Background:Cochlear hair cell injury is a common pathological feature of hearing loss.The basic helix-loop-helix family,member e40(Bhlhe40),a gene belonging to the basic helix-loop-helix(bHLH)family,exhibits strong tr...Background:Cochlear hair cell injury is a common pathological feature of hearing loss.The basic helix-loop-helix family,member e40(Bhlhe40),a gene belonging to the basic helix-loop-helix(bHLH)family,exhibits strong transcriptional repression activity.Methods:Oxidative damage,in House Ear Institute-Organ of Corti 1(HEI-OC1)cells,was caused using hydrogen peroxide(H2O2).The Ad-Bhlhe40 particles were constructed to overexpress Bhlhe40 in HEI-OC1 cells.Various assays including cell counting kit-8(CCK-8),terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling assay(TUNEL),flow cytometry,immunofluorescence,and corresponding commercial kits were employed to investigate the impacts of Bhlhe40 on cell viability,apoptosis,oxidative stress levels,mitochondrial membrane potential and cellular senescence.Additionally,a dual-luciferase reporter assay was performed to confirm the targeting of the histone deacetylases 2(Hdac2)by Bhlhe40.Results:The results revealed that Bhlhe40 was downregulated in H_(2)O_(2)-treated HEI-OC1 cells,but its overexpression improved cell viability and mitigated H_(2)O_(2)-induced oxidative injury in HEI-OC1 cells with increase of superoxide dismutase(SOD),catalase(CAT)and glutathione peroxidase(GPx)activities and decrease of reactive oxygen species(ROS)levels.Besides,overexpression of Bhlhe40 suppressed H_(2)O_(2)-triggered cell senescence,as evidenced by the fact that the upregulation of P53,P21,and P16 in HEI-OC1 cells treated with H2O2 were all alleviated by Bhlhe40 overexpression.And we further verified that overexpression of Bhlhe40 could inhibit the expression of Hdac2,which may be related to the repression of Hdac2 transcription.Conclusion:This study suggests that Bhlhe40 plays a protective role against senescence and oxidative damage in cochlear hair cells exposed to H2O2.展开更多
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 ...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.展开更多
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...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. .展开更多
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(ope...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.展开更多
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 ...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.展开更多
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 radiatio...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.展开更多
Explaining the mechanism of the cochlear active phonosensitive amplification has been a major problem in medicine.The basilar membrane(BM)is the key infrastructure.In 1960,Nobel Laureate von B′ek′esy first discovere...Explaining the mechanism of the cochlear active phonosensitive amplification has been a major problem in medicine.The basilar membrane(BM)is the key infrastructure.In 1960,Nobel Laureate von B′ek′esy first discovered BM's traveling wave motion.Since that time,BM's models only have considered the traveling wave but not the biological activity.Therefore,a new model considering changes of BM's stiffness in space and time is established based on the immersed boundary method to describe its biological activity.It not only reproduces the results of traveling wave motion but also explains the mechanization on the generation of traveling wave.An important discovery is that changes of BM's stiffness in space and time will cause the unstable global resonance,which will induce amplification of sounds in cochlea.An important inference is that biological activity shall be included in the application of mechanical principles to the analysis of life,which is the essential difference between biomechanics and general mechanics.展开更多
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 spee...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.展开更多
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 exclusio...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<sup>®</sup> 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.展开更多
This paper proposes a cochlear prosthetic system with an implanted digital signal processor (DSP). This system transmits voice-band signals with a low data rate through the wireless link, free of the data-rate limit...This paper proposes a cochlear prosthetic system with an implanted digital signal processor (DSP). This system transmits voice-band signals with a low data rate through the wireless link, free of the data-rate limitation and suitable for future development. By optimizing the speech processing algorithm and the DSP hardware design, the implanted DSP manages to execute the continuous interleaved sampling (CIS) algorithm at a clock frequency of 3MHz and a power consumption of only 1.91mW. With an analytic power-transmission efficiency of the wireless inductive link (40%), the power overhead caused by the implanted DSP is derived as 2.87roW,which is trivial when compared with the power consumption of existing cochlear prosthetic systems (tens of milliwatts). With the DSP implanted,this new system can.be easily developed into a fully implanted cochlear prosthesis.展开更多
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 t...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.展开更多
