Analysis of the Influence of Sound Source Position and Electrode Placement on the Stimulation Pattern of the Cochlear Implant under Noise Conditions: Optimization of Auditory Perceptual Effects

Background: Cochlear implants (CI) are widely used to restore hearing in people with severe to profound hearing loss. However, optimizing CI performance, especially in difficult listening environments with background noise, remains a major challenge. Understanding the influence of factors such as sound source position and electrode placement on CI stimulation patterns is critical to improving auditory perception. Methods: In this study, an analysis was conducted to investigate the influence of sound source position and electrode placement on CI stimulation patterns under noisy conditions. For this purpose, a special measurement setup with a CI speech processor-microphone test box was used to simulate realistic listening scenarios and measure CI performance. Results: The results show that the effectiveness of CI noise reduction systems is influenced by factors such as the position of the sound source and electrode placement. In particular, the beamforming ultra zoom mode showed significantly better noise reduction than the omnidirectional mode, especially under real listening conditions. Furthermore, differences in electrode responses indicate individual variability in the CI user experience, highlighting the importance of personalized fitting algorithms. Conclusions: The results demonstrate the importance of considering environmental factors and individual differences when optimizing CI performance. Future research efforts should focus on the development of personalized fitting algorithms and the exploration of innovative strategies, such as the integration of artificial intelligence, to improve CI functionality in different listening environments. This study contributes to our understanding of CI stimulation patterns and lays the foundation for improving auditory perception in CI users.

The role of cochlear implantation in alleviating Tumarkin drop attacks of Meniere's disease;a case report

Ménière’s disease(MD)patients may suffer episodes of sudden falls,named Tu markin drop attacks(DAs).This fall occurs abruptly and without warning or loss of consciousness.DAs usually aggravate the clinical picture of MD and are challenging to manage.The present report describes a case treated by cochlear implantation(CI)due to concomitant deafness and offers some clinical considerations for this condition.A male patient aged 48 years with a 10-year history of definite bilateral MD had profound SNHL on the right and severe SNHL on the left side.He suffered from intermittent attacks of vertigo,ear fullness,and tinnitus and,in the last year,had developed DAs and experienced 14 episodes in the previous six months.The preoperative category of acoustic performance was 3.The Dizziness Handicap Inventory(DHI)questionnaire showed a total score of 46,which indicated a moderate degree of disability.A CI was planned for the right side.The patient did not report any further DAs episode for two years since then.The postoperative category of acoustic performance became 11,and the postoperative DHI questionnaire showed a decrease in the total score(from 46 to 19),which indicated a mild disability.Unilateral CI effectively alleviated the DAs associated with bilateral MD.Our report proposes a new modality for managing vertiginous symptoms in cases of MD with hearing loss without the need for more aggressive surgical interventions with the need for clinical trials to confirm our results.

Long-term complications of the transmeatal approach (Open Transcanal) in cochlear implants: A follow-up study

Objective: Multiple alternative approaches of cochlear implant surgery have been described, such as the suprameatal approach, transcanal approach, transmeatal approach and middle cranial fossa approach.Transmeatal(open trnascanal) approach has not been adapted since first described in the clinical field.we aimed to assess the long-term complications of the transmeatal approach in a series of 131 patients at our center between 2004 and 2008.Methods: This study was a retrospective case series of all patients who underwent cochlear implants with the transmeatal(open transcanal) approach from May 2004 to December 2008 at King Faisal Specialist and Research Hospital(Riyadh, Saudi Arabia), which were conducted by the same surgeon.Results: Complications were observed often with various combinations-recurrent otitis externa, posterior tympanic membrane perforation, electrode extrusion, cholesteatoma, and chronic mastoiditis. The overall long-term complication rate was 16%(21/131). The gap between the implantation and the diagnosis of a complication ranged from <1 year to 11 years. Major complications were as follows:cholesteatoma in 5(3.8%) patients, extrusion of the electrode in 5(3.8%) patients, and tympanic membrane perforation or deep retractions in 5(3.8%) patients. Minor complications were as follows: recurrent mastoiditis with/without concomitant temporary facial nerve palsy in 4(3%) patients, recurrent otitis externa infections in 7(5%) patients, and weakness of the posterior canal wall in 1 patient.Conclusion: The transmeatal approach posed an high rate of complications on long-term follow-up such as cholestetoma formation, extrusion of electrode or perielectrode reaction formation to tympanic membrane and external auditory canal.

