Hearing loss and pigmentary disturbances in Waardenburg syndrome with reference to WS Type II

1995 ◽  
Vol 109 (2) ◽  
pp. 96-100 ◽  
Author(s):  
Xuezhong Liu ◽  
Valerie Newton ◽  
Andrew Read
Keyword(s):  
Hearing Loss ◽  
Common Type ◽  
Hearing Impaired ◽  
Type I ◽  
Type Ii ◽  
Waardenburg Syndrome ◽  
Profound Hearing Loss ◽  
Significant Difference ◽  
Impaired Group ◽  
Pigmentation Disorders

AbstractThirty families in which there were 149 individuals affected by the Waardenburg syndrome (WS) were investigated for penetrance of hearing loss and pigmentary abnormalities. Twenty two families contained 89 individuals affected by WS Type II and eight families with 60 individuals affected by Type I. A bilateral symmetrical sensorineural hearing loss was found to be the most common type of hearing loss. The most frequent degree of hearing loss category was a hearing loss of > 100dB HL with no difference between syndrome types.Although there was no significant difference in the frequency of pigmentation disorders between Type I and II, considering these abnormalities as a whole, heterochromia irides was more common in Type II than Type I and other pigmentation disorders were more frequent in Type I: 62 per cent of Type I patients had more than one pigmentary defect, but only 28 per cent of Type II. Pigmentary disturbances were not significantly more frequent in the hearing impaired group than in the normally hearing group in either Type I or II when taking into account the occurrence of only one pigmentation disorder. There was a significantly higher proportion of pigmentary defects in the hearing impaired group with Type I when only those having more than one pigmentary abnormality were compared: 93.3 per cent of Type I patients and 88.5 per cent of Type II patients with a profound hearing loss had pigmentary defects. The frequency of pigmentation disorders was not greater when the hearing loss was more severe in either type. Penetrance for hearing loss and pigmentary abnormalities showed marked intrafamilial and interfamilial variation.

scholarly journals Production of Greek vowels by hearing-impaired children

2020 ◽  
Author(s):  
Elina Nirgianaki ◽  
Maria Bitzanaki
Keyword(s):  
Hearing Loss ◽  
Cochlear Implants ◽  
High Dimension ◽  
Normal Hearing ◽  
Hearing Impaired ◽  
Acoustic Characteristics ◽  
Significant Difference ◽  
Impaired Group ◽  
Impaired Children ◽  
Hearing Impaired Children

The present study investigates the acoustic characteristics of Greek vowels produced by hearing-impaired children with profound prelingual hearing loss and cochlear implants. The results revealed a significant difference between vowels produced by hearingimpaired children and those produced by normal-hearing ones in terms of duration. Stressed vowels were significantly longer than non-stressed for both groups, while F0, F1 and F2 did not differ significantly between the two groups for any vowel, with the exception of /a/, which had significantly higher F1 when produced by hearingimpaired children. Acoustic vowel spaces were similar for the two groups but shifted towards higher frequencies in the low-high dimension and somehow reduced in the front-back dimension for the hearing-impaired group.

scholarly journals Analysis of sagittal profile and radiographic parameters in symptomatic thoracolumbar disc herniation patients

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ang Gao ◽  
Yongqiang Wang ◽  
Miao Yu ◽  
Xiaoguang Liu
Keyword(s):  
Disc Herniation ◽  
Thoracic Spine ◽  
Normal Population ◽  
Sagittal Profile ◽  
Sacral Slope ◽  
Type I ◽  
Type Ii ◽  
Radiographic Parameters ◽  
Significant Difference ◽  
Thoracolumbar Region

