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reserved by the HUGO Gene Nomenclature Committee on January 30, 2003 and February 10, 2009, respectively, for as yet unpublished loci for otosclerosis.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 58 free, full-text research articles on human participants. First 3 results:

Correlation of computed tomography with histopathology in otosclerosis.
 

Author(s): Alicia M Quesnel, Gul Moonis, Jason Appel, Jennifer T O'Malley, Michael J McKenna, Hugh D Curtin, Saumil N Merchant

Journal: Otol. Neurotol.. 2013 Jan;34(1):22-8.

 

Until now, the use of computed tomography (CT) in the diagnosis and evaluation of otosclerosis has been based on correlation of radiologic findings to patient histories, intraoperative examinations, and audiologic data. The purpose of this study was to compare CT findings in otosclerosis ...

Last Updated: 13 Dec 2012

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Undiagnosed severe cochlear otosclerosis as a cause of profound hearing loss.
 

Author(s): Christina Hederstierna, Sebahattin Cureoglu, Michael M Paparella

Journal: Otol. Neurotol.. 2013 Apr;34(3):e14-5.

 

Last Updated: 15 Mar 2013

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Oval window niche height: quantitative evaluation with CT before stapes surgery for otosclerosis.
 

Author(s): E Ukkola-Pons, D Ayache, Y Pons, M Ratajczak, C Nioche, M Williams

Journal: AJNR Am J Neuroradiol. 2013 May;34(5):1082-5.

 

Stapes surgery for otosclerosis can be challenging when the oval window niche is narrow. We analyzed the reliability of CT to evaluate the height of the OWN and propose a quantitative criterion to distinguish normal and narrow OWNs.

Last Updated: 16 May 2013

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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 5 free, full-text review articles on human participants. First 3 results:

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: 9 Sep 2010

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Pediatric otosclerosis: case report and literature review.
 

Author(s): Raquel Salomone, Paulo Emmanuel Riskalla, Andy de Oliveira Vicente, Maria Carmela Cundari Boccalini, Adriana Gonzaga Chaves, Renata Lopes, Gilberto Bolivar Felin Filho

Journal: Braz J Otorhinolaryngol. ;74(2):303-6.

 

Otospongiosis is an osteodystrophy of the temporal bone, characterized by disordered neoformation and deposition of bone, characterized by the presence of a progressive conductive, sensorineural or mixed hearing loss and tinnitus. Typically, otospongiosis presents as a slowly progressive ...

Last Updated: 23 Jun 2008

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[Otosclerosis imaging: matching clinical and imaging data].
 

Author(s): F Veillon, J L Stierle, J Dussaix, L Ramos-Taboada, S Riehm

Journal: J Radiol. 2006 Nov;87(11 Pt 2):1756-64.

 

Otosclerosis is a primitive osteodystrophia of the labyrinthine bone. Its diagnosis must be confirmed by a CT scan in order to eliminate the other causes that may lead to conductive hearing loss with an absence of stapedial reflex: fixation of the head of the malleus to the lateral ...

Last Updated: 24 Nov 2006

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Comprehensive Wide Bandwidth Test Battery of Auditory Function in Veterans
 

Status: Not yet recruiting

Condition Summary: Hearing Loss, Sensorineural; Hearing Loss, Conductive; Hearing Loss, Mixed Conductive-Sensorineural; Otosclerosis; Tympanic Membrane Perforation; Otitis Media With Effusion; Cholesteatoma

 

Last Updated: 21 May 2014

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Clinical Assessment of the External Sound Processor Worn by Patients Implanted With Codacs
 

Status: Recruiting

Condition Summary: Severe to Profound Mixed Hearing Loss

 

Last Updated: 3 Jun 2014

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