Long-term Airway Outcomes and Interventions in Children With Oesophageal Atresia With Tracheoesophageal Fistula: A 20-year Single Centre Observational Study.

BACKGROUND: Airway anomalies, symptoms and interventions are commonly reported in children with oesophageal atresia with tracheoesophageal fistula (OA/TOF). The purpose of this study was to assess the incidence of these airway pathologies and those requiring interventions in the long-term. METHODS: A retrospective case note review of all patients admitted to the Neonatal Unit at the Royal Hospital for Children, Glasgow between January 2000 and December 2015 diagnosed with OA/TOF. Included patients had a minimum of 5 years follow-up. RESULTS: 121 patients were identified. 118 proceeded to OA/TOF repair. 115 patients had long-term follow-up data. Ninety-five (83%) children had one or more airway symptom recorded. Thirty-six (31%) neonates underwent airway endoscopy at the time of their initial OA/TOF repair. Forty-six (40%) children underwent airway endoscopy at a later date due to airway symptoms. Airway pathologies identified included airway malacia, thirty-two (28%), subglottic stenosis, eleven (10%), tracheal pouch, twenty-five (22%), laryngeal cleft, seven (6%) and recurrent fistula, five (4%). Airway interventions included endoscopic division of tracheal pouch, ten (9%), tracheostomy, seven (6%), aortopexy, six (5%), repair of recurrent fistula, five (4%), endoscopic repair of laryngeal cleft, three (3%) and four (3%) required open airway reconstruction for subglottic stenosis. One child (1%) remains tracheostomy dependent. CONCLUSIONS: Long-term airway pathologies are common in children with OA/TOF. Many of these are remediable with surgical intervention. Clinicians should be cognisant of this and refer to Airway Services appropriately.

The impact of intranasal corticosteroids in a prospective cohort of children with sleep disordered breathing.

INTRODUCTION: Sleep disordered breathing (SDB) is common in children and the most common reason for adenotonsillectomy. This large observational cohort study from a specialist outpatient clinic describes the impact of intranasal steroids (INS) on symptom improvement and the need for surgery. METHOD: Observational cohort study of 568 children assessing the impact of INS using the OSA-5 questionnaire with clinical and surgical outcome measures. RESULTS: The mean OSA-5 score at first visit was 7.78. Symptoms were persistent for a median 9 months (range 2-72). 51% underwent a trial of INS with 56% reporting symptomatic improvement. The mean score decreased from 8.2 to 5.5 (p < 0.0001) in those prescribed INS. They had a significantly higher symptom load (p < 0.01), turbinate size (p < 0.005) and history of atopy (p < 0.01) than the non-trial group. The rate of surgery in the non-trial group was 56% compared with 38% in those who had INS (p < 0.001). With increasing symptom burden, the reported improvement with INS and comparative reduction in surgery increased. Baseline OSA-5 scores were predictive of rates of surgery. Atopic status or age did not influence response to INS. CONCLUSION: The mean score at first visit and the median duration of symptoms indicated significant persistent symptoms in this cohort. The use of INS improved symptoms of SDB in 56%. The need for surgery in the group that received INS was 38% compared with 56% in those not trialling INS, despite the non-trial group having significantly less symptoms and signs. Symptomatic improvement was not influenced by age or atopic status.

Spontaneous abscess of the nasal septum in children: a 10-year series.

BACKGROUND: This paper reports a 10-year series of spontaneous nasal septal abscesses in immune-competent children, with suggestions for optimal management. METHODS: A retrospective case note review was conducted of children undergoing an operation for incision and drainage of nasal septal abscesses between 2013 and 2023. RESULTS: Six children were identified via electronic hospital records during the 10-year review period, five with a spontaneous abscess. The children were aged 10-14 years. All were immunocompetent and none had active sinus infection. The most common presenting features were nasal swelling, facial swelling, headache, nasal congestion and fever. The most common bacterial isolate was Staphylococcus aureus. All children received prompt surgical drainage and intravenous antibiotic therapy. Complications were seen in three children, with one child developing significant intracranial complications. CONCLUSION: To our knowledge, this is the first series of spontaneous nasal septal abscesses in immunocompetent children. The high prevalence of Staphylococcus aureus suggests spread from the nasal mucosa or vestibule. Early recognition, computed tomography scanning, surgical drainage and antibiotic therapy are the mainstays of treatment, to prevent potentially life-threatening complications.

