A National Iranian Cochlear Implant Registry (ICIR): cochlear implanted recipient observational study.

BACKGROUND AND OBJECTIVE: Patients who receive cochlear implants (CIs) constitutes a significant population in Iran. This population needs regular monitor on long-term outcomes, educational placement and quality of life. Currently, there is no national or regional registry on the long term outcomes of CI users in Iran. The present study aims to introduce the design and implementation of a national patient-outcomes registry on CI recipients for Iran. This Iranian CI registry (ICIR) provides an integrated framework for data collection and sharing, scientific communication and collaboration inCI research. METHODS: The national ICIR is a prospective patient-outcomes registry for patients who are implanted in one of Iranian centers. The registry is based on an integrated database that utilizes a secure web-based platform to collect response data from clinicians and patient's proxy via electronic case report forms (e-CRFs) at predefined intervals. The CI candidates are evaluated with a set of standardized and non-standardized questionnaires prior to initial device activation(as baseline variables) and at three-monthly interval follow-up intervals up to 24 months and annually thereafter. RESULTS: The software application of the ICIR registry is designed in a user-friendly graphical interface with different entry fields. The collected data are categorized into four subsets including personal information, clinical data, surgery data and commission results. The main parameters include audiometric performance of patient, device use, patient comorbidities, device use, quality of life and health-related utilities, across different types of CI devices from different manufacturers. CONCLUSION: The ICIR database could be used by the increasingly growing network of CI centers in Iran. Clinicians, academic and industrial researchers as well as healthcare policy makers could use this database to develop more effective CI devices and better management of the recipients as well as to develop national guidelines.

Correlation between Auditory Spectral Resolution and Speech Perception in Children with Cochlear Implants.

BACKGROUND: Variability in speech performance is a major concern for children with cochlear implants (CIs). Spectral resolution is an important acoustic component in speech perception. Considerable variability and limitations of spectral resolution in children with CIs may lead to individual differences in speech performance. The aim of this study was to assess the correlation between auditory spectral resolution and speech perception in pediatric CI users. METHODS: This cross-sectional study was conducted in Shiraz, Iran, in 2017. The frequency discrimination threshold (FDT) and the spectral-temporal modulated ripple discrimination threshold (SMRT) were measured for 75 pre-lingual hearing-impaired children with CIs (age=8-12 y). Word recognition and sentence perception tests were completed to assess speech perception. The Pearson correlation analysis and multiple linear regression analysis were used to determine the correlation between the variables and to determine the predictive variables of speech perception, respectively. RESULTS: There was a significant correlation between the SMRT and word recognition (r=0.573 and P<0.001). The FDT was significantly correlated with word recognition (r=0.487 and P<0.001). Sentence perception had a significant correlation with the SMRT and the FDT. There was a significant correlation between chronological age and age at implantation with SMRT but not the FDT. CONCLUSION: Auditory spectral resolution correlated well with speech perception among our children with CIs. Spectral resolution ability accounted for approximately 40% of the variance in speech perception among the children with CIs.

Comparison of Auditory Perception in Cochlear Implanted Children with and without Additional Disabilities.

BACKGROUND: The number of children with cochlear implants who have other difficulties such as attention deficiency and cerebral palsy has increased dramatically. Despite the need for information on the results of cochlear implantation in this group, the available literature is extremely limited. We, therefore, sought to compare the levels of auditory perception in children with cochlear implants with and without additional disabilities. METHODS: A spondee test comprising 20 two-syllable words was performed. The data analysis was done using SPSS, version 19. RESULTS: Thirty-one children who had received cochlear implants 2 years previously and were at an average age of 7.5 years were compared via the spondee test. From the 31 children, 15 had one or more additional disabilities. The data analysis indicated that the mean score of auditory perception in this group was approximately 30 scores below that of the children with cochlear implants who had no additional disabilities. CONCLUSION: Although there was an improvement in the auditory perception of all the children with cochlear implants, there was a noticeable difference in the level of auditory perception between those with and without additional disabilities. Deafness and additional disabilities depended the children on lip reading alongside the auditory ways of communication. In addition, the level of auditory perception in the children with cochlear implants who had more than one additional disability was significantly less than that of the other children with cochlear implants who had one additional disability.

Tracing of helicobacter pylori in patients of otitis media with effusion by polymerase chain reaction.

Otitis media with effusion (OME) is one of the most common causes of hearing loss (HL) in children. It has been reported that several factors such as eustachian tube dysfunction, insufficiencies in the aeration of the mastoid cells, allergies, immunity, and infections play an important role in the etiology of the disease. Little is known about the role of Helicobacter pylori (H. pylori) in extragastric diseases. Because of the near location of the nose, sinuses, tonsils, and adenoids to the eustachian tube and middle ear, we believe it is possible to have H. pylori in the middle ear. The present study was designed to investigate the presence of H. pylori by polymerase chain reaction (PCR) in the middle ear effusion of patients with OME. The study was performed on 21 patients, 19 patients were affected bilaterally, and 2 patients were affected unilaterally, from which 40 specimens were collected. OME was diagnosed through findings by otoscopic examination and tympanogram. The middle ear fluid samples were collected under sterile conditions. A total of 40 samples was stored at -80°C until analyzed by PCR assay. From 40 specimens, 2 specimens were serosal and 38 specimens were mucoid. PCR results of the study in assays for Helicobacter pylori were not positive in all collected specimens. Overall, probably there was no H. pylori organism in free-floating form and thus could not be detected by PCR.

Amniotic membrane in reconstruction of larynx following chondrosarcoma resection: a case report.

Chondrosarcomas of the larynx are rare malignancies and frequently located in cricoid cartilage. They are characterized by a low tendency to metastasis (low grade type). The treatment of choice is surgery, which may be endoscopic or open partial surgery, if extension of the cancer is limited. Prognosis is generally good. In this report, a case of low grade chondrosarcoma of the larynx is presented, which was treated surgically with a combined use of amniotic membrane and stent in airway reconstruction following laryngofissure approach for resection of the tumor.

Cochlear Implantation with a Combined Approach: A Case Report.

Introduction: Ear symptoms of granulomatosis with polyangiitis can range from ear fullness and otalgia to conductive or sensory neural hearing loss and sudden deafness. Cochlear implantation in these patients faces two challenges: access to the round window and control of mastoid and middle ear inflammation. The combined approach in cochlear implantation is a classic trans-facial recess approach facilitated by a trans-canal view. Case Report: In this case report, we present the "combined approach" in a 20-year-old lady with granulomatosis with polyangiitis who underwent cochlear implantation successfully using the combined approach. Conclusion: Post-operative results suggest that the "combine approach" seems to be a safe, easy, and fast cochlear implantation technique for chronic otitis media with an atelectatic middle ear and retracted tympanic membrane or narrow facial recess space. It is a single-stage surgery that has no need for the obliteration of the ear and has less morbidity.

Evaluating the Relation Between Mothers' Depression Level and Their Cochlear Implanted Children's Social Performance.

Since the child's sensorineural hearing loss and its negative consequences, including weak social skills, is one of the most challenging problems for parents and other family members, the main aim of the present study is to investigate if there is any probable relation between the cochlear implanted children's social performance and their mothers' depression level,in this cross-sectional observational analytic research, we implemented the Beck Depression Inventory version- II to evaluate the level of depression in 29 mothers of cochlear implanted children. Also, we assessed their children's social abilities through the Vineland Adaptive Behavior Scale. Finally, the analysis of variance (ANOVA) was carried out to find any probable relation between the mothers' depression level and their children's social performance. The mean difference was significant at the 0.05 level,results indicated no significant difference in the mean social performance of the children mothers encountered with mild or moderate degrees of depression. However, the mean social performance of the children with severely depressed mothers was significantly less than the others,severe to profound sensorineural hearing loss in children might cause various degrees of depression in mothers. Mothers who suffer from severe depression cannot effectively communicate with their children. Consequently, the child will not improve in social interaction well.

Exploring the OTITIS Research Landscape Through a Scientometric Approach.

