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    Otosclerosis (Otospongiosis)

    Otosclerosis (Otospongiosis) is a topic covered in the 5-Minute Clinical Consult.

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    Basics

    Description

    • A primary bone dyscrasia involving the otic capsule
    • This is the leading cause of conductive hearing loss in adults.
    • There are two forms:
      • Histologic otosclerosis: asymptomatic form in which abnormal bone spares vital structures of the ear
      • Clinical otosclerosis: Abnormal spongy bone involves ossicular chain or other structures, leading to altered physiology.
    • System(s) affected: sensory, hearing

    Epidemiology

    Incidence
    • Predominant age
      • Clinical onset usually in early 20s
      • Peak incidence 3rd to 5th decade
    • Predominant gender: female > male (2:1)
    Pregnancy Considerations
    • Progression may accelerate during pregnancy.
    • Some women first notice hearing loss at this time.

    Prevalence
    • Histologic form: 4–8% among U.S. Caucasian population; 1% among African Americans
    • Histologic form: U.S. Caucasian population: 5,000/100,000; African Americans: 1,000/100,000
    • Clinical form: 0.3–0.4%

    Etiology and Pathophysiology

    • Unknown; possible association with measles virus infection, hormonal factors, cytokine-related inflammation, fluoride metabolism, and oxidative stress

    Genetics
    • 50–60% of those affected give positive family history.
    • Appears to be transmitted by autosomal dominant gene with variable expressivity

    Risk Factors

    Unknown, possibly measles virus infection

    Commonly Associated Conditions

    • Van der Hoeve syndrome (rare triad of osteogenesis imperfecta, blue sclera, and otospongiosis)
    • Tinnitus, usually low frequency

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    Citation

    Stephens, Mark B., et al., editors. "Otosclerosis (Otospongiosis)." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_.
    Otosclerosis (Otospongiosis). In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_. Accessed April 21, 2019.
    Otosclerosis (Otospongiosis). (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_
    Otosclerosis (Otospongiosis) [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 21]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_.
    * Article titles in AMA citation format should be in sentence-case
    TY - ELEC T1 - Otosclerosis (Otospongiosis) ID - 816029 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_ PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -