Stereotactic Radiosurgery and Radiotherapy for Vestibular Schwannoma in NF2-Related Schwannomatosis.

OBJECTIVES: To determine the long-term control rates and hearing outcomes for growing vestibular schwannoma in NF2-related schwannomatosis (NF2) treated with stereotactic radiosurgery (SRS) and fractionated radiotherapy (FRT). METHODS: Retrospective review of all patients treated with SRS/FRT between 1986 and2021 from a tertiary NF2 unit. Overall tumor control was defined as: (1) growth control (growth failure was defined as growth in any dimension of 3 millimetres or more from baseline post-SRS/FRT), and (2) treatment control (no need for further intervention). Loss of serviceable hearing was defined as a drop in speech discrimination score below 50% after SRS/FRT. RESULTS: There were 81 cases, with a mean duration of follow-up of 125 months. Overall control rate was 72% (58/81), with 80% (65/81) growth control and 74% (60/81) treatment control. There was a 5-year actuarial survival of 77% and 10-year survival of 71%. Forty-three percent (30/69) of cases did not have serviceable hearing at baseline. Of those remaining, 49% (19/39) preserved serviceable hearing during follow-up at a mean of 106 months. Actuarial survival for preservation of serviceable hearing at 5 and 10 years was 69% and 53%. There were poorer outcomes with increasing genetic severity, and with baseline tumor size >3 cm. No cases of SRS/FRT-related malignancy were identified at a mean follow-up of 10 years. CONCLUSION: Stereotactic radiosurgery/fractionated radiotherapy are an effective option to treat growing vestibular schwannoma in patients with NF2 with the potential for hearing preservation in a proportion of patients. LEVEL OF EVIDENCE: 4-Case Series Laryngoscope, 134:2364-2371, 2024.

Cochlear Implantation in Children with Autism Spectrum Disorder: A Systematic Review and Pooled Analysis.

OBJECTIVE: To determine outcomes following cochlear implantation (CI) in children with autism spectrum disorder (ASD). DATABASES REVIEWED: MEDLINE, Embase, Web of science, Cochrane Library, and Clinicaltrial.gov. METHODS: The review was performed according to the PRISMA statement. Primary outcomes measures were changes in speech perception and speech production scores. Secondary outcome measures included communication mode, device use, parental recommendation of implant, postoperative hyperacusis, and quality of life measures. Pooled analysis of outcomes was performed if possible. RESULTS: Twenty-four studies reported on 159 children with ASD. There were improvements in speech perception in 78% of cases and in speech expression in 63% of cases, though the extent of this improvement was variable. Seventy-four percent of children with ASD and CI are nonoral communicators. Intermittent/nonuse rate was 31%. Hearing outcomes are worse compared to children with other disabilities. The vast majority of parents would recommend CI based on their experiences. CONCLUSION: Outcome in children with ASD and CI are highly variable and significantly poorer compared to non-ASD children. Despite this, most parents report positive experiences and the evidence supports the use of CI in children with ASD.

Assessment of the cochlear implant pathway for newborn hearing screening referrals.

OBJECTIVE: To evaluate the cochlear implant (CI) assessment pathway for children referred through the newborn hearing screening programme (NHSP) and identify potential reasons for delay in CI surgery at a large UK centre. METHODS: Retrospective analysis of electronic notes of children referred through NHSP from 2016 to 2019. Key points in the assessment pathway from referral to switch-on were assessed. RESULTS: Of the 46 referrals, 44 went on to have CI surgery. Median age at CI surgery was 15 months (interquartile range (IQR) 13 to 17 months). Only 5% of referrals were implanted before the age of 12 months. Delayed referral and the presence of otitis media with effusion (OME) were associated with delayed implantation. The median period of monitoring for OME was 48 days (IQR 20 to 93 days), with a natural resolution rate of only 11%. The median time from surgery to switch-on was 5 weeks (IQR 4.1 to 5.4 weeks). CONCLUSION: Achieving implantation prior to the age of 12 months for NHSP referrals can be challenging for a variety of reasons. The delays in the system and the steps that our implant programme has taken to address these will be highlighted.

Development of electrophysiological and behavioural measures of electrode discrimination in adult cochlear implant users.

The plasticity of the auditory system enables it to adjust to electrical stimulation from cochlear implants (CI). Whilst speech perception may develop for many years after implant activation, very little is known about the changes in auditory processing that underpin these improvements. Such an understanding could help guide interventions that improve hearing performance. In this longitudinal study, we examine how electrode discrimination ability changes over time in newly implanted adult CI users. Electrode discrimination was measured with a behavioural task as well as the spatial auditory change complex (ACC), which is a cortical response to a change in place of stimulation. We show that there was significant improvement in electrode discrimination ability over time, though in certain individuals the process of accommodation was slower and more limited. We found a strong relationship between objective and behavioural measures of electrode discrimination using pass-fail rules. In several cases, the development of the spatial ACC preceded accurate behavioural discrimination. These data provide evidence for plasticity of auditory processing in adult CI users. Behavioural electrode discrimination score but not spatial ACC amplitude was found to be a significant predictor of speech perception. We suggest that it would be beneficial to measure electrode discrimination in CI users and that interventions that exploit the plastic capacity of the auditory system to improve basic auditory processing, could be used to optimize performance in CI users.

