Stapedectomy in Teunissen–Cremers Syndrome: Intraoperative Findings and Hearing Outcomes

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To describe the intraoperative findings and outcomes of stapedectomy surgery in Teunissen–Cremers syndrome.


A family of three patients with bilateral conductive hearing loss because of Teunissen–Cremers syndrome.


Six exploratory tympanotomies and stapedectomies, including one revision operation.

Main Outcome Measures:

Intraoperative findings and postoperative hearing 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.


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.

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