The Prevalence of Hearing Impairment in Infants and Children with Down Syndrome a Cross Sectional Study in a Tertiary Care Center

Overview

Journal Sci Rep

Specialty Science

Date 2025 Mar 4

PMID 40038479

Authors

Sohier Yahia

Marwa Metawea

Ahmed Megahed

Wessam ELshawaf

Yahya Wahba

Ranim Mahmoud

Affiliations

Soon will be listed here.

Abstract

Down syndrome is the most common chromosomal disorder in humans. Hearing impairment is a common feature of Down syndrome. To assess the prevalence of hearing impairment in children with DS attending the Mansoura University Children's Hospital. The study is a descriptive cross-sectional study on 170 pediatric patients with genetically confirmed DS. Patients were recruited from the genetic outpatient clinic of the Mansoura University Children's Hospital from October 2021 to October 2022. All infants and children were subjected to full history taking, and a lateral X-ray on the nasopharynx with open mouth and extended neck. The ears were examined and cleared from accumulated cerumen if present. The presence of middle ear pathology was assessed through an otoscopic examination of the tympanic membrane. Eustachian tube dysfunction and otitis media with effusion (OME) were assessed using tympanometry. Appropriate hearing tests including pure tone audiometry (PTA) and auditory brain stem response (ABR) were used. Conductive hearing loss (CHL) was observed in 48.8% of studied children with DS and 4.1% had sensorineural hearing loss (SNHL). Among patients with hearing impairment, 86.5% had bilateral affection. The severity of hearing loss was mild in 59.1% of patients with CHL and 71.4% with SNHL. Of the patients with CHL, HL remained stationary in 45.8%, regressed from moderate to mild HL in 15.7%, and normalized in 38.6%. SNHL was permanent in all 7 affected patients with a stationary course in 4 and a progressive nature in 3. OME, upper respiratory tract infections (URTI), and adenoid enlargement were commonly associated with CHL in infants and children with DS. Most children with DS have bilateral and mild hearing loss. HL is mostly conductive. Otitis media with effusion, adenoid enlargement, and recurrent upper RTI are common in patients with conductive hearing loss. Hearing assessment should be considered in all infants and children with Down syndrome.

References

Nightengale E, Yoon P, Wolter-Warmerdam K, Daniels D, Hickey F . Understanding Hearing and Hearing Loss in Children With Down Syndrome. Am J Audiol. 2017; 26(3):301-308. DOI: 10.1044/2017_AJA-17-0010. View

Baiduc R, Poling G, Hong O, Dhar S . Clinical measures of auditory function: the cochlea and beyond. Dis Mon. 2013; 59(4):147-56. PMC: 3603279. DOI: 10.1016/j.disamonth.2013.01.005. View

Lau W, Ko C, Cheng W . Prevalence and parental awareness of hearing loss in children with Down syndrome. Chin Med J (Engl). 2015; 128(8):1091-5. PMC: 4832951. DOI: 10.4103/0366-6999.155105. View

Paulson L, Weaver T, Macarthur C . Outcomes of tympanostomy tube placement in children with Down syndrome--a retrospective review. Int J Pediatr Otorhinolaryngol. 2013; 78(2):223-6. DOI: 10.1016/j.ijporl.2013.10.062. View

Laws G, Hall A . Early hearing loss and language abilities in children with Down syndrome. Int J Lang Commun Disord. 2014; 49(3):333-42. DOI: 10.1111/1460-6984.12077. View

Mesolella M, Iorio B, Ricciardiello F, Motta G . Efficacy of crenotherapy by Politzer in the treatment of otitis media with effusion in children with down syndrome. Int J Pediatr Otorhinolaryngol. 2019; 130:109803. DOI: 10.1016/j.ijporl.2019.109803. View

Austeng M, Akre H, Falkenberg E, Overland B, Abdelnoor M, Kvaerner K . Hearing level in children with Down syndrome at the age of eight. Res Dev Disabil. 2013; 34(7):2251-6. DOI: 10.1016/j.ridd.2013.04.006. View

Fujioka M, Young L, GIRDANY B . Radiographic evaluation of adenoidal size in children: adenoidal-nasopharyngeal ratio. AJR Am J Roentgenol. 1979; 133(3):401-4. DOI: 10.2214/ajr.133.3.401. View

McPherson B, Lai S, Leung K, Ng I . Hearing loss in Chinese school children with Down syndrome. Int J Pediatr Otorhinolaryngol. 2007; 71(12):1905-15. DOI: 10.1016/j.ijporl.2007.09.003. View

10.

