Type Of Deafness Research Articles

Molecular and Clinical Characterization of a Cohort of Autosomal Recessive Sensorineural Hearing Loss in Egyptian Patients

Hearing loss (HL) is one of the most common health problems worldwide. Autosomal recessive non-syndromic sensorineural hearing loss (ARNSHL) represents a large portion of congenital hereditary HL. Our study was conducted on 13 patients from 13 unrelated families. The majority of patients presented with congenital severe to profound bilateral sensorineural HL. All patients were subjected to detailed family history and three-generation pedigree analysis to exclude any environmental cause and to ensure an autosomal recessive mode of inheritance. Molecular analysis was performed using the whole exome sequencing (WES) technique for the recruited patients. Three variants in the MYO7A and OTOF genes were reported for the first time in patients with ARNSHL (one nonsense, one frameshift, and one splice variant). Ten previously reported variants were detected in seven genes (GJB2, MYO15A, BSND, OTOF, CDH23, SLC26A4, and TMIE). They varied between missense, nonsense, frameshift, and splice variants. This study expands the molecular spectrum of two types of autosomal recessive deafness (types 2 and 9).

Otosyphilis-A Type of Refractory Deafness.

Otosyphilis-A Type of Refractory Deafness.

Genotype-phenotype analysis of hearing function in patients with DFNB1A caused by the c.-23+1G>A splice site variant of the GJB2 gene (Cx26).

The audiological features of hearing loss (HL) in patients with autosomal recessive deafness type 1A (DFNB1A) caused by splice site variants of the GJB2 gene are less studied than those of patients with other variants of this gene. In this study, we present the audiological features of DFNB1A in a large cohort of 134 patients with the homozygous splice site variant c.-23+1G>A and 34 patients with other biallelic GJB2 genotypes (n = 168 patients with DFNB1A). We found that the preservation of hearing thresholds in the speech frequency range (PTA0.5,1.0,2.0,4.0 kHz) in patients with the c.[-23+1G>A];[-23+1G>A] genotype is significantly better than in patients with the "severe" c.[35delG];[35delG] genotype (p = 0.005) and significantly worse than in patients with the "mild" c.[109G>A];[109G>A] genotype (p = 0.041). This finding indicates a "medium" pathological effect of this splice site variant on hearing function. A detailed clinical and audiological analysis showed that in patients with the c.[-23+1G>A];[-23+1G>A] genotype, HL is characterized as congenital or early onset (57.5% onset before 12 months), sensorineural (97.8%), bilateral, symmetrical (82.8%), variable in severity (from mild to profound HL, median hearing threshold in PTA0.5,1.0,2.0,4.0 kHz is 86.73±21.98 dB), with an extremely "flat" audioprofile, and with a tendency toward slow progression (a positive correlation of hearing thresholds with age, r = 0.144, p = 0.041). In addition, we found that the hearing thresholds in PTA0.5,1.0,2.0,4.0 kHz were significantly better preserved in females (82.34 dB) than in males (90.62 dB) (p = 0.001). We can conclude that in patients with DFNB1A caused by the c.-23+1G>A variant, male sex is associated with deteriorating auditory function; in contrast, female sex is a protective factor.

Analysis of clinical characteristics and curative effects of 169 patients with bilateral sudden sensorineural hearing loss

