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Sample records for profound sensorineural deafness

  1. Visual impairment in severe and profound sensorineural deafness.

    OpenAIRE

    Armitage, I M; Burke, J. P.; Buffin, J T

    1995-01-01

    The frequency of reversible and irreversible visual impairment was determined in children with severe and profound sensorineural deafness, as subnormal vision can adversely affect their educational and social development. Eighty three of 87 such children attending an audiology service were examined to assess the incidence and severity of visual impairment. Each child underwent a detailed ophthalmic assessment. The criteria for visual impairment were visual acuity < 6/9 Snellen or equivalent a...

  2. Prevalence of Mutations in Deafness-Causing Genes in Cochlear Implanted Patients with Profound Nonsyndromic Sensorineural Hearing Loss in Shandong Province, China.

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    Luo, Jianfen; Bai, Xiaohui; Zhang, Fengguo; Xiao, Yun; Gu, Lintao; Han, Yuechen; Fan, Zhaomin; Li, Jianfeng; Xu, Lei; Wang, Haibo

    2017-11-01

    The mutations of GJB2, SLC26A4, and mtDNA12SrRNA are the most common inherited causes of nonsyndromic sensorineural hearing loss (NSHL) in China, yet previous genetic screenings were mainly carried on patients with moderate-to-profound impairment. We aimed to detect the mutation frequencies in NSHL population within a more specified range of severity. Patients with profound NSHL who had undergone cochlear implantation in the Shandong Provincial Hospital (Shandong, China) were recruited. The majority (n  =  472) were between 0.7 and 6 years old, and the remaining (n  =  63) were between 6 and 70 years old. In total, 115 mutation alleles of the three genes were screened with SNP scan assay. Of the patients, 19.44% (104/535) were found to have GJB2 mutations, and the most common allele was c.235delC, followed by c.299_300delAT and c.109G>A. SLC26A4 mutations were detected in 13.46% patients (72/535), and the most common allele was c.919-2A>G (IVS7-2A>G), followed by c.1174A>T and c.2168A>G. Seven patients (1.31%) carried mutations in mtDNA12SrRNA, with the alleles of m.1555A>G and m.1494C>T. We found the allele frequency of c.109G>A (GJB2) was relatively lower in the profound NSHL population in comparison to the moderate-to-profound ones, and the c.1174A>T (SLC26A4) relatively higher. It suggests those mutations may be connected with the degree of deafness, which needs more observations and analyses to support. © 2017 John Wiley & Sons Ltd/University College London.

  3. An epidemiological and genetic study of congenital profound deafness in Tunisia (governorate of Nabeul).

    OpenAIRE

    Ben Arab, S; Bonaïti-Pellié, C.; Belkahia, A

    1990-01-01

    An epidemiological and genetic study of profound deafness has been undertaken in the governorate of Nabeul in Tunisia. This paper deals with sensorineural deafness with no associated abnormalities. The prevalence was estimated to be 0.0007 and four clusters could be identified, two of which represent 51% and 34% respectively of the total number of cases. Segregation analysis performed in 29 pedigrees containing 415 subjects with 129 affected cases provided evidence for simple recessive inheri...

  4. Acute profound sensorineural hearing loss as the initial manifestation of acute leukemia--report of a case.

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    Harada, T; Namiki, S; Kawabata, I

    2000-10-01

    This paper reports an unusual case in which acute lymphocytic leukemia presented acute profound sensorineural hearing loss as the initial manifestation of the disease. The patient is a 55-year-old woman who complained of left hearing loss of sudden onset. Pure tone audiometry revealed profound sensorineural hearing loss of the left ear at mid and low frequencies. The patient was tentatively diagnosed as idiopathic sudden deafness and admitted for the treatment, but her laboratory data indicated that she was at an advanced stage of leukemia. The patient's hearing loss did not improve subjectively until she deceased 1 year after the admission. The mechanism producing acute hearing loss in leukemic patients is reviewed and discussed, and the importance of differentiating possible underlying diseases before we diagnose idiopathic sudden deafness is stressed.

