Objectives Gradually progressive sensorineural hearing loss (SNHL) is a prevalent sensory defect. It is generally untreatable, making rehabilitation by hearing aid or cochlear implantation the only option. However, SNHL as one of the symptoms of the hereditary autoinflammatory systemic disease cryopyrin-associated periodic syndrome, or as the only symptom of the cochlea-specific form (DFNA34), was suggested to respond to IL-1 antagonist (anakinra) therapy, which ameliorates NLRP3 variants-induced over-secretion of IL-1β. We analysed genotypic and phenotypic spectrum of cryopyrin-associated periodic syndrome or DFNA34, specifically focusing on the responsiveness of SNHL to anakinra. Methods Seventeen families diagnosed with either cryopyrin-associated periodic syndrome or DFNA34 were recruited. Genotyping and phenotyping including audiogram, MRI findings, and in vitro IL-1β assay were performed. Results Our cohort had an etiologic homogeneity of 94.1% to NLRP3 variants and a high de novo occurrence (84.6%). We identified the second DNFA34 pedigree worldwide with a novel NLRP3 variant supported by in vitro analysis. Significant improvement of hearing status against the natural course, showing response to anakinra, was identified in three probands, one of whom used to have severe SNHL. Hearing threshold worse than 60 dB at the start of anakinra and cochlear enhancement on brain MRI seemed to be related with poor audiologic prognosis and responsiveness to anakinra therapy despite stabilized systemic symptoms and inflammatory markers. Conclusion We propose a constellation of biomarkers comprising NLRP3 genotypes, hearing status at diagnosis, and cochlear radiological findings as prognostic factors of hearing status after anakinra treatment and possibly as sensitive parameters for treatment dosage adjustment.

We compared the therapeutic efficacy of facial nerve decompression (FND) and conservative treatment in patients with Bell’s palsy through a systematic review and meta-analysis. Primary database search was performed in PubMed, Medline, and Embase. After screening, 13 studies were assessed for their eligibility. Among them, seven studies employing either the House-Brackmann grading system (HBGS) or May’s classification (modified HBGS) were selected for quantitative and qualitative analysis. Based on May’s classification, the degree of recovery was classified into complete (HBGS I), fair (HBGS II–III), or failed (HBGS IV–VI) recovery. The outcomes were assessed between 6 and 12 months after surgery. The estimated pooled odds ratio (OR) and 95% confidence interval (CI) were calculated using random effects model. Cohorts were comprised of patients who underwent FND (n=202, 53.0%) and conservative treatments (n=179, 47.0%). In pooled analysis, the rate of complete recovery was significantly higher in the FND group than in the control group (OR, 2.06; 95% CI, 1.22 to 3.48; P=0.007) showing neither heterogeneity nor publication bias. Meanwhile, the rates of fair recovery (OR, 0.71; 95% CI, 0.42 to 1.21; P=0.208) and failed recovery (OR, 0.60; 95% CI, 0.22 to 1.67; P=0.327) in the FND group were similar to that in the control group. In subgroup analyses, there was no significant difference in the OR according to the operation timing and surgical approach. FND can be a possible treatment option for patients with complete Bell’s palsy, especially for complete recovery, which provide insights on decision-making and outcome prediction. However, FND should be determined carefully given the risk of small study effects and possible complications.

The Journal of International Advanced Otology (J Int Adv Otol) is an international, peer reviewed, open access publication that is fully sponsored and owned by the European Academy of Otology and Neurotology and the Politzer Society. The journal is published triannually in April, August, and December and its publication language is English. The scope of the Journal is limited with otology, neurotology, audiology (excluding linguistics) and skull base medicine. The Journal of International Advanced Otology aims to publish manuscripts at the highest clinical and scientific level. J Int Adv Otol publishes original articles in the form of clinical and basic research, review articles, short reports and a limited number of case reports. Controversial patient discussions, communications on emerging technology, and historical issues will also be considered for publication. Target audience of J Int Adv Otol includes physicians and academics who work in the fields of otology, neurotology, audiology and skull base medicine.

Background and Objectives: Bilateral microphones with contralateral routing of signal (BiCROS) hearing aid is an option for hearing rehabilitation in individuals with asymmetric sensorineural hearing loss (ASNHL). The clinical factors influencing the trial and purchase of BiCROS were investigated. Subjects and Methods: We reviewed the medical records of 78 patients with ASNHL who were recommended to use BiCROS and analyzed the demographic and audiological factors influencing the trial and purchase of BiCROS. Results: Among the 78 patients, 52 (66.7%) availed of the free BiCROS trial and 21 (26.9%) purchased BiCROS. The mean pure tone audiometry (PTA) air conduction (AC) threshold of the better-and worse-hearing ears were 44.2±12.8 dB and 90.7±22.5 dB HL, respectively. The decision for trial or purchase of BiCROS was not influenced by age, sex, duration of hearing loss of the worse-hearing ear, or PTA AC threshold or speech discrimination score of both ears. The first and third quartiles of the PTA AC thresholds for the better-hearing ear of BiCROS buyers were 38.75 dB and 53.75 dB HL, respectively. The counterpart values for the worse-hearing ear were 72.50 dB and 118.75 dB HL, respectively. Conclusions: The clinical factors analyzed in this study were found to be irrelevant to the trial and purchase of BiCROS in patients with ASNHL. Nevertheless, the distribution range of the auditory thresholds of the subjects using BiCROS can be a useful basis for the counseling of patients with ASNHL and selection of candidates for BiCROS use.