Sensitivity of transient evoked and distortion product otoacoustic emissions to the direct effects of noise on the human cochlea

Measurement of otoacoustic emissions (OAEs) has been proposed as a sensitive test to reliably assess the effects of noise exposure. The present study in humans was designed to evaluate the sensitivity and applicability of transient evoked OAEs (TEOAEs) and 2f1-f2 distortion product OAEs (DPOAEs) as quantitative indices of the functional integrity of the outer hair cells (OHC) during growth of and recovery from temporary threshold shift (TTS). This was examined in two different groups of volunteers by measuring the per- and post-stimulatory effects of a one hour BBN and an on-site five hour exposure to loud music from a discotheque. The results of both experiments show consistent growth and recovery patterns for both DPOAEs and TEOAEs. For TEOAEs, both the reproducibility scores and signal to noise ratio values for the 4 kHz frequency band exhibited the greatest sensitivity. The DPOAEs, on the other hand, showed the greatest sensitivity between 2 and 5.5 kHz. Thus, both the TEOAEs and DPOAEs have a great potential in the detection of TTS after noise exposure.     

Renal Granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature

We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leucocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. Light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1–11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. Conclusion Renal failure, proteinuria, leucocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure. Received: 4 December 1997 / Accepted in revised form: 22 June 1998     

High circulating proviral load with oligoclonal expansion of HTLV-1 bearing T cells in HTLV-1 carriers with strongyloidiasis

Adult T cell leukemia (ATLL) develops in 3 - 5% of HTLV-1 carriers after a long period of latency during which a persistent polyclonal expansion of HTLV-1 infected lymphocytes is observed in all individuals. This incubation period is significantly shortened in HTLV-1 carrier with Strongyloides stercoralis (Ss) infection, suggesting that Ss could be a cofactor of ATLL. As an increased T cell proliferation at the asymptomatic stage of HTLV-1 infection could increase the risk of malignant transformation, the effect of Ss infection on infected T lymphocytes was assessed in vivo in HTLV-1 asymptomatic carriers. After real-time quantitative PCR, the mean circulating HTLV-1 proviral load was more than five times higher in HTLV-1 carriers with strongyloidiasis than in HTLV-1+ individuals without Ss infection (P<0.009). This increased proviral load was found to result from the extensive proliferation of a restricted number of infected clones, i.e. from oligoclonal expansion, as evidenced by the semiquantitative amplification of HTLV-1 flanking sequences. The positive effect of Ss on clonal expansion was reversible under effective treatment of strongyloidiasis in one patient with parasitological cure whereas no significant modification of the HTLV-1 replication pattern was observed in an additional case with strongyloidiasis treatment failure. Therefore, Ss stimulates the oligoclonal proliferation of HTLV-1 infected cells in HTLV-1 asymptomatic carriers in vivo. This is thought to account for the shortened period of latency observed in ATLL patients with strongyloidiasis. Oncogene (2000) 19, 4954 - 4960     

Pallister-Killian syndrome: characterization of the isochromosome 12p by fluorescent in situ hybridization.

The isochromosome 12p (i(12p)) in fibroblasts of 3 patients with Pallister-Killian syndrome and one decreased prematurely born neonate, was characterized by fluorescent in situ hybridization (FISH) using chromosome 12-specific DNA probes. FISH is a useful technique for rapid and reliable detection and characterization of the i(12p) chromosome in Pallister-Killian patients. Detection was possible also in interphase cells. In addition, the in vitro selection against i(12p) cells at different passages in fibroblast cultures of two patients was monitored.     

Combined operation: cataract and glaucoma with implantation. Results of a series of 28 cases

28 eyes underwent a combined procedure: trabeculectomy, extra-capsular cataract extraction and implantation of a posterior chamber intraocular lens. With a minimum of 9 months follow-up, 23 eyes had intraocular pressure controlled of all antiglaucoma medications, and 4 additional eyes achieved control with only the use of timolol maleate drops. (1 eye was lost to follow-up.) Preoperative near visual acuity was less than Parinaud 5 (Jaeger 7) in all eyes. Post-operative near visual acuity was Parinaud 2 (Jaeger 1) in 17 eyes. Senile macular degeneration was responsible for less than Parinaud 2 visual acuity in 4 additional eyes. An extracapsular extraction has advantages over the intracapsular method, both in terms of less post-operative vitreoretinal complications for the cataract extraction itself, and for the creation of a functional trabeculectomy by keeping the vitreous away from the filtration site. Use of a posterior chamber intraocular lens implant as part of the combined procedure appears to be highly acceptable. Complications and limits of the combined procedure are discussed.