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Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report.
Childs Nerv Syst. 2015 Sep; 31(9):1613-6.CN

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.

Authors+Show Affiliations

Department of Neurological Surgery, New Jersey Medical School, Rutgers University, 90 Bergen Street, Newark, NJ, 07103, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

26201554

Citation

Hernandez, R Nick, et al. "Cerebrospinal Fluid Otorrhea and Pseudomonal Meningitis in a Child With Mondini Dysplasia: Case Report." Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery, vol. 31, no. 9, 2015, pp. 1613-6.
Hernandez RN, Changa AR, Bassani L, et al. Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report. Childs Nerv Syst. 2015;31(9):1613-6.
Hernandez, R. N., Changa, A. R., Bassani, L., Jyung, R. W., & Liu, J. K. (2015). Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report. Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery, 31(9), 1613-6. https://doi.org/10.1007/s00381-015-2836-x
Hernandez RN, et al. Cerebrospinal Fluid Otorrhea and Pseudomonal Meningitis in a Child With Mondini Dysplasia: Case Report. Childs Nerv Syst. 2015;31(9):1613-6. PubMed PMID: 26201554.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report. AU - Hernandez,R Nick, AU - Changa,Abhinav R, AU - Bassani,Luigi, AU - Jyung,Robert W, AU - Liu,James K, Y1 - 2015/07/23/ PY - 2015/03/10/received PY - 2015/07/14/accepted PY - 2015/7/24/entrez PY - 2015/7/24/pubmed PY - 2016/6/4/medline SP - 1613 EP - 6 JF - Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery JO - Childs Nerv Syst VL - 31 IS - 9 N2 - 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. SN - 1433-0350 UR - https://www.unboundmedicine.com/medline/citation/26201554/Cerebrospinal_fluid_otorrhea_and_pseudomonal_meningitis_in_a_child_with_Mondini_dysplasia:_case_report_ L2 - https://doi.org/10.1007/s00381-015-2836-x DB - PRIME DP - Unbound Medicine ER -