Introduction: Orthotopic heart transplantation (OHT) increases the risks of varicella-zoster reactivation, and severe complications may arise due to infection in the setting of active immunosuppression. Complications include meningitis, facial nerve palsy (known as Ramsay Hunt Syndrome), and bacterial superinfection. When severe bacterial ear infections spread to the petrous apex, facial pain accompanied by otitis media and abducens nerve palsy may also occur, classically known as Gradenigo Syndrome. Case Report: We present a case of a patient 15 months post OHT who came in with left-sided facial droop and pain, abducens palsy, crusting facial rash, and ear swelling. Imaging revealed necrotizing otitis externa, with associated otitis media, petrous apicitis, and signs suggestive of encephalitis. A viral panel resulted positive for zoster infection, with superimposed methicillin-resistant staphylococcus aureus infection. Despite treatment with antibiotics and antivirals, the patient's mentation declined, with subsequent lumbar puncture revealing zoster meningoencephalitis. The patient’s mycophenolate mofetil (MMF) immunosuppression was suspended, and he was continued solely on therapeutic tacrolimus. His antiviral was switched to intravenous acyclovir and he was continued on six weeks of antibiotic therapy. The patient demonstrated resolution of his acute infection, however he continues to have residual facial and abducens nerve palsies. On discharge, the patient was resumed on MMF without any cardiac complications. Conclusions: This is the first documented case of Ramsay Hunt Syndrome, complicated by Gradenigo Syndrome and varicella-zoster virus meningoencephalitis in an OHT recipient. Our patient's immunosuppression level was the most important factor leading to the concurrence of these novel complications. Physicians should consider and assess for symptoms of these potential sequelae in all transplant patients, and carefully manage immunosuppressive agents to allow for resolution of the infection, while avoiding rejection. While residual neurologic deficits may be challenging to manage, this case demonstrated successful treatment of these complications while avoiding significant cardiac sequelae.
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