Cochlear Implantation in Brown-Vialetto-Van-Laere syndrome.

OBJECTIVE: To report outcomes for the first known cochlear implantation procedures in two patients with Brown-Vialetto-Van-Laere syndrome. PATIENTS: Two adult patients (a brother and sister) with post-lingual sensorineural deafness associated with Brown-Vialetto-Van-Laere syndrome. The female patient presented with a milder form of the syndrome. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURE: Post-implantation speech discrimination scores. RESULTS: Auditory evoked potential testing suggested pathological changes in both patients' cochleae, auditory nerves, brainstem and (probably) central auditory pathways. In the male patient, despite implantation of the better ear, the Bamford-Kowal-Bench sentence score was zero at 21 months post-implantation. In the female patient, Bamford-Kowal-Bench sentence scores at six months post-implantation were 25 per cent in quiet and 3 per cent in noise. CONCLUSION: These poor clinical outcomes appear to be related to retrocochlear and probable central auditory pathway degeneration.

Proposals that the International Committee on Systematics of Prokaryotes establish a public database of validly published names and that the Bacteriological Code be amended to change the prescription for citation of validly published names.

It is proposed that the International Committee on Systematics of Prokaryotes formally maintain a readily accessible database of validly published names and that Rule 33b of the International Code of Nomenclature of Bacteria be revised to allow a new, abbreviated, format as the sole method by which authors indicate validation of previously published names.

Auditory sensory memory and working memory processes in children with normal hearing and cochlear implants.

There can be wide variation in the level of oral/aural language ability that prelingually hearing-impaired children develop after cochlear implantation. Automatic perceptual processing mechanisms have come under increasing scrutiny in attempts to explain this variation. Using mismatch negativity methods, this study explored associations between auditory sensory memory mechanisms and verbal working memory function in children with cochlear implants and a group of hearing controls of similar age. Whilst clear relationships were observed in the hearing children between mismatch activation and working memory measures, this association appeared to be disrupted in the implant children. These findings would fit with the proposal that early auditory deprivation and a degraded auditory signal can cause changes in the processes underpinning the development of oral/aural language skills in prelingually hearing-impaired children with cochlear implants and thus alter their developmental trajectory.

Proposal that Agrobacterium radiobacter has priority over Agrobacterium tumefaciens. Request for an opinion.

It is proposed that Agrobacterium radiobacter has priority as the earlier heterotypic (subjective) synonym when it is united with Agrobacterium tumefaciens. The nomenclatural status of A. tumefaciens as a later heterotypic synonym of the united species is not lost and it remains the type species of the genus. Request for an opinion.

Reconsideration of Arthrobacter ilicis (Mandel et al. 1961) Collins et al. 1982 as a plant-pathogenic species. Proposal to emend the authority and description of the species. Request for an opinion.

Strains now considered to represent the type strain of Arthrobacter ilicis, described as a pathogen of American holly, are not identical. The designated type strain does not represent this pathogen. However, one of the other strains sourced to the type strain of the pathogen does appear to be authentic, but is not a member of A. ilicis. It is proposed that A. ilicis is an unrelated species, not a pathogen of American holly. The nomenclature of A. ilicis can be rectified by emending the authority and by emending the species description to recognize this species as a novel species that is not a plant pathogen. The pathogen of American holly then becomes a novel pathovar, Curtobacterium flaccumfaciens pv. ilicis. The opinion of the Judicial Commission is sought.

The impact of impaired vocal quality on children's ability to process spoken language.

This paper investigated the effect of voice quality on children's ability to process spoken language. A group of 24 children, mean age 11 years 5 months, listened to a series of recorded short passages, half spoken by a female with normal voice and half spoken by a female with a classic vocal impairment (dysphonic voice). The children were tested for their ability to recall words and to draw a final target inference. Children performed better on both preceding indices when listening to the normal voice. The implications of the findings are discussed, with particular reference to the classroom situation.

