So unilaterally taste on the antero-lateral portion of the tongue may be diminished.
Without adequate treatment, the prognosis of facial palsy is very poor. Untreated, the chance of a restitutio ad integrum is as little as 10% in complete and about 70% incomplete paralysis . With the nowadays generally accepted treatment the outcome is much more favorable.
Zoster-related hearing disorders may occur at different intervals in the course of the disease, but mostly follow the cutaneous symptoms. Often, there is an abrupt onset, resembling sudden hearing loss. The common finding is a sen-sorineural deafness, mostly in the high frequency range, sometimes accompanied by tinnitus. Profound inner ear hearing loss or even complete deafness is a very rare event. In some cases, so-called hyperacousis is found, meaning that the pain threshold for noise or tones is decreased, though (mostly) the hearing threshold is raised.
Vertigo may come along with the hearing disorders or appear at intervals, often with sensations of spinning or twirling motion.
Zoster oticus infection may cause neurologic complications such as cere-brospinal fluid changes, involvement of other cranial nerves (V, IX, X), aseptic meningitis, myelitis, encephalitis, acute ascending polyradiculitis, thrombotic cerebral vasculopathy etc. There are publications describing extensive involvement of more cranial nerves such as XI and XII , very rarely I, II, III and IV cervical dermatomes C2-4 or even bilateral facial paralysis [18, 23]. In those cases, the spread of infection through meningeal inflammation should be considered.
Post-herpetic neuralgia can persist for long time after the disease, meaning a special therapeutic challenge.
In cases with characteristic pathology (pain, herpetiform eruptions of the auricle, facial palsy, may be unilateral hearing loss, vertigo) the diagnosis may be fairly easy. However, there are many atypical courses, especially those without any cutaneous symptoms ('zoster sine herpete'), in which only serologic examinations can verify the diagnosis 'zoster'.
Serological assays confirming the diagnosis include verification of significant increase of Anti-VZV-antibodies of the IgG-, IgM-, respectively, IgA-fraction (Enzym-linked immunosorbent assay or immune fluorescence).
In some cases (and for scientific purpose), detection of viral DNA (VZV DNA) via polymerase chain reaction in cerebrospinal fluid, saliva tissue etc. can be helpful [24, 25].
In case of facial nerve involvement, the severity code is classified by the criteria of House and Brackmann . The course of facial nerve palsy can be followed electrophysiologically (electromyography, electroneurography).
Hearing loss in the context of zoster oticus infection is documented and controlled by audiometry (pure tone audiometry, supra-threshold tests, impedance audiometry). In certain cases, objective audiometry via evoked potentials (e.g. BERA) can be helpful for identifying the site of the lesion (cochlear/retro-cochlear) [27, 28]. In early stages registration of otoacoustic emissions can make sense .
In cases of zoster-linked dizziness or vertigo [30, 31], detection and registration of nystagmus is important. For clinical purpose, observation of eye movement, e.g. with special glasses ('Frenzel's goggles') in a darkened room may be sufficient. For more detailed examinations nystagmography (electro-nystagmography, or video-oculography) is inevitable (fig. 4). Caloric testing with water  for detecting unilateral vestibular disorders may be painful because of zoster papules, even the use of heated or cooled air may not be tolerated in single cases.
Additional hints concerning the site of the zoster-linked lesion may be given by magnetic resonance tomography of the temporal bone (fig. 5). Typically, gadolinium enhancement in the course of the facial nerve, the area of the geniculate ganglion, the labyrinth or the meatus acousticus internus may indicate inflammation within the temporal bone and help estimating the main focus of the disease [33-35]. Some authors advocate transcranial magnetic stimulation of the nerve, by which a more precise localization of the lesion within the Fallopian channel is possible. Moreover, additional electric stimulation via the same route may help to establish correct diagnosis and prognosis .
If there are further neurological signs or any apprehension of meningitis, lumbal puncture should be performed for examining CNS fluid.
As soon as the diagnosis zoster oticus has been made, systemic antiviral chemotherapy should be started urgently. Substances like acyclovir, valacy-clovir, famciclovir and brivudin are available. In most studies, high doses of acyclovir were administered intravenously (e.g. 5-10 mg, 3 times a day for 7-10 days), with good results [37, 38]. So far, in Germany oral application of antiviral agents, even brivudin as a very potent drug, has not been widely accepted for zoster oticus treatment, though in other localization of zoster it proved to be effective.
Once there are any signs of involvement of the Vllth or Vlllth cranial nerve (facial weakness, hearing loss, tinnitus, dizziness/vertigo), a simultaneous application of cortisone is indicated (e.g. 60 mg prednisolone daily for the first 10 days, subsequently 10 mg for another 10 days or starting with 200 mg prednisolone i.v., progressively decreasing till 10 mg p.o. within 2 weeks).
It seems crucial, not to delay the onset of the combined therapy (at least stay within the 72 h range), because the prognosis deteriorates markedly [39, 40].
With this therapy, prognosis of facial palsy has improved so much, that indications for surgical procedures for the nerve became very rare . Some decades ago, nerve decompression for stopping 'self-strangulation' of the ede-matous nerve by removing the bony walls of its canal was a rather common operation . Surgery ranged from simple mastoidectomy (descending part of the nerve) to transtemporal approaches, from which the entire course of the nerve from the internal auditory canal till the foramen stylomastoideum could be exposed [42, 43].
If the Vlllth cranial nerve is involved (hearing loss, tinnitus, dizziness/ vertigo), a simultaneous infusion therapy (similar to that in sudden hearing loss) can be added. In Germany, the combination of plasma volume expanders (like hydroxyethyl starch) and vasoactive substances (e.g. pentoxiphylline) is widely used (e.g. 500 ml HAES plus pentoxiphylline per day for approximately 1 week).
In cases of vestibular imbalance with dizziness and vertigo sometimes causing vomitus, additional treatment with antivertiginous drugs: e.g. anticholinergics, antihistamines, Ca-channel blockers may be indicated.
Heavy pain is treated with analgesics (e.g. tramadol) in appropriate doses, best combined with a neuroactive agent (e.g. amitriptyline) .
Local treatment of the skin includes antisepsis and drying in the early stages (e.g. lotio alba, vioform zinc mixture), later cautious removal of crusts.
Prior to the era of early and simultaneous antiviral and antiphlogistic therapy, zoster-related affections of the Vllth and Vlllth cranial nerves had a poor prognosis. Nowadays, in case of early onset of medical treatment (as possible within the first 72 h), in most cases an almost or complete restitution can be expected. The main difficulty for the otologist is the correct interpretation of clinical symptoms, which may be misleading at least in the early phase of the disease. As indicative cutaneous symptoms may be missing ('zoster sine herpete'), the combination of local pain (earache) and hearing loss (sensorineural), vertigo or any kind of facial weakness should always be suspicious for zoster oticus.
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Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde Kopf- und Hals-Chirurgie, 'Otto Körner' der Universität Rostock Doberaner Strasse 137-139 DE-18057 Rostock (Germany)
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Gross G, Doerr HW (eds): Herpes Zoster. Monogr Virol. Basel, Karger, 2006, vol 26, pp 58-68
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