A New Community-to-Hospital Specialist Otology Pathway Using Telemedicine: A Pilot Study of 50 Patients.

INTRODUCTION: Pathway innovation using smartphone otoscopy and tablet-based audiometry technologies to deliver ear and hearing services via trained audiologists may improve efficiency of the service. An ENT-integrated-community-ear service (ENTICES-combining community audiology management, remote ENT review and novel technologies) was piloted. We aimed to assess the efficiency and safety of ENTICES. METHOD: ENTICES was a community-based and audiologist-led pathway. Patients with otological symptoms were self-referred to this service. Smartphone otoscopy and tablet-based audiograms were performed. Two otologists reviewed all decisions made in the community by audiologists based on video-otoscopy, hearing tests and chart reviews. Data on the first 50 consecutive new patients attending either consultant-led hospital otology clinics (HOC), audiologist-led hospital advanced audiology diagnostics (AAD) or ENTICES clinics were collected between 1 August 2021 and 31 December 2021. Data were collected through chart reviews and questionnaires to compare the three pathways with respect to efficiency, patient satisfaction, technology utility and safety. RESULTS: No audiology-led ENTICES decisions were amended by hospital otologists following remote review. Remote review of video-otoscopy with history was sufficient for a diagnosis in 80% of cases. Adding hearing tests and standardised history increased the diagnostic yield to 98%. Patient satisfaction scores showed 100% service recommendation. The cost per patient, per visit, was £83.36, £99.07 and £69.72 for AAD, HOC or ENTICES, respectively. CONCLUSION: ENTICES provides a safe ear and hearing service that patients rated highly. Thirty-two per cent of hospital otology patients were eligible for this service. For those patients, ENTICES is 20% more cost-effective and can reduce the number of clinic visits by up to 60% compared with HOC.

Do Filling Defects of the Vestibule Represent a Neuroradiological Marker for Congenital Cytomegalovirus? A Retrospective Case Series.

INTRODUCTION: The aim of this study was to investigate whether radiological marker(s) of the inner ear can be detected in congenital cytomegalovirus (cCMV) patients with severe-profound sensorineural hearing loss. METHODS: A retrospective imaging review of confirmed cCMV paediatric patients that had undergone consecutive cochlear implantation was performed at a tertiary hospital. Available pre- and postoperative imaging was examined, and abnormalities of the labyrinth were catalogued by a consultant neuroradiologist in the study group and control group. RESULTS: Twenty-eight paediatric patients with cCMV having undergone cochlear implantation were identified between the ages of 1-15 years (mean 4.7 years) at the time of implantation. Increased density of the vestibule on computed tomography (CT) or filling defects of the vestibule on magnetic resonance imaging (MRI) were identified in 11 and 4 patients, respectively, of the 24 in the case series. No filling defects were identified in any of the 48 CT and MRI control group. CONCLUSION: This study demonstrates a potential novel radiological finding of the inner ear of patients with cCMV. With more research, greater onus placed on MRI and CT for inner ear assessment may facilitate early detection and treatment for patients at risk of significant hearing loss. Further prospective studies in this area will help to validate radiological markers in order to establish a comprehensive inner ear classification system for neuroradiological features in cCMV.

Cochlear Implantation in Elderly Patients: Survival Duration, Hearing Outcomes, Complication Rates, and Cost Utility.

INTRODUCTION: The prevalence of hearing loss and its consequences is increasing as the elderly population grows. As the guidelines for cochlear implantation (CI) expand, the number of elderly CI recipients is also increasing. We report complication rates, survival duration, and audiological outcomes for CI recipients aged 80 years and over and discuss the cost utility of CI in this age group. METHODS: A retrospective cohort study was undertaken of all CI recipients (126 cases), aged 80 years and over at the time of their surgery, implanted at our institution (Cambridge University Hospitals) during a period from January 1, 2001, to March 31, 2019. Data on survival at 1, 3, and 5 years post-implantation, post-operative complications and functional hearing outcomes including audiometric and speech discrimination outcomes (Bamford-Kowal-Bench sentence test) have been reported. RESULTS: The mean age at implantation was 84 years. The mean audiometric score improved from 108 dB HL to 28 dB HL post-implantation. The mean Bamford-Kowal-Bench score improved from 14% to 66% and 73% at 2 and 12 months post-implantation, respectively. The complication rate was 15.3%. The survival probability at 1 year post-implantation was 0.95 for females and 0.93 for males, at 3 years was 0.89 for females and 0.81 for males, and at 5 years was 0.74 for females and 0.54 for males. CONCLUSION: CI is safe and well-tolerated in this age group and elderly patients gain similar audiometric and functional benefit as found for younger age groups.

