Background Transmembrane inner ear (TMIE) protein is an essential component of the mechanotransdu... more Background Transmembrane inner ear (TMIE) protein is an essential component of the mechanotransduction complex. In collaboration with other components, TMIE aids the maintenance and function of the sensory hair cells. Autosomal recessive deafness-6 (DFNB6) is caused by mutated TMIE, a gene in the high genetic heterogeneity spectrum of deafness. Hearing loss has a significant impact on the global economy and the quality of life of affected persons, their families, and society. Here, three unrelated families with TMIE variants are presented. All three cases were found while studying the genetic causes of an Iranian cohort of subjects with cochlear implants. Methods Whole exome sequencing was performed to find possible genetic etiology in probands of families after a comprehensive medical evaluation for hearing loss. Co-segregation analysis in probands and other family members was performed by Sanger sequencing. The variants were interpreted per the American College of Medical Genetics...
Objectives: Age-related hearing impairment, or presbycusis, is the most common communication diso... more Objectives: Age-related hearing impairment, or presbycusis, is the most common communication disorder and neurodegenerative disease in the elderly. Its prevalence is expected to increase, due to the trend of growth of the elderly population. The current diagnostic test for detection of presbycusis is implemented after there has been a change in hearing sensitivity. Identification of a pre-diagnostic biomarker would raise the possibility of preserving hearing sensitivity before damage occurs. Mitochondrial dysfunction, including the production of reactive oxygen species and induction of expression of apoptotic genes, participates in the progression of presbycusis. Mitochondrial DNA sequence variation has a critical role in presbycusis. However, the nature of the relationship between mitochondrial DNA copy number, an important biomarker in many other diseases, and presbycusis is undetermined. Methods: Fifty-four subjects with presbycusis and 29 healthy controls were selected after ear, nose, throat examination and pure-tone audiometry. DNA was extracted from peripheral blood samples. The copy number of mitochondrial DNA relative to the nuclear genome was measured by quantitative real-time polymerase chain reaction. Results: Subjects with presbycusis had a lower median mitochondrial DNA copy number than healthy subjects and the difference was statistically significant (P=0.007). Mitochondrial DNA copy number was also significantly associated with degree of hearing impairment (P=0.025) and audiogram configuration (P=0.022). Conclusion: The findings of this study suggest that lower mitochondrial DNA copy number is responsible for presbycusis through alteration of mitochondrial function. Moreover, the significant association of mitochondrial DNA copy number in peripheral blood samples with the degree of hearing impairment and audiogram configuration has potential for use as a standard test for presbycusis, providing the possibility of the development of an easy-to-use biomarker for the early detection of this condition.
Waardenburg syndrome (WS) is a disease of abnormal neural-crest derived melanocyte development ch... more Waardenburg syndrome (WS) is a disease of abnormal neural-crest derived melanocyte development characterized by hearing loss and pigmentary disturbances in hair, eyes and skin. WS is subdivided into four major types, WS1-WS4, where WS1 is recognized by the presence of dystopia canthorum, with PAX3 being the only known gene involved. This study aimed at investigating PAX3 mutations and clinical characteristics of WS1 in a group of Iranian patients. A total of 12 WS1 patients from four unrelated Iranian families were enrolled. Waardenburg consortium guidelines were used for WS1 diagnosis. A detailed family history was traced and a thorough clinical examination was performed for all participants. Furthermore, WS1 patients underwent screening for PAX3 mutations using PCR-sequencing. Dystopia canthorum, broad high nasal root and synophrys were observed in all patients. Early graying, hair discoloration, hypoplastic blue eyes (characteristic brilliant blue iris) and hearing loss were the most common features observed, while heterochromia iridis was the least frequently observed sign among the studied Iranian WS1 patients. Genetic analysis of PAX3 revealed four mutations including c.667C>T, c.784C>T, c.951delT and c.451+3A>C. Two of the four mutations reported here (c.951delT and c.451+3A>C) are being reported for the first time in this study. Our data provide insight into genotypic and phenotypic spectrum of WS1 in an Iranian series of patients. Our results expand the spectrum of PAX3 mutations and may have implications for the genetic counseling of WS in Iran.