What is the annual risk of infection in congenital midline neck cysts in children? Thyroglossal duct cysts versus dermoid cysts.

INTRODUCTION: Midline neck lumps in children are mostly found to be thyroglossal duct cysts or dermoid cysts. Thyroglossal duct cysts often have an associated sinus tract which may connect all the way to the foramen caecum on the tongue, while dermoids have no such connection. This study aims to estimate the annual infection risk for midline neck cysts based on our patient series, and to see if this differs between thyroglossal duct cysts and dermoid cysts. METHODS: All children seen at the Royal Hospital for Children, Glasgow who underwent surgical excision of a midline neck cyst between 1st January 2017 and 31st December 2021 were identified. In those whose cyst had been infected prior to surgical excision, the age at which the first episode of infection occurred was recorded and used to calculate a survival curve. RESULTS: We identified 53 children (29 male, 24 female) aged 1-16 years (median 4) at the time of surgical excision. There were 26 thyroglossal and 24 dermoid cysts, plus 2 with indeterminate histology and 1 lymph node. Of the 24 dermoids, 4 suffered infection prior to surgery (17%), and 2 of these recurred after surgery (8%). Of the 26 thyroglossal cysts, 16 suffered infection prior to surgery (62%) and 5 of these recurred (19%). 78% of thyroglossal and dermoid cysts had at least 1 episode of infection by age 10 years. DISCUSSION: In a child with a congenital midline neck cyst that has never been infected, deferring surgery for a year comes with a 7.8% risk that the cyst will get infected.

Managing recurrent nosebleeds in children: a retrospective review of 718 children attending a nurse-led epistaxis clinic.

OBJECTIVE: This review assessed the effectiveness of the nurse-led children's epistaxis clinic in streamlining patient care and avoiding unnecessary general anaesthesia. METHODS: A retrospective case note review was conducted of children attending the nurse-led epistaxis clinic between 2019 and 2021. RESULTS: A total of 718 children were seen over three years. Twelve (1.7 per cent) had a known coagulopathy. Of the children, 590 (82 per cent) had visible vessels and 29 (4 per cent) had mucosal crusting. Silver nitrate cautery was attempted under topical anaesthesia in 481 children, with 463 (96 per cent) successful cauterisations. Fifteen (3 per cent) were cauterised under general anaesthesia. Of the children, 706 (99 per cent) were prescribed nasal antiseptic preparations; this was the sole treatment for 58 (8 per cent). Blood investigations were requested for eight children (1 per cent) and haematology referral for three (0.4 per cent). CONCLUSION: This is the largest published series of children's nosebleeds. Given the short-lived benefit from cautery, it is suggested that general anaesthesia should not be offered routinely. However, improved haematology referral criteria are required to increase underlying diagnosis.

Retrospective observational study analysing the trends in ventilation tube insertion in children in Scotland between 2001 and 2018.

OBJECTIVES: Otitis media with effusion (OME) is common, affecting up to 90% of children. Around 25% will have a persistent effusion with conductive hearing loss which can impact their development. Ventilation tubes (VTs) can improve their hearing in the short term. This study aims to analyse the trends in VT insertion rates across Scotland. DESIGN: Retrospective observational study. SETTING: All mainland Scottish health boards. PARTICIPANTS: All children aged 0-16 who underwent a VT insertion procedure from 2001 to 2018 were included. MAIN OUTCOME MEASURES: Data were provided by the Scottish Public Health Observatory, using Scottish Morbidity Records. Mid-year population estimates were obtained from the National Records Office of Scotland. Socioeconomic deprivation was estimated based on area of residence using the Scottish Index of Multiple Deprivation. VT insertion rates were calculated and trends analysed. RESULTS: A total of 35 878 VT procedures were performed in total with a mean rate of 2.02 per 1000 children per year. The highest insertion rates were observed in children aged 4-6. VT insertion rates reduced during the study period (R = -0.729, p = .001). Variability in VT insertion rates between health boards reduced. There was a significant association between socioeconomic deprivation and VT insertion rate, with the most deprived children having the highest rate (p < .001). CONCLUSIONS: VT insertion rates and the variability between Scottish health boards have reduced over the past two decades, suggesting a more equitable system. Our data intimates that the decision to perform VT insertion is based upon disease prevalence rather than clinician preference.

Prevalence of thyroid gland tissue in midline neck dermoid cysts in children and a proposed new 'thyroglossal entrainment' hypothesis for their formation.

BACKGROUND: Thyroglossal duct cysts and dermoid cysts both commonly present as midline neck lumps in children. They are treated as separate entities with different embryological origins. There are isolated reports of thyroid gland tissue in a dermoid cyst, concurrent thyroglossal and dermoid cysts, and cysts with mixed histology. It is not known if these are rare or common. METHODS: All children undergoing excision of a congenital midline neck cyst between January 2017 and December 2022 were identified. Histopathology slides were reviewed in detail. RESULTS: In 53 children, there were 26 thyroglossal duct cysts, 24 dermoids, 1 lymph node and 2 with no diagnostic material identified. Five dermoids (28 per cent) had associated thyroid gland tissue, and 1 (4 per cent) had hybrid histology with keratinising and respiratory epithelium. Infection occurred in 17 per cent of dermoids prior to excision and 8 per cent of dermoids recurred after excision. CONCLUSION: Hybrid histology, infection and recurrence are all common in midline neck dermoids. A new theory for their embryological origin is proposed, with the suggestion that some may need more extensive surgery.

International pediatric otolaryngology group (IPOG) consensus on approach to aspiration.

OBJECTIVE: To provide recommendations for a comprehensive management approach for infants and children presenting with symptoms or signs of aspiration. METHODS: Three rounds of surveys were sent to authors from 23 institutions worldwide. The threshold for the critical level of agreement among respondents was set at 80 %. To develop the definition of "intractable aspiration," each author was first asked to define the condition. Second, each author was asked to complete a 5-point Likert scale to specify the level of agreement with the definition derived in the first step. RESULTS: Recommendations by the authors regarding the clinical presentation, diagnostic considerations, and medical and surgical management options for aspiration in children. CONCLUSION: Approach to pediatric aspiration is best achieved by implementing a multidisciplinary approach with a comprehensive investigation strategy and different treatment options.

External peri-stomal skin granulations in paediatric tracheostomy: Incidence, outcomes and a proposed treatment algorithm.

BACKGROUND: External peri-stomal skin granulations after tracheostomy in children are common and may interfere with routine tube changes. This study is the first attempt to describe the incidence and outcomes, along with a proposed treatment algorithm. METHODS: A retrospective review of all inpatient children with a tracheostomy between January 2020 and May 2022 at the Royal Hospital for Children (RHC) in Glasgow. The presence of external peri-stomal granulation, date of onset and resolution, recurrence and treatment modalities were noted. All tracheostomy tubes used during the study period were made of silicone. RESULTS: A total of 50 episodes of peri-stomal granulation were identified in 27 children (52 %). Median age at the end of the study period was 4.3 years, with younger children experiencing more frequent granulation. 3 episodes interfered with tracheostomy tube changes. Time to resolution of the granulation was significantly longer with topical steroid/antimicrobial ointment monotherapy, but recurrence was less common when this was used a first treatment modality. CONCLUSION: Non-invasive measures such as topical anti-microbials should be used in the first instance when managing external stoma-site granulations. More invasive measures, such as silver nitrate cautery and surgical excision, should be considered if the granulation tissue is not improving or when it poses a risk to safe tube changes.

Comprehensive management of anterior drooling: An International Pediatric Otolaryngology Group (IPOG) consensus statement.

OBJECTIVE: To provide guidance for the comprehensive management of children referred for anterior drooling. The mission of the International Pediatric Otolaryngology Group (IPOG) is to develop expertise-based recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. METHODS: Survey of expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The recommendations are derived from current expert consensus and critical review of the literature. RESULTS: Consensus recommendations include initial care and approach recommendations for health care providers who commonly evaluate children with drooling. This includes evaluation and treatment considerations for commonly debated issues in drooling management, initial work-up of children referred for anterior drooling, treatment recommendations, indications and contra-indications for rehabilitation, medical, and surgical management, as well as pros and cons of different surgical procedures in the hands of drooling management experts. CONCLUSION: Anterior drooling consensus recommendations are aimed at improving patient-centered care in children referred for sialorrhea.

How common are ear, nose and throat disorders in children with Noonan syndrome and other RASopathies?

