Hearing rehabilitation with a binaural cochlear implant in a patient with Erdheim-Chester disease.

OBJECTIVE AND IMPORTANCE: Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. This paper reports an exceptional case of bilateral neural involvement, responsible for profound hearing loss. Bilateral cochlear implantation was performed. CLINICAL PRESENTATION: We present a 57-year-old man affected by ECD with profound bilateral hearing loss. INTERVENTION: The patient underwent cochlear implantation with a binaural Digisonic(®) cochlear implant, 7 years after the initial diagnosis. RESULTS: Speech intelligibility rose to a plateau after about 6 months of cochlear implant use. The average outcome of speech intelligibility over time was 55% for dissyllabic words without lip reading, and 70% for sentences. Perception score decreased before the patient died from ECD. CONCLUSION: A description of the ECD and its otological manifestations is presented. This paper reports the effective hearing rehabilitation of profound bilateral hearing loss by the means of a binaural Digisonic(®) cochlear implant.

Yield of bone marrow examination in diagnosing the source of fever of unknown origin.

BACKGROUND: Fever of unknown origin (FUO) still remains a diagnostic challenge, while diagnosis may remain obscure for several weeks or months. The role of tissue biopsy is crucial in the diagnostic approach. We report a series of 130 consecutive patients with FUO who had undergone a bone marrow biopsy (BMB). METHODS: Among 280 consecutive nonimmunocompromised patients hospitalized between 1995 and 2005 for a febrile illness of uncertain cause, lasting at least 3 weeks, with no diagnosis after an appropriate minimal diagnostic workup, 130 underwent BMB. RESULTS: Overall, a specific diagnosis was achieved by BMB and histological examination in 31 cases (diagnostic yield, 23.7%). Three types of diseases were found: hematological malignant diseases in 25 cases, including 19 patients with malignant lymphoma, 4 with acute leukemia, 1 with hairy cell leukemia, and 1 with multiple myeloma; infectious diseases in 3 cases; systemic mastocytosis in 2 cases; and disseminated granulomatosis in 1 case. Thrombocytopenia (odds ratio, 4.9; 95% confidence interval, 1.04-9.30) and anemia (odds ratio, 3.24; 95% CI, 1.13-9.34) were the most reliable predictive factors regarding the usefulness of BMB. Bone marrow cultures had very limited value in our cohort. Finally, corticosteroid use did not seem to affect the yield of BMB. CONCLUSIONS: Bone marrow biopsy is a useful technique for the diagnosis of prolonged fever in immunocompetent patients. Thrombocytopenia and anemia seem to be correlated with the value of this test.

Adult Kawasaki disease: report of two cases and literature review.

OBJECTIVES: To describe 2 cases of adult Kawasaki Disease (KD) and to review the medical literature to better define the epidemiological, clinical, laboratory, histopathological, cardiovascular, and therapeutic aspects of adult KD compared with pediatric KD. METHODS: Report of 2 cases, and review of the literature using a Medline search from 1967 to June 2003. RESULTS: Including our 2 cases, there are 57 reports of adult KD, 74% among patients aged 18 to 30 years. Nine cases of KD associated with human immunodeficiency virus (HIV) infection were described, suggesting that an immunocompromised state may predispose to this syndrome. The incidence of specific diagnostic criteria was roughly similar in adults and in children. However, cheilitis, meningitis, and thrombocytosis were observed in a larger percentage of children, while arthralgia, adenopathy, and liver function abnormality were more common in adults. Although adult KD often was diagnosed after the acute phase, when a significant beneficial effect from gammaglobulin infusion could not be expected, this treatment did appear to shorten the course of the disease. Coronary aneurysms were less frequent in adults than in children. Prognosis was more favorable in adults, with less cardiovascular complications and no deaths. CONCLUSIONS: Adult KD is a rare condition, which may go unrecognized. Other known disease processes with similar clinical presentations such as hypersensitivity drug reaction and toxic shock syndrome must be ruled out. For adult KD, exclusion criteria such as absence of hypotension, visceral impairment, staphylococcal infection, and any drug able to induce a drug hypersensitivity reaction are suggestive of the diagnosis, in the presence of the inclusion criteria, rash, conjunctival effusion, oropharynx changes, extremity changes, or adenopathy.

Brain abscesses caused by Abiotrophia defectiva: complication of immunosuppressive therapy in a patient with connective-tissue disease.

We report the case of a patient who developed brain abscesses caused by Abiotrophia defectiva. The patient was treated with prednisone and cyclophosphamide for connective-tissue disease (lupus-Sjögren's overlap syndrome). A few cases of central nervous system infections due to Abiotrophia species have been previously reported in immunocompetent patients following neurosurgical procedures, suggesting possible introduction of this organism at the time of surgery. This case report is the first report of brain abscesses due to A. defectiva in a pharmacologically immunosuppressed patient.