Добірка наукової літератури з теми "Christophe Cochet"

Maison, Stéphane, Christophe Micheyl, and Lionel Collet. Medial olivocochlear efferent system in humans studied with amplitude-modulated tones. J. Neurophysiol. 77: 1759–1768, 1997. Evoked otoacoustic emissions (EOAEs) are assumed to be generated by outer hair cells (OHCs). It is now generally accepted that EOAEs represent a means of functional exploration of the active micromechanical properties of OHCs. Efferent fibers of the medial olivocochlear system (MOCS) are connected along the sides and the bases of OHCs. Some studies have shown that a suppression effect on EOAE amplitude is induced by the MOCS neurons during contralateral stimulation, presumably by modification of OHC motility. The contralateral acoustic stimuli used in experiments on the EOAE suppression effect have consisted mainly of sounds without a slow temporal fluctuation in their envelopes (broad-band noise, narrow-band noise, pure tones, or clicks). To elucidate further the parameters of MOCS activation, in the present study we looked at the contralateral suppression effect of amplitude-modulated (AM) tones. The results showed that EOAE amplitude was reduced with AM tones compared with no contralateral acoustic stimulation. The suppression effect mainly depended on three parameters. 1) Contralateral stimulation intensity: EOAE suppression occurred only with intensities ≥40 dB SL. 2) The greater the modulation depth, the greater the suppression effect: statistical analysis showed a significant effect for 75 and 100% modulation depth. 3) The 100- and 140-Hz modulation frequencies gave the greatest suppression effect for 100 and 75% modulation depths. The suppression effect was frequency specific. The greatest decreases were observed when the carrier frequency of the contralateral AM tone was close to the frequency of the EOAE under study, i.e., 1 and 2 kHz. Acoustic cross talk and middle ear effects, which cannot be completely excluded, are discussed. However, the demonstrated frequency specificity of the EOAE suppression effect, together with observed presence of contralateral EOAE suppression in patients without stapedial reflex and the very weak intensities used (i.e., below acoustic reflex threshold), suggested that it was unlikely that the observed effects were due merely to middle ear reflexes. Our results confirm further the contralateral suppression effect on human cochlea mechanisms and show that the suppression effect can be influenced by amplitude modulations of the suppressor, characteristic of sounds in the environment.

Background:Hydroxychloroquine (HCQ) is a well-tolerated drug that contributes to downregulating the immune response against autoantigens and it has been used in several autoimmune diseases. In systemic sclerosis (SSc) it is used to treat inflammatory arthritis without proof of efficacy.Objectives:Our aim was to evaluate the use of HCQ and its impact on Health Assessment Questionnaire disability index (HAQ-DI) and the Cochin Hand Function Status (CHFS). in a large SSc cohort compared to a propensity matched group of SSc patients not using HCQ.Methods:SSc patients from the European Scleroderma Trials and Research (EUSTAR) data base treated with HCQ for at least 6 months were evaluated. Demographic and clinical data, concomitant drugs, duration of HCQ treatment and reasons for its discontinuation, HAQ-DI and CHFS (at least 2 evaluation) were recorded and were the outcome variables of interest. Statistical analysis was performed using propensity score matching for age, gender, disease duration, corticosteroids, immunosuppressives, vasoactive drugs, DMARDs in a 3:1 control:HCQ ratio. Standard descriptive statistics and Student’s t-test and Chi-square test were used to assess the propensity-matched groups.Results:1,636 of 17,805 SSc patients (9.2%) were treated with HCQ for at least 6 months; out of these 3% (50/1636). had at least a baseline and follow-up HAQ-DI evaluation, (and 44/1636 (2.7%) had at least a baseline and follow-up CHFS evaluation. Propensity matching assured that pts were matched for demographic variables such as gender (mean on HCQ vs no HCQ:femals:92.0 vs 85.3), age(49.8 vs 49.97yrs) disease duration(8.3 vs 9.1 yrs), limited disease(55.3 vs 62.6%) as well as background medications (P>0.1-0.9). We did not find any significant changes in HAQ or CHFS (difference in slope) over 365 days of treatment, comparing the HCQ-treated group to the non-HCQ treated patients (p=0.240 for both (Figure 1).Conclusion:Results from the EUSTAR registry showed that HCQ was used by 9.2% of SSc patients. HCQ use did not improve the HAQ or CHFS, comparing HCQ users to non-HCQ users.Disclosure of Interests:Silvia Bellando Randone: None declared, Holly Wilhalme: None declared, Cosimo Bruni: None declared, Elise Siegert: None declared, Paolo Airò: None declared, Rosaria Irace: None declared, Oliver Distler: None declared, Andrea Doria: None declared, Lidia P. Ananieva: None declared, László Czirják: None declared, Christopher Denton: None declared, Yannick Allanore: None declared, Valeria Riccieri: None declared, ALESSANDRA VACCA: None declared, Ivan Foeldvari Consultant of: Gilead, Novartis, Pfizer, Hexal, BMS, Sanofi, MEDAC, Anna-Maria Hoffmann-Vold Speakers bureau: Actelion, Boehringer Ingelheim, Roche, Merck Sharp & Dohme, Lilly and Medscape, Consultant of: Actelion, Boehringer Ingelheim, Roche, Bayer, ARXX, and Medscape, Grant/research support from: Boehringer Ingelheim, Armando Gabrielli: None declared, Marco Matucci-Cerinic: None declared, Daniel Furst: None declared

BackgroundHand involvement is a major cause of disability in systemic sclerosis (SSc) patients. Loss of hand function is the result of a complex and overlapping series of manifestations including Raynaud’s, cutaneous ulcerations as well as skin fibrosis, joint inflammation, and contractures. The natural history of hand involvement in SSc and potential biomarkers to predict its outcome are still poorly defined. Type 1 Interferon (IFN) activation has been extensively correlated with skin fibrosis, joint disease activity, vascular manifestations, and poor prognosis in SSc patients.ObjectivesTo characterize hand disability burden in SSc and explore its relationship with IFN activation in a national, multicenter, longitudinal, observational cohort of patients with SSc.MethodsThe Cochin Hand Function Scale (CHFS) was assessed in consecutively enrolled SSc patients at baseline and after 12 months. CHFS values above the patient acceptable symptom state (PASS)(CHFS>25)1 were considered as clinically meaningful hand impairment (CMHI). Minimal clinically important difference (MCID) in CHFS for improvement (reduction of 13.1%) and worsening (increase >24.6%) were assessed in longitudinal analysis. Serum IFN score was evaluated as previously described2.ResultsA total of 397 SSc patients from 10 centers (female 85.3%, aged 54.9±11.5 years, white Caucasian 88.2%) were available for longitudinal (12m) analysis. The median disease duration was 9 (IQR 3-16) years, 37.1% of patients had a diffuse cutaneous variant, while anticentromere (ACA) and anti-Scl70 antibody positivity was reported in 41.2% and 33.5% of cases, respectively. Hand digital ulcers, forearm-hand-finger skin score ≥6, and tenosynovitis/arthritis were clinically reported in 24.0%, 15.3%, and 17.9% of patients, respectively. 37.3% of patients reported a CHFS > PASS at baseline. CMHI was associated with male gender (p<.001), diffuse cutaneous variant (p<.001), anti Scl70 positivity (p<.001), ACA negativity (p=.002), and digital ulcers (p=.001). Patients with CMHI had greater serum IFN score than patients with CHFS < PASS (p=.002). In multivariate logistic regression analysis, high serum IFN score remained associated with CHFS>PASS when adjusted for male gender, ACA positivity, anti-Scl70 positive, diffuse subset, and current digital ulcers (OR 2.67, p=.005). Over the 12-month follow-up, vasoactive and immunosuppressive treatment were escalated or introduced in 7.2 and 7.8% of patients, respectively. Median CHFS worsened over time (from 18 (IQR 5-37) to 21 (IQR 6-37), p=.002)) with 32.5% of patients having a clinically meaningful worsening and 32.0% improving their hand function. Functional hand worsening was associated with lower baseline CHFS (p=.001) and ACA negativity (p=.002), while improving with female gender (p=.047), limited cutaneous subset (p=.029), higher baseline CHFS (p=.001), and active baseline tenosynovitis (p=.014).ConclusionOne third of the patients within our cohort complain of a significant hand impairment. This is associated with higher IFN activation and worsens at group level in patients despite standard of care treatment.References[1]Daste C et al. Semin Arthritis Rheum. 2019;48(4):694-700. [2] Hinchcliff M et al. Arthritis Rheumatol. 2021; 73 (suppl 10).Disclosure of InterestsEnrico De Lorenzis: None declared, Vishal Kakkar: None declared, rebecca ross: None declared, Stefano Di Donato: None declared, Theresa Barnes: None declared, Benazir Saleem: None declared, Ariane Herrick: None declared, Muhammad Nisar: None declared, Catherine Morley: None declared, Karen Douglas: None declared, Christopher P Denton: None declared, Emma Derrett-Smith: None declared, Philip Helliwell Consultant of: PH received consulting fees (Eli Lilly) and fees for educational services (Abbvie, Amgen, Novartis, Janssen), Grant/research support from: PH received consulting fees (Eli Lilly) and fees for educational services (Abbvie, Amgen, Novartis, Janssen), Francesco Del Galdo Consultant of: FDG has received research support and personal fees, not directly related to the content of this study, fromAbbvie, AstraZeneca, Boehringer-Ingelheim, Capella Biosciences, Chemomab LTD, Janssen, Kymab LTD, Mitsubishi-Tanabe, Grant/research support from: FDG has received research support and personal fees, not directly related to the content of this study, fromAbbvie, AstraZeneca, Boehringer-Ingelheim, Capella Biosciences, Chemomab LTD, Janssen, Kymab LTD, Mitsubishi-Tanabe

Cochlear implants are now the treatment of choice for patients with severe to profound hearing loss. Inclusion criteria for cochlear implantation have expanded, and a whole array of implantable hearing devices have been introduced over the years. To date, more than 250 cochlear implantations have now been performed in the Philippines (Figure 1). In 2006, the first auditory brainstem implantation, and first vibroplasty or middle ear implantation in the country were done at the Philippine General Hospital (PGH). In 2008, the first electroacoustic stimulation or partial deafness cochlear implantation surgery in the country was performed at the Capitol Medical Center by Professor Joachim Müeller of the University of Würzburg and the author. This concept, that cochlear implantation can be performed for patients with residual hearing or only partial deafness, is quite novel. There are patients whose low frequency hearing below 1.5 kHz is still be quite good while high frequency hearing loss above 1.5 kHz is in the severe to profound range (Figure 2). For such patients speech discrimination scores will typically fall below 60% at 65 dB sound pressure level (SPL) in the best aided condition. This technological advancement, often called electroacoustic stimulation (EAS), was developed in 1999 after Christoph Von Ilberg demonstrated preserved residual low frequency hearing in a patient who underwent cochlear implantation such that the patient wore a hearing aid in the implanted ear.1 Currently, EAS devices are available from two manufacturers. Contraindications to the use of EAS are shown in Table 1. Candidates for EAS devices should have stable low frequency hearing. There should be no progressive or autoimmune sensorineural hearing loss. Also there should be no history of meningitis, otosclerosis, or any other malformation that might cause an obstruction. The patient’s air-bone gap should be < 15 dB. Finally, there should not be any external auditory canal problems that can impede placement of the ear mould for the acoustic component. There are two main components of the EAS system (Figure 3). The external component is made up of a microphone that picks up sounds and a processor that separately encodes low and high frequency energy. After processing, low frequency energy is converted into an acoustic signal via the loudspeaker located in the ear hook and delivered into the external auditory canal. This acoustic signal will vibrate the tympanic membrane and ossicles so that cochlear fluids as well as the relatively intact structures of the cochlea in the apical region are stimulated. In contrast, high frequency energy is coded into radio-wave-like signals which are transmitted transcutaneously to the internal receiver. There, electric signals are delivered to the electrode array that has been surgically implanted into the cochlea. Thus the auditory nerve receives information using two different pathways from low and high frequency sounds, and the auditory nerve signals are then transmitted to the brain. Our Experience: Of the more than 100 implantations done under the Philippine National Ear Institute “CHIP” or Cochlear and Hearing Implants Programme only one was a case of EAS implantation. This particular case demonstrates key principles and concepts that every otolaryngologist should consider. Among these are audiological evaluation, temporal bone imaging, surgical technique for hearing preservation and some quality of life issues. Audiological Evaluation A 33 year old man had been seen at the clinic for over 7 years, with serial audiograms (Figure 4-6) illustrating the presence of good and stable low frequency hearing while high frequency hearing loss increased somewhat. The patient had been continually advised to get the best hearing aids available. However, a series of high-end hearing aids did not solve his problem of poor hearing in noisy places nor his difficulty understanding words when watching television and movies. Figure 7A shows the speech perception scores of this patient obtained with a Word Intelligibility by Picture Identification (WIPI) test, a “closed-set test” using isolated words while Figure 7B represents speech scores when “open-set” Bamford-Kowal-Bench (BKB) Sentence Lists were presented to the listener in both quiet and noise prior to the implantation. Temporal bone imaging A combination of high resolution computerized tomography (HRCT) of the temporal bone with both coronal and axial cochlear views, and T2-weighted normal anatomic Fast Spin Echo (T2 FSE) or 3D Constructive Interference in Steady State (3D CISS) MRI sequences of the inner ear should be done. Results from both studies should ascertain whether the cochlear duct is patent, ruling out any cochlear fibrosis or obstructive pathology. This patient’s HRCT and 3-D CISS MRI studies showed no such cochlear obliteration that would have posed intraoperative difficulties and constituted contraindications to EAS surgery (Figure 8). Surgical Technique for Hearing Preservation A variety of techniques have evolved over the years into what is now commonly called minimally invasive cochlear implantation. Using minimally invasive techniques, residual hearing can indeed be preserved in over 80%-90% of patients 3,4 Initially, a “Soft Cochleostomy” technique was introduced. This entailed careful low-speed drilling of the promontory with a Skeeter® drill (Medtronic Xomed, Jacksonville FL, USA) followed by the use of a mini-lancet to make an opening in the membranous labyrinth. This method avoids direct suctioning and prevents ingress of blood and bone dust into the intracochlear compartment. Also, for this method, the endosteum is left intact after drilling a cochleostomy antero-inferior to the round window. This allows proper placement of the electrode into the scala tympani with less chance of injury to the basilar membrane. Later, a round window approach was introduced, and it also proved to be a reliable way to preserve residual hearing during cochlear implantation. For this method, a more direct round window approach is performed after careful drilling of the round window niche. A limited incision is made just large enough to allow the electrode to be inserted. For both methods, after the endosteal or round window membrane incision is made with a micro lancet, a very flexible electrode of 20 mm length is slowly inserted. During the insertion process, the cochleostomy or round window is kept under direct vision so that insertion forces are minimized. Topical antibiotics and steroids are applied at this time to reduce any inflammatory or apoptotic reactions related to the trauma of opening the cochlea and introducing an electrode. Finally, a soft tissue plug is placed tightly around the electrode entry point into the membranous labyrinth to prevent perilymph leakage. New electrode designs that are thinner and more flexible are important contributors to the preservation of hearing. Postoperative Outcomes and Quality of Life After about 4-6 weeks from the time of surgery the EAS implant is switched on. Based on our experience and that of others,3 speech perception performance improves with prolonged experience with the implant. Roughly 1 ½ years post-surgery this patient has achieved dramatic improvement in hearing both in quiet and in noise using the EAS compared to using only the hearing aid component or the CI component alone. Figure 9 shows this dramatic improvement in free-field pure tone thresholds. Figure 10 demonstrates the speech perception following EAS implantation compared to pre-EAS implantation. Audiologic evaluation done at the PGH Ear Unit using 20 phonetically balanced Filipino words familiar to the patient in quiet and with 55 dB masking noise in the side of the implanted ear clearly showed an advantage with the EAS configuration compared to either hearing aid or CI component alone. Even with noise, this patient actually performed better presumably because he may have concentrated more with the introduction of masking noise. Another factor of course is that the words have now become familiar to the patient with the previous testing done in quiet. Notably, he reported great subjective improvement after only 10 months post-surgery.5 Interestingly the patient’s only complaint during his last follow-up was that he had not been offered bilateral EAS implantation. It is always important for the otolaryngologist to consider the quality of hearing and quality of life of patients with hearing loss. Intervention should not end with a referral note to a hearing aid center or dispenser. It is important to request proof of improvement not only of hearing thresholds but of speech perception outcomes in quiet and in noise. That is, one should document actual performance with the device in place, regardless of the type of device (hearing aid, an EAS device, or a Cochlear implant). Minimal disturbance of the remaining intact structures of the cochlea of patients with low frequency residual hearing can be achieved by employing a meticulous surgical technique, by using the advanced and flexible electrodes developed by some manufacturers, and instilling intraoperative antibiotics and steroids. Thus when one is faced with a ski-slope type audiogram it is likely the patient with this audiogram will not benefit from hearing aids. Such patients should be offered the option of EAS implantation which combines good acoustic stimulation with electric stimulation using a shorter (than conventional cochlear implantation) but very flexible electrode system. Counseling must also be done with a special emphasis on the risk of losing residual hearing, and noting that post-operative rehabilitation may take a long period of time. This patient now has a better quality of life than was obtainable from the most expensive and advanced hearing aids in the market, and has demonstrated a new implantable solution to partial deafness. Truly, EAS technology has opened a new era in prosthetic rehabilitation for hearing impaired adults and children.5 Acknowledgement Dr. Maria Rina Reyes-Quintos is gratefully acknowledged for performing all the excellent audiological testing following the surgery while Susan Javier and Angie Tongko of Manila Hearing Aid Center performed all the audiological testing prior to the surgery. Ms. Celina Ann Tobias, Professional Education Manager of Med-El is also credited with thanks for preparing the figures, reviewing the manuscript and interviewing the patient regarding his hearing performance following the surgery.

BackgroundToday, there are still no DMARDs licensed for primary Sjögren Syndrome (pSS) patients. Among the explanations, are the limitations of current outcome measures used as primary endpoints: e.g; high placebo response rate, evaluation of either symptoms or systemic activity, and important features not being assessed. The NECESSITY consortium (https://www.necessity-h2020.eu/), including pSS experts from academia, pharmaceutical industry and patient groups formed to develop a new composite responder index, the Sjögren’s Tool for Assessing Response (STAR) that solve the issues of current outcome measures in pSS and is intended for use in clinical trials as an efficacy endpoint.ObjectivesTo develop a composite responder index in primary Sjögren’s syndrome (pSS): the STAR.MethodsTo develop the STAR, the NECESSITY consortium used data-driven methods, based on 9 randomized controlled trials (RCTs), and consensus techniques, involving 78 experts and 20 patients. Based on reanalysis of rituximab trials (TRACTISS and TEARS) and literature review, the Delphi panel identified a core set of domains to include in the STAR, with their respective outcome measures. STAR options combining these domains were designed and proposed to the panel to select and improve them. For each STAR option, sensitivity to change was estimated by the C-index (derived from Effect size) in all 9 RCTs. Delphi rounds were run for selecting STAR among these options. The Delphi panel also voted to classify trials as positive, negative or “in between” in regards to primary but also key secondary endpoints. For the options remaining before the final vote, meta-analyses of the RCTs were performed separately for positive and “in between” trials together, and for negative trials.ResultsThe Delphi panel identified 5 core domains (systemic activity, patient symptoms, lachrymal gland function, salivary gland function and biological parameters), and 227 STAR options, combining these domains, were selected to be tested for sensitivity to change. After two Delphi rounds, meta-analyses of the 20 remaining options were performed. The candidate STAR was selected by a final vote based on metrological properties and clinical relevance. In positive/in between trials, candidate STAR detected a difference between arms (OR 3.29, 95%-CI [2.07;5.22], whereas it did not in negative trials (OR 1.53, 95%-CI [0.81;2.91]).ConclusionThe candidate STAR is a composite responder index, including in a single tool all main disease features, and is designed for use as a primary endpoint in pSS RCTs. Its rigorous and consensual development process ensures its face and content validity. The candidate STAR showed good sensitivity and specificity to change. The candidate STAR will be prospectively validated in a dedicated three arms RCT of the NECESSITY consortium that will evaluate combination of synthetic DMARDs (hydroxychloroquine + lefunomide or hydroxychloroquine + mycophenolate vs placebo). We encourage the use of STAR in any ongoing and future trials.Table 1.Candidate STARDomainPointDefinition of responseSystemic activity3Decrease of clinESSDAI ≥ 3Patient reported outcome3Decrease of ESSPRI ≥ 1 point or ≥ 15%Lachrymal gland function1Schirmer:If abnormal score at baseline: increase ≥ 5 mm from baselineIf normal score at baseline: no change to abnormalOrOcular Staining Score:If abnormal score at baseline: decrease ≥ 2 points from baselineIf normal score at baseline: no change to abnormalSalivary gland function1Unstimulated Whole Salivary Flow:If score > 0 at baseline: increase ≥ 25% from baselineIf score is 0 at baseline: any increase from baselineorUltrasound:Decrease ≥ 25% in total Hocevar score from baselineBiological1Serum IgG levels: decrease ≥ 10%orRheumatoid Factor levels: decrease ≥ 25%Candidate STAR responder≥ 5 pointsESSDAI: EULAR Sjögren syndrome disease activity index; ESSPRI: EULAR Sjögren syndrome patient reported index; IgG: Immunoglobulin G;AcknowledgementsNECESSITY WP5 STAR development participants: Suzanne Arends (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Francesca Barone (Centre for Translational Inflammation Research, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK), Albin Björk (Division of Rheumatology, Department of Medicine, Karolinska Institutet, Stockholm, Sweden), Coralie Bouillot (Association Française du Gougerot Sjögren et des Syndromes Secs, France), Guillermo Carvajal Alegria (University of Brest, Inserm, CHU de Brest, LBAI, UMR1227, Brest, France; Service de Rhumatologie, Centre de Référence Maladies Autoimmunes Rares CERAINO, CHU Cavale Blanche, Brest, France), Wen-Hung Chen (GlaxoSmithKline, Research Triangle Park, North Carolina, USA), Kenneth Clark (GlaxoSmithKline Medicines Research Centre, Gunnels Wood Road, Stevenage, Hertfordshire SG1 2NY, United Kingdom), Konstantina Delli (Department of Oral and Maxillofacial Surgery, University Medical Center Groningen (UMCG), University of Groningen, The Netherlands), Salvatore de Vita (Rheumatology Clinic, University Hospital of Udine, Italy), Liseth de Wolff (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Jennifer Evans (Novartis Pharmaceuticals corporation USA), Stéphanie Galtier (Institut de Recherches Internationales Servier (IRIS), Suresnes Cedex, France), Saviana Gandolfo (Rheumatology Clinic, Department of Medical area, University of Udine, ASUFC, 33100 Udine, Italy), Mickael Guedj (Institut de Recherches Internationales Servier (IRIS), Suresnes Cedex, France), Dewi Guellec (CHU de Brest, Service de Rhumatologie, Inserm, CIC 1412, Brest, France), Safae Hamkour (Center of Translational Immunology, Department of Immunology, University Medical Center Utrecht, Utrecht 3584 GA, Netherlands), Dominik Hartl (Novartis Institutes for BioMedical Research, Basel, Switzerland), Malin Jonsson (Section for Oral and Maxillofacial Radiology, Department of Clinical Dentistry, Faculty of Medicine and Dentistry, University of Bergen, Norway), Roland Jonsson (Broegelmann Research Laboratory, Department of Clinical Science, University of Bergen, Department of Rheumatology, Haukeland University Hospital, Bergen, Norway), Frans Kroese (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Aike Albert Kruize (University Medical Center Utrecht, Department Rheumatology and Clinical Immunology, Utrecht, Netherlands), Laurence Laigle (Institut de Recherches Internationales Servier (IRIS), Suresnes Cedex, France), Véronique Le Guern (AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne, Paris, France), Wen-Lin Luo (Department of Biometrics and Statistical Science, Novartis Pharmaceuticals, East Hanover, New Jersey), Esther Mossel (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Wan-Fai Ng (Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne NE2 4HH, UK), Gaëtane Nocturne (Department of Rheumatology, Université Paris-Saclay, INSERM U1184: Centre for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Le Kremlin Bicêtre, Paris, France), Marleen Nys (Global Biometric Sciences, Bristol Myers Squibb, Braine L’Alleud, Belgium), Roald Omdal (Clinical Immunology Unit, Department of Internal Medicine, Stavanger University Hospital, PO Box 8100, 4068, Stavanger, Norway), Jacques-Olivier Pers (LBAI, UMR1227, University of Brest, Inserm, Brest, France and CHU de Brest, Brest, France), Maggy Pincemin (Association Française du Gougerot Sjögren et des Syndromes Secs, France), Manel Ramos-Casals (Department of Autoimmune Diseases, Hospital Clinic de Barcelona Institut Clinic de Medicinai Dermatologia, Barcelona, Catalunya, Spain), Philippe Ravaud (Centre d’Epidémiologie Clinique, Hôpital Hôtel-Dieu, Assistance Publique-Hôpitaux de Paris, Paris, France), Neelanjana Ray (Global Drug Development - Immunology, Bristol Myers Squibb Company, Princeton, New Jersey, USA), Alain Saraux (HU de Brest, Service de Rhumatologie, Univ Brest, Inserm, UMR1227, Lymphocytes B et Autoimmunité, Univ Brest, Inserm, LabEx IGO, Brest, France), Athanasios Tzioufas (Rheumatology Clinic, Department of Medical area, University of Udine, ASUFC, 33100 Udine, Italy), Gwenny Verstappen (University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen 9700 RB, Netherlands), Arjan Vissink, Marie Wahren-Herlenius (Division of Rheumatology, Department of Medicine, Karolinska Institutet, Stockholm, Sweden). We thank the following experts: Esen Karamursel Akpek, Alan Baer, Chiara Baldini, Elena Bartoloni, Marí-Alfonso Begona, Johan Brun, Vatinee Bunya, Laurent Chiche, Troy Daniels, Paul Emery, Robert Fox, Roberto Giacomelli, John Gonzales, John Greenspan, Robert Moots, Susumu Nishiyama, Elizabeth Price, Christophe Richez, Caroline Shiboski, Roser Solans Laque, Muthiah Srinivasan, Peter Olsson, Tsutomu Takeuchi, Frederick Vivino, Paraskevi Voulgari, Daniel Wallace, Ava Wu, Wen Zhang. We thank the anonymous patients from the NECESSITY Patient Advisory Group and the Sjögren Foundation for their valuable contribution to the Delphi process. We thank EW StClair and AN Baer who generated the baminercept data and made them publicly available.Disclosure of InterestsRaphaèle Seror Consultant of: GlaxoSmithKline, Boehringer, Janssen and Novartis, Grant/research support from: GlaxoSmithKline and Amgen, Gabriel Baron: None declared, Marine Camus: None declared, Divi Cornec Consultant of: GlaxoSmithKline, Bristol Myers Squibb, Janssen, Amgen, Pfizer and Roche, Elodie Perrodeau: None declared, Simon J. Bowman Consultant of: Abbvie, Astra Zeneca, Galapagos and Novartis Pharmaceuticals, Michele Bombardieri Consultant of: UCB, Amgen/Medimmune, Janssen, and GlaxoSmithKline, Grant/research support from: Amgen/Medimmune, Janssen, and GlaxoSmithKline, Hendrika Bootsma: None declared, Jacques-Eric Gottenberg Consultant of: AbbVie, Bristol Myers Squibb, Eli Lilly, Galapagos, Gilead, Pfizer, Roche, Sanofi, Novartis, MSD, CSL-Behring and Genzyme, Grant/research support from: Bristol Myers Squibb, Benjamin Fisher Speakers bureau: Bristol Myers Squibb and Novartis, Consultant of: Novartis, Bristol Myers Squibb, Janssen and Servier, Grant/research support from: Servier, Galapagos and Janssen, Wolfgang Hueber Shareholder of: Novartis Pharma, Employee of: Novartis Pharma, Joel van Roon: None declared, Valerie Devauchelle-Pensec: None declared, Peter Gergely Shareholder of: Novartis Pharma, Employee of: Novartis Pharma, Xavier Mariette Consultant of: Bristol Myers Squibb, Galapagos, GlaxoSmithKline, Janssen, Novartis, Pfizer and UCB, Grant/research support from: Ose Pharmaceuticals, Raphaël Porcher: None declared

