Down syndrome and Hearing

Articles written by Marit Erna Austeng in connection with her PhD thesis; Hearing loss, otitis media with effusion and obstructive sleep apnea in children with Down syndrome

Austeng, M. E., et al. (2014). "Obstructive sleep apnea in younger school children with Down syndrome." International Journal of Pediatric Otorhinolaryngology.

Objective - We aimed to assess the prevalence of obstructive sleep apnea (OSA) in 8 year old school children with Down syndrome (DS). While the prevalence in otherwise healthy children is below 5%, the prevalence estimates in children with DS are uncertain (30–80%). OSA directly affects cognitive development and school performance. Study designPopulation based cross sectional study in a limited geographical area. MethodsPolysomnography (PSG) with video and audio recordings was performed in 8-year-old children with DS in a pediatric sleep unit according to the guidelines of American Academy of Sleep Medicine. Twenty-nine of all 32 children with DS within a restricted area comprising >50% of the Norwegian population and 54% of the children with DS born in Norway in 2002 were enrolled. ResultsThis study reports an apnea hypopnea index AHI>1.5 in 28 of 29 children and an obstructive apnea index (OAI)>1 in 24 of 29 children. 19 children (66%) had an AHI>5 and 17 children (59%) had an OAI>5 which indicated moderate to severe OSA. No correlation was found between OSA and obesity or gender. ConclusionThe high prevalence of disease found in these previously undiagnosed 8-year-old children underlines the importance of performing OSA diagnostics in children with DS throughout childhood. These findings suggest that the prevalence of OSA remains high up to early school years. In contrast to earlier publications, this current study has the advantage of being population based, the study is performed on children of a narrow age band to estimate prevalence of disease and the diagnostic gold standard of PSG is applied.

Austeng, M. E., et al. (2013). "Hearing level in children with Down syndrome at the age of eight." Research in developmental disabilities 34(7): 2251.

This study examines the prevalence of hearing loss in children with Down syndrome at the age of 8. All children were examined in the ENT-departments of public hospitals in Norway and the study population consisted of children born in Norway in 2002 with Down syndrome. Hearing loss was defined as pure-tone air-conduction reduction by on average more than 25 dB HL in the best hearing ear. A cross sectional clinical and audiological population based study was chosen as study design. Hearing loss more than 25 dB HL in the best hearing ear was found in 17/49 children (35%). Mild hearing loss was found in 13 children (26%), moderate in 3 (6%) children and severe hearing loss in 1 child (2%). Conductive hearing loss was found in 8 children (16%), 9 children (18%) had a sensory-neural hearing loss, and mixed hearing loss was found in 3 children. Mean hearing level among boys and girls were 30.0 dB HL (SD 15.7) and 25.5 dB HL (SD13.7) respectively, a non-significant difference (p=0.139). In conclusion this study indicates that both conductive and sensorineural hearing loss, is still common in children with Down syndrome children at the age of eight and as much as two thirds of the children may have a bilateral impairment. The study population was under diagnosed in terms of hearing loss and thus our findings underline the importance of continuous audiological follow up of this group of children throughout childhood.

Austeng, M. E., et al. (2013). "Otitis media with effusion in children with in Down syndrome." International Journal of Pediatric Otorhinolaryngology 77(8): 1329-1332.

ObjectiveTo determine the prevalence of otitis media with effusion (OME) in children with Down syndrome (DS), and the associated to hearing loss at the age of 8 years. Study designA national population based clinical study of all children with DS born in Norway in 2002. ResultsOME was found in 20 out of 52 (38%) children. Those with OME had a significant lower hearing level with a mean pure tone average (PTA) of 33.4dB HL compared to children with no OME whose mean PTA was 21.7dB HL (p<0.0001). Verified hearing loss above 25dB HL in the better hearing ear was found in 12 out of the 20 with OME, compared to 5 out 31 without OME. ConclusionThe findings of this present study uncover the increased risk of OME in eight year old children with DS as current otitis media was found in one of three. This reduced hearing ability in children with DS due to OME at age of 8 strongly emphasizes the need for optimal treatment and follow up to optimize hearing rehabilitation. The findings are further supported by the population based study design, the focus on the narrow age band and the high response rate.

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