Table 3.
Type of Hearing Loss by Group
| Mutation Group | Sensorineurala | Mixeda |
|---|---|---|
|
| ||
| PEX1 – G843D/Null | 1/1 (100%) | 0/1 (0%) |
| PEX1 – G843D/G843D | 31/49 (63.3%) | 18/49 (36.7%) |
| Other PEX – Intermediate & Mild | 11/16 (68.8%) | 5/16 (31.2%) |
| DBPD | 11/15 (73.3%) | 4/15 (26.7%) |
a
Data indicates number (percentage) of audiograms. All audiograms included were required to have ear-specific AC and BC thresholds from 0.5 – 4kHz, and/or tympanogram data. Hearing loss was defined as conductive if BC thresholds were normal and at least one AC threshold exceeded 25 dB HL, sensorineural if all AC and BC thresholds were greater than 25 dB HL and within 10dB or less of one another, and mixed if both AC and BC thresholds were abnormal with a 15dB or greater air-bone gap at two or more frequencies or an air-bone gap of 20dB or greater at one or more frequencies.