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.

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.

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.

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.

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.

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.

Bipolar loop device versus bipolar diathermy for tonsillectomy: A pilot randomized controlled trial.

OBJECTIVE: Tonsillectomy is one of the most common surgeries performed in the pediatric population. Although different forms of instruments and various methods are used to perform tonsillectomy, none of them is still recognized as the best global technique. This study aimed to compare the outcomes of the new Bipolar Loop tonsillectomy versus bipolar diathermy technique. METHODS: This study is a pilot randomized clinical trial and was conducted on 40 pediatric patients who were the candidate of tonsillectomy. Patients were divided into two groups of Bipolar Loop or bipolar diathermy. Operation time, intraoperative bleeding, tonsillar fossa wound, postoperative complications, and duration of return to normal diet were evaluated in the current study. RESULTS: In both of the Bipolar Loop and bipolar diathermy groups, no significant difference was found in terms of sex, age, and weight. The average amount of the operative time, intra-operative blood loss, and postoperative pain loss were significantly less in the Bipolar Loop group (P < .001). In addition, the tonsillar fossa wound healing scores were significantly better (on the 14th day, P = 0.009). However, there was no significant difference between the two groups in terms of postoperative bleeding, duration of return to a normal diet, and postoperative symptoms of fever, otalgia, or voice change between the groups. CONCLUSION: The study showed that blood loss and postoperative pain through day 7 were significantly less in the Bipolar Loop group. Tonsillectomy with Bipolar Loop can be recommended as one of the methods for tonsillectomy, especially in pediatric patients.

Vowel duration measurement in school-age children with cochlear implant.

OBJECTIVES: Normal hearing system is an essential factor for accurate production of speech segments. It seems that cochlear implant prosthesis helps children with hearing impairment to speak more accurately than before receiving prosthesis. The current research aimed to compare the vowel duration in school-aged children with cochlear implants and that in children with normal hearing. Additionally, the performance of girls and boys in two groups were investigated. METHODS: A cross-sectional and descriptive-analytical study was carried out to compare the vowel duration in 9- to 12-year-old children with cochlear implant and those with normal hearing. Participants were 52 children who were matched by age and sex. We asked the children to read the target words with each word including one vowel and then participants' voice samples were recorded. Then, vowel duration was measured using Praat software. Finally, the vowel duration was compared between the two groups running independent sample t-test. The level of significance was (P < 0.05). RESULTS: There was no statistically significant difference observed between the two groups in the mean values of the vowel duration for the six Persian vowels (P > 0.05). Also, no significant difference was found between boys and girls in the mean value of the vowel duration between the two groups (P > 0.05). CONCLUSION: Persian vowels in school-aged children with cochlear implant and with typical hearing are produced with similar durations. This finding is probably related to the increased duration of using prosthesis in this age range and speech mode used to measure vowel duration in the present study.

Cerebrospinal fluid gusher in cochlear implant and its associated factors.

Introduction: Cochlear implants (CI) is considered a safe procedure with overall complication rate of 12.5% amongst adult and pediatric population. Cerebrospinal fluid (CSF) gusher is regarded as a common CI intraoperative complication.Objective: In this study, we determined the association between probable associated factors and occurrence of intraoperative CSF gusher.Method: In a retrospective survey, 394 patients with severe to profound sensorineural hearing loss who underwent primary cochlear implantation surgery were evaluated. Patients with incomplete electronic or manual file and those with revision, explantation or reimplantation surgery were excluded.Result: three hundred and ninety-four CI patients with a mean age of 8.74 ± 12.21 years were reviewed 49.62% of them were female. CSF gusher developed in 22 patients (5.58%). Patients with CSF gusher had more structural abnormalities in their CT scans' report. Common cavity malformation and Mondini dysplasia were the most common abnormal reports amongst those with intra-operative CSF gusher.Conclusion: In conclusion, cochlea structural abnormalities affect the incidence of CSF gusher in CI recipients. Common cavity malformation and Mondini dysplasia were associated with an increased incidence of CSF gusher. We insist on more precise pre-operative imaging of those with abnormal cochlea structures to provide the needed management.

Cochlear implantation in children with inner ear malformation: A multicenter study on auditory performance and speech production outcomes.

OBJECTIVES: This multicenter study evaluated the auditory performance and speech production outcomes of cochlear implantation in children with inner ear anomaly and compared the outcomes of patients with different kinds of malformation. METHODS: Cochlear implantation was performed in 107 children with inner ear malformation at four tertiary academic centers. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated preoperatively and one year and two years postoperatively. RESULTS: Types of inner ear malformation and their frequencies were: incomplete partition type-I, 19 (17.8%) patients; incomplete partition type-II, 31 (29%), common cavity, 17 (15.9%), cochlear hypoplasia, 17 (15.9%), and isolated enlarged vestibular aqueduct (isolated EVA), 23 (21.5%) patients. EVA was the coexisting anomaly in 27(25.2%) subjects. The median CAP and SIR scores improved significantly during the first two years after cochlear implantation in all groups (p-values <0.001 and < 0.001, respectively). No significant difference was seen in CAP and SIR scores of children with different inner ear malformations (p-value = 0.147 and 0.570, respectively) or in patients with isolated EVA compared to coexisting EVA (p-value = 0.538 and 0.075, respectively). CONCLUSION: The median CAP and SIR scores two years after surgery were 5 (Understanding of common phrases without lip-reading) IQR: 4-6, and 3 (Connected speech is intelligible to a listener who concentrates and lip-reads within a known context) IQR: 3-4, respectively. Auditory performance and speech production were significantly improved in all inner ear malformation patient groups, and no significant difference was observed between the scores of patients with different types of anomaly.

