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Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome

INTRODUCTION: Noonan syndrome (NS) is a heterogeneous genetic disease that affects many parts of the body. It was named after Dr. Jacqueline Anne Noonan, a paediatric cardiologist. CASE REPORT: We report audiological tests and auditory brainstem response (ABR) findings in a 5-year old Malay boy with...

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Autores principales: Jalaei, Bahram, Zakaria, Mohd Normani, Sidek, Dinsuhaimi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Mashhad University of Medical Sciences 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5307306/
https://www.ncbi.nlm.nih.gov/pubmed/28229064
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author Jalaei, Bahram
Zakaria, Mohd Normani
Sidek, Dinsuhaimi
author_facet Jalaei, Bahram
Zakaria, Mohd Normani
Sidek, Dinsuhaimi
author_sort Jalaei, Bahram
collection PubMed
description INTRODUCTION: Noonan syndrome (NS) is a heterogeneous genetic disease that affects many parts of the body. It was named after Dr. Jacqueline Anne Noonan, a paediatric cardiologist. CASE REPORT: We report audiological tests and auditory brainstem response (ABR) findings in a 5-year old Malay boy with NS. Despite showing the marked signs of NS, the child could only produce a few meaningful words. Audiological tests found him to have bilateral mild conductive hearing loss at low frequencies. In ABR testing, despite having good waveform morphology, the results were atypical. Absolute latency of wave V was normal but interpeak latencies of wave’s I-V, I-II, II-III were prolonged. Interestingly, interpeak latency of waves III-V was abnormally shorter. CONCLUSION: Abnormal ABR results are possibly due to abnormal anatomical condition of brainstem and might contribute to speech delay.
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spelling pubmed-53073062017-02-22 Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome Jalaei, Bahram Zakaria, Mohd Normani Sidek, Dinsuhaimi Iran J Otorhinolaryngol Case Report INTRODUCTION: Noonan syndrome (NS) is a heterogeneous genetic disease that affects many parts of the body. It was named after Dr. Jacqueline Anne Noonan, a paediatric cardiologist. CASE REPORT: We report audiological tests and auditory brainstem response (ABR) findings in a 5-year old Malay boy with NS. Despite showing the marked signs of NS, the child could only produce a few meaningful words. Audiological tests found him to have bilateral mild conductive hearing loss at low frequencies. In ABR testing, despite having good waveform morphology, the results were atypical. Absolute latency of wave V was normal but interpeak latencies of wave’s I-V, I-II, II-III were prolonged. Interestingly, interpeak latency of waves III-V was abnormally shorter. CONCLUSION: Abnormal ABR results are possibly due to abnormal anatomical condition of brainstem and might contribute to speech delay. Mashhad University of Medical Sciences 2017-01 /pmc/articles/PMC5307306/ /pubmed/28229064 Text en This is an Open Access article distributed under the terms of the Creative Commons Attribution License, (http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Jalaei, Bahram
Zakaria, Mohd Normani
Sidek, Dinsuhaimi
Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome
title Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome
title_full Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome
title_fullStr Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome
title_full_unstemmed Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome
title_short Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome
title_sort abnormal auditory brainstem response (abr) findings in a near-normal hearing child with noonan syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5307306/
https://www.ncbi.nlm.nih.gov/pubmed/28229064
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