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.