For describing structure and quality of spirit in traditional medicine sources and achieving a co... more For describing structure and quality of spirit in traditional medicine sources and achieving a common language between today and past knowledge, we search traditional medicine sources in Islamic era (dark ages in European history). In this way Avicenna's notes was our main source. This study presents a comparison between some spirit replacement candidates and spirit characteristics in Canon on Medicine. Traditional medicine in Islamic era follows both ancient Egyptians' belief in immortal soul and Aristotle's belief in material quality of soul. Islamic scientists divided it to two parts. Material part (known as medical spirit) acts as a connector of immortal part (soul) to body. This material part initially is produced in heart and is known as vital spirit. That is origin of other spirits. Similarity of vital spirit to heart electrical activities is more prominent than the other equal candidates. This can leads us to a new understanding of spirit. Keywords:spirit – tradi...
Waardenburg syndrome (WS) is a disease of abnormal neural-crest derived melanocyte development ch... more Waardenburg syndrome (WS) is a disease of abnormal neural-crest derived melanocyte development characterized by hearing loss and pigmentary disturbances in hair, eyes and skin. WS is subdivided into four major types, WS1-WS4, where WS1 is recognized by the presence of dystopia canthorum, with PAX3 being the only known gene involved. This study aimed at investigating PAX3 mutations and clinical characteristics of WS1 in a group of Iranian patients. A total of 12 WS1 patients from four unrelated Iranian families were enrolled. Waardenburg consortium guidelines were used for WS1 diagnosis. A detailed family history was traced and a thorough clinical examination was performed for all participants. Furthermore, WS1 patients underwent screening for PAX3 mutations using PCR-sequencing. Dystopia canthorum, broad high nasal root and synophrys were observed in all patients. Early graying, hair discoloration, hypoplastic blue eyes (characteristic brilliant blue iris) and hearing loss were the most common features observed, while heterochromia iridis was the least frequently observed sign among the studied Iranian WS1 patients. Genetic analysis of PAX3 revealed four mutations including c.667C>T, c.784C>T, c.951delT and c.451+3A>C. Two of the four mutations reported here (c.951delT and c.451+3A>C) are being reported for the first time in this study. Our data provide insight into genotypic and phenotypic spectrum of WS1 in an Iranian series of patients. Our results expand the spectrum of PAX3 mutations and may have implications for the genetic counseling of WS in Iran.
International Journal of Pediatric Otorhinolaryngology, 2011
We also aimed to document the development of auditory perception in these patients. Material and ... more We also aimed to document the development of auditory perception in these patients. Material and Methods: We examined the records of 915 cochlearimplanted, prelingually deaf patients who had received a cochlear implant at least one year previously. Results: A total of 125 cochlear-implanted, prelingually deaf patients were diagnosed with additional disabilities. All patients showed significant development in speech perception, except for autistic and congenitally deaf-blind patients. Conclusion: Although cochlear implantation is not contraindicated in prelingually deaf persons with additional disabilities, congenitally deaf-blind and autistic patients showed limited development in auditory perception as a main outcome of cochlear implantation.
International Journal of Pediatric Otorhinolaryngology, 2011
We also aimed to document the development of auditory perception in these patients. Material and ... more We also aimed to document the development of auditory perception in these patients. Material and Methods: We examined the records of 915 cochlearimplanted, prelingually deaf patients who had received a cochlear implant at least one year previously. Results: A total of 125 cochlear-implanted, prelingually deaf patients were diagnosed with additional disabilities. All patients showed significant development in speech perception, except for autistic and congenitally deaf-blind patients. Conclusion: Although cochlear implantation is not contraindicated in prelingually deaf persons with additional disabilities, congenitally deaf-blind and autistic patients showed limited development in auditory perception as a main outcome of cochlear implantation.
... IN THE THREE MONTHS AFTER RECEIVING THE DEVICE IN CHILDREN WITH COCHLEAR IMPLANT POURJAVID AR... more ... IN THE THREE MONTHS AFTER RECEIVING THE DEVICE IN CHILDREN WITH COCHLEAR IMPLANT POURJAVID AR*,ADEL GHAHRAMAN M.,SEDAIE ... receiving the device with approximately one-month intervals in all the children who were implanted in the Amir Alam and ...
Aim: To present the outcomes of cochlear implantation (CI) in children with auditory neuropathy s... more Aim: To present the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorders (ANSD). Materials and methods: The pre-and post-CI hearing outcomes in children with ANSD were retrospectively evaluated. Performance was assessed with categories of auditory performance (CAP) and the Manchester spoken language development scale (MSLDS). Results: Full data were available in 27 implanted children with ANSD with average age at implantation 35.4 months (range 19-68 months). Nine children were implanted bilaterally, while 13 were bimodal. The pre-CI CAP and MSLDS scores were 2.5 (range 0-5) and 2.5 (range 0-6), while the post-CI scores 5.8 (range 2-9) and 7.7 (range 3-10), respectively. Conclusions: Although the outcome of CI in children with ANSD might vary, it is favourable in most of the cases. CI seems a justified hearing rehabilitation option for children with ANSD and limited benefits from conventional hearing aids.
Background and Aim: Sound processors in cochlear implant (CI) cannot encode low frequency informa... more Background and Aim: Sound processors in cochlear implant (CI) cannot encode low frequency information and discard much of the temporal fine structure required to perceive fundamental frequency. Hearing aids can transmit low frequency information, which is important for pitch perception and provides many advantages for the users. This study aimed to compare aural/oral performance of bimodal cochlear implants with unilateral ones in children using parents' evaluation of aural/oral performance of children (PEACH) questionnaire. Methods: Twenty children with unilateral cochlear implant and 20 ones with bimodal cochlear implants were selected for this study. Of them, 23 had cochlear devices, 10 possessed Med-El ones, and 7 wore advanced bionics ones. Bimodal group had at least 7 months of hearing experience with digital hearing aid in non-implanted ear. In order to compare the aural/oral performance in these groups, we used the PEACH questionnaire. Results: In unilateral and bimodal ...
International Journal of Pediatric Otorhinolaryngology, 2011
Aim: Finding surgical complications of cochlear implantation and their prevalence among patients ... more Aim: Finding surgical complications of cochlear implantation and their prevalence among patients undergoing surgery so we can pursue methods for preventing and treating these complications. Methods and Materials: A cross-sectional study of 1487 cochlearimplanted patients who had received a cochlear implant during 1991-2009. All Aetiologies of deafness, surgical complications (intra-operative and postoperative) were registered and analyzed. Results: Complications occurred in 120 patients (8%).Two most common surgical complications were device failure (5.04%) and Gusher (1.34%). Another complications were facial paresis (0.4%); Device extrusion (0.2%), flap necrosis (0.33%); Meningitis (0.13%); sensitivity to prosthesis (0.13%); Device break (0.13%); Electrode extrusion (0.2%); Incorrect device positioning (0.067%) and wound dehiscence (0.067%). Conclusion: Despite this fact that cochlear implantation is a procedure with minimal complications, but precise preoperative evaluation of patients results in better information about structural anomalies and prevention of unwanted adverse effects. These evaluations include preoperative Imaging and also considering administration of Proper antibiotic for preventing infectious complications.
Background: Auditory scene analysis (ASA) is the process by which the auditory system separates i... more Background: Auditory scene analysis (ASA) is the process by which the auditory system separates individual sounds in natural-world situations. ASA is a key function of auditory system, and contributes to speech discrimination in noisy backgrounds. It is known that sensorineural hearing loss (SNHL) detrimentally affects auditory function in complex environments, but relatively few studies have focused on the influence of SNHL on higher level processes which are likely involved in auditory perception in different situations.
The use of cochlear implantation to treat patients with inner ear malformations such as Mondini d... more The use of cochlear implantation to treat patients with inner ear malformations such as Mondini dysplasia has been increasingly successful. Until now, conventional hearing aids in these patients have not performed well. Consequently, the hearing problem for patients with this condition has been somewhat improved with the use of cochlear implants. Various results of cochlear implantation have been reported in these patients so far. This is a report of 5 patients with Mondini malformation who have undergone cochlear implant surgery.
