Recurrent meningitis secondary to idiopathic oval window CSF leak

Abstract

Bacterial meningitis remains a life-threatening infection even in the present antibiotic era; thus, any abnormality which predisposes a patient to a recurrence of this serious disease, must be identified and corrected. This report describes the histroy of a 12-year old boy with a profound neurosensory hearing loss, a related absence of vestibular function and a Mondini-type of temporal bone dysplasia who developed recurrent episodes of meningitis which were due to an idiopathic cerebrospinal fluid otorrhea. Even though the meningitis was labyrinthogenic in origin, the patient did not experience the associated symptoms of hearing loss and/or vertigo since the affected inner ear was clinically unreactive. By surgically exploring the middle ear, the presence of a cerebrospinal fluid otorrhea was confirmed. The leak was observed to be coming from a defect in the stapes footplate, and it was controlled by firmly packing the inner ear vestibule with muscle. A remarkable similarity exists between the patient described above and the 15 previously reported cases of meningitis due to a spontaneous cerebrospinal fluid otorrhea. Generally, the problem occurred in young children, the average age being 6.4 years; male and female were equally afflicted. All 15 previously reported cases had a severe neurosensory hearing loss which was unilateral in 10 individuals and bilateral in the other five. In 11 of the case reports, the vestibular function was evaluated, and the labyrinth was noted to be unreactive in the affected ear. An associated congenital abnormality of the inner ear was described in 11 of the patients reviewed. Anatomically, in 13 cases, the leak was observed to be coming from the oval window area. Other affected sites included one report of a fissure of the promontory and one report of a defect in the roof of the eustachian tube. Multiple surgical procedures were required in 11 of the 15 patients in order to identify the exact source of the otorrhea and to seal it permanently. In three cases, the successful procedure was a middle ear exploration with stapedectomy and packing of the inner ear vestibule. Overall, a total of 36 operations was performed in the 15 patients reviewed. In conclusion, when the physician is confronted by a case of meningitis in a patient with a unilateral or bilateral total loss of hearing and vestibular function, the possible presence of an idiopathic cerebrospinal fluid leak should be considered, expecially if radiographic studies demonstrate a temporal bone dysplasia. In these selected cases, if the etiology of the meningitis is obscure, a middle ear exploration should be performed both for diagnostic purposes as a means to ascertain definitely the presence of a leak and for therapeutic purposes to seal it effectively.