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Findings of Impaired Hearing in Patients With Nonfluent/Agrammatic Variant Primary Progressive Aphasia.
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Hardy, CJD; Frost, C; Sivasathiaseelan, H; Johnson, JCS; Agustus, JL; Bond, RL; Benhamou, E; Russell, LL; Marshall, CR; Rohrer, JD; Bamiou, D-E; Warren, JD. - : American Medical Association, 2019
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Abstract:
Importance: Despite being characterized as a disorder of language production, nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) is frequently associated with auditory symptoms. However, to our knowledge, peripheral auditory function has not been defined in this condition. Objective: To assess peripheral hearing function in individuals with nfvPPA compared with healthy older individuals and patients with Alzheimer disease (AD). Design, Setting, and Participants: This cross-sectional single-center study was conducted at the Dementia Research Centre of University College London between August 2015 and July 2018. A consecutive cohort of patients with nfvPPA and patients with AD were compared with healthy control participants. No participant had substantial otological or cerebrovascular disease; all eligible patients fulfilling diagnostic criteria and able to comply with audiometry were included. Main Outcomes and Measures: We measured mean threshold sound levels required to detect pure tones at frequencies of 500, 1000, 2000, 4000, and 6000 Hz in the left and right ears separately; these were used to generate better-ear mean and worse-ear mean composite hearing threshold scores and interaural difference scores for each participant. All analyses were adjusted for participant age. Results: We studied 19 patients with nfvPPA (9 female; mean [SD] age, 70.3 [9.0] years), 20 patients with AD (9 female; mean [SD] age, 69.4 [8.1] years) and 34 control participants (15 female; mean [SD] age, 66.7 [6.3] years). The patients with nfvPPA had significantly higher scores than control participants on better-ear mean scores (patients with nfvPPA: mean [SD], 36.3 [9.4] decibels [dB]; control participants: 28.9 [7.3] dB; age-adjusted difference, 5.7 [95% CI, 1.4-10.0] dB; P = .01) and worse-ear mean scores (patients with nfvPPA: 42.2 [11.5] dB; control participants: 31.7 [8.1] dB; age-adjusted difference, 8.5 [95% CI, 3.6-13.4] dB; P = .001). The patients with nfvPPA also had significantly higher better-ear mean scores than patients with AD (patients with AD: mean [SD] 31.1 [7.5] dB; age-adjusted difference, 4.8 [95% CI, 0.0-9.6] dB; P = .048) and worse-ear mean scores (patients with AD: mean [SD], 33.8 [8.2] dB; age-adjusted difference, 7.8 [95% CI, 2.4-13.2] dB; P = .005). The difference scores (worse-ear mean minus better-ear mean) were significantly higher in the patients with nfvPPA (mean [SD], 5.9 [5.2] dB) than control participants (mean [SD], 2.8 [2.2] dB; age-adjusted difference, 2.8 [95% CI, 0.9-4.7] dB; P = .004) and patients with AD (mean [SD], 2.8 [2.1] dB; age-adjusted difference, 3.0 [95% CI, 0.9-5.1] dB; P = .005). Conclusions and Relevance: In this study, patients with nfvPPA performed worse on pure-tone audiometry than healthy older individuals or patients with AD, and the difference was not attributable to age or general disease factors. Cases of nfvPPA were additionally associated with increased functional interaural audiometric asymmetry. These findings suggest conjoint peripheral afferent and more central regulatory auditory dysfunction in individuals with nfvPPA. ; The Dementia Research Centre is supported by Alzheimer's Research UK, Brain Research Trust, and The Wolfson Foundation. This work was supported by the Alzheimer’s Society (grant AS-PG-16-007 [Dr Warren]), the National Institute for Health Research University College London Hospitals Biomedical Research Centre, the University College London Leonard Wolfson Experimental Neurology Centre (grant PR/ylr/18575), an Action on Hearing Loss–Dunhill Medical Trust Pauline Ashley Fellowship (grant PA23_Hardy [Dr Hardy]), a Medical Research Council PhD Studentship (Ms Bond), a Medical Research Council Clinician Scientist Fellowship (Dr Rohrer), an Association of British Neurologists Clinical Research Training Fellowship (Dr Johnson), Wolfson Foundation Clinical Research Fellowships (Drs Marshall and Sivasathiaseelan), and the Wellcome Trust (grant 091673/Z/10/Z [Dr Warren]).
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Keyword:
Impaired Hearing; primary progressive aphasia
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URL: https://qmro.qmul.ac.uk/xmlui/handle/123456789/56181 https://doi.org/10.1001/jamaneurol.2018.4799
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Impaired Interoceptive Accuracy in Semantic Variant Primary Progressive Aphasia
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Behavioural and neuroanatomical correlates of auditory speech analysis in primary progressive aphasias
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Teaching NeuroImages: Nonfluent variant primary progressive aphasia A distinctive clinico-anatomical syndrome
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