RÉSUMÉ
Outline the hearing and cognitive profile of children with Down syndrome in relation to speech and language aptitudes in order to recognize other underlying etiological factors of communicative disability beside mental handicap that would improve the rehabilitation of those children. Twenty children with Down syndrome and 20 age and sex-matched normal subjects were subjected to comprehensive audiological evaluation including pure tone audiometry, tympanometry, Auditory brainstem response [ABR], and Event-related potentials [ERPs] in addition to vocal tract examination, and communicative and psychometric evaluation. Conductive hearing loss was detected in 5 [25%] as a complication of recurrent otitis media and repeated insertion of ventilation tubes. ABR wave V detection thresholds were compatible with behavioural pure tone audiometry. Shortened absolute latency of waves I, III and V as well as interpeak latency of I-III, III-V and I-V was observed in 10 patients [50%]. In 8 patients [40%] ERPs showed delay in latency of N[1], P[2], N[2] and P[3] components. In comparison to normal subjects, all patients showed variable degrees of subnormal mentality and social immaturity [p<0.001] as well as delayed language [language age] development [p<0.001]. Auditory dysfunction in patients with Down syndrome can occur at many levels either middle ear, brainstem and higher cortical cognitive progressing. Such findings may contribute to lower functioning of those patients in cognitive, linguistic and social aptitudes. Early audiological screening specific management and well-tailored rehabilitation strategy may allow improved prognosis and good quality of life in patients with Down syndrome