Deaf Mutism Research Articles

Comparative Study between Dexmedetomidine vs Midazolam-Fentanyl on Emergence Agitation in Children Undergoing Cochlear Implantation in Sohag University Hospital

ABSTRACT Background: Surgery of cochlear implantation is a great advance in otology for patients with deaf mutism, but it carries a great challenge to the anesthesiologist. Objective: The aim of the current work was to compare effect of bolus Dexmedetomidine infusion versus bolus Midazolam- fentanyl infusion as regards; emergence agitation & recovery time, hemodynamics, postoperative pain and complication. Patient and Methods: This study included a total of 40 pediatric patients (ASA I or II), undergoing cochlear implantation, attending at Sohag University Hospital. They were randomized divided into dexmedetomidine (D) group and midazolam (M) group. Results: There was no significant difference between both groups as regard intraoperative mean blood pressure. There was a significant difference at discharge time. There was statistically insignificant decrease in heart rate (HR) in group D than M group. There was statistically significant difference at 1 min and discharge measurements. There was a highly significant difference between both groups as the time for recovery being more rapid in D group than in M group. Mask Acceptance Scale (MAS) was better within D group than M group without statistically significant difference. There was a no significant difference in emergence agitation between both groups. There was no significant difference between both groups as regard objective pain score. As regard nausea and vomiting, there was a highly significant difference between both groups. Conclusion: It could be concluded that dexmedetomidine infusion in cochlear implantation in pediatric patients was equal as midazolam-fentanyl in inducing hypotension, emergence agitation and giving post-operative analgesia. However, dexmedetomidine infusion is better as regardrapid recovery without inducing nausea and vomiting.

Alexander Lobanov: The Reception of the Political in Soviet Outsider Art

The analysis of the oeuvre of the outsider artist Alexander Lobanov (1924–2003) reveals the mechanisms of influence of Soviet visual propaganda. This article examines the total influence of ideology and visual narratives on an artist even when they seem to have been completely excluded from artistic life (in Lobanov’s case, due to his deaf mutism). The author refers to Irving Goffman’s self-presentation theory, the works on political power and its influence by Boris Groys, and the works of psychologists on the peculiarities of compensatory activity in the deaf and mute. The work is relevant as it reflects the importance of outsider artists as part of the art process. The author mostly refers to works by Lobanov in non-Russian and Russian private and institutional collections, as well visual propaganda from the Soviet period. Works by Lobanov and other Soviet outsider artists have not been studied from the perspective chosen by the article’s author. The artist’s oeuvre is examined by Élisabeth Anstett from the discourse point of view, connected with visual images from Soviet propaganda. The author of the article singles out the peculiarities of the appropriation by the outsider artist of the ideological visual narrative, the circle of borrowed images (militarism, hero, Stalin – “the father of nations”), and certain approaches (recurrence, ornamentality, and the presence of text). As a result of an artistic and formalist analysis, the author reveals specific borrowings of approaches and stylistics from the Soviet art (poster, illustration, and easel painting) of the 1930s to the mid‑1950s. Additionally, an important aspect of the study is that the author reveals the peculiar intellectualisation of compensatory mechanisms in the construction of social representation addressing the dominant images of visual Soviet propaganda. The interdisciplinary approach of the research, with certain exceptions, could be used to analyse the creative work of other Soviet outsider artists.

Hearing balance and communication jubilees exponents of the “twenties”: Dagobert Schwabach (1846–1920) and his tuning fork test

Purpose: To honour the 100 year death jubilee of Dagobert Schwabach (1846–1920), and his eponymly named tuning fork test.Method: We will present original documentation and historical references.Result: After a biography of Schwabach, a paragraph will present the original description of the test, followed by another paragraph discussing its acceptation by his colleagues.Conclusion: Schwabach published numerous articles on hearing tests, deaf mutism and statistisc of deaf mutism, collecting many cases of otological interest.

ERRATUM to "Good clinical practice advice: Thyroid and pregnancy" [Int J Gynecol Obstet 144(2019) 347-351

The International Journal of Gynecology and Obstetrics regrets that, in the above article, an error appeared in the section, Requirement of iodine ‘Requirement of iodine’ on page 348, 1st paragraph, line 11–12: “Children born to mothers with severe iron deficiency during pregnancy exhibited cretinism, characterized by profound mental retardation, motor rigidity, and deaf mutism”. should have been: “Children born to mothers with severe iodine deficiency during pregnancy exhibited cretinism, characterized by profound mental retardation, motor rigidity, and deaf mutism”.

