- Email: [email protected]
B063 Jervell and Lange-Nielsen Syndrome
Abstracts, ESPCI 2011 / International Journal of Pediatric Otorhinolaryngology 75 (2011) 10–32
adhesive otitis with near location of sigmoid sinus and other). In these cases cochlear implantation combined with functional reconstructive operation. All patients had sound feelings after connection of speech processor on all channels. Conclusion: Application of functional reconstructive methods of microsurgery ear and modern types of cochlear implants allows to conduct the high-quality patient’s rehabilitation and to remove the pathological process in middle ear. B059 Audiologic evaluation after cochlear implantation in single sided deafness K. Boeheim, S.M. Pok, M. Schloegel. ENT Dep. Landesklinikum St. Poelten, Austria Aim: To evaluate speech recognition in quiet and noise and effects on incapacitating tinnitus after cochlear implantation in unilateral deafness. Material and Methods: 9 subjects with unilateral postlingual deafness and incapacitating tinnitus received a cochlear implant (MedEl Sonata). Hearing in the contralateral ear ranged from normal hearing to moderate SNHL with conventional HA fitting. A binaural test battery was applied to assess the pre- and postoperative speech recognition in quiet and noise, including an adaptive speech reception threshold test in noise (Oldenburg sentence test). Results: With the contralateral ear plugged, all subjects achieved open-set speech recognition with the CI. In follow up intervals up to 24 months, all subjects show continuous improvement, especially in the most challenging binaural hearing tasks. Conclusion: In single sided deafness, most improvement with the CI was observed for (1) patients suffering a chronic, incapacitating tinnitus and (2) patients with SNHL on the not implanted ear. B060 The role of trauma in cochlear implant malfunction in children J. Kronenberg1 , Y. Shapira1 , Z. Yakir2 , L. Migirov1 . 1 Department of Otolaryngology Head and Neck Surgery, 2 Department of Communication Disorders, Sheba Medical Center, Tel Aviv University, Israel Aim: The rate of cochlear implant malfunction due to trauma is higher in children. This can be explained by the hyperactivity of children and the anatomy of the pediatric skull. Material and Methods: This study includes 559 children aged up to 10 years who underwent cochlear implantation at the Sheba Medical Center between 1993 and 2010. The malfunctioned implants were investigated for faults and sent to the companies for further examination. Results: Fifty nine (11.8%) device malfunctions were encountered in this series (compared to 4.3% in the adults group). Twenty three of them (32.9%) were caused by trauma. The main traumatic reasons for device malfunction were breakage of the electrodes (34.8%), defective case (26.1%), skin flap brake- down (17.3%) and magnet dislodgment 13%). Conclusion: The rate of traumatic implant malfunction was found to be higher in children than in adults. This rate of malfunction may be minimized by using several protective techniques. Correspondence: [email protected] B061 Consanguineous marriage among parents of iranian deaf children M. Ajalloueyan, S. Amirsalari, M.A. Raeesi. Iran cochlear implant centre, Tehran, Iran Aim: To document the causes of profound bilateral sensorineural hearing loss among Iranian children, candidate for cochlear implantation. Materials and Methods: A total of 310 deaf children attended CI Center between 2007 and 2009 ranging in age from 6 to
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48 months were included in this study. Chi2 test was used to assess statistical difference between the frequency of parental consanguinity between deaf and normal population. Result: At 203 (65%) children had parents married with their relatives 132 parents were first cousins, 37 (11.8%) patrilateral parallel cousins, 38 (12.2%) multilateral parallel cousins, 57 (18.3%) cross-cousins, 54 (17.4%) of parents were second cousins and the remained 17 (5.2%) parents were beyond second-cousins. Conclusion: These data demonstrated 65% parental consanguineous marriage in deaf children. Percentage of consanguineous marriage among Iranian population is (38%) that indicates an obvious relation between sever hearing loss in offspring’s and consanguineous marriage. B062 What happens to the bony electrode groove? – A classical technique in the light of revision surgery W. Pethe, J. Langer, K. Begall. HNO-Klinik, AMEOS Klinikum Halberstadt, Germany Aim: To investigate the behaviour of the receiver-stimulator package and the