Congenital hearing loss is a chronic condition which occurs worldwide. In the past, investigations focused on testing the most common genes associated with hearing loss (such as Connexin 26-related hearing loss). Targeted testing of specific genes was requested only when a particular syndrome was suspected. Recent advances have led to the development of a large gene panel which utilises next-generation sequencing to simultaneously test for pathogenic variants in many genes associated with hearing loss.

This review article aims to highlight the changes in the approach to congenital hearing loss in the context of the R67 gene panel, and how its use may increase the efficiency of the diagnosis and management of this condition.

The use of this large gene panel has revolutionised the approach to hearing loss. Uptake of this large gene panel has resulted in prompter diagnosis and therefore more appropriate clinical management.

This study aimed to evaluate clinical characteristics, treatments and outcomes of paediatric temporal bone fractures at our institute.

A retrospective study of paediatric skull fractures confirmed by imaging from January 2010 to December 2022. Data on demographics, clinical presentations, injury mechanisms and complications were analysed, and fractures were categorised into otic capsule sparing (OCS) and violating (OCV).

Of 369 skull fracture cases, 88 (24 per cent) involved temporal bones, predominantly caused by falls and vehicle accidents. Common symptoms were loss of consciousness, hematoma, and hemotympanum, with complications like facial nerve injury and cerebrospinal fluid leaks in 3.4 per cent of cases. OCV fractures led to more severe complications, including hearing loss. Audiology showed 65 per cent without hearing impairment, while others had various degrees of loss.

Paediatric temporal bone fractures, particularly OCV types, pose significant challenges. Early detection and thorough management are vital, underscoring the need for consistent data collection and regular audiometric monitoring.

For 50 years, the Thomas Wickham-Jones (TWJ) Foundation has promoted the advancement of otology and audiology in the UK and Republic of Ireland through a series of overseas Fellowships and other grants.

The paper examines the history of the Foundation since its establishment in 1974, drawing upon the Foundation's archives and personal recollections. The analysis is located within a framework concerning the factors that shape the success or failure of a foundation including vision, strategy, information, leadership and finance.

The activities of the TWJ Foundation are charted over five decades, and the governance of the Foundation is detailed. Particular attention is given to the Major Fellowships offered, at first in North America, and to their subsequent development into the Foundation's current rotation.

The paper offers an assessment of the TWJ Foundation's impact on the training of otologists in the British Isles and concludes with a brief self-reflective analysis.

This pilot study aimed to evaluate a training programme for primary care physiotherapists focused on the assessment and management of benign paroxysmal positional vertigo.

A six-month training programme and toolkit utilising the revised Standards for Quality Improvement Reporting Excellence (‘SQUIRE 2.0’) guidelines was developed to facilitate the learning of new knowledge and skills in the assessment and management of benign paroxysmal positional vertigo following Gagne's model of instructional design. A pre- and post-training knowledge and confidence questionnaire evaluated the impact of the training programme.

Eleven participants started the training programme and five completed it. On average, knowledge increased by 54 per cent (range, 41–95 per cent) and confidence increased by 45 per cent (range, 31–76 per cent). A 73 per cent improvement in practical skills acquisition was demonstrated after the initial training session.

A structured approach to learning demonstrates improvements in knowledge, skills and confidence of physiotherapists in the evidence-based management of benign paroxysmal positional vertigo.

Driving capacity is affected by vestibular disorders and the medications used to treat them. Driving is not considered during medical consultations, with 92 per cent of patients attending a centre for dizziness not discussing it with the doctor.

To investigate if medical record prompts facilitate dizziness and driving conversations in ENT balance clinics.

A questionnaire was designed to reflect the current standards of practice and advice given regarding driving and dizziness during balance clinic consultations.

Medical record prompts facilitated the improved frequency and recording of shared decision-making conversations about driving and dizziness in 98 per cent of consultations.

This study highlights the benefits of medical record prompts for documented and accurate shared decision-making conversations surrounding dizziness, vertigo, vestibular conditions and driving. This potentially improves safety for all road users, and protects the patient and clinician in the event of road traffic accidents and medico-legal investigations.

Balance dysfunction and vestibular conditions are major problems requiring significant resources. There is significant national and international variation in management pathways for such patients.

This paper outlines a collaborative project run by the ENT department and two vestibular rehabilitation trained physiotherapists to establish a clinic to manage patients referred to ENT with vestibular and/or balance complaints. As part of a six-month pilot, two physiotherapy-led balance clinics were provided per week.

A total of 159 new patients were seen, with only 15 needing ENT consultant input. This led to the successful creation of substantive posts; the clinic has seen 698 patients in its first two years.

Patient outcomes and experience have been positive, and accompanied by reduced waiting and in-service times. The authors discuss some of the pitfalls, challenges and opportunities of developing this type of clinic.

Differential diagnosis of acute vertigo syndrome is challenging given the similarities between clinical presentations of posterior circulation stroke and peripheral vestibular dysfunction. The Head Impulse, Nystagmus, Test of Skew (‘HINTS’) assessment is a clinical bedside test used to aid diagnosis.

