Temporal Bone Findings in Klippel-Fell Syndrome

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Klippel-Feil syndrome which is characterized by a fusion of two or more cervical vertebrae and manifested by the triad of short neck, limitation of movement of the head and low posterior hairline, is often associated with other congenital anomalies. Other abnormalities associated with Klippel-Feil syndrome are found in the musculoskeletal, nervous, gastrointestinal, urogenital, cardiovascular and respiratory systems and in the ears. Deafness is the second most common associated abnormality and has been estimated to occur in up to 30% of patients with Klippel-Feil syndrome. There has been only one temporal bone study in this syndrome.Temporal bone findings in one patient with the Klippel-Feil syndrome are presented here, A histological study revealed thickened mucous membranes in the middle ear with otitis media, confluence between the right saccule and utricle and numerous anomalies in the left ear aural atresia, filled middle ear cavity, hypoplasia and maldevelopment of the ossicles, abnormal facial nerve, large bony semicircular canals, and shortened cochlea.
耳鼻咽喉科臨床学会の論文