One of the most important factors in auditory speech perception of cochlear-implanted children is... more One of the most important factors in auditory speech perception of cochlear-implanted children is age. The goal of this study was to compare auditory speech perception among children implanted from 6 different age groups: 0 to 3, 4 to 5, 6 to 7, 8 to 9, 10 to 11, and >12 years. The subjects of this study were matched based on socioeconomic status, residual hearing before cochlear implantation, the kinds of cochlear implant device, speech processing strategy, communication mode after implantation, and primary language in family. All of them have used the device minimally for 2 years. The subjects were tested with a range of closed and open-set auditory speech perception tests, and the levels of auditory speech perception in different age groups were compared. Results showed that the children who received an implant at 0 to 3 years of age had maximum auditory speech perception.
To investigate the prevalence of mutations in the coding exon of the GJB2 gene in Iranian childre... more To investigate the prevalence of mutations in the coding exon of the GJB2 gene in Iranian children with cochlear implants, and to compare the outcomes of auditory perception and speech production in cochlear-implanted children with and without GJB2 mutation. One hundred and sixty-six prelingually deaf children who had undergone cochlear implantation at the Iranian Cochlear Implant Center, Tehran, were selected from a pool of 428 implanted children. The prevalence of GJB2 gene mutations was assessed using nested polymerase chain reaction and direct sequencing. To enable comparisons, we also identified 36 implanted children with non-GJB2 deafness. Patients' speech perception and speech production were assessed using the Categorization of Auditory Performance and Speech Intelligibility Rating scales. Thirty-three of 166 probands (19.9 per cent) were found to have GJB2 deafness-causing allele variants and were diagnosed with DFNB1 deafness. Results also indicated a significant improvement in speech perception and production scores in both GJB2 and non-GJB2 patients over time. Children with GJB2-related deafness benefit from cochlear implantation to the same extent as those with non-GJB2-related deafness.
Background Transmembrane inner ear (TMIE) protein is an essential component of the mechanotransdu... more Background Transmembrane inner ear (TMIE) protein is an essential component of the mechanotransduction complex. In collaboration with other components, TMIE aids the maintenance and function of the sensory hair cells. Autosomal recessive deafness-6 (DFNB6) is caused by mutated TMIE, a gene in the high genetic heterogeneity spectrum of deafness. Hearing loss has a significant impact on the global economy and the quality of life of affected persons, their families, and society. Here, three unrelated families with TMIE variants are presented. All three cases were found while studying the genetic causes of an Iranian cohort of subjects with cochlear implants. Methods Whole exome sequencing was performed to find possible genetic etiology in probands of families after a comprehensive medical evaluation for hearing loss. Co-segregation analysis in probands and other family members was performed by Sanger sequencing. The variants were interpreted per the American College of Medical Genetics...
Objectives: Age-related hearing impairment, or presbycusis, is the most common communication diso... more Objectives: Age-related hearing impairment, or presbycusis, is the most common communication disorder and neurodegenerative disease in the elderly. Its prevalence is expected to increase, due to the trend of growth of the elderly population. The current diagnostic test for detection of presbycusis is implemented after there has been a change in hearing sensitivity. Identification of a pre-diagnostic biomarker would raise the possibility of preserving hearing sensitivity before damage occurs. Mitochondrial dysfunction, including the production of reactive oxygen species and induction of expression of apoptotic genes, participates in the progression of presbycusis. Mitochondrial DNA sequence variation has a critical role in presbycusis. However, the nature of the relationship between mitochondrial DNA copy number, an important biomarker in many other diseases, and presbycusis is undetermined. Methods: Fifty-four subjects with presbycusis and 29 healthy controls were selected after ear, nose, throat examination and pure-tone audiometry. DNA was extracted from peripheral blood samples. The copy number of mitochondrial DNA relative to the nuclear genome was measured by quantitative real-time polymerase chain reaction. Results: Subjects with presbycusis had a lower median mitochondrial DNA copy number than healthy subjects and the difference was statistically significant (P=0.007). Mitochondrial DNA copy number was also significantly associated with degree of hearing impairment (P=0.025) and audiogram configuration (P=0.022). Conclusion: The findings of this study suggest that lower mitochondrial DNA copy number is responsible for presbycusis through alteration of mitochondrial function. Moreover, the significant association of mitochondrial DNA copy number in peripheral blood samples with the degree of hearing impairment and audiogram configuration has potential for use as a standard test for presbycusis, providing the possibility of the development of an easy-to-use biomarker for the early detection of this condition.
