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Building a Multidisciplinary Cochlear Implant Team

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Abstract

Cochlear implantation is evolving into a common modality of auditory rehabilitation for the patient with severe to profound sensorineural hearing loss. In order to provide the patient considering cochlear implantation with an understanding of how best to integrate the device into his daily life, adequate evaluation, counseling, and instruction are required. Using the multidisciplinary model, our initial experience has shown that evaluation by specialists in audiology, otolaryngology, speech pathology, pediatric genetics, social work, and child life may lead to better patient and family understanding of cochlear implantation.

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... There is still knowledge difference and the importance of social workers in cochlear implant team is not known by many yet they social workers are the ones who obtain psychosocial information related to family dynamics, school and social issues and are crucial in informing the patients and parents about the lengthy rehabilitation needs after cochlear implantation [20]. ...
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Background: Cochlear implantation is one of the most performed surgery worldwide currently to people mostly children with sensorineural hearing loss to solve the effects of severe to profound hearing loss and without this, these children would be left with deafness for life. Tanzania has also developed and started to perform cochlear implantation to children in 2017. Objective: The main aim of this study was to assess Knowledge on cochlear Implants among senior medical students and interns who are going to be the primary care providers and will be required to make proper diagnosis and referrals for children requiring cochlear implants to the right facilities for further management hence their knowledge is of great essence. Methodology: Descriptive cross sectional study was used, a structured questionnaire was used to collect data and Stratified random sampling method was used to get participants. Self-administered questionnaires was used for data collection and collected data was coded and analysed using IBM SPSS version 24.0. Results: In this study which involved 203 participants, we found that only 87.2% have ever heard about cochlear implants and only 80.2% knew that cochlear implants may restore hearing to the deaf and 11.3% did not know that children who received cochlear implant could learn spoken language also 35% of the participants did not know that cochlear implant is being done in Tanzania despite starting performing the surgery since 2017. More than half of the participants are knowledgeable to different candidacy criteria for cochlear implantation however only 23.2% of the participants knew that a child without a cochlear is not a candidate for cochlear implants. More than 90% of participants knew that Audiologist, Otolaryngologist and speech therapist are key members of cochlear implant team but only 65% knew that a social worker is also a member of the team. We also found that more than half of the participants knew that infections, device failures, cerebral spinal fluid fistulas facial nerve paresis and cholesteatomas are complications associated with cochlear implantations. It was also found that there was an association between the university studied/studying and the level of knowledge, Majority of government owned university students having high knowledge while those of private owned having moderate knowledge. Citation: Massawe Richard Enica., et al. “Knowledge on Cochlear Implants among Medical Students, Tanzania". Acta Scientific Otolaryngology 3.3 (2021). Conclusion: This study provides a general image on knowledge of cochlear implants among intern doctors and final year medical students in Tanzania and still there is a knowledge gap among the participants and few still don’t know that cochlear implantation surgery is done in Tanzania which is a very good step in the country but also a call to the primary care physicians to be well knowledgeable in indications, possible complications and the whole process involved in cochlear implantation to be able to properly manage these children and be able to refer them timely for the surgery. Also, a call to Ear Nose and Throat Specialists and Academicians to ensure this knowledge gap is covered. Keywords: Cochlear Implant; Hearing Loss; Knowledge
Article
Cochlear implantation is a timeous and cost-effective solution for severe-profound sensorineural hearing loss, and has transformed the lives of many individuals with significant hearing loss. The advent of cochlear implantation has meant that, for the first time, one of the senses (hearing), having been entirely lost, can be restored. This, the first of a two-part series on cochlear implantation in South Africa (SA) puts global (and especially SA) hearing loss in perspective, sets out the principles of cochlear implantation and briefly sketches the history of this world-changing technology.
