Prevalence and etiology of sensorineural hearing loss in children with down syndrome: A cross-sectional study.

BACKGROUND

The prevalence and causes of sensorineural hearing loss (SNHL) in children with Down syndrome (DS) are poorly delineated.

OBJECTIVE

To describe the prevalence, severity, laterality and underlying etiology of SNHL in a cohort of children with DS.

METHODS

A cross-sectional study was performed among all children with DS followed at the multidisciplinary Downteam of the Antwerp University Hospital. Patients' characteristics, risk factors for hearing loss, audiometric data and results of an etiological work-up were collected.

RESULTS

Among 291 patients in follow-up, 138 patients (47.4%) presented with hearing loss. In the majority this was caused by middle ear effusion and only 13 patients (4.5%) had sensorineural hearing loss, 7 boys and 6 girls with a mean age of 14.4 ± 7.4 years. Hearing loss was bilateral in 8 cases. Hearing loss severity was graded as mild in 38.5%, moderate in 30.8% and profound in 30.8% of the patients. An etiological work-up was completed in 9 children. Four patients presented with single sided deafness due to cochlear nerve deficiency. One patient had a genetic cause and in 2 patients the hearing loss was attributed to excessive noise exposure. The etiology of hearing loss was unknown in 6 patients.

CONCLUSION

Sensorineural hearing loss is uncommon in children with DS with a prevalence of 4.5%. Etiological work-up may allow identifying a specific underlying cause. Cochlear nerve deficiency was found in 4 children with DS and single sided deafness.