Hearing preservation in paediatric cochlear implantation with the Nucleus Slim Straight Electrode - our experience.

OBJECTIVE: to evaluate the levels of successful hearing preservation and preservation of functional hearing following cochlear implantation (HPCI) in children using the Cochlear Nucleus® Slim Straight Electrode (SSE). DESIGN: retrospective case note review of paediatric HPCI cases in our CI centre from 2013 to 2023. Inclusion criteria were attempted hearing preservation surgery, SSE used for implantation, pre-operative hearing thresholds ≤80dBHL at 250 Hz, CI before 18 years of age. Patients were excluded if no postoperative unaided PTA was obtained (poor attendance). Primairy outcome was hearing preservation using the HEARRING group formula; secondary outcome was residual functional hearing (≤80dBHL at 250 Hz/<90dB LFPTA). STUDY SAMPLE: 56 patients with 94 CI's were included for review. RESULTS: Hearing preservation was achieved in 94.7% (89/94) of ears and complete preservation in 72% (68/94)). Average functional hearing was preserved in 89% using both criteria for preservation. Long-term follow up data was available for 36 ears (average 35.2 months), demonstrating 88.9% (32/36) complete preservation. CONCLUSION: We have reliably achieved and maintained a high success rate of HPCI using the SSE in our paediatric population. The field of HPCI would benefit from unification of outcome reporting in order to optimise the evidence available to professionals, patients and their carers.

The benefits of preserving residual hearing following cochlear implantation: a systematic review.

OBJECTIVE: Systematically review the current literature for evidence on the "real-life" benefits of hearing preservation cochlear implantation (HPCI) for children and adults. DESIGN: Systematic search of Pubmed, MEDLINE, EMBASE, CINHAL and Cochrane Library for MesH terms hearing¸ preservation and cochlear implantation. Inclusion criteria were the "real-life" benefit of HPCI i.e. other than pre- and post-operative pure tone thresholds. Exclusion criteria were non-English language, conference abstracts, reviews and animal and cadaveric studies. Risk of bias was assessed using the Evidence Project Tool. STUDY SAMPLE: 37 studies that matched criteria for review with 8/37 including children and 29/37 including adults. RESULTS: HPCI was associated with better speech perception in noise in 18/26 papers and better music perception in 4/5 papers. There was no significant benefit reported in speech perception in quiet (14/20 papers) or binaural cues (3/4 papers), nor was there convincing evidence of HPCI outperforming bimodal users (5/7 papers). QoL scores were high amongst HPCI patients (2/2 papers). Interpretation of findings was hindered by small study groups and significant heterogeneity in various parameters. CONCLUSION: Current literature on the "real-life" benefit of HPCI, although limited, supports the existence of meaningful benefit, especially in speech perception in noise and music perception.

'Real-life' benefit of hearing preservation cochlear implantation in the paediatric population: a single-site case-control study.

INTRODUCTION: Cochlear implantation with hearing preservation (HPCI) has allowed a cochlear implant (CI) electrode to be implanted while trying to preserve residual acoustic low-frequency hearing. The concept arises from the importance of this low-frequency information and the limitations of a CI in several auditory domains. The combination of electrical hearing with either preserved acoustic hearing or amplified 'natural' hearing has the potential to address these issues and enable children with HPCI to closely follow normal auditory development.The aim of this study is to evaluate the 'real-life' benefit of preserved acoustic low-frequency hearing in children with a CI, understand the benefits of preserved natural hearing in complex listening situations and so enable parents and children to make an informed choice about implantation. Ultimately, helping to ensure the maximum number of children benefit from this life-changing intervention. METHODS AND ANALYSIS: Nineteen ears in children and young people aged 6-17 years old with 'successful' HPCI will be subjected to a test battery consisting of: (1) spatial release from masking; (2) complex pitch direction discrimination; (3) melodic identification; (4) perception of prosodic features in speech and (5) threshold equalising noise test. Subjects will be tested in the electro-acoustic stimulation (EAS)/electro-natural stimulation (ENS) and the electric-only (ES) condition, thereby acting as their own control group. Standard demographic and hearing health information will be collected. In the absence of comparable published data to power the study, sample size was determined on pragmatic grounds. Tests are exploratory and for hypothesis-generating purposes. Therefore, the standard criterion of p<0.05 will be used. ETHICS AND DISSEMINATION: This study has been approved by the Health Research Authority and NHS Research Ethics Committee (REC) within the UK (22/EM/0017). Industry funding was secured via a competitive researcher-led grant application process. Trial results will be subject to publication according to the definition of the outcome presented in this protocol.

Children using a unilateral cochlear implant and contralateral hearing aid: bimodal hearing outcomes when one ear is outside the UK (NICE 2009) audiological criteria for cochlear implantation - a single site case-control study.

