Cases reported "Deafness"

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1/170. Contralateral deafness following unilateral suboccipital brain tumor surgery in a patient with large vestibular aqueduct--case report.

    A 68-year-old female developed contralateral deafness following extirpation of a left cerebellopontine angle epidermoid cyst. Computed tomography showed that large vestibular aqueduct was present. This unusual complication may have been caused by an abrupt pressure change after cerebrospinal fluid release, which was transmitted through the large vestibular aqueduct and resulted in cochlear damage.
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ranking = 1
keywords = aqueduct, duct
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2/170. 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 = 1.9626194321062
keywords = perilymphatic, duct
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3/170. Genetic factors in human sleep disorders with special reference to Norrie disease, prader-willi syndrome and Moebius syndrome.

    Sleep-wake problems are common in specific inborn errors of metabolism and structure of the central nervous system. Psychological factors, behavioural difficulties, metabolic disturbances, and widespread rather than focal damage to the nervous system are present in many of these diseases and all influence the sleep-wake cycle. However, a number of conditions cause relatively focal damage to the neuroanatomical substrate of sleeping and waking. These include fatal familial insomnia, with involvement of the prion protein gene on chromosome 20, Norrie disease, the prader-willi syndrome and the Moebius syndrome. The last three important conditions, although rare, are considered in detail in this review. They result in sensory deprivation, hypothalamic and mid-brain damage, and involve the X-chromosome, chromosome 15, and chromosome 13, respectively. These conditions cause a wide variety of sleep disturbance, including parasomnias, daytime sleepiness, and a condition like cataplexy. The place of the relevant gene products in normal sleep regulation needs further exploration.
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ranking = 0.00016651975170097
keywords = duct
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4/170. Sign-language interpretation in psychotherapy with deaf patients.

    Sporadic encounters with deaf patients seeking psychotherapy present a challenge to general clinicians outside of specialized services for the deaf. Skills for working with people who do not share one's own language mode and culture are not routinely taught in most training programs, so clinicians may be unprepared when they first encounter a deaf patient. While it would be ideal to be able to match deaf patients with therapists fluent in their preferred language mode, this is often not feasible in smaller centers. Working with a trained professional sign-language interpreter can be a productive alternative, as long as patient, therapist, and interpreter understand and are comfortable with the process. Peer-reviewed literature on sign language interpretation in psychotherapy is sparse, but some practical guidelines can be gleaned from it and supplemented by information provided by the deaf community through the internet. This paper arose from one psychiatric resident's first experience of psychotherapy working with a sign-language interpreter, and summarizes the literature search that resulted from a quest for understanding of deaf culture and experience, of the unique characteristics of sign language, and of the effects on the therapeutic relationship of the presence of the interpreter.
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ranking = 0.00016651975170097
keywords = duct
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5/170. Bilateral sensorineural deafness, partial agenesis of the corpus callosum, and arachnoid cysts in two sisters.

    We describe two sisters (ages 10 and 3 years, respectively) with a normal development and a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, arachnoid cyst, and hydrocephalus. Neither girl has distinctive physical anomalies. In the oldest girl, there was a hearing loss of 80 dB bilaterally, and the most severe loss on audiogram was seen at 2,000-4,000 Hz. In the youngest girl, there was a hearing loss of 100 dB bilaterally. Above 2,000 Hz no neural reactions were seen. Cerebral magnetic resonance imaging in one girl and computed tomography in the other showed a partial agenesis of the corpus callosum and a cyst in the pineal region, causing an aqueduct stenosis by compression and consequent hydrocephalus. The parents have normal hearing, and brain magnetic resonance imaging showed no abnormalities. They are nonconsanguineous but from the same small village. This is the first report of a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, and an arachnoid cyst. The pattern of inheritance is probably autosomal recessive.
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ranking = 0.16666666666667
keywords = aqueduct, duct
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6/170. Mitochondrial maculopathy: geographic atrophy of the macula in the MELAS associated A to G 3243 mitochondrial dna point mutation.