Objective: To analyze the clinical characteristics, curative effect related factors and follow-up situation of bilateral sudden sensorineural hearing loss (BSSHL). Methods: The clinical data of 169 patients(338 ears) with BSSHL were retrospectively summarized, and the demographic characteristics, predisposing factors, concomitant symptoms and diseases, and audiological characteristics were statistically described. Additionally, influencing factors of curative effect and prognosis were statistically analyzed. Results: Among the 169 patients, 50.9% (86/169) of patients had at least one incentive, with cold and fatigue being the most common incentives(both 23/169). There were high rates of accompanying symptoms including tinnitus (150/169, 88.8%) and dizziness (100/169, 59.2%). Hypertension(49/169, 29.0%)and diabetes(23/169, 13.6%)were the most common concomitant diseases observed. Most cases exhibited all frequencies involvement, with flat type and total deafness type accounting for 83.1%(281/338 ears). The most common degree of hearing loss was total deafness(86/338 ears), with approximately 60.1%(203/338 ears) of the cases being severe or worse. The total effective rate of treatment was only 29.0%, but it increased to 39.5% for patients with course of disease≤14 days, however, when course of disease>30 days, the effective rate decreased sharply to 3.6%, showing a significant difference between these two groups(χ2=13.776,<0.01). Different types of hearing curves showed statistically significant difference in efficiency(χ2=14.782, P<0.01). Comparing the hearing improvements of 28 BSSHL patients from admission to discharge and from discharge to follow-up, it was found that the hearing improvement of the two groups showed statistically significant difference at the frequencies of L-250 Hz, L-500 Hz, R-125 Hz, R-250 Hz and R-500 Hz(Z value was -2.495, -3.083, -3.970, -3.388 and -3.264 respectively, all P<0.05). The proportion of patients with elevated serum IgE was much higher than that of the normal population. Conclusion: BSSHL patients suffer from serious hearing loss, and many also experience tinnitus and vertigo symptoms. Due to the poor efficiency of treatment, it is better for patients to be treated within 30 days of onset. For patients of hearing loss in the low frequency range, hearing improvement is more significant during hospitalization. And the occurrence of BSSHL may involve an immune mechanism.

Metrology analysis and thoughts of literatures on tinnitus associated with sudden deafness based on CiteSpace

Objective: To explore the incidence of sudden deafness accompanied with tinnitus, the selection of examination protocols and treatment, and to provide reference for the establishment of new guidelines for sudden deafness. Methods: CiteSpace software was used for analysis and data mining to analyze and summarize the computer-retrieved articles on diagnostic examination and treatment of sudden deafness accompanied with tinnitus collected from CNIC, Wanfang and Web of Science databases from 2011 to 2021. Results: A total of 207 randomized controlled studies were retrieved in this study, including 121 in Chinese and 86 in English. Finally, 74 Chinese literatures and 16 English literatures were included. Among the 74 valid Chinese literatures, 64 (86.5%) were accompanied with tinnitus, 58 (78.4%) with dizziness/vertigo, 25 (33.8%) with aural fullness, 10 (13.5%) with headache, 4 (5.4%) with insomnia, 4 (5.4%) with a mixture of dizziness and tinnitus, and 2 (2.7%) with vomiting. Among the 16 English literatures, 15 (93.8%) were accompanied with tinnitus, 12 (75.0%) with vertigo, 1 (6.3%) with aural fullness, and 1 (6.3%) with a mixture of various symptoms. Among the 64 Chinese articles mentioning tinnitus, only 9 mentioned tinnitus matching tests, and 1 mentioned that the treatment for tinnitus accompanying symptoms was sound therapy and psychological counseling. The incidence rates of tinnitus accompanying four different types of sudden deafness, from low to high, are as follows: low-to-mid frequency, 82.4%; mid-to-high frequency, 90.7%; complete deafness, 92.4%; and flat type, 92.8%. Conclusion: Tinnitus is the most common accompanying symptom of sudden deafness, and tinnitus matching test is an effective evaluation method. When establishing a scientific, comprehensive, and systematic diagnosis and treatment system or guidelines for sudden deafness, attention should be paid to the diagnosis and treatment of tinnitus symptoms and their adverse psychological reactions, in order to reduce the incidence of tinnitus patients in the later stage of recovery from sudden deafness.

Carrier screening for 223 monogenic diseases in Chinese population: a multi-center study in 33 104 individuals

To investigate the epidemiological characteristics and mutation spectrum of monogenic diseases in Chinese population through a large-scale, multicenter carrier screening. This study was conducted among a total of 33 104 participants (16 610 females) from 12 clinical centers across China.Carrier status for 223 genes was analyzed using high-throughput sequencing and different PCR methods. The overall combined carrier frequency was 55.58% for 197 autosomal genes and 1.84% for 26 X-linked genes in these participants.Among the 16 669 families, 874 at-risk couples (5.24%) were identified.Specifically, 584 couples (3.50%) were at risk for autosomal genes, 306(1.84%) for X-linked genes, and 16 for both autosomal and X-linked genes.The most frequently detected autosomal at-risk genes included GJB2(autosomal recessive deafness type 1A, 393 couples), HBA1/HBA2(α-thalassemia, 36 couples), PAH (phenylketonuria, 14 couples), and SMN1(spinal muscular atrophy, 14 couples).The most frequently detected X-linked at-risk genes were G6PD (G6PD deficiency, 236 couples), DMD (Duchenne muscular dystrophy, 23 couples), and FMR1(fragile X syndrome, 17 couples).After excluding GJB2 c.109G>A, the detection rate of at-risk couples was 3.91%(651/16 669), which was lowered to 1.72%(287/16 669) after further excluding G6PD.The theoretical incidence rate of severe monogenic birth defects was approximately 4.35‰(72.5/16 669).Screening for a battery of the top 22 most frequent genes in the at-risk couples could detect over 95% of at-risk couples, while screening for the top 54 genes further increased the detection rate to over 99%. This study reveals the carrier frequencies of 223 monogenic genetic disorders in the Chinese population and provides evidence for carrier screening strategy development and panel design tailored to the Chinese population.In carrier testing, genetic counseling for specific genes or gene variants can be challenging, and the couples need to be informed of these difficulties before testing and provided with options for not screening these genes or gene variants.

Prevalence of Ear and Nasal Diseases in Bangladeshi Patients

Background: The magnitude of health problems related to ear and nose in Bangladesh has not been estimated in a larger scale and very little is known about the prevalence and types of ENT diseases. Commonest cause of hearing impairment in our country is middle ear infection. Conductive type of deafness mostly occurs due to otitis media with or without its complications. Goal of primary health eare services is to achieve "Sound hearing by 2030" will never be achieved if we could not find out possible association of middle ear disease with other health conditions. Objective: To determine the prevalence of ear and nasal diseases in Bangladeshi patients. Methods: Two hundred patients complaining of middle ear discharge and or nasal symptoms were included in this study, to see the association of COM with nasal disorders. This cross sectional study was conducted in Dhaka Medical College Hospital from July 2012 to June 2014.Sample was taken by purposive sampling. Sample was divided into four groups as exposed and diseased, exposed and not diseased, not exposed diseased and not exposed and not diseased. Results: Two hundred patients were studied in the department of ENT and Head Neck Surgery of Dhaka Medical College Hospital complaining of either COM or Sinonasal disease. Among them 107 were female and 93 were male and male female ratio was 1:1.15. On analyzing age distribution it was ranging from 10 years to 50 years with mean age 30±7.86% clustering around 25 years. This study revealed that 65 % of COM is in the age group of 10-30 years. Conclusion: A study on the prevalence of the Ear Nose throat and Head and Neck diseases in developing country like Bangladesh can provide basic data which can be relevant and beneficial to the development of medical ENT curriculum in the regional setup and subsequent medical practice.

Whole Exome Sequencing of Non-Syndromic Hearing Loss Patients.

Hearing loss is the second most common disease after mental retardation in Iran. Autosomal recessive non-syndromic hearing loss (ARNSHL) is an extreme and highly heterogeneous disease, for which more than 70 genes have been identified. Considering the frequency of family marriage as well as the importance of ARNSHL in Iran, we evaluated the genetic factors involved in this type of deafness. We performed the whole exome sequencing (WES) of eight Iranian subjects with severe nonsyndromic hearing loss selected from 110 well-characterized subjects with non-syndromic hearing loss from 2017-2019. The patients with mutated GJB2 and GJB6 genes were excluded from the study. The use of the whole exome sequencing method revealed 10 different mutations in 7 genes, including SLC26A4 (c.1234G>T), FGF3 (c.45DelC, c.466T>C), ADGRV1 (c.12528-2A>C, c.16226-16227insAGTC), OTOG (c.7454delG), OTOF (c.3570+2T>C), ESPN (c.992G>A), OTOA (c.2359G>T, c.2353A>C). Seven new variants were observed in seven families including SLC26A4 (c.1234G>T), FGF3 (c.45DelC), ADGRV1 (c.12528-2A>C), OTOG (c.7454delG), ADGRV1 (c.16226-16227insAGTC), OTOF (c.3570+2T>C). The causal mutation of ARNSHL was found in all patients using the WES. Meta-analysis studies can help to identify common mutations causing deafness in any population to facilitate identification of carriers and subjects with deafness.

