Hard to Swallow Results.

The American Neurogastroenterology and Motility Society (ANMS) proposed quality measures (QMs) for performance and interpretation of esophageal manometry (EM). We implemented a quality improvement (QI) study at a large community hospital to assess and improve procedural adherence and interpretation of EM studies based on the ANMS QMs using the Chicago Classification 3.0 (CC) Guidelines. For pre-intervention, three motility independent reviewers reinterpreted 60 EM studies conducted by community gastroenterologists without Tier II-III motility training from October to December 2018 for compliance with pre-procedural, procedural, and data interpretation ANMS QMs. In December 2018, we developed a pre-procedural form, educated nurses on EM procedural compliance, and provided preliminary pre-intervention results to gastroenterologists along with literature utilizing the CC 3.0 Guidelines. For post-intervention, we reinterpreted 54 EM studies from January to August 2019 and investigated whether they met QMs for data interpretation with respect to the CC Guidelines and resulted in appropriate treatment. We found a statistically significant improvement in procedural compliance among nursing staff for 30 s of swallows (76% post-intervention versus 12% pre-intervention, p < 0.001) and 7 evaluable swallows (94% post-intervention versus 53% pre-intervention, p < 0.001). However, quality metrics within data interpretation by physicians post-intervention showed mixed results. An incorrect diagnosis was made in 50% (n = 27)) of studies with 72% (n = 39) having at least one missing item based on the CC. The most missed diagnosis was fragmented peristalsis (30%, n = 29). Among the 39% (n = 21) of surgery referrals, 24% (n = 5) were incorrectly referred. Our study shows poor data interpretation by community gastroenterologists without formal motility training despite adequate performance by nursing staff. This further supports the need for a national ANMS certification process for formal HRM education.

International survey of cochlear implant candidacy.

BACKGROUND: The goal of this work was to determine international differences in candidacy based on audiometric and speech perception measures, and to evaluate the information in light of the funding structure and access to implants within different countries. METHOD: An online questionnaire was circulated to professionals in 25 countries. There were 28 respondents, representing the candidacy practice in 17 countries. RESULTS: Results showed differences in the funding model between countries. Unilateral implants for both adults and children and bilateral implants for children were covered by national funding in approximately 60% of countries (30% used medical insurance, and 10% self-funding). Fewer countries provided bilateral implants routinely for adults: national funding was available in only 22% (37% used medical insurance and 41% self-funding). Main evolving candidacy areas are asymmetric losses, auditory neuropathy spectrum disorders and electro-acoustic stimulation. For countries using speech-based adult candidacy assessments, the majority (40%) used word tests, 24% used sentence tests, and 36% used a mixture of both. For countries using audiometry for candidacy (70-80% of countries), the majority used levels of 75-85 dB HL at frequencies above 1 kHz. The United Kingdom and Belgium had the most conservative audiometric criteria, and countries such as Australia, Germany, and Italy were the most lenient. Countries with a purely self-funding model had greater flexibility in candidacy requirements.

Comparing the epidemiology of hospital-acquired methicillin-resistant Staphylococcus aureus clone groups in Alberta, Canada.

Patients with methicillin-resistant Staphylococcus aureus (MRSA) clones, which were traditionally seen in the community setting (USA400/CMRSA7 and USA300/CMRSA10), are often identified as hospital-acquired (HA) infections using Infection Prevention and Control (IPC) surveillance definitions. This study examined the demographics and healthcare risk factors of patients with HA-MRSA to help understand if community MRSA clones are from a source internal or external to the hospital setting. Despite USA300/CMRSA10 being the predominant clone in Alberta, hospital clones (USA100/CMRSA2) still dominated in the acute care setting. In the Alberta hospitalized population, patients with USA400/CMRSA7 and USA300/CMRSA10 clones were significantly younger, had fewer comorbidities, and a greater proportion had none or ambulatory care-only healthcare exposure. These findings suggest that there are two distinct populations of HA-MRSA patients, and the patients with USA400/CMRSA7 and USA300/CMRSA10 clones identified in hospital more greatly resemble patients affected by those clones in the community. It is possible that epidemiological assessment overidentifies HA acquisition of MRSA in patients unscreened for MRSA on admission to acute care.

