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

OBJECTIVE

To report on a patient with bilateral meningoencephalocele 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 fossa approach, 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 of the 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.