Abstract
Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF) leakage, and recurrent bacterial meningitis (RBM), which is defined as two or more episodes of meningitis separated by a period of convalescence and the complete resolution of all signs and symptoms. An accurate diagnosis of the underlying pathology is crucial to prevent further episodes from occurring. Herein, we present a three-year-old boy with RBM and unilateral sensorineural hearing loss. During the evaluation to determine the etiology of the RBM, cystic malformation in the cochlea and vestibular dilatation consistent with Mondini dysplasia were detected via computerized tomography (CT) of the temporal bone.
Highlights
Recurrent bacterial meningitis (RBM) is defined as two or more episodes of meningitis that are separated by a period of convalescence and the complete resolution of all signs, symptoms, and laboratory findings [1,2,3]
Mondini dysplasia is the most frequent anomaly associated with cerebrospinal fluid (CSF) leaks originating in the temporal bone in children
Examples of temporal bone anomalies that can lead to RBM are Mondini dysplasia, stapedial anomalies, Klippel-Feil syndrome, Pendred syndrome, petromastoid fistulae, a widened cochlear aqueduct, and Hyrtl’s fissure [1]
Summary
Recurrent bacterial meningitis (RBM) is defined as two or more episodes of meningitis that are separated by a period of convalescence and the complete resolution of all signs, symptoms, and laboratory findings [1,2,3]. The mortality rate for RBM episodes is lower than what is found with acute bacterial meningitis [2,3,4,5]. A variety of cranial and spinal anatomic defects can facilitate the migration of microorganisms to the CSF spaces. Another frequent abnormality is congenital inner ear malformations, which is the leading cause of RBM episodes. When these are present, nasopharyngeal commensal microorganisms migrate from the nasopharynx to the middle ear via the Eustachian tube and travel on to the CSF spaces through the abnormal inner ear fistulous connections. The vestibular structures and their associated neural elements may be underdeveloped
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