A systematic review on prevention and management of wound infections from cochlear implantation.

OBJECTIVE OF REVIEW: Surgical site infections are a recognised complication of cochlear implant (CI) surgery with significant morbidity. Our aim was to search for the optimum prevention and management strategy to deal with this issue. TYPE OF REVIEW: Systematic review. SEARCH STRATEGY: A systematic literature search was undertaken from the databases of Embase, CINAHL, MEDLINE® , Web of Science, Scopus and Cochrane Library according to the predefined inclusion and exclusion criteria. EVALUATION METHOD: All relevant titles, abstracts and full-text articles were reviewed by two authors who resolved any differences by discussion and consultation with senior authors. RESULTS: Fourteen articles were included in our review. The overall quality of evidence was low with the vast majority of the studies being retrospective case series and expert opinions. No randomised controlled trials were noted. We found consistent reports that intraoperative prophylactic antibiotics should be given to all patients undergoing CI and that the vast majority of CI wound infections had grown Staphylococcal spp. or Pseudomonas spp. CONCLUSION: Our review has not identified any reliable or reproducible strategies to prevent and deal with wound infections after CI. We strongly encourage further research within this field and would suggest that a consensus of opinions from a multidisciplinary panel of experts may be a pragmatic way forward as an effective guide.

The Impact of Personal Protective Equipment on Speech Discrimination and Verbal Communication in the Operating Room and the Role of Audio Communication Devices.

INTRODUCTION: Recent work has highlighted communication difficulties when wearing personal protective equipment (PPE) in the clinical setting, but currently, there are little objective data on its effects. We assessed the impact of PPE on verbal communication in a simulated operating room and evaluated use of an audio communication device. METHODOLOGY: Frontline health professionals across specialties including surgery, anesthetics, and nursing undertook speech discrimination testing with and without standardized levels of PPE in a simulated operating room environment. Background noise (30- and 70-a-weighted decibel multitalker babble) at 2 distances (2 and 4 m) were selected representative of operating room environments. Bamford-Kowal-Bench (BKB) scoring (192 sentences per participant) was performed. A Digital Multichannel Transceiver System (DMTS) was evaluated. We assessed the effects of PPE use, distance, and use of the DMTS with pairwise comparisons, using a Bonferroni correction, and assessed participant experience via Likert scales. RESULTS: Thirty-one healthcare professionals were tested. Without PPE in 70-a-weighted decibel "babble," median BKB sentence scores were 90% and 76% at 2 and 4 m (adjusted P < 0.0005). The median BKB sentence scores dropped to 8% and 4% at 2 and 4 m in PPE (adjusted P < 0.0005). Improved speech discrimination was achieved with DMTS use to 70% and 76% at 2 and 4 m. Personal protective equipment led to a statistically significant reduction in BKB scores across all conditions compared with baseline. Overall participant confidence in PPE clinical communication was low. CONCLUSIONS: Addition of PPE dramatically impairs speech discrimination and communication in high levels of background noise characteristic of clinical environments, which can be significantly improved using DMTS. Measures should be taken by teams through both through reduction of background noise and consideration of assistive technologies maximizing patient safety. This may be further rehearsed in a simulation environment.

Management and Clinical Outcomes of 37 Patients with Necrotizing Otitis Externa: Retrospective Review of a Standardized 6-Week Treatment Pathway.

