ear, nose, and throat specialist registrar 1 , Farah Latif core medical trainee 2 , Sandeep Berry ear, nose, and throat consultant 1A 76 year old man presented to the emergency department with a three day history of a painful rash on his right cheek, associated with right sided otalgia and sore throat. He had noticed a right sided facial droop over the past 24 hours. He had no history of other neurological disturbances, otological symptoms, or head trauma.Ten years earlier he had received a right cochlear implant, which was still functioning, and he also had benign prostatic hypertrophy. He was on aspirin 75 mg once daily and tamsulosin 400 µg once daily and had no known allergies.He was afebrile and had partial right facial nerve palsy with incomplete eye closure; his forehead muscles were also affected. Neurological examination was otherwise normal. Examination of the right ear identified vesicles on the external auditory canal; the right tympanic membrane was normal. The left ear was normal. A rash comprising lentil sized vesicles, in part confluent and pustular with surrounding erythema, was noted in the dermatomal distribution of the maxillary branch of the right trigeminal nerve. A vesicular rash was noted on the right side of the hard palate (figure); the oropharynx was normal. Examination of the neck showed no parotid swelling or cervical lymphadenopathy.Vesicular rash on the right side of the hard palate Questions 1. On the basis of the history and clinical findings, what is the likely diagnosis? 2. What system is usually used to grade facial nerve palsy?3. How is this condition managed?
Answers
On the basis of the history and examination findings, what is the likely diagnosis? Short answerRamsay Hunt syndrome, which is caused by reactivation of the varicella zoster virus (VZV) in the geniculate ganglion of the facial nerve. Clinically, it usually comprises a painful vesicular rash on the external ear (herpes zoster oticus) associated with ipsilateral lower motor neurone facial nerve palsy. Because it is a cranial polyneuritis, other cranial nerves-most often the vestibular, cochlear, and trigeminal nerves-can also be affected.
Long answerRamsay Hunt syndrome refers to reactivation of VZV, usually in the geniculate ganglion of the facial nerve. It was initially described to comprise the clinical features of lower motor neurone facial nerve palsy and painful vesicular rash in the ipsilateral ear (herpes zoster oticus) and palate. 1 Other possible features include hyperacusis and vesicular rash on the anterior two thirds of the tongue, taste disturbance, and reduced lacrimation owing to impaired functioning of the facial nerves. Hearing loss, tinnitus, vertigo, nausea, and vomiting can occur if the neighbouring cochlear and vestibular nerves are inflamed. The virus is thought to enter the geniculate ganglion during the initial chickenpox infection through the sensory branches of the facial nerve located on the ear and tongue. 2The associated dermatological signs and symptoms can be explained by viral infec...
