Abstract
Purpose: To record language and auditory skills development before and after cochlear implantation (CI) in children with Auditory neuropathy spectrum disorder (ANSD), and to determine the outcome after cochlear implant in patients with ANSD in comparison to patients with sensorineural hearing loss (SNHL). Materials and methods: Cases Included in this study were divided into two groups. Group I: includes, 13 children diagnosed with ANSD, of them 7 cases were subjected to CI. Group II: includes, 20 cases of SNHL, of them 10 patients were subjected to cochlear implant. For all cases language therapy was given regularly for 6 months pre-operatively and 6 months post-operatively. Auditory Skills Checklist (ASC) and The Arabic language test (receptive, expressive and total language Quotients) were used to monitor the progress concerning auditory skills and language development. Results: There was significant improvement in SNHL group and ANSD group after cochlear implantation regarding auditory skills (AS) and language development and almost the same outcome was obtained in both groups. Conclusion: Cases with ANSD improved markedly after cochlear implantation and No differences were noticed in outcome between SNHL & ANSD groups.
Highlights
Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder characterized by normal functioning of outer hair cells, evidenced by intact cochlear microphonic (CM) potentials and Otoacoustic emissions (OAEs) with absent or severely desynchronized auditory brainstem response (ABRs) [1]
In this study we found no benefit of H.As. and amplification for cases with ANSD, this coincide with the conclusion reached by Shallop et al [11] who reported limited success with H-A, FM systems and tactile aids, as the problem of neural desynchrony is not addressed and even lead to a detrimental effect
The results obtained because all candidates were young children and the lesion was mostly presynaptic, the inner hair cells within the synapses were the site of lesion and most of auditory nerve fibers are intact
Summary
Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder characterized by normal functioning of outer hair cells, evidenced by intact cochlear microphonic (CM) potentials and Otoacoustic emissions (OAEs) with absent or severely desynchronized auditory brainstem response (ABRs) [1]. It is much more common than most investigators initially thought. Current incidence of ANSD is 0.5% to 1.3% of patients attending the audiology clinics [2]. They account about 12% to 14% of patients having absent ABR. Hyperbilirubinemia, kernicterus and anoxia are possible factors [4]
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