Hearing Impaired

Two experiments were performed to explore the feasibility of deriving an articulation index {AI) based scheme for quantifying residual auditory function in listeners with sensorineural hearing impairment. In the first experiment the AI procedure was applied to audiograms of normal and sensorineural hearing-impaired individuals in order to predict speech discrimination under various conditions. Good predictions were found for the normal and the less impaired subjects, but not for those with greater impairments. These subjects exhibited a disproportionate loss in speech discrimination compared to that predicted on the basis of the AI procedure. In the second experiment it was investigated whether the AI predictions for presbycusic individuals could be improved by making use of the proficiency factor that is not a function of frequency. The results indicate that the deficit in suprathreshold speech processing is frequency-specific and that, therefore, the proficiency factor is not independent of frequency. It assumes the value of 1 at those frequencies where hearing sensitivity is normal and is substantially reduced for frequencies where hearing threshold is elevated.

Final approval and acceptance of this dissertation is contingent upon the candidate's submission of the final copies of the dissertation to the Graduate College. I hereby certify that I have read this dissertation prepared under my direction and recommend that it be accepted as fulfilling the dissertation requirement.

Final approval and acceptance of this dissertation is contingent upon the candidate's submission of the final copies of the dissertation to the Graduate College. I hereby certify that I have read this dissertation prepared under my direction and recommend that it be accepted as fulfilling the dissertation requirement.

Mutations in the connexin 31 (GJB3) gene have been found in subjects with dominant and recessive deafness and in patients with erythrokeratodermia variabilis. We report here a dominant mutation in the GJB3 gene (D66del) in a family affected with peripheral neuropathy and sensorineural hearing impairment. A wide range of disease severity for peripheral neuropathy, from asymptomatic cases to subjects with chronic skin ulcers in their feet and osteomyelitis leading to amputations, was detected in D66del patients. Mild, often asymmetrical, hearing impairment was found in all but one patient with mutation D66del of this family and the same mutation was present in an independent family ascertained because of hearing impairment. We have found mouse connexin 31 (Gjb3) gene expression in the cochlea and in the auditory and sciatic nerves, showing a pattern similar to that of Gjb1 (connexin 32), of which the human ortholog (GJB1) is involved in X-linked peripheral neuropathy. This expression pattern, together with auditory-evoked brainstem anomalous response in D66del patients, indicates that hearing impairment due to GJB3 mutations involves alterations in both the cochlea and the auditory nerve. Peripheral neuropathy is the third phenotypic alteration linked to GJB3 mutations, which enlarges the list of genes that cause this group of heterogeneous disorders.

Objective: To describe the relationship between atherosclerosis and hearing thresholds in prediabetic patients with impaired fasting glucose (IFG) and to determine the efficacy of glycated albumin in predicting carotid artery atherosclerosis in patients with isolated IFG. Subjects and Methods: The study included 82 patients (aged 53.73-80 years) divided into two groups based on fasting glucose levels, the IFG group: 59 patients (32 females, 54.2%), and the normal fasting plasma glucose level group: 23 patients (12 females, 52.2%). Patients underwent audiological testing to determine hearing thresholds, and carotid intima-media thickness (CIMT) was measured using carotid artery Doppler sonography. Multivariate analyses were performed to determine whether or not the plasma glycated albumin levels could predict hearing loss and CIMT. Results: Patients in the IFG group (mean age: 59.8 ± 9.5 years) had higher hearing thresholds and pure-tone average scores (PTA) than those in the group w...

We report the case of a 12 year-old boy with oto-palato-digital syndrome type II (OPD II). He had various anomalies at birth, including bilateral cataracts, bilateral glaucoma, bilateral severe hearing impairment, congenital heart defect, umbilical herniation, bowed extremities and constrictions of various joints. These clinical features and whole body X-ray findings were compatible with OPD II. However, his ocular disorders such as congenital cataract and glaucoma, and congenital heart defect have never been associated with OPD II as far as we know. His chromosomal analysis revealed normal karyotype, 46,XY. Analysis of the filamin A gene using a standard PCRdirect sequencing method determined a C586T (Arg196Trp) missense mutation in exon 3. Interestingly, the same C586T mutation was reported previously in a patient with OPD I (mild form). Thus, phenotype-genotype correlation of OPD is lacking in those patients. Further clinical and genetic studies are needed to clarify the relationship between phenotypes and genotypes, or to identify other factor(s) that influence the clinical features of this syndrome.