INTRODUCTION: Noonan syndrome and related conditions (RASopathies) are known to be associated with abnormalities in many organ systems. It is our impression that few otolaryngologists are familiar with the manifestations of these syndromes and we therefore reviewed our hospital's patient cohort to identify the prevalence of ear, nose and throat disorders in these children. METHODS: We cross-referenced various hospital department databases (otolaryngology, audiology, cardiology, haematology and genetics) to try to identify as many children with Noonan and other RASopathies as possible. We then performed a retrospective review of electronic patient records. RESULTS: We identified 67 children with Noonan, Costello, LEOPARD and other RASopathy syndromes. Around half have been seen in otolaryngology and audiology clinics. Otitis media with effusion requiring ventilation tubes occurred in 4% of children. 10% have suffered recurrent acute otitis media. 9% have a sensorineural hearing loss. 7% have undergone adenotonsillectomy for obstructive sleep apnoea. Airway anomalies and head and neck malformations occur but are rare. DISCUSSION: Children with Noonan and other RASopathies present commonly to otolaryngology and audiology clinics. The prevalence of sensorineural hearing loss is high and audiological screening is likely to be worthwhile. Surgeons should be aware that complications of surgery are common and can be very severe, especially in those with cardiac anomalies.

Effectiveness of Intranasal Mometasone Furoate vs Saline for Sleep-Disordered Breathing in Children: A Randomized Clinical Trial.

Importance: Obstructive sleep-disordered breathing (SDB) in children is characterized by snoring and difficulty breathing during sleep. SDB affects at least 12% of otherwise healthy children and is associated with significant morbidity. Evidence from small clinical trials suggests that intranasal corticosteroids improve SDB as measured by polysomnography; however, the effect on symptoms and quality of life is unclear. Objective: To determine whether intranasal mometasone furoate is more effective than intranasal saline for improving symptoms and quality of life in children with SDB. Design, Setting, and Participants: The MIST trial was a multicenter, randomized, double-blind, placebo-controlled trial, recruiting participants from June 8, 2018, to February 13, 2020. Children aged 3 to 12 years who were referred to a specialist for significant SDB symptoms were included; exclusions were previous adenotonsillectomy, body mass index greater than the 97th percentile, and severe SDB. Randomization was stratified by site, and data were analyzed on an intention-to-treat basis from October 28, 2020, to September 25, 2022. Interventions: Participants were randomly assigned to receive mometasone furoate, 50 µg, or sodium chloride (saline), 0.9%, 1 spray per nostril daily, dispensed in identical bottles. Main Outcomes and Measures: The primary outcome was resolution of significant SDB symptoms (ie, reduction to a level no longer requiring referral to a specialist as per the American Academy of Pediatrics guidelines) at 6 weeks, measured by parental report of symptoms using the SDB Score. Results: A total of 276 participants (mean [SD] age, 6.1 [2.3] years; 146 male individuals [53%]) were recruited, 138 in each treatment arm. Resolution of significant SDB symptoms occurred in 56 of 127 participants (44%) in the mometasone group and 50 of 123 participants (41%) in the saline group (risk difference, 4%; 95% CI, -8% to 16%; P = .51) with 26 participants lost to follow-up and missing values managed by multiple imputation. The main adverse effects were epistaxis, affecting 12 of 124 participants (9.7%) in the mometasone group and 18 of 120 participants (15%) in the saline group, and nasal itch/irritation, affecting 12 of 124 participants (9.7%) in the mometasone group and 22 of 120 participants (18%) in the saline group. Conclusions and Relevance: Results of this randomized clinical trial suggest that there was no difference in treatment effect between intranasal mometasone and saline for the management of SDB symptoms. The results suggest that almost one-half of children with SDB could be initially managed in the primary care setting and may not require referral to specialist services, as is currently recommended. Trial Registration: Australian New Zealand Clinical Trials Registry: ANZCTRN12618000448246.

Measuring benefit from non-surgical interventions in otolaryngology for different conditions, using the revised 5-factor Glasgow Benefit Inventory.

