Actively Recruiting

Age: 12Years - 20Years
All Genders
ID05419960

Audio-vestibular Evaluation of Children and Young Adults With Osteogenesis Imperfecta

Led by Assistance Publique - Hôpitaux de Paris · Updated on 2025-09-12

44

Participants Needed

1

Research Sites

N/A

Total Duration

On this page

Sponsors

A

Assistance Publique - Hôpitaux de Paris

Lead Sponsor

U

URC-CIC Paris Descartes Necker Cochin

Collaborating Sponsor

AI-Summary

What this Trial Is About

Researchers are investigating whether vestibular problems occur in people aged 12 to 20 with Osteogenesis Imperfecta (OI), a genetic bone disorder also linked to hearing loss. They aim to find if there's a relationship between the type and severity of OI and specific hearing and balance issues. The study involves detailed hearing and balance tests to better understand these connections and to explore bone changes seen on imaging scans. Participants will undergo several hearing tests including audiometry and immittance assessments, along with vestibular evaluations such as videonystagmography, video Head Impulse Test, and vestibular evoked muscular potentials. If hearing loss or vestibular deficits are found, patients will have a CT scan of the ear bones. Those with sensorineural hearing loss or abnormal CT results will be offered an MRI to further assess inner ear and nerve structures. During the study, participants will complete these tests as part of their annual medical visit, with results tracked over 24 months. Researchers will measure various vestibular functions and hearing abilities, as well as assess the severity of OI and bone changes on imaging. This comprehensive evaluation aims to clarify the connection between OI and balance or hearing problems while monitoring patients' health over two years.

CONDITIONS

Brief Title

Audio-vestibular Evaluation of Children and Young Adults With Osteogenesis Imperfecta

Who Can Participate

Age: 12Years - 20Years
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Patients between the ages of 12 and 20 years at the time of inclusion
  • Diagnosis of Osteogenesis Imperfecta of any type
  • Currently followed by a physician at the CRMR OI
  • Provided information and non-opposition consent by major patients, holders of parental authority, and minor patients to participate in the study
Not Eligible

You will not qualify if you...

  • Hearing loss from causes other than Osteogenesis Imperfecta (e.g., cochlear nerve deficiency, atresia)
  • Neurological or developmental deficits limiting participation
  • Cervico-occipital instability such as Chiari's malformation
  • Limitations in spine mobility like scoliosis or spinal fracture fusion
  • Ophthalmologic conditions such as strabismus or severe refraction disorder
  • Patients under State Medical Aid (AME)
  • Protected adults, adults unable to consent, pregnant or breastfeeding women

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person)

Diagnostic Evaluation

Duration - Annual visits over 24 months

Participants undergo audiometric, immittance, and vestibular assessments during their annual visit to evaluate hearing and vestibular function.

Annual visits for up to 24 months

Diagnostic Evaluation

Duration - As needed during the study period

For participants with conductive or mixed hearing loss or identified vestibular deficits, a CT scan without injection is performed as part of standard care.

1 to 2 visits depending on hearing loss and vestibular assessment results

Diagnostic Evaluation

Duration - As needed during the study period

Participants with sensorineural hearing loss or abnormal CT results may undergo an MRI with contrast injection as part of standard care.

1 visit if MRI is required

Long-term Monitoring

Duration - 24 months

Participants are monitored to assess vestibular function and hearing over 24 months to explore correlations with Osteogenesis Imperfecta severity and radiological findings.

Assessments aligned with annual visits

Trial Site Locations

Total: 1 location

1

Hôpital Necker-Enfants Malades

Paris, France, 75015

Actively Recruiting

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Research Team

N

Natalie Loundon, MD, PhD

H

Hélène Morel

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

1

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