Cochlear-implanted children from homes where English is an additional language: findings from a recent audit in one London centre.

A 5-year retrospective audit of demographic, audiological, and other records of 147 children implanted at one London centre was conducted. The aim was to detail the number of children implanted, with a specific focus on children from families with English as an additional language (EAL), and to compare these children with children from monolingual English-speaking families on a variety of characteristics known to affect paediatric cochlear implant outcomes. In all, 28% of children were from families where English is an additional language, with 15 different languages recorded. There were no differences between EAL and English-speaking children with respect to age of implantation; bilateral versus unilateral implants or hearing levels in better ear. There were differences between these groups in aetiology, in the occurrence of additional needs, and in educational placements. Information about speech and language outcomes was difficult to gather. Conclusions indicate the need for more detailed record-keeping especially about children's home languages for purposes of planning intervention and for the inclusion of children with EAL in future studies.

A case of linear epidermal naevus presenting as genital warts--a cautionary tale.

Linear epidermal naevus (LEN) in the genital area is quite rare. It may present at birth or appear later on in life, in infancy or childhood and occasionally for the first time in adult life. There are several variants of epidermal naevi (EN), which, to the less experienced, can be mistaken for warts. When extensive, it can be associated with abnormalities in other organ systems (epidermal naevus syndrome). The definitive treatment of LEN is surgical ablation with excision of underlying dermis, but this frequently leads to scarring. Laser therapy is an alternative treatment modality and good results have been shown. We report an unusual case of LEN in the genital area in a 60-year-old man presenting as genital warts.

Effect of 24 months of recombinant growth hormone on height and body proportions in SHOX haploinsufficiency.

Leri-Weill syndrome (LWS) is a skeletal dysplasia with mesomelic short stature, bilateral Madelung deformity (BMD) and SHOX (short stature homeobox-containing gene) haploinsufficiency. The effect of 24 months of recombinant human growth hormone (rhGH) therapy on the stature and BMD of two females with SHOX haploinsufficiency (demonstrated by fluorescence in situ hybridisation) and LWS was evaluated. Both patients demonstrated an increase in height standard deviation score (SDS) and height velocity SDS over the 24 months of therapy. Patient 1 demonstrated a relative increase in arm-span and upper segment measurements with rhGH while patient 2 demonstrated a relative increase in lower limb length. There was appropriate advancement of bone age, no adverse events and no significant deterioration in BMD. In this study, 24 months of rhGH was a safe and effective therapy for the disproportionate short stature of SHOX haploinsufficiency, with no clinical deterioration of BMD.

Familial growth and skeletal features associated with SHOX haploinsufficiency.

This study was designed to determine the intrafamilial effect of SHOX haploinsufficiency on stature, by comparing the growth and phenotype of 26 SHOX haploinsufficient individuals with 45 relatives and population standards. It confirmed that SHOX haploinsufficiency leads to growth restriction from birth to final height. Compared to unaffected siblings, the SHOX haploinsufficient cohort was 2.14 SDS (3.8 cm) shorter at birth and 2.1 SDS shorter through childhood. At final height females were 2.4 SDS (14.4 cm) shorter and males 0.8 SDS (5.3 cm) shorter than normal siblings. The family height analysis suggests that the effect of SHOX haploinsufficiency on growth may have been previously underestimated at birth and overestimated in males at final height. SHOX haploinsufficiency leads to short arms in 92%, bilateral Madelung deformity in 73% and short stature in 54%. Females were more severely affected than males. We conclude that SHOX is a major growth gene and that mutations are associated with a broad range of phenotype.

Decision making and memory: a critique of Juslin and Olsson's (1997) sampling model of sensory discrimination.

P. Juslin and H. Olsson's (1997) distinction between Thurstonian and Brunswikian uncertainty is examined and their sampling model of sensory discrimination analyzed as a representative of the class of memoryless decision processes. The separate characteristics and combined behavior of 4 main components of the model are explored: (a) the basic decision process, (b) the assumption of deadline responding, (c) the moving window model of memory, and (d) the hypothesized basis for confidence. It is argued that grafting a moving window memory onto a memoryless decision process has several undesirable consequences. Moreover, the suggested basis for confidence leads to predictions that are counterintuitive and unsupported by empirical evidence. It is concluded that the window-sampling model is a maladapted combination of inappropriate elements, which is implausible as a model of decision making, memory, or confidence, in sensory discrimination.

