Ear surgery in osteogenesis imperfecta. Clinical findings and short-term and long-term results.

Preoperative and postoperative hearing results and long-term results of stapedectomy have been investigated in 58 ears (47 patients) with osteogenesis imperfecta. After 3 months, hearing gain had been achieved in 49 (85%) of 58 ears. Twenty-seven (68%) of 40 ears followed up for an average of 9.6 years (range, 2 to 24 years) had no deterioration of their immediate postoperative hearing gain. In the other ears, the decrease in hearing gain in the long term was due to progression of the sensorineural component of the hearing loss. Complete closure of the air-bone gap remained unchanged in 26 (70%) of 37 ears. In 5 (9%) of 58 ears the sensorineural component of the hearing loss increased as an immediate result of the operation. In 6 other ears (10%) progressive sensorineural hearing loss was seen only after more than 1 year. A natural course of the disease is assumed as the cause because progressive sensorineural hearing loss has also been seen in the nonoperated on contralateral ears of these patients.