Ramsay Hunt Syndrome

AFFILIATIONS:
¹Emergency Department, Jefferson Health New Jersey – Stratford, Stratford, NJ
²Emergency Department, Jefferson Health New Jersey – Washington Township, Washington Township, NJ
³Emergency Department, Jefferson Health New Jersey – Cherry Hill, Cherry Hill, NJ

CITATION:
Lucerna A, Espinosa J, Coleman T, Lee J. Ramsay Hunt syndrome. Consultant. 2023;63(5):e6. doi:10.25270/con.2023.03.000004

Received June 14, 2022. Accepted December 22, 2022. Published online March 21, 2023.

DISCLOSURES:
The authors report no relevant financial relationships.

ACKNOWLEDGEMENTS:
None.

CORRESPONDENCE:
Alan Lucerna, Emergency Department, Jefferson Health New Jersey – Stratford, 18 East Laurel Road, Stratford, NJ 08084 (lucernaa@gmail.com)

Introduction. A 55-year-old man presented to the emergency department with a 4-day history of rash, redness, swelling, and pain of his left ear.

History. The patient reported discomfort on the left ear two days prior to noticing the rash and the redness. When the redness and pain started, it was associated with decreased hearing and intermittent left facial paresthesias. The patient denied any preceding upper respiratory infection or any recent illness. There was no history of trauma. The patient’s medical history included stroke, hyperlipidemia, and peripheral vascular disease. His surgical history was significant for remote angioplasty and stent placement of his left femoral artery. He had a 20-pack year smoking history but denied any other drug or alcohol use.

The vital signs on arrival were as follows: 119/88 mm Hg, 79 beats/min, 17 breaths/min, 98.1°F (taken orally), and 96% oxygen saturation level on room air. On physical examination, a crop of vesicular lesions in the concha of the left ear was identified (Figure 1). The helix of the left ear was erythematous (Figure 2). The left ear canal was swollen. There was no drainage observed from the ear canal. The left tympanic membrane could not be visualized secondary to swelling of the left ear canal. No other lesions were noted in the face, eyes, nose, mouth, or other surrounding areas. The patient was able to do full eye closure, and there were no reported visual changes. No facial asymmetry was appreciated. There was no tenderness to palpation of the left mastoid area.

Figure 1. A crop of vesicular lesions in the concha of the patient’s left ear.

Figure 2. Helix of the patient’s left ear showing erythema and edema.

Diagnostic testing. Magnetic resonance imaging was ordered and showed no acute stroke.

Differential diagnoses. The differential diagnosis includes Bell palsy, Ramsay Hunt syndrome, and post-herpetic neuralgia. The patient had no facial palsy and there was no previous history of a previous herpetic infection of the face or ear.

Treatment and management. The patient was admitted to the hospital and started on valacyclovir 1000 mg every 8 hours as well as prednisone 60 mg for 5 days by mouth. The patient was seen by the otolaryngologist, who agreed with the diagnosis of Ramsay Hunt syndrome. Due to the patient’s history of stroke and peripheral vascular disease, a neurologist was also consulted, who ruled out a cerebral vascular accident.

Outcome and follow-up. The patient was discharged home on valacyclovir for 7 days and a 12-day prednisone taper. Two weeks after hospital discharge, the patient noted via telemedicine that he was pain-free and that the rash had resolved completely.

Discussion. Around the turn of the 20th century, James Ramsay Hunt, MD, described a case of a patient with ear pain associated with a vesicular rash of the ear, which he attributed to what we now know as varicella-zoster virus (VZV). His research led to a better understanding of the function of cranial nerve VII.¹

Ramsay Hunt syndrome, also called herpes zoster oticus, is a group of possible complications of VZV infection which has a classic triad of symptoms. These symptoms include ear pain with vesicles within the ear canal, in association with facial paralysis of the affected side.² Symptoms may also include tinnitus, hearing loss, vertigo, hyperacusis, and decreased tearing.³ The nature of the symptoms makes it apparent that more than cranial nerve VII may be involved. Cranial nerves VIII, IX, V, and VI may also be affected.³

The essential pathophysiology is reactivation of latent VZV infection predominantly affecting the geniculate ganglion of cranial nerve VII, but with the potential to spread to cranial nerves that are in close proximity, with associated edema and inflammation.¹

