Cochlear implant outcomes in patients with Meniere's disease: a large case series.

OBJECTIVE: To perform a matched cohort study to assess whether patients with Meniere's Disease (MD) require more intensive auditory rehabilitation following cochlear implantation (CI) and identify factors that may affect outcomes in patients with MD. METHODS: A retrospective case review was performed. MD and control patients were matched for age, biological sex, implant manufacturer and electrode design. Outcomes measured were speech scores, number of visits to audiology department following switch-on, and post-operative active MD. RESULTS: Forty consecutive implanted MD patients were identified between May 1993 and May 2019. Patients with active MD following CI required significantly more visits to the audiology department compared to controls (P < 0.01) and patients who had inactive MD post-operatively (P < 0.01). However, in MD patients, active MD was less likely following CI (P = 0.03). In patients who continued to experience active MD post-operatively, further medical and surgical ablative intervention was required to control ongoing Meniere's attacks. CONCLUSION: We present the largest case series of performance outcomes in CI patients with MD. Although speech outcomes in MD patients are comparable to controls, patients with active MD pre-operatively are more likely to experience variation in CI performance requiring a prolonged period of auditory rehabilitation compared to inactive preoperative MD.

Home energy efficiency and radon: An observational study.

Exposure to radon gas is the second leading cause of lung cancer worldwide behind smoking. Changing the energy characteristics of a dwelling can influence both its thermal and ventilative properties, which can affect indoor air quality. This study uses radon measurements made in 470 689 UK homes between 1980 and 2015, linked to dwelling information contained within the Home Energy Efficiency Database (HEED). The linked dataset, the largest of its kind, was used to analyze the association of housing and energy performance characteristics with indoor radon concentrations in the UK. The findings show that energy efficiency measures that increase the airtightness of properties are observed to have an adverse association with indoor radon levels. Homes with double glazing installed had radon measurements with a significantly higher geometric mean, 67% (95% CI: 44, 89) greater than those without a recorded fabric retrofit. Those with loft insulation (47%, 95% CI: 26, 69) and wall insulation (32%, 95% CI: 11, 53) were also found to have higher radon readings. Improving the energy performance of the UK's housing stock is vital in meeting carbon emission reduction targets. However, compromising indoor air quality must be avoided through careful assessment and implementation practices.

Outcomes in implanted teenagers who do not meet the adult candidacy criteria.

Severe to profoundly deaf adults who score 50% or over on the Bamford-Kowal-Bench (BKB) sentence test currently cannot obtain NHS funding for a cochlear implant according to the NICE guidelines (NICE Technical Appraisal Guidance (TAG166), 2009. Cochlear implants for children and adults with severe to profound deafness. NICE technology appraisal guidance [TAG166]. http://www.nice.org.uk/ta166 accessed 08/02/2016). There is no cut-off restriction from the BKB score for children. This study challenges this restrictive criteria for adults, by presenting the outcomes of cochlear implantation in older children who scored over 50% on BKB sentence testing pre-implantation and therefore would not have been implanted under the adult NICE guidelines. Outcomes are presented using the Speech, Spatial and Qualities of Hearing Scale Version C (SSQ-C) (Gatehouse, S., Noble, W. 2004. The Speech, Spatial and Qualities of Hearing Scale (SSQ). International Journal of Audiology, 43: 85-99.). This study suggests a greater proportion of adults who are currently being restricted from having a cochlear implant would benefit from implantation.

Outdoor wood furnaces create significant indoor particulate pollution in neighboring homes.

CONTEXT: The use of outdoor wood furnaces (OWFs) is common in many parts of the United States. Little published information exists on the concentrations of outdoor and indoor fine particulates found near OWFs. OBJECTIVE: To compare PM2.5 (cts) and PM0.5 (cts) particle concentrations inside four Connecticut homes located 30.5-259 m from OWFs, and inside six Connecticut control homes located more than 2 km from the nearest OWF. MATERIALS AND METHODS: PM2.5 (cts) and PM0.5 (cts) measurements were made with a Dylos light-scattering particulate counter. RESULTS: Mean PM2.5 (cts) concentrations were 4.21 times as great in the four OWF exposed homes than the six control homes (0.302 × 10(6) counts/m(3) versus 0.0718 counts × 10(6)/m(3) p < 0.001). The mean PM2.5 (cts) concentrations inside the four OWF exposed homes roughly corresponds to a mass PM2.5 of 37 µg/m(3), which is above the US EPA 24-h PM2.5 limit of 35 µg/m(3). Mean PM0.5 (cts) concentrations were 3.44 times as great in the four OWF exposed homes than in the six control homes (0.657 versus 0.191 × 10(6)/m(3) p < 0.001). Mean PM2.5 (cts) and PM0.5 (cts) concentrations were significantly higher in the house 259 m from an OWF as compared with the mean of the six control homes. CONCLUSION: Existing regulations, such as the present Connecticut law requiring a 61 meter distance between an OWF and neighboring homes, are not adequate to protect the health of neighboring residents.

Cochlear implant programming: a global survey on the state of the art.

