European Annals of Otorhinolaryngology, Head and Neck diseases (2012) 129, e22e25

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ORIGINAL ARTICLE

Ramsay Hunt syndrome

R. Zainine , M. Sellami , A. Charfeddine , N. Beltaief , S. Sahtout , G. Besbes

Service dORL et de chirurgie maxillofaciale, hpital La Rabta, Jabbari, 1007 Tunis, Tunisia

Available online 24 February 2012

KEYWORDS Summary
Ramsay Hunt Introduction: Ramsay Hunt syndrome is a viral infection that combines facial palsy and pinnal
syndrome; vesicular rash.
Peripheral facial Objectives: To detail diagnostic criteria, treatment and prognosis in Ramsay Hunt syndrome.
palsy; Patients and methods: A retrospective study of 15 patients with Ramsay Hunt syndrome admit-
Acyclovir; ted to the ENT department of La Rabta Hospital (Tunis) from 2003 to 2009.
Steroid therapy Results: The main presenting symptom was facial palsy associated with conchal vesicular rash.
House-Brackmann classication of facial nerve function ranged from III to V. All patients were
treated with acyclovir and corticosteroids. Mean follow-up was 8 months. Ten patients showed
improvement in facial palsy, four had permanent palsy and one moved from grade IV to grade
III.
Conclusion: Ramsay Hunt syndrome involves severe dysfunction, with poorer facial nerve prog-
nosis than in Bells palsy.
2011 Published by Elsevier Masson SAS.

Introduction The present study describes the clinical presentation,

In 1907, J. Ramsay Hunt described a syndrome associating
facial palsy, inner ear dysfunction, pinnal pain and vesicular
rash [1].
Now referred to as Ramsay Hunt syndrome, it is a rare
cause of facial palsy, possibly originating in resurgence of
varicella-zoster virus (VZV) present in the geniculate gan-
glion from the rst infestation, although many authors have
questioned this theory [2].
Treatment is empiric; several drugs have been reported,
including corticosteroids, vasodilators, vitamins and antivi-
ral agents.
diagnosis, treatment and prognosis of this rare but severe
complication of herpes zoster.
Patients and methods
A retrospective study included 15 of the 30 Ramsay Hunt
patients managed in the ENT department of the Rabta Uni-
versity Hospital Center of Tunis (Tunisia) over a 7-year period
(20032009). Unusable les and patients lost to follow-up
were excluded. Diagnosis was clinical.
Results

Corresponding author. Tel.: +216 98 61 45 25; Eight men and seven women, mean age 46 years (range,
fax: +216 71 56 18 89. 1275 yrs), consulted at a mean 8 days (range, 221 days)
E-mail address: rimzainine@yahoo.fr (R. Zainine). after symptom onset. Four patients had been exposed to

1879-7296/$ see front matter 2011 Published by Elsevier Masson SAS.

doi:10.1016/j.anorl.2011.08.003
Ramsay Hunt syndrome e23

Figure 1 Vesicular lesions of the concha.