Otitis media is a common health problem affecting people of all ages and significantly impacting public health and healthcare costs. Otitis media, a type of middle ear disease, is one of the most common types. This scientometric study aimed to provide an overview of the knowledge domain in otitis media research. Documents were retrieved from the Web of Science database. A scientometric study was then performed on a sample of 27,213 documents. This study found that research on otitis media has increased significantly in recent years, with an annual growth rate of 4.58%. The average age of the documents analyzed was 18 years, with an average of 21.88 citations and an average of 4.58 authors. The United States, the United Kingdom, and Japan ranked first to third in terms of number of publications. Still, the United States, China, and Sweden were in a better position in terms of impact on the research network. Co-occurrence word analysis showed that significant attention was given to topics such as chronic inflammation in autism, acute inflammation in otitis media, and increased fluid in the middle ear. This study highlights the need to prioritize and focus attention on otitis, particularly otitis media, due to its prevalence and impact on public health. The use of scientometric software, such as Biblioshiny and CiteSpace, provides a valuable means of assessing research trends and identifying important areas for future study in the field of knowledge.

Cochlear Implantation in Children with Meningitis: A Multicenter Study on Auditory Performance and Speech Production Outcomes.

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.

Comparing the Patients with or without Inner Ear Malformations in Terms of Intra and Postoperative Complications of Cochlear Implantation.

Introduction: Since 20% of the patients with sensorineural hearing loss have confirmed radiographically inner ear malformation, this study aimed to compare the incidence rate of intra and postoperative complications of cochlear implantation amongst the patients with or without inner ear malformations. Materials and Methods: In this retrospective study, we evaluated the medical record of 954 patients who had undergone cochlear implantation. Seventeen patients had inner ear malformations and were selected as the case group, and 25 patients with normal inner ear were selected as the control group. Patient's information; including intraoperative complications, post-operative complications and neural response telemetry (NRT) immediately after the surgery were recorded. Finally, the collected data were analyzed, using the SPSS software, version 21. Results: According to the CT-scan findings, the most observed internal ear abnormality was the temporal bone Mondini in 8 patients (47%), and the next inline was the common cavity at a frequency of (23.52%). Cerebrospinal fluid (CSF) gusher was detected in 11 patients (64.7%) of the case group, which was significantly higher than the other group. This includes mild CSF gusher in 7 cases (41.17%) and severe CSF gusher in 5 cases (29.41%). Interestingly, no significant post-operative complications were observed in either group, minor side effects were limited and not significantly different. Conclusion: In conclusion, despite the limitations of cochlear implantation surgery amongst patients with inner ear malformation with potential risk of early or late complications, it can still be recommended as an appropriate procedure to acquire hearing as well as auditory and speech perception.

Molecular strategy for the direct detection and identification of the most common fungal community in cerumen specimens by multiplex PCR.

Introduction. Otomycosis is a superficial fungal infection that is responsible for approximately 9-27 % of otitis externa. However, fungal communities in otomycosis are varied, but Aspergillus spp. and Candida spp. are the most common causes of this infection.Hypothesis Statement. The multiplex PCR assay is postulated to be able to directly detect more than one fungal genus in cerumen specimens.Aim. This study aimed to develop and evaluate the role of the multiplex PCR assay in detecting the most common genus of fungi that cause otomycosis directly from the cerumen specimens.Methodology. To detect Candida and Aspergillus/Penicillium genera, three pairs of primers, including pan-fungal, pan-Candida, and pan-Aspergillus/Penicillium, were used in a multiplex PCR. In order to evaluate the performance and reproducibility of the multiplex PCR. the cerumen of 140 patients suspected of otomycosis were investigated.Results. Pan-Candida and pan-Aspergillus/Penicillium primers were designed to amplify the ITS1-5.8S-ITS2 region and the ß-tubulin gene, respectively. In the multiplex PCR assay, 64 (47.40 %) and 118 (87.40 %) specimens were positive with pan-Candida and pan-Aspergillus/Penicillium primers, respectively. Double amplicon bands of Candida and Aspergillus were obtained in 51 (37.77 %) specimens. In the culture method, yeast (n=18, 13.33 %) and mould (n=117, 86.66 %) were isolated from 135 cerumen specimens. The sensitivity, specificity, and positive and negative predictive values of the multiplex PCR assays using culture method results as the gold standard were determined to be 94, 33, 97, and 22 %, respectively.Conclusion. In our study, multiplex PCR assays enabled simultaneous detection of two common genera of the causative agent of otomycosis in a cerumen specimen. Regarding the high sensitivity of the first step of the multiplex PCR assay, this assay may be used for the direct detection of Candida and Aspergillus genera in other clinical specimens.

