scholarly journals Hearing Loss in Beta-Thalassemia: Systematic Review

2021 ◽  
Vol 11 (1) ◽  
pp. 102
Author(s):  
Immacolata Tartaglione ◽  
Roberta Carfora ◽  
Davide Brotto ◽  
Maria Rosaria Barillari ◽  
Giuseppe Costa ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Clinical Phenotype ◽  
Diagnostic Tools ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Laboratory Findings ◽  
Pathogenic Factors ◽  
Age And Sex

In the last half century, the life expectancy of beta-thalassemia patients has strikingly increased mostly due to regular blood transfusions and chelation treatments. The improved survival, however, has allowed for the emergence of comorbidities, such as hearing loss, with a non-negligible impact on the patients’ quality of life. This thorough review analyzes the acquired knowledge regarding hearing impairment in this hereditary hemoglobinopathy, aiming at defining its prevalence, features, course, and possible disease- or treatment-related pathogenic factors. Following PRISMA criteria, we retrieved 60 studies published between 1979 and 2021. Diagnostic tools and criteria, forms of hearing impairment, correlations with beta-thalassemia phenotypes, age and sex, chelation treatment and laboratory findings including iron overload, were carefully searched, analyzed and summarized. In spite of the relatively high number of studies in the last 40 years, our knowledge is rather limited, and large prospective studies with homogeneous diagnostic tools and criteria are required to define all the aforementioned issues. According to the literature, the overall prevalence rate of hearing impairment is 32.3%; age, sex, and laboratory findings do not seem to correlate with hearing deficits, while the weak relationship with clinical phenotype and chelation treatment seems to highlight the presence of further yet to be identified pathogenic factors.

Down Regulation of Hepcidin and Interleukin 1-Alpha in Pbmc from Patients with Beta thalassemia

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2880-2880
Author(s):  
Maria-Eliana Lai ◽  
Stefania Vacquer ◽  
Maria Paola Carta ◽  
Claudia Mulas ◽  
Pierluigi Cocco ◽  
...  
Keyword(s):  
Iron Overload ◽  
Iron Homeostasis ◽  
Mononuclear Cells ◽  
Interleukin 1 ◽  
Diagnostic Tools ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Mrna Levels ◽  
Down Regulation ◽  
Hepcidin Mrna

Abstract Thalassemia is an inherited blood disease characterized by low levels or absence of normal globin chains. Severe forms of thalassemia, thalassemia major (TM), require regular blood transfusions, the main cause of secondary hemosyderosis. However, iron overload is a potential complication, even in patients (pts) with thalassemia intermedia (TI), who do not require transfusions. Among them, iron overload is mainly the result of an excessive absorption of dietary iron, caused by a down-regulation of hepcidin, an hepatic hormone that acts as a major regulator of systemic iron homeostasis. Very low mRNA levels of hepcidin have been reported in urine as well as in liver biopsies from patients with TI. It has been also reported that, interleukin-1 alpha (IL-1α) stimulates hepcidin transcription, raising the question as to whether a down-regulation of this cytokine may be responsible, at least partially, for the excessive iron absorption occurring in patients with TI. Here, we assessed whether IL-1α was involved in hepcidin regulation in β-thalassemia. Peripheral blood mononuclear cells (PBMCs) were isolated from 6 TM pts, 8 TI pts and 6 controls. mRNA was obtained from PBMCs by RT-PCR. Hepcidin mRNA levels were reduced in both TM and TI pts when compared to the age matched normal controls. Neverthless, hepcidin mRNA levels were significantly lower in TI pts, as compared to TM pts receiving regular blood transfusions. Among TI pts lower hepcidin mRNA level was associated with lower level of IL-α. Tumor necrosis factor alpha (TNF-α) does not seem to be involved in the regulation of hepcidin transcription. Interestingly, the levels of mRNA expression of acyl-CoA-cholesterol acyltransferase (ACAT), the enzyme responsible for intracellular cholesterol ester accumulation, and thus, for atherosclerotic plaque formation were strongly induced in PBMCs in TI pts. These results suggest that PBMCs hepcidin and IL-1-α measurements could possibly be used in the future as simple, ease and sensible diagnostic tools for the detection of iron overload in patients with thalassemia. ACAT expression may even be used as therapeutic target in preventing atherosclerotic complications such as pulmonary thromboembolism, cerebral thrombosis, and leg ulcers. frequently occurring in such patients.

