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1/14. metronidazole ototoxicity--report of two cases.

    Two cases of bilateral moderate to severe sensorineural hearing loss due to oral administration of metronidazole are reported. There has been only one case report of deafness following metronidazole therapy in the world literature. The hearing loss recovered gradually in a period of four to six weeks following withdrawal of drug and oral steroid therapy. The possible mechanism of ototoxicity is discussed. awareness by the treating physician of ototoxicity due to any drug is stressed.
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2/14. Conservative facial nerve management in jugular foramen schwannomas.

    OBJECTIVE: Although transposition of the facial nerve is crucial in infiltrative vascular lesions involving the jugular foramen, the objective was to show that a conservative approach to management of the facial nerve is sufficient with jugular foramen neuromas because of their noninfiltrative, less vascular nature and medial location in the jugular foramen. STUDY DESIGN: Retrospective case review. SETTING: Tertiary, private, multiphysician, otologic practice. patients: Sixteen patients with jugular foramen schwannoma (18 procedures) treated between January 1975 and October 1995. The 8 male and 8 female patients ranged in age from 13 to 66 years (mean age 47.7 years). INTERVENTION: One-stage, total jugular foramen neuroma removal without transposition of the facial nerve, using a variety of surgical approaches. MAIN OUTCOME MEASURES: facial nerve transposition (yes or no), House-Brackmann facial nerve grade, lower cranial nerve status, complications. RESULTS: One-stage total tumor removal was accomplished in all the cases. In 13 (72%) of the neuromas, removal was accomplished without facial nerve transposition. Transposition was performed in 2 revision cases in which scar tissue from a previous operation prevented complete control of the carotid artery and safe removal, 2 cases with large tumor extension anteriorly to the petrous apex, and 1 case with extensive involvement of the middle ear. A House-Brackmann facial nerve Grade I or II was obtained in 16 of the 18 procedures, with 1 Grade III and 1 case that remained Grade V, as it was preoperatively. CONCLUSIONS: One-stage, total tumor removal can be achieved with excellent control of the important vascular structures and without transposition of the facial nerve in a majority of jugular foramen schwannomas.
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3/14. mtDNA disease in the primary care setting.

    Disorders of mitochondrial dna (mtDNA) may commonly present to primary care physicians but go undiagnosed. A 36-year-old man with a 15-year history of psychosis, seizures, and sensorineural hearing loss and a family history of diabetes mellitus and heart disease presented to our hospital without a unifying diagnosis. Physiologic, biochemical, and genetic testing revealed deficient aerobic metabolism, a defect in mitochondrial electron transport, and the presence of an A-to-G point mutation at position 3243 of the mitochondrial leucine-transfer rna gene, establishing the diagnosis of mitochondrial encephalopathy, lactic acidosis, and strokelike syndrome (MELAS). Diagnosing mtDNA disorders requires a careful integration of clinical signs and symptoms with pedigree analysis and multidisciplinary testing. Diagnosis is important to provide genetic counseling, avoid unnecessary evaluation, and facilitate therapy for symptomatic relief.
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4/14. Autoimmune sensorineural hearing loss improved by tumor necrosis factor-alpha blockade: a case report.

    Autoimmune inner ear disease is a treatable cause of sensorineural hearing loss and it is important for physicians and hearing health professionals to recognize that early diagnosis and proper management strategies may result in stabilization and improvement in hearing. The pathogenesis of autoimmune sensorineural hearing loss remains unclear but antibodies directed against the inner ear and/or cellular effectors have been proposed. Therefore, immunosuppressive drugs such as steroids and methotrexate are administered to interfere with the progression of hearing loss and in some cases have been found to improve hearing. We report herein the history of a patient who was treated by systemic administration of anti-tumor necrosis factor-alpha antibodies for Crohn's disease and who also had associated sensorineural hearing loss. Audiometric follow-up revealed not only the efficacy of tumor necrosis factor-alpha blockade in arresting the hearing loss but also an improvement in hearing of 15 dB on average across all frequencies. Hearing remained stable afterwards.
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5/14. Massive endolymphatic sac and vestibular aqueduct in Mondini dysplasia.

