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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.[4987] 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.[4987] The condition can also predispose affected individuals to recurrent meningitis.[4985] It is caused by disruption in the embryonic development of the inner ear during the seventh week of gestation.[4988] The condition may be isolated (occurring with no other conditions or malformations) or may occur with other ear malformations or a number of syndromes.[4986] Treatment options may include surgical repair of the defect to prevent recurrent meningitis; amplification aids for those with residual hearing; and cochlear implantation.[4989]
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