Ear and Hearing最新文献

{"title":"Social Predictors of Hearing Aid Purchase: Do Stigma, Social Network Composition, Social Support, and Loneliness Matter?","authors":"Gurjit Singh, Huiwen Goy, Kay Wright-Whyte, Alison L Chasteen, M Kathleen Pichora-Fuller","doi":"10.1097/AUD.0000000000001656","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001656","url":null,"abstract":"<p><strong>Objectives: </strong>The purpose of this study was to evaluate the extent to which four different social factors (stigma, social network composition, social support, and loneliness) predict the purchase of hearing aids in a sample of older adults with impaired hearing who had not previously tried hearing aids and visited a hearing care clinic for the first time.</p><p><strong>Design: </strong>Data collection took place across 130 different hearing care clinics (Connect Hearing) in Canada. A total of 4630 participants were recruited for the study from notices in the waiting rooms of the clinics or by advertising in local newspapers. The final sample consisted of 753 adults (mean age = 69.2 years; SD = 9.0; 57.4% male) who were all recommended to try hearing aids. Clinical records were tracked for a minimum of 3 months and a maximum of 15 months after the appointment to determine if they obtained hearing aids. Participants completed a 56-item questionnaire before their appointment and then experienced standard care at the clinic (i.e., hearing evaluation, hearing rehabilitation if desired, etc.). Key factors assessed by the questionnaire included stigma related to age, stigma related to hearing aids, social network composition, perceived levels of social support, loneliness, self-reported hearing disability, and demographic information.</p><p><strong>Results: </strong>Data were analyzed using two methods, a penalized logistic regression and a classification tree analysis, to identify statistical predictors and meaningful clinical cutoff scores, respectively. Both models found that hearing aid adoption was best predicted by being older and having greater self-reported hearing disability. Hearing aid uptake was also predicted by social factors, but these predictors were less robust than age and self-reported hearing disability. Participants were more likely to adopt hearing aids if they reported less hearing aid stigma and had a social network that included at least 1 person with a suspected hearing loss. Loneliness and social support did not predict hearing aid adoption. Some model-specific variables also emerged.</p><p><strong>Conclusions: </strong>Using a prospective research design, the study provides novel quantitative evidence of the role of different social factors regarding the uptake of hearing aids. The research findings may be used to better identify individuals more and less likely to obtain hearing aids, inform hearing rehabilitation, and motivate the use of interventions designed to lessen the impact of stigma on hearing rehabilitation.</p>","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143733299","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Changes in Patient Characteristics of Pediatric Cochlear Implant Candidates Over a 20-Year Timeframe Affect Language Outcomes and Equity of Healthcare.","authors":"Julie Hare, Tracey Sear, Deborah A Vickers","doi":"10.1097/AUD.0000000000001639","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001639","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;To determine how the patient profile of pediatric cochlear implant recipients changed over a 20-year period in an inner-city clinic (typical of many larger clinics), and to understand how these changes were influenced by clinical practice and changes in society. To relate changes in patient profile to long-term language outcomes, the primary purpose of pediatric cochlear implantation.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;A retrospective, observational study of children implanted between 1998 and 2019 was conducted. Longitudinal language outcomes from preimplant to 5-year postimplant were collected from all children reaching the 5-year clinical review (179 children). Demographic factors of home language, onset of severe to profound deafness (congenital, progressive, or acquired), age at implantation, device configuration (unilateral, bimodal, bilateral), and socioeconomic status were collected for the entire sample (414 children) to understand changes over time. Chi-square, Kruskal-Wallis, and Analysis of Variance tests were conducted to determine if demographic factors changed over time and Logistic Regressions were conducted to understand which factors predicted language outcomes.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Over the 20-year period, we observed a significant increase in the percentage of children from non-native English-speaking families (24 to 67%), influenced by population migration. There was a significant increase in the percentage of children with progressive onset of deafness (8 to 45%), influenced by UK National Institution of Health and Care Excellence guidance which saw a relaxation in audiometric criteria such that greater numbers of children with progressive losses were eligible. Age at implantation significantly decreased due to greater surgical confidence, increased awareness of the benefits of implanting babies under 12 months and the introduction of newborn hearing screening. There was a significant reduction in the Index of Multiple Deprivation (proxy for socioeconomic status) believed to be related to recession, austerity, and population migratory trends. Regression analyses suggested that onset of deafness, age at implantation, year of implantation, income deprivation, and parental education were key predictors of 5-year post-implantation language abilities.