额颞叶痴呆综合征中的听觉改变言语感知

Acoustically Altered Speech Perception in Frontotemporal Dementia Syndromes.

作者信息

Johnson Jeremy C S, Jiang Jessica, Requena-Komuro Mai-Carmen, Benhamou Elia, Sivasathiaseelan Harri, Nelson Annabel, Bamiou Doris-Eva, Hardy Chris J D, Warren Jason D

机构信息

Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, United Kingdom.

Basic & Clinical Neurosciences, Kings College London & King's College Hospital NHS Foundation Trust, United Kingdom.

出版信息

Neurology. 2025 Oct 7;105(7):e214022. doi: 10.1212/WNL.0000000000214022. Epub 2025 Sep 11.

DOI:10.1212/WNL.0000000000214022

PMID:40934459

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12427881/

Abstract

BACKGROUND AND OBJECTIVES

Hearing impairment is a major clinical issue in dementia. Acoustically altered speech (AAS) is a typical real-world listening challenge, but markers of AAS perception, especially in frontotemporal dementias (FTDs), remain undefined. We investigated whether AAS perception differentiates FTD syndromes and predicts real-world hearing symptoms.

METHODS

In this cross-sectional case-control study, participants meeting diagnostic criteria for FTD and primary progressive aphasia syndromes were recruited at a UK tertiary center. Participants performed pure-tone audiometry (PTA) and 3 AAS perception tests: speech-in-babble (SIB), spectrally filtered speech (SFS), and time-compressed speech (TCS). Real-world hearing abilities were assessed through the modified Amsterdam Inventory for Auditory Disability and Handicap (mAIAD). Group characteristics were compared using analysis of variance and Fisher exact tests. Regression models adjusted for PTA and Wechsler Abbreviated Scale of Intelligence Matrices scores compared group performance on AAS tests. The Spearman test examined correlations with mAIAD scores.

RESULTS

Among 44 participants, the mean (SD) age was 66 (6.23) years and 13 (29.5%) were female. 29 patients with FTD (6 with nonfluent/agrammatic variant PPA [nfvPPA], 8 with semantic variant PPA [svPPA], and 15 with behavioral variant FTD [bvFTD]) were compared with 15 age-matched controls. Patients with nfvPPA had significantly higher mean PTA thresholds than controls (mean difference 12 decibels [95% CI 0.7-23.4], = 0.034). Those with nfvPPA performed significantly worse on the SFS test than all other groups (vs controls: difference 6.3 [9.4-3.1], < 0.001; vs svPPA group: difference 2.4 [0.5-7.3], = 0.0018; vs bvFTD group: difference 2.0 [1.45-7.0], = 0.0012) and significantly worse on the TCS test than controls and patients with bvFTD (vs controls: difference 88.2 [29.7-146.7], < 0.01; vs bvFTD group: difference 76.1 [26.7-125.5], < 0.01). The svPPA group performed significantly worse than controls on TCS (difference 57.4 [16.38-98.4], = 0.01). Patients with nfvPPA (difference 27.2 [9.3-45.2], < 0.001) and svPPA (difference 25.7 [11.8-39.5], = 0.0013) had significantly worse mAIAD scores than controls. Over the combined patient cohort, mAIAD scores correlated more strongly with AAS than PTA thresholds (TCS, ρ = 0.64, < 0.001; SFS, ρ = 0.53, = 0.003; SIB, ρ = 0.5, = 0.004; PTA, ρ = 0.37, = 0.028).

DISCUSSION

AAS perception stratifies FTD syndromes and constitutes a "real-world audiogram" in these diseases.

摘要

背景与目的

听力障碍是痴呆症中的一个主要临床问题。声学改变的语音（AAS）是现实世界中典型的听力挑战，但AAS感知的标志物，尤其是在额颞叶痴呆（FTD）中，仍不明确。我们研究了AAS感知是否能区分FTD综合征并预测现实世界中的听力症状。

方法

在这项横断面病例对照研究中，在英国一家三级中心招募了符合FTD和原发性进行性失语综合征诊断标准的参与者。参与者进行了纯音听力测定（PTA）和3项AAS感知测试：嘈杂环境中的言语（SIB）、频谱滤波语音（SFS）和时间压缩语音（TCS）。通过改良的阿姆斯特丹听觉残疾和障碍量表（mAIAD）评估现实世界中的听力能力。使用方差分析和Fisher精确检验比较组间特征。针对PTA和韦氏智力简表矩阵分数进行调整的回归模型比较了AAS测试中的组间表现。Spearman检验检查与mAIAD分数的相关性。

结果

在44名参与者中，平均（标准差）年龄为66（6.23）岁，13名（29.5%）为女性。将29例FTD患者（6例非流利/语法缺失型原发性进行性失语[nfvPPA]、8例语义变异型原发性进行性失语[svPPA]和15例行为变异型FTD [bvFTD]）与15名年龄匹配的对照者进行比较。nfvPPA患者的平均PTA阈值显著高于对照组（平均差异12分贝[95%可信区间0.7-23.4]，P = 0.034）。nfvPPA患者在SFS测试中的表现明显比所有其他组差（与对照组相比：差异6.3[9.4-3.1]，P < 0.001；与svPPA组相比：差异2.4[0.5-7.3]，P = 0.0018；与bvFTD组相比：差异2.0[1.45-7.0]，P = 0.0012），在TCS测试中的表现明显比对照组和bvFTD患者差（与对照组相比：差异88.2[29.7-146.7]，P < 0.01；与bvFTD组相比：差异76.1[26.7-125.5]，P < 0.01）。svPPA组在TCS测试中的表现明显比对照组差（差异57.4[16.38-98.4]，P = 0.01）。nfvPPA患者（差异27.2[9.3-45.2]，P < 0.001）和svPPA患者（差异25.7[11.8-39.5]，P = 0.0013）的mAIAD分数明显比对照组差。在整个患者队列中，mAIAD分数与AAS的相关性比与PTA阈值的相关性更强（TCS，ρ = 0.64，P < 0.001；SFS，ρ = 0.53，P = 0.003；SIB，ρ = 0.5，P = 0.004；PTA，ρ = 0.37，P = 0.028）。