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Updated: May 11, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Risk analysis of unilateral severe-to-profound sensorineural hearing loss in children
Adva B Friedman1, Ryan Guillory, Raghu H Ramakrishnaiah
1Department of Otolaryngology/Head and Neck Surgery, University of Arkansas for Medical Sciences, 4301 West Markham, Little Rock, AR 72205, USA.
Insights
The cause of pediatric unilateral severe-to-profound sensorineural hearing loss (USPSNHL) is identified in 40% of cases, often linked to perinatal events or family history. Importantly, children with USPSNHL show no increased risk of hearing loss in the other ear.
Area of Science:
- Pediatric Otolaryngology
- Neuroscience
- Genetics
Background:
- Unilateral severe-to-profound sensorineural hearing loss (USPSNHL) in children presents diagnostic challenges.
- Understanding the etiology and contralateral ear risk is crucial for management.
Purpose of the Study:
- To investigate the causes of pediatric USPSNHL.
- To assess the risk of hearing deterioration in the contralateral ear.
Main Methods:
- Retrospective review of 84 pediatric patients with USPSNHL undergoing bone-anchored hearing aid evaluation.
- Analysis of clinical charts, audiograms, and temporal bone imaging.
Main Results:
- Etiology identified in 41.7% of cases; perinatal events (16.2%) and family history (10.0%) were most common.
- Inner ear anatomical abnormalities found in 40.8% of temporal bone scans.
- No contralateral ear hearing loss progression observed during a mean follow-up of 31.9 months.
Conclusions:
- Pediatric USPSNHL has diverse etiologies, with identifiable causes in approximately 40% of patients.
- Temporal bone imaging reveals frequent inner ear anomalies but rarely impacts clinical decisions.
- Children with USPSNHL do not appear to have an elevated risk of contralateral ear hearing loss.
Objective:
To explore the etiology of pediatric unilateral severe-to-profound sensorineural hearing loss (USPSNHL) and the risk of hearing loss in the contralateral ear.
Methods:
Pediatric patients with USPSNHL referred to a tertiary children's hospital for unilateral bone-anchored hearing aid implantation were identified for study. Clinical charts, audiograms, and radiographic imaging of the temporal bones were reviewed.
Results:
Eighty-four children with USPSNHL were reviewed (40 male, 44 female). The etiology of USPSNHL could be identified in 35 patients (41.7%), with perinatal events (16.2%) and family history of hearing loss (10.0%) being the most common identifiable risk factors. Forty percent of children with available newborn hearing screens passed in both ears but later developed USPSNHL. Progressive unilateral hearing loss leading to USPSNHL was found in 21% percent of patients. No patient experienced worsening hearing in the contralateral ear (mean follow-up=31.9 months). Of the 49 temporal bone scans available for review, 40.8% demonstrated aberrant inner ear anatomy, including semicircular canal dysplasia (10.2%), cochlear aperture stenosis (10.2%), hypoplastic cochlea (8.1%), enlarged vestibular aqueduct (14.3%), incomplete partition I/II (6.1%), and anomalous internal auditory canal (2.0%).
Conclusion:
Pediatric USPSNHL can be attributed to a variety of sources with a cause identifiable in approximately 40% of patients. Temporal bone CT scan acquisition appears to be relatively high yield in this patient population, but only rarely do results have clinical implications. Early follow-up suggests that this population of children with USPSNHL does not appear to have a significantly increased risk for hearing loss in the contralateral ear.