Quality of life measures in otitis media

2013 ◽  
Vol 127 (5) ◽  
pp. 442-447 ◽  
Author(s):  
E J Maile ◽  
R Youngs
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Hearing Impairment ◽  
Otitis Media ◽  
Chronic Otitis Media ◽  
Pure Tone Audiometry ◽  
Quality Of Life Measures ◽  
Generic Measures ◽  
Disease Specific

AbstractDisabling hearing impairment is the world's most common disability. Traditionally, hearing levels measured by pure tone audiometry have been used to define and quantify hearing loss. The effects of disabling hearing loss on patients' quality of life can be profound, and audiometric data alone may not correlate with quality of life measures. Generic measures of quality of life can be used to compare different diseases, and as such are useful in resource allocation and burden of disease studies. Their disadvantage is that they are not disease-specific and can therefore under-estimate the effects of a disease on patients' quality of life. Disease-specific measures are more sensitive. In chronic otitis media, additional factors such as discharge augment the effect of hearing loss alone on quality of life. Many of the quality of life measures developed for chronic otitis media have been used to assess improvement following reconstructive surgery. Quality of life measures have also been used to assess the effect of paediatric otitis media. Quality of life measures also have utility in the developing world, where hearing impairment is a huge burden.

scholarly journals Study of pulmonary function tests in multitransfused children with beta thalassemia major: an observational study

2020 ◽  
Vol 7 (12) ◽  
pp. 2307
Author(s):  
Kavya Shivaswamy ◽  
Somashekhar Chikkanna ◽  
Jagdish Singh ◽  
Keerthana T. N. Gubbari
Keyword(s):  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Beta Thalassemia Major ◽  
Function Tests ◽  
Asymptomatic Patients ◽  
Multiple Blood ◽  
Age And Sex

Background: Thalassemia is a hemoglobinopathy requiring multiple blood transfusions leading to iron deposition in various organs. Abnormalities in pulmonary function in thalassemia major patients have been found in various studies. Our aim was to study pulmonary function tests (PFT) in multitransfused children with beta thalassemia major.Methods: PFT was done in forty beta thalassemia major (TM) patients and forty normal healthy age and sex matched individuals and the results were analyzed.Results: Out of 40 thalassemia major patients, 20 (50%) had normal PFT and the rest 20 (50%) had an abnormal PFT out of which 18 (90%) had restrictive PFT and 2 (10%) had obstructive PFT.  Out of 40 controls, all 40 (100%) had normal PFT.Conclusions: Alteration of pulmonary function can be seen even in well-chelated TM patients. The respiratory system should be evaluated annually by PFTs even in asymptomatic patients to prevent pulmonary sequelae.

scholarly journals Assessment of Quality of Life in Adults with Hearing Impairment

2021 ◽  
pp. 11-18
Author(s):  
Lubna Noureen ◽  
Muhammad Sikardar Ghayas Khan ◽  
Atif Ikram ◽  
Rashid Hussain ◽  
Fazaila Ehsaan ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Sample Size ◽  
Sexual Activity ◽  
Hearing Impairment ◽  
Significant Relationship ◽  
Negative Impact ◽  
P Value ◽  
Life Questionnaire

Background: Hearing loss (HL) in adults is loss of ability to perceive surrounding environmental sounds. There may be environmental or genetic cause of this disability. Presently expectancy of old adult age is going to increase as in the same way hearing problems and its negative impact on health wellbeing standard of living also increased, the beneficial of current study is to be addressed, or give awareness about health consequences to adult population with hearing impairment. Aim: To determine the quality of life in adults with hearing impairment. Place and Duration of Study: Riphah international University, Lahore campus between December 2018 to May 2019. Methodology: A cross sectional survey was conducted on 67 adults with hearing impairment (sample size was calculated on the base of prevalence of hearing loss in the Express Tribute, (March 4, 2014) which was a Pakistani  study on hearing impaired  population  the president of famous organization the  developmental diseases and community of auditory handicap  MR Afzal  estimated the   occurrence of auditory deficits  in Pakistan region was in between  7 to 8 percent on per 10,000 living birth ,this prevalence was used to calculate sample size by using online calculator).(1), their age range were 18 to 55 (elder adults) and 56 to 97(older adults). Data collection tool which was used in this research was 15D Quality of life questionnaire to find out the influence of hearing loss (HL) on daily living activities in adult’s life. Sampling technique used in this research was convenient sampling. For this purpose, descriptive analysis was done for demographics and chi square was used for inferential analysis by using SPSS20. Endnote was used for references. Results: There are four domains of quality of life (speech, usual activities, mental functioning, and vitality status of participants) which have highly significant relationship value(<0.001) with hearing impairment, in other domains of quality of life p value is less then (<0.05) this means that other domains of quality of life have also significant relationship with hearing impairment except visual or sexual activity of the participants which have no significant association p- value(>0.05)with hearing impairment. Conclusion:  Research concluded that domains of quality of life are significantly associated with hearing loss which have negative impact on the QOL(quality of life) except visual or sexual activity, but these two domains may also indirectly have associated or may affect the quality of life individually in adults with hearing impairment.

