Identity and dilemma:the ‘native speaker’ English language teacher in a globalising world

Globalisation (increasing international flows of finance, culture, technological know-how, information, people etc.) has created pressure for a lingua franca. It is widely accepted that English now fulfils this role, with some academics in English language teaching suggesting that the language is no longer owned by ‘native speakers’ and requesting a re-evaluation of the ‘native speaker’ English language teacher in terms of his/her traditional importance in the field. These academics have queried, for example, the continued relevance of ‘native speaker’ pronunciation, methodology and the professional status of the ‘native speaker’ teacher compared with the ‘non-native speaker’ English language teacher. In this study the professional identities of a small group of ‘native speaker’ teachers are explored through data obtained from interviews, field-notes, critical incidents in the researcher-as-teacher’s professional life and by e-mail correspondence. From the collected data it appears that these ‘native speaker’ English language teachers retain a view of themselves as having a superior professional identity, based on their pronunciation, classroom practices, ethnicity, British educational backgrounds and their relational stance to ‘non-native speaker’ teachers. On the other hand, the teachers’ ambivalent relationship with both the new academic understandings of English language teaching and their own professional development appear to contribute to a dilemma in their superior identity constructs. Only one teacher in the group manages to engage with the new understandings and is thus able to conceptualise a professional identity as an English language teacher which seems more in tune with the new global role of English. Overall, in fact, this study reveals a considerable discrepancy between the lived reality of the ‘native speaker’ teachers’ professional lives and the new understandings of academics about English language teaching in a globalising world. The study also highlights a concerning gap between the teachers’ current self-constructs and the implications for the development of practice of new academic theory

The Cone Dysfunction Syndromes

The cone dysfunction syndromes are a heterogeneous group of inherited, predominantly stationary retinal disorders characterised by reduced central vision, and varying degrees of colour vision abnormalities, nystagmus and photophobia. This review details the following conditions: complete and incomplete achromatopsia, blue-cone monochromatism, oligocone trichromacy, bradyopsia, and Bornholm eye disease. We describe the clinical, psychophysical, electrophysiological and imaging findings that are characteristic to each condition, in order to aid their accurate diagnosis, as well as highlight some classically held notions about these diseases that have come to be challenged over recent years. The latest data regarding the genetic aetiology and pathological changes observed in the cone dysfunction syndromes are discussed, and, where relevant, translational avenues of research, including completed and anticipated interventional clinical trials, for some of the diseases described herein will be presented. Finally, we briefly review the current management of these disorders

A Quantitative and Qualitative Exploration of Photoaversion in Achromatopsia

Purpose: Photoaversion (PA) is a disabling and ubiquitous feature of achromatopsia (ACHM). We aimed to help define the characteristics of this important symptom, and present the first published assessment of its impact on patients' lives, as well as quantitative and qualitative PA assessments. Methods: Molecularly confirmed ACHM subjects were assessed for PA using four tasks: structured survey of patient experience, novel quantitative subjective measurement of PA, visual acuities in differing ambient lighting, and objective palpebral aperture-related PA testing. Results: Photoaversion in ACHM was found to be the most significant symptom for a substantial proportion (38%) of patients. A novel subjective PA measurement technique was developed and demonstrated fidelity with more invasive paradigms without exposing often very photosensitive patients to brighter light intensities used elsewhere. An objective PA measurement was also refined for use in trials, indicating that higher light intensities than previously published are likely to be needed. Monocular testing, as required for trials, was also validated for the first time. Conclusions: This study offers new insights into PA in ACHM. It provides the first structured evidence of the great significance of this symptom to patients, suggesting that PA should be considered as an additional outcome measure in therapeutic trials. It also offers new insights into the characteristics of PA in ACHM, and describes both subjective and objective measures of PA that could be employed in clinical trials

Vision in observers with enhanced S-cone syndrome: an excess of S-cones connected mainly to conventional S-cone pathways but also a faster pathway

