Cerebrospinal fluid otorhinorrhoea due to inner-ear malformations: clinical presentation and new perspectives in management

Abstract

In children cerebrospinal fluid (CSF) otorhinorrhoea can be due to congenital inner-ear malformations. Cochlear dysplasia associated with a defect in the stapes footplate is usually the cause of the CSF leak. Repair of the CSF leak in these cases is usually done by packing the vestibule with muscle or fascia. This traditional method of repair has a 30 to 60 per cent failure rate. CSF otorhinorrhoea due to congenital inner-ear anomalies in four patients was repaired using multiple-layer packing of the vestibule with glue, muscle and fascia, reinforced by a pedicle temporalis muscle graft. Intra-operatively, continous lumbar drainage was performed. Repair of the CSF leak was successful in four cases and no recurrence was noticed. Inner-ear magnetic resonance imaging (MRI) using three-dimensional fast-spin echo (3D FSE) T2-weighted imaging (T2WI) and 3D steady-state free precession (FIESTA) sequences was found to be a very helpful, noninvasive investigation to localize the site and route of the CSF leak. Children presenting with recurrent meningitis and CSF rhinorrhoea should be investigated for congenital inner-ear anomalies. In these cases CSF fistulae should be repaired using fibrin or cyanoacrylate glue and intra-operative continuous lumbar drainage; 3D FSE T2WI and 3D FIESTA MRI sequences of the inner ear are helpful.