Neurodevelopmental disorders in children with severe to profound sensorineural hearing loss: a clinical study

Abstract

PDD Pervasive developmental disorder SNHL Sensorineural hearing lossAIM The effects of sensorineural hearing loss (SNHL) are often complicated by additional disabilities, but the epidemiology of associated disorders is not clearly defined. The aim of this study was to evaluate the frequency and type of additional neurodevelopmental disabilities in a sample of children with SNHL and to investigate the relation between these additional disabilities and the aetiology of deafness.METHOD One hundred children with severe ⁄ profound SNHL (60 males, 40 females; mean age 5y 7mo, SD 3y 6mo, range 8mo-16y) were investigated using a diagnostic protocol including neurodevelopmental, genetic, neurometabolic, and brain magnetic resonance imaging (MRI) assessment.RESULTS Forty-eight per cent of the sample exhibited one or more additional disabilities, with cognitive, behavioural-emotional, and motor disorders being the most frequent. The risk of additional disabilities varied according to the type of aetiology. Thirty-seven out of 80 individuals with available MRIs showed signal abnormalities, in particular brain malformations (46%) and white matter abnormalities (54%). Frequency and type of disability were associated with aetiology (p=0.015) and MRI data (p<0.001).INTERPRETATION A multidimensional evaluation, including aetiological, neurodevelopmental, and MRI investigation, is needed for planning therapeutic intervention, such as cochlear implantation in children with severe to profound hearing impairment. The aetiology of deafness is a relevant risk indicator for the presence of an associated disorder.According to the literature, sensorineural hearing loss (SNHL) is complicated by additional disabilities in about 30% of individuals, 1-5 but the epidemiology of associated disorders, in terms of type, frequency, and aetiology, is still not clearly defined. A wide variety of additional disabling conditions have been described in children with severe to profound hearing impairment, sometimes as part of a named syndrome (i.e. CHARGE [coloboma, heart defects, choanal atresia retarded growth and development, ear anomaly ⁄ deafness], Waardenburg, Usher, and Goldenhar syndromes), or as comorbid to deafness of known or unknown aetiology. Studies on additional disabilities are difficult to perform and interpret. The heterogeneity of definition and inclusion criteria (such as severity of cognitive disability) has led to widely differing results from various studies. Moreover, the relation between deafness and disability can raise contradictory interpretations that may be related to aetiology or a secondary association.Additional disabilities in a child with severe to profound hearing impairment have important consequences when assessing and choosing a therapeutic treatment, in particular when considering cochlear implantation or hearing aids.Given the costs of cochlear implantation, clear criteria for evaluating possible risks and benefits and meeting parents' expectations are indispensable. 6 The preoperative assessment and the postope...

Childhood hearing impairment in northern Finland, etiology and additional disabilities

“…Thus, it is conceivable that in our study, children with acquired HI had more often additional disabilities than those children with genetic or unknown etiology of HI. Our findings are in line with those recently reported [13].…”

“…The presence or absence of additional disabilities is a significant predictor of outcome in children with HI [10][11][12]. Most studies on additional disabilities in hearing impaired children have focused on children with a cochlear implant or children with severe or profound HI [9,13], whereas little is known about additional disabilities in children with mild or moderate HI.…”

Profound childhood hearing loss in a South Africa cohort: Risk profile, diagnosis and age of intervention

“…Profound childhood hearing loss is more than just a sensory loss, since central nervous system consequences of congenital deafness are aggravated with an increase in degree of hearing loss [10]. Also, approximately 30% of children with a profound hearing loss are reported to have an additional disability, with cognitive impairment and neurodevelopmental disabilities being the most common [36,37].…”

The Clinical Significance of Preoperative Brain Magnetic Resonance Imaging in Pediatric Cochlear Implant Recipients

“…In addition, cytomegalovirus infection, rubella, syphilis, toxoplasmosis, or other viral infections during the prenatal period can lead to profound hearing loss as well as brain lesions [Kral and O'Donoghue, 2010]. For example, abnormal white matter signals are typically related to either pre-perinatal problems, such as hypoxic brain damage, or intrauterine infections [Chilosi et al, 2010]. Hence, there is an increased incidence of brain abnormalities in children with hearing loss.…”