Title

Effects of Bisphosphonates on OI-Related Hearing Loss
Effects of Bisphosphonates on OI-Related Hearing Loss: A Pilot Study
  • Phase

    Phase 4
  • Study Type

    Interventional
  • Status

    Recruiting
  • Study Participants

    100
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.
Study Started
Nov 02
2019
Primary Completion
Nov 30
2024
Anticipated
Study Completion
Nov 30
2024
Anticipated
Last Update
Apr 08
2022

Drug Risedronate Oral Tablet

Oral bisphosphonate

  • Other names: actonel

Adult Treatment Arm Experimental

Intervention treatment arm. Adults (18+ years) with type 1 OI. Must have at least mild hearing loss. Will receive Risedronate (35mg, 0-2x/week as clinically indicated) for duration of study. Changes in hearing, quality of life, and bone density will be monitored.

Child (Bisphosphonate Arm) No Intervention

Observational (no investigational intervention) arm. Children (6-17 years) with any type of OI who are already receiving bisphosphonate treatment as standard of care treatment for orthopedic symptoms. Changes in hearing, quality of life, and bone density will be observed for the duration of the study.

Child (Control Arm) No Intervention

Observational arm. Children (6-17 years) with any type of OI who are not receiving bisphosphonate treatment. Changes in hearing, quality of life, and bone density will be observed for the duration of the study.

Adult Control Arm No Intervention

Observational arm. Adults (18+ years) with type 1 OI. Changes in hearing, quality of life, and bone density will be monitored.

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 (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
No Results Posted