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Pediatr Radiol. 2006 Mar;36(3):241-3. Epub 2005 Nov 18.

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

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Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario M5G 1X8, Canada.


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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