Download PDF
Semin Hear 2003; 24(3): 201-210
DOI: 10.1055/s-2003-41219
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Perspectives on the Audiological Evaluation of Individuals with Down Syndrome

Marilyn F. Dille
  • The University of Arizona, Department of Speech and Hearing Sciences, Tucson, Arizona
Further Information

Publication History

Publication Date:
14 August 2003 (online)

    Permissions and Reprints

ABSTRACT

Patients with Down syndrome often present with a complicated history of otologic disease, hearing loss, slow speech and language development, and cognitive delay. As audiologists, we are called upon to coordinate audiological care in the context of a team that includes healthcare and educational professionals. Our recommendations need to acknowledge and reflect the uniqueness of this population among the many populations that we serve. This article reviews several unique features characteristic of this population including: (1) the complexity of the middle ear disease that often is chronic and intractable, (2) the strategies for effective testing and common findings across the lifespan, (3) interpretation of objective measures in light of the syndrome, and (4) the unique aging process that is characteristic to Down syndrome.

KEYWORDS

Down syndrome - developmental disability - pediatric audiology

REFERENCES

  • 1 Diefendorf A O, Bull M J, Casey-Harvey D. et al . Down syndrome: a multidisciplinary perspective.  J Am Acad Audiol . 1995;  6 39-46
    PubMedGoogle Scholar
  • 2 Hartley X Y. A summary of recent research into the development of children with Down's syndrome.  J Ment Defic Res . 1986;  30 1-14
    PubMedGoogle Scholar
  • 3 Green G E. Evaluation of neurologic syndromes with mental retardation and auditory sequelae.  Semin Hear . 2003;  24 179-188
    Article in Thieme ConnectPubMedGoogle Scholar
  • 4 Wishart J G. The development of learning difficulties in children with Down's syndrome.  J Intellect Disab Res . 1993;  37 389-403
    PubMedGoogle Scholar
  • 5 Wishart J G. Cognitive abilities in children with Down syndrome: development instability and motivational deficits.  Prog Clin Biol Res . 1995;  393 57-91
    PubMedGoogle Scholar
  • 6 Buchanan L H. Early onset of presbyacusis in Down syndrome.  Scand Audiol . 1990;  19 103-110
    PubMedGoogle Scholar
  • 7 Schupf N, Kapell D, Lee J H. et al . Onset of dementia is associated with apolipoprotein E e4 in Down's syndrome.  Ann Neurol . 1996;  40 799-801
    CrossrefPubMedGoogle Scholar
  • 8 Strome S E, Strome M. Down syndrome: an otolaryngologic perspective.  J Otolaryngol . 1992;  21 394-397
    PubMedGoogle Scholar
  • 9 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 43-49
    CrossrefPubMedGoogle Scholar
  • 10 Selikowitz M. Short-term efficacy of tympanostomy tubes for secretory otitis media in children with Down syndrome.  Dev Med Child Neurol . 1993;  35 511-515
    PubMedGoogle Scholar
  • 11 Zigman W, Schupf N, Sersen E, Silverman W. Prevalence of dementia in adults with and without Down syndrome.  Am J Ment Retard . 1995;  100 403-412
    PubMedGoogle Scholar
  • 12 Campbell-Taylor I. Communication Impairments in Alzheimer Disease and Down Syndrome. New York: Oxford University Press; 1993
    Google Scholar
  • 13 Evenhuis H M. The natural history of dementia in Down's syndrome.  Arch Otolaryngol . 1990;  47 263-267
    PubMedGoogle Scholar
  • 14 Thase M E. The Relationship between Down Syndrome and Alzheimer's Disease. Cambridge, MA: MIT Press; 1988
    Google Scholar
  • 15 Moss S E, Tomoeda C K, Bayles K A. Comparison of the cognitive-linguistic profiles of Down syndrome adults with and without dementia to individuals with Alzheimer's disease.  J Med Speech-Lang Pathol . 2000;  8 69-81
    PubMedGoogle Scholar
  • 16 Young E C, Kramer B M. Characteristics of age-related language decline in adults with Down syndrome.  Ment Retard . 1991;  29 75-79
    PubMedGoogle Scholar
  • 17 Grundfast K M, Camilon F. External auditory canal stenosis and partial atresia without associated anomalies.  Ann Otol Rhinol Laryngol . 1986;  95 505-509
    PubMedGoogle Scholar
  • 18 Balkany T J, Mischke R E, Downs M P, Jafek B W. Ossicular abnormalities in Down's syndrome.  Otolaryngol Head Neck Surg . 1979;  87 372-384
    PubMedGoogle Scholar