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Multiple factors affect long-term speech and language outcomes in children growing up using cochlear implants. Influential factors can alter over time due to changes in clinical practice/guidance or changes in society affecting cultural/linguistic distribution. If this complex and dynamically changing landscape of influential factors is well understood, appropriate interventions can be introduced for families that are most in need of them to facilitate faster rates of language acquisition. Clinical services should be streamlined and changes in patient characteristics monitored to provide equitable treatment.&lt;","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143722505","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Let's See If You Can Hear: The Effect of Stimulus Type and Intensity to Pupil Diameter Response in Infants and Adults.","authors":"Amanda Saksida, Sašo Živanović, Saba Battelino, Eva Orzan","doi":"10.1097/AUD.0000000000001651","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001651","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;Pupil dilation can serve as a measure of auditory attention. It has been proposed as an objective measure for adjusting hearing aid configurations, and as a measure of hearing threshold in the pediatric population. Here we explore (1) whether the pupillary dilation response (PDR) to audible sounds can be reliably measured in normally hearing infants within their average attention span, and in normally hearing adults, (2) how accurate within-participant models are in classifying PDR based on the stimulus type at various intensity levels, (3) whether the amount of analyzed data affects the model reliability, and (4) whether we can observe systematic differences in the PDR between speech and nonspeech sounds, and between the discrimination and detection paradigms.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;In experiment 1, we measured the PDR to target warble tones at 500 to 4000 Hz compared with a standard tone (250 Hz) using an oddball discrimination test. A group of normally hearing infants was tested in experiment 1a (n = 36, mean [ME] = 21 months), and a group of young adults in experiment 1b (n = 12, ME = 29 years). The test was divided into five intensity blocks (30 to 70 dB SPL). In experiment 2a (n = 11, ME = 24 years), the task from experiment 1 was transformed into a detection task by removing the standard warble tone, and in experiment 2b (n = 12, ME = 29 years), participants listened to linguistic (Ling-6) sounds instead of tones.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;In all experiments, the increased PDR was significantly associated with target sound stimuli on a group level. Although we found no overall effect of intensity on the response amplitude, the results were most clearly visible at the highest tested intensity level (70 dB SPL). The nonlinear classification models, run for each participant separately, yielded above-chance classification accuracy (sensitivity, specificity, and positive predictive value above 0.5) in 76% of infants and in 75% of adults. Accuracy further improved when only the first six trials at each intensity level were analyzed. However, accuracy was similar when pupil data were randomly attributed to the target or standard categories, indicating over-sensitivity of the proposed algorithms to the regularities in the PDR at the individual level. No differences in the classification accuracy were found between infants and adults at the group level, nor between the discrimination and detection paradigms (experiment 2a versus 1b), whereas the results in experiment 2b (speech stimuli) outperformed those in experiment 1b (tone stimuli).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;The study confirms that PDR is elicited in both infants and adults across different stimulus types and task paradigms and may thus serve as an indicator of auditory attention. However, for the estimation of the hearing (or comfortable listening) threshold at the individual level, the most efficient and time-effective protocol with the","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143671879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Relationships Between Subjective and Objective Measures of Listening Accuracy and Effort in an Online Speech-in-Noise Study.","authors":"Ian M Wiggins, Jemaine E Stacey, Graham Naylor, Gabrielle H Saunders","doi":"10.1097/AUD.0000000000001662","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001662","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;Speech-in-noise performance is of paramount importance to daily function, and there exists a bewildering array of outcome measures to capture the many dimensions of this concept. The aim of the present study was to provide insight into how different speech-in-noise outcome measures relate to one another, how they behave under different test conditions, and how researchers or practitioners might go about selecting an outcome measure (or measures) depending on the context and focus of their enquiry.