Skull Base 2008; 18(6): 423-428
DOI: 10.1055/s-0028-1087217

© Thieme Medical Publishers

Bilateral Meningoencephalocele Repair Complicated by Superior Semicircular Canal Dehiscence: Case Report

Anthony A. Mikulec1 , Aayesha M. Khan1 , Fred G. Barker2  II , Michael J. McKenna3
  • 1Department of Otolaryngology Head and Neck Surgery, Saint Louis University, Saint Louis, Missouri
  • 2Neurosurgery Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
  • 3Department of Otology and Laryngology, Harvard Medical School/Massachusetts Eye and Ear Infirmary, Boston, Massachusetts
Further Information

Publication History

Publication Date:
07 October 2008 (online)


To describe an unusual case of bilateral meningoencephaloceles with concurrent bilateral superior semicircular canal dehiscene (SSCD) and to discuss the clinical presentation, diagnosis, and treatment of SSCD. A 34-year-old man presented with unsteadiness and bilateral conductive hearing loss. He was diagnosed with bilateral meningoencephaloceles and underwent staged middle fossa approaches for repair. Following the second (right-sided) surgery, he developed sensorineural hearing loss and severe dizziness, indicating labyrinthine insult in the operated ear. He was then referred to our institution for further management. On our evaluation, the patient was continuing to experience disequilibrium and sensitivity to loud sounds. Examination revealed a positive Hennebert's sign and nystagmus consistent with symptomatic SSCD in the left ear. Computed tomography scanning with reformatting into Poeschel and Stenvers views identified bilateral SSCD. Plugging of the left SSCD was performed via a middle cranial fossa approach and resulted in improvement of the conductive hearing loss and after a period of compensation, resolution of the vestibular symptoms. This case illustrates that tegmental defects may result in simultaneous meningoencepaholcele and SSCD that may complicate their repair. The importance of having a high index of suspicion and evaluation with high resolution CT scanning with appropriate reformatting is emphasized. When present and symptomatic, SSCD can be successfully managed by plugging the canal.


Anthony A Mikulec, M.D. 

Assistant Professor, Chief, Otologic and Neurotologic Surgery, Department of Otolaryngology

3660 Vista Avenue, Suite 312, St. Louis, MO 63110

Email: [email protected]