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Updated: Jun 14, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Neurodevelopmental disorders in children with severe to profound sensorineural hearing loss: a clinical study
Anna M Chilosi1, Alessandro Comparini, Maria F Scusa
1Department of Developmental Neuroscience, IRCCS Stella Maris, Pisa, Italy. achilosi@inpe.unipi.it
Insights
Nearly half of children with severe/profound sensorineural hearing loss (SNHL) have additional neurodevelopmental disabilities. The cause of deafness and MRI findings are linked to the presence and type of these associated disorders.
Area of Science:
- Neuroscience
- Genetics
- Pediatrics
Background:
- Sensorineural hearing loss (SNHL) in children can be associated with other disabilities, but their prevalence and nature are not well-established.
- Understanding these co-occurring conditions is crucial for comprehensive patient care and intervention planning.
Purpose of the Study:
- To determine the frequency and types of additional neurodevelopmental disabilities in children with SNHL.
- To explore the relationship between these disabilities and the underlying causes of deafness.
Main Methods:
- A cohort of 100 children with severe/profound SNHL underwent a detailed diagnostic assessment.
- Evaluations included neurodevelopmental screening, genetic testing, neurometabolic workup, and brain magnetic resonance imaging (MRI).
Main Results:
- 48% of children with SNHL presented with at least one additional disability, most commonly cognitive, behavioral-emotional, or motor impairments.
- Brain MRI revealed abnormalities in 37 out of 80 children, including malformations and white matter changes.
- The occurrence and nature of disabilities were significantly associated with the etiology of SNHL and MRI findings.
Conclusions:
- A thorough, multidimensional assessment is essential for children with severe to profound SNHL to identify associated disorders.
- The etiology of SNHL serves as a key indicator for the risk of co-existing neurodevelopmental conditions, informing therapeutic strategies like cochlear implantation.
Aim:
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 5 y 7 mo, SD 3 y 6 mo, range 8 mo-16 y) 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.