    A postmortem study was performed on the bilateral temporal bones of a 90-year-old woman who had a lifelong profound hearing impairment. The histopathologic findings included severe Mondini dysplasia with unusual enlargement of the vestibular aqueducts and endolymphatic sacs. Dehiscent carotid arteries and dehiscent facial nerves were also present. The clinical and surgical aspects of the case are discussed based on the histopathologic findings. The physician should be prepared to diagnose congenital hearing impairment promptly to avoid complications during the clinical and surgical treatment of patients with Mondini dysplasia.
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6/14. Acute hearing loss after airbag deployment.

    hearing loss from airbag deployment after motor vehicle crashes has been documented multiple times in the adult population, but rarely in the pediatric population. We report a case of significant sensorineural hearing loss following airbag deployment in a pediatric patient who presented to the ED on the day of the crash. This report highlights the need for pediatric physicians to be aware of the potential of minor to significant hearing loss in patients who present for medical evaluation after airbag deployment in motor vehicle crashes and to be aware of the importance of follow-up in these children.
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7/14. Acute sensorineural hearing loss associated with peginterferon and ribavirin combination therapy during hepatitis c treatment: outcome after resumption of therapy.

    Peginterferon and ribavirin combination therapy for the treatment of hepatitis c virus (HCV) is well known to be associated with significant adverse effects. Sensorineural hearing loss, that in most cases is unilateral, has been reported as a consequence of therapy with both non-pegylated and pegylated interferon (pegIFN) but is not a well-known adverse effect. We report a 45-year-old Caucasian woman who developed acute sensorineural hearing loss 2 mo after starting therapy with pegIFN-alpha 2b and ribavirin for the treatment of chronic HCV, genotype 1a. She did not report the hearing loss to the hepatitis clinic until 1 mo, later whereupon therapy was promptly discontinued. Although her serum alanine aminotransferase (ALT) normalized and her HCV-rna became undetectable after 12 wk of pegIFN and ribavirin therapy, after discontinuation, her HCV-rna became detectable with significant elevations of serum ALT. Four months after initial discontinuation, the patient re-commenced pegIFN and ribavirin combination therapy. After 44 of 48 wk of therapy, the patient's liver biochemistry has normalized and the HCV-rna is undetectable. She has not developed worsening of her hearing loss and hearing on the left-side is unaffected. Both patients and physicians should be aware that sensorineural hearing loss may occur with pegIFN therapy. Our experience suggests that re-institution of therapy is not always associated with further hearing impairment.
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8/14. Transient hemiageusia in cerebrovascular lateral pontine lesions.

    knowledge of human central taste pathways is largely based on textbook (anatomical dissections) and animal (electrophysiology in vivo) data. It is only recently that further functional insight into human central gustatory pathways has been achieved. magnetic resonance imaging studies, especially selective imaging of vascular, tumoral, or inflammatory lesions in humans has made this possible. However, some questions remain, particularly regarding the exact crossing site of human gustatory afferences. We present a patient with a pontine stroke after a vertebral artery thrombosis. The patient had infarctions in areas supplied by the anterior inferior cerebellar artery and showed vertical diplopia, right sided deafness, right facial palsy, and transient hemiageusia. A review of the sparse literature of central taste disorders and food preference changes after strokes with a focus on hemiageusia cases is provided. This case offers new evidence suggesting that the central gustatory pathway in humans runs ipsilaterally within the pons and crosses at a higher, probably midbrain level. In patients with central lesions, little attention has been given to taste disorders. They may often go unnoticed by the physician and/or the patient. Central lesions involving taste pathways seem to generate perceptions of quantitative taste disorders (hemiageusia or hypogeusia), in contrast to peripheral gustatory lesions that are hardly recognised as quantitative but sometimes as qualitative (dysgeusia) taste disorders by patients.
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9/14. Sudden sensorineural hearing loss.

    Sudden sensorineural hearing loss has many causes and treatments. The family physician can identify the hearing loss, perform audiometry, identify likely systemic causes and counsel the patient on the prognosis. Most patients are ultimately found to have idiopathic sensorineural hearing loss, and the spontaneous recovery rate is high. If no improvement occurs within one month, evaluation to rule out tumor is recommended.
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10/14. erythromycin ototoxicity: analysis and conclusions based on 22 case reports.

    Although the majority of patients receiving erythromycin experience no hearing loss, certain patients are susceptible. erythromycin ototoxicity data were analyzed on 20 patients from 13 reports in the English language literature as well as on two patients from the University of missouri-Columbia. patients were classified with respect to age, sex, premorbid diagnosis, renal/hepatic function, type of erythromycin used, dosage, duration of therapy, and route of administration. The following auditory effects were considered: onset of symptoms, degree and configuration of hearing loss, and recovery of hearing. The factors which, when combined with the use of high-dose erythromycin (greater than or equal to 2 gm/day), might place patients at risk for erythromycin ototoxicity are preexisting renal or hepatic disease, age (elderly), and perhaps being female. With knowledge of the predisposing factors and auditory effects, the clinician can more easily recognize hearing loss caused by erythromycin and properly counsel referring physicians and affected patients.
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