Young deaf children with hearing aids or cochlear implants: early assessment package for monitoring progress

BACKGROUND: Very few assessment measures exist for evaluating progress in young deaf children with hearing aids and cochlear implants. OBJECTIVE: To introduce and describe an early assessment package that covers auditory perception, communication/language development, and speech production in very young deaf children. MAIN OUTCOME MEASURES: Seven of the assessment measures (Listening Progress Profile, Categories of Auditory Performance, Tait Video Analysis, Stories-Narratives Assessment Procedure, Profile of Actual Linguistic Skills, Speech Intelligibility Rating, and the Profile of Actual Speech Skills) have been specifically developed at the Nottingham Cochlear Implant Programme, and a further one (Meaningful Auditory Integration Scale) was modified for use within the package. Moreover, two commercially available tests (Pragmatics Profile of Everyday Communication Skills and Preschool Language Scale) are included to complete the package. METHODS: The present paper describes each measure, how to use it, and its time frame. In addition, two case studies demonstrate the usefulness of the package as a whole. RESULTS AND CONCLUSIONS: The Nottingham Early Assessment Package (NEAP) offers a framework with which to assess in young deaf children the use of audition and language and communication in real-life situations. Being simple, reliable, and time effective can be used in everyday clinical practice. NEAP is innovative in design and offers a structured approach to monitor very young deaf children, both in short and long term. In addition, it allows the identification of additional problems and areas of difficulty as well as specific abilities and skills. This enables the clinician to determine appropriate intervention strategies.     

A submillimeter resolution fluorescence molecular imaging system for small animal imaging

Most current imaging systems developed for tomographic investigations of intact tissues using diffuse photons suffer from a limited number of sources and detectors. In this paper we describe the construction and evaluation of a large dataset, low noise tomographic system for fluorescence imaging in small animals. The system consists of a parallel plate-imaging chamber and a lens coupled CCD camera, which enables conventional planar imaging as well as fluorescence tomography. The planar imaging data are used to guide the acquisition of a Fluorescence Molecular Tomography (FMT) dataset containing more than 106 measurements, and to superimpose anatomical features with tomographic results for improved visual representation. Experimental measurements exhibited good agreement with the diffusion theory models used to predict light propagation within the chamber. Tests of the instrument's capacity to quantitatively reconstruct fluorochrome distributions in three dimensions showed less than 5% errors between actual fluorochrome concentrations and FMT findings, and suggested a detection threshold of approximately 100 femptomoles for small localized objects. Experiments to assess the instrument's spatial resolution demonstrated the ability of the system to resolve objects placed at clear distances of less than 1 mm. This is a significant resolution increase over previously developed systems for animal imaging, and is primarily due to the large dataset employed and the use of inversion methods. Finally, the in vivo imaging capacity is showcased. It is expected that the large dataset collected can enable superior imaging of molecular probes in vivo and improve quantification of fluorescence signatures.     

Recurrent ceruminous adenocarcinoma of the external auditory canal

Ceruminous adenocarcinoma is a rare malignant neoplasm of the glandular structures of the external auditory canal. The true incidence and behavior of these rare tumors are still unclear due to confusing terminology, classification and histological definitions. Therefore, the ENT surgeon faces major difficulties in choosing the method of management--conservative or more radical surgery--with the addition or not of radiotherapy. We report a 57-year-old male patient with a recurrence of a previously excised (maybe partially) and irradiated ceruminous adenocarcinoma of the right external auditory canal. Aggressive surgery was considered as the treatment of choice. However, the patient refused this approach and, as a consequence, a conservative excision was performed but with histologically confirmed healthy margins. To our surprise, the patient showed an excellent response and he is disease free 3 years following the last operation. Although recurrences usually occur within months after inadequate management, some may happen even 7 years post treatment. Therefore, routine long-term follow-up was advised.     

Long-term outcome of patients with avascular necrosis,after internal fixation of femoral neck fractures

We retrospectively reviewed 84 patients who underwent internal fixation of an intracapsular femoral neck fracture. The mean age was 58 years and the time from injury to operative treatment was 5.3 days. The mean follow-up was 4.7 years (range, 2-8 years). At the latest follow-up, in the 46 patients with undisplaced (Garden I, II) fractures, nonunion occurred in two patients and avascular necrosis of the femoral head in nine. Six of these nine patients had a good or excellent result, one had a fair result, and two had a poor result. Of 35 patients with no sign of avascular necrosis, 32 patients had a good or excellent result, two a fair and one had a poor result. In the group of 38 patients with displaced (Garden III, IV) fractures, nonunion occurred in six patients and avascular necrosis of the femoral head in 15. Of these 15 patients, 10 had a good or excellent result, two had a fair result, and three had a poor result. Of 17 patients with no sign of avascular necrosis, 14 had an excellent result and three patients a poor result. Overall only five of the 24 patients who developed avascular necrosis of the femoral head had undergone total hip arthroplasty. Internal fixation remains a simple and safe, method of treatment for both undisplaced and displaced femoral neck fractures in middle-age patients. Despite the relatively high rate of avascular necrosis after internal fixation of femoral neck fractures, only a few of these patients (20%) required further surgical treatment in the follow-up period of this study.     

Role of mitochondrial aldehyde dehydrogenase in nitrate tolerance

Glyceryl trinitrate (GTN) is used in the treatment of angina pectoris and cardiac failure, but the rapid onset of GTN tolerance limits its clinical utility. Research suggests that a principal cause of tolerance is inhibition of an enzyme responsible for the production of physiologically active concentrations of NO from GTN. This enzyme has not conclusively been identified. However, the mitochondrial aldehyde dehydrogenase (ALDH2) is inhibited in GTN-tolerant tissues and produces NO2- from GTN, which is proposed to be converted to NO within mitochondria. To investigate the role of this enzyme in GTN tolerance, cumulative GTN concentration-response curves were obtained for both GTN-tolerant and -nontolerant rat aortic rings treated with the ALDH inhibitor cyanamide or the ALDH substrate propionaldehyde. Tolerance to GTN was induced using both in vivo and in vitro protocols. The in vivo protocol resulted in almost complete inhibition of ALDH2 activity and GTN biotransformation in hepatic mitochondria, indicating that long-term GTN exposure results in inactivation of the enzyme. Treatment with cyanamide or propionaldehyde caused a dose-dependent increase in the EC50 value for GTN-induced relaxation of similar magnitude in both tolerant and nontolerant aorta, suggesting that although cyanamide and propionaldehyde inhibit GTN-induced vasodilation, these inhibitors do not affect the enzyme or system involved in tolerance development to GTN. Treatment with cyanamide or propionaldehyde did not significantly inhibit 1,1-diethyl-2-hydroxy-2-nitrosohydrazine-mediated vasodilation in tolerant or nontolerant aorta, indicating that these ALDH inhibitors do not affect the downstream effectors of NO-induced vasodilation. Immunoblot analysis indicated that the majority of vascular ALDH2 is present in the cytoplasm, suggesting that mitochondrial biotransformation of GTN by ALDH2 plays a minor role in the overall vascular biotransformation of GTN by this enzyme.     

Effect of atorvastatin on the concentration,relative distribution,and chemical composition of lipoprotein subfractions in patients with dyslipidemias of type IIA and IIB

The authors investigated the effect of atorvastatin (40 mg qd) on low-density lipoprotein (LDL) particle distribution in patients with dyslipidemias of type IIA (n = 55) and IIB (n = 21). Atorvastatin therapy induced a significant decrease in total and LDL cholesterol in both patient groups. A significant reduction in triglyceride values, which was more profound in type IIB patients, was also observed. In type IIA patients, LDL-3 was the predominant subfraction. Atorvastatin therapy induced a significant reduction in total LDL mass in this group of patients that was mainly due to the reduction in large and intermediate subspecies (LDL-1 to LDL-3), whereas the mass of dense LDL particles (LDL-4 and LDL-5) remained unchanged. As a consequence, the percentage contribution of dense subfractions to the total LDL mass increased significantly after atorvastatin therapy. The dense LDL-4 subfraction was the predominant one in type IIB patients. In this group, atorvastatin therapy resulted in a significant reduction in the total LDL mass, which was due to the reduction in all LDL subfractions. Thus, the percentage mass distribution of LDL particles remained unaffected. These results suggest that the effect of atorvastatin on LDL subfractions is affected by the underlying genetic defect.     

Biweekly administration of low-dose docetaxel in hormone-resistant prostate cancer: pilot study of an effective subtoxic therapy

Taxane-based chemotherapy has shown activity but also toxicity when administered at standard doses in patients with hormone-resistant prostate cancer (HRPC). In this pilot study, we investigated biweekly low-dose docetaxel in patients with HRPC as a convenient regimen with low toxicity. Sixteen patients with metastatic HRPC entered the study. Median age was 73 years, median performance status (PS) was 2, and median Gleason score was 9. All patients had undergone and failed combined androgen-blockade therapy (luteinizing hormone-releasing hormone analogue plus antiandrogen) for their metastatic disease; 3 had also been treated with mitoxantrone. Treatment consisted of docetaxel 30 mg/m2 administered every 2 weeks. Prostate-specific antigen (PSA) response, characterized by a 50% decrease of PSA level confirmed 4 weeks later, was the primary endpoint. Durations of PSA response and toxicity assessment were secondary endpoints. A total of 136 biweekly docetaxel doses were administered, with a median of 8.5 doses per patient (range, 2-24). Six patients (38%; 95% confidence interval, 25%-43%) fulfilled the criteria of PSA response. Median duration of PSA response was 4.5 months (range, 3-12). Toxicity was negligible: myelotoxicity was practically absent, whereas 3 patients developed grade 1 alopecia and 1 patient developed dacryorrhea. We conclude that our study provides evidence that biweekly docetaxel at 30 mg/m2 can be considered an effective nontoxic therapeutic option for patients with HRPC. Confirmation of these preliminary data in larger-scale trials is justified     

Giant adrenal myelolipoma with minimal fat content in a patient with homozygous beta-thalassemia: appearance on MRI

Giant adrenal myelolipoma is an uncommon entity. We present the atypical MR imaging findings of a giant adrenal myelolipoma in a patient with homozygous beta-thalassemia with histopathology correlation. The tumor showed a drop in signal on the opposed-phase images, with no evidence of macroscopic fat contents, and demonstrated very high signal intensity on T2-weighted images. Giant adrenal myelolipoma should be considered in the differential diagnosis of tumors with the combination of the above MR imaging characteristics.