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ORIGINAL ARTICLE
Evaluation of hearing impairment in patients with Down syndrome
1
Department of Logopedics and Applied Linguistics, University of Maria Curie-Skłodowska, Lublin, Poland
2
World Hearing Center, Institute of Physiology and Pathology of Hearing,
Warsaw/Kajetanty, Poland
A - Research concept and design; B - Collection and/or assembly of data; C - Data analysis and interpretation; D - Writing the article; E - Critical revision of the article; F - Final approval of article;
Submission date: 2023-07-25
Final revision date: 2023-08-09
Acceptance date: 2023-08-09
Online publication date: 2023-09-21
Publication date: 2023-09-21
Corresponding author
Katarzyna Sienkiewicz
Department of Logopedics and Applied Linguistics, University of Maria Curie-Skłodowska, Sowińskiego 17, 20-400, Lublin, Poland
Department of Logopedics and Applied Linguistics, University of Maria Curie-Skłodowska, Sowińskiego 17, 20-400, Lublin, Poland
J Hear Sci 2023;13(3):36-41
KEYWORDS
otoacoustic emissionsABRhearing disordersDown syndrome (DS)objective hearing testsimpedance audiometry
TOPICS
ABSTRACT
Introduction:
It is estimated that the prevalence of hearing loss in children with Down syndrome (DS) ranges from 38% to 82%. However, the diagnosis of hearing loss in DS is difficult, due to an impaired ability to cooperate for subjective tests. Thus, objective tests such as impedance audiometry, otoacoustic emissions, and auditory brainstem responses (ABRs) may be more appropriate. In this study objective methods without anesthesia were used to determine the amount, type, and prevalence of hearing loss in people with DS.
Material and methods:
The study included 39 subjects with DS, ranging in age from 1 year to 27 years. Hearing tests in DS subjects were performed during physiological sleep or while awake. Otoscopic examination was performed in all DS subjects. If no abnormalities were seen, tympanometry, otoacoustic emissions, and recording of ABRs were attempted.
Results:
Objective hearing tests showed that the DS group had various types of hearing disorders. Based on all objective tests carried out in all ears (n = 78), the following diagnoses were made: normal hearing, 36 ears (46%); cochlear hearing loss, 27 ears (35%); conductive hearing loss, 8 ears (10%); suspected deafness, 7 ears (9%). Based on wave V thresholds, the following degrees of hearing loss were established: normal hearing, 36 ears (46%); 21–40 dB nHL, 21 ears (27%); 50–60 dB nHL, 8 ears (10%); 70–80 dB nHL, 6 ears (8%); > 80 dB nHL, 7 ears (9%).
Conclusions:
In previous studies of DS subjects by other authors, conductive hearing loss predominated, followed by sensorineural hearing loss. In the present study, more sensorineural than conductive hearing loss was diagnosed. In most cases, the diagnosis of the type of hearing disorder was based on the ABR result, and other tests such as tympanometry and otoacoustic emissions played a supporting role. Due to the often limited cooperation of the patient, the DPOAE test was difficult to perform.
It is estimated that the prevalence of hearing loss in children with Down syndrome (DS) ranges from 38% to 82%. However, the diagnosis of hearing loss in DS is difficult, due to an impaired ability to cooperate for subjective tests. Thus, objective tests such as impedance audiometry, otoacoustic emissions, and auditory brainstem responses (ABRs) may be more appropriate. In this study objective methods without anesthesia were used to determine the amount, type, and prevalence of hearing loss in people with DS.
Material and methods:
The study included 39 subjects with DS, ranging in age from 1 year to 27 years. Hearing tests in DS subjects were performed during physiological sleep or while awake. Otoscopic examination was performed in all DS subjects. If no abnormalities were seen, tympanometry, otoacoustic emissions, and recording of ABRs were attempted.
Results:
Objective hearing tests showed that the DS group had various types of hearing disorders. Based on all objective tests carried out in all ears (n = 78), the following diagnoses were made: normal hearing, 36 ears (46%); cochlear hearing loss, 27 ears (35%); conductive hearing loss, 8 ears (10%); suspected deafness, 7 ears (9%). Based on wave V thresholds, the following degrees of hearing loss were established: normal hearing, 36 ears (46%); 21–40 dB nHL, 21 ears (27%); 50–60 dB nHL, 8 ears (10%); 70–80 dB nHL, 6 ears (8%); > 80 dB nHL, 7 ears (9%).
