Otology & Neurotology最新文献

{"title":"Assessing Chronic Ear Symptoms in Bone-Conduction Hearing Implant (BCHI) Patients Using the Chronic Otitis Media Benefit Inventory (COMBI) Score.","authors":"Rachael Collins, John Phillips, Junaid Hanif, Ian Nunney, Amanda Collett","doi":"10.1097/MAO.0000000000004255","DOIUrl":"10.1097/MAO.0000000000004255","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to determine improvement in health-related quality of life (HRQoL) using a validated disease-specific patient-reported outcome measure (PROM) questionnaire in patients undergoing bone-conduction hearing implant (BCHI) insertion.</p><p><strong>Study design: </strong>A mixed retrospective and prospective correlational study.</p><p><strong>Setting: </strong>Single tertiary referral center in the United Kingdom.</p><p><strong>Patients: </strong>All adult patients undergoing their first BCHI over 6 years (April 1, 2017, to March 3, 2023).</p><p><strong>Main outcome measures: </strong>The Chronic Otitis Media Benefit Inventory (COMBI) score (postintervention) and the Glasgow Health Status Inventory (GHSI) (pre-and post-BCHI questionnaire).</p><p><strong>Results: </strong>Improvements were seen across all COMBI domains. The mean total COMBI score was 46.3 (standard deviation = 5.3). Although expected significant improvements were seen in hearing and social domains, there were also notable gains in ear symptoms and reduced medical intervention post-BCHI. There was a statistically significant improvement in all GHSI scores post-BCHI (median total difference 67.1, p < 0.0001).</p><p><strong>Conclusions: </strong>This study reports very favorable outcomes for BCHI patients using two different PROMs: COMBI and GHSI. Although these PROMs complement each other, they also offer different perspectives on the same cohort of patients, with COMBI providing a unique insight into specific ear symptoms. This is the first reported study using this complement of PROMS in BCHI patients and offers further evidence for the wide-reaching improvements BCHI can have for patients.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141760199","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":"Hearing Loss From a Retained Dental Needle Traversing the Carotid Canal and Cochlea.","authors":"Michael S Castle, Weitao Wang, Matthew T Bender, Benjamin T Crane, Hitomi Sakano","doi":"10.1097/MAO.0000000000004249","DOIUrl":"10.1097/MAO.0000000000004249","url":null,"abstract":"","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141492934","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":"Auditory Localization Performance in Cochlear Implant Recipients With Single-Sided Deafness: The Challenges and Limitations of Current Outcome Metrics.","authors":"Chioma Anidi, Obada Abdulrazzak, Gerilyn Jones, Madison V Epperson, Nadine I Ibrahim, Renee M Banakis Hartl","doi":"10.1097/MAO.0000000000004273","DOIUrl":"10.1097/MAO.0000000000004273","url":null,"abstract":"<p><strong>Hypothesis: </strong>Acoustic localization accuracy metrics currently employed in clinical literature both overestimate and underestimate performance benefit of cochlear implantation (CI) for single-sided deafness (SSD).</p><p><strong>Background: </strong>Although localization in SSD with CI has been investigated, performance characterization has relied heavily on average error. Although attractively concise, this measure may misrepresent performance. Here, we characterize frequency-specific localization on a granular level in subjects with CI for SSD as a critical analysis of localization outcome metrics.</p><p><strong>Methods: </strong>Eight CI recipients with SSD were recruited. Stimuli of broadband (BBN) and narrowband noise (NBN) at low (500 Hz), mid (1000 Hz), and high (4000 Hz) frequencies were presented in a semianechoic chamber. Localization accuracy was quantified in mean angular error (MAE) and linear regression slope.</p><p><strong>Results: </strong>Use of a CI for SSD subjects improved localization performance by slope for all stimuli ( p ≤ 0.0033) to a level that was equal to normal-hearing controls at 1 and 4 kHz ( p ≥ 0.2281). MAE was also significantly improved for SSD subjects using CI for BBN stimuli ( p ≪ 0.0001); however, no statistically significant improvement in MAE was seen for NBN ( p ≥ 0.5773) with CI use. Descriptive analysis of individual subject performance highlights the reasons for contradictory results.</p><p><strong>Conclusion: </strong>There is inherent challenge in characterizing localization benefit for individuals with CI for SSD. Our data demonstrate the limitations of utilization of average error as the sole metric for outcome benefit. We emphasize the importance of continued research investigating alternative outcome measures as we work toward a more refined understanding of the potential benefits and limitations of cochlear implantation for SSD.