Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology · 2025
Deafness Progressing to Cochlear Implant Eligibility Is Eight Times More Likely in the Hypoplastic Than the Degenerative Endotype of Menière's Disease.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Brühlmann Catrin, Spiegel Jennifer L, Mühle Agnes, Dalbert Adrian, Lin Vincent Y W, Le Trung N, Schade-Mann Thore, Knoppik Jessica, Veraguth Dorothe, Röösli Christof, Huber Alexander, Dlugaiczyk Julia, Rauch Steven D, Löwenheim Hubert, Chen Joseph M, Juliano Amy F, Eckhard Andreas H, Bächinger David
Аннотация
OBJECTIVE: To investigate whether one of the two recently described MD endotypes-defined by either endolymphatic sac degeneration (MD-dg patients) or hypoplasia (MD-hp patients)-is associated with an increased likelihood of undergoing CI.
STUDY DESIGN: Retrospective multicenter cross-sectional study.
SETTING: Five tertiary referral centers.
PATIENTS: CI cohort: 115 adult MD patients with a history of uni- or bilateral CI. Non-CI cohort: 72 MD patients with no CI history. All included patients matched current diagnostic criteria for definite MD.
INTERVENTION: Cochlear implantation.
MAIN OUTCOME MEASURES: Endotype distribution (MD-dg versus MD-hp) between the CI cohort and the non-CI cohort. The endotype was determined using high-resolution CT data based on the angular trajectory of the vestibular aqueduct, following established protocols. Secondary outcomes included disease laterality, age at MD diagnosis, duration of MD, and pre-CI hearing thresholds.
RESULTS: The CI cohort included significantly more MD-hp patients than the non-CI cohort (72% versus 24%, p < 0.0001). The odds ratio of CI for an MD-hp patient relative to an MD-dg patient was 8.4 (95% confidence interval, 4.3-16.1). Pre-CI audiometric data showed no significant differences in hearing thresholds between endotypes, neither in the implanted nor in the non-implanted ear.
CONCLUSIONS: The MD-hp endotype, frequently associated with bilateral disease and early-age disease onset, is strongly linked to a higher likelihood of CI. Endotyping of MD patients based on endolymphatic sac pathology can effectively stratify their risk of severe hearing loss, guiding personalized audiological follow-up and clinical decisions regarding potential CI.
STUDY DESIGN: Retrospective multicenter cross-sectional study.
SETTING: Five tertiary referral centers.
PATIENTS: CI cohort: 115 adult MD patients with a history of uni- or bilateral CI. Non-CI cohort: 72 MD patients with no CI history. All included patients matched current diagnostic criteria for definite MD.
INTERVENTION: Cochlear implantation.
MAIN OUTCOME MEASURES: Endotype distribution (MD-dg versus MD-hp) between the CI cohort and the non-CI cohort. The endotype was determined using high-resolution CT data based on the angular trajectory of the vestibular aqueduct, following established protocols. Secondary outcomes included disease laterality, age at MD diagnosis, duration of MD, and pre-CI hearing thresholds.
RESULTS: The CI cohort included significantly more MD-hp patients than the non-CI cohort (72% versus 24%, p < 0.0001). The odds ratio of CI for an MD-hp patient relative to an MD-dg patient was 8.4 (95% confidence interval, 4.3-16.1). Pre-CI audiometric data showed no significant differences in hearing thresholds between endotypes, neither in the implanted nor in the non-implanted ear.
CONCLUSIONS: The MD-hp endotype, frequently associated with bilateral disease and early-age disease onset, is strongly linked to a higher likelihood of CI. Endotyping of MD patients based on endolymphatic sac pathology can effectively stratify their risk of severe hearing loss, guiding personalized audiological follow-up and clinical decisions regarding potential CI.