The Role of Platelet-Rich Plasma in the Management of Sensorineural Hearing Loss: Current Evidence and Emerging Trends.

Sensorineural hearing loss (SNHL) is a common form of hearing impairment characterized by damage to the inner ear or auditory nerve, resulting in significant communication difficulties and reduced quality of life. Current treatment options, including hearing aids, cochlear implants, and corticosteroids, primarily focus on symptom management and do not address the underlying pathophysiological damage. Platelet-rich plasma (PRP), an autologous concentrate rich in platelets and growth factors, has emerged as a potential regenerative therapy due to its ability to promote tissue repair and cellular regeneration. This review provides a comprehensive overview of the role of PRP in the management of SNHL, examining the current evidence from preclinical and clinical studies. We discuss the mechanisms through which PRP may promote auditory tissue regeneration and repair, analyze its efficacy and safety profile, and explore innovative approaches and future directions in its application for SNHL. Despite promising preliminary findings, further research is needed to optimize PRP protocols, establish standardized treatment guidelines, and conduct large-scale randomized controlled trials to validate efficacy. This review aims to highlight the potential of PRP as a novel therapeutic strategy in treating SNHL and its possible integration into current clinical practices, offering new hope for patients with this debilitating condition.

Unveiling a Rare Phenomenon: Sudden Sensorineural Hearing Loss Following Skin Prick Testing.

Introduction: The skin prick test (SPT) is a standard method for diagnosing allergic diseases, while sudden sensorineural hearing loss (SSNHL) remains a perplexing condition often of unknown etiology. We present a unique case of SSNHL emerging shortly after an SPT, suggesting a potential link between allergic reactions and inner ear disorders. Case Report: A 27-year-old male presented with unilateral hearing loss following an SPT for allergic rhinitis. Audiometric findings revealed SSNHL in the affected ear. Treatment with corticosteroids led to a full recovery of hearing function. Conclusion: This case highlights the rare occurrence of SSNHL possibly triggered by a type 1 allergic reaction to an SPT. Prompt corticosteroid therapy proved effective in restoring hearing. Further research is needed to understand the relationship between allergies and SSNHL and explore alternative treatment options.

Atypical Mumps; are We Heading Towards an Outbreak?

Mumps, caused by the mumps virus, is a contagious disease primarily affecting children and young adults. While typically presenting with salivary gland swelling and systemic symptoms, mumps can lead to various complications including SNHL, orchitis/ oophoritis, aseptic meningitis. Recent observations suggest atypical features in mumps cases, raising concerns of a potential outbreak in India. To discuss the etiopathogenesis and clinical presentation in cases of atypical mumps with increasing number of cases, a prospective multicentric study was conducted across five major centers - SMS Medical College Jaipur, RDBP Jaipuria Hospital, Jaipur, Shri Ashwini Saxena ENT Hospital Rewari, AIIMS Bhubaneswar and SP Medical College Bikaner, in India to evaluate patients with acute salivary gland swellings. Clinical and laboratory data were collected, including demographics, presenting symptoms, history of vaccination, imaging findings, and treatment outcomes. Patients were followed for four weeks post-treatment to monitor for delayed complications. Among 53 patients, a bimodal age distribution was observed, with peaks in early adolescents and middle-aged individuals. Vaccination status was recorded based on recall. Fever and salivary gland swelling were predominant symptoms, with a significant proportion experiencing submandibular gland involvement. Elevated serum amylase and CRP levels correlated with disease severity and prolonged symptomatic resolution. Notably, cases of sensorineural hearing loss (SNHL) and airway complications emerged as significant concerns. The study highlights a shift in mumps demographics, with higher age groups affected and increased incidence of complications like SNHL and airway compromise. International trends also suggest periodic outbreaks and evolving clinical manifestations post-COVID-19 pandemic. Factors contributing to mumps resurgence include lack of vaccination or vaccine efficacy, population immunity, and seasonal variations. India appears to be facing a potential mumps outbreak, characterized by atypical features and increased risk of complications like SNHL and airway compromise. Serum amylase and CRP serve as valuable markers for disease severity. Early recognition and management of complications are crucial, emphasizing the importance of mumps immunization to mitigate the impact of the disease. ENT specialists should remain vigilant for emerging complications, particularly SNHL, advocating for comprehensive immunization strategies.

