Objectives: Ear involvement is a common feature of antineutrophil antibody (ANCA)-associated vasculitis (AAV). The vigilance of otolaryngologists often determines early diagnosis of AAV, thereby reducing the risk of irreversible organ damage and improving the quality of life of these patients. The goal of this study was to assess the quantitative and qualitative hearing impairment in patients with active AAV and to identify an audiological test that can detect of early deterioration of hearing even in patients without hearing loss. Methods: A study group of 46 patients with ANCA-associated vasculitis (AAV) hospitalized at the Department of Internal Diseases, Nephrology and Dialysis, Military Institute of Medicine—National Research Institute and a control group of 20 patients without a diagnosis of ANCA vasculitis and with no reported hearing disturbances were assessed prospectively. A battery of audiologic tests were carried out including High Frequency Audiometry, Impedance Audiometry, Otoacoustic Emissions, and Auditory Brainstem Responces (ABR). Computed Tomography of temporal bones and paranasal sinuses were performed, and audiologic anamnesis was gathered. Results: Pure-tone audiometry (PTA) demonstrated hearing loss in 58.6% (51/87) of the ears in the study group. The predominant type of damage was sensorineural hearing loss (SNHL). No correlation was found between hearing loss and AAV activity, duration of the disease, number of relapses, or ANCA antibody type. The statistically significant differences between control group and study group, even after excluding patients with hearing loss, were observed for high frequency audiometry (p < 0.001, for all tested frequencies excluding 14,000 Hz). The otoacoustic emissions showed to be statistically insignificant after exclusion of patients with hearing loss. Conclusions: Hearing involvement is common in patients with AAV regardless of the type of ANCA antibodies. High Frequency Audiometry could be an important audiologic screening test in this group of patients, and should be incorporated to diagnostic test battery in AAV. Otoacoustic emissions and ABR can be a handful in uncertain cases.

Objectives: ANCA-associated vasculitis (AAV) is a relapsing–remitting systemic disease in which auditory involvement is increasingly recognized as a determinant of quality of life. The objective of this study was to evaluate hearing outcomes during induction therapy in AAV and to assess the applicability of sudden sensorineural hearing loss (SSNHL) criteria for monitoring treatment response. Methods: A prospective study included 27 patients with AAV hospitalized at the Department of Internal Medicine, Nephrology and Dialysis, Military Institute of Medicine–National Research Institute. Audiologic assessment included pure-tone audiometry (including high-frequency audiometry), immittance audiometry, otoacoustic emissions, and auditory brainstem response (ABR). Examinations were performed at baseline (induction phase) and after remission. Hearing outcomes were evaluated using SSNHL criteria defined by the Ministry of Health and Welfare of Japan. Associations between hearing loss and organ involvement were also analyzed. Results: Improvement to normal hearing in pure-tone audiometry was observed in three ears, while deterioration from normal hearing occurred in four ears. No statistically significant differences were found in overall otoacoustic emission responses between baseline and follow-up. A statistically significant association was identified between hearing loss and peripheral nervous system involvement (50.0% vs. 11.1%, p = 0.057). No significant correlations were observed between hearing outcomes and other clinical parameters. Conclusions: SSNHL-based hearing outcome criteria may represent a practical tool for assessing auditory dysfunction and monitoring treatment response in AAV. High-frequency audiometry and structured outcome measures may improve detection of subtle cochlear changes. Further studies with larger cohorts are required to validate these findings and clarify the relationship between systemic disease activity and hearing impairment.

Objectives: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of vasculitides sharing a common pathophysiology, which affects small and medium blood vessels. Sinonasal involvement is one of the most common manifestations of AAV. The goal of this study was to find the most suitable method to assess paranasal sinus changes in a group of patients with ANCA-associated vasculitis and renal involvement. Subjective scales like Lund–Mackay and Zinreich were compared with a three-dimensional (3D) volumetric method. Pre- and post-treatment computer tomography were compared. Methods: Computer tomography, nasal symptoms, and endoscopy of 28 patients hospitalized at the Department of Internal Diseases, Nephrology and Dialysis, Military Institute of Medicine—National Research Institute were assessed retrospectively. Paranasal sinus tomography was performed during treatment induction and after achieving disease remission (BVAS = 0) to assess treatment effectiveness. Radiological analysis was performed with the Lund–Mackay scoring system, Zinreich scoring system, and 3D volumetric scoring system with the usage of Slicer 3D analysis. The radiologic scoring systems were compared. Results: The statistically significant differences in treatment effectiveness were observed for the Zinreich scale on both the right and left side. Similar to the 3D volumetric scoring system, the right and left maxillary sinuses demonstrated statistically significant differences. On the other hand, no statistically significant differences were found between the first and second visits for the Lund–Mackay or total Global Osteitis scores on either side. The strongest correlation was achieved between the Zinreich scoring system and 3D volumetric scale. Conclusions: The three-dimensional CT volumetric analysis demonstrated higher SRM (standardized response mean) values than the Zinreich score on both sides, but the differences were not statistically significant. The Zinreich scoring system should be used instead of the Lund–Mackay scale in everyday clinical practice.