The journal of international advanced otology最新文献

{"title":"Next-Generation Sequencing to Detect Mutations for the Molecular Diagnosis of Auditory Neuropathy Spectrum Disorder in a Chinese Series.","authors":"Fan Yuan, Zhe Chen, Yanmei Zhang, Yueqi Wang, Jing Xie, Yuhe Liu","doi":"10.5152/iao.2025.241690","DOIUrl":"10.5152/iao.2025.241690","url":null,"abstract":"<p><strong>Background: </strong>Auditory neuropathy spectrum disorder (ANSD) encompasses a range of hearing impairments caused by disrupted sound transmission from the cochlea to the brain. The atypical symptoms or signs of ANSD often complicate both diagnosis and treatment. To improve the identification of lesion sites and gain insights into the disease mechanisms, we employed next-generation sequencing (NGS) to detect mutations in ANSD-related genes.</p><p><strong>Methods: </strong>We studied 23 patients with ANSD from non-consanguineous Chinese families. Clinical data were collected and analyzed from medical records. Genomic DNA was extracted from blood samples, followed by whole-exome capture, NGS, and confirmation through bidirectional Sanger sequencing.</p><p><strong>Results: </strong>Based on ANSD classification, 10 patients had non-syndromic (NS) ANSD, 7 had syndromic peripheral neuropathy, and 6 had syndromic central neuropathy. Thirteen novel variants (8 missense variants and 1 deletion variant) and 21 previously reported variants were identified in 23 patients. Several cases exhibited mild-to-profound hearing loss.</p><p><strong>Conclusion: </strong>Multiple genes have been identified to cause ANSD. Next-generation sequencing plays a role in differentiating ANSD from other clinical conditions and identifying it as a symptom of syndromic ANSD. Molecular diagnosis offers valuable insights into prognosis and helps guide treatment strategies.</p>","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 1","pages":"1-8"},"PeriodicalIF":0.0,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12128310/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144129883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transmeatal Section of the Array in Cochlear Implant Explantation and Reimplantation: How I Do It.","authors":"Stefano Concheri, Elisabetta Zanoletti, Sebastiano Franchella","doi":"10.5152/iao.2025.241771","DOIUrl":"10.5152/iao.2025.241771","url":null,"abstract":"<p><strong>Background: </strong>Cochlear implant explantation and reimplantation (ERI) are rarely required, but the widespread use of implants has made it a procedure that every otologic surgical center must address. Although generally described as safe, ERI presents specific surgical challenges, particularly concerning the replacement of the array.</p><p><strong>Methods: </strong>The transmeatal section of the array as the initial step of cochlear implant ERI is proposed and described.</p><p><strong>Results: </strong>A brief case series did not report any specific complications.</p><p><strong>Conclusion: </strong>The transmeatal section of the array allows for the early and safe removal of the cable portion within the mastoid, enabling the surgeon to address scar tissue and new bone formation without the hindrance of the cable and preventing the premature removal of the array in the cochlea.</p>","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 1","pages":"1-3"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12128287/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144129898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cochlear Implantation: A Treatment Approach of Hearing Loss in Superficial Siderosis.","authors":"Enrico Liaci, Francesco Maccarrone, Maurizio Negri, Martina Silvestri, Benincasa Paola, Maurizio Guida, Rosa Maria Minniti, Sauro Tassi, Filippo Di Lella","doi":"10.5152/iao.2025.241682","DOIUrl":"10.5152/iao.2025.241682","url":null,"abstract":"<p><strong>Background: </strong>Superficial siderosis (SS) is a chronic condition characterized by progressive hemosiderin accumulation in the central nervous system (CNS) due to chronic subarachnoid hemorrhage. Common clinical features include progressive bilateral sensorineural hearing loss (SNHL), cerebellar ataxia and myelopathy. The aim of the study was to analyze the clinical presentation, management, and outcomes of patients affected by SS with bilateral severe-to-profound hearing loss and treated with cochlear implantation (CI), with a particular focus on the course of hearing benefit.</p><p><strong>Methods: </strong>A retrospective monocentric review was performed, including patients affected by CNS SS and profound SNHL that underwent CI between January 2012 and December 2021. Outcomes were assessed by comparing pre- and post-operative tonal and vocal hearing thresholds together with verbal perception tests.