Highest reported visual acuity after electronic retinal implantation.

Purpose: To report the highest attained visual acuity with an electronic retinal implant for the treatment of advanced retinal degeneration following a novel intensive period of visual training.

Methods: A case study as part of the prospective, international, multi-centre, interventional clinical trial (ClinicalTrials.gov NCT02720640 and NCT01024803) of patients with the Retina Implant Alpha AMS (Retina Implant AG, Reutlingen, Germany) for advanced retinal degeneration.

CD4 T Follicular Helper Cells in Human Tonsils and Blood Are Clonally Convergent but Divergent from Non-Tfh CD4 Cells.

T follicular helper (Tfh) cells are fundamental for B cell selection and antibody maturation in germinal centers. Circulating Tfh (cTfh) cells constitute a minor proportion of the CD4 T cells in peripheral blood, but their clonotypic relationship to Tfh populations resident in lymph nodes and the extent to which they differ from non-Tfh CD4 cells have been unclear. Using donor-matched blood and tonsil samples, we investigate T cell receptor (TCR) sharing between tonsillar Tfh cells and peripheral Tfh and non-Tfh cell populations.

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

Background: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a rare clinical syndrome of unknown cause usually identified in children. Tonsillectomy is considered a potential treatment option for this syndrome. This is an update of a Cochrane Review first published in 2010 and previously updated in 2014.

Progression of hearing loss in neurofibromatosis type 2 according to genetic severity.

Objectives/hypothesis: This study set out to describe the progression of hearing loss in patients with neurofibromatosis type 2 (NF2), treated in a quaternary multidisciplinary clinic. It also aimed to compare hearing loss across patients grouped according to a known genetic severity score to explore its utility for prognostication.

Study Design: Retrospective cohort study.

Assessment of the Electronic Retinal Implant Alpha AMS in Restoring Vision to Blind Patients with End-Stage Retinitis Pigmentosa.

Purpose: To report the initial efficacy results of the Retina Implant Alpha AMS (Retina Implant AG, Reutlingen, Germany) for partial restoration of vision in end-stage retinitis pigmentosa (RP).

Design: Prospective, single-arm, investigator-sponsored interventional clinical trial. Within-participant control comprising residual vision with the retinal implant switched ON versus OFF in the implanted eye.

Interim Results of a Multicenter Trial with the New Electronic Subretinal Implant Alpha AMS in 15 Patients Blind from Inherited Retinal Degenerations.

We assessed the safety and efficacy of a technically advanced subretinal electronic implant, RETINA IMPLANT Alpha AMS, in end stage retinal degeneration in an interim analysis of two ongoing prospective clinical trials. The purpose of this article is to describe the interim functional results (efficacy). The subretinal visual prosthesis RETINA IMPLANT Alpha AMS (Retina Implant AG, Reutlingen, Germany) was implanted in 15 blind patients with hereditary retinal degenerations at four study sites with a follow-up period of 12 months (www.

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

Background: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a rare clinical syndrome of unknown cause usually identified in children. Tonsillectomy is considered a potential treatment option for this syndrome. This is an update of a Cochrane review first published in 2010.

Simultaneous labyrinthectomy with cochlear implantation in patients with bilateral Ménière's disease.

Objective: The development of second-side Ménière's disease in the only remaining serviceable ear is difficult to treat. We describe an intervention to control disabling disease combining a labyrinthectomy and cochlear implant to restore hearing.

Methods: Following a thorough preoperative assessment and consenting process, 2 patients underwent labyrinthectomy of the affected ear with simultaneous cochlear implantation.

Periodic botulinum toxin injections for paradoxical vocal fold motion in a child with cerebral palsy: a case study.

We describe an unusual case of paradoxical vocal fold motion in a child with cerebral palsy. Clinically, the child presented with mild stridor, which worsened over months, eventually requiring emergency intubation. After an unsuccessful trial of medical management, microlaryngoscopy revealed abnormal adduction of the vocal folds during inspiration.

Cochlear implants to treat deafness caused by vestibular schwannomas.

Objective: Rehabilitation of hearing is complicated in patients with profound bilateral hearing loss in the presence of sporadic vestibular schwannoma (VS) or neurofibromatosis 2 (NF2), especially if the tumor does not need to be removed. We present the outcome of patients who have had a cochlear implant in the tumor affected ear without removal of the primary tumor.

Design: This is a retrospective multicentre study investigating outcomes of cochlear implantation in profoundly deaf patients with vestibular schwannoma in the implanted ear.

Adenotonsillectomy for the management of obstructive sleep apnea in children with congenital craniosynostosis syndromes.

