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Intrathecal Application of a Fluorescent Dye for the Identification of Cerebrospinal Fluid Leaks in Cochlear Malformation
Published on: February 29, 2020
[Mondini dysplasia: traumatic cerebrospinal fluid otorrhea with meningitis]
H Kaftan1, M Adamaszek, W Hosemann
1Klinik und Poliklinik für Hals-, Nasen- und Ohrenkrankheiten, Kopf- und Hals-Chirurgie, Ernst-Moritz-Arndt-Universität Greifswald. H.Kaftan@web.de
Insights
Mondini dysplasia, a rare inner ear malformation, can lead to hearing loss and meningitis. Unsuspected cases may present with cerebrospinal fluid leaks after head trauma, requiring surgical intervention.
Area of Science:
- Otolaryngology
- Neurology
- Genetics
Background:
- Mondini dysplasia is a congenital inner ear anomaly characterized by malformation of the cochlea.
- It often leads to sensorineural hearing loss and vestibular dysfunction.
- Patients are at increased risk for spontaneous cerebrospinal fluid (CSF) leaks and meningitis.
Observation:
- A 65-year-old man with undiagnosed unilateral Mondini dysplasia presented with meningoencephalitis.
- The condition was precipitated by minor head trauma, leading to a CSF leak.
- Two surgical procedures were required to successfully repair the defect.
Findings:
- Undiagnosed Mondini dysplasia can remain asymptomatic for decades.
- Head trauma can trigger serious complications like CSF leaks and meningitis in affected individuals.
- Early diagnosis and awareness are crucial for managing this condition.
Implications:
- Patients with Mondini dysplasia require counseling regarding the risks associated with head trauma.
- Prompt diagnosis and management are essential to prevent severe neurological complications.
- This case highlights the importance of considering rare congenital malformations in adult-onset neurological symptoms.
Abstract:
Mondini dysplasia is a rare malformation of the inner ear commonly associated with loss of hearing and vestibular function. Children with Mondini dysplasia are predisposed to developing a spontaneous cerebrospinal fluid (CSF) leak and recurrent meningitis. If there is no CSF leak but a unilateral hearing loss, the condition may go undiagnosed for years. We describe a 65-year-old man with unrecognized unilateral Mondini dysplasia who presented with CSF leak and meningoencephalitis after minor head trauma. Two operative interventions were undertaken to close the defect properly. Patients with Mondini dysplasia or their parents should be cautioned about the potential hazards of any head trauma.
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