Echoes of the Mind: Auditory Charles Bonnet Syndrome.

Musical hallucinations (MH) represent a rare and complex auditory phenomenon where individuals perceive music without external stimuli. This case study explores auditory Charles Bonnet syndrome (ACBS) in a 51-year-old male with a history of bilateral sensorineural hearing loss. The patient reported hearing recognizable prayer chants, initially perceived as external sounds from a nearby temple. Over time, these hallucinations persisted and interfered with his daily activities, prompting medical consultation. Despite the absence of psychiatric illness, the patient was diagnosed with ACBS and treated with risperidone, an atypical antipsychotic. The intervention led to a significant reduction in the frequency and intensity of the hallucinations, alongside improved sleep and concentration. The patient also experienced a recurrence of symptoms upon discontinuation of the medication, highlighting the importance of adherence to treatment. This case underscores the need for awareness and understanding of non-psychotic auditory hallucinations in individuals with hearing impairments. The pathophysiology of MH is not fully understood but is believed to involve abnormal activity in the auditory associative cortices due to sensory deprivation. Treatment approaches often include both pharmacological and non-pharmacological strategies, such as optimizing hearing with aids and providing psychoeducation. This study contributes to the limited literature on ACBS and emphasizes the efficacy of antipsychotics in managing MH. Further research is essential to explore the underlying mechanisms and to develop comprehensive management plans for patients experiencing these distressing auditory phenomena. The findings advocate for a multidisciplinary approach to treatment, integrating audiological and psychiatric care to improve patient outcomes.

Automated segmentation of clinical CT scans of the cochlea and analysis of the cochlea's vertical profile.

Purpose: Knowledge of the cochlear anatomy in individual patients is helpful for improving electrode selection and placement during cochlear implantation, as well as in surgical planning. The aim of this study was to develop a model-free automated segmentation algorithm to obtain 3D surfaces from clinical computed tomography (CT) scans that describe an individual's cochlear anatomy and can be used to quantitatively analyze the cochlea's vertical trajectory. Methods: Clinical CT scans were re-oriented and re-sliced to obtain mid-modiolar slices. Using these slices, we segmented the cross-section of the cochlea. Results: 3D surfaces were obtained for the first 1.5 turns of 648 cochleae. Validation of our algorithm against the manually segmented ground truth obtained from 8 micro-CT scans showed good agreement, with 90 % area overlap and an average distance of 0.11 mm between the segmentation contours. The average cochlear duct length for the basal turn was 16.1 mm along the central path and 22.4 mm along the outer wall. The use of an intrinsic, observer-independent coordinate system and principal component analysis enabled unambiguous quantitative evaluation of the vertical trajectory of the cochlea, revealing only a weak correlation between the symmetry of the commonly used basal turn diameters (B-ratio of A and B diameters) and the profile of the vertical trajectory. Conclusion: A model-free segmentation algorithm can achieve similar accuracy as previously published methods relying on statistical shapes. Quantitative analysis of the vertical trajectory can replace the categorization into rollercoaster, sloping, or intermediate vertical trajectory types.

Inflammatory diet, gut microbiota and sensorineural hearing loss: a cross-sectional and Mendelian randomization study.

Aims: Inflammatory diets can trigger chronic inflammation and affect gut microbiota. However, the relationship between dietary preferences and sensorineural hearing loss (SNHL) remains unclear. This study aims to elucidate the relationship between different dietary preferences and sensorineural deafness. Methods: The Dietary Inflammation Index (DII) and SNHL were defined by data from the National Health and Nutrition Examination Survey (NHANES), and exploring their relationship. Using Mendelian randomization (MR) to analyze the relationship between 34 dietary preferences, 211 gut microbiota, and SNHL. Results: Smooth curve fitting indicated that the risk of SNHL increased with increasing DII score when the DII score was greater than 5.15. MR results suggest that a diet including both oily and non-oily fish can substantially reduce the risk of SNHL. Additionally, six specific gut microbiota were found to have significant causal relationship with SNHL. Conclusion: An inflammatory diet may increase the risk of developing SNHL. The observed relationship between fish consumption, gut microbiota, and SNHL suggests the existence of a gut-inner ear axis.

The role of selective serotonin reuptake inhibitors and tricyclic antidepressants in addressing reduction of Meniere's disease burden: A scoping review.

