Klippel-Feil syndrome and associated ear deformities

The Klippel-Feil syndrome is characterized by a short, thick neck with restricted head mobility and a low occipital hairline. The head appears to rest directly on the thorax and the trapezius muscles flare out on either side of the neck, producing a pterygium-like effect. Cervical roentgenograms demonstrate fusion of some or even all of the vertebral bodies into bony blocks. Ear deformities occur in approximately one-third of the Klippel-Feil patient population. We report five cases of Klippel-Feil syndrome and describe their otologic and roentgenographic features. Although no single characteristic otologic or audiologic problem is identified, a proclivity for major malformations of involved ears is demonstrated. Early audiometric and otologic evaluation is indicated when the diagnosis of Klippel-Feil syndrome is made.