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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.
Insights
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.
Abstract:
Prevalence of progressive sensorineural hearing loss in childhood seems to be extremely variable, as percentages reported range from 4 to 30%. Differences in the criteria employed for identifying the deterioration, in the groups of patients, and the age range, could explain this wide range of reported figures. The etiology of the progressive sensorineural hearing loss in infants can be hereditary or acquired. Hereditary causes are divided into syndromic and non-syndromic, whereas the acquired causes include congenital or acquired infection (syphilis, cytomegalovirus, rubella virus and toxoplasma infections, bacterial meningitis and acquired viral infections) and congenital inner ear anomalies (Mondini's dysplasia, large vestibular aqueduct, large cochlear aqueduct). Other acquired causes such as disorders of the metabolism, chronic use of ototoxic drugs, autoimmune diseases, perilymphatic fistula and head or acoustic trauma are less common. The age of onset of deterioration shows a great variability because even the congenital hearing losses may occur late after birth. The progressive evolution seems to be binaural in most patients, but more commonly it presents interaural differences, and when the hearing deficit is initially asymmetrical the deterioration is usually greater in the ear which appeared least affected in the first audiogram. Furthermore, at the different frequencies, there is a tendency to a greater deterioration at the frequencies initially least affected, but some authors are not in agreement because they report a uniform pattern of progression in the range of 0.5 to 4 kHz with no modification of the audiometric shape in most of the examined patients.