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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
Otosclerosis

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: Hussam@med.umich.edu

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