{"title":"一名佩利泽斯-梅尔茨巴赫氏病患者的听前庭症状","authors":"Pradeep Yuvaraj, Suman Narayana Swamy, Kallahalli Chethan, Ragavendra Kenchaiah, Gautham Arunachal Udupi, Aravind Kumar Rajasekaran","doi":"10.5152/iao.2024.231261","DOIUrl":null,"url":null,"abstract":"<p><p>Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive rare disease condition in which audiological deficit is also observed. A 4-year-old male child with PMD underwent an audiological evaluation. The results suggested normal middle ear and outer hair cells functioning, with only peak I of the auditory brainstem response present until 30 dBnHL. Further, the cervical vestibular evoked myogenic potential showed delayed latencies with normal amplitudes. In this case report, we attempt to explain the audio-vestibular test results and correlate them with the pathophysiology. This is the first report on the cervical vestibular myogenic potentials in patients with PMD.</p>","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11363208/pdf/","citationCount":"0","resultStr":"{\"title\":\"Audio-vestibular Findings in a Patient with Pelizaeus- Merzbacher Disease.\",\"authors\":\"Pradeep Yuvaraj, Suman Narayana Swamy, Kallahalli Chethan, Ragavendra Kenchaiah, Gautham Arunachal Udupi, Aravind Kumar Rajasekaran\",\"doi\":\"10.5152/iao.2024.231261\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive rare disease condition in which audiological deficit is also observed. A 4-year-old male child with PMD underwent an audiological evaluation. The results suggested normal middle ear and outer hair cells functioning, with only peak I of the auditory brainstem response present until 30 dBnHL. Further, the cervical vestibular evoked myogenic potential showed delayed latencies with normal amplitudes. In this case report, we attempt to explain the audio-vestibular test results and correlate them with the pathophysiology. This is the first report on the cervical vestibular myogenic potentials in patients with PMD.</p>\",\"PeriodicalId\":94238,\"journal\":{\"name\":\"The journal of international advanced otology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11363208/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"The journal of international advanced otology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.5152/iao.2024.231261\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"The journal of international advanced otology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.5152/iao.2024.231261","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Audio-vestibular Findings in a Patient with Pelizaeus- Merzbacher Disease.
Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive rare disease condition in which audiological deficit is also observed. A 4-year-old male child with PMD underwent an audiological evaluation. The results suggested normal middle ear and outer hair cells functioning, with only peak I of the auditory brainstem response present until 30 dBnHL. Further, the cervical vestibular evoked myogenic potential showed delayed latencies with normal amplitudes. In this case report, we attempt to explain the audio-vestibular test results and correlate them with the pathophysiology. This is the first report on the cervical vestibular myogenic potentials in patients with PMD.
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