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Primary--i.e., nonsyndromal-postlingual deafness is inherited as an autosomal dominant phenotype in a large kindred in Costa Rica. Genetically susceptible individuals begin to lose hearing at low frequencies at about age 10 years, after language and speaking are learned. Deafness inevitably progresses by age 30 years to bilateral hearing loss of all frequencies. Intelligence, fertility, and life expectancy are normal. The family traces its ancestry to an affected founder born in Costa Rica in 1754. We have mapped the gene for deafness in this kindred to chromosome 5q31, between the markers IL9 and GRL, by linkage analysis involving 99 informative relatives.
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This study was performed to investigate factors contributing to auditory, speech, language, and reading outcomes after 4 to 6 years of multichannel cochlear implant use in children with prelingual deafness. The analysis controlled for the effects of child, family, and implant characteristics so that the educational factors most conducive to maximum implant benefit could be identified. We tested 136 children from across the United States and Canada. All were 8 or 9 years of age, had an onset of deafness before 3 years of age, underwent implantation by 5 years of age, and resided in a monolingual English-speaking home environment. Characteristics of the child and the family (primarily nonverbal IQ) accounted for approximately 20% of the variance in outcome after implantation. An additional 24% was accounted for by implant characteristics and 12% by educational variables, particularly communication mode. Oral education appears to be an important educational choice for children who have undergone cochlear implantation before 5 years of age.
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In June 2002, the Food and Drug Administration received reports of bacterial meningitis in patients with cochlear implants for treatment of hearing loss. Implants that included a positioner (a wedge inserted next to the implanted electrode to facilitate transmission of the electrical signal by pushing the electrode against the medial wall of the cochlea) were voluntarily recalled in the United States in July 2002. We identified patients with meningitis and conducted a cohort study and a nested case-control investigation involving 4264 children who had received cochlear implants in the United States between January 1, 1997, and August 6, 2002, and who were less than six years of age when they received the implants. We calculated the incidence of meningitis in the cohort and assessed risk factors for meningitis among patients and among 199 controls, using data from interviews with parents and abstracted from medical records. We identified 26 children with bacterial meningitis. The incidence of meningitis caused by Streptococcus pneumoniae was 138.2 cases per 100,000 person-years--more than 30 times the incidence in a cohort of the same age in the general U.S. population. Postimplantation bacterial meningitis was strongly associated with the use of an implant with a positioner (odds ratio, 4.5 [95 percent confidence interval, 1.3 to 17.9], with adjustment for medical, surgical, and environmental factors) and with the joint presence of radiographic evidence of a malformation of the inner ear and a cerebrospinal fluid leak (adjusted odds ratio, 9.3 [95 percent confidence interval, 1.2 to 94.5]). The incidence of meningitis among patients who had received an implant with a positioner remained higher than the incidence among those whose implants did not have a positioner for the duration of follow-up (24 months from the time of implantation). Parents and health care providers should ensure that all children who receive cochlear implants are appropriately vaccinated and are then monitored and treated promptly for any bacterial infections after receiving the implant.
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Mutations in the gene for connexin 26, GJB2, are the most common cause of hearing loss in American and European populations, with a carrier rate of about 3%—a rate similar to that for cystic fibrosis. A single mutation, 35delG, is responsible for most of this autosomal recessive hearing loss, DFNB1. A broad spectrum of mutations in GJB2 has been found to be associated with hearing loss, including another deletion mutation, 167delT, which has a carrier rate of about 4% in the Ashkenazi Jewish population. Mutations in GJB2 have also been found to be associated with dominant nonsyndromic hearing loss, DFNA3. Clinical studies have shown that the recessive hearing loss can vary from mild to profound, even within the same sibship. This type of hearing loss is nonsyndromic and is accompanied by normal vision, vestibular responses, and no malformations of the inner ear detectable by computed tomography scanning. Progressive and asymmetrical hearing loss has been noted in some cases, but it accounts for fewer than one-third of the cases of this type of hearing loss. The discovery of mutations in GJB2 that cause hearing loss has profound implications in the early diagnosis of hearing loss in general. The relative ease of diagnosis by genetic testing of Cx26 permits early identification of children with GJB2/DFNB1 hearing loss. This testing, coupled with hearing loss diagnosed by infant auditory brainstem response audiometry, will ensure that hearing-impaired children and their parents receive proper medical, audiologic, genetic, and educational counseling. Am. J. Med. Genet. (Semin. Med. Genet.) 89:130–136, 1999. © 2000 Wiley-Liss, Inc.
