Isaac L Alter, Alexander Chern, Megan E Kuhlmey, Meghan A Despotidis, Scott Kelly, Michael W Denham, Alexis S Leiderman, Anil K Lalwani
{"title":"Emotional Responses to Music Are Diminished in Bilateral Cochlear Implant Users.","authors":"Isaac L Alter, Alexander Chern, Megan E Kuhlmey, Meghan A Despotidis, Scott Kelly, Michael W Denham, Alexis S Leiderman, Anil K Lalwani","doi":"10.1159/000543299","DOIUrl":"https://doi.org/10.1159/000543299","url":null,"abstract":"<p><strong>Introduction: </strong>Emotional response to music, though a central facet of music listening, remains poorly understood. In this study, we investigate the emotional responses to music among bilateral cochlear implantees.</p><p><strong>Methods: </strong>Bilateral CI users and normal-hearing (NH) controls were recruited from a tertiary academic center and community hearing loss groups. Participants listened (via an online survey) to ten previously validated 15-second musical clips representing multiple genres and wide range of valence (happiness vs. sadness) and arousal (excitement vs. calm), and rated the musical clips on validated nine-point visual analog scales of valence and arousal.</p><p><strong>Results: </strong>In total, 25 bilateral CI users and 18 NH controls completed the study. Compared to NH controls, CI users demonstrated significantly increased error in valence identification (mean (SD) 1.75 (0.61) vs. 1.16 (0.29), p<0.001), but equivalent arousal error. There were significant differences for eight of 20 total ratings between NH and CI groups. Additionally, CI users demonstrated a significantly constricted range of arousal (4.69 (1.39) vs. 6.04 (1.33), p=0.0025), but a preserved range of valence (4.99 (1.57) vs. 5.62 (1.13), p=0.13). Implantees reported significantly lower music enjoyment (5.78 (3.57) vs. 8.57 (1.17), p=0.0016) than NH controls, and enjoyment was significantly associated with both valence and arousal range.</p><p><strong>Conclusions: </strong>Bilateral CI users' emotional response to music is significantly constricted compared to NH controls. Blunted appreciation of emotional content likely contributes to reduced music enjoyment among cochlear implantees and is a critical target for improvement.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-16"},"PeriodicalIF":1.6,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143694576","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}
Nicole Knölke, Susan Busch, Thomas Lenarz, Hannes Maier
{"title":"Vibrant Soundbridge Round Window Vibroplasty: Safety, Coupling Efficiency and Speech Outcome of the Most Common Coupling Modalities.","authors":"Nicole Knölke, Susan Busch, Thomas Lenarz, Hannes Maier","doi":"10.1159/000545335","DOIUrl":"https://doi.org/10.1159/000545335","url":null,"abstract":"<p><p>Introduction The Floating Mass Transducer (FMT) of the Vibrant Soundbridge (VSB) is successfully used for reverse stimulation of the round window membrane (RWM) since 2005, enabling the treatment of conductive and mixed hearing loss. The FMT can be placed directly to the RWM, or indirectly by using an interposed material such as fascia and Tutopatch, or a coupler, e.g. the Round Window Coupler (RWC), the Round Window Soft Coupler (RWS), or the custom-made Hannover Coupler (HCV2). The aim of the study was to retrospectively evaluate the long-term outcome of the most common VSB RW coupling modalities in our clinic with regards to (1) safety and preservation of residual hearing, (2) coupling efficiency and (3) speech outcome. Methods A total of 111 Round Window (RW) vibroplasties, a surgical procedure including the implantation of the VSB, in 102 ears (95 patients) performed at the Hannover Medical School between 2006 and 2021 were analyzed. Seventy-nine RW vibroplasties in ears with available preoperative BC PTA4 were included in the safety analysis, focusing on adverse events (AE). Furthermore, the audiological outcomes were evaluated in 56 ears (52 patients, 4 bilateral implanted) preoperatively, at initial activation (6-8 weeks), 2 years (2Y) and 5 years (5Y), including bone conduction pure tone average (BC; PTA4 at 0.5, 1, 2, 4 kHz), coupling efficiency (Ceff, in situ measurement - BC PTA4), effective gain (EG, BC PTA4 - sound field threshold) and monosyllable word recognition score (WRS; Freiburg monosyllable test at 65 dB SPL). Results The safety analysis showed a total mean AE rate of 29.1%. A statistically significant decline over time in BC PTA4 was found in groups RWS and HCV2 by 7.6 dB and 8.8 dB (paired t-test, p=0.022 and p≤0.001, respectively), at 2Y. The WRS improved over time in all groups, resulting