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Updated: Jul 19, 2026
Intrathecal Application of a Fluorescent Dye for the Identification of Cerebrospinal Fluid Leaks in Cochlear Malformation
Published on: February 29, 2020
[Surgical management of Mondini dysplasia with cerebrospinal fluid leakage]
Weirnin Li1, Dongyi Han, Weiyan Yang
1Department of Otolaryngology-Head and Neck Surgery, General Hospital of PLA, Beijing, 100853, China.
Objective:
To investigate the etiology, clinical presentation, surgical treatment and prevention of Mondini dysplasia with cerebrospinal fluid otorrhea.
Method:
Fifteen cases of Mondini dysplasia were collected from our department during 1998-2004. The clinical presentations, surgical treatment and image characters were analysed. Surgical therapy methods included: checking fenestra vestibuli, fenestra cochlear or other fistula and repairing the fistulas by plugging with fascial tissue via tympanoplastic approach with endaural incision.
Result:
Severe sensorineural hearing loss and spontaneous recrudescent meningitis were the characteristics of Mondini dysplasia. The leakage was from fenestra vestibuli, fenestra cochlear or other fragile region nearby. In these cases, ten cases underwent one plugging operation and cured, five cases underwent two or three operation and cured. All these cases had no leakage or meningitis recrudescence after ten months follow up.
Conclusion:
Repairing fistula via tympanoplastic approach is an effective therapeutic method. Avoiding nosogenesis, fitting hearing aid or taking cochlear implantation are important means of prevention and cure.
Insights
Mondini dysplasia, characterized by hearing loss and meningitis, can be effectively treated by surgically repairing cerebrospinal fluid fistulas. Prevention strategies include avoiding nosogenesis and utilizing hearing aids or cochlear implants.
Area of Science:
- Otolaryngology
- Neurosurgery
- Genetics
Context:
- Mondini dysplasia is a congenital inner ear malformation.
- Cerebrospinal fluid (CSF) otorrhea is a rare but serious complication.
- This condition presents challenges in diagnosis and management.
Purpose:
- To explore the causes, symptoms, surgical interventions, and preventive measures for Mondini dysplasia with CSF otorrhea.
- To analyze clinical data from 15 patients diagnosed with Mondini dysplasia.
Summary:
- The study reviewed 15 cases of Mondini dysplasia, noting severe sensorineural hearing loss and recurrent meningitis as key features.
- CSF leakage originated from fistulas in the vestibule or cochlea.
- Surgical repair via a tympanoplastic approach with fascial plugging was effective, with all patients cured after one or multiple operations and showing no recurrence during a ten-month follow-up.
Impact:
- Surgical repair of fistulas is a successful treatment for Mondini dysplasia with CSF otorrhea.
- Preventive measures and long-term management involve avoiding iatrogenic complications, and considering hearing aids or cochlear implantation for hearing restoration.