Screening of 35delG, W24X, W77X and Q124X Variants in connexin 26 gene associated with Non-Syndromic Hearing Impairment

Introduction: Hearing deficiency is the most common sensory incompetence that affects millions of people all over the world. Profound Deafness is a common disorder that occurs in 1:1000 neonates and the cause is hereditary. Gap Junction Beta2 (GJB2) gene which codes for connexion 26 protein is implicated in hearing. Mutation or defect in this gene leads to the most common form of the congenital disorder called Deaf Mutism (DM). The present study aims to screen the common variants associated with hearing impairment. Objective: To Screen common variants 35delG, W24X, W77X, and Q124X in connexin 26 gene of the North Karnataka population. Methods: 613 deaf-mute children from districts of North Karnataka were screened and 184 Non Syndromic Sensorineural Hearing-impaired children were included in the study. Genomic DNA was isolated using peripheral blood. Our gene of interest was amplified by Polymerase Chain Reaction (PCR) using target-specific primers. The PCR product was subjected to sequencing to analyze the mutations. Result: In our population prevalence of W24X, W77X variants were 25% & 5.4% respectively and 35 del G and Q124X mutations were absent. Along with this common variant, I33T (5.4%) and R127H (14.6%) were also observed. Conclusion: above findings suggests that Gap junction Beta 2 gene plays a crucial role in hearing impairment. Mutation analysis of the entire GJB2 gene in our population may help to define the etiology of deafness as well as the development of population-specific prenatal and perinatal genetic markers.

A Cross-sectional study assessing the Prevalence ofIodine Deficiency Disorders among school children aged6 to 12 years in Tumakuru district of Karnataka

Background Iodine deficiency disorders IDD constitute a major public health problem in India. Deficiency of iodine can cause physical and mental retardation cretinism abortions stillbirth deaf mutism squint amp various types of goitre. Methodology A community ndash based cross section survey was undertaken during the February-March 2019 in Tumakuru district of Karnataka to find out prevalence of goitre estimate urinary iodine excretion in 6-12 years old school children and household salt iodine concentrations. 2875 school children aged 6-12 years were examined for goitre. Urine samples of 270 childrenwere collected for estimation of urinary iodine using Sandell-Kolthoff method. Household salt samples of the 540 selected children from schools were analyzed for its iodine content by standard iodometric titration method. Results The overall prevalence of Goitre was found to be 9.5 among children examined. The median urinary iodine excretion UIE was 104 mugL. All the household salt samples were adequately iodized having Iodine content of gt15ppm. Conclusion Considering prevalence of goitre in 6-12 years children Tumakuru district is lsquoendemicrsquo for IDD. But as inferred from median UIE obtained IDD is not a public health problem of significance. Household level salt is adequately iodized as per NIDDCP recommendations

SEX & AGE-WISE DISTRIBUTION AND CAUSES OF DYSPHONIA IN CHILDREN

Background: Dysphonia is common in children due to which they suffer adversely both at home and at school. The objectives of this study were to determine the sex & age-wise distribution and causes of dysphonia in children. Materials & Methods: This descriptive cross-sectional study was conducted at the Department of ENT and Head & Neck surgery, Hayatabad Medical Complex, Peshawar, Pakistan from January 1, 2017 to December 31, 2018. Sample size consisted of 72 patients presenting with chronic dysphonia, selected using the non-probability consecutive sampling technique Inclusion criteria were children presenting with dysphonia for more than 4 weeks. Exclusion criteria were children with stammering, puberphonia, acute respiratory distress, deaf mutism, speech articulation and increased or decreased nasal twang in voice. The demographic variables were sex and age groups and research variables were causes of dysphonia. All variables except age being categorical were analyzed by frequency and percentages carried out using SPSS 16.0 for windows. Results: Mean age of the sample of patients was 8.78+3.6 with range of 12 years (3-15). Out of 72 patients, 44(61.11%) were boys and 28(38.89%) girls with boy to girl ratio of 1.57:1. Chronic nonspecific laryngitis was the commonest etiology followed by reflux laryngitis. Functional dysphonia was noted only in girls and vocal nodules, vocal hematoma and post traumatic laryngeal stenosis were noted only in boys. Conclusion: Chronic nonspecific laryngitis and reflux laryngitis were the commonest causes especially in males less than 9 years of age. Vocal nodules, vocal hematoma and post traumatic laryngeal stenosis were found only in boys and functional dysphonia was noted only in adolescent girls.

