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Objectives: Evidence suggests that Cochlear Implantation (CI) is a beneficial approach for auditory and speech skills improvement in children with severe to profound hearing loss. However, it remains controversial if implantation in children <12 months is safe and effective compared to older children. The present study aimed to determine whether children's ages affect surgical complications and auditory and speech development., Methods: The current multicenter study enrolled 86 children who underwent CI surgery at <12 months of age (group A) and 362 children who underwent implantation between 12 and 24 months of age (group B). The Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were determined pre-impanation, and "one-year" and "two-year" post-implantation., Results: All children had full insertions of the electrode array. Four complications (overall rate: 4.65%; three minor) occurred in group A and 12 complications (overall rate: 4.41%; nine minor) occurred in group B. We found no statistically significant difference in the complication rates between the groups (p > 0.05). The mean SIR and CAP scores improved over time following CI activation in both groups. However, we did not find significant differences in CAP and SIR scores between the groups across different time points., Conclusion: Cochlear implantation in children younger than 12 months is a safe and efficient procedure, providing substantial auditory and speech benefits. Furthermore, rates and nature of minor and major complications in infants are similar to those of children undergoing the CI at an older age., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objective: This multicenter study aimed to evaluate the auditory and speech outcomes of cochlear implantation (CI) in deaf-blind patients compared with deaf-only patients., Study Design: Retrospective cohort study., Setting: Multiple cochlear implant centers., Patients: The current study was conducted on 17 prelingual deaf-blind children and 12 postlingual deaf-blind adults who underwent CI surgery. As a control group, 17 prelingual deaf children and 12 postlingual deaf adults were selected., Intervention: Cochlear implantation., Main Outcome Measures: Auditory and linguistic performances in children were assessed using the categories of auditory performance (CAP) and Speech Intelligibility Rating (SIR) scales, respectively. The word recognition score (WRS) was also used to measure speech perception ability in adults. The mean CAP, SIR, and WRS cores were compared between the deaf-only and deaf-blind groups before CI surgery and at "12 months" and "24 months" after device activation. Cohen's d was used for effect size estimation., Results: We found no significant differences in the mean CAP and SIR scores between the deaf-blind and deaf-only children before the CI surgery. For both groups, SIR and CAP scores improved with increasing time after the device activation. The mean CAP scores in the deaf-only children were either equivalent or slightly higher than those of the deaf-blind children at "12 months post-CI" (3.94 ± 0.74 vs 3.24 ± 1.25; mean difference score, 0.706) and "24 months post-CI" (6.01 ± 0.79 vs 5.47 ± 1.06; mean difference score, 0.529) time intervals, but these differences were not statistically significant. The SIR scores in deaf-only implanted children were, on average, 0.870 scores greater than the deaf-blind children at "12 months post-CI" (2.94 ± 0.55 vs 2.07 ± 1.4; p = 0.01, d = 0.97) and, on average, 1.067 scores greater than deaf-blind children at "24 months post-CI" (4.35 ± 0.49 vs 3.29 ± 1.20; p = 0.002; d = 1.15) time intervals. We also found an improvement in WRS scores from the "preimplantation" to the "12-month post-CI" and "24-month post-CI" time intervals in both groups. Pairwise comparisons indicated that the mean WRS in the deaf-only adults was, on average, 10.61% better than deaf-blind implanted adults at "12 months post-CI" (62.33 ± 9.09% vs 51.71 ± 10.73%, p = 0.034, d = 1.06) and, on average, 15.81% better than deaf-blind adults at "24-months post-CI" (72.67 ± 8.66% vs 56.8 ± 9.78%, p = 0.002, d = 1.61) follow-ups., Conclusion: Cochlear implantation is a beneficial method for the rehabilitation of deaf-blind patients. Both deaf-blind and deaf-only implanted children revealed similar auditory performances. However, speech perception ability in deaf-blind patients was slightly lower than the deaf-only patients in both children and adults., Competing Interests: The authors have no relevant financial conflicts or other conflicts of interest to disclose., (Copyright © 2022, Otology & Neurotology, Inc.)