Purpose: The effect of increased numbers of S-cone photoreceptors in enhanced S-cone syndrome (ESCS) was investigated psychophysically in six ESCS observers to understand more about the relative cone sensitivities and postreceptoral organization. Methods: Measures of temporal sensitivity or delay were made: S- and L-cone temporal acuity (critical flicker fusion or cff), S-cone temporal contrast sensitivity, and S-cone delay. Results: ESCS observers showed uniform enhancements of S-cone cff of between 0.85 and 6.25 Hz, but reductions in L-cone cff. They also showed higher S-cone temporal-contrast-sensitivities at medium and high S-cone adaptation levels with sensitivity functions that peaked near 7.5 Hz but fell off at lower and higher frequencies; in contrast, the mean normal function was flat at low frequencies and fell-off only at high frequencies. The S-cone signal, as in the normal, is subject to large phase delays. Conclusions: We interpret the enhancements in cff as increases in S-cone number in ESCS of between 1.39 and 11.32 times normal density (with a mean of 3.48). The peaked ESCS contrast-sensitivity functions are consistent with S-cone signal interactions that increase sensitivity at intermediate frequencies through constructive interference but decrease it at lower and higher frequencies through destructive interference. Measures of S-cone delays relative to L- and M-cone signals show that the predominant S-cone signals in ESCS are negative and delayed as in normal observers, but reveal another faster, positive S-cone signal. This signal is also likely to be the cause of constructive and destructive interference in the contrast-sensitivity data of ESCS observers

Investigation of Aberrant Splicing Induced by AIPL1 Variations as a Cause of Leber Congenital Amaurosis

PURPOSE: Biallelic mutations in AIPL1 cause Leber congenital amaurosis (LCA), a devastating retinal degeneration characterized by the loss or severe impairment of vision within the first few years of life. AIPL1 is highly polymorphic with more than 50 mutations and many more polymorphisms of uncertain pathogenicity identified. As such, it can be difficult to assign disease association of AIPL1 variations. In this study, we investigate suspected disease-associated AIPL1 variations, including nonsynonymous missense and intronic variants to validate their pathogenicity. METHODS: AIPL1 minigenes harboring missense and intronic variations were constructed by amplification of genomic fragments of the human AIPL1 gene. In vitro splice assays were performed to identify the resultant AIPL1 transcripts. RESULTS: We show that all nine of the suspected disease-associated AIPL1 variations investigated induced aberrant pre-mRNA splicing of the AIPL1 gene, and our study is the first to show that AIPL1 missense mutations alter AIPL1 splicing. We reveal that the presumed rare benign variant c.784G>A [p.(G262S)] alters in vitro AIPL1 splicing, thereby validating the disease-association and clarifying the underlying disease mechanism. We also reveal that in-phase exon skipping occurs normally at a low frequency in the retina, but arises abundantly as a consequence of specific AIPL1 variations, suggesting a tolerance threshold for the expression of these alternative transcripts in the retina normally, which is exceeded in LCA. CONCLUSIONS: Our data confirm the disease-association of the AIPL1 variations investigated and reveal for the first time that aberrant splicing of AIPL1 is an underlying mechanism of disease in LCA

Longitudinal Assessment of Retinal Structure in Achromatopsia Patients With Long-Term Follow-up

PURPOSE: To longitudinally characterize structural retinal changes in achromatopsia (ACHM) over extended follow-up. METHODS: Fifty molecularly confirmed ACHM subjects underwent serial spectral-domain optical coherence tomography (SD-OCT) and fundus autofluorescence (FAF) imaging. Foveal structure on SD-OCT was graded and compared for evidence of progression, and foveal total retinal thickness (FTRT) and outer nuclear layer (ONL) thickness were serially measured. FAF patterns were characterized and compared over time. RESULTS: Mean SD-OCT follow-up was 61.6 months (age range at baseline, 6–52 years). Forty-five of the subjects had serial FAF (mean follow-up: 48.5 months). Only 6 (12%) of the subjects demonstrated qualitative change on serial foveal SD-OCT scans. Among the entire cohort, there was no statistically significant change over time in FTRT (P = 0.2459) or hyporeflective zone (HRZ) diameter (P = 0.3737). There was a small—but statistically significant—increase in ONL thickness (P = 0.0084). Three different FAF patterns were observed: centrally increased FAF (13/45), normal FAF (14/45), and well-demarcated reduced FAF (18/45), with the latter group displaying a small gradual increase in the area of reduced FAF of 0.055 mm2 over 43.4 months (P = 0.0011). CONCLUSIONS: This longitudinal study of retinal structure in ACHM represents the largest cohort and longest follow-up period to date. Our findings support the presiding notion that ACHM is essentially a stationary condition regarding retinal structure, and any change over time is likely to be small, slow, and variable across patients. This may potentially afford a wider window for therapeutic intervention

