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Surgical Treatment of Hearing Loss When Otosclerosis Coexists With Superior Semicircular Canal Dehiscence Syndrome

Pritchett, Cedric V.*; Spector, Matthew E.; Kileny, Paul R.*; Heidenreich, Katherine D.*; El-Kashlan, Hussam K.*

doi: 10.1097/MAO.0000000000000470

Objective Document a case of bilateral otosclerosis with coexisting bilateral superior semicircular canal dehiscence syndrome and the treatment of hearing loss in this setting.

Patient A 33-year-old woman presented with bilateral mixed hearing loss; worse in the left ear. This was gradual in onset, and she denied dizziness. Computerized tomographic scan revealed fenestral otosclerosis and a large dehiscence of the superior semicircular canal bilaterally. She declined amplification.

Intervention Sequential laser-assisted stapedotomy with insertion of a Kurz titanium CliP Piston prosthesis.

Main Outcome Measure Comparison of audiovestibular symptoms, hearing thresholds, and neurodiagnostic testing results preoperatively and postoperatively.

Results Hearing improved bilaterally with closure of the air-bone gaps at most frequencies, and she has not had permanent vestibular symptoms. Postoperative follow-up time is 37 months for the left ear and 13 months for the right ear.

Conclusion When otosclerosis and superior semicircular canal dehiscence syndrome coexist and hearing loss is the dominant symptom, stapes surgery can be effective for improving hearing without permanent vestibular symptoms.

*Divisions of Otology-Neurotology, and †Head and Neck Surgery, Department of Otolaryngology–Head and Neck Surgery, University of Michigan Health System, Ann Arbor, Michigan, U.S.A.

Address correspondence and reprint requests to Hussam El-Kashlan, M.D., Division of Otology-Neurotology, Department of Otolaryngology–Head and Neck Surgery, University of Michigan Health System, 1904 Taubman Center, 1500 E. Medical Center Dr, Ann Arbor, MI, U.S.A.; E-mail:

Dr. Kileny had past consulting relationship with GN Otometrics.

Sources of funding: Dr. Kileny has a current grant from the U.S. Navy, Office of Naval Research.

Copyright © 2014 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company