Objective: To assess the effect of selective serotonin reuptake inhibitors (SSRIs) and tricyclic antidepressants (TCAs) in reducing vertigo, tinnitus, and hearing loss among patients with Meniere's disease (MD). Data Sources: The following databases were utilized in this scoping review: Ovid Medline, PubMed-NCBI, CINAHL, Cochrane Library, Web of Science, and Clinicaltrials.gov. Method: Studies were identified through the following search phrases: "serotonin specific reuptake inhibitors" OR "tricyclic antidepressants" AND "Meniere's disease." References from included manuscripts were examined for possible inclusion of additional studies. Results: The literature search yielded 23 results, which were screened by three independent reviewers. Seventeen studies and three duplicates were excluded. An examination of references from the included studies yielded two additional publications. A total of four published studies assessing SSRIs and TCAs among 147 patients with MD were ultimately included. Four studies described significant reductions in vertigo attack frequency among patients treated with either SSRIs or TCAs compared to their pretreatment baseline. Three studies assessed the drugs' effects on hearing, of which none found a significant difference among patients treated with SSRIs or TCAs. One study found a significant decrease in patient-reported tinnitus following treatment with TCAs or SSRIs compared to their pretreatment baseline. Conclusions: Data exploring SSRIs and TCAs among patients with MD suggests that these medications may reduce the frequency of tinnitus and vertigo, although there was significant heterogeneity in outcome reporting. There remains a need for larger-scale prospective studies that emphasize objective data to evaluate their effectiveness in reducing common MD symptoms.

Sudden sensorineural hearing loss: audiological profile during the COVID-19 pandemic.

Introduction: Sudden sensorineural hearing loss (SSNHL) is an otological emergency that requires prompt recognition and intervention to prevent devastating impacts on people's lives. During the COVID-19 pandemic, sensory deprivations have been reported in patients positive for SARS-CoV-2 virus, including deleterious effects on the auditory pathway. This study aims to describe the audiological profile of individuals with SSNHL during the COVID-19 pandemic and to correlate hearing recovery in subgroups of individuals with or without COVID-19. Methods: Prospective cohort including patients diagnosed with SSNHL evaluated in a tertiary care center between March 2020 and September 2022. Hearing loss was confirmed with pure-tone and speech audiometry, with Speech Recognition Threshold (SRT) and word recognition score (WRS). Audiometric testing was performed at the moment of diagnosis, then 7, 30 and 120 days after diagnosis. The average degree of hearing loss was assessed by calculating the 4-frequency pure tone average (4fPTA). The investigation of COVID-19 included RT-PCR technique for the SARS-CoV-2 virus and collection of information regarding disease severity. A statistical analysis was performed using an analysis of covariance (ANCOVA) model to compare the 4fPTA between the four groups (with and without a history of COVID-19, unilateral and bilateral cases) at the end of the follow-up period. Results: Fifty-two patients with SSNHL were assessed, 40 (76.9%) with unilateral and 12 (23.1%) with bilateral hearing loss, totaling 64 ears included. Of those, 15 (28.8%) patients tested positive for SARS-CoV-2 and were symptomatic for COVID-19. Of all unilateral cases, 22.5% were seropositive and showed symptoms of COVID-19, a number that increased to 50% for bilateral cases. Comparing the COVID-19 positive groups, individuals with unilateral SSNHL went from 40 dB as their average 4fPTA at onset to 20 dB as their average 4fPTA after 120 days, whereas those with bilateral SSNHL went from an initial average of 60 dB to a final average of 66 dB. Although the 4fPTA value of individuals with unilateral SSNHL improved in 7 days, the mean values showed no significant difference between positive and negative groups. There was a higher incidence of bilateral simultaneous SSNHL in patients who had not been vaccinated against COVID-19 and who presented with symptoms of severe COVID-19. Conclusion: Infection with SARS-CoV-2 resulted in more severe SSNHL, in bilateral SSNHL, and in poorer recovery from SSNHL in bilateral cases. Bilateral SSNHL was seen more frequently in patients who had not received vaccination against COVID-19.

Differences Among Elderly Patients Having Asymmetric Sensorineural Hearing Loss With and Without Cerebellopontine Angle Tumors.