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;An online speech-in-noise study was conducted using the Labvanced experimental platform. A total of 67 participants (42 who reported having normal hearing, 25 who said they had some degree of hearing loss) completed the Effort Assessment Scale (a self-reported measure of daily-life listening effort), followed by a sentence recognition task in which BKB sentences were presented in speech-shaped noise at signal to noise ratios (SNRs) of -8, -4, 0, +4, +8, and +20 dB. Participants were instructed to listen to each sentence and then repeat aloud what they heard. Responses were recorded through participants' webcams and later independently scored by 2 research assistants. Several outcome measures were used to tap into both accuracy and listening effort. Specifically, we examined: (1) objective intelligibility (percentage of keywords correctly repeated); (2) subjective intelligibility; (3) subjective listening effort; (4) subjective tendency to give up listening; and (5) verbal response time (VRT) extracted from the audio recordings. Data were analyzed using Bayesian statistical methods.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Hearing loss and age were associated with speech-in-noise outcomes. Specifically, we observed lower intelligibility (objective and subjective), higher subjective listening effort, and longer VRT (time to verbal response onset) in hearing-impaired compared with normal-hearing listeners, and reduced objective intelligibility and longer VRT in older compared with younger listeners. When moving from highly favorable to more adverse listening conditions, subjective listening effort was the first measure to show sensitivity to worsening SNR, followed by subjective intelligibility, objective intelligibility, subjective tendency to give up listening, and, finally, VRT. Participants, especially those with normal hearing, consistently underestimated their own performance.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;The present findings offer useful insight into how different subjective and objective measures of listening accuracy and effort respond to variation in hearing status, age, and SNR. Although speech intelligibility remains a measure of primary importance, it is a sensitive measure only under adverse listening conditions, which may not be representative of everyday listening. Under more ecologically relevant listening conditions (generally speaking, at moderate, positive SNRs), listening eff","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143675000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Vocal Emotion Recognition in School-Age Children With Hearing Aids.","authors":"Laura Rachman, Gizem Babaoğlu, Başak Özkişi Yazgan, Pinar Ertürk, Etienne Gaudrain, Leanne Nagels, Stefan Launer, Peter Derleth, Gurjit Singh, Frédérick Uhlemayr, Monita Chatterjee, Esra Yücel, Gonca Sennaroğlu, Deniz Başkent","doi":"10.1097/AUD.0000000000001645","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001645","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;In individuals with normal hearing, vocal emotion recognition continues to develop over many years during childhood. In children with hearing loss, vocal emotion recognition may be affected by combined effects from loss of audibility due to elevated thresholds, suprathreshold distortions from hearing loss, and the compensatory features of hearing aids. These effects could be acute, affecting the perceived signal quality, or accumulated over time, affecting emotion recognition development. This study investigates if, and to what degree, children with hearing aids have difficulties in perceiving vocal emotions, beyond what would be expected from age-typical levels.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;We used a vocal emotion recognition test with non-language-specific pseudospeech audio sentences expressed in three basic emotions: happy, sad, and angry, along with a child-friendly gamified test interface. The test group consisted of 55 school-age children (5.4 to 17.8 years) with bilateral hearing aids, all with sensorineural hearing loss with no further exclusion based on hearing loss degree or configuration. For characterization of complete developmental trajectories, the control group with normal audiometric thresholds consisted of 86 age-matched children (6.0 to 17.1 years), and 68 relatively young adults (19.1 to 35.0 years).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Vocal emotion recognition of the control group with normal-hearing children and adults improved across age and reached a plateau around age 20. Although vocal emotion recognition in children with hearing aids also improved with age, it seemed to lag compared with the control group of children with normal hearing. A group comparison showed a significant difference from around age 8 years. Individual data indicated that a number of hearing-aided children, even with severe degrees of hearing loss, performed at age-expected levels, while some others scored lower than age-expected levels, even at chance levels. The recognition scores of hearing-aided children were not predicted by unaided or aided hearing thresholds, nor by previously measured voice cue discrimination sensitivity, for example, related to mean pitch or vocal tract length perception.