Background and Objectives: The Klippel-Feil syndrome is a congential anomaly characterized by fusion of the cervical vertebrae. It is often associated with serious congenital anomalies of the nervous, cardivascular, respiratory, and
urogenital
systems. Another anomaly often associated is hearing loss. For those cases of the Klippel-Feil syndrome accompanying hearing loss, the middle and inner ear deformities were reported in some, whereas aural atresia was extremely rare. The purpose
of
this
paper is to report the fact that Klippel-Feil syndrome can be combined with congenital aural atresia and deafness. Material & Method: Recently we experienced a case of Klippel-Feil syndrome in a 28 year old female. In this case, the second
and
third cervical vertebrae were fused. The patient also presented a short neck, cardiovascular anomaly, bilateral congenital deafness, and bilateral congenital aural atresia. Radiographical observation showed soft tissue density in the bilateral
external
auditory canals and stenosis of bilateral internal auditory canals, but no definite middle and inner ear anomalies in the temporal bone were found by computerized tomogram. Result: As operative procedures, canaloplasty and meatoplasty were
performed after cholesteatoma removal from the external auditory canal. The external auditory canal has been well maintained following the surgery. Conclusion: For patients with the Klippel-Feil syndrome, we must do full evaluation of the
whole
body in order to treat first any life threatening problems. Evaluation of hearing should then be followed to implement appropriate treatment and early rehabilitation.
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