Background: Patients with aspirin-exacerbated respiratory disease (AERD) frequently experience symptoms consistent with eustachian tube dysfunction (ETD), which can substantially impair patient quality of life. Methods: We analyzed a cohort of 98 adult patients with AERD who participated in a longitudinal, survey-based study. Results: By assessing data over 1 year, we established that, in patients with AERD, the ear/facial subdomain of the 22-item Sino-Nasal Outcome Test (SNOT-22) questionnaire could predict performance on the 7-item Eustachian Tube Dysfunction Questionnaire, a validated instrument for the diagnosis of ETD. We then performed a re-analysis of data from a prospective, open-label study of 22 adult patients with AERD treated with dupilumab for 3 months. We found that treatment with dupilumab was associated with a significant decrease in the SNOT-22 ear/facial subdomain score, which reflects a substantial reduction in otologic symptoms and ETD within 1 month of initiating dupilumab and was sustained for 3 months afterward. Conclusion: Our findings provide evidence that dupilumab significantly improved ETD and otologic symptoms in AERD, evidenced by changes in the SNOT-22 ear/facial subdomain score. The presence of ETD and otologic symptoms should be considered when determining the optimal therapeutic course for patients with AERD.