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Now, parents of deaf children have at hand a complete guide to the process of cochlear implantation. Written by two eminent professionals in deaf education, The Parents’ Guide to Cochlear Implants explains in a friendly, easy-to-follow style each stage of the process. Parents will discover how to have their child evaluated to determine her or his suitability for an implant. They’ll learn about implant device options, how to choose an implant center, and every detail of the surgical procedure. The initial “switch-on� is described along with counseling about device maintenance. Most importantly, parents will learn their roles in helping their child adjust to and successfully use the cochlear implant. The Parents’ Guide to Cochlear Implants emphasizes such critical subjects as learning to listen through home activities, implants as tools for language development, and critical issues regarding school placement. This encouraging book considers the implications for performance in light of the whole child, including issues related to Deaf culture and cochlear implants. The authors also include brief stories by parents whose children have had implants that provide reassuring actual experiences to parents considering the procedure for their own child. With a last word on parenting perspectives and a rich source of resources in the appendices, this one-of-a-kind guide will arm parents of deaf children with complete confidence to make informed decisions about cochlear implantation. Patricia M. Chute is Associate Professor in the Department of Communication Disorders at Mercy College, Dobbs Ferry, NY. Mary Ellen Nevins is Professor in the Department of Communication Disorders and Deafness at Kean University, Union, New Jersey.
The availability of cochlear implant technology has made mainstreaming a more reachable social and academic goal for profoundly deaf children. Traditionally, the profoundly deaf child has required more self-contained education. It has been the hard-of-hearing child who reached the mainstream education classroom during the elementary years. Cochlear implant recipients, implanted early and receiving appropriate educational services that maximize learning across all domains, have shown a significant trend toward moving from a more self-contained to a less restrictive educational environment. Children with implants are making these transitions earlier than the larger majority of profoundly deaf children using traditional amplification.
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To evaluate the intraoperative problems and postoperative complications in children receiving cochlear implants. A retrospective analysis of the clinical records of 366 children, aged 1 to 14 years, who had received cochlear implants. Intraoperatively, obliteration of the cochlea occurred in 66 patients, cochlear dysplasia in 8, and CSF leakage in 7, and nearly 5% of patients had signs of infection in the mucosa of the middle ear. Postoperatively, early complications occurred in 1% to 2.5% of patients: flap complications, electrode dislocation, facial nerve problems, and incorrect insertion of the electrode. Delayed complications included otitis media and stimulation of the facial nerve. Proper preparation of the implantation site, experienced and well-trained surgeons, and awareness of the operative and postoperative risks are necessary.
Article
Mutations in the gene for connexin 26, GJB2, are the most common cause of hearing loss in American and European populations, with a carrier rate of about 3%-a rate similar to that for cystic fibrosis. A single mutation, 35delG, is responsible for most of this autosomal recessive hearing loss, DFNB1. A broad spectrum of mutations in GJB2 has been found to be associated with hearing loss, including another deletion mutation, 167delT, which has a carrier rate of about 4% in the Ashkenazi Jewish population. Mutations in GJB2 have also been found to be associated with dominant nonsyndromic hearing loss, DFNA3. Clinical studies have shown that the recessive hearing loss can vary from mild to profound, even within the same sibship. This type of hearing loss is nonsyndromic and is accompanied by normal vision, vestibular responses, and no malformations of the inner ear detectable by computed tomography scanning. Progressive and asymmetrical hearing loss has been noted in some cases, but it accounts for fewer than one-third of the cases of this type of hearing loss. The discovery of mutations in GJB2 that cause hearing loss has profound implications in the early diagnosis of hearing loss in general. The relative ease of diagnosis by genetic testing of Cx26 permits early identification of children with GJB2/DFNB1 hearing loss. This testing, coupled with hearing loss diagnosed by infant auditory brainstem response audiometry, will ensure that hearing-impaired children and their parents receive proper medical, audiologic, genetic, and educational counseling. Am. J. Med. Genet. (Semin. Med. Genet.) 89:130-136, 1999.
