Cochlear implant eligibility in an adult hearing aid population: a multi-perspective service evaluation of a patient referral pathway at a British district general hospital.

OBJECTIVES: To quantify patient eligibility for cochlear implantation following National Institute for Health and Care Excellence 2019 guidelines (TA566) over five years at our institution, and identify factors influencing patients' decisions surrounding cochlear implantation referral. METHODS: A multi-perspective service evaluation was conducted at a district general hospital, comprising cochlear implantation eligible patients. The main outcome measures were: eligibility numbers for 2014-2019, comparing application of TA566 versus 2009 (TA166) guidelines; and patient interview transcripts and questionnaires. RESULTS: There was a 259 per cent average increase in cochlear implantation eligibility from 2014 to 2019. Most patients' thresholds were 80 dB HL or more at 3 kHz and 4 kHz. There are several cochlear implantation barriers, including patient-centred issues (e.g. health-related anxieties, implantation misperceptions) and external barriers (difficulty getting to regional implant centres). Motivating factors for cochlear implantation include improved quality of life and access to local cochlear implantation services. CONCLUSION: The TA566 guidelines have increased cochlear implantation eligibility, putting pressure on cochlear implantation centres and referring hospitals. Current referral systems have external and patient-centred implantation barriers. British cochlear implantation delivery may need rethinking to meet increasing populational demands and improve accessibility for those most vulnerable to these barriers.

Plasma cell depletion with bortezomib in the treatment of refractory N-methyl-d-aspartate (NMDA) receptor antibody encephalitis. Rational developments in neuroimmunological treatment.

BACKGROUND AND PURPOSE: The aim was to assess the therapeutic potential of bortezomib in the treatment of refractory N-methyl-d-aspartate receptor (NMDAR) antibody encephalitis and its potential in other immune-mediated, B-cell-driven neurological diseases. METHODS: Two cases of severe NMDAR antibody encephalitis, resistant to first and second line therapy with steroids, intravenous immunoglobulins, plasma exchange, cyclophosphamide and rituximab, were treated with four and five cycles of 1.3 mg/m2 bortezomib at 350 and 330 days following initial presentation. RESULTS: Both patients showed significant clinical improvement with reductions of NMDAR antibody titres following bortezomib treatment. This is the first case in the literature where the NMDAR antibody level was undetectable following treatment with bortezomib. CONCLUSION: Bortezomib's unique ability to target long-lived autoreactive plasma cells appears to be a useful adjunct to standard second line immunosuppressive therapy in treatment-refractory NMDAR antibody encephalitis. The drug's pharmacodynamics, cell targeting and mechanism of action are reviewed, and it is postulated that bortezomib may be useful in a host of B-cell-driven neuroimmunological diseases.