Otosclerosis

Common Name(s)

Otosclerosis

Clinical otosclerosis, the single most common cause of hearing impairment, is characterized by isolated endochondral bone sclerosis of the labyrinthine capsule. Otosclerotic foci invade the stapediovestibular joint (oval window) and interfere with free motion of the stapes. Mean age of onset is in the third decade and 90% of affected persons are under 50 years of age at the time of diagnosis. Approximately 10% of affected persons develop profound sensorineural hearing loss across all frequencies (summary by {16:Tomek et al., 1998}). Genetic Heterogeneity of Otosclerosis The locus associated with otosclerosis-1 (OTSC1) has been mapped to chromosome 15q26.1. Other loci associated with otosclerosis include OTSC2 ({605727}) on chromosome 7q; OTSC3 ({608244}) on chromosome 6p; OTSC4 ({611571}) on chromosome 16q; OTSC5 ({608787}) on chromosome 3q22-q24; OTSC7 ({611572}) on chromosome 6q13; OTSC8 ({612096}) on chromosome 9p13.1-q21.11; and OTSC10 ({615589}) on chromosome 1q41-q44. The symbols OTSC6 and OTSC9 were reserved by the HUGO Gene Nomenclature Committee on January 30, 2003 and February 10, 2009, respectively, for as yet unpublished loci for otosclerosis.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Otosclerosis" for support, advocacy or research.

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Vestibular Disorders Association

VEDA's mission is to serve people with vestibular (inner ear balance) disorders by providing access to information, offering a support network, and elevating awareness of the challenges associated with these conditions.

Last Updated: 20 Feb 2013

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Otosclerosis" for support, advocacy or research.

Logo
Vestibular Disorders Association

VEDA's mission is to serve people with vestibular (inner ear balance) disorders by providing access to information, offering a support network, and elevating awareness of the challenges associated with these conditions.

https://vestibular.org

Last Updated: 20 Feb 2013

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Otosclerosis" returned 102 free, full-text research articles on human participants. First 3 results:

The surgical treatment results of otosclerosis at the Department of Otolaryngology Silesian Medical University in Zabrze in years 2000-2010.
 

Author(s): Gabriela Czerwińska, Wojciech Ścierski, Grzegorz Namysłowski, Grażyna Lisowska, Maciej Misiołek

Journal: Otolaryngol Pol. 2017 Apr;71(2):16-21.

 

Otosclerosis is a cause of 5-9% of all hearing loss. The most effective treatment of otoslerosis is stapedotomy.

Last Updated: 31 Dec 1969

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[Cochlear implantation in far advanced otosclerosis: series of four cases].
 

Author(s): İsmail Yılmaz, M Volkan Akdoğan, Fulya Özer, Haluk Yavuz, Cabbar Çadırcı, Levent N N Özlüoğlu

Journal: Kulak Burun Bogaz Ihtis Derg. ;26(6):360-5.

 

In this article, we present four patients who underwent cochlear implantation due to far advanced otosclerosis. Preoperative evaluations, intraoperative findings, complications, and postoperative benefits were analyzed. Cochlear implantation is a treatment option providing excellent ...

Last Updated: 31 Dec 1969

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Does Otosclerosis Affect Dark and Transitional Cells in the Human Vestibular Labyrinth?
 

Author(s): Serdar Kaya, Michael M Paparella, Sebahattin Cureoglu

Journal: Otol. Neurotol.. 2017 02;38(2):234-238.

 

The density of vestibular dark cells (DCs) and vestibular transitional cells (TCs) can be quantitatively decreased in human temporal bones with otosclerosis.

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Otosclerosis" returned 7 free, full-text review articles on human participants. First 3 results:

Update on the imaging diagnosis of otosclerosis.
 

Author(s): J Gredilla Molinero, M Mancheño Losa, N Santamaría Guinea, N Arévalo Galeano, M Grande Bárez

Journal: Radiologia. ;58(4):246-56.

 

Otosclerosis is a primary osteodystrophy of the temporal bone that causes progressive conductive hearing loss. The diagnosis is generally clinical, but multidetector CT (MDCT), the imaging technique of choice, is sometimes necessary. The objective of this article is to systematically ...

Last Updated: 31 Dec 1969

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Juvenile otosclerosis: a case presentation and review of the literature.
 

Author(s): Konstantinos Markou, Marios Stavrakas, Petros Karkos, Georgios Psillas

Journal:

 

Otosclerosis in childhood and adolescence or juvenile otosclerosis is a rare disorder resulting in conductive hearing loss. A 9-year-old boy presented to our clinic, suffering from moderate hearing loss. According to his parents, his hearing acuity had progressively deteriorated over ...

Last Updated: 31 Dec 1969

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Cochlear otosclerosis.
 

Author(s): Sebahattin Cureoglu, Muzeyyen Y Baylan, Michael M Paparella

Journal: Curr Opin Otolaryngol Head Neck Surg. 2010 Oct;18(5):357-62.

 

The aim of this study is to summarize current advances in research and clinical aspects of cochlear otosclerosis.

Last Updated: 31 Dec 1969

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Cone Beam and CT Scan for the Diagnosis of Otosclerosis (TACOS)
 

Status: Recruiting

Condition Summary: Otosclerosis

 

Last Updated: 7 Feb 2018

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Study of Binaural Squelch Effect in Unilateral Otosclerosis
 

Status: Recruiting

Condition Summary: Otosclerosis of Middle Ear; Unilateral Hearing Loss

 

Last Updated: 18 Sep 2018

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