Paediatric percutaneous bone anchored hearing aid implant failures: Comparing the experience of a tertiary centre with a systematic review of the literature and meta-analysis.

BACKGROUND: Despite the proven audiological benefits of Percutaneous Bone Anchored Hearing Aids (BAHAs) in paediatric patients with conductive or mixed hearing loss, their adoption has been limited due to concerns over implant failure and associated complications. This paper conducts a systematic review and meta-analysis to assess the prevalence of implant failure in paediatric populations, combined with a case series from our tertiary referral centre. METHODS: A comprehensive literature search identified 562 articles, from which 34 were included in the review, covering 1599 implants in 1285 patients. Our retrospective case series included consecutive patients from our tertiary referral centre who underwent percutaneous BAHA implantation from 2003-2019. RESULTS: Meta-analysis revealed an overall implant failure rate of 11%, predominantly attributed to traumatic extrusion. Our retrospective case series comprised 104 implantations in 76 patients, with a 4.8% failure rate. DISCUSSION: Factors contributing to the lower-than-expected failure rates in the case series likely included consistent use of 4 mm fixtures from a single manufacturer and older age at implantation. The study underscores the need for standardised reporting formats in bone conduction implants research, given the systematic review's limitations in study design heterogeneity, especially with the expected rise in the adoption of novel active devices.

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.

Risk factors associated with the development of chronic suppurative otitis media in children: Systematic review and meta-analysis.

OBJECTIVES: Chronic suppurative otitis media (CSOM) is defined as persistent discharge through a tympanic membrane perforation for greater than 2 weeks. It is associated with a significant disease burden, including hearing loss, and reducing its incidence could significantly improve short- and long-term health. We aimed to identify risk factors associated with the development of CSOM in children. DESIGN AND SETTING: Systematic review and meta-analysis of studies set in community, primary and secondary care settings, identified from Medline, Embase and Cochrane databases from 2000 to 2022. PARTICIPANTS: Children 16 years old and below. MAIN OUTCOME MEASURES: Clinical diagnosis of CSOM. RESULTS: In total, 739 papers were screened, with 12 deemed eligible for inclusion in the systematic review, of which, 10 were included in the meta-analysis. Risk factors examined included perinatal, patient, dietary, environmental and parental factors. Meta-analysis results indicate that atopy (RR = 1.18, 95% CI [1.01-1.37], p = .04, 2 studies); and birth weight <2500 g (RR = 1.79 [1.27-2.50], p < .01, 2 studies) are associated with an increased risk of CSOM development. Factors not associated were male sex (RR = 0.96 [0.82-1.13], p = .62, 8 studies); exposure to passive smoking (RR = 1.27 [0.81-2.01], p = .30, 3 studies); and parental history of otitis media (RR = 1.14 [0.59-2.20], p = .69, 2 studies). CONCLUSION: Optimal management of risk factors associated with CSOM development will help reduce the burden of disease and prevent disease progression or recurrence. The current quality of evidence in the literature is variable and heterogeneous. Future studies should aim to use standardised classification systems to define risk factors to allow meta-analysis.

Cochlear implantation in children with auditory neuropathy spectrum disorder: an updated systematic review.

