title: Stapedectomy in Teunissen-Cremers Syndrome: Intraoperative Findings and Hearing Outcomes.
abstract: 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.
  
  
  
  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.
authors.affiliation: *Department of Otolaryngology Head and Neck Surgery, Dunedin Hospital, Dunedin †Department of Otolaryngology Head and Neck Surgery, Christchurch Hospital, Christchurch, New Zealand.
authors.forename: Alice C
authors.initials: AC
authors.lastname: Coombs
authors.forename: Philip A
authors.initials: PA
authors.lastname: Bird
mesh_terms: D000293:Adolescent; D000328:Adult; D002348:Carpal Bones / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D004429:Ear Ossicles / Q000473:pathology / Q000601:surgery; D005260:Female; D005532:Foot Deformities, Congenital / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006228:Hand Deformities, Congenital / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006314:Hearing Loss, Conductive / Q000209:etiology* / Q000601:surgery*; D006320:Hearing Tests; D006801:Humans; D008297:Male; D008875:Middle Aged; D009998:Ossicular Prosthesis; D013199:Stapes / Q000002:abnormalities* / Q000473:pathology; D013201:Stapes Surgery / Q000379:methods*; D013580:Synostosis / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D013639:Tarsal Bones / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery