Whole exome sequencing study identifies candidate loss of function variants and locus heterogeneity in familial cholesteatoma.

Cholesteatoma is a rare progressive disease of the middle ear. Most cases are sporadic, but some patients report a positive family history. Identifying functionally important gene variants associated with this disease has the potential to uncover the molecular basis of cholesteatoma pathology with implications for disease prevention, surveillance, or management.

Do patients report quality of life improvements after fitting of their unilateral bone conducting hearing implant?

Objective: The audiological benefits of a bone conducting hearing implant are well documented; however, there is a paucity of literature comparing pre- and post-operative quality of life benefits. This study assessed the quality of life status before and after the device is implanted.

Methods: A prospective study was conducted of all adult bone conducting hearing implants inserted in a teaching hospital between 2012 and 2017.

Cholesteatoma and family history: An international survey.

Objective: To explore the relative frequency of a family history of cholesteatoma in patients with known cholesteatoma, and whether bilateral disease or earlier diagnosis is more likely in those with a family history. Associations between cleft lip or palate and bilateral disease and age of diagnosis were also explored.

Design: An online survey of patients with diagnosed cholesteatoma was conducted between October 2017 and April 2019.

Cholesteatoma in children and adults: are there really any differences?

Background: Cholesteatoma is widely considered to be more aggressive in children than adults, yet few studies have directly compared the operative findings and surgical outcomes between these two groups. This study aimed to assess differences between childhood and adult cholesteatoma.

Methods: The operative caseload of a single consultant surgeon was reviewed between January 2006 and May 2017 using the online Common Otology Audit database.

Nasal oncocytoma causing unilateral epiphora in an elderly patient: the vital role of nasendoscopy.

Oncocytomas are rare glandular tumours, typically found in salivary glands and kidneys. A 76-year-old man presented to an ophthalmology department after 3 months of persistent right-sided epiphora. Lacrimal syringing and a fluorescein dye test demonstrated an obstruction at the level of the nasolacrimal duct (NLD).

The genetics of cholesteatoma. A systematic review using narrative synthesis.

Objective: A cholesteatoma is a mass of keratinising epithelium in the middle ear. It is a rare disorder that is associated with significant morbidity, and its causative risk factors are poorly understood; on a global scale, up to a million people are affected by this each year. We have conducted a systematic literature review to identify reports about the heritability of cholesteatoma or any constitutional genetic factors that may be associated with its aetiology.

Results of revision myringoplasty: are they different to those of primary myringoplasty?

Unlabelled: Audit question: Does revision myringoplasty have worse outcomes than primary surgery?

Methods: The International Otology Database has been used to record data on surgery for middle-ear disease in Norfolk, UK, over nine years. The data show the results of all myringoplasty cases and the results of revision cases. Outcome measures are perforation and hearing change.

Combined external and endonasal approach to fronto-ethmoidal mucocele involving the orbit.

Purpose: To present a technique to improve the surgical treatment of frontal sinus mucocele and its recurrence.

Methods: Nine procedures performed on eight patients by a team of ENT and Ophthalmic orbital surgeons. Data collected included patient demographics, surgical details, pathological findings and complications.

The burden of revision sinonasal surgery in the UK-data from the Chronic Rhinosinusitis Epidemiology Study (CRES): a cross-sectional study.

Objectives: The aim of this study was to investigate the surgical revision rate in patients with chronic rhinosinusitis (CRS) in the UK CRS Epidemiology Study (CRES). Previous evidence from National Sinonasal Audit showed that 1459 patients with CRS demonstrated a surgical revision rate 19.1% at 5 years, with highest rates seen in those with polyps (20.

Hearing loss following myringoplasty - implications for informed consent.

Background: There are many reports of operations performed to successfully close ear drum perforations. Hearing deterioration after myringoplasty is not a widely published topic. This paper presents an audit of this complication.

An audit of 'dead ear' after ear surgery.

Introduction: 'Dead ear' is a rare and serious complication of ear surgery. This paper presents an audit of this complication.

Method: Over 6 years, data for all 617 middle-ear operations performed under the care of a single consultant were recorded for the International Otology Audit.

An unusual headache: sphenoiditis in children and adolescents.

Sphenoid sinus infection is a rare cause of headaches in children and adolescents. Its symptoms are often non-specific and confusing. The diagnosis is made on the history, examination, nasendoscopy, cultures and CT or MRI.

Initial UK experience of patient satisfaction with the Meniett® device for Ménière's disease treatment.

Objectives: To evaluate patient satisfaction and symptom improvement following treatment of Ménière's disease with the Meniett® device.

Methods: Retrospective, questionnaire-based audit and analysis of unilateral Ménière's disease patients' records, following on from a previous study from our departments on intra-tympanic gentamicin for Ménière's disease, using the Vertigo Symptom Scale and Glasgow Benefit Inventory as outcome measures.

Results: Of 33 consecutive patients treated with the Meniett® device for four to six weeks, 30 responded to the questionnaires (90.

Tattooing and the risk of wrong-site ear surgery.

Wrong-site surgery could occur in cases of bilateral pathology or in patients with normal tympanic membranes such as those undergoing stapedectomy. This report highlights the pitfalls in current surgical checklists despite best efforts being put into their design. The practice of marking the earlobe in ear surgery may be less safe than using larger arrows on the neck to indicate the side of surgery.