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141788765","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":"Regulation of the p53/SLC7A11/GPX4 Pathway by Gentamicin Induces Ferroptosis in HEI-OC1 Cells.","authors":"Yingying Li, Hui Xu, Jinping Shi, Cong Li, Mengxin Li, Xiaoling Zhang, Qing Xue, Jingjing Qiu, Limei Cui, Yan Sun, Xicheng Song, Liang Chen","doi":"10.1097/MAO.0000000000004271","DOIUrl":"10.1097/MAO.0000000000004271","url":null,"abstract":"<p><strong>Background: </strong>Gentamicin is a commonly used aminoglycoside antibiotic, with ototoxicity as a significant side effect. Ferroptosis, an iron-dependent form of cell death, has been implicated in a variety of disorders. Whether ferroptosis impacts gentamicin ototoxicity is not yet known. The current work used an in-vitro model to examine the influence of gentamicin-induced ferroptosis on cochlear hair cell damage and probable molecular biological pathways.</p><p><strong>Methods: </strong>House Ear Institute-Organ of Corti 1 (HEI-OC1) cells were treated with different concentrations of gentamicin for 24 hours, with or without ferrostatin-1 pretreatment, to observe gentamicin-induced ferroptosis. The role of p53/solute carrier family 7 member 11 (SLC7A11)/glutathione peroxidase 4 (GPX4) signaling in gentamicin-induced ferroptosis was explored by pretreating cells with the p53 inhibitor pifithrin-α (PFT-α). We investigated the effect of gentamicin on cells by assessing cell viability. Cellular proteins were isolated and Western blots were performed to detect changes in the expression of p53, SLC7A11, and GPX4. Fluorescence staining was used to assess levels of reactive oxygen species. An enzymatic detection kit was used to detect glutathione, Fe, and malondialdehyde markers.</p><p><strong>Results: </strong>Gentamicin reduced cell viability, glutathione content, and SLC7A11 and GPX4 protein levels, and increased levels of p53 protein, reactive oxygen species, malondialdehyde, and Fe. These effects were largely blocked by pretreatment with ferrostatin-1. Pretreatment with the p53 inhibitor PFT-α prevented the gentamicin-induced reduction in SLC7A11 and GPX4, which alleviated several features of ferroptosis including glutathione depletion, iron overload, and lipid peroxidation build-up.</p><p><strong>Conclusion: </strong>Gentamicin induces ferroptosis in the HEI-OC1 cell line, and the mechanism may be related to the p53/SLC7A11/GPX4 signaling pathway.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11462884/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141788766","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Amplitude Parameters Are Predictive of Hearing Preservation in a Randomized Controlled Trial of Intracochlear Electrocochleography During Cochlear Implant Surgery.","authors":"Michael S Harris, Kanth Koka, Adam Thompson-Harvey, Erin Harvey, William J Riggs, Shaza Saleh, Jordan T Holder, Robert T Dwyer, Sandra M Prentiss, Shannon M Lefler, Kristin Kozlowski, Meghan M Hiss, Amanda J Ortmann, Erin R Nelson-Bakkum, Andreas Büchner, Rolf Salcher, Steven A Harvey, Michael E Hoffer, Jorge E Bohorquez, Farid Alzhrani, Rana Alshihri, Fida Almuhawas, Christopher J Danner, David R Friedland, Michael D Seidman, Thomas Lenarz, Fred F Telischi, Robert F Labadie, Craig A Buchman, Oliver F Adunka","doi":"10.1097/MAO.0000000000004286","DOIUrl":"10.1097/MAO.0000000000004286","url":null,"abstract":"<p><strong>Objective: </strong>To prospectively evaluate the association between hearing preservation after cochlear implantation (CI) and intracochlear electrocochleography (ECochG) amplitude parameters.</p><p><strong>Study design: </strong>Multi-institutional, prospective randomized clinical trial.</p><p><strong>Setting: </strong>Ten high-volume, tertiary care CI centers.</p><p><strong>Patients: </strong>Adults (n = 87) with sensorineural hearing loss meeting CI criteria (2018-2021) with audiometric thresholds of ≤80 dB HL at 500 Hz.</p><p><strong>Methods: </strong>Participants were randomized to CI surgery with or without audible ECochG monitoring. Electrode arrays were inserted to the full-depth marker. Hearing preservation was determined by comparing pre-CI, unaided low-frequency (125-, 250-, and 500-Hz) pure-tone average (LF-PTA) to LF-PTA at CI activation. Three ECochG amplitude parameters were analyzed: 1) insertion track patterns, 2) magnitude of ECochG amplitude change, and 3) total number of ECochG amplitude drops.</p><p><strong>Results: </strong>The Type CC insertion track pattern, representing corrected drops in ECochG amplitude, was seen in 76% of cases with ECochG \"on,\" compared with 24% of cases with ECochG \"off\" ( p = 0.003). The magnitude of ECochG signal drop was significantly correlated with the amount of LF-PTA change pre-CI and post-CI ( p < 0.05). The mean number of amplitude drops during electrode insertion was significantly correlated with change in LF-PTA at activation and 3 months post-CI ( p ≤ 0.01).</p><p><strong>Conclusions: </strong>ECochG amplitude parameters during CI surgery have important prognostic utility. Higher incidence of Type CC in ECochG \"on\" suggests that monitoring may be useful for surgeons in order to recover the ECochG signal and preventing potentially traumatic electrode-cochlear interactions.