Steady-state evoked potential testing offers a means of obtaining accurate, frequency specific estimates of hearing threshold in subjects with even severe to total hearing losses. As such, the technique can play an important role in the pre operative evaluation of young cochlear implant candidates.

With more children receiving cochlear implants during infancy, there is a need for validated assessments of pre-verbal and early verbal auditory skills. The LittlEARS Auditory Questionnaire is presented here as the first module of the LittlEARS test battery. The LittlEARS Auditory Questionnaire was developed and piloted to assess the auditory behaviour of normal hearing children and hearing impaired children who receive a cochlear implant or hearing aid prior to 24 months of age. This paper presents results from two studies: one validating the LittlEARS Auditory Questionnaire on children with normal hearing who are German speaking and a second validating the norm curves found after adaptation and administration of the questionnaire to children with normal hearing in 15 different languages. Scores from a group of 218 German and Austrian children with normal hearing between 5 days and 24 months of age were used to create a norm curve. The questionnaire was adapted from the German orig...

To compare the performance of hearing-impaired and normal-hearing people on phonologic and semantic verbal fluency tests. A cross-sectional study was conducted with 48 hearing-impaired adults and 42 individuals (control group) with no hearing or language complaints. Sociodemographic data were collected, as well as the characteristics of hearing loss and of the electronic auditory device (hearing aids or cochlear implant), when relevant. Verbal fluency was tested in two different tasks: by semantic category (animals) and by phonology (letter F). Educational level has influenced the results of fluency tests in both groups, with more evidence in the hearing-impaired subjects (p<0.001). Hearing-impaired subjects showed worse performance in verbal fluency tests when compared to normal-hearing people in groups with up to 10 years of schooling. In the comparison of performance in the two tests, both groups showed better results in the semantic fluency task. The hearing-impaired subjects...

Background: Fanconi's anemia is an autosomal recessive disease associated with chromosomal breakage as well as pancytopenia, skin pigmentation, renal hypoplasia, cardiac defects, microcephaly, congenital malformations of the skeleton, hypogonadism, and increased risk of leukemia. The present report describes the periodontal clinical and microbiological status of an 11‐year old male having Fanconi's anemia.Methods: Polymerase chain reaction analysis to detect human cytomegalovirus (HCMV), Epstein‐Barr type 1 virus, and herpes simplex virus (HSV) was performed on paper‐point samples pooled from either 3 periodontal sites with advanced attachment loss or 3 gingivitis sites with no clinical attachment loss. Anaerobic bacterial culture examination was performed on the pooled periodontitis sample.Results: The patient suffered from pancytopenia, allergy, asthma, hearing impairment, and mental retardation. Dentition consisted of 7 primary teeth, 11 erupted permanent teeth, and 14 un...

By using homozygosity mapping in a consanguineous Pakistani family, we detected linkage of nonsyndromic hearing loss to a 7.6 Mb region on chromosome 3q13.31-q21.1 within the previously reported DFNB42 locus. Subsequent candidate gene sequencing identified a homozygous nonsense mutation (c. 1135G>T [p.Glu379X]) in ILDR1 as the cause of hearing impairment. By analyzing additional consanguineous families with homozygosity at this locus, we detected ILDR1 mutations in the affected individuals of 10 more families from Pakistan and Iran. The identified ILDR1 variants include missense, nonsense, frameshift, and splice-site mutations as well as a start codon mutation in the family that originally defined the DFNB42 locus. ILDR1 encodes the evolutionarily conserved immunoglobulinlike domain containing receptor 1, a putative transmembrane receptor of unknown function. In situ hybridization detected expression of Ildr1, the murine ortholog, early in development in the vestibule and in hair cells and supporting cells of the cochlea. Expression in hair cell-and supporting cell-containing neurosensory organs is conserved in the zebrafish, in which the ildr1 ortholog is prominently expressed in the developing ear and neuromasts of the lateral line. These data identify loss-of-function mutations of ILDR1, a gene with a conserved expression pattern pointing to a conserved function in hearing in vertebrates, as underlying nonsyndromic prelingual sensorineural hearing impairment.

