Clinical and imaging analysis of white matter lesions combined with congenital sensorineural hearing loss in children

Objective To analyze the clinical and imaging characteristics of congenital sensorineural hearing loss (CSNHL) children combined with white matter (WM) lesions in order to provide evidence for clinical practice. Methods With referral to the Department of Pediatrics, Peking University First Hospital from November 2011 to December 2015, documents of 78 patients of CSNHL combined with WM lesions were collected and analyzed for the clinical and imaging characteristics. Results Bilateral severe-profound hearing loss existed in all 78 cases, 48.1%(25/52 cases) of the patients exhibited gross motor development delay, 98.1%(51/52 cases)of them had normal cognition development.One hundred percent (61/61 cases) of patients had abnormal language development.Infection occurring during pregnancy existed in 21.2%(11/52 cases) of the patients, the premature and smaller for the gestational age infants accounted for 28.9% (15/52 cases). The bilateral multiple WM lesions from the brain MRI were in dot to flake sizes with sharp boundary, the intensity of T1-weighted imaging decreased, T2-weighted imaging and fluid attenuated inversion recovery increased.Eighty-two point one percent (64/78 cases) of the patients were found to have the periventricular and subcortical WM involvement.The most frequently affected periventricular region was the posterior horn (91.9%, 68/74 cases), followed by the anterior horn and temporal horn, and the least with the body involvement.The former three had a combined lesion tendency (55.4%-68.9%). There was an extensive involvement in the subcortical WM of parietal, frontal, temporal and occipital lobes respectively(73.5%-88.2%). Subcortical WM involvement of multiple lobes was common (accounted for 67.6%-77.9%). The enlargement of bilateral ventricles existed in 37.2%(29/78 cases) of the patients and cystic changes in the subcortical WM of anterior temporal lobe could be found in 9.0% (7/78 cases) patients.Calcification in 2 CT cases was reported.Corpus callosum and basal ganglia of all cases were normal.For cases with MRI scans more than once, WM lesions of 96.0%(24/25 cases) patients became silent or self-restored. Conclusions The clinical presentations of CSNHL combined with WM lesions are mild, not paralleled with their multiple foci.It is considered as demyelination or a delay of myelination.Due to its benign course, it is probably not the contraindication for the cochlear implantation. Key words: Congenital sensorineural hearing loss; White matter; Magnetic imaging resonance; Child