Comparing accuracy of cochlear measurements on magnetic resonance imaging and computed tomography:A step towards radiation-free cochlear implantation

Objective:Computed Tomography(CT)and Magnetic Resonance Imaging(MRI)are commonly employed in pre-operative evaluation for cochlear implant surgery.However,with a decrease in the age of implantation,even minor radiation exposure can cause detrimental effects on children over their lifetime.The current study compares different cochlear measurements from CT and MRI scans and evaluates the feasibility of using only an MRI scan for radiological evaluation before cochlear implantation.Methods:A longitudinal observational study was conducted on 94 ears/47 children,employing CT and MRI scans.The CT and MRI scan measurements include,A value,B value,Cochlear duct length(CDL),twoturn cochlear length,alpha and beta angles to look for cochlear orientation.Cochlear nerve diameter was measured using MRI.The values were compared.Results:The mean difference between measurements from CT and MRI scans for A value,B value,CDL,and two-turn cochlear length values was 0.567±0.413 mm,0.406±0.368 mm,2.365±1.675 mm,and 2.063±1.477 mm respectively without any significant difference.The alpha and beta angle measures were comparable,with no statistically significant difference.Conclusion:The study suggests that MRI scans can be the only radiological investigation needed with no radiation risk and reduces the cost of cochlear implant program in the paediatric population.There is no significant difference between the measurements obtained from CT and MRI scans.However,observed discrepancies in cochlear measurements across different populations require regionally or race-specific standardized values to ensure accurate diagnosis and precision in cochlear implant surgery.This aspect must be addressed to ensure positive outcomes for patients.

Stages of Emotional Experience of Parents of Children with Cochlear Implantation (CI)

Rehabilitation is a set of measures aimed at compensating or fully restoring the functions of the patient impaired by the disease. In the rehabilitation of children with a cochlear implant, a comprehensive, systematic approach is essential in the work of doctors, teachers, sign language teachers, psychologists, and families. Rehabilitation and social adaptation of children with a cochlear implant depend not only on specialists but also on the ability of parents to help the child organize educational activities in an optimistic mood and the ability to provide emotional support. This means that the role of parents in the way to successful rehabilitation is high, and therefore this topic is especially relevant today. .

Barriers to Early Pediatric Cochlear Implantation in Kenya

Background: Cochlear implantation is the best management option for children with profound hearing loss and has received no benefit from hearing aids. Early implantation for these children is associated with good speech and language outcomes. Objectives: To determine the barriers to early pediatric cochlear implantation. Methodology: A qualitative cross-sectional study was conducted at Hearing Implants Centre in Nairobi Kenya from August 2022 to February 2023. The target population was 40 children who had undergone cochlear implantation under the auspices of Cochlear Implant Group of Kenya but data was only collected from 30 of them. The remaining were ruled out because 3 were unreachable over the phone, 5 refused to participate and 2 did not meet the inclusion criteria. Results: Patient file reviews and parental telephone interviews were conducted to collect information and analyzed using Microsoft excel and presented using graphs, tables and pie charts. The analysis of the gender showed 46.67% were male and 53.33% were female. Analysis on newborn screening showed that none had it done. The mode age of hearing loss suspicion was between the ages of 2 - 3 years. The hearing loss suspicion done was done by the mothers at 20 children the reminder being 3 by the father, 1 by a family friend, 4 by the school-teacher and 2 by the child’s grandmother. A total of 17 participants noted a delayed in speech and language, 9 noted that the child did not respond to loud sounds, 4 noted that the children did not turn when called. Once hearing loss was identified, 73% saw the ENT, 17% saw a pediatrician, 7% went to see an Audiologist, and 3% saw a speech therapist. The mode age at diagnosis was 1.5 years. The mode age at implantation was 5 years. The mode time from diagnosis was 2 years. Conclusions: This study sought to investigate the barriers to pediatric cochlear implantation in Kenya. From the results it was determined that factors such as lack of newborn screening, high cost of cochlear implantation, lack of awareness have led to late cochlear implantation.

Preliminary Results of Cochlear Implantation in Underdeveloped Countries: The Experience of Cameroon

Introduction: Deafness, is the most common neurosensory deficit in humans. The origins can be diverse: congenital or acquired and sometimes of an etiology that is difficult to specify. The main risk is social exclusion. The advent of cochlear implants is a solution of choice for severe to profound sensorineural hearing loss. This innovative therapeutic modality is new to Cameroon, so we proposed to evaluate the preliminary results of cochlear implantation at the General Hospitals of Yaoundé and Douala, by addressing the epidemiological, clinical and paraclinical, surgical, and prosthetic aspects. Methodology: We conducted a descriptive and prospective cross-sectional study over a period of two years and eight months, from January 2019 to 31 August 2021. The study sites were: the general hospitals of Yaounde and Douala, as well as the private practices of speech therapists in the said cities. We collected socio-demographic, clinical, paraclinical variables and data on surgical, prosthetic and speech therapy management which were processed. Results: We recruited 15 cochlear implant patients, one adult and 14 children. The sex ratio was 1.14 in favour of girls, the average age of the child population was 4.9 years and one subject was 57 years old. These children were mostly in school (85.7%) and mostly (86.7%) living in urban areas. The average period of sound deprivation was 3.9 years. The deafness of the children was 100% prelingual and the acquired cause was evoked in front of the risk factors (prematurity, low birth weight, neonatal asphyxia, jaundice, meningitis, neuromalaria) for 57.7% of them. The adult deafness was postlingual and post-traumatic. The associated clinical conditions found in 4 (26.8%) of the patients were an ocular refraction disorder, a chronic otitis media sequelae, cerebral palsy and minor head trauma injuries. There was no syndromic or malformative picture. The deafness was bilateral in all cases, asymmetric in 22.2% of cases and severe to profound sensorineural. The threshold of the deafness was deep in 78.6% of cases, with a more marked involvement on the right. Imaging studies (MRI and CT scans of the cranium, brain and rock) carried out in our series showed abnormalities in 4 (26.7%) of the children, but none of these abnormalities were an absolute contraindication to implantation. The surgical management was done with oticon® Neuro ZTI implants. Implantations were unilateral and mostly right, with one case of stenosis of the round window recess observed. The postoperative course was simple for 92.8% of patients. One case of superinfection of the surgical wound. The activations were performed within four to five weeks after surgery and the implant was functional in fourteen patients and dysfunction was observed in one patient. Conclusion: The cochlear implant is an effective solution in the fight against severe to profound sensorineural deafness. The diffusion of this therapeutic tool in our environment is still hampered by the youth of the teams, the lack of equipment and the insufficient financial means.

Usher syndrome:Genetic diagnosis and current therapeutic approaches

Usher Syndrome(USH)is the most common deaf-blind syndrome,affecting approximately 1 in 6000 people in the deaf population.This genetic condition is characterized by a combination of hearing loss(HL),retinitis pigmentosa,and,in some cases,vestibular areflexia.Among the subtypes of USH,USH type 1 is considered the most severe form,presenting profound bilateral congenital deafness,vestibular areflexia,and early onset RP.USH type 2 is the most common form,exhibiting congenital moderate to severe HL for low frequencies and severe to profound HL for high frequencies.Conversely,type 3 is the rarest,initially manifesting mild symptoms during childhood that become more prominent in the first decades of life.The dual impact of USH on both visual and auditory senses significantly impairs patients'quality of life,restricting their daily activities and interactions with society.To date,9 genes have been confirmed so far for USH:MYO7A,USH1C,CDH23,PCDH15,USH1G,USH2A,ADGRV1,WHRN and CLRN1.These genes are inherited in an autosomal recessive manner and encode proteins expressed in the inner ear and retina,leading to functional loss.Although non-genetic methods can assist in patient triage and disease extension evaluation,genetic and molecular tests play a pivotal role in providing genetic counseling,enabling appropriate gene therapy,and facilitating timely cochlear implantation(CI).The CRISPR/Cas9 system and viral-based gene replacement therapy have recently emerged as highly promising techniques for treating USH.Regarding drug therapy,PTC-124 and Nb54 have been identified as promising drug interventions for genetic HL in USH.Simultaneously,CI has proven to be critical in the restoration of hearing.This review aims to summarize the genetic and molecular diagnosis of USH and highlight the importance of early diagnosis in Cuzzuol BR et al.Diagnosis and current treatments of USH WJO https://www.wjgnet.com 2 January 19,2024 Volume 11 Issue 1 guiding appropriate treatment strategies and improving patient prognosis.