OBJECTIVES: The Glasgow Benefit Inventory (GBI) has been extensively used to report the benefit from otolaryngological surgery. Benefit from non-surgical management has not been reported, despite this being the outcome of most otolaryngology and audiology consultations. DESIGN: GBI responses from 4543 adults from the Scottish ENT Outcome Study were categorised by diagnosis. Benefit scores for different interventions within diagnostic categories for which surgery was not a potential management are reported using the revised 5-factor Glasgow Benefit Inventory (GBI-5F; 15 questions and 5 factors). SETTING: Adult otolaryngology outpatient clinics in six university hospitals. PARTICIPANTS: Adults seen with conditions that had no surgical option and given non-surgical management. RESULTS: Overall, 80% of participants managed in Scottish Ear Nose and Throat Outcome Study (SENTOS) did not have surgery. A total of 1373 (30%) participants with various diagnoses were given reassurance and advice with no active intervention. There was no change in their GBI-5F total or factor scores, suggesting that they did not come to harm from their lack of active intervention. Hearing aids for bilateral sensorineural hearing loss gave greater benefit than reassurance in all factors, though individuals with a conductive impairment reported greater benefit in the Quality of life factor than those with a sensorineural impairment. Hearing aids and maskers produced benefit in the Support factor for patients with tinnitus. Epley's manoeuvre for benign paroxysmal positional vertigo gave benefit in the total score and the Quality of life factor compared with reassurance. Systemic medication for laryngo-pharyngeal reflux and topical medication for otitis externa gave no greater benefit in any factor or the total score compared with reassurance. CONCLUSION: The GBI-5F and its five factors give useful information for reporting the benefit of non-surgical interventions in adult otolaryngology and audiology clinics.

Outcomes and complications of simple layered closure of persistent tracheocutaneous fistula after tracheostomy in childhood.

BACKGROUND: Up to half of all children who have a tracheostomy will develop a persistent tracheo-cutaneous fistula (TCF) after decannulation. Surgical closure of the TCF is technically easy but post-operative complications can be immediate and life-threatening. These include air leak from the tracheal repair leading to massive surgical emphysema or pneumothorax. We reviewed our experience of TCF closure to try to identify potential risk factors for complications. METHOD: Retrospective case record review of all children (0-16 years) who underwent surgical TCF closure between January 2010 and December 2021 following development of a persistent TCF after decannulation of a tracheostomy. RESULTS: We identified 67 children. They ranged in age from 14 months to 16 years (median 3 years 10 months) at the time of the TCF closure. Major medical comorbidities were present in 90%. Pre-operative pulse oximetry with the fistula occluded was used in 29 children (43%). An underwater leak test was performed in 28 (42%). A non-suction drain was used in 29 children (43%). Prophylactic antibiotics were prescribed for 30 children (45%). Post-operative complications occurred in 15 children (22%). Life-threatening air leak occurred in the immediate post-operative period in 2 children (3%). Respiratory distress occurred in 3 children (4%) in the recovery area immediately after surgery. None required re-tracheostomy. Three children suffered post-operative pneumonia (4%), and wound infections occurred in 8 children (12%). We were unable to show a significant association between patient or surgical factors and complications. DISCUSSION: Complications for TCF closure are unfortunately common and it is unclear from the available evidence how best to prevent them. Further research is required.

Exploring the Factor Structure of the Glasgow Children's Benefit Inventory: New Recommendations for Reporting Results.

OBJECTIVE: Patient-reported outcomes can be useful for reporting benefit from non-life-saving interventions, but often they report a single overall score, which means that much information on the specific areas of benefit is lost. Our aim was to perform a new factor analysis on the Glasgow Children's Benefit Inventory (GCBI) to create subscales reflecting domains of benefit. Further aims were to assess the internal consistency of the GCBI, and to develop guidelines for reporting both a total score and sub-scales in future studies. METHODS: We collected 4 existing datasets of GCBI data from children who have undergone tonsillectomy, ventilation tube insertion, pinnaplasty, and submucous diathermy to the inferior turbinates. We performed exploratory factor analysis with principal axis factoring with varimax rotation, we sought redundancy in question items, and we measured internal consistency. RESULTS: Using the combined dataset of 772 cases, we found 4 factors which accounted for 64% of the variance and which we have labeled "Psycho-social," "Physical health," "Behavior," and "Vitality." Subscale results varied in predictable ways depending on the nature of the intervention. Cronbach's alpha was 0.928. Item-total correlations were high, and no item could be deleted to improve alpha. Floor effects were apparent for various questions but were not consistent between different interventions. CONCLUSIONS: The GCBI contains a range of questions which each add value in different clinical interventions. We can now make recommendations for reporting the results of the GCBI and its 4 new subscales.

Trends in tonsillectomy surgery in children in Scotland 2000-2018.