Gene therapy: an emerging therapy for hair cells regeneration in the cochlea.

Sensorineural hearing loss is typically caused by damage to the cochlear hair cells (HCs) due to external stimuli or because of one's genetic factors and the inability to convert sound mechanical energy into nerve impulses. Adult mammalian cochlear HCs cannot regenerate spontaneously; therefore, this type of deafness is usually considered irreversible. Studies on the developmental mechanisms of HC differentiation have revealed that nonsensory cells in the cochlea acquire the ability to differentiate into HCs after the overexpression of specific genes, such as Atoh1, which makes HC regeneration possible. Gene therapy, through in vitro selection and editing of target genes, transforms exogenous gene fragments into target cells and alters the expression of genes in target cells to activate the corresponding differentiation developmental program in target cells. This review summarizes the genes that have been associated with the growth and development of cochlear HCs in recent years and provides an overview of gene therapy approaches in the field of HC regeneration. It concludes with a discussion of the limitations of the current therapeutic approaches to facilitate the early implementation of this therapy in a clinical setting.

The GJB2 (Cx26) Gene Variants in Patients with Hearing Impairment in the Baikal Lake Region (Russia).

The GJB2 (Cx26) gene pathogenic variants are associated with autosomal recessive deafness type 1A (DFNB1A, OMIM #220290). Direct sequencing of the GJB2 gene among 165 hearing-impaired individuals living in the Baikal Lake region of Russia identified 14 allelic variants: pathogenic/likely pathogenic-nine variants, benign-three variants, unclassified-one variant, and one novel variant. The contribution of the GJB2 gene variants to the etiology of hearing impairment (HI) in the total sample of patients was 15.8% (26 out of 165) and significantly differed in patients of different ethnicity (5.1% in Buryat patients and 28.9% in Russian patients). In patients with DFNB1A (n = 26), HIs were congenital/early onset (92.3%), symmetric (88.5%), sensorineural (100.0%), and variable in severity (moderate-11.6%, severe-26.9% or profound-61.5%). The reconstruction of the SNP haplotypes with three frequent GJB2 pathogenic variants (c.-23+1G>A, c.35delG or c.235delC), in comparison with previously published data, supports a major role of the founder effect in the expansion of the c.-23+1G>A and c.35delG variants around the world. Comparative analysis of the haplotypes with c.235delC revealed one major haplotype G A C T (97.5%) in Eastern Asians (Chinese, Japanese and Korean patients) and two haplotypes, G A C T (71.4%) and G A C C (28.6%), in Northern Asians (Altaians, Buryats and Mongols). The variable structure of the c.235delC-haplotypes in Northern Asians requires more studies to expand our knowledge about the origin of this pathogenic variant.

