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Related Experiment Videos

Ossicular abnormalities in Down's syndrome.

T J Balkany, R E Mischke, M P Downs

    Otolaryngology and Head and Neck Surgery
    |May 1, 1979
    PubMed
    Summary
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    Down syndrome is linked to significant hearing loss, primarily conductive. Beyond common ear infections, structural middle ear abnormalities like stapes fixation are frequent causes in affected children.

    Area of Science:

    • Otolaryngology
    • Genetics
    • Audiology

    Background:

    • Down syndrome (DS) is frequently associated with hearing loss and otitis media.
    • Detailed otologic studies are limited due to examination challenges in this population.
    • Understanding ear disease in DS is crucial given its prevalence (1 in 600 live births).

    Observation:

    • A comprehensive otologic and audiometric evaluation was conducted on 107 patients with Down syndrome.
    • Methods included otoscopy, pure-tone and speech audiometry, and impedance tympanometry.
    • Histologic examination of temporal bones and surgical findings were also analyzed.

    Findings:

    • 64% of patients exhibited hearing deficiency, with 83% of these being conductive hearing loss.
    • Middle ear effusion or tympanic membrane perforation explained only 60% of conductive losses.

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  • Histologic and operative findings revealed stapes fixation, superstructure deformity, and fallopian canal dehiscence.

    • Implications:

    • Conductive hearing loss in Down syndrome stems from more than just middle ear effusion.
    • Underlying structural abnormalities require consideration in managing hearing loss in DS.
    • Further research into surgical and audiological interventions for these specific abnormalities is warranted.