Background and Objective: Patients with profound Sensorineural Hearing Loss (SNHL) are susceptible to vestibular disturbances following Cochlear Implant (CI) surgery. This study aimed to evaluate vestibular dysfunctions following unilateral CI in the congenitally deaf children., Methods: This was a cross-sectional study conducted on 24 children (mean age: 10.56 ± 5.49 years old) who underwent unilateral CI and 24 age-matched controls (mean age: 11.13 ± 6.21 years old). Vestibular functions were assessed by Vestibular Evoked Myogenic Potential (VEMP) and Computerized Dynamic Posturography (CDP). The VEMP test was performed for otolith's function (especially saccule) evaluation. Sensory Organization Test (SOT) protocol of CDP was also utilized to differentiate the role of various sensory systems contributing to postural stability. In addition, total equilibrium score was calculated. The variables were comparatively assessed between the two groups., Results: The mean p13-n23 amplitude in the CI users was significantly lower than the controls (p<0.05). However, the two groups showed no significant difference in cVEMP latency values (p>0.05). The SOT analysis revealed that 45.83% (11/24) of the CI subjects had some kind of sensory abnormalities: 7 cases (29.17%) vestibular, 2 cases (8.33%) visual, 2 cases (8.33%) vestibular and somatosensory involvements. Furthermore, total equilibrium score was significantly reduced in implanted group than the controls (p<0.001). At least, 70.59% (12/24) CI patients showed abnormal values in the CDP or cVEMP examinations., Conclusion: This study shows functional vestibular impairments in children who underwent CI. These patients showed significantly increased postural instability which was more evident in dynamic conditions. These findings provide the basis for better pre-operative counseling and postoperative vestibular rehabilitation to CI recipients.

Background: The performance of a cochlear implant (CI), especially in conveying pitch depends on its electrical stimulation strategy., Objective: The present study proposes a variable-rate stimulation algorithm which improves speech emotion perception by using temporal fine-structure cues and electrophysiological parameters of the patient., Methods: This method is based on the coding of the phase information at the peak time intervals of the band-passed signals. The stimulation pulse is generated at the time of peak occurrence, which is able to excite the number of fibers with a discharge probability above a threshold. Calculating the discharge probability is based on the excitable fiber model and taking into account the biological characteristics of the patient, such as the fiber threshold and the distribution of remaining intact fibers., Results: The results of the emotion detection test on selective reconstructed sentences from the Persian emotional speech database (Persian ESD) indicated that the listeners have been able to detect the emotion by an average of 83.82% using the proposed stimulation algorithm while it was 75% and 48.03% for the zero-crossing and the continuous interleaved sampling (CIS), respectively. Furthermore, the number of pulses compared to the zero-crossing and the CIS has decreased by 76.3% and 75.4%, respectively., Conclusions: In this paper, a stimulation method was proposed for cochlear implants by considering the patient's biological parameters. It has been successful in transmitting speech emotion despite the reduction of stimulating pulses. This has some advantages such as reducing the interaction of current fields between electrodes during stimulation and reducing battery usage.

Objectives: The purpose of this study is to retrospectively review the complications of paediatric patients undergoing cochlear implantation at four major Iranian cochlear implant centres., Methods: A retrospective analysis was performed of all patients who underwent primary cochlear implantation from January 1991 to December 2013. The patients were reviewed for demographic information, and complications including cerebrospinal fluid leak, meningitis, facial palsy, and wound infection., Results: 4400 records were reviewed. Fifty-four patients were lost to follow-up; therefore, 4346 records were analysed. The most common aetiology of hearing loss was non-syndromic genetic sensori-neural hearing loss (69%). Other less common aetiologies of hearing loss included TORCH (Toxoplasmosis, Other infections, Rubella, Cytomegalovirus, Herpes) (11%), syndromic hearing loss (7%), ototoxicity (5%), and autoimmune inner-ear disease (4%). The most common major complications were CSF leak (0.4%), skin necrosis (0.2%), meningitis (0.1%), facial paralysis (0.07%) and massive haemorrhage (0.05)., Conclusion: Cochlear implantation continues to be reliable and safe in experienced hands, with a very low percentage of severe complications., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: New cochlear implant (CI) designs and developments in implantation techniques have revolutionized the management of hearing loss. However, cochlear implantation still has some disadvantages, such as its potential to initiate an inflammatory response that may lead to further hair cell damage. Recent topics of investigation have been the effect of glucocorticoids on inflammatory tissue response reduction, glucocorticoid dosage levels, and drug-delivery methods. In the present study, dexamethasone delivery via a drug-eluting CI was evaluated histologically through assessing inflammatory cell infiltration., Methods and Materials: Thirty healthy, adult male guinea pigs were included and randomly assigned to one of three surgical groups that underwent cochleostomy of the basal turn. The experimental group (Group 1) of 12 animals were implanted with a dexamethasone-loaded silicone elastomer shaped like a CI electrode. The primary control group (Group 2) of 12 animals were implanted with a simple CI (non-eluting). A second control group (Group 3) of six animals underwent cochleostomy only. Inflammatory responses were compared between groups by evaluating inflammatory cell infiltration in inner-ear specimens at days 3 and 13., Results: The Mann‐Whitney test revealed reduction in most of the inflammatory indices in Group 1 compared with Group 2. This was significant for fibrocyte, macrophage, and giant cell infiltration at day 3 as well as lymphocyte, macrophage infiltration, and capillary formation at day 13., Conclusion: This study showed some attenuation in inflammatory response following insertion of a dexamethasone-eluting CI, suggesting that it could be a route for local drug delivery into the cochlea.