In a large consanguineous family of Turkish origin, genome-wide homozygosity mapping revealed a locus for recessive nonsyndromic hearing impairment on chromosome 14q24.3-q34.12. Fine mapping with microsatellite markers defined the critical linkage interval to a 18.7 cM region flanked by markers D14S53 and D14S1015. This region partially overlapped with the DFNB35 locus. Mutation analysis of ESRRB, a candidate gene in the overlapping region, revealed a homozygous 7 bp duplication in exon 8 in all affected individuals. This duplication results in a frame shift and premature stop codon. Sequence analysis of the ESRRB gene in the affected individuals of the original DFNB35 family and in three other DFNB35-linked consanguineous families from Pakistan revealed four missense mutations. ESRRB encodes the estrogen-related receptor beta protein, and one of the substitutions (p.A110V) is located in the DNA-binding domain of ESRRB, whereas the other three are substitutions (p.L320P, p.V342L, and p.L347P) located within the ligand-binding domain. Molecular modeling of this nuclear receptor showed that the missense mutations are likely to affect the structure and stability of these domains. RNA in situ hybridization in mice revealed that Esrrb is expressed during inner-ear development, whereas immunohistochemical analysis showed that ESRRB is present postnatally in the cochlea. Our data indicate that ESRRB is essential for inner-ear development and function. To our knowledge, this is the first report of pathogenic mutations of an estrogen-related receptor gene.

Myosin VI, found in organisms from Caenorhabditis elegans to humans, is essential for auditory and vestibular function in mammals, since genetic mutations lead to hearing impairment and vestibular dysfunction in both humans and mice. Here, we show that a missense mutation in this molecular motor in an ENU-generated mouse model, Tailchaser, disrupts myosin VI function. Structural changes in the Tailchaser hair bundles include mislocalization of the kinocilia and branching of stereocilia. Transfection of GFP-labeled myosin VI into epithelial cells and delivery of endocytic vesicles to the early endosome revealed that the mutant phenotype displays disrupted motor function. The actin-activated ATPase rates measured for the D179Y mutation are decreased, and indicate loss of coordination of the myosin VI heads or 'gating' in the dimer form. Proper coordination is required for walking processively along, or anchoring to, actin filaments, and is apparently destroyed by the proximity of the mutation to the nucleotide-binding pocket. This loss of myosin VI function may not allow myosin VI to transport its cargoes appropriately at the base and within the stereocilia, or to anchor the membrane of stereocilia to actin filaments via its cargos, both of which lead to structural changes in the stereocilia of myosin VI-impaired hair cells, and ultimately leading to deafness.

This paper reports a research on Hearing Impaired (HI) learners in Malaysia which focuses on beginner level. This research has been carried out with the intention to solve problem faced by parents of HI children in teaching their children to know the six (6) basic sounds which is known as Ling-6 Sound. The HI learners were assisted by specially-developed electroniclearning application or known as Assistive Courseware (AC) in their teaching and learning process. The AC utilizes the mother tongue language. The objectives of the study include 1) To identify the characteristic of AC for HI learners, 2) To design the storyboard of AC for HI learners, and 3) To design the interface of AC for HI learners based on MI theory. This study adapts the Iterative Triangulation Methodology, and it is integrated with the IntView Methodology. The storyboard of the prototype is outlined next. The findings of this paper highlight the prototype of the AC labelled with certain characteristics representing MI theory which is suitable for HI learners and the result is based on interview with parents. The final part concludes this paper.

Popular theory on the tendency to cradle an infant to the left side points to the specialization of the right hemisphere for the perception and expression of emotion. J. S. Sieratzki and B. Woll (1996) recently suggested that more emphasis be placed on the auditory modality, specifically focusing on the role of prosodic information. In this study, the direction of the lateral cradling bias in a group of profoundly deaf children, a group of deaf adults, and a control group of adults with no hearing impairment was investigated. The authors found a strong leftward cradling bias in all groups, a bias that was, if anything, stronger in the deaf participants. Given that people who are profoundly deaf, especially those who have been deaf from birth, have not been exposed to auditory prosody, the data do not support the suggestion that such prosodic information is the basis for the leftward cradling bias.

