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Hearing loss and vestibular dysfunction in congenital CMV infection: Could it be due to endolymphatic pressure
Laureline Kahn1, Guillaume Poillon2, Monique Elmaleh-Bergès3
1Department of Paediatric Otolaryngology, Robert Debre Hospital, Assistance Publique Hôpitaux de Paris (APHP), Paris Cité University, Paris, France.
Objectives:
To describe the inner ear sectors after an inner ear MRI protocol and search for the presence of endolymphatic pressure anomaly in patients presenting with a congenital CMV infection and audio-vestibular dysfunction.
Methods:
A 3D FLAIR MRI sequence, 4 h after gadolinium injection, was performed in patients with sensory-neural hearing loss secondary to a congenital CMV infection in order to analyse the morphology of the endolymphatic space.
Results:
Two patients presented with a unilateral SNHL and 4 patients a bilateral SNHL. Seven ears with SNHL demonstrated an endolymphatic hydrops on MRI images and 2 showed a membranous labyrinth atelectasis. All ears but two had a marked enhancement in the perilymph of the basal turn of the cochlea. One ear, with a normal hearing threshold but altered vestibular function, demonstrated cochlear and saccular hydrops. Two ears with normal or near normal hearing and normal vestibular function were radiologically normal on the MRI.
Conclusion:
The compartmental endolymphatic study using delayed contrast-enhanced MRI sequences in children with cCMV infection suggests a relationship between inner ear involvement and endolymphatic pressure anomaly.
Insights
Congenital CMV infection in infants can cause inner ear problems, including endolymphatic hydrops, detected by specialized MRI. This suggests a link between CMV and abnormal inner ear pressure.
Area of Science:
- Otolaryngology
- Pediatric Neurology
- Medical Imaging
Background:
- Congenital Cytomegalovirus (cCMV) infection is a leading cause of non-genetic sensorineural hearing loss (SNHL) in children.
- Audio-vestibular dysfunction is a common sequela of cCMV infection, impacting a child's development.
- The precise mechanisms of inner ear damage in cCMV are not fully understood.
Purpose of the Study:
- To investigate inner ear morphology using a specific MRI protocol in children with cCMV.
- To identify potential endolymphatic pressure anomalies in affected individuals.
- To correlate imaging findings with audio-vestibular dysfunction in cCMV patients.
Main Methods:
- Utilized a 3D FLAIR MRI sequence performed 4 hours post-gadolinium injection.
- Analyzed the morphology of the endolymphatic space in pediatric patients with SNHL secondary to cCMV.
- Correlated MRI findings with clinical audio-vestibular assessments.
Main Results:
- Seven out of ten ears with SNHL showed evidence of endolymphatic hydrops.
- Two ears exhibited membranous labyrinth atelectasis.
- A significant enhancement in the cochlear perilymph was observed in most affected ears, with some cases showing cochlear and saccular hydrops despite normal hearing.
Conclusions:
- Delayed contrast-enhanced MRI can reveal endolymphatic pressure anomalies in children with cCMV.
- Findings suggest a direct relationship between cCMV infection and inner ear structural changes.
- This imaging approach may aid in understanding the pathophysiology of cCMV-related hearing and balance disorders.
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