Autoinflammatory & rare systemic diseases

Sensorineural hearing loss in anti-interleukin-1 treated Cryopyrin-Associated Periodic Syndromes (CAPS) patients: risk factors and real-life barriers—an observational study

Rheumatology (Oxford)Published Longitudinal cohort study

Authors' conclusion

Early diagnosis, timely intervention and a refined Treat-to-Target approach are vital for hearing in lifelong CAPS management.

Study context

Population
Consecutive paediatric and adult anti-IL-1-treated CAPS patients with sensorineural hearing loss, 2006–202420 males · 16 females
Participants
36 participants
Outcome
WHO grade of hearing impairment
Follow-up
Last
Methodological detailssingle-centre, observational, consecutive patients
  • Single-centre
  • Observational
  • Consecutive patients

Main finding

As reported in the abstract

WHO grade of hearing impairment at last follow-up

Hearing loss present at last follow-up

88%

Observational data: associations, not effects.

Further findings

Hearing loss at baseline

Hearing loss present
83%
HF-PTA sensitivity
100%

Therapy escalation due to progressive hearing loss

Patients requiring escalation
10
Of whom carried pathogenic mutations
8
Early progression primarily driven by disease activity; late progression predominantly by non-compliance

Hearing course over time

Stable hearing
86%
Improvement
8%
Worsening
6%

Original publication

Sensorineural hearing loss in anti-interleukin-1 treated CAPS patients: risk factors and real-life barriers—an observational study. Rheumatology (Oxford). Published 1 August 2025. doi:10.1093/rheumatology/keaf355

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