Chan Jess C S, Stout Julie C, Shirbin Christopher A, Vogel Adam P
Centre for Neuroscience of Speech, University of Melbourne, Victoria, Australia.
Turner Institute for Brain and Mental Health, School of Psychological Sciences, Monash University, Victoria, Australia.
J Huntingtons Dis. 2022;11(1):71-79. doi: 10.3233/JHD-210501.
Subtle progressive changes in speech motor function and cognition begin prior to diagnosis of Huntington's disease (HD).
To determine the nature of listener-rated speech differences in premanifest and early-stage HD (i.e., PreHD and EarlyHD), compared to neurologically healthy controls.
We administered a speech battery to 60 adults (16 people with PreHD, 14 with EarlyHD, and 30 neurologically healthy controls), and conducted a cognitive test of processing speed/visual attention, the Symbol Digit Modalities Test (SDMT) on participants with HD. Voice recordings were rated by expert listeners and analyzed for acoustic and perceptual speech features.
Listeners perceived subtle differences in the speech of PreHD compared to controls, including abnormal pitch level and speech rate, reduced loudness and loudness inflection, altered voice quality, hypernasality, imprecise articulation, and reduced naturalness of speech. Listeners detected abnormal speech rate in PreHD compared to healthy speakers on a reading task, which correlated with slower speech rate from acoustic analysis and a lower cognitive performance score. In early-stage HD, continuous speech was characterized by longer pauses, a higher proportion of silence, and slower rate.
Differences in speech and voice acoustic features are detectable in PreHD by expert listeners and align with some acoustically-derived objective speech measures. Slower speech rate in PreHD suggests altered oral motor control and/or subtle cognitive deficits that begin prior to diagnosis. Speakers with EarlyHD exhibited more silences compared to the PreHD and control groups, raising the likelihood of a link between speech and cognition that is not yet well characterized in HD.
在亨廷顿舞蹈症（HD）诊断之前，言语运动功能和认知就开始出现细微的渐进性变化。
确定与神经功能正常的对照组相比，临床前和早期HD（即PreHD和EarlyHD）患者在听众评定的言语差异的性质。
我们对60名成年人（16名PreHD患者、14名EarlyHD患者和30名神经功能正常的对照组）进行了言语测试，并对HD患者进行了处理速度/视觉注意力的认知测试，即符号数字模态测试（SDMT）。由专业听众对语音录音进行评分，并分析声学和感知言语特征。
与对照组相比，听众察觉到PreHD患者的言语存在细微差异，包括音高异常、语速异常、响度降低和响度变化、音质改变、鼻音过重、发音不清晰以及言语自然度降低。在阅读任务中，与健康说话者相比，听众发现PreHD患者的语速异常，这与声学分析得出的较慢语速以及较低的认知表现得分相关。在早期HD中，连续言语的特点是停顿时间更长、沉默比例更高以及语速更慢。
专业听众可在PreHD患者中检测到言语和语音声学特征的差异，且这些差异与一些从声学得出的客观言语指标相符。PreHD患者较慢的语速表明在诊断之前就出现了口腔运动控制改变和/或细微的认知缺陷。与PreHD组和对照组相比，EarlyHD患者的沉默更多，这增加了HD中尚未得到充分表征的言语与认知之间存在联系的可能性。
