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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Huang et al. (2026) studied this question.
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