Ramsay Hunt syndrome with atypical auricular findings: Reports of two cases

Ramsay Hunt syndrome (RHS) is caused by the reactivation of the varicella-zoster virus (VZV) and is characterized by a classical triad of auricular vesicles, otalgia, and peripheral facial palsy.1 Early treatment within 3 days of disease onset is recommended to minimize neurological sequelae.2 Although the diagnosis is usually clinical, few reports have focused on auricular findings. Herein, we report two pediatric cases of RHS that were initially misdiagnosed as bacterial auricular infections owing to atypical auricular findings. Case 1.A 12-year-old male patient who underwent ciliary astrocytoma surgery at the age of three presents with severe mental retardation, making communication about pain unfeasible. The patient had not undergone chemotherapy. He had a mild varicella infection at 12 months old and had not been vaccinated against VZV. Six days before admission, redness appeared on the right auricle, and the patient began to scratch the area. Examination revealed erythema, swelling, crusting, and blisters (Figure 1a). The patient was diagnosed with auricular cellulitis. Despite intravenous ampicillin/sulbactam administration, the symptoms persisted. On the third day of hospitalization (ninth day of illness), the patient developed right facial palsy. The diagnosis of RHS was made. Acyclovir and prednisolone treatment was initiated on day 4 of hospitalization. Serological results were positive for VZV IgG and IgM. The patient completed a 7-day course of acyclovir and a 13-day course of steroid therapy. Two years later, the patient experienced persistent twitching while smiling. Case 2.A 12-year-old boy with diffuse large B-cell lymphoma undergoing chemotherapy developed left ear pain, fever (38.0°C), and auricular swelling with a crust on the antitragus (Figure 1b). He had a varicella infection at 12 years old (several months before administration) but was not vaccinated. Blood tests revealed a white blood cell count of 3010/μL (30.5% neutrophils and 6.0% lymphocytes) and a C-reactive protein level of 3.52 mg/dL. Due to the dark purple lesion and immunocompromised status, ecthyma gangrenosum was suspected, and cefepime was initiated. However, the symptoms did not improve. On day 5, a VZV rapid antigen test of the crust was positive, and a 14-day course of acyclovir was initiated. Although the auricular pain improved, left facial palsy appeared on day 13. The diagnosis of RHS was made. A 6-day course of prednisolone was administered, leading to full recovery without any sequelae. In both cases, the initial auricular findings lacked typical vesicles, leading to a misdiagnosis of bacterial infection. The diagnosis of RHS often relies on auricular lesions; however, some patients present with erythema, swelling, or crusting without clear vesicles, especially in early or atypical cases. Moreover, facial palsy may occur days later, further complicating diagnosis. Additionally, in Case 1, it was difficult to diagnose RHS because of the difficulty in assessing otalgia due to mental retardation. We could not find any cohort studies that systematically analyzed auricular findings; therefore, we reviewed pediatric RHS reports from the past 15 years using PubMed with the keywords “Ramsay Hunt syndrome” and “children,” identifying eight patients with color images (Table S1). Otalgia was observed in 8 patients (80%) and 2 patients could not be assessed due to their age or mental retardation. Auricular redness was observed in all 10 patients (100%), swelling in 6 (60%), vesicles in 9 (90%), and crusting without vesicles in 1 (10%). Pinna protrusion, a suggestive finding of mastoiditis, was observed in 1 (10%). Four (40%) patients lacked facial palsy at the initial presentation, and in 57% of the cases, auricular signs preceded facial palsy. In one report of 32 children with RHS, blisters preceded facial palsy in 15.6% of cases, occurred simultaneously in 34.4%, and followed facial palsy in 50%.3 In another report, pediatric RHS without any auricular findings was reported,4 so we should consider RHS when we observed otalgia and facial palsy. In Case 2, the auricular finding appeared mild. Leukopenia induced by chemotherapy may have impaired the host inflammatory response, resulting in less local inflammation. We should recognize that although VZV infection in immunosuppressed patients can become severe, local findings may not always be severe. In conclusion, RHS can initially mimic bacterial auricular infections, particularly in children with atypical auricular findings and delayed-onset facial palsy. Awareness of such presentations and consideration of RHS, even in the absence of vesicles, are critical. HS, RH, SE, and SH provided the patient care. HS and MI drafted the manuscript. YA and AS supervised the study and reviewed the final manuscript. All authors contributed to writing the final manuscript and met the ICMJE authorship criteria. All authors gave approval of the final version. Informed consent was obtained to publish the clinical course from his parent in Case 1 and from the patient and his parent in Case 2. We thank Editage (www.editage.jp) for English language editing. This study did not receive any specific grants from funding agencies in the public, commercial, or nonprofit sectors. The authors declare no conflict of interest. The data that support the findings of this study are available upon request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions. Table S1. Literature review comparing auricular findings and symptoms of RHS in children. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. 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