This paper discusses clinical features and management aspects of an extremely rare entity, neurofibroma of the external nose.

Database searches were performed using PubMed, Embase and Google Scholar to identify previously published articles.

Twelve articles comprising 13 patients with neurofibroma of the external nose were included. The mean age of presentation was 31 years. Sixty-nine per cent of patients were diagnosed at final histopathology. External approach rhinoplasty was performed in 76.9 per cent of patients, while the intranasal approach was used in 15.3 per cent of patients. There was a 15.3 per cent association with neurofibromatosis type 1. Recurrence was noted in 23 per cent of patients.

It may be challenging to diagnose this entity clinically because of its rarity and striking features on histopathology. Neurofibroma of the external nose should be kept in mind as a differential diagnosis for any soft progressive swelling over the nose. Management requires complete excision, with cosmesis and restoration of functions.

Otosclerosis affects women twice as often as men, especially during fertile age. A role of female hormones has been claimed, but controversy still exists regarding the influence of pregnancy. The purpose of this research was to analyse the role of pregnancy on the course of otosclerosis.

PubMed was searched in May 2019 using the terms ‘otosclerosis AND pregnancy’, ‘otosclerosis AND pregnant’, ‘otosclerosis AND parous’, ‘otosclerosis AND parity’, and ‘otosclerosis AND puerperium’. Age at diagnosis, number of pregnancies and the temporal relationship of the disease with childbearing were considered.

From 65 articles, 11 were chosen for review. They described 2323 women affected by otosclerosis: 1805 had at least 1 pregnancy, while 518 did not. During childbearing, otosclerosis began in 1 per cent of pregnant women, worsened in 21 per cent and worsened during puerperium in 4 per cent. Often, the authors reported hearing change with pregnancy without details, so a further group has been considered composed of women belonging to any of the groups just mentioned or to another group of women not further characterised. Overall, hearing change occurred during pregnancy in 44 per cent. A statistically significant correlation emerged between hearing change and number of pregnancies (p = 0.003).

Because of wide data heterogeneity and the difficulty in analysing a single factor, absolute statements could not be formulated. According to this review, pregnancy seems to have a worsening effect on the course of otosclerosis.

Magnetic resonance imaging scans of the internal acoustic meatus are commonly requested in the investigation of audio-vestibular symptoms for potential vestibular schwannoma. There have been multiple studies into protocols for requesting magnetic resonance imaging for vestibular schwannoma, but none have been reported based on UK National Institute for Health and Care Excellence guidelines for investigating audio-vestibular symptoms. This study intended to identify the local magnetic resonance imaging detection rates and patterns of vestibular schwannoma, and to audit the conformity of scan requests with the National Institute for Health and Care Excellence guidelines, with a review of relevant literature.

A retrospective analysis of 1300 magnetic resonance imaging scans of the internal acoustic meatus, compared against National Institute for Health and Care Excellence guidelines, was conducted over two years.

Sixteen scans were positive for vestibular schwannoma, with a detection rate of 1.23 per cent. All positive cases fit the guidelines; three of these could have been missed using other criteria. A total of 281 requests did not meet the guideline criteria but revealed no positive results, supporting the use of National Institute for Health and Care Excellence guidelines in planning magnetic resonance imaging scans for audio-vestibular symptoms.

Nasal chondromesenchymal hamartoma is a rare pathology that can present with a myriad of symptoms to ENT, maxillofacial and ophthalmology departments. This study reviewed the literature on nasal chondromesenchymal hamartoma as well as adding three new cases to the reported literature.

This study was an up-to-date review of the world literature with the addition of three new cases to provide the most comprehensive review to date.

A total of 56 patients with nasal chondromesenchymal hamartoma were identified, ranging from children to adults. Nasal symptoms and ocular symptoms were most common, and surgical resection was the most frequent treatment modality.

