Quality of life of Nepali patients with ear disease before and after corrective surgery.

OBJECTIVES: Hearing impairment is a significant burden in the developing world. However, no suitable quality of life (QoL) measures exist for use in Nepal. We aimed to amend and translate the Glasgow Health Status Inventory (GHSI), assessing QoL at any given time, and the Glasgow Benefit Inventory (GBI), assessing change in QoL following intervention, into Nepali and to assess the impact of ear disease and effect of surgery on QoL. METHODS: The GHSI and GBI were translated into Nepali and independently verified. The GHSI was administered by interview to patients before surgery, and the GBI was administered 6 months after surgery. The Mann-Whitney U-test was used for hypothesis testing. RESULTS: The GHSI was administered to 242 patients. In total, 205 had chronic suppurative otitis media (CSOM) without cholesteatoma and 37 had cholesteatoma. The mean GHSI score was 47.9. There was no significant difference in GHSI scores between patients with CSOM without cholesteatoma and those with cholesteatoma. The GBI was administered to 161 patients, 73 of whom had also been in the GHSI group. In total, 130 had CSOM without cholesteatoma, 31 had cholesteatoma. The mean GBI score was +38.4 with no significant difference between disease groups. CONCLUSIONS: Ear disease in Nepal is associated with reduced QoL, and surgical intervention is associated with improved QoL. There is no difference in QoL or benefit following surgery for CSOM between patients with or without cholesteatoma. There are few QoL measures suitable for the developing world. It is essential to invest in these measures to guide health interventions.

Cochlear Implantation in Neurofibromatosis Type 2: Experience From the UK Neurofibromatosis Type 2 Service.

OBJECTIVE: To review the outcomes of cochlear implants (CI) in patients with neurofibromatosis type 2 (NF2) in a large cohort, and identify factors associated with poor hearing benefit. STUDY DESIGN: Fifteen-year retrospective national observational case series. SETTING: United Kingdom regional NF2 multidisciplinary teams. PATIENTS: Consecutive patients with NF2 receiving a CI. INTERVENTIONS: CI for hearing rehabilitation. MAIN OUTCOME MEASURES: 1) Audiometric performance at 9 to 12 months after implantation using City University of New York (CUNY) sentence recognition score, and Bamford- Kowal-Bench (BKB) word recognition score in quiet (BKBq), and in noise (BKBn). 2) CI use at most recent review. RESULTS: Sixty four consecutive patients, median age 43 years, were included. Nine to 12 months mean audiometric scores were: CUNY 60.9%, BKBq 45.8%, BKBn 41.6%. There was no difference in audiometric outcomes between VS treatment modalities. At most recent review (median 3.6 years from implantation), 84.9% with device in situ/available data were full or part-time users. Between 9 and 12 months and most recent review there was an interval reduction in mean audiometric scores: CUNY -12.9%, BKBq -3.3%, BKBn -4.9%. Larger tumor size and shorter duration of profound hearing loss were the only variables associated with poorer audiometric scores. Tumor growth at the time of surgery was the only variable associated with CI non-use. Individual patient response was highly variable. CONCLUSIONS: CI can provide significant and sustained auditory benefits to patients with NF2 independent of tumor treatment modality, with the majority of those implanted becoming at least part-time users. Larger datasets are required to reliably assess the role of independent variables.

ENT Quality Improvement Program as a tool to improve the collection of morbidity and mortality data: a multisite audit carried out over 6 months.

OBJECTIVE: This project aims to assess the role of a standardised process of data collection to improve morbidity and mortality data across the region. DESIGN: Six hospitals within the North West (UK) were recruited and adopted the ENT Quality Improvement Program (QIP) into their daily practice. Monthly anonymous data were sent back to the reviewer for trend analysis. OUTCOME MEASURES: Four outcome measures were defined: (1) number of cases recorded within the region each month; (2) assessment of the severity of cases and trends; (3) assessment of action plans reviewing any changes in practice made as a result of using this tool; (4) long-term use of the tool and qualitative feedback from units. RESULTS: 162 patients over the 6 months were included with 180 case discussions. 170 of these were morbidities and 10 were mortalities. Mortality was more frequent in patients with a diagnosis of head and neck cancer. Of the 162 patients, 133 encountered postoperative complications. Post-tonsillectomy (62/133 47%) and post-thyroid surgery (19/133 14%) complications were the most frequently encountered. 66% of the complications were low grade with 18% requiring management under general anaesthetic. Actions plans included four policy reviews with the introduction of three new policies. All sites found the tool user-friendly and are continuing to use it beyond the data collection period. CONCLUSIONS: The ENT QIP has been found to be a simple, user-friendly tool which has improved the quality of data over the six sites and resulted in improvements in practice. Implementation of the tool allows clinicians to critically appraise their practice and to reflect as well as to demonstrate how complications have resulted in change.

