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Prevalence of Cochlear Nerve Deficiency and Hearing Device Use in Children With Single-Sided Deafness
Kristina M Ward1,2, Alanna J Coughran2, Monterosa Lee2
1Department of Pediatric Audiology, Stanford Medicine Children's Health, Palo Alto, California, USA.
Insights
Cochlear nerve deficiency (CND) is common in children with single-sided deafness (SSD). Magnetic resonance imaging (MRI) is crucial for diagnosis, and early intervention with hearing devices is needed.
Area of Science:
- Audiology
- Pediatric Otolaryngology
- Neuroradiology
Background:
- Single-sided deafness (SSD) in children presents unique audiological challenges.
- Cochlear nerve deficiency (CND) is a potential underlying cause of SSD, impacting auditory pathway integrity.
- Understanding CND prevalence is vital for guiding diagnostic and therapeutic strategies in pediatric SSD.
Purpose of the Study:
- To determine the prevalence of cochlear nerve deficiency (CND) in pediatric patients diagnosed with single-sided deafness (SSD).
- To analyze trends in the utilization of hearing interventions and devices among these children.
- To evaluate the diagnostic accuracy of different imaging modalities for CND in SSD.
Main Methods:
- Retrospective chart review of 190 pediatric patients (0-21 years) with SSD.
- Inclusion criteria: severe-to-profound unilateral sensorineural hearing loss with normal contralateral hearing.
- Diagnostic criteria for CND: neuroradiologist-reported aplastic/hypoplastic nerve (MRI) or stenotic cochlear aperture (CT).
Main Results:
- CND prevalence varied by imaging: 42% (CT only), 76% (MRI only), 63% (both MRI and CT).
- High concordance (90%) between MRI and CT for CND diagnosis.
- Hearing device use: 36% (bone conduction/contralateral routing), 3% (cochlear implant), 40% (no device).
Conclusions:
- CND is highly prevalent in pediatric SSD, underscoring the need for MRI confirmation.
- Current hearing device implementation for pediatric SSD is limited, indicating a need for improved early intervention.
- Further research and support are essential to optimize auditory outcomes for children with SSD and CND.
Objective:
This study aimed to assess the prevalence of cochlear nerve deficiency (CND) in a cohort of pediatric patients with single-sided deafness (SSD). A secondary objective was to investigate trends in intervention and hearing device use in these children.
Study Design:
Case series with chart review.
Setting:
Pediatric tertiary care center.
Methods:
Children ages 0 to 21 years with SSD (N = 190) who underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI) were included. Diagnostic criteria for SSD included unilateral severe-to-profound sensorineural hearing loss with normal hearing sensitivity in the contralateral ear. Diagnostic criteria for CND included neuroradiologist report of an "aplastic or hypoplastic nerve" on MRI or a "stenotic cochlear aperture" on CT.
Results:
The prevalence of CND was 42% for children with CT only, 76% for children with MRI only, and 63% for children with both MRI and CT. Of the children with MRI and CT, there was a 90% concordance across imaging modalities. About 36% of children with SSD had hearing devices that routed sound to the normal hearing ear (ie, bone conduction hearing device/contralateral routing of signal), while only 3% received a cochlear implant. Approximately 40% did not have a hearing device. Hearing device wear time averaged 2.9 hours per day and did not differ based on cochlear nerve status.
Conclusion:
There is a high prevalence of CND in children with SSD. Cochlear nerve status should be confirmed via MRI in children with SSD. The limited implementation and use of hearing devices observed for children with SSD reinforce the need for increased support for early and continuous intervention.
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