Hearing loss in Wegener's granulomatosis

To describe the frequency, type, and clinical course of hearing loss in Wegener's granulomatosis and assess hearing loss as an indicator of disease activity.

Retrospective cohort review of all patients with Wegener's granulomatosis seen in 1 year at an academic medical center.

Hearing loss documented by pure-tone audiogram.

Thirty-six patients were included in the analysis: 20 men and 16 women, with a mean age of 55.5 years (range, 22-87 yr); 30 (83%) were antineutrophil cytoplasmic autoantibodies-positive, and the mean disease duration was 47 months (range, 2-196 mo). Twenty patients (56%) had documented hearing loss: there were 17 (47%) cases of sensorineural hearing loss and 12 (33%) cases of conductive hearing loss. Seven of 12 cases of conductive hearing loss improved with immunosuppressive treatment of Wegener's granulomatosis, 2 worsened, and 3 remained stable. Of 17 cases of sensorineural hearing loss, 3 improved, 4 worsened, and 10 remained stable. Seven patients had hearing loss requiring amplification. Five of 35 (14%) patients had established hearing loss months to years before diagnosis of Wegener's granulomatosis. Hearing loss occurred both on initial presentation and with disease relapse. The rates of conductive hearing loss (38%) and sensorineural hearing loss (31%) were also high in the subset of patients 65 years of age or younger and without history of noise exposure.

Both sensorineural hearing loss and conductive hearing loss are common in Wegener's granulomatosis, may result in significant morbidity, and may precede the diagnosis of Wegener's granulomatosis by years. Both types of hearing loss in patients with Wegener's granulomatosis may be used as an indicator of disease. These data suggest that it may be appropriate to obtain screening audiograms in all patients with newly diagnosed or relapsing Wegener's granulomatosis.