Type Of Deafness Research Articles

Clinical Value of 3D-FLAIR MRI in Idiopathic Sudden Sensorineural Hearing Loss

Three-dimensional fluid-attenuated inversion recovery sequence magnetic resonance imaging (3D-FLAIR MRI) has been used in the diagnosis of inner ear diseases. However, the relevance of 3D-FLAIR MRI appearances with multiple features and prognosis of patients with idiopathic sudden sensorineural hearing loss (ISSNHL) remains unclear. This study was a retrospective trial. We recruited 1300 patients with unilateral ISSNHL hospitalized from May 2017 to January 2019. They were divided into four groups according to their 3D-FLAIR MRI appearances: normal (n = 739), inner ear hemorrhage (n = 218), increased protein content (n = 288), and blood-labyrinth barrier damage (n = 55). The correlation between 3D-FLAIR MRI appearances and the degree or type of deafness of the participants was analyzed. There was a statistical difference in the deafness side (p < 0.001) and vestibular dysfunction (p < 0.001) among the four groups. There was a statistical difference in the duration of treatment (p < 0.001) and the incidence of dizziness or vertigo (p < 0.001) for patients among these groups. The degree of deafness in the patients in the inner ear hemorrhage group was significantly more severe than that of the patients in the other three groups (p < 0.001). 3D-FLAIR MRI appearances were correlated with the prognosis of patients with ISSNHL.

Analysis on the correlation of auditory brain/stem response and 40 Hz auditory event related potential by pure tone audiometry in adult patients with sudden deafness

Objective:To study the value of auditory brainstem response（ABR） and 40 Hz auditory evoked potential（40Hz AERP） in adult patients with sudden deafness. Methods:Pure tone audiometry, ABR and 40 Hz AERP were performed in 184 adult patients with sudden deafness before treatment. According to the frequency and severity of hearing loss revealed by pure tone audiometry, the patients were divided into low-frequency decline type（86 cases）, high-frequency decline type（60 cases）, flat type（32 cases） and total deafness type（6 cases, statistical analysis were not conducted in this group due to the incomplete elicitation of reaction threshold）. Data from 178 ears were collected for statistical results. After treatment, pure tone audiometry, ABR and 40 Hz AERP were performed. Results:As for ABR threshold, its correlation with 500 Hz threshold of subjective pure tone test was poor（r=0.233, P=0.706）, and was worse with 1000 Hz threshold（r=0.472, P=0.345）. ABR threshold was closely correlated with 2000 Hz threshold（r=0.878, P=0.021） and 4000 Hz threshold（r=0.800, P=0.010） of subjective pure tone test. As for 40 Hz AERP threshold, its correlation with 500 Hz threshold of subjective pure tone audiometry was good（r=0.992, P=0）, and was better with 1000 Hz threshold（r=0.912, P=0.110）. 40 Hz AERP threshold was poorly correlated with 2000 Hz threshold（r=0.210, P=0.690） and 4000 Hz threshold（r=0.370, P=0.945） of subjective pure tone audiometry. ABR and subjective pure tone audiometry have high correlation at high frequency, while 40 Hz AERP and subjective pure tone audiometry have high correlation at low frequency. Conclusion:ABR and 40 Hz AERP can comprehensively reflect the severity of hearing loss in adult patients with sudden deafness.