BACKGROUND: Necrotizing otitis externa is an invasive infection, affecting older patients, with significant associated morbidity. Despite this, there are no randomized controlled trials that address management, and therefore, treatment approaches may vary considerably. We describe the management and outcomes of 37 patients managed using a multidisciplinary treatment pathway for necrotizing otitis externa over a 5-year period. The pathway is based on a standardized antibiotic regime of 3 weeks of intravenous ceftazidime plus oral ciprofloxacin, followed by a further 3 weeks of ciprofloxacin. METHODS: This is a retrospective review of all patients diagnosed with necrotizing otitis externa since the introduction of our pathway in 2016. We include data on patient demographics, comorbidities, microbiology, length of stay, and length of antimicrobial treatment. Outcome data, including mortality, relapse and treatment failure, and adverse effects of treatment, are presented. RESULTS: The median age of our patients was 82 years. About 54% of patients had diabetes mellitus or another cause of immunocompromise. Pseudomonas aeruginosa was isolated in 68%. The median duration of inpatient stay was 9 days, and median treatment duration was 6 weeks. Of 37 patients, 32 were cured (86%), and of the remaining 5 patients, there were 2 mortalities unrelated to necrotizing otitis externa and 3 patients with recurrent infections due to anatomical abnormalities. CONCLUSION: We note favorable treatment outcomes when using a standardized multidisciplinary pathway and a 6-week course of antibiotic therapy.

Cricopharyngeal dilatation for the long-term treatment of dysphagia in oculopharyngeal muscular dystrophy.

Oculopharyngeal muscular dystrophy (OPMD) is a rare autosomal dominant, progressive degenerative muscle disorder featuring dysphagia with limited therapeutic options. The aim of this study was to evaluate the safety and efficacy of repeated endoscopic dilatation for OPMD over a 15-year period. All patients seen at our Regional Swallowing Clinic with OPMD confirmed by genetic analysis were included. Cricopharyngeal dilatation was performed as an outpatient procedure using a wire-guided 18-mm (54 Fr) Savary-Gilliard bougie with the patient under sedation. Patients were offered repeat endoscopic dilatation when symptoms recurred. Symptom severity prior to initial dilatation and at follow-up was evaluated using the Sydney Swallow Questionnaire (SSQ). Nine patients (7 female, 2 male) were included for analysis. Median total treatment period was 13 years (range = 3-15), median number of dilatations per patient was 7.2 (range = 1-16), and median interval between treatments was 15 months (range = 4.5-45). All patients recorded sustained symptom improvement. Mean SSQ score (out of 1,700) was 1,108.11 (SD ± 272.85) prior to first dilatation and 297.78 (SD ± 189.14) at last follow-up, representing a 73% decrease (95% CI = 52-94) in degree of dysphagia symptoms (paired t-test, P = 0.0001). All mean scores for individual questions also showed significant improvement (P < 0.05). No adverse events were reported with all patients maintaining oral feeding at last follow-up. Repeated cricopharyngeal dilatation is a safe, effective, well-tolerated, and long-lasting treatment for dysphagia in OPMD.

Delivery of remote otology care: a UK pilot feasibility study.

INTRODUCTION: The COVID-19 pandemic has catalysed the need to implement the National Health Service Long-Term Plan to deliver more care in the community and to reduce face-to-face hospital appointments by up to 33%. This study aimed to assess the feasibility of a remote otology service from triage through to delivery. METHODS: New adult otology referrals at a tertiary ear, nose and throat (ENT) hospital aged between 18 and 70 with hearing loss or tinnitus were included. Patients attended an audiology-led community clinic where they underwent a focused history, audiometric testing, and a smartphone-based application and otoscope (Tympa System) was used to capture still and video images of their eardrums. The information was reviewed by ENT clinicians using a remote review platform with a subset of patients subsequently undergoing an in-person review to measure concordance between the two assessments. RESULTS: 58 patients participated. 75% of patients had their pathways shortened by one hospital visit with 65% avoiding any hospital attendances. 24% required an additional face-to-face appointment due to incomplete views of the tympanic membrane or need for additional examinations. Electronic validation by a blinded consultant otologist demonstrated a diagnosis concordance of 95%, and concordance between remote-review and in-person consultations in the 12 patients who agreed to attend for an in-person review was 83.3%. 98% of patients were satisfied with the pathway. CONCLUSION: This pilot service is feasible, safe and non-inferior to the traditional outpatient model in the included patient group. There is potential for the development of a community audiology-led service or use for general practioner advice and guidance.

Cochlear implant outcomes in patients with Meniere's disease: a large case series.