Abstract Background Few studies describe thoracolumbar disc herniation (TLDH) as an isolated category, it is frequently classified as the lower thoracic spine or upper lumbar spine. Thus, less is known about the morphology and aetiology of TLDH compared to lumbar disc herniation (LDH). The aim of study is to investigate sagittal alignment in TLDH and analyze sagittal profile with radiographic parameters. Methods Data from 70 patients diagnosed with TLDH were retrospectively reviewed. The thoracic-lumbar alignment was depicted by description of curvatures (the apex of lumbar curvature, the apex of thoracic curvature, and inflexion point of the two curvatures) and radiographic parameters from complete standing long-cassette spine radiographs. The rank sum test was utilised to compare radiographic parameter values in each subtype. Results We found two subtypes differentiated by the apex of thoracic kyphotic curves. The sagittal profile was similar to that of the normal population in type I, presenting the apex of the thoracic kyphotic curve located in the middle thoracic spine. The well aligned thoracic-lumbar curve was disrupted in type II, presenting the apex of the thoracic kyphotic curve located in the thoracolumbar region in type II patients. Thirty-six patients were classified as type I, and 34 patients were classified as type II. The mean sagittal vertical axis, T1 pelvic angle and L1 pelvic angle were 27.9 ± 24.8°, 8.2 ± 7.3° and 6.2 ± 4.9°, respectively. There was significant difference (p < 0.001) of thoracolumbar angle between type I (14.9 ± 7.9°) and type II patients (29.1 ± 13.7°). Conclusions We presented two distinctive sagittal profiles in TLDH patients, and a regional kyphotic deformity with a balanced spine was validated in both subtypes. In type I patients, disc degeneration was accelerated by regional kyphosis in the thoracolumbar junction and eventually caused disc herniation. In type II patients, excessive mechanical stress was directly loaded at the top of the curve (thoracolumbar apex region) rather than being diverted by an arc as in a normal population or type I patients. Mismatch between shape and sacral slope value was observed, and better agreement was found in Type II patients.

scholarly journals Perception and learning of relative pitch by musicians using the vibrotactile mode

2021 ◽  
pp. 102986492110152
Author(s):  
Carl Hopkins ◽  
Saúl Maté-Cid ◽  
Robert Fulford ◽  
Gary Seiffert ◽  
Jane Ginsborg
Keyword(s):  
Hearing Loss ◽  
Music Education ◽  
Success Rate ◽  
Normal Hearing ◽  
Pitch Discrimination ◽  
Professional Musicians ◽  
Profound Hearing Loss ◽  
Relative Pitch ◽  
Significant Difference ◽  
Schools For The Deaf

This study investigated the perception and learning of relative pitch using vibrotactile stimuli by musicians with and without a hearing impairment. Notes from C3 to B4 were presented to the fingertip and forefoot. Pre- and post-training tests in which 420 pairs of notes were presented randomly were carried out without any feedback to participants. After the pre-training test, 16 short training sessions were carried out over six weeks with 72 pairs of notes per session and participants told whether their answers were correct. For amateur and professional musicians with normal hearing and professional musicians with a severe or profound hearing loss, larger pitch intervals were easier to identify correctly than smaller intervals. Musicians with normal hearing had a high success rate for relative pitch discrimination as shown by pre- and post-training tests, and when using the fingertips, there was no significant difference between amateur and professional musicians. After training, median scores on the tests in which stimuli were presented to the fingertip and forefoot were >70% for intervals of 3–12 semitones. Training sessions reduced the variability in the responses of amateur and professional musicians with normal hearing and improved their overall ability. There was no significant difference between the relative pitch discrimination abilities between one and 11 semitones, as shown by the pre-training test, of professional musicians with and without a severe/profound hearing loss. These findings indicate that there is potential for vibration to be used to facilitate group musical performance and music education in schools for the deaf.

scholarly journals Perception of Auditory Distance in Normal-Hearing and Moderate-to-Profound Hearing-Impaired Listeners

2019 ◽  
Vol 23 ◽  
pp. 233121651988761 ◽  
Author(s):  
Gilles Courtois ◽  
Vincent Grimaldi ◽  
Hervé Lissek ◽  
Philippe Estoppey ◽  
Eleftheria Georganti
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Normal Hearing ◽  
Hearing Impaired ◽  
Spatial Cues ◽  
Linear Amplification ◽  
Profound Hearing Loss ◽  
Wireless Microphone ◽  
Auditory Distance ◽  
Non Linear

