Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-08-06DOI: 10.1097/MAO.0000000000004553
Andrea Vambutas, Virginia Mullooly, Sharon Weber, Shresh Pathak
{"title":"Corticosteroid-Resistant and Corticosteroid-Dependent AIED Patients Treated With Anakinra: A Decade of Experience.","authors":"Andrea Vambutas, Virginia Mullooly, Sharon Weber, Shresh Pathak","doi":"10.1097/MAO.0000000000004553","DOIUrl":"10.1097/MAO.0000000000004553","url":null,"abstract":"<p><strong>Objective: </strong>Patients with autoimmune inner ear disease (AIED) are particularly vulnerable if they stop responding to corticosteroids or if they are dependent on high-dose corticosteroids to maintain hearing, as they have no therapeutic alternatives. We initially demonstrated the efficacy of anakinra in a small open-label clinical trial in corticosteroid-resistant AIED and are conducting a larger phase 2 placebo-controlled clinical trial. A large number of screened patients were ineligible or refused participation; however, for those we could obtain insurance coverage, we have used anakinra to improve and/or stabilize hearing.</p><p><strong>Study design: </strong>Retrospective case review.</p><p><strong>Setting: </strong>Tertiary referral center.</p><p><strong>Patients/intervention: </strong>A total of 49 patients representing three cohorts were treated with anakinra from 2013 to 2023, and hearing results followed over time: cohort A, corticosteroid-resistant AIED patients (n = 28); cohort B, corticosteroid-dependent AIED patients (n = 12); and cohort C, AIED patients who have participated in our anakinra trials with hearing improvement but were unable to maintain hearing off drug (n = 9).</p><p><strong>Outcome measures/results: </strong>The anakinra response rate was 46% for cohort A, 83% for cohort B, and 78% for cohort C. In cohort A, a positive durable response was measured by hearing improvement by PTA: in this cohort of 13 patients, 14 ears improved by an average of 14 dB PTA. If we adopt a more conservative measure of improvement in both the PTA and WRS domains, eight ears in eight patients improved by 22% in WRS in this PTA-responsive cohort. After removing three responders who exhibited a WRS ceiling effect and three patients deemed nonresponders as they improved by WRS only, the overall rate of improvement drops to 32% or 7 of 22 evaluable patients. In cohort B, a positive response was considered to achieve hearing stability, with a concomitant reduction in corticosteroid dose by at least 50%. In cohort C, a positive response was to achieve hearing stability.</p><p><strong>Conclusion: </strong>Anakinra seems to be efficacious in some patients with corticosteroid-resistant and corticosteroid-dependent AIED.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1059-1069"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-08-01DOI: 10.1097/MAO.0000000000004618
Saúl Astray-Gómez, Ana Belén Castilla-Jiménez, Vanesa Pérez-Guillén
{"title":"Eslicarbazepine as a Treatment for Vestibular Paroxysmia: Preliminary Results.","authors":"Saúl Astray-Gómez, Ana Belén Castilla-Jiménez, Vanesa Pérez-Guillén","doi":"10.1097/MAO.0000000000004618","DOIUrl":"10.1097/MAO.0000000000004618","url":null,"abstract":"<p><strong>Objective: </strong>To assess the potential of eslicarbazepine as an effective and tolerable treatment for vestibular paroxysmia compared to standard drugs.</p><p><strong>Patients: </strong>A cohort of five patients fulfilling criteria of probable or definite vestibular paroxysmia were included in the study.</p><p><strong>Intervention: </strong>Patients were treated with eslicarbazepine in daily regime with an initial dose of 400 mg.</p><p><strong>Main outcome measure: </strong>Reduction in number of crises of vestibular paroxysmia per week was measured according to patients' self-report, to evaluate clinical efficacy.</p><p><strong>Results: </strong>Mean treatment period was 10.6 months. Mean follow-up period was 21 months. Four of the five patients treated with eslicarbazepine achieved a total response with one of the five patients having a partial response with a mean reduction of 70% in the number of crises. The response to the withdrawal of the drug was highly variable in each patient. The most common adverse effects identified in the cohort were nausea, vomiting, and mild biochemical abnormalities. Two of the five patients experienced stochastic effects, which motivated drug removal.</p><p><strong>Conclusion: </strong>Eslicarbazepine shows good preliminary results achieving symptomatic relief in patients diagnosed with vestibular paroxysmia. Its enhanced safety profile with less adverse effects and drug interactions with respect to standard therapies could facilitate therapeutical adherence without detriment of its efficacy.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e443-e446"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789661","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-08-04DOI: 10.1097/MAO.0000000000004617