Complications and outcomes of cochlear implantation in children younger than 12 months: A multicenter study.

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.

Prevalence of GJB2 (CX26) gene mutations in south Iranian patients with autosomal recessive nonsyndromic sensorineural hearing loss.

Hereditary hearing loss is a genetically heterogeneous disorder. Mutations in connexin 26 (CX26), are a major cause in many countries and are largely dependent on ethnic groups. The purpose of our study was to evaluate the prevalence of GJB2 mutations among affected individuals from south of Iran. Fifty patients presenting with autosomal recessive non-syndromic hearing loss from Fars, province in south of Iran, were studied for mutations in GJB2 gene and screened by direct sequencing. Mutations were detected in 15 out of 50 patients (30 %). Eight different mutations were identified; six of them were previously identified (35delG, V27I M34V, V153I, A149T, V198M). The remaining two alleles, L28I and N169T, were novel variants. The most common mutations were 35delG followed by V153I with an allele frequency of 7 and 6 %, respectively. In this study, 30 % of our subjects were found to have the causative variants or polymorphisms in GJB2 and the c.35delG mutation was the most common cause in our patients. However, more study with larger sample size as well as in vitro functional study for these new variants in Xenopus oocytes is required.

"Mucosal pocket" myringoplasty: a modification of underlay technique for anterior or subtotal perforations.

PURPOSE: Chronic otitis media surgery is the most common procedure in otology in developing countries. Subtotal and total tympanic membrane (TM) perforation with inadequate anterior remnant is associated with higher rate of graft failure. It was the goal of this study to test the anatomical and functional outcomes of a modified underlay myringoplasty technique. MATERIALS AND METHODS: In a prospective clinical study, 45 patients with subtotal or total TM perforation and inadequate anterior remnant underwent tympanoplasty (+/-mastoidectomy). The anterior tip of the temporalis fascia was secured in a mucosal pocket on the lateral wall of eustachian tube orifice. Data on graft take rate, preoperative and postoperative hearing status, and intraoperative findings were analyzed. RESULTS: We achieved the graft success rate of 91.1%, without lateralization, blunting, atelectasia, or epithelial pearls. Approximately 24% patients had air-bone gap within 25 dB before intervention, which increased to 71% postoperatively (P < .001). CONCLUSION: We believe that this technique could be a convenient and suitable method for cases with subtotal or total TM perforation and inadequate anterior remnant.

Cochlear implantation outcomes in children with global developmental delay.

OBJECTIVE: As the number of hearing loss cochlear implant candidates who suffer from global developmental delay has dramatically increased, we aimed to study the prognosis of implantation in this group. MATERIALS AND METHODS: In this cross-sectional case-control study, we utilized the Ages and Stages Questionnaire third edition (ASQ-3) to investigate the prognosis of cochlear implantation and its rehabilitation in 26 congenitally deaf children who suffered from global developmental delay compared with those in 25 non-delayed cases with the same conditions in two time periods, namely the first diagnosis of hearing loss and 18 months after the surgery and rehabilitation program. The data were analyzed using Statistical Package for Social Sciences, version 21 (SPSS-21). RESULTS: By the time of hearing loss diagnosis (six months old), the performance of all the global developmentally delayed hearing loss children in five subtests of the ASQ-3 scale was significantly lower than that of their non-delayed peers. Meanwhile, they improved significantly in two gross motor and social development subtests 18 months after the surgery and rehabilitation. CONCLUSION: Along with the general improvement of delay developed children with sensorineural hearing loss after cochlear implantation, global developmental assessment in the process of candidacy and after implantation is an essential factor that needs to be considered.

Auditory and speech outcomes of cochlear implantation in post-meningitis deafness.

PURPOSE: This study aims to identify the auditory, speech, and surgical outcomes of cochlear implantation in patients with profound SNHL following bacterial meningitis. METHODS: Subjects with bilateral severe to profound SNHL who underwent unilateral cochlear implantation from 2003 to 2020 were included in this historical cohort study. The main outcomes were assessed using Categories of Auditory Performance (CAP) and Speech Intelligibility Ratings (SIR) scores. The CAP and SIR outcomes were collected as three-time points after surgery: 6, 12, and 24 months. In order to achieve the strength of the relationship and for computing the Risk Ratio (RR) by log-binominal regression method, we used two binary categorizations of CAP and SIR in our analyses. RESULTS: The mean of age at implantation of the study and control group were 144.30 (156.90) and 121.10 (133.70) months, respectively. In the study group, 19 of 35 (54.3%) patients were male, and 16 (45.7%) were female. In the control group, 34 of 81 (42.0%) patients were male and 47 (58.0%) were female. The mean scores of CAP and SIR in our study improved significantly during the time in both groups. All p-values (p) were significant in both groups (T2 vs T1, T3 vs T1, and T3 vs T2). Our analysis by log-binomial regression and computing the RR based on the first and second categorization of CAP and SIR showed moderate to strong relationships between the presence of a history of meningitis and inappropriate CAP and SIR outcomes in these patients. CONCLUSIONS: Although subjects who were deafened due to meningitis benefit significantly from cochlear implantation, we found moderate to strong relationships between the history of meningitis and inappropriate CAP and SIR outcomes in these patients.

Hyperbilirobinemia and Its Effect on Auditory Perception and Speech Intelligibility in Cochlear Implanted Children (Cochlear Implantation Outcomes in Children Who Have Experienced Hyperbilirobinemia).

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.

The Outcome of Cochlear Implantations in Deaf-Blind Patients: A Multicenter Observational Study.

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.

Is There Any Association Between Language Acquisition and Cognitive Development in Cochlear-Implanted Children?

OBJECTIVE: Different studies on normal children and children with a sensory or intellectual disability indicate a strong correlation between the child's vocabulary domain and his cognitive abilities. Based on this, the main focus of the present study was to investigate the cognitive performance of cochlear-implanted children after a cognition-based language intervention program. METHODS: In this experimental study, 60 cochlear-implanted children were selected and randomly allocated into case and control groups. The control group received auditory verbal therapy (AVT), while the intervention group was trained by using both AVT and a language intervention protocol that was recently developed by the authors. Finally, the participants' communication abilities were assessed through the adapted version of the language subtest of Bayley Scales of Infant and Toddler Development - Third Edition (BSID 3). Five months later, the cognitive subtest was carried out. The data gathered were then analyzed using SPSS software. RESULTS: The study was performed on 2 groups of 20- to 24-month-old cochlear implant users, and our results confirmed a high correlation between language acquisition and cognitive development (r = 0.76). In addition, the cognitive and language performance of the participants who were trained by the new and specifically designed language intervention protocol as well as AVT was significantly higher than that of the control group (P ≤ .001). CONCLUSION: The new and specifically designed language intervention protocol that was mainly established based on cognitive factors such as attention and semantic memory enhancement in cochlear-implanted children improved not only their language acquisition but also their cognitive development.

Identification of homozygous mutations for hearing loss.

BACKGROUND: Hearing loss is the most common sensory disorder worldwide, affecting about 1 out of every 1000 newborns. The disease has major genetic components, and can be inherited as a single gene disorder either in autosomal dominant or recessive fashions. Due to the high rate of consanguineous unions, Iran has one of the highest prevalence of autosomal recessive nonsyndromic deafness (ARNSD) in the world. METHODS: We carried out a genetic screening of ten Iranian kindreds with more than one offspring affected by ARNSD caused by consanguineous unions. Sanger sequencing and whole exome sequencing together with in silico 3D structure modeling and protein stability prediction were used to identify the underlying disease causing genes. CONCLUSION: We identified the causes of deafness in all 10 kindred. In six kindreds homozygous mutations were identified in GJB2 gene by Sanger sequencing. By using whole exome sequencing (WES), a homozygous missense mutation was identified in ESRRB gene as the first ever reported disease gene in Iran. Also two novel homozygous frameshift and missense mutations were identified in MYO15A gene and one previously reported mutation in TMC1 gene in three independent kindred. Our study shows the efficacy of WES for unraveling new pathogenic mutations in ARNSD patients and expands the spectrum of genes contributing to ARNSD in the Iranian population. The findings of our study can facilitate future genetic screening of patients with ARNSD , early screening and optimal design of novel therapeutics.