Adductor focal laryngeal Dystonia: correlation between clinicians' ratings and subjects' perception of Dysphonia.

BACKGROUND: Although considerable research has focused on the etiology and symptomology of adductor focal laryngeal dystonia (AD-FLD), little is known about the correlation between clinicians' ratings and patients' perception of this voice disturbance. This study has five objectives: first, to determine if there is a relationship between subjects' symptom-severity and its impact on their quality of life; to compare clinicians' ratings with subjects' perception of the individual characteristics and severity of AD-FLD; to document the subjects' perception of changes in dysphonia since diagnosis; to record the frequency of voice arrest during connected speech; and, finally, to calculate inter-clinician reliability based on results from the Unified Spasmodic Dysphonia Rating Scale (USDRS) (Stewart et al, J Voice 1195-10, 1997). METHODS: Sixty subjects with AD-FLD who were receiving ongoing injections of BoNT participated in this study. Subjects' mean age was 60.78 years and their mean duration of symptoms was 16.1 years. Subjects completed the Disease Symptom Questionnaire (DSQ) (specifically designed for this study) and the Voice Handicap Index-10 (VHI-10) (Jacobson et al, Am J Speech Lang Pathol 6:66-70, 1997) to measure the symptoms of their dysphonia and the impact of the disease on their quality of life.Two speech-language pathologists and two laryngologists used the Voice Arrest Measure (VAM) (specifically designed for this study) and the USDRS to independently rate voice recordings of 56/60 subjects. RESULTS: The mean VHI-10 score was 21.3 which is clinically significant. The results of the DSQ and the USDRS were highly correlated. The most severe symptoms identified by both subjects and clinicians were roughness, strain-strangled voice quality, and increased expiratory effort. Voice arrest, aphonia, and tremor were uncommon. Subjects rated their current voice quality at the time of reinjection (i.e., at the time of the study) as significantly better than at the time of their initial AD-FLD diagnosis (p < 0.0001). Inter-clinician reliability on the USDRS was significant at the 0.001 level. CONCLUSIONS: The findings from the VHI-10 suggest that AD-FLD has a profound impact on quality of life. The results of the DSQ and the USDRS suggest that there is a strong correlation between subjects' perception and clinicians' assessment of the individual symptoms and the severity of the dysphonia. The findings from the VAM suggest that voice arrests are infrequent in subjects with AD-FLD who are receiving ongoing BoNT injections. The strong inter-clinician reliability on the USDRS suggests that it is an appropriate measure for identifying symptoms and severity of AD-FLD.

Discriminative Properties of the Spinal Appearance Questionnaire Compared With the Scoliosis Research Society-22 Revised.

STUDY DESIGN: Longitudinal cohort. OBJECTIVES: To determine the responsiveness of the Spinal Appearance Questionnaire (SAQ) in patients with adolescent idiopathic scoliosis (AIS) undergoing surgical correction of the deformity. SUMMARY OF BACKGROUND DATA: The SAQ has been found to be a valid and reliable measure in patients with AIS. A recently published factor analysis and scoring system has been shown to be applicable to all Lenke types and had greater correlation to the curve magnitude than the Scoliosis Research Society (SRS) Appearance and Total score. METHODS: From a prospective multicenter database, 126 AIS patients who underwent correction of the spinal deformity with complete SAQ and SRS-22 Revised data at baseline and 2-year follow-up were identified. Discriminative properties of the SAQ domains (Expectations, Appearance, and Total) and SRS domains (Appearance, Activity, Pain, Mental, Satisfaction, and Total) were compared by computing the effect size (ES) and the standardized response mean (SRM). RESULTS: The SAQ Total had the largest ES (1.8) and SRM (1.5). This was followed by the SAQ Appearance, with an ES of 1.7 and SRM of 1.4; and the SAQ Expectations, with an ES of 1.5 and SRM of 1.2. Among the different SRS domains, only the Appearance (ES = 1.2, SRM = 1.1), Satisfaction (ES = 0.8, SRM = 0.6), and Total scores (ES = 0.8, SRM = 0.9) had effect sizes that were considered large. The SRS Mental domain had a moderate effect size (ES = 0.3, SRM = 0.3), whereas the Activity (ES = 0.0, SRM = 0.0) and Pain (ES = 0.2, SRM = 0.2) domains had small effect sizes. CONCLUSIONS: The SAQ is sensitive and responsive to change, as evidenced by the large effect size for both domain and the Total score. The effect sizes are larger than those for any of the SRS domains, including Appearance and Total scores.