Cisplatin belongs to platinum-based drugs and is widely used in cancer chemotherapy.Ototoxicity is one of the major dose limiting side-effects of cisplatin.For toxicity to occur cisplatin must first be transported fro...Cisplatin belongs to platinum-based drugs and is widely used in cancer chemotherapy.Ototoxicity is one of the major dose limiting side-effects of cisplatin.For toxicity to occur cisplatin must first be transported from the bloodstream into cochlear cells.Three copper transporters are considered pathways for regulating the uptake and translocation of cisplatin into cells:Ctr1,ATP7A and ATP7B.Our recent study with cochlear organotypic cultures shows that cochlear hair cells can be destroyed by cisplatin at low concentrations from 10μm to 100μn.However,high doses of cisplatin cannot damage hair cells,maybe due to intrinsic feedback reactions that increase export of platinum by ATP7B when the platinum concentration is high in extracellular space.Cimitidine is a specific copper transporter inhibitor that can block the entrance of copper and platinum,and may prevent cisplatin-induced cochlear hair cell injury.To evaluate this hypothesis,we treated cochlear organotypic cultures with cisplatin (10 μm or 50 μm) alone,or cisplatin combined with cimitidine at concentrations ranging from 10-2000 μm for 48 hours.cisplatin at 10 μm damaged about 20% hair cells.In contrast,when cimitidine (10 μm,100 μm and 2000 μm) was added to the culture,near 100% cochlear hair cell survived.At higher concentration (50 μm),cisplatin destroyed about 80% of cochlear hair cells.However,100 μmcimitidine rescued about 50% hair cells from cisplatin damage,and 2000μm cimitidine protected about 80% hair cells.The data of western blot showed that CTR1 and ATP7B expressions were increased in cisplatin treated cochlear tissue,but cimitidine significantly reduced CTR1 and ATP7B.In addition,ATP7A expression was depressed a little after cisplatin treatment.Considering that Ctr1 is involved in copper and platinum influx,but the ATP7A and ATP7B are copper export transporters,the results suggest that cimitidine can effectively block the entrance by copper transporters and stop the influx of cisplatin.展开更多
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 imp...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/).展开更多
Objectives:This study aimed to assess the effect of hearing and speech rehabilitation in patients with Nurotron■cochlear implants.Design:Ninety-eight paediatric patients with bilateral severe-to-profound sensorineura...Objectives:This study aimed to assess the effect of hearing and speech rehabilitation in patients with Nurotron■cochlear implants.Design:Ninety-eight paediatric patients with bilateral severe-to-profound sensorineural deafness who received cochlear implantation were divided into three groups according to age:group A(≤3 years),group B(4-7 years),and group C(8-16 years).All patients were followed up for one year for hearing and speech performance after the surgery.The comprehensive Auditory Perception Assessment,MAIS,CAP and SIR hearing and speech assessments and rating materials were used for assessment before the surgery and at 3,6,and 12 months after implant activation.Results:The scores of patients in the open-set speech assessment,Chinese Auditory Perception Assessment,MAIS,CAP and SIR significantly improved after cochlear implantation in all age groups.The younger the age at implantation,the better the results.Moreover,the hearing and speech performance of cochlear implant recipients gradually improved with the extension of rehabilitation time.Conclusions:Nurotron■Venus^TM cochlear implantation can improve the hearing and speech performance of patients with bilateral severe-to-profound sensorineural deafness.展开更多
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 child...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.展开更多
Objective: A common cavity deformity(CCD) is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity that results in profound hearing loss. There are few studie...Objective: A common cavity deformity(CCD) is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity that results in profound hearing loss. There are few studies paying attention to common cavity. Our group is engrossed in observing the improvement of auditory and verbal abilities in children who have received cochlear implantation(CI), and comparing these targets between children with common cavity and normal inner ear structure.Material and methods: A retrospective study was conducted in 12 patients with profound hearing loss that were divided into a common cavity group and a control group, six in each group matched in sex, age and time of implantation, based on inner ear structure. Categories of Auditory Performance(CAP) and speech intelligibility rating(SIR) scores and aided hearing thresholds were collected and compared between the two groups. All patients wore CI for more than 1 year at the Cochlear Center of Anhui Medical University from 2011 to 2015.Results: Postoperative CAP and SIR scores were higher than before operation in both groups(p < 0.05), although the scores were lower in the CCD group than in the control group(p < 0.05). The aided threshold was also lower in the control group than in the CCD group(p < 0.05).Conclusion: Even though audiological improvement in children with CCD was not as good as in those without CCD, CI provides benefits in auditory perception and communication skills in these children.展开更多
Tuberculous otitis media(TOM) is rare in ENT department,and is frequently misdiagnosed as otitis media.Thus early systemic treatment is very important for TOM.We reported a case report with TOM to highlight developmen...Tuberculous otitis media(TOM) is rare in ENT department,and is frequently misdiagnosed as otitis media.Thus early systemic treatment is very important for TOM.We reported a case report with TOM to highlight development of the disease and difficulties in clinical treatment in late stage of TOM.Implantation of ossified and eroded cochlea poses many unique challenges to both the surgeon and programming learn.With thorough preparation and complete knowledge about characters of specific issues,implantation would be performed successfully,and patients with ossified cochlear could benefit from cochlear implantation.展开更多
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 w...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.展开更多
Objective:To review developments in sound processors over the past 30 years that have resulted in significant improvements in outcomes for Nucleus~ recipients.