electrodes in the bony groove after cochlear implantation. Material and Methods: Digital intraoperative photos and operation reports of 41 cases of cochlear implant revision surgery had been analyzed retrospectively. Results: In all cases the receiver-stimulator package had been in place. It was always covered in connective tissue. In only two cases it was partially overgrown by osseous tissue. In 28 out of 41 cases a drilling burr had been used to free the electrode from the bone. Remnants of the return electrode had to been left 9 times. Conclusions: Deep grooves for the receiver-stimulator package and the electrodes help to secure the positioning. Healing processes including osteoneogenesis lead to a partial or complete bony cover of the mastoidectomy and the eletrode groove. Slenderized implants and shallow electrode grooves can probably be beneficial for cochlear implant revision surgery. B063 Jervell and Lange-Nielsen Syndrome A. Eftekharian, N. Alani. Cochlear Implant Center, Department of Otolaryngology, Shaheed-beheshti University of Medical sciences, Tehran, Iran Aim: Jervell and Lange-Nielsen syndrome (JLNS) is characterized by deafness and prolongation of the QT interval with a high risk of life-threatening arrhythmias. Disease prevalence varies depending on the population studied. The aim of this study is to investigate its frequency in our population, to compare it with the literature, and to present its pre-, pri and post-operative hazards and precautions. Material and Methods: Electrocardiograms of 203 children with profound hearing loss which were candidates of cochlear implantation at Loghman hospital, Tehran, Iran were evaluated. Frequency of JLNS and its clinical presentations were compared with the literature results. Results: There was only one case of proved JLNS in our studied population (Frequency: 0.49%). Comparing to the literature, it seems that its frequency is less than Europe. Conclusions: Although it’s a rare disease, physicians must be aware of its pre-, pri and post-operative hazards that will be discussed in the presentation. B064 Cochlear implantation in deaf children with neurodevelopmental disorders S. Hassanzadeh. Faculty of Psychology and Education, University of Tehran, Tehran, Iran Aim: We aimed to identify the frequency with which the following conditions were present as a second disability in cochlear-
adhesive otitis with near location of sigmoid sinus and other). In these cases cochlear implantation combined with functional reconstructive operation. All patients had sound feelings after connection of speech processor on all channels. Conclusion: Application of functional reconstructive methods of microsurgery ear and modern types of cochlear implants allows to conduct the high-quality patient’s rehabilitation and to remove the pathological process in middle ear. B059 Audiologic evaluation after cochlear implantation in single sided deafness K. Boeheim, S.M. Pok, M. Schloegel. ENT Dep. Landesklinikum St. Poelten, Austria Aim: To evaluate speech recognition in quiet and noise and effects on incapacitating tinnitus after cochlear implantation in unilateral deafness. Material and Methods: 9 subjects with unilateral postlingual deafness and incapacitating tinnitus received a cochlear implant (MedEl Sonata). Hearing in the contralateral ear ranged from normal hearing to moderate SNHL with conventional HA fitting. A binaural test battery was applied to assess the pre- and postoperative speech recognition in quiet and noise, including an adaptive speech reception threshold test in noise (Oldenburg sentence test). Results: With the contralateral ear plugged, all subjects achieved open-set speech recognition with the CI. In follow up intervals up to 24 months, all subjects show continuous improvement, especially in the most challenging binaural hearing tasks. Conclusion: In single sided deafness, most improvement with the CI was observed for (1) patients suffering a chronic, incapacitating tinnitus and (2) patients with SNHL on the not implanted ear. B060 The role of trauma in cochlear implant malfunction in children J. Kronenberg1 , Y. Shapira1 , Z. Yakir2 , L. Migirov1 . 