Late-onset, progressive sensorineural hearing loss in the paediatric population: a systematic review.

PURPOSE: To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection. METHODS: PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16 years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare. RESULTS: 37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204 months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156 months, mean 38.7 ± 40.7). CONCLUSIONS: cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5 days, prematurity ≤ 34 weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3 months of age up to at least 3-4 years of age, and at least annually, should be recommended.

Hodgkin Lymphoma Presenting With Spinal Cord Compression: Challenges for Diagnosis and Initial Management.

Hodgkin lymphoma (HL) can present with extra-nodal disease, but spinal cord compression is exceptionally rare. We describe a 15-year-old presenting with hip/back pain with normal initial examination. Persistent pain and raised inflammatory markers prompted further investigation with MRI, which revealed an epidural mass causing spinal cord compression. On examination, there was no palpable lymphadenopathy or cauda equina syndrome, but absent lower limb reflexes were noted. Following multidisciplinary discussion, it was determined that cauda equina syndrome was imminent and therefore surgical debulking was undertaken, both to prevent this complication and establish a diagnosis. At surgery, the tumor was highly vascular. Frozen section confirmed lesional material. Following surgery, and given the frozen section findings, a short course of steroids was commenced to reduce any peri-surgical edema. Unfortunately, histopathology was ultimately non-diagnostic, due to failure of immunohistochemistry on technically challenging material. Consequently, ultrasound-guided excision biopsy of a (non-palpable) cervical lymph node was performed five days later; histopathology showed typical effacement of the normal architecture and a conspicuous population of CD15/CD30-positive larger pale cells present, confirming nodular sclerosis classic HL, despite recent steroids. We review the available literature for HL presenting with spinal cord compression and describe the challenges for diagnosis and initial management in such cases.

Fifteen-minute consultation: Child with persistent runny nose.

A child with persistent runny nose may cause significant parental anxiety and healthcare utilisation. While the most common diagnoses are recurrent acute viral upper respiratory tract infections and allergic rhinitis, a careful history and examination is necessary to exclude other causes and to identify comorbidities. Treatment can then be tailored to the underlying cause. The aim of this article is to provide a systematic approach to such patients.

Synchronous airway lesions in children with severe, progressive and atypical laryngomalacia - Experience of a UK tertiary referral centre.

OBJECTIVES: Laryngomalacia is the commonest cause of stridor in the paediatric population. Whilst usually self-limiting, studies have suggested that the presence of synchronous airway lesions (SALs) might be associated with more severe clinical presentation and additional morbidity. However, evidence in the literature is scarce, contributing to controversy regarding the appropriate investigation and management of children with laryngomalacia. The aim of this study was to explore the prevalence, clinical significance and risk factors of SALs in children with severe, progressive and atypical laryngomalacia symptoms. METHODS: Retrospective analysis of the records of all paediatric patients diagnosed with laryngomalacia in a UK tertiary referral centre, over a 2-year period, was undertaken. Data on demographics, comorbidities, management and presence of SALs were collected. RESULTS: The study included 130 patients with laryngomalacia. Seventy-eight patients required investigation with microlaryngobronchoscopy (MLB), based on clinical presentation. SALs were identified in 22 patients (28.2%), 6 of which (7.7%) required surgical intervention. The commonest airway lesions included tracheomalacia, bronchomalacia and subglottic stenosis. A correlation between the presence of neurodevelopmental conditions in children and SALs was noted (p value < 0.05). The presence of SALs was not associated with sex, gestational age at birth or other medical comorbidities. CONCLUSION: The results of this study allow professionals to counsel families about the utility of MLB in children with severe, progressive and atypical laryngomalacia symptoms and inform management pathways for clinicians not working in airway centres. We support the clinical practice that progressive, atypical or severe laryngomalacia symptoms should prompt consideration of an MLB, to identify potential synchronous airway lesions. Furthermore, we propose that a higher index of suspicion and more thorough investigation might be indicated in children with neurodevelopmental disorders.

Methicillin-resistant Staphylococcus aureus (MRSA) submandibular abscess in a neonate.

Neonatal suppurative submandibular sialadenitis and abscess formation is an exceedingly rare entity. This report describes a complex case of a male neonate with a methicillin-resistant Staphylococcus aureus (MRSA) submandibular abscess, requiring emergency intubation due to acute airway compromise. The patient was admitted to the paediatric intensive care unit, received appropriate antibiotic treatment and underwent urgent surgical drainage of the abscess. He made a full recovery and remains well 18 months later. No comorbidities or common risk factors for the disease were identified. Although extremely uncommon, neonatal submandibular abscesses can lead to significant morbidity. Neonates tend to present insidiously, and sudden clinical deterioration with airway compromise is possible. MRSA has been increasingly implicated in these infections, even in the absence of relevant risk factors. As such, continued clinical vigilance is essential for prompt diagnosis and prevention of life-threatening complications. Multidisciplinary input is paramount for appropriate management of these complex infections.

Anatomic findings in revision endoscopic sinus surgery: Case series and review of contributory factors.