Waardenburg syndrome (WS) is a disease of abnormal neural-crest derived melanocyte development ch... more Waardenburg syndrome (WS) is a disease of abnormal neural-crest derived melanocyte development characterized by hearing loss and pigmentary disturbances in hair, eyes and skin. WS is subdivided into four major types, WS1-WS4, where WS1 is recognized by the presence of dystopia canthorum, with PAX3 being the only known gene involved. This study aimed at investigating PAX3 mutations and clinical characteristics of WS1 in a group of Iranian patients. A total of 12 WS1 patients from four unrelated Iranian families were enrolled. Waardenburg consortium guidelines were used for WS1 diagnosis. A detailed family history was traced and a thorough clinical examination was performed for all participants. Furthermore, WS1 patients underwent screening for PAX3 mutations using PCR-sequencing. Dystopia canthorum, broad high nasal root and synophrys were observed in all patients. Early graying, hair discoloration, hypoplastic blue eyes (characteristic brilliant blue iris) and hearing loss were the most common features observed, while heterochromia iridis was the least frequently observed sign among the studied Iranian WS1 patients. Genetic analysis of PAX3 revealed four mutations including c.667C>T, c.784C>T, c.951delT and c.451+3A>C. Two of the four mutations reported here (c.951delT and c.451+3A>C) are being reported for the first time in this study. Our data provide insight into genotypic and phenotypic spectrum of WS1 in an Iranian series of patients. Our results expand the spectrum of PAX3 mutations and may have implications for the genetic counseling of WS in Iran.
For describing structure and quality of spirit in traditional medicine sources and achieving a co... more For describing structure and quality of spirit in traditional medicine sources and achieving a common language between today and past knowledge, we search traditional medicine sources in Islamic era (dark ages in European history). In this way Avicenna's notes was our main source. This study presents a comparison between some spirit replacement candidates and spirit characteristics in Canon on Medicine. Traditional medicine in Islamic era follows both ancient Egyptians' belief in immortal soul and Aristotle's belief in material quality of soul. Islamic scientists divided it to two parts. Material part (known as medical spirit) acts as a connector of immortal part (soul) to body. This material part initially is produced in heart and is known as vital spirit. That is origin of other spirits. Similarity of vital spirit to heart electrical activities is more prominent than the other equal candidates. This can leads us to a new understanding of spirit. Keywords:spirit – tradi...
Waardenburg syndrome (WS) is a disease of abnormal neural-crest derived melanocyte development ch... more Waardenburg syndrome (WS) is a disease of abnormal neural-crest derived melanocyte development characterized by hearing loss and pigmentary disturbances in hair, eyes and skin. WS is subdivided into four major types, WS1-WS4, where WS1 is recognized by the presence of dystopia canthorum, with PAX3 being the only known gene involved. This study aimed at investigating PAX3 mutations and clinical characteristics of WS1 in a group of Iranian patients. A total of 12 WS1 patients from four unrelated Iranian families were enrolled. Waardenburg consortium guidelines were used for WS1 diagnosis. A detailed family history was traced and a thorough clinical examination was performed for all participants. Furthermore, WS1 patients underwent screening for PAX3 mutations using PCR-sequencing. Dystopia canthorum, broad high nasal root and synophrys were observed in all patients. Early graying, hair discoloration, hypoplastic blue eyes (characteristic brilliant blue iris) and hearing loss were the most common features observed, while heterochromia iridis was the least frequently observed sign among the studied Iranian WS1 patients. Genetic analysis of PAX3 revealed four mutations including c.667C>T, c.784C>T, c.951delT and c.451+3A>C. Two of the four mutations reported here (c.951delT and c.451+3A>C) are being reported for the first time in this study. Our data provide insight into genotypic and phenotypic spectrum of WS1 in an Iranian series of patients. Our results expand the spectrum of PAX3 mutations and may have implications for the genetic counseling of WS in Iran.