Safety and outcomes of cochlear implantation in the elderly:A review of recent literature

Global demographic changes related to longevity are leading to increasing numbers of the elderly, for whom hearing loss is a significant cause of morbidity and disability. Once met with reticence, severely hearing impaired older adults are increasingly being considered for cochlear implantation (CI). Significant data indicate that CI in the elderly population is safe, well-tolerated, and effective. Risks from CI surgery and anesthesia are low and generally comparable to rates in other age groups. Outcomes studies regarding CI in older adults have shown excellent improvements to speech perception, quality of life, and even cognition. Overall, currently available data suggests that advanced age should not, in itself, be considered a barrier to implantation. This review paper will highlight selected articles from recent medical literature regarding the safety and efficacy of CI in the elderly population. Copyright ? 2016, PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

After cochlear implantation: Complications related to flap around implants

Objective: To report complications related to flap around implants after cochlear implantation, possible causes of such complications and treatments. Methods and material:We performed a retrospective analysis of children in whom complications related to flap around implants occurred after undergoing cochlear implantation in our department from 2005 to 2016. Results:Complications among 1500 cochlear implantation (CI) recipients by the same surgeon included hematoma (n ? 20) and seroma around implants (n ? 15), of which most (n ? 10) recovered in 2 weeks after effective drainage, utility of antibiotics and pressure dressing, but 5 developed flap necrosis and had to undergo contralateral re-implantation. Four patients developed abscess around implants, of whom 2 recovered after 2 weeks of drainage, gentamicin irrigation and use of antibiotics, but 2 patients ended up with flap necrosis and had to receive contralateral reimplantation. Conclusions: Immediate drainage, pressure dressing and antibiotics can be used to effectively control seroma around implants. For seroma lasting for more than two weeks without improvement, surgical drainage may be need.

MUSIC AND COCHLEAR IMPLANTS

Currently, most people with modern multichannel cochlear implant systems can understand speech in qui-et environment very well. However, studies in recent decades reported a lack of satisfaction in music percep-tion with cochlear implants. This article reviews the literature on music ability of cochlear implant users by presenting a systematic outline of the capabilities and limitations of cochlear implant recipients with regard to their music perception as well as production. The review also evaluates the similarities and differences be-tween electric hearing and acoustic hearing regarding music perception. We summarize the research results in terms of the individual components of music (e.g., rhythm, pitch, and timbre). Finally, we briefly intro-duce the vocal singing of prelingually-deafened children with cochlear implants as evaluated by acoustic measures.

Speech intelligibility and auditory perception of pre-school children with Hearing Aid,cochlear implant and Typical Hearing

Purpose:There is a growing interest in speech intelligibility and audito ry perception of deaf children.The aim of the present study was to compare speech intelligibility and auditory perception of pre-school children with Hearing Aid(HA),Cochlear Implant(Cl),and Typical Hearing(TH).Methods:The research design was descriptive-analytic and comparative.The participants comprised 75 male pre-school children aged 4-6 years in the 2017-2018 from Tehran,Iran.The participants were divided into three groups,and each group consisted of 25 children.The first and second groups were respectively selected from pre-school children with HA and CI using the convenience sampling method,while the third group was selected from pre-school children with TH by random sampling method.All children completed Speech Intelligibility Rating and Catego ries of Auditory Performance Questionnaires.Results:The findings indicated that the mean scores of speech intelligibility and auditory perception of the group with TH were significantly higher than those of the other groups(P<0.0001).The mean scores of speech intelligibility in the group with CI did not significantly differ from those of the group with HA(P<0.38).Also,the mean scores of auditory perception in the group with CI were significantly higher than those of the group with HA(P<0.002).Conclusion:The results showed that auditory perception in children with CI was significantly higher than children with HA.This finding highlights the importance of cochlear implantation at a younger age and its significant impact on auditory perception in deaf children.

The history of Cochlear^(TM) Nucleus~ sound processor upgrades:30 years and counting

Objective:To review developments in sound processors over the past 30 years that have resulted in significant improvements in outcomes for Nucleus~ recipients.