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;In line with previous literature, even in normal hearing, vocal emotion recognition develops over many years toward adulthood, likely due to interactions with linguistic and cognitive development. Given the long development period, any potential difficulties for vocal emotion recognition in children with hearing loss can only be identified with respect to what would be realistic based on their age. With such a comparison, we were able to show that, as a group, children with hearing aids also develop in vocal emotion recognition, however, seemingly at a slower pace. Individual data indicated a number of the hearing-aided children showed age-expected vocal emotion recognition. Hence, even tho","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143671881","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Investigations on Directional Hearing With One-Sided Fitting of an Active Middle Ear Implant or Bone Conduction Hearing Implant.","authors":"Christoph Müller, Hannes Seidler, Janina Kuch, Anna Tsypina, Thomas Zahnert, Susen Lailach","doi":"10.1097/AUD.0000000000001650","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001650","url":null,"abstract":"<p><strong>Objective: </strong>In patients with conductive or combined unilateral hearing loss, implantable hearing systems can be a treatment option. Due to the overlapping indications of hearing implants, a systematic evaluation of audiologic differences in terms of speech intelligibility and binaural hearing abilities is necessary. Because of the unilateral cochlear stimulation in patients implanted with an active middle ear implant, we expect superior binaural hearing performance compared with patients implanted with a bone-conducting implant that causes bilateral cochlear stimulation. This study focuses especially on comparing directional hearing abilities between the aforementioned groups of implantable hearing aid users.</p><p><strong>Methods: </strong>In 13 patients unilaterally fitted with Vibrant Soundbridge (VSB) and 8 patients fitted with Bonebridge (BB) (both implants manufactured by MED-EL, Austria) (implantation at least 6 months ago, contralateral ear with at most mild hearing loss [pure tone average across 4 frequencies <30 dB]), sound localization ability, speech intelligibility (Freiburger monosyllabic word test and Oldenburgsentencetest), audiometric threshold-based measurements and patient-reported outcome measures (International Outcome Inventory for Hearing Aids and Speech, Spatial and Qualities of Hearing Scale 12) have been examined.</p><p><strong>Results: </strong>The groups did not differ significantly (p > 0.05) in terms of patient age (VSB: 44.6 ± 14.4 years [SD]; BB: 44.5 ± 17.3 years), pure tone average across 4 frequencies of bone conduction (VSB: 26.4 ± 6.9 dB; BB: 23.3 ± 6.7 dB), speech intelligibility (VSB: 80.0% ± 16.7%; BB: 69.4% ± 13.2% [Freiburger]) and Oldenburgsentencetest (VSB -8.9 ± 2.6 dB; BB: -7.2 ± 4.4 dB). Implantation was 4.2 ± 2.7 years (VSB) and 7.5 ± 3.5 years (BB) (p < 0.05). Sound tended to be localized more frequently (56% ± 16%) within the reference range in the VSB group than in the BB group (49% ± 12.9%) (p > 0.05). The VSB group tended to show a smaller lateral deviation of sound detection from the actual sound presentation direction, especially with frontal sound presentation, compared with the BB group. Lateral sound presentations above 60° were increasingly perceived in the direction contralateral to the sound source in both groups (p > 0.05). Subjective hearing disabilities were scored significantly lower in the VSB group compared with the BB group.</p><p><strong>Conclusion: </strong>When comparing the sound localization ability between BB and VSB users, the study displayed a trend toward better results with the VSB. Further measurement data of patient cohorts with larger group sizes have to be collected for a final judgment on the clinical significance of these differences.</p>","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143665411","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effects of Masker Intelligibility and Talker Sex on Speech-in-Speech Recognition by Mandarin Speakers Across the Lifespan.","authors":"Duo-Duo Tao, Yuhui Fan, John J Galvin, Ji-Sheng Liu, Qian-Jie Fu","doi":"10.1097/AUD.0000000000001655","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001655","url":null,"abstract":"<p><strong>Objectives: </strong>Speech perception develops during childhood, matures in early adulthood, and declines in old age. Everyday listening environments often contain competing sounds that may interfere with the perception of the signal of interest. With competing speech, listeners often experience informational masking, where the intelligibility and acoustic characteristics (e.g., talker sex differences) of the maskers interfere with understanding of target speech. Across the lifespan, utilization of segregation cues in competing speech is not well understood. Furthermore, there is a dearth of research regarding speech-in-speech recognition across the lifespan in speakers of tonal languages such as Mandarin Chinese.</p><p><strong>Design: </strong>Speech recognition thresholds (SRTs) were measured in listeners with age-adjusted normal hearing; the age range of participants was 5 to 74 years old. All participants were native speakers of Mandarin Chinese. SRTs were measured in the presence of two-talker Forward or Reverse speech maskers where the masker sex was the same as or different from the target.