BACKGROUND: The goal of managing auditory neuropathy spectrum disorder (ANSD) is to restore the children's ability to discriminate auditory information. Children who are not making sufficient progress in speech comprehension, and speech and language development after receiving adequate auditory re/habilitation and/or acoustic amplification may be candidates for cochlear implantation (CI). Despite the growing number of published literature on CI outcomes in children with ANSD, the current evidence is primarily based on case reports or retrospective chart reviews some of which had a limited number of children. In addition, the outcomes of CI seem to vary between children with ANSD. Thus, compelling evidence is lacking. This updated systematic review evaluated the speech perception, language, and speech intelligibility outcomes of children with ANSD post-CI. METHODS: An online bibliographic search was conducted in PubMed, Scopus, Web of Science, and CENTRAL databases. We included both interventional and observational studies that assessed the outcomes of the CI in  children with ANSD. RESULTS: Thirty-three studies were included in this systematic review. Several tests were used to assess speech perception following CI in children with ANSD. The findings of this study revealed that  children with ANSD had mean Categories of Auditory Performance scores ranging from 4.3 to 7 post-operatively, this result was better than the pre-operative scores which ranged between 0.4 to 2.5. Likewise, the Infant-Toddler Meaningful Auditory Integration Scale, Phonetically Balanced Kindergarten, and multisyllabic lexical neighborhood test showed clinically relevant improvement after CI. The same findings were reported for language and speech intelligibility scores. One study investigated the quality of life/children satisfaction after CI and showed overall good satisfaction with the outcomes. CONCLUSIONS: The present systematic review suggests that CI is a feasible and effective hearing  rehabilitation modality for children with ANSD. REGISTRATION AND PROTOCOL: PROSPERO ID: CRD42021279140.

Outcome measures for use in trials of paediatric otorrhoea: A systematic review.

INTRODUCTION: Paediatric otorrhoea (PO) describes a middle ear infection that results in a perforation of the tympanic membrane and ear discharge, in children and young people (CYP). Prolonged infection may be associated with hearing loss and developmental delay. The current management of paediatric otorrhoea is variable, including non-invasive treatments (conservative, oral antibiotics, topical antibiotics) and surgery, reflecting the lack of a sufficiently strong evidence base. Outcome reporting is fundamental to producing reliable and meaningful evidence to inform best practice. OBJECTIVES: Primary objective: to determine which outcome measures are currently used to evaluate treatment success in studies of non-surgical treatments for paediatric otorrhoea. SECONDARY OBJECTIVES: to identify outcome measurement instruments used in the literature and assess their applicability for use in clinical trials of PO. METHODS: This systematic review was registered with PROSPERO (CRD42023407976). Database searches of EMBASE, MEDLINE and Cochrane was performed on June 6, 2023, covering from Jan 1995 to May 2023. Randomised controlled trials or study protocols involving CYP with PO were included following PRISMA guidelines. Risk of bias was assessed with Cochrane's tool. RESULTS: Of the 377 papers identified, six were included in the systematic review. The primary outcome of five of the studies related to otorrhoea cessation; both time to cessation and proportion recovered at various time points were used as measures. Two measurement instruments were identified: Otitis Media-6 Questionnaire and the Institute for Medical Technology Assessment Productivity Cost Questionnaire. Both were shown to be applicable measurement instruments when used in clinical trials of PO. CONCLUSIONS: To promote homogeneity and facilitate meaningful comparison and combination of studies, we propose that time to cessation of otorrhoea from onset of otorrhoea should be used as the primary outcome in future studies. Further research is needed to establish if this is the most important outcome to children and their caregivers.

Protocol for the Paediatric Otorrhoea Study (POSt): a multi-methods study to understand the burden of paediatric otorrhoea in the UK.

INTRODUCTION: Paediatric otorrhoea (PO) refers to the leakage of fluid through a perforation in the ear drum, resulting from an infection of the middle ear of a child or young person (CYP). PO frequently results in hearing loss which may lead to developmental delay, restricted communication and reduced educational attainment.Epidemiological information for PO is largely derived from low-income countries. The aim of this study will be to establish the incidence of PO within the UK and to understand the impact of PO on CYP and their families' everyday lives. It will build the foundations for a randomised controlled trial investigating the best antibiotic treatment for PO. METHODS AND ANALYSIS: The study will consist of two work packages. (1) Data from the Clinical Practice Research Datalink (CPRD), January 2005 to July 2021, will be used to determine the incidence of patient presentations with PO to primary care in the UK. It will also explore the current antimicrobial prescribing practice for PO in primary care. (2) Thirty semi-structured interviews will be conducted from 13 July to 31 October 2023 with CYP and their parents/carers to help identify the impact of PO on everyday life, the patient journey and how service users define treatment success. Three medical professional focus groups will be used to understand the current management practice, how treatment success is measured and acceptability to randomise patients. Thematic analysis will be used. ETHICS AND DISSEMINATION: The Health Research Authority, The Health and Social Care Research Ethics Committee (23/NI/0082) and the CPRD's research data governance panel (22_002508) reviewed this study. Results will be disseminated at medical conferences, in peer-reviewed journals and via social media. The study will cocreate a webpage on healthtalk.org, with the Dipex Charity, about PO to ensure members of the public can learn more about the condition. TRIAL REGISTRATION NUMBER: ISRCTN46071200.

