Background and Objectives: Granulomatosis with Polyangiitis (GPA) is a rare, autoimmune, multisystemic disease characterized by vasculitis and necrotizing granuloma that commonly affects the upper and lower respiratory tract and kidneys. Audiovestibular dysfunction in GPA diseases may have different clinical presentations. The aim of the present study was to evaluate hearing function in patients with GPA and to compare the results with a healthy control group. Materials and Methods: A total of 34 individuals participated in the study. The GPA group consisted of 14 participants, and the control group was composed of 20 healthy participants with no signs or symptoms of ear disease. The ages ranged from 18 to 65 years old, with a mean age of 43.8 years. The participants underwent a complete audiological evaluation using otoscopy, impedance audiometry, pure tone audiometry, speech audiometry—evaluation of speech thresholds, and speech recognition in quiet. Both ears were tested. All of the participants of the study were native Lithuanian speakers. Data were statistically analyzed using the Statistical Analysis System software SAS^® Studio 3.8. A p value < 0.05 was regarded as statistically significant. Results: 92.85% of patients from the GPA group reported hearing-related symptoms: hearing loss, tinnitus, and fullness in the ears. The arithmetic means of all hearing thresholds at frequencies from 125 Hz to 8000 Hz were significantly higher in the GPA group. The results revealed statistically significant differences between the two groups in the Speech Detection Threshold, Speech Recognition Threshold, Speech Discomfort level, and Word Recognition Scores. Conclusions: The frequency of hearing loss, the average hearing thresholds, and speech thresholds were higher in GPA patients than in healthy individuals. The most common type of hearing loss was sensorineural. Audiological assessments should be considered during the routine evaluation of patients with GPA disease to prevent hearing-related disabilities.

Background and Objectives: Systemic small-vessel vasculitis (SV) represents a group of rare autoimmune disorders with varied etiologies and clinical manifestations. Audiovestibular involvement in SV may present with a broad spectrum of symptoms, often complicating diagnosis and management. This study aimed to evaluate auditory function and speech perception in individuals diagnosed with SV and to investigate associations with disease-specific clinical parameters. Materials and Methods: A total of 40 patients diagnosed with SV (mean age: 48.9 years; range: 28–65 years) were recruited for comprehensive audiological assessment. The evaluation protocol included otoscopic examination, tympanometry, pure-tone audiometry, and speech audiometry. Statistical analysis was conducted using R software (version 4.3.1), and significance was set at p < 0.05. Results: Diagnoses included granulomatosis with polyangiitis (52.5%), eosinophilic granulomatosis with polyangiitis (27.5%), necrotizing vasculopathy (12.5%), and microscopic polyangiitis (7.5%). Mean disease duration was 4.14 years. Hearing complaints were reported by 77.5%; in 20%, they were the initial symptoms. Audiometry identified hearing loss in 50% of patients—predominantly sensorineural (33.8%), followed by mixed (13.7%) and conductive (2.5%) types. Hearing loss was most frequent in necrotizing vasculopathy (60%) and among ANCA-positive individuals (53.7%). Conclusions: Sensorineural hearing loss is common in SV, particularly in ANCA-positive patients, highlighting the need for routine hearing assessment in SV management.

Background and Objectives: Speech audiometry employs standardized materials, typically in the language spoken by the target population. Language-specific nuances, including phonological features, influence speech perception and recognition. The material of speech audiometry tests for the assessment of word recognition comprises lists of words that are phonemically or phonetically balanced. As auditory perception is influenced by a variety of linguistic features, it is necessary to develop test materials for the listener’s mother tongue. The objective of our study was to compose and evaluate new lists of Lithuanian words to assess speech recognition abilities. Materials and Methods: The main criteria for composing new lists of Lithuanian words included the syllable structure and frequency, the correlation between consonant and vowel phonemes, the frequency of specific vowel and consonant phonemes, word familiarity and rate. The words for the new lists were chosen from the Frequency Dictionary of Written Lithuanian according to the above criteria. Word recognition was assessed at different levels of presentations. The word list data were analyzed using a linear mixed-effect model for repeated measures. Results: Two hundred bisyllabic words were selected and organized into four lists. The results showed no statistically significant difference between the four sets of words. The interaction of the word list and presentation level was not statistically significant. Conclusions: Monaural performance functions indicated good inter-list reliability with no significant differences between the word recognition scores on the different bisyllabic word lists at each of the tested intensities. The word lists developed are equivalent, reliable and can be valuable for assessing speech recognition in a variety of conditions, including diagnosis, hearing rehabilitation and research.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition which could present as local infiltration of skull base structures. We report a case of IgG4-RD with infiltration of the temporal bone and surrounding structures in a patient with systemic vasculitis on systemic steroids. A 31-year-old woman presented with right-sided facial weakness, headache, and right ear hearing loss. On examination, redness and retraction of the right tympanic membrane and facial paresis (House-Brackman IV) were noted. Computed tomography imaging showed mastoiditis, temporal lobe stroke, and brain abscess. Magnetic resonance imaging (MRI) showed infiltration in the infratemporal fossa, nasopharynx, spreading along the Eustachian tube and perineurally along the branches of CN V and CN VII intracranially, forming a dural based mass in the middle cranial fossa. Intracranial mass compressed the temporal lobe of the brain, causing perifocal brain edema. Endoscopic biopsy of the nasopharynx was chosen as the least invasive method. It showed marked fibrosis of the tissue, dense lymphoplasmacytic infiltrates, and an increased number of IGG4-positive plasma cells. Serum IgG4 levels were below the diagnostic criteria of IgG4-RD, but histological characteristics of IgG4-RD were met. The patient was treated with high-dose oral prednisolone. Resolution of symptoms, including facial nerve paresis, was observed and infiltration in the nasopharynx, infratemporal fossa decreased on subsequent MRI tests. No recurrence was noted on the follow-up of 16 months. The case presented itself as a diagnostic challenge for a multidisciplinary team to differentiate pathology caused by either IgG4-RD, systemic vasculitis, or atypic mastoiditis. MRI and histological reports were essential to establish a correct diagnosis.

Bipolar cancellation (BPC) is an efficiency-limiting phenomenon in bipolar nanosecond pulsed electric field (nsPEF) exposures, in which the second, opposite-polarity phase reduces or partially reverses the electroporation induced by the first phase. Nisin, a cationic antibiotic peptide, has been reported to interact with lipid membranes in bacterial systems and artificial bilayer models, where it may contribute to membrane destabilization and increased permeability during pulsed electric field exposure. This study investigated whether nisin may enhance the efficacy of bleomycin electrochemotherapy (ECT) in the presence of bipolar nanosecond pulses, which are typically associated with pronounced BPC effects. Pulsed electric field (PEF) parameters and drug concentrations were selected based on preliminary viability and Yo-Pro-1 uptake experiments in CLS-354 human squamous cell carcinoma cells. To evaluate the effect of nisin, cell viability and membrane permeabilization were assessed following exposure to 300 ns pulses across a range of bipolar PEF protocols, with or without nisin, while identical unipolar pulses were used for comparison. Nisin (50 µg/mL) increased membrane permeabilization across the tested range of field amplitudes (9–15 kV/cm) and burst repetition frequencies (0.1–1.66 MHz). The presence of nisin was also associated with increased efficacy of bleomycin-based ECT under both unipolar and symmetrical bipolar PEF conditions. Under the optimized parameters tested (13 kV/cm; 150 pulses of 300 ns at 1.66 MHz), bipolar nsPEFs in combination with nisin reached levels of efficacy comparable to those observed with unipolar waveforms, suggesting a potential attenuation of bipolar cancellation effects.