{"title":"PLA2G6 相关神经变性患儿的听力发现。","authors":"Valiyaparambath Purushothaman Vandana, Jeevendra Kumar Darshini, Bindu Parayil Sankaran","doi":"10.1055/a-1877-2546","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong> Audiological manifestations of patients with PLA2G6-associated neurodegeneration are limited.</p><p><strong>Objective: </strong> To analyze the audiological findings in a cohort of 13 children with infantile neuroaxonal dystrophy (INAD).</p><p><strong>Method: </strong> Patients underwent a battery of audiological tests including tympanometry, distortion product otoacoustic emissions, impedance audiometry, and Brainstem Auditory Evoked Potentials (BAEPs).</p><p><strong>Results: </strong> Audiological studies of 13 children indicated sensorineural hearing loss in six children and auditory neuropathy spectrum disorder in four children.</p><p><strong>Conclusion: </strong> This study may extend the auditory findings for INAD. Additional studies on quality of life and cognitive-brain degeneration related to this disease are required before making appropriate recommendations for aural rehabilitation.</p>","PeriodicalId":50021,"journal":{"name":"Journal of the American Academy of Audiology","volume":null,"pages":null},"PeriodicalIF":1.0000,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Audiological Findings in Children with PLA2G6-Associated Neurodegeneration.\",\"authors\":\"Valiyaparambath Purushothaman Vandana, Jeevendra Kumar Darshini, Bindu Parayil Sankaran\",\"doi\":\"10.1055/a-1877-2546\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong> Audiological manifestations of patients with PLA2G6-associated neurodegeneration are limited.</p><p><strong>Objective: </strong> To analyze the audiological findings in a cohort of 13 children with infantile neuroaxonal dystrophy (INAD).</p><p><strong>Method: </strong> Patients underwent a battery of audiological tests including tympanometry, distortion product otoacoustic emissions, impedance audiometry, and Brainstem Auditory Evoked Potentials (BAEPs).</p><p><strong>Results: </strong> Audiological studies of 13 children indicated sensorineural hearing loss in six children and auditory neuropathy spectrum disorder in four children.</p><p><strong>Conclusion: </strong> This study may extend the auditory findings for INAD. Additional studies on quality of life and cognitive-brain degeneration related to this disease are required before making appropriate recommendations for aural rehabilitation.</p>\",\"PeriodicalId\":50021,\"journal\":{\"name\":\"Journal of the American Academy of Audiology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.0000,\"publicationDate\":\"2022-06-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of the American Academy of Audiology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1055/a-1877-2546\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2022/6/15 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q3\",\"JCRName\":\"AUDIOLOGY & SPEECH-LANGUAGE PATHOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the American Academy of Audiology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1055/a-1877-2546","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2022/6/15 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"AUDIOLOGY & SPEECH-LANGUAGE PATHOLOGY","Score":null,"Total":0}
Audiological Findings in Children with PLA2G6-Associated Neurodegeneration.
Background: Audiological manifestations of patients with PLA2G6-associated neurodegeneration are limited.
Objective: To analyze the audiological findings in a cohort of 13 children with infantile neuroaxonal dystrophy (INAD).
Method: Patients underwent a battery of audiological tests including tympanometry, distortion product otoacoustic emissions, impedance audiometry, and Brainstem Auditory Evoked Potentials (BAEPs).
Results: Audiological studies of 13 children indicated sensorineural hearing loss in six children and auditory neuropathy spectrum disorder in four children.
Conclusion: This study may extend the auditory findings for INAD. Additional studies on quality of life and cognitive-brain degeneration related to this disease are required before making appropriate recommendations for aural rehabilitation.
期刊介绍：
The Journal of the American Academy of Audiology (JAAA) is the Academy''s scholarly peer-reviewed publication, issued 10 times per year and available to Academy members as a benefit of membership. The JAAA publishes articles and clinical reports in all areas of audiology, including audiological assessment, amplification, aural habilitation and rehabilitation, auditory electrophysiology, vestibular assessment, and hearing science.
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