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Published on: September 11, 2020
A case of Mondini dysplasia with recurrent Streptococcus pneumoniae meningitis
Dilek Yilmaz Ciftdoğan1, Nuri Bayram, Yasemin Ozdemir
1Division of Pediatric Infectious Diseases, Department of Pediatrics, Faculty of Medicine, Ege University, Bornova, Izmir 35100, Turkey. drdilekyilmaz@hotmail.com
Abstract:
Mondini's dysplasia is a developmental anomaly of the middle ear characterized by cochlear malformation with dilation of the vestibular aquaduct, vestibule, and ampullar ends of the semicircular canals. These deformities may result in a connection between subarachnoid space and the middle ear resulting in recurrent episodes of meningitis. Additionally, it is commonly associated with hearing impairment. We describe here a boy with recurrent meningitis and unilateral sensorineural hearing loss. Mondini dysplasia was demonstrated with computed tomographic scans of the temporal bones in the search for pathogenesis of recurrent meningitis.
Insights
Mondini dysplasia, a middle ear anomaly, can cause recurrent meningitis and hearing loss. This case highlights the connection between cochlear malformation and these serious conditions.
Area of Science:
- Otolaryngology
- Neurology
- Genetics
Background:
- Mondini dysplasia is a congenital malformation of the inner ear.
- It involves cochlear hypoplasia and abnormalities of the vestibular aqueduct and semicircular canals.
- This can lead to cerebrospinal fluid (CSF) leaks, increasing meningitis risk.
Observation:
- A pediatric case presenting with recurrent meningitis and unilateral sensorineural hearing loss.
- Computed tomographic (CT) scans of the temporal bones were utilized for diagnosis.
- The scans revealed characteristic features of Mondini dysplasia.
Findings:
- The patient exhibited cochlear malformation and dilated vestibular and semicircular canal structures.
- A potential communication pathway between the subarachnoid space and the middle ear was identified.
- Unilateral sensorineural hearing loss was confirmed.
Implications:
- Mondini dysplasia is a significant risk factor for recurrent bacterial meningitis in children.
- Early identification through imaging is crucial for managing hearing impairment and preventing meningitis.
- Understanding the pathogenesis aids in developing targeted diagnostic and management strategies.
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