ADVANCING CLINICAL PRACTICE AND ESTABLISHING FOUNDATIONAL THEORY FOR AUDITORY FUNCTION IN INDIVIDUALS WITH DOWN SYNDROME

项目摘要/摘要 高达80%的唐氏综合症患者在一生中都有听力损失，包括波动 传导性听力损失和永久性感音神经性听力损失。早发性老年性耳聋也是观察到的 这一群体与在其他领域观察到的性早衰一致。然而，大多数现有的流行率 数据来自对生活在机构中的个人的医疗记录审查或评估，这些机构使 适用于普通人群中的唐氏综合症患者。患有唐氏综合症的人 与神经典型个体相比，颅骨解剖学上的结构差异，包括可以预测的 高听力损失和渗出性中耳炎(OME)。然而，这些因素也会影响刺激措施。 演示级别。具体地说，耳道狭窄和OME都会导致刺激水平增加；听力 如果在刺激校准或呈现过程中不考虑这些因素，则可能低估了损失。 关键的是，这个问题可能促成了先前发现的听性脑干反应(ABR)缩短。 唐氏综合征患者相对于年龄匹配的神经典型同龄人的波形潜伏期，这是一个有用的指标 用于临床诊断对声音有可靠行为反应的个人的传导性听力损失 是无法获得的。目标1定义听力损失的患病率、类型和轨迹 唐氏综合症在普通人群中的流行。临床听力学的重大创新是通过使用STRICAL 校准技术，这将解决ABR延迟问题，并提供关于外围设备的全面数据 整个生命周期的敏感度。就像神经典型人群一样，听力损失会让人患上唐氏症 在沟通、学习、社交和就业机会方面面临重大挑战的综合症。 具体地说，感觉神经性听力损失会导致较宽的听觉滤波带宽，限制了准确的识别 语音的声音。唐氏综合征患者的小脑和皮质体积减少 与跨域的时间处理困难相关，并可能影响简要说明的重要性 在背景噪声中收听时，信噪比得到改善。鉴于认知和语言 有助于在神经典型人群中成功地进行掩蔽语音识别，语言使用率高 困难和智力残疾可能会削弱唐氏综合症患者的功能性听力 综合症。目标2建立了关于个体功能性听觉能力的基础理论 由掩蔽言语感知模型指导的唐氏综合症，该模型包括听觉、心理物理 光谱和时间分辨率的测量以及选择性注意的神经心理学因素，工作 记忆力和词汇知识。

PROJECT SUMMARY/ABSTRACT Up to 80% of individuals with Down syndrome have hearing loss across the lifespan, including fluctuating conductive hearing loss and permanent sensorineural hearing loss. Early onset presbycusis is also observed for this population, consistent with precocious aging observed in other domains. However, most existing prevalence data were obtained from medical record review or assessment of individuals living in institutions complicating application to individuals with Down syndrome in the general population. Individuals with Down syndrome have structural differences in cranial anatomy compared to neurotypical individuals, including that which would predict high rates of hearing loss and otitis media with effusion (OME). However, these factors can also impact stimulus presentation level. Specifically, stenotic ear canals and OME both result in increased stimulus levels; hearing loss may be underestimated if these factors are not considered during stimulus calibration or presentation. Critically, this issue may have contributed to previous findings of shortened auditory brainstem response (ABR) waveform latency for individuals with Down syndrome relative to age-matched neurotypical peers, a metric useful for clinical diagnosis of conductive hearing loss in individuals for whom a reliable behavioral response to sound is unable to be obtained. AIM 1 defines the prevalence, type, and trajectory of hearing loss for individuals with Down syndrome in the general population. Significant innovation to clinical audiology is gained using rigorous calibration techniques, which will resolve the issue of ABR latency and provide comprehensive data on peripheral sensitivity across the lifespan. Just as for neurotypical populations, hearing loss puts individuals with Down syndrome at risk for major challenges to communication, learning, socialization, and employment opportunities. Specifically, sensorineural hearing loss results in broad auditory filter bandwidths limiting accurate identification of speech sounds. Low cerebellar and cortical volumes observed in individuals with Down syndrome are associated with temporal processing difficulties across domains and may impact the saliency of brief improvements in signal-to-noise ratio when listening in background noise. Whereas cognition and language contribute to successful masked speech recognition in neurotypical populations, high rates of language difficulties and intellectual disability are likely to undermine functional hearing abilities for individuals with Down syndrome. AIM 2 establishes foundational theory regarding the functional auditory abilities of individuals with Down syndrome guided by a model of masked speech perception that includes audibility, psychophysical measures of spectral and temporal resolution, and neuropsychological factors of selective attention, working memory, and vocabulary knowledge.