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Evaluation and therapy outcome in children with auditory neuropathy spectrum disorder (ANSD)
18 April 2018 (online)
ANSD is a special form of sensorineural hearing loss in which the diagnosis and adequate therapy are quite challenging. The prevalence of an ANSD is reported to be about 10% in literature. Most common causes are genetic mutations, extreme prematurity as well as perinatal hyperbilirubinemia and hypoxia. The aim of this study was to describe the procedure and the results of confirming the diagnosis and to show the outcome of the different therapies.
25 children, who were diagnosed and treated at the ENT department Wuerzburg were examined. A free field or pure tone audiometry was performed in all children. In addition OAEs, an ABR, ASSR as well as an electrocochleography and a cMRI were done. Following suitable provision pedaudiological tests, a psychological developmental diagnostic and speech and language evaluation were done at regular intervals. OAEs could be recorded in all children initially. Most of the children had no responses in ABR. Long oscillating cochlear microphonics could be found in all children who received an electrocochleography. 8 children were provided with hearing aids and 17 received a CI. The functional gain with hearing aids was between 32 and 65dB, the one with CI within 32 to 50dB. In the Freiburgers' monosyllabic test, a speech discrimination level between 35 to 100% could be reached. Regarding the outcome of therapy in ANSD, only some case reports could be found in literature. Provision with a suitable hearing device depends on audiological results and on the speech and language development of the individual child as well as on accompanying disorders. Repeated audiological evaluations, interdisciplinary diagnostics and an intense hearing and speech therapy are indispensable for the adequate rehabilitation of this group of children.