With the growing demand of educational efficiency, it is observed that the evolution in different education system for special need child have also been attended. There has been a significant rise in addressing the problems and educational need of physically challenged children. Specifically, the mute and deaf children who wants to study and develop their educational background (Skyer, 2023). It begins with an overview of the international effort towards inclusive education, particularly focussing on students with special education needs. In recent years, there has been a global movement towards inclusive education that aims to provide learning opportunities for all students, irrespective of their physical or mental disabilities. According to UNESCO, (2024a), ensuring inclusive education is important and essential for upholding human dignity and rights with the Salamanca statement in 1994 marking a significant milestone in international support for inclusive education policies. However, despite these efforts’ challenges persist in effectively implementing inclusive practises, particularly for students with sensory impairment like deafness, in conventional schools or classrooms.

A consanguineous family with autosomal recessive nonsyndromic hearing impairment (NSHI) was ascertained in Pakistan and displayed significant evidence of linkage to 3q13.31-q22.3. The novel locus (DFNB42) segregating in this kindred, maps to a 21.6 cM region according to a genetic map constructed using data from both the deCode and Marshfield genetic maps. This region of homozygosity is flanked by markers D3S1278 and D3S2453. A maximum multipoint LOD score of 3.72 was obtained at marker D3S4523. DFNB42 represents the third autosomal recessive NSHI locus to map to chromosome 3.

This study is aimed at investigating the teacher's strategies in teaching English for the hearing-impaired students of a special education school in Surakarta, to find out the teacher's problems in teaching English, and to find out how the teaching strategies help the hearing-impaired students to learn English. The method used in this research is a case study. The qualitative data used in this research were collected from observation, interview, and documentation. The techniques used to analyze the data are from Miles and Huberman (1994), which include data reduction, data display and conclusion drawing or verification. From the result of this research, it showed that the strategies the teacher used in teaching English for the hearing-impaired students were drilling and visual scaffolding. The result of this research also showed the strategies the teacher used can help the students in memorizing new vocabulary and help the students in understanding better.

Persons with Disabilities are the world's largest minority. This group, like others, needs information and skills to play a positive role in the community. Public libraries play an important role in providing services to the community, including People with Disabilities, by providing a variety of sources and services. The present study has been conducted using the qualitative content analysis method for identifying the types of library services and programs required for people with visually impaired, physically disabled, and hearing impaired using a semi-structured interview. The sample of the study consisted of 84 individuals, including librarians of public libraries and the disabled in the three groups mentioned. The identified library needs and services are categorized into eight categories: planning and budgeting, advertising and service development, library accessibility and physical condition, services and programs, human resources and training, collection, information equipment, and technology and welfare facilities. The category of services and programs had the highest frequency in all three groups of the disabled. In the group of physically disabled individuals, the category of accessibility ranked highest, and in the two groups of the blind and deaf, services and programs had the highest frequency. The need to know about library services in different ways (email, SMS, and social media, …) had the highest frequency. Public libraries should plan to make the library accessible, and provide a variety of services and programs for individuals with disabilities.

The congenital middle ear anomalies are the cause for rare congenital conductive hearing loss (1). The incidence of Congenital Middle ear Anomalies (CMEAs) is around 0.28 per 100,000 persons (2). The CMEAs can occur unilaterally or bilaterally. This article aimed to profile the audiological and radiological findings and test battery approach which helped to diagnose ossicular fixation in a 4 year old child.

Introduction: Deafness is the invisible disability and the commonest human sensory defects.  It leads to difficult speech development, poor educational and employment prospects of  chilhood. Comprehensive otologic and audiological evaluations are very much essential for  etiological assessment of hearing impaired children and management efficacy.Methods: A cross-sectional study was carried out with the aim to evaluate the etiological  factors, degree of hearing loss, type of hearing loss, and results of aided audiogram among  the below 12 years deaf children. The study included 100 deaf children below 12 years with  history of deafness and non-development of speech.Result: The etiology of deaf Children was diverse. Infection was the predominating etiological  factor (38%). The infections were: measles (31.5%), pneumonia (26.2%), typhoid (21.5%),  maternal rubella (5.2%), varicella (5.2%), mumps (5.2%) and meningitis (5.2%). 48% of deaf  child had a parental suspicion of deafness bel...