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.

'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.

Listening effort and downstream effects due to hearing loss in children and young people: an online quantitative questionnaire-based observational study.

INTRODUCTION: The clinical application of listening effort (LE) is challenging due to the lack of consensus regarding measuring the concept. Correlational analysis between different measuring instruments shows conditional and weak relationships, indicating they capture different dimensions of LE. Current research has suggested possible links between LE and downstream consequences such as fatigue, stress and confidence. One way to clinically measure LE would be to focus on its corollaries. Further research is needed to explore whether tools used to measure these downstream effects can be applied to capture LE. This study explores using existing questionnaire-based outcome instruments to evaluate LE and its associated consequences in children and young people (CYP), with and without hearing loss. METHODS AND ANALYSIS: One hundred CYP aged 12-17 years with normal hearing and a range of hearing loss levels will be invited to complete a series of online questionnaires (Speech, Spatial and Qualities, Vanderbilt Fatigue Scale-Child, Perceived Stress Scale and Rosenberg Self-Esteem Scale) and a hearing test (Digits in Noise). They will complete the questionnaires at two time points (1) at the end of a rest day and (2) at the end of a workday. Standard demographic and hearing health information will be collected. The sample size was determined pragmatically due to a lack of comparable published data to power the study. Tests are exploratory and for generating hypotheses; therefore, the standard criterion of p<0.05 will be used. ETHICS AND DISSEMINATION: This study has been reviewed within the funding organisation (Cochlear Research and Development Limited) by an independent and relevant peer reviewer/committee. This study has had a favourable ethics committee review by both NHS ethics and University of Manchester ethics. The study will be disseminated through newsletters, publication and presentations at conferences. The results will be made available to participants on request.

Exploring the Correlations Between Measures of Listening Effort in Adults and Children: A Systematic Review with Narrative Synthesis.

Listening effort (LE) describes the cognitive resources needed to process an auditory message. Our understanding of this notion remains in its infancy, hindering our ability to appreciate how it impacts individuals with hearing impairment effectively. Despite the myriad of proposed measurement tools, a validated method remains elusive. This is complicated by the seeming lack of association between tools demonstrated via correlational analyses. This review aims to systematically review the literature relating to the correlational analyses between different measures of LE. Five databases were used- PubMed, Cochrane, EMBASE, PsychINFO, and CINAHL. The quality of the evidence was assessed using the GRADE criteria and risk of bias with ROBINS-I/GRADE tools. Each statistically significant analysis was classified using an approved system for medical correlations. The final analyses included 48 papers, equating to 274 correlational analyses, of which 99 reached statistical significance (36.1%). Within these results, the most prevalent classifications were poor or fair. Moreover, when moderate or very strong correlations were observed, they tended to be dependent on experimental conditions. The quality of evidence was graded as very low. These results show that measures of LE are poorly correlated and supports the multi-dimensional concept of LE. The lack of association may be explained by considering where each measure operates along the effort perception pathway. Moreover, the fragility of significant correlations to specific conditions further diminishes the hope of finding an all-encompassing tool. Therefore, it may be prudent to focus on capturing the consequences of LE rather than the notion itself.