Objective assessment of electrode discrimination with the auditory change complex in adult cochlear implant users.

The spatial auditory change complex (ACC) is a cortical response elicited by a change in place of stimulation. There is growing evidence that it provides a useful objective measure of electrode discrimination in cochlear implant (CI) users. To date, the spatial ACC has only been measured in relatively experienced CI users with one type of device. Early assessment of electrode discrimination could allow auditory stimulation to be optimized during a potentially sensitive period of auditory rehabilitation. In this study we used a direct stimulation paradigm to measure the spatial ACC in both pre- and post-lingually deafened adults. We show that it is feasible to measure the spatial ACC in different CI devices and as early as 1 week after CI switch-on. The spatial ACC has a strong relationship with performance on a behavioural discrimination task and in some cases provides information over and above behavioural testing. We suggest that it may be useful to measure the spatial ACC to guide auditory rehabilitation and improve hearing performance in CI users.

A report on 15 years of clinical negligence claims in rhinology.

BACKGROUND: This study was designed to determine the characteristics of medical negligence claims in rhinology. In 2010-2011 the National Health Service (NHS) litigation bill surpassed 1 billion Great British Pounds (GBP; 1.52 billion U.S. dollars [US$]). Systematic analysis of malpractice complaints allows for the identification of errors and can thereby improve patient safety and reduce the burden of litigation claims on health services. METHODS: Claims relating to ear, nose, and throat between 1995 and 2010 were obtained from the NHS Litigation Authority and were analyzed. RESULTS: The series contains 65 closed claims that resulted in payment totaling 3.1 million GBP (US$4.7 million). Fifty claims were related to surgical complications. Functional endoscopic sinus surgery and septoplasty were the procedures most commonly associated with successful claims. There were 11 cases of orbital injury including 6 cases of visual loss and 5 cases of diplopia. The most common cause of a claim was failure to recognize the complication or manage it appropriately. Lack of informed consent was claimed in eight cases. Other claims arose because of errors in outpatient procedures (two), diagnosis (six), delayed surgery (one), and errors in medical management (three). CONCLUSION: This is the first study to report the outcomes of negligence claims in rhinology in the United Kingdom. Claims in rhinology are associated with a high success rate. Steps that can be taken to reduce litigation include careful patient workup and ensuring adequate informed consent. Where there is a suspicion of orbital damage early recognition and intervention is needed to reduce long-term injury to the patient.

Cochlear implantation in a patient with combined renal and liver transplantation.

OBJECTIVE AND IMPORTANCE: Patients who have undergone solid organ transplantation and continuing immunosuppressant medication are at a higher risk of wound problems and infections following cochlear implantation. This risk is theoretically even further increased in multi-organ transplant recipients due to the increased doses of immunosuppressive medications that these patients are administered. CLINICAL PRESENTATION AND INTERVENTION: Here, we present the first reported case of successful cochlear implantation in a patient who had previously undergone successful combined liver and kidney transplant. She had no significant complications from the surgery and had good audiological outcomes 3 months post-operatively. CONCLUSION: As we continue our advances in the use of cochlear implant technology, our report adds to the growing evidence of its benefits in transplant recipients. However, there are important pre- and peri-operative considerations in this group of patients which can improve safety and outcome.

Analysis of clinical negligence claims following tonsillectomy in England 1995 to 2010.

OBJECTIVES: We determined the characteristics of medical negligence claims following tonsillectomy. METHODS: Claims relating to tonsillectomy between 1995 and 2010 were obtained from the National Health Service Litigation Authority database. The number of open and closed claims was determined, and data were analyzed for primary injury claimed, outcome of claim, and associated costs. RESULTS: Over 15 years, there were 40 claims of clinical negligence related to tonsillectomy, representing 7.7% of all claims in otolaryngology. There were 34 closed claims, of which 32 (94%) resulted in payment of damages. Postoperative bleeding was the most common injury, with delayed recognition and treatment of bleeding alleged in most cases. Nasopharyngeal regurgitation as a result of soft palate fistulas or excessive tissue resection was the next-commonest cause of a claim. The other injuries claimed included dentoalveolar injury, bums, tonsillar remnants, and temporomandibular joint dysfunction. Inadequate informed consent was claimed in 5 cases. CONCLUSIONS: Clinical negligence claims following tonsillectomy have a high success rate. Although postoperative bleeding is the most common cause of negligence claims, a significant proportion of claims are due to rare complications of surgery. Informed consent should be tailored to the individual patient and should include a discussion of common and serious complications.

Toward safer practice in otology: a report on 15 years of clinical negligence claims.

