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Genetic and Rare Diseases Information Center (GARD)

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Mondini dysplasia

Other Names for this Disease
  • Familial nonsyndromal Mondini dysplasia (subtype)
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How might Mondini dysplasia be treated?

Surgery to repair the defect present with Mondini dysplasia is typically necessary to prevent recurrent meningitis.[1][2] Prophylactic antimicrobial therapy (such as antibiotics) to prevent infection and conjugate pneumococcal vaccination are helpful in reducing the formation of bacteria in affected individuals.[1] If an individual has residual hearing, hearing amplification aids may be useful.[3] The use of cochlear implants to treat patients with inner ear malformations such as Mondini dysplasia has been increasingly successful. Various results of cochlear implantation in individuals with Mondini dysplasia have been reported in the literature.[3]
Last updated: 2/27/2012

  1. 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.
  2. Tullu MS, Khanna SS, Kamat JR, Kirtane MV. Mondini dysplasia and pyogenic meningitis. Indian J Pediatr. July 2004; 71(7):655-657.
  3. Daneshi A, Hassanzadeh S, Abasalipour P, Emamdjomeh H, Farhadi M. Cochlear implantation in Mondini dysplasia. ORL J Otorhinolaryngol Relat Spec. January-February 2003; 65(1):39-44.

Clinical Trials & Research for this Disease

  • lists trials that are studying or have studied Mondini dysplasia. Click on the link to go to to read descriptions of these studies.