Acute coalescent mastoiditis and acoustic sequelae in an infant with severe congenital neutropenia.

We report a 2-month-old boy with severe congenital neutropenia (SCN), who developed acute necrotizing otitis media and coalescent mastoiditis due to methicillin-sensitive Staphylococcus aureus. The infection fulminantly progressed within a day to a subtotal perforation of the tympanic membrane, destructive bony changes of ossicles, lateral subperiosteal abscess, and suppurative labyrinthitis. Despite the combined treatment with intravenous antibiotics and granulocyte colony-stimulating factor, the infection resulted in mixed hearing impairment. Much attention should be given to prompt diagnosis of otomastoiditis in SCN, a rare congenital disorder, since resultant acoustic sequelae may affect subsequent speech development and intellectual ability.