OBJECTIVES/HYPOTHESIS: To determine the characteristics of medical negligence claims arising from otological practice. STUDY DESIGN: Retrospective analysis of medical negligence claims contained in the National Health Service Litigation Authority (NHSLA) database. METHODS: Claims relating to otology and neurotology between 1995 and 2010 were obtained from the NHSLA database and analyzed for cause of injury, type of injury, outcome of claim and costs. RESULTS: Over 15 years there were 137 claims in otology, representing 26% of all the claims in otolaryngology. Of these, 116 have been closed, and 84% of closed claims resulted in payment. Of the 97 successful claims, 63 were related to operative complications. This included six cases of wrong side/site surgery, and 15 cases of inadequate informed consent. The most common injuries claimed were hearing loss, facial paralysis, and additional/unnecessary surgery. Middle ear ventilation and mastoid surgery were the procedures most commonly associated with a successful claim. There were 15 successful claims of misdiagnosis/delayed diagnosis, with chronic suppurative otitis media the condition most frequently missed. There were nine successful claims related to outpatient procedures, of which seven were for aural toilet and six claims of medical mismanagement, including three cases of ototoxicity from topical medications. There were also four successful claims for morbidity due to delayed surgery. CONCLUSIONS: This is the first study to report outcomes of negligence claims in otology. Claims in otology are associated with a high success rate. A significant proportion of claims are not related to surgery and represent areas where safety should also be addressed.

Overcoming barriers to secondary prevention in hip fracture patients: An electronic referral and management system for osteoporosis.

BACKGROUND: Numerous studies have shown that osteoporosis is both under diagnosed and under treated in patients with fragility fractures. We describe the use of an electronic automated referral system to enable multidisciplinary assessment and treatment of osteoporosis in patients admitted with fragility fracture of the hip. METHODS: An electronic referral system was developed from the hospital's trauma software database. This automatically refers patients admitted with fragility fracture of the hip to osteoporosis services. Data were collected prospectively from May to July 2007, when referral was dependent on members of the orthopaedic team and from August to October 2007, after the implementation of the electronic referral system. Primary outcomes were presence of a referral to osteoporosis services, organisation of bone density scan and follow up appointment in patients less than 75 years, and treatment with osteoporosis medication in patients greater than 75 years of age. RESULTS: There were a total of 90 patients, 47 in the non-intervention group and 43 in the intervention group. In the non-intervention group 8/47 patients (17%) were referred to osteoporosis services whilst 43/43 (100%) were referred in the intervention group. Of patients greater than 75 years, 10/32 (31.3%) were started on a bisphosphonate in the non-intervention group compared to 28/34 (82.4%) in the intervention group. Of patients less than 75 years, 1/15 (7%) in the non-intervention group had a DEXA scan booked and appropriate follow up arranged, compared to 7/9 (78%) in the intervention group. There was significant difference between both groups in all outcomes (p<0.0001). CONCLUSION: We present an electronic system which facilitates delivery of osteoporosis services and significantly improves management of osteoporosis in patients admitted with fragility fracture. We recommend the use of such programmes to facilitate multidisciplinary assessment and treatment of osteoporosis in orthopaedic trauma patients.

Development of electronic software for the management of trauma patients on the orthopaedic unit.

INTRODUCTION: Continuity of patient care is an essential prerequisite for the successful running of a trauma surgery service. This is becoming increasingly difficult because of the new working arrangements of junior doctors. Handover is now central to ensure continuity of care following shift change over. The purpose of this study was to compare the quality of information handed over using the traditional ad hoc method of a handover sheet versus a web-based electronic software programme. It was hoped that through improved quality of handover the new system would have a positive impact on clinical care, risk and time management. METHODS: Data was prospectively collected and analyzed using the SPSS 14 statistical package. The handover data of 350 patients using a paper-based system was compared to the data of 357 cases using the web-based system. Key data included basic demographic data, responsible surgeon, location of patient, injury site including site, whether fractures were open or closed, concomitant injuries and the treatment plan. A survey was conducted amongst health care providers to assess the impact of the new software. RESULTS: With the introduction of the electronic handover system, patients with missing demographic data reduced from 35.1% to 0.8% (p<0.0001) and missing patient location from 18.6% to 3.6% (p<0.0001). Missing consultant information and missing diagnosis dropped from 12.9% to 2.0% (p<0.0001) and from 11.7% to 0.8% (p<0.0001), respectively. The missing information regarding side and anatomical site of the injury was reduced from 31.4% to 0.8% (p<0.0001) and from 13.7% to 1.1% (p<0.0001), respectively. In 96.6% of paper ad hoc handovers it was not stated whether the injury was 'closed' or 'open', whereas in the electronic group this information was evident in all 357 patients (p<0.0001). A treatment plan was included only in 52.3% of paper handovers compared to 94.7% (p<0.0001) of electronic handovers. A survey revealed 96% of members of the trauma team felt an improvement of handover since the introduction of the software, and 94% of members were satisfied with the software. CONCLUSIONS: The findings of our study show that the use of web-based electronic software is effective in facilitating and improving the quality of information passed during handover. Structured software also aids in improving work flow amongst the trauma team. We argue that an improvement in the quality of handover is an improvement in clinical practice.