Audiology and Neuro-Otology最新文献

{"title":"The Impact of Auditory Input on Postural Control in Adults with Unilateral Cochlear Implants.","authors":"Volkan Tutar, Isa Tuncay Batuk, Merve Ozbal Batuk","doi":"10.1159/000543402","DOIUrl":"10.1159/000543402","url":null,"abstract":"<p><strong>Introduction: </strong>This study aimed to investigate the impact of auditory input on postural control in young adult cochlear implant users with profound sensorineural hearing loss. The research explores the relationship between auditory cues and static postural stability in individuals with hearing impairment.</p><p><strong>Methods: </strong>Thirty-four young adult cochlear implant users, consisting of 15 males and 19 females aged 18-35 years, underwent various balance tests, including the modified Clinical Tests of Sensory Interaction on Balance (mCTSIB) and the unilateral stance test (UST), under different auditory conditions: (a) white noise stimulus present with the sound processor activated, (b) ambient noise present with the sound processor activated, and (c) sound processor deactivated. Statistical analysis was performed to evaluate the participants' postural control performance in relation to auditory stimuli.</p><p><strong>Results: </strong>Statistical analysis revealed that participants exhibited better postural control in the presence of white noise stimuli compared to when the sound processor was turned off in the UST (p = 0.001) and Fukuda Stepping Test (in terms of displacement [p = 0.004]). The composite mCTSIB scores showed no significant difference between sound processors off, sound processors on with ambient noise, and sound processors on with broadband white noise conditions.</p><p><strong>Conclusion: </strong>The findings suggest that access to auditory cues through cochlear implants plays a crucial role in preserving static postural control in young adults with hearing impairment. This study highlights the positive effect of auditory information on balance performance in individuals with cochlear implants and profound hearing loss. Future research is recommended to further explore the impact of auditory stimuli on dynamic postural abilities and to include preoperative balance assessments in individuals undergoing cochlear implantation.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-9"},"PeriodicalIF":1.6,"publicationDate":"2025-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142973425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intra-Cochlear Electrode Position Impacts the Preservation of Residual Hearing in an Animal Model of Cochlear Implant Surgery.","authors":"Max Meuser, Susanne Schwitzer, Mario Thiele, Patrick Boyle, Arne Ernst, Dietmar Basta","doi":"10.1159/000540266","DOIUrl":"10.1159/000540266","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Preservation of residual hearing after cochlear implantation remains challenging. There are several approaches to preserve residual hearing, but the configuration of the implant electrode array seems to play a major role. Lateral wall electrode arrays are seemingly more favorable in this context. To date, there are no experimental data available which correlate the spatial electrode position in the scala tympani with the extent of hearing preservation.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;Based on micro-computed tomography (µCT) imaging data, this study analyses the exact position of a pure silicone electrode array inserted into the cochlea of four guinea pigs. Array position data were correlated with the extent of hearing loss after implantation, measured using auditory brainstem measurements in the frequency range of the area occupied by the electrode array area as well as apical to the array.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The use of pure silicone arrays without electrodes resulted in artifact-free, high-resolution µCT images that allowed precise determination of the arrays' positions within the scala tympani. The electrode arrays' locations ranged from peri-modiolar to an anti-modiolar. These revealed a correlation of a lower postoperative hearing loss with a higher spatial proximity to the lateral wall. This correlation was found in the low-frequency range only. A significant correlation between the inter-individual differences in the diameter of the scala tympani and the postoperative hearing loss could not be observed.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;This study demonstrates the importance of the intra-cochlear electrode array's position for the preservation of residual hearing. The advantage of such an electrode array's position approximated to the lateral wall suggests, at least for this type of electrode array applied in the guinea pig, it would be advantageous in the preservation of residual hearing for the apical part of the cochlea, beyond the area occupied by the electrode array.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Preservation of residual hearing after cochlear implantation remains challenging. There are several approaches to preserve residual hearing, but the configuration of the implant electrode array seems to play a major role. Lateral wall electrode arrays are seemingly more favorable in this context. To date, there are no experimental data available which correlate the spatial electrode position in the scala tympani with the extent of hearing preservation.