When Hearing Loss Occurs with Multiple Disabilities

Many deaf and hard-of-hearing children have, in addition to hearing loss, one or more conditions that result in multiple disabilities. The most prevalent conditions that co-occur with hearing loss are learning disorders, intellectual disabilities, attention disorders, visual impairment, and cerebral palsy. In this article we examine these conditions and their implications for audiologic management, including assessment of hearing, selection and fitting of hearing aids, and clinical application of various hearing technologies. The need for specialized medical management is considered along with the role of the pediatrician and medical home. Also considered are issues relevant to educational placement, assessment, and intervention by professionals from multiple disciplines, and parent-professional communication. The article concludes with the personal perspective of a parent whose child experienced severe illness at birth and now has multiple disabilities.

KEYWORDS

Multiple disabilities - children - hearing loss - pediatric assessment and intervention

REFERENCES

• 1 Pagon R, Graham J, Zonana J, Yong S. Coloboma, congenital heart disease, and choanal atresia with multiple anomalies: CHARGE association.  J Pediatr. 1981;  99 223-227
• 2 Edwards B, Kileny P, Van Riper L. CHARGE syndrome: a window of opportunity for audiologic intervention.  Pediatrics. 2002;  110 119-126
• 3 Vohr B, Widen J, Cone-Wesson B et al.. Identification of neonatal hearing impairment: characteristics of infants in the neonatal intensive care unit and well-baby nursery.  Ear Hear. 2000;  21 373-382
• 4 Gallaudet Research Institute. Regional and National Summary Report of Data from the 2001-2002 Annual Survey of Deaf and Hard of Hearing Children and Youth. Washington, DC; GRI, Gallaudet University 2003
• 5 Beitchman J, Young A. Learning disorders with a special emphasis on reading disorders: a review of the past 10 years.  J Am Acad Child Adolesc Psychiatry. 1997;  36 1020-1032
• 6 Cantwell D, Baker L. Developmental Speech and Language Disorders. New York; Guilford 1987
• 7 Cantwell D, Baker L. Psychiatric and Developmental Disorders in Children with Communication Disorders. Washington, DC; American Psychiatric Press 1991
• 8 Hammill D. On defining learning disabilities: an emerging consensus.  J Learn Disabil. 1990;  23 74-84
• 9 Kavale K, Forness S, Lotsbach T. Definition for definitions of learning disabilities.  Learning Disability Quarterly. 1991;  14 257-268
• 10 Kavale K, Forness S. The Nature of Learning Disabilities: Critical Elements of Diagnosis and Classification. Mahwah, NJ; Lawrence Erlbaum 1995
• 11 Shaw S, Cullen J, McGuire J, Brinckerhoff L. Operationalizing a definition of learning disabilities.  J Learn Disabil. 1995;  28 586-597
• 12 Hallahan D, Kauffman J, Lloyd J. Introduction to Learning Disabilities, 3rd ed. Englewood Cliffs, NJ; Prentice Hall 1996
• 13 Love R, Webb W. Neurology for the Speech-Language Pathologist. Boston; Butterworth-Heinemann 1992
• 14 Murdoch, B Acquired Neurological Speech/Language Disorders in Childhood. London; Taylor and Francis 1991
• 15 Rourke B. Nonverbal Learning Disabilities: The Syndrome and the Model. New York; Guilford 1989
• 16 Swanson H. Information processing theory and learning disabilities: an overview.  J Learn Disabil. 1987;  20 3-7
• 17 Swanson H. Memory subtypes in learning disabled readers.  Learn Disabil. 1988;  11 342-357
• 18 Zemlin W. Speech and Hearing Sciences: Anatomy and Physiology, 3rd ed. Englewood Cliffs, NJ; Prentice Hall 1988
• 19 Hurst J, Baraitser M, Auger E, Graham F, Norell S. An extended family with a dominantly inherited speech disorder.  Dev Med Child Neurol. 1990;  32 352-355
