Fig. 6.

Otoacoustic emissions. Otoacoustic emissions (OAEs) were present, present but reduced, or initially present and lost over time in over 80% of cases (A). The proportion of cases with present OAEs decreases in older age groups (B; n = 5 cases with OAEs that were initially present and lost over time are represented twice, once at their oldest age with present OAEs and once at their youngest age with absent OAEs). Patients with mild or temperature-sensitive sensorineural hearing loss were more likely to have present OAEs than patients with other phenotypes (C), and patients with at least one non-truncating (NT) allele were more likely to have present OAEs than patients with two truncating (T) alleles (D). Only hearing loss phenotypes classified using behavioral audiometry were included in C. Year of Life 1 = birth to 1 year old; Year of Life 2 = 1.01 years old to 2 years old; etc.