Overview

Effects of Bisphosphonates on OI-Related Hearing Loss

Status:
Recruiting
Trial end date:
2024-11-01
Target enrollment:
0
Participant gender:
All
Summary
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, ages 18-100, who have documented hearing loss. 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.
Phase:
Phase 4
Accepts Healthy Volunteers?
Accepts Healthy Volunteers
Details
Lead Sponsor:
Hospital for Special Surgery, New York
Collaborators:
Northwell Health
The New York Community Trust
Weill Cornell Medicine
Weill Medical College of Cornell University
Treatments:
Diphosphonates
Risedronic Acid
Criteria
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 (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