Int. Adv. Otol. 2010; 6(3) 415-418

Mondini dysplasia presenting as recurrent meningitis.
Cengiz Ozcan, Yusuf Vayisoglu, Kemal Gorur, Altan Yildiz, Onur İsmi, Necdet  Kuyucu, Department of Otorhinolaryngology, School of Medicine, Mersin University, Turkey

Mondini dysplasia (MD) is the most common congenital malformation of the cochlea associated with sensorineural hearing loss in variable degrees and recurrent meningitis. It occurs due impairment of the embryonic development of the inner ear during the seventh week of fetal life. Congenital anomalies of temporal bone may cause fistulisation between the middle ear and subarachnoid space. MD is often associated with spontaneous cerebrospinal fluid leakage that leads to recurrent bacterial meningitis. MD generally remains undiagnosed until the radiological investigation for hearing loss and recurrent meningitis. Computed tomography of the temporal bone is the best method of MD diagnosis. Immediate surgical closure of the defect is the treatment of choice to prevent further episodes of meningitis. In this article, we presented a case of Mondini dysplasia in a 5-year-old patient with recurrent meningitis as an initial symptom and discussed the pathogenesis of meningitis and treatment methods. We also reviewed pediatric MD cases with recurrent meningitis since 1990.