Abstract            Volume:4  Issue-8  Year-2016         Original Research Articles

Online ISSN : 2347 - 3215 Issues : 12 per year Publisher : Excellent Publishers Email : editorijcret@gmail.com

Phenylketoneuria (PKU) is a metabolic disease caused by a mutation in the gene that codes the phenylalanine hydroxylase enzyme, which is responsible for transforming the amino acid phenylalanine into tyrosine. Elevated levels of phenylalanine in the blood results in a toxic effect with mental retardation being the most significant manifestation of this disease. The aim of this study is to evaluate the audiometry and auditory brainstem responses (ABRs) to determine the effects of treatment on the neurologic evolution of the auditory pathway in patients with PKU. In a descriptive cross-sectional study, a group consisting of 50 children with PKU was compared with 50 healthy children, in terms of the Audiometry, ABRs and their affecting factors. In this study, results of ABRs in the patient group compared to normal values confirms the relative delay )though generally in the normal range( in the intervals between the waves I-III, III-V and I-V and developmental delays and mental retardation and seizures were more prevalent in Phenylketoneuria patients. The results of this study showed that verbal and developmental disorders may be affected by undiagnosed hearing loss, thus, we recommend that all infants, especially Phenylketoneuria patients should be evaluate for hearing and speech disorders.

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