Cases reported "Deafness"

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1/18. 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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2/18. 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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3/18. Hearing habilitation with auditory brainstem implantation in two children with cochlear nerve aplasia.

    patients with aplasia and hypoplasia of the cochlear nerve have no chance of having their hearing restored by stimulating the periphery of the auditory system using the traditional cochlear implant. A possible approach to auditory rehabilitation may be direct electrical stimulation of the cochlear nuclei with an auditory brainstem implant (ABI). Recently, two children, aged 4 and 3 years, respectively, with bilateral severe cochlear malformations and cochlear nerve aplasia received an ABI. The present paper reports the technique and the preliminary results of this experience. The classic retrosigmoid approach was used. The correct position of the electrodes was estimated with the aid of EABRs and neural response telemetry (NRT). No postoperative complications were observed. High-resolution CT scans with a bone algorithm reconstruction technique were taken postoperatively to evaluate electrode placement before discharge. The ABI was activated 30 days after implantation in both patients. To date 16 and 13 electrodes, respectively, have been activated in the two children. Three months after activation the first patient had achieved good environmental sound awareness, good speech detection and some speech discrimination. The second child, 1 month after activation, had achieved good environmental sound awareness and moderate speech detection. To the best of our knowledge this is the first report of patients with hypoplasia of the cochlea and aplasia of the cochlear nerve, aged below 5 years and treated with an ABI.
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4/18. Fluctuant, progressive hearing loss associated with Meniere like vertigo in three patients with the Pendred syndrome.

    OBJECTIVE: To evaluate vestibular and long-term audiometric findings in patients with Pendred syndrome. Study design: Retrospective analysis of long-term clinical data. SETTING: University hospital department. patients: Three patients with Pendred syndrome caused by a mutation in the SLC26A4 gene. methods: Perchlorate discharge test, mutation analysis of the SLC26A4 gene, MR imaging of temporal bones, vestibular function test (in two cases) and serial audiometry. A saturation hyperbola with onset age was fitted to the audiometric threshold-on-age data using a nonlinear regression method. The residues remaining after regression were analyzed in a correlation analysis to detect significant ipsilateral or contralateral cofluctuation. RESULTS: All three patients had a mutation in the SLC26A4 gene and bilateral enlarged vestibular aqueduct; two of them had a positive perchlorate discharge test but in one of two siblings this test was negative. hearing loss was significantly progressive with significant ipsilateral and contralateral cofluctuation in all evaluable cases, combined with episodes of Meniere like vertigo in two cases. The episodes of vertigo are as seen in meniere disease. One case had unilateral caloric areflexia and one had bilateral vestibular hyporeflexia, proven to be progressive in a repeat examination. CONCLUSIONS: patients with Pendred syndrome may exhibit progressive and fluctuant hearing loss with episodes of vertigo.
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5/18. Hearing restoration with auditory brainstem implant in three children with cochlear nerve aplasia.

    OBJECTIVE: To verify the possibility of auditory habilitation in children with aplasia and hypoplasia of the cochlear nerve by direct electrical stimulation of the cochlear nuclei with an auditory brainstem implant. STUDY DESIGN: Retrospective case review. SETTING: Study conducted at the Ear, nose, and Throat Department of the University of Verona, italy. patients: Three children, aged 4, 3, and 2 years, respectively, with severe bilateral cochlear malformations and cochlear nerve aplasia have received an auditory brainstem implant at this institution in the past 2 years. INTERVENTION: The classic retrosigmoid approach was used. Correct positioning of the electrodes was evaluated using electric auditory brainstem responses and neural response telemetry. Before the patients were discharged, high-resolution computed tomography with a bone algorithm reconstruction technique was performed to evaluate electrode placement. The auditory brainstem implant was activated 30 to 60 days after implantation. RESULTS: No postoperative complications were observed. To date, 21, 18, and 8 electrodes, respectively, have been activated in the three children. The first patient, 12 months after activation, had achieved good environmental sound awareness, good speech detection, and some speech recognition. The second child, 8 months after activation, had achieved good environmental sound awareness and moderate speech detection. The third patient, 1 month after activation, had obtained good environmental sound awareness. CONCLUSION: This study indicates that auditory brainstem implantation is technically feasible in children with cochlear nerve aplasia. The early results suggest the possibility of achieving auditory habilitation with auditory brainstem implantation in this population.
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6/18. Pendred's syndrome.