</p><p><strong>Results: </strong>A total of 4 patients were identified, with a mean follow-up time of 48.25 months. All patients showed significant improvement of hearing threshold and verbal perception in the first 6 months after surgery (preoperative mean aided PTA of 58.3 dB with a mean open-set speech perception score of 20.3% versus postoperative mean PTA in bimodal stimulation of 41.1 dB and open-set speech perception score of 60.1%). At the last follow-up evaluation, despite progressive deterioration of auditory performances, all patients were users and maintained subjective benefit from CIs.</p><p><strong>Conclusion: </strong>Patients affected by SS who underwent CI showed a significant improvement in auditory and auditory-verbal performances in the first post-operative period. Despite a progressive decline of hearing benefit due to retrocochlear disease progression, CI represents valid support for lip-reading and spatial orientation even in the advanced stages of the disease.</p>","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 1","pages":"1-5"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12128349/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144129824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erratum.","authors":"","doi":"10.5152/jiao.2025.2500001","DOIUrl":"10.5152/jiao.2025.2500001","url":null,"abstract":"","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 3","pages":"1"},"PeriodicalIF":0.0,"publicationDate":"2025-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12147345/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144304136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Role of Non-Invasive Imaging in Differentiating LCH and Cholesteatoma in Clinical Practice.","authors":"Ammara Jamil","doi":"10.5152/iao.2025.241742","DOIUrl":"https://doi.org/10.5152/iao.2025.241742","url":null,"abstract":"","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 2","pages":"1-2"},"PeriodicalIF":0.0,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12001534/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144015920","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Temporal Bone Magnetic Resonance Imaging in Sudden Sensorineural Hearing Loss: Low Frequency Versus Other Types.","authors":"Yun Ji Lee, Doyeon Kim, Joong Ho Ahn, Hong Ju Park, Jong Woo Chung, Woo Seok Kang","doi":"10.5152/iao.2025.241490","DOIUrl":"https://doi.org/10.5152/iao.2025.241490","url":null,"abstract":"<p><strong>Background: </strong>Gadolinium-enhanced magnetic resonance imaging (MRI) is the gold standard for diagnosing vestibular schwannoma (VS). This study aimed to compare the incidence of VS among patients with low-frequency, high-frequency, and flat-type sudden sensorineural hearing loss (SSNHL) and to assess the effectiveness of MRI for each SSNHL type.</p><p><strong>Methods: </strong>We analyzed 755 patients diagnosed with SSNHL at a single tertiary center between January 2014 and March 2020. All underwent temporal bone MRI. Logistic regression was used to determine the associations of SSNHL types with VS. Additionally, we conducted a correlation analysis to examine the relationship between word recognition scores (WRS) and the size of tumors measured on MRI at the time of diagnosis.</p><p><strong>Results: </strong>Magnetic resonance imaging identified VS in 23 of 755 cases (3.0%). Of the patients, 123 (16.3%) had low-frequency SSNHL; none of these patients had VS. The incidence of VS differed significantly between the low-frequency SSNHL group and other SSNHL types (P < .001). A significant negative correlation was found between initial tumor size and WRS (r2 = 0.2905, P=.008).</p><p><strong>Conclusion: </strong>The prevalence of VS was rare in patients with low-frequency SSNHL. The pattern of hearing loss may provide insights into the likelihood of tumor presence, thereby aiding in more effective audiometric screening and decision-making regarding MRI for detecting VS.</p>","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 2","pages":"1-6"},"PeriodicalIF":0.0,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12001430/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144059370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Audio-Vestibular Findings in Young Regular and Non-Regular Personal Music System Users.","authors":"Darshan Devananda, Vipin Ghosh, Nayana Benny, Mereena James","doi":"10.5152/iao.2025.241676","DOIUrl":"https://doi.org/10.5152/iao.2025.241676","url":null,"abstract":"<p><strong>Background: </strong>Younger adults frequently utilize personal music systems (PMSs) for extended periods for leisure. It has been reported in the literature that hearing abilities are affected in such individuals. However, its effect on auditory processing abilities and the vestibular system remains unclear. Hence, the present study was carried out to investigate the audiological and vestibular functioning in young adults who use PMSs regularly.