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141760198","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":"RESPONSE TO LETTER TO THE EDITOR.","authors":"Zachary A Kons, Lawrance Lee, Daniel H Coelho","doi":"10.1097/MAO.0000000000004262","DOIUrl":"10.1097/MAO.0000000000004262","url":null,"abstract":"","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141982947","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":"Influence of Anatomy-Based Fitting in Cochlear Implant Users on Music Perception Using the Montreal Battery of Evaluation of Amusia.","authors":"Felix S Heitkötter, Bianca A Krämer, Achim G Beule, Claudia Rudack","doi":"10.1097/MAO.0000000000004292","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004292","url":null,"abstract":"<p><strong>Objectives: </strong>The aim is to investigate the influence of the anatomy-based fitting (ABF) in cochlear implants (CI) on the perception of specific musical features.</p><p><strong>Design: </strong>The Montreal Battery of Evaluation of Amusia (MBEA) test battery was performed with a collective of 16 CI users with and without an anatomy-based fitting and 9 normal hearing subjects. The insertion angles of the intracochlear electrode arrays were calculated using Otoplan® (MED-EL, Innsbruck, Austria) software.</p><p><strong>Results: </strong>CI users with an anatomy-based fitting achieve better results in the MBEA in all categories, especially in rhythm, meter, and memory. There is no effect of insertion depth alone on the ability to perceive musical features, even after anatomy-based fitting.</p><p><strong>Conclusions: </strong>The results of the CI users in comparison to normal hearing people confirm previous studies. Apart from better speech understanding in CI users, which has been demonstrated in other studies, the anatomy-based fitting also contributes to a better perception of musical features. Currently, there are no comparable studies on this relationship. Investigations with further parameters with the help of the MBEA are pending.</p><p><strong>Significance: </strong>Our results show that anatomy-based fitting of cochlear implants significantly improves the perception of specific musical attributes and thus leads to an increased enjoyment of music.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141982900","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":"Simulator Verification Is Potentially Beneficial for the Fitting of Softband Bone Conduction Hearing Devices in Young Children.","authors":"Le Sun, Lu Ping, Xinmiao Fan, Jian Wang, Xiaowei Chen","doi":"10.1097/MAO.0000000000004245","DOIUrl":"10.1097/MAO.0000000000004245","url":null,"abstract":"<p><strong>Hypothesis: </strong>The current study employed a skull-simulator verification method to assess whether the output of softband bone conduction hearing devices (BCHDs) at the manufacturer's default settings deviated widely from the target determined by the fitting formula.</p><p><strong>Background: </strong>Real ear analysis is utilized for the verification of the fitting of air conduction hearing devices (ACHDs) in a variety of institutions. This procedure, however, has not been used in the fitting of BCHDs, largely due to the difficulty of testing the output of these devices to temporal bones. Despite the availability of skull simulators, they have not been utilized clinically to measure BCHD output.</p><p><strong>Materials and methods: </strong>This prospective, single-center study enrolled 42 subjects, aged 3 months to 10 years, with microtia-atresia-associated mild-to-severe bilateral conductive hearing loss. Hearing sensitivity was evaluated behaviorally by pure tone audiometry (PTA) in 22 subjects 4 years or older (the PTA group), and by auditory brainstem response (ABR) in 20 subjects younger than 4 years (the ABR group). Following 6 months of subjects wearing the prescribed softband BCHDs, their dial level (DL) thresholds were reassessed while using their own BCHDs, configured with zero gain across all frequencies, functioning solely as a bone vibrator. These DL thresholds were inputted into the fitting formula, desired sensation level-bone conduction devices (DSL-BCD) for children, to obtain the target values of BCHD output. The simulator output of the BCHD programmed at the manufacturer's default setting was measured in response to speech presented at 55, 65, and 80 dB SPL, followed by gain adjustment based on the differences between the simulator output and the target. Aided speech intelligibility index (SII) was measured before and after the gain adjustment.</p><p><strong>Results: </strong>The softband BCHDs at the manufacturer's settings generally had lower output than the prescribed target values. This difference was larger at low frequencies and low levels. Across the 12 points tested (four frequencies from 500 to 4000 Hz multiplied by three levels), 22 (52.3%) and 42 (100%) BCHDs had deviations of +7 and +5 dB, respectively, at one point or more. The gain adjustments reduced the deviation and improved the SII values at the two lower levels of speech presented.