Rance Gary, Zanin Julien
Department of Audiology and Speech Pathology, The University of Melbourne, Parkville VIC 3052, Australia.
J Clin Med. 2024 Dec 2;13(23):7311. doi: 10.3390/jcm13237311.
Chiari malformation is a condition involving caudal descent of the hindbrain which herniates the cerebellar tonsils through the foramen magnum. The purpose of this study was to quantify auditory deficits in an affected individual and to explore the hypothesis that cerebellar malformation specifically disrupts binaural processing. We present audiometric, electrophysiologic, imaging and auditory perceptual findings for a 17-year-old female with Chiari 1 malformation and for a cohort of 35 hearing- and age-matched controls. The patient presented with auditory deficit consistent with cerebellar disorder-that is, an impaired ability to judge the duration of auditory stimuli. In addition, she showed evidence of abnormal binaural processing affecting her capacity to localise sound sources to optimise speech perception in background noise. The provision of a remote microphone listening device was successful in improving her perceptual ability to normal levels. Despite normal sound detection ability, this child with Chiari 1 malformation suffered functional hearing deficits severe enough to impact everyday listening/communication and educational progress. These limitations were ameliorated through auditory intervention.
Chiari畸形是一种涉及后脑尾端下移的疾病，小脑扁桃体通过枕骨大孔疝出。本研究的目的是量化一名患病个体的听觉缺陷，并探讨小脑畸形特别干扰双耳加工的假说。我们展示了一名患有Chiari 1畸形的17岁女性以及一组35名听力和年龄匹配的对照者的听力测定、电生理、影像学和听觉感知结果。该患者表现出与小脑疾病一致的听觉缺陷，即判断听觉刺激持续时间的能力受损。此外，她还表现出双耳加工异常的证据，影响了她在背景噪声中定位声源以优化语音感知的能力。提供远程麦克风聆听设备成功地将她的感知能力提高到正常水平。尽管声音检测能力正常，但这名患有Chiari 1畸形的儿童仍存在严重的功能性听力缺陷，足以影响日常听力/交流和教育进展。通过听觉干预，这些限制得到了改善。
