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Titolo:
Goldenhar's syndrome: Congenital hearing deficit of conductive or sensorineural origin? Temporal bone histopathologic study
Autore:
Scholtz, AW; Fish, JH; Kammen-Jolly, K; Ichiki, H; Hussl, B; Kreczy, A; Schrott-Fischer, A;
Indirizzi:
Innsbruck Univ, Dept Otolaryngol, A-6020 Innsbruck, Austria Innsbruck Univ Innsbruck Austria A-6020 yngol, A-6020 Innsbruck, Austria Innsbruck Univ, Dept Anat Pathol, A-6020 Innsbruck, Austria Innsbruck Univ Innsbruck Austria A-6020 athol, A-6020 Innsbruck, Austria
Titolo Testata:
OTOLOGY & NEUROTOLOGY
fascicolo: 4, volume: 22, anno: 2001,
pagine: 501 - 505
SICI:
1531-7129(200107)22:4<501:GSCHDO>2.0.ZU;2-A
Fonte:
ISI
Lingua:
ENG
Soggetto:
AURICULO-VERTEBRAL SPECTRUM; CHARGE ASSOCIATION; MONOZYGOTIC TWINS; ABNORMALITIES; MALFORMATIONS; MICROSOMIA; DYSPLASIA; DEAFNESS;
Keywords:
human; Goldenhar's syndrome; inner ear; middle ear;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
35
Recensione:
Indirizzi per estratti:
Indirizzo: Scholtz, AW Innsbruck Univ, Dept Otorhinolaryngol, Anichstr 35, A-6020 Innsbruck, Austria Innsbruck Univ Anichstr 35 Innsbruck Austria A-6020 , Austria
Citazione:
A.W. Scholtz et al., "Goldenhar's syndrome: Congenital hearing deficit of conductive or sensorineural origin? Temporal bone histopathologic study", OTOL NEURO, 22(4), 2001, pp. 501-505

Abstract

Background: Oculoauriculovertebral dysplasia (OAVD) (Goldenhar's syndrome)is a congenital syndrome with ipsilateral deformity of the ear and face, epibulbar lipodermoids, coloboma, and vertebral anomalies. Goldenhar's anomaly has often been associated with a degree of congenital hearing deficits, almost always of a conductive origin, but a sensorineural component is alsosuspected in some cases, evident through malformations of the inner ear. Patients and Methods: Both temporal bones of a 10-day-old deceased patientwith oculoauriculovertebral dysplasia were examined by light microscopy. Results: The ear deformities included deformity of the auricle, atresia ofthe external auditory canal, and malformation of the tympanic cavity and ossicles. Abnormalities of the stria vascularis and the semicircular canals were also demonstrated. Further inner ear deformities were not identified in this case. Conclusion: These histopathologic findings appear to confirm the conductive component of the congenital hearing deficit, but a sensorineural component could not be omitted. The ear alterations favor early developmental fielddefects. The causes of this condition are controversial. Recent results ingenetic research pertaining to the MSX class genes permit better understanding of the variety, variability, and different degrees of severity of the anomalies described here.

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Documento generato il 10/12/19 alle ore 01:25:46