Reclassification of flat type sudden deafness

Objective: To reclassify the flat type sudden deafness according to the types of audiogram shape, and to explore the correlation between different pattern of hearing loss and prognosis. Methods: All of 1 024 patients with unilateral sudden deafness (492 males and 532 females, aged from 19 to 65 years, with an average age of 41.2 years old) admitted to 33 hospitals nationwide from August 2007 to October 2011 were divided into four types according to Chinese Guideline of Sudden Deafness(2015): low-frequency, high-frequency, flat and total deafness. Then, 402 patients with flat type sudden deafness were further divided into ascending type, descending type and consistent type according to the audiogram shapes. First, we compared the clinical characteristics and prognosis among these three subtypes of flat deafness, then compared the clinical characteristics and prognosis between ascending flat deafness and low-frequency deafness, descending flat deafness and high-frequency deafness, consistent flat deafness and total deafness, explored the factors related to the prognosis of flat deafness. SPSS 21.0 software, ANOVA, χ2 test, t-test and Logistic regression were used to analyze the data. Results: The cure rates of flat ascending, flat descending and flat consistent sudden deafness groups were 70.7%, 17.1% and 34.0% respectively, with a statistically significant difference (χ2=33.984, P<0.001); However, there was no significant difference in age, sex and affected side (all P>0.05). The independent related factors for the recovery of flat type sudden deafness were as follows: whether there was dizziness [OR=0.459; 95% confidence interval (CI): 0.271-0.777], the type of audiogram shape (OR=0.721; 95%CI: 0.530-0.981), and days from onset to therapy (OR=0.903, 95%CI: 0.835-0.978), all of which had P values<0.05. There was no significant difference in the cure rates between ascending flat sudden deafness and low-frequency descending sudden deafness, descending flat sudden deafness and high-frequency descending sudden deafness (all P>0.05). The pure tone average(PTA) of flat consistent sudden deafness and total deafness were (69.1±18.9) and (101.7±17.7) dB HL, respectively, with a statistically significant difference (t=20.890, P<0.001), and the cure rates were 34.0% and 14.5%, respectively, with a statistically significant difference (χ2=29.012, P<0.001). Conclusion: According to the audiogram shape, the flat type sudden deafness can be further divided into ascending flat sudden deafness, descending flat sudden deafness and consistent flat sudden deafness, which can more effectively evaluate the prognosis. The cure rate of ascending flat sudden deafness is similar to that of low-frequency sudden deafness, and the prognosis is well; The cure rate of descending flat sudden deafness is similar to that of high-frequency descending sudden deafness, and the prognosis is poor. The cure rate of consistent flat sudden deafness is higher than that of total deafness. PTA plays an important role in the prognosis of consistent flat sudden deafness and total deafness. Total deafness can be regarded as a single type of sudden deafness.

Characteristics of deafness in the elderly patients at Sanglah general hospital period march 2020-march 2021

Introduction: Elderly is defined as an individual who has reached the age of 60 years and over. the number of elderly population in Indonesia currently reaches 28.7 million people, 10.6% of the total population of Indonesia. In 2020 Bali has entered the aging population, namely when the percentage aged 60 years and over reaches more than 10%. In line with the increasing life expectancy of people in Indonesia, health problems for the elderly will increase. One of the health problems of the elderly is hearing loss. Pathological changes in the hearing organ due to the degeneration process in the elderly can cause deafness. Objective: To determine the characteristics of deafness in elderly patients at Sanglah General Hospital during the period March 2020-March 2021. Methods: This study used a retrospective descriptive research design with cross sectional design, by taking secondary data from medical records of elderly patients with deafness who came to the ENT’s polyclinic at Sanglah General Hospital during the period March 2020-March 2021. Result: Characteristics deafness in elderly patients based on gender, the most found in male group compare to female group (53,3%: 46,5%), the largest age group was in the 60-70 years group as many as 33 people or 56.9%. Based on audiometric examination 39 elderly patients with deafness or 67.2% with sensorineural deafness. Characteristics based on the degree of deafness in elderly patients most found intensity in range between 56-70 dB, 20 people or 34.5% who experienced moderate deafness. The most common based on the side of the ear 53 people with deafness (91.4%) on both sides of the ear or bilateral. Conclusion: This study found that deafness in elderly patients is more commonly found in male, at age groups 60-70 years old with the most type of sensorineural deafness type, the most degree of deafness is moderate to severe degree, and deaf complaints on the both sides.

