Congenital inner ear deformity with spontaneous cerebrospinal fluid leakage: a case series report of 9 children

Abstract

Objective To investigate the clinical features, diagnosis and treatment of congenital inner ear deformity with spontaneous cerebrospinal fluid leakage in children. Methods Clinical data of 9 children suffering from inner ear deformity with spontaneous cerebrospinal fluid leakage treated in our hospital from 2012 to 2021 were collected retrospectively. The clinical manifestations, results of auxiliary examinations, surgical management and outcomes were analyzed. Results All the 9 children had unilateral disease, including 1 case in the left ear and 8 cases in the right ear, and were accompanied with fever. Some of them had the symptoms like headache, vomiting, convulsion, otorrhea and earache. Hearing examination indicated more severe sensorineural hearing loss, and CT scanning indicated bone defects in the bottom of the inner hear duct. All the children were complicated with bacterial meningitis, and 4 children with common cavity also had different severities of hydrocephalus, status convulsion secondary epilepsy and peripheral facial paralysis. All the 9 cases were repaired through mastoid approach. Eight children were successfully operated at the first time, and one underwent second operation 2 months after first surgery. All the children were followed up from 3 months to 2 years, and no recurrence was found. Conclusion Congenital inner ear deformity with spontaneous cerebrospinal fluid otorrhea is often found in children due to symptoms related to bacterial meningitis. Timely hearing examination and high resolution CT scanning of temporal bone are helpful for early diagnosis. Surgery is the most effective treatment.

Keywords

• congenital inner ear malformation
• spontaneous cerebrospinal fluid leakage
• diagnosis
• case series report