DEPRESSIVE SYMPTOMATOLOGY AMONG IN-SCHOOL ADOLESCENTS WITH IMPAIRED HEARING

Purpose of the study: This study determined incidences of depressive symptoms and associated causative contextual factors among in-school adolescents with impaired hearing in South West, Nigeria. The study determined symptoms of depression exhibited by in-school adolescents with impaired hearing vis-à-vis some associated intrapersonal causative (age, religion, gender, the onset of hearing loss, satisfaction with the home environment, and family type) variables.&#x0D; Methodology: Descriptive survey design was adopted for the study. Data were collected from 236 adolescents who are either prelingually (39.1%) and postlingually (60.9%) hearing impaired with the Depressive Symptomatology Questionnaire (α = .89). Data collected over a period of 10 weeks were analysed with both descriptive and inferential statistics via SPSS version 23.&#x0D; Main Findings: 58.8% of the participants scored higher on the depression scale, which is mostly characterized by loss of appetite and fearfulness. There is a significant difference (F = 7.61; p &lt; 0.05) in the expression of depressive symptoms between gender and across variants in the onset of deafness (F = 3.59; p &lt; 0.05) and family type (F = 23.14; p &lt; 0.05). Academic stressor and lack of access to information were found to be the major source of their depression.&#x0D; Applications of this study: This study is of importance to counselling psychologist and mental health practitioners for the development of strategic therapeutic approach geared towards positive psychosocial development of individuals with impaired hearing&#x0D; Novelty/Originality of this study: The study confirms the manifestation of moderate to a high level of depressive symptoms among those in-school adolescents with impaired hearing. Academic anxiety/low grade, communication difficulties, and negative societal attitude were the major source of depression for adolescents with impaired hearing in high schools.

Acoustic and Speech Perceptual Evaluation of health related Quality of Life among adults with Chronic Suppurative Otitis Media

Background: Chronic Suppurative Otitis Media, patients suffer from hearing loss causing communication problems and social withdrawal. Objective: To assess the acoustic iand speech perceptual evaluation iof health related quality of life among adults iwith Chronic Suppurative Otitis Media. Methodology: This cross sectional study was performed in ENT/Audiology Departments of District Headquarter Hospital, Hafizabad and Saqib Bashir General Hospital, Hafizabad, from March, 2015 to September, 2018, after the approval of synopsis. Covenient sampling method was used to induct 101 patients with Chronic Suppurative Otitis Media meeting inclusion criteria. Pure itone audiometry and speech audiometry were performed. A statement based questionnaire COMOT-15 was used to measure the scoring difference in cases. Results: The mean age of patients was 33±10 years and 53 (52.47%) were male. Out of 101 patients,the speech deficit was moderate in 7 (6.93%) patients, moderately severe in 19 (18.81%), severe in 42 (41.58%) and speech deficit was profound in 33 (32.67%) patients. There were 10 (9.90%) patients with conductive type of hearing loss, 91 (90.09%) with mixed type of hearing loss. Chronic Otitis Media Outcome Test-15 (COMOT-15), overall score was between 41-50 in 11 (10.89%) patients, 51-60 in 62 (61.38%) patients and Chronic Otitis Media Outcome Test-15 (COMOT-15) overall score was between 61-70 in 28 (27.72%) patients. Conclusion: This study showed that speech deficit was severe to profound in majority of patients with Chronic Suppurative Otitis Media. Mixed type of hearing loss is commonest type of deafness and very high scores were observed on COMOT-15 scale, which reflects a significant impairment in quality of life.

Correlation between effectual time and the curative effect in patients with all frequency descending sudden deafness after treatment.

To discuss the correlation between effectual time and the curative effect in patients with all frequency descending sudden deafness. According to effectual time, the subjects were divided into first week effectual group and second week effectual group and the curative effect of each group was compared. In patients with flat descent sudden deafness, the curative rate of the first week effectual group was higher than that of the second week effectual group, but there was no significant difference between the two groups (χ2 = 1.584, P = 0.208). Meanwhile, the total significant effective rate of the first week effectual group was higher than that of the second week effectual group, without obvious difference between the two groups (χ2 = 0.227, P = 0.634). Furthermore, in patients with total deafness type of sudden deafness, the curative rate of the first week effectual group was higher than that of the second week effectual group, showing no remarkable difference between the two groups (χ2 = 2.726, P = 0.099). Besides, there was no remarkable difference in the comparison of the total significant effective rate (χ2 = 2.933, P = 0.087), which was higher in the first week effectual group than that in the second week effectual group. The course of treatment should be at least 2 weeks in patients with all frequency descending sudden deafness after onset.

Decline in prevalence of congenital sensorineural deafness in Dalmatian dogs in the United Kingdom.