The utility of the exoscope compared to the operating microscope in simulated temporal bone surgery.

OBJECTIVE: To objectively assess the utility of an exoscope during simulated otological surgery. DESIGN: Cohort study. SETTING: Tertiary referral otolaryngology centre. PARTICIPANTS: Seven experienced otologists undertook simulated temporal bone surgery on plastic temporal bones using the Zeiss Kinevo microscope with both a microscope and exoscope facility. OUTCOME MEASURES: The utility of microscope and exoscope was compared using a Likert scale from 1 to 10 with and without PPE. Attributes assessed included image quality, depth perception, adequacy of view, exoscope positioning, surgeon comfort, surgeon safety and adequacy of image and protection for assistants and observers. RESULTS: The exoscope in 3D mode performed as well as or better than the microscope for image quality, field of view and manoeuvrability. It outperformed the microscope for compatibility with PPE, surgeon comfort and assistant/observer experience. It scored almost as highly as the microscope for depth perception. CONCLUSION: There is likely to be a learning curve but this initial assessment of the exoscope shows significant potential as an alternative to the operating microscope in otological surgery but with the advantage of allowing the use of appropriate PPE and better ergonomics for both surgeon and assistant/observer.

Acute paediatric mastoiditis in the UK before and during the COVID-19 pandemic: A national observational study.

OBJECTIVES: To explore the impact of COVID-19 on the management and outcomes of acute paediatric mastoiditis across the UK. DESIGN: National retrospective and prospective audit. SETTING: 48 UK secondary care ENT departments. PARTICIPANTS: Consecutive children aged 18 years or under, referred to ENT with a clinical diagnosis of mastoiditis. MAIN OUTCOME MEASURES: Cases were divided into Period 1 (01/11/19-15/03/20), before the UK population were instructed to reduce social contact, and Period 2 (16/03/20-30/04/21), following this. Periods 1 and 2 were compared for population variables, management and outcomes. Secondary analyses compared outcomes by primary treatment (medical/needle aspiration/surgical). RESULTS: 286 cases met criteria (median 4 per site, range 0-24). 9.4 cases were recorded per week in period 1 versus 2.0 in period 2, with no winter increase in cases in December 2020-Febraury 2021. Patient age differed between periods 1 and 2 (3.2 vs 4.7 years respectively, p < 0.001). 85% of children in period 2 were tested for COVID-19 with a single positive test. In period, 2 cases associated with P. aeruginosa significantly increased. 48.6% of children were scanned in period 1 vs 41.1% in period 2. Surgical management was used more frequently in period 1 (43.0% vs 24.3%, p = 0.001). Treatment success was high, with failure of initial management in 6.3%, and 30-day re-admission for recurrence in 2.1%. The adverse event rate (15.7% overall) did not vary by treatment modality or between periods 1& 2. CONCLUSION: The COVID-19 pandemic led to a significant change in the presentation and case mix of acute paediatric mastoiditis in the UK.

Characterization of tracheobronchomalacia in infants with hypophosphatasia.

BACKGROUND: Perinatal and infantile hypophosphatasia (HPP) are associated with respiratory failure and respiratory complications. Effective management of such complications is of key clinical importance. In some infants with HPP, severe tracheobronchomalacia (TBM) contributes to respiratory difficulties. The objective of this study is to characterize the clinical features, investigations and management in these patients. METHODS: We report a case series of five infants with perinatal HPP, with confirmed TBM, who were treated with asfotase alfa and observed for 3-7 years. Additionally, we reviewed respiratory function data in a subgroup of patients with perinatal and infantile HPP included in the clinical trials of asfotase alfa, who required high-pressure respiratory support (positive end-expiratory pressure [PEEP] ≥6 cm H2O and/or peak inspiratory pressure ≥18 cm H2O) during the studies. RESULTS: The case series showed that TBM contributed significantly to respiratory morbidity, and prolonged respiratory support with high PEEP was required. However, TBM improved over time, allowing weaning of all patients from ventilator use. The review of clinical trial data included 20 patients and found a high degree of heterogeneity in PEEP requirements across the cohort; median PEEP was 8 cm H2O at any time and some patients presented with high PEEP (≥8 cm H2O) over periods of more than 6 months. CONCLUSION: In infants with HPP presenting with persistent respiratory complications, it is important to screen for TBM and initiate appropriate respiratory support and treatment with asfotase alfa at an early stage. TRIAL REGISTRATION: ClinicalTrials.gov numbers: NCT00744042 , registered 27 August 2008; NCT01205152 , registered 17 September 2010; NCT01176266 , registered 29 July 2010.

