Klippel-Feil syndrome and associated ear deformities

R. T. Miyamoto, H. Y. Yune, W. H. Rosevear

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Abstract

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 described 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.

Original languageEnglish (US)
Pages (from-to)113-119
Number of pages7
JournalAmerican Journal of Otology
Volume5
Issue number2
StatePublished - Dec 1 1983

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ASJC Scopus subject areas

  • Otorhinolaryngology

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