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Progressive sensorineural hearing loss in childhood
S Berrettini1, F Ravecca, S Sellari-Franceschini
1Neuroscience Department, University of Pisa, Italy.
Insights
Progressive sensorineural hearing loss in children affects 6.2% of cases. Most cases begin after age four, often impacting both ears, with unknown causes in many instances.
Area of Science:
- Pediatric Audiology
- Genetics of Hearing Loss
- Otolaryngology
Background:
- Progressive sensorineural hearing loss in childhood has a highly variable reported prevalence (4–30%).
- Etiologies include hereditary, developmental, and infectious causes, but are often unknown.
- Previous studies lack consistent criteria for identifying hearing deterioration, age groups, and examination ranges.
Purpose of the Study:
- To determine the prevalence of progressive sensorineural hearing loss in a pediatric cohort.
- To investigate the onset, progression, and etiology of progressive hearing loss in children.
Main Methods:
- Audiologic tests were used to examine 178 children with bilateral sensorineural hearing loss between 1971 and 1993.
- Children with syndromal genetic hearing loss were excluded from the analysis.
Main Results:
- A progressive hearing loss was identified in 11 subjects, yielding a prevalence of 6.2%.
- The onset of deterioration occurred after age four in 73% of affected children.
- Hereditary deafness was the most common known etiology (5/11), followed by congenital infection (1/11) and inner ear anomaly (1/11); 4/11 had unknown etiology.
Conclusions:
- Progressive sensorineural hearing loss affects a significant minority of children with hearing impairment.
- Late onset (after age four) and binaural, asymmetric progression are characteristic features.
- Unknown etiologies remain a significant challenge in childhood progressive hearing loss.
Abstract:
A progressive sensorineural hearing loss in childhood, with an extremely variable prevalence (from 4% to 30%), has been reported in the literature. This wide range of reported figures could depend on the different criteria used for identifying the deterioration, the groups, and the examined age ranges. The most frequent etiology of progressive sensorineural hearing loss in childhood includes hereditary causes, both syndromic and nonsyndromic, and developmental and infectious causes, whereas metabolic, toxic, autoimmune, traumatic, and vascular etiologies are less common; however, the origin of the hearing impairment often remains unknown. The population for this study consisted of 178 children with bilateral sensorineural hearing loss who were examined between 1971 and 1993 using audiologic tests. Syndromal genetic hearing loss was excluded from the study. A progressive loss of acuity was found in 11 subjects, with a prevalence of 6.2%. The etiology was hereditary deafness in five patients, congenital infection in one, and congenital inner ear anomaly in another patient; in the last four children the etiology was unknown. Onset of deterioration was after 4 years of age in 73% of the patients. The progressive evolution was binaural in almost all patients (10 of 11) and asymmetric in most, with a tendency to a greater deterioration at the frequencies initially least affected.