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Other Names for this Disease
- Familial nonsyndromal Mondini dysplasia (subtype)
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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.
- Masri A. et al. Mondini malformation associated with diastematomyelia and presenting with recurrent meningitis. J Child Neurol. May 2011.
- Kamata A, Obinata K, Kon N, Suganuma H, Niizuma T, Kinoshita K. Recurrent meningitis with Mondini dysplasia after the operation and vaccination. Pediatr Int. April 2010.
- Liu FC, Chen PY, Huang FL, Lee CY, Lin CF. Recurrent bacterial meningitis in a child with mondini dysplasia. Clin Pediatr (Phila). November 2009.
- Huang S. et al. Extremely discrepant mutation spectrum of SLC26A4 between Chinese patients with isolated Mondini deformity and enlarged vestibular aqueduct. J Transl Med. September 2011.
- Tullu MS, Khanna SS, Kamat JR, Kirtane MV. Mondini dysplasia and pyogenic meningitis. Indian J Pediatr. July 2004.
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- PubMed is a searchable database of medical literature and lists journal articles that discuss Mondini dysplasia. Click on the link to view a sample search on this topic.