Deletion of the Notch ligand Jagged1 during cochlear maturation leads to inner hair cell defects and hearing loss

The mammalian cochlea is an exceptionally well-organized epithelium composed of hair cells, supporting cells, and innervating neurons. Loss or defects in any of these cell types, particularly the specialized sensory hair cells, leads to deafness. The Notch pathway is known to play a critical role in the decision to become either a hair cell or a supporting cell during embryogenesis; however, little is known about how Notch functions later during cochlear maturation. Uniquely amongst Notch ligands, Jagged1 (JAG1) is localized to supporting cells during cell fate acquisition and continues to be expressed into adulthood. Here, we demonstrate that JAG1 in maturing cochlear supporting cells is essential for normal cochlear function. Specifically, we show that deletion of JAG1 during cochlear maturation disrupts the inner hair cell pathway and leads to a type of deafness clinically similar to auditory neuropathy. Common pathologies associated with disruptions in inner hair cell function, including loss of hair cells, synapses, or auditory neurons, were not observed in JAG1 mutant cochleae. Instead, RNA-seq analysis of JAG1-deficient cochleae identified dysregulation of the Rho GTPase pathway, known to be involved in stereocilia development and maintenance. Interestingly, the overexpression of one of the altered genes, Diaph3, is responsible for autosomal dominant auditory neuropathy-1 (AUNA1) in humans and mice, and is associated with defects in the inner hair cell stereocilia. Strikingly, ultrastructural analyses of JAG1-deleted cochleae revealed stereocilia defects in inner hair cells, including fused and elongated bundles, that were similar to those stereocilia defects reported in AUNA1 mice. Taken together, these data indicate a novel role for Notch signaling in normal hearing development through maintaining stereocilia integrity of the inner hair cells during cochlear maturation.

Novel POU3F4 variants identified in patients with inner ear malformations exhibit aberrant cellular distribution and lack of SLC6A20 transcriptional upregulation.

Hearing loss (HL) is the most common sensory defect and affects 450 million people worldwide in a disabling form. Pathogenic sequence alterations in the POU3F4 gene, which encodes a transcription factor, are causative of the most common type of X-linked deafness (X-linked deafness type 3, DFN3, DFNX2). POU3F4-related deafness is characterized by a typical inner ear malformation, namely an incomplete partition of the cochlea type 3 (IP3), with or without an enlargement of the vestibular aqueduct (EVA). The pathomechanism underlying POU3F4-related deafness and the corresponding transcriptional targets are largely uncharacterized. Two male patients belonging to a Caucasian cohort with HL and EVA who presented with an IP3 were submitted to genetic analysis. Two novel sequence variants in POU3F4 were identified by Sanger sequencing. In cell-based assays, the corresponding protein variants (p.S74Afs*8 and p.C327*) showed an aberrant expression and subcellular distribution and lack of transcriptional activity. These two protein variants failed to upregulate the transcript levels of the amino acid transporter gene SLC6A20, which was identified as a novel transcriptional target of POU3F4 by RNA sequencing and RT-qPCR. Accordingly, POU3F4 silencing by siRNA resulted in downregulation of SLC6A20 in mouse embryonic fibroblasts. Moreover, we showed for the first time that SLC6A20 is expressed in the mouse cochlea, and co-localized with POU3F4 in the spiral ligament. The findings presented here point to a novel role of amino acid transporters in the inner ear and pave the way for mechanistic studies of POU3F4-related HL.

Surdité dans l’artérite à cellules géantes : à propos d’une observation

IntroductionHearing loss is a rare manifestation in giant cell arteritis. The different types of deafness are possible with a predominance of sensorineural deafness. Case reportWe report a 75-year-old woman who presented with typical manifestations of giant cell arteritis associated concomitantly with the occurrence of bilateral mixed hearing loss confirmed on the audiogram. Corticosteroids allowed a rapidly favorable clinical and biological outcome. The follow-up audiogram at 3 months was markedly improved and showed a decrease in sensorineural hearing loss and disappearance of conductive hearing loss. ConclusionAny rapid onset deafness in an inflammatory context in the elderly should lead to a search for giant cell arteritis. The diagnosis can be difficult in the absence of other typical manifestations, especially since the biopsy of the temporal artery most often comes back negative. Corticosteroids are usually effective.