Spinal appearance questionnaire: factor analysis, scoring, reliability, and validity testing.

STUDY DESIGN: Cross sectional. OBJECTIVE: This study presents the factor analysis of the Spinal Appearance Questionnaire (SAQ) and its psychometric properties. SUMMARY OF BACKGROUND DATA: Although the SAQ has been administered to a large sample of patients with adolescent idiopathic scoliosis (AIS) treated surgically, its psychometric properties have not been fully evaluated. This study presents the factor analysis and scoring of the SAQ and evaluates its psychometric properties. METHODS: The SAQ and the Scoliosis Research Society-22 (SRS-22) were administered to AIS patients who were being observed, braced or scheduled for surgery. Standard demographic data and radiographic measures including Lenke type and curve magnitude were also collected. RESULTS: Of the 1802 patients, 83% were female; with a mean age of 14.8 years and mean initial Cobb angle of 55.8° (range, 0°-123°). From the 32 items of the SAQ, 15 loaded on two factors with consistent and significant correlations across all Lenke types. There is an Appearance (items 1-10) and an Expectations factor (items 12-15). Responses are summed giving a range of 5 to 50 for the Appearance domain and 5 to 20 for the Expectations domain. The Cronbach's α was 0.88 for both domains and Total score with a test-retest reliability of 0.81 for Appearance and 0.91 for Expectations. Correlations with major curve magnitude were higher for the SAQ Appearance and SAQ Total scores compared to correlations between the SRS Appearance and SRS Total scores. The SAQ and SRS-22 Scores were statistically significantly different in patients who were scheduled for surgery compared to those who were observed or braced. CONCLUSION: The SAQ is a valid measure of self-image in patients with AIS with greater correlation to curve magnitude than SRS Appearance and Total score. It also discriminates between patients who require surgery from those who do not.

Newborn hearing screening in the NICU: profile of failed auditory brainstem response/passed otoacoustic emission.

OBJECTIVE: Incidence of a specific pattern of auditory responses, absent auditory brainstem responses (ABRs) and present otoacoustic emissions (OAEs), in newborn hearing screening in a regional perinatal center neonatal intensive care unit (NICU) is described. This profile, labeled auditory neuropathy or auditory dyssynchrony (AN/AD), is a dysfunction in neural/brainstem transmission that occurs in individuals whose outer hairs cells are functioning normally. Although the AN/AD profile has been associated with various risk factors, incidence and prediction are unknown. METHOD: Analysis of electrophysiologic measures and medical record reviews of the first 22 months of the universal newborn hearing-screening program was conducted. Association of the AN/AD profile was evaluated with the following factors: gender, gestational age, ototoxic drug regimen, low birth weight, hyperbilirubinemia, hydrocephalus, low Apgar score, anoxia, respiratory distress syndrome, pulmonary hypertension, intraventricular hemorrhage, multiple birth, seizure activity, and family history. RESULTS: One hundred fifteen (24.1%) of the 477 infants failed the ABR in 1 or both ears and passed OAEs bilaterally. Comparisons of infants fitting the AN/AD profile with those not fitting the AN/AD profile were negative with 3 exceptions: those with hyperbilirubinemia and those who were administered vancomycin or furosemide. A logistic-regression analysis model failed to predict which infants would be at risk for the AN/AD profile either unilaterally or bilaterally. CONCLUSIONS: Screening of NICU infants should be conducted with ABR first, followed by OAE after failure on ABR. Because the incidence of the AN/AD profile was found to be 24% in this at-risk population, additional study is warranted.

