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

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

Otol Neurotol. 2016 Aug 12;

Authors: Coombs AC, Bird PA

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.

PMID: 27525709 [PubMed - as supplied by publisher]

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