Infant Alveolar Macrophages Are Unable to Effectively Contain Mycobacterium tuberculosis.

Infants are more likely to develop lethal disseminated forms of tuberculosis compared with older children and adults. The reasons for this are currently unknown. In this study we test the hypothesis that antimycobacterial function is impaired in infant alveolar macrophages (AMϕs) compared with those of adults. We develop a method of obtaining AMϕs from healthy infants using rigid bronchoscopy and incubate the AMϕs with live virulent Mycobacterium tuberculosis (Mtb). Infant AMϕs are less able to restrict Mtb replication compared with adult AMϕs, despite having similar phagocytic capacity and immunophenotype. RNA-Seq showed that infant AMϕs exhibit lower expression of genes involved in mycobactericidal activity and IFNγ-induction pathways. Infant AMϕs also exhibit lower expression of genes encoding mononuclear cell chemokines such as CXCL9. Our data indicates that failure of AMϕs to contain Mtb and recruit additional mononuclear cells to the site of infection helps to explain the more fulminant course of tuberculosis in early life.

Cochlear implantation in adolescents: Factors influencing compliance.

OBJECTIVES: To quantify rates of non- and partial-use of cochlear implants (CIs) in adolescent patients implanted in adolescence and childhood and identify factors influencing compliance. METHODS: A retrospective case note review undertaken at The Manchester Auditory Implant Centre. Adolescents were defined as young people aged 11-18 years. Individuals implanted in adolescence were defined as Group 1, individuals implanted in childhood under the age of 3 years and currently adolescents were defined as Group 2 and individuals implanted between the age of 3 and11 years and currently adolescents were defined as Group 3. Non-use was defined as not using the CI at all and partial use was defined as consistently using the CI less than full-time, or fluctuating periods of full and less than full-time use. RESULTS: In Group 1 there was 1 non-user (1.3%) and 11 partial-users (13.9%), with an overall non-compliance rate of 15.2%. In Group 2 there was one non-user (1.9%) and one partial-user (1.9%) with an overall non-compliance rate of 3.8%. In Group 3 there were no non-users and eight partial-users (9%), with an overall non-compliance rate of 9%. The factors influencing compliance differed between groups with the most common factor in Group 1 being a preference for the auditory input gained from the contralateral hearing aid (50%). In Groups 2 and 3 the main factors influencing compliance were behavioural and related to wearing the implant only at school (50 and 75%, respectively). CONCLUSIONS: Patients implanted during adolescence have higher rates of non- and partial-use compared with their adolescent counterparts who have been implanted during childhood. It is important to investigate factors influencing non-compliance so appropriate support may be provided to the patient and their family.

Progress of hearing loss in neurofibromatosis type 2: implications for future management.