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;Based on micro-computed tomography (µCT) imaging data, this study analyses the exact position of a pure silicone electrode array inserted into the cochlea of four guinea pigs. Array position data were correlated with the extent of hearing loss after implantation, measured using auditory brainstem measurements in the frequency range of the area occupied by the electrode array area as well as apical to the array","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"34-44"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809461/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141565193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparison between the International Outcome Inventory for Hearing Aids Questionnaire and Real-Ear Measurement.","authors":"Gi-Sung Nam, Young Jae Lee, Hansoo Song, Jeonghyun Oh, Sung Il Cho","doi":"10.1159/000540738","DOIUrl":"10.1159/000540738","url":null,"abstract":"<p><strong>Introduction: </strong>The international outcome inventory for hearing aids (IOI-HA) is a questionnaire widely used to assess the subjective benefits of hearing aids. This study aimed to evaluate the relationship between IOI-HA outcomes and target mismatch in real-ear measurement (REM).</p><p><strong>Methods: </strong>Thirty-four ears of 25 patients who had worn hearing aids were evaluated using the Korean version of the IOI-HA and REM after fitting for 2 months. Real-ear insertion gain (REIG) was measured at three different levels of input intensity - 50, 65, and 80 dB sound pressure level (SPL) - and a frequency range of 0.25-6 kHz. Factors 1 and 2 and total IOI-HA scores were compared with the mismatches of REIGs and target gains of REM.</p><p><strong>Results: </strong>Factor 1, factor 2, and total IOI-HA scores were 14.6 ± 3.5, 11.4 ± 2.2, and 25.9 ± 5.1, respectively. The averages of the difference of REIGs and target gains in REM at 50, 65, and 80 dB SPL input levels were -3.1 ± 6.7, -2.3 ± 7.2, and -3.0 ± 8.2, respectively. Factors 1 and 2 scores of the IOI-HA showed significant correlations with target mismatch in REM at 1 kHz and 0.75 kHz frequencies, respectively. Total IOI-HA scores had significant correlations with target mismatches in REM at 0.75 and 1 kHz frequencies.</p><p><strong>Conclusion: </strong>IOI-HA scores correlated with target mismatch in REM at mid frequencies. The IOI-HA can be a useful screening measure for evaluating the necessity of further adjustments in hearing aids through REM at mid frequencies.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"52-57"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141894918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impedance and Functional Outcomes in Robotic-Assisted or Manual Cochlear Implantation: A Comparative Study.","authors":"Guillaume Gersdorff, Nicolas Peigneux, Unal Duran, Severine Camby, Philippe P Lefebvre","doi":"10.1159/000540577","DOIUrl":"10.1159/000540577","url":null,"abstract":"<p><strong>Introduction: </strong>Preservation of residual hearing, mainly the low frequencies, is the current main objective of cochlear implantation. New electrode arrays and the development of minimally invasive surgery have allowed electroacoustic stimulation. Over the past several years, robotic-assisted cochlear implant surgery aimed to improve the insertion process while respecting inner ear structures. However, the introduction of a foreign body inside the cochlea can lead to the development of fibrous tissue around the electrode array, or even induce osteogenesis. These histological changes disrupt the parameters of the cochlear implant, resulting in elevated impedance. In addition, long-term auditory performance can be affected, with a deterioration in word comprehension. We evaluated the potential impact of RobOtol® on impedance changes over time, leading to potentially positive functional outcomes.</p><p><strong>Methods: </strong>Cochlear implant surgery with a round window approach was performed under general anesthesia. Fifteen Med-El Flex24 electrode arrays were inserted manually and 24 using RobOtol®. All subjects underwent pure-tone audiometry tests before the surgery and at regular intervals up to 1 year after the surgery. Based on the pure-tone average at the low frequencies from 250 to 1,000 Hz, we divided the patients according to the degree of auditory preservation (full preservation ≤15 dB, partial preservation 15 dB-30 dB, significant loss >30 dB). These different groups were compared in terms of impedance changes and auditory performance, specifically word recognition score.</p><p><strong>Results: </strong>We found proportionally fewer patients who experienced significant low-frequency hearing loss after robotic insertion (53.33% in the manual group compared to 41.67% in the robot-assisted insertion group). Impedance changes at the apex of the electrode array, especially at the first electrode (p = 0.04), after robotic surgery, with less overall variability, a continuous decreasing trend without secondary elevation, and lower values in cases of complete residual hearing preservation (for the three first electrodes: p = 0.017, p = 0.04, p = 0.045). The speech intelligibility amelioration over time showed favorable evolution in patients with complete residual hearing preservation regardless of the insertion method. However, in the absence of auditory preservation, the positive evolution continued more than 6 months after robotic surgery but stagnated after manual insertion (difference at 1 year, p = 0.038; median auditory capacity index 83% vs. 57%).