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.

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.

Computerized cognitive training for improving cochlear-implanted children's working memory and language skills.

Sensory deprivation, including hearing loss, can affect different aspects of a person's life. Studies on children with hearing impairment have shown that such patients, especially those with cochlear implants (CIs), suffer from cognitive impairments, such as working memory problems and poor language skills. The present study aimed to examine the efficacy of cognitive computer training in improving working memory and language skills in children with a CI. This research was a quasi-experimental study with a pre-test-post-test design and a control group. Fifty-one children with a CI aged 6-12 years were recruited through convenience sampling and randomly assigned to the control and treatment groups. The Wechsler Working Memory Subtest and the Test of Language Development (TOLD) were used to evaluate children's working memory and language skills pre- and post-treatment. The treatment group attended twenty 50-60-minute cognitive computer training sessions three times a week. Sina-Working Memory Training was used to provide the treatment group with working memory training, whereas no intervention was provided to the control group. Univariate and multivariate analyses of covariance were used to analyze data. The results demonstrated the efficacy of cognitive computer training in improving the performance of cochlear-implanted children's working memory (auditory and visual-spatial) (P < 0.01). The results also pointed to improved performance in sentence imitation (P < 0.01), word discrimination (P < 0.01), and phonemic analysis subtests (P < 0.01). Overall, the findings indicated that cognitive computer training might improve working memory and language skills for children with CI. Therefore, the development and execution of such programs for children with CIs seem to improve their cognitive functions, such as working memory and language skills.

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.

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.

Cochlear implantation in children with auditory neuropathy spectrum disorder: A multicenter study on auditory performance and speech production outcomes.

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.

The effects of cochlear implantation on vestibular function in 1-4 years old children.

OBJECTIVES: Although cochlear implants offer an effective hearing restoration option in children with severe to profound hearing loss, concern continues to exist regarding the possible effects of cochlear implantation on the vestibular system and balance. METHODS: In a prospective cohort study, 27 children with bilateral profound hearing loss (all candidates for cochlear implantation) were evaluated for their vestibular function before and after cochlear implantation. Vestibular evaluations consisted of Vestibular Evoked Myogenic Potentials, caloric testing and the Head-Impulse Test. RESULTS: Mean age at the time of cochlear implantation was 27.19 months. Without considering vestibular evaluation results, one of the ears was selected for surgery. Vestibular tests after surgery were not indicative of any statistically significant change in vestibular system or balance. CONCLUSION: This limited data shows that cochlear implantation did not impair the vestibular system of these patients. By the results of our study we may conclude that round window implantation does not have any disturbing impact on vestibular function in children. The generalization of this result needs further research.

Complications in a series of 4400 paediatric cochlear implantation.

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.

Bacterial Meningitis after Cochlear Implantation among Children without Polyvalent Conjugate Vaccine: A Brief Report of an Iranian Cohort Study on 371 Cases.

BACKGROUND: Regarding risk of bacterial meningitis (BM) after Cochlear implantation (CI), it was suggested to receive polyvalent conjugate vaccine. We aimed to estimate the prevalence of BM post CI in child recipients who do not receive polyvalent vaccine. METHODS: We enrolled 371 children who had received cochlear implants from 2007 to 2010. None of them received pre or post implantation polyvalent conjugate vaccine for BM. We followed all of them for BM for 2 years after implantation. RESULTS: We detected only one female case of BM (0.3% of patients) with the age of 24 months. The mean age of noninfected children was 36.7 ± 23.2 months. The education level of parents was "college level or higher" in less than half of them, and about 65% of patients were products of consanguineous marriage. CONCLUSIONS: Our findings indicated that the incidence of BM was not higher in our cochlear implanted children who did not receive immunization than patients from countries in which routine vaccination is done. We suggest that although proper immunization is recommended before surgery, this procedure could be performed without vaccination, especially in developing countries that face financial problems for preparing vaccines.

A model portraying experimental loss of hair cell: the use of distortion product otoacoustic emission in the assessment of rat's ear.