BACKGROUND: Tonsillectomy is one of the most common surgical procedures in children but indications and surgical practice change over time. OBJECTIVES: We aimed to identify trends in tonsillectomy procedures in children, in particular the number of procedures performed, the age of child undergoing tonsillectomy and the type of hospital in which the surgery was performed. DESIGN: Review of Scottish Morbidity Records data (SMR01) which are routinely collected after everyday case procedure or overnight stay in all Scottish NHS hospitals. SETTING: All NHS hospitals in all 14 Scottish health boards. PARTICIPANTS: All children (0-16 years) undergoing tonsillectomy, 2000-2018. MAIN OUTCOME MEASURES: Number of tonsillectomy procedures; rate of tonsillectomy per 1000 children in the population; number of children aged 0-2 years and 3-4 years undergoing tonsillectomy; health board in which the surgery occurred; diagnostic coding for these episodes; length of stay and readmission within 30 days of surgery. RESULTS: During 2000-2018, there were 50,208 tonsillectomies performed in children in Scotland (mean 2642/year). The number of tonsillectomies per year remained constant (R = 0.322, P = .178) but tonsillectomies performed in children 0-2 years rose from 0.41 to 1.56 per 1000 (R = 0.912, P < .001), and 3-4 years from 3.06 to 6.93 per 1000 (R = 0.864, P < .001). The proportion of all children's tonsillectomies performed up to age 4 rose from 20.6% to 35.9% and up to age 2 from 2.4% to 8.1%. All specialist children's hospitals showed a significant increase in surgery in very young children. CONCLUSIONS: Tonsillectomy rates remained static between 2000 and 2018, despite a falling population. More tonsillectomies are now performed for obstructive sleep apnoea, at a young age and in regional children's hospitals. This has important implications for the workload of these specialist hospitals.

Revised 15-item Glasgow Benefit Inventory with five factors based on analysis of a large population study of medical and surgical otorhinolaryngological interventions.

OBJECTIVES: To review, using confirmatory factor analysis, the widely used 18-question Glasgow Benefit Inventory [GBI] that has three factors. Thereafter to develop, using exploratory factor analysis, a more coherent, revised version of the GBI. DESIGN: Confirmatory and exploratory factor analysis of a large national GBI data set of ORL interventions. SETTING: Adult otorhinolaryngology outpatient clinics in six University Hospital departments. PARTICIPANTS: One thousand nine hundred eighty adult patients who had complete GBI data and who underwent an active (medical or surgical) intervention, out of the total data set of 9005 patients from the original Scottish ENT Outcome Study [SENTOS]. RESULTS: One of the 18 questions was discarded from the data base because it was not answered by 8% of respondents. Two of the original factors remained (Physical Health, renamed General Health, and Social Support, renamed Support). The General factor was split into three new factors (Quality of Life, Self-Confidence and Social Involvement). The three new factors were found to give additional information regarding the area of benefit. This reduced number of questions does not make any material difference to the results of the >196 existing GBI papers in the literature. CONCLUSION: A 15-question GBI with five factors is provided that is more explanatory of the areas of benefit.

Prevalence, risk factors and management strategies for otological problems in girls with Turner syndrome.

AIM: To determine the prevalence and risk factors of ear disease in Turner syndrome (TS), propose an algorithm for future surveillance and recommend preventative strategies. METHODS: Review of TS patients seen in the West of Scotland between 1989 and 2015, with questionnaire follow-up in 2015. RESULTS: Of 168 girls, median age 27.3 (3.8-47.2) years, ear problems occurred more frequently with 45,X and 45,X/46,XiXq than other karyotypes: 71/103 (69%) versus 23/65 (35%). Recurrent acute otitis media (AOM) first developed at 0-5 years in 23 (40%) girls, persisting in 16 (10%) at 5-10 years; and first developing at 5-10 years in 11 (7%). Persistent otitis media with effusion (OME) first developed at 0-5 and 5-10 years in 23 (40%) and 14 (8%) girls. Recurrent AOM was significantly linked with cholesteatoma in 8 (4.9%) girls (7 aged >10 years). Permanent hearing loss was documented in 28 girls (16.7%), with 16 (9.5%) receiving hearing aids (bone-anchored in 3). CONCLUSION: Acute otitis media and OME occur commonly in preschool TS girls and may persist or newly develop in later childhood. Recurrent AOM predisposes to cholesteatoma. Strategies to reduce otological morbidity include: intensive patient education, annual audiology, vaccinations and a randomised trial of antibiotic prophylaxis in high-risk groups.