in an overall mean score of 81.1% in the range 70.0% to 90.8% at 2Y, with a statistically significant improvement between IA and 2Y and the best mean WRS of 90.8% at 2Y in group HCV2 (paired t-test, p≤0.001). While the Ceff was stable and below 25 dB at all time points in most groups, it improved statistically significant in group RWC by a total of 9.9 dB between IA and 5Y to 16.8 dB (paired t-test, p≤0.001). Conclusion The RW vibroplasty is a safe surgery, though complex and challenging. All investigated coupling modalities provided effective treatment for conductive and mixed hearing loss. Patients in group RWC and HCV2 tended to perform noticeably better in terms of aided speech understanding and coupling efficiency than those in the other groups.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-17"},"PeriodicalIF":1.6,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143694579","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":"Erratum.","authors":"","doi":"10.1159/000543746","DOIUrl":"https://doi.org/10.1159/000543746","url":null,"abstract":"","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1"},"PeriodicalIF":1.6,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143665431","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}
Saad Elzayat, Hussein A El-Shirbeny, Ibrahim Gehad, Ahmed Morshedy, Wessam Mostafa Essawy, Ashraf Fayed, Salma Ragab, Haitham H Elfarargy
{"title":"Validation and Adaptation of the Arabic Version of the Skarzynski Tinnitus Scale.","authors":"Saad Elzayat, Hussein A El-Shirbeny, Ibrahim Gehad, Ahmed Morshedy, Wessam Mostafa Essawy, Ashraf Fayed, Salma Ragab, Haitham H Elfarargy","doi":"10.1159/000544805","DOIUrl":"10.1159/000544805","url":null,"abstract":"<p><strong>Introduction: </strong>This study aimed to translate the Skarzynski Tinnitus Scale (STS) into Arabic, conduct a cross-cultural adaptation, and validate its psychometric properties.</p><p><strong>Methods: </strong>The translation and cross-cultural adaptation of the STS were carried out in five main steps. A total of 152 participants were divided into two groups: 79 (52%) cases complaining of tinnitus and 73 (48%) in the control group without tinnitus.</p><p><strong>Results: </strong>The scale required no substantial modifications during the translation process. Internal consistency was measured by Cronbach α for each of the three subscales and the total score. The overall psychological distress subscale, Cronbach α was 0.815; for the functional scale α was 0.787; for the coping subscale α was 0.555; and for global STS, α was 0.921. The STS global score and the psychological distress subscale had extremely high consistency.</p><p><strong>Conclusion: </strong>The translation and adaptation of the STS established linguistic and Arabic cultural equivalence with the original version. Furthermore, the adapted version demonstrated good internal consistency. The results suggest that the STS is suitable for use in a clinical setting.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-8"},"PeriodicalIF":1.6,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143588242","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":"Investigation of the Effect of Cognitive Additional Tasks on Functional Head Impulse Test in Healthy Individuals.","authors":"Büşra Sezer, Mine Baydan Aran","doi":"10.1159/000544719","DOIUrl":"10.1159/000544719","url":null,"abstract":"<p><strong>Introduction: </strong>Performing two tasks simultaneously may present various challenges for the human cognitive system. This study investigates how concurrent cognitive tasks impact the performance of the functional head impulse test (fHIT) in healthy adults. It was hypothesized that adding a cognitive load during fHIT would reduce the test's scores due to shared attentional resources.</p><p><strong>Method: </strong>Twenty-seven participants completed the functional head impulse test (fHIT) and verbal fluency tests simultaneously. The study was designed in three stages: (1) recording fHIT results without any cognitive task, (2) completing the categorical fluency test and fHIT concurrently, and (3) administering the phonemic fluency test and fHIT simultaneously, with results recorded.</p><p><strong>Results: </strong>Significant differences were observed in fHIT performance with and without additional cognitive tasks. Categorical tasks (e.g., animal and furniture categories) and phonemic tasks (e.g., words starting with \"K,\" \"A,\" or \"S\") impacted fHIT results, with notable performance reductions.