BackgroundCongenital sensorineural deafness (CSD) is the most common type of deafness in Dalmatian dogs.ObjectivesTo use results of CSD screening in Dalmatian dogs in the United Kingdom in genetic analysis and to determine any changes in the prevalence of CSD in this breed over time.AnimalsA total of 8955 Dalmatian puppies undergoing hearing function screening using brainstem auditory evoked response (BAER) between July 1992 and February 2019.MethodsResults of BAER testing and pigmentation phenotypic data were linked to the UK Kennel Club Dalmatian pedigree database. Mixed model analysis was used to estimate variance parameters.ResultsThe overall prevalence of CSD was 17.8% (13.4%, unilateral; 4.4%, bilateral). Heritability of CSD was approximately 0.3 (across models) and significantly >0. Genetic correlations between CSD and blue irises (+0.6) and pigmented head patch (−0.86) were large in magnitude and significantly different form 0. Significant improving phenotypic and genetic trends were identified, likely as the result of selection against deafness, equivalent to avoiding breeding with the 4% to 5% of animals with the highest genetic risk of CSD.Conclusions and Clinical ImportanceA decrease in the prevalence and genetic risk of CSD implies breeders have been selecting for hearing dogs. Selective breeding based on estimated breeding values (EBVs) can help further decrease the prevalence of CSD in Dalmatians in the future.

Polymerase Chain Reaction and Serology Test to Detect Rubella Virus in Congenital Rubella Syndrome Patients with Hearing Loss

Rubella infections in pregnant women, especially during the first trimester, often lead to manifest as congenital rubella syndrome (CRS). This syndrome consists of several inherited disorders such as deafness, cataracts, and cardiac abnormalities. Deafness is the most common manifestation of CRS, 70-90% of all cases with deafness type are sensory neural deafness. The mechanism of hearing loss due to rubella virus is caused by hypoxia resulting from endothelial vascular damage in cochlea, followed by cell death in the organ of Corti and stria vascularis.Cochlea’s stria damage makes alterations in the composition of endolymph. This study aimed to confirm rubella infection using PCR and serological tests in patients with CRS who were clinically deaf. A suspected CRS reported was a 2-month-old patient with an unclear response to surround sound. The patient had been already diagnosed with congenital cataracts before. Prenatal, perinatal and postnatal history was evaluated. Hearing screening was done by OAE (Biological), and AABR (Beraphone). Anti-rubella IgM and IgG was obtained from blood serum. PCR was carried out from a throat swab and the eye-lens specimen. OAE and AABR results ‘refer’ to both sides of the ear indicated a sensorineural hearing loss. The serological examination of IgM was positive with an index of 15.00 and IgG rubella positive with a titer&gt; 500 IU / ml. Positive results from the rubella virus were identified from the throat swab and lens specimens using the PCR method. Detection of rubella virus in CRS sufferers with clinical deafness of both ears gives positive results using PCR and serology methods. In the future PCR can be potential for prenatal diagnosis of rubella virus aimed at the provision of early prevention.

Evaluation of Gene Variants in TGFB1, SERPINF1 and MEPE in a Spanish Family Affected by Otosclerosis and Tinnitus

Otosclerosis (OTSC) is a common type of deafness affecting up to 0.4 % of Caucasians. Its familial form is inherited in an autosomal dominant fashion, although to this date, no definitive cause for OTSC has been found. In the development of OTSC, three recent genetic association studies have suggested the participation of particular point mutations and small indels in the TGFB1, SERPINF1, and MEPE genes. Consequently, replicative studies are needed to confirm the role of the proposed mutations in OTSC patients. The goal of this study was to test the presence of the candidate variants described in the genes TGFB1, SERPINF1, and MEPE in a new case of familial OTSC with seven affected individuals. DNA was extracted from saliva samples of a Spanish family with several members affected by OTSC. PCR amplified target regions of some candidate genes, and the products were purified, Sanger-sequenced, and analyzed in silico. The family subject of the study did not carry the candidate variants for OTSC described in the genes TGFB1, SERPINF1, and MEPE, although it cannot be ruled out the involvement of other mutations in genes related to their same signaling pathways. This result highlights the importance of performing replicative studies for complex diseases, such as OTCS, in families of diverse origins. Additionally, a significant association of subjective tinnitus with OTSC has been found in this family, although the link between the two pathologies should be studied further.