Geographical distribution and occurrence of Endemic Goitre

AIM- The aim of this article is to write review the geographical distribution and occurrence of endemic goiter OBJECTIVE- In this review, we will understand the etiology of the disease and its treatment. We will also understand how the goiter occurs in people with respect to their location near the coast and away from it. BACKGROUND- Endemic goiter is the swelling of the thyroid gland due to iodine deficiency. This type of goiter is very common in people living in regions away from the shore where the iodine resources are very minimal. Some common symptoms seen in hyperthyroidism are palpitations, nervousness, tremor, etc. Hypothyroid individuals have weight gain despite poor appetite, cold intolerance, constipation and lethargy. Treatment involves medical and surgical techniques. In case of iodine deficiency, the person is asked to consume more iodine in his/her diet. If the goiter is associated with an underactive thyroid, thyroid supplements are used as treatment. In extreme cases, a partial or complete thyroidectomy is required (removal of the goiter). However numerous causes attribute to goiter. They may include vitamin A deficiency, reduced carotene content and a defect in calcium metabolism .Goiter is also accompanied by other disorders like deaf mutism and cretinism. REASON- From this article one can gather all information about the endemic goiter and its geographical distribution occurring worldwide.

Prevalence of neonatal hypothyroidism in Kangra Valley, Himachal Pradesh.

Iodine deficiency (ID) is an endemic health problem in Kangra District, Himachal Pradesh (HP). ID in pregnant mothers leads to neonatal hypothyroidism (NH), mental retardation, deaf mutism, squint, dwarfism, spastic dysplasia, neurological defects and congenital anomalies. NH can be assessed by estimating the thyroid stimulating hormone (TSH) in cord blood samples. The present study was conducted with an objective to assess the prevalence of NH in district Kangra, HP. In district Kangra, all the hospitals providing obstetric services were enlisted. Three hospitals conducting more than 100 deliveries per year were selected randomly. A total of 613 umbilical cord blood samples of neonates were collected on filter papers and analyzed for TSH. TSH was estimated by enzyme-linked immunosorbent assay method. Neonates with TSH levels ⩾20 mIU/l were recalled for reassessment of TSH for confirmation of NH. Prevalence of NH was found to be 4.4%. This finding suggests the need for the implementation of a neonatal screening program for early detection of children with ID.

Iodine nutrition status amongst neonates in Kangra district, Himachal Pradesh

Iodine deficiency (ID) is an endemic health problem in Kangra district, Himachal Pradesh (HP) state. ID leads to mental retardation, deaf mutism, squint, dwarfism, spastic diplegia, neurological defects and congenital anomalies. Iodine nutrition status amongst neonates can be assessed by estimating thyroid stimulating hormone (TSH). The present study was conducted with an objective to assess the iodine nutrition status amongst Neonates in Kangra district, HP. All of the hospitals in the district which provide obstetric services were enlisted, of which three were selected for this survey. A total of 613 umbilical cord blood samples of neonates were collected on filter paper and analyzed for TSH. WHO (2007) reported that that a <3% frequency of TSH concentrations above 5mIU/L in samples collected 3–4 days after birth indicates iodine sufficiency in a population. In our study we found that 73.4% of the neonates had TSH levels of more than 5mlU/l, thus indicating ID in the population studied. Iodine deficiency continues to be a public health problem in Kangra district, Himachal Pradesh.

Iodine Nutritional Status Among Neonates in the Solan District, Himachal Pradesh, India

Iodine nutrition status amongst neonates can be assessed by estimating thyroid stimulating hormone (TSH). According to WHO, if more than 3 % of the neonates have TSH levels of 5 mlU/l and more in a population, it indicates presence of iodine deficiency (ID). Iodine deficiency is an endemic health problem in Solan district, Himachal Pradesh (HP) state. ID leads to mental retardation, deaf mutism, squint, dwarfism, spastic diplegia, neurological defects and congenital anomalies. The aim is to determine iodine nutrition status of neonates of Solan district. In Solan district, six hospitals/community health centers providing obstetric services and conducting more than 100 deliveries per annum were identified and enlisted. Two hospitals were selected keeping in view of operational feasibility. A total of 683 umbilical cord blood samples of neonates were collected on filter paper and analyzed for TSH. It was found that 63.2 % of the neonates had TSH levels of more than 5 mlU/l indicating iodine deficiency in the Solan district. Iodine deficiency was a public health problem in Solan district, HP.