Milan P Fehrenbach, Asher C Park, Anthony A Mangino, Matthew L Bush
{"title":"Evaluating the Distribution of Otological and Neurotological Surgeons in the United States With the Social Vulnerability Index.","authors":"Milan P Fehrenbach, Asher C Park, Anthony A Mangino, Matthew L Bush","doi":"10.1097/MAO.0000000000004617","DOIUrl":"10.1097/MAO.0000000000004617","url":null,"abstract":"<p><strong>Objective: </strong>Assess the relationship between neighborhood-level social determinants of health (SDoH) and the distribution of otology and neurotology surgeons (ONSs) with the Social Vulnerability Index (SVI).</p><p><strong>Study design: </strong>Cross-sectional study.</p><p><strong>Setting: </strong>United States.</p><p><strong>Methods: </strong>A dataset of US-based ONS and their practice addresses were obtained from the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and American Neurotology Society (ANS) directories. The overall SVI and its four subthemes, socioeconomic status (SES), household composition and disability status (HH), racial-ethnic minority status (RE), and housing and transportation status (HT), were used to quantify neighborhood-level disadvantage at the census-tract level. The distance from each census-tract to the nearest ONS was calculated. Subsequently, multivariable and univariable linear regression analyses were performed to assess associations between SVI quartiles and distance to the closest ONS.</p><p><strong>Results: </strong>This study included 415 otology and neurotology surgeons. The most vulnerable quartile of overall SVI, SES, and HH was associated with greater distances to the nearest otologist ( p < 0.001). Conversely, the most vulnerable quartile of RE and HT were associated with decreased distance to the nearest ONS ( p < 0.001).</p><p><strong>Conclusion: </strong>Regions experiencing greater social vulnerability across specific SDoH are further from ONS. SVI may be useful in identifying regions deficient in ONSs while characterizing the factors that contribute to this disparity. The projected workforce deficit highlights the need for proactive measures to ensure equitable access to ONS care, guiding future research and policy efforts aimed to improve healthcare equity and access.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1015-1021"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789662","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-07-10DOI: 10.1097/MAO.0000000000004597
David Shimunov, Huseyin Isildak
{"title":"Impact of the COVID-19 Pandemic and Vaccination on Bell's Palsy: A Retrospective Comprehensive Analysis Using TriNetX Data.","authors":"David Shimunov, Huseyin Isildak","doi":"10.1097/MAO.0000000000004597","DOIUrl":"10.1097/MAO.0000000000004597","url":null,"abstract":"<p><strong>Background: </strong>Bell's palsy (BP), a sudden onset of facial paralysis, has been reported in patients with COVID-19. The relationship between COVID-19 infection and BP remains unclear, as does the impact of COVID-19 vaccination on its incidence. This study aims to assess the occurrence of BP before and after the COVID-19 pandemic and to explore any potential effects of COVID-19 vaccination on BP cases.