    This report describes Pendred's syndrome in three siblings of a consanguineous marriage, belonging to Rahim YarKhan. The children presented with deafmutism and goiters. The investigations included scintigram, perchlorate discharge test and audiometery. The perchlorate discharge was positive in index case. Bilateral sensorineural hearing defect was detected on Pure Tone Average (PTA) audiometry. Meticulous clinical and laboratory evaluation is mandatory for the detection of rare disorders like Pendred's syndrome.
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7/18. genetic heterogeneity of KID syndrome: identification of a Cx30 gene (GJB6) mutation in a patient with KID syndrome and congenital atrichia.

    connexins are integral membrane proteins forming aqueous gap junction channels that allow the diffusional exchange of ions and small metabolites between cells, thus coordinating metabolic activities in multicellular tissues. Dominant mutations in the Cx26 gene GJB2 have been shown to cause keratitis-ichthyosis-deafness (KID) syndrome, palmoplantar keratoderma associated with hearing loss, and Vohwinkel syndrome. Missense mutations in the closely related Cx30 gene GJB6 underlie Clouston syndrome (autosomal dominant hidrotic ectodermal dysplasia). We report a 6-y-old boy with phenotypic characteristics of KID syndrome as well as atrichia. In contrast to other KID syndrome patients, molecular analysis of the connexin gene GJB2 did not disclose a pathogenic mutation, although the patient was homozygous for a common polymorphism (V27I) in the coding sequence of Cx26. Nevertheless, screening of GJB6 revealed a heterozygous missense mutation (V37E) predicted to alter sequence and charge of the first transmembrane helix of Cx30, which was previously implicated in Clouston syndrome (Smith et al, 2002). The presence of a pathogenic Cx30 mutation and the lack of a pathologic molecular change in Cx26 in this patient, whose clinical features predominantly resemble KID syndrome, suggest genetic heterogeneity of KID syndrome and underscore that mutations in Cx30, similar to those in Cx26 or Cx31, can cause different phenotypes. Based on our results, connexin gene mutations should be considered in patients presenting with congenital sensorineural hearing loss and disorders of cornification, and screening of several connexin genes with known cutaneous phenotype, such as those for Cx26, Cx30, Cx30.3, and Cx31, may be required.
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8/18. Isolated deafness following recovery from neurologic injury and adult respiratory distress syndrome. A sequela of intercurrent aminoglycoside and diuretic use.

    We report two children who survived neurologic injury (near-drowning and Reye's syndrome) and adult respiratory distress syndrome and who required prolonged ventilatory support. Follow-up examination in both children showed steady neurologic recovery, but five months following discharge from their acute illness, profound hearing loss was diagnosed in both children. A review of the literature is reported and the hypothesis that combined aminoglycoside antibiotic and loop diuretic therapy caused the hearing loss is presented. Recommendation is made for audiologic assessment within six months of recovery from critical illness of pediatric patients in whom therapy has included loop diuretic and aminoglycoside antibiotic therapy.
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9/18. early diagnosis and evolution of deafness in childhood bacterial meningitis: a study using brainstem auditory evoked potentials.

    Fifty-one children with bacterial meningitis were studied prospectively using serial recordings of brainstem auditory evoked potential (BAEP) from the earliest phase of the disease, according to a standardized protocol. The objectives were to make an early diagnosis and follow the evolution of deafness in the course of meningitis and evaluate the prognostic value of BAEP. Thirty-five children (68.6%) always had normal recordings; 11 children (21.6%) had transient BAEP abnormalities (prolonged wave V latency or elevated threshold for wave I), and five children (9.8%) had persistent pathologic BAEP recordings from the first examination at 48 hours until discharge from the hospital and have a persistent deafness. All recordings that were normal or pathologic at discharge were confirmed by behavioral audiometry 3 months later. These results show the early occurrence of deafness in the course of meningitis with a crucial phase of possible recovery or worsening occurring during the first 2 weeks. There were no cases of "late" deafness or "late" recovery (there was sometimes slight improvement) occurring after discharge; thus BAEPs have a prognostic value. However, observation of a child with clinically proven selective high-frequency postmeningitic deficit but without a hearing handicap, a disorder that was diagnosed early with BAEP (which tests only the high frequencies), is a warning that this method alone is insufficient and that clinical auditory surveillance and conventional audiometry remain necessary.
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10/18. Intrauterinely acquired Pseudomonas infection in the neonate.

    The case is presented of a premature infant with pseudomonas aeruginosa infection, apparently acquired in utero. After a complicated postnatal course, the child was noted to have a profound hearing loss. This infection itself, was rapid and progressive, with the infant showing signs and symptoms characteristic of Pseudomonas infection, such as necrotizing skin vasculitis and "green" purulent discharge. Pseudomonas infection poses a virulent and life-threatening challenge to the immunologically immature infant. infection with this organism, uncommon in the neonate, results in significant morbidity and mortality.
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