Iodine deficiency, thyroid function and hearing deficit: a review

Iodine deficiency affects an estimated 241 million school-aged children in the world. Little is known about iodine deficiency in relation to auditory function, except for the fact that deaf-mutism is one of the features of cretinism. In the present review, we documented the scientific knowledge on the role of iodine and hypothyroidism in the auditory system. We found that ear development and hearing function depend on thyroid hormones. Multiple pathways are involved in this, including both inner ear morphology as well as neurological processes. Conductive as well as sensorineural hearing loss is found in studies with animals that were rendered hypothyroidic. In humans, auditory impairment is reported frequently in relation to hypothyroidism, ranging from mild disturbances to severe handicap. Auditory impairment has been related more explicitly to congenital hypothyroidism than to acquired hypothyroidism. The critical period for thyroid function-related hearing maturation is the first and second trimesters of pregnancy. Although only a limited number of studies have directly investigated the relationship between iodine deficiency and auditory function, most studies point toward an association. However, evidence from good randomised controlled trials is lacking. Inclusion of auditory outcomes in iodine supplementation studies is therefore to be recommended, especially for trials in pregnancy. Hearing deficit is an invisible abnormality, but has major consequences for educational and social skills if not detected. In view of this, auditory impairment should be mapped in iodine-deficient areas in order to realistically estimate the magnitude of the problem.

Interactive Sound Glove System for deaf mutism - Mainly with Kodaly Method -

Interactive Sound Glove System for deaf mutism - Mainly with Kodaly Method -

Clinical profile of coexisting conditions in type 1 diabetes mellitus patients

AimsType 1 diabetes mellitus (T1DM) is associated with various genetic and autoimmune diseases implicated in its etiopathogenesis. We hereby profile the clinical association of such diseases among patients from our center. MethodsConsecutive patients of T1DM presenting to department of Endocrinology from May 1997 to December 2011 were retrospectively analyzed in context of associated clinical profile. ResultsAmong 260 patients diagnosed as T1DM, 21 (8%) had hypothyroidism, 4 (1.5%) had hyperthyroidism and 2 (0.7%) had primary adrenal insufficiency. Eighteen patients (7%) had celiac disease, 9 (3.5%) had Turner's syndrome, 5 patients (1.9%) had Klinefelter's syndrome, whereas Down's syndrome and Noonan's syndrome was present in 2 and 1 patients (0.7%) respectively. One patient had Wolframs’ syndrome and 1 patients had myasthenia gravis. Systemic lupus erythematosus and rheumatoid arthritis were present in 3 and 1 patients respectively. Total of 5 patients with cerebral palsy, 4 cases with deaf mutism, 4 cases with acute psychosis and 16 patients with depression were noted. Mean age of study patients was 20.8±9.8 years (range, 3–23 years). ConclusionVarious conditions including genetic (Down, Turner, Noonan, and Klinefelter's), autoimmune (thyroid and adrenal disorders, myasthenia gravis, SLE, rheumatoid arthritis) and central nervous system diseases were the associated diseases encountered in our patients. Routine screening is required for early diagnosis and treatment of associated co morbidities.

Cretinism revisited

Endemic cretinism includes two syndromes: a more common neurological disorder with brain damage, deaf mutism, squint and spastic paresis of the legs and a less common syndrome of severe hypothyroidism, growth retardation and less severe mental defect. Both conditions are due to dietary iodine deficiency and can be prevented by correction of iodine deficiency before pregnancy. Endemic cretinism is now included in the spectrum of the effects of iodine deficiency in a population termed the 'iodine deficiency disorders (IDDs)', which also includes a wide range of lesser degrees of cognitive defect that can be prevented by the correction of iodine deficiency. Iodine deficiency is now recognised by the World Health Organization (WHO) as the most common preventable cause of brain damage with in excess of 2 billion at risk from 130 countries. A global United Nations (UN) programme of prevention has achieved 68% household usage of iodised salt by the year 2000 compared with less than 20% prior to 1990.

