The Klippel-Feil syndrome is a congenital anomaly characterized by the fusion of the cervical vertebrae with malformation of the atlas and axis. Radiologic studies have reported a fusion or confluency of some or all the cervical vertebrae into
one
homogeneous bony mass, named block vertebra.
Deafness is the second most common finding and has been esti-mated up to 30% of patients with Klippel-Feil syndrome.
Recently we experienced a case of Klippel-Feil syndrome having a imperforate anus, congenital heart disease, torticollis, short neck, and anomalies of vertebrae in a 2-years old boy with congenital deafness.
We present the patient's hearing with the auditory brainstem response(ABR), and the anomalies of cervical vertebrae with 3-dimensional CT. (Korean J Otolaryngol 39 : 3, 1996)
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