Spontaneous Otogenic CSF Leaks: A Presentation of Cases and Review of Literature and Management Options

Objective: Spontaneous otogenic CSF leak is a life-threatening problem. The types of spontaneous CSF fistulae have been previously classified into defects through, adjacent to, or distal to the otic capsule. This paper presents case reviews representative of these three different spontaneous CSF fistulae and reviews pertinent literature. The clinical evaluation and surgical management of each type of CSF fistula are reviewed, highlighting the importance of variables such as the functional status of the ear, age-related limitations, and potential for concomitant anatomic anomalies.

Study Design: The study consists of a case series and literature review.

Setting: The study was conducted at an academic tertiary referral practice.

Patients: Two patients with leaks through malformed inner ears are presented—one with persistent tympanomeningeal fissure and one with middle fossa encephalocele with leak.

Intervention(s): Skull base closure techniques, selective middle ear packing, middle fossa craniotomy and CSF diversion were interventions chosen based on hearing status and site of leak.

Main Outcome Measure(s): Resolution of CSF leakage and absence of meningitis were indicators of successful outcomes.

Results: None of the cases described in this series had return of CSF otorrhea in the postoperative period, and none of them had subsequent episodes of meningitis. Each type of CSF fistula required individualized diagnostic and therapeutic strategy with regard to preoperative workup, type of surgical procedure, adjunctive lumbar drainage and postoperative functional expectations.

Conclusions: Otogenic CSF fistulae can occur through, adjacent to, and distant to the otic capsule. Identification of leak type and concomitant potential pitfalls impact treatment options and outcomes.