Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report

R. Nick Hernandez, Abhinav R. Changa, Luigi Bassani, Robert W. Jyung, James K. Liu

Research output: Contribution to journalArticlepeer-review

6 Scopus citations

Abstract

Purpose: Mondini dysplasia is a rare congenital inner ear malformation that presents with abnormal cochlear development with accompanied vestibular dilation and vestibular aqueduct enlargement. This dysfunctional anatomy provides the potential for sensorineural hearing deficits, cerebrospinal fluid leaks, and severe cases of recurrent meningitis. Methods: We present the case of a child with Mondini dysplasia who presented with unilateral hearing loss and cerebrospinal fluid (CSF) otorrhea that was surgically repaired through a combined middle fossa/transmeatal middle ear approach to alleviate any recurrence of infection and cerebrospinal fluid otorrhea. Results: Postoperatively, the patient remained neurologically stable without any further CSF leakage. CSF cultures revealed a Pseudomonas aeruginosa infection, a rare occurrence within the context of Mondini dysplasia. Retrograde bacterial spread from the external ear canal into the CSF space has been theorized as the possible pathogenesis of the resulting meningitis. The patient was successfully treated with intravenous antibiotics without any neurologic complications. Conclusions: Although Mondini dysplasia is a rare malformation, the life-threatening sequelae of meningitis that can result from the dysfunctional anatomy makes it a condition that requires elevated clinical vigilance, especially when considering children with hearing loss associated with recurrent meningitis, otorrhea, or rhinorrhea.

Original languageEnglish (US)
Pages (from-to)1613-1616
Number of pages4
JournalChild's Nervous System
Volume31
Issue number9
DOIs
StatePublished - Sep 10 2015

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Keywords

  • CSF otorrhea
  • Meningitis
  • Mondini dysplasia
  • Skull base

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