Hearing loss and vestibular dysfunction in congenital CMV infection: Could it be due to endolymphatic pressure anomaly? A preliminary study.

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.