Herpes Zoster Oticus with Cranial Polyneuropathy without Involvement of Facial Nerve.

Choi, Ha Na; Kim,  Ji Eun; Chung,  Dae Young; Park,  So Young; Oh,  Jeong Hoon

Case Report

Auditory and vestibular disorders

Korean Journal of Audiology 2011;15(3):155-158.

Herpes Zoster Oticus with Cranial Polyneuropathy without Involvement of Facial Nerve.

Ha Na Choi, Ji Eun Kim, Dae Young Chung, So Young Park, Jeong Hoon Oh

Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, The Catholic University of Korea, Seoul, Korea. ojhent@catholic.ac.kr

Abstract

Herpes zoster oticus is caused by herpetic viruses including varicella zoster and most commonly affects cranial nerves (CN) VII and VIII. With a review of literature, we report a case of herpes zoster oticus with selective involvement of CN VIII, IX and X. Interestingly, the motor fibers of CN VII were spared while ipsilateral recurrent laryngeal nerve involvement was evident. The patient was treated with antiviral medication and systemic steroids and symptoms were improved.

Keywords: Herpes zoster oticus;Polyneuropathy;Vocal cord paralysis

Address for correspondence : Jeong-Hoon Oh, MD, Department of Otolaryngology-Head and Neck Surgery, College of Medicine, The Catholic University of Korea, 180 Wangsan-ro, Dongdaemun-gu, Seoul 130-709, Korea
Tel : +82-2-958-2148, Fax : +82-2-959-5375, E-mail : ojhent@catholic.ac.kr

Introduction

Herpes zoster resulting from reactivation of preexisting Varicella zoster virus (VZV) is characterized by unilateral painful herpetic vesicular eruption along the dermatome. In the head and neck region, the most common presentation of herpes zoster is Ramsay Hunt syndrome which involves the neurons in the geniculate ganglion of the cranial nerve (CN) VII. The patients present with facial nerve palsy, otalgia, and herpetic auricular vesicular lesions, with or without auditory or vestibular involvement.¹⁾ However, VZV infection of the head and neck may present as multiple cranial neuropathies that are not necessarily associated with typical signs of Ramsay Hunt syndrome. Herpes zoster oticus involving vestibular nerve without facial nerve involvement is uncommon and is only reported in a limited body of literature.^2,³⁾ To our knowledge, VZV infection involving multiple lower CN without facial paralysis has not yet been reported. The authors describe a rare case of herpes zoster oticus involving the lower CN VIII, IX and X, but sparing the motor fibers of CN VII.

Case Report

A 65-year-old male presented with severe otalgia in the right ear continuous for 5 days. He complained of otorrhea, hoarseness and difficulties in swallowing after the onset of otalgia. He denied hearing loss or vertigo initially. The patient did not report any specific medical or surgical history. On physical examination, the right auricle was reddish and swollen with crusted scars and erythematous vesicles (Fig. 1). Function of the facial nerve was intact and paralysis of the facial muscles was not observed. The involvement of right glossopharyngealnerve (CN IX) was evident from neurological examination (palatal paresis with dysphagia). Flexible laryngoscopy revealed the paretic right vocal cord fixed in paramedian position and mucosal swelling with ulceration along the lateral wall of hypopharynx and aryepiglottic fold in the right side (Fig. 2). T1-enhanced magnetic resonance imaging (MRI) showed neither abnormal enhancement along the course of facial nerve nor any abnormal signal intensity in the visualized brain parenchyma including brainstem. Neck CT showed no significant abnormality except mucosal thickening of right hypopharyngeal wall (Fig. 3). After admission, the patient complained of continuous vertigo. Physical evaluation revealed a left-beating gaze nystagmus and 97% weakness of the caloric test in the right ear (Fig. 4). Electronystagmography showed normal tracking on optokinetic tasks and direction-changing nystagmus on positioning in one trace. Sero
logic tests confirmed acute VZV infection with elevated titers of IgM antibody to VZV. The audiological examination was within normal range. Treatment was started with intravenous acyclovir and a short course of corticosteroids. Daily dressing of the ear was performed and pain was managed adequately. After a week of hospitalization, swallowing difficulty and otalgia were significantly improved, and the movement of the right true vocal cord returned to normal. Although the patient had complained of mild dizziness, evoked nystagmus subsided after discharge.

Discussion

Differentiation of Ramsay Hunt syndrome or herpes zoster oticus associated with cranial polyneuropathy from other diseases is essential for accurate diagnosis and proper management.⁴⁾ The symptoms and signs of VZV in the head and neck region may have a wide spectrum, including a painful skin rash, pain but without skin manifestations, multiple CN palsies, CN palsies but without skin or mucosal eruptions, and mucosal eruptions only.^1,^4,⁵⁾ There have been many case reports of patients with glossopharyngeal and vagus nerve palsy caused by VZV infection.^5,^6,^7,⁸⁾ However, paralysis of such lower CN is accompanied by facial nerve paralysis with or without auricular skin manifestations in most cases. To our knowledge, this is the first report of combined herpes zoster oticus and laryngitis without involvement of the motor component of facial nerve. Herpes zoster laryngitis usually indicates a presenting symptom that manifests as pharyngeal and laryngeal mucosal eruptions.^8,⁹⁾ When it is associated with vocal cord palsy, the infrequent condition is known as laryngeal zoster,^8,¹⁰⁾ which may be further associated with prelaryngeal skin erythema. It is thought to be caused by VZV infection involving the sensory fibers of both glossopharyngeal and vagus nerves considering their anatomic correlation and virus spreading.¹⁾
Although the MRI can show the enhancement of the involved nerve in some cases, the findings do not provide information about severity or prognosis.^11,¹²⁾ In our case, no enhancement of involved CN was noted on MRI with gadolinium enhancement even with multiple involvement of CN VIII, IX and X, and contrast-enhanced neck CT scans showed only mucosal thickening of right hypopharyngeal wall. Some authors suggested that the presence of multiple CN involvement results in greater loss of function and is considered a negative prognostic indicator for recovery of vocal fold function.⁷⁾ The cause of multiple CN involvement has been explained as a consequence of an inflammation-induced infarction of a small vessel knowing that a small branch of the carotid artery supplies two or three contiguous nerves.⁸⁾ Therefore, consideration must be given to the possibility of multiple CN involvement, even in case of herpes zoster oticus not accompanying facial paralysis.

REFERENCES

Lin YY, Kao CH, Wang CH. Varicella zoster virus infection of the pharynx and larynx with multiple cranial neuropathies. Laryngoscope 2011;121:1627-30.
Boo SH, Bok KH, Ryu NG, Chung WH. A case of herpes zoster oticus involving vestibular nerve without facial nerve palsy. J Korean Bal Soc 2006;5:311-6.
Van de Steene V, Kuhweide R, Vlaminck S, Casselman J. Varicella zoster virus: beyond facial paralysis. Acta Otorhinolaryngol Belg 2004;58:61-6.
Kim YH, Chang MY, Jung HH, Park YS, Lee SH, Lee JH, et al. Prognosis of Ramsay Hunt syndrome presenting as cranial polyneuropathy. Laryngoscope 2010;120:2270-6.
Nakagawa H, Nagasao M, Kusuyama T, Fukuda H, Ogawa K. A case of glossopharyngeal zoster diagnosed by detecting viral specific antigen in the pharyngeal mucous membrane. J Laryngol Otol 2007;121:163-5.
Adachi M. A case of Varicella zoster virus polyneuropathy: involvement of the glossopharyngeal and vagus nerves mimicking a tumor. AJNR Am J Neuroradiol 2008;29:1743-5.
Coleman C, Fozo M, Rubin A. Ramsay Hunt Syndrome With Severe Dysphagia. J Voice 2011. [Epub ahead of print]
Van Den Bossche P, Van Den Bossche K, Vanpoucke H. Laryngeal zoster with multiple cranial nerve palsies. Eur Arch Otorhinolaryngol 2008;265:365-7.
Watelet JB, Evrard AS, Lawson G, Bonte K, Remacle M, Van Cauwenberge P, et al. Herpes zoster laryngitis: case report and serological profile. Eur Arch Otorhinolaryngol 2007;264:505-7.
Nishizaki K, Onoda K, Akagi H, Yuen K, Ogawa T, Masuda Y. Laryngeal zoster with unilateral laryngeal paralysis. ORL J Otorhinolaryngol Relat Spec 1997;59:235-7.
Korzec K, Sobol SM, Kubal W, Mester SJ, Winzelberg G, May M. Gadolinium-enhanced magnetic resonance imaging of the facial nerve in herpes zoster oticus and Bell's palsy: clinical implications. Am J Otol 1991;12:163-8.
Engström M, Abdsaleh S, Ahlström H, Johansson L, Stålberg E, Jonsson L. Serial gadolinium-enhanced magnetic resonance imaging and assessment of facial nerve function in Bell's palsy. Otolaryngol Head Neck Surg 1997;117:559-66.