Table 3.
Distribution of the Type and Degree of Hearing Loss Among the CAPS Groups, n ears (%)
| NOMID (n = 38) | NOMID/MWS (n = 14) | MWS (n = 12) | FCAS (n = 12) | |
|---|---|---|---|---|
| Typea | ||||
| Normal | 6 (16) | 2 (14) | 8 (67) | 9 (75) |
| CHL | 4 (11) | 0 (0) | 0 (0) | 0 (0) |
| MHL | 5 (13) | 2 (14) | 0 (0) | 0 (0) |
| SNHL | 23 (61) | 10 (71) | 4 (33) | 3 (25) |
| Degreeb | ||||
| Normal | 9 (24) | 2 (14) | 8 (67) | 9 (75) |
| Mild | 7 (18) | 3 (21) | 0 (0) | 3 (25) |
| Moderate | 15 (39) | 8 (57) | 4 (33) | 0 (0) |
| Severe | 4 (11) | 0 (0) | 0 (0) | 0 (0) |
| Profound | 3 (8) | 1 (7) | 0 (0) | 0 (0) |
Abbreviations: CHL, conductive hearing loss; FCAS, familial cold autoinflammatory syndrome; MHL mixed hearing loss; MWS, Muckle-Wells syndrome; NOMID, neonatal-onset multisystem inflammatory disease; SNHL, sensorineural hearing loss.
a
Type of hearing loss was based on a 3-frequency (0.5/1/2 kHz) pure-tone average for air and bone conduction thresholds.
b
Degree of hearing loss was based on a 4-frequency (0.5/1/2/4 kHz) pure-tone average for air conduction thresholds.