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
R Nick HernandezJames K Liu

Abstract

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. 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. 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. Although Mondini dysplasia is a rare malformation, the life-threatening sequelae of meningitis that can result from the dysfunctional ana...Continue Reading

References

May 1, 1990·Archives of Otolaryngology--head & Neck Surgery·L A OhlmsR J Smith
Oct 1, 1972·Archives of Otolaryngology·P Illum
Aug 23, 2000·Revista clínica española·A Rodríguez GuardadoJ M Arribas Castrillo
Mar 7, 2002·International Journal of Pediatric Otorhinolaryngology·Yiqing ZhengMichael M Paparella
Dec 4, 2002·The Laryngoscope·Levent Sennaroglu, Isil Saatci
Jan 2, 2013·Pediatrics and Neonatology·Chien-Yu LinNan-Chang Chiu

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Citations

Jan 23, 2019·Journal of Zhejiang University. Science. B·Bing WangBing Chen
Jun 6, 2020·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Emilie BoisThierry Van Den Abbeele
Jun 5, 2020·International Journal of Pediatric Otorhinolaryngology·Wenmin DengXiangmin Zhang

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