In this study, drug-eluting cochlear implant (CI) electrodes were prepared, and the amount of drug released was determined. Dexamethasone (DEX) (0.25-2% w/w, the weight percent of the final cured polymer) was used as a bioactive agent to suppress postsurgical inflammations upon mixing with a two-part nonrestricted pourable medical-grade silicone elastomer. Batch reproducibility analysis was performed on three consecutive batches. Drug release experiments were accomplished in normal saline medium, where DEX was analyzed via a validated HPLC method. The drug loading percentage and the device surface area were the most dominant parameters explored to monitor the drug release behavior from CI coatings. Total cumulative amount of DEX released from various loaded samples was in the order of 2 > 1 > 0.5 > 0.35 > 0.25% w/w, but the cumulative percentage of drug released showed a reverse order. The DEX dosages between 0.1 and 1 microg were released from samples of smallest to highest loadings during the initial 24 h, and dosages <1-5 microg were released from similar samples of various loadings at the first patency 2 weeks. The extent of crosslinking was only effective on release profile at lower drug loadings of 0.25% w/w relative to 0.5%. It was also found that release profile was not affected by postcuring. (c) 2010 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater, 2010.

Conclusion: The results of this study show that R- prelingually cochlear-implanted children are not optimal candidates for cochlear implantation. However, if this group are implanted, other rehabilitation methods should be incorporated into their rehabilitation program in order to achieve better results and maximize the efficacy of their prosthesis., Objective: To demonstrate that the blink reflex or auropalpebral reflex evaluation can be used as a prognostic factor for the assessment of auditory and speech perception levels in prelingually cochlear-implanted children., Material and Methods: In an observational, analytical, prospective study conducted at a single cochlear implant rehabilitation center in 85 prelingually cochlear-implanted children, the presence or absence of the blink reflex (BR) was evaluated and the results of auditory and speech perception tests were compared between reflex-positive (R+) and reflex-negative (R-) patients. To obtain the BR, four electrodes were applied in both the Nucleus and MED-EL systems and then stimulated by means of current levels higher than the previously detected most comfortable level of the patient until the reflex appeared. Auditory and speech perception levels were measured using the vowels-confusion test and the categorization of auditory perception scale., Results: The mean results of auditory and speech perception tests were significantly higher in R+ compared to R- patients.

One of the most important factors in auditory speech perception of cochlear-implanted children is age. The goal of this study was to compare auditory speech perception among children implanted from 6 different age groups: 0 to 3, 4 to 5, 6 to 7, 8 to 9, 10 to 11, and >12 years. The subjects of this study were matched based on socioeconomic status, residual hearing before cochlear implantation, the kinds of cochlear implant device, speech processing strategy, communication mode after implantation, and primary language in family. All of them have used the device minimally for 2 years. The subjects were tested with a range of closed and open-set auditory speech perception tests, and the levels of auditory speech perception in different age groups were compared. Results showed that the children who received an implant at 0 to 3 years of age had maximum auditory speech perception.

