Petrified ears in a patient with Keutel syndrome: temporal bone CT findings.
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.
Parmar
Hemant
H
Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario M5G 1X8, Canada. parurad@hotmail.com
Blaser
Susan
S
Unger
Sheila
S
Yoo
Shi-Joon
SJ
Papsin
Blake
B
D000015:Abnormalities, Multiple; D001842:Bone and Bones / Q000002:abnormalities; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000473:pathology*; D002648:Child; D003241:Consanguinity; D004431:Ear, External / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D006314:Hearing Loss, Conductive / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D013577:Syndrome; D013701:Temporal Bone / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed