Forschen Sci Open HUB for Scientific Research Pediatrics and Neonatal Nursing: Open Access Open Access Copyright: © 2015 Sinha VR, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Volume: 1.2 Research Article Cochlear Implants and Auditory Neuropathy Spectrum Disorder VR Sinha 1 , Ruchika Mittal 1 *, Ajith Nilkanthan 1 , Awadhesh Mishra 1 , DK Singh 1 , AV Ramesh 2 , Satish Nair 3 , Poonam Raj 1 1 Army Hospital (R&R) Delhi Cantt – 110010, India 2 Command Hospital, Lucknow, India 3 Command Hospital, Kolkatta, India Received date: 24 August 2015; Accepted date: 17 October 2015; Published date: 25 October 2015 Citation: Sinha VR, Mittal R, Nilkanthan A, Mishra A, Singh DK, et al. (2015) Cochlear Implants and Auditory Neuropathy Spectrum Disorder. Pediatr Neonatal Nurs 1(2): doi http://dx.doi.org/10.16966/pnnoa.105 Copyright: © 2015 Sinha VR, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. * Corresponding author: Dr. Ruchika Mittal, M-37C Rajouri Garden, New Delhi 110027, India, Tel: +919810961266; E-mail: ruchikamittal07@gmail.com Abstract Objective: The results of cochlear implantation in patients that present with auditory neuropathy spectrum disorder (ANSD) patients have been variably reported. The effectiveness of the treatment modality varies in such patients. Hearing rehabilitation in this group of patients has been very challenging. Considerable controversy exists whether to provide conventional ampliication (hearing aids or personal FM systems) or cochlear implants (CI) to children with ANSD. With this background, we present our experience with select subset of ANSD cases that derived beneit from cochlear implant. Methodology: We studied and evaluated all the paediatric cases (i.e. less than 12 years of age) using audiological test battery. The cases with other co-morbidities, abnormality of auditory nerve and cochlea were excluded. Diagnosed cases of ANSD were given bilateral, behind the ear, digital hearing aids as per their behavioural responses for 6 months and enrolled for communication development in the auditory verbal habilitation program. They were evaluated for auditory perception using Category of Auditory Performance scoring system. The ANSD cases that derived “intermediate beneit” as per our criteria at 6 months post hearing aids were taken up for unilateral cochlear implant. All the implanted ANSD cases continued in auditory verbal habilitation program of our hospital and their progress on auditory perception post cochlear implant was monitored using CAP scoring system. Results: A total of 1313 cases were evaluated for hearing loss. Out of these 65 cases were detected to have ANSD (42 bilateral and 23 unilateral ANSD).Unilateral ANSD cases were excluded from the study. Hearing aids were itted in all the bilateral ANSD cases. After 6 months of hearing aid itting thirteen ANSD cases showed “intermediate beneit” and were taken up for cochlear implant. After 6 months of implant usage all ANSD cases showed “good” progress on CAP. Conclusion: Hearing aid trial should be given to all the cases diagnosed with ANSD and those who derive “intermediate beneit” from hearing aids and AVT should be considered for cochlear implant. Introduction Auditory Neuropathy Spectrum Disorder (ANSD) is a pattern of hearing loss characterized by preservation of outer hair cell function despite absent brainstem auditory evoked responses. Intact outer hair cell function is demonstrated by the presence of Otoacoustic Emissions (OAEs) and / or measurable Cochlear Microphonics (CM) whereas no synchronous neural activity is seen on acoustically evoked auditory evoked brainstem response testing [1]. he underlying cause may be congenital or acquired. Congenital causes include genetic abnormalities, perinatal asphyxia, orhyperbilirubinaemia. Acquired causes include infections, demyelination disorders and vascular causes. Postulated sites of lesion in ANSD include selected outer hair cells, inner hair cells, the synapse between hair cells and the auditory nerve, neural dendrite/axon, myelin sheath, or spiral ganglion cells [2,3]. ANSD was initially believed to be very rare. However, various studies have shown that ANSD is much more frequent than previously believed, with its prevalence estimated to range from 0.5% to 15% [4] and incidence ranging from 2.4% to 14-15% in children with severe to profound sensori-neural hearing loss [1]. herefore, dealing with hearing loss in ANSD is important. he efectiveness of the treatment modality varies on an individual basis. Hearing rehabilitation in this group of patients has been very challenging. Hearing aids, frequency modulation (FM) system and cochlear implantation are current treatment options for AN/AD, although the beneits of these interventions are variable and under much investigation [5]. Considerable controversy exists whether to provide conventional ampliication (hearing aids or personal FM systems) to children with ANSD. About 50% of the children in one study gained signiicant beneit [6], although some clinics report much lower success rates [7]. Moreover, cochlear implants (CI) in ANSD are also a debatable issue. If the site of the lesion is the cochlea, then bypassing the inner hair cells with direct stimulation of the vestibulocochlear nerve should produce good results. However, if the pathological condition lies in the nerve itself as in ANSD, then electrical stimulation (cochlear implants) might be expected to be subject to the same limitations as acoustic stimulation (hearing aids) [8]. hus it may be logically inferred that those ANSD cases that get some beneit from hearing aids would beneit better from cochlear implants, as it suggest that the nerve is at least partially functional. With this background we planned to evaluate the hearing outcomes in those ANSD cases that derived some beneit from hearing aids and subsequently underwent implantation. Methodology We studied all the paediatric cases (i.e. less than 12 years of age) referred to our tertiary care hospital’s outpatient department of ENT for hearing evaluation from March 2008 to March 2013. All the cases were evaluated using audiological test battery consisting of Behavioural Observation Audiometry (BOA) / Pure Tone Audiometry (PTA), Impedance Audiometry, Diagnostic Otoacoustic Emissions and click evoked Brain Stem Evoked Response Audiometry (BERA). BERA with both