</p><p><strong>Conclusion: </strong>This study demonstrates that dual-task scenarios involving cognitive and vestibular demands adversely affect fHIT performance. The findings underscore the importance of attentional capacity in tasks requiring simultaneous cognitive and visual-oculomotor processing. These insights could guide future clinical applications in assessing and rehabilitating vestibular and cognitive deficits.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-6"},"PeriodicalIF":1.6,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143588239","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":"Effects of Wearing Hearing Aids on Gait and Cognition: A Pilot Study.","authors":"Ryota Sakurai, Maki Nishinakagawa, Keigo Hinakura, Masatoki Takahashi","doi":"10.1159/000544829","DOIUrl":"10.1159/000544829","url":null,"abstract":"<p><strong>Introduction: </strong>Positive effects of hearing aids (HA) have been suggested. However, their impact on gait performance, which is closely associated with hearing loss and cognition, remains unclear. This pilot study assessed the impact of wearing HA on gait performance among patients with age-related hearing loss (ARHL).</p><p><strong>Methods: </strong>This single-arm trial compared gait performance of patients with ARHL before and after wearing HA. Participants diagnosed with ARHL and prescribed HA by an audiologist wore the HA gradually over 1 week. They then underwent a baseline assessment before wearing HA consistently. After wearing the HA for 1 year, participants underwent a follow-up assessment that included evaluations of gait and cognitive performance, fear of falling, incidental falls, and well-being using WHO-5.</p><p><strong>Results: </strong>Of the 10 participants included, one withdrew during the follow-up period. Intention-to-treat analyses showed improvements in gait step time at both usual and maximum paces due to HA use. Cognitive function, including Montreal Cognitive Assessment scores and delayed verbal recall on the logical memory test, also significantly improved at follow-up. Furthermore, reduced fear of falling and increased WHO-5 scores was observed after 1 year of HA use. Conversely, the rate of incidental falls did not decrease.</p><p><strong>Conclusion: </strong>HA use may contribute to improved gait performance and reduced anxiety related to physical function, in addition to cognitive function and well-being. Although the results should be interpreted with caution due to the non-randomized controlled trial design and small sample size, the findings suggest that improving hearing acuity among older adults may enhance overall health status.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-7"},"PeriodicalIF":1.6,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143538188","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}
Tzu-Pu Chang, Ying-Chi Hong, Yu-Hung Kuo, Michael C Schubert
{"title":"Visual Vertigo Is Not Correlated with Perception of Dynamic Verticality.","authors":"Tzu-Pu Chang, Ying-Chi Hong, Yu-Hung Kuo, Michael C Schubert","doi":"10.1159/000543868","DOIUrl":"10.1159/000543868","url":null,"abstract":"<p><strong>Introduction: </strong>We conducted a prospective study determining the relationship between severity of visual vertigo and extent of visual dependence by comparing the visual vertigo analogue scale (VVAS) with the dynamic subjective visual vertical (dSVV) test.</p><p><strong>Methods: </strong>A total of 76 patients with chronic dizziness (without peripheral or central vestibular lesions) and 43 healthy controls completed VVAS and dSVV testing. The VVAS uses nine visual analog scales to query visual vertigo across nine specific situations. The dSVV measures visual dependence under three conditions: static background, counterclockwise (CCW) rotating background, and clockwise (CW) rotating background (20°/s). Finally, each patient completed a simulator sickness questionnaire (SSQ) to evaluate the dizziness induced from the dSVV. VVAS, dSVV, and SSQ were compared between the patient and the control groups. Among the patient group, the correlation between VVAS and dynamic SVV were determined. Associations of outcomes measures and gender, age, and migraine were also assessed.</p><p><strong>Results: </strong>Patients with dizziness had significantly greater VVAS (16.9 vs. 1.6, p < 0.001) and SSQ (25.7 vs. 6.5, p < 0.001) scores, yet less deviation of dSVV (CCW background: -9.2 vs. -11.5, p = 0.05; CW background: 7.4 vs. 10.1, p = 0.02) than controls. In the patient group, VVAS was not correlated with dynamic SVV for either CCW (r = 0.12, p = 0.30) or CW backgrounds (r = -0.01, p = 0.92). During CCW rotation, the SVV deviation was greater in older aged patients and slightly greater in female. During CW rotation, the SVV deviation was less in the patients with migraine.</p><p><strong>Conclusion: </strong>In chronic dizzy patients that have no measurable peripheral or central vestibular lesion, severity of visual vertigo is not correlated with visual dependence.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-8"},"PeriodicalIF":1.6,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143451060","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}