Conclusions:
In previous studies of DS subjects by other authors, conductive hearing loss predominated, followed by sensorineural hearing loss. In the present study, more sensorineural than conductive hearing loss was diagnosed. In most cases, the diagnosis of the type of hearing disorder was based on the ABR result, and other tests such as tympanometry and otoacoustic emissions played a supporting role. Due to the often limited cooperation of the patient, the DPOAE test was difficult to perform.
REFERENCES (33)
1.
Korenberg JR, Chen XN, Schipper R, et al. Down syndrome phenotypes the consequences of chromosomal imbalance. Proc Natl Acad Sci USA, 1994; 91(11): 4997–5001. https://doi.org/10.1073/pnas.9....
2.
Korniszewski L. [Taking care of a child with Down syndrome]. Medipress Pediatria, 1995; 1(1): 5–10 [in Polish].
3.
Opitz JM, Gilbert-Barness EF. Reflections on the pathogenesis of Down syndrome. Am J Med Genet 1990; 37(Suppl 7): 38–51. https://doi.org/10.1002/ajmg.1....
4.
Maurizi M, Ottaviani F, Paludetti G, Lungarottic S, Audiological findings in Down’s children. Int J Pediatr Otorhinolaryngol, 1985; 9(3): 227–32. https://doi.org/10.1016/s0165-....
5.
Maroudias N, Economides J, Christodoulou P, Helidonis E. A study on the otoscopical and audiological findings in patients with Down’s syndrome in Greece. Int J Pediatr Otorhinolaryngol, 1994; 29(1): 43–9. https://doi.org/10.1016/0165-5....
6.
Pilewski W. [Establishment of the auditory pathways in newborns in the light of studies of evoked potentials from the brainstem]. PhD thesis. Medical Academy; 1989 [in Polish].
7.
Roizen NJ, Wolters C, Nicol TA, Blondis TA. Hearing loss in children with Down syndrome. J Pediatr, 1993; 123(1): 9–12. https://doi.org/10.1016/s0022-....
8.
Sadowska L, Mysłek-Prucnal M, Choioska AM, Mazur A. [Diagnostics and therapy of children with Down’s syndrome based on own research and literature review]. Przegląd Medyczny Uniwersytetu Rzeszowskiego, 2009; 1: 10–12 [in Polish].
9.
Kręcicki T, Zalesska-Kręcicka M, Kubiak K, Gawron W. Brain auditory evoked potentials in children with Down syndrome. Int J Pediatr Otorhinolaryngol, 2005; 69: 615–20. https://doi.org/10.1016/j.ijpo....
10.
Sato M, Suzuki S. Auditory brainstem responses in newborns with Down syndrome at a Japanese Perinatal Center. Int J Clin Pediatr, 2014; 3(3): 69–71. https://doi.org/10.14740/ijcp1....
11.
Squires N, Aine C, Buchwald J, Norman R, Galbraith G. Auditory brainstem responses abnormalities in severely and profoundly retarded adults. Electroencephalogram Clin Neurophysiol, 1980; 50: 172–85. https://doi.org/10.1016/0013-4....
12.
Cooley WC, Graham JM. Down syndrome: an update and review for the primary pediatrician. Clin Pediatr (Phila), 1991; 30(4): 233–53. https://doi.org/10.1177/000992....
13.
Szyfter W, Łączkowska-Przybylska J. [Hearing impairment in children with Down’s syndrome]. Otolaryngol Pol, 1995; 49(5): 436–44 [in Polish].
14.
Rodman R, Pine HS. The otolaryngologist’s approach to the patient with Down syndrome. Otolaryngol Clin North Am, 2012; 45(3): 599–629. https://doi.org/10.1016/j.otc.....
15.
Venail F, Gardiner Q, Mondain M. ENT and speech disorders in children with Down’s syndrome: an overview of pathophysiology, clinical features, treatments, and current management. Clin Pediatr (Phila), 2004; 43(9): 783–91. https://doi.org/10.1177/000992....