OBJECTIVE: To perform a matched cohort study to assess whether patients with Meniere's Disease (MD) require more intensive auditory rehabilitation following cochlear implantation (CI) and identify factors that may affect outcomes in patients with MD. METHODS: A retrospective case review was performed. MD and control patients were matched for age, biological sex, implant manufacturer and electrode design. Outcomes measured were speech scores, number of visits to audiology department following switch-on, and post-operative active MD. RESULTS: Forty consecutive implanted MD patients were identified between May 1993 and May 2019. Patients with active MD following CI required significantly more visits to the audiology department compared to controls (P < 0.01) and patients who had inactive MD post-operatively (P < 0.01). However, in MD patients, active MD was less likely following CI (P = 0.03). In patients who continued to experience active MD post-operatively, further medical and surgical ablative intervention was required to control ongoing Meniere's attacks. CONCLUSION: We present the largest case series of performance outcomes in CI patients with MD. Although speech outcomes in MD patients are comparable to controls, patients with active MD pre-operatively are more likely to experience variation in CI performance requiring a prolonged period of auditory rehabilitation compared to inactive preoperative MD.

Thromboprophylaxis in trauma: a review of methods, evidence and guidelines.

Venous thromboembolism is a common cause of morbidity and mortality in trauma patients. Mechanical and pharmacological methods of thromboprophylaxis are available and guidelines relating to thromboprophylaxis in trauma include those published by the American College of Chest Physicians, the UK-based National Institute of Clinical Excellence, the Scottish Intercollegiate Guidelines Network and US-based Eastern Association for the Surgery of Trauma. All four guidelines have in common a recommendation for the use of low-molecular-weight heparin except where contraindicated. However, there is little consensus between guidelines relating to which mechanical methods should be used. Given the risk of thromboembolism in trauma patients and increasing interest in this condition clinicians should be aware of local and national guidelines relating to venous thromboembolism prevention in trauma patients. This paper reviews methods of thromboprophylaxis and compares guidelines relating to their use in trauma patients.

Stapes Surgery for 121 Patients With Mixed Hearing Loss.

This study aims to determine the benefit of stapes surgery for otosclerosis in 121 patients with a mixed hearing loss and a preoperative bone conduction (BC) threshold >30 dB. Average postoperative air conduction (AC) improved from 61.5 dB to 34.3 dB. Average air-bone gap closed from 27.1 dB to 6.1 dB. Bone conduction improved from 34.3 dB to 28.2 dB, with 38% of patients achieving a postoperative AC of <30 dB. Glasgow Benefit Inventory scores showed significantly increased quality of life postoperatively in the 88 patients who responded to follow-up, with an average score of 56. There was a mean reduction in daily hearing aid use postsurgery of 5.48 hours, with 56% of patients who responded to follow-up questionnaire no longer needing to use one. When assessing suitability for stapes surgery, surgeons should consider that preoperative BC thresholds may be a poor indicator of the true cochlear reserve and therefore the potential for improvement in AC thresholds and quality of life.

Acute otitis externa: Consensus definition, diagnostic criteria and core outcome set development.

OBJECTIVE: Evidence for the management of acute otitis externa (AOE) is limited, with unclear diagnostic criteria and variably reported outcome measures that may not reflect key stakeholder priorities. We aimed to develop 1) a definition, 2) diagnostic criteria and 3) a core outcome set (COS) for AOE. STUDY DESIGN: COS development according to Core Outcome Measures in Effectiveness Trials (COMET) methodology and parallel consensus selection of diagnostic criteria/definition. SETTING: Stakeholders from the United Kingdom. SUBJECTS AND METHODS: Comprehensive literature review identified candidate items for the COS, definition and diagnostic criteria. Nine individuals with past AOE generated further patient-centred candidate items. Candidate items were rated for importance by patient and professional (ENT doctors, general practitioners, microbiologists, nurses, audiologists) stakeholders in a three-round online Delphi exercise. Consensus items were grouped to form the COS, diagnostic criteria, and definition. RESULTS: Candidate COS items from patients (n = 28) and literature (n = 25) were deduplicated and amalgamated to a final candidate list (n = 46). Patients emphasised quality-of-life and the impact on daily activities/work. Via the Delphi process, stakeholders agreed on 31 candidate items. The final COS covered six outcomes: pain; disease severity; impact on quality-of-life and daily activities; patient satisfaction; treatment-related outcome; and microbiology. 14 candidate diagnostic criteria were identified, 8 reaching inclusion consensus. The final definition for AOE was 'diffuse inflammation of the ear canal skin of less than 6 weeks duration'. CONCLUSION: The development and adoption of a consensus definition, diagnostic criteria and a COS will help to standardise future research in AOE, facilitating meta-analysis. Consulting former patients throughout development highlighted deficiencies in the outcomes adopted previously, in particular concerning the impact of AOE on daily life.

