Independent hearing evaluation in frontotemporal lobar degeneration (Utianski et al., 2025)

Background and Objectives: Hearing loss is relatively common in older individuals, and individuals with frontotemporal lobar degeneration (FTLD) are no exception; this can exacerbate communication difficulties stemming from speech-language impairments. The co-occurrence of these symptoms can contribute to increased frustration and isolation for patients and their communication partners. The objective of this study was to evaluate the feasibility of self-administered hearing testing. Ultimately, this could be administered remotely, which would increase access to assessment and inform management for this population.

Method: Seventeen patients with underlying FTLD, clinically presenting with progressive apraxia of speech with and without agrammatic aphasia, independently completed the Home Hearing Test supervised while in office.

Results: Three patients self-reported hearing loss, but results for 100% recommended formal evaluation. Eleven patients demonstrated good test accuracy. Six patients had “poor” or not computable accuracy; among them, five had evidence of cognitive impairment and/or behavioral changes, and one had known severe hearing loss, although differences between those with good and poor test accuracy were not significant. Patients with poor test accuracy had more difficulty with limb praxis.

Conclusions: Most participants independently completed the test, though some had reduced test accuracy that may have been associated with cognitive impairment and/or limb apraxia. This study supports the need for hearing screenings in all patients with progressive apraxia of speech and aphasia and highlights both the feasibility and caution required in implementing self-administered testing protocols. Further work should evaluate the feasibility of in-home hearing screenings in a broader range of FTLD phenotypes and directly compare sensitivity and specificity of hearing loss detection with in-clinic administration.

Supplemental Material S1. Example output for two patients, who each denied concerns for hearing loss; both results indicate some degree of hearing loss is likely present and suggest referral for formal evaluation. The top panel indicates POOR test accuracy, and the bottom panel indicates GOOD test accuracy.

Utianski, R. L., Meade, G., Meyer, M. J., Duffy, J. R., Clark, H. M., Whitwell, J. L., Josephs, K. A., & Poling, G. A. (2025). Testing feasibility of a self-administered hearing test for patients with progressive apraxia of speech and aphasia. American Journal of Audiology, 34(1), 220–226. https://doi.org/10.1044/2024_AJA-24-00197