Table 1.

Baseline characteristics

	Boys	Girls	p-value
n	20	27
Age (years); median (range)	11.6 (5.1–17.9)	12.1 (6.2–18.0)	0.24
DS3 score; median (range)	7 (1–15)	3 (0–12)	0.002
Follow up (years); median (range)	2 (0–11)	2 (0–8)
Baseline (sub)clinical HL; n (%)	2 (10.5%)	1 (3.6%)

Hearing loss was defined on the basis of low frequency thresholds (PTA_.5,1,2) of the most severely affected ear. HL = hearing loss, DS3 = disease severity scoring system