Hearing 7 22.09.00 BASIC MEDICAL SURVEILLANCE ESSENTIALS FOR PEOPLE WITH DOWN'S SYNDROME. HEARING IMPAIRMENT (One of a set of guidelines drawn up by the Down's Syndrome Medical Interest Group. Approved by BACDA and BAAP. Sept 2000) 1. Well over 50% of people with Down's syndrome have significant hearing impairment which may be mild, moderate, severe or profound. Sensorineural and/or conductive loss may be present at any age. 3.4.5.7.17. Hearing impairment can be successfully managed in this population. If undetected it is likely to be a significant cause of preventable secondary handicap 3.10.12.13.21. Lifelong audiological surveillance is essential for all. The main cause of conductive loss is persistent otitis media with effusion (OME, glue ear). The natural history of OME and response to intervention differ from that in the general population hence local surveillance and management protocols need to be set up specific to people with Down's syndrome.3.5.9.19 2. People with Down's syndrome of all ages should have rapid access to specialist audiology services 3. 3. Because of an increased incidence of congenital sensorineural loss newborns should be included in neonatal screening programmes 1.14. This does not preclude the need for ongoing surveillance 8. 4. Guidance for parents of children with Down's syndrome should include discussion about hearing problems and their management, supported by good quality written information.15 5. Whether or not a baby has passed a neonatal screen all should have full audiological assessment between age 6 and 10 months. This should include measurement of auditory thresholds, impedance testing and otoscopy 18. To ensure inclusion of the child with Down's syndrome participation in existing child health hearing surveillance programmes should be encouraged. 6. Therefore by 10 months it should have been established whether or not a child has any degree of permanent hearing loss with or without OME. A clear management plan must have been agreed with the parents and intervention instigated where necessary. 7. In the second year (usually around 18 months) all children ­ whatever their previous hearing status - should have further audiological review carried out in a manner appropriate for a child with learning disability. This should include assessment of auditory thresholds, impedance testing and otoscopy. This should be repeated at least yearly until age 5 and thereafter 2 yearly for life. More frequent testing will be necessary if problems exist. 8. Transition of care from paediatric to adult services should involve direct transfer of care to a named person. --------------------------------------------------------------------------------------------------------------------------------------------------------------------------- DSMIG (UK) 2001 www.dsmig.org.uk 15.01.02 Hearing 7 22.09.00 9. At all ages people with Down's syndrome have narrow ear canals which predispose to accumulation of wax4. This may affect impedance testing and hearing. 10. Most people with Down's syndrome are able to respond to standard tests ­ eg distraction; speech discrimination; pure tone audiometry (play or standard); and visual reinforcement audiometry ­ as long as these are carried out by testers with expertise in working with people with learning disability. Threshold measurement tests appropriate to developmental age must be used 6.20. 11. Because of increased incidence of sensorineural as well as conductive loss the frequency range tested should include 8000Hz whenever feasible as this may be an early warning of impending sensorineural deafness 11.22. 12. Diagnostic Auditory Brain Stem (ABR) responses in people with Down's syndrome must be interpreted with caution 7.22 13. As in the general population all those who are hearing impaired should have access to specialist hearing support services (Speech and Language Therapy; Teachers of the deaf; hearing Therapists etc) 14. At all ages particular attention should be paid to the treatment of suppurative nasal and ear conditions 3.16. 