The auditory system allows the estimation of the distance to sound-emitting objects using multiple spatial cues. In virtual acoustics over headphones, a prerequisite to render auditory distance impression is sound externalization, which denotes the perception of synthesized stimuli outside of the head. Prior studies have found that listeners with mild-to-moderate hearing loss are able to perceive auditory distance and are sensitive to externalization. However, this ability may be degraded by certain factors, such as non-linear amplification in hearing aids or the use of a remote wireless microphone. In this study, 10 normal-hearing and 20 moderate-to-profound hearing-impaired listeners were instructed to estimate the distance of stimuli processed with different methods yielding various perceived auditory distances in the vicinity of the listeners. Two different configurations of non-linear amplification were implemented, and a novel feature aiming to restore a sense of distance in wireless microphone systems was tested. The results showed that the hearing-impaired listeners, even those with a profound hearing loss, were able to discriminate nearby and far sounds that were equalized in level. Their perception of auditory distance was however more contracted than in normal-hearing listeners. Non-linear amplification was found to distort the original spatial cues, but no adverse effect on the ratings of auditory distance was evident. Finally, it was shown that the novel feature was successful in allowing the hearing-impaired participants to perceive externalized sounds with wireless microphone systems.

The Effect of Conventional and Transparent Surgical Masks on Speech Understanding in Individuals with and without Hearing Loss

2017 ◽  
Vol 28 (01) ◽  
pp. 058-067 ◽  
Author(s):  
Samuel R. Atcherson ◽  
Lisa Lucks Mendel ◽  
Wesley J. Baltimore ◽  
Chhayakanta Patro ◽  
Sungmin Lee ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Hearing Impairment ◽  
Visual Input ◽  
Acoustic Properties ◽  
Speech Understanding ◽  
Profound Hearing Loss ◽  
Cross Sectional ◽  
Surgical Masks ◽  
Significant Difference

AbstractIt is generally well known that speech perception is often improved with integrated audiovisual input whether in quiet or in noise. In many health-care environments, however, conventional surgical masks block visual access to the mouth and obscure other potential facial cues. In addition, these environments can be noisy. Although these masks may not alter the acoustic properties, the presence of noise in addition to the lack of visual input can have a deleterious effect on speech understanding. A transparent (“see-through”) surgical mask may help to overcome this issue.To compare the effect of noise and various visual input conditions on speech understanding for listeners with normal hearing (NH) and hearing impairment using different surgical masks.Participants were assigned to one of three groups based on hearing sensitivity in this quasi-experimental, cross-sectional study.A total of 31 adults participated in this study: one talker, ten listeners with NH, ten listeners with moderate sensorineural hearing loss, and ten listeners with severe-to-profound hearing loss.Selected lists from the Connected Speech Test were digitally recorded with and without surgical masks and then presented to the listeners at 65 dB HL in five conditions against a background of four-talker babble (+10 dB SNR): without a mask (auditory only), without a mask (auditory and visual), with a transparent mask (auditory only), with a transparent mask (auditory and visual), and with a paper mask (auditory only).A significant difference was found in the spectral analyses of the speech stimuli with and without the masks; however, no more than ∼2 dB root mean square. Listeners with NH performed consistently well across all conditions. Both groups of listeners with hearing impairment benefitted from visual input from the transparent mask. The magnitude of improvement in speech perception in noise was greatest for the severe-to-profound group.Findings confirm improved speech perception performance in noise for listeners with hearing impairment when visual input is provided using a transparent surgical mask. Most importantly, the use of the transparent mask did not negatively affect speech perception performance in noise.

