Klippel-Feil syndrome is characterized by a short-neck and a low occip
ital hairline due to such deformaties of the cervical spine as aplasia
, dysplasia and fusions of the cervical and thoracic vertebrae. Approx
imately 1/3, of cases have sensorineural deafness, although occasional
unilateral or bilateral conductive hearing loss due to middle ear mal
formations have been described. We have now treated two cases with uni
lateral middle ear malformations in patients with known Klippel-Feil s
yndrome. In the first case a completely malformed stapes was found as
well as an atypical course of the facial nerve, which was found to be
exactly over an absent oval window. In the second case aplasia of the
stapes was seen and the oval and round windows were absent. No epitymp
anum was identifiable and a compact mastoid was found. In addition to
audiometry, high-resolution CT was indispensable in diagnosis, However
, surgical procedures in cases with unilateral middle ear deformations
should be performed only on explicit request of the patient and not b
efore the age of 16.