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

Abstract

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.

Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

“…In some instances where insufficient data were presented in the manuscript to categorize cases adequately, the authors were contacted and asked to provide the missing details. Publications with substantial remaining uncertainties or insufficient details were not included (71,89,182,200,207,213).…”

Long-term outcomes of a transmastoid lateral semicircular canal approach to congenital CSF otorrhea in children associated with recurrent meningitis and severe inner ear malformation

“…However, with malformed ears, particularly in cases with severe inner ear malformations of IP-Type I and common cavity, greater CSF pressure acts on the media wall of the middle ear because of fundus deficiency as well as lack of modulus [8,17,32]. Therefore, with the classical stapedectomy approach, i.e., packing the CSF leakage through the oval window direction from middle ear cavity to the vestibule, several previous studies have indicated various recurrence rates due to packing materials not being strong enough to withstand the high-pressure of CSF, even CSF drainage and post-operation medication were used [12,16,18]. Wetmore et al showed that 54% (30/56) cases of childhood CSF otorrhea had undergone an average of three operations most of which were stapedectomy with packing of the vestibule with muscle graft [3].…”

“…Furthermore, to strengthen the stability of the packing materials, and reduce recurrent of CSF otorrhea, revised techniques of stapedectomy approach were reported [11,18,24]. For example, Wilson et al [11] performed blind sac closure of the external auditory canal to obtain a vigorous obliteration of the Eustachian tube and middle ear cavity, whereas, Stevenson et al [24] placed grafts around the stapes footplate to prevent the possibility of a CSF gusher, particularly in case of severe inner ear malformation.…”

“…For example, Wilson et al [11] performed blind sac closure of the external auditory canal to obtain a vigorous obliteration of the Eustachian tube and middle ear cavity, whereas, Stevenson et al [24] placed grafts around the stapes footplate to prevent the possibility of a CSF gusher, particularly in case of severe inner ear malformation. In addition, Tyagi et al [18] M A N U S C R I P T A C C E P T E D ACCEPTED MANUSCRIPT 8 described a multiple layer repair modality, including packing of the vestibule with muscle, fascia and glue, and reinforcing the repair with a pedicled temporalis muscle graft, as well as an intra and postoperative lumbar drainage of CSF. Unfortunately, as the literatures has indicated, packing of the vestibule through stapedectomy approach with muscle or fascia still has a high failure rate (i.e., 30-60%) [3,10,18,19].…”

“…Repeated surgery may be needed to achieve an ear that is 'safe' without recurrent meningitis particularly with severe inner ear malformation, in which the greater CSF pressure acts directly on the oval window region [8,17]. Various studies have shown that recurrence of CSF leak can reach 30-60% [10,18,19].…”