Cyborgs already walk among us. (“Cures to Come” 76) This essay was begun as a reaction to a Hallmark Hall of Fame television movie called Sweet Nothing in My Ear (2008), which follows the lives of two parents, Dan, who is hearing (played by Jeff Daniels), and Laura, who is deaf (Marlee Matlin), as they struggle to make a decision about whether or not to give their 11-year-old son, Adam (late-deafened), a cochlear implant. Dan and Laura represent different perspectives, hearing and deaf perspectives. The film dramatizes the parents’ conflict and negotiation, exposing audiences to both sides of the cochlear implant debate, albeit in a fairly simplistic way. Nevertheless, it represents the lives of deaf people and gives voice to debates about cochlear implants with more accuracy and detail than most film and television dramas. One of the central scenes in the film is what I call the “activation scene”, quite common to cochlear implant narratives. In the scene, the protagonists witness a child having his implant activated or turned on. The depiction is reminiscent of the WATER scene in the film about Helen Keller, The Miracle Worker, employing a sentimental visual rhetoric. First, the two parents are shown seated near the child, clasping their hands as if in prayer. The audiologist, wielder of technology and therefore clearly the authority figure in the scene, types away furiously on her laptop. At the moment of being “turned on,” the child suddenly “hears” his father calling “David! David!” He gazes angelically toward heaven as piano music plays plaintively in the background. The parents all but fall to their knees and the protagonist of the film, Dan, watching through a window, weeps. It is a scene of cure, of healing, of “miracle,” a hyper-sentimentalised portrait of what is in reality often a rather anti-climactic event. It was certainly anti-climactic in my son, Michael’s case. I was taken aback by how this scene was presented and dismayed overall at some of the inaccuracies, small though they were, in the portrayal of cochlear implants in this film. It was, after all, according to the Nielsen ratings, seen by 8 million people. I began to wonder what kinds of misconceptions my son was going to face when he met people whose only exposure to implants was through media representations. Spurred by this question, I started to research other recent portrayals of people with implants on U.S. television in the past ten years, to see how cochlear implant (hereafter referred to as CI) identity has been portrayed by American media. For most of American history, deaf people have been portrayed in print and visual media as exotic “others,” and have long been the subject of an almost morbid cultural fascination. Christopher Krentz suggests that, particularly in the nineteenth century, scenes pairing sentimentality and deafness repressed an innate, Kristevan “abject” revulsion towards deaf people. Those who are deaf highlight and define, through their ‘lack’, the “unmarked” body. The fact of their deafness, understood as lack, conjures up an ideal that it does not attain, the ideal of the so-called “normal” or “whole” body. In recent years, however, the figure of the “deaf as Other” in the media, has shifted from what might be termed the “traditionally” deaf character, to what Brenda Jo Brueggeman (in her recent book Deaf Subjects: Between Identities and Places), calls “the new deaf cyborg” or the deaf person with a cochlear implant (4). N. Katharine Hailes states that cyborgs are now “the stage on which are performed contestations about the body boundaries that have often marked class, ethnic, and cultural differences” (85). In this essay, I claim that the character with a CI, as portrayed in the media, is now not only a strange, “marked” “Other,” but is also a screen upon which viewers project anxieties about technology, demonstrating both fascination fear. In her book, Brueggeman issues a call to action, saying that Deaf Studies must now begin to examine what she calls “implanting rhetorics,” or “the rhetorical relationships between our technologies and our identity” and therefore needs to attend to the construction of “the new deaf cyborg” (18). This short study will serve, I hope, as both a response to that injunction and as a jumping-off point for more in-depth studies of the construction of the CI identity and the implications of these constructions. First, we should consider what a cochlear implant is and how it functions. The National Association of the Deaf in the United States defines the cochlear implant as a device used to help the user perceive sound, i.e., the sensation of sound that is transmitted past the damaged cochlea to the brain. In this strictly sensorineural manner, the implant works: the sensation of sound is delivered to the brain. The stated goal of the implant is for it to function as a tool to enable deaf children to develop language based on spoken communication. (“NAD Position”) The external portion of the implant consists of the following parts: a microphone, which picks up sound from the environment, which is contained in the behind-the-ear device that resembles the standard BTE hearing aid; in this “hearing aid” there is also a speech processor, which selects and arranges sounds picked up by the microphone. The processor transmits signals to the transmitter/receiver, which then converts them into electric impulses. Part of the transmitter sits on the skin and attaches to the inner portion of the transmitter by means of a magnet. The inner portion of the receiver/stimulator sends the impulses down into the electrode array that lies inside the cochlea, which in turn stimulates the auditory nerve, giving the brain the impression of sound (“Cochlear Implants”). According to manufacturer’s statistics, there are now approximately 188,000 people worldwide who have obtained cochlear implants, though the number of these that are in use is not known (Nussbaum). That is what a cochlear implant is. Before we can look at how people with implants are portrayed in the media, before we examine constructions of identity, perhaps we should first ask what constitutes a “real” CI identity? This is, of course, laughable; pinning down a homogeneous CI identity is no more likely than finding a blanket definition of “deaf identity.” For example, at this point in time, there isn’t even a word or term in American culture for someone with an implant. I struggle with how to phrase it in this essay - “implantee?” “recipient?” - there are no neat labels. In the USA you can call a person deaf, Deaf (the “D” representing a specific cultural and political identity), hearing impaired, hard of hearing, and each gradation implies, for better or worse, some kind of subject position. There are no such terms for a person who gets an implant. Are people with implants, as suggested above, just deaf? Deaf? Are they hard of hearing? There is even debate in the ASL community as to what sign should be used to indicate “someone who has a cochlear implant.” If a “CI identity” cannot be located, then perhaps the rhetoric that is used to describe it may be. Paddy Ladd, in Understanding Deaf Culture, does a brilliant job of exploring the various discourses that have surrounded deaf culture throughout history. Stuart Blume borrows heavily from Ladd in his “The Rhetoric and Counter-Rhetoric of a 'Bionic' Technology”, where he points out that an “essential and deliberate feature” of the history of the CI from the 60s onward, was that it was constructed in an overwhelmingly positive light by the mass media, using what Ladd calls the “medical” rhetorical model. That is, that the CI is a kind of medical miracle that promised to cure deafness. Within this model one may find also the sentimental, “missionary” rhetoric that Krentz discusses, what Ladd claims is a revival of the evangelism of the nineteenth-century Oralist movement in America. Indeed, newspaper articles in the 1980s and 90s hailed the implant as a “breakthrough”, a “miracle”; even a quick survey of headlines shows evidence of this: “Upton Boy Can Hear at Last!”, “Girl with a New Song in Her Heart”, “Children Head Queue for Bionic Ears” (Lane). As recently as January 2010, an issue of National Geographic featured on its cover the headline Merging Man and Machine: The Bionic Age. Sure enough, the second photograph in the story is of a child’s bilateral cochlear implant, with the caption “within months of the surgery (the child) spoke the words his hearing parents longed for: Mama and Dada.” “You’re looking at a real bionic kid,” says Johns Hopkins University surgeon John Niparko, proudly (37). To counter this medical/corporate rhetoric of cure, Ladd and Blume claim, the deaf community devised a counter-rhetoric, a discourse in which the CI is not cast in the language of miracle and life, but instead in terms of death, mutilation, and cultural oppression. Here, the implant is depicted as the last in a long line of sadistic experiments using the deaf as guinea pigs. Often the CI is framed in the language of Nazism and genocide as seen in the title of an article in the British Deaf News: “Cochlear Implants: Oralism’s Final Solution.” So, which of these two “implanting rhetorics” is most visible in the current construction of the CI in American television? Is the CI identity presented by rendering people with CIs impossibly positive, happy characters? Is it delineated using the metaphors of the sentimental, of cure, of miracle? Or is the CI identity constructed using the counter-rhetorical references to death, oppression and cultural genocide? One might hypothesize that television, like other media, cultivating as it does the values of the hearing hegemony, would err on the side of promulgating the medicalised, positivist rhetoric of the “cure” for deafness. In an effort to find out, I conducted a general survey of American television shows from 2000 to now that featured characters with CIs. I did not include news shows or documentaries in my survey. Interestingly, some of the earliest television portrayals of CIs appeared in that bastion of American sentimentality, the daytime soap opera. In 2006, on the show “The Young and the Restless”, a “troubled college student who contracted meningitis” received an implant, and in 2007 “All My Children” aired a story arc about a “toddler who becomes deaf after a car crash.” It is interesting to note that both characters were portrayed as “late-deafened”, or suddenly inflicted with the loss of a sense they previously possessed, thus avoiding any whiff of controversy about early implantation. But one expects a hyper-sentimentalised portrayal of just about everything in daytime dramas like this. What is interesting is that when people with CIs have appeared on several “reality” programs, which purport to offer “real,” unadulterated glimpses into people’s lives, the rhetoric is no less sentimentalized than the soaps (perhaps because these shows are no less fabricated). A good example of this is the widely watched and, I think, ironically named show “True Life” which appears on MTV. This is a series that claims to tell the “remarkable real-life stories of young people and the unusual subcultures they inhabit.” In episode 42, “ True Life: I’m Deaf”, part of the show follows a young man, Chris, born deaf and proud of it (his words), who decides to get a cochlear implant because he wants to be involved in the hearing world. Through an interpreter Chris explains that he wants an implant so he can communicate with his friends, talk with girls, and ultimately fulfill his dreams of having a job and getting married (one has to ask: are these things he can’t do without an implant?). The show’s promo asks “how do you go from living a life in total silence to fully understanding the spoken language?” This statement alone contains two elements common to the “miracle” rhetoric, first that the “tragic” deaf victim will emerge from a completely lonely, silent place (not true; most deaf people have some residual hearing, and if you watch the show you see Chris signing, “speaking” voluminously) to seamlessly, miraculously, “fully” joining and understanding the hearing world. Chris, it seems, will only come into full being when he is able to join the hearing world. In this case, the CI will cure what ails him. According to “True Life.” Aside from “soap opera” drama and so-called reality programming, by far the largest dissemination of media constructions of the CI in the past ten years occurred on top-slot prime-time television shows, which consist primarily of the immensely popular genre of the medical and police procedural drama. Most of these shows have at one time or another had a “deaf” episode, in which there is a deaf character or characters involved, but between 2005 and 2008, it is interesting to note that most, if not all of the most popular of these have aired episodes devoted to the CI controversy, or have featured deaf characters with CIs. The shows include: CSI (both Miami and New York), Cold Case, Law and Order (both SVU and Criminal Intent), Scrubs, Gideon’s Crossing, and Bones. Below is a snippet of dialogue from Bones: Zach: {Holding a necklace} He was wearing this.Angela: Catholic boy.Brennan: One by two forceps.Angela {as Brennan pulls a small disc out from behind the victim’s ear} What is that?Brennan: Cochlear implant. Looks like the birds were trying to get it.Angela: That would set a boy apart from the others, being deaf.(Bones, “A Boy in the Tree”, 1.3, 2005) In this scene, the forensics experts are able to describe significant points of this victim’s identity using the only two solid artifacts left in the remains, a crucifix and a cochlear implant. I cite this scene because it serves, I believe, as a neat metaphor for how these shows, and indeed television media in general, are, like the investigators, constantly engaged in the business of cobbling together identity: in this particular case, a cochlear implant identity. It also shows how an audience can cultivate or interpret these kinds of identity constructions, here, the implant as an object serves as a tangible sign of deafness, and from this sign, or clue, the “audience” (represented by the spectator, Angela) immediately infers that the victim was lonely and isolated, “set apart from the others.” Such wrongheaded inferences, frivolous as they may seem coming from the realm of popular culture, have, I believe, a profound influence on the perceptions of larger society. The use of the CI in Bones is quite interesting, because although at the beginning of the show the implant is a key piece of evidence, that which marks and identifies the dead/deaf body, the character’s CI identity proves almost completely irrelevant to the unfolding of the murder-mystery. The only times the CI character’s deafness is emphasized are when an effort is made to prove that the he committed suicide (i.e., if you’re deaf you are therefore “isolated,” and therefore you must be miserable enough to kill yourself). Zak, one of the forensics officers says, “I didn’t talk to anyone in high school and I didn’t kill myself” and another officer comments that the boy was “alienated by culture, by language, and by his handicap” (odd statements, since most deaf children with or without implants have remarkably good language ability). Also, in another strange moment, the victim’s ambassador/mother shows a video clip of the child’s CI activation and says “a person who lived through this miracle would never take his own life” (emphasis mine). A girlfriend, implicated in the murder (the boy is killed because he threatened to “talk”, revealing a blackmail scheme), says “people didn’t notice him because of the way he talked but I liked him…” So at least in this show, both types of “implanting rhetoric” are employed; a person with a CI, though the recipient of a “miracle,” is also perceived as “isolated” and “alienated” and unfortunately, ends up dead. This kind of rather negative portrayal of a person with a CI also appears in the CSI: New York episode ”Silent Night” which aired in 2006. One of two plot lines features Marlee Matlin as the mother of a deaf family. At the beginning of the episode, after feeling some strange vibrations, Matlin’s character, Gina, checks on her little granddaughter, Elizabeth, who is crying hysterically in her crib. She finds her daughter, Alison, dead on the floor. In the course of the show, it is found that a former boyfriend, Cole, who may have been the father of the infant, struggled with and shot Alison as he was trying to