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-y...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.展开更多
基金This research was supported by the Special Fund for Economic and Technological Development of Longgang District,Shenzhen(LGKCYLWS2021000030).
文摘Background:Cochlear hair cell injury is a common pathological feature of hearing loss.The basic helix-loop-helix family,member e40(Bhlhe40),a gene belonging to the basic helix-loop-helix(bHLH)family,exhibits strong transcriptional repression activity.Methods:Oxidative damage,in House Ear Institute-Organ of Corti 1(HEI-OC1)cells,was caused using hydrogen peroxide(H2O2).The Ad-Bhlhe40 particles were constructed to overexpress Bhlhe40 in HEI-OC1 cells.Various assays including cell counting kit-8(CCK-8),terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling assay(TUNEL),flow cytometry,immunofluorescence,and corresponding commercial kits were employed to investigate the impacts of Bhlhe40 on cell viability,apoptosis,oxidative stress levels,mitochondrial membrane potential and cellular senescence.Additionally,a dual-luciferase reporter assay was performed to confirm the targeting of the histone deacetylases 2(Hdac2)by Bhlhe40.Results:The results revealed that Bhlhe40 was downregulated in H_(2)O_(2)-treated HEI-OC1 cells,but its overexpression improved cell viability and mitigated H_(2)O_(2)-induced oxidative injury in HEI-OC1 cells with increase of superoxide dismutase(SOD),catalase(CAT)and glutathione peroxidase(GPx)activities and decrease of reactive oxygen species(ROS)levels.Besides,overexpression of Bhlhe40 suppressed H_(2)O_(2)-triggered cell senescence,as evidenced by the fact that the upregulation of P53,P21,and P16 in HEI-OC1 cells treated with H2O2 were all alleviated by Bhlhe40 overexpression.And we further verified that overexpression of Bhlhe40 could inhibit the expression of Hdac2,which may be related to the repression of Hdac2 transcription.Conclusion:This study suggests that Bhlhe40 plays a protective role against senescence and oxidative damage in cochlear hair cells exposed to H2O2.
文摘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.
文摘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. .
文摘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.
文摘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.
文摘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.
基金Project supported by the Key Projects of National Natural Science Foundation of China(No.11932010)。
文摘Explaining the mechanism of the cochlear active phonosensitive amplification has been a major problem in medicine.The basilar membrane(BM)is the key infrastructure.In 1960,Nobel Laureate von B′ek′esy first discovered BM's traveling wave motion.Since that time,BM's models only have considered the traveling wave but not the biological activity.Therefore,a new model considering changes of BM's stiffness in space and time is established based on the immersed boundary method to describe its biological activity.It not only reproduces the results of traveling wave motion but also explains the mechanization on the generation of traveling wave.An important discovery is that changes of BM's stiffness in space and time will cause the unstable global resonance,which will induce amplification of sounds in cochlea.An important inference is that biological activity shall be included in the application of mechanical principles to the analysis of life,which is the essential difference between biomechanics and general mechanics.
文摘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.
文摘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<sup>®</sup> 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.
基金the National Natural Science Foundation of China(No.60475018)~~
文摘This paper proposes a cochlear prosthetic system with an implanted digital signal processor (DSP). This system transmits voice-band signals with a low data rate through the wireless link, free of the data-rate limitation and suitable for future development. By optimizing the speech processing algorithm and the DSP hardware design, the implanted DSP manages to execute the continuous interleaved sampling (CIS) algorithm at a clock frequency of 3MHz and a power consumption of only 1.91mW. With an analytic power-transmission efficiency of the wireless inductive link (40%), the power overhead caused by the implanted DSP is derived as 2.87roW,which is trivial when compared with the power consumption of existing cochlear prosthetic systems (tens of milliwatts). With the DSP implanted,this new system can.be easily developed into a fully implanted cochlear prosthesis.
文摘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.
