Perfil audiológico de indivíduos portadores da síndrome de Goldenhar
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Data
2004-10-01
Autores
Brosco, Karina Costa
Zorzetto, Neivo Luiz [UNESP]
Costa, Antonio Richieri da
Título da Revista
ISSN da Revista
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Editor
ABORL-CCF Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial
Resumo
A síndrome de Goldenhar é uma anomalia congênita rara, de etiologia ainda desconhecida e caracterizada por uma tríade clássica de alterações oculares, auriculares e vertebrais. FORMA DE ESTUDO: Estudo de série. MATERIAL E MÉTODO: Este trabalho foi realizado com 30 indivíduos portadores da síndrome de Goldenhar regularmente matriculados no HRAC, de ambos os gêneros, com faixa etária variando de 8 a 34 anos de idade. OBJETIVO: foi caracterizar o perfil audiológico dos indivíduos portadores dessa síndrome, garantindo assim um melhor tratamento e orientação para os mesmos, assim como também estabelecer a freqüência do comprometimento auditivo contralateral nos indivíduos com o clássico envolvimento unilateral. A avaliação audiológica do estudo constou de ATL, timpanometria, EOA-T e BERA. RESULTADO: de acordo com os resultados concluímos que 34% (=10) dos indivíduos apresentaram como característica de seu perfil audiológico perda auditiva do tipo sensório-neural mista com grau variando de moderado a profundo (7 uni e 3 bilaterais); 13% (n=4) apresentaram perda do tipo condutiva (bilateralmente) com grau de leve a severo e 3% (n=1) apresentaram ou perda do tipo sensório-neural profundo unilateral. Encontramos 40% (n=12) com audição normal bilateralmente e em 10% (n=3) não foi possível estabelecer a característica do perfil audiológico por utilizarmos como avaliação apenas o BERA como pesquisa de limiar eletrofisiológico. Dos doze (12) indivíduos com malformação de OE unilateral encontramos apenas dois (02) com comprometimento auditivo na orelha contralateral, sendo um do tipo misto de grau severo e um condutivo de grau moderado. Com relação a variável sexo encontramos predominância maior da síndrome de Goldenhar no gênero feminino (57%) do que no masculino (43%), mas considerado estatisticamente sem significância, assim como também o lado anatomicamente afetado, que foi predominantemente o direito.
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.
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.
Descrição
Palavras-chave
Goldenhar, síndrome de Goldenhar, displasia óculo-aurículo-vertebral, audiologia, Goldenhar's syndrome, oculoauriculovertebral dysplasia, audiology
Como citar
Revista Brasileira de Otorrinolaringologia. ABORL-CCF Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial, v. 70, n. 5, p. 645-649, 2004.