Results of stapedotomy in otosurgical treatment of adult patients with osteogenesis imperfecta.

OBJECTIVE

This is a retrospective study of hearing results and characteristics of osteogenesis imperfecta (OI) patients treated for hearing loss by stapedotomy at tertiary reference center.

METHODS

This study enrolled 20 patients with a clinical diagnosis of OI- (11M:9F). 18 patients (90%) underwent surgery due to hearing loss in the period 2003-16. The audiometric analysis provides the pure tone audiometry results of stapedotomy in adult patients in 2 periods (≤12 months and >12 months). Air-bone gap (ABG), hearing gain (HG), and changes in air and bone conduction thresholds after surgical treatment were analyzed.

RESULTS

In short-time follow-up we noted statistically significant improvement in mean AC thresholds and ABG (p < 0.001 for both), change in mean BC thresholds was statistically negligible. Comparing the observation periods short-term and long-term, it was found that AC thresholds, ABG, HG, ABG closure did not significantly change, although BC thresholds and BC closure deteriorated significantly (p < 0.05).

CONCLUSION

Stapes surgery for OI can be considered as a method of treating the conductive and/or mixed hearing loss suffered by these patients; however, the surgery is more difficult than that for otosclerosis because OI cases often have extremely difficult anatomical conditions. The hearing results of OI stapes surgery differ from typical otosclerosis cases, with the ABG closure not being as good. In addition, sensorineural hearing loss inevitably progresses.