Ann-Charlotte Persson, Måns Eeg-Olofsson, André Sadeghi, Margret Lepp
{"title":"Patients' Experiences of an Active Transcutaneous Implant: The Bone Conduction Implant.","authors":"Ann-Charlotte Persson, Måns Eeg-Olofsson, André Sadeghi, Margret Lepp","doi":"10.1159/000544774","DOIUrl":"10.1159/000544774","url":null,"abstract":"<p><strong>Introduction: </strong>The aim of this qualitative study was to explore and describe patients' experiences of using and living with the bone conduction implant (BCI).</p><p><strong>Methods: </strong>Semi-structured interviews were conducted with 10 BCI users and analyzed according to the phenomenographic approach.</p><p><strong>Results: </strong>Four conceptual themes were formed during the analysis; (1) conceptions of the process receiving the BCI, (2) conceptions of handling the BCI on a daily basis, (3) conceptions of hearing with the BCI, and (4) conceptions of health care issues related to the BCI. The participants' statements include experiences of improved hearing and self-esteem by using the BCI. Noisy situations and not being able to hear in daily life situations causes frustrations. The participants described anxiety about consequences following an MRI examination. The audio processor is easy to handle but the fact that it is not waterproof raise concerns. Despite some frustration and concerns, participants state that the audio processor has become a part of them, and they cannot imagine being without it.</p><p><strong>Conclusion: </strong>The ability to hear and communicate with other people has a great impact on the participants' daily life quality, and their statements show the importance hearing has on their lives and how they perceive themselves. The BCI seems to be a good hearing rehabilitation alternative for the participants, and they state that the audio processor is easy to use and handle.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-12"},"PeriodicalIF":1.6,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143451058","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}
Pauline Roger, Thomas Lespargot, Catherine Boiteux, Eric Bailly-Masson, Fabien Auberger, Sandrine Mouysset, Bernard Fraysse
{"title":"Predicting Hearing Aid Outcomes Using Machine Learning.","authors":"Pauline Roger, Thomas Lespargot, Catherine Boiteux, Eric Bailly-Masson, Fabien Auberger, Sandrine Mouysset, Bernard Fraysse","doi":"10.1159/000543916","DOIUrl":"10.1159/000543916","url":null,"abstract":"<p><strong>Introduction: </strong>The aims of this study were to measure the effectiveness of hearing aid (HA) fitting in improving understanding in quiet and in noise and to investigate the factors that significantly influence these results. This study will be carried out through a retrospective analysis of the results obtained from patients fitted with HAs at Amplifon HA centers between 2018 and 2021. This study explores and classifies the predictive factors of HAs outcomes, looking at the impact of HA technology, personalized adjustments made by the hearing care professional, and patient follow-up and daily use (data logging).</p><p><strong>Methods: </strong>The study is based on the analysis of a large population of HA users who were fitted in HA centers between 2018 and 2021. It included 77,661 patients. HA outcome is measured through the improvement of intelligibility in quiet and noise. eXtreme Gradient Boosting machine learning method is used to identify predictive factors of HA outcome. SHapley Additive exPlanations Value analysis derived from the game theory is used to evaluate the individual impact of each factor.</p><p><strong>Results: </strong>HA outcomes are significant in terms of both average improvement per patient of speech intelligibility and the percentage of patients improved. The analysis shows that the level of aided speech perception in quiet and noise is impacted by the choice of technology (category level and manufacturer), fitting parameters (amplification level and binaural loudness balancing) as well as by a high therapy adherence. In particular, binaural loudness balancing was shown to be systematically beneficial to all patients.