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


Online ISSN : 2347 - 3215
Issues : 12 per year
Publisher : Excellent Publishers
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Evaluation of Developmental and Hearing disorders in Phenylketonuric patients
Yalda Jabbari Moghadam1, Siamak Shiva2, A. Mehrnoush Mousavi1, Younes Ebrahimzadeh1* and Hamidreza Mahboobi3
1Pediatric Health Research Center, Department of Otolaryngology, Head and Neck Surgery, Faculty of Medicine, Tabriz University of Medical Sciences, Iran 2Pediatric Health Research Center, Department of Pediatrics, Faculty of Medicine, Tabriz University of Medical Sciences, Iran 3Department of Internal Medicine, Faculty of Medicine, Bandar-e Abbas University of Medical Sciences, Iran
*Corresponding author
Abstract:

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.

Keywords: Phenylketoneuria, Hearing loss, ABR
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How to cite this article:

Yalda Jabbari Moghadam, Siamak Shiva, A. Mehrnoush Mousavi, Younes Ebrahimzadeh and Hamidreza Mahboobi. 2016. Evaluation of Developmental and Hearing disorders in Phenylketonuric patients.Int.J.Curr.Res.Aca.Rev. 4(8): 222-226
doi: http://dx.doi.org/10.20546/ijcrar.2016.408.019