Syndrome of recurrent meninigitis due to congenital perilymph fistula with two different clinical presentations

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

“…Only in three patients with inner ear malformations the initial episode of meningitis occurred in adulthood, compared with 39 cases (93%) in childhood (164,165,178). In patients with cranial meningocele/meningoencephalocele, there was also a tendency to presentation in childhood (median age, 7 years), although this pattern was less clear, as almost half of the publications did not report the age at which the respective patient had experienced the first episode of meningitis.…”

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

“…Surgical packing of the vestibule to close off the defect is mandatory [3,9,11,12,22,23]. The present study showed no recurrence of CSF leakage and meningitis using TMLSCCA after a long-term follow-up (1-8.5 years).…”

“…A C C E P T E D ACCEPTED MANUSCRIPT 10 Furthermore, pathological communications between subarachnoid space and middle ear cavity may be in multiple locations, such as adjacent to, distal to, the labyrinthine [1,11,23].…”

Prevention of Meningitis

“…If cochlear implant surgery is indicated, the repair can be performed at the time of the implantation, if feasible. Since malformations and a fistula of the oval window have been reported to be associated with recurrent meningitis, 9,11,12,15 the entire stapes should be examined and repaired if a defect is uncovered. If a middle ear abnormality is diagnosed and the patient has a malformation of the contralateral inner ear, that middle ear should also be explored.…”