</p><p><strong>Conclusion: </strong>The simulator output of softband bone conduction hearing devices (BCHDs) with the manufacturer's settings may exhibit significant deviations from the formula. Objective output verification should be considered a beneficial step in BCHD fitting and is recommended when applicable.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141458591","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":"Clinical Outcomes of Endoscopic Butterfly Inlay Cartilage Myringoplasty.","authors":"Hye Ah Joo, Hyeon Chang Choi, Dong Kyu Lee, Joong Ho Ahn, Hong Ju Park, Jong Woo Chung, Woo Seok Kang","doi":"10.1097/MAO.0000000000004234","DOIUrl":"10.1097/MAO.0000000000004234","url":null,"abstract":"<p><strong>Objective: </strong>To review a 3-year case series of endoscopic butterfly inlay cartilage myringoplasty performed by a single surgeon (W.S.K.) and analyze the clinical surgical outcomes.</p><p><strong>Study design: </strong>Retrospective study.</p><p><strong>Setting: </strong>Tertiary care academic center.</p><p><strong>Patients: </strong>We enrolled 60 ears with tympanic membrane (TM) perforation, receiving endoscopic inlay butterfly myringoplasty between 2019 and 2022.</p><p><strong>Main outcomes and measures: </strong>We reviewed patients' demographics, size and location of TM perforation, operation time, complications, and postoperative pain evaluated by the numerical rating scale (NRS). We analyzed the graft uptake success rate in 5 weeks and the perforation closure rate in 4 months after surgery. We also compared the air-bone gap (ABG) before and after the surgery.</p><p><strong>Results: </strong>Among the 60 ears included, the mean age was 57.0 years, and 78.3% (47 of 60) had small perforations. The average operation time was 48.9 ± 11.5 minutes, and the postoperative NRS was 2.0 ± 1.6. The immediate graft uptake success rate evaluated at postoperative 5 weeks was 96.7% (58 of 60), with myringitis occurring in three ears. Except for 11 patients lost to follow-up, the perforation closure rate evaluated at postoperative 4 months was 100% (49 of 49). The mean ABG significantly improved from preoperative status (8.87 ± 5.51 dB HL) to postoperative 4 months (6.22 ± 6.03 dB HL) ( p = 0.019).</p><p><strong>Conclusions: </strong>A single surgeon's success rate for endoscopic butterfly inlay cartilage myringoplasty was almost 100%. This surgical procedure is safe and effective, with a high graft success rate.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141492920","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":"Charcot-Marie-Tooth Disease and Hearing Loss: A Systematic Review With Meta-Analysis.","authors":"John F Mills, Luke D Heiland, Shaun A Nguyen, Michaela F Close, Ted A Meyer","doi":"10.1097/MAO.0000000000004243","DOIUrl":"10.1097/MAO.0000000000004243","url":null,"abstract":"<p><strong>Objective: </strong>To characterize the pattern of hearing loss in Charcot-Marie-Tooth (CMT) disease to help guide clinical management.</p><p><strong>Databases reviewed: </strong>CINAHL, PubMed, and Scopus.</p><p><strong>Methods: </strong>Two independent investigators selected studies on CMT patients with pure-tone average (PTA) and auditory brainstem response (ABR) data. Case reports, case series <5 patients, and data that overlapped with another study were excluded. Investigators performed data extraction, quality rating, and risk-of-bias assessment using the Newcastle-Ottawa Scale. Meta-analysis of mean difference using fixed/random effects models was used. Also, data were analyzed using a weighted one-way analysis of variance, with post-hoc Tukey's test for comparison.</p><p><strong>Results: </strong>Ultimately, 6 prospective studies (N = 197) were included. The most common demyelinating subtype (CMT1A) had significantly prolonged ABR latency values across wave III (0.20 ms, 95% confidence interval [CI]: 0.05-0.35), wave V (0.20 ms, 95% CI: 0.01-0.39), waves I-III (0.20 ms, 95% CI: 0.01-0.39), and waves I-V (0.20 ms, 95% CI: 0.01-0.39) when compared to matched controls. The autosomal recessive demyelinating subtype (CMT4C) had significantly worse PTA when compared to the most common subtype (CMT1A) (Δ 28.93 dB, 95% CI 18.34-39.52) and nondemyelinating subtype (CMT2A) (Δ 28.3 dB, 95% CI: 15.98-40.62).</p><p><strong>Conclusions: </strong>Patients with CMT can present with a variety of phenotypes depending on the causative mutation. The ABR interpeak latency values for the most common demyelinating form of CMT are delayed when compared to matched controls. Most subtypes have normal hearing thresholds, apart from CMT4C, which presents with mild hearing loss on average.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141492919","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}