Histopathological analysis of Leri-Weill dyschondrosteosis: disordered growth plate.

Leri-Weill syndrome (LWS) is a dominant (pseudoautosomal) skeletal dysplasia with mesomelic short stature and bilateral Madelung deformity, due to dyschondrosteosis of the distal radius. It results from the loss of one copy of the Short Stature Homeobox Gene (SHOX) from the tip of the short arm of the X or Y chromosome. SHOX molecular testing enabled us to evaluate the histopathology of the radial physis in LWS patients with a documented SHOX abnormality. A widespread disorganisation of physeal anatomy was revealed with disruption of the normal parallel columnar arrangement of chondrocytes. Tandem stacking of maturing chondrocytes within columns was replaced by a side-by-side arrangement. The presence of hypertrophic osteoid with micro-enchondromata in the radial metaphysis suggests abnormal endochondral ossification. The Vickers' ligament was confirmed to blend with the triangular fibrocartilage complex (TFCC). This histopathological study demonstrates that the zone of dyschondrosteosis in LWS is characterised by marked disruption of normal physeal chondrocyte processes and that a generalised physeal abnormality is present.

Human performance on visually presented Traveling Salesman problems.

Little research has been carried out on human performance in optimization problems, such as the Traveling Salesman problem (TSP). Studies by Polivanova (1974, Voprosy Psikhologii, 4, 41-51) and by MacGregor and Ormerod (1996, Perception & Psychophysics, 58, 527-539) suggest that: (1) the complexity of solutions to visually presented TSPs depends on the number of points on the convex hull; and (2) the perception of optimal structure is an innate tendency of the visual system, not subject to individual differences. Results are reported from two experiments. In the first, measures of the total length and completion speed of pathways, and a measure of path uncertainty were compared with optimal solutions produced by an elastic net algorithm and by several heuristic methods. Performance was also compared under instructions to draw the shortest or the most attractive pathway. In the second, various measures of performance were compared with scores on Raven's advanced progressive matrices (APM). The number of points on the convex hull did not determine the relative optimality of solutions, although both this factor and the total number of points influenced solution speed and path uncertainty. Subjects' solutions showed appreciable individual differences, which had a strong correlation with APM scores. The relation between perceptual organization and the process of solving visually presented TSPs is briefly discussed, as is the potential of optimization for providing a conceptual framework for the study of intelligence.

Effects of low-pass filtering on the intelligibility of speech in quiet for people with and without dead regions at high frequencies.

A dead region is a region of the cochlea where there are no functioning inner hair cells (IHCs) and/or neurons; it can be characterized in terms of the characteristic frequencies of the IHCs bordering that region. We examined the effect of high-frequency amplification on speech perception for subjects with high-frequency hearing loss with and without dead regions. The limits of any dead regions were defined by measuring psychophysical tuning curves and were confirmed using the TEN test described in Moore et al. [Br. J. Audiol. 34, 205-224 (2000)]. The speech stimuli were vowel-consonant-vowel (VCV) nonsense syllables, using one of three vowels (/i/, /a/, and /u/) and 21 different consonants. In a baseline condition, subjects were tested using broadband stimuli with a nominal input level of 65 dB SPL. Prior to presentation via Sennheiser HD580 earphones, the stimuli were subjected to the frequency-gain characteristic prescribed by the "Cambridge" formula, which is intended to give speech at 65 dB SPL the same overall loudness as for a normal listener, and to make the average loudness of the speech the same for each critical band over the frequency range important for speech intelligibility (in a listener without a dead region). The stimuli for all other conditions were initially subjected to this same frequency-gain characteristic. Then, the speech was low-pass filtered with various cutoff frequencies. For subjects without dead regions, performance generally improved progressively with increasing cutoff frequency. This indicates that they benefited from high-frequency information. For subjects with dead regions, two patterns of performance were observed. For most subjects, performance improved with increasing cutoff frequency until the cutoff frequency was somewhat above the estimated edge frequency of the dead region, but hardly changed with further increases. For a few subjects, performance initially improved with increasing cutoff frequency and then worsened with further increases, although the worsening was significant only for one subject. The results have important implications for the fitting of hearing aids.