Effect of tympanic dexamethasone injection in the treatment of different types of sudden deafness

Objective:The aim of this study is to explore the best administration, timing and efficacy of dexamethasone and Mison in the treatment of different types of sudden deafness. Method:242 cases of sudden deafness first diagnosed in our department were selected. According to the guidelines（2015）, the patients were divided into low frequency descending type （49 cases）, high frequency descending type （66 cases）, flat descending type （71 cases） and total deafness （56 cases）. Different types of patients were randomly divided into tympanic injection group and systemic administration group on the basis of routine treatment. Tympanic injection group was further divided into initial injection group and delayed injection group. Tympanic injection was performed under ear endoscope, once every other day, three times for low frequency descending deafness, and five times for other types of deafness. Result:In comparison of total effective rate, there were significant differences among the three treatments in 49 cases of low frequency descending type, 71 cases of flat descending type and 56 cases of total deafness type （P<0.05）. In 66 cases of high frequency descending type, there was no significant difference among the three treatments （P>0.05）. In the comparison of cure rate, the difference of cure rate among the three treatment methods was also significant in low frequency descending type （P<0.05）. In the other three types of deafness, there was no significant difference among the three treatment methods （P>0.05）. There was no significant difference in the effective rate between men and women （P>0.05） in all patients treated by tympanic injection. There was significant difference in the effective rate of tympanic injection within 7 days of onset and 7 days after onset （P<0.05）. Conclusion:Intratympanic injection of dexamethasone is safe, effective, and easy to use as an initial treatment for low frequency descent, flat, and full deafness, and the sooner the better.

Hearing Level Comparison of Noisy and Silent Area

This prospective study was carried out in the Department of ENT and Head Neck Surgery, Sheikh Hasina medical college, Tangial, Bangladesh in a period of 6 months from January 2017 July 2017 among those complaining hearing loss attending in ENT out patient department. The aim of the study was to find the out pattern of hearing loss and hearing level comparison between noisy area (such as bazaar, bus stands, factory, school etc) to silent area. In this study 103 cases of hearing impaired people were taken and the data were collected by interviewing the cases as per questionnaire from history, examination and investigation report. Working place of most of the hearing impaired people was in noisy area 88 (85.4%). Among them most of the patients was male (58%) and female was (42%) and male:female ratio 1.4:1. The commonest type of hearing loss was sensorineural type. In the right ear 55 cases (53.4%) of sensorineural type deafness, 19 cases (18.4%) of mixed type of deafness and 21 cases (20.4%) of conductive type deafness. In the left ear 46 cases (47.7%) of sensorineural type deafness, 18 cases (17.5%) of mixed type deafness and 24 cases (23.3%) of conductive type deafness. This study showed that male (58%) were mostly affected than female (42%) and vehicle drivers (25.5%) were commonest group of people. From this study we got both ears were involved by disease process in 77.6% of patients and only right ear was 14.6% and only left ear was 10% of the patient of hearing loss.&#x0D; Bangladesh J Otorhinolaryngol; October 2017; 23(2): 159-163

A CASE OF MID-FREQUENCY SENSORINEURAL HEARING LOSS

Introduction: Mid-frequency sensorineural hearing loss (MFSNHL) is an unusual audiometric finding with a debatable etiology with unclear long-term results. In general, the middle frequencies are affected first, and the process progresses slowly to include all frequencies. There are also some cases where provocative factors have been reported. Purpose: The aim of the work is to present a clinical case in our practice of the rare occurrence of mid-frequency acoustic hearing loss and its diagnostic approach. Material and methods: The patient has undergone a number of manual and instrumental tests to confirm or reject a specific cause of the hearing loss condition. Results: A 52-year-old man was hospitalised with complaints of hearing loss for about a year, judging by the growing difficulty in making a phone call. Conclusions: Generally, a U-shaped audiogram is thought to indicate hearing loss of genetic origin. However, this type of deafness is not unique to one particular type of genetic mutation. There are also numerous descriptions of non-genetic mid-frequency hearing loss in the literature. In the particular clinical case presented by us, it was concluded that this was a hearing loss, possibly of hereditary nature, after excluding another provocative cause, as well as due to anamnestic data on premature hearing loss in one of the parents.

