Jaro-Journal of the Association for Research in Otolaryngology最新文献

Management Strategies for Chronic Suppurative Otitis Media and Why They Fail. 慢性化脓性中耳炎的治疗策略及其失败的原因。

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-16 DOI: 10.1007/s10162-025-00996-z

Nanki Hura, Anping Xia, Peter L Santa Maria

{"title":"Management Strategies for Chronic Suppurative Otitis Media and Why They Fail.","authors":"Nanki Hura, Anping Xia, Peter L Santa Maria","doi":"10.1007/s10162-025-00996-z","DOIUrl":"https://doi.org/10.1007/s10162-025-00996-z","url":null,"abstract":"Chronic suppurative otitis media (CSOM) affects up to 330 million people globally and is one of the leading causes of pediatric hearing loss. Defined by a state of chronic infection in the middle ear in the setting of a tympanic membrane perforation, CSOM is traditionally most frequently associated with infection with Pseudomonas and Staphylococcus aureus species. The current therapeutic options for CSOM include medical therapy in the form of topical antibiotics or antiseptics (i.e., boric acid, acetic acid, povo-iodine), as well as surgical intervention with tympanoplasty or tympanomastoidectomy in refractory cases. While topical fluoroquinolones have the strongest level of evidence supporting their use for CSOM treatment, they are frequently associated with long-term treatment failure. Treatment failure is secondary to the presence of persister cells in CSOM, which are antibiotic tolerant and have the potential to proliferate and gain additional antibiotic resistance. As biofilms and persister cells are not routinely tested for in clinical medicine, there is limited data on therapeutic options that may eradicate biofilms and persister cells while limiting ototoxic effects. While future research should aim to identify such ototopical treatment options, clinicians may also consider surgical intervention earlier in patients with disease refractory to topical treatment to both minimize the risk of encouraging antibiotic resistance and to maximize the ability to debride the biofilm.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310903","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}

引用次数: 0

Tinnitus Measured in Everyday Life: A Literature Review of Ecological Momentary Assessment Studies. 日常生活中耳鸣的测量：生态瞬时评价研究的文献综述。

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-09 DOI: 10.1007/s10162-025-00995-0

Milena Engelke, Sebastian Müller, Berthold Langguth, Rüdiger Pryss, Winfried Schlee

{"title":"Tinnitus Measured in Everyday Life: A Literature Review of Ecological Momentary Assessment Studies.","authors":"Milena Engelke, Sebastian Müller, Berthold Langguth, Rüdiger Pryss, Winfried Schlee","doi":"10.1007/s10162-025-00995-0","DOIUrl":"https://doi.org/10.1007/s10162-025-00995-0","url":null,"abstract":"Tinnitus, a common auditory phenomenon, often presents with considerable between-person heterogeneity and within-person fluctuations. To understand the pathophysiological mechanisms and advance patient-centred care, it is essential to recognise these variations. Ecological Momentary Assessment (EMA) is a (close-to) real-time data collection method that offers insights into short- and long-term fluctuations of subjective symptoms and their interaction with psychological, environmental, and physiological factors. EMA applied in tinnitus research has shown promise in capturing the nuances of tinnitus experience in naturalistic settings, minimizing recall bias inherent in traditional retrospective methods. This narrative literature review aims to provide a comprehensive up-to-date picture of EMA in tinnitus research by describing previous and current applications, summarising scientific findings, and identifying research gaps by drawing lessons from adjacent mental health fields. 28 publications were identified and assigned to six different topics based on thematic and methodological matters. We highlight contributions of EMA methodology for tinnitus research such as findings on momentary and longitudinal symptom interactions, circadian rhythms, individual differences in symptom patterns and its contributions to treatment evaluation. Emerging technologies, including machine learning, are opening new avenues for personalised tinnitus understanding and management. Despite promising advances, challenges such as data reliability, participant compliance, and integration with sensor-based passive data collection remain areas for further exploration. Drawing lessons from adjacent mental health fields, we propose future directions for EMA in tinnitus research, emphasizing the integration of multimodal data, advanced analytics, and ecological validity to enhance the understanding and management of chronic tinnitus.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144250984","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}

引用次数: 0

The Transmission of Sound to the Cochlea in Normal and Pathological Human Middle Ears. 正常和病理中耳中声音向耳蜗的传递。

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-05 DOI: 10.1007/s10162-025-00997-y

John J Rosowski

引用次数: 0

How Exceptional Is the Ear? 耳朵有多特别？

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-01 Epub Date: 2025-05-12 DOI: 10.1007/s10162-025-00988-z

Christopher Bergevin, Dennis M Freeman, Allison Coffin

{"title":"How Exceptional Is the Ear?","authors":"Christopher Bergevin, Dennis M Freeman, Allison Coffin","doi":"10.1007/s10162-025-00988-z","DOIUrl":"10.1007/s10162-025-00988-z","url":null,"abstract":"Studies of hearing often conclude that the ear is \"remarkable\" or that its performance is \"exceptional.\" Some common examples include the following: <math><mo>▹</mo></math> the ears of mammals are encased in the hardest bone in the body; <math><mo>▹</mo></math> the ear contains the most vascularized tissue in body; <math><mo>▹</mo></math> the ear has the highest resting potential in the body; <math><mo>▹</mo></math> ears have a unique \"fingerprint\"; <math><mo>▹</mo></math> the ear can detect signals below the thermal noise floor; and <math><mo>▹</mo></math> the ear is highly nonlinear (or highly linear, depending upon who you ask). Some claims hold up to further scrutiny, while others do not. Additionally, several claims hold for animals in one taxon, while others are shared across taxa. Most frequently, our sense of wonder results from the differences between ears as products of natural selection (over eons) and artificial systems as products of engineering design. Our goal in analyzing claims of remarkable or exceptional performance is to deepen our appreciation of these differences.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":"203-223"},"PeriodicalIF":2.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133661/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144043427","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}

引用次数: 0

Multidimensional Feature Analysis of Meniere's Disease and Vestibular Migraine: Insights from Machine Learning and Vestibular Testing. 梅尼埃病和前庭偏头痛的多维特征分析：来自机器学习和前庭测试的见解。

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-01 Epub Date: 2025-04-28 DOI: 10.1007/s10162-025-00990-5

Yi Du, Xingjian Liu, Lili Ren, Yu Wang, Ziming Wu

{"title":"Multidimensional Feature Analysis of Meniere's Disease and Vestibular Migraine: Insights from Machine Learning and Vestibular Testing.","authors":"Yi Du, Xingjian Liu, Lili Ren, Yu Wang, Ziming Wu","doi":"10.1007/s10162-025-00990-5","DOIUrl":"10.1007/s10162-025-00990-5","url":null,"abstract":"Objective: Differentiating between Meniere's disease (MD) and vestibular migraine (VM) is challenging due to overlapping symptoms and limited diagnostic tools. Traditional statistical methods often rely on physician judgment and struggle with complex, high-dimensional data. This study applies the random forest (RF) machine learning algorithm to enhance the clinical differentiation between MD and VM.Methods: We retrospectively analyzed data from 36 VM (26 female) and 100 unilateral MD patients (51 female). The data were anonymized and labeled. Symptomatic and examination parameters were selected as features, and exploratory data analysis identified key parameters for diagnosis. An RF model was used to rank these features.Results: MD patients more commonly experienced ear-related symptoms, while VM patients reported more headaches and dizziness. Examination findings showed greater asymmetry in vHIT saccade latency in MD patients, particularly on the affected side. A total of 40 key parameters were identified. Heatmap and clustering analysis revealed that time constant (Tc) in velocity step test (VST) correlated more strongly with headache and other symptoms, while saccade latencies and velocities correlated with pure tone averages. The RF model selected 27 parameters for prediction, achieving 91.86% accuracy (95% confidence interval [85.37%, 95.18%]). Tc and saccade velocity were among the top 10 contributing features. Additionally, MD patients had earlier saccades and shorter Tc values on the affected side compared to both healthy controls and VM patients.Conclusions: Machine learning successfully classified MD and VM patients, with Tc and saccade velocity identified as key diagnostic indicators alongside symptoms.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":"287-300"},"PeriodicalIF":2.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12134248/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144051296","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}