AGE-RELATED ISSUES OF THE QUALITY OF LIFE IN HEARING IMPAIRED PATIENTS

2021 ◽  
pp. 885-892
Author(s):  
Л. Е. Голованова ◽  
Е. А. Огородникова ◽  
Е. С. Лаптева ◽  
М. Ю. Бобошко
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Hearing Aids ◽  
Pure Tone Audiometry ◽  
Hearing Impaired ◽  
Hearing Disorders ◽  
Age Related ◽  
Sf 36 ◽  
Significant Difference

Целью исследования было изучение качества жизни лиц с нарушениями слуха в разных возрастных группах. Обследованы 100 пациентов, обратившихся в городской сурдологический центр для взрослых в связи с нарушением слуха: 50 человек - 34-59 лет, 50 - 60 лет и старше, из которых 32 человека были пожилого возраста (60-74 года)и 18 - старческого (75-86 лет). Степень тугоухости оценивали на основании результатов тональной пороговой аудиометрии. Для исследования качества жизни все пациенты заполняли общий опросник MOS SF-36, отражающий физический и психологический компоненты здоровья, а также специальный опросник HHIА(E)-S для лиц с нарушениями слуха. Установлено, что шкала HHIА(E)-S демонстрирует высокую корреляцию со степенью тугоухости у пациентов моложе 60 лет ( R =0,98; достоверность различий на уровне p <0,05), которая снижается у пациентов 60 лет и старше ( R =0,94; различия в оценках при разной степени тугоухости недостоверны). Значительные трудности в старшей возрастной группе могут быть связаны с тем, что людям пожилого и, особенно, старческого возраста сложно пользоваться слуховыми аппаратами (или они для них неэффективны) и оценивать свои затруднения по шкалам опросника. Целесообразно использовать шкалу HHIА(Е)-S в качестве скринингового инструмента для раннего выявления тугоухости, направления пациентов к сурдологу и своевременного слухопротезирования. The aim of the research was to study the quality of life in hearing impaired patients of different age. 100 patients referred to the city audiology centre because of their hearing disorders were examined: 50 patients from 34 to 59 years old and 50 patients from 60 years and older, from which 32 patients were of older age (60 to 74 years old) and 18 of oldest age (75 to 86 years old). A degree of hearing loss was assessed according to results of pure tone audiometry. To study the quality of life all patients filled in the questionnaire MOS SF-36, which evaluates physical and psychological components of health, and the questionnaire HHIA(E)-S, designed specifically for patients with hearing disorders. The HHIA(E)-S scale was found to show high correlation with hearing loss degree in patients younger than 60 years old ( R =0,98 with statistically significant difference, p <0,05), with decreasing correlation in patients from 60 years and older ( R =0,98; no significant difference while assessing various hearing loss degrees). Considerable difficulties in this age group may be explained by the fact, that older and especially oldest patients have a challenge with hearing aids usage (or they are of low efficiency for them) and with assessing theirs difficulties on the questionnaire scales. The HHIA(E)-S scale is useful as a screening tool for early detection of hearing loss, referral of patients to an audiologist and prompt hearing aid fitting.

RA05.04: MANAGEMENT & OUTCOMES OF ANASTOMOTIC LEAKS FOLLOWING ESOPHAGECTOMY: A PROSPECTIVE 10-YEAR SINGLE-SITE EXPERIENCE

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 28-28
Author(s):  
Joel Lambert ◽  
Sanya Caratella ◽  
Eloise Lawrence ◽  
Bilal Alkhaffaf
Keyword(s):  
Minimally Invasive ◽  
Anastomotic Leak ◽  
Surgical Intervention ◽  
Type I ◽  
Oral Contrast ◽  
Open Approach ◽  
Type Ii ◽  
Anastomotic Leaks ◽  
Type Iii ◽  
Significant Difference

