Klippel-Feil Syndrome and Associated Hearing Loss

Klippel-Feil syndrome is a clinical triad consisting of short neck, decreased mobility of head movement, and low occipital hairline. Manifestation of these characteristics varies widely. Prevalence and etiologic basis of the cervical deformity are uncertain. Various musculoskeletal anomalies and defects of other organ systems, including hearing loss, have been reported in association with the syndrome. Four cases of children with the Klippel-Feil syndrome with confirmed hearing impairments show that no single audiologic problem appears to be characteristic of the syndrome. It is recommended that audiologic and otologic information be obtained as part of the total diagnostic work-up.