</p><p><strong>Conclusion: </strong>Atraumatic electrode array insertion with consistent, slow speed and the assistance of RobOtol® minimizes disturbances in the delicate neurosensory structures of the inner ear and leads to better auditory performance.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"80-88"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142057400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Residual Dizziness Characteristics of Idiopathic Sudden Sensorineural Hearing Loss Patients with Benign Paroxysmal Positional Vertigo.","authors":"Jing Yang, Gaoyun Xiong, Hongyi Lu, Xiandan Luo, Xiaoxing Xie, Aoling Shao","doi":"10.1159/000540036","DOIUrl":"10.1159/000540036","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;ISSNHL, a common clinical condition, can be accompanied by vertigo. Initially, research on sudden deafness primarily focused on the hearing loss itself, with less emphasis on episodic vertigo. However, as vertigo research has advanced, it has been recognized that BPPV is a frequent accompaniment to ISSNHL-associated vertigo. Even after treatment, some patients may experience residual dizziness. This study investigates the characteristics of patients with ISSNHL accompanied by BPPV and the impact of residual dizziness on their lives.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This study is being conducted on patients with ISSNHL accompanied by BPPV, analyzing the characteristics of such patients and the impact of residual dizziness on their lives. Overall, 54 adult inpatients with ISSNHL and BPPV were included in this study. All patients received 50 mg of intravenous prednisolone for 5 consecutive days and hemodilution agents for 10 days. At the same time, BPPV was treated with repositioning by the same therapist using the SRM-IV vertigo diagnostic and treatment system, and different repositioning methods were used for different types of otolithiasis. Patients were grouped according to the absence of residual dizziness when the nystagmus disappeared at the time of discharge.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;There were 24 cases in the group with residual symptoms, including 10 males and 14 females. The proportion of females was 58.33%, with an average age of 46.75 ± 13.80. The group without residual symptoms consisted of 30 cases, including 13 males and 17 females. The female proportion was 56.67%, with an average age of 45.77 ± 11.86. There is no statistical significance between the two groups in the pre-treatment hearing status and DHI scores. The HAMA (Hamilton Anxiety Rating Scale) scores before treatment were compared, revealing a significant statistical difference.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;ISSNHL-associated BPPV may be caused by vascular embolism or thrombosis in the cochlear or spiral modiolar artery. This disrupts blood flow, leading to ischemia in the otolithic membrane and subsequent detachment of otoconia. Because this detachment often occurs within 24 h of the initial event, patients experience positional vertigo early in the course of the disease.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;ISSNHL, a common clinical condition, can be accompanied by vertigo. Initially, research on sudden deafness primarily focused on the hearing loss itself, with less emphasis on episodic vertigo. However, as vertigo research has advanced, it has been recognized that BPPV is a frequent accompaniment to ISSNHL-associated vertigo. Even after treatment, some patients may experience residual dizziness. This study investigates the characteristics of patients with ISSNHL accompanied by BPPV and the impact of residual dizziness on their lives.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This study is being conducted on patients with ISSNHL accompa","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"45-51"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809453/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141789934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Speech Comprehension by Cochlear Implant Users Assessed with Evoked Potentials and Response Times.","authors":"Pushkar Deshpande, Lindsey Van Yper, Stine Christiansen, Chrisitian Brandt, Stefan Debener, Tobias Neher","doi":"10.1159/000538701","DOIUrl":"10.1159/000538701","url":null,"abstract":"<p><strong>Introduction: </strong>Cochlear implant (CI) users differ greatly in their rehabilitation outcomes, including speech understanding in noise. This variability may be related to brain changes associated with intact senses recruiting cortical areas from stimulation-deprived senses. Numerous studies have demonstrated such cross-modal reorganization in individuals with untreated hearing loss. How it is affected by regular use of hearing devices remains unclear, however. To shed light on this, the current study measured cortical responses reflecting comprehension abilities in experienced CI users and normal-hearing controls.