</p><p><strong>Methods: </strong>Forty participants between 18 and 25 years of age were divided into 2 groups. Group 1 included 20 regular PMSs users from 2 to 3 years, and group 2 comprised 20 participants who were non-regular PMSs users. Detailed audiological evaluations were carried out on 15 participants in each group, and vestibular evaluations were carried out on all the participants.</p><p><strong>Results: </strong>It was observed that the extended high-frequency hearing thresholds and otoacoustic emissions were affected in the regular PMSs users. The gap in noise test and vestibular evoked myogenic potential testing revealed that temporal resolution abilities and vestibular system functioning are also compromised among regular PMS users.</p><p><strong>Conclusion: </strong>Thus, this study highlights the subtle vestibular and auditory impairments that PMS may produce in young adults, as well as the significance of a battery of tests to detect them.</p>","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 2","pages":"1-7"},"PeriodicalIF":0.0,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12001432/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144056279","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Advancing AI Horizons: Scientific Conversations on Tympanoplasty Postoperative Management.","authors":"Aynur Aliyeva","doi":"10.5152/iao.2025.2419152","DOIUrl":"https://doi.org/10.5152/iao.2025.2419152","url":null,"abstract":"","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 2","pages":"1-2"},"PeriodicalIF":0.0,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12001463/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144036471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Impact of High Glycated Hemoglobin A1c on Prognosis in Patients with Bell's Palsy: A Propensity Score Matchingatched Analysis.","authors":"Erhui Yu, Binyan Yu, Fanyuan Jin, Huafeng Cai, Jinhua Hu, Yingtong Chen, Runcheng Wang, Xiuzhen Xie, Shuhan Yang, Lihua Xuan","doi":"10.5152/iao.2025.241759","DOIUrl":"https://doi.org/10.5152/iao.2025.241759","url":null,"abstract":"<p><strong>Background: </strong>Glycated hemoglobin A1c (HbA1c) is an indicator of blood glucose levels, but the impact of hyperglycemia on Bell's palsy (BP) remains unclear. This study aims to assess the influence of high and low HbA1c levels on the prognosis of patients with BP.</p><p><strong>Methods: </strong>This monocentric, retrospective study included 712 patients with BP, divided into 103 patients with HbA1c ≥ 6.5% and 609 patients with HbA1c < 6.5%. Receiver operating characteristic curve analysis was used to evaluate the main factors affecting HbA1c levels. Propensity score matching (PSM) was further utilized to avoid selection bias and disproportionate distributions of confounding factors between the 2 groups. The House-Brackmann (H-B) facial nerve grading system was employed to assess the severity of facial motor dysfunction.</p><p><strong>Results: </strong>Analysis showed that high HbA1c patients were older, had higher body mass index, less frequently suffered from dysgeusia, and more often had hypertension (P < .05). According to the area under the curve, age had the greatest impact on HbA1c levels (95% CI=0.748-0.803, P <.001). After PSM 1 : 1 matching, there was no statistical difference in initial H-B grade between the 2 groups, but there was a statistical difference in final H-B grade (P = .023), indicating a worse prognosis for patients with BP in the high HbA1c group.</p><p><strong>Conclusion: </strong>The study, after controlling for confounding factors, showed that patients with BP and high HbA1c have a worse prognosis, suggesting that controlling blood glucose levels has a positive significance for the recovery of patients with BP.</p>","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 2","pages":"1-8"},"PeriodicalIF":0.0,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12001523/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144039526","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cochlear Implantation in Patient with Arnold-Chiari Malformation.","authors":"Paulina Podlawska-Nowak, Anna Bartochowska, Andrzej Balcerowiak, Wojciech Gawecki","doi":"10.5152/iao.2024.231273","DOIUrl":"https://doi.org/10.5152/iao.2024.231273","url":null,"abstract":"<p><p>We report a case of a 74-year-old patient with Arnold-Chiari syndrome (type 1) who, due to the bilateral profound hearing loss, was qualified for cochlear implantation. The difficult anatomy of the temporal bone necessitated special preparation for the procedure. The use of neuronavigation, in combination with facial nerve monitoring, enabled a safe surgical approach to the cochlea and ensured accurate placement of the implant.</p>","PeriodicalId":94238,"journal":{"name":"The journal of international advanced otology","volume":"21 2","pages":"1-4"},"PeriodicalIF":0.0,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12001461/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144040369","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}