Dilemmas in the treatment of concurrent bilateral meningoencephalocele and superior semicircular canal dehiscence

To report on a patient with bilateralmeningoencephalocele complicated by bilateral asymptomatic superior semicircular canal dehiscence and discuss dilemmas associated with his successful surgical treatment. Study Design: Case capsule report. Setting: Tertiary academic medical center. Patient: A 56-year-old man with 6 years of progressive conductive hearing loss and recent spontaneous cerebrospinal fluid leak from the right ear diagnosed as having bilateral large temporal bone meningoencephalocele (ME) and concomitant bilateral asymptomatic superior semicircular canal dehiscence (SSCD). Results: The right ME was repaired through the middle fossaapproach, and the right SSCD was plugged. To avoid the risk associated with bilaterally plugging the SSCDs, the left ME was sealed by subtotal petrosectomy, blind sac closure of the external auditory canal, plugging ofthe eustachian tube orifice, and obliteration of the residual space with an abdominal fat graft. The herniated brain and meninges were amputated, and the tissue present in the attic was not disrupted. Hearing was reconstructed with a two-staged Bone-anchored Hearing Aid procedure to avoid the risk of cerebrospinal fluid leaking through the skin defect of the Bone-anchored Hearing Aid abutment. Conclusion: Temporal bone ME can be associated with asymptomatic SSCD. We illustrate an example of how patients with bilateral pathologies can be managed effectively with good vestibular, auditory, and cosmetic results.