Endocrinologic and metabolic complications of Alagille syndrome]

BACKGROUND: First language acquisition depends on intermodal perception, especially auditive, tactile-kinesthetic, and partly visual in addition to sensomotoric integration. The influence of the vestibular function for a physiological language development is still unknown. PATIENT: A case history of a child with bilateral aplasia of all semicircular canals, normacusis in the right ear, and severe sensorineural hearing loss in the left (hearing aid supplied), without mental deficiency, allows us to draw initial conclusions. RESULTS: Logopedic diagnostics revealed only a mild articulation disorder (interdental sigmatism, inconstant gammacism) as a possible consequence of recurrent middle ear effusions since early childhood. Language development diagnostics did not demonstrate any significant norm deviation. CONCLUSIONS: In contrast to the statomotoric disorder, no evidence for a specific language impairment was found. Our report elucidates the importance of a selected diagnostic imaging (spiral CT and MIP MRI) in the phoniatric-ped-audiological field.