Visual Psychophysics and Physiological Optics Vision in Observers With Enhanced S-Cone Syndrome: An Excess of S-Cones but Connected Mainly to Conventional S-Cone Pathways

Citation: Ripamonti C, Aboshiha J, Henning GB, et al. Vision in observers with enhanced S-cone syndrome: an excess of S-cones but connected mainly to conventional S-cone pathways. Invest Ophthalmol Vis Sci. 2014;55:963-976. DOI:10.1167/iovs. 13-12897 PURPOSE. The effect of increased numbers of S-cone photoreceptors in enhanced S-cone syndrome (ESCS) was investigated psychophysically in six ESCS observers to understand more about relative cone sensitivities and postreceptoral organization. METHODS. Measures of temporal sensitivity or delay were made: S-and L-cone temporal acuity (critical flicker fusion, or CFF), S-cone temporal contrast sensitivity, and S-cone delay. RESULTS. ESCS observers showed uniform enhancements of S-cone CFF of between 0.85 and 6.25 Hz, but reductions in L-cone CFF. They also showed higher S-cone temporal contrast sensitivities at medium and high S-cone adaptation levels, with sensitivity functions that peaked near 7.5 Hz but fell off at lower and higher frequencies. In contrast, the mean normal function was flat at low frequencies and fell off only at high frequencies. The S-cone signal, as in the normal, is subject to large phase delays. CONCLUSIONS. We interpret the enhancements in CFF as increases in S-cone number in ESCS of between 1.39 and 11.32 times normal density (with a mean of 3.48). The peaked ESCS contrast-sensitivity functions are consistent with S-cone signal interactions that increase sensitivity at intermediate frequencies through constructive interference but decrease it at lower and higher frequencies through destructive interference. Measurements of S-cone delays relative to L-and M-cone signals show that the predominant S-cone signals in ESCS are negative and delayed as in normal observers, but reveal another faster, positive S-cone signal. This signal is also likely to be the cause of constructive and destructive interference in the contrast-sensitivity data of ESCS observers Keywords: enhanced S-cone syndrome, flicker sensitivity, critical flicker fusion, temporal processing, NR2E3, temporal acuity, short-wavelength-sensitive cones. E nhanced S-cone syndrome (ESCS) is a rare inherited degenerative retinal disease named after the associated unusual gain in function-an increase in short-wavelengthsensitive (S) cone sensitivity. 1 The syndrome is also characterized by severely reduced rod sensitivity (night blindness), foveal schisis and macular cysts, varying degree of visual acuity loss, and atypical ERGs that show little or no responses to dim rod (scotopic) stimuli, but have large, slow responses to brighter cone (photopic) stimuli. 1-4 The photopic ERG was originally thought to be of rod origin, 5-7 but spectral measurements have shown that it is dominated by S-cones with reduced contributions from long-and middle-wavelength-sensitive (L and M) cones. 9,10,12 Hood et al. 10 More direct evidence for a relative increase in the number of Scones was provided by histological examination of a disordered ESCS retina of a 77-year-old woman, 12 in which twice the normal number of cones was found, 92% of which were Scones. A more recent study used adaptive optics imaging to attempt to visualize individual cones directly in vivo in three young adults with ESCS. The excess of S-cones can be related to a molecular defect. The gene NR2E3 codes for a photoreceptor-specific nuclear receptor NR2E