{"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":22007,"journal":{"name":"Strength of Materials","volume":"15 1","pages":"324-329"},"PeriodicalIF":0.9000,"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\":22007,\"journal\":{\"name\":\"Strength of Materials\",\"volume\":\"15 1\",\"pages\":\"324-329\"},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2022-06-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Strength of Materials\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1055/a-1877-2546\",\"RegionNum\":4,\"RegionCategory\":\"材料科学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2022/6/15 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"MATERIALS SCIENCE, CHARACTERIZATION & TESTING\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Strength of Materials","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1055/a-1877-2546","RegionNum":4,"RegionCategory":"材料科学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2022/6/15 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"MATERIALS SCIENCE, CHARACTERIZATION & TESTING","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：
Strength of Materials focuses on the strength of materials and structural components subjected to different types of force and thermal loadings, the limiting strength criteria of structures, and the theory of strength of structures. Consideration is given to actual operating conditions, problems of crack resistance and theories of failure, the theory of oscillations of real mechanical systems, and calculations of the stress-strain state of structural components.
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