An Elusive Diagnosis: Delays in Treatment and Opportunities for Improvement in Temporal Encephalocele and CSF Leak

Abstract

Objective: Symptoms of temporal encephalocele or cerebrospinal fluid (CSF) leak causing middle ear effusion or otorrhea can be nonspecific and mistaken for other common diagnoses, leading to delays in diagnosis, failed treatments, and a risk of meningitis. This study sought to investigate the association between symptomatology and time to definitive surgical management. Study Design: Retrospective cohort. Setting: Single tertiary care academic medical center. Patients: Adults treated surgically for temporal encephalocele or CSF leak. Revision cases were excluded. Interventions: Chart review was performed to identify pertinent symptoms at presentation. Multivariable regression was performed to analyze the association between symptoms and time to definitive management. Main Outcome Measures: Otologic and related symptoms present prior to middle cranial fossa (MCF). Time between symptom onset and surgical treatment. Results: Thirty-four patients had symptoms present a median of 15.5 months (interquartile range, 8–35 months; range, 1 month to 12 years) prior to surgery. The most common symptoms were subjective hearing loss in the affected ear (76.5%) and aural fullness (73.5%). Otorrhea was present in 55.9%, and 42.9% had a history of otorrhea after myringotomy with or without tube insertion. Meningitis occurred in 5 patients (14.7%). Only the absence of otalgia was statistically significantly associated with decreased time between symptoms onset and surgery (P = 0.01). Conclusions: Encephalocele and CSF leak were most commonly associated with aural fullness and hearing loss. Medical treatment for presumed Eustachian tube dysfunction or chronic ear disease were commonly observed. Patients had symptoms for a median of almost 1 and a half years prior to surgical management.