The clinical and financial impact of port-access coronary revascularization.

OBJECTIVE: Port-access coronary bypass grafting (CABG)was performed in an attempt to impact the clinical course of patients with coronary artery disease. METHODS: One hundred patients (56 men and 44 women) with a median age of 61 years underwent port-access coronary revascularization. The clinical and financial profiles of these patients were compared with fiscal year 1997 patients (n = 531) who underwent standard median sternotomy coronary bypass. RESULTS: Preoperative clinical demographics were similar in both groups of patients. Among the port-access population there were no incidences of aortic dissection, deep vein thrombosis, conversion to median sternotomy, or death. Total time in the Intensive Care Unit (ICU), incidence of atrial fibrillation, transfusion requirements, and (subjective) pain rating at 28 days postoperatively were less in the port-access group. The average hospital cost per case was $2703.00 (US dollars) more in the port-access patients, despite a similar length of stay versus conventional sternotomy patients. CONCLUSIONS: Coronary bypass surgery can be performed safely with port-access technology with significant clinical benefits in selected patients. Currently these benefits are attained at a significant cost to the institution.

Modification of port-access coronary artery bypass in high-risk patients.

BACKGROUND: Minimally invasive port-access coronary artery bypass surgery has many potential advantages over routine median sternotomy coronary revascularization in patients with serious co-morbid conditions. The common femoral artery and vein have been the standard peripheral cannulation and balloon deployment sites. However, these sites present some risk, especially from proximal arteriosclerotic or aneurysmal disease. METHODS: We utilized Heartport endovenous and endoaortic cannulas (Heartport Inc, Redwood City, CA) for axilloaxillary or femoral-descending aortic cardiopulmonary bypass in 9 patients in an attempt to avoid potential cerebral and systemic embolization. All patients were successfully cannulated and the endoaortic clamp was deployed to perform a total of eleven grafts (five right coronary arteries and six circumflex coronary arteries). The patients ranged from 66 to 80 years of age. Five patients had abdominal aortic aneurysmal disease and 4 had severe peripheral vascular disease. RESULTS: All patients achieved full cardiopulmonary bypass with flows in excess of 3 L/min. without difficulty. There were no complications referable to the arm and its neurovascular structures. All axillary arteriotomies were closed primarily, without the need for thrombectomy or reconstruction. There were no neurological complications and 30-day survival was 100%. Three patients underwent successful abdominal aortic aneurysm resection prior to discharge. CONCLUSIONS: Axilloaxillary and femoral-descending aortic cannulation utilizing standard Heartport cannulas (Heartport, Inc) offer alternative sites for cardiopulmonary bypass in patients with severe peripheral vascular disease.

A study of the effects of cochlear loss on the auditory brainstem response (ABR) specificity and false positive rate in retrocochlear assessment.

The auditory brainstem response (ABR) test is widely used as a screen for retrocochlear dysfunction in individuals suffering idiopathic auditory and vestibular disturbance. Although its sensitivity for lesions of the lower brainstem is well established, hearing loss is known to have significant effects upon the test's specificity and false positive rate. This study analysed the effects of aspects of cochlear hearing loss including level, slope and general audiogram shape on these properties of the ABR test in a large clinical group. The study comprised 306 patients (153 male, 153 female) referred for ABR screening to exclude retrocochlear dysfunction. Of 566 ears meeting the inclusion criteria, 85 (15 per cent) failed the test. On follow-up eight ears (3 per cent) were confirmed to have some form of retrocochlear abnormality, seven of which were pathological. All had abnormal ABR results. No cases of retrocochlear abnormality were found in the ABR pass group. In those patients diagnosed as free of retrocochlear problems, the importance of the I-V interval as a primary index of function was confirmed. It displayed a high specificity (>90 per cent) which was largely independent of the level or slope of high-frequency hearing loss. In contrast wave V specificity decreased with increasing loss and increasing slope. Its value as a functional index was limited with losses greater than 70 dB HL in whom specificity fell below 70 per cent. The study concluded that, despite reducing interval availability above 60 dB HL, wave V latency cannot be used as an alternative index because it displays weakening specificity over this range of loss. With the problems inherent in applying latency corrections, the author advocates the use of improved procedures for wave I identification in these cases.

