Madrid 1997 World Down Syndrome Conference Neurological and Sensorial Therapies Workshops


Wisniewski, K.E., Kida, E.
Institute for Basic Research in Developmental Disabilities. 1050 Forest Hill Road, Staten Island NY 10314 (USA) Phone: 1718-4945202 Fax: 1718-6983803

We have studied, at the George A. Jervis Clinic of our institution, several hundred children with Down Syndrome (DS) and found that all have neurological abnormalities, e.g., different degrees of developmental delay, deficit in language and intellectual function, unusual personality, and occasionally, seizures, autism, and/or behavioral problems. The most characteristic neurological features of children with DS are mild to moderate mental retardation and impairment of fine and gross motor coordination. In addition, a universal feature of children with DS is central hypotonia, which manifests as decreased physiological reflexes (e.g., Moro, parachute) during early infancy, but tends to improve with age. Other findings may be present, e.g., ocular nystagmus, myopia, astigmatism alternating with esotropía, conductive and/or sensorineural hearing impairment, and rarely, complications associated with atlantoaxial subluxation, predisposing to upper cervical spinal cord injury.The neuropathological (brain) abnormalities that occur in individuals with DS are microcephaly (small brain) associated with abnormal brain shape, fewer neurons (20-50% less), reduced axons and dendritic ramifications, synaptic structural abnormalities, alterations of cortical lamination, especially II and IV, and myelilnation delay. These abnormalities start during early gestation and may reflect genetically determined, altered brain programming, as suggested also by our recent data showing decreased immunoreactivity of developing DS brain to Bcl-2, a factor involved in nerve cell survival.

Hugo, R., Louw, B., Kritzinger, A.
Dpto. of Communicat. Paathology. Univ. Of Pretoria, Pretoria 0002, (South Africa). Phone: 27-12-4202357 Fax: 27-12-4203517 E-mail:

Practical experience indicates a lack of clinical evaluation procedures for the evaluation of the listening behaviour of children with Down Syndrome. This is especially important because these children are at risk for developmental communication delays concomitant to the high prevalence of recurrent Otitis Media and resultant auditory processing disorders. The aim of this study was to develop and apply an evaluation procedure for listening behaviour to 10 children with Down syndrome. Thereafter this scale was evaluated in terms of it's usefulness to a specific population of infants who are at risk for delay in communication disorders by applying it to 56 children (younger than 3 years) attending an early intervention programme. It was found that:- the listening scale can be used to differentiate between good and bad listeners;- there was a good correlation between the two situations used for the application of the evaluation scale;- that the children with Down syndrome are the second worst performers (compared to other high-risk aetiologies like children with cleft-lip and palate, low birth weight or neurological impairment) in listening behaviour. Subsequently this scale was applied in the clinical situation to 32 children with Downs Syndrome and evaluated as being a useful practical instrument for the assessment of listening behaviour.
Hearing and its implication in Down Syndrome children's speech and language

Limongi,S.C.O., Carvallo, R.M.M., Mustacchi, Z., Souza, E.R.
Rua Paraguaçu 479 apto 22, Perdizes, Sao Paulo (Brasil) CEP 05006-010 Phone: 55-11-884.19.44 Fax: 55-11-815.0589