scholarly journals Hearing Impairment and Quality of Life in Adults with Asymmetric Hearing Loss: Benefits of Bimodal Stimulation

2019 ◽  
Vol 15 (1) ◽  
pp. 62-69 ◽  
Author(s):  
Ignacio Sanhueza ◽  
◽  
Raquel Manrique-Huarte ◽  
Diego Calavia ◽  
Alicia Huarte ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Hearing Impairment ◽  
Bimodal Stimulation ◽  
Asymmetric Hearing Loss

Quality of life in patients with untreated age-related hearing loss

2010 ◽  
Vol 124 (8) ◽  
pp. 835-841 ◽  
Author(s):  
H S Chew ◽  
S Yeak
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Hearing Impairment ◽  
Health Survey ◽  
Short Form ◽  
The Elderly ◽  
Short Form 36 ◽  
Age Related ◽  
Hearing Handicap

AbstractBackground:Hearing loss commonly affects quality of life in the elderly, yet is often neglected.Objectives:To investigate the impact of untreated age-related hearing loss on the quality of life of elderly individuals, and to assess the usefulness of quality of life questionnaires as screening tools for significant hearing loss.Methods:We recruited 80 patients aged 50 years or more with untreated hearing impairment. The Short Form 36 Health Survey and the Hearing Handicap Inventory for the Elderly Screening Version questionnaire were administered.Results:There was no significant association between severity of hearing impairment and Short Form 36 Health Survey scores. However, dose-graded correlation was observed between severity of hearing loss and Hearing Handicap Inventory for the Elderly Screening Version questionnaire scores (p < 0.001). A score for the latter questionnaire of more than 8 was 72.8 per cent sensitive and 71.4 per cent specific in detecting clinically significant hearing loss of at least 40 dB (receiver operating characteristic = 0.83).Conclusion:The Short Form 36 Health Survey, a generic measure, lacked specificity and sensitivity in detecting clinically significant hearing loss. However, significant hearing impairment was reflected in the Hearing Handicap Inventory for the Elderly Screening Version questionnaire scores, suggesting that this is a good, disease-specific screening tool. A combination of functional (i.e. the Hearing Handicap Inventory for the Elderly Screening Version questionnaire) and physiological (i.e. audiometric) assessment is recommended to investigate hearing loss in elderly individuals.

scholarly journals Hearing impairment and associated socio-demographic characteristics in patients with tinnitus

2021 ◽  
Author(s):  
Danish A. Khan ◽  
Pramod K. Varshney ◽  
M. Yasir Zubair ◽  
M. Anas
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Hearing Impairment ◽  
Rural Areas ◽  
Early Recognition ◽  
Pure Tone Audiometry ◽  
Study Data ◽  
Considerable Proportion ◽  
Socio Demographic Factors

Background: Tinnitus is a common ear problem and causes various somatic and psychological effects that has a bearing on the quality of life. Considerably high prevalence, distressing nature of the problem and the fact that it is associated with hearing impairment warrants an early recognition and appropriate timely intervention.Methods: A total of 120 patients (60 with tinnitus and 60 without tinnitus) were included in the study. Data of putative socio-demographic factors and clinical examination findings were collected on a semi-structured questionnaire.Results: The mean age of patients with tinnitus was 38.12±13.39 while that of patients without tinnitus was 40.85±15.26 (p=0.299). Around two-thirds (63.33%, 95% CI, 51.3–75.7%) of patients with tinnitus were males and the other one-third (36.67%, 95% CI, 24.3–48.7%) were females. Two-thirds (66.7%, 95% CI, 54.6–76.7%) of patients with tinnitus belonged to urban locality and the remaining one-third (33.3%, 95% CI, 23.3–45.4%) were from rural areas. On pure tone audiometry (PTA), hearing impairment was detected in 32 (53.3%, 95% CI, 42.0–68.3%) patients with tinnitus as compared to 13 (21.7%, 95% CI, 9.3–35.4%) patients without tinnitus.Conclusions: Tinnitus was associated with hearing loss in considerable proportion irrespective of patients’ own awareness of hearing loss. Therefore, patients should be educated regarding importance of seeking early healthcare. Although the benefits of available treatments are small, and the perception of tinnitus does not wane completely, it improves quality of life and reduces distress resulting from tinnitus.