Hypothyroidism: Clinical Features

Hypothyroidism: Clinical Features

Changing trends in otorhinolaryngological diseases at a non-government clinic in Jaipur

Otorhinolaryngological (ENT) diseases are major health problem in India but the trends in occurrence of various disorders in clinical practice have not been well studied. To assess the types of such diseases and to determine changing trends in their incidence we performed this study. The study was done at a non-government ENT center at Jaipur. From 1975 to 2005, >125000 patients of different diseases were evaluated here. The study includes randomly selected patients (n = 11454) from years 1980 to 2000 at an interval of 5 years to evaluate disease trends. Significance of trends was evaluated using least squares regression. In the years 1980, 1985, 1990, 1995 and 2000 mean age of patients was 26.60 ± 17.81 (range 0.16 to 85), 27.07 ± 16.91 (0.08 to 90), 28.30 ± 17.73 (0.25 to 90), 28.79 ± 17.8 (0.25 to 90) and 28.74 ± 17.81 (0.25 to 85) years respectively. 51 types of ENT diseases were observed of which 19 contributed to 76.8% (8807) patients and analysis was restricted to them. Chronic suppurative otitis media (2203, 19.2%), otitis externa (859, 7.5%), deviated nasal septum with nasal obstruction (717, 6.3%) and chronic tonsillitis (695, 6.1%) were the most common, followed by ear wax (569, 4.9%), sensorineural hearing loss (545, 4.7%), chronic rhinosinusitis (428, 3.7%) and epistaxis (320, 2.8%). There was increasing trend for stomatitis (b = 0.0014), deviated nasal septum (b = 0.0290), allergic rhinitis (b = 0.0023), epistaxis (b = 0.0002), acute tonsillitis (b = 0.0003), hoarseness (b = 0.0017), deaf mutism (b = 0.0005), sensorineural hearing loss (b = 0.0038), tinnitus (b = 0.0006) and ear wax (b = 0.0050). Declining trend was observed for chronic rhinosinusitis (b = -0.0155), otitis externa (b = -0.0063), chronic suppurative otitis media (b = -0.0001), acute otitis media ( = -0.0007), secretory otitis media (b = -0.0013), otosclerosis (b = -0.0007), vertigo (b = -0.0007), neck swelling (b = -0.0005) and chronic tonsillitis (b = -0.0194). This study from an Indian urban ENT center shows a significantly increasing trend in chronic and degenerative ear diseases and decline in infection related diseases.

Clinical profile of special children at asha centre

Background: The care of special children suffering from cerebral palsy, deaf mutism, mental retardation (MR) and post encephalitic sequelae etc. is done in the armed forces at “ASHA” centre supported by Army Wives Welfare Association (AWWA). Methods: The clinical profile and underlying etiological factors in these children were studied. Result: Out of 30 children studied, majority were males. The commonest disability was cerebral palsy, seen in 13 (43%) cases followed by mental retardation in six (20%) and post encephalitic sequlae in four (13%) cases. Convulsions were noticed in 12 (40%) cases. The delayed speech was a significant handicap observed in 27 (90%) cases. Conclusion: Among the etiological factors, natal causes and infections are leading factors in these children and there is an urgent need to strengthen the existing maternal and child health services in our country.

A Rare Association of Deaf Mutism, Syndactyly and Squamous cell carcinoma of the Temporal bone

Malignant tumours of the temporal bone are rare, the majority being squamous cell carcinoma. We here report a rare syndromic complex of squamous cell carcinoma of the temporal bone, deaf-mutism & syndactyle in a 12 year old male child, presented in Department of Otorhinolaryngology, Hamidia Hospital, Bhopal, with one month history of mass in left external auditory canal & lower motor neuron facial nerve palsy on a background of left chronic suppurative otitis media since childhood.

Cochleosaccular dysplasia: a morphometric and histopathologic study in a series of temporal bones.

The objective of this study was to perform a morphometric analysis of a series of temporal bones with cochleosaccular dysplasia to clarify the extent of inner ear changes in this disease. This human temporal bone histopathologic study of a series of deaf-mute cases involves morphometric analysis, including stria vascularis and spiral ligament area measurements and spiral ganglion and hair cells counts. Thirteen temporal bones were selected from 35 with deaf mutism based on the histopathologic findings described by Scheibe. Twenty normal age-matched control subjects were used for comparisons. All temporal bones had the main histopathologic findings described by Scheibe, as well as severe affected stria vascularis. Seven temporal bones had cystic areas in the stria and three had concretions. Cross-sectional strial areas in temporal bones with cochleosaccular dysplasia were smaller than normal in all cochlear turns; however, no difference was found in spiral ligament cross-sectional areas. Reissner's membrane was hydropic in three temporal bones and the organ of Corti was absent in at least one cochlear turn in five. Concretions were present in the macula of seven temporal bones. Twelve temporal bones showed some level of spiral ganglion cell loss. No hair cells were observed in any temporal bone. A familial history of deafness was found in three cases. Pathologic findings were variable and limited to the saccule and scala media. The variation, perhaps, reflects the different etiologies involved in the origin of cochleosaccular dysplasia.