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Deafness is a complex disorder that involves a high number of genes and environmental factors. There has been enormous progress in non-syndromic deafness research during the last five years, with the identification of over 50 loci and 15 genes. Among these, three genes, GJB2, GJB3, and GJB6, encode for connexin proteins (Connexin26, Connexin31, and Connexin30, respectively). Another connexin (Connexin32, encoded by GJB1) is involved in X-linked peripheral neuropathy and hearing impairment. Mutations in these genes cause autosomal recessive (GJB2 and GJB3), autosomal dominant (GJB2, GJB3, and GJB6) or X-linked (GJB1) hearing impairment, both syndromic (GJB2, keratoderma; GJB3 erythrokeratodermia variabilis; and GJB1, peripheral neuropathy), and non-syndromic (GJB2, GJB3, and GJB6). Among these genes, mutations in GJB2 account for about 50% of all congenital cases of hearing impairment. Three mutations in GJB2 (35delG, 167delT, and 235delC) are particularly common in specific populations (Caucasoid, Jewish Ashkenazi, and Oriental, respectively), leading to carrier frequencies between one in 30 and one in 75. Over 50 mutations have been identified in the GJB2 gene, of which some missense changes (M34T, W44C, G59A, D66H, and R75W) have a negative dominant action in hearing impairment, with partial to full penetrance. Functional studies for some missense mutations in connexins 26, 30, and 32 have indicated abnormal gap junction conductivity. Expression patterns in mouse and rat cochlea indicate that Connexin26 and Connexin30 are expressed in the supportive cells of the cochlea, suggesting a potential role in endolymph potassium recycling. The high prevalence of mutations in GJB2 in some populations provides the tools for molecular diagnosis, carrier detection, and prenatal diagnosis of congenital hearing impairment.
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Over the past two decades, cochlear implantation has become a widely accepted treatment of deafness in children. Over 20,000 children have received cochlear implants worldwide. Hearing, language and social development outcomes have been positive. We review current issues in cochlear implantation, candidacy, evaluation, surgery, habilitation, ethics and outcomes.
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Genetic testing for deafness has become a reality. It has changed the paradigm for evaluating deaf and hard-of-hearing persons and will be used by physicians for diagnostic purposes and as a basis for treatment and management options. Although mutation screening is currently available for only a limited number of genes, in these specific instances, diagnosis, carrier detection, and reproductive risk counseling can be provided. In the coming years there will be an expansion of the role of genetic testing and counseling will not be limited to reproductive issues. Treatment and management decisions will be made based on specific genetic diagnoses. Although genetic testing may be a confusing service for the practicing otolaryngologist, it is an important part of medical care. New discoveries and technologies will expand and increase the complexity of genetic testing options and it will become the responsibility of otolaryngologists to familiarize themselves with current discoveries and accepted protocols for genetic testing.
Study clinical presentation, diagnosis and treatment of cochlear implant patients diagnosed with bacterial meningitis. Cochlear implantation in children is necessary for the optimal development of speech in the developing child diagnosed with profound sensorineural hearing loss. Approximately 60,000 devices have been inserted in adults and children worldwide to date. The Department of Pediatric Otolaryngology of a tertiary care children's hospital. All patients undergoing cochlear implantation from April 1997 were identified. Patients diagnosed with bacterial meningitis after implantation were selected for study. Of 30 children, two developed bacterial meningitis after cochlear implantation. One patient developed Streptococcus pneumoniae meningitis. One patient developed nontypable Haemophilus influenzae bacterial meningitis. Both patients made a complete recovery. Prompt diagnosis and treatment of meningitis is essential to optimize outcome.
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