期刊论文详细信息
Revista Brasileira de Otorrinolaringologia
Audiology profile in patients with Goldenhar's syndrome
Brosco, Karina Costa1  Universidade de São Paulo, Bauru1  Zorzetto, Neivo Luiz1  UNESP, Marilia1  Costa, Antonio Richieri da1 
关键词: Goldenhar's syndrome;    oculoauriculovertebral dysplasia;    audiology.;   
DOI  :  10.1590/S0034-72992004000500011
学科分类:医学(综合)
来源: Associacao Brasileira de Otorrinolaringologia e Cirurgia Cervicofacial
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【 摘 要 】

The Goldenhar's syndrome is a rare congenital anomaly, of which the etiology is yet unknown, and characterized by a classical triad of ocular, auricular and vertebral abnormalities.FORMA DE ESTUDO: Serie report.MATERIAL AND METHOD: This study used 30 individuals presenting Goldenhar's syndrome, that were regularly enrolled in the Craniofacial Anomaly Rehabilitation Hospital (HRAC), of both sexes, varying from 8 to 34 years old.AIM: The goal was to characterize the audiologic profile to the individuals presenting this syndrome, thus, assuring a better treatment and orientation for them, and also the establishment of the frequency of contra-lateral auditory commitment in individual with the classical unilateral involvement.RESULTS: The auditory assessment of this study showed ATL, tympanometry, EOA-T and BERA. According to the results, we concluded that 35% (N=10) of the individuals presented, as a characteristic of the audiologic profile, loss of hearing type sensorioneural, mixed with varied levels from moderate to deep (7 uni and 3 bilateral); 13% (n=4) presented conductive loss (bilaterally) with levels mild to severe and 3% (n=1) presented a unilateral deep type of sensorioneural loss. This study showed 40% n=12) with normal bilateral hearing and in 10% (n=3) it wasn't possible to establish the characteristic of the audiologic profile, since only the BERA was used as an assessment tool for establishing the electro-physiologic threshold. Of the twelve (12) individuals presenting unilateral malformations of the left ear, only two (02) presented contra-lateral auditory commitment of the ear, one type mixed with a severe level and one conductive of a moderate level. Considering the sex variable, the mayor predominance of the Goldenhar's syndrome was for females (57%) compared to the males (43%), but these results weren't statistically significant, as well as for the unaffected anatomical side, that was predominantly the right side.

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