Stapes footplate deformity leading to perilymphatic fistula and recurrent meningitis

Abstract

We present a case of a 23-month old female with congenital malformation of her inner ear and perilymphatic fistula due to stapes footplate defect leading to recurrent episodes of bacterial meningitis and resulting bilateral profound hearing loss. Multiple-layer closure with obliteration of the malformation, middle ear and mastoid along with closure of the external auditory canal was performed to achieve definitive repair. Cochlear implantation in the contralateral ear resulted in age-appropriate language proficiency with excellent CI speech perception over a 12-year follow up. The importance of multiple-layer closure for adequate repair and early consideration of cochlear implantation are highlighted.