Cochlear nerve dysplasia in unilateral severe to profound congenital sensorineural hearing loss - Prevalence in Australian children and the impact of socioeconomic disadvantage on its management.

INTRODUCTION: Congenital unilateral sensorineural hearing loss (cuSNHL) carries potentially significant social, educational, and developmental consequences. Early diagnosis enables investigation, and consideration of options for management and early intervention, helping to mitigate the effects of hearing loss. Cochlear nerve dysplasia (CND) is a prominent cause of cuSNHL and may affect candidacy for cochlear implantation. Socioeconomic disadvantage may impact on a patient's family's capacity to participate in necessary intervention and follow-up. METHODS: Infants with severe-profound cuSNHL referred to a large Australian quaternary pediatric center between October 2004 and December 2020 were retrospectively included. Audiometric and clinical data, and the presence of hearing loss risk factors were obtained from a prospectively collated database. In Australia MRI scans are provided free-of-charge to citizens and residents. MRI scans were reviewed to determine the status of the nerves within the internal acoustic meatus (IAM grade) along with attendance rates. Travel distance to the hospital was also calculated. Reasons for non-attendance at MRI were obtained from patient medical records and correspondence. Socioeconomic, educational, and occupational indices, and travel distances were obtained using patient residential postcodes with reference to Australian Bureau of Statistics data. RESULTS: A total of 98 patients were reviewed, 64.3% (n = 63) of whom underwent MRI. The median age at diagnosis was 40 days (IQR 27). The prevalence of CND was 75% (n = 47). Importantly, there was no significant difference in the degree of hearing loss between IAM grades (F(4,57) = 1.029, p = 0.405). Socioeconomic indices were significantly lower in patients not attending MRI investigations compared with patients who did attend. Travel distance was not significantly different between the two groups. CONCLUSION: CND is a prominent cause of cuSNHL in Australian infants. MRI at a young age allows parent education regarding management options and timely intervention where indicated. Socioeconomic disadvantage significantly impacts on participation in further routine assessment of cuSNHL, potentially limiting management options for these children long term.

Restructuring ENT out-patient services during the coronavirus disease 2019 pandemic - an iterative approach.

BACKGROUND: Coronavirus disease 2019 has demanded enormous adjustments to National Health Service provisions. Non-urgent out-patient work was initially postponed or performed virtually, but is now being re-established. In ENT surgery, aerosol-generating procedures pose a particular challenge in out-patient settings. OBJECTIVE: A rapid restructuring of ENT out-patient services is required, to safely accommodate aerosol-generating procedures and increase in-person attendances, whilst coronavirus disease 2019 persists. METHODS: Data were collected prospectively over four consecutive cycles. Two surveys were conducted. Results were analysed and disseminated, with recommendations for service restructuring implemented at cycle end-points. RESULTS: Out-patient activity increased four-fold, associated with a significant rise in aerosol-generating procedures during the study period. Mean aerosol-generating procedure duration dropped weekly, implying a learning curve. Service restructuring occurred at cycle end-points. CONCLUSION: Iterative data gathering, results analysis and outcome dissemination enabled a swift, data-driven approach to the restructuring of ENT out-patient services. Patient and staff safety was ensured, whilst out-patient capacity was optimised.

Surgical treatment of chronic ear disease in remote or resource-constrained environments.

BACKGROUND: Surgery for chronic suppurative otitis media performed in low- and middle-income countries creates specific challenges. This paper describes the equipment and a variety of techniques that we find best suited to these conditions. These have been used over many years in remote areas of Nepal. RESULTS AND CONCLUSION: Extensive chronic suppurative otitis media is frequently encountered, with limited pre-operative investigation or treatment possible. Techniques learnt in better-resourced settings with good follow up need to be modified. The paper describes surgical methods suitable for resource-poor conditions, with rationales. These include methods of tympanoplasty for subtotal wet perforations, hearing reconstruction in wet ears and open cavities, large aural polyps, and canal wall down mastoidectomy with cavity obliteration. Various types of autologous ossiculoplasty are described in detail for use in the absence of prostheses. The following topics are discussed: decision-making for surgery on wet or best hearing ears, children, bilateral surgery, working with local anaesthesia, and obtaining adequate consent in this environment.

Implementation and results of a surgical training programme for chronic suppurative otitis media in Cambodia.