Comprehensive training on use of the Head Impulse, Nystagmus, Test of Skew assessment was provided to one stroke consultant, and the effectiveness of the test in that setting was assessed. Further education was completed with more members of the stroke and emergency department multi-disciplinary team. Quality improvement measures including magnetic resonance imaging use and bed utilisation were explored.

Following training of one stroke consultant, the Head Impulse, Nystagmus, Test of Skew assessment was found to be a feasible, accurate bedside test within this acute stroke service. Further training for the multi-disciplinary team was completed, but outcome measures were not explored because of the coronavirus disease 2019 pandemic and maternity leave.

There is a role for trained members of the multi-disciplinary team to successfully use the Head Impulse, Nystagmus, Test of Skew assessment in hyperacute stroke settings, to aid diagnosis in acute vertigo syndrome.

To examine the newly established role of a primary contact physiotherapist in an ENT clinic, in an Australian cohort and context, over two phases of development.

A retrospective cohort study was conducted with data collected from a medical record audit. Over the study duration, the primary contact physiotherapist completed initial appointments with patients; follow-up appointments were subsequently conducted by medical staff.

There was a 46 per cent reduction in patients with suggested vestibulopathy requiring an ENT medical review. This reduction could hypothetically increase to 71 per cent with follow-up primary contact physiotherapist appointments. Improvements in the service delivery model and a primary contact physiotherapist arranging diagnostic assessments could improve waitlist times and facilitate better utilisation of medical staff time.

The primary contact physiotherapist can help in the management of patients with suspected vestibulopathy on an ENT waitlist. This is achieved through: a reduction of patients requiring ENT review, improvements to waitlist time and improved utilisation of medical specialists’ time.

Benign paroxysmal positional vertigo is the most common episodic vestibular disorder, although it is often quiescent by the time patients visit a specialist clinic, making the diagnosis difficult to confirm. Patients fear the consequences of a relapse and it is not clear what follow up should be provided.

This evaluation reviewed the results of an open access pathway that offered priority appointments to patients with a history of positional vertigo.

In total, 664 patients were included in the analysis, 52 per cent of whom had unconfirmed benign paroxysmal positional vertigo. Open follow up improved diagnostic rates by approximately 40 per cent. The rate of benign paroxysmal positional vertigo recurrence was 34 per cent in patients with follow up of at least one year, 41 per cent with follow up of one to two years, and 53 per cent with follow up of two years or more. One in five recurrences occurred in a different semi-circular canal. These results suggest that specialist follow up is required for management of recurrent benign paroxysmal positional vertigo.

Persistent postural-perceptual dizziness classifies patients with chronic dizziness, often triggered by an acute episode of vestibular dysfunction or threat to balance. Unsteadiness and spatial disorientation vary in intensity but persist for over three months, exacerbated by complex visual environments.

Literature suggests diagnosis relies on a clinical history of persistent subjective dizziness and normal vestibular and neurological examination findings. Behavioural diagnostic biomarkers have been proposed, to facilitate diagnosis.

Research has focused on understanding the neural mechanisms that underpin this perceptual disorder, with imaging data supporting altered connectivity between neural brain networks that process vision, motion and emotion. Behavioural research identified the perceptual and motor responses to a heightened perception of imbalance.

Management utilises head and body motion detection, and downregulation of visual motion excitability, reducing postural hypervigilance and anxiety. Combinations of physical and cognitive therapies, with antidepressant medications, help if the condition is associated with mood disorder.

Patients presenting to the emergency department with acute vertigo pose a diagnostic challenge. While ‘benign’ peripheral vestibulopathy is the most common cause, the possibility of a posterior circulation stroke is paradoxically the most feared and missed diagnosis in the emergency department.

This review will attempt to cover the significant advances in the ability to diagnose acute vertigo that have occurred in the last two decades. The review discusses the role of neurological examinations, imaging and specific oculomotor examinations. The review then discusses the relative attributes of the Head Impulse-Nystagmus-Test of Skew plus hearing (‘HINTS+’) examination, the timing, triggers and targeted bedside eye examinations (‘TiTrATE’), the associated symptoms, timing and triggers, examination signs and testing (‘ATTEST’) algorithm, and the spontaneous nystagmus, direction, head impulse testing and standing (‘STANDING’) algorithm. The most recent technological advancements in video-oculography guided care are discussed, as well as other potential advances for clinicians to look out for.

PTEN hamartoma tumour syndrome (PHTS) comprises a group of genetic disorders with varied clinical presentations, including macrocephaly, developmental delay, and increased cancer susceptibility. Recent reports have highlighted the occurrence of tonsil-related issues in PHTS.

Clinical data focusing on tonsil-related pathology and tonsillectomy details (indications, histology and post-operative complications) were collected from 53 patients with PHTS.

Tonsil issues affected 58 per cent of the cohort, with 43 per cent requiring tonsillectomy. Primary indications for tonsillectomy included obstructive sleep apnoea (43 per cent), recurrent tonsillitis (17 per cent) and other causes (17 per cent). Tonsil-related problems were observed both before (45 per cent) and after (55 per cent) PHTS. Tonsillectomy with adenoidectomy was the predominant surgical intervention performed (87 per cent), spanning a broad age range (1–27 years old).