Figure 2 Axial T1-weighted MRI: strong contrast uptake in the

cold during the days preceding onset. The main presenting right intratemporal facial nerve.
symptom was in all cases facial asymmetry associated with
conchal vesicular rash. Thirteen patients had associated
otalgia. Nine showed partial hearing loss, with rotational
Discussion
vertigo and unstable gait in four cases. Two patients pre-
sented with herpes zoster of the V3 territory. Otic involvement in herpes zoster viral infection is impli-
Clinical examination found peripheral facial palsy with cated in 12% of cases of peripheral facial palsy [4]. Latent
muscular test ranging from 1/30 to 18/30 (mean, 7/30) or VZV infection is reactivated in the geniculate ganglion, caus-
III to V on the House-Brackmann grading system [3]. Vesicular ing otalgia, pinnal vesicular rash and peripheral facial palsy.
lesions of the ipsilateral Ramsay-Hunt region were observed It is a rare pathology, with an incidence of 5/100,000 per
in all patients, and founded diagnosis (Fig. 1). Neurovestibu- year; nevertheless, it is the second most frequent etiology
lar examination found harmonious vestibular syndrome in in non-traumatic peripheral facial palsy [5].
four cases. Prognosis for facial palsy is poorer in Ramsay Hunt syn-
Systematic audiography found unilateral perception drome than in idiopathic forms [6]. Only 10% of patients with
hearing loss in 11 patients, with a mean threshold of 55 dB complete facial palsy are totally cured [4].
(range, 3090 dB). Stapedial reex was absent ipsilaterally Facial nerve involvement is initially due to inammation
to the facial palsy in all cases. caused by the viral neuronitis and secondarily to the facial
Caloric test, performed in the four patients with ver- nerve edema [1,7].
tigo, showed hyporeexia in three cases and areexia in the Strictly, Ramsay Hunt syndrome is dened as facial palsy
fourth. with associated erythematovesicular rash of the pinna or
All patients received per os acyclovir (Zovirax ) antiviral mouth. Possible involvement of Cortis (hearing loss) or
treatment and high-dose corticosteroids by general route Scarpas (vertigo) ganglions completes the total otic form
for a mean 8 days. They had also undergone motor physio- of pinnal herpes zoster.
therapy and received local care for the pinnal lesions. Five Diagnosis is founded on clinical history and physical
underwent brain and internal auditory canal MRI at a mean examination [8]. This was the case in all the present series.
30 days after diagnosis, revealing facial neuritis in four cases
(Fig. 2).
Over a mean follow-up of 8 months (range, 3 to Table 1 House-Brackmann facial nerve function grading
48 months), facial palsy improved in 10 patients, with test system before and after treatment.
scores of 20 to 30/30 and grades I or II; in four patients,
Baseline facial nerve Facial nerve function Total
palsy remained stable (grade IV or V) and one patient showed
function after treatment
improvement from grade IV to grade III (Table 1).
Cochleovestibular evolution showed persistent vertigo in I II III IV V
one patient at 7 months FU and persistent perception hea-
III 1 0 0 0 0 1
ring loss in six patients. Three of the other patients showed
IV 4 2 1 1 0 8
improvement in vertigo and ve in hearing loss, with mean
V 0 3 0 0 3 6
perception hearing loss of 35 dB (range, 2540 dB) on control
Total 5 5 1 1 3 15
audiogram.
e24 R. Zainine et al.

Table 2 Therapeutic protocols in management of Ramsay Hunt syndrome.

Series Corticosteroids Dose Route Antiviral Dose Route

Uri et al. [13] Hydrocortisone 100 mg 3/d IV Acyclovir 5 mg/kg/8h IV

Murakami et al. [15] Prednisolone 1 mg/kg/d PO Acyclovir 800 mg 5/d PO
Kinishi et al. [14] Methylprednisolone 500 mg/da IV Acyclovir 800 mg 5/d PO
Yeo et al. [11] Prednisolone 1 mg/kg/d PO AcyclovirFamciclovir 5mg/kg/8 h500mg/db IV PO
Present series Hydrocortisone 100 mg 3/d IV Acyclovir 800 mg 5/d PO
PO: per os; IV: intravenous.
a Methylprednisolone 500 mg/d the 1st day, then 250 mg/d the 2nd and 3rd days, and 100 mg/d the last 4 days.
b Acyclovir for 5 days, then Famciclovir 500 mg/d for 7 days.

Gadolinium-enhanced MRI and CSF analysis have no diagnos-
tic or prognostic value [9].
The natural history of pinnal Ramsay Hunt syndrome
was described in a retrospective study of 102 non-treated
patients [10]: facial nerve functional impairment reached a
maximum in the rst week.
A retrospective study [11] of 81 treated patients, inclu-
ding 55 cases of Bells palsy and 26 of Ramsay Hunt syn-
drome, found recovery from palsy, with House-Brackmann
grades of I or II in 96.3% of Bells palsy cases and in 84.6%
of palsies associated with Ramsay Hunt syndrome. Advanced
age, elevated arterial blood pressure, vertigo and diabetes
were factors of poor prognosis in Ramsay Hunt syndrome.
The neural edema in a narrow space in the Fallopian
canal or internal auditory canal induces neural compres-
sion and hypoxia, exacerbating the neural degeneration. The
powerful anti-edematous action of corticosteroids has been
clinically conrmed: high-dose corticosteroids is one of the
recognized treatments for Ramsay Hunt syndrome [12].
The classical high-dose corticosteroid treatment for
Ramsay Hunt syndrome has been changed by the advent of
new virostatic agents, notably acyclovir, which has an effec-
tive virostatic action on herpes group viruses by interference
with DNA polymerase and inhibition of replication. Results
are best with early administration [13]. In the present series,
all patients received an association of per os acyclovir
and high-dose injected corticosteroids, resulting in clear
improvement in 10 of the 15 patients (Table 2).
Kinishi et al. [14] compared isolated corticotherapy (47
patients) and associated acyclovir and high-dose injected
corticosteroids (91 patients). Prognosis was signicantly bet-
ter in the latter group, with good nervous function and
improved recovery rate.
Acyclovir is administered at 15 mg/kg per day. As diges-
tive tract absorption concerns only 1520% of the ingested
dose, higher doses are recommended in per os delivery:
800 mg per os ve times daily for 710 days in adults [15].
There was no signicant difference in prognosis according
to per os versus i.v. administration [15].
Murakami et al. [15], in a large retrospective series
of 80 patients, found signicant improvement with associ-
ated acyclovir and prednisone administered within 3 days
of symptom onset. Recovery was complete in 75% of
patients treated with three days, compared to only 30%
of those treated after 7 days. This suggests that early
diagnosis and management improves prognosis in Ramsay
Hunt syndrome.
Conclusion
Ramsay Hunt syndrome is one of the least frequent causes
of facial palsy, inducing severe dysfunction and of poorer
prognosis for the facial nerve than Bells palsy.
Associated acyclovir and high-dose corticosteroids
improves facial nerve functional recovery.
Disclosure of interest
The authors declare that they have no conicts of interest
concerning this article.
References
[1] Hunt JR. On herpetic inammations of the geniculate gan-
glion, a new syndrome and its complication. J Nerve Ment Dis
1907;34:7396.
[2] Aviel A, Marshak G. Ramsay Hunt syndrome: a cranial polyneu-
ropathy. Am J Otolaryngo 1982;3:616.
[3] House JW, Brackmann DE. Facial nerve grading system. Oto-
laryngol Head Neck Surg 1985;93:1467.
[4] Robillard ZRB, Hilsinger Jr RL, Adour KK. Ramsay Hunt facial
paralysis: clinical analysis of 185 patients. Otolaryngol Head
Neck Surg 1986;95:2927.
[5] Adour KK. Otological complications of herpes zoster. Ann Neu-
rol 1994;35(Suppl.):S624.
[6] Devriese PP, Moesker WH. The natural history of facial paralysis
in herpes zoster. Clin Otolaryngol 1988;13:289.
[7] Blackley B, Friedmann I, Wright I. Herpes zoster auris associ-
ated with facial nerve palsy and auditory nerve symptoms: a
case report with histopathological ndings. Acta Otolaryngol
(Stockh) 1967;63:53350.
[8] Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neu-
rosurg Psychiatry 2001;71:14954.
[9] Jonsson L, Tien R, Engstrom M, et al. Gd-DPTA enhanced MRI
in Bells palsy and herpes zoster oticus: an overview and impli-
cations for future studies. Acta Otolaryngol 1995;115:57784.
[10] Devriese PP, Moesker WH. The natural history of facial paralysis
in herpes zoster. Clin Otolaryngol 1988;13:28998.
[11] Yeo SW, Lee DH, Jun BC, et al. Analysis of prognostic factors
in Bells palsy and Ramsay Hunt syndrome. Auris Nasus Larynx
2007;34:15964.
Ramsay Hunt syndrome
[12] KinishiM, HosomiH, Amatsu M, et al. Conservative treat-
ment of Hunt syndrome. J Otolaryngol Jpn 1992;95:
6570.
[13] Uri N, Greenberg E, Kitzes-Cohen R, et al. Acyclovir in the
treatment of Ramsay Hunt syndrome. Otolaryngol Head Neck
Surg 2003;129:37981.
e25
[14] Kinishi M, Amatsu M, Mohri M, et al. Acyclovir improves recov-
ery rate of facial nerve palsy in Ramsay Hunt syndrome. Auris
Nasus Larynx 2001;28:2236.
[15] Murakami S, Hato N, Horiuchi J, et al. Treatment of Ramsay
Hunt syndrome with acyclovir-prednisone: signicance of early
diagnosis and treatment. Ann Neurol 1997;41:3537.