International Journal of Pediatric Otorhinolaryngology, 2011
We also aimed to document the development of auditory perception in these patients. Material and ... more We also aimed to document the development of auditory perception in these patients. Material and Methods: We examined the records of 915 cochlearimplanted, prelingually deaf patients who had received a cochlear implant at least one year previously. Results: A total of 125 cochlear-implanted, prelingually deaf patients were diagnosed with additional disabilities. All patients showed significant development in speech perception, except for autistic and congenitally deaf-blind patients. Conclusion: Although cochlear implantation is not contraindicated in prelingually deaf persons with additional disabilities, congenitally deaf-blind and autistic patients showed limited development in auditory perception as a main outcome of cochlear implantation.
International Journal of Pediatric Otorhinolaryngology, 2011
We also aimed to document the development of auditory perception in these patients. Material and ... more We also aimed to document the development of auditory perception in these patients. Material and Methods: We examined the records of 915 cochlearimplanted, prelingually deaf patients who had received a cochlear implant at least one year previously. Results: A total of 125 cochlear-implanted, prelingually deaf patients were diagnosed with additional disabilities. All patients showed significant development in speech perception, except for autistic and congenitally deaf-blind patients. Conclusion: Although cochlear implantation is not contraindicated in prelingually deaf persons with additional disabilities, congenitally deaf-blind and autistic patients showed limited development in auditory perception as a main outcome of cochlear implantation.
... IN THE THREE MONTHS AFTER RECEIVING THE DEVICE IN CHILDREN WITH COCHLEAR IMPLANT POURJAVID AR... more ... IN THE THREE MONTHS AFTER RECEIVING THE DEVICE IN CHILDREN WITH COCHLEAR IMPLANT POURJAVID AR*,ADEL GHAHRAMAN M.,SEDAIE ... receiving the device with approximately one-month intervals in all the children who were implanted in the Amir Alam and ...
Aim: To present the outcomes of cochlear implantation (CI) in children with auditory neuropathy s... more Aim: To present the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorders (ANSD). Materials and methods: The pre-and post-CI hearing outcomes in children with ANSD were retrospectively evaluated. Performance was assessed with categories of auditory performance (CAP) and the Manchester spoken language development scale (MSLDS). Results: Full data were available in 27 implanted children with ANSD with average age at implantation 35.4 months (range 19-68 months). Nine children were implanted bilaterally, while 13 were bimodal. The pre-CI CAP and MSLDS scores were 2.5 (range 0-5) and 2.5 (range 0-6), while the post-CI scores 5.8 (range 2-9) and 7.7 (range 3-10), respectively. Conclusions: Although the outcome of CI in children with ANSD might vary, it is favourable in most of the cases. CI seems a justified hearing rehabilitation option for children with ANSD and limited benefits from conventional hearing aids.
Background and Aim: Sound processors in cochlear implant (CI) cannot encode low frequency informa... more Background and Aim: Sound processors in cochlear implant (CI) cannot encode low frequency information and discard much of the temporal fine structure required to perceive fundamental frequency. Hearing aids can transmit low frequency information, which is important for pitch perception and provides many advantages for the users. This study aimed to compare aural/oral performance of bimodal cochlear implants with unilateral ones in children using parents' evaluation of aural/oral performance of children (PEACH) questionnaire. Methods: Twenty children with unilateral cochlear implant and 20 ones with bimodal cochlear implants were selected for this study. Of them, 23 had cochlear devices, 10 possessed Med-El ones, and 7 wore advanced bionics ones. Bimodal group had at least 7 months of hearing experience with digital hearing aid in non-implanted ear. In order to compare the aural/oral performance in these groups, we used the PEACH questionnaire. Results: In unilateral and bimodal ...
