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Inner-ear malformations as a cause of single-sided deafness
E Tahir1, M D Bajin2, S Jafarov2
1Department of Otolaryngology, Ondokuz Mayıs University School of Medicine, Samsun, Turkey.
The Journal of Laryngology and Otology
|June 9, 2020
Summary
Inner-ear malformations are common in congenital single-sided deafness, affecting nearly half of cases. Early radiological evaluation is crucial for understanding the cause and guiding treatment for affected children.
Area of Science:
- Otolaryngology
- Pediatric Radiology
- Developmental Biology
Background:
- Congenital single-sided deafness (SSD) presents diagnostic challenges.
- Understanding the underlying inner ear malformations is critical for prognosis and management.
- Previous studies have not fully elucidated the prevalence and specific types of malformations in pediatric SSD.
Purpose of the Study:
- To investigate the prevalence and distribution of inner ear malformations in children with congenital single-sided deafness.
- To identify specific types of malformations and their association with cochlear nerve status.
- To emphasize the importance of radiological assessment in managing pediatric SSD.
Main Methods:
- Retrospective analysis of 90 pediatric patients (<16 years) with congenital SSD.
- Evaluation of radiological findings using computed tomography (CT) and magnetic resonance imaging (MRI).
- Identification of inner ear malformations and assessment of cochlear nerve status.
Main Results:
- Inner ear malformations were present in 46.7% of cases (42 out of 90 ears).
- Most common anomalies included isolated cochlear aperture stenosis (20%) and atresia (12.2%), and cochlear hypoplasia (7.8%).
- Cochlear nerve deficiency (45.6%) and internal auditory canal stenosis (54.4%) were frequently observed.
Conclusions:
- Inner ear malformations, particularly cochlear aperture anomalies, are a significant factor in congenital SSD.
- The etiology of SSD varies considerably between congenital and adult-onset cases.
- Radiological evaluation is essential for all children with SSD to inform prognosis, management, and etiological understanding.
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