Otological Manifestations in Oral Submucous Fibrosis

Background: Oral submucous fibrosis (OSMF) is a chronic premalignant condition seen in the oral cavity, but sometimes extends to the pharynx, esophagus, or even the larynx. OSMF causes stiffness in the oral cavity leads to trismus and affects speech and swallowing. Sometimes this lesion affects the Eustachian tube opening at the nasopharynx which results in Eustachian tube dysfunction followed by middle ear diseases. The purpose of the present work is to evaluate otological manifestations in OSMF patients. Materials and Methods: Patients with OSMF were evaluated for otological manifestations such as hearing impairment and fullness in the ear. All of them were evaluated with pure tone audiometry and tympanometry. Results: In this study group of 58 patients of OSMF comprising 116 ears, the hearing was within the normal limit in 60 ears (51.72%), a mild degree of conductive deafness in 52 ears (44.82%), a mild degree of sensorineural deafness in 2 ears (1.72%) and a moderate degree of sensorineural deafness were present in 2 ears (1.72%). There is a significant difference in mean quantitative hearing impairment was seen in OSMF groups C and group D with respect to the control group. Conclusion: Involvements of palatal muscles in OSMF patients reduce the patency of the Eustachian tube, resulting in a conductive type of deafness. OSMF patients should be assessed for hearing impairment and suggested for proper management.

The Analysis of Presbycusis Type and Lesion Location Based on Audiogram Description, Speech Audiometry, and Otoaccoustic Emission

Background: This study aims to determine the type of presbycusis and the location of the lesion based on audiogram images, speech audiometry and Otoacoustic Emission.&#x0D; Method: This study was an observational study of 36 presbycusis patients (72 ear samples). Pure tone audiometry, speech audiometry and Otoacoustic Emission were examined to determine the most types of presbycusis and the location of the lesion. A cross-sectional descriptive research method was conducted to analyze the dynamics of the correlation between risk factors and effects by using approaching, observing or collecting data at once (point time approach). Each research subject was observed only once, and measurements were performed towards the character status or subject variables during the examination.&#x0D; Result: The results showed that presbycusis was primarily obtained in the 60-69 years age group (69.4%). And the most types of presbycusis based on the audiogram were metabolic presbycusis (36.1%). The average hearing range based on the audiogram was at a frequency of 26-40 dB or the degree of mild deafness. Most types of deafness were Sensorineural Hearing Loss (SNHL). In speech audiometry, most NPT and NDT were mild and normal deafness as much as 44.4% in NPT and 56.9% in NDT. Based on the OAE, 72 samples showed the results of the referrals. In addition, regarding the results of the audiogram, speech audiometry and OAE, the location of the lesions of all samples were in the cochlea (100%).&#x0D; Conclusion: The most common type of presbycusis based on audiogram images is metabolic presbycusis with a mild hearing loss.

Analysis of coagulation state in sudden deafness patients with total deafness and vertigo