Sudden irreversible hearing loss post COVID-19.

Sudden onset sensorineural hearing loss (SSNHL) is frequently seen by otolaryngologists. The exact pathophysiology of the disease is still unknown, with the most likely causative factor being following a viral infection. Immediate steroids are the best treatment to improve prognosis. Despite a plethora of papers in the literature describing SSNHL, there are only a few reported cases of hearing loss following COVID-19, none of which have been reported in the UK. This paper presents the first UK case of SSNHL following COVID-19. Physical examination and imaging excluded any other cause of hearing loss. A literature review showed that four other cases have been previously described. Hearing loss can be a significant cause of morbidity and can easily be missed in the intensive care setting. Being aware and screening for SSNHL following COVID-19 enables an early course of steroids, which offers the best chance of recovering hearing.

Hearing Preservation Outcomes With Standard Length Electrodes in Adult Cochlear Implantation and the Uptake of Electroacoustic Stimulation.

BACKGROUND: Cochlear implantation with preservation of residual low-frequency hearing enables patients to utilize acoustic and electrical stimulation. It is widely accepted that preservation of residual low-frequency hearing is beneficial in both background noise and for music appreciation. The extent to which patients may benefit is not fully understood, but the importance of these concepts is reflected in electrode design developments and also refinement of surgical technique. Greater understanding is needed around factors that may affect hearing preservation. This study reports experience in adults using standard length cochlear implant arrays. OBJECTIVE: The study reviews hearing preservation outcomes using the HEARRING GROUP method for factors such as gender, electrode type, insertion depth, laterality, preoperative hearing level, and time between surgery and audiogram. Furthermore, the study reviews rates of electroacoustic stimulation use in those with postoperative functional residual low-frequency hearing. METHODOLOGY: Retrospective case series. INCLUSION CRITERIA: preoperative ≤ 85 dB HL at 250 Hz and aged ≥ 18 years. The hearing preservation percentages were calculated using the HEARRING group formula S=[1 - ((PTApost - PTApre)/(PTAmax - PTApre))*100]%. Preservation of > 75% was considered complete, 25 to 75% partial, and 1 to 25% minimal. Standardized operative technique with facial recess approach, posterior tympanotomy, and minimally traumatic round window insertion was performed for each implant. RESULTS: Fifty-three implantations in 52 patients met the inclusion criteria. The mean age at implantation was 55.5 years. The average time since the last audiogram was 10 months. The mean average total pre and postoperative pure-tone averages were 92.4 dB, 99.2 dB, respectively, using minimum reporting standards for adult cochlear Implant (CI). Thirty percent demonstrated complete hearing preservation, 35.8% partial hearing preservation, and 20.8% minimal hearing preservation. Overall, mean hearing preservation was 52.9%. Sex, age at implantation, insertion depth, lateral versus perimodiolar electrode, and preoperative hearing level did not statistically significantly affect rates of hearing preservation in our study. There was a statistically significant deterioration in hearing preservation outcomes difference at 3 months compared with 12 months postoperatively. Only two patients within our study out of 17 with functional postoperative hearing went on to use electroacoustic stimulation. CONCLUSION: Hearing preservation varies between patients and postoperative outcomes are difficult to predict. This study adds to existing literature in terms of likelihood of hearing preservation following cochlear implantation. In turn, this improves our ability to counsel patients as to the chances of preserving residual low-frequency hearing postoperatively and their ability to use electroacoustic stimulation.

Prognostic factors for outcomes of idiopathic sudden sensorineural hearing loss: protocol for the SeaSHeL national prospective cohort study.