  5. How do profoundly deaf children learn to read?

    OpenAIRE

    伊藤, 泰子

    2013-01-01

    We know that children who were born profoundly deaf have much difficulty to learn to speak English or Japanese. But is it possible that profoundly deaf children learn to read written English or Japanese? Some researchers mention that early exposure to fingerspelling actually helps deaf children become better readers. Then I tried to find the reason why fingerspelling helps deaf children develop their reading ability and examined how to develop deaf children’s reading ability with fingerspelli...

  6. Epilepsy, ataxia, sensorineural deafness, tubulopathy, and KCNJ10 mutations.

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    Bockenhauer, Detlef; Feather, Sally; Stanescu, Horia C; Bandulik, Sascha; Zdebik, Anselm A; Reichold, Markus; Tobin, Jonathan; Lieberer, Evelyn; Sterner, Christina; Landoure, Guida; Arora, Ruchi; Sirimanna, Tony; Thompson, Dorothy; Cross, J Helen; van't Hoff, William; Al Masri, Omar; Tullus, Kjell; Yeung, Stella; Anikster, Yair; Klootwijk, Enriko; Hubank, Mike; Dillon, Michael J; Heitzmann, Dirk; Arcos-Burgos, Mauricio; Knepper, Mark A; Dobbie, Angus; Gahl, William A; Warth, Richard; Sheridan, Eamonn; Kleta, Robert

    2009-05-07

    Five children from two consanguineous families presented with epilepsy beginning in infancy and severe ataxia, moderate sensorineural deafness, and a renal salt-losing tubulopathy with normotensive hypokalemic metabolic alkalosis. We investigated the genetic basis of this autosomal recessive disease, which we call the EAST syndrome (the presence of epilepsy, ataxia, sensorineural deafness, and tubulopathy). Whole-genome linkage analysis was performed in the four affected children in one of the families. Newly identified mutations in a potassium-channel gene were evaluated with the use of a heterologous expression system. Protein expression and function were further investigated in genetically modified mice. Linkage analysis identified a single significant locus on chromosome 1q23.2 with a lod score of 4.98. This region contained the KCNJ10 gene, which encodes a potassium channel expressed in the brain, inner ear, and kidney. Sequencing of this candidate gene revealed homozygous missense mutations in affected persons in both families. These mutations, when expressed heterologously in xenopus oocytes, caused significant and specific decreases in potassium currents. Mice with Kcnj10 deletions became dehydrated, with definitive evidence of renal salt wasting. Mutations in KCNJ10 cause a specific disorder, consisting of epilepsy, ataxia, sensorineural deafness, and tubulopathy. Our findings indicate that KCNJ10 plays a major role in renal salt handling and, hence, possibly also in blood-pressure maintenance and its regulation. 2009 Massachusetts Medical Society

  7. Cochlear implantation in autistic children with profound sensorineural hearing loss.

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    Lachowska, Magdalena; Pastuszka, Agnieszka; Łukaszewicz-Moszyńska, Zuzanna; Mikołajewska, Lidia; Niemczyk, Kazimierz

    2016-11-19

    Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. The aim of this study was to assess the benefits from cochlear implantation in deafened children with autism as the only additional disability. This study analyzes data of six children. The follow-up time was at least 43 months. The following data were analyzed: medical history, reaction to music and sound, Ling's six sounds test, onomatopoeic word test, reaction to spoken child's name, response to requests, questionnaire given to parents, sound processor fitting sessions and data. After cochlear implantation each child presented other communication skills. In some children, the symptoms of speech understanding were observed. No increased hyperactivity associated with daily use cochlear implant was observed. The study showed that in autistic children the perception is very important for a child's sense of security and makes contact with parents easier. Our study showed that oral communication is not likely to be a realistic goal in children with cochlear implants and autism. The implantation results showed benefits that varied among those children. The traditional methods of evaluating the results of cochlear implantation in children with autism are usually insufficient to fully assess the functional benefits. These benefits should be assessed in a more comprehensive manner taking into account the limitations of communication resulting from the essence of autism. It is important that we share knowledge about these complex children with cochlear implants. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  8. THIAMINE–RESPONSIVE MEGALOBLASTIC ANEMIA, SENSORINEURAL DEAFNESS AND DIABETES MELLITUS

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    M. Kadivar R