The Patients selected according to audiological evaluation (ABR- OAEs and Cochlear microphonic). This study was conducted on children presented to ENT Center, King Fahd Hospital, Jeddah, from the period of April, 2010 to April, 2011 complaining of hearing impairment and delayed language development.Ģ. To compare the outcome after CI in points with AN Vs Pts. To explore development of auditory skills and language development before and after cochlear implantation in children with AN.Ģ. They also mentioned that hearing aids, FM systems, tactile aids are seldom of benefit.ġ. Poor speech discrimination, absent or severely abnormal ABR may also be encountered. reported that the patients may show varied SNHL mild to severe and usually bilateral. Patients with AN may show poor temporal encoding and degradation of speech perception, ranging from limited to no open-set discrimination. (3) Auditory nerve axons (demyelinated, axonal degeneration). ġ) Inner hair cells (IHC) and auditory nerve dendrites.Ģ) Spiral ganglion, synaptic junction between IHC and auditory nerve. Hyperbilirubinemia, kernicterus and anoxia are possible factors. ANSD may be caused by Hereditary, metabolic, toxic or inflammatory conditions. The vast majority of the patients have absent ABR and Wave I is always absent, CM is Present in all cases of ANSD, OAEs may miss up to 50% of cases. They account about 12% to 14% of patients having absent ABR. Current incidence of ANSD is 0.5% to 1.3% of patients attending the audiology clinics. It is much more common than most investigators initially thought. 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.Ĭonclusion: Cases with ANSD improved markedly after cochlear implantation and No differences were noticed in outcome between SNHL & ANSD groups.Īuditory 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). 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. For all cases language therapy was given regularly for 6 months pre-operatively and 6 months post-operatively. Group II: includes, 20 cases of SNHL, of them 10 patients were subjected to cochlear implant. Group I: includes, 13 children diagnosed with ANSD, of them 7 cases were subjected to CI. Materials and methods: Cases Included in this study were divided into two groups. 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).
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