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Mondini dysplasia

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.[1] 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.[1] The condition can also predispose affected individuals to recurrent meningitis.[2] It is caused by disruption in the embryonic development of the inner ear during the seventh week of gestation.[3] The condition may be isolated (occurring with no other conditions or malformations) or may occur with other ear malformations or a number of syndromes.[4] Treatment options may include surgical repair of the defect to prevent recurrent meningitis; amplification aids for those with residual hearing; and cochlear implantation.[5]
Last updated: 2/24/2012


  1. Masri A. et al. Mondini malformation associated with diastematomyelia and presenting with recurrent meningitis. J Child Neurol. May 2011; 26(5):622-624.
  2. 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;
  3. Liu FC, Chen PY, Huang FL, Lee CY, Lin CF. Recurrent bacterial meningitis in a child with mondini dysplasia. Clin Pediatr (Phila). November 2009; 48(9):975-977.
  4. 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; 9:167.
  5. Tullu MS, Khanna SS, Kamat JR, Kirtane MV. Mondini dysplasia and pyogenic meningitis. Indian J Pediatr. July 2004; 71(7):655-657.
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