1 Department of Otolaryngology Head and Neck Surgery, 2 Department of Communication Disorders, Sheba Medical Center, Tel Aviv University, Israel Aim: The rate of cochlear implant malfunction due to trauma is higher in children. This can be explained by the hyperactivity of children and the anatomy of the pediatric skull. Material and Methods: This study includes 559 children aged up to 10 years who underwent cochlear implantation at the Sheba Medical Center between 1993 and 2010. The malfunctioned implants were investigated for faults and sent to the companies for further examination. Results: Fifty nine (11.8%) device malfunctions were encountered in this series (compared to 4.3% in the adults group). Twenty three of them (32.9%) were caused by trauma. The main traumatic reasons for device malfunction were breakage of the electrodes (34.8%), defective case (26.1%), skin flap brake- down (17.3%) and magnet dislodgment 13%). Conclusion: The rate of traumatic implant malfunction was found to be higher in children than in adults. This rate of malfunction may be minimized by using several protective techniques. Correspondence: [email protected] B061 Consanguineous marriage among parents of iranian deaf children M. Ajalloueyan, S. Amirsalari, M.A. Raeesi. Iran cochlear implant centre, Tehran, Iran Aim: To document the causes of profound bilateral sensorineural hearing loss among Iranian children, candidate for cochlear implantation. Materials and Methods: A total of 310 deaf children attended CI Center between 2007 and 2009 ranging in age from 6 to
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48 months were included in this study. Chi2 test was used to assess statistical difference between the frequency of parental consanguinity between deaf and normal population. Result: At 203 (65%) children had parents married with their relatives 132 parents were first cousins, 37 (11.8%) patrilateral parallel cousins, 38 (12.2%) multilateral parallel cousins, 57 (18.3%) cross-cousins, 54 (17.4%) of parents were second cousins and the remained 17 (5.2%) parents were beyond second-cousins. Conclusion: These data demonstrated 65% parental consanguineous marriage in deaf children. Percentage of consanguineous marriage among Iranian population is (38%) that indicates an obvious relation between sever hearing loss in offspring’s and consanguineous marriage. B062 What happens to the bony electrode groove? – A classical technique in the light of revision surgery W. Pethe, J. Langer, K. Begall. HNO-Klinik, AMEOS Klinikum Halberstadt, Germany Aim: To investigate the behaviour of the receiver-stimulator package and the electrodes in the bony groove after cochlear implantation. Material and Methods: Digital intraoperative photos and operation reports of 41 cases of cochlear implant revision surgery had been analyzed retrospectively. Results: In all cases the receiver-stimulator package had been in place. It was always covered in connective tissue. In only two cases it was partially overgrown by osseous tissue. In 28 out of 41 cases a drilling burr had been used to free the electrode from the bone. Remnants of the return electrode had to been left 9 times. Conclusions: Deep grooves for the receiver-stimulator package and the electrodes help to secure the positioning. Healing processes including osteoneogenesis lead to a partial or complete bony cover of the mastoidectomy and the eletrode groove. Slenderized implants and shallow electrode grooves can probably be beneficial for cochlear implant revision surgery. B063 Jervell and Lange-Nielsen Syndrome A. Eftekharian, N. Alani. Cochlear Implant Center, Department of Otolaryngology, Shaheed-beheshti University of Medical sciences, Tehran, Iran Aim: Jervell and Lange-Nielsen syndrome (JLNS) is characterized by deafness and prolongation of the QT interval with a high risk of life-threatening arrhythmias. Disease prevalence varies depending on the population studied. The aim of this study is to investigate its frequency in our population, to compare it with the literature, and to present its pre-, pri and post-operative hazards and precautions. Material and Methods: Electrocardiograms of 203 children with profound hearing loss which were candidates of cochlear implantation at Loghman hospital, Tehran, Iran were evaluated. Frequency of JLNS and its clinical presentations were compared with the literature results. Results: There was only one case of proved JLNS in our studied population (Frequency: 0.49%). Comparing to the literature, it seems that its frequency is less than Europe. Conclusions: Although it’s a rare disease, physicians must be aware of its pre-, pri and post-operative hazards that will be discussed in the presentation. B064 Cochlear implantation in deaf children with neurodevelopmental disorders S. Hassanzadeh. Faculty of Psychology and Education, University of Tehran, Tehran, Iran Aim: We aimed to identify the frequency with which the following conditions were present as a second disability in cochlear-