15. In adults with the syndrome hearing assessment is essential in the differential diagnosis of depression and dementia 7. References 1. Barnet,AB.,Weiss,IP.,Aysun,S.,Bernardo,EB.,Saumweger,RW.,Hines,A.(1988) Hearing loss in infants with Down's syndrome. Paediatric Research.25. 289A 2. Bennett.KE.,Haggard,MP.(1999) Behaviour and cognitive outcomes from middle ear disease. Arch.Dis.Child.80.28-35 3. Cunningham,C.,McArthur,K.,(1981) Hearing loss and treatment in young Down's syndrome children. Child: care,health and development. 7 : 357-374. 4. Dahle,AJ.,McCollister,FP.,(1986) Hearing and otologic disorders in children with Down syndrome. American Journal of Mental Deficiency. 90 (6) : 636-642. 5. Davies,B.,Penniceard,RM.(1980) Auditory function and receptive vocabulary in Down's syndrome children. In.'Disorders of Auditory Function lll' Eds Taylor,IG.,Markides,A. Academic Press. 6. Evenhuis,H.M. (1996) Dutch consensus on diagnosis and treatment of hearing impairment in children and adults with intellectual disability. J.Intel. Disabil. Res. 40(1) 451-456 7. Evenhuis,HM.,van Zanten,GA.,Brocnar,MP.,Roerdinkholder,WHM.(1992) Hearing loss in middle- age persons with Down syndrome. Am.J.on Mental Retardation.97.47-56. --------------------------------------------------------------------------------------------------------------------------------------------------------------------------- DSMIG (UK) 2001 www.dsmig.org.uk 15.01.02 Hearing 7 22.09.00 8. Hall,DMB.,(1996) Screening for hearing defects. Health for All Children, Oxford Univ. Press. 3rd Edition : 146-162. 9. Iino,Y.,Imamura,Y.,Harigai,S.,Tanaka,Y. (1999) Efficacy of tympanostomy tube insertion for otitis media with effusion in children with Down syndrome. Int. J.of Ped. Otorhinolaryngology. 49(2) 143-149 10. Kaplan,DJ.,Fleshman,JK.,Bender,TR.,Baum,C.,Clark,PS.(1973) Long term effects of otitis media. A ten year cohort of Alaskan Eskimo Children. Pediatrics.52.577-585 11. Keiser,H.,Montague,J.,Wold,D.,Maune,S.,Pattison,D.(1981) Hearing loss of Down's syndrome adults. Amer.J.Ment. deficiency.85 467-472 12. Libb.JW. et al.(1985) Hearing disorder and cognitive function of individuals with Down syndrome. Am.J of Mental Deficiency.90. 353-6 13. Menyuk,P.,(1979) Design factors in the assessment of language development in children with otitis media. Annals of Otology, Rhinology & Laryngology - supplement. 88 (5 Pt. 2 Suppl 60) : 78-87 14. National Deaf Children's Society (1994) Quality standards in Paediatric audiology. Volume 1. Guidelines for early identification of hearing impairment. ISBN 0904691 36 5 15. NICE.(2000) Referral practice for persistent otitis media with effusion in young children. NICE referral practice. May 2000. Pub NICE. ISBN. 1-84257-020-X 16. Polnay,L.,Hull,D.,(1993) Hearing. Community Paediatrics, Churchill Livingston. 2nd Edition : 323-334. 17. Roizen,N.(1997) Hearing loss in children with Down's syndrome: a review. Down Syndrome Quarterly.2(4).1-4 18. Schwartz,DM.,Schwartz,RH.(1978) Acoustic impedance and otoscopic findings in young children with Down's syndrome. Arch Otolaryngol.104.652-656 19. Selikowitz,M. (1993) Short-term efficacy of tympanostomy tubes for secretory otitis media in children with Down's syndrome. Dev.Med and child. Neurol. 35 511-515 20. Sonsken,P.M. (1985) A developmental reappraisal of clinical tests of hearing for normal and handicapped children. Part 3.The handicapped child. Mat and Ch Health. June 1985. 170-175 21. Whiteman,BC.,Simpson,GB.,Compton,WC.(1986) Relationship of otitis media and language impairment of adolescents with Down's syndrome. Mental Retardation.24. (6) : 353-356. 22. Widen,JE.,Folsom,RC.,Thompson,G.,Wilson,WR.(1987) Auditory brainstem responses in young adults with Down syndrome. Am.J.Mental Deficiency.91.472-479 Enquiries to: Down's Syndrome Medical Information Services Children's Centre, City Hospital Campus, Nottingham NG5 1PB. UK Tel (0)115 962 7658. Ext 45667. (0)115 934 5502 (answerphone). Fax (0)115 962 7915 Email:info@dsmig.org.uk --------------------------------------------------------------------------------------------------------------------------------------------------------------------------- DSMIG (UK) 2001 www.dsmig.org.uk 15.01.02