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Elucidating the relationship between vestibular aqueduct size and hearing loss progression may inform the prognosis and counseling of patients who have an enlarged vestibular aqueduct (EVA).To examine the association between vestibular aqueduct size and repeated measures of hearing loss.For this retrospective medical record review, 52 patients with a diagnosis of hearing loss and radiologic diagnosis of EVA according to the Valvassori criterion were included. All available speech reception threshold and word recognition score data was retrieved; mixed-effects models were constructed where vestibular aqueduct size, age at diagnosis of hearing loss, and time since diagnosis of hearing loss were used to predict repeated measures of hearing ability. This study was performed at an academic tertiary care center.Variable vestibular aqueduct size, age at first audiogram, length of time after first audiogram.Speech reception threshold (dB) and word recognition score (%) during routine audiogram.Overall, 52 patients were identified (29 females [56%] and 23 males [44%]; median age at all recorded audiograms, 7.8 years) with a total of 74 ears affected by EVA. Median (range) vestibular aqueduct size was 2.15 (1.5-5.9) mm, and a median (range) of 5 (1-18) tests were available for each patient. Each millimeter increase in vestibular aqueduct size above 1.5 mm was associated with an increase of 17.5 dB in speech reception threshold (95% CI, 7.2 to 27.9 dB) and a decrease of 21% in word recognition score (95% CI, −33.3 to −8.0 dB). For each extra year after a patient’s first audiogram, there was an increase of 1.5 dB in speech recognition threshold (95% CI, 0.22 to 3.0 dB) and a decrease of 1.7% in word recognition score (95% CI, −3.08 to −0.22 dB).Hearing loss in patients with an EVA is likely influenced by vestibular aqueduct midpoint width. When considering hearing loss prognosis, vestibular aqueduct midpoint width may be useful for the clinician who counsels patients affected by EVA.