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

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Abstract

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.

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