引用次数: 0

Acoustical Effects of Tympanostomy Tube Attachment to Human Tympanic Membrane. 鼓膜造瘘管附着于人鼓膜的声学效应。

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-01 Epub Date: 2025-04-29 DOI: 10.1007/s10162-025-00989-y

Arash Ebrahimian, Hossein Mohammadi, Hamid Motallebzadeh, Nima Maftoon

{"title":"Acoustical Effects of Tympanostomy Tube Attachment to Human Tympanic Membrane.","authors":"Arash Ebrahimian, Hossein Mohammadi, Hamid Motallebzadeh, Nima Maftoon","doi":"10.1007/s10162-025-00989-y","DOIUrl":"10.1007/s10162-025-00989-y","url":null,"abstract":"Purpose: Several therapeutic approaches for hearing disorders involve attaching medical devices to the tympanic membrane. The attachment of these devices can change the mechanical and acoustical properties of the middle ear, affecting the middle-ear vibrations. The alteration of passive mechanical properties results from the mass, stiffness, and geometry of the attached device. Additionally, procedures like tympanostomy tube attachment create perforations on the tympanic membrane, altering both the mechanical and acoustical properties of the middle ear. This study examined the acoustical effects of these as well as the combination of acoustical and mechanical effects of the attached devices on middle-ear vibrations.Methods: A finite-element model of the middle ear, including the middle-ear cavity, was used to systematically study the effects of perforation size and location on vibration outputs. Experimental data from the literature were used to tune the model. This model was then employed to investigate the combined mechanical and acoustical effects of tympanostomy tubes on vibration outputs.Results: In presence of both the mechanical effects of the device (due to its mass and stiffness) and the acoustical effects of it (due to perforations), the reduction in the motion of the stapes footplate resulting from the acoustical effects is more remarkable at low frequencies (below about 1 kHz). However, at higher frequencies, the mechanical effects of the device are dominant.Conclusion: The findings of this study provide insights into the optimal design of the shape, location, and other characteristics of medical devices implanted on the tympanic membrane.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":"271-286"},"PeriodicalIF":2.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12134247/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144053656","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}

引用次数: 0

Temporal Pitch Perception of Multi-Channel Stimuli by Cochlear-Implant Users. 人工耳蜗使用者对多通道刺激的时间音高感知

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-01 Epub Date: 2025-03-28 DOI: 10.1007/s10162-025-00983-4

Evelien de Groote, Olivier Macherey, John M Deeks, Stéphane Roman, Robert P Carlyon

{"title":"Temporal Pitch Perception of Multi-Channel Stimuli by Cochlear-Implant Users.","authors":"Evelien de Groote, Olivier Macherey, John M Deeks, Stéphane Roman, Robert P Carlyon","doi":"10.1007/s10162-025-00983-4","DOIUrl":"10.1007/s10162-025-00983-4","url":null,"abstract":"Purpose: To explore the feasibility of cochlear-implant (CI) processing strategies that aim to improve pitch perception by presenting information on the stimulus temporal fine structure (TFS) in low-frequency channels to the corresponding apical electrodes.Methods: Eight users of the MED-EL CI pitch-ranked stimuli consisting of isochronous pulse trains presented concurrently to the four most apical CI electrodes.Results: When the same rate was applied to all electrodes, pitch ranks increased with increasing rates up to 200-300 pulses-per-second (pps), consistent with previous research. Presenting rates of 100, 200, 300, and 400 pps to one electrode per rate produced a pitch rank between that of the 100- and 200-pps same-rate stimuli. The assignation of pulse rate to electrode did not have a consistent effect on pitch ranks. However, maximising the delay between pulses on the different electrodes generally produced higher pitch ranks compared to when the between-electrode pulse delay was very short.Conclusion: Our results show no evidence that listeners combine the rates of TFS applied to different channels so as to estimate the fundamental frequency but do show that pitch can be affected by between-electrode delays. We conclude that presenting different temporal patterns to adjacent electrodes is unlikely to produce a clear and robust pitch and propose an alternative method for conveying the F0 of complex sounds on multiple electrodes of a CI.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":"301-315"},"PeriodicalIF":2.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133670/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143736180","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}