The effect of hospital admission on the opinions and knowledge of elderly patients regarding cardiopulmonary resuscitation.

OBJECTIVE: to determine (i) if the opinions of elderly people, regarding their wish for cardiopulmonary resuscitation (CPR), change after staying in hospital, (ii) how much elderly people wish to be involved in making decisions about CPR and (iii) the degree of knowledge they use to make their decisions. DESIGN: consecutive sample survey. SETTING: assessment, treatment and rehabilitation unit. PATIENTS: 95 elderly inpatients (63% of all admissions) without a terminal illness who could give informed consent, interviewed after hospital admission. Sixty-seven were interviewed again at hospital discharge and three were interviewed 16-35 days after admission. INTERVENTION: patient education and semi-structured questionnaire. OUTCOME MEASURES: patients' knowledge and opinions on basic knowledge of CPR, preference for CPR, who should decide and how this should be documented. RESULTS: knowledge improved after intervention, although patients persistently overestimated the success rate of CPR. Eighty percent on admission and 69% following a hospital stay wished to have CPR if necessary. Men were more likely to want CPR. On admission, 74% stated the patient should make the decision regarding CPR. This rose to 84% after a hospital stay. Only 57% wished to have their preference recorded in the hospital record and only 43% wanted their general practitioner notified of their wishes. Ninety-four percent felt comfortable with the interview. CONCLUSIONS: elderly people wish to be consulted about CPR but many do not wish their preference to be committed to paper. Most older patients want CPR but these wishes may change with time. It is important that any recorded directive from a patient is updated frequently.

The effects of cochlear hearing loss, age and sex on the auditory brainstem response.

This study analyzed the effects of auditory impairment, age and sex on the auditory brainstem response (ABR) wave latencies. ABR wave I, wave V and I-V interval measures were extracted from the clinical records of 201 patients with cochlear hearing loss. Females had consistently earlier wave V latencies and shorter I-V intervals than males. No age effects were observed. Degree of impairment had a systematic effect on ABR wave latencies and I-V intervals. Wave I displayed latency extension with increasing levels of high-frequency hearing loss, whilst for wave V increases in latency were dependent upon both degree and slope of the hearing loss. Present results suggest that many of the previously reported sex differences and variable interactions seen for the ABR can be accounted for by differences in the underlying distribution of audiogram shapes within and between study populations. Different audiometric configurations were found to produce consistent differential effects on both wave I and wave V latency and thus influence the I-V interval. This study underlines the need to develop a more detailed model of impairment effects if correction factors are to be employed more effectively in ABR testing for retrocochlear pathology.

Auditory brainstem response screening for hearing loss in high risk neonates.

The present paper reports the findings of a 7 year study evaluating the use of the auditory brainstem response (ABR) as the basis of a hearing screening procedure in a group of newborns at increased risk of hearing impairment. A Special Care Baby Unit (SCBU) population of 417 infants with diverse clinical backgrounds and treatment histories was tested for hearing impairment at birth using ABR audiometry. Some 332 passed the original screen at 30 dBnHL test level in both ears. Of the failure group, 18 did not survive and 32 had some degree of hearing impairment confirmed, nine of which were sensorineural in origin. An increased incidence of persistent middle ear disease was also noted in the failure group. A detailed operational analysis demonstrates that provided appropriate pass/fail criteria are adopted, the ABR technique offers excellent sensitivity and specificity for the detection of significant hearing loss in the test population. Furthermore, the study establishes that implementation of an ABR-based screening programme could reduce the average age at detection of permanent hearing loss by 7 months. A cost assessment shows that the introduction of such a targetted screening procedure could be done at a reasonable outlay.