16.
Intrapiromkul J, Aygun N, Tunkel DE, Carone M, Yousem DM. Inner ear anomalies seen on CT images in people with Down syndrome. Pediatr Radiol, 2012; 42(12): 1449–55. https://doi.org/10.1007/s00247....
17.
Folsom RC, Widen JE, Wilson WR. Auditory brain-stem responses in infants with Down’s syndrome. Arch Otolaryngol, 1983; 109(9): 607–10. https://doi.org/10.1001/archot....
18.
Hassmann E, Skotnicka B, Midro A, Musiatowicz M. Distortion products otoacoustic emissions in diagnosis of hearing loss in Down syndrome. Int J Pediatr Otorhinolaryngol, 1998; 45(3): 199–206. https://doi.org/10.1016/s0165-....
19.
Iino Y, Imamura Y, Harigi S, Tanaka Y. Efficacy of tympanostomy tube insertion for otitis media with effusion in children with Down syndrome. Int J Pediatr Otorhinolaryngol, 1999; 49(2): 143–9. https://doi.org/10.1016/s0165-....
20.
Kaga K, Marsh R. Auditory brainstem responses in young children with Down’s syndrome. Int J Pediatr Otolaryngol, 1986; 11(1): 29–38. https://doi.org/10.1016/s0165-....
21.
Pappas DG, Flexer C, Shackelford MA. Otological and habilitative management of children with Down syndrome. Laryngoscope, 1994; 104(9): 1065–70. https://doi.org/10.1288/000055....
22.
Shott SR, Joseph A, Heithaus D. Hearing loss in children with Down syndrome. Int Pediatr Otorhinolaryngol, 2001; 61(3): 199–205. https://doi.org/10.1016/s0165-....
23.
Davis B. Auditory disorders in Down’s syndrome. Scand Audiol Suppl, 1998; 30: 65–8.
24.
Balkany TDM. Hearing loss in Downs syndrome: a treatable handicap more common than generally recognized. Clin Pediatr, 1979; 18(2): 116–8. https://doi.org/10.1177/000992....
25.
McPherson B, Lai SP, Leung KK. Hearing loss in Chinese school children with Down syndrome. Int J Pediatr Otorhinolaryngol, 2007; 71(12): 1905–15. https://doi.org/10.1016/j.ijpo....
26.
Buchanan LH. Early onset of presbyacusis in Down syndrome. Scand Audiol, 1990; 19(2): 103–10. https://doi.org/10.3109/010503....
27.
Brooks DN, Wooley H, Kanjilal GC. Hearing loss and middle ear disorders in patients with Down’s syndrome (Mongolism). J Ment Defic Res, 1972; 16(1): 21–9. https://doi.org/10.1111/j.1365....
28.
Krmpotic-Nemanic J. Down’s syndrome and presbyacousis. Lancet, 1970; 2(7674): 670–1. https://doi.org/10.1016/s0140-....
29.
Jiang ZD, Wu YY, Liu XY. Early development of brainstem auditory evoked potentials in Down’s syndrome. Early Hum Dev, 1990; 23(1): 41–51. https://doi.org/10.1016/0378-3....
30.
Sienkiewicz K, Piłka A, Kochanek K. [Evaluation of the usefulness of objective methods of hearing testing in children and adolescents with Down syndrome: preliminary study]. Now Audiofonol, 2014; 3(3): 24–8 [in Polish].
31.
Hoda AK, Ribha FJ, Ziad ZM. A pilot study of the relationship between Down’s syndrome and hearing loss. Saudi Med J, 2000; 21(10): 931–3.
32.
Raut P, Sriram B, Yeoh A, Hee KYM, Lim SB, Daniel ML. High prevalence of hearing loss in Down syndrome at first year of life. Ann Acad Med Singapore, 2011; 40: 493–8.
33.
Soares J, Urosas JG, Calarga KS, Pichelli TS, Limongi SCO, Navid Shahnaz N, et al. Wideband reflectance in Down syndrome. Int J Ped Otolaryngol, 2016; 87: 164–71. https://doi.org/10.1016/j.ijpo....
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