</p><p><strong>Conclusion: </strong>Big data analysis is a new relevant method to evaluate predictive factors for HA outcomes. It demonstrates HA efficiency to improve intelligibility in quiet and noise and shows the impact of hearing care professionals in maximizing patient's outcome through the selection of the most appropriate technology, fitting parameters, and a regular follow-up ensuring a high daily usage. However, global results must be interpreted with caution on such a heterogeneous population. They would need to be refined by an approach using clusters of patients with similar audiological profiles.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-9"},"PeriodicalIF":1.6,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143124222","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}
Julie Moyaert, Hanne Gommeren, Griet Mertens, Marc J W Lammers, Olivier Vanderveken, Sebastien Janssens de Varebeke, Nicolas Verhaert, Sam Denys, Raymond van de Berg, Ronald Pennings, Erik Fransen, Vincent Van Rompaey, Annick Gilles
{"title":"Early Deficits in Speech Perception in Carriers of the p.Pro51Ser Variant in the COCH Gene: A Prospective Longitudinal Evaluation of Speech Perception in Quiet and Noise.","authors":"Julie Moyaert, Hanne Gommeren, Griet Mertens, Marc J W Lammers, Olivier Vanderveken, Sebastien Janssens de Varebeke, Nicolas Verhaert, Sam Denys, Raymond van de Berg, Ronald Pennings, Erik Fransen, Vincent Van Rompaey, Annick Gilles","doi":"10.1159/000543263","DOIUrl":"10.1159/000543263","url":null,"abstract":"<p><strong>Background and aim: </strong>The primary concern among adults with regard to their hearing is the difficulty in comprehending speech, particularly in noisy environments. The constant need to listen attentively leads to heightened frustration, fatigue, and decreased concentration. According to research, high-frequency hearing loss could have negative implications on speech perception and make it even harder to communicate. In this study, the focus is on patients with DFNA9 as it is one of the most common forms of non-syndromic high-frequency hearing loss. These patients carry the p.Pro51Ser variant in the COCH gene, which leads to progressive decline of hearing and vestibular function. Despite various cross-sectional studies on the natural progression of hearing levels, speech perception in silence and noisy surroundings is largely unexplored in this group of patients.</p><p><strong>Method: </strong>For the longitudinal analysis of speech perception, 101 heterozygous carriers of the p.Pro51Ser variant in the COCH gene were enrolled. In addition, a control group composed of individuals with normal hearing, who matched the patients with DFNA9 in the study in terms of age and sex, was recruited. All patients underwent pure-tone audiometry, speech perception in quiet (SPIQ), and speech perception in noise (SPIN).</p><p><strong>Results: </strong>The SPIQ outcomes reveal a mean speech reception threshold (SRT) of 28.18 dB SPL for male carriers and 29.29 dB SPL for female carriers in the youngest age-group (18-29 years). With increasing age, a steep decrease was noticed, and no speech discrimination ability in quiet remained for carriers in their seventh decade. Differences between carriers and control participants seem evident in the third decade of life and become more pronounced in the decades that follow. The SPIN displayed a similar trend, varying from -5 dB SNR in the youngest age-group, to no speech-in-noise thresholds in patients above the age of 60 years. In contrast, the matched group exhibited a SRT range from -5.5 to -3.25 dB SNR for males, and from -6.23 to -4.58 dB SNR for females from the second/third to the seventh decade. This stands in contrast to the DFNA9 population, where male carriers reach values of -5.18 dB SNR and female carriers reach -3.12 dB SNR as early as in the fourth decade.</p><p><strong>Conclusion: </strong>This study indicates poor performance on speech understanding in quiet and noise in DFNA9 patients in comparison with the group with normal hearing, even at a young age. Therefore, future research should not only investigate pure-tone audiometry, but also speech perception. Moreover, reimbursing hearing aids based on speech-in-noise testing could prove to be more advantageous than based on pure-tone audiometry.</p>","PeriodicalId":55432,"journal":{"name":"Audiology and Neuro-Otology","volume":" ","pages":"1-15"},"PeriodicalIF":1.6,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048874","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}