Audio-vestibular Findings in a Patient with Pelizaeus- Merzbacher Disease.

Pradeep Yuvaraj, Suman Narayana Swamy, Kallahalli Chethan, Ragavendra Kenchaiah, Gautham Arunachal Udupi, Aravind Kumar Rajasekaran
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Abstract

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.

一名佩利泽斯-梅尔茨巴赫氏病患者的听前庭症状
佩利泽尤斯-梅尔茨巴赫病(PMD)是一种X连锁隐性罕见病,患者也会出现听力障碍。一名患有佩利泽斯-梅尔茨巴赫病的 4 岁男童接受了听力评估。结果表明,中耳和外毛细胞功能正常,听性脑干反应只有峰值 I,直到 30 dBnHL。此外,颈前庭诱发肌源性电位显示延迟潜伏期,但振幅正常。在本病例报告中,我们试图解释听觉前庭测试结果,并将其与病理生理学联系起来。这是第一份关于 PMD 患者颈前庭肌源性电位的报告。
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