Sign languages that exist around the world are usually identified by the country where they are used such as Ethiopian sign language. Mostly, the communication among the hearing-impaired people involves signs that stand for words by themselves. However, to make a sign language complete as a spoken language, the hearing-impaired community around the world use manual alphabets for names, technical terms, and sometimes for emphasis. As there are different alphabets for different spoken languages such as Amharic, there are manual alphabets or finger spellings used by the deaf people. Therefore, sign language in general is a tool that deaf communities use to communicate with each other.
There is no problem when the communication is limited between the deaf, but they struggle to communicate with hearing people due to the language barrier. Using translators was the solution for filling the communication gap especially in Ethiopia, even if it has its own draw backs with respect to economy or privacy issue. Consequently, developing software which fills the communication gap between the deaf and hearing people is a best solution.
This thesis contributes on the development of a model and system for Amharic sentence to Ethiopian sign language translator which accepts Amharic sentences, letters, or numbers, and outputs 3D animation of Ethiopian sign language based on the pre-lingual deaf grammar. The model bases on rule-based machine translation approaches and the developed system has three basic components: the interface component, the back-end component, and the database component. The first component (front-end) acts as a bridge between the users and the back-end component. The back-end component has three modules: Amharic text analysis, natural language processing (NLP), and text-to-sign mapping. Amharic text analysis modules analyze Amharic sentence and pass Romanized sentence to the NLP module. The NLP module accepts the Romanized Amharic sentence and performs all language processing and return sentence in Ethiopian Sign Language with including of morphological information. Then the final module (text-to-sign mapping) maps each word with the SiGML (sign script) and send to the interface component and the 3D avatar animation display the sign. In addition to enhance the quality of the translator we use a POS tagging which combine the previous work (naïve Byes classifier) and the new created one, using a brill tagging approach.
The translator performance evaluated into three classes; at sentences level, letter level, and number level and the result ranked into three categories; number of correctly translated sentences, number of understandable sentences, and number of wrong translations. All results without any errors were considered as correctly translated sentences. The results that conveyed meaning but not clear sense was considered as understandable sentences. But the results that did not covey meaning as well as sense were considered as wrong translations. Finally, the system gave an accuracy of 58.77%, 75.76%, and 84% at sentence, letter, and number level respectively.

El alto porcentaje de jóvenes rechazados en el ingreso laboral en Argentina por problemas auditivos sin justificación clínica, motivó el desarrollo de un modelo de medición para estudiar la inmisión sonora de los adolescentes durante sus hábitos recreativos y las consecuencias en la función auditiva. Consistió en un estudio longitudinal interdisciplinario, psicosocial, audiológico y acústico, con 102 varones y 71 niñas de dos escuelas de la ciudad de Córdoba, Argentina, con edades entre 14/15 años al comienzo del estudio y re-testeados anualmente durante cuatro años. Se muestran los resultados más importantes de los tres estudios y sus interrelaciones, analizando más detalladamente el psicosocial. Estos resultados fundamentan el actual Programa de Intervención para Promoción y Conservación de la Audición en Adolescentes delineado en breve síntesis.

In this study we applied synthetic aperture magnetometry (SAM) to investigate active cortical areas associated with magnetically recorded transient and steady-state auditory evoked responses. For transient evoked responses, SAM images reveal an activated volume of cortical tissue within the lateral aspect of the superior temporal plane. The volume of cortical activation for steady-state responses was located more medially than that for transient evoked responses. Additionally, SAM also reveals a small overlap of activated areas between transient and steady-state evoked responses, which has not be demonstrated when using equivalent current dipole (ECD) source modeling. Source waveforms from SAM and ECD analyses show comparable temporal information. Results from this study suggest that SAM is a useful technique for imaging cortical structures involved in processing perceptual information.

Objective:Possible therapeutic and protective benefits of intratympanic autologous serum application in amikacin-induced ototoxicity were investigated.Methods:Twenty-four guinea pigs were separated equally into two groups: therapeutic (group A) and protective (group B). Transient evoked otoacoustic emissions were recorded before and after autologous serum application. Apoptotic cells were identified in the organ of Corti, spiral limbus and spiral ganglion by the terminal deoxynucleotidyl transferase-mediated dUTP nick-end labelling (‘TUNEL’) method.Results:Transient evoked otoacoustic emission responses at 1, 1.4 and 2.8 kHz improved without significance after autologous serum application in group A (p > 0.05). A significantly protective effect of autologous serum was determined at 4 kHz in group B (p < 0.05). There were significantly fewer apoptotic cells at the spiral limbus in the therapeutic and protective groups compared to the control group (p < 0.05).Conclusion:Autol...

In an experimental task was tested the ability often year old hearing impaired subjects to preserve the phonological and acoustic form of actual words and nonsense words. One of the changes the words were undergoing was a great variation in duration of different sub morphemic segments.

Introduction: Hearing loss is one of the most disabling impairments. Using a hearing aid as an attempt to improve the hearing problem can positively affect the quality of life for these people. This research was aimed to assess satisfaction of hearing impaired patients with their hearing aids regarding the employed technology and style. Materials and methods: This descriptive-analytic cross-sectional research was conducted on 187 subjects with hearing loss who were using a hearing aid. The subjects were over 18 years of age and were using a hearing aid for at least 6 months. The Persian version of Satisfaction with Amplification in Daily Life (SADL) questionnaire was the instrument which was used for assessing satisfaction with the hearing aid. Cronbach's alpha was calculated to be 0.80 for instrument reliability. Results: A significant difference was observed among satisfaction subscales' mean scores with hearing aid technology. Also a significant difference was observed between the total satisfaction score and the hearing aid model. With respect to the analysis of satisfaction with the hearing aid and its style, cost and services was the only subscale which showed a significant difference (P=0.005). Conclusion: Respondents using hearing aids with different technology and style were estimated to be quite satisfied. Training audiologists in using more appropriate and fitting hearing aids in addition to using self-reporting questionnaires like SADL for estimating patients' social condition and participation in their life can essentially change their disability condition and countervail their hearing loss.

The paper describes an approach to specifically tailored user interface design, to adapt the user interface to the specific needs of mobility impaired travellers. Given a user has some interaction impairments or s/he is in a situation that causes an interaction impairment (i.e. noisy environment has the same consequences as hearing impairment), another modality is to be used or adapted to compensate this impairment. As sound has other interaction characteristics than graphical user interface elements (e.g. information can not be presented in parallel, but sequentially), rules for substituting some modalities through others are described.

Polymorphisms of constitutive hetrochomatin, in particular fluorescence polymorphisms after QFQstaining, show variations in size and staining character with unequal intra-and interchromosomal distribution in the chromosomes 1, 3, 4, 9, 13, 14, 15, 16, 21, 22 and Y, revealing significant patterns. In some cases, significant differences are found in size as well as in fluorescence, according to the type of cell culture analysed (amniocytes or lymphocytes). Within the individual sizes, the findings for flurescence are almost equivalent in both groups. Complete pericentric inversions show a maximum in chromosomes 4. The majority of polymorphisms in the acrocentrics 13 and 22 for p11.2 and in 15 and 22 for p13. Fields of application show analyses of polymorphisms of constitutive heterochromatin in twins and triplets demonstrating discrepancies in dizygotics and trizygotics but identical polymorphisms in monozygotic twins. Case presentations deal with proof or exclusion of percentage on the basis of comparison of polymorphisms of the constitutive heterochromatin. satellite-DNA type I-IV and αand β-DNA. Constitutive heterochromatin partially stains bright with QFQ, defined as i(5) according to ISCN 1995, but does not distinguish between different DNA-types. Each centromer is surrounded by pericentromeric heterochromatin with a different structure and size in each single chromosome. Four human chromosomes (1, 9, 16, Y) show large blocks of heterochromatin of chromosomespecific tandem repeats of a pentanucleotide. They are located proximally in the long arm of the autosomes 1, 9, and 16 and distally in the Ychromosome. These regions used to be considered to be without any function. They react less to DNAase-digestion as compared to euchromatin. Replicating late, constitutive heterochromatin prolongs the S-phase of the cell cycle and tends to cluster in the interphase. These chromosomes with a large block of heterochromatin are known to separate late in the anaphase together with the acrocentric chromosomes. In pachytene of meiosis I, heterochromatin maintains a special behaviour without any crossing-over. Recent investigations revealed the fact that constitutive heterochromatin is important for gene regulation, thereby modifying gene inactivity in various ontogenetic phases and specific tissues ).

Non syndromic hearing impairment is a common sensory disorder, which affects one in 600 newborns. Though more than 50 nuclear genes are involved in causing non syndromic hearing impairment, mutations in the connexin 26 (GJB2) gene explain a high proportion of congenital deafness in several populations worldwide. The diversity of genes and genetic loci implicated in hearing loss defines the complexity of the genetic basis of hearing. This review focuses on the role of connexin 26 and mitochondrial 12S rRNA genes in hearing which will be helpful for better understanding of genes in sporadic and aminoglycoside-induced non syndromic hearing impairment.

Academic achievement largely depends on the students’ discipline among other factors in any institution of learning. Studies show that indiscipline students perform poorly in both internal and external examinations including KCPE performance. Hearing impaired students, studies suggest, perform poorly because they display more behavior problems. For instance, there were poor examination results in both internal and national KCPE exams results for the year’s 2007and 2008 in primary schools for the hearing impaired in Vihiga County. This was attributed to high indiscipline cases (over 60%) as reflected in the school internal discipline records. The present study investigated disciplinary problems that existed and modes of punishment used to manage discipline problems in schools for the hearing impaired in Vihiga County. The main objective and research question of this study was to find out the common disciplinary problems that existed and modes of punishment used in schools for the hea...

Background: The objectives of the study are: 1. To determine the profile of patients presenting with hearing loss. 2. To determine the causes of hearing loss in adult patients of various age groups. The cases for the study comprised of adult patients aged >/=20years, presenting to the ENT Out-patient Department with complaint of hearing loss. Detailed history thorough ENT examination including Examination of the ear, nose and throat with Bull’s eye lamp, Otoscopic/Otomicroscopic/Otoendoscopic examination with photo-documentation of the ear status when required, tuning fork tests, and a battery of audiological tests including pure-tone audiometry, impedance audiometry and Brain-stem Evoked Response audiometry (when required) were conducted and the reports of the same were enclosed with the Pro forma of the patients. Results and Discussion: Hearing loss is more common in old age and more male patients presented with hearing loss. The most common symptom seen with hearing loss was tinnitus which was commonly ringing type and this was commonly seen in older patients associated with sensori-neural hearing loss. The most common type of hearing loss was sensori-neural hearing loss seen more commonly in older patients and associated with tinnitus. The definitive diagnosis for patients with sensori-neural hearing loss was difficult to specify with the most common diagnosis being presbyacusis, followed by noise induced hearing loss. The second common diagnosis was Chronic Otitis Media(COM).

Persons with Disabilities are the world's largest minority. This group, like others, needs information and skills to play a positive role in the community. Public libraries play an important role in providing services to the community, including People with Disabilities, by providing a variety of sources and services. The present study has been conducted using the qualitative content analysis method for identifying the types of library services and programs required for people with visually impaired, physically disabled, and hearing impaired using a semi-structured interview. The sample of the study consisted of 84 individuals, including librarians of public libraries and the disabled in the three groups mentioned. The identified library needs and services are categorized into eight categories: planning and budgeting, advertising and service development, library accessibility and physical condition, services and programs, human resources and training, collection, information equipment, and technology and welfare facilities. The category of services and programs had the highest frequency in all three groups of the disabled. In the group of physically disabled individuals, the category of accessibility ranked highest, and in the two groups of the blind and deaf, services and programs had the highest frequency. The need to know about library services in different ways (email, SMS, and social media, …) had the highest frequency. Public libraries should plan to make the library accessible, and provide a variety of services and programs for individuals with disabilities.

