Cases reported "Deafness"

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1/55. Direct measurement of cerebrospinal fluid pressure through the cochlea in a congenitally deaf child with Mondini dysplasia undergoing cochlear implantation.

    OBJECTIVE: perilymph/cerebrospinal fluid (CSF) "gushers" may occur at cochleostomy during cochlear implant surgery, particularly in patients with congenital cochlear duct malformation in which CSF in the internal auditory meatus is in direct communication with the perilymphatic space in the cochlea. The object of the study was to measure the pressure and flow of a CSF gusher at cochleostomy. STUDY DESIGN: The design was a preoperative pressure measurement. SETTING: The setting was a multidisciplinary cochlear implant program. PATIENT: A 4-year-old girl with bilateral Mondini deformity undergoing cochlear implantation was studied. INTERVENTION: A size 23 FG intravenous cannula was inserted into the cochlea and connected to a pediatric drip set to form an improvised manometer. MAIN OUTCOME MEASURE: Intracochlear fluid pressure was measured at 14 cm H2O, equivalent to the normal CSF pressure that would be recorded in a child of this age at lumbar puncture. An indirect measurement of the likely size of the CSF/perilymph defect was made. RESULTS: This technique may allow better assessment of the risk of postoperative CSF leakage and meningitis. CONCLUSION: This simple technique of measuring the pressure in a perilymph gusher can be used to assess the need for careful sealing of the cochleostomy, to measure the reduction in pressure produced by head elevation or a spinal drain, and to assess the probable size of a defect in the lamina cribrosa.
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ranking = 1
keywords = communication
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2/55. The assessment and treatment of deaf children with psychiatric disorders.

    The assessment and treatment of deaf children with psychiatric disorder is intimately related to the individual child's communication, which in turn is affected by a number of factors, medical, social, and cultural. The deafness can be aetiologically related to the psychiatric disorder or can be incidental. Treatment strategies should be adapted to meet the individual child and family's needs. Deaf professionals have a vital role in mental health services for this population. The use of an interpreter can clarify communication and cultural issues for deaf and hearing children, families, and professionals.
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ranking = 2
keywords = communication
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3/55. Interstitial deletion of 4p15.32p16.3 in a boy with minor anomalies, hearing loss, borderline intelligence, and oligodontia.

    We describe an 11-year-old boy of Saudi origin with an interstitial deletion in the short arm of chromosome 4 (p15.32p16.3) as determined by G-banding and fluorescent in situ hybridization. His clinical manifestations were similar but not identical to previously reported cases of interstitial deletion in the same chromosomal region, and were not those associated with wolf-hirschhorn syndrome. The boy had normal facial characteristics, short stature, minor anomalies of hands and feet, amblyopia of the right eye, bilateral hearing loss, and hypotonia. On developmental testing, he had borderline intelligence, with a severe sensory integration and motor planning disorder, and severe deficits in the communication domain. In addition, he had severe oligodontia affecting his secondary dentition. This finding supports the presence of one or more genes involved in dentition in this chromosomal region.
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ranking = 1
keywords = communication
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4/55. Deaf murderers: clinical and forensic issues.

    Data are reported on 28 deaf individuals who were convicted, pled guilty, or have been charged and awaiting trial for murder. The unique forensic issues raised by these cases are discussed, and their clinical picture presented. A significant percentage of these deaf murderers and defendants had such severely limited communication skills in both English and American sign language that they lacked the linguistic ability to understand the charges against them and/or to participate in their own defense. As such, they were incompetent to stand trial, due not to mental illness or mental retardation, but to linguistic deficits. This form of incompetence poses a dilemma to the courts that remains unresolved. This same linguistic disability makes it impossible for some deaf suspects to be administered Miranda Warnings in a way comprehensible to them. This paper identifies the reasons for the communication problems many deaf persons face in court and offers remedial steps to help assure fair trials and police interrogations for deaf defendants. The roles and responsibilities of psychiatric and psychological experts in these cases are discussed. Data are provided on the etiology of the 28 individuals' hearing losses, psychiatric/psychological histories, IQs, communication characteristics, educational levels, and victim characteristics.
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ranking = 3
keywords = communication
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5/55. cochlear implantation after labyrinthectomy.

    OBJECTIVE: The goal was to report a method used for intraoperative ear selection for cochlear implantation using electrical brainstem response. Initial patient response and the longer-term results of cochlear implantation after labyrinthectomy were compared. STUDY DESIGN: This was a specific retrospective review of a single case of cochlear implantation after labyrinthectomy. SETTING: The study involved a tertiary referral center in both an ambulatory and a hospital setting. PATIENT: The study involved a report of a single patient who was evaluated for a possible cochlear implant and successfully underwent cochlear implantation. INTERVENTIONS: A case study of a profoundly deaf individual is presented, including the diagnostic measures used to determine the candidacy for cochlear implantation, the ear selected, and the rehabilitation. MAIN OUTCOME MEASURES: Both early (3 months) and later (14 months) postoperative results clearly demonstrate that a cochlear implant in a patient with a previous labyrinthectomy can be beneficial. RESULTS: The early and later results after cochlear implantation are compared in a single case study. CONCLUSION: This case study demonstrates that there is improvement in sound awareness, speech recognition, and communication after cochlear implantation in a previously labyrinthectomized ear.
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ranking = 1
keywords = communication
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6/55. Functional differentiation of the auditory association area in prelingually deaf subjects.