INTRODUCTION: The mainstay of treatment for idiopathic sudden sensorineural hearing loss (SSNHL) includes oral steroids, intratympanic steroid injections or a combination of both. The National Institute for Health and Care Excellence, in their recent hearing loss guidelines, highlighted the paucity of evidence assessing the comparative effectiveness of these treatments; and the National Institute for Health Research (NIHR) Health Technology Assessment Programme has since released a commissioned call for a trial to identify the most effective route of administration of steroids as a first-line treatment for idiopathic SSNHL. For such trials to be run effectively, reliable information is needed on patients with SSNHL: where they present, numbers, demographics, treatment pathways, as well as outcomes. This study will collect these data in a nationwide cohort study of patients presenting with SSNHL across 97 National Health Service (NHS) trusts. The study will be delivered through ear, nose and throat (ENT) trainee networks, the NIHR Clinical Research Network (CRN) Audiology Champions and the NIHR CRN. Importantly, this study will also provide a dataset to develop a prognostic model to predict recovery for patients with idiopathic SSNHL. The study objectives are to: (1) map the patient pathway and identify the characteristics of adult patients presenting to NHS ENT and hearing services with SSNHL, (2) develop a prognostic model to predict recovery for patients with idiopathic SSNHL and (3) establish the impact of idiopathic SSNHL on patients' quality of life (QoL). METHODS AND ANALYSIS: Study design: national multicentre prospective cohort study across 97 NHS trusts. INCLUSION CRITERIA: adult patients presenting to NHS ENT and hearing services with SSNHL. OUTCOMES: change in auditory function; change in QoL score. ANALYSIS: multivariable prognostic model, using prespecified candidate predictors. Mean change in QoL scores will be calculated from initial presentation to follow-up. ETHICS AND DISSEMINATION: Health Research Authority and NHS Research Ethics Committee approved the study. Publication will be on behalf of study sites and collaborators. TRIAL REGISTRATION NUMBER: ClinicalTrials.gov Registry (NCT04108598).

Stapes Surgery for Profound Hearing Loss Secondary to Otosclerosis.

The objective of this study is to evaluate stapes surgery in patients with otosclerosis and "profound" hearing loss. This means they meet hearing threshold criteria for cochlear implantation (CI). We performed a retrospective study and patient questionnaire. The results from 33 patients (35 ears) were recorded (mean age: 63.6, range: 40-85). The primary outcome measure was hearing thresholds recorded before and after surgery at 0.5, 1, 2, 3, and 4 kHz. Hearing thresholds at 2 and 4 kHz were also analyzed. Glasgow Benefit Inventory (GBI) was used in 21 patients to assess life quality changes. Hearing thresholds improved in 80% of ears (mean improvement, 26.3 dB), were unchanged in 11.4%, and worsened in 8.6%. Mean GBI score was +20.7. Hearing aid use decreased in 23.8% and ceased in 28.6%. One patient subsequently underwent CI. For patients with profound otosclerosis, stapes surgery provides a quantitative improvement in hearing thresholds and improvement in quality of life, with reduced reliance on hearing aids. This avoids CI, auditory rehabilitation, and a change in quality and tonality of sound.

Hearing Preservation With Standard Length Electrodes in Pediatric Cochlear Implantation.

OBJECTIVE: Preserving low frequencies following cochlear implantation improves outcomes and allows patients to use a combination of electrical and acoustic stimulation. This importance has been reflected in advances in electrode design and refined surgical techniques. Full insertion of standard length electrodes may be advantageous over shortened electrodes because more electrodes can be activated over time if low frequency hearing loss progresses. Surgeons must counsel patients over this choice but data is lacking regarding the degree and likelihood of hearing preservation achievable with standard length electrodes in children. We report our experience using standard length cochlear implant arrays for hearing preservation in children. METHODS: Retrospective case series. INCLUSION CRITERIA: preoperative hearing ≤85 dB HL at 250 Hz and aged ≤18 years. Hearing preservation percentages are calculated using the HEARRING group formula. (Equation is included in full-text article.)Preservation of > 75% was considered complete, 25 to 75% partial, and 1 to 25% minimal. Patients were implanted with either MED-EL FLEX28 or Cochlear Nucleus CI522. Standardized operative technique with facial recess approach, posterior tympanotomy and minimally traumatic round window insertion. RESULTS: Fifty-two implantations in 27 pediatric patients met inclusion criteria. Mean age at implantation: 9.8 years. Average latest audiogram: 8 months. Mean total pre- and postoperative pure-tone averages were 82.8 and 92.6 dB. Seventeen (33%) ears demonstrated complete hearing preservation, 22 (42%) ears partial hearing preservation, 7 (13%) minimal hearing preservation, and 6 (12%) exhibited no acoustic hearing postoperatively. Mean hearing preservation was 55.5%. CONCLUSION: Hearing preservation is achievable to varying degrees in pediatric cochlear implantation using standard length electrodes though it is difficult to predict preoperatively which children may benefit. This study is among the largest additions to the knowledge base for this patient group.