INTRODUCTION: In the new revised National Institute for Health & Care Excellence (NICE, TA566, 2019) guidelines for cochlear implantation (CI) have clearly stipulated that the hearing loss must be bilateral. Prior to this revision, children and young people (CYP) with asymmetrical thresholds have been considered for unilateral CI when one ear was in audiological criteria. Children with asymmetrical hearing loss represent an important cohort of potential CI candidates, who will continue to be prevented from benefiting from CI unless evidence is produced to support implantation and maximise subsequent benefit.The aim of this study is to evaluate the 'real-life' hearing performance in a group of children who have received a unilateral CI and who have hearing thresholds in the contralateral ear that are outside the current UK NICE 2019 audiological criteria for CI. The contralateral ear will be aided using a conventional hearing aid (HA). The outcomes from this 'bimodal' group will be compared with a group of children who have received bilateral CI, and a group of children using bilateral HA, to extend the current knowledge about the different performance levels between bilateral CI, bilateral HA and bimodal hearing in CYP. METHODS AND ANALYSIS: Thirty CYP aged 6-17 years old, 10 bimodal users, 10 bilateral HA users and 10 bilateral cochlear implant users will be subjected to a test battery consisting of: (1) spatial release from masking, (2) complex pitch direction discrimination, (3) melodic identification, (4) perception of prosodic features in speech and (5) TEN test. Subjects will be tested in their optimal device modality. Standard demographic and hearing health information will be collected. In the absence of comparable published data to power the study, sample size was determined on pragmatic grounds. Tests are exploratory and for hypothesis generating purposes. Therefore, the standard criterion of p<0.05 will be used. ETHICS AND DISSEMINATION: This has been approved by the Health Research Authority and NHS REC within the UK (22/EM/0104). Industry funding was secured via a competitive researcher-led grant application process. Trial results will be subject to publication according to the definition of the outcome presented in this protocol.

The Need for Emergency Laparotomy With Open Abdomen Therapy in the Course of ECMO-A Retrospective Analysis of Course and Outcome.

Background: Abdominal compartment syndrome (ACS) can occur in patients placed on extra corporeal membrane oxygenation (ECMO). This implies the necessity of decompressive laparotomy followed by an open abdomen (OA) to prevent complications such as multi-organ-failure or death. Methods: We searched for ECMO patients in our hospital database between July 2015 and April 2020 and selected those with an emergency laparotomy and OA therapy. Of these, we analyzed only patients who were treated with an OA after establishing the ECMO regarding patient-related parameters like sex, age, height, weight, and indications for ECMO as well as outcome parameters like complete fascial closure rate, mortality, length of stay in intensive care unit (ICU), length and kind of OA therapy, number of surgical procedures, dressing changes concerning negative pressure wound therapy (NPWT), and number of surgical revisions. Results: In eight out of 421 patients (1.9%), a laparostoma had to be created during ECMO support. For temporary closure, either NPWT, abdominal packing, or both were used. The median length of OA therapy was 17 days, and the median length of stay in ICU was 42 days in total. The median number of surgical procedures and NPWT dressing changes was seven. In three of the eight patients, a surgical revision was necessary. The total mortality rate was 50%. In 75%, the fascia could be closed. Two patients died before final closure. In all deceased patients, an abdominal packing was necessary during the course of treatment; in the survivors, only once. No enteroatmospheric fistula or abscesses occurred. Conclusions: ACS in patients placed on ECMO is a very rare condition with a considerable mortality rate but high secondary closure rate of the fascia. A necessary abdominal packing due to a severe bleeding seems to be a risk factor with a potentially fatal outcome.

Cochlear implantation in children with auditory neuropathy: Lessons from Brown-Vialetto-Van Laere syndrome.

OBJECTIVE: Brown-Vialetto-Van Laere syndrome (BVVL) is a rare neurodegenerative disorder associated with auditory neuropathy (AN). The decision process for CI in AN is evolving with increasing evidence of efficacy. We evaluated the benefit of CI in children with BVVL syndrome. METHODS: A retrospective study reviewed the pre- and post-operative hearing outcomes of three patients with BVVL who presented for CI. A fourth patient with BVVL who was not suitable for CI is also discussed. The primary outcomes were hearing thresholds and auditory perception. Outcome measurement instruments included visual reinforcement audiometry (VRA) or Play Audiometry (PA), Categories of Auditory Performance (CAP) and Auditory Speech Sound Evaluation (ASSE). Secondary outcomes were parental report (BAPP questionnaire), the perception of our SaLT and compliance. RESULTS: Patient 1 had ASSE levels of 40-45 dB HL 1 year post-operatively, and CAP score had improved from 2 to 5. At 2-year review, aided thresholds were 40 dB at 2-4 kHz. Three months following CI, the CAP score of Patient 2 had improved from 3 to 5. At 6 months, thresholds were 25-30 dB at 2-4 kHz. Single words/phrases are used by both patients and benefit is reported by both families. Patient 3 has recently undergone CI, having been previously rejected at another centre. Three months following CI, his thresholds were 35-40 dB at 2-4 kHz and increased use of sign and vocalization is reported. CONCLUSION: CI in children with AN complicating BVVL has a variable, but a positive effect. Other manifestations of BVVL make measuring benefit challenging, in the absence of a 'bespoke' measurement instrument for children with complex needs. This study provides further evidence for the benefit of CI in children with AN.

Transnasal adenoidectomy in mucopolysaccharidosis.