High-frequency pulsed electromagnetic energy in tinnitus treatment.

OBJECTIVES/HYPOTHESIS: Electromagnetic therapy has been used with reported success in multiple clinical settings, including the treatment of seizure disorders, brain edema, migraine headaches, revascularization of burn wounds, and diabetic ulcers. The purpose of the study was to investigate the effect of pulsed high-frequency electromagnetic therapy on chronic tinnitus. STUDY DESIGN: A randomized, prospective, double-blind, placebo-controlled pilot study was conducted to evaluate the effectiveness of high-frequency pulsed electromagnetic energy using the Diapulse device in the treatment of chronic tinnitus. Thirty-seven adult patients with chronic tinnitus of at least 6 months' duration were recruited and randomly assigned to either a treatment or a placebo group. METHODS: Patients received 30-minute treatments with the Diapulse device three times a week for 1 month. The unit was set to deliver electromagnetic energy at a frequency of 27.12 MHz at a repetition rate of 600 pulses per second. All subjects had pretreatment and post-treatment audiological testing, including tinnitus frequency and intensity matching. They responded to a tinnitus rating questionnaire and the Tinnitus Handicap Inventory before and after treatment. RESULTS: There was no significant change in the pretreatment and post-treatment audiometric thresholds in either group. There were no significant differences between the pretreatment and post-treatment Tinnitus Handicap Inventory scores or the tinnitus rating scores in either subject group (Student t test). No diagnosis-specific differences were identified. CONCLUSION: High-frequency pulsed electromagnetic energy (Diapulse) at the settings used in the study showed no role in the therapy of patients with chronic tinnitus.

Growth and skeletal maturation after pediatric cardiac transplantation.

To examine the effects of cardiac transplantation on skeletal maturation and linear growth, we retrospectively evaluated annual bone age determinations and growth parameters of pediatric cardiac transplant recipients followed at our center. Included in the analysis were records of 86 patients (32 females) who had received a cardiac transplant at our institution between 1984 and 1998. Bone age delay of > or =12 months was apparent in 38.5% at the time of transplantation. At some point in their post-transplant course, 23 patients (29%) had one or more bone age measurements that were > or =36 months delayed with respect to chronological age. Children transplanted before age seven and those with a pretransplantation diagnosis of cardiomyopathy experienced the most significant decrement in skeletal maturation after transplantation. High cyclosporin A levels and low body mass index were the only parameters found to be associated with delayed bone age. Although the majority of children grew at a normal rate after transplantation, height Z scores and height age were adversely affected regardless of the type of heart disease or the age at transplantation. The pathogenesis of both delayed skeletal maturation and growth retardation in this population warrant further investigation.

A home-based resistance-training program using elastic bands for elderly patients with orthostatic hypotension.

UNLABELLED: Falls are a common hazard in the elderly. Orthostatic hypotension (OH) is prevalent in older patients and contributes to the high incidence of falls. Our aim was to test whether a home-based resistance-training program (HBRT) using elastic resistance bands could safely and effectively increase muscle strength and functional ability, and attenuate the orthostatic fall in blood pressure. Eight subjects (> 60 yrs) underwent orthostatic provocations; muscle strength testing (isometric and dynamic), and a functional test of gait and mobility (Timed Up & Go), at baseline and following 8 weeks of training. Ten exercises were assigned and customized to each participant. At 8 weeks, significant increases occurred in dynamic strength in the chest press ( p = 0.017), quadriceps extension ( p = 0.017), and leg press (p = 0.025); no significant differences occurred in isometric strength or in blood pressures. Functional mobility increased in 7 of 8 subjects. There were no falls during the investigation period. CONCLUSIONS: A HBRT program using elastic resistance bands effectively increases dynamic muscle strength in elderly individuals with OH. Although no changes occurred in orthostatic blood pressures, which could be attributed to the limited length of the program, this therapy may be recognized as a safe method to improve strength, functional ability, and promote physical activity - variables that can reduce the incidence of falls and enhance the quality of life in this population.