Objective: Asymmetric sensorineural hearing loss (ASNHL) exhibits a higher prevalence among the elderly compared to younger individuals, yet optimal management remains subject to ongoing debate. We aimed to elucidate the clinical disparities among elderly patients with ASNHL, distinguishing between those with and without cerebellopontine angle (CPA) tumors. Methods: A retrospective analysis was conducted on elderly patients (aged ≥65 years) diagnosed with ASNHL who underwent magnetic resonance imaging (MRI) between January 2012 and December 2022 at our tertiary referral center. Results: A total of 119 patients were enrolled, with a median age of 71 years (range: 65-89 years). Among them, 11 patients (9.2%) exhibited abnormal MRI findings. In the CPA tumors group, vestibular schwannoma was the most prevalent abnormality (63.6%), with a mean growth rate of 0.53 mm/year (range: 0-1.33 mm/year). The prevalence of CPA tumors in patients with diabetes mellitus (DM) and ASNHL was significantly lower than in those without DM (P = .021). Vertigo emerged as a significant associated symptom in cases with CPA tumors (P = .011). However, there were no significant differences in mean hearing thresholds or asymmetry of hearing loss at individual frequencies between the 2 groups. Conclusions: Elderly patients with ASNHL and vertigo should undergo radiological assessment. Patients with DM exhibit a lower prevalence of CPA tumors than those without DM, warranting careful observation and follow-up due to the limited diagnostic yield of MRI. No discernible differences in audiometric patterns were detected between patients with and without CPA tumors.

Ototoxicity-Alleviating and Cytoprotective Allomelanin Nanomedicine for Efficient Sensorineural Hearing Loss Treatment.

Sensorineural hearing loss (SNHL) represents a significant clinical challenge, predominantly attributed to oxidative stress-related mechanisms. In this work, we report an innovative antioxidant strategy for mitigating SNHL, utilizing synthetically engineered allomelanin nanoparticles (AMNPs). Empirical evidence elucidates AMNPs' profound capability in free radical neutralization, substantiated by a significant decrement in reactive oxygen species (ROS) levels within HEI-OC1 auditory cells exposure to cisplatin or hydrogen peroxide (H2O2). Comparative analyses reveal that AMNPs afford protection against cisplatin-induced and noise-induced auditory impairments, mirroring the effect of dexamethasone (DEX), a standard pharmacological treatment for acute SNHL. AMNPs exhibit notable cytoprotective properties for auditory hair cells (HCs), effectively preventing ototoxicity from cisplatin or H2O2 exposure, as confirmed by both in vitro assays and cultured organ of Corti studies. Further in vivo research corroborates AMNPs' ability to reverse auditory brainstem response (ABR) threshold shifts resulting from acoustic injury, concurrently reducing HCs loss, ribbon synapse depletion, and spiral ganglion neuron degeneration. The therapeutic benefits of AMNPs are attributed to mitigating oxidative stress and inflammation within the cochlea, with transcriptome analysis indicating downregulated gene expression related to these processes post-AMNPs treatment. The pronounced antioxidative and anti-inflammatory effects of AMNPs position them as a promising alternative to DEX for SNHL treatment.

Hearing loss secondary to variants in the OTOF gene.

OBJECTIVE: Genetic variants in the OTOF gene are responsible for non-syndromic hearing loss with an autosomal recessive inheritance pattern. The objective of our work was to evaluate the clinical characteristics of patients with biallelic pathogenic variants in OTOF and their evolution after treatment. METHODS: A cohort of 124 patients with prelingual hearing loss, studied from 1996 to 2023, was included in this study. A genetic analysis was conducted to identify the type and frequency of variants in the OTOF gene and their relation to the clinical characteristics of the patients. RESULTS: The homozygous p. Gln829* variant in the OTOF gene was detected in 3 probands (2.4 %) of a group 124 individuals with prelingual hearing loss. Another 6 family members to a total of 9 individuals were finally included. All presented with severe/profound bilateral sensorineural hearing loss of congenital onset. Three of these individuals were diagnosed with auditory neuropathy spectrum disorder. One individual passed the OAE test during the screening program, and since he did not have risk factors for hearing loss that would warrant ABR testing, this led to a delay in his hearing loss diagnosis. Four individuals underwent cochlear implants (three bilateral) with good functional outcomes. In three of them. However, in 17 familial cases with heterozygous variants, either no hearing loss was observed or it was within the expected range for their age. CONCLUSIONS: Hearing loss secondary to the p. Gln829* variant of the OTOF gene is relatively rare in our medical area. Its presence in homozygosity is the cause of severe/profound bilateral prelingual sensorineural hearing loss, responsible for auditory neuropathy with a good response to cochlear implantation.

Predictors of Delays in the Management of Idiopathic Sudden Sensorineural Hearing Loss.

OBJECTIVE: To study predictors of delayed presentation, diagnosis, and treatment of idiopathic sudden sensorineural hearing loss (ISSNHL). STUDY DESIGN: Retrospective medical chart review and patient telephone survey. SETTING: Tertiary medical center. SUBJECTS AND METHODS: All patients who initially presented or referred with unilateral ISSNHL between 2016 and 2020 were included. Associations between epidemiological, demographic, and socioeconomic profiles and delays in presentation, diagnosis and treatment were studied. RESULTS: A total of 518 patients were suitable for study inclusion. The total delay in the emergency department (ED) setup was a median (interquartile range, IQR) of 1 (0-1) day, 7 (6-12) days in a community otolaryngologist setup, and 15 (8-25) days in a general practitioner setup. Patients who presented to the ED first also had fewer diagnostic and treatment delays. Those who presented to a community otolaryngologist first had significantly longer presentation delay (5 [4-8] days p < 0.001) and significantly longer treatment delays (1 [1-3] days p < 0.001). Patients who presented to a general practitioner first had significantly longer presentation delays compared with ED presentation, and the longest diagnostic and treatment delays (3 [2-5], 8 [4-12] days, and 4 [2-7] days, p = < 0.01, p = <0.01, and p < 0.001, respectively). There was no association between socioeconomic status or demography and presentation, diagnostic, or treatment delays. CONCLUSION: Total delay in ISSNHL management is affected by the venue of the first medical encounter. General practitioners' level of awareness of the need for empiric steroidal treatment of ISSNHL without delay should be raised. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

Bilaterally positive head-impulse tests can differentiate AICA infarction from labyrinthitis.

Introduction: Video head-impulse tests (video-HITs) often fail to detect anterior inferior cerebellar artery (AICA) infarction due to peripheral and central vestibular system involvement. Anecdotal studies suggest that video-HITs may reveal bilateral impairment in AICA infarction. However, the diagnostic utility of video-HITs has not been established, particularly when compared to labyrinthitis, which accounts for the majority of acute audiovestibular syndrome (AAVS) cases. Methods: We reviewed the medical records of consecutive patients presenting with new-onset acute hearing loss and spontaneous vertigo (i.e., AAVS) between March 2018 and July 2023 at a tertiary hospital in South Korea. Video-HIT patterns were categorized as follows: (1) ipsilaterally positive, (2) contralaterally positive, (3) bilaterally normal, and (4) bilaterally positive. Results: Twenty-eight patients with AICA infarction (mean age ± standard deviation = 67 ± 15 years; 14 men) and 51 with labyrinthitis (63 ± 17 years, 26 men) were included in the analyses. Among the 28 patients with AICA infarction, 15 presented with AAVS in isolation, without other co-morbid neurologic deficits (15/28, 54%). The vestibulo-ocular reflex (VOR) gains of ipsilesional horizontal canals (HCs) ranged from 0.21 to 1.22 (median = 0.81, interquartile range [IQR] = 0.50-0.89). However, those for contralateral HC gain ranged from 0.57 to 1.19 (median = 0.89 [IQR = 0.73-0.97]). Collectively, HITs were bilaterally positive in 13 patients (including 12 patients with bilaterally positive HITs for the horizontal canal), normal in eight, ipsilesionally positive in six, and contralesionally positive in one patient with AICA infarction. The VOR gains were typically decreased ipsilaterally in 28 (28/51, 55%), normal in 17 (17/51, 33%), and decreased bilaterally in six patients with labyrinthitis (6/51, 12%). Logistic regression analysis revealed that bilaterally positive HITs (p = 0.004) and multiple vascular risk factors (p = 0.043) were more frequently associated with AICA infarction than labyrinthitis. Discussion: Among patients presenting with AAVS, bilaterally positive HITs can be indicative of AICA infarction in patients with multiple vascular risk factors.

From Spinal Ependymoma to Superficial Siderosis: A Bottom to Top Cause of Progressive Neurological Deterioration.

