Audiological findings in children with ataxia-telangiectasia (A-T) syndrome

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• 作者：Pretty O. Afifi^a ; ^{pretty_afifi@hotmail.com} ; Hanaa Hussein Elsanadiky^b
• 关键词：Ataxia-telangiectasia ; Audiological assessment ; ABR findings
• 刊名：International Journal of Pediatric Otorhinolaryngology
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：92
• 期：Complete
• 页码：94-98
• 全文大小：740 K
• 卷排序：92

文摘

To assess peripheral and central hearing in children with A-T.Method3 children diagnosed with A-T according to the diagnostic criteria for A-T of the European Society for Immunodeficiencies. Involuntary movements were seen in the form of chorea-athetosis together with tremors. They were examined to assess both peripheral and central hearing was assessed (hearing thresholds). Sound-field testing, tympanometry, acoustic reflexes, Otoacoustic Emissions (OAEs) and Auditory Brainstem Responses (ABR) were done for all of them.ResultsBasic Audiological evaluation is of a limited value as the children are not co-operative. Sound field testing could not be done. Bilateral normal middle ear functions as reflected by Tympanometry and Acoustic Reflexes. Advanced Audiological evaluation including OAEs and ABR are more valuable in assessing hearing in children with A-T. Bilateral pass response at all test frequencies in DPOAEs. Abnormal ABR findings were obtained in the form of a delay in wave V latency more than 2 SD with subsequent increased in I-V interpeak latency with no significant interaural latency difference.ConclusionConsistent with bilateral normal peripheral hearing sensitivity with central hearing affection.LimitationsThe rarity of the disease, make it difficult to be applied on many cases.