The relative role of beats and combination tones in determining the shapes of masking patterns at 2 kHz: I. Normal-hearing listeners.

Masking patterns for a 2-kHz sinusoidal masker at 45, 65 or 85 dB SPL were measured for three normal-hearing subjects, using a 3-AFC method with feedback (condition 1). The patterns showed distinct irregularities, particularly at the highest masker level. In condition 2, a lowpass noise was added to mask combination tones. The noise increased thresholds mainly for the 85 dB masker, for signal frequencies of 2.3-3.0 kHz. In condition 3, a pair of high-frequency tones ('modulation detection interference (MDI) tones') was used to introduce beats at the same rate as produced by the interaction of the masker and signal. Thresholds were higher than for condition 1, particularly for signal frequencies adjacent to the masker frequency. In condition 4, the lowpass noise was presented simultaneously with the MDI tones. Thresholds were well predicted as a combination of the effects of the lowpass noise and the MDI tones. In condition 5, a pair of low-frequency MDI tones was added to the masker. The thresholds had the same overall pattern as in condition 4. We conclude that the shapes of masking patterns measured using a 2-kHz masker are influenced by the detection of beats for masker-signal frequency separations up to at least 300 Hz and by the detection of combination tones for separations between 300 and 1000 Hz.

A test for the diagnosis of dead regions in the cochlea.

Hearing impairment may sometimes be associated with complete loss of inner hair cells (IHCs) over a certain region of the basilar membrane. We call this a 'dead region'. Amplification (using a hearing aid) over a frequency range corresponding to a dead region may not be beneficial and may even impair speech intelligibility. However, diagnosis of dead regions is not easily done from the audiogram. This paper reports the design and evaluation of a method for detecting and delimiting dead regions. A noise, called 'threshold equalizing noise' (TEN), was spectrally shaped so that, for normally hearing subjects, it would give equal masked thresholds for pure tone signals at all frequencies within the range 250-10,000 Hz. Its level is specified as the level in a one-ERB (132 Hz) wide band centred at 1000 Hz. Measurements obtained from 22 normal-hearing subjects and TEN levels of 30, 50 and 70 dB/ERB confirmed that the signal level at masked threshold was approximately equal to the noise level/ERB and was almost independent of signal frequency. Masked thresholds were measured for 20 ears of 14 subjects with sensorineural hearing loss, using TEN levels of 30, 50 and 70 dB/ERB. Psychophysical tuning curves (PTCs) were measured for the same subjects. When there are surviving IHCs corresponding to a frequency region with elevated absolute thresholds, a signal in that frequency region is detected via IHCs with characteristic frequencies (CFs) close to that region. In such a case, threshold in the TEN is close to that for normal-hearing listeners, provided that the noise intensity is sufficient to produce significant masking. Also, the tip of the PTC lies close to the signal frequency. When a dead region is present, the signal is detected via IHCs with CFs different from that of the signal frequency. In such a case, threshold in the TEN is markedly higher than normal, and the tip of the PTC is shifted away from the signal frequency. Generally, there was a very good correspondence between the results obtained using the TEN and the PTCs. We conclude that the measurement of masked thresholds in TEN provides a quick and simple method for the diagnosis of dead regions.

The importance of the convex hull for human performance on the traveling salesman problem: a comment on MacGregor and Ormerod (1996)

MacGregor and Ormerod (1996) have presented results purporting to show that human performance on visually presented traveling salesman problems, as indexed by a measure of response uncertainty, is strongly determined by the number of points in the stimulus array falling inside the convex hull, as distinct from the total number of points. It is argued that this conclusion is artifactually determined by their constrained procedure for stimulus construction, and, even if true, would be limited to arrays with fewer than around 50 points.

A prospective randomized trial comparing four biofeedback techniques for patients with faecal incontinence.