</p><p><strong>Methods: </strong>This study utilized data from the TriNetX global health research network, specifically the US Collaborative Network of 68 healthcare organizations, encompassing 99,852,106 patients with ICD codes from 2016 to 2024. BP cases were identified using ICD-10 code G51.0, while COVID-19 vaccinations and infections were identified using CPT codes and TNX Curated 9088, respectively. We conducted an incidence and prevalence analysis of BP cases annually over the 8-year period to detect trends and compared BP rates between prepandemic (2016-2020) and postpandemic (2020-2024) periods using odds ratios. Additionally, we assessed BP incidence among COVID-19 patients and performed a subgroup analysis on vaccinated individuals to evaluate the impact of vaccination on BP incidence. To reduce potential confounding, we employed propensity score matching using the greedy nearest neighbor method available in the TriNetX platform, matching patients on age, sex, race, type 2 diabetes mellitus, and vascular commodities. Statistical analyses were executed within the TriNetX platform and supplemented by online AI tools using Python.</p><p><strong>Results: </strong>The analysis of BP cases from 2016 to 2024 revealed an upward trend in both incidence and prevalence. The incidence proportion rose from 48 cases per 100,000 people in 2016-2017 to 69 cases per 100,000 by 2023-2024, with a significant increase noted from 2021 onwards. Similarly, the incidence rate increased from 0.00000155 to 0.00000333 over the same period. Prevalence also showed a steady rise, from 228 to 434 cases per 100,000 people. Trend analyses confirmed these increases as statistically significant, with p -values indicating upward trends in incidence and prevalence. Comparing prepandemic (2016-2020) to postpandemic (2020-2024) periods, there was a significant rise in incidence proportions ( p -value ≈ 0.0074) and prevalence rates ( p -value = 0.0180). Among COVID-positive patients, the incidence proportion remained stable, while prevalence increased, indicating a higher burden compared to the general population. After propensity score matching, vaccinated individuals exhibited a significantly lower risk of BP compared to unvaccinated individuals (hazard ratio: 0.723, 95% CI: 0.618-0.84, p < 0.001).</p><p><strong>Conclusion: </strong>This highlights a significant increase in the incidence and prevalence of BP during the COVID-19 pandemic years compared to prepandemic levels. While the incidence of BP among vaccinated individuals showed a gradual increase, when m","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1164-1170"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675433","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-07-10DOI: 10.1097/MAO.0000000000004594
Ke Qiu, Junhong Li, Ping An, Lin Lou, Tianyi Gu, Xiuli Shao, Min Chen, Minzi Mao, Wendu Pang, Yongbo Zheng, Di Deng, Wei Xu, Jianjun Ren, Yu Zhao
{"title":"Genome- and Exome-Wide Identification of Common-to-Rare Variants Associated with Middle Ear Cholesteatoma.","authors":"Ke Qiu, Junhong Li, Ping An, Lin Lou, Tianyi Gu, Xiuli Shao, Min Chen, Minzi Mao, Wendu Pang, Yongbo Zheng, Di Deng, Wei Xu, Jianjun Ren, Yu Zhao","doi":"10.1097/MAO.0000000000004594","DOIUrl":"10.1097/MAO.0000000000004594","url":null,"abstract":"<p><strong>Hypothesis: </strong>To investigate the genetic susceptibility of middle ear cholesteatoma (MEC) and construct an MEC risk prediction model by integrating genetic risk with clinical factors.</p><p><strong>Background: </strong>MEC represents a relatively rare disorder that is associated with high morbidity, whereas its genetic etiology remains poorly understood.</p><p><strong>Methods: </strong>Using genetic data from the UK Biobank (UKB), we performed both genome-wide association study (GWAS) and exome-wide association study (ExWAS) involving 702 MEC patients and 491,503 controls. Gene-based and gene set-based association studies were then performed to identify risk genes and gene sets of MEC, respectively. In addition, logistic regression models were applied to identify clinically significant MEC-associated diseases, of which the genetic and causal relationships with MEC were further characterized using linkage disequilibrium score regression, genetic analysis incorporating pleiotropy and annotation, and Mendelian randomization. Moreover, logistic regression models were employed to construct MEC risk prediction models by integrating genetic risk with clinical factors.</p><p><strong>Results: </strong>Our study identified 159 common variants across 8 genomic loci and 39 rare variants spanning 17 genomic regions that were significantly associated with MEC, with PLD1 being prioritized as the top-ranked MEC candidate target gene. Additionally, 10 different types of diseases showed significant associations with MEC, but no inconclusive genetic or causal relationship was established between them. Moreover, we successfully constructed a high-performance MEC risk prediction model with an area under the curve of 0.704, showing the potential for clinical application.