Background: acclimatization is the period that succeeds the fitting of hearing aids, when a progressive improvement of the hearing and speech recognition abilities is observed due to the new speech cues that are available to the hearing aid user. Aim: to verify acclimatization after the fitting of hearing aids through objective (speech tests) and subjective (Questionnaire) evaluations. Method: 16 hearing impaired individuals were evaluated on the first day of hearing aid fitting and were monthly reassessed for three months. In all three months the following speech tests were used: PISR -percentage index of speech recognition through monosyllabic words and SRT -speech recognition threshold through sentences, determining the speech/ noise ratio (S/N). The International Outcome Inventory of Hearing Aid (IOI-HA) was also applied in the first and third months after the hearing aids were fitted. Results: the comparison between the first day, first, second and third months after the hearing aids were fitted revealed a statistically significant improvement (p<0.001) between the results, not only for the PISR but also for the S/N ratio. No statistically significant difference was found for the results obtained through the questionnaire applied in the first and third months. Conclusion: the objective evaluation, using speech recognition tests, presents better results in the months following the hearing aids fitting indicating a progressive improvement in the speech abilities from the first month onwards. The subjective evaluation does not reveal an improvement when comparing the first and third moths after the hearing aids were fitted.

In a large consanguineous family of Turkish origin, genome-wide homozygosity mapping revealed a locus for recessive nonsyndromic hearing impairment on chromosome 14q24.3-q34.12. Fine mapping with microsatellite markers defined the critical linkage interval to a 18.7 cM region flanked by markers D14S53 and D14S1015. This region partially overlapped with the DFNB35 locus. Mutation analysis of ESRRB, a candidate gene in the overlapping region, revealed a homozygous 7 bp duplication in exon 8 in all affected individuals. This duplication results in a frame shift and premature stop codon. Sequence analysis of the ESRRB gene in the affected individuals of the original DFNB35 family and in three other DFNB35-linked consanguineous families from Pakistan revealed four missense mutations. ESRRB encodes the estrogen-related receptor beta protein, and one of the substitutions (p.A110V) is located in the DNA-binding domain of ESRRB, whereas the other three are substitutions (p.L320P, p.V342L, and p.L347P) located within the ligand-binding domain. Molecular modeling of this nuclear receptor showed that the missense mutations are likely to affect the structure and stability of these domains. RNA in situ hybridization in mice revealed that Esrrb is expressed during inner-ear development, whereas immunohistochemical analysis showed that ESRRB is present postnatally in the cochlea. Our data indicate that ESRRB is essential for inner-ear development and function. To our knowledge, this is the first report of pathogenic mutations of an estrogen-related receptor gene.

The detection of amplitude modulation (AM) of a carrier can be impaired by additional (masker) AM applied to the same carrier (within-carrier modulation masking, MM) or to a different carrier (across-carrier MM). These two types of MM were compared for young normal-hearing and older hearing-impaired subjects. The signal was 4- or 16-Hz sinusoidal AM of a 4000-Hz carrier. Masker AM with depth 0.4 was applied either to the same carrier or to a carrier at 3179 or 2518 Hz. The masker AM rate was 0.25, 0.5, 1, 2, or 4 times the signal rate. The signal AM depth was varied adaptively to determine the threshold. Both within-carrier and across-carrier MM patterns were similar for the two groups, suggesting that the hypothetical modulation filters are not affected by hearing loss or age. The signal AM detection thresholds were also similar for the two groups. Thresholds in the absence of masker AM were lower (better) for the older hearing-impaired than for the young normal-hearing subjects. S...

Recent years the noise is one of the leading pollutants in working and living environment. Although in some cases the noise levels do not exceed proposed levels, people often have the opposite impression. While for the most activities noise regulation exists, for public events this is not the case. There are certain guidelines, however established levels differ from country to country. In order to determine the noise level during public events the equivalent level of noise was measured at one public event on which approximately 50,000 people was present. The results show significantly high levels of noise, especially during rock concert and firework, when certain protective measures should be implemented.

This paper describes a new meeting support system that helps the hearing impaired to understand the contents of the meeting. The proposed system distinguishes the mainstream of the discussion from other chattering based on the utterances. The situation of the meeting is acquired as a picture using an omni-directional vision sensor, and the system analyzes speaker's relations from the captured image by using face directions for the participants. The system shows the mainstream and the chattering of a meeting by using the analyzed result and speech-recognition.