Case Presentation: A 63-year-old woman presented with a 4-day history of progressive dysphagia, first to solid food, then to liquids and oral secretions. She endorsed hoarseness and mild lateral neck and chin pain, but denied trauma, headache, neck stiffness, and rash. Her past medical history included gastroesophageal reflux and remote esophageal erosions with normal follow-up endoscopy. She quit smoking 17 years ago. On admission, pulse was 84, temperature 36.6C, respiratory rate 16, blood pressure 129/79, and oxygen saturation 94% on room air. Her neck was supple without masses, but was mildly tender to palpation over the right sternocleidomastoid and submandibular areas. Oropharynx was unremarkable; electrolytes, white blood cell count, and thyroid-stimulating hormone were normal. On day 2, she reported sinus congestion and scant purulent drainage from the right ear; levofloxacin was initiated for bacterial rhinosinusitis. Esophagogastroduodenoscopy found no strictures, and nasopharyngolaryngoscopy demonstrated edema of right aryepiglottic fold and arytenoid. Neck CT revealed mass-like fullness in right post-pharyngeal mucosal space and mild vocal cord asymmetry, so vancomycin and dexamethasone were added for supraglottitis. Swallowing improved slightly, however, she noted new loss of throat sensation, burning neck pain extending behind the right ear, dizziness, and decreased hearing on the right. Brain MRI was normal. On day 6, vesicular lesions appeared in her right auricle, and asymmetric elevation of her soft palate was noted. Repeat nasopharyngolarynogscopy showed new right true vocal cord paresis. Vesicle fluid was positive for varicella zoster virus (VZV) DNA by polymerase chain reaction, and serum VZV IgM was elevated, confirming Ramsay Hunt Syndrome. Acyclovir and prednisone were initiated, resulting in recovery of swallow function.
Discussion: This case highlights dysphagia as an under-recognized potential symptom of Ramsay Hunt syndrome (RHS). Classically defined by otalgia, facial nerve palsy, and hearing and balance problems caused by VZV reactivation in cranial nerve (CN) VII/VIII, there is a growing literature describing RHS as a VZV-related polycranioneuropathy, with reports of deficits in CN III, IV, VI, IX, and X. This patient’s dysphagia, asymmetric palate elevation, and loss of throat sensation suggest involvement of CN IX; right vocal cord paresis and hoarseness implicate CN X and its recurrent laryngeal branch. In a review compiling 14 atypical cases of RHS, 13 were characterized by dysphagia, odynophagia, or regurgitation. The severity of incapacity varies, from need for gastric tube placement to full recovery.
Conclusions: VZV-related cranial neuropathies are gaining increasing attention as a potentially reversible cause of rapidly progressive oropharyngeal dysphagia. It is important for generalists to recognize this rare presentation of a common disease and be aware that Ramsay Hunt syndrome can involve cranial nerves other than those typically affected.