International Journal of Pediatric Otorhinolaryngology, 2011
Aim: Finding surgical complications of cochlear implantation and their prevalence among patients ... more Aim: Finding surgical complications of cochlear implantation and their prevalence among patients undergoing surgery so we can pursue methods for preventing and treating these complications. Methods and Materials: A cross-sectional study of 1487 cochlearimplanted patients who had received a cochlear implant during 1991-2009. All Aetiologies of deafness, surgical complications (intra-operative and postoperative) were registered and analyzed. Results: Complications occurred in 120 patients (8%).Two most common surgical complications were device failure (5.04%) and Gusher (1.34%). Another complications were facial paresis (0.4%); Device extrusion (0.2%), flap necrosis (0.33%); Meningitis (0.13%); sensitivity to prosthesis (0.13%); Device break (0.13%); Electrode extrusion (0.2%); Incorrect device positioning (0.067%) and wound dehiscence (0.067%). Conclusion: Despite this fact that cochlear implantation is a procedure with minimal complications, but precise preoperative evaluation of patients results in better information about structural anomalies and prevention of unwanted adverse effects. These evaluations include preoperative Imaging and also considering administration of Proper antibiotic for preventing infectious complications.
Background: Auditory scene analysis (ASA) is the process by which the auditory system separates i... more Background: Auditory scene analysis (ASA) is the process by which the auditory system separates individual sounds in natural-world situations. ASA is a key function of auditory system, and contributes to speech discrimination in noisy backgrounds. It is known that sensorineural hearing loss (SNHL) detrimentally affects auditory function in complex environments, but relatively few studies have focused on the influence of SNHL on higher level processes which are likely involved in auditory perception in different situations.
The use of cochlear implantation to treat patients with inner ear malformations such as Mondini d... more The use of cochlear implantation to treat patients with inner ear malformations such as Mondini dysplasia has been increasingly successful. Until now, conventional hearing aids in these patients have not performed well. Consequently, the hearing problem for patients with this condition has been somewhat improved with the use of cochlear implants. Various results of cochlear implantation have been reported in these patients so far. This is a report of 5 patients with Mondini malformation who have undergone cochlear implant surgery.
One of the most important factors in auditory speech perception of cochlear-implanted children is... more One of the most important factors in auditory speech perception of cochlear-implanted children is age. The goal of this study was to compare auditory speech perception among children implanted from 6 different age groups: 0 to 3, 4 to 5, 6 to 7, 8 to 9, 10 to 11, and >12 years. The subjects of this study were matched based on socioeconomic status, residual hearing before cochlear implantation, the kinds of cochlear implant device, speech processing strategy, communication mode after implantation, and primary language in family. All of them have used the device minimally for 2 years. The subjects were tested with a range of closed and open-set auditory speech perception tests, and the levels of auditory speech perception in different age groups were compared. Results showed that the children who received an implant at 0 to 3 years of age had maximum auditory speech perception.
To investigate the prevalence of mutations in the coding exon of the GJB2 gene in Iranian childre... more To investigate the prevalence of mutations in the coding exon of the GJB2 gene in Iranian children with cochlear implants, and to compare the outcomes of auditory perception and speech production in cochlear-implanted children with and without GJB2 mutation. One hundred and sixty-six prelingually deaf children who had undergone cochlear implantation at the Iranian Cochlear Implant Center, Tehran, were selected from a pool of 428 implanted children. The prevalence of GJB2 gene mutations was assessed using nested polymerase chain reaction and direct sequencing. To enable comparisons, we also identified 36 implanted children with non-GJB2 deafness. Patients' speech perception and speech production were assessed using the Categorization of Auditory Performance and Speech Intelligibility Rating scales. Thirty-three of 166 probands (19.9 per cent) were found to have GJB2 deafness-causing allele variants and were diagnosed with DFNB1 deafness. Results also indicated a significant improvement in speech perception and production scores in both GJB2 and non-GJB2 patients over time. Children with GJB2-related deafness benefit from cochlear implantation to the same extent as those with non-GJB2-related deafness.
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