Background: This study aims to evaluate speech production outcomes and auditory performance in children with post-meningitis deafness who were treated with cochlear implants. Additionally, the study assesses the impact of electrode insertion depth on surgical outcomes.". Methods: We conducted a study on 66 pediatric patients with bilateral postmeningitis hearing loss who were being prepared for cochlear implantation at four tertiary referral academic institutions. The speech intelligibility rating (SIR) and categories of auditory performance (CAP) were evaluated after the first and second years following implantation. The patients were divided into two groups based on electrode insertion depth: one group had full electrode insertion (more than two-thirds), while the other had partial electrode insertion (less than two-thirds). We compared the SIR and CAP scores between the two groups to assess the impact of electrode insertion depth on outcomes. Results: Before implantation, the median CAP score was one, but it improved significantly to six within two years after the procedure (P-value < 0.001). Similarly, the median SIR score before implantation was one, but it improved significantly to three within two years after surgery (P-value < 0.001). However, there was no significant difference between the partial and full electrode insertion groups in terms of CAP and SIR scores during the follow-up evaluations conducted after the first and second years. Conclusion: The study found that cochlear implantation significantly improved speech production skills and auditory performance in children with postmeningitis deafness. Importantly, the amount of electrode insertion at the time of implantation did not have a significant impact on the outcomes. [ABSTRACT FROM AUTHOR]

The goal of this study was to investigate the probable difference in auditory perception and speech intelligibility performance amongst cochlear implanted children who experienced hyperbilirubinemia or auditory neuropathy in comparison to the cochlear implanted children with unknown etiology for hearing loss. This case–control study was carried out on 106 cochlear implanted children with mean age of 32.36 ± 11.98 months who were purposively selected and allocated into four groups. Out of the total, 30 had no specific etiology for hearing loss, while the others had experienced auditory neuropathy or hyperbilirubinemia with/without blood exchange. The auditory perception and speech intelligibility performance of all the participants who had received auditory verbal therapy were assessed after 6 and 12 months of rehabilitation. Then, the data was analyzed, using the Statistical Package for Social Sciences-version 21(SPSS-21). Results indicated poor auditory perception and speech intelligibility performance of the cochlear implanted children with hyperbilirubinemia and blood exchange (P ≤ 0.05), while the participants in the control group with no specific etiology for hearing loss, the children with hyperbilirubinemia with no blood exchange, and those who suffered from auditory neuropathy performed better, respectively. Also, a significant correlation between auditory neuropathy and hyperbilirubinemia was observed. Despite lower improvement of auditory perception and speech intelligibility of the hearing impaired children who were experiencing moderate to severe degrees of hyperbilirubinemia or auditory neuropathy, cochlear implantation is highly recommended not only for children with unknown etiology for severe hearing loss but also for this group of hearing impaired children. [ABSTRACT FROM AUTHOR]

Background: Transmembrane inner ear (TMIE) protein is an essential component of the mechanotransduction complex. In collaboration with other components, TMIE aids the maintenance and function of the sensory hair cells. Autosomal recessive deafness-6 (DFNB6) is caused by mutated TMIE, a gene in the high genetic heterogeneity spectrum of deafness. Hearing loss has a significant impact on the global economy and the quality of life of affected persons, their families, and society. Here, three unrelated families with TMIE variants are presented. All three cases were found while studying the genetic causes of an Iranian cohort of subjects with cochlear implants. Methods: Whole exome sequencing was performed to find possible genetic etiology in probands of families after a comprehensive medical evaluation for hearing loss. Co-segregation analysis in probands and other family members was performed by Sanger sequencing. The variants were interpreted per the American College of Medical Genetics and Genomics guidelines. Results: Three different variants associated with TMIE were confirmed as reasons for autosomal recessive non-syndromic deafness. The first novel ~ 10-kb deletion surrounding exon 1 of TMIE along with two previously reported variants co-segregated with families including a frameshift variant c.122_125dup (p.Pro43fs) and a missense variant c.250 C > T; p.(Arg84Trp) in exons 2, and 3, respectively. Conclusion: This study increases the mutational spectrum of the TMIE gene and highlights the importance of the large deletion of this gene as a reason for hearing loss. Moreover, an efficient and simple multiplex PCR assay was developed to determine the exact breakpoints of the TMIE deletion. [ABSTRACT FROM AUTHOR]

Introduction: Patients with sensorineural hearing loss suffer concomitant vestibular dysfunction that is more prevalent in patients with inner ear anomaly and could be aggravated with cochlear implantation. To assess the vestibular dysfunction in patients with cochlear implantation, we compared their results with those of patients with and without inner ear anomaly. Materials and methods: This is a historical cohort study lasting for 20 years on 50 patients with cochlear implantation. All patients underwent dynamic posturography and Bruininks‐Oseretsky Test. Results: Twenty‐two (44%) of the participants showed some types of inner ear anomaly. The frequency of abnormal Bruininks‐Oseretsky Test was 45.5% and 10.7% (P =.005, odds ratio [OR] = 6.9). Abnormal composite was seen in 77.3% and 21.4%, respectively (P <.001; OR = 12.5). The mean strategy score in the fifth condition of the sensory organization test was 25.0 ± 20.4 in patients with inner ear anomaly, whereas it was 44.1 ± 18.9 in those without it (P =.001). Conclusion: Balance capability in cochlear implantation patients with inner ear anomaly compared to those without inner ear anomaly was worse. More vestibular rehabilitation treatment plans are suggested for these patients. [ABSTRACT FROM AUTHOR]