The use of rats in research academies to study deafness is widespread, meanwhile medicinal methods to eliminate hair cells is also increasing. Thus, aminoglycosides and loop diuretics have grasped more attention. This study aimed at establishing an animal model in which a rapid distortion of the hair cell of cochlea administering amikacin and furosemide and using distortion product otoacoustic emission (DPOAE) the functioning of rat's ear would be assessed. Forty-eight male Sprague-Dawley rats (mean weight 200-250g) were randomly divided into six equal groups. Except the control group the rest received 0.5mg/g, 0.75mg/g, 1mg/g, 1.25mg/g, and 1.5mg/g, of subcutaneous amikacin respectively. 30 minutes later every rat received 0.1mg/g of furosemide intrapritoneally. DPOAE of rats was measured before these injections and 72 hours later. Then tissue sections of the rat's cochlea were prepared. All the cases had a significant decrease in their DPOAE with the frequencies 2KHz-8KHz (p<0.05). The most change in DPOAE was in rats which had received 1mg/g - 1.5mg/g amikacin. Histological studies approved distortion of hair cell even the apical turn. To establish a deafness model due losing hair cells, it is possible to use a combination of 1mg/g amikacin and 0.1mg/g furosemide. Besides, to approve deafness DPOAE resulted can be used.

The specificity and sensitivity of transient otoacustic emission in neonatal hearing screening compared with diagnostic test of auditory brain stem response in tehran hospitals.

OBJECTIVE: Since early detection (specially before 6 months of age) of deaf people leads to better hearing and speech outcome after treatment, several clinical trials have been performed in order to find a cost effective, short duration screening test for diagnosis of neonatal hearing impairment. The aim of this study was to assess the sensitivity and specificity of Transient Otoacustic Emission (TEOAE) test in newborns comparing with auditory brain stem response (ABR) in the age of 3 months and to analyze the association between risk factors and hearing loss in neonates. METHODS: A cross-sectional study was conducted January2008 - May 2009 in Tehran. 1000 newborns (526 boys and 474 girls) were assessed. First, all of neonates were evaluated by TEOAE 24h after birth. If responses of OAE were failing, they were retested 10 to 15 days after birth by TEOAE. Also, All Neonates were assessed by ABR in the age of 3 months. Descriptive Statistics was used to analyze data. FINDINGS: Eighteen out of 1000 neonates failed double-checked TEOAE tests, of which 6 were confirmed by ABR test (12 false positive results). Nine out of 1000 neonates had impaired ABR tests, from these patients, 6 had failed OAE as well, but 3 had normal OAE (3 false negative results). From these 9 patients 2 had profound hearing loss and received cochlear implantation. We found that OAE has 66.7% sensitivity and 98.8% specificity in diagnosis of neonatal hearing impairment. Its positive and negative predictive value was 33.3% and 99.7% respectively. Also we did not find statistically significant relationship between hearing loss and risk factors. CONCLUSION: TEOAE as a simple, non-invasive, short duration and cost effective method, is a suitable test for neonatal hearing screening. Even though only two thirds of patients were detected by this method, 99.7% negative predictive value makes it a good screening test. We recommend OAE as a suitable primary neonatal hearing screening all over the country.

Stem cell transplantation in noise induced hearing loss.

OBJECTIVE: To investigate efficacy of bone marrow stem cell implantation in rehabilitation of noise induced hearing loss in rats. MATERIALS AND METHODS: Hearing loss was induced in male rats by a continuous wide-band noise (8-16 kHz/120 dB/120 min). Ten microliter of stem cell containing solution was injected by a Hamilton syringe with 30 G needle through the round window membrane. Hearing status was examined by, distortion product otoacoustic emissions using DP-OAE. Animals were studied in 4 different groups: (1) Normal hearing animals, undergoing sham surgery (no injection done, only round window membrane ruptured and sealed). (2) Deaf animals, undergoing sham surgery. (3) Deaf animals undergoing surgery and injection of solvent (artificial perilymph). (4) Deaf animals undergoing surgery and injection of artificial perilymph containing BMSCs. RESULTS: DP-Gram in rat with normal hearing undergoing sham surgery show that procedure has neither negative impact on normal cochlear nor on deaf cochleas. No significant difference (p=0.25) between ears excludes artificial perilymph as a confounding factor. There is no significant difference between ears in animals receiving BMSCs. CONCLUSIONS: Implanted cells with normal histologic structures have no physiologic function and hearing rehabilitation. Further studies by monitoring the survival of these cells with histologic and appropriate biomarkers will help to investigate differentiation process of these cells.

Bilateral Cochlear Implantation in Meningitis, Is it Mandatory? A Case Report.

These days cochlear implantation is the accepted modality to rehabilitate deafened people. Meningitis is still a life threatening disease which may lead to deafness due to sole disease or secondary to ototoxic drugs used to stop the disaster [1]. Sepsis and/or meningitis may harm neonates whom are taking care in nurseries. TEOAE neonatal hearing screening programs are unable to find all of these deafened children and ABR would be necessary to explore most of them [2].Cochlear implantation should be performed as soon as possible and before complete ossification of cochlea.

