Transient “deafness” accompanies auditory development during metamorphosis from tadpole to frog

During metamorphosis, ranid frogs shift from a purely aquatic to a partly terrestrial lifestyle. The central auditory system undergoes functional and neuroanatomical reorganization in parallel with the development of new sound conduction pathways adapted for the detection of airborne sounds. Neural responses to sounds can be recorded from the auditory midbrain of tadpoles shortly after hatching, with higher rates of synchronous neural activity and lower sharpness of tuning than observed in postmetamorphic animals. Shortly before the onset of metamorphic climax, there is a brief “deaf” period during which no auditory activity can be evoked from the midbrain, and a loss of connectivity is observed between medullary and midbrain auditory nuclei. During the final stages of metamorphic development, auditory function and neural connectivity are restored. The acoustic communication system of the adult frog emerges from these periods of anatomical and physiological plasticity during metamorphosis.

Human deafness dystonia syndrome is a mitochondrial disease

The human deafness dystonia syndrome results from the mutation of a protein (DDP) of unknown function. We show now that DDP is a mitochondrial protein and similar to five small proteins (Tim8p, Tim9p, Tim10p, Tim12p, and Tim13p) of the yeast mitochondrial intermembrane space. Tim9p, Tim10p, and Tim12p mediate the import of metabolite transporters from the cytoplasm into the mitochondrial inner membrane and interact structurally and functionally with Tim8p and Tim13p. DDP is most similar to Tim8p. Tim8p exists as a soluble 70-kDa complex with Tim13p and Tim9p, and deletion of Tim8p is synthetically lethal with a conditional mutation in Tim10p. The deafness dystonia syndrome thus is a novel type of mitochondrial disease that probably is caused by a defective mitochondrial protein-import system.

Steps in theory-of-mind development for children with deafness or autism

Prior research demonstrates that understanding theory of mind (ToM) is seriously and similarly delayed in late-signing deaf children and children with autism. Are these children simply delayed in timing relative to typical children, or do they demonstrate different patterns of development? The current research addressed this question by testing 145 children (ranging from 3 to 13 years) with deafness, autism, or typical development using a ToM scale. Results indicate that all groups followed the same sequence of steps, up to a point, but that children with autism showed an importantly different sequence of understandings (in the later steps of the progression) relative to all other groups.

The role of audiometry in the prevention of occupational deafness

The research set out to test three main hypotheses derived from a summary of literature relevant to the use of audiometry in industry. These hypotheses were: (1) performing audiometry increases the probability that hearing protectors, once issued, will be worn; (2) audiometry is considered by workers to be evidence of their employer's concern for their welfare; (3) audiometry is associated with common law claims by workers against employers for alleged occupational deafness. Six subsidiary hypotheses were also developed. Four methods of data collection were used: (1) attitude questionnaires were administered to samples of workers drawn from an industrial company performing audiometry and two industrial companies not performing audiometry; (2) a postal questionnaire was sent out to industrial medical officers; (3) surveys were undertaken to assess the proportion of the workforce in each of eight industrial companies that was wearing personal hearing protectors that had been provided; (4) structured interviews were carried out with relevant management level personnel in each of five industrial companies. Factor analysis was the main statistical analytic technique used. The data supported all three main hypotheses. Audiometry was also examined as an example of medical screening procedure. It was argued that the validation of medical screening procedures requires the satisfaction of attitudinal or motivational validation criteria in addition to the biological and economic criteria currently used. It was concluded that industrial audiometry failed to satisfy such attitudinal or motivational criteria and so should not be part of a programme of screening for occupational deafness. It was also concluded that industrial audiometry may be useful in creating awareness, amongst workers, of occupational deafness. It was argued that the only profitable approach to investigating the role of audiometry in preventing occupational deafness is to study the attitudes and perceptions of everyone involved.

Validation of an Australian sign language instrument of outcome measurement for adults in mental health settings

Objective: There are currently no adult mental health outcome measures that have been translated into Australian sign language (Auslan). Without a valid and reliable Auslan outcome measure, empirical research into the efficacy of mental health interventions for sign language users is unattainable. To address this research problem the Outcome Rating Scale (ORS), a measure of general functioning, was translated into Auslan and recorded on to digital video disk for use in clinical settings. The purpose of the present study was therefore to examine the reliability, validity and acceptability of an Auslan version of the ORS (ORS-Auslan). Method: The ORS-Auslan was administered to 44 deaf people who use Auslan as their first language and who identify as members of a deaf community (termed ‘Deaf’ people) on their first presentation to a mental health or counselling facility and to 55 Deaf people in the general community. The community sample also completed an Auslan version of the Depression Anxiety Stress Scale-21 (DASS-21). Results: t-Tests indicated significant differences between the mean scores for the clinical and community sample. Internal consistency was acceptable given the low number of items in the ORS-Auslan. Construct validity was established by significant correlations between total scores on the DASS-21-Auslan and ORS-Auslan. Acceptability of ORS-Auslan was evident in the completion rate of 93% compared with 63% for DASS-21-Auslan. Conclusions: This is the only Auslan outcome measure available that can be used across a wide variety of mental health and clinical settings. The ORS-Auslan provides mental health clinicians with a reliable and valid, brief measure of general functioning that can significantly distinguish between clinical and non-clinical presentations for members of the Deaf community.

The development and evaluation of a culturally affirmative counselling model for deaf clients in Australia

In Australia, there is only one, newly established, dedicated mental health service catering specifically for the signing *Deaf community. It is staffed by four part-time hearing professionals and based in Brisbane. There are currently no Deaf psychologists or psychiatrists and there is no valid or reliable empirical evidence on outcomes for Deaf people accessing specialised or mainstream mental health services. Further compounding these issues, is the fact that there are no sign language versions of the most common standardised mental health or psychological instruments available to clinicians in Australia. Contemporary counselling literature is acknowledging the role of the therapeutic alliance and the impact of 'common factors' on therapeutic outcomes. However, these issues are complicated by the relationship between the Deaf client and the hearing therapist being a cross-cultural exchange. The disability model of deafness is contentious and few professionals in Australia have the requisite knowledge and understanding of deafness from a cultural perspective to attend to the therapeutic relationship with this in mind. Consequently, Deaf people are severely disadvantaged by the current lack of services, resources and skilled professionals in the field of deafness and psychology in this country. The primary aim of the following program of research has been to propose a model for culturally affirmative service delivery and to provide clinicians with tools to evaluate the effect of their therapeutic work with Deaf people seeking mental health treatment. The research document is presented as a thesis by publication and comprises four specific objectives formulated in response to the lack of existing services and resources. The first objective was to explore the use of social constructionist counselling techniques and a reflecting team with Deaf clients, hearing therapists and an interpreter. Following the establishment of a pilot counselling clinic, indepth semi-structured interviews were conducted with two long-term clients following the one year pilot of this service. These interviews generated recommendations for the development of a new 'enriched' model of counselling to be implemented and evaluated in later stages of the research program. The second objective was to identify appropriate psychometric measures that could be translated into Australian Sign Language (Auslan) for research into efficacy, effectiveness and counselling outcomes. Two instruments were identified as potentially suitable; the Outcome Rating Scale (ORS), a measure of global functioning, and the Session Rating Scale (SRS), a measure of therapeutic alliance. A specialised team of bi-lingual and bi-cultural interpreters, native signers and the primary researcher for this thesis, produced the ORS-Auslan and the SRS-Auslan in DVD format, using the translation and back-translation process. The third objective was to establish the validity and reliability of these new Auslan measures based on normative data from the Deaf community. Data from the ORS-Auslan was collected from one clinical and one non-clinical sample of Deaf people. Statistical analyses revealed that the ORS-Auslan is reliable, valid and adequately distinguishes between clinical and non-clinical presentations. Furthermore, construct validity has been established using a yet to be validated sign language version of the Depression, Anxiety and Stress Scale-21 items (DASS-21), providing a platform for further research using the DASS-21 with Deaf people. The fourth objective was to evaluate counselling outcomes following the implementation of an enriched counselling service, based on the findings generated by the first objective, and using the newly translated Auslan measures. A second university counselling clinic was established and implemented over the course of one year. Practice-based evidence guided the research and the ORS-Auslan and the SRS-Auslan were administered at every session and provided outcome data on Deaf clients' global functioning. Data from six clients over the course of ten months indicated that this culturally affirmative model was an effective approach for these six clients. This is the first time that outcome data have been collected in Australia using valid and reliable Auslan measures to establish preliminary evidence for the effectiveness of any therapeutic intervention for clinical work with adult, signing Deaf clients. The research generated by this thesis contributes theoretical knowledge, professional development and practical resources that can be used by a variety of mental health clinicians in the context of mental health service delivery to Deaf clients in Australia.

Preventing the selection of “deaf embryos” under the Human Fertilisation and Embryology Act 2008 : problematizing disability?

Section 14(4) of the Human Fertilisation and Embryology Act 2008 imposes – within the general licensing conditions listed in the Human Fertilisation and Embryology Act 1990 – a prohibition to prevent the selection and implantation of embryos for the purpose of creating a child who will be born with a “serious disability.” This article offers a perspective that demonstrates the problematic nature of the consultation, review, and legislative reform process surrounding s 14(4). The term “serious disability” is not defined within the legislation, but we highlight the fact that s 14(4) was passed with the case of selecting deaf children in mind. We consider some of the literature on the topic of disability and deafness, which, we think, casts some doubt on the view that deafness is a “serious disability.” The main position we advance is that the lack of serious engagement with alternative viewpoints during the legislative process was unsatisfactory. We argue that the contested nature of deafness necessitates a more robust consultation process and a clearer explanation and defence of the normative position that underpins s 14(4).

Cetuximab plus chemotherapy in patients with advanced non-small-cell lung cancer (FLEX) : an open-label randomised phase III trial

Background: Use of cetuximab, a monoclonal antibody targeting the epidermal growth factor receptor (EGFR), has the potential to increase survival in patients with advanced non-small-cell lung cancer. We therefore compared chemotherapy plus cetuximab with chemotherapy alone in patients with advanced EGFR-positive non-small-cell lung cancer. Methods: In a multinational, multicentre, open-label, phase III trial, chemotherapy-naive patients (≥18 years) with advanced EGFR-expressing histologically or cytologically proven stage wet IIIB or stage IV non-small-cell lung cancer were randomly assigned in a 1:1 ratio to chemotherapy plus cetuximab or just chemotherapy. Chemotherapy was cisplatin 80 mg/m 2 intravenous infusion on day 1, and vinorelbine 25 mg/m 2 intravenous infusion on days 1 and 8 of every 3-week cycle) for up to six cycles. Cetuximab-at a starting dose of 400 mg/m 2 intravenous infusion over 2 h on day 1, and from day 8 onwards at 250 mg/m 2 over 1 h per week-was continued after the end of chemotherapy until disease progression or unacceptable toxicity had occurred. The primary endpoint was overall survival. Analysis was by intention to treat. This study is registered with ClinicalTrials.gov, number NCT00148798. Findings: Between October, 2004, and January, 2006, 1125 patients were randomly assigned to chemotherapy plus cetuximab (n=557) or chemotherapy alone (n=568). Patients given chemotherapy plus cetuximab survived longer than those in the chemotherapy-alone group (median 11·3 months vs 10·1 months; hazard ratio for death 0·871 [95% CI 0·762-0·996]; p=0·044). The main cetuximab-related adverse event was acne-like rash (57 [10%] of 548, grade 3). Interpretation: Addition of cetuximab to platinum-based chemotherapy represents a new treatment option for patients with advanced non-small-cell lung cancer. Funding: Merck KGaA. © 2009 Elsevier Ltd. All rights reserved.