Audiology Services in India

2011 ◽  
Vol 1 (1) ◽  
pp. 21-26 ◽  
Author(s):  
Kalyani Mandke ◽  
Rashmi Deshpande
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Social Economic ◽  
World Health ◽  
Low Profile ◽  
The World ◽  
Life Issues ◽  
Health Organization ◽  
The Individual

Hearing impairment is common throughout the world, and it is estimated that 50% of all deafness and hearing impairment is preventable. The World Health Organization (WHO, 1999) estimates that there are 255 million people worldwide with a hearing impairment; of those, two-thirds live in the developing countries. Although hearing loss is not a death causing disease, the implication of hearing impairment for the individual, family, and community is tremendous. The far-reaching implications of hearing loss, both in terms of development of communication skills as well as social, economic, and quality of life issues, warrant highlighting the magnitude and severity of the problem. This disability cannot be “seen” and, therefore, has been low-profile. At the same time, hearing loss programs in India are less developed than programs for individuals diagnosed with blindness. It is well documented that hearing loss and deafness is preventable, provided it is detected early and managed properly through appropriate health education and program development.

scholarly journals Prevalence and etiology of hearing impairment in urban area of Shimla, Himachal Pradesh, India: a cross sectional observational study

2017 ◽  
Vol 5 (4) ◽  
pp. 1252
Author(s):  
Trilok C. Guleria ◽  
Shobha Mohindroo ◽  
Narender K. Mohindroo ◽  
Ramesh K. Azad
Keyword(s):  
Hearing Loss ◽  
Observational Study ◽  
Hearing Impairment ◽  
Urban Area ◽  
Himachal Pradesh ◽  
Age Group ◽  
Cross Sectional ◽  
Sensitive Organ ◽  
Loss Maximum

Background: The ear is a marvelously complex and sensitive organ. Disease of the ear have profound effect on the health and quality of life of millions of people around the globe. Data regarding the magnitude of hearing impairment in our country is limited and the literature search revealed that no such studies have been conducted in this region.Methods: A community based cross sectional observational study was carried out among the 306 individuals in urban area of Shimla, Himachal Pradesh, India. Information was obtained by a structured questionnaire, clinical ENT examination and audiological tests after obtaining informed consent.Results: The study group had 48.4% males and 51.6% females. Maximum 29.4% of individuals were in the age group of 31-45 years. Mean age was 32.22±20.15. The prevalence of hearing impairment was 13.1%. Among individuals with hearing loss, maximum 57.5% were in the age group of ≥60 years. Sensorineural hearing loss was found in 70%, maximum 55% had mild hearing loss. Among majority of individuals with hearing loss cause was presbyacusis 57.5% followed by infectious ear disease 27.5%. Among majority of individuals with hearing loss 37.5% required no further action at the time of study.Conclusions: In the present study, prevalence of hearing loss was found to be 13.1% and predominantly mild sensorineural or conductive type of hearing loss. An early intervention and quality patient education was necessary for prevention of hearing impairment in majority of cases.

scholarly journals Auditory cortex hypoperfusion: a metabolic hallmark in Beta Thalassemia

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Renzo Manara ◽  
Sara Ponticorvo ◽  
Silverio Perrotta ◽  
Maria Rosaria Barillari ◽  
Giuseppe Costa ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Auditory Cortex ◽  
Pure Tone ◽  
Chelation Therapy ◽  
Clinical Laboratory ◽  
Clinical Phenotype ◽  
Iron Chelation ◽  
Pure Tone Audiometry ◽  
Cognitive Test ◽  
Beta Thalassemia

Abstract Background Sensorineural hearing loss in beta-thalassemia is common and it is generally associated with iron chelation therapy. However, data are scarce, especially on adult populations, and a possible involvement of the central auditory areas has not been investigated yet. We performed a multicenter cross-sectional audiological and single-center 3Tesla brain perfusion MRI study enrolling 77 transfusion-dependent/non transfusion-dependent adult patients and 56 healthy controls. Pure tone audiometry, demographics, clinical/laboratory and cognitive functioning data were recorded. Results Half of patients (52%) presented with high-frequency hearing deficit, with overt hypoacusia (Pure Tone Average (PTA) > 25 dB) in 35%, irrespective of iron chelation or clinical phenotype. Bilateral voxel clusters of significant relative hypoperfusion were found in the auditory cortex of beta-thalassemia patients, regardless of clinical phenotype. In controls and transfusion-dependent (but not in non-transfusion-dependent) patients, the relative auditory cortex perfusion values increased linearly with age (p < 0.04). Relative auditory cortex perfusion values showed a significant U-shaped correlation with PTA values among hearing loss patients, and a linear correlation with the full scale intelligence quotient (right side p = 0.01, left side p = 0.02) with its domain related to communication skills (right side p = 0.04, left side p = 0.07) in controls but not in beta-thalassemia patients. Audiometric test results did not correlate to cognitive test scores in any subgroup. Conclusions In conclusion, primary auditory cortex perfusion changes are a metabolic hallmark of adult beta-thalassemia, thus suggesting complex remodeling of the hearing function, that occurs regardless of chelation therapy and before clinically manifest hearing loss. The cognitive impact of perfusion changes is intriguing but requires further investigations.

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