Cases reported "Deafness"

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1/13. 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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2/13. leopard syndrome associated with hyperelastic skin: analysis of collagen metabolism in cultured skin fibroblasts.

    We present a patient with leopard syndrome and hyperelastic skin. Biochemical analysis using cultured skin fibroblasts showed normal type III and V collagen synthesis, lysyl hydroxylation level of type I procollagen and processing of pro-alpha(1) and alpha(2)(I). Our results suggest that molecular defects of hyperelasticity in leopard syndrome are not related to abnormal collagen metabolism, although not all steps of collagen synthesis have been investigated.
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3/13. Necrotizing otitis externa caused by staphylococcus epidermidis.

    We present a case of malignant necrotizing otitis externa (MNOE) caused by staphylococcus epidermidis, which is usually a non-pathogenic microorganism. The patient is an otherwise healthy, nondiabetic 58-year-old white man. Contributory history began in 1994 after surgery for bilateral exostoses of the external auditory canals. Between April 1994 and May 1998 persistent otalgia occurred, with progressive mixed hearing losses, purulent discharge from both ears, spontaneous perforations of the tympanic membranes and ulceration of canal wall skin. From the beginning, Staph. epidermidis was isolated in all but one culture, but was not recognized as the pathological agent because of the presence of other more frequently involved bacteria and fungi. After multiple intravenous and oral antibiotics and antifungal treatments failed, further management involved frequent debridement of both external auditory canals and tympanic membranes, right tympanoplasty, bilateral mastoidectomy, revision tympanomastoidectomies and left modified radical mastoidectomy. Antistaphylococcal therapy including ceftazidime, vancomycin, teicoplanin, clindamycin and rifampicin was tried. Following the modified radical radical mastoidectomy, normalization of the status of his ears took approximately 2 months and has since remained stable to date. His left ear is deaf because of vancomycin administration, while magnetic resonance imaging and gallium scintigraphy have shown persistent inflammation of the skull base.
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4/13. streptococcus suis meningitis: report of a case.

    A 50-year-old policeman who presented with subacute meningitis, bilateral rectus muscle palsies, dizziness and early bilateral deafness was reported. cerebrospinal fluid (CSF) revealed polymorphonuclear pleocytosis with gram-positive cocci. blood and CSF cultures grew Streptococcus viridans which subsequently identified to be streptococcus suis. The patient improved after treatment but deafness persisted.
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5/13. Deaf culture and alcohol and substance abuse.

    Alcohol and substance abuse problems are now of concern among persons who are deaf. The problems of cultural influence, prevalence of the problem, and factors contributing to isolation and denial are addressed in this article. In addition, the issues of accessibility and service delivery are explored. Current programs that are accessible and provide alcohol and substance abuse recovery are identified.
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6/13. Chronic pseudomonas infections of cochlear implants.

    OBJECTIVE: To discuss chronic, refractory pseudomonas infections of cochlear implants and their management. DESIGN: Retrospective case series. SETTING: Two university-based cochlear implant programs. patients: Twenty-eight-year-old (Case 1) and 4-year-old (Case 2), different devices. INTERVENTIONS: Medical and surgical management. MAIN OUTCOME MEASURES: Clinical course. RESULTS: Both patients had delayed presentations, 4 months and 3 years postimplantation, respectively, with fluctuating scalp edema and pain resistant to multiple courses of oral antibiotics. Infections began as localized granulation and progressed to complete encasement of both devices with rubbery, poorly vascularized tissue. In each case, two different strains of multiresistant pseudomonas aeruginosa were cultured. Infections progressed despite local debridement and targeted antipseudomonal antibiotic coverage, and sensitive organisms continued to appear in cultures of refractory granulation tissue. Both patients underwent partial explantation, with the electrode array left in the cochlea, then received 2 to 3 more months of further medical therapy and observation and then were reimplanted successfully with new devices. Both have shown excellent performance and no sign of recurrent infection. CONCLUSIONS: Infections of cochlear implants are uncommon, and cases of successful conservative management without device explantation have been reported. However, our experience and the implanted device literature suggest that chronic pseudomonas infections may represent a distinct clinical entity, likely to fail protracted therapy and ultimately require device removal. Fortunately, successful reimplantation is possible.
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7/13. Chronic neurobrucellosis due to brucella melitensis.

