Point-of-care hearing screening using consumer wireless earbuds in patients with USH2A-associated retinal disease: a prospective pilot feasibility study

Usher syndrome type 2 (USH2) may be under-recognized in ophthalmology practice due to variable hearing loss severity and limited point-of-care audiometry. In this prospective pilot study at the Singapore National Eye Centre (November 2024 to February 2025) we evaluated a consumer earbud hearing test in patients with USH2A-associated retinal disease and explored cross-sectional relationships between hearing and visual measures. Thirteen patients with biallelic USH2A pathogenic variants (median age 45 years, range 29-81) and thirteen age- and sex-matched comparison participants underwent supervised air-conduction threshold estimation using AirPods Pro 2. All participants completed testing without failure, repetition or technical error. Among patients, median test duration was 4.5 minutes (range 3.6–5.1), and three required physical guidance. These feasibility outcomes are reported descriptively and post hoc. Right-ear pure-tone average (PTA4-R) ranged from 13.00 to 84.25 dB HL (median 48.25 dB HL) and ellipsoid zone (EZ) width ranged from 0 to 7,972 μm (median 1,037 μm). After adjustment for age, higher PTA4-R showed a negative association estimate with EZ width (β = −57.36 μm/dB, 95% CI −117.56 to 2.83; nominal p = 0.0597) and a positive association estimate with best-corrected visual acuity (BCVA) in logMAR units (β = 0.02793 logMAR/dB, 95% CI 0.00429 to 0.05156; nominal p = 0.0251). Neither met the Bonferroni-adjusted significance threshold (adjusted p = 0.1194 and 0.0501, respectively). Earbud-derived threshold configurations resembled previously described USH2A-associated patterns, but agreement with calibrated clinical audiometry was not assessed. Formal audiological evaluation remains necessary for diagnosis and management.

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Journal
Scientific Reports
Published
2026-10-16
DOI
https://doi.org/10.1038/s41598-026-75833-1
Primary Topic
Hearing, Cochlea, Tinnitus, Genetics
Type
article
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article

Point-of-care hearing screening using consumer wireless earbuds in patients with USH2A-associated retinal disease: a prospective pilot feasibility study

Beau James Fenner, Hiok Hong Chan, Ian Y S Yeo, Mathur Ranjana et al.
Scientific Reports
Hearing, Cochlea, Tinnitus, Genetics
article

Point-of-care hearing screening using consumer wireless earbuds in patients with USH2A-associated retinal disease: a prospective pilot feasibility study

Beau James Fenner, Hiok Hong Chan, Ian Y S Yeo, Mathur Ranjana, Chui M G Cheung, Lisa George, Antoine Huang, Barrie Y. B. Tan, Rachael W. C. Tang, Anna C. S. Tan, Choi Mun Chan, Charles Ong, Shaan Wirayasaputra, Farah N. I. Ibrahim, Christopher Sun
article en

Abstract

Usher syndrome type 2 (USH2) may be under-recognized in ophthalmology practice due to variable hearing loss severity and limited point-of-care audiometry. In this prospective pilot study at the Singapore National Eye Centre (November 2024 to February 2025) we evaluated a consumer earbud hearing test in patients with USH2A-associated retinal disease and explored cross-sectional relationships between hearing and visual measures. Thirteen patients with biallelic USH2A pathogenic variants (median age 45 years, range 29-81) and thirteen age- and sex-matched comparison participants underwent supervised air-conduction threshold estimation using AirPods Pro 2. All participants completed testing without failure, repetition or technical error. Among patients, median test duration was 4.5 minutes (range 3.6–5.1), and three required physical guidance. These feasibility outcomes are reported descriptively and post hoc. Right-ear pure-tone average (PTA4-R) ranged from 13.00 to 84.25 dB HL (median 48.25 dB HL) and ellipsoid zone (EZ) width ranged from 0 to 7,972 μm (median 1,037 μm). After adjustment for age, higher PTA4-R showed a negative association estimate with EZ width (β = −57.36 μm/dB, 95% CI −117.56 to 2.83; nominal p = 0.0597) and a positive association estimate with best-corrected visual acuity (BCVA) in logMAR units (β = 0.02793 logMAR/dB, 95% CI 0.00429 to 0.05156; nominal p = 0.0251). Neither met the Bonferroni-adjusted significance threshold (adjusted p = 0.1194 and 0.0501, respectively). Earbud-derived threshold configurations resembled previously described USH2A-associated patterns, but agreement with calibrated clinical audiometry was not assessed. Formal audiological evaluation remains necessary for diagnosis and management.

Scientific Reports
Singapore National Eye Center (SG), Gleneagles Hospital (SG), Sorbonne Université (FR), Singapore Eye Research Institute (SG), Duke-NUS Medical School (SG)
Duke-NUS Medical School, SingHealth Foundation
Good health and well-being
Openalex Percentile: Top 16%
Hearing, Cochlea, Tinnitus, Genetics
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