文摘Cisplatin belongs to platinum-based drugs and is widely used in cancer chemotherapy.Ototoxicity is one of the major dose limiting side-effects of cisplatin.For toxicity to occur cisplatin must first be transported from the bloodstream into cochlear cells.Three copper transporters are considered pathways for regulating the uptake and translocation of cisplatin into cells:Ctr1,ATP7A and ATP7B.Our recent study with cochlear organotypic cultures shows that cochlear hair cells can be destroyed by cisplatin at low concentrations from 10μm to 100μn.However,high doses of cisplatin cannot damage hair cells,maybe due to intrinsic feedback reactions that increase export of platinum by ATP7B when the platinum concentration is high in extracellular space.Cimitidine is a specific copper transporter inhibitor that can block the entrance of copper and platinum,and may prevent cisplatin-induced cochlear hair cell injury.To evaluate this hypothesis,we treated cochlear organotypic cultures with cisplatin (10 μm or 50 μm) alone,or cisplatin combined with cimitidine at concentrations ranging from 10-2000 μm for 48 hours.cisplatin at 10 μm damaged about 20% hair cells.In contrast,when cimitidine (10 μm,100 μm and 2000 μm) was added to the culture,near 100% cochlear hair cell survived.At higher concentration (50 μm),cisplatin destroyed about 80% of cochlear hair cells.However,100 μmcimitidine rescued about 50% hair cells from cisplatin damage,and 2000μm cimitidine protected about 80% hair cells.The data of western blot showed that CTR1 and ATP7B expressions were increased in cisplatin treated cochlear tissue,but cimitidine significantly reduced CTR1 and ATP7B.In addition,ATP7A expression was depressed a little after cisplatin treatment.Considering that Ctr1 is involved in copper and platinum influx,but the ATP7A and ATP7B are copper export transporters,the results suggest that cimitidine can effectively block the entrance by copper transporters and stop the influx of cisplatin.
文摘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/).
文摘Objectives:This study aimed to assess the effect of hearing and speech rehabilitation in patients with Nurotron■cochlear implants.Design:Ninety-eight paediatric patients with bilateral severe-to-profound sensorineural deafness who received cochlear implantation were divided into three groups according to age:group A(≤3 years),group B(4-7 years),and group C(8-16 years).All patients were followed up for one year for hearing and speech performance after the surgery.The comprehensive Auditory Perception Assessment,MAIS,CAP and SIR hearing and speech assessments and rating materials were used for assessment before the surgery and at 3,6,and 12 months after implant activation.Results:The scores of patients in the open-set speech assessment,Chinese Auditory Perception Assessment,MAIS,CAP and SIR significantly improved after cochlear implantation in all age groups.The younger the age at implantation,the better the results.Moreover,the hearing and speech performance of cochlear implant recipients gradually improved with the extension of rehabilitation time.Conclusions:Nurotron■Venus^TM cochlear implantation can improve the hearing and speech performance of patients with bilateral severe-to-profound sensorineural deafness.
文摘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.
文摘Objective: A common cavity deformity(CCD) is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity that results in profound hearing loss. There are few studies paying attention to common cavity. Our group is engrossed in observing the improvement of auditory and verbal abilities in children who have received cochlear implantation(CI), and comparing these targets between children with common cavity and normal inner ear structure.Material and methods: A retrospective study was conducted in 12 patients with profound hearing loss that were divided into a common cavity group and a control group, six in each group matched in sex, age and time of implantation, based on inner ear structure. Categories of Auditory Performance(CAP) and speech intelligibility rating(SIR) scores and aided hearing thresholds were collected and compared between the two groups. All patients wore CI for more than 1 year at the Cochlear Center of Anhui Medical University from 2011 to 2015.Results: Postoperative CAP and SIR scores were higher than before operation in both groups(p < 0.05), although the scores were lower in the CCD group than in the control group(p < 0.05). The aided threshold was also lower in the control group than in the CCD group(p < 0.05).Conclusion: Even though audiological improvement in children with CCD was not as good as in those without CCD, CI provides benefits in auditory perception and communication skills in these children.
基金funded by Chinese National Scientifie Research Special-Purpose Project for Public Health Profession(No.200802070)National Program on Key Basic Research(2011CB504503)
文摘Tuberculous otitis media(TOM) is rare in ENT department,and is frequently misdiagnosed as otitis media.Thus early systemic treatment is very important for TOM.We reported a case report with TOM to highlight development of the disease and difficulties in clinical treatment in late stage of TOM.Implantation of ossified and eroded cochlea poses many unique challenges to both the surgeon and programming learn.With thorough preparation and complete knowledge about characters of specific issues,implantation would be performed successfully,and patients with ossified cochlear could benefit from cochlear implantation.
基金supported by Grant No.CX2012B101 from Scientific Innovation Fund of Hunan ProvinceGrant No.201206370106 from China State Scholarship Fund
文摘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.
基金funded by Cochlear Limited,the manufacturer of Nucleus implant systems
文摘Objective:To review developments in sound processors over the past 30 years that have resulted in significant improvements in outcomes for Nucleus~ recipients.
基金supported by grants from the National Basic Research Program of China(973Program)(#2012CB9679002011CBA01000)National Hi-Tech Research and Development Program of China(863Program)(#2007AA02Z150)to SMYthe National Natural Science Foundation of China(NSFC)(#30730040)to Yang Shi-ming
文摘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.