BACKGROUND: Mucopolysaccharide (MPS) diseases are a heterogeneous group of inherited, metabolic disorders characterized by accumulation of partially degraded glycosaminoglycans (GAG) in multiple organ systems. Due to accumulation in the airway, patients often present with multilevel airway obstruction and obstructive sleep apnoea (OSA). Adenotonsillar surgery leads to a significant improvement in the severity of OSA in MPS patients. However, access to secure the airway and for conventional surgery can be challenging, due to limited neck extension, macroglossia and reduced mouth opening. This study was undertaken to evaluate the role of transnasal microdebridement and radiofrequent plasma ablation (Coblation) in adenoidectomy to treat OSA in patients with MPS and restricted airway access. METHODS: A retrospective case review was performed including patients with MPS undergoing adenoidectomy for OSA in the period between June 2015 and March 2017. In all cases, either a microdebrider (Gyrus Diablo) or a Coblation wand (EVAC70, Smith&Nephew) was used via a transnasal approach guided by nasendoscopy. The primary outcome was effect upon OSA, measured by sleep oximetry and parental report of benefit. The secondary outcomes were surgical complications and risk factors for persistent OSA after surgery. RESULTS: A total of nine patients were identified with a mean age of 9 years (range 3-14 years) at surgery. Post-operative sleep study data was available for eight patients (8/9). Six patients (6/8) had improvement in 4% oxygen desaturation index (ODI-4) with a mean of 8.11 pre-operatively (range 2.69-14.0) and 4.99 postoperatively (range 0.68-8.48). ODI-4 did not improve in two (2/8) patients. Irrespective of sleep oximetry results, improvement in OSA-related symptoms was noted by all parents postoperatively. No risk factors for persistent OSA were identified. Furthermore, no complications were noted in this cohort. CONCLUSION: Transnasal Coblation and Microdebrider adenoidectomy is a safe and effective surgical treatment for OSA in patients with Mucopolysaccharidosis and adenoidal hypertrophy. As lifespan increases for patients with the Mucopolysaccharidoses, greater emphasis is being given to optimising airway management over the longer-term. This technical note describes the novel application of endoscopic techniques for the management of primary adenoidal hypertrophy when transoral access is restricted, or to debulk recurrent disease that would be challenging to remove via the standard transoral route.

Intrauterine Lipopolysaccharide-Induced Chorioamnionitis in a Sheep: Does It Affect the Auditory System?

BACKGROUND: Fetal exposure to in utero inflammation such as chorioamnionitis is related to central nervous system injury. We hypothesized that chorioamnionitis can provoke inflammatory changes in the perilymph and alter hearing outcome. METHODS: Pregnant ewes were randomized into 2 groups: intrauterine injection with lipopolysaccharide (LPS; n = 19) or saline (n = 21). In the first experiment, fetal perilymph samples were taken for cytokine analysis. In the second experiment, consecutive bone-conducted auditory brain stem responses were obtained from 1 to 7 months after birth. RESULTS: Perilymph samples showed a significant elevation in interleukin 8 in the LPS group. Auditory brain stem response analysis demonstrated higher response thresholds and a prolongation of absolute peak V and interpeak intervals I to V and III to V in the LPS group compared to sham treatment. CONCLUSION: Our study confirms the hypothesis that an intrauterine inflammation by LPS can result in a fetal perilymphatic inflammatory response and functional impaired hearing outcomes after birth in a sheep model.

Screening for and validated quantification of amphetamines and of amphetamine- and piperazine-derived designer drugs in human blood plasma by gas chromatography/mass spectrometry.

The classical stimulants amphetamine, methamphetamine, ethylamphetamine and the amphetamine-derived designer drugs MDA, MDMA ('ecstasy'), MDEA, BDB and MBDB have been widely abused for a relatively long time. In recent years, a number of newer designer drugs have entered the illicit drug market. 4-Methylthioamphetamine (MTA), p-methoxyamphetamine (PMA) and p-methoxymethamphetamine (PMMA) are also derived from amphetamine. Other designer drugs are derived from piperazine, such as benzylpiperazine (BZP), methylenedioxybenzylpiperazine (MDBP), trifluoromethylphenylpiperazine (TFMPP), m-chlorophenylpiperazine (mCPP) and p-methoxyphenylpiperazine (MeOPP). A number of severe or even fatal intoxications involving these newer substances, especially PMA, have been reported. This paper describes a method for screening for and simultaneous quantification of the above-mentioned compounds and the metabolites p-hydroxyamphetamine and p-hydroxymethamphetamine (pholedrine) in human blood plasma. The analytes were analyzed by gas chromatography/mass spectrometry in the selected-ion monitoring mode after mixed-mode solid-phase extraction (HCX) and derivatization with heptafluorobutyric anhydride. The method was fully validated according to international guidelines. It was linear from 5 to 1000 micro g l(-1) for all analytes. Data for accuracy and precision were within required limits with the exception of those for MDBP. The limit of quantification was 5 micro g l(-1) for all analytes. The applicability of the assay was proven by analysis of authentic plasma samples and of a certified reference sample. This procedure should also be suitable for confirmation of immunoassay results positive for amphetamines and/or designer drugs of the ecstasy type.