</p><p><strong>Methods: </strong>Using multichannel electroencephalography, we tested CI users who had used their devices for at least 12 months and closely matched controls (N = 2 × 13). Cortical responses reflecting comprehension abilities - the N400 and late positive complex (LPC) components - were evoked using congruent and incongruent digit-triplet stimuli. The participants' task was to assess digit-triplet congruency by means of timed button presses. All measurements were performed in speech-shaped noise 15 dB above individually measured speech recognition thresholds. Three stimulus presentation modes were used: auditory-only, visual-only, and visual-then-auditory.</p><p><strong>Results: </strong>The analyses revealed no group differences in the N400 and LPC responses. In terms of response times, the CI users were slower and differentially affected by the three stimulus presentation modes relative to the controls.</p><p><strong>Conclusion: </strong>Compared to normal-hearing controls, experienced CI users may need more time to comprehend speech in noise. Response times can serve as a proxy for speech comprehension by CI users.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-13"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140873473","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exploring CHAPS as a Potential Measurement for Auditory Processing and Cognitive Ability in Children with Hearing Loss.","authors":"Deniz Tuz, Ceren Bodur, Beyza Akti, Samet Kılıç, Gülce Kirazlı, Pelin Piştav Akmeşe","doi":"10.1159/000539570","DOIUrl":"10.1159/000539570","url":null,"abstract":"<p><strong>Objectives: </strong>The primary goal was to investigate the suitability of CHAPS for assessing cognitive abilities and auditory processing in people with hearing loss (HL), specifically in the domains of auditory processing, verbal working memory, and auditory attention.</p><p><strong>Method: </strong>The study comprised 44 individuals between the ages of seven and 14, 22 with HL (N = 11 males) and 22 with normal hearing (N = 10 males). Individuals' auditory attention, working memory, and auditory processing skills were assessed in the study, and self-report questionnaires were used. The evaluation utilized the Sustained Auditory Attention Capacity Test (SAACT), Working Memory Scale (WMS), Filtered Words Test, Auditory Figured Ground Test (AFGT), and the Children's Auditory Performance Scale (CHAPS). Analyses were conducted, including group comparisons, correlation examinations, and receiver operating characteristic evaluations.</p><p><strong>Results: </strong>There were significant differences in CHAPS total, attention, noise, quiet, and multiple inputs between groups. No significant differences were seen in CHAPS_ideal and CHAPS_auditory memory across groups. The study of SAACT and its subscores, WMS and its subscores, FWT, and AFGT revealed a significant difference between groups, caused by the poor performance of persons in the HL group compared to those in the NH group. The SAACT and its subscores correlated significantly with CHAPS_attention. The AUC calculation showed that The SAACT and CHAPS_attention distinguished persons with or without HL (p < 0.05). WMS_STM and WMS_total correlated with CHAPS auditory memory subscale; however, WMS_VWM did not. AUC values for WMS and its subscores showed significant discrimination in identifying children with or without HL (p < 0.05), whereas CHAPS_auditory memory did not (AUC = 0.665; p = 0.060). FWT and AFGT had a significant relationship with CHAPS_noise and CHAPS_multiple inputs subscales. The CHAPS_quiet and CHAPS_ideal subtests only correlated with AFGT. CHAPS_quite and CHAPS_ideal did not exhibit significant discriminative values (p < 0.05) for identifying children with or without HL, while CHAPS_noise, CHAPS_multiple inputs, FWT, and AFGT did.</p><p><strong>Conclusion: </strong>The CHAPS_attention subscale could be a trustworthy instrument for assessing auditory attention in children with HL. However, the CHAPS_auditory memory subscale may not be suitable for testing working memory. While performance-based auditory processing tests showed improved discrimination, the CHAPS_noise and CHAPS_multiple inputs subtests can still assess hearing-impaired auditory processing. The CHAPS_quiet and CHAPS_ideal subtests may not evaluate auditory processing.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"14-24"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141332595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sudden Hearing Loss before, during, and after the Pandemic: Investigating COVID-19 Illness and Vaccine-Related Symptoms.","authors":"Elaine C Thompson, Khaled Altartoor, Esther X Vivas","doi":"10.1159/000539791","DOIUrl":"10.1159/000539791","url":null,"abstract":"<p><strong>Introduction: </strong>Accumulating reports suggest an increase in sudden sensorineural hearing loss during the COVID-19 pandemic and vaccination periods. However, clear evidence is lacking. The goal of this study was to determine if sudden sensorineural hearing loss is associated with COVID-19 illness or its vaccine.</p><p><strong>Methods: </strong>Retrospective chart review of 50 randomly selected patients from three, 6-month time periods: \"pre-pandemic,\" \"early pandemic,\" and \"late pandemic.