Mitochondrial DNA mutations play an important role in causing sensorineural hearing loss. The purpose of this study was to determine the association of the mitochondrial genes RNR1, MT-TL1, and ND1 as well as the nuclear genes GJB2 and GJB6 with audiological examinations in nonfamilial Iranians with cochlear implants, using polymerase chain reaction, DNA sequencing, and RNA secondary structure analysis. We found that there were no novel mutations in the mitochondrial gene 12S rRNA (MT-RNR1) in patients with and without GJB2 mutation (GJB2(+) and GJB2(-), respectively), but a total of six polymorphisms were found. No mutations were observed in tRNA(Leu) (() (UUR) ()) (MT-TL1). Furthermore, eight polymorphisms were found in the mitochondrial ND1 gene. Additionally, no mutations were observed in the nuclear GJB6 gene in patients in the GJB2(-) and GJB2(+) groups. The speech intelligibility rating and category of auditory perception tests were statistically assessed in patients in the GJB2(-) and GJB2(+) groups. The results indicated that there was a significant difference (P<0.05) between the categories of auditory perception score in the GJB2(-) group compared to that in the GJB2(+) group. Successful cochlear implantation was observed among individuals with GJB2 mutations (GJB2(+)) and mitochondrial polymorphisms compared to those without GJB2 mutations (GJB2(-)). In conclusion, the outcome of this study suggests that variation in the mitochondrial and nuclear genes may influence the penetrance of deafness. Therefore, further genetic and functional studies are required to help patients in making the best choice for cochlear implants. [ABSTRACT FROM AUTHOR]

Background and Aim: In neural response telemetry (NRT), intracochlear electrodes stimulate the auditory nerve and record the neural responses. The electrical stimulation send to the auditory nerve by an electrode and the resulted response, called electrically evoked compound action potential (ECAP), is recorded by an adjacent electrode. The most important clinical applications of this test are evaluation and monitoring the intra and postoperative responses of auditory nerve and help to primary setting of speech processor. The aim of this study was evaluating of the potential's threshold changes in three monthes after receiving the devise in pediatric cochlear implant recipients. Materials and Methods: This longitudinal study evaluated the potential's threshold in four given electrodes in four sessions after receiving the device by approximately one months intervals in children implanted in Amir Alam and Hazrat-e-Rasoul hospitals in 2007, July to December. Results: ECAP mean threshold level of each electrode did not significantly change in differnent sessions, while there was significant difference between apical and basal electrodes' responses in every session(p<0.001). Conclusion: The reliabiliy of the responses result in more certainty of clinician to fit the speech processor for a long time. Better responses in apical electrodes may lead to develope an effective coding strategy. [ABSTRACT FROM AUTHOR]

Objectives To evaluate the auditory performance and speech production outcome in children with auditory neuropathy spectrum disorder (ANSD). The effect of age on the outcomes of the surgery at the time of implantation was also evaluated. Methods Cochlear implantation was performed in 136 children with bilateral severe-to- profound hearing loss due to ANSD, at four tertiary academic centers. The patients were divided into two groups based on the age at the time of implantation; Group I: Children ≤24 months, and Group II: subjects >24 months. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated after the first and second years of implantation. The differences between the CAP and SIR scores in the two groups were assessed. Results The median CAP scores improved significantly after the cochlear implantation in all the patients (p value < 0.001). The improvement in the CAP scores during the first year in Group II was greater than Group I (p value: 0.007), but the improvement in CAP scores tended to be significantly higher in patients who were implanted at ≤24 months (p value < 0.001). There was no significant difference between two groups in SIR scores at first-year and second-year follow-ups. The evaluation of the SIR improvement revealed significantly higher values for Group I during the second-year follow-up (p value: 0.003). Conclusion The auditory performance and speech production skills of the children with ANSD improved significantly after cochlear implantation, and this improvement was affected by age at the time of implantation. [ABSTRACT FROM AUTHOR]