</p><p><strong>Results: </strong>In general, SRTs were highest (poorest) with the Forward same-sex maskers and lowest (best) with the Reverse different-sex maskers. SRT data were analyzed for 5 age groups: child (5 to 9 years), youth (10 to 17 years), adult (18 to 39 years), middle-aged (40 to 59 years), and elderly (60 to 74 years). Overall, SRTs were significantly higher for the child group than for the youth, adult, middle-aged, and elderly groups (p < 0.05), and significantly higher for the elderly than for the adult group (p < 0.05). There was a significant interaction among age group, speech direction, and talker sex cues, where SRTs were significantly higher for Forward than for Reverse speech, and significantly higher for same-sex than for different-sex maskers for all age groups (p < 0.05), except for the child group.</p><p><strong>Conclusions: </strong>Consistent with previous studies with non-tonal language speakers, the present SRTs with tonal language speakers were best in the adult group and poorest in the child and elderly groups. The child and youth groups demonstrated greater masking release with Reverse speech than with different-sex maskers, while the elderly group exhibited greater release with the different-sex maskers than with Reverse speech. This pattern of results may reflect developmental effects on utilization of talker sex cues in children; in older adults, enhanced top-down processes may compensate for the age-related declines in processing of temporal envelope and temporal fine structure information.</p>","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652100","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Variant Reclassification in Underrepresented Minority Children With Sensorineural Hearing Loss.","authors":"Sonia M Scaria, Jacqueline Harris, Noura Ismail Mohamad, Emily Taketa, Yesai Park, Dylan K Chan","doi":"10.1097/AUD.0000000000001653","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001653","url":null,"abstract":"<p><strong>Objectives: </strong>Underrepresented minority (URM, comprising Hispanic, non-Hispanic Black, and Native American) children with sensorineural hearing loss have fivefold lower odds of receiving a genetic diagnosis after undergoing hearing loss gene-panel testing. Using hearing loss-specific American College of Medical Genetics (ACMG)/Association for Molecular Pathology (AMP) guidelines applied to a URM-specific cohort demonstrates the utility of these guidelines in reducing the disparity in diagnostic efficacy of genetic testing for URM populations.</p><p><strong>Design: </strong>A total of 2740 variants from 715 patients with sensorineural hearing loss (1275 variants from 348 URM patients) were queried. ACMG variant interpretation guidelines with hearing loss expert specification were used to attempt reclassification of multihit (≥2 occurrences) variants of uncertain significances (VUSs), focusing on case-control analysis relative to ancestry-matched controls and computational prediction.</p><p><strong>Results: </strong>Before curation, only 198 of the 1275 variants (15.52%) in the URM population were classified as likely pathogenic. Sixty-one multihit VUSs, including variants in OTOG, TJP2, COL11A2, and 34 other genes, were probed using hearing loss-specific ACMG/AMP guidelines, resulting in reclassification of 19 variants. For the remaining 42 VUSs, reclassification would require parental testing and segregation analysis. In addition to these VUSs that appeared at least twice in our dataset, many additional VUSs appeared only once, but were extremely rare or absent from ancestry-matched databases and could be reclassified with additional information.</p><p><strong>Conclusions: </strong>This study demonstrates the utility of the application of HL-specific ACMG/AMP classification to specifically URM variants and the dramatic effects it can have on clarifying pathogenicity of VUSs, thus contributing to clinicians' ability to improve the standard of care for URM patients with improved genetic testing accuracy and subsequent early intervention.</p>","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143607250","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Concurrent Compensation for Auditory and Visual Processing in Individuals With Single-Sided Deafness.","authors":"Yufei Qiao, Jiayan Yang, Min Zhu, Qiaoyu Liu, Yuanshun Long, Hepeng Ke, Chang Cai, Yingying Shang","doi":"10.1097/AUD.0000000000001658","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001658","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;Auditory deprivation results in functional enhancement of the remaining intact visual modality, and the underlying mechanisms include cross-modal recruitment of additional resources from the auditory cortex and compensatory reorganization of the visual network in bilateral deafness. However, how resources are allocated between hearing and vision has not been determined in patients with partial auditory deprivation. This study aimed to investigate the relationship between functional plasticity of the visual and auditory pathways in patients with congenital single-sided deafness (SSD), a typical partial deprivation condition.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;The cross-sectional cohort was comprised of 25 patients with congenital SSD (mean age ± SD = 31.6 ± 5.2 years, 13 males) and 25 normal hearing (NH) controls (mean age ± SD = 30.9 ± 7.5 years, 13 males). Both visual-evoked potentials (VEPs) and auditory-evoked potentials (AEPs) were assessed for all participants. For assessment of AEPs, auditory stimuli were presented unilaterally through the hearing ear in the SSD group, while the auditory stimuli were presented unilaterally (left and right) and bilaterally in the NH group. Event-related potential analyses focused on the differences in latency and amplitude of VEPs and AEPs between groups. Dipole source analyses of VEPs and AEPs were implemented to measure the dipole strengths and latencies of the bilateral primary visual and auditory cortex and comparisons were made both within and between groups.