引用次数: 0

Contributions of Auditory Nerve Density and Synchrony to Speech Understanding in Older Cochlear Implant Users. 听觉神经密度和同步对老年人工耳蜗使用者言语理解的贡献。

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-01 Epub Date: 2025-04-04 DOI: 10.1007/s10162-025-00984-3

Kara C Schvartz-Leyzac, Carolyn M McClaskey, James W Dias, Bryan E Pfingst, Kelly C Harris

{"title":"Contributions of Auditory Nerve Density and Synchrony to Speech Understanding in Older Cochlear Implant Users.","authors":"Kara C Schvartz-Leyzac, Carolyn M McClaskey, James W Dias, Bryan E Pfingst, Kelly C Harris","doi":"10.1007/s10162-025-00984-3","DOIUrl":"10.1007/s10162-025-00984-3","url":null,"abstract":"Purpose: The majority of adult cochlear implant (CI) recipients are over the age of 65, and previous research in non-implanted older adults shows that auditory nerve (AN) pathophysiology contributes to senescent declines in speech understanding. However, age-related changes to AN structure and function have not yet been explored as a contributory factor to poorer speech understanding outcomes in older CI users. Here, we explore how estimates of AN disengagement (i.e., AN density) and dyssynchrony in CI users contribute to poorer speech recognition performance observed in older CI users.Methods: We examined electrically evoked compound action potentials (ECAPs) in 47 adult (Male = 25) CI recipients. We measured the interphase gap (IPG) effect for the amplitude-growth function (AGF) slope and the N1-P2 interpeak latency as independent metrics of AN density and dyssynchrony, respectively.Results: Estimates of AN density and dyssynchrony worsen with increasing age in older CI listeners. These measures were not significantly correlated with one another, but were independently related to speech recognition in noise performance. Lower ECAP IPG effect values (lower density of AN fibers) are observed in older CI users. Longer N1-P2 interpeak latency values (poorer neural synchrony) are also observed in older CI users. When controlling for listener age, poorer AN dyssynchrony contributes to declines in speech-recognition-in-noise performance in CI users.Conclusion: These results suggest that AN dyssynchrony rather than density is the primary contributing factor to age-related declines in speech understanding in CI users. These results have important implications for better understanding neural contributions to speech understanding in adult CI users.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":"317-329"},"PeriodicalIF":2.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133654/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143781994","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}

引用次数: 0

Haploinsufficiency of Whrn Contributes to Progressive Sensorineural Hearing Loss in C57BL6 Mice. Whrn单倍性不足导致C57BL6小鼠进行性感音神经性听力丧失

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-01 Epub Date: 2025-05-13 DOI: 10.1007/s10162-025-00991-4