• 20 Tomblin J, Buckwalter P. Studies of the genetics of specific language impairment. In: Watkins T, Rice M Specific Language Impairments in Children. Baltimore, MD; Paul H. Brookes 1994: 17-34
• 21 Pennington B. Genetics of learning disabilities.  J Child Neurol. 1995;  10(suppl x1) 69-77
• 22 Huttenlocher J, Haight W, Bryk A, Seltzer M, Lyson T. Early vocabulary growth: relation to language input and gender.  Dev Psychol. 1991;  27 236-248
• 23 Shywitz S. Dyslexia.  Sci Am. 1996;  275 98-104
• 24 Torgesen J, Wagner R, Rashotte C. Longitudinal studies of phonological processing and reading.  J Learn Disabil. 1994;  27 276-286
• 25 National Deaf Education Center, Gallaudet University. Deaf children with multiple disabilities. Available at: http://clerccenter.gallaudet.edu/InfoToGo/141.html Accessed February 9, 2004
• 26 American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 4th Washington, DC; APA 1994
• 27 Fraser G. Profound childhood deafness.  J Med Genet. 1964;  1 118-151
• 28 Holland A. Genetic aspects of visual and auditory impairment in mentally handicapped people. In: Ellis D Sensory Impairments in Mentally Handicapped People. Beckenham, KY; Croom-Helm 1986: 149-166
• 29 Balkany T. Otologic aspects of Down’s syndrome.  Semin Speech Lang. 1980;  1 39-48
• 30 Bond D. Mental health in children who are deaf and have multiple disabilities>. In: Hindley P, Kitson N Mental Health and Deafness. London; Whurr Publishers 2000: 127-148
• 31 Hildmann A, Hildmann H, Kessler A. Hearing disorders in children with Down’s syndrome.  Laryngorhinootologie. 2002;  81 3-7
• 32 Shott S R, Joseph A, Heithaus D. Hearing loss in children with Down syndrome.  Int J Pediatr Otorhinolaryngol. 2001;  61 199-205
• 33 Mazzoni D, Ackley R, Nash D. Abnormal pinna type and hearing loss correlations in Down's syndrome.  J Intellect Disabil Res. 1994;  38 549-560
• 34 Buchanan L. Early onset of presbyacusis in Down syndrome.  Scand Audiol. 1990;  19 103-110
• 35 Yeates S. The incidence and importance of hearing loss in people with severe learning disability: the evolution of a service.  Br J Learn Disabil. 1995;  23 79-84
• 36 Carvill S. Sensory impairments, intellectual disability and psychiatry.  J Intellect Disabil Res. 2001;  45 467-483
• 37 Wetherby A. Communication and language disorders in infants, toddlers, and preschool children. In: Shames G, Wiig E, Secord W Human Communication Disorders: An Introduction, 5th ed. Boston; Allyn and Bacon 1998: 155-184
• 38 Levine M. Developmental Variation and Learning Disorders, 2nd ed. Cambridge, MA; Educators Publishing Service Inc 2001
• 39 Kelly D, Aylward G. Attention deficits in school-aged children and adolescents: current issues and practice.  Pediatr Clin North Am. 1992;  39 487-512
• 40 Chess S, Fernandez P. Impulsivity in rubella deaf children: a longitudinal study.  Am Ann Deaf. 1980;  125 505-509
• 41 Levine M. Attention deficits: the diverse effects of weak control systems in childhood.  Pediatr Ann. 1987;  16 117-131
• 42 August G, Garfunkel B. Co-morbidity of ADHD and reading disability among clinic-referred children.  J Abnorm Child Psychol. 1990;  18 29-45
• 43 Kelly D, Kelly B, Jones M, Moulton N, Verhulst S, Bell S. Attention deficits in children and adolescents with hearing loss: a survey.  Am J Dis Child. 1993;  147 737-741
• 44 Kelly D, Forney G, Parker-Fisher S, Jones M. The challenge of attention deficit disorder in children who are deaf or hard of hearing.  Am Ann Deaf. 1993;  138 343-348
• 45 Kelly D, Forney G, Parker-Fisher S, Jones M. Evaluating and managing attention deficit disorder in children who are deaf or hard of hearing.  Am Ann Deaf. 1993;  138 349-357
• 46 Barkley R. Attention Deficit Hyperactivity Disorder: A Handbook for Diagnosis and Treatment. New York; Guilford Press 1990
• 47 Van Dijk J. Rubella handicapped children. In: Blank M, Bakker D Modern Approaches to the Diagnosis and Instruction of Multi-Handicapped Children. Lisse, The Netherlands; Swets and Zeitlinger 1982: 7-179
