Prediction of the Outcome of Cochlear Implantation in the Patients with Congenital Cytomegalovirus Infection based on Magnetic Resonance Imaging Characteristics

Abstract

The goal of this study was to elucidate radiologic biomarker that can predict the outcome of cochlear implantation (CI) in congenital cytomegalovirus (cCMV) related deafness. A retrospective survey of speech perception after CI and an evaluation of brain magnetic resonance imaging (MRI) findings were performed in 10 cochlear implantees with cCMV-related prelingual deafness. Specifically, a special attention was paid to the degree of white matter (WM) abnormality shown in brain MRI, which was used to divide our cohort into two groups: The mild and severe pathology groups. Age-matched prelingual deaf patients with idiopathic sensorineural hearing loss were selected as controls. Subjects in mild pathology groups showed higher a Category of Auditory Performance (CAP) score (5.2 ± 0.8) than those with severe pathologies (3.4 ± 1.5) (P = 0.041). Importantly, speech performance from subjects with mild pathology was comparable to that of the control group (mean CAP score of 5.2 ± 0.8 vs. 5.1 ± 1.2) (P = 0.898). Mild pathologies related to the limited WM lesion in MRI not accompanied by severe MRI pathologies, such as diffuse WM abnormality, myelination delay, ventriculomegaly, migration abnormality, and cerebellar hypoplasia, can be tolerated and do not adversely affect the CI outcome in cCMV deafness.

Keywords: cochlear implantation; cytomegalovirus; hearing loss; magnetic resonance imaging; outcome; radiologic biomarker; speech perception; white matter.

Figure 1

Various features of T2-weighted MR images in patients with cytomegalovirus infection. (a) Multifocal white matter (WM) lesions (white arrows) in deep WM of frontal and parietal lobe (CH-4). (b) Extensive WM lesions (white arrows) in periventricular and deep WM of whole brain areas (CH-9). (c) Diffuse WM lesion (white arrows) combined with periventricular cyst (black arrow), ventriculomegaly (black asterisk), cerebellar hypoplasia (white asterisk), and polymicrogyria (white arrowhead) (CH-3).

Figure 2

The progression of speech perception performance after cochlear implantation (CI) in patients with congenital cytomegalovirus (cCMV) infection and idiopathic sensorineural hearing loss (SNHL). Among the participants with cCMV infection, the mild pathology group having only limited white matter (WM) abnormalities in their MRIs showed rapid progression of speech perception performance after CI. At 2 years after CI, the categories of auditory performance score of mild pathology group was similar to that of the idiopathic SNHL group, whereas the CI outcomes of the severe pathology group with one or more of cCMV infection-related severe pathologic MRI findings, was significantly poorer than the mild pathology group and idiopathic SNHL group.