    BACKGROUND: it is believed that the number of neurons of the human cortex increases rapidly in the first postnatal year, and then decreases gradually towards adult level as their functions are revised up to 11 years of age ('synaptic revision'). It is also confirmed that regional cerebral blood flow (rCBF) at rest represents the density of the neurons and decreases in accordance with the synaptic revision in process. If synaptic revision does not occur, rCBF remains at high level. Thus, we can evaluate whether functional differentiation has occurred in the human cortex by measuring rCBF at resting state. OBJECTIVE: to examine functional differentiation of the auditory association area (A2) in prelingually deaf subjects. methods: six postlingually and six prelingually deaf subjects who had undergone cochlear implant (CI) were involved in the current study. All prelingually deaf subjects underwent CI over 8 years old. The rCBF in A2 was examined during resting and listening to speech sounds using positron emission tomography (PET) and H2(15)O intravenous injection. Twelve normal subjects' rCBFs were also measured as control. Furthermore, three prelingually deaf subjects underwent follow up PET studies in which cortical activities in A2 for listening and lipreading were examined. RESULTS: in the examination of rCBF at rest, rCBFs of prelingually deaf subjects in A2 showed significantly higher than those of either the postlingually deaf subjects or normal subjects. During listening, rCBFs in A2 increased in postlingually deaf subjects and normal subjects, while there was no significant rCBF increase in the prelingually deaf. High rCBF level in A2 at rest observed in prelingually deaf subjects implied a lack of synaptic revision, and it was suggested that the functional differentiation for auditory processing was little in the A2 of prelingually deaf subjects. In the follow up study for three prelingually deaf subjects, activation of A2 was observed during lipreading but not during listening in two cases, who had developed the skill of lipreading while speech recognition was not improved by CI. In contrast, the other case had not used any visual clues in daily communication prior to CI, and the hearing acuity was well improved by CI. This case demonstrated an activity in A2 during listening, while lipreading induced no activation. CONCLUSION: it is suggested that functional differentiation of A2 should differ according to which of visual and auditory clue is chiefly used during critical periods for speech acquisition. The findings are thought to be important for us to schedule the education and treatment for prelingually deaf children.
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ranking = 1
keywords = communication
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7/55. Documenting English syntactic development in face-to-face signed communication.

    The authors explored the face-to-face English competence of five students who were participating in a larger study of teachers' use of English-based signing. Using case studies, the authors report on the students' development of English-based signing at the beginning and end of their involvement in this 4-year study. Grammatical forms similar in English and American sign language (ASL) were initially more readily produced when tested for in English, and showed consistently higher attainment levels across all the students, than grammatical forms that are different in English and ASL. The authors found emerging English forms that could be documented (a) between prompted and imitated utterances and (b) within blocks of test items examining the same grammatical constructions. The authors conclude that teachers' concerted efforts to use English-based signing as a language of instruction enhance deaf students' English acquisition. Such signing helps build a bridge between native sign language and the development of English skills necessary for literacy.
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ranking = 4
keywords = communication
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8/55. Unpredictable hearing loss after intratympanic gentamicin treatment for vertigo. A new theory.

    A new hypothesis is advanced suggesting that unpredictable cases of profound hearing loss after intratympanic gentamicin treatment (IGT) may be caused by decreased patency of the communication routes between the inner ear and the cerebrospinal fluid, primarily of the cochlear aqueduct. A tympanic displacement analyzer, which can indirectly analyze inner ear and intracranial pressure changes and can also evaluate the efficiency of communication between these two compartments, was used. Two cases are presented: in the first, a patient who became deaf after IGT showed signs of decreased patency of the communication routes with the tympanic membrane displacement (TMD) test; in the second, a patient without hearing damage after IGT had efficient communication evaluated by the TMD test. These preliminary findings are in accordance with the proposed pathophysiology. If future clinical studies confirm the present theory and findings, it may prove possible to predict and prevent deafness after IGT and possibly also after systemic aminoglycoside treatment.
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ranking = 4
keywords = communication
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9/55. Obstetric handling of a deaf patient.

    We report our experience of obstetric handling of a deaf pregnant patient antenatally, in labor and postpartum. The patient was deaf from childhood. The attending obstetrician had no training in the necessary skills for communication with the deaf. Fortunately, the patient could read and write English very well and communication was carried out through pen and paper. This proved to be difficult, time-consuming and required a lot of patience. The clinical, psychological and human aspects of the management were gratifying. The patient brought up interesting aspects that need to be considered when dealing with similar patients. Experience of deaf mothers and their ingenious approaches in dealing with babies, in the postpartum period, are quoted in this communication.
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ranking = 3
keywords = communication
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10/55. Postlingual collapse of language and its recovery after cochlear implantation.

    A 6-year-old boy lost his normally-developed language ability within 2 months after bilateral sudden peripheral deafness. The boy became non-communicative with others, restless with frequent meaningless bursts of shouts (communication skills equivalent to 9-11 months of language development: a rapid breakdown of language). Since conservative methods were ineffective, cochlear implantation was performed. A surprising success was observed: he regained the language retracing the normal developmental stages and caught up with his contemporaries in 2 years. Behavioral improvement paralleled his language development. This case (1) provides insights into the brain function with respect to language acquisition, in relation to the plasticity during the 'critical period' of language learning, (2) reveals the close relationship between language development and behavior, and (3) suggests the predominance of auditory stimulation in learning language.
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ranking = 1
keywords = communication
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