This study advocates for increased awareness of the condition associations for nasal chondromesenchymal hamartoma, multi-specialty treatment and the role for the ENT surgeon in treatment of the condition.

Necrotising otitis externa is an invasive, infective condition, with minimal evidence underpinning its diagnosis and management. This work aimed to analyse literature from the past decade, to identify emerging themes and important topics for future research.

A robust literature search and review were conducted by two researchers. Sixty studies were filtered into the final review. A grounded theory approach was used to identify core themes. Data within these themes formed the basis of the review.

There is no consensus regarding a clinical definition or outcome measures of necrotising otitis externa, and there exists no level 1, 2 or 3 evidence to diagnose, investigate, monitor or treat necrotising otitis externa. Emerging themes in the literature direct researchers to important topics for future clinical trials, including risk factors, microbiological culture, management strategies and radiology.

In order to optimise understanding and management of necrotising otitis externa, future research requires robust clinical trials and consistently reported outcome measures.

Paediatric otogenic cerebral venous sinus thrombosis is a rare, heterogeneous and life-threatening condition, with possible otological, neurological and ophthalmological sequelae. Its course and outcomes can be widely variable. The publications available often consider individual aspects of paediatric otogenic cerebral venous sinus thrombosis management. The condition itself and the nature of the currently available guidance can lead to uncertainties when holistically managing patients with paediatric otogenic cerebral venous sinus thrombosis.

Clear recommendations for the comprehensive assessment and management of paediatric otogenic cerebral venous sinus thrombosis are presented, along with the literature review upon which they are based. Its clinical and radiological assessment are discussed.

A multidisciplinary approach to assessment and management is recommended, inclusive of infectious diseases, ENT surgery, neurology, ophthalmology and haematology. On balance, anticoagulation is recommended for three months. Follow-up imaging is not recommended in the absence of clinical concern. Follow up by ENT surgery, neurology and ophthalmology departments is recommended.

Described just over 20 years ago, superior semicircular canal dehiscence remains a relatively unknown and easily missed cause of dizziness and auditory symptoms.

This review focused on the origin, presenting symptoms and underlying pathophysiology of superior semicircular canal dehiscence, and the available treatment options.

The bony dehiscence acts as a ‘third window’, affecting inner-ear homeostasis, and resulting in hypersensitivity and a vestibular response to lower sound level stimuli. The third window effect explains the pressure- and sound-induced vertigo, oscillopsia, and nystagmus, as well as autophony, conductive hyperacusis and tinnitus. The origin of superior semicircular canal dehiscence is linked to the combination of a congenital or developmental factor, and a ‘second event’ like head trauma, rapid pressure changes or age-related factors. Computed tomography of the temporal bone and reduced vestibular-evoked myogenic potential thresholds can confirm the diagnosis. Despite only retrospective cohorts, surgery is considered a safe treatment option, targeting mainly vestibular but also auditory symptoms, with transmastoid approaches gaining popularity.

To compare the efficacy and safety characteristics of endoscopic and microscopic stapes surgery based on current evidence.

A systematic literature search was conducted of three medical databases, focusing on randomised, controlled studies or observational studies. Data related to the efficacy and safety of each technique were extracted. Outcome data were summarised using the pooled mean differences or pooled odds ratios, along with their 95 per cent confidence intervals.

Thirteen studies were included in the meta-analysis. Success rate was evaluated by estimating air–bone gap improvement; this revealed comparable outcomes for the two techniques (mean difference = −0.20; 95 per cent confidence interval = −0.53, 0.14). No statistically significant difference was detected concerning post-operative complications, except for dysgeusia (odds ratio = −1.12; 95 per cent confidence interval = −1.97, −0.28) and pain (odds ratio = −2.00; 95 per cent confidence interval = −2.97, −1.04), which favoured the endoscopic approach.

Though both techniques result in commensurate outcomes concerning success rate, post-operative pain and dysgeusia favour the endoscopic approach. Further high-quality studies are needed to adequately compare the two methods.