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

260 weeks

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 deficits occur in children and young adults with Osteogenesis Imperfecta (OI), a genetic bone disorder with varying severity and types. The study also aims to find if there is a connection between the genetic type and severity of OI and the presence of hearing and balance issues. The condition is known to be linked with hearing loss, but the extent and relationship with vestibular problems in younger patients are less clear. Participants aged 12 to 20 with OI will undergo a comprehensive evaluation including audiometric, immittance, and vestibular tests such as videonystagmography, video Head Impulse Test, and vestibular evoked myogenic potentials. Those with conductive or mixed hearing loss or vestibular deficits will be offered a CT scan of the petrous bone without injection. If sensorineural hearing loss or abnormal CT findings are present, an MRI with contrast enhancement will be performed as part of their care. During the study, participants will be assessed through various diagnostic tests to measure their hearing and vestibular function over a 24-month period. Key outcome measures include central vestibular function, subjective visual vertical, ocular and cervical vestibular evoked myogenic potentials, and semicircular canal function. The research team will analyze these results to determine the presence and severity of vestibular deficits and their correlation with radiological findings and hearing loss type. Safety and follow-up are integrated with the participants' routine care visits at the specialized reference center in Paris.

CONDITIONS

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

You will not qualify if you...

  • Patients with hearing loss of alternate origin e.g. Cochlear nerve deficiency, atresia, etc.
  • Neurological or developmental deficits limiting participation
  • Cervico-occipital instability e.g. Chiari's malformation
  • Limitations in mobility of the spine e.g. scoliosis, spinal fractural fusion
  • Ophthalmologic pathologies e.g. strabism or severe refraction disorder
  • Patients under AME (State Medical Aid)
  • Protected adult patients, adults unable to express their consent, pregnant or breastfeeding women

AI-Screening

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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

Frequently Asked Questions

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