Clinical Characteristics, Surgical Outcomes, and Recurrence Factors of Cerebrospinal Fluid Otorrhea Associated With Inner Ear Malformations: A Retrospective Cohort Study.

OBJECTIVE

To characterize the clinical features, management, outcomes, and predictors of recurrence of cerebrospinal fluid otorrhea in patients with inner ear malformations.

METHODS

This retrospective cohort study included 40 patients with inner ear malformation-associated cerebrospinal fluid otorrhea who underwent surgical repair at our institution between January 2009 and December 2024. Data on patient demographics, clinical presentation, imaging findings, surgical management, and outcomes were collected and analyzed.

RESULTS

The cohort predominantly comprised minors (97.5%, 39/40), with a mean age of onset of 5.83 years. Young children (≤7 y) accounted for 80% (32/40) of patients. Meningitis was the most common presenting symptom (75%), and the overall incidence throughout the disease course was 97.5% (39/40). Recurrent meningitis was observed in 57.5% (23/40) of patients preoperatively, with the frequency of episodes significantly correlating with the duration of the preoperative interval. Radiologically, an incomplete partition type I (IP-I) malformation was the most prevalent inner ear anomaly, and the cerebrospinal fluid (CSF) leakage sites were almost exclusively localized to the oval window region. The initial surgical success rate was 77.5% (31/40), with most recurrences occurring within 2 years postoperatively. Causes of recurrence included reperforation at the primary leak site, misdiagnosis, missed diagnosis, and failure to address the oval window region.

CONCLUSIONS

Cerebrospinal fluid otorrhea associated with inner ear malformations primarily afflicts the pediatric population and most commonly presents as meningitis. Early and accurate diagnosis is critical for definitive management. The selection of an optimal surgical approach and meticulous repair technique are pivotal for achieving a high cure rate and minimizing the risks of failure or recurrence.