Cochlear implant outcomes in children with attention-deficit/hyperactivity disorder: Comparison with controls.

OBJECTIVES: The coincidence of attention-deficit/hyperactivity disorder (ADHD) and hearing loss in the children has adverse effects in speech, cognition, communication and motor development. This can influence cochlear implant (CI) outcomes negatively. The aim of this study was to compare auditory, language, speech, cognition, communication and motor outcomes between CI children with versus without ADHD. METHODS: Nineteen CI children with ADHD and twenty-three age and gender matched children without ADHD at the Shiraz CI center ranging in age from 37 to 60 months were participated in this cross-sectional study. The developmental quotient in auditory, receptive and expressive language, speech and cognition skills was evaluated through Newsha Developmental Scale. The Persian version of the Ages and Stages Questionnaire (ASQ) was used to assess children's developmental status in fine and gross movements, communication, problem solving, and personal-social domains. A comparison of the results between two groups was made by the Mann-Whitney test. RESULTS: CI children with ADHD had significantly lower Newsha developmental quotients in cases of auditory, receptive and expressive language, speech and cognition skills compared to the control group (P = 0.027 to <0.001). A significant difference was observed between children with and without ADHD in fine and gross movements, communication, problem solving, and personal-social domains of ASQ at 60 months (P = 0.029 to 0.003). CONCLUSION: Children with ADHD showed decreased ability in auditory, language, speech, cognition, motor and communication skills following CI compared to children without ADHD. It can guide clinician to provide these children with more specific rehabilitation program to improve their skills.

Identification of novel microsatellite markers flanking GJB2 gene in order to use in preimplantation genetic diagnosis of hearing loss: A comparison of whole-genome amplification and semi-nested PCR.

Hearing loss is the most prevalent sensorineural disorder which can be caused by genetic factors in more than half of the cases. GJB2 mutations with the frequency of 18.7% are the most common cause of autosomal recessive non-syndromic hearing loss (ARNSHL) in the Iranian population. The aim of the current study was to genotype 100 healthy individuals for eight microsatellite markers flanking the GJB2 gene, and to study markers on ten blastomeres using semi-nested PCR and Whole-genome amplification (WGA). All microsatellite markers within 1 Mb flanking the GJB2 gene were identified. From the identified markers, four with potentially high heterozygosity values were selected. The heterozygosity indices of four newly discovered markers and four previously reported markers were calculated. The markers and the GJB2 gene were also validated on single lymphocytes and blastomeres. Totally, 77 alleles were observed in eight loci. D13S046 showed the highest polymorphism and D13S141 showed the lowest. The observed heterozygosities of all markers, except D13S141, were higher than 50%. All single cells were genotyped successfully by the two techniques. Our findings indicate a high degree of polymorphism of the selected markers. Due to the high rate of successful amplification of markers in all ten blastomeres and the low level of allelic drop out (ADO), a combination of these eight microsatellite markers in conjunction with direct mutation detection is suggested for performing preimplantation genetic diagnosis (PGD) of hearing loss due to GJB2 mutations.

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.

The effectiveness of a specifically-designed language intervention protocol on the cochlear implanted children's communication development.

OBJECTIVE: Although various studies have depicted the importance of language intervention programs in facilitating language acquisition in cochlear implanted children, to this date rarely has a specific language intervention approach been recommended as better than the other and no best practice has been introduced in terms of language acquisition outcomes. Thus, the therapists remain uncertain as to which approach to follow and how to apply evidence to practice. Hence, the main goal of this study was to take a step in this regard by comparing the communication development of pediatric cochlear implant users who enrolled in two different language intervention approaches: the routine auditory-verbal approach, and the routine auditory-verbal approach plus a new intervention protocol specifically designed to enhance receptive vocabulary development in cochlear implanted children. METHOD AND MATERIALS: This prospective experimental study compared the receptive and expressive communication developmental scores of 26 cochlear implanted 20-24 months old children who received both a routine auditory-verbal intervention and a new cognitive based intervention protocol specifically designed to enhance receptive vocabulary development, with that of a group of 25 participants whose intervention program was only auditory-verbal. The children were recruited from Fars cochlear implant center situated in the city of Shiraz in Iran, and were assigned randomly to the two groups. The communication development of both groups was evaluated by the Bayley scales of Infant and Toddler Development- Third Edition, and statistical analysis was performed using the Statistical Package for Social Sciences-version 21(SPSS-21). RESULTS: The two groups were not significantly different in terms of age, sex and parents' educational level. Both the receptive and expressive communication outcomes of the children who received auditory-verbal as well as the new specifically-designed cognitive-based intervention protocol focusing on receptive vocabulary enhancement, were significantly higher than the control group (p < 0.001). CONCLUSION: The new specifically-designed cognitive-based language intervention protocol focusing on receptive vocabulary enhancement was significantly more effective in promoting and enhancing the communication development of cochlear implanted children than the routine auditory-verbal intervention.