Mitwalli M, Wahba Y, Shaltout A, Gouida M . Lymphocyte subgroups and recurrent infections in children with Down syndrome - a prospective case control study. Cent Eur J Immunol. 2018; 43(3):248-254. PMC: 6305610. DOI: 10.5114/ceji.2018.80042. View

11.

Abou-Youssef H, Kamal M, Mehaney D . Triple test screening for Down syndrome: an Egyptian-tailored study. PLoS One. 2014; 9(10):e110370. PMC: 4203785. DOI: 10.1371/journal.pone.0110370. View

12.

Maris M, Wojciechowski M, Van de Heyning P, Boudewyns A . A cross-sectional analysis of otitis media with effusion in children with Down syndrome. Eur J Pediatr. 2014; 173(10):1319-25. DOI: 10.1007/s00431-014-2323-5. View

13.

Martin T, Smith A, Breatnach C, Kent E, Shanahan I, Boyle M . Infants Born with Down Syndrome: Burden of Disease in the Early Neonatal Period. J Pediatr. 2017; 193:21-26. DOI: 10.1016/j.jpeds.2017.09.046. View

14.

Chhajed M, Madaan P, Saini L, Kaur S, Udani V . Normative Values for Auditory Brainstem Responses and Wave V Thresholds in Indian Neonates and Infants. Indian J Otolaryngol Head Neck Surg. 2023; 74(Suppl 3):3658-3661. PMC: 9895165. DOI: 10.1007/s12070-020-02347-1. View

15.

Tedeschi A, Roizen N, Taylor H, Murray G, Curtis C, Parikh A . The prevalence of congenital hearing loss in neonates with Down syndrome. J Pediatr. 2014; 166(1):168-71. DOI: 10.1016/j.jpeds.2014.09.005. View

16.

Blanc F, Ayache D, Calmels M, Deguine O, Francois M, Leboulanger N . Management of otitis media with effusion in children. Société française d'ORL et de chirurgie cervico-faciale clinical practice guidelines. Eur Ann Otorhinolaryngol Head Neck Dis. 2018; 135(4):269-273. DOI: 10.1016/j.anorl.2018.04.008. View

17.

Corsello A, Milani G, Picca M, Buzzetti R, Carrozzo R, Gambino M . Recurrent upper respiratory tract infections in early childhood: a newly defined clinical condition. Ital J Pediatr. 2024; 50(1):30. PMC: 10873936. DOI: 10.1186/s13052-024-01600-5. View

18.

Lertsburapa K, Schroeder Jr J, Sullivan C . Assessment of adenoid size: A comparison of lateral radiographic measurements, radiologist assessment, and nasal endoscopy. Int J Pediatr Otorhinolaryngol. 2010; 74(11):1281-5. DOI: 10.1016/j.ijporl.2010.08.005. View

19.

De Schrijver L, Topsakal V, Wojciechowski M, Van de Heyning P, Boudewyns A . Prevalence and etiology of sensorineural hearing loss in children with down syndrome: A cross-sectional study. Int J Pediatr Otorhinolaryngol. 2018; 116:168-172. DOI: 10.1016/j.ijporl.2018.10.048. View

20.

Mircher C, Toulas J, Cieuta-Walti C, Marey I, Conte M, Briceno L . Anthropometric charts and congenital anomalies in newborns with Down syndrome. Am J Med Genet A. 2017; 173(8):2166-2175. DOI: 10.1002/ajmg.a.38305. View