Video head impulse testing: Pitfalls in neurological patients.

The video head impulse test (vHIT) assesses the vestibulo-ocular reflex (VOR) during a rapid high-velocity low amplitude (10°-20°) head rotation. Patients with peripheral vestibulopathy have a reduced VOR gain with corrective catch-up saccades during the head turn. There are several pitfalls, mainly technical, which may interfere with interpretation of vHIT data. In addition, intrusive eye movement disorders such as spontaneous nystagmus that affect normal eye position and tracking can affect the vHIT results. To date there has been little study of neurological saccadic eye movements that may interfere with the interpretation of vHIT data. Here, in ten patients with a range of central neurological disorders, we describe oculomotor abnormalities on vHIT in the presence of normal range VOR gain values, recorded at a tertiary vestibular neurology service.

Autoimmune Vestibulopathy-A Case Series.

Autoimmune inner ear disease (AIED) is a rare clinical entity. Its pathogenicity, heterogenous clinical presentation in the context of secondary systemic autoimmune disease and optimal treatment avenues remain poorly understood. Vestibular impairment occurring in the context of AIED is rarely subject to detailed investigation given that the auditory symptoms and their responsiveness to immunosuppression are the focus of the few proposed diagnostic criteria for AIED. We present three cases of vestibulopathy occurring in the context of autoimmune inner ear conditions, including the first known report of autoimmune inner ear pathology arising with a temporal association to administration of the Pfizer-BioNTech SARS-CoV2 vaccination. We review the available literature pertinent to each case and summarise the key learning points, highlighting the variable presentation of vestibular impairment in AIED.

Improving transition outcomes in adolescents with permanent hearing loss.

Young people with permanent hearing loss need to transition into adult services and engagement with the transition process is required to achieve this. Although there are clear national recommendations about the transition process and transfer to the adult services limited evidence has been published about the implementation and efficacy of transition programmes in young adults with permanent hearing loss. The primary aim of this quality improvement project was to significantly increase the number of teenagers with a clear transition protocol documented in their notes. The secondary aims were to ensure good medical and audiological outcomes based on all completing their medical investigations prior to transfer to the adult services and also based on a survey regarding attitudes to hearing aids use. A multiphase intervention programme was developed; (1) staff update with national transition protocols, (2) development of a clear transition protocol, (3) allocated key worker (4) access and (5) engagement. Through these interventions we increased the number of teenagers who had a clear transition protocol documented in their notes from 11% to 91%. 84% of our teenagers had completed their medical investigations, although documented discussion about the investigations had been done with 100% of the cohort. 9.2 % of adolescents felt pressurised to use hearing aids compared to 35% prior to the interventions. This project resulted in clear, measurable conclusions and we have shown that we better understand the needs of our patients and the improvement is sustainable.

Lambda light chain revision in the human intestinal IgA response.

Revision of Ab L chains by secondary rearrangement in mature B cells has the potential to change the specific target of the immune response. In this study, we show for the first time that L chain revision is normal and widespread in the largest Ab producing population in man: intestinal IgA plasma cells (PC). Biases in the productive and non-productive repertoire of lambda L chains, identification of the circular products of rearrangement that have the characteristic biases of revision, and identification of RAG genes and protein all reflect revision during normal intestinal IgA PC development. We saw no evidence of IgH revision, probably due to inappropriately orientated recombination signal sequences, and little evidence of kappa-chain revision, probably due to locus inactivation by the kappa-deleting element. We propose that the lambda L chain locus is available and a principal modifier and diversifier of Ab specificity in intestinal IgA PCs.