Abstract Background Anastomotic leak after esophagectomy is associated with high levels of morbidity and may impact negatively on oncological outcomes. The aim of this single centre study was to describe our experience in managing these complications Methods From 2007–2017 data was reviewed retrospectively from our prospectively maintained electronic database. All patients underwent either 2 or 3 phase esophagectomy for cancer of the oesophagus or esophago-gastric junction. All histological sub-types and stage of cancer were included in the analysis. Anastomotic leaks were classified according to the Esophagectomy Complications Consensus Group (ECCG) guidelines; type I—conservative management, type II—non-surgical intervention, type III—surgical intervention. Results 224 esophagectomies were included in our analysis (104 (46%) minimally invasive, 120 (54%) open approach). The incidence of all anastomotic leaks was 10% (23/224). Surgical approach did not influence the incidence of anastomotic leak (minimally invasive 10 (43%), open approach 13(57%), P = 0.76). Five patients (22%) had a type I leak, 9 patients (39%) type II and 9 (39%) had a type III leak. There was an increase in the number of leaks managed non-surgically over the last 5 years compared to those in the first five years of our dataset (2012–2017: 11/23 (48%) vs 2007–2012: 4/23 (17%) P = 0.08). The median time for leak diagnosis was 8 days. Most leaks were diagnosed with oral contrast CT 19 (83%). Median hospital stay after anastomotic leak was 58.5 days. Type III leaks were associated with an increased length of stay (median 84 days) compared to type I&II leaks (median (38.5 days) (P = 0.002 95% CI 18.19- 74.41). There was no significant difference in 30-day mortality between type I&II (0 patients) and type III leaks (1 patient) P = 0.260. Conclusion Low mortality rates with anastomotic leak can be achieved. In centres with experienced radiological and endoscopic skills, most anastomotic leaks can be managed non-surgically. Disclosure All authors have declared no conflicts of interest.

Close Reduction Percutaneous Pinning (CRPP) versus Open Reduction Internal Fixation (ORIF) for Pediatric Supracondylar Humerus Fractures Gartland Type II and III: A Systematic Review and Meta-analysis

2021 ◽  
Vol 71 (4) ◽  
pp. 187-193
Author(s):  
Putu Astawa ◽  
Made Agus Maharjana ◽  
Surya Adisthanaya ◽  
Made Winatra Satya Putra ◽  
Agus Suarjaya Putra ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Reports ◽  
Meta Analysis ◽  
Fixed Effect Model ◽  
Cosmetic Outcome ◽  
Type I ◽  
Type Ii ◽  
Supracondylar Humerus Fractures ◽  
Significant Difference ◽  
Humerus Fractures

Introduction: Displaced supracondylar fracture in children is a challenging injury that may result in impaired functional and cosmetic outcome if not well-treated. Utilization of Closed Reduction and Percutaneus Pinning (CRPP) increased for this pathology, some authors believe ORIF results better anatomical reduction and lower rate of loss of reduction. Study aims to compare CRPP and ORIF for pediatric supracondylar humerus fracture. Method: Systematic review was conducted based on PRISMA guideline. Inclusion criteria were age <18 years old, comparing CRPP and ORIF for Supracondylar Humerus Fractures Gartland Type II, II.Studies of one surgical technique, Gartland type I, case reports were excluded. For meta-analysis, 6 studies were included and fixed effect model used to pool the result. In each study, mean difference (MD) with 95% confidence interval (CI) was calculated for dichotomous outcomes using Review Manager. Result: Total of 252 patients aged 0-15 years old were included. CRPP more often performed than ORIF. Satisfactory outcomes measured by Flynn’s criteria were achieved in 87.74% in CRPP and 86.73% in ORIF patient group, indicating significant difference (Heterogeneity, I2 = 23%; WMD, 1.26; 0.58 to 2.73; P =0.56). Conclusion: Current systematic review and meta-analysis suggest that for displaced supracondylar humerus fractures, ORIF offers a comparable functional and cosmetic outcome compared to CRPP.

Should All Cases of High-Grade Serous Ovarian, Tubal, and Primary Peritoneal Carcinomas Be Reclassified as Tubo-Ovarian Serous Carcinoma?

