Timing of cochlear implantation in large vestibular aqueduct syndrome–a retrospective cohort analysis
IntroductionLarge vestibular aqueduct syndrome (LVAS) typically manifests fluctuating, progressive, or sudden hearing loss. Cochlear implantation (CI) is a critical intervention for LVAS patients when hearing aids (HA) no longer confer sufficient benefit. However, determining the optimal timing for...
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Frontiers Media S.A.
2025-03-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2025.1562198/full |
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| author | Xiao Liu Wanting Huang Yunxiu Wang Jingjing Xu Lulu Xie Lin Liu Jia Chen |
| author_facet | Xiao Liu Wanting Huang Yunxiu Wang Jingjing Xu Lulu Xie Lin Liu Jia Chen |
| author_sort | Xiao Liu |
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| description | IntroductionLarge vestibular aqueduct syndrome (LVAS) typically manifests fluctuating, progressive, or sudden hearing loss. Cochlear implantation (CI) is a critical intervention for LVAS patients when hearing aids (HA) no longer confer sufficient benefit. However, determining the optimal timing for CI remains challenging due to the heterogeneous and unpredictable nature of hearing loss progression, particularly when audiological criteria for CI are met, and HA can still provide benefits. This study aimed to address these complexities by analyzing real-world data on the timing of CI and clinical decision-making processes in pediatric LVAS patients.MethodsThis retrospective cohort study reviewed the medical records of 74 pediatric patients (<18 years) with LVAS who underwent CI at a tertiary care hospital in China between 2010 and 2023. Clinical data, including newborn hearing screening (NBHS) results, methods of hearing loss identification, hearing levels at the initial audiological assessment (IAA), and patterns of hearing loss progression, were analyzed. Additionally, key milestones were evaluated, including age at hearing loss identification, IAA, and CI, and the durations between these events.ResultsThe median age at CI was 4.9 years (IQR: 3.0–6.8), with a median duration from IAA to CI of 2.9 years (IQR: 1.6–5.2). Patients identified through NBHS underwent CI earlier than those identified through poor response to sound or language learning difficulties. Moreover, patients with poor performance at IAA had an earlier age at CI and shorter duration from IAA to CI. CI timing was comparable among different hearing loss progression patterns. Finally, among patients meeting CI criteria but still benefiting from HA, while those who directly underwent CI had an earlier age at implantation, their interval from IAA to CI was similar to those who initially underwent HA fitting.ConclusionThe majority of LVAS patients experience progressive hearing loss and undergo CI during early childhood. Failure of NBHS and poor auditory performance at IAA are indicative of rapid hearing deterioration. Once audiological criteria for CI are met, prolonged observation appears unnecessary. Nevertheless, further prospective longitudinal studies are warranted to refine the timing and decision-making process. |
| format | Article |
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| institution | Kabale University |
| issn | 1664-2295 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Neurology |
| spelling | doaj-art-2876cadbfddd486bbd1ade01f36709a42025-08-20T03:41:51ZengFrontiers Media S.A.Frontiers in Neurology1664-22952025-03-011610.3389/fneur.2025.15621981562198Timing of cochlear implantation in large vestibular aqueduct syndrome–a retrospective cohort analysisXiao Liu0Wanting Huang1Yunxiu Wang2Jingjing Xu3Lulu Xie4Lin Liu5Jia Chen6Department of Otorhinolaryngology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Otorhinolaryngology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Otorhinolaryngology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Radiology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Otorhinolaryngology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Otorhinolaryngology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Otorhinolaryngology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaIntroductionLarge vestibular aqueduct syndrome (LVAS) typically manifests fluctuating, progressive, or sudden hearing loss. Cochlear implantation (CI) is a critical intervention for LVAS patients when hearing aids (HA) no longer confer sufficient benefit. However, determining the optimal timing for CI remains challenging due to the heterogeneous and unpredictable nature of hearing loss progression, particularly when audiological criteria for CI are met, and HA can still provide benefits. This study aimed to address these complexities by analyzing real-world data on the timing of CI and clinical decision-making processes in pediatric LVAS patients.MethodsThis retrospective cohort study reviewed the medical records of 74 pediatric patients (<18 years) with LVAS who underwent CI at a tertiary care hospital in China between 2010 and 2023. Clinical data, including newborn hearing screening (NBHS) results, methods of hearing loss identification, hearing levels at the initial audiological assessment (IAA), and patterns of hearing loss progression, were analyzed. Additionally, key milestones were evaluated, including age at hearing loss identification, IAA, and CI, and the durations between these events.ResultsThe median age at CI was 4.9 years (IQR: 3.0–6.8), with a median duration from IAA to CI of 2.9 years (IQR: 1.6–5.2). Patients identified through NBHS underwent CI earlier than those identified through poor response to sound or language learning difficulties. Moreover, patients with poor performance at IAA had an earlier age at CI and shorter duration from IAA to CI. CI timing was comparable among different hearing loss progression patterns. Finally, among patients meeting CI criteria but still benefiting from HA, while those who directly underwent CI had an earlier age at implantation, their interval from IAA to CI was similar to those who initially underwent HA fitting.ConclusionThe majority of LVAS patients experience progressive hearing loss and undergo CI during early childhood. Failure of NBHS and poor auditory performance at IAA are indicative of rapid hearing deterioration. Once audiological criteria for CI are met, prolonged observation appears unnecessary. Nevertheless, further prospective longitudinal studies are warranted to refine the timing and decision-making process.https://www.frontiersin.org/articles/10.3389/fneur.2025.1562198/fullcochlear implantationlarge vestibular aqueduct syndromeenlarged vestibular aqueductintervention criteriadecision-makingnewborn hearing screening |
| spellingShingle | Xiao Liu Wanting Huang Yunxiu Wang Jingjing Xu Lulu Xie Lin Liu Jia Chen Timing of cochlear implantation in large vestibular aqueduct syndrome–a retrospective cohort analysis Frontiers in Neurology cochlear implantation large vestibular aqueduct syndrome enlarged vestibular aqueduct intervention criteria decision-making newborn hearing screening |
| title | Timing of cochlear implantation in large vestibular aqueduct syndrome–a retrospective cohort analysis |
| title_full | Timing of cochlear implantation in large vestibular aqueduct syndrome–a retrospective cohort analysis |
| title_fullStr | Timing of cochlear implantation in large vestibular aqueduct syndrome–a retrospective cohort analysis |
| title_full_unstemmed | Timing of cochlear implantation in large vestibular aqueduct syndrome–a retrospective cohort analysis |
| title_short | Timing of cochlear implantation in large vestibular aqueduct syndrome–a retrospective cohort analysis |
| title_sort | timing of cochlear implantation in large vestibular aqueduct syndrome a retrospective cohort analysis |
| topic | cochlear implantation large vestibular aqueduct syndrome enlarged vestibular aqueduct intervention criteria decision-making newborn hearing screening |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2025.1562198/full |
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