Stapedectomy in Teunissen-Cremers Syndrome: Intraoperative Findings and Hearing Outcomes.

Objective: To describe the intraoperative findings and outcomes of stapedectomy surgery in Teunissen-Cremers syndrome. Patients: A family of three patients with bilateral conductive hearing loss because of Teunissen-Cremers syndrome. Intervention: Six exploratory tympanotomies and stapedectomies, including one revision operation. Main Outcome Measures: Intraoperative findings and postoperative hearing results. Results: There was an increased distance between the incus and the vestibule, a thicker long process of the incus, and slight variation in the relative position of the ossicles in all patients. Surgery resulted in closure of the air-bone gap to less than 10 dB in all five operated ears. Conclusion: We describe anatomic abnormalities of the ossicular chain in Teunissen-Cremers syndrome. Prosthesis availability should include prostheses that can adapt to these potential anatomic abnormalities. Stapedectomy resulted in good long-term hearing outcomes in this series. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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