Klippel-Feil syndrome with congenital conductive deafness: report of a case and review of literature.

Klippel-Feil syndrome is a clinical triad consisting of short neck, decreased head mobility, and low occipital hairline. Additional deformities of the musculoskeletal and the neural system may also be present. Otological defects occur in about one third of these patients. They are seen either unilaterally or bilaterally and accompanied by deafness which may be perceptive, or mixed, or rarely conductive in type. They are caused either by dysplasia of the labyrinth and/or internal auditory canal, or by the middle and the outer ear. We present a report of a case of Klippel-Feil syndrome with congenital conductive deafness. Detailed radiological investigations failed to show any malformations of the middle ear. At operations, thick glue was removed from the middle ear cavities, and dehiscent fallopian canals and bulging of the facial nerve in the tympanic segment were seen. One year postoperatively, audiometry showed a hearing gain of only 20 dB on both sides. Review of the literature shows that there is a trend among the contemporary otologic surgeons to undertake curative surgery whenever possible, so as to improve the hearing, despite isolated reports of various difficulties and complications of curative surgery.