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;For VEPs, SSD patients exhibited a greater amplitude and a shorter latency than NH controls. For dipole source analysis of VEPs, no interhemispheric asymmetry or between-group difference was observed. For AEPs, the amplitude of SSD patients was greater than that of NH controls under the monaural condition but did not exceed that evoked by binaural stimuli in NH controls. For dipole source analysis of AEPs, interhemispheric strength asymmetry was observed in NH controls in response to monaural stimuli but was less clear in SSD subjects. Considering the side of deafness, interhemispheric strength asymmetry was hardly observed in left SSD (LSSD) patients, and was also weakened in right SSD (RSSD) patients. The interhemispheric difference index of dipole strength in LSSD patients was significantly lower than that in NH controls in response to right monaural stimuli. Furthermore, the dipole strength of the ipsilateral hemisphere in both LSSD and RSSD patients was greater than that in NH controls in response to monaural stimuli.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Neural activity and efficiency in the early stage of cortical visual processing in SSD patients were enhanced. The monaural responses of the auditory pathway have lost the typical contralateral organization, becoming more symmetric due to the increased ipsilateral pathways. These findings suggest concurrent compensation for auditory an","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143598551","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early Intervention Influences 9-Year Speech, Language, Cognitive, and Quality-of-Life Outcomes in Deaf or Hard-of-Hearing Children.","authors":"Teresa Y C Ching, Linda Cupples, Mark Seeto, Vicky Zhang, Sanna Hou, Angela Wong, Christopher Flynn, Vivienne Marnane, Greg Leigh, Harvey Dillon","doi":"10.1097/AUD.0000000000001657","DOIUrl":"https://doi.org/10.1097/AUD.0000000000001657","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;Early identification of congenital deafness enables early intervention, but evidence on the influence of age at fitting of hearing aids (HAs) or cochlear implants (CIs) on outcomes in school-aged children who are deaf or hard of hearing (DHH) is limited. This study (1) described developmental outcomes and health-related quality of life in DHH children; and (2) examined the relationships among demographic factors, including age at fitting of HAs or CIs, and outcomes.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;This prospective cohort study included participants in a population-based study who were followed up at 9 years of age. Children who are DHH and who first received hearing habilitation services before 3 years of age from the government-funded national hearing service provider in the states of New South Wales, Victoria, and Southern Queensland in Australia were invited to enroll in the study. At 9 years of age, enrolled children were assessed using standardized measures of language, cognitive abilities, and speech perception. The children also completed questionnaire ratings on their quality of life. Parents provided demographic information about their child, family, and education; and completed ratings on their child's quality of life. Audiological data were retrieved from the client database of the hearing service provider and records held at CI centers. Descriptive statistics were used to report quantitative outcomes. The relationships among demographic characteristics, including age at fitting of HAs or CIs, and children's outcomes were examined using structural equation modeling.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;A total of 367 children, 178 (48.5%) girls, completed assessments at age 9.4 (SD = 0.3) years. On average, performance was within 1 SD of the normative mean for language, cognitive functioning, and health-related quality of life; but much below norms for speech perception. The modeling result is consistent with verbal short-term memory having a mediating effect on multiple outcomes. Better verbal short-term memory is significantly associated with no additional disabilities, earlier age at CI activation, use of an oral communication mode in early intervention, and higher maternal education. In turn, verbal short-term memory directly and positively affects speech perception, language, and health-related quality of life. Maternal education directly and positively affects language outcomes, and indirectly via its effects on nonverbal I.Q. and verbal short-term memory. Better language is directly associated with a better quality of life.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;This study found evidence consistent with early hearing intervention having a positive effect on speech perception and language via its effect on verbal short-term memory. Children who had better language also had better quality of life. The importance of early hearing for cognitive development lends support to early detection and early hearing int","PeriodicalId":55172,"journal":{"name":"Ear and Hearing","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143588385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}