Children with congenital craniosynostosis syndromes have a high incidence of obstructive sleep apnea (OSA). Obstructive sleep apnea has significant consequences including impaired growth, learning and behavioral problems, and cardiovascular morbidity. Adenotonsillectomy is the treatment of choice for OSA in otherwise healthy children.

European Bilateral Pediatric Cochlear Implant Forum consensus statement.

This is a consensus statement on pediatric cochlear implantation by the European Bilateral Pediatric Cochlear Implant Forum. The consensus statement was determined by review of current scientific literature to identify areas of scientific and clinical agreement of current understanding of bilateral cochlear implantation. The statement is "Currently we feel that the infant or child with unambiguous cochlear implant candidacy should receive bilateral cochlear implants simultaneously as soon as possible after definitive diagnosis of deafness to permit optimal auditory development; an atraumatic surgical technique designed to preserve cochlear function, minimize cochlear damage, and allow easy, possibly repeated re-implantation is recommended.

Cochlear implantation in superficial siderosis: a viable option?

Objective: Superficial siderosis of the central nervous system is characterized by accumulation of haemosiderin in the subpial layers of the brain and spinal cord. The evidence largely suggests a retro-cochlear cause for hearing loss with questionable involvement of cochlea. We present our experience with two patients of superficial siderosis who underwent cochlear implantation, and discuss their outcomes and the underlying pathology.

Traumatic perilymphatic fistula with pneumolabyrinth: diagnosis and management.

A perilymphatic fistula (PLF) is an abnormal communication between the inner and middle ear resulting in vestibular or cochlear symptoms. We review three pediatric traumatic temporal bone fractures with pneumolabyrinth, confirmed radiologically by the presence of air within the cochlea (pneumocochlea) or vestibule (pneumovestibule). Patients were treated conservatively with complete resolution of vestibulopathy.

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

Background: PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome) is a rare clinical syndrome of unknown cause usually identified in children.

Objectives: To assess the efficacy of tonsillectomy (with or without adenoidectomy) in children with PFAPA.

Search Strategy: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library, 2010 Issue 1); MEDLINE (PubMed); EMBASE; CINAHL; mRCT (metaRegister of clinical trials, including ClinicalTrials.

Bilateral simultaneous cochlear implantation in children: our first 50 cases.

Objectives/hypothesis: To assess the safety and surgical technique of bilateral simultaneous cochlear implantation in children.

Study Design: Prospective case series at a tertiary academic pediatric hospital.

Methods: Surgical times, complications, and patient outcomes are reported from the first 50 consecutive simultaneous cochlear implants performed at the Hospital for Sick Children between 2005 and 2008.

Parental and program's decision making in paediatric simultaneous bilateral cochlear implantation: who says no and why?

Objective: To evaluate initial candidacy for bilateral simultaneous cochlear implantation in children.

Design: Prospective case series.

Setting: Tertiary academic pediatric hospital.

Soft tissue complications after small incision pediatric cochlear implantation.

Objectives/hypothesis: To report and analyze the soft tissue complications of 462 consecutive cochlear implants using a minimal access approach at a single institution.

Study Design: A retrospective case series analysis was performed.

Methods: A database of all patients implanted at our institution between January 2002 and December 2007 was searched, and 385 consecutive patients were identified.

Choanal atresia and choanal stenosis.

Congenital narrowing of the nasal airway at the posterior choanae, which can be uni- or bilateral, is an uncommon condition in pediatric patients. The surgical management of choanal atresia varies widely in different centers. This article discusses the different surgical strategies including: dilation and stenting; trans-palatal repair; and transnasal resection utilizing endoscopic sinus surgery (ESS) techniques.

Gradenigo's syndrome: successful conservative treatment in adult and paediatric patients.

A triad of retro-ocular pain, discharging ear and abducens nerve palsy, as described by Gradenigo, has been recognized for 150 years. It has traditionally been treated with surgery, but recent advances in imaging, allied with improved antibiotic treatment, allow conservative management of these cases. We present two cases of Gradenigo's syndrome: a 6-year-old child and a 70-year-old man, both without cholesteatoma, who were managed without mastoidectomy.

Complete inhibition of goiter in mice requires combined gene therapy modification of angiopoietin, vascular endothelial growth factor, and fibroblast growth factor signaling.

In goiter, increased expression of growth factors and their receptors occurs. We have inhibited the action of some of these growth factors, alone and in combination, to determine which are important in goitrogenesis. Recombinant adenovirus vectors (RAds) expressing truncated, secreted forms of human Tie2 (RAd-sTie2) and vascular endothelial growth factor receptor 1 (RAd-sVEGFR1) or a truncated, dominant-negative fibroblast growth factor receptor 1 (RAdDN-FGFR1) were used.