BACKGROUND: Chronic suppurative otitis media is a massive public health problem in numerous low- and middle-income countries. Unfortunately, few low- and middle-income countries can offer surgical therapy. METHODS: A six-month long programme in Cambodia focused on training local surgeons in type I tympanoplasty was instigated. Qualitative educational and quantitative surgical outcomes were evaluated in the 12 months following programme completion. A four-month long training programme in mastoidectomy and homograft ossiculoplasty was subsequently implemented, and the preliminary surgical and educational outcomes were reported. RESULTS: A total of 124 patients underwent tympanoplasty by the locally trained surgeons. Tympanic membrane closure at six weeks post-operation was 88.5 per cent. Pure tone audiometry at three months showed that 80.9 per cent of patients had improved hearing, with a mean gain of 17.1 dB. The trained surgeons reported high confidence in performing tympanoplasty. Early outcomes suggest the local surgeons can perform mastoidectomy and ossiculoplasty as safely as overseas-trained surgeons, with reported surgeon confidence reflecting these positive outcomes. CONCLUSION: The training programme has demonstrated success, as measured by surgeon confidence and operative outcomes. This approach can be emulated in other settings to help combat the global burden of chronic suppurative otitis media.

Lyme disease: sudden hearing loss as the sole presentation.

BACKGROUND: Lyme disease is an uncommon tick-borne multisystemic infection caused by Borrelia burgdorferi. The most common clinical manifestation is erythema migrans. In this report, a very unusual presentation of this condition is described, in which sudden onset sensorineural hearing loss was the sole presenting symptom. METHODS: Case report and review of English-language literature. RESULTS: A patient presented with sensorineural hearing loss, with no other symptoms or signs. Acute Lyme infection was detected by laboratory tests. Magnetic resonance imaging showed signs of labyrinthitis of the same inner ear. After hyperbaric oxygen and systemic antibiotic treatment, the patient showed total hearing recovery, and magnetic resonance imaging showed complete resolution of the labyrinthitis. CONCLUSION: To our knowledge, this is the first reported case of Lyme disease presenting only with sensorineural hearing loss. Borreliosis should be considered as an aetiological factor in sensorineural hearing loss. Adequate treatment may provide total recovery and prevent more severe forms of Lyme disease.

Prospective cohort comparison of bioactive glass implants and conchal cartilage in reconstruction of the posterior canal wall during tympanomastoidectomy.

OBJECTIVE: To compare the effectiveness of bioactive glass implants and conchal cartilage in reconstructing the posterior canal wall during tympanomastoidectomy. STUDY DESIGN: Prospective cohort clinical study. SETTING: Teaching hospital. PATIENTS: Patients with clinically diagnosed chronic suppurative otitis media and cholesteatoma awaiting tympanomastoidectomy were recruited. INTERVENTION: All patients underwent tympanomastoidectomy by the same surgeon. A first cohort of 12 patients underwent posterior canal wall reconstruction with autogenous conchal cartilage. A second cohort of 12 patients underwent such reconstruction with prefabricated bioactive glass. MAIN OUTCOME MEASURES: Primary- All patients underwent out-patient review at 1, 3, 6 and 12 months postoperatively, after which a second-look procedure was performed. Reconstructions were inspected for evidence of epithelialization, granulation, infection, stenosis, depression and extrusion. Secondary- All patients had perioperative serial pure-tone audiometry to check for any change in hearing levels upto 1 year postoperatively. RESULTS: By 1 year postoperatively, both reconstructive graft materials showed good epithelialization, no granulation, no infection, no ear canal stenosis, no depression and no extrusion. At operative second-looks, bioactive glass particularly showed good tissue bonding, including both neovascularization and connective tissue integration. Overall clinical outcome was equivalent for both materials. Both graft materials showed no statistically significant difference in postoperative hearing levels. CONCLUSIONS: Bioactive glass and conchal cartilage showed equivalent clinical outcome in reconstructing the posterior canal wall without significantly affecting hearing levels. As bioactive glass does not require second site morbidity and thus also reduces operative time, we prefer it for reconstructing the posterior canal wall following tympanomastoidectomy.

A combined third and fourth branchial arch anomaly: clinical and embryological implications.

Embryological abnormalities of the branchial apparatus present an interesting diagnostic and surgical challenge. Thymic cysts are a rare form of branchial apparatus anomaly, resulting from abnormal development of the third pharyngeal pouch. We present two cases of a thymic cyst coexisting with a non recurrent inferior laryngeal nerve (NRILN), two anomalies that to our knowledge have not been associated previously. A possible embryological explication for this double abnormality is discussed, while the clinical implications of this association are presented.