Sudden hearing improvement under triple immunosuppression in subacute unilateral deafness in granulomatosis with polyangiitis

Introduction A 44-year-old female presented to our department with subacute unilateral deafness. She had been first diagnosed with granulomatosis with polyangiitis (GPA) at that time. Literature shows that hearing improvement can result from immunosuppressive therapy in patients with GPA and the present symptomatology. However, external cMRI showed cochlear signal reduction in CIS3D sequence (contrast agent was not given due to renal failure), which may indicate the onset of obliteration and thus require immediate cochlear implantation (CI) or placeholder insertion.

Methods With a PTA4 of 94 dB HL and an initial speech understanding of 5% at 100 dB on the Freiburg monosyllable word test (FMWT), we performed the CI pre-examination and planned for cochlear placeholder insertion. Simultaneously, triple immunosuppression with steroids, rituximab, and cyclophosphamide was induced due to progressive renal failure. Ten days later, the patient noticed a hearing improvement. Results The control audiogram showed significant hearing improvement (PTA4: 84 dB HL, FMWT: 30% at 95 dB). Short-term follow-up by cMRI and audiogram showed a normalized cochlear fluid signal in the CIS3D sequence and further hearing improvement (PTA4: 65 dB HL, FMWT: 25% at 65 dB).

Conclusion  Immunosuppressive therapy can lead to hearing improvement in GPA and, as in the present case, to remission of unilateral deafness with cochlear signal reduction. CI indication must be made carefully and possible remission must be considered. We recommend close monitoring and interdisciplinary care of these patients to achieve the best possible audiological and clinical outcome.

Publication History

Article published online:
12 May 2023