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Progression of Unilateral Hearing Loss in Children With and Without Ipsilateral Cochlear Nerve Canal Stenosis: A
Patricia L Purcell1, Justin R Shinn, Scott S Coggeshall
1*Department of Otolaryngology †Division of Pediatric Otolaryngology, Seattle Children's Hospital ‡Department of Biostatistics §Department of Radiology, University of Washington, Seattle, Washington.
Insights
Children with bony cochlear nerve canal (BCNC) stenosis face a higher risk of hearing loss progression in the affected ear. Regular audiometric and medical follow-up is recommended for these children.
Area of Science:
- Pediatric audiology
- Neurotology
- Radiology
Background:
- Unilateral hearing loss in children can have various underlying causes.
- Bony cochlear nerve canal (BCNC) stenosis is a temporal bone anomaly that may impact hearing.
- Understanding the progression of hearing loss in affected children is crucial for management.
Purpose of the Study:
- To investigate the risk of hearing loss progression in children with unilateral hearing loss and ipsilateral bony cochlear nerve canal (BCNC) stenosis.
- To compare hearing loss progression rates between children with and without BCNC stenosis.
Main Methods:
- A prospective audiological database was used to identify children with unilateral hearing loss and temporal bone CT imaging.
- Two pediatric radiologists assessed imaging for temporal bone anomalies and measured BCNC width.
- Audiometric thresholds were reviewed, defining progression as a 10 dB increase in air conduction pure-tone average.
Main Results:
- Of 128 children, 22 (17%) had ipsilateral BCNC stenosis.
- Children with BCNC stenosis showed a significantly greater risk of ipsilateral hearing loss progression (HR 2.17, p=0.046).
- No hearing loss progression was observed in the contralateral ear among children with BCNC stenosis.
Conclusions:
- Bony cochlear nerve canal (BCNC) stenosis may increase the risk of ipsilateral hearing loss progression in children.
- Children diagnosed with BCNC stenosis warrant careful audiometric and medical follow-up.
- Further research may elucidate the precise mechanisms linking BCNC stenosis to hearing loss progression.
Objective:
To investigate the risk of hearing loss progression in each ear among children with unilateral hearing loss associated with ipsilateral bony cochlear nerve canal (BCNC) stenosis.
Setting:
Tertiary pediatric referral center.
Patients:
Children diagnosed with unilateral hearing loss who had undergone temporal bone computed tomography imaging and had at least 6 months of follow-up audiometric testing were identified from a prospective audiological database.
Interventions:
Two pediatric radiologists blinded to affected ear evaluated imaging for temporal bone anomalies and measured bony cochlear canal width independently. All available audiograms were reviewed, and air conduction thresholds were documented.
Main Outcome Measure:
Progression of hearing loss was defined by a 10 dB increase in air conduction pure-tone average.
Results:
One hundred twenty eight children met inclusion criteria. Of these, 54 (42%) had a temporal bone anomaly, and 22 (17%) had ipsilateral BCNC stenosis. At 12 months, rates of progression in the ipsilateral ear were as follows: 12% among those without a temporal bone anomaly, 13% among those with a temporal bone anomaly, and 17% among those with BCNC stenosis. Children with BCNC stenosis had a significantly greater risk of progression in their ipsilateral ear compared with children with no stenosis: hazard ratio (HR) 2.17, 95% confidence interval (CI) (1.01, 4.66), p value 0.046. When we compared children with BCNC stenosis to those with normal temporal bone imaging, we found that the children with stenosis had nearly two times greater risk estimate for progression, but this difference did not reach significance, HR 1.9, CI (0.8, 4.3), p = 0.1. No children with BCNC stenosis developed hearing loss in their contralateral year by 12 months of follow-up.
Conclusion:
Children with bony cochlear nerve canal stenosis may be at increased risk for progression in their ipsilateral ear. Audiometric and medical follow-up for these children should be considered.
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