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==Risk Factors==
==Risk Factors==
 
*;Genetic factor:
Otosclerosis is an autosomal dominant disease with incomplete penetrance. When 60% of patients were found with family history, 40-50% of patients have it with variable patterns of inheritance.


== Screening==
== Screening==

Revision as of 05:59, 6 November 2020

Wikidoc


Otosclerosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] CSN, M.D. Christina Ninan, M.D.


Synonyms and keywords: Otospongiosis

Overview

Otosclerosis is a word derived from the Greek word "sklērós" meaning hardening and oto meaning ear. Otosclerosis is a disorder in which the footplate of the stapes is replaced by an abnormal bone, thereby affecting sound transmission to the inner ear at the level of the oval window.[1] osseous dyscrasia limited to the temporal bone that results in slow, progressive conductive hearing loss(emedicine) (CT) scanning of the temporal bone can often demonstrate foci of demineralization in the otic capsule in cases of cochlear otosclerosis

These changes can occur at many locations in the inner ear. The majority of cases consist of changes in the oval window, but they have also been described in the round window, cochlear apex, posterior to the oval window, posterior and anterior wall of the internal auditory canal (IAC), cochlear aqueduct, semicircular canals, and within the stapes footplate.


Historical perspective

  • 1704:Antonio Maria Valsalva identified fixation of stapes as a cause of hearing loss[2]
  • 1841:Toyn bee stated, "osseous ankylosis of the stapes to the fenestra ovalis as one of the causes of deafness".[3]
  • 1876:Johannes Kessel described stapes surgery as the treatment.[4]
  • 1930-1950: Used Julius Lempert’s single-stage fenestration of stapes as treatment.[5]
  • 1956:John Shea modernized stapedectomy.[6]

Classification

Embryology

Pathophysiology

Etiology

Differentiating otosclerosis from other diseases

Conditions mimicking Otosclerosis
  • Serous otitis media
  • Adhesive otitis media
  • Congenital stapes fixation
  • Meniere disease
  • Tympanosclerosis
  • Attic fixation of head of the malleus
  • Ossicular discontinuity

Epidemiology and demographics

  • Greater preponderance in women compared to men in a ratio of 2:1.[7]
  • Even though the disease begins in the second and third decade of life, it doesn't result in hearing loss until the fourth decade.[8]
  • Clinical prevalence of otosclerosis is found to be higher in Caucasians by 0.04%-1% while the histological incidence of otosclerosis increases to 10% in the same.[9].The incidence of histological otosclerosis is 1% and 5% in African and Asian population respectively.[10]
  • Autosomal dominant mode of inheritance with incomplete penetrance in certain cases.

Risk Factors

  • Genetic factor

Otosclerosis is an autosomal dominant disease with incomplete penetrance. When 60% of patients were found with family history, 40-50% of patients have it with variable patterns of inheritance.

Screening

Clinincal associations and Complications

Clinical presentaion

Diagnosis

Treatment

SAMPLE

  • USMLE STEP 1
  • USMLE STEP 2
    • USMLE STEP 2 CK
    • USMLE STEP 2 CS
  • USMLE STEP 3
    • USMLE STEP 3 MCQ
    • USMLE STEP 3 CCS92F + atrial fibrillation + numbness/tingling of her right leg; where is the stroke lesion?
  1. left or right side of the brain?
à answer = left (contralateral).
  1. medial or lateral cerebral hemisphere?
àanswer = medial (homunculus for lower limb is

medial, upper limb and face are lateral).

  1. anterior or posterior to the central sulcus?
ànswer = posterior (primary sensory cortexis posterior; primary motor is anterior))
  1. ↑ Rajput MSA, Arain AA, Rajput AA, Adeel M, Suahil A, Awan MS (2020). "Otosclerosis: Experience With Stapes Surgery". Cureus. 12 (5): e7927. doi:10.7759/cureus.7927. PMC 7265776 Check |pmc= value (help). PMID 32499972 Check |pmid= value (help).
  2. ↑ Makarem AO, Hoang TA, Lo WW, Linthicum FH, Fayad JN (2010). "Cavitating otosclerosis: clinical, radiologic, and histopathologic correlations". Otol Neurotol. 31 (3): 381–4. doi:10.1097/MAO.0b013e3181d275e8. PMC 2880664. PMID 20195188.
  3. ↑ Nazarian R, McElveen JT, Eshraghi AA (2018). "History of Otosclerosis and Stapes Surgery". Otolaryngol Clin North Am. 51 (2): 275–290. doi:10.1016/j.otc.2017.11.003. PMID 29502722.
  4. ↑ Wielgosz R, Mroczkowski E (2008). "[History of endaural surgery]". Otolaryngol Pol. 62 (3): 348–50. doi:10.1016/S0030-6657(08)70269-5. PMID 18652164.
  5. ↑ Pietruski J (1998). "[Juliusz Lempert (1890-1959): the author of the fenestration technique]". Otolaryngol Pol. 52 (3): 341–6. PMID 9760779.
  6. ↑ Cheng HCS, Agrawal SK, Parnes LS (2018). "Stapedectomy Versus Stapedotomy". Otolaryngol Clin North Am. 51 (2): 375–392. doi:10.1016/j.otc.2017.11.008. PMID 29397948.
  7. ↑ Crompton M, Cadge BA, Ziff JL, Mowat AJ, Nash R, Lavy JA; et al. (2019). "The Epidemiology of Otosclerosis in a British Cohort". Otol Neurotol. 40 (1): 22–30. doi:10.1097/MAO.0000000000002047. PMC 6314447. PMID 30540696.
  8. ↑ Fitzgerald DC (1985). "The aging ear". Am Fam Physician. 31 (2): 225–32. PMID 3883726.
  9. ↑ Declau F, Spaendonck MV, Timmermans JP, Michaels L, Liang J, Qiu JP; et al. (2007). "Prevalence of histologic otosclerosis: an unbiased temporal bone study in Caucasians". Adv Otorhinolaryngol. 65: 6–16. doi:10.1159/000098663. PMID 17245017.
  10. ↑ Tato JM, Tato JM (1967). "Otosclerosis and races". Ann Otol Rhinol Laryngol. 76 (5): 1018–25. doi:10.1177/000348946707600512. PMID 6074235.
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