Late laryngeal findings in sulfur mustard poisoning.

INTRODUCTION: Sulfur mustard is an alkylating warfare agent and its inhalation results in early and late toxic effects. This agent was used widely against both military and civilian population by Iraqi forces in the Iran-Iraq war (1983-88). The purpose of the study was to describe abnormal laryngeal findings in a group of chemical war injury patients (soldiers) 20 years after an acute exposure to sulfur mustard. METHODS: In this observational case series study, 50 male patients who were suffering from chronic respiratory symptoms and because of exacerbation of these problems, were consecutively selected and clinically examined for dysphonia by a speech-language pathologist, and then underwent fiberoptic laryngobronchoscopy to describe anatomical and functional pathologic findings of the larynx. RESULTS: In objective speech evaluation, different degrees of dysphonia including harshness and hoarseness were observed in 46% of patients. Inflammation was present in supraglottic and subglottic regions of 9 (18%) and 3 (6%) patients, respectively. Hyperfunction of the false vocal cords was observed in 24 (48%) patients. CONCLUSION: Chronic laryngitis was seen in a considerable number of exposed victims nearly 20 years after exposure, in spite of different treatments received.

Use of laser for dacrocystorhinostomy.

OBJECTIVE: To compare outcomes between conventional external dacrocystorhinostomy (ext DCR) and endonasal laser-assisted DCR (ELADCR). DESIGN: Prospective randomized trial. PATIENTS: The study included 210 consecutive patients (244 eyes) referred to hospital eye and ear, nose, and throat clinics. MAIN OUTCOME MEASURES: Success rates and complications of ext DCR and ELADCR were compared after lacrimal ducts requiring DCR were randomly chosen and divided into 2 groups (ext DCR and ELADCR). RESULTS: The success rate was statistically equal in both groups (92.4% for ext DCR and 94.2% for ELADCR); however, morbidity (eg, intraoperative hemorrhage and wound scar) and operation time were less in the ELADCR group. CONCLUSION: Preoperative patient consultation for selection of the surgical modality may help select the procedure of choice for each patient with regard to aesthetics, anesthesia, operation time, and costs.

Impact of body mass index (BMI) on ventilation during low-frequency jet ventilation.

OBJECTIVE: Percutaneous trans-tracheal jet ventilation (PTJV) is an alternative ventilatory approach in airway surgery. We evaluated the effects of body mass index (BMI) on ventilation during low-frequency jet ventilation. STUDY DESIGN AND SETTING: Forty-two patients undergoing micro-laryngeal surgery under total anesthesia were studied. Low-frequency jet ventilation was applied through an injector inserted into the trachea via cricothyroid membrane; ventilation was assessed during the operation by arterial blood gas sampling. RESULT: The age range of the patients was 43.54 +/- 12.04 years, weight was 69.97 +/- 11.66 kg, and BMI 24.80 +/- 2.78 (mean +/- SD). There was a strong correlation (P < 0.05) and a good correspondence between the BMI and mean PaCO(2), and arterial pH. CONCLUSION AND SIGNIFICANCE: This method was effective in maintaining gas exchange in the presence of micro-laryngeal surgery for low-BMI patients. It provided a nice visible surgical field, avoiding the use of combustible material inside the larynx or trachea.

Experience with surgical management of cholesteatomas.

OBJECTIVE: To evaluate long-term results after at least 10 years of follow-up for a common method of ear surgery for managing cholesteatomas. METHODS: This retrospective study was performed by assessing the medical records of adult patients who underwent canal-wall-down mastoidectomy for management of cholesteatomas. RESULTS: From January 1, 1989, through December 31, 1995, 148 patients underwent surgery for cholesteatoma. Of these, 72 (48%) were treated by using the canal-wall-down method. During follow-up (10-16 years), 5 (7%) patients underwent at least 1 revision operation. Ten years after primary surgery, 69 (96%) of the ears were dry and 55 (76%) had intact tympanic membranes with fair hearing in 30 (42%). One patient who was deaf was seen in this group of 72 patients. The recurrence rate was 7%, independent of the size of the cholesteatoma, mastoid status, or labyrinth or footplate erosion. The major predisposing factor for recurrence was retraction, especially in the posterior superior quadrant. CONCLUSION: Our results showed that the surgical technique could be improved by using endoscopes to lower the recurrence rate and improve hearing results.