2015 ◽  
Vol 25 (7) ◽  
pp. 1201-1207 ◽  
Author(s):  
Esther Louise Moss ◽  
Tim Evans ◽  
Philippa Pearmain ◽  
Sarah Askew ◽  
Kavita Singh ◽  
...  
Keyword(s):  
Overall Survival ◽  
Clear Cell ◽  
Stage Iii ◽  
Serous Carcinoma ◽  
Low Grade ◽  
Type I ◽  
High Grade ◽  
Type Ii ◽  
Ovarian Serous Carcinoma ◽  
Significant Difference

IntroductionThe dualistic theory of ovarian carcinogenesis proposes that epithelial “ovarian” cancer is not one entity with several histological subtypes but a collection of different diseases arising from cells of different origin, some of which may not originate in the ovarian surface epithelium.MethodsAll cases referred to the Pan-Birmingham Gynaecological Cancer Centre with an ovarian, tubal, or primary peritoneal cancer between April 2006 and April 2012 were identified from the West Midlands Cancer Registry. Tumors were classified into type I (low-grade endometrioid, clear cell, mucinous, and low-grade serous) and type II (high-grade serous, high-grade endometrioid, carcinosarcoma, and undifferentiated) cancers.ResultsOvarian (83.5%), tubal (4.3%), or primary peritoneal carcinoma (12.2%) were diagnosed in a total of 583 woman. The ovarian tumors were type I in 134 cases (27.5%), type II in 325 cases (66.7%), and contained elements of both type I and type II tumors in 28 cases (5.7%). Most tubal and primary peritoneal cases, however, were type II tumors: 24 (96.0%) and 64 (90.1%), respectively. Only 16 (5.8%) of the ovarian high-grade serous carcinomas were stage I at diagnosis, whereas 240 (86.6%) were stage III+. Overall survival varied between the subtypes when matched for stage. Stage III low-grade serous and high-grade serous carcinomas had a significantly better survival compared to clear cell and mucinous cases,P= 0.0134. There was no significant difference in overall survival between the high-grade serous ovarian, tubal, or peritoneal carcinomas when matched for stage (stage III,P= 0.3758; stage IV,P= 0.4820).ConclusionsType II tumors are more common than type I and account for most tubal and peritoneal cancers. High-grade serous carcinomas, whether classified as ovarian/tubal/peritoneal, seem to behave as one disease entity with no significant difference in survival outcomes, therefore supporting the proposition of a separate classification of “tubo-ovarian serous carcinoma”.

Reliability of Threshold, Slope, and PB Max for Monosyllabic Words

1985 ◽  
Vol 50 (2) ◽  
pp. 166-178 ◽  
Author(s):  
Randall C. Beattie ◽  
Michael J.M. Raffin
Keyword(s):  
Hearing Loss ◽  
Hearing Impaired ◽  
Sensation Level ◽  
Step Size ◽  
Binomial Theorem ◽  
Large Variability ◽  
Impaired Group ◽  
Word Lists ◽  
Monosyllabic Words ◽  
Single List

The Auditec recordings of the CID W-22 monosyllables were used to generate test and retest intelligibility functions on normally hearing listeners and subjects with mild-to-moderate sensorineural hearing loss. The normally hearing subjects were tested with 50-word lists at SPLs ranging from 15 to 50 dB. Lists of 25 words were used with the hearing-impaired group. The functions were analyzed to assess the reliability of threshold (50% point), slope (20%–80% points), and maximum intelligibility (PB max). The 50% point was obtained at 28 dB SPL for the normally hearing listeners and at a sensation level (SL) of 12 dB re spondaic thresholds for the hearing-impaired group. Very stable monosyllabic thresholds were found because 95% of the test-retest values were within 6 dB for both subject groups. Slopes of 4.9% per dB and 2.7% per dB were obtained for the normally hearing and hearing-impaired groups, respectively. Fair reliability was observed; 95% of the test-retest values encompassed a range of ± 1.9% per dB for the normally hearing subjects and ± 1.1% per dB for the hearing-impaired group. Although group slopes provide useful information for selecting the range and step size for generating psychometric functions, the value of routinely obtaining slope on an individual basis has not been demonstrated. Even though the same word lists were used for both test and retest measurements, reliability closely agreed with predicted results based on the binomial theorem. In contrast to the large variability for a single list of 25 words, very stable PB max scores were found when two or three scores were averaged on the plateau of the function.

Sign in / Sign up

Export Citation Format

Share Document