    A 20-year-old male Turkish immigrant to norway suffering from severe chronic neurobrucellosis with spastic paraplegia and deafness is presented. The diagnosis was established by isolation of brucella melitensis from cerebrospinal fluid (CSF) culture. Brucella antibody agglutination titers were high in serum and CSF. In spite of intensive, prolonged treatment with a combination of trimethoprim-sulfamethoxazole (TPM-SMZ), rifampicin and doxycycline, the course of the illness was characterized by relapses and severe neurological defects.
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8/13. Inherited superactivity of phosphoribosylpyrophosphate synthetase: association of uric acid overproduction and sensorineural deafness.

    PURPOSE: Superactivity of 5-phosphoribosyl 1-pyrophosphate (PP-Rib-P) synthetase, inherited as an x chromosome-linked trait, has been reported in nearly 20 families in which overproduction of uric acid is invariably present in hemizygous affected males. Clinical manifestations of PP-Rib-P synthetase superactivity are mainly limited to gout in early adulthood. Neurologic deficits, including sensorineural deafness, have rarely been described. We herein document the association of PP-Rib-P synthetase superactivity, gout with excessive uric acid synthesis, and sensorineural deafness in an additional family. patients AND methods: Two members of a Spanish family were studied: an eight-year-old boy (Patient 1) with tophaceous gout, purine nucleotide and uric acid overproduction, and sensorineural deafness, and his 27-year-old mother (Patient 2), who had gout. Fibroblast cultures were initiated from skin biopsy specimens, and measurements of PP-Rib-P and purine nucleotide metabolism in the fibroblasts were performed. RESULTS: A labile but superactive PP-Rib-P synthetase was demonstrated in the fibroblasts cultured from both patients 1 and 2. The kinetic basis of PP-Rib-P synthetase superactivity in this family was resistance to purine nucleotide inhibition of enzyme activity. More severe derangements in the enzyme and in PP-Rib-P and purine synthesis in Patient 1's cells than in Patient 2's cells suggest that Patient 1 is hemizygous and Patient 2 is heterozygous for an x chromosome-linked genetic defect. Limited pedigree data support this view. Compared with affected members of seven other families with PP-Rib-P synthetase superactivity, these patients are intermediate in the range of clinical expression and in the severity of the enzyme defect as measured by the degree of aberration of PP-Rib-P and purine nucleotide synthesis in fibroblasts. Metabolic abnormalities were more severe in Patient 1's cells than in the cells of most male patients (in whom clinical expression is limited to early adult-onset gout) but were less severe than in the cells of two patients in whom more complex enzyme defects were associated with uric acid overproduction and neurodevelopmental abnormalities (including deafness) in male children and adult women. CONCLUSION: Certain defects resulting in PP-Rib-P synthetase superactivity may be causally related to neurologic impairment, most commonly sensorineural deafness.
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9/13. Nonspecific necrotizing petrositis: an unusual complication of otitis in children.

    Three cases of an unusual form of suppurative chronic otitis media in children 2 and 3 years old are presented. The clinical feature common to all cases was otorrhea, beginning at the first months of life, with a silent evolution to a complete peripheral facial paralysis. In one case the facial paralysis was bilateral. The treatment applied was surgical: subtotal petrosectomy. The surgical findings were bloody granulation tissue, sequestering completely the otic capsule. The facial nerve was destroyed almost the total length of the Fallopian canal in all cases. The histopathologic examination revealed a destructive and nonspecific chronic inflammatory process. The culture showed Gram-negative organisms, such as pseudomonas aeruginosa and proteus mirabilis. Etiologic aspects involved in the process are discussed and the name non-specific necrotizing petrositis is suggested to individualize this rare form of otitis which, with morbidity, leads to severe complications such as facial paralysis and deafness.
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10/13. pasteurella ureae meningitis and septicaemia.

    A 55-year-old male diabetic admitted with deafness, nystagmus, headache and vomiting was found to have meningitis due to pasteurella ureae and responded to treatment with ampicillin. The P. ureae was unusual in showing X dependency. The family's dogs had had ear infections but no P. ureae were recovered from them when cultured three months later.
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