To report a case of pediatric recurrent meningitis that is associated with an underlying petrous apex encephalocele in a patient with bilateral cochlear implants and reviews the literature of similar petrous apex lesions. A 5-year-old girl presented during her third episode of meningitis. Her first episode of meningitis left her with profound bilateral sensorineural hearing loss for which she received cochlear implants. During her assessment, she was found to have a petrous apex encephalocele, which was successfully repaired via a middle cranial fossa approach. Literature review demonstrates that petrous apex cephaloceles (PAC) are associated with meningitis, particularly in children. No previous reports exist of PAC in patients with cochlear implants. This case illustrates the importance of identifying an underlying anatomic abnormality in recurrent meningitis. PAC are rare abnormalities that can cause cerebrospinal fluid leaks leading to intracranial hypotension, cranial neuropathies, and infectious complications.
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