\" Group comparisons were performed for demographics, comorbid conditions, audiologic history, audiometric data, speech reception thresholds, and word recognition.</p><p><strong>Results: </strong>One hundred 50 patients were included in this study. A mean difference was observed in that the relative percentage of sensorineural hearing loss (SNHL) cases increased over time, corresponding to a relative decrease in conductive hearing loss cases. However, this change was not explained by proportional changes in sudden SNHL. Patients in the early pandemic time period were more likely to report tinnitus. Otherwise, the patient groups did not differ on demographic variables, hearing health history, hearing loss presentation, pure tone averages, speech reception thresholds, or word recognition performance.</p><p><strong>Conclusions: </strong>Proportion of patients with sudden sensorineural hearing loss did not change over time from the pre-pandemic period to the early or late pandemic phases. Despite a randomized sample, these findings do not support the hypothesis that COVID-19 illness or vaccine is associated with sudden sensorineural hearing loss.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"25-33"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141332596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Etiologic Diagnosis of Genetic Hearing Loss in an Ethnically Diverse Deafness Cohort.","authors":"Denise Yan, Aria Nawab, Molly Smeal, Xue-Zhong Liu","doi":"10.1159/000540202","DOIUrl":"10.1159/000540202","url":null,"abstract":"<p><strong>Introduction: </strong>Hearing loss is a common sensory disorder that impacts patients across the lifespan. Many genetic variants have been identified that contribute to non-syndromic hearing loss. Yet, genetic testing is not routinely administered when hearing loss is diagnosed, particularly in adults. In this study, genetic testing was completed in patients with known hearing loss.</p><p><strong>Methods: </strong>A total of 104 patients who were evaluated for hearing loss were enrolled and received genetic testing.</p><p><strong>Results: </strong>Of those 104 patients, 39 had available genetic testing, 20 had one missing allele, and 45 yielded no genetic diagnosis. Of the 39 cases with genetic testing data, 24 were simplex cases, and 15 were multiplex cases. A majority of patients presented with an autosomal recessive inheritance pattern (n = 32), 26 of whom presented with congenital hearing loss. 38% of cases were positive for GJB2 mutation with c.35delG being the most common pathogenic variant. These findings are consistent with previous literature suggesting GJB2 mutations are the most common causes of non-syndromic hearing loss.</p><p><strong>Conclusion: </strong>Given the frequency of genetic variants in patients with hearing loss, genetic testing should be considered a routine part of the hearing loss work-up, particularly as gene therapies are studied and become more widely available.</p><p><strong>Lay summary: </strong>Many genetic variants have been identified that contribute to non-syndromic hearing loss. Given the frequency of genetic variants in patients with hearing loss, genetic testing should be considered a routine part of the hearing loss work-up.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"70-79"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142057399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Estimation of Hearing Thresholds with Auditory Steady-State Responses to Narrow-Band Chirps in Children.","authors":"Hiroyuki Chiba, Tsukasa Ito, Toshinori Kubota, Hirooki Matsui, Chikako Shinkawa, Seiji Kakehata","doi":"10.1159/000541180","DOIUrl":"10.1159/000541180","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study was to evaluate the utility of auditory steady-state responses (ASSRs) to narrow-band chirps (NB-chirps) for estimating hearing levels in children.</p><p><strong>Design: </strong>Thresholds from the NB-chirp ASSR were evaluated in 30 sedated children with normal hearing or hearing loss. The correlation between the NB-chirp ASSR and pure-tone audiometry (PTA) thresholds was analyzed, and the difference score (DS) between these thresholds was calculated. Data from the NB-chirp ASSR were compared to retrospective data from conventional ASSR to exponentially amplitude-modulated tones in 25 sedated children.</p><p><strong>Results: </strong>Positive correlations between the NB-chirp ASSR and PTA thresholds were found at 500, 1,000, 2,000, and 4,000 Hz. Multiple comparisons of the DSs for the NB-chirp ASSR and PTA revealed significant differences at 500-2,000 Hz and 4,000 Hz, as well as 1,000-2,000 Hz, and 4,000 Hz. Comparisons of the DSs demonstrated that the DS of the NB-chirp ASSR was significantly smaller than that of the conventional ASSR at 2,000 Hz. Furthermore, the testing times for the NB-chirp ASSR were significantly shorter than those for the conventional ASSR.</p><p><strong>Conclusion: </strong>The NB-chirp ASSR closely reflected the PTA thresholds, and the testing time was shorter than that of the conventional ASSR. Thus, this study demonstrated that the NB-chirp ASSR is useful for hearing threshold estimation in children.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"89-100"},"PeriodicalIF":1.6,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142134557","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}