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Audio-vestibular Evaluation of Children and Young Adults With Osteogenesis Imperfecta
Sponsor: Assistance Publique - Hôpitaux de Paris
Summary
The aim is to determine whether vestibular deficits are present in OI, then to establish whether a correlation exists between genetic type, severity of OI and audiovestibular phenotype. OI patients aged 12 to 20 years will undergo an audiometric, immittance, and vestibular assessment. When hearing loss is conductive or mixed or in cases where vestibular deficits are identified, a CT scan without injection will be performed. In case of sensorineural hearing loss or abnormal CT results, an MRI will be performed.
Key Details
Gender
All
Age Range
12 Years - 20 Years
Study Type
OBSERVATIONAL
Enrollment
44
Start Date
2022-12-22
Completion Date
2027-12-22
Last Updated
2025-09-12
Healthy Volunteers
No
Conditions
Interventions
Vestibular Assessment
During the usual hearing assessment (immittance testing and pure-tone and speech audiometry) carried out by patients, a vestibular assessment will be done for the study to detect vestibular disorders: * Videonystagmography (VNG) Is the study of eye movement with the help of infrared googles. * Subjective Visual Vertical (SVV) The patient is asked to orient a line on the vertical axis. * Video Head Impulse Test (vHIT) * Cervical and Ocular Vestibular Evoked Myogenic Potential (cVEMP and oVEMP)
Petrous bone Computed Tomography (CT)
For patients diagnosed with hearing loss, whether conductive, mixed, and/or a vestibular deficit, a CT scan (without injection) will be proposed for their care. Petrous bone Computed Tomography (CT) is an imaging technique used as the standard method of evaluation of the boney structures in the ear.
Magnetic Resonance Imaging (MRI)
Patients that have done a CT and for who CT results was abnormal, will additionally require an MRI for their standard care. Magnetic Resonance Imaging (MRI) is an imaging technique used as the standard method of evaluation of the cochlear, retrocochlear and central and peripheral nervous system. An injection of Gadolinium contrast media will be used to enhance certain recordings.
Locations (1)
Hôpital Necker-Enfants Malades
Paris, France