Table 2 Audiologic findings in a cohort of pediatric and adult MWS patients at baseline and following treatment with anti-IL-1-therapy utilizing the standard 4PTA and the proposed high frequency (HF-PTA) instrument
From: Early detection of sensorineural hearing loss in Muckle-Wells-syndrome
|  | Patients N = 23 |
|---|---|
Assessments (ears) N = 46 | |
Hearing loss at baseline | |
 Patients with clinical hearing loss (%) | 21/23 (91) |
 Ears (assessments) with hearing loss (total) (%) | 42/46 (91) |
 Adults with hearing loss (%) | 11/11 (100) |
  • Abnormal assessments (adults) (%) | 22/22 (100) |
 Children with hearing loss (%) | 10/12 (83) |
  •Abnormal assessments (children) (%) | 20/24 (83) |
Hearing thresholds at baseline | |
 4-frequency pure tone average (4PTA), median (range)* | 28 (0–72) |
 Number of abnormal 4PTA assessments (%)* | 34/46 (74) |
  •Adult assessments with abnormal 4PTA (%) | 22/22 (100) |
  •Pediatric assessments with abnormal 4PTA (%) | 12/24 (50) |
 High Frequency HF-PTA median (range)* | 45 (0–114) |
 Abnormal HF-PTA hearing assessments (%) | 42/46 (91) |
  •Adult assessments with abnormal HF-PTA (%) | 22/22 (100) |
  •Pediatric assessments with abnormal HF-PTA (%) | 20/24 (83) |
Hearing thresholds at last follow-up | |
 4 Pure Tone Average (4PTA); median (range)* | 26 (0–56) |
 Abnormal 4PTA assessments (%) | 34/46 (74) |
 High Frequency HF-PTA; median (range)* | 43 (0–114) |
 Abnormal HF-PTA assessments (%) | 42/46 (83) |
Sensitivity of hearing assessments | |
 Detection of clinical hearing loss by 4PTA | 81 % |
 Detection of clinical hearing loss by HF-PTA | 100 % |