Related Experiment Videos
Progressive sensorineural hearing loss in childhood.
S Berrettini1, F Ravecca, S Sellari-Franceschini
1Neuroscience Department, University of Pisa, Italy.
Pediatric Neurology
|March 19, 1999
Summary
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.