Ramsay Hunt syndrome (RHS) is characterized by facial paralysis, inner ear dysfunction, periauricular pain, and herpetiform vesicles. The reported incidence in children is 2.7/100,000. The pathogenesis involves the reactivation of latent varicella zoster virus (VZV) in the geniculate ganglion of the facial nerve. The recovery rate is better in children than in adults. This paper discusses a 12-year-old girl with a rare case of peripheral facial paralysis caused by RHS and reviews the literature.
In 1907, Hunt first described Ramsay Hunt syndrome (RHS), a disease characterized by facial paralysis, inner ear dysfunction, periauricular pain, and herpetiform vesicles [
Congenital anomalies, trauma, otitis media, Lyme disease, metabolic and genetic factors, and malignancy of the temporal bone or parotid gland can cause peripheral facial paralysis in children. Herpes simplex virus (HSV-1), Epstein-Barr virus (EBV), cytomegalovirus (CMV), human herpes virus (HHV 6 and 7), and mumps virus can all cause acute peripheral paralysis. However, the etiology is unknown in most cases, resulting in a diagnosis of idiopathic peripheral facial paralysis or Bell’s palsy. Bell’s palsy is the most common peripheral facial paralysis in children, and it is reported to cause 24–70% of all cases of peripheral facial paralysis in children [
Although RHS is the second most common atraumatic cause of facial paralysis, it is very rare in children. Hato et al. reported that RHS caused 16.7% of all peripheral facial palsies in children [
This study presents a case of peripheral facial paralysis caused by RHS and discusses the current literature.
The patient was a 33 kg, 138 cm-tall, 12-year-old girl. She came to the pediatrics clinic with a chief complaint of facial asymmetry. She first developed a sore throat, cough, and runny nose, and this was followed 2 days later by pain around the right ear and a vesicular rash on the right ear pinna and behind the ear. The facial asymmetry started 3 days after the periauricular rash. She presented to our clinic on the second day of facial paralysis. She had no audiovestibular complaints, such as vertigo, tinnitus, nausea, vomiting, or hearing loss. Her medical history included varicella infection at 5 years of age.
The patient had Grade 4 peripheral facial paralysis according to the House-Brackmann (HB) classification (Figure
Grade 4 peripheral facial paralysis on the right.
Crusted herpetic vesicles on the right ear pinna.
Based on the clinical findings, RHS was diagnosed at this point. The patient was started on methylprednisolone (2 mg/kg/day, maximum 60 mg/day) and oral acyclovir (800 mg/day) simultaneously. The methylprednisolone dose was tapered to 1 mg/kg at the beginning of the second week, tapered during the second week, and stopped on day 14. The acyclovir was continued for 5 days. Artificial tear drops were recommended to protect the cornea. Facial electromyography (EMG) was not required, as the patient’s clinical picture improved by day 21. Cranial imaging was also not required, as the patient had no history of peripheral facial paralysis and no neurological manifestations.
Herpes zoster oticus or Ramsay Hunt syndrome is a disease that affects cranial nerves 7 and 8. The acute peripheral facial paralysis is characterized by vestibulocochlear dysfunction and a herpetic rash around the ear pinna and outer ear canal. It is reported to be the cause of 2–10% of acute peripheral facial paralysis cases [
The primary VZV infection is varicella. The virus moves retrogradely and stays latent in the geniculate ganglion of the facial nerve, and the clinical picture of RHS presents after the reactivation of VZV there.
Typically, the clinical picture begins with otalgia that lasts 1~3 days. Then, herpetic vesicles appear in the outer ear canal, tympanic membrane, mouth, and distal 2/3 of the tongue, which can also cause losing taste sensation. If the eighth cranial nerve is also affected, nausea, vomiting, vertigo, nystagmus, tinnitus, and hearing loss can occur [
The disease can also present with only acute peripheral facial paralysis, without the vesicular rash. This condition, termed “zoster sine herpete,” tends to be misdiagnosed as Bell’s palsy [
To diagnose RHS in children, enzyme-linked immunosorbent assay (ELISA) serum anti-VZV IgG and IgM antibody titers are recommended [
Starting steroids and antiviral agents in the early phase of the disease is recommended. Antivirals prevent VZV replication and facial nerve involvement, and the steroids prevent inflammation and edema. Murakami et al. examined the recovery of facial nerve function after starting treatment in the first 3 days, at 3–7 days, or later than 7 days and found that the recovery was best when treatment was started within 3 days of disease presentation [
Kim and Bhimani recommended starting oral acyclovir at a dose of 800 mg for 7–10 days and prednisone 1 mg/kg/day for 5 days, followed by weaning over the next 6-7 days [
The prognosis of the facial paralysis in RHS is worse than that in Bell’s palsy, and only 10% of complete facial paralysis in RHS recovers completely [
With this case report, we show that VZV reactivation can be a rare cause of acute peripheral facial paralysis in children aged 6–15 years and that early diagnosis and treatment have a positive effect on disease prognosis.
The authors declare that there is no conflict of interests regarding the publication of this paper.
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