Otosclerosis (Otospongiosis)
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
- 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
Domino, Frank J., et al., editors. "Otosclerosis (Otospongiosis)." 5-Minute Clinical Consult, 33rd ed., Wolters Kluwer, 2025. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_.
Otosclerosis (Otospongiosis). In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2025. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_. Accessed November 14, 2024.
Otosclerosis (Otospongiosis). (2025). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (33rd ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_
Otosclerosis (Otospongiosis) [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2025. [cited 2024 November 14]. 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
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T1 - Otosclerosis (Otospongiosis)
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ED - Baldor,Robert A,
ED - Golding,Jeremy,
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BT - 5-Minute Clinical Consult, Updating
UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816029/all/Otosclerosis__Otospongiosis_
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