The incidence of Ramsay Hunt syndrome is considered rare; it has been estimated to affect between 5 and 30 individuals per 100,000 population.^4-6 The peak incidence of Ramsey Hunt syndrome is the eighth decade of life.⁷ Bell palsy is overall more common than Ramsay Hunt syndrome.³

It is possible to have Ramsay Hunt syndrome in the absence of the cutaneous rash. This has been referred to as “zoster sine herpete” and is estimated to occur in 8% to 12% of cases of unilateral facial nerve palsy in adult patients.^1,3

Ramsay Hunt syndrome often follows a viral prodrome with upper respiratory tract symptoms.⁵ Most patients present with ear pain that precedes the rash by several days. Facial weakness may present after the onset of rash and ear pain and may reach maximum severity in 5 to 7 days. The rash most commonly affects the pinna and external auditory canal, as was seen in our patient, and may involve the soft palate and tongue.

The diagnosis of Ramsay Hunt syndrome is essentially clinical. Diagnosis is based on the patient’s history, clinical findings, and neurologic examination.⁸

The mainstay of treatment for Ramsay Hunt syndrome continues to be oral antiviral medications, such as acyclovir and oral corticosteroids. Only 20% of people achieve complete recovery without proper treatment.⁶ There are no high-quality randomized clinical trials to guide the duration of treatment or to show the effect of treatment on outcome. Early administration of oral antivirals appears to be related to better facial nerve outcomes and decreased risk of post-herpetic neuralgia.^3,4 Sweeney and colleagues¹ recommend famciclovir or acyclovir for 7 to 10 days with oral prednisone for 3 to 5 days.

Morrow and colleagues⁹ support the use of both antiviral therapy and oral prednisone in Ramsay Hunt syndrome despite what they note to be weak evidence to prove the effect of the combination. They recommend prednisone at a higher dose for 5 to 10 days, followed by a tapering dose.

A small study by Furuta and colleagues¹⁰ supports combination treatment of antivirals and corticosteroids. Similarly, Murakami and colleagues¹¹ support the combination treatment approach. A systematic review by Uscategui and colleagues¹² concluded that evidence was weak to support antiviral and corticosteroid combination treatment. Ocular lubricants and opthalmologic care may be needed if ocular symptoms, such as ptosis, are found in an individual case.

There are some data on prognosis. As a general principle, it appears that facial paralysis, if present, overall has a better prognosis with Bell palsy than with Ramsay Hunt syndrome.^4,11 Facial paralysis outcome studies use objective scales such as the House-Brackmann scale, although modified and newer grading systems are used in some studies.¹³ The House-Brackmann scale has six grades, with grade 1 indicating normal function and grade 6 indicating total paralysis. ¹⁴ Ryu and colleagues⁴ studied 115 patients with Ramsay Hunt syndrome and found increasing age to be a negative risk factor. Diabetes and hypertension were risk factors for a poorer prognosis. When facial paralysis was part of the Ramsay Hunt constellation, increasing peak severity after onset appears to be associated with a less favorable outcome.⁴ Yeo and colleagues¹⁵ studied 26 patients with Ramsay Hunt syndrome and determined that the negative prognostic features were age greater than 61 years, diabetes, hypertension, and presence of vertigo at the time of initial presentation. Coulson and colleagues⁵ studied 101 patients with Ramsay Hunt syndrome involving the facial nerve and found that incomplete eye closure and dry eye were poorer prognostic indicators. They also reported that 59% of patients with Ramsay Hunt syndrome achieved a satisfactory recovery by 1 year, defined as a House-Brackmann scale of grade 1 or grade 2.⁵

Conclusion. Ramsay Hunt syndrome is a rare manifestation of varicella zoster virus reactivation with a constellation of symptoms, including possible facial paralysis, tinnitus, hearing loss, vertigo, hyperacusis, and decreased tearing. In the case presented, there was no facial palsy noted. Although Ramsay Hunt syndrome may mimic Bell palsy, it is overall less common than Bell palsy. The diagnosis is essentially clinical, and the treatment mainstay continues to be oral antiviral medications, such as acyclovir and oral corticosteroids. Areas for future research might include high-quality randomized clinical trials to guide the duration of treatment and to show the effect of treatment on outcome.