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Revista Cubana de Pediatría

versión impresa ISSN 0034-7531


QUINTERO NOA, Julianis Loraine et al. Mondini dysplasia associated to recurrent bacterial meningitis-a clinical and imaging correlation. Rev Cubana Pediatr [online]. 2014, vol.86, n.1, pp.115-122. ISSN 0034-7531.

Mondini dysplasia associated to hearing loss and recurrent bacterial meningitis was described in this paper. Mondini malformation accounts for 30% of congenital anomalies in the inner ear, either unilateral or bilateral, and its main characteristic is the incomplete cochlear development causing various grades of neurosensory hypoacusis. It is considered that the occurrence of this malformation results from the disruption of the embryonal development on the 7th week of gestation when the cochlear development ceases. This is the case of 12 years-old girl who was attended to at the pediatric and otorhinolaryngology service of "William Soler" university pediatric hospital because she presented with three meningoencephalic infections from which Streptococcus pneumonia serotype 19F was isolated. It was also confirmed that she suffered severe neurosensory hypoacusis in her left ear. High-resolution computerized axial tomography of the ear (axial and coronal planes) evinced the cochlear and vestibular malformation, with tissue of the middle ear communicating directly with the inner ear of the left size at the oval window. This paper underlined the importance of the clinical suspicion of Mondini disease, and the scientific impact of the computerized axial tomography of the temporal bone in order to early diagnose the congenital fistula in the inner ear associated to recurrent bacterial meningitis.

Palabras clave : Mondini congenital malformation and severe unilateral neurosensory hypoacusis; recurrent bacterial meningitis; computerized tomography; cerebrospinal fluid fistula.

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