Ramsay Hunt syndrome

Dear Editor, Ramsay Hunt syndrome (RHS), or herpes zoster oticus, is a triad of infranuclear facial nerve palsy, herpetiform eruptions over the ipsilateral pinna and external auditory canal, and severe otalgia. Although these patients primarily present to an otorhinolaryngologist due to otalgia and skin eruptions over the pinna, some of them may first visit an ophthalmologist due to ocular complaints. A 55-year-old gentleman presented to the ophthalmology outpatient department with the inability to close the right eye and left-sided facial deviation for the past 7 days. He also had rashes over his right pinna for the same duration. There was no history of fever, ear pain, or ear discharge. No complaint of eye-watering, dryness, redness, diminution of vision, or taste alteration. No prior history of diabetes and hypertension was present. He had no history of repeated sinopulmonary infections, intravenous drug abuse, and multiple sexual partners. On examination, the patient had right infranuclear facial nerve palsy House–Brackmann Grade 5 [Figure 1a and b] with multiple erythematous healing lesions over the conchal bowl of the right pinna [Figure 1c]. Debris was present over the right external auditory canal walls and tympanic membrane. There was no conjunctival congestion, conjunctival, or corneal xerosis. His blood sugars were within normal range, and the test for HIV was negative. He was started on oral prednisolone 1 mg/kg in tapering doses over 3 weeks along with tablet acyclovir 400 mg five times a day for 7 days. He was advised night taping of the right eye and frequent use of tear supplement in the affected eye to avoid exposure to keratopathy as eye care. Facial exercises were also advised and taught for the rehabilitation.Figure 1: (a and b) Right-sided infranuclear facial nerve palsy, (c) Herpetiform eruptions over right pinnaRHS, first described by Ramsay Hunt in 1907, is the manifestation of reactivated varicella zoster virus (VZV) which resides latent in the geniculate ganglion. Immunocompromised conditions such as diabetes mellitus and HIV are the common triggers for reactivation, COVID-19 also being a recently reported trigger.[1] However, in our case, none of the above listed causes were present. Apart from the clinical triad, diagnosis of RHS can be by demonstration of VZV in samples from vesicular lesions by polymerase chain reaction (PCR) or by viral culture. Serological tests for VZV antibodies can be used in case of equivocal results on PCR or culture. Increased absolute values indicate active VZV infection, whereas a serial increase in titers of antibodies can also be used for diagnosis in case of less typical presentation. Magnetic resonance imaging demonstrates enhancement of affected facial nerve near the geniculate ganglion, the extent of involvement, and other affected cranial nerves. It also excludes other possible causes for the condition.[2,3] However, these investigations are not strictly necessary for diagnosis. The treatment of the condition includes corticosteroids, antiviral agents, eye care, and facial exercises. The choice of corticosteroids has classically been oral prednisolone 1 mg/kg in tapering doses. In a review by Monsanto et al., methylprednisolone (81.3%) had a better effect as compared to prednisolone (61.4%).[4] Acyclovir has been traditionally used as an antiviral agent; however, no significant difference in outcome of facial palsy was found by intravenous or oral route.[5] The compliance of patients is better with valacyclovir 1000 mg given thrice a day for 7 days. Combining acyclovir with prednisolone resulted in reduced nerve degeneration and better hearing recovery.[5] The medical management should start as soon as possible for good recovery, preferably within the first 5 days.[4-6] In case of delayed presentation up to 1 week, offering treatment is helpful for recovery although the outcome may not be as good as compared to early treatment.[3] It has a good rate of recovery though when compared with Bell's palsy, has a poorer rate of recovery along with a higher rate of synkinesis. Postherpetic neuralgia may persist up to 3 months. In conclusion, the patients with RHS may first report to an ophthalmologist with ocular complaints such as inability to close the eye, watering in the affected eye, and even exposure keratopathy to the grade of vision loss, if not diagnosed and treated timely. A high degree of clinical suspicion and timely multidisciplinary medical management can provide a favorable outcome in most of the cases. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.