A Rare Case of Klippel-Feil Syndrome with Cholesteatoma, Thrice Operated with Final Outcome of Cochlear Implant

Abstract

Klippel-Feil syndrome (KF) is defined as the congenital fusion of two or more vertebrae. Due to skeletal abnormalities, patients with KF syndrome may potentially present with hearing loss. To the best of our knowledge, this is a rare case of an adolescent patient with underlying KF syndrome and cholesteatoma. Our patient underwent three successive mastoid operations, primarily due to middle ear disease, with cochlear implantation for hearing rehabilitation.