Translabyrinthine Fistula Closure in Inner Ear Malformations with Otogenic Cerebrospinal Fluid Leak

Area of Science:

• Neurosurgery
• Otolaryngology
• Pediatric Medicine

Background:

• Inner ear malformations can lead to cerebrospinal fluid (CSF) leakage into the subarachnoid space, causing otogenic meningitis.
• Recurrent meningitis from persistent CSF leaks is associated with poor neurological outcomes, necessitating timely diagnosis and surgical intervention.
• Current surgical strategies like middle ear and inner ear obliteration lack standardized definitions and consistent effectiveness.

Purpose of the Study:

• To describe and evaluate a translabyrinthine fistula closure technique for treating pediatric inner ear malformations with recurrent meningitis.
• To clarify the classification, indications, and limitations of surgical strategies for otogenic CSF leakage.

Main Methods:

• Two pediatric patients with incomplete partition type I (IP-I) malformation and recurrent meningitis underwent translabyrinthine fistula closure via the lateral semicircular canal.
• The technique involved direct identification and sealing of the fistula using a three-layer construct under direct visualization.
• Surgical approach was chosen based on high vestibular CSF pressure and internal auditory canal fundus hypoplasia.

Main Results:

• Both patients experienced successful and secure fistula closure with no recurrence of meningitis during long-term follow-up (>8 years for Case 1, >1 year for Case 2).
• The intervention was effective even in a patient refractory to multiple previous window closure surgeries.
• No postoperative complications were reported, though inner ear function was sacrificed.

Conclusions:

• Translabyrinthine fistula closure is a potentially effective surgical option for managing cerebrospinal fluid leakage in severe inner ear malformations, particularly in refractory cases.
• This technique offers a viable treatment for recurrent meningitis associated with these malformations, even at the expense of hearing.
• The study contributes to clarifying surgical approaches for otogenic CSF leakage by presenting a case-based review.