Conclusions

This case underscores the importance of recognizing congenital inner ear malformation as a potential cause of CSF otorrhea in pediatric patients. Although myringotomy is a routine and generally safe procedure, it may unmask a previously unrecognized CSF fistula in children with underlying labyrinthine anomalies, resulting in high-flow leakage and significant risk of meningitis. Prompt intraoperative recognition, confirmatory testing, and definitive surgical repair are critical to achieving favorable outcomes. Successful management in this case required a multilayered reconstructive approach combined with permanent external auditory canal and Eustachian tube closure. Surgeons performing middle-ear procedures in children with profound sensorineural hearing loss should be aware of this condition, consider preoperative temporal bone imaging when appropriate, and be prepared to identify and temporarily manage an unexpected high-flow leak during surgery.