Introduction. The sensory neural and/or conductive auditory impairment occurrence has been described in cognitive impairments syndromes (Webb, Kinde, Weber & Beedlr, 1966; Lloyd & Reid, 1967; Fulton & Lloyd, 1975). The auditory impairments associated with conductive disturbances in subjects with Down's Syndrome, mainly serous otitis media, was reported by many investigators (Fulton & Lloyd, 1968; Brooks, Wooley & Kanjilal, 1972; Balkany, Downs, Jafek & Krajicek, 1979).Down's Syndrome children can present oral communication development bellow that expected for their cognitive level. It is known that there is a high prevalence of middle eair eimpairments that affects the hearing of these children, in degrees varying from mild to moderate hearing loss . This loss repercussion in Central Auditory Processing abilities performance, such as attention, memory, localisation, recognition and discrimination, can affect these children more because of the associated cognitive disadvantage. Objective The aim of this research was to verify the Down's Syndrome children performance in Central Auditory Processing tests involving attention, verbal sequential memory and sound localisation, independent of their auditory condition. Methods. The audiological tests battery has been applied in fourteen Down's Syndrome children, aged between 5 and 11 years old. All of them have been attended at Child Hospital Darcy Vargas by: 1) genetic and paediatric clinic; 2) immitance audiometry, pure tone audiometry and speech tests, and auditory abilities screening, by Central Auditory Processing Assessment Protocol of Ambulatory Program of Syndromes and Sensory-Motor Disabilities and Clinical Audiology Program, both of the Speech-Language Course of Medicine Faculty of University of São Paulo. Results The results have revealed hearing pure tone average (PTA) less than 25 dBHL for 7 children and between 25 and 40 dBHL for 7 others; normal immitance results on 4 children and abnormal on 10. Aural-palpebral reflex present in both sides on 12 children and absent on 2 children ;adequate localisation answers to 5 direction on 4 children and abnormal on 10; verbal sequential memory abnormal on 13 children (one of them has not been tested). Conclusion The high prevalence of alterated auditory responses on Down's Syndrome children shows the importance of audiological attendance for these subjects. The knowledge about the hearing level and about the possible disorders in Central Auditory Processing abilities gives important additional information to the speech-language therapist to improve their planning and therapeutic process in language.To consider the Down's Syndrome children and the variable inherent alterations to the pathology, mainly the cognitive disadvantage, it is more clearly established the importance to reveal the audiological and auditory processing data to understanding the communicative manifestations and to improve the therapeutic work to be developed.

López, C.
C/ Puentesuela, 32 2° Drcha 18002 Granada (España) Phone: 958-26.27.88

El padecimiento de problemas audiológicos en pacientes con Síndrome de Down origina graves deficiencias en la adquisición del lenguaje. Si añadimos a ambos factores el característico retraso psicomotor del paciente, obtendremos como resultado la presencia de serias dificultades en el proceso de aprendizaje. En este estudio presentamos la experiencia realizada sobre nuestro grupo de pacientes, afectados por el Síndrome de Down a los que se les estudió, mediante otoscopia, timpanometría, audiometría, y Potenciales Evocados auditivos del Tronco, la posible existencia de patología audiológica, y se valoró su umbral auditivo.
Se estudiaron 85 pacientes con edades que oscilaban entre los 3 meses y los 23 años. De ellos, 53 varones (62,35%) y 32 mujeres (37,65%). A todos ellos se les realizó previamente una historia clínica de O.R.L. incluyendo los factores de riesgo pre y postnatales, una otoscopia bajo control microscópico, y una audiometría. Dependiendo de la edad y colaboración de cada uno, se realizó también una timpanometría al 50,58% una audiometría tonal al 12.94% una audiometría conductual 20% y potenciales auditivos del tronco al 67,05%.
Anamnesis; Encontramos pacientes con factores de riesgo prenatales en 2,35%; con factores de riesgo postnatales en 5,88%; retraso en la adquisición del lenguaje, 100%; antecedentes de patología inflamatoria aguda de otitis media:28,23%; alteraciones en la otoscopia; 21,11%. En la impedanciometría encontramos timpanometrías anormales 69,76%. En la valoración del umbral auditivo mediante Audiometría tonal convencional o conductual encontramos un 32,94% de pérdida, que oscilaban entre 30 y 70 dB, con una media de 45 dB y una desviación standard de 11,785. En los niños en que con la audiometría conductual no obtengamos resultados fiables se realizaron Potenciales evocados auditivos del tronco (en 57 niños) mostrando un aumento en los umbrales 40,35% de los casos con pérdidas. De las cuales encontramos una leve (1,75%), 20 moderadas (35,08%) y dos severas (3,50%).
Hemos detectado un elevado número de problemas auditivos en estos pacientes siendo las más frecuentes las otitis serosas y las hipoacusias: leves (4,70%), moderadas (30,50%) y severas (2,35%). Los estudios realizados nos permiten afirmar que las hipoacusias profundas no aparecen en ningún caso de forma estable, sólo en dos casos observamos laberintizaciones de su problema inflamatorio de oído medio que desaparecieron en el tratamiento.
Revised: January 4, 2001.