Estetyczne aspekty tworzenia i odbioru sztuki przez osoby z wadami słuchu

The aim of the article is to analyse the aesthetic aspects of the reception and creation of art by people with hearing impairments. For most people, hearing loss is unimaginable and is often understood only in the context of communication problems. Meanwhile, deaf people perceive their otherness completely differently. Deafness is a total experience, affecting not only the physical aspect, but also changing the perception of reality as a whole. Divergences in the way of perceiving the world, resulting from the exclusion of one of the basic senses, are particularly visible in the field of art, especially in those areas that use sound effects. The large variety of types of deafness does not allow for broad generalizations or building some kind of universal theory of reception. Therefore, from the perspective of deaf people, contact with art is very individual and intimate, which also affects the issue of their artistic creation. The author of the article touches upon the problem of the visuality of sign language and its artistic possibilities. She analyses the perception preferences of people with hearing impairments, exposing the issue of polysensory perception, and presents the most interesting artistic projects created both by the deaf and those that allow to build multidirectional relations between the parallel worlds of the deaf and hearing people.

Estetyczne aspekty tworzenia i odbioru sztuki przez osoby z wadami słuchu

The aim of the article is to analyse the aesthetic aspects of the reception and creation of art by people with hearing impairments. For most people, hearing loss is unimaginable and is often understood only in the context of communication problems. Meanwhile, deaf people perceive their otherness completely differently. Deafness is a total experience, affecting not only the physical aspect, but also changing the perception of reality as a whole. Divergences in the way of perceiving the world, resulting from the exclusion of one of the basic senses, are particularly visible in the field of art, especially in those areas that use sound effects. The large variety of types of deafness does not allow for broad generalizations or building some kind of universal theory of reception. Therefore, from the perspective of deaf people, contact with art is very individual and intimate, which also affects the issue of their artistic creation. The author of the article touches upon the problem of the visuality of sign language and its artistic possibilities. She analyses the perception preferences of people with hearing impairments, exposing the issue of polysensory perception, and presents the most interesting artistic projects created both by the deaf and those that allow to build multidirectional relations between the parallel worlds of the deaf and hearing people.

Congenital or Early Acquired Deafness: An Overview of the Portuguese Situation, from Diagnosis to Follow-Up

Congenital deafness or early acquired deafness affects 1 to 3 out of 1000 newborns without risk factors and 20 to 40 out of 1000 newborns with risk factors. The universal newborn hearing screening enables its early identification. Children with congenital deafness/early acquired deafness have a higher prevalence of other conditions, especially ophthalmologic and neurodevelopmental ones, and at least 30% to 40% have at least one associated comorbidity. We carried out a cross-sectional, multicenter study in which 83% (n = 30) of the hospitals/maternity hospitals of the National Health Service participated. All surveyed hospitals/maternity hospitals routinely performed universal newborn hearing screening to all newborns before discharge; 63% referred children with risk factors for hearing loss to Otorhinolaryngology. All children with congenital deafness/early acquired deafness are referred to: Pediatrics in 23% hospitals/maternity hospitals. In 23 hospitals/maternity hospitals, all children with congenital deafness/early acquired deafness are referred to: Speech Therapy in 44% hospitals/ maternity hospitals; Ophthalmology in 17% hospitals/maternity hospitals; National System of Early Intervention in Childhood in 30% hospitals/maternity hospitals; 22% of hospitals/maternity hospitals refer all children with congenital deafness/early acquired deafness, with no identified cause, to Clinical Genetics clinics. The number of diagnoses of deafness in the years 2014 and 2015 was 2.5 and 1.5 per 1000 newborns, respectively, in 15 hospitals/maternity hospitals. Awareness of universal newborn hearing screening seems to be widely spread in the National Health Service. The number of children with SC / SPA, as well as the percentage of different types of deafness diagnosed, were identical to those found in other studies and shows its importance. The assessment / follow-up of these children by specialties other than the otolaryngology was heterogeneous in different health entities and revealed that not all children with risk factors for deafness follow up advised by existing standards. Results show that Portugal made an important path in the screening and follow-up of children with SC / SPA. It is important, with the ultimate aim of continually improving the care of these children, to reflect on the involvement of specialties other than otolaryngology, such as the National Early Childhood Intervention System in the follow-up of these children.

