Perspectives on the Audiological Evaluation of Individuals with Down Syndrome

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
• 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
• 3 Green G E. Evaluation of neurologic syndromes with mental retardation and auditory sequelae.  Semin Hear . 2003;  24 179-188
• 4 Wishart J G. The development of learning difficulties in children with Down's syndrome.  J Intellect Disab Res . 1993;  37 389-403
• 5 Wishart J G. Cognitive abilities in children with Down syndrome: development instability and motivational deficits.  Prog Clin Biol Res . 1995;  393 57-91
• 6 Buchanan L H. Early onset of presbyacusis in Down syndrome.  Scand Audiol . 1990;  19 103-110
• 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
• 8 Strome S E, Strome M. Down syndrome: an otolaryngologic perspective.  J Otolaryngol . 1992;  21 394-397
• 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
• 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
• 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
• 12 Campbell-Taylor I. Communication Impairments in Alzheimer Disease and Down Syndrome. New York: Oxford University Press; 1993
• 13 Evenhuis H M. The natural history of dementia in Down's syndrome.  Arch Otolaryngol . 1990;  47 263-267
• 14 Thase M E. The Relationship between Down Syndrome and Alzheimer's Disease. Cambridge, MA: MIT Press; 1988
• 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
• 16 Young E C, Kramer B M. Characteristics of age-related language decline in adults with Down syndrome.  Ment Retard . 1991;  29 75-79
• 17 Grundfast K M, Camilon F. External auditory canal stenosis and partial atresia without associated anomalies.  Ann Otol Rhinol Laryngol . 1986;  95 505-509
• 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
• 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
• 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
• 21 Igarashi M, Takahashi M, Alford B R, Johnson P E. Inner ear morphology in Down's syndrome.  Acta Otolaryngol . 1977;  83 175-181
• 22 Bilgin H, Kasemsuwan L, Schachern P, Paparella M, Le C. Temporal bone study of Down's syndrome.  Arch Otolaryngol . 1996;  122 271-275
• 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
• 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
• 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
• 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
• 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
• 28 Matkin N. Assessment of Hearing Sensitivity during the Preschool Years. New York: Grune and Stratton; 1977
• 29 Maurizi M, Ottaviani F, Paludetti G, Lungarotti S. Audiological findings in Down's children.  Int J Pediatr Otorhinolaryngol . 1985;  9 227-232
• 30 Roizen N J, Wolters C, Blondis T A. Hearing loss in children with Down syndrome.  J Pediatr . 1993;  123 S9-S12
• 31 Marcell M M, Cohen S. Hearing abilities of Down syndrome and other mentally handicapped adolescents.  Res Dev Disabil . 1992;  13 533-551
• 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
• 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
• 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
• 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
• 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
• 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
• 38 Kaga K, Marsh R R. Auditory brainstem responses in young children with Down's syndrome.  Intern J Pediatr Otorhinolaryngol . 1986;  11 29-38
• 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
• 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
• 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
• 42 Hearing Screening. American Speech-Language-Hearing Association. Available at: www.asha.org/ hearing/testing/index.cfm. Accessed November 27 2002