Double challenge:cochlear implantation in the only hearing ear with progressive hearing loss following meningitis and vestibular dysfunction after implantation

Objective:Vestibular dysfunction associated with cochlear implantation is rare.It is usually seen in patients with otosclerosis due to spread of electrical activity throughout the demineralized bone.A 17-year old female with progressive hearing loss 2 years after meningitis and vestibular dysfunction in the implanted ear is presented in this study.Findings:The patient had mild hearing loss in the right ear and total hearing loss on the left side because of complete ossification of the cochlea following meningitis.She had to have cochlear implantation in the right ear because of progression of hearing loss.She had successful implantation but she experienced vestibular dysfunction following activation of cochlear electrodes.Closure of two electrodes caused disruption of auditory programming.Then the patient was subjected to long term vestibular rehabilitation program.Conclusion:Timing for implantation before the completion of cochlear ossification is crucial not to miss the chance for hearing restoration.However,difficulties in hearing rehabilitation due to extensive ossification can be doubled by vestibular problems triggered by stimulation of the vestibular nerve by cochlear electrodes.Attempts to reduce the balance problem will complicate auditory programming.Vestibular rehabilitation for long term helps to carry on hearing progress.

Cochlear implantation in a child with auditory neuropathy spectrum disorder

Objective To report outcomes of cochlear implantation (CI) in a child with auditory neuropathy spectrum disorder (ANSD) and to provide preliminary clinical evidence of the efficacy of CI in ANSD patients.Methods A 4-year-old boy with diagnosed auditory neuropathy spectrum disorder (ANSD) received implantation of a Nucleus CI24R after an unsatisfactory trial of amplification.Post-implantation performance in both hearing sensitivity and speech recognition was assessed in different sessions.Aided hearing thresholds were tested by behavioral audiometry.Mandarin Early Speech Perception Test (MESP),Meaningful Auditory Integration Scale (MAIS),category of auditory performance (CAP) and Speech Intelligibility Rating (SIR) were used to assess the benefits in auditory skills or speech recognition the boy obtained from CI.The tests were administered before surgery and at 3 months and 7 months after opening.Results The boy demonstrated improved auditory sensitivity by using CI.Concerning speech recognition and communication,both speech audiometry and questionnaires showed an obvious benefit from CI.Conclusions CI has worked efficiently in this ANSD boy.But because of limited understanding of ANSD and rehabilitation effect by cochlear implantation in this condition,the clinical decision to implant should be cautious and only after a thorough evaluation.Meanwhile,well controlled and long term studies are needed to confirm the efficacy of cochlear implantation in patients with ANSD.

Binaural integration abilities in bilateral cochlear implant user

Bilateral Cochlear implants (CIs) improved speech intelligibility, speech perception in background noise, and sound localization in quiet and noisy situations. However, it is unclear whether these advantages essentially result in binaural integration of acoustic stimuli from each ear. In this study, we investigated the effectiveness of binaural integration by bilateral CIs placement using binaural hearing tests and subjective auditory perceptual assessment. A 61-year-old bilateral CIs subject underwent the following four tests:the Japanese Hearing in Noise Test (HINT-J), the dichotic listening test (DLT), the Rapidly Alternating Speech Perception (RASP) test, and subjective auditory perceptual assessment. The HINT-J score was significantly higher for bilateral CIs than for a unilateral CI. However, DLT and the RASP test revealed contradictory results. Subjective auditory perceptual assessment revealed active and bright impressions for bilateral hearing, which were also noisy and strong compared with those for unilateral hearing. The results of this study revealed that bilateral CIs improved speech perception in background noise and an improved auditory impression, although the bilateral integration abilities were not improved. This was probably because the patient was required to combine information from the two ears into a single perception in DLT and the RASP test. More longitudinal data should be collected and analyzed in future studies to evaluate the long-term effects of bilateral CIs. Copyright ? 2016, PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

One-year experience with the Cochlear^(TM) Paediatric Implanted Recipient Observational Study(Cochlear P-IROS) in New Delhi, India

