Page 137 - Characterization of genetic neurodevelopmental disorders at adult age, with a focus on 22q11.2 deletion syndrome - Emma Boersma

Hearing loss and otolaryngological conditions
steeply sloping, we considered audiometric configurations showing a
decrease of ≥15-29 dB HL or ≥30 dB HL between 4 and 8 kHz gently- and
steeply-sloping as well (Table 1).
Tympanometry
If 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.14
Statistical analyses
For 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 Two-
tailed p-values <0.05 were considered statistically significant. All statistical
analyses were performed with IBM SPSS Statistics 25 (SPSS Inc., Chicago,
Illinois, USA).
4
Table 1. Definitions
Hearing loss, grading of severity a
Normal or borderline hearing (0-1) ≤20 dB HL over all frequencies
Loss, 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 HL
Loss, 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
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