</p><p><strong>Conclusions: </strong>These findings advance our understanding of the genetic susceptibility of MEC and provide insights into its risk prediction, thus contributing to improved MEC prevention and management.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e403-e411"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675431","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-07-21DOI: 10.1097/MAO.0000000000004595
Melanie L Gilbert, Rebecca M Lewis, Mickael L D Deroche, Nicole T Jiam, Patpong Jiradejvong, Jonathan Mo, Daniel L Cooke, Charles J Limb
{"title":"Speech, Timbre, and Pitch Perception in Cochlear Implant Users With Flat-Panel CT-Based Frequency Reallocations: A Longitudinal Prospective Study.","authors":"Melanie L Gilbert, Rebecca M Lewis, Mickael L D Deroche, Nicole T Jiam, Patpong Jiradejvong, Jonathan Mo, Daniel L Cooke, Charles J Limb","doi":"10.1097/MAO.0000000000004595","DOIUrl":"10.1097/MAO.0000000000004595","url":null,"abstract":"<p><strong>Hypothesis: </strong>To determine whether chronic use of experimental computed tomography (CT)-based frequency allocations would improve cochlear implant (CI) user performance in the areas of speech and music perception, as compared to the clinical default frequency mapping provided by the CI manufacturer.</p><p><strong>Background: </strong>CIs utilize default frequency maps to distribute the frequency range important for speech perception across their electrode array. Clinical default frequency maps do not address the significant frequency-place mismatch that is inherent after cochlear implantation, nor the variability between individual anatomy or array lengths. Recent research has utilized postoperative high-resolution flat-panel CT imaging to measure the precise location of electrode contacts within an individual's cochlea, in order to generate a custom frequency map and decrease the frequency-place mismatch.</p><p><strong>Methods: </strong>A cohort of 10 experienced CI users (14 CI ears) was recruited to receive CT scans and then use an experimental CT-based frequency map for 1 month. The efficacy of these maps was measured using a battery of speech and music tests.</p><p><strong>Results: </strong>No change in speech or music performance between the Experimental and Clinical Maps was found at the group level, although there was large variability within the cohort. Greater benefit from the Experimental Map on speech in quiet tasks was correlated with better electrode array alignment in the apical (low frequency) region (rho 14 = -0.55 to -0.72, p < 0.05).</p><p><strong>Conclusion: </strong>This application of strict CT-based mapping was most beneficial for CI users with the least amount of apical-mid array frequency-place mismatch, and least beneficial for CI users with overly deep or shallow insertions. Results may be limited by long acclimation periods to clinical default frequency maps prior to CT map usage, intervention bias, and small sample size.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e384-e394"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675436","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Asymmetrical Auditory Dysfunction as a Potential Nonmotor Lateralizing Sign in Parkinson's Disease: A Case-Control Study.","authors":"Reham Mamdouh Lasheen, Mohamed Nasser Elsheikh, Mohamed Osama Tomoum","doi":"10.1097/MAO.0000000000004598","DOIUrl":"10.1097/MAO.0000000000004598","url":null,"abstract":"<p><strong>Introduction: </strong>Parkinson's disease (PD) is a complex neurodegenerative disorder that often presents with unilateral motor symptoms at the onset. While motor symptoms such as tremor, bradykinesia, rigidity, and postural instability are the signs of PD, nonmotor symptoms also significantly impact the patient's quality of life. One such nonmotor symptom is auditory dysfunction, which has been increasingly recognized as a feature of PD.</p><p><strong>Objectives: </strong>Our purpose is to evaluate auditory impairment in PD patients and its asymmetry as a biomarker to differentiate PD from other neurodegenerative disorders that do not exhibit this lateralized auditory dysfunction.</p><p><strong>Subjects and methodology: </strong>Our study included 60 patients with parkinsonism \"the study group\", and 40 healthy individuals \"the control group.\" All participants underwent three auditory tests: pure tone audiometry (PTA), auditory brainstem response (ABR), and otoacoustic emissions (OAE).</p><p><strong>Results: </strong>PTA thresholds were significantly higher in the PD group, especially on the affected side. ABR absolute latencies and interpeak latencies were significantly delayed in PD, with the delays being more pronounced on the affected side. The distortion product OAEs showed a significant decrease in amplitude on the affected side compared to the nonaffected side. Furthermore, its amplitude was highly significantly reduced when compared to the control group.</p><p><strong>Conclusion: </strong>The asymmetry of the audiological responses in PD patients could be a valuable nonmotor marker of PD, enhancing our understanding of its broader impact on sensory systems and aiding in more precise diagnosis and monitoring of the disease.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e370-e376"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675426","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-07-07DOI: 10.1097/MAO.0000000000004600
Guangwei Zhou, Hannah Peterson, Alice Yun, Jacob Brodsky
{"title":"Vestibular Dysfunction in Pediatric Patients With Congenital Cytomegalovirus Infection and Hearing Loss: Occurrence and Characteristics.","authors":"Guangwei Zhou, Hannah Peterson, Alice Yun, Jacob Brodsky","doi":"10.1097/MAO.0000000000004600","DOIUrl":"10.1097/MAO.0000000000004600","url":null,"abstract":"<p><strong>Objective: </strong>To explore the occurrence of vestibular dysfunction in pediatric patients with congenital cytomegalovirus (CMV) infection and define the characteristics of vestibular loss in this population.</p><p><strong>Study design: </strong>Retrospective study with controls.</p><p><strong>Setting: </strong>Tertiary pediatric referral center.</p><p><strong>Patients: </strong>Pediatric patients with hearing loss associated with congenital CMV infection and GJB2 mutation(s).</p><p><strong>Interventions: </strong>Balance and vestibular evaluation.</p><p><strong>Main outcome measures: </strong>Normal versus abnormal results of laboratory vestibular testing.</p><p><strong>Results: </strong>A total of 50 pediatric patients with congenital CMV infection, average age = 3.4 years (ranging from 7 mo to 12 yr, SD = 2.9 yr), underwent vestibular workup, including Videonystagmography, rotary chair test, and cervical vestibular evoked myogenic potential test. Of these children with CMV infection, 35 (70%) had bilateral vestibular loss, 7 (14%) had unilateral loss, and only 8 (16%) had a normal vestibular workup. In contrast, among the 48 pediatric patients without a history of CMV infection and with hearing loss associated with GJB2 mutation(s), only 9 (19%) had bilateral vestibular loss, 2 (4%) had unilateral loss, while 37 (77%) had a normal vestibular workup. Developmental/motor delay was documented in 35 (70%) patients with congenital CMV infection and hearing loss.</p><p><strong>Conclusions: </strong>Children with congenital CMV infection are at high risk for vestibular loss, which is frequently bilateral, impacting the semicircular canals and otolith organs, not just the well-known hearing loss. A comprehensive balance and vestibular workup is warranted for children with congenital CMV infection with the goal of early identification of vestibular dysfunction and earlier management.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1124-1128"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-07-22DOI: 10.1097/MAO.0000000000004591
Levent Sennaroglu, Mounika Naidu Boya, Miriam I Redleaf
