Evaluation of hearing in the Stickler s Syndrome and association with risk factors of hearing loss in infancy / Avaliação da audição na Síndrome de Stickler e associação com fatores de risco de perda auditiva na infância

AUTOR(ES)
DATA DE PUBLICAÇÃO

2006

RESUMO

Hearing loss is an important characteristic of the Stickler’s Syndrome. In literature, few studies have defined the characterization of this loss, and there are no studies about the effect of other risk factors of hearing in Sticker. This present work has been made with the purpose of verifying the clinical and audiological phenotype of a group of 26 children with average age of 5 years and 7 months, affected by SS, at the Hospital de Reabilitação de Anomalias Craniofaciais. After analyzing the data of the clinical and audiological exams, we concluded that 80.76% (IC 95%, 60,65 – 93,44) of the children have presented hearing loss, being 34.61% (IC 95%, 17,21 – 55,66) of sensorial neural loss, associated with SS, typically for high frequencies; 11.53% (IC 95%, 2,44 – 30,15) of mixed loss; and 34.61% (IC 95%, 17,21 – 55,66) of conductive hearing loss, which is common in children with SS, due to the presence of cleft palate and other craniofacial anomalies, leading to a disfunction of the mid ear, with harming of hearing sensibility. The degree of hearing loss has varied from mild to severe. The statistical analysis through Fisher’s test (p<0.05) didn’t reveal any significant effect of the exposition of children to other risk factors of hearing loss to the sensorial neural loss of SS. Due to the impossibility of the realization of the molecular genetic exam, the clinical phenotype, associated with the audiological evaluation, may suggest the diagnosis of the kind of SS.

ASSUNTO(S)

fatores de risco de perda auditiva stickler’s syndrome audiological evaluation fenótipo clínico avaliação audiológica clinical phenotype risk factors of hearing loss síndrome de stickler

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