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Titolo:
Mondini dysplasia without functional impairment in a case of CHARGE association.
Autore:
Acham, A; Walch, C;
Indirizzi:
Graz Univ, HNO Klin, Klin Abt Neurootol, A-8036 Graz, Austria Graz Univ Graz Austria A-8036 , Klin Abt Neurootol, A-8036 Graz, Austria
Titolo Testata:
LARYNGO-RHINO-OTOLOGIE
fascicolo: 7, volume: 80, anno: 2001,
pagine: 381 - 384
SICI:
1615-0007(200107)80:7<381:MDWFII>2.0.ZU;2-O
Fonte:
ISI
Lingua:
GER
Soggetto:
PENDREDS SYNDROME; INNER-EAR; MALFORMATIONS;
Keywords:
CHARGE association; Mondini dysplasia; cochlear malformation; hearing loss;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
14
Recensione:
Indirizzi per estratti:
Indirizzo: Acham, A Graz Univ, HNO Klin, Klin Abt Neurootol, Auenbruggerpl 26-28, A-8036 Graz,Austria Graz Univ Auenbruggerpl 26-28 Graz Austria A-8036 6 Graz,Austria
Citazione:
A. Acham e C. Walch, "Mondini dysplasia without functional impairment in a case of CHARGE association.", LARY RH OTO, 80(7), 2001, pp. 381-384

Abstract

A male child with fully developed CHARGE association was referred to our hospital several times because of choanal atresia and suspected hearing lossin his first two years of life. As far as they are a main symptom malformations of the ear appear frequently in CHARGE association. This report describes that an obvious anatomic dysplasia of the inner ear can have normal function, which is shown by objective measurement. Methods and Results: Computed tomography of the petrous bone showed bilateral Mondini malformation ofthe cochlea combined with bulky deformation of middle ear ossicles. By theage of one year brainstem evolved response audiometry (BERA) was performed. The result demonstrated a hearing loss of 60 dB on the right ear, but no measurable hearing loss on the left ear. Conclusion: This case report proves that a Mondini-type malformation of the cochlea does not need to be combined with hearing loss.

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Documento generato il 09/12/19 alle ore 11:44:18