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Titolo:
MRI of the cerebellopontine angle in patients with cleido-cranial dysostosis
Autore:
Scherer, A; Engelbrecht, V; Nawatny, J; Messing-Junger, M; Reifenberger, G; Modder, U;
Indirizzi:
Univ Dusseldorf, Inst Diagnost Radiol, D-40225 Dusseldorf, Germany Univ Dusseldorf Dusseldorf Germany D-40225 , D-40225 Dusseldorf, Germany Univ Dusseldorf, Neurochirurg Klin, D-4000 Dusseldorf, Germany Univ Dusseldorf Dusseldorf Germany D-4000 in, D-4000 Dusseldorf, Germany Univ Dusseldorf, Inst Neuropathol, D-4000 Dusseldorf, Germany Univ Dusseldorf Dusseldorf Germany D-4000 ol, D-4000 Dusseldorf, Germany
Titolo Testata:
ROFO-FORTSCHRITTE AUF DEM GEBIET DER RONTGENSTRAHLEN UND DER BILDGEBENDEN VERFAHREN
fascicolo: 4, volume: 173, anno: 2001,
pagine: 315 - 318
SICI:
1438-9029(200104)173:4<315:MOTCAI>2.0.ZU;2-R
Fonte:
ISI
Lingua:
GER
Soggetto:
CLEIDOCRANIAL DYSPLASIA; INNER-EAR; CT; ABNORMALITIES;
Keywords:
cleidocranial dysostosis; Scheuthauer-Marie-Sainton syndrome schwannoma; hearing loss; magnetic resonance imaging;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
15
Recensione:
Indirizzi per estratti:
Indirizzo: Scherer, A Univ Dusseldorf, Inst Diagnost Radiol, Moorenstr 5, D-40225 Dusseldorf, Germany Univ Dusseldorf Moorenstr 5 Dusseldorf Germany D-40225 Germany
Citazione:
A. Scherer et al., "MRI of the cerebellopontine angle in patients with cleido-cranial dysostosis", ROFO-F RONT, 173(4), 2001, pp. 315-318

Abstract

MRI of the cerebellopontine angle in patients with cleidocranial dysostosis. Purpose: Cleidocranial dysostosis (CCD) is an autosomal dominant bone disorder in which deafness is common secondary to malformation of the middle ear structures. The study aimed at MRI evaluation of the cerebellopontine angle in 7 patients with a history of CCD - two generation spanned relatives. Material and methods: Cranial MRI in 7 patients with CCD (4 women/3 men aged between 8 and 46 years) was performed. In two patients hearing disorders were present. The examinations encompassed multi-planar spin-echo sequences of the cerebellopontine angle in 3-mm slice thickness before and after administration of contrast medium. Results: The clinically most conspicuous female patient (hearing loss, ataxia, headache) showed a strongly contrast-enhancing tumor in MRI that was histologically proved to be an acoustic schwannoma. Concerning the other family members, no pathological findings werenoted except for non-pneumatized mastoids. Conclusion: The first report ofa patient with CCD and an acoustic schwannoma shows that in case of hearing loss in these patients also a retrocochlear cause must be considered.

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Documento generato il 20/02/20 alle ore 03:23:48