Superficial siderosis (SS) is an infrequent condition characterized by hemosiderin deposition in the central nervous system, resulting from chronic subarachnoid hemorrhage, often linked to dural mater diseases. Through a case report of a 50-year-old male with severe sensorineural hearing loss and newly diagnosed epilepsy, we explore SS triggered by a spinal ependymoma, diagnosed via resonance magnetic imaging (MRI). This case highlights the necessity of comprehensive neuroaxis imaging to identify treatable etiologies. The complexity of SS, with its varied clinical presentations, necessitates early detection and a multidisciplinary treatment approach. Despite limited treatment options and the uncertain efficacy of therapies like deferiprone, early intervention is crucial for mitigating irreversible neurological damage and enhancing patient prognosis.

Cochlear Implantation in a Patient with Intractable Tinnitus: A Case Report of an Endoscope-Assisted Approach.

Preservation of residual hearing and vestibular function is a crucial factor in cochlear implantation (CI), especially in patients with residual low-frequency hearing thresholds. We report a case of a patient who underwent unilateral endoscope-assisted CI with a challenging surgical view following rigorous posterior tympanotomy. A 53-year-old male presented with left-sided intractable tinnitus due to sudden sensorineural hearing loss that had occurred 10 years prior. Due to the abnormal location of the round window (RW), which was far more posterior and inferior than usual and impeded insertion of the electrode using the conventional RW approach, endoscope-assisted CI was performed. Pure-tone audiometry at 3 months after CI revealed satisfactory hearing thresholds. Furthermore, there was alleviation of the left-sided tinnitus, which was indicated by a marked decrease in both the subjective visual analog scale loudness and Tinnitus Handicap Inventory scores. With proper indications, we strongly recommend applying the RW approach with endoscopic assistance over conventional bony cochleostomy for the preservation of low-frequency hearing thresholds in cases where RW visualization is insufficient following posterior tympanotomy.

A sensorineural hearing loss harboring novel compound heterozygous variant in the TRIOBP gene: A case report.

Background: Autosomal recessive non-syndromic deafness-28 (DFNB28; OMIM #609823) specifically refers to prelingual sensorineural hearing loss (SNHL) resulting from homozygous or compound heterozygous mutations in the TRIO- and F-actin-binding protein, TRIOBP gene. In this report, we present a pediatric patient exhibiting novel compound heterozygous deleterious variants in the TRIOBP gene. Methods: The auditory brainstem response result revealed both left- and right-sided deafness with a threshold of 20 dB normal hearing level in the proband. A comprehensive trio whole exome sequencing (WES) using the Celemics G-Mendeliome Whole Exome Sequencing Panel was employed. Results: The WES analysis revealed compound heterozygous TRIOBP variants in the proband, namely c.1192_1195delCAACinsT/p.Gln398* classified as pathogenic and c.3661C > T/p.Arg1221Trp categorized as a variant of uncertain significance according to American College of Medical Genetics and Genomics guidelines. These variants are considered the most probable cause of the proband's SNHL. Conclusion: TRIOBP isoforms are predominantly expressed in the inner ear, contributing to the formation of stereocilia rootlets. Further investigations are required to fully understand the phenotypic variability and establish the pathogenicity of the identified variant in relation to the TRIOBP gene and SNHL.

Targeting the NLRP3 inflammasome in cochlear macrophages protects against hearing loss in chronic suppurative otitis media.

The activation of the NLRP3 inflammasome has been linked to several inflammatory and autoinflammatory diseases. Despite cases of potential hearing improvement in immune-mediated diseases, direct evidence of the efficacy of targeting this mechanism in the inner ear is still lacking. Previously, we discovered that macrophages are associated with Sensorineural Hearing loss (SNHL) in Chronic Suppurative Otitis Media (CSOM), the leading cause of this permanent hearing loss in the developing world and incurring costs of $4 to $11 billion dollars in the United States. However, the underlying mechanism remained unknown. Here, we investigate how macrophages drive permanent hearing loss in CSOM. We first confirmed the occurrence of NLRP3 inflammasome activation in cochlear macrophages in CSOM. We then revealed that Outer Hair Cells (OHCs) were protected in CSOM by macrophage depletion and subsequently confirmed the same protection in the NLRP3 knockout condition. Furthermore, we showed that therapeutic inhibition of NLRP3 inflammasome activation and downstream inhibition of IL-1ß protects OHCs in CSOM. Collectively, our data demonstrates that the main driver for hearing loss in CSOM is NLRP3 inflammasome activation in cochlear macrophages and this is therapeutically targetable, leading the way for the development of interventions to prevent the leading cause of permanent hearing loss and a costly disease in the developed world.

