Ear Deformities Associated with the Klippel-Feil Syndrome

Abstract

Congenital spinal fusion, constituting the Klippel-Feil anomaly, has long been known to be associated with severe deafness in many patients. Ten such cases are described, with audiometric and tomographic assessment of the ear lesions. Of the 20 ears examined, 12 showed evidence of severe hearing loss and of these, 11 had evidence of significant inner ear dysplasia on tomography. Middle and external ear abnormalities were also demonstrated. The significance of these findings and the relation to other syndromes is discussed.