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[Progressive sensorineural hearing loss in childhood].
S Berrettini1, F Ravecca, S Sellari-Franceschini
1Dipartimento di Neuroscienze, Università degli Studi di Pisa.
Summary
Childhood progressive sensorineural hearing loss varies widely in prevalence due to differing diagnostic criteria. Causes are hereditary or acquired, impacting hearing development and progression patterns.
Area of Science:
- Pediatrics
- Audiology
- Genetics
Context:
- Progressive sensorineural hearing loss (PSNHL) in children presents a diagnostic challenge with reported prevalence ranging from 4% to 30%.
- Variability in prevalence estimates stems from diverse criteria for identifying hearing deterioration, patient cohorts, and age ranges studied.
- Understanding the etiology and progression of PSNHL is crucial for timely intervention and management.
Purpose:
- To review the variable prevalence, diverse etiologies (hereditary and acquired), and progression patterns of progressive sensorineural hearing loss in childhood.
- To highlight the complexities in diagnosing and characterizing PSNHL in pediatric populations.
- To synthesize current knowledge on the factors influencing PSNHL onset and evolution.
Summary:
- PSNHL etiology is broadly categorized into hereditary (syndromic/non-syndromic) and acquired causes, including infections, inner ear anomalies, metabolic disorders, ototoxic drug use, autoimmune diseases, and trauma.
- The onset of hearing deterioration in PSNHL is highly variable, with congenital cases sometimes manifesting later.
- Progression is typically bilateral but often asymmetrical, with potential for greater deterioration in initially less affected ears or specific frequency ranges, though patterns can vary.
Impact:
- Provides a comprehensive overview for clinicians and researchers on the multifaceted nature of pediatric PSNHL.
- Highlights the need for standardized diagnostic criteria to improve prevalence estimations and comparative studies.
- Informs clinical practice regarding the diverse causes and unpredictable progression of hearing loss in children, aiding in better patient counseling and management strategies.