• 48 Aitken S. Understanding deafblindness. In: Aitken S, Buultjens M, Clark C, Eyre J, Pease L Teaching Children Who Are Deaf-Blind. London; David Fulton Publishers Ltd 2000: 1-34
• 49 Baldwin V. Annual Deaf-Blind Census. Monmouth, OR; Teaching Research Division 1994
• 50 Jensema C. A profile of deaf-blind children within various types of educational facilities.  Deaf-Blind News. 1980;  80 896-900
• 51 Miles B. Overview on deaf-blindness. DB-LINK: The National Clearinghouse on Children who are Deaf-Blind. 2003 Available at: http://www.tr.wou.edu/dblink/overview.htm Accessed December 10, 2003
• 52 Shafer S, Moss K. Cerebral palsy and children with vision and hearing loss. http://www.deafblind.com/cerebral.html Accessed December 2003
• 53 Robinson R. The frequency of other handicaps in children with cerebral palsy.  Dev Med Child Neurol. 1983;  15 305-312
• 54 Winter S, Autry A, Boyle C, Yeargin-Allsopp M. Trends in the prevalence of cerebral palsy in a population-based study.  Pediatrics. 2002;  110 1220-1225
• 55 Bhushan V, Paneth N, Kiely J. Impact of improved survival of very low birth weight infants on recent secular trends in the prevalence of cerebral palsy.  Pediatrics. 1993;  91 1094-1100
• 56 Centers for Disease Control and Prevention (CDC) . Economic costs associated with mental retardation, cerebral palsy, hearing loss, and vision impairment.  Morb Mortal Wkly Rep. 2004;  53 57-59
• 57 Harrison M, Roush J, Wallace J. Trends in the age of identification and intervention for deaf and hard-of-hearing infants.  Ear Hear. 2003;  24 89-95
• 58 Tharpe A M. Cochlear implantation of children with multiple disabilities. Paper presented at the annual convention of the American Academy of Audiology Salt Lake City, UT; 2004
• 59 Pediatric Working Group of the Conference on Amplification for Children with Auditory Deficits. Amplification for infants and children with hearing loss.  Am J Audiol. 1996;  5 53-68
• 60 Desired Sensation Level Method .University of Western Ontario. Available at: http://www.dslio.com Accessed January 2004
• 61 Tharpe A, Ashmead D, Ricketts T, Rothpletz A, Wall R. Optimization of amplification for deaf-blind children. In: Seewald, RC, Gravel JS A Sound Foundation through Early Amplification 2001: Proceedings of the Second International Conference. Suffolk, UK; St. Edmundsbury Press 2000
• 62 Zimmerman-Phillips S, Osberger M, Robbins A. Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS). Sylmar, CA; Advanced Bionics Corp 1997
• 63 Niparko J, Blankenhorn R. Cochlear implants in young children.  Ment Retard Dev Disabil Res Rev. 2003;  9 267-275
• 64 Waltzman S, Scalchunes V, Cohen N. Performance of multiply handicapped children using cochlear implants.  Am J Otol. 2000;  21 329-335
• 65 Joint Committee on Infant Hearing . Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs.  Pediatrics. 2000;  106 798-817
• 66 American Academy of Pediatrics, Ad Hoc Task Force on Definition of the Medical Home. The medical home.  Pediatrics. 1992;  90-774
• 67 Individuals with Disabilities Education Act Amendments of 1997, PL no. 105-17,111, Stat. 38 (1997).  Codified as amended at 20 USC Section. 1400-1485
• 68 Diefendorf A, Bull M, Casey-Harvey D et al.. Down syndrome: a multidisciplinary perspective.  J Am Acad Audiol. 1995;  6 39-46
• 69 Cherow E, Matkin N, Trybus R Hearing Impaired Children and Youth with Developmental Disabilities: An Interdisciplinary Foundation for Service. Washington, DC; Gallaudet University Press 1985
• 70 Jones T. Behavior modification studies with hearing-impaired students: a review.  Am Ann Deaf. 1984;  129 451-458
• 71 Ewing K, Jones T. An educational rationale for deaf students with multiple disabilities.  Am Ann Deaf. 2003;  148 267-271