MELAS Syndrome is a mitochondrial cytopathy characterized by mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes. Sensorineural hearing loss is a frequent symptom, occurring in 44-75% of patients. The document discusses the role of otologists in diagnosing and managing MELAS, including screening all patients for hearing loss and following up regularly as hearing loss is a cardinal feature and patients require multidisciplinary treatment and guidance.