Mondini Dysplasia

Common Name(s)

Mondini Dysplasia

Mondini dysplasia is a type of inner ear malformation that is present at birth (congenital). Individuals with Mondini dysplasia have one and a half coils of the cochlea instead of the normal two coils. It may occur in one ear (unilateral) or both ears (bilateral) and can cause varying degrees of sensorineural hearing loss, although most individuals have profound hearing loss. The condition can also predispose affected individuals to recurrent meningitis. It is caused by disruption in the embryonic development of the inner ear during the seventh week of gestation. The condition may be isolated (occurring with no other conditions or malformations) or may occur with other ear malformations or a number of syndromes. Treatment options may include surgical repair of the defect to prevent recurrent meningitis; amplification aids for those with residual hearing; and cochlear implantation.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

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

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

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 "Mondini Dysplasia" returned 6 free, full-text research articles on human participants. First 3 results:

The development of auditory skills in young children with Mondini dysplasia after cochlear implantation.
 

Author(s): Xueqing Chen, Fei Yan, Bo Liu, Sha Liu, Ying Kong, Jun Zheng, Yongxin Li, Shusheng Gong, Demin Han, Luo Zhang

Journal:

 

The aim of this study is to survey and compare the development of auditory skills in young children with Mondini dysplasia and profoundly-deaf young children with radiologically normal inner ears over a period of 3 years after cochlear implantation. A total of 545 young children (age ...

Last Updated: 24 Sep 2014

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Recurrent bacterial meningitis in a child with hearing impairment, mondini dysplasia: a case report.
 

Author(s): Behdad Gharib, Sara Esmaeili, Golnaz Shariati, Narges Mazloomi Nobandegani, Mehrzad Mehdizadeh

Journal: Acta Med Iran. 2012 ;50(12):843-5.

 

Recurrent bacterial meningitis is not a common disease and makes physicians seek underlying predisposing factors which can result from anatomic anomalies or immunodeficiency. In this paper we present a boy with recurrent bacterial meningitis with the history of trauma and sensorineural ...

Last Updated: 4 Mar 2013

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Mondini dysplasia presenting as otorrhea without meningitis.
 

Author(s): Chien-Yu Lin, Hung-Ching Lin, Chun-Chih Peng, Kuo-Sheng Lee, Nan-Chang Chiu

Journal: Pediatr Neonatol. 2012 Dec;53(6):371-3.

 

Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed ...

Last Updated: 1 Jan 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 "Mondini Dysplasia" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.