Annals of Indian Academy of Neurology
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Year : 2020  |  Volume : 23  |  Issue : 6  |  Page : 835-836

Application of internal auditory canal MRI in ramsay hunt syndrome involving multiple cranial nerves

Department of Neurology, Beijing Chaoyang Hospital, Capital Medical University, Jingyuan Road 5, Beijing, China

Date of Submission11-Jan-2020
Date of Decision21-Jan-2020
Date of Acceptance16-Feb-2020
Date of Web Publication07-Apr-2020

Correspondence Address:
Prof. Lichun Zhou
Department of Neurology, Beijing Chaoyang Hospital, Capital Medical University, Jingyuan Road 5, Beijing
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aian.AIAN_20_20

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How to cite this article:
Li C, Li J, Zhou L. Application of internal auditory canal MRI in ramsay hunt syndrome involving multiple cranial nerves. Ann Indian Acad Neurol 2020;23:835-6

How to cite this URL:
Li C, Li J, Zhou L. Application of internal auditory canal MRI in ramsay hunt syndrome involving multiple cranial nerves. Ann Indian Acad Neurol [serial online] 2020 [cited 2021 Jan 25];23:835-6. Available from:


Acute infection with the varicella-zoster virus (VZV) causes a series of neurological syndromes including Ramsay Hunt syndrome (RHS). However, multiple cranial nerve (CN) involvement is rare in RHS and its diagnosis is further difficult. In most cases, brain magnetic resonance image (MRI) shows no abnormalities. Conversely, MRI of the internal auditory canal (IAC) was seldom applied, which was helpful in the diagnosis and differential diagnosis. Herein, we present a case of the clinical and IAC MRI characteristic of RHS with multiple cranial involvements.

A 68-year-old male presented to the hospital with a 4-day history of right-sided otalgia and upper respiratory tract infection. Right facial paralysis started after 2 days accompanied by hoarseness, slight choking, hearing loss and tinnitus on the right side after 4 days. Vesicular eruptions manifested itself in the right ear auricle, behind the ear and on the face on the second day of admission. After 3 days of admission, he developed dystaxia. His neurologic exam was remarkable for peripheral facial paralysis, vestibular ataxia, in addition to lower motor neuron paralysis of the right-sided glossopharyngeal vagus nerve and vestibulocochlear nerve. Serological investigations included the percent of neutrophile granulocyte which elevated up to 80% (normal: 50–70%). Analysis of the cerebrospinal fluid (CSF) revealed a WBC count of 39 × 106/L including 71.8% monocytes and 28.2% polykaryocytes, and protein 50.9 mg/dL (normal: 15–45 mg/dL). Given his multiple CN involvement, Guillain-Barré spectrum disorders were highest on our differential diagnosis. This hypothesis was evaluated with GM1, GQ1b, GD1b antibodies in CSF and serum and needle electromyography, nerve conduction velocity, all of which were negative. Most importantly, PCR for VZV in serum and CSF was positive. Otolaryngological examinations suggested right vocal fold paralysis and hearing loss in his right ear. Brain MRI showed no abnormality. While IAC MRI showed enhancement of the right CN VII, VIII, IX and X [Figure 1]. Later, the patient was given acyclovir and methylprednisolone. Consequently, there was a significant improvement in hoarseness and hearing impairment, and only slight facial weakness persisted during a follow-up period of 6 months.
Figure 1: Post-contrast 3D T1-weighted image of IAC MRI in a patient with Ramsay Hunt syndrome showing cranial polyneuropathy. (a) There is the enhancement of the labyrinthine segment of the right facial nerve (small arrow) and right vestibular nerve (large arrow). (b) Enhancement of the cisternal segment of right vagus nerve (arrow) is visualised. (c) This image shows enhancement of the cisternal segment of the right glossopharyngeal nerve (arrow)

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Although the VZV remains latent in both CN and dorsal root ganglia, it may reactivate causing a diverse spectrum of neurological syndromes including the well-known RHS.[1] RHS consists of a unilateral otalgia associated with a vesicular eruption in the external auditory canal and sometimes in the pharynx and other parts of the cranial integument, accompanied by a severe peripheral facial palsy, sometimes as well as hearing loss and disequilibrium involving the facial and the vestibulocochlear nerve.[2]

Multiple CN involvement is rare in RHS which was reported only in some cases.[3] In our case this patient showed CN involvement in CN VII, VIII, IX and X. The diagnosis of cranial polyneuropathy in RHS is difficult since it can be associated with neurological symptoms mimicking other diseases such as Bell's palsy, Guillain-Barré spectrum disorders, vestibular neuritis, acute labyrinthitis of various causes and so on. In most cases, brain MRI showed no abnormalities. Conversely, IAC MRI was seldom applied.[4] This case indicated that early diagnosis of RHS with atypical clinical manifestations can be prompted by IAC MRI findings which can show enhancement in the affected CN (CN VII, VIII, IX, X). It has been attributed to the swelling of CN caused by edema and inflammatory cells, and the breakdown of the blood-peripheral nerve barrier and so on.[3] A combination of systemic antiviral and steroid therapy has a beneficial response in patients with RHS.[1] The prognosis of our patient improved with the advent of optimal doses of antiviral and steroid treatment on the day of admission for a sufficient time period. Our case concludes that when patients show unilateral palsy of the VII, VIII, IX and X CN, the infection of VZV should be considered in the differential diagnosis, and IAC MRI should be recommended which may facilitate early diagnosis of RHS in these patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Gilden DH, Kleinschmidt-DeMasters BK, LaGuardia JJ, Mahalingam R, Cohrs RJ. Neurologic complications of the reactivation of varicella-zoster virus. N Engl J Med 2000;342:635-45.  Back to cited text no. 1
Hunt JR. On herpetic inflammation of geniculate ganglion. A new syndrome and its complications. J Nerve Ment Dis 1907;34:73-96.  Back to cited text no. 2
Gunbey HP, Kutlar G, Aslan K, Sayit AT, Incesu L. Magnetic resonance imaging evidence of varicella zoster virus polyneuropathy: Involvement of the glossopharyngeal and vagus nerves associated with ramsay hunt syndrome. J Craniofac Surg 2016;27:721-3.  Back to cited text no. 3
Choi JW, Nahm H, Shin JE, Kim C. Atypical clinical manifestations of herpes zoster oticus: Diagnostic usefulness of magnetic resonance imaging. J Neurovirol 2019;25:874-82.  Back to cited text no. 4


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