The objective of this study was to describe changes in hearing over time in patients with neurofibromatosis type 2 (NF2) treated conservatively. A retrospective case review was conducted in a tertiary referral centre. Pure tone audiometry, speech discrimination scores, serviceable hearing (American Academy of Otolaryngology class A or B) and measurement of vestibular schwannoma (VS) size on magnetic resonance imaging were evaluated in 56 patients (89 ears) with NF2 with at least one conservatively managed VS. Over a mean follow-up period of 7 years (range 0.8-21 years) pure tone average thresholds increased gradually with a mean annual rate of 1.3 dB for the right ear (p = 0.0003) and 2 dB for the left ear (p = 0.0009). Speech discrimination scores dropped with an average annual rate of 1.3 and 0.34% in the right and left ear, respectively. Patients maintained serviceable hearing for an average of 7.6 years (range 2.7-19.3 years). The average annual VS growth was 0.4 mm without any correlation with hearing loss. There was a correlation between patients' age and pure tone threshold increase (p < 0.05 for both ears). In this selected population of patients with NF2, hearing threshold increases were very slow. In NF2 patients with indolently behaving tumours, serviceable hearing can be maintained for a significant length of time, making conservative management an attractive option.

Tracheostomy in mucopolysaccharidosis type II (Hunter's Syndrome).

OBJECTIVE: Patients with mucopolysaccharidosis type II (MPS II) may develop progressive multi-level upper airway obstruction. Despite the unique challenges presented by these complex patients, tracheostomy remains an important intervention to safeguard the airway when other interventions have failed or when the airway obstruction involves multiple sites. Airway involvement is largely responsible for the significant anaesthetic risk seen in MPS II. We reviewed our tertiary unit's experience of tracheostomies in patients with MPS II. STUDY DESIGN: Retrospective study. METHODS: Case note review of MPS II patients requiring tracheostomy at our tertiary institution. The primary outcome measure used for this study was complications following tracheostomy. RESULTS: We identified 10 MPS II patients requiring tracheostomy to manage upper airway obstruction. Mean age at which tracheostomy was 11 years 2 months (range 4 years 6 months to 28 years 10 months). Tracheostomy insertion was indicated in 3 scenarios: (1) to safeguard an anticipated difficult airway prior to a planned non-ENT surgical procedure, (2) to treat refractory progressive upper airway obstruction and (3) emergency airway management. Complications recorded included infratip and suprastomal granulations, local wound infection and skin ulceration from mechanical trauma. There were no immediate postoperative complications. CONCLUSIONS: Progressive upper airway obstruction is common in children with MPS II. Tracheostomy is an effective way of managing airway obstruction when less invasive interventions are no longer adequate. Tracheostomy in these patients can be technically difficult and although the complications of tracheostomy in MPS II do not significantly differ from other patient groups, the implications and management complexity vary considerably. The impact of ERT on airway obstruction is not yet fully understood, with tracheostomies likely to remain an important airway adjunct in some patients who fail to respond to ERT, or in those patients surviving into adulthood. It is vital that a multidisciplinary team, comprising clinicians with experience in managing such patients, are involved in airway management of patients with MPS II to enable the best standard of care to be given. The significant additional implications of a tracheostomy in a patient with MPS II, in terms of safety, aftercare and potentially life-threatening complications must be discussed in detail with the patient's family and/or carers. LEVEL OF EVIDENCE: 2c.

Cochlear implantation in patients deafened by ototoxic drugs.

OBJECTIVE: To investigate the outcome of cochlear implantation (CI) in patients deafened by ototoxic drugs and to compare this, with the outcome of CI in sudden sensorineural hearing loss (SSNHL) with a similar duration of deafness. METHODS: The Manchester Auditory Implant Centre database was reviewed to identify patients who were implanted to rehabilitate profound sensorineural hearing loss resulting from treatment with ototoxic agents and patients with SSNHL group. A retrospective case note review of selected patients was carried out. Primary outcome measure was post-implantation Bamford-Kowal-Bench (BKB) score in quiet in both the groups. Secondary outcome measure was any significant complications following implantation. RESULTS: We identified 14 patients in the ototoxic group, which were matched with 13 patients in the SSNHL group. The post-operative BKB score in the ototoxic group ranged from 33 to 100% (median score 91%). One patient had bilateral CI. One patient required explantation following an infection. The post-operative BKB score in the SSNHL group ranged from 16 to 100% (median score 88%). One patient in this group could not be tested using this method as they did not have open set speech discrimination. Two patients in this group had bilateral CI. The data were analysed using Mann-Whitney U test. There was no statistically significant difference in the BKB scores in the two groups of patients (P value -0.983). CONCLUSION: Patients with profound hearing loss secondary to ototoxic agents can be rehabilitated successfully with CI. The outcomes may be variable and may be dependent on the underlying pathology for which the ototoxic agents were prescribed.