I-Cell Disease (Mucolipidosis II): A Case Series from a Tertiary Paediatric Centre Reviewing the Airway and Respiratory Consequences of the Disease.

BACKGROUND: Inclusion cell disease (I-cell) is a rare autosomal recessive metabolic disease involving multiple organ systems, associated with a severely restricted life expectancy. No curative therapy is currently available, with management aimed at symptom palliation. METHODS: We present a retrospective, single-centre, case series of children referred to a tertiary paediatric metabolic service. The clinical presentation, demographics, genetics and natural history of the condition are investigated. RESULTS: Five patients with I-cell disease were referred over a 10-year period. All patients were born with dysmorphic features and had a family history of I-cell disease on further exploration. Phenotypic variation was seen within patients with the same genetic profile. Airway problems were common with 100% of the documented sleep oximetry studies suggesting sleep-disordered breathing. Of the two patients who had tracheal intubation anaesthetic difficulties we encountered, one required intraoperative reintubation, and one suffered a failed intubation with subsequent death. All five patients required oxygen therapy with the use of CPAP and BiPAP also seen. Feeding issues were almost universal with four of the five patients requiring nasogastric feeding. Four patients had died in the 10-year period with a mean life expectancy of 36 months. Cause of death for three of the four patients was respiratory failure. CONCLUSIONS: Airway problems, including sleep-disordered breathing, were ubiquitous in this cohort of children. Any intervention requiring a general anaesthetic needs careful multidisciplinary consideration due to significant associated risks and possibly death. Management as a result is generally non-surgical and symptomatic. This case series demonstrates universal involvement of the airway and respiratory systems, an important consideration when selecting meaningful outcomes for future effectiveness studies of novel therapies.

The trumpet player with a swelling in the neck.

Bilateral neck swelling in patients following valsalva manouveres could lead to a diagnosis of either a pharyngocele or laryngocele. Distinguishing between them can be complicated but is vital given the possibility for an acute airway in patients with laryngoceles. A 20-year-old trumpet player presents with a 5-year history of neck swelling. Clinical suspicion is that of a pharyngocele but imaging introduces some confusion with the diagnosis. Both pharyngoceles and laryngoceles can occur as a result of prolonged positive pressure. Accurate assessment with fibreoptic examination and imaging is needed to confirm the diagnosis. Pharyngoceles are often misdiagnosed as laryngoceles. Though treatment is similar between the two patient groups it is vital that a distinction is made to enable careful observation of the airway in patients with laryngoceles.

Early Experience with a Hepatobiliary and Pancreatic Quality Improvement Program.

This project aims to assess the impact of the introduction of a hepatobiliary and pancreatic (HPB) Quality Improvement Program (QIP) on postoperative complications following liver, biliary and pancreatic surgery. A prospective analysis of postoperative complications over a six month period was performed. Complications were analysed and graded according to internationally agreed definitions. Justification was sought and errors identified. Weekly meetings were performed to review each complication enabling an action plan to be created to prevent future recurrence. Rates were compared with previously audited and published results, using the chi-square test. A total of 326 procedures were performed over the six months including 30 pancreatectomies, 45 liver resections and 251 other procedures. 37 patients developed complications (11.3%) with 47 complications in total including two deaths. Using the ISGPS grading, eight complications were identified; two grade A, four grade B and two grade C. There were three grade A ISGLS complications and one grade B. 30 complications were justified as unavoidable, 16 as avoidable and one as indeterminate. Action plans included continued monitoring (n=41), formulation of new policy (n=3), individual counselling (n=4), educational offering (n=4). When compared with 2010 complication rates, 114 complications occurred in 233 liver operations during the baseline period, compared with 17 complications in 45 liver operations during the QIP period, OR=0.63 (95% CI: 0.33 to 1.22), p=0.17 and 86 complications occurred in 99 pancreatic resections during the baseline period, compared with 20 complications in 30 pancreatic resections during the QIP period OR=0.30 (95% CI: 0.12 to 0.79), p=0.01 The HPB QIP is a rigorous approach to grade every complication and death. A statistically significant reduction in pancreas related complications has already been obtained. Further work is required to determine the persistence and magnitude of this quality improvement.