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Crian as com fenilcetonúria: avalia o audiológica básica e supress o das otoemiss es Children with phenylketonuria: basic audiological evaluation and suppression of otoacoustic emissions

DOI: 10.1590/s1516-80342012000300003

Keywords: Audi o , Fenilcetonúrias , Células ciliadas auditivas , Transtornos da audi o , Emiss es otoacústicas espontaneas , Hearing , Phenylketonurias , Hair cells , auditory , Hearing disorders , Otoacoustic emissions , spontaneous

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Abstract:

OBJETIVO: Avaliar a via auditiva de crian as com fenilcetonúria tratadas precocemente, por meio de audiometria, imitanciometria e supress o das emiss es otoacústicas transientes. MéTODOS:Estudo prospectivo transversal comparativo com amostra composta por 28 crian as, sendo 12 com fenilcetonúria e 16 sem a doen a. Foi realizada a pesquisa dos limiares de audibilidade por via aérea e óssea, logoaudiometria, imitanciometria e supress o das emiss es otoacústicas transientes. RESULTADOS: A audiometria e a logoaudiometria estiveram normais em todos os participantes. Foram encontrados piores resultados para o índice de reconhecimento de fala (IRF) no grupo com fenilcetonúria. A imitanciometria revelou curva normal para todas as crian as, mas a pesquisa dos reflexos estapedianos demonstrou que as crian as do grupo com fenilcetonúria apresentaram aumento nos seus limiares nas frequências de 2 e 4 kHz. A supress o das emiss es otoacústicas transientes n o revelou diferen a na compara o entre os grupos. CONCLUS O: A avalia o audiológica básica n o identifica altera es na audi o das crian as com fenilcetonúria, mas há pior discrimina o ao IRF e aumento nos limiares de reflexos estapedianos nessas crian as, podendo indicar distúrbios do processamento auditivo. O estudo da supress o das otoemiss es demonstra integridade do sistema eferente olivococlear medial nas crian as com fenilcetonúria. PURPOSE: To evaluate the auditory pathways of children with early-treated phenylketonuria through audiometry, immitance tests, and suppression of transient otoacoustic emissions. METHODS: Prospective cross-sectional study with sample composed by 28 children: 12 with phenylketonuria and 16 without the disease. Participants underwent auditory evaluations composed of air- and bone-conduction pure-tone audiometry, speech audiometry, immittance tests and suppression of transient otoacoustic emissions. RESULTS: All participants presented normal results in pure-tone and speech audiometry; however, speech discrimination scores were lower on the phenylketonuria group. Immitance tests revealed normal tympanograms for all children, but stapedial reflex thresholds demonstrated higher thresholds in 2 and 4 kHz for children with phenylketonuria. The suppresion of transient otoacoustic emissions did not show difference in the comparison between groups. CONCLUSION: The basic audiologic assessment do not identify hearing disorders in children with phenylketonuria; however, speech discrimination scores were lower and stapedial reflexes were higher in these children, which may indicate auditor

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