kidnap the baby. Apparently Cole “got his hearing back” with a cochlear implant, no longer considered himself Deaf, and wanted the child so that she wouldn’t be raised “Deaf.” At the end of the show, Cole tries to abduct both grandmother and baby at gunpoint. As he has lost his external transmitter, he is unable to understand what the police are trying to tell him and threatens to kill his hostages. He is arrested in the end. In this case, the CI recipient is depicted as a violent, out of control figure, calmed (in this case) only by Matlin’s presence and her ability to communicate with him in ASL. The implication is that in getting the CI, Cole is “killing off” his Deaf identity, and as a result, is mentally unstable. Talking to Matlin, whose character is a stand-in for Deaf culture, is the only way to bring him back to his senses. The October 2007 episode of CSI: Miami entitled “Inside-Out” is another example of the counter-rhetoric at work in the form of another implant corpse. A police officer, trying to prevent the escape of a criminal en route to prison, thinks he has accidentally shot an innocent bystander, a deaf woman. An exchange between the coroner and a CSI goes as follows: (Alexx Woods): “This is as innocent as a victim gets.”(Calleigh Duquesne): “How so?”AW: Check this out.”CD: “I don’t understand. Her head is magnetized? Steel plate?”AW: “It’s a cochlear implant. Helps deaf people to receive and process speech and sounds.”(CSI dramatization) AW VO: “It’s surgically implanted into the inner ear. Consists of a receiver that decodes and transmits to an electrode array sending a signal to the brain.”CD: “Wouldn’t there be an external component?”AW: “Oh, she must have lost it before she was shot.”CD: “Well, that explains why she didn’t get out of there. She had no idea what was going on.” (TWIZ) Based on the evidence, the “sign” of the implant, the investigators are able to identify the victim as deaf, and they infer therefore that she is innocent. It is only at the end of the program that we learn that the deaf “innocent” was really the girlfriend of the criminal, and was on the scene aiding in his escape. So she is at first “as innocent” as they come, and then at the end, she is the most insidious of the criminals in the episode. The writers at least provide a nice twist on the more common deaf-innocent stereotype. Cold Case showcased a CI in the 2008 episode “Andy in C Minor,” in which the case of a 17-year-old deaf boy is reopened. The boy, Andy, had disappeared from his high school. In the investigation it is revealed that his hearing girlfriend, Emma, convinced him to get an implant, because it would help him play the piano, which he wanted to do in order to bond with her. His parents, deaf, were against the idea, and had him promise to break up with Emma and never bring up the CI again. His body is found on the campus, with a cochlear device next to his remains. Apparently Emma had convinced him to get the implant and, in the end, Andy’s father had reluctantly consented to the surgery. It is finally revealed that his Deaf best friend, Carlos, killed him with a blow to the back of the head while he was playing the piano, because he was “afraid to be alone.” This show uses the counter-rhetoric of Deaf genocide in an interesting way. In this case it is not just the CI device alone that renders the CI character symbolically “dead” to his Deaf identity, but it leads directly to his being literally executed by, or in a sense, excommunicated from, Deaf Culture, as it is represented by the character of Carlos. The “House Divided” episode of House (2009) provides the most problematic (or I should say absurd) representation of the CI process and of a CI identity. In the show, a fourteen-year-old deaf wrestler comes into the hospital after experiencing terrible head pain and hearing “imaginary explosions.” Doctors Foreman and Thirteen dutifully serve as representatives of both sides of the “implant debate”: when discussing why House hasn’t mocked the patient for not having a CI, Thirteen says “The patient doesn’t have a CI because he’s comfortable with who he is. That’s admirable.” Foreman says, “He’s deaf. It’s not an identity, it’s a disability.” 13: “It’s also a culture.” F: “Anything I can simulate with $3 earplugs isn’t a culture.” Later, House, talking to himself, thinks “he’s going to go through life deaf. He has no idea what he’s missing.” So, as usual, without permission, he orders Chase to implant a CI in the patient while he is under anesthesia for another procedure (a brain biopsy). After the surgery the team asks House why he did it and he responds, “Why would I give someone their hearing? Ask God the same question you’d get the same answer.” The shows writers endow House’s character, as they usually do, with the stereotypical “God complex” of the medical establishment, but in doing also they play beautifully into the Ladd and Blume’s rhetoric of medical miracle and cure. Immediately after the implant (which the hospital just happened to have on hand) the incision has, miraculously, healed overnight. Chase (who just happens to be a skilled CI surgeon and audiologist) activates the external processor (normally a months-long process). The sound is overwhelming, the boy hears everything. The mother is upset. “Once my son is stable,” the mom says, “I want that THING out of his head.” The patient also demands that the “thing” be removed. Right after this scene, House puts a Bluetooth in his ear so he can talk to himself without people thinking he’s crazy (an interesting reference to how we all are becoming cyborgs, more and more “implanted” with technology). Later, mother and son have the usual touching sentimental scene, where she speaks his name, he hears her voice for the first time and says, “Is that my name? S-E-T-H?” Mom cries. Seth’s deaf girlfriend later tells him she wishes she could get a CI, “It’s a great thing. It will open up a whole new world for you,” an idea he rejects. He hears his girlfriend vocalize, and asks Thirteen if he “sounds like that.” This for some reason clinches his decision about not wanting his CI and, rather than simply take off the external magnet, he rips the entire device right out of his head, which sends him into shock and system failure. Ultimately the team solves the mystery of the boy’s initial ailment and diagnoses him with sarcoidosis. In a final scene, the mother tells her son that she is having them replace the implant. She says it’s “my call.” This show, with its confusing use of both the sentimental and the counter-rhetoric, as well as its outrageous inaccuracies, is the most egregious example of how the CI is currently being constructed on television, but it, along with my other examples, clearly shows the Ladd/Blume rhetoric and counter rhetoric at work. The CI character is on one hand portrayed as an innocent, infantilized, tragic, or passive figure that is the recipient of a medical miracle kindly urged upon them (or forced upon them, as in the case of House). On the other hand, the CI character is depicted in the language of the counter-rhetoric: as deeply flawed, crazed, disturbed or damaged somehow by the incursions onto their Deaf identity, or, in the worst case scenario, they are dead, exterminated. Granted, it is the very premise of the forensic/crime drama to have a victim, and a dead victim, and it is the nature of the police drama to have a “bad,” criminal character; there is nothing wrong with having both good and bad CI characters, but my question is, in the end, why is it an either-or proposition? Why is CI identity only being portrayed in essentialist terms on these types of shows? Why are there no realistic portrayals of people with CIs (and for that matter, deaf people) as the richly varied individuals that they are? These questions aside, if these two types of “implanting rhetoric”, the sentimentalised and the terminated, are all we have at the moment, what