OBJECTIVE: To present power stapes, stapedotomy, and middle ear implantation with Vibrant SoundBridge (VSB) performed in a one-step surgery as an alternative option for hearing rehabilitation in patients with osteogenesis imperfecta (OI). STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral ear center. PATIENTS: A family with genetically proven OI Type I. INTERVENTIONS: Two patients, mother and son, with severe to profound mixed hearing loss underwent 3 power stapes, 1 unilateral and 1 bilateral sequential. MAIN OUTCOME MEASURES: Thorough audiological diagnostic batteries including aided and unaided pure-tone and free-field audiometry and Freiburg monosyllabic word test were used to assess the preoperative status and the postoperative hearing outcome. High-resolution computed tomography of the temporal bones was performed as well. Surgical procedure and any special considerations were analyzed in detail. RESULTS: The hearing outcome was favorable in all cases, showing in comparison to the preoperative values an average improvement of 36.8 dB. Severe intraoperative bleeding of the middle ear mucosa was the only complication and could be easily controlled by allowing short time intervals. Inner ear trauma did not occur in any case. CONCLUSION: Power stapes represents a safe and promising procedure for treating hearing loss in selected patients with OI. Furthermore, it introduces a new, advantageous VSB application in cases of mixed hearing loss with severe otosclerosis and increased bone-conduction thresholds.
OBJECTIVE: To present power stapes, stapedotomy, and middle ear implantation with Vibrant SoundBridge (VSB) performed in a one-step surgery as an alternative option for hearing rehabilitation in patients with osteogenesis imperfecta (OI). STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral ear center. PATIENTS: A family with genetically proven OI Type I. INTERVENTIONS: Two patients, mother and son, with severe to profound mixed hearing loss underwent 3 power stapes, 1 unilateral and 1 bilateral sequential. MAIN OUTCOME MEASURES: Thorough audiological diagnostic batteries including aided and unaided pure-tone and free-field audiometry and Freiburg monosyllabic word test were used to assess the preoperative status and the postoperative hearing outcome. High-resolution computed tomography of the temporal bones was performed as well. Surgical procedure and any special considerations were analyzed in detail. RESULTS: The hearing outcome was favorable in all cases, showing in comparison to the preoperative values an average improvement of 36.8 dB. Severe intraoperative bleeding of the middle ear mucosa was the only complication and could be easily controlled by allowing short time intervals. Inner ear trauma did not occur in any case. CONCLUSION: Power stapes represents a safe and promising procedure for treating hearing loss in selected patients with OI. Furthermore, it introduces a new, advantageous VSB application in cases of mixed hearing loss with severe otosclerosis and increased bone-conduction thresholds.
Authors: Burkard Schwab; Eugen Kludt; Hannes Maier; Thomas Lenarz; Magnus Teschner Journal: Eur Arch Otorhinolaryngol Date: 2015-06-20 Impact factor: 2.503
Authors: Daniel Dejaco; David Riedl; Timo Gottfried; Thomas Rasse; Natalie Fischer; David Prejban; Viktor Koci; Herbert Riechelmann; Joachim Schmutzhard; Thomas Keintzel Journal: Otol Neurotol Date: 2019-03 Impact factor: 2.311
Authors: Daniel Dejaco; David Riedl; Anna Elisabeth Cassar; Timo Gottfried; Thomas Rasse; Natalie Fischer; Armina Kreuzer-Simonyan; Josef Seebacher; Herbert Riechelmann; Thomas Keintzel; Joachim Schmutzhard Journal: Otol Neurotol Date: 2022-04-01 Impact factor: 2.311