Objective: To describe a case of insulinoma presenting with tinnitus and hearing loss. Case Report: A 57-year-old man presented with tinnitus, hearing loss and dizziness. Endogenous hyperinsulinemia was suspected because of episodic hypoglycemic symptoms associated with low plasma glucose and elevated C-peptide. In-hospital 72-hour fasting and advanced image confirmed the diagnosis and localized the lesion. Results: The positive finding of the 72-hour fasting test was consistent with endogenous hyperinsulinemia. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a pancreatic head tumor. Surgical enucleation was performed. After surgery, all the auditory symptoms resolved. Conclusions: Endogenous hyperinsulinemia should be suspected if recurrent tinnitus and hearing loss are simultaneously associated with hypoglycemic episodes.