    PURPOSE: To report ocular findings in the mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (melas syndrome) in a family with the A to G 3243 mitochondrial (mt) dna point mutation. methods: case reports. Ocular findings are described from four family members with the MELAS associated A to G 3243 mt dna point mutation. RESULTS: Findings included ophthalmoplegia, neurosensory deafness, reduction of photopic and scotopic electroretinogram b-wave amplitudes, and myopathy, as well as macular retinal pigment epithelial atrophy. No family members had nyctalopia, attenuation of retinal blood vessels, or retinal bone spicule pigmentation. CONCLUSION: The finding of slowly progressive macular retinal pigment epithelial atrophy expands the reported phenotypic diversity of patients with A3243G mt dna mutations.
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ranking = 0.00016651975170097
keywords = duct
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7/170. Ebstein's anomaly of the tricuspid valve associated with congenital deafmutism.

    Ebstein's anomaly is the most common congenital abnormality of the tricuspid valve and accounts for about 0.5% of cases of the congenital heart diseases. It has occasionally been associated with other syndromes but not with the congenital deafmutism. The first case of Ebstein's anomaly associated with the congenital deafmutism is reported. The patient remained asymptomatic until the age of 35 years and presented with palpitations and dizzy spells. This patient also manifested mitral valve prolapse and wolff-parkinson-white syndrome with right-sided accessory conduction pathway.
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ranking = 0.00016651975170097
keywords = duct
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8/170. Primary basal cell carcinoma of the middle ear presenting as recurrent cholesteatoma.

    OBJECTIVE: This study aimed to sensitize the practitioner to the unusual presentation of basal cell carcinoma in a recurrent cholesteatoma mass. STUDY DESIGN: The study design was a case report format. SETTING: The study was conducted at a tertiary referral hospital. PATIENT: The patient studied was a contralaterally anacusic veteran. INTERVENTION: Interventions were excision of cholesteatoma and withholding of adjunctive radiation. CONCLUSION: Primary basal cell carcinoma may present in a revision cholesteatoma field.
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ranking = 0.00016651975170097
keywords = duct
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9/170. Psychological factors in the etiology of ulcerative colitis: objectlessness and rage.

    In this second investigation of psychological factors in the etiology of ulcerative colitis, the author again utilizes unusual cases characterized by limited variables preceding illness. The first investigation involved several patients whose partial deafness antedated their illness. A significant point was that prior to developing their illness, all these patients had arrived at a state of objectlessness which was abetted by the deafness. This report presents four ulcerative colitis patients with pre-existing organic problems affecting their mental status. Two of the patients are severely mentally retarded. A third patient has rapidly advancing multiple sclerosis, and a fourth--actually an addition to the earlier study--has long-standing partial deafness. Aside from the objectlessness which was also prominent in the earlier group, all four patients demonstrate a consistent pattern of vicious self-directed rage. This paper takes into account the interaction between the rage and the state of objectlessness in the production of illness.
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ranking = 0.00016651975170097
keywords = duct
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10/170. Interarticulator co-ordination in deaf signers with Parkinson's disease.

    Motor control deficits in signers with Parkinson's disease (PD) were examined through analysis of their production of American sign language (ASL) fingerspelling, which is sequential and rapid motor behavior that has theoretical models of its underlying structure. Free conversation of two Deaf signers with PD and two Deaf control signers was analysed. In addition, scripted productions of one control signer were also analysed and directly compared to the same productions by the signers with PD. A featural analysis of ASL fingerspelling and a frame-by-frame analysis of multiple articulator movements were used to examine the fingerspelled productions. On the basis of the featural analysis, the signers with PD showed a variety of error patterns, all of which reflected attempts to reduce the motoric demands of coarticulation and thereby facilitate ease of articulation. Signers with PD either held individual segments in a fingerspelling sequence for a long time (segmentation), blended adjacent segments into a single segment (sequential blending), or broke handshapes down sequentially into their component features (featural unraveling). The results of both the featural analysis and the frame-by-frame analysis show that the PD signers have difficulty co-ordinating the movements of independent articulators in complex sequences. For example, the movements of independent articulators for fingerspelling (the thumb, fingers, and wrist) were markedly farther apart in time and more variable for the signers with PD. In addition, the signers with PD used fewer wrist movements while fingerspelling. Such deficits are consistent with claims that patients with PD are impaired in their ability to use ongoing sensorimotor information to program multi-articulator movements.
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ranking = 0.0006660790068039
keywords = duct
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