Supporting information

Supporting Information 1 Table S1: Operational definitions of pure tone averages, degree, and type of hearing loss.

Supporting Information 2 Table S2: Post hoc comparisons and visit slopes of hearing by genotype and OI type (4F‐PTA).

Supporting Information 3 Table S3: Final decade of completed audiological measures by gene and decade.

Supporting Information 4 Table S4: PTA as a function of OI type, gene variant, and visit.

Supporting Information 5 Figure S1: Normal hearing, degree of hearing loss and progression by OI type. All ears (individual lines) with normal hearing (WNL = within normal limits; gray circles), hearing loss (horizontal dashed line represent degree of hearing loss designation; > 20 dBHL is hearing loss), and type (CHL = blue triangle, MHL = yellow inverted triangle, and SNHL = red square) indicated as a function of age (years). Each row represents LF‐, 4F‐, and HF‐PTA with columns noting normal hearing, stable or progressive hearing loss, by OI Type III or IV. Bone conduction not measured indicated by “x,” and unfilled circles represent cases unable to be categorized by hearing loss type. Refer to Figure 1 for representation of only ears with hearing loss.

Supporting Information 6 Figure S2: OI Type IV hearing by PTA for quartiles. All ears (individual lines) representing degree and type of hearing loss by (rows) LF‐, 4F‐, HF‐PTA and age (years) sorted by quartile (columns; Q1, Q2, Q3, and Q4). One individual had a bilateral high frequency hearing loss in Q3 in the fourth decade of life, but hearing was not measured in the third decade. Same colors and representation of symbols as Figure 1.

Supporting Information 7 Figure S3: Hearing by gene variant and OI type. All ears (individual lines) represent degree and type of hearing loss by age (years). Columns represent gene variant and rows represent LF‐, 4F‐, and HF‐PTA. Normal hearing, stable hearing, and progressive hearing loss are shown together. Same colors and representation of symbols as Figure 1.