Audio-vestibular Evaluation of Children and Young Adults With Osteogenesis Imperfecta
Assistance Publique - Hôpitaux de Paris
44 participants
Dec 22, 2022
OBSERVATIONAL
Conditions
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.
Eligibility
Inclusion Criteria4
- 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
Exclusion Criteria7
- 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
Interested in this trial?
Get notified about updates and connect with the research team.
Interventions
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)
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.
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)
View Full Details on ClinicalTrials.gov
For the most up-to-date information, visit the official listing.
NCT05419960