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Hearing loss and otolaryngological conditions1354steeply sloping, we considered audiometric configurations showing a decrease of ≥15-29 dB HL or ≥30 dB HL between 4 and 8 kHz gently- andsteeply-sloping as well (Table 1).TympanometryIf deemed to be indicated (n=24; 40%), tympanometry was used to measure the tympanic membrane’s response to changes in pressure in order to detect effusion or depression in the middle ear. Abnormal responses were classified using the Jerger classification that was adapted for adults.14Statistical analysesFor hearing loss and history of otolaryngological conditions, we calculated prevalence rates and related 95% confidence intervals (CI) using the formula CI= p ± 1.96* √(p(1-p)/n). We calculated the prevalence of hearing loss of at least one ear, in addition to the better-hearing ear. We used ordinal regression analysis to evaluate the effect of age, sex, FSIQ and history of chronic otitis media on the severity of hearing loss of the better-hearing ear according to the Muenster classification. Mathematica (Wolfgang mathematica 13.2, Oxfordshire, United Kingdom) was used to plot the sensory thresholds (dB HL) in adults with 22q11.2DS at 0.25, 0.5, 1, 2, 4 and 8 kHz, relative to 50th and 90th percentiles in the general population.15 Twotailed p-values <0.05 were considered statistically significant. All statistical analyses were performed with IBM SPSS Statistics 25 (SPSS Inc., Chicago, Illinois, USA).Table 1. DefinitionsHearing loss, grading of severity aNormal or borderline hearing (0-1) ≤20 dB HL over all frequenciesLoss, limited to high-frequencies (2) ≥4 kHz >20 dB HL Mild (2a) >20 ≤40 dB HL Moderate (2b) >40 ≤60 dB HL Severe (2c) >60 dB HLLoss, corrigible with hearing aids (3) <4 kHz >20 dB HL Mild (3a) >20 ≤ 40 dB HL Moderate (3b) >40 ≤ 60 dB HL Severe (3c) >60 dB HL