Table 1.
Characteristics of 126 Patients With SNHL and GJB2 Mutations
| Characteristic | Patientsa |
|---|---|
| Sex | |
| Girls | 76 (60.3) |
| Boys | 50 (39.6) |
| Race/ethnicity | |
| White, non-Hispanic | 94 (74.6) |
| Hispanic | 10 (7.9) |
| Asian | 17 (13.5) |
| African-American and white | 23 (1.6) |
| Asian and white | 2 (1.6) |
| Unknown | 1 (0.7) |
| Progressive hearing loss | |
| Yes | 47 (37.3) |
| No | 37 (29.4) |
| Not evaluableb | 42 (33.3) |
| Baseline hearing loss, median (range), dBc | 74 (5–125) |
| Baseline age, median (range), y | 2.5 (0–15.9) |
Abbreviation: SNHL, sensorineural hearing loss.
a
Data are given as number (percentage) of patients unless otherwise indicated.
b
Twenty-five patients were profoundly deaf at presentation, precluding the measurement of significant progression; 17 had insufficient follow-up data to evaluate progression.
c
Four-frequency pure-tone average in the better ear of 118 patients. Baseline audiometric measurements were not available for 8 patients.