Correlation analysis of incidence, season and temperature parameters of different types of sudden deafness

Objective:The objective of this study was to investigate the correlation between the onset of different types of sudden sensorineural hearing loss（SSNHL） with temperature parameters and seasons. Method:We retrospectively reviewed the medical charts of 175 patients who were diagnosed as SSNHL, precisely collected the exact date and city of onset, confirmed the season, and obtained the meteorological data including maximum temperature（Tmax）, minimum temperature（Tmin）, mean temperature（T）, day-to-day change of mean temperature（ΔT）, and diurnal temperature range（Trange） at the same day, then analyzed the relation between season and temperature with the onset of different types of SSNHL. Result:There was a significant difference of Trange between different types of SSNHL（P=0.001）. Trange on the onset date of all-frequency SSNHL（including flat and profound type） was significantly higher than low and high frequency descending type（P=0.001, P<0.05 respectively）. Types of SSNHL had weak association with Trange groups（P=0.03, Cramer's V=0.220）. An increase of 1℃ in Trange increased the risk of flat type SSNHL by 23.9% and 16.5% compared with low and high frequency descending type, respectively, and for profound type, the risk was increased by 22.4% and 15.1%. No significant differences were observed between seasons and SSNHL types（P=0.666）. Conclusion:The incidence of different types of sudden sputum may be related to the worse temperature on the day, and has nothing to do with the disease season.

The correlation between plasma fibrinogen level and different types of sudden sensorineural hearing loss

Objective: To investigate the correlation between plasma fibrinogen level and the incidence of sudden hearing loss. Method: A total of 225 patients (age ranging from 18 to 70 years) with sudden sensorineural hearing loss(SSNHL) were selected. The disease onset was within 2 weeks. No patient received previous medical intervention. Depending on the types of auditory threshold curve, SSNHL was divided into low frequency descending type, high frequency descending type, flat desending type and total deafness type. Two hundred and twenty-nine cases with normal hearing and no inflammatory diseases in the same period was selected as control group. Fibrinogen level and Blood Routine Indexes were detected for the purpose of retrospective cohort study.Result: The levels of fibrinogen in SSNHL group(2.98±0.59) g/L were significantly higher than that in control group(2.66±0.36) g/L (P<0.01). According to the types of auditory threshold curve, SSNHL groups were divided into group A(55 cases, 24.44%), Group B(36 cases, 16.00%), Group C(43 cases,19.11%) and Group D(91 cases, 40.44%). The average fibrinogen levels before treatment were at(2.75±0.46)g/L, (3.16±0.61) g/L, (3.02±0.63) g/L and(3.03±0.63) g/L respectively. There was no significant difference in fibrinogen level(P=0.286) between group A and the control group. Fibrinogen levels of group B, group C and group D were significantly elevated compared to control group (P<0.01). Conclusion: The level of fibrinogen is not significantly correlated with development of sudden deafness of low frequency descending type. The incidence of high frequency descending type, flat descending type and profound deafness type are all correlated with the level of high fibrinogen at the onset of sudden deafness. The classification of sudden deafness based on audiogram curves could be of great significance for analysis of possible causes and selection of treatment options.

MON-376 A Novel Mutation in VHL Gene as Posible Cause of Metastasic Pheocromocytoma