A review of computerized electronystagmography technology.

Analysis of nystagmus using manual methods is time consuming, reliant on considerable experience and subject to observer bias. Like many electrophysiological responses, nystagmus waveforms are suited to acquisition and analysis by digital computer techniques. Development of computerized eye movement analysis procedures commenced in the late 1960s. Initially programs were mostly researched-based with the data often acquired on FM tape recorders and evaluated on mini-computers. As computer technology evolved and equipment prices decreased, development of portable software and hardware for use in general clinical assessment became feasible. The development of computer programs for the acquisition and analysis of eye movements is described and the limitations of analysis procedures discussed.

Reliability and effectiveness of screening for hearing loss in high risk neonates.

OBJECTIVE: To establish the reliability and effectiveness of screening for hearing loss by brainstem auditory evoked potential testing in high risk neonates. DESIGN: Seven year investigation of newborn babies admitted to a special care baby unit and monitored through a regional children's audiology unit. SETTING: Special care baby unit and children's audiology department, Belfast. SUBJECTS: 405 neonates admitted to the baby unit, during 1 October 1982 to 31 March 1987. MAIN OUTCOME MEASURES: Presence of hearing impairment, type and severity of hearing impairment, mortality. RESULTS: 85 children failed the screening test, 62 of whom were followed up. Five children had severe bilateral sensorineural impairment and 12 had conductive impairment requiring surgical intervention. A further 18 had severe neurological disorder detected. The sensitivity of screening was 100% and specificity was 88%. If the procedure was introduced into routine clinical practice the mean age at diagnosis for all children with severe perinatal hearing impairment would be 11 (median 1) months. The mean age at diagnosis with the health visitor screening service was 23 (19) months (difference 10 months, 95% confidence interval 6 to 16 months; p < 0.0001). CONCLUSION: Screening for hearing loss in high risk neonates is highly reliable and cost effective. It also provides valuable neurophysiological information. Routine testing of these infants would result in over half of all children with severe bilateral perinatal sensorineural hearing impairment being identified by 2 months of age. This would make an important contribution to the habilitation of this socially, emotionally, and educationally vulnerable group.

A neurophysiological study of children with reading, writing and spelling difficulties.

Recordings of early and late auditory evoked potentials and spectral analysis of scalp EEGs of 14 eight- and nine-year-old boys with difficulties in reading, writing and spelling were compared with those of 18 matched controls. The recordings of the poor readers lacked the normal asymmetry of central conduction time and wave amplitude in the early (brainstem) auditory evoked potentials, and late (cortical) evoked potential components were reduced in amplitude. Spontaneous EEG activity was significantly increased in power in all spectral bands, and this activity was less responsive to changes in sensory stimulation. These observations provide evidence for disturbed early and late auditory processing and altered cortical function. Correlations with behavioural measures considered to be indices of reading, writing and spelling ability support the view that the electrophysiological differences in the group of poor readers reflect a disturbance in the mechanisms subserving the acquisition of written language. These differences are consistent with a primary disturbance of selective attention, which may contribute to a less efficient cognitive strategy for the acquisition of reading skills.

A computer-managed clinical evaluation system: implications for competency-based dietetic education programs.

Instruments for evaluating clinical performance of students were developed, tested, and designed for a computer-managed evaluation system for the competency-based dietetic internship program at Emory University, Atlanta. The development, evaluation, and utilization of the instruments are described. The method of development included: (a) analysis of validated competencies and performance criteria to determine aspects of behavior appropriate to measure in clinical settings, (b) specification of major areas to be evaluated, (c) specification of component behaviors within each of the major areas, (d) specification of criteria to be used in evaluating component behaviors, and (e) definition of the rating continuum. Validation of the instruments consisted of determinations of content validity, interrater reliability, and practicality. Results of the use of the computer-managed evaluation system indicated that the instruments were valid, reliable, and practical to use for evaluating students' clinical performance.