  • 19 Mazzoni D S, Ackley R S, Nash D J. Abnormal pinna type and hearing loss correlations in Down's syndrome.  J Intellect Disabil Res . 1994;  38 549-560
    PubMedGoogle Scholar
  • 20 Brown P M, Lewis G TR, Parker A J, Maws A R. The skull base and nasopharynx in Down's syndrome in relation to hearing impairment.  Clin Otolaryngol . 1989;  14 241-246
    PubMedGoogle Scholar
  • 21 Igarashi M, Takahashi M, Alford B R, Johnson P E. Inner ear morphology in Down's syndrome.  Acta Otolaryngol . 1977;  83 175-181
    PubMedGoogle Scholar
  • 22 Bilgin H, Kasemsuwan L, Schachern P, Paparella M, Le C. Temporal bone study of Down's syndrome.  Arch Otolaryngol . 1996;  122 271-275
    PubMedGoogle Scholar
  • 23 Iino Y, Imamura Y, Harigai S, Tanaka Y. Efficacy of tympanostomy tube insertion for otitis media with effusion in children with Down syndrome.  Int J Pediatr Otorhinolaryngol . 1999;  49 143-149
    CrossrefPubMedGoogle Scholar
  • 24 Greenberg D B, Wilson W R, Moore J M, Thompson G. Visual reinforcement audiometry (VRA) with young Down's syndrome children.  J Speech Hear Disord . 1978;  43 448-458
    PubMedGoogle Scholar
  • 25 Thompson G, Wilson W R, Moore J M. Application of visual reinforcement audiometry (VRA) to low functioning children.  J Speech Hear Disord . 1979;  44 80-90
    PubMedGoogle Scholar
  • 26 Werner L A, Mancl L R, Folsom R C. Preliminary observations on the development of auditory sensitivity in infants with Down syndrome.  Ear Hear . 1996;  17 455-468
    PubMedGoogle Scholar
  • 27 Olsho L W, Koch E G, Halpin C F, Carter E A. An observer-based psychoacoustic procedure for use with young infants.  Dev Psychol . 1987;  23 627-640
    PubMedGoogle Scholar
  • 28 Matkin N. Assessment of Hearing Sensitivity during the Preschool Years. New York: Grune and Stratton; 1977
    Google Scholar
  • 29 Maurizi M, Ottaviani F, Paludetti G, Lungarotti S. Audiological findings in Down's children.  Int J Pediatr Otorhinolaryngol . 1985;  9 227-232
    PubMedGoogle Scholar
  • 30 Roizen N J, Wolters C, Blondis T A. Hearing loss in children with Down syndrome.  J Pediatr . 1993;  123 S9-S12
    CrossrefPubMedGoogle Scholar
  • 31 Marcell M M, Cohen S. Hearing abilities of Down syndrome and other mentally handicapped adolescents.  Res Dev Disabil . 1992;  13 533-551
    PubMedGoogle Scholar
  • 32 van Gorp E, Baker R. The incidence of hearing impairment in a sample of Down's syndrome schoolchildren.  Int J Rehab Res . 1984;  7 198-199
    PubMedGoogle Scholar
  • 33 Widen J E, Folsom R C, Thompson G, Wilson W R. Auditory brainstem responses in young adults with Down syndrome.  Am J Ment Defic . 1987;  91 472-479
    PubMedGoogle Scholar
  • 34 Dean J, Harris F. Adaptive hierarchical test procedures for developmentally delayed adults: taking the "difficult" out of "difficult to test." Semin Hear .  2003;  24 247-262
    PubMedGoogle Scholar
  • 35 Hassmann E, Skotnicka B, Midro A, Musiatowicz M. Distortion products otoacoutic emissions in diagnosis of hearing loss in Down syndrome.  Int J Pediatr Otorhinolaryngol . 1998;  45 199-206
    CrossrefPubMedGoogle Scholar
  • 36 Jiang Z D, Wu Y Y, Liu X Y. Early development of brainstem auditory evoked potentials in Down's syndrome.  Early Human Development . 1990;  23 41-51
    CrossrefPubMedGoogle Scholar
  • 37 Folsom R C, Widen J E, Wilson W R. Auditory brain-stem responses in infants with Down's syndrome.  Arch Otolaryngol . 1983;  109 607-610
    PubMedGoogle Scholar
  • 38 Kaga K, Marsh R R. Auditory brainstem responses in young children with Down's syndrome.  Intern J Pediatr Otorhinolaryngol . 1986;  11 29-38
    PubMedGoogle Scholar
  • 39 Squires N, Aine C, Buchwald J, Norman R, Galbraith G. Auditory brain stem response abnormalities in severely and profoundly retarded adults.  Electroencephal Clin Neurophysiol . 1980;  50 172-185
    PubMedGoogle Scholar
  • 40 Evenhuis H M, van Zanten A G, Brocaar M P, Broerdinkholder W HM. Hearing loss in middle-age persons with Down syndrome.  Am J Ment Defic . 1992;  97 47-56
    PubMedGoogle Scholar
  • 41 Keiser H, Montague J, Wold D, Maune S, Pattison D. Hearing loss of Down syndrome adults.  Am J Ment Defic . 1981;  85 467-472
    PubMedGoogle Scholar
  • 42 Hearing Screening. American Speech-Language-Hearing Association. Available at: www.asha.org/ hearing/testing/index.cfm. Accessed November 27 2002
    Google Scholar
      >