Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening catastrophic and rarely seen complication of EBV infection especially in adults. While typical presentation of EBV infection is easily diagnosed as mononucleosis syndrome in teenagers and adults, some atypical clinical presentations may be challenged. We did not encounter any patient presenting with sudden sensorineural hearing loss associated with EBV infection in our English medical literature research (1966-2016). In this study, we report an adult patient who was complicated with EBV-HLH under high dose steroid therapy after diagnosis as sensorineural hearing loss. Our aim is to emphasise the atypical presentation of EBV infection and to discuss steroid therapy complication in sensorineural hearing loss that had been simply defined as idiopathic.

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