A 62-year-old woman with no family history of hereditary diseases presented to the ER department in 2008 with the diagnosis of a subarachnoid hemorrhage that required urgent decompressive craniotomy. On February 2011, due to resistant hypertension, an abdominal CT scan was performed, revealing a left adrenal gland-dependent tumor of 70x86 mm, with additional increased normetanephrine levels in a 24-hour urine sample. Laparoscopic left adrenalectomy was performed on June 3, 2011, with a histological report of pheochromocytoma. It was also documented an elevation of iPTH (301 pg/ml) with corrected calcium of 11 mg/dL and phosphorus of 2.7 mg/dL. Parathyroidectomy was performed with the report of parathyroid adenoma. On January 2015, an abdominal CT scan was performed, revealing recurrence of the pheochromocytoma at the surgical site. A MIBG PET-CT confirmed the recurrence and detected a left retroperitoneal lesion close to the tail of the pancreas. Metastasectomy of the retroperitoneal lesions, distal pancreatectomy, and dissection of tumor adjacent to left kidney were performed with a histopathologic report of pheochromocytoma with nest pattern and extensive necrosis, negative margins, and three positive lymph nodes. During follow-up a significant increase of normetanephrine in 24-hour urine sample was documented (3 months after surgery). A Ga-DOTATOC PET-CT revealed the presence of hepatic metastases. In the 131-MIBG SPECT-CT performed one year later, a decreased height of C3 vertebral body with an abnormal concentration of the radiopharmaceutical in relation to secondary deposit was also found. On August 2017 the patient was treated with a therapeutic dose of 131-MIBG, achieving complete biochemical remission during 14 months of follow-up. On March 2018 an Invitae Multi-Cancer Panel reported the germline variant C. 416C > G (P. Ser139Cys) related to VHL without mutations in other pathogenic genes related to pheochromocytoma. VHL c.416C>G (at the cDNA level ) is a germline variant of Von Hipple Lindau (VHL) disease, also known as p.Ser139Cys (S139C) at the protein level. It results in the change of a Serine to a Cysteine (TCT>TGT), located in 3p25.3. This variant has been reported in population databases (rs587780732, ExAC 0.06%) and related to familiar erythrocytosis, monogenic diabetes (likely benign) and a type of autosomal recessive A1 deafness (uncertain significance); however, to our knowledge it has not been previously published in the literature as a pathogenic nor benign form of the VHL disease. It was also not observed in the NHLBI Exome Sequencing Project that included 6,500 individuals of European and African American ancestry, indicating it is not a common benign variant in these populations. Because of the lack of evidence to determine its role in disease, to date, it is considered a variant of uncertain significance.

Hypnotherapy in the treatment of conversion disorder blindness and deafness type

Conversion disorder is characterized by the loss of one or several neurological functions, with no apparent organic cause. In such cases, vision may be compromised in up to 5% of the patients in ophthalmologic emergencies. Hypnosis is a suggestion technique that has been used for years in the treatment of conversion disorder with positive results. Reported here is the case of a patient with conversion disorder, blindness, and deafness type whose evolution was satisfactory after one session of hypnotherapy.

Study of Incidence and Type of Deafness in Children Withdelayed and Non Development of Speech in Apediatric Tertiary Health Care Centre Among 5500 Childrenover 5 Years

Study of Incidence and Type of Deafness in Children Withdelayed and Non Development of Speech in Apediatric Tertiary Health Care Centre Among 5500 Childrenover 5 Years

Effect of Propolis Combination with Cefadroxil in Accelerating Clinical Improvement in Benign Chronic Suppurative Otitis Media

Chronic suppurative otitis media has a high prevalence and is a health problem in the community. The study aims to assess the effect of propolis combined with cefadroxil in accelerating clinical improvement in patients with benign type CSOM. This study used experimental clinical trials, carried out at the clinic of ENT-HN RSUP Dr. Wahidin Sudirohusodo and Unhas Hospital during the period of October-December 2018. A total of 15 samples were divided into 2 groups, namely: the treatment group (given propolis 2 x 200 mg therapy combined with cefadroxil 2 x 500 mg and the control group (cefadroxil 2 x 500 mg and methylprednisolone 2x4 mg) In this study, the initial and final examination of treatment was carried out, namely: physical examination of ENT, Pure Tone Audiometry (PTA), laboratory, and endoscopy. The data were presented in the form of tabulations and narratives. The results show that administration of propolis and cefadroxil show improved hearing through a decrease of 7.09% air conduction compared to the control group and a 13.06% decrease in bone conduction compared to the control group, while inflammatory improvement marked by a decrease in leucocyte levels was 3.68% compared to the control group, a decrease in neutrophil levels 3.8% compared to the control group, the decrease in eosinophil levels was 4.71% compared to the control group while the size of the perforation, type and degree of deafness do not occur between the treatment and control groups. From the results of this study it can be concluded that in patients with benign type CSOM for the treatment group given propolis therapy combined with cefadroxil better and faster clinical improvement (tympanic membrane mucosa is not hyperemic and secretions dry), namely: on the day 3 to 7 days compared to the control group given cefadroxil and methylprednisolone therapy clinical improvement occurred in the 7th to 11th day range.