{"title":"Histopathological Factors Contributing to Recurrent Meningitis in Inner Ear Malformations.","authors":"Levent Sennaroglu, Mounika Naidu Boya, Miriam I Redleaf","doi":"10.1097/MAO.0000000000004591","DOIUrl":"10.1097/MAO.0000000000004591","url":null,"abstract":"<p><strong>Objective: </strong>To analyze histopathological factors contributing to recurrent meningitis in patients with inner ear malformations (IEMs) through examination of two cases from the archives of an otopathology laboratory.</p><p><strong>Patients: </strong>Two cases with documented IEMs and a history of recurrent meningitis were analyzed from archived human temporal bone specimens in July 2024.</p><p><strong>Intervention: </strong>Histopathological examination and analysis of temporal bone specimens to identify anatomical defects and infection pathways.</p><p><strong>Main outcome measure: </strong>Identifying specific anatomical defects and pathways contributing to recurrent meningitis in IEM cases.</p><p><strong>Results: </strong>Case 1 demonstrated an incomplete partition type I (IP-I) malformation with a stapes footplate fistula, which provided a direct pathway for infection from the middle ear to the inner ear spaces. Case 2 revealed an incomplete partition type III (IP-III) malformation with a deficient otic capsule, presenting multiple potential routes for infection spread due to irregular erosion and minimal bone barrier between middle and inner ear spaces. Both cases resulted in fatal meningitis despite medical intervention.</p><p><strong>Conclusions: </strong>Recurrent meningitis in IEMs can occur through different pathophysiological mechanisms: either through a discrete stapes footplate fistula or via a deficient otic capsule with multiple potential infection pathways. Management strategies should be tailored to the specific anatomical defect, with surgical intervention focusing on defect repair in stapes footplate fistulas and consideration of subtotal petrosectomy in cases of deficient otic capsule. Vaccination and careful selection of surgical procedures are crucial preventive measures.</p><p><strong>Level of evidence: </strong>IV.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e395-e398"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144691176","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Otology & NeurotologyPub Date : 2025-10-01Epub Date: 2025-06-25DOI: 10.1097/MAO.0000000000004578
Mohanad Almashhadani, Anna Lisa Giannuzzi, Mohammed Alkhateeb, Elisabetta Rebecchi, Francesco Di Pierro, Mario Sanna
{"title":"Hearing and Vestibular Outcomes in Patients Undergoing Labyrinthectomy and Cochlear Implant in End-Stage Menière's Disease.","authors":"Mohanad Almashhadani, Anna Lisa Giannuzzi, Mohammed Alkhateeb, Elisabetta Rebecchi, Francesco Di Pierro, Mario Sanna","doi":"10.1097/MAO.0000000000004578","DOIUrl":"10.1097/MAO.0000000000004578","url":null,"abstract":"<p><strong>Objective: </strong>To analyze the results of hearing, vertigo, and tinnitus after simultaneous or sequential labyrinthectomy and cochlear implant (CI) and evaluate the effectiveness of end-stage Menière's disease (MD) treatment.</p><p><strong>Study design: </strong>Retrospective study.</p><p><strong>Method: </strong>The medical records of 39 patients who underwent labyrinthectomy and CI ipsilaterally for intractable vertigo and hearing loss with preoperative and postoperative documents were evaluated. Auditory outcomes were assessed with pure tone and speech audiometry (word test, closed set) and compared with the preoperative audiometric evaluation. Dizziness was graded according to the Dizziness Handicap Inventory Questionnaire (DHI). Tinnitus outcomes were assessed by the Tinnitus Handicap Inventory (THI).</p><p><strong>Results: </strong>Paired t tests revealed significant improvements in post-CI pure tone average ( p = 0.0001), speech discrimination ( p = 0.009), and tinnitus ( p = 0.0001). Fifty-eight percent of patients had complete resolution of the vestibular symptoms in the operated ear, while 42% showed little or no improvement. However, over half (54.5%) of the patients with no or partial improvement were over 65 years old, as evidenced by the postoperative DHI. Patients demonstrated significant reductions in tinnitus as indicated by a significant improvement on the THI ( p = 0.0001).</p><p><strong>Conclusion: </strong>Patients with vertigo and profound hearing loss who have end-stage Menière's disease (MD) or secondary Menière's disease can benefit from labyrinthectomy with a cochlear implant (CI). Older individuals should be treated with caution due to the possibility of chronic instability. Cochlear implantation greatly benefits both tinnitus suppression and hearing restoration.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1077-1085"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675432","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}