Background： Currently, there is a significant lack of data concerning long-term outcomes following paediatric cochlear implantation in terms of quality of life. There is a need for a long-term, prospective study in this regard. This study aims at highlighting the preliminary results, one year post surgery of a five year prospective study. Methods： The CochlearTM Paediatric Implanted Recipient Observational Study （P-IROS） is a prospective, patient outcomes registry for routinely implanted children. The study collects data using questionnaires post-surgery and at regular intervals up to five years. Results： At our Centre, 159 cochlear implant surgery procedures were carried out between January 2014 and December 2014. Category of Auditory Performance II score increased from ‘0＇ to ‘3＇ at six months and to ‘5＇ at 12 months for children aged 0-3 years, although this was not statistically significant. However, the same trend was statistically significant for the age 3-6 year and age 6-10 year brackets. The quality of life of the child improved significantly. Analysis of communication mode revealed a statistically significant overall shift to the auditory-oral mode from total communication. Conclusion： Cochlear implantation is a life-changing intervention. The evidence in support of what it can achieve safely is clear. However, the costs associated with it raise the question if it will remain an effective option for life in all children. The Cochlear P-IROS is an attempt to answer the same over a five year period. Our study in New Delhi, so far concludes that cochlear implantation in a population with limited access to funds is very effective, one year after surgery.

Effects of Delayed Second Cochlear Implant

Objective Since Helms’ successful bilateral cochlear implantation with good results in 1996, there have been increasing number of reports on bilateral cochlear implantation. Most second device have been implantated within one year after the first. Considering effects of long time auditory deprivation, it is not clear whether a delayed second cochlear implant serves to add additional benefits and how it may interact with central nervous system plasticity. Methods Three cases who received delayed second cochlear implants at People’s Hospital of Peking University from 2002 to 2005 were reviewed. The interval between the first and second implants was longer than 2 years in all three patients. Sound perception, and unilateral/bilateral speech discrimination in quiet and noise were evaluated. In addition, GAP detection test was conducted in one patient. Results In one case, having both implants on provided improved performance compared to using only one implant both in quiet and noise. Presumably due to visual interference from lip-reading or short interval between second implant and testing, one patient showed no improvement from using the second implant either in quiet or noise, while the last case demonstrated additional benefits from the second implant only in quiet. In all three patients, performance in recognizing the four tones in Mandarin was superior over word recognition. Conclusions Considerable plasticity in the cerebral auditory center is preserved, despite long acoustic deprivation in some children who have received unilateral cochlear implant. Delayed second implants can result in significant improvements in some of these children. Visual interference from lip-reading may be an obstacle during retraining. The better recognition of tones in the Mandarin language may represent a different sound discrimination mechanism in the auditory system, although it may also be related to the signal processing mechanisms of the implant used (MED-EL COMBI 40+).

Molecular mechanisms and roles of inflammatory responses on low-frequency residual hearing after cochlear implantation

Preservation of low-frequency residual hearing is very important for combined electro-acoustic stimulation after cochlear implantation.However,in clinical practice,loss of low-frequency residual hearing often occurs after cochlear implantation and its mechanisms remain unclear.Factors affecting lowfrequency residual hearing after cochlear implantation are one of the hot spots in current research.Inflammation induced by injury associated with cochlear implantation is deemed to be significant,as it may give rise to low-frequency residual hearing loss by interfering with the blood labyrinth barrier and neural synapses.Pathological changes along the pathway for low-frequency auditory signals transmission may include latent factors such as damage to neuroepithelial structures,synapses,stria vascularis and other ultrastructures.In this review,current research on mechanisms of low-frequency residual hearing loss after cochlear implantation and possible roles of inflammatory responses are summarized.

Communication outcomes following cochlear implantation in a child with cystic cochleovestibular anomaly

Cochlear implantation is one of the best amongst the various management options available for children and adults with severe to profound sensorineural hearing loss.Inner ear and internal auditory canal(IAC) malformations accounts to approximately 25% of congenital sensorineural hearing loss in children.The primary goal of this report was to evaluate the communication outcomes after cochlear implantation in a child with cystic cochleovestibular anomaly(CCVA).The child was evaluated through various standardized outcome measures at regular intervals to track the progress in terms of auditory and spoken language skills.The scores on Categories of Auditory Perception(CAP),Meaningful Auditory Integration Scale(MAIS),Speech Intelligibility Rating(SIR),Meaningful Use of Speech Scale(MUSS),and listening and spoken language skills showed a significant leap in 12 months duration post implantation.The report thus highlights and correlates the significant progress in auditory and spoken language skills of the child with congenital malformations to appropriate auditory rehabilitation and intensive parental training.