Cochlear implantation in patients profoundly deafened after head injury.

OBJECTIVE: There is very little information in the literature regarding outcomes of cochlear implantation in patients profoundly deafened after head injury. The aim of this study was to assess outcomes in this group of patients. STUDY DESIGN: Retrospective case review. SETTING: The Manchester Cochlear Implant Programme, University of Manchester. PATIENTS: Profoundly deafened patients after head injury who proceeded to cochlear implantation for auditory rehabilitation. MAIN OUTCOME MEASURES: Mean age and duration of deafness at implantation. Preimplantation and postimplantation speech perception outcomes were measured using Bench Kowel Bamford (BKB) sentences in quiet and noise, City University of New York sentences with lip reading and Arthur Boothroyd words scoring the percentage phonemes correct. RESULTS: Twenty patients received 23 cochlear implants. Mean age at implantation was 51 years (standard deviation, 12 yr). Mean duration of deafness at implantation was 12 years (range, 1-30 yr).Preimplantation BKB score in quiet of 0%. Mean postimplantation BKB score in quiet was 64% (range, 0%-100%) and in noise was 61% (range, 0%-97%). Three were nonusers, and 1 required reimplantation. There was a moderately negative correlation between outcome and age at implantation (r = -0.41, p < 0.05) and between outcome and duration of deafness (r = -0.52, p < 0.05). CONCLUSION: Cochlear implantation is an effective method for hearing rehabilitation in profoundly deafened patients after head injury. However, negative factors, such as significant injury to the central auditory pathway, basal turn obliteration, long duration of deafness, and nonauditory stimulation, should be considered in the preoperative assessment of these patients.

Cochlear implantation after bacterial meningitis in children: outcomes in ossified and nonossified cochleas.

OBJECTIVE: To investigate the outcome of cochlear implantation in children with postmeningitic deafness and to compare the outcomes in ossified and nonossified cochleas. STUDY DESIGN: A retrospective case note review of children with postmeningitic deafness requiring cochlear implantation. SETTING: Manchester Auditory Implant Centre--a tertiary referral center. PATIENTS: Children younger than 16 years with postmeningitic deafness. INTERVENTION: Cochlear implantation to aid auditory habilitation. MAIN OUTCOME MEASURE: Outcomes were measured using Categories of Auditory Performance Score and the Manchester Speech and Language Development Scale. RESULTS: Fifty-two children underwent cochlear implantation. Patients were allocated to 2 groups--Group 1 with nonossified cochleas and Group 2 with cochlear ossification. Group 1 comprised 25 children, of whom, 22 had Categories of Auditory Performance Score of 5 or higher. Nineteen of them were able to use simple phrases of 3 words or more on the Manchester Speech and Language Development Scale. Group 2 comprised 27 children. Fourteen had partial ossification with complete standard electrode insertion in all instances. Of the 13 children with gross ossification, 3 had scala vestibuli insertion, 7 had split electrode insertion and 3 had partial electrode insertion. In this group, 20 children had Categories of Auditory Performance Score of 5 or higher. Nineteen children were able to use simple phrases of 3 words or more on Manchester Speech and Language Development Scale. CONCLUSION: Children with postmeningitic deafness benefit significantly from cochlear implantation. However, the audiologic outcomes are difficult to predict, especially in the presence of cochlear ossification. Acquisition of speech and language in these children after cochlear implantation is possible even in the presence of gross cochlear ossification.