Our findings highlight the complex nature of PHTS and its association with tonsil-related pathology, demonstrating its relevance for ENT surgeons. Early recognition and intervention are pivotal for managing sleep apnoea and the associated health problems.

While the pathogenesis of sudden sensorineural hearing loss is thought to be localised to the cochlea, recent microRNA findings suggest a neuro-topic localisation in some patients. This study distinguishes if neural and non-neural groups differ in hearing recovery.

Neural-type hearing loss was defined as a presenting word recognition score less than 60 per cent, with a word recognition score reduction greater than 20 per cent than expected based on the averaged pure tone audiometry. Hearing recovery was defined as an improvement of greater than or equal to 10 decibels in pure tone audiometric thresholds.

Eight of 12 and 24 of 36 of neural and non-neural hearing loss patients demonstrated hearing recovery, respectively. The affected ear's word recognition score (per cent) change with treatment were different between the neural and non-neural groups (46.9 ± 29.8 vs 3.2 ± 25.8 (p < 0.0001)).

The hearing recovery rate in neural and non-neural hearing loss groups was similar. Patients with neural-type hearing loss demonstrated greater word recognition score recovery post treatment than those in the sensory group.

This retrospective study aimed to establish a robust rating system for assessing post-operative outcomes in congenital aural atresia patients undergoing auricular reconstruction. The newly introduced EAR scale, a weighted grading system, not only considers anatomical landmarks but also factors such as ear alignment. In addition, the outer-ear cartilage scale and the visual analogue scale (VAS) were introduced. These scales were compared among themselves and against two established scales.

Nine raters assessed 17 eligible patients who underwent auricular reconstruction between 2001 and 2020.

The study compared inter-rater agreement among scales, with the EAR scale proving the most reliable (Krippendorff's alpha coefficient, α = 0.45), outperforming existing measures. The outer-ear cartilage scale and the VAS exhibited lower inter-rater agreement, indicating inferiority in assessing aesthetic outcomes.

The EAR scale emerged as an effective tool for evaluating post-operative outcomes in congenital aural atresia auricular reconstruction.

To determine the prevalence of laryngeal muscle tension in patients with sinonasal diseases.

The medical records and video-recordings of patients with a history of sinonasal disease were reviewed to identify one of four muscle tension patterns during phonation. A control group with no history of sinonasal diseases was matched according to age and gender.

Seventy-seven patients were divided into a study group (n = 47) and a control group (n = 30). In the study group, 29 patients had at least one muscle tension pattern compared with only 9 in the control group (p = 0.007). The most common muscle tension patterns observed in the study and control groups were muscle tension patterns II and III. In the study group, 79.3 per cent of patients with at least one muscle tension pattern reported dysphonia compared with only 33.3 per cent in the control group.

Patients with sinonasal diseases are more likely to exhibit laryngeal muscle tension and dysphonia in comparison with healthy subjects.

For over a century, circumferential pharyngoesophageal junction reconstruction posed significant surgical challenges. This review aims to provide a narrative history of pharyngoesophageal junction reconstruction from early surgical innovations to the advent of modern free-flap procedures.

The review encompasses three segments: (1) local and/or locoregional flaps, (2) visceral transposition flaps, and (3) free-tissue transfer, focusing on the interplay between pharyngoesophageal junction reconstruction and prevalent surgical trends.

Before 1960, Mikulicz-Radecki's flaps and the Wookey technique prevailed for circumferential pharyngoesophageal junction reconstruction. Gastric pull-up and colonic interposition were favoured visceral techniques in the 1960s–1990s. Concurrently, deltopectoral and pectoralis major flaps were the preferred cutaneous methods. Free flaps (radial forearm, anterolateral thigh) revolutionised reconstructions in the late 1980s, yet gastric pull-up and free jejunal transfer remain in selective use.

Numerous pharyngoesophageal junction reconstructive methods have been trialled in the last century. Despite significant advancements in free-flap reconstruction, some older methods are still in use for challenging clinical situations.

Most otolaryngologists advocate absolute voice rest after laryngeal surgery, which proves difficult for patients, so we decided to evaluate the role of absolute voice rest versus relative voice rest in the post-operative management of benign lesions.

Forty patients were recruited and divided in two groups: absolute voice rest and relative voice rest. Pre- and post-operative voice analysis (fundamental frequency, jitter, shimmer, voice handicap index, voice-related quality-of-life scale scores and compliance) were noted at one week and one month.

Voice analysis parameters including jitter (p = 0.035), shimmer (p = 0.020), voice handicap index (p < 0.001) and compliance (p < 0.001) were better in the relative voice-rest group. Frequency, number of voice breaks and voice-related quality of life showed no statistically significant results.

There was no significant benefit of absolute voice rest on post-operative outcomes as determined by acoustic variables. Compliance and quality-of-life scores were low in the strict voice-rest group. Therefore, we should reconsider post-surgical voice-rest protocol.