The programming of CIs is essential for good performance. However, no Good Clinical Practice guidelines exist. This paper reports on the results of an inventory of the current practice worldwide. A questionnaire was distributed to 47 CI centers. They follow 47600 recipients in 17 countries and 5 continents. The results were discussed during a debate. Sixty-two percent of the results were verified through individual interviews during the following months. Most centers (72%) participated in a cross-sectional study logging 5 consecutive fitting sessions in 5 different recipients. Data indicate that general practice starts with a single switch-on session, followed by three monthly sessions, three quarterly sessions, and then annual sessions, all containing one hour of programming and testing. The main focus lies on setting maximum and, to a lesser extent, minimum current levels per electrode. These levels are often determined on a few electrodes and then extrapolated. They are mainly based on subjective loudness perception by the CI user and, to a lesser extent, on pure tone and speech audiometry. Objective measures play a small role as indication of the global MAP profile. Other MAP parameters are rarely modified. Measurable targets are only defined for pure tone audiometry. Huge variation exists between centers on all aspects of the fitting practice.

Variable long-term outcomes from cochlear implantation in children with hypoplastic auditory nerves.

This paper describes the outcomes in six children with hypoplastic cochlear nerves, demonstrated on magnetic resonance imaging who were selected for implantation because they had clear responses to sound, with hearing aids. In four of the six children the internal auditory canals were abnormally narrow; however in the remaining two the internal auditory canals were of normal width. After activation of their devices, all six children had auditory thresholds within the normal range for implanted congenitally deaf children. However the progress of all these children after 2 to 6 years of implant use turned out to be disappointing. Five of the children developed only the ability to detect the presence of sound, and were unable to discriminate accurately between speech sounds. They remain at Category of Auditory Performance (CAP) level 2: sign is their primary mode of communication. All five children also remain at Speech Intelligibility Rating (SIR) level 2, with unintelligible connected speech. The sixth child was able to develop some understanding of common phrases through listening alone, however he remains at CAP level 4 and his SIR level also remains at 2. The benefit of cochlear implantation in this group of children is likely to be considerably restricted. In specific cases it may be appropriate to offer a cochlear implant to a child with hypoplastic auditory nerves but careful counselling of the family about likely outcomes is essential.

Coordinating outcomes measurement in ataxia research: do some widely used generic rating scales tick the boxes?

The objective of this study was to examine the psychometric properties of four widely used generic health status measures in Friedreich's ataxia (FA), to determine their suitability as outcome measures. Fifty-six people with genetically confirmed FA completed the Barthel Index (BI), General Health Questionnaire (GHQ-12), EuroQol (EQ-5D), and Medical Outcomes Study 36-item Short Form Health Survey (SF-36) by means of postal survey. Six psychometric properties (data quality, scaling assumptions, acceptability, reliability, validity, and responsiveness) were examined. The response rate was 97%. In general, the psychometric properties of the four measures satisfied recommended criteria. However, closer examination highlighted limitations restricting their use for treatment trials. For example, the BI had high levels of missing data, EQ-5D had poor discriminant ability, and five SF-36 scales had high floor and/or ceiling effects. Most scale scores did not span the entire scale range, had means that differed notably from the scale mid-point, and had wide confidence intervals. Effect sizes (ES) were small for all four measures raising questions about their ability to detect clinically significant change. Results highlight the potential limitations of these four scales for evaluating health outcomes in FA and suggest the need for new disease-specific patient-based measures of its impact.

Antioxidant treatment of patients with Friedreich ataxia: four-year follow-up.

BACKGROUND: Decreased mitochondrial respiratory chain function and increased oxidative stress have been implicated in the pathogenesis of Friedreich ataxia (FRDA), raising the possibility that energy enhancement and antioxidant therapies may be an effective treatment. OBJECTIVE: To evaluate the long-term efficacy of a combined antioxidant and mitochondrial enhancement therapy on the bioenergetics and clinical course of FRDA. DESIGN: Open-labeled pilot trial over 47 months.Patients Seventy-seven patients with clinical and genetically defined FRDA. Intervention A combined coenzyme Q(10) (400 mg/d) and vitamin E (2100 IU/d) therapy of 10 patients with FRDA over 47 months. MAIN OUTCOME MEASURES: Clinical assessment using echocardiography and the International Cooperative Ataxia Rating Scale and cardiac and skeletal muscle bioenergetics as assessed using phosphorus P 31 magnetic resonance spectroscopy. RESULTS: There was a significant improvement in cardiac and skeletal muscle bioenergetics that was maintained throughout the 47 months of therapy. Echocardiographic data revealed significantly increased fractional shortening at the 35- and 47-month time points. Comparison with cross-sectional data from 77 patients with FRDA indicated the changes in total International Cooperative Ataxia Rating Scale and kinetic scores over the trial period were better than predicted for 7 patients, but the posture and gait and hand dexterity scores progressed as predicted. CONCLUSION: This therapy resulted in sustained improvement in mitochondrial energy synthesis that was associated with a slowing of the progression of certain clinical features and a significant improvement in cardiac function.