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Video

Otology/Neurotology

(A867) The role of age and otitis media in hearing loss patterns in mucopolysaccharidosis

Monday, October 13, 2025
12:00 PM - 1:00 PM EDT
Location: OTO EXPO, Posters

    Presenting Author(s)

  • Murilo W. de Santana Hager, BS

    Medical Student
    Jacobs School of Medicine and Biomedical Sciences at the University at Buffalo
    Buffalo, New York, United States

    • Co-Author(s)

  • GV

    Gaayathri Varavenkataraman, MA

    Research Assistant
    Department of Otolaryngology, Jacobs School of Medicine and Biomedical Sciences at the University at Buffalo
    East Amherst, New York, United States

    • Senior Author(s)

  • MC

    Michele M. Carr, DDS MD PhD

    Professor
    Department of Otolaryngology, Jacobs School of Medicine and Biomedical Sciences at the University at Buffalo
    Williamsville, New York, United States

Disclosure(s):
Murilo W. de Santana Hager, BS: No relevant relationships to disclose.
Michele M. Carr, DDS MD PhD: No relevant relationships to disclose.


Introduction: Mucopolysaccharidosis (MPS) is a group of inherited metabolic disorders causing glycosaminoglycan accumulation, resulting in skeletal, neurological, and auditory impairments. Despite the known risk of hearing loss (HL) in MPS, data on specific types of HL and the role otitis media (OM) plays are limited. This retrospective cohort study analyzed HL prevalence in MPS patients, stratified by age, and examined the impact of OM on outcomes.

Methods: Data from the TriNetX US Network identified MPS patients (ICD-10 E76) from 1/1/2006 to 12/31/2024. HL prevalence was stratified by age group, and HL types were categorized as conductive (CHL; ICD-10 H90.0, H90.1, H90.2, H90.A1), sensorineural (SNHL; ICD-10 H90.3, H90.4, H90.5, H90.A2), and mixed (MHL; ICD-10 H90.6, H90.7, H90.8, H90.A3). MPS patients with/without a history of OM (ICD-10 H65, H66, H67) were propensity score matched by demographics, and HL outcomes were analyzed. HL outcomes were also compared between matched OM patients with/without MPS.

Results: Among 3,288 MPS patients (mean age=26.5 years, SD=25.5) 1,600 (48.7%) male, 1,429 (43.5%) female, and 259 (7.8%) unknown, SNHL was the most common type of HL affecting 433 (13.2%) patients, followed by CHL 229 (7.0%) and MHL 177 (5.4%). Peak CHL occurred at 6-10 years (N=47, 15.8%), SNHL at 11-15 years (N=80, 21.6%), and MHL at 16-20 years (N=43, 11.6%). Assessing the impact of OM on HL, MPS patients with a history of OM (N=569) had higher HL rates compared to those without OM: CHL (21.4% vs. 3.2%; OR=8.4, p<.001), SNHL (29.7% vs. 9.3%; OR=4.1, p<.001), and MHL (16.9% vs. 2.5%; OR=8.0, p<.001) when compared with patients without OM. Assessing the impact of MPS on HL, MPS patients with OM (N=586) had elevated HL risks compared to non-MPS patients with OM: CHL (22.2% vs. 6.7%, OR=4.0, p<.001), SNHL (30.7% vs. 3.2%, OR=13.2, p<.001), and MHL (17.7% vs. 1.7%, OR=12.4, p<.001) when compared to non-MPS patients.

Conclusions: OM significantly increases the risk for all types of HL in MPS patients, highlighting the need for routine auditory monitoring and proactive OM management to optimize patient outcomes in this group.