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Temporal bone CT findings in Cornelia de Lange syndrome.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Kim, J. H. Kim, Eun Young Lee, June Seung Lee, Wang Soo Kim, Ha Neul |
| Copyright Year | 2008 |
| Abstract | BACKGROUND AND PURPOSE Cornelia de Lange syndrome is a rare developmental malformation syndrome with a high prevalence of hearing impairment. The purposes of this study were to describe the characteristic temporal bone CT findings in patients with Cornelia de Lange syndrome and to correlate audiometric data with radiologic findings in these patients. MATERIALS AND METHODS Ten children (6 girls and 4 boys; mean age, 42.0 months) who were clinically diagnosed with Cornelia de Lange syndrome (classic, n = 5; mild form, n = 5) were enrolled. Temporal bone CT was prospectively performed, and 32 aspects of each temporal bone CT were analyzed, 21 by direct measurement and 11 by visual inspection. Twenty age-matched children (n = 20 ears) with normal temporal bone CT scans served as a control group. Audiologic tests were also performed on all patients. RESULTS Characteristic temporal bone CT findings of Cornelia de Lange syndrome were external auditory canal stenosis, soft-tissue opacification of the hypoplastic tympanomastoid cavity, dysmorphic ossicle, hypoplastic cochlea, and dysplastic vestibule, all of which were more prevalent in patients with the classic form of the disease than in those with the mild form. Children who had more severe structural abnormalities on temporal bone CT had worse hearing levels compared with those without structural abnormalities. CONCLUSION Temporal bone CT scans in Cornelia de Lange syndrome could document combined structural abnormalities of the external, middle, and inner ear, which were one of the characteristic clinical manifestations; CT abnormalities were well correlated with the audiometric data. |
| Starting Page | 46 |
| Ending Page | 46 |
| Page Count | 1 |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://www.ajnr.org/content/ajnr/29/3/569.full.pdf |
| Alternate Webpage(s) | http://www.ajnr.org/content/ajnr/early/2007/12/07/ajnr.A0888.full.pdf |
| PubMed reference number | 18065514v1 |
| Volume Number | 29 |
| Issue Number | 3 |
| Journal | AJNR. American journal of neuroradiology |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | Body cavities Bone Cysts Cochlear structure Conductive hearing loss Congenital Abnormality Congenital hypoplastic anemia Cornelia De Lange Syndrome Ear Diseases Ear ossicle structure Ear structure External auditory canal structure Gilles de la Tourette syndrome Histopathology Mastoid process Obstructive Ureterocele Patients Stenosis of external auditory canal Temporal bone structure Tomography, Spiral Computed Tooth Abnormalities Vestibular Labyrinth X-Ray Computed Tomography abnormal cellular structure or growth hearing impairment |
| Content Type | Text |
| Resource Type | Article |