does it mean? As I mentioned early in this essay, deaf people, along with many “others,” have long helped to highlight and define the hegemonic “norm.” The apparent cultural need for a Foucauldian “marked body” explains not only the popularity of crime dramas, but it also could explain the oddly proliferant use of characters with cochlear implants in these particular shows. A person with an implant on the side of their head is definitely a more “marked” body than the deaf person with no hearing aid. The CI character is more controversial, more shocking; it’s trendier, “sexier”, and this boosts ratings. But CI characters are, unlike their deaf predecessors, now serving an additional cultural function. I believe they are, as I claim in the beginning of this essay, screens upon which our culture is now projecting repressed anxieties about emergent technology. The two essentialist rhetorics of the cochlear implant, the rhetoric of the sentimental, medical model, and the rhetoric of genocide, ultimately represent our technophilia and our technophobia. The CI character embodies what Debra Shaw terms a current, “ontological insecurity that attends the interface between the human body and the datasphere” (85). We are growing more nervous “as new technologies shape our experiences, they blur the lines between the corporeal and incorporeal, between physical space and virtual space” (Selfe). Technology either threatens the integrity of the self, “the coherence of the body” (we are either dead or damaged) or technology allows us to transcend the limitations of the body: we are converted, “transformed”, the recipient of a happy modern miracle. In the end, I found that representations of CI on television (in the United States) are overwhelmingly sentimental and therefore essentialist. It seems that the conflicting nineteenth century tendency of attraction and revulsion toward the deaf is still, in the twenty-first century, evident. We are still mired in the rhetoric of “cure” and “control,” despite an active Deaf counter discourse that employs the language of the holocaust, warning of the extermination of yet another cultural minority. We are also daily becoming daily more “embedded in cybernetic systems,” with our laptops, emails, GPSs, PDAs, cell phones, Bluetooths, and the likes. We are becoming increasingly engaged in a “necessary relationship with machines” (Shaw 91). We are gradually becoming no longer “other” to the machine, and so our culturally constructed perceptions of ourselves are being threatened. In the nineteenth century, divisions and hierarchies between a white male majority and the “other” (women, African Americans, immigrants, Native Americans) began to blur. Now, the divisions between human and machine, as represented by a person with a CI, are starting to blur, creating anxiety. Perhaps this anxiety is why we are trying, at least in the media, symbolically to ‘cure’ the marked body or kill off the cyborg. Future examinations of the discourse should, I believe, use these media constructions as a lens through which to continue to examine and illuminate the complex subject position of the CI identity, and therefore, perhaps, also explore what the subject position of the post/human identity will be. References "A Boy in a Tree." Patrick Norris (dir.), Hart Hanson (by), Emily Deschanel (perf.). Bones, Fox Network, 7 Sep. 2005. “Andy in C Minor.” Jeannete Szwarc (dir.), Gavin Harris (by), Kathryn Morris (perf.). Cold Case, CBS Network, 30 March 2008. Blume, Stuart. “The Rhetoric and Counter Rhetoric of a “Bionic” Technology.” Science, Technology and Human Values 22.1 (1997): 31-56. Brueggemann, Brenda Jo. Deaf Subjects: Between Identities and Places. New York: New York UP, 2009. “Cochlear Implant Statistics.” ASL-Cochlear Implant Community. Blog. Citing Laurent Le Clerc National Deaf Education Center. Gallaudet University, 18 Mar. 2008. 29 Apr. 2010 ‹http:/ /aslci.blogspot.com/2008/03/cochlear-implant-statistics.html›. “Cures to Come.” Discover Presents the Brain (Spring 2010): 76. Fischman, Josh. “Bionics.” National Geographic Magazine 217 (2010). “House Divided.” Greg Yaitanes (dir.), Matthew V. Lewis (by), Hugh Laurie (perf.). House, Fox Network, 22 Apr. 2009. “Inside-Out.” Gina Lamar (dir.), Anthony Zuiker (by), David Caruso (perf.). CSI: Miami, CBS Network, 8 Oct. 2007. Krentz, Christopher. Writing Deafness: The Hearing Line in Nineteenth-Century American Literature. Chapel Hill: UNC P, 2007. Ladd, Paddy. Understanding Deaf Culture: In Search of Deafhood. Clevedon, UK: Multilingual Matters Limited, 2002. Lane, Harlan. A Journey Into the Deaf-World. San Diego: DawnSignPress, 1996. “NAD Position Statement on the Cochlear Implant.” National Association of the Deaf. 6 Oct. 2000. 29 April 2010 ‹http://www.nad.org/issues/technology/assistive-listening/cochlear-implants›. Nussbaum, Debra. “Manufacturer Information.” Cochlear Implant Information Center. National Deaf Education Center. Gallaudet University. 29 Apr. 2010 < http://clerccenter.gallaudet.edu >. Shaw, Debra. Technoculture: The Key Concepts. Oxford: Berg, 2008. “Silent Night.” Rob Bailey (dir.), Anthony Zuiker (by), Gary Sinise (perf.). CSI: New York, CBS Network, 13 Dec. 2006. “Sweet Nothing in My Ear.” Joseph Sargent (dir.), Stephen Sachs (by), Jeff Daniels (perf.). Hallmark Hall of Fame Production, 20 Apr. 2008. TWIZ TV scripts. CSI: Miami, “Inside-Out.” “What Is the Surgery Like?” FAQ, University of Miami Cochlear Implant Center. 29 Apr. 2010 ‹http://cochlearimplants.med.miami.edu/faq/index.asp›.

La tesi si propone di indagare la produzione in bronzo a Napoli, in rapporto alla scultura di grande formato, in un arco cronologico che va dagli anni novanta del Cinquecento fino agli anni settanta del Seicento. Lo studio mira a dimostrare come nella capitale del Viceregno, a cavallo tra i due secoli, si assista alla nascita e sviluppo di una tradizione fusoria locale al pari di altri centri dell'industria del bronzo della Penisola come Firenze, Milano, Roma e il Veneto. Il primo capitolo circoscrive i ''contesti del bronzo'' a Napoli, nello specifico l'Arsenale e le corporazioni dedite alla lavorazione dei metalli. La macchina della committenza, oggetto del secondo capitolo, funge da osservatorio privilegiato per misurare il mutamento del gusto condizionato dai dettami tridentini. Gli Ordini degli Oratoriani, dei Gesuiti e dei Teatini furono i principali interpreti delle istanze postconciliari e veicolo per l'arrivo nel Viceregno di artisti e architetti provenienti da Roma. La seconda parte del lavoro intende tracciare una storia della scultura in bronzo napoletana, che tenga conto della collaborazione con i fusores quali co-autori delle opere in metallo. Il terzo capitolo si apre con Michelangelo Naccherino (1550-1622), la cui attività viene riletta alla luce di una nuova documentazione e di un possibile viaggio a Roma. L'analisi dell'attività di comprimari come Andrea Bolgi (1605-1656), Christophe Cochet (1606-1634), Camillo Mariani (1567-1611), Tommaso Montani (doc. 1593-1622), Cristoforo (doc. 1583-1622) e Giovan Domenico Monterosso (doc. 1603-1630) contribuisce a tracciare un quadro più articolato. L'avvio verso il barocco (1635-1669), il quarto e ultimo capitolo della tesi, è incentrato sulla decorazione del Tesoro di San Gennaro, dalla serie bronzea dei Santi Protettori di Giuliano Finelli (1601-1653) alla travagliata gestazione della Cancellata di ottone di Cosimo Fanzago (1591-1678). In parallelo, il caso del convento dei Santi Apostoli offre l'occasione per fare un bilancio sull'arrivo e diffusione a Napoli di modelli romani attraverso il legame con la Casa madre di Sant'Andrea della Valle. La tesi è corredata da un Repertorio degli scultori e fonditori attivi a Napoli dal 1596 al 1669, in cui sono raccolte le notizie edite e inedite su questi artefici, e da un apparato illustrativo, in gran parte inedito, condotto attraverso un'apposita campagna fotografica.