Inflammatory labyrinthitis is defined as a fluctuant vestibulo-cochlear syndrome associated with an impairment of the blood-labyrinthine barrier (BLB) on delayed FLAIR MRI sequences. Systemic and intratympanic corticosteroids are the gold standard treatment but their effect is frequently insufficient. The objective is here to determine whether infliximab could be of value in the treatment of bilateral inflammatory labyrinthitis. A retrospective monocentric study was conducted between January 2013 and December 2021. All patients included in the study were affected with a bilateral vestibulo-cochlear syndrome associated with bilateral blood-labyrinthine barrier impairment. Patients were administered infliximab at the dose of 5 mg/kg every 6 weeks for 6 months. Audiometry, MRI with delayed FLAIR sequences on the labyrinth, and corticosteroid doses still required were assessed both before and after treatment with infliximab was completed. Pure-tone average (PTA) was the primary outcome. The secondary outcomes were the speech recognition threshold (SRT), the Dizziness Handicap Inventory (DHI) score, and the corticosteroid (CS) dose. A total of nine patients including five men and four women were enrolled in the study. Thirteen ears were analyzed. After a 6-month period of treatment, the mean PTA (54 ± 24 db versus 66 ± 22 db; p = 0.027), SRT (54 ± 37 db versus 66 ± 32 db; p = 0.041) and DHI score (27 ± 15 versus 9 ± 2; p = 0.032) significantly improved. After the 6-month treatment period, the mean CS dose decreased from 38 ± 33 to 6 ± 5 mg/day (p = 0.003). We conclude that infliximab substantially improves the vestibulo-cochlear function in patients with bilateral inflammatory labyrinthitis and could be of value in corticosteroid-dependent cases.

Background: The main clinical features of pseudohypoparathyroidism (PHP)/inactivating parathyroid hormone/parathyroid hormone-related protein signaling disorders (iPPSD), including parathyroid hormone (PTH) resistance, brachydactyly and short stature, develop during middle and late childhood. Very few studies have addressed hearing loss in PHP/iPPSD patients, and these studies have yielded widely divergent conclusions. The aim of our study was to assess hearing and determine the predictive factors of hearing loss in patients with PHP/iPPSD. Methods: Our retrospective cohort study was conducted between March 2019 and May 2020 in the Otolaryngology Department and the calcium phosphate reference center for rare diseases in Bicêtre Paris-Saclay Hospital, France. We retrospectively collected data from patients with PHP/iPPSDs (age, sex, genetic mutations, height, body mass index (BMI), PTH resistance, presence or absence of ectopic ossifications and brachydactyly). All patients underwent auditory investigations, including tonal and vocal audiometry. The primary outcome was the pure tone average (PTA). The PTA was compared with the norm according to the International Organization for Standardization. Hearing loss was defined as a PTA ≥ 20 db. Results: The median age of the patients was 15.6 years [9.5, 28.5]. Thirty-six patients were diagnosed with iPPSD2, and eight were diagnosed with iPPSD3. Twenty-six of them (59%) were female. Hearing impairment was confirmed in 17 patients (39%). The mean PTA and the mean SRT of the deaf ears were 40 ± 26 db and 31 ± 14 db. The mean difference in the PTA between the patients and the normal controls was 11.4 db (p = 0.00002). Short stature and the presence of ectopic ossifications were two significant predictive factors of hearing loss (p = 0.009 and p = 0.03, respectively). Sex, BMI, PTH resistance, mutation category and brachydactyly were not associated with an increased risk of hearing loss (p = 0.19, p = 0.41, p = 0.13, p = 0.50, p = 0.19, respectively). Conclusion: Our study confirmed the frequency of hearing loss in patients with PHP/iPPSD disease (prevalence = 39%). A diagnosis of PHP/iPPSD should trigger auditory investigations and follow-up, especially when short stature and/or ectopic ossifications are present.

Objective: To characterize the clinical, radiologic, and electrophysiological features of spontaneous autoplugging in superior semicircular canal dehiscence (SSCD) and compare them to untreated and surgically treated cases. Methods: This retrospective observational study was conducted in a tertiary referral center in Paris, France. Fifty-nine SSCD ears were categorized into 3 groups: untreated (n=36), autoplugging (n=5), and surgically plugged (n=18). Autoplugging was defined as the absence of a fluid signal in the dehiscent canal on high-resolution 3D T2-weighted MRI in patients with no history of ear surgery. All patients underwent clinical assessment, audiometry, video head impulse test (vHIT), cervical vestibular-evoked myogenic potentials (cVEMP), and high-resolution imaging. Group comparisons used nonparametric tests with FDR correction and power analysis. Results: vHIT gain was significantly reduced in both the autoplugging (0.56 ± 0.09) and surgical groups (0.57 ± 0.13) compared with untreated SSCD (0.77 ± 0.19, P =0.0003). The reduction was confined to the superior canal, whereas lateral and posterior canal gains remained normal. Autophony persisted in the autoplugging group (80%) but was significantly reduced in the surgical group (22.2%, P <0.0001). cVEMP thresholds were abnormally low in untreated (62.0 ± 11.1 dB) and autoplugging ears (62.5 ± 9.6 dB), and significantly higher post-surgery (83.5 ± 12.3 dB, P <0.001). Conclusion: Autoplugging mimics surgical plugging in terms of canal dysfunction but fails to alleviate third-window symptoms. It may represent an incomplete, spontaneous adaptation in SSCD pathophysiology. Level of Evidence: Level 3.

Background: Non‐infectious labyrinthitis (NIL) is an inflammatory disorder of the inner ear characterized by cochlear and vestibular symptoms associated with impairment of the blood–labyrinth barrier on delayed FLAIR MRI. Although the condition may initially present unilaterally, some patients subsequently develop bilateral disease, which can lead to greater functional disability. Identifying factors associated with bilateralization (i.e., progression from unilateral to bilateral involvement) remains clinically important. Methods: We conducted a retrospective case–control study in a tertiary referral center to identify factors associated with bilateral involvement in NIL. Fifty patients diagnosed with NIL between 2018 and 2025 were included: 25 with bilateral NIL (BNIL) and 25 with unilateral NIL (UNIL). Clinical, audiometric, vestibular, and biological data were collected from medical records. Logistic regression analyses were performed to identify factors associated with bilateralization. Results: Compared with UNIL patients, BNIL patients were significantly younger (42.4 ± 10.9 vs. 54.8 ± 11.6 years, p = 0.0003) and more frequently female (60% vs. 24%, OR = 4.75, p = 0.010). Systemic disease was more frequent in BNIL patients (36% vs. 4.0%, OR = 13.50, p = 0.011). In multivariate analysis, younger age, female gender, and the presence of systemic disease were independently associated with bilateral involvement. Conclusions: Younger age, being female, and systemic disease are associated with bilateralization in NIL. These findings may help identify subgroups who warrant closer clinical monitoring. However, given the retrospective case–control design, small single‐center sample, referral bias, and sparse data (particularly for systemic disease), these results should be interpreted as exploratory associations and hypothesis‐generating rather than definitive prognostic predictors ready for clinical risk scoring. Level of Evidence: 3.