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Hearing loss and otolaryngological conditions1454design. On the one hand, data concerning the medical history may not be complete resulting in an underestimation of some otolaryngologic conditions. On the other hand, referral bias may have resulted in higher prevalence rates because individuals with 22q11.2DS with a more severe clinical presentation may be more likely to be referred to a 22q11 specialty clinic. In addition, some adults did not have audiometric results because of difficulties with performing the test or noncooperation, which may have influenced results. Last, due to missing bone conduction thresholds at higher frequencies, the type of hearing impairment could not be established for some adults with 22q11.2DS, mainly those with loss limited to the higher frequencies, probably resulting in an underestimation of sensorineural hearing loss.ConclusionsHearing loss at a relatively young age, especially at the higher frequencies, appears to be common in adults with 22q11.2DS. Higher age and history of chronic otitis media are associated with more severe hearing loss. Therefore, we recommend periodic audiometric screening, including highfrequency testing (8 kHz), from early adulthood in all adults with 22q11.2DS.AcknowledgementsAvE is member of European Reference Network ITHACA.Funding This work was supported financially by Stichting Wetenschappelijk Onderzoek, ‘s Heeren Loo (#2210100). The funder had no role in the design and conduct of the study, preparation of the review, or approval of the manuscript.DisclosuresThe authors declare no conflicts of interest.Data availability statementThe data are not publicly available due to privacy and ethical restrictions. Any data requests can be directed to the corresponding author.