BACKGROUND: It is recognized that patients who undergo endoscopic sinus surgery (ESS) do not always achieve control of their disease. The causes are multifactorial; variations in surgical practice have been identified as possible factors in refractory disease. OBJECTIVE: To reflect on the frequent anatomic findings of patients with chronic rhinosinusitis (CRS) who require revision ESS. METHODS: A retrospective review of patients who required revision ESS at a tertiary institution over a 3-year period. Patients for whom maximal medical therapy failed for CRS underwent computed tomography of the paranasal sinuses and image-guided surgery. Surgical records of anatomic findings were reviewed and analyzed. RESULTS: Over 3 years, a total of 75 patients underwent revision procedures, 28% of all ESS performed in the unit. The most frequent finding was a residual uncinate process in 64% of the patients (n = 48); other findings included a maxillary antrostomy not based on the natural ostium of the maxillary sinus in 47% (n = 35), an oversized antrostomy in 29% (n = 22), resected middle turbinates in 35% (n = 26), middle meatal stenosis in 15% (n = 11), synechiae in 29% (n = 22), and osteitic bone that required drilling in 13% (n = 10). CONCLUSION: Surgical technique can give rise to anatomic variations that may prevent adequate mucociliary clearance and medication delivery, which leads to failure in ESS in patients with CRS. This study demonstrated the surgical findings encountered in revision ESS that should be highlighted in the training of Ear, Nose and Throat surgeons to help prevent primary failure and reduce health care costs.

Quality-of-life outcomes after sinus surgery in allergic fungal rhinosinusitis versus nonfungal chronic rhinosinusitis.

BACKGROUND: Given the differences in pathophysiology between allergic fungal rhinosinusitis (AFRS) and other chronic rhinosinusitis (CRS) subgroups, it remains unclear about whether these patients respond differently to a combination of surgical and medical treatments. OBJECTIVE: To evaluate differences in quality-of-life (QoL) outcomes for a cohort of patients who underwent endoscopic sinus surgery (ESS) for CRS. METHODS: This retrospective review included patients with CRS who underwent ESS between 2010 and 2013. QoL was measured by using the 22-item Sino-Nasal Outcome Test (SNOT-22). Variables collected included baseline demographics, SNOT-22 scores before ESS and at 1, 3, 6, 9, and 12 months after ESS. Groups tested were CRS with nasal polyposis, CRS without nasal polyposis (CRSsNP), and patients with AFRS. A linear mixed- effects regression model was used to calculate the adjusted mean QoL differences. RESULTS: Among the 250 patients included, 61.6% had CRS with nasal polyposis (n = 154), 28.8% had CRSsNP (n = 72), and 9.6% had AFRS (n = 24). Significant differences were seen in SNOT-22 scores between pre- and postoperative visits and between the etiologic subgroups (p < 0.001). Multivariate analysis revealed significantly greater improvement in QoL for patients with AFRS in comparison with those with CRSsNP at the 9-month follow-up (change in SNOT-22 score, 22.6 [95% confidence interval, 1.2-44.1]; p < 0.0) and the 12-month follow-up (change in SNOT-22 score, 20.2 [95% confidence interval, 0.5-39.9]; p < 0.04). CONCLUSIONS: Patients with AFRS experienced a more-prolonged QoL benefit from surgical and targeted medical intervention compared with those with CRSsNP, which may reflect the severity of inflammation that they presented with compared with other CRS subtypes.

A previously unreported serious adverse event during balloon sinuplasty.

Balloon sinuplasty is considered as a minimally invasive procedure for the treatment of chronic rhinosinusitis. In this case report we describe how a patient undergoing balloon sinuplasty sustained an intraoperative cardiac arrest with no cardiac history. Postoperative investigations were all normal. The patient was discharged on the first postoperative day and further outpatient tests were normal. We postulate that this event occurred due to profound vagal stimulation either on instrumentation of the maxillary mucosa or when the orbit was accidently breached during instrumentation of the right frontal recess. The authors conclude that balloon sinuplasty, despite being minimally invasive surgery, should be performed under the supervision of an anaesthesiologist with the ability of cardiac monitoring and immediate treatment because of possible arrhythmias.

Diffuse large B-cell lymphoma: an unusual cause of severe otalgia and facial palsy.

An elderly woman, two months after chemotherapy for diffuse large B-cell lymphoma, presented with left-sided otalgia, discharge and facial nerve palsy. MRI showed an active left mastoid infection with an ear canal lesion, likely to be a cholesteatoma. However, a biopsy of the mass showed recurrent high-grade lymphoma. Following diagnosis, the patient opted for palliative care within the community and consequently passed away a few weeks later.

Cochlear implantation update: contemporary preoperative imaging and future prospects - the dual modality approach as a standard of care.

The selection of cochlear implant (CI) candidates requires consideration of a variety of clinical and radiographic factors. The present article reviews the current knowledge regarding the preoperative imaging of CI candidates and explores emerging developments in different imaging modalities. Preoperative radiologic assessment should evaluate the status of the middle/inner ear, auditory nerve and central acoustic pathways. Preoperative computed tomography displays anatomic middle ear variations of surgical importance. MRI can demonstrate fluid/obliteration in the inner ear and depict the retrocochlear auditory pathways. Dual modality imaging with high-resolution computed tomography and MRI of the petrous bone and brain can provide the maximum information regarding surgical landmarks and detect deafness-related abnormalities. Cost-effectiveness issues also justify its use. New systems are now becoming available, offering improved soft-tissue delineation, sophisticated segmentation techniques, volumetric measurements, semitransparent views and superior surface resolution, thus significantly advancing our diagnostic acumen and making the preoperative evaluation of CI candidates more accurate and reliable.