Han-Gyu Bae, Sean Kashiwagura, Andrew Jung, Elizabeth Gould, Jun Hee Kim

{"title":"Haploinsufficiency of Whrn Contributes to Progressive Sensorineural Hearing Loss in C57BL6 Mice.","authors":"Han-Gyu Bae, Sean Kashiwagura, Andrew Jung, Elizabeth Gould, Jun Hee Kim","doi":"10.1007/s10162-025-00991-4","DOIUrl":"10.1007/s10162-025-00991-4","url":null,"abstract":"Purpose: Whrn, encoding whirlin, is one of the genes highly relevant to Usher syndrome (USH) that has been known as an autosomal recessive genetic disorder that is characterized with sensorineural hearing loss with retinitis pigmentosa. Although recent studies on the other USH genes, PDZD7 and Ush1 g, showed a possibility of haploinsufficiency effect, the potential contribution of heterozygous Whrn loss to hearing loss remains unclear.Methods: To investigate the effect of Whrn haploinsufficiency, we conducted a longitudinal study assessing auditory function in heterozygous Whrn mutant (Whrn+/-) mice in which long isoform of Whrn was deleted by replacing exon 1 with Neo cassette without disturbing short isoform. The threshold of auditory brainstem responses (ABRs) was measured on 135 Whrn+/- mice and littermate 133 wild-type (WT) mice from 1 to 6 months of ages. From those data, the threshold from male and female were separately analyzed to investigate sex-dependent effect. To further investigate underlie mechanisms, hair cell death was investigated using immunohistostaining from 4 to 5 WT, 5 female Whrn+/-, and 7 male Whrn+/- mice at 4-5 months old.Results: Hearing threshold was significantly increased with aging in Whrn+/- mice compared to WT controls. Both WT and Whrn+/- mice exhibited sex-dependent variations in hearing sensitivity. Notably, Whrn+/- males showed a progressive hearing loss with age, while Whrn+/- females exhibited elevated hearing thresholds as early as 1-2 month of age compared to WT females.Conclusion: These results provide evidence for a haploinsufficiency effect of Whrn on auditory function and highlight its potential role in progressive sensorineural hearing loss.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":"239-247"},"PeriodicalIF":2.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133618/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144059892","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}

引用次数: 0

Cochlear Implantation Outcomes in Genotyped Subjects with Sensorineural Hearing Loss. 感音神经性听力损失基因分型受试者的人工耳蜗植入结果。

IF 2.4 3区医学

Jaro-Journal of the Association for Research in Otolaryngology Pub Date : 2025-06-01 Epub Date: 2025-04-23 DOI: 10.1007/s10162-025-00987-0

M L A Fehrmann, L Haer-Wigman, H Kremer, H G Yntema, M E G Thijssen, E A M Mylanus, W J Huinck, C P Lanting, R J E Pennings

{"title":"Cochlear Implantation Outcomes in Genotyped Subjects with Sensorineural Hearing Loss.","authors":"M L A Fehrmann, L Haer-Wigman, H Kremer, H G Yntema, M E G Thijssen, E A M Mylanus, W J Huinck, C P Lanting, R J E Pennings","doi":"10.1007/s10162-025-00987-0","DOIUrl":"10.1007/s10162-025-00987-0","url":null,"abstract":"Purpose: Cochlear implants (CIs) are an effective rehabilitation option for individuals with severe-to-profound sensorineural hearing loss (SNHL). While genetic factors play a significant role in SNHL, the variability in CI outcomes remains unclear. This study evaluated short- and long-term CI outcomes in a large genotyped cohort and investigated correlations with genetic defects and their cochlear site-of-lesion.Methods: This retrospective, single-center, cohort study included 220 subjects (127 females; 299 ears) with pathogenic variants identified in 31 different nuclear genes and in mitochondrial genes. Audiological outcomes were measured pre- and post-implantation. Cochlear site-of-lesion was categorized as pre-synaptic, post-synaptic, or mitochondrial, based on gene function or expression. Multiple regression analysis assessed factors influencing outcomes, including age at implantation, SNHL duration, hearing aid (HA) use, and cochlear site-of-lesion.Results: Results showed a median phoneme score of 90%, with better outcomes in early implantation (≤ 6 years). Variability in outcomes was not linked to cochlear site-of-lesion, but to subject-specific factors, such as age at implantation, duration of SNHL, pre-implantation HA use, and CI experience. A model incorporating these subject-specific factors explained 19% of the total variance in outcomes. Poorer outcomes (phoneme scores < 70%) were more common in individuals with prolonged auditory deprivation or older age at implantation.Conclusion: Genotyped CI recipients demonstrated excellent outcomes, with variability largely attributed to non-genetic factors. These findings show that cochlear implantation is a beneficial type of rehabilitation for most individuals with hereditary SNHL and underscore the importance of early implantation.","PeriodicalId":56283,"journal":{"name":"Jaro-Journal of the Association for Research in Otolaryngology","volume":" ","pages":"331-348"},"PeriodicalIF":2.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133674/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144041845","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}

引用次数: 0