Exploring the application of stem cell technology in treating sensorineural hearing loss.

Sensorineural deafness mainly occurs due to damage to hair cells, and advances in stem cell technology, especially the application of induced pluripotent stem cells (iPSCs) and adult stem cells, provides new possibilities for hair cell regeneration. This review describes the basic knowledge of stem cells and their important applications in regenerative medicine, as well as recent progress in stem cell research in the field of hair cell regeneration, especially the induced differentiation of hair-like cells. At the same time, we also point out the challenges facing current research, including differentiation efficiency, cell stability issues, and treatment safety and long-term efficacy considerations. Finally, we look forward to the direction of future research, and emphasize the importance of the cell differentiation mechanism, simulation of the inner ear microenvironment, safety assessment, and personalized treatment strategies. In conclusion, despite many challenges, stem cell technology has shown great potential in the field of hearing research and is expected to bring revolutionary treatment options for patients with sensorineural hearing loss in the future.

Cochlear implantation in a familial rare syndromic ossification-related deafness and literature review.

BACKGROUND: Kenny-Caffey Syndrome type 2 (KCS2) is a genetic disease affecting bone metabolism. However, cochlear implantation (CI) results have yet to be published in detail. OBJECTIVE: This study presents the gene, clinical characteristics, surgical outcomes, and literature review of 2 patients with sensorineural hearing loss related to KCS2. To enhance diagnostic detection and accuracy, we also compare the differential diagnosis between KCS2, otosclerosis, and Cogan's syndrome (CS). METHODS: Prior to CI, patients with KCS2 and CS underwent comprehensive audiological and radiological evaluations. Postoperative auditory speech outcomes and impedance values were recorded and analyzed statistically. A systematic search of the literature was conducted to summarize clinical characteristics. RESULTS: Patients diagnosed with KCS2 exhibit more pronounced changes in the inner ear. The impedance values in the KCS2 cohort were considerably higher (Mean = 12.13 kΩ) than those with CS (Mean = 8.8 kΩ) one year post-activation. The literature review exhibits the clinical manifestations associated with KCS2. CONCLUSION: CI is an effective treatment for KCS2 to restore hearing loss. More frequent programming and accurate adjustment of stimulation is of great necessity. A thorough examination, including temporal bone HRCT, 3D-MRI, audiological evaluations, and whole-exome sequencing, is essential for the diagnosis and treatment of KCS2.

The relationship between changes in functional connectivity gradients and cognitive-emotional disorders in sudden sensorineural hearing loss.

Sudden sensorineural hearing loss, a prevalent emergency in otolaryngology, is known to potentially precipitate cognitive and emotional disorders in affected individuals. Extensive research has documented the phenomenon of cortical functional reorganization in patients with sudden sensorineural hearing loss. However, the potential link between this neural functional remodelling and cognitive-emotional disorders remains unclear. To investigate this issue, 30 bilateral sudden sensorineural hearing loss patients and 30 healthy adults were recruited for this study. We collected clinical data and resting-state functional magnetic resonance imaging data from the participants. Gradient mapping analysis was employed to calculate the first three gradients for each subject. Subsequently, gradient changes in sudden sensorineural hearing loss patients were compared with healthy controls at global, regional and network levels. Finally, we explored the relationship between gradient values and clinical variables. The results revealed that at the global level, sudden sensorineural hearing loss did not exhibit significant differences in the primary gradient but showed a state of compression in the second and third gradients. At the regional level, sudden sensorineural hearing loss patients exhibited a significant reduction in the primary gradient values in the temporal pole and ventral prefrontal cortex, which were closely related to neuro-scale scores. Regarding the network level, sudden sensorineural hearing loss did not show significant differences in the primary gradient but instead displayed significant changes in the control network and default mode network in the second and third gradients. This study revealed disruptions in the functional hierarchy of sudden sensorineural hearing loss, and the alterations in functional connectivity gradients were closely associated with cognitive and emotional disturbances in patients. These findings provide new evidence for understanding the functional remodelling that occurs in sudden sensorineural hearing loss.