Overview
Osteogenesis Imperfecta-related hearing loss usually occurs in individuals with mild (type I) OI and is much earlier in onset than age-related hearing loss, with the majority of individuals experiencing some minor hearing loss in their 20s. Bisphosphonates have been successfully used to treat otosclerosis, a common cause of hearing loss similar to OI-related hearing loss. As many individuals with OI-related hearing loss also present with otosclerosis and because of their mechanistic similarities, the investigators propose studying the effects of bisphosphonate treatment on individuals diagnosed with both OI type I and hearing loss, thereby determining its effectiveness as a potential treatment for hearing loss.
The investigators will enroll 50 individuals diagnosed with type I OI and age 18-100. 25 adults will be enrolled into the treatment arm and receive bisphosphonate treatment (must have at least mild hearing loss), while 25 adults will be enrolled into the control arm. The investigators will enroll 25 children (6-17 years of age) diagnosed with OI who are currently receiving bisphosphonate treatment as part of their care for orthopedic symptoms. The investigators will also observe 25 children (6-17 years of age) diagnosed with OI who are NOT currently receiving bisphosphonate treatment. The study duration is 63 months (approximately 5 years). Enrollment is anticipated to begin in November 2019.
Eligibility
Inclusion Criteria (Adult Treatment Arm):
- Diagnosis of OI type I
- Diagnosis of at least mild hearing loss (>20dB pure tone average) by audiogram testing
- 18+
- Vitamin D level > 30
Inclusion Criteria (Adult Control Arm):
- Diagnosis of OI type I
Inclusion Criteria (Child Observational Bisphosphonate Arm)
- Diagnosis of OI
- Age 6-17 years
- Currently receiving bisphosphonate treatment as standard of care
Inclusion Criteria (Child Observational No Treatment Arm)
- Diagnosis of OI
- Age 6-17 years
- NOT receiving bisphosphonate treatment and will not receive bisphosphonate treatment for the duration of the study
Exclusion Criteria (ALL ARMS):
- Family history of hearing-loss (not related to OI or occupational hearing loss)
- Pregnancy