Deafness Progressing to Cochlear Implant Eligibility Is Eight Times More Likely in the Hypoplastic Than the Degenerative Endotype of Menière's Disease.

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.