Petrified ears in a patient with Keutel syndrome: temporal bone CT findings

H Parmar, S Blaser, S Unger, SJ Yoo, B Papsin - Pediatric radiology, 2006 - Springer
H Parmar, S Blaser, S Unger, SJ Yoo, B Papsin
Pediatric radiology, 2006Springer
We present unusual imaging findings of petrified ears in a 9-year-old girl with Keutel
syndrome. The patient presented for a temporal bone study for hearing loss. CT scan
showed middle and inner ear abnormalities along with extensive and unsuspected
calcification of the external ears and ossicular ligaments. On further investigation, the patient
was found to have diffuse cartilage calcification in the larynx and tracheobronchial tree,
brachytelephalangism and peripheral pulmonary stenosis suggestive of Keutel syndrome …
Abstract
We present unusual imaging findings of petrified ears in a 9-year-old girl with Keutel syndrome. The patient presented for a temporal bone study for hearing loss. CT scan showed middle and inner ear abnormalities along with extensive and unsuspected calcification of the external ears and ossicular ligaments. On further investigation, the patient was found to have diffuse cartilage calcification in the larynx and tracheobronchial tree, brachytelephalangism and peripheral pulmonary stenosis suggestive of Keutel syndrome. Confirmation was obtained by mutation analysis.
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