Prevalence and etiology of sensorineural hearing loss in children with down syndrome: A cross-sectional study. (January 2019)

• Record Type: Journal Article
• Authors: De Schrijver, L.; Topsakal, V.; Wojciechowski, M.; Van de Heyning, P.; Boudewyns, A.
• Is Part Of: International journal of pediatric otorhinolaryngology. Volume 116(2019:Jan.)
• Page Start: 168
• Page End: 172
• Publication Date: 2019-01
• Subjects: Down syndrome -- Trisomy 21 -- Sensorineural hearing loss -- Single sided deafness; Otolaryngology -- Periodicals; Pediatrics -- Periodicals; Otolaryngology -- Periodicals; Pediatrics -- Periodicals; Oto-rhino-laryngologie -- Périodiques; Pédiatrie -- Périodiques; 618.9209751
• DOI: 10.1016/j.ijporl.2018.10.048
• Languages: English
• ISSNs: 0165-5876

Abstract

Abstract: 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.