OBJECTIVE: The aim of this study was to compare four methods of biofeedback therapy for patients with faecal incontinence (FI). PATIENTS AND METHODS: All patients with FI who were ineligible for surgical management were prospectively randomized using a computer generated randomization method into one of four protocols: 1, out-patient intra-anal electromyographic biofeedback training (EMG); 2, EMG plus intrarectal balloon training (BT); 3, EMG plus a home trainer (HT); and 4, EMG, BT and HT. All patients received weekly, 1 h, out-patient biofeedback training. Success for patients with FI was measured by a reduction in incontinent episodes (days/week). In all instances, patients maintained a daily log in which documentation was recorded regarding each bowel evacuation. RESULTS: Forty patients were randomized into one of the four groups. Six patients withdrew after one session and were not included in the analysis. Therefore, 34 patients (23 female and 11 male) with a mean incontinence score of 12 (range 7-14) were randomized to one of the four groups (n=8, 8, 8, and 10, respectively). There was a statistically significant reduction in incontinent episodes for all groups. However, there were no significant differences in treatment outcome found in comparisons among the four groups. CONCLUSION: Biofeedback therapy significantly improves FI. Moreover, EMG training was as effective alone as was the addition of HT, BT or both for the treatment of FI.

Prospective, randomized trial comparing four biofeedback techniques for patients with constipation.

PURPOSE: The aim of this study was to compare four methods of biofeedback for patients with constipation. METHODS: Thirty-six patients were prospectively, randomly assigned to one of four protocols: 1) outpatient intra-anal electromyographic biofeedback training; 2) electromyographic biofeedback training plus intrarectal balloon training; 3) electromyographic biofeedback training plus home training; or 4) electromyographic biofeedback training, balloon training, and home training. All 36 patients received weekly one-hour outpatient biofeedback training. Success was measured by increased unassisted bowel movements and reduction in cathartic use. In all instances patients maintained a daily log in which documentation was maintained regarding each bowel evacuation and the need for any cathartics. RESULTS; There was a statistically significant increase in unassisted bowel movements for Groups 1, 2, and 4 (P < 0.05) and a reduction in the use of cathartics in Groups 1, 2, and 3 (P < 0.05). CONCLUSION: There was a significant improvement in outcome after all four treatment protocols for constipation; however, no significant difference was found among the treatments. Therefore, electromyographic biofeedback training alone is as effective as with the addition of balloon training, home training, or both.

Inter-relationship between different psychoacoustic measures assumed to be related to the cochlear active mechanism.

The active mechanism in the cochlea is thought to depend on the integrity of the outer hair cells (OHCs). Cochlear hearing loss is usually associated with damage to both inner hair cells (IHCs) and OHCs, with the latter resulting in a reduction in or complete loss of the function of the active mechanism. It is believed that the active mechanism contributes to the sharpness of tuning on the basilar membrane (BM) and is also responsible for compressive input-output functions on the BM. Hence, one would expect a close relationship between measures of sharpness of tuning and measures of compression. This idea was tested by comparing three different measures of the status of the active mechanism, at center frequencies of 2, 4, and 6 kHz, using subjects with normal hearing, with unilateral or highly asymmetric cochlear hearing loss, and with bilateral loss. The first measure, HLOHC, was an indirect measure of the amount of the hearing loss attributable to OHC damage; this was based on loudness matches between the two ears of subjects with unilateral hearing loss and was derived using a loudness model. The second measure was the equivalent rectangular bandwidth (ERB) of the auditory filter, which was estimated using the notched-noise method. The third measure was based on the slopes of growth-of-masking functions obtained in forward masking. The ratio of slopes for a masker centered well below the signal frequency and a masker centered at the signal frequency gives a measure of BM compression at the place corresponding to the signal frequency; a ratio close to 1 indicates little or no compression, while ratios less than 1 indicate that compression is occurring at the signal place. Generally, the results showed the expected pattern. The ERB tended to increase with increasing HLOHC. The ratio of the forward-masking slopes increased from about 0.3 to about 1 as HLOHC increased from 0 to 55 dB. The ratio of the slopes was highly correlated with the ERB (r = 0.92), indicating that the sharpness of the auditory filter decreases as the compression on the BM decreases.