Acute airway obstruction secondary to vocal fold heterotopic ossification.

A 29-year old man of Eritrean origin presented with acute stridor and respiratory distress on a background 1 year history of progressive breathing difficulty and worsening inspiratory stridor. Fibreoptic laryngoscopy revealed an indeterminate swelling of the left vocal fold leaving no clear airway visible. The patient refused surgical tracheostomy. Microlaryngoscopy revealed a hard, calcified mass arising from the left cord preventing intubation. Histological analysis after excision revealed features consistent with heterotopic ossification. At 4 months repeat microlaryngoscopy was performed revealing normal appearance of the larynx and subglottis. Heterotopic ossification in the larynx is a very rare condition that presents a diagnostic and therapeutic challenge. In the first documented case in the larynx, we describe how the disease caused life threatening airway obstruction, but was managed in a way that led to preservation of laryngeal function and complete resolution of the condition.

Head and neck teratomas in children--A series of 23 cases at Great Ormond Street Hospital.

OBJECTIVE: Head and neck teratomas are rare and malignant change is rarer still. This is a report detailing all of the teratomas treated by the ear, nose and throat or craniofacial teams over the last 28 years at our institution. Examining the common presenting and radiological features as well as our success rates compared to the current literature. METHOD: A retrospective review of all cases presenting to our institution with a head and neck teratoma were analysed. Data regarding the following characteristics were collected: antenatal history, clinical features, biological serum makers, radiological and pathological characteristics. Surgical treatment, rates of reoccurrence and the degree of post-operative follow up were also analysed. RESULTS: 23 cases in total were included: 8 cervical, 6 nasopharyngeal, 5 thyroid, 2 thymus, 2 temporal. One had malignant change. The majority of children presented at birth with respiratory distress, 5 cases were picked up antenatally and one case presented at 10 years of age. All were treated surgically; with complete excision in 20 patients. No clinical recurrence occurred but further surgery was performed for radiologically suspected residual disease in one case. DISCUSSION: This is the largest detailed case series in literature in regard to head and neck teratomas. Illustrating that this is frequently a benign disease process disease in the head and neck region and has an excellent long term prognosis following surgery. In the situation of incomplete resection careful meticulous follow up with radiological imaging and a multidisciplinary team approach is a safe and viable alternative.

Primary cervical ganglioneuroblastoma.

OBJECTIVES: Ganglioneuroblastomas represent a histological subgroup of the rare neuroblastic tumours with intermediate malignant potential arising from neural crest progenitor cells of sympathetic nerves. Diagnosis can often be difficult based on imaging alone. We describe 4 cases of children presenting with a solitary neck mass with histology ultimately revealing ganglioneuroblastoma. METHODS: A retrospective case note review was carried out of all patients with cervical ganglioneuroblastoma seen at Great Ormond Street Hospital, UK. RESULTS: Mean age at presentation was 5 years. Based on imaging, the initial diagnoses for three of the cases were: lymphatic malformation, carotid body tumour, paraganglioma, respectively, whilst the remaining case had an immediate incisional biopsy revealing the correct diagnosis. All cases were managed by surgical excision with no evidence of recurrence after a median follow up of 6 years. CONCLUSION: Otolaryngologists should be aware of ganglioneuroblastoma when establishing the differential diagnosis of a child presenting with a neck mass. Biopsy is recommended as the gold standard investigation to avoid an incorrect diagnosis.