Objective:To analyze the coagulation status and prognosis of sudden deafness patients with total deafness accompanied by vertigo, and to provide basis for improving the treatment of this disease. Methods:From January 2017 to December 2020, 33 patients with total deafness and vertigo sudden deafness who were hospitalized in the Department of Otolaryngology Head and Neck Surgery, Hebei Provincial People's Hospital were selected as the research group. During the same period, 33 cases of low frequency type, full frequency type, total deafness and 26 cases of high frequency type were treated as control group.Thirty-three cases of inpatients without history of middle ear and inner ear diseases were treated as normal control group. The levels of fibrinogen（FIB）, D-Dimer（D-D）, Prothrombin Time（PT）, Activated Partial Thrombin Time（APTT） between the research group and the control group were analyzed, and the therapeutic effects of different types of sudden deafness patients were analyzed. Results:FIB and D-D of total deafness with vertigo were 2.50（2.11, 2.95）and 0.27（0.16, 0.51） respectively, which were higher than 2.31（1.92, 2.50） and 0.17（0.12, 0.21） of normal group. APTT was 25.2（23.1, 28.1）, lower than 27.3（26.4, 29.7） in the normal group, the differences were statistically significant（P<0.01）. ② FIB of total deafness with vertigo was 2.50（2.11, 2.95）, which was higher than that of low frequency group 2.37（1.81, 2.68）. D-D was 0.27（0.16, 0.51）, higher than low frequency group 0.16（0.12, 0.25）, high frequency group of 0.13（0.11, 0.23）, the whole frequency group 0.16（0.11, 0.28）, total of 0.18（1.45, 0.30）. APTT was 25.75±3.18/25.2（23.1, 28.1）, lower than 27.72±2.22 in low frequency group and 26.7（25.8, 28.7） in full frequency group, with statistical significance（P<0.05）. ③ The total deafness with vertigo group had the worst curative effect（ineffective rate was 63.6%）, and the low frequency group had the best curative effect（recovery rate was 75.8%）. The difference of curative effect among different types of sudden deafness groups was statistically significant（P<0.05）. Conclusion:Hypercoagulability and thrombosis may be one of the influencing factors of total sudden deafness. The hypercoagulable state of sudden deafness patients with total deafness and vertigo is more serious than that of total deafness and other types of sudden deafness, and the prognosis is the worst.

Deafness: Epidemiological, Clinical and Therapeutic Aspects in 15 Year Olds and over at the University Hospital Center Gabriel Toure of Bamako

Objective: To study the epidemiological, clinical and therapeutic aspects of hearing loss in 15-year-olds and over at the CHU GT of Bamako. Materials and Method: This is a longitudinal prospective study carried out in the ENT department of the University hospital Gabriel Toure in Bamako over 13 months (from September 2018 to September 2019). We carried out an exhaustive sampling of all the patients who consulted in the department for hearing loss and whose deafness was confirmed at pure tone audiometry with age greater than or equal to 15 years. The exclusion criteria were all patients under the age of 15 as well as a hearing loss related to earwax or foreign bodies and refusals to participate in the study. Results: Two hundred and thirty (230) patients were collected during the study. Dominated by the male sex with a sex ratio of 1.5 or 3 men for 2 women, with an average age of 38.16 ± 19 years with extremes ranging from 15 to 86 years. Among our patients, 37.40% had unilateral deafness, progressive installation evolving more than 12 weeks with a rate of cases had headaches 41% and had a history of chronic otitis media (CMO) 27%. There were a few cases of mixed type deafness, 30% had mild deafness, 22% moderate deafness and 40% severe deafness, according to the BIAP classification. Among our patients, 22.16% had conductive hearing loss, and those with sensorineural hearing loss accounted for 15.24%. Conclusion: Pure tone audiometry remains essential in the diagnosis of deafness. It not only makes it possible to confirm or invalidate the deafness, but also to define the type of deafness and especially to classify the deafness according to the degree of the average hearing loss established by the BIAP.

Ayurvedic Management of Sensory Neural Hearing Loss- A Case Study

Introduction: Sensory neural hearing loss occurs when there is damage to tiny hair cells in the cochlear or the auditory nerve. It is characterized by Sudden or steady loss of hearing, Muffled hearing, Difficulty for understanding speech, Ear fullness, Tinnitus and dizziness. According to WHO It is considered the main type of Deafness, which also affects the quality of daily life. In contemporary medicine there is no ideal cure for it, except the application of hearing aid and surgical intervention. In Ayurveda we can correlate it with Badhirya and treat according to it. Thus there is a need to develop an Ayurvedic treatment protocol which is effective and economically viable. Methods: A 34- year old male adult approached OPD of SJIIM Bengaluru, complaining of decreased hearing in both the ears along with tinnitus since 1and a half months. He was taken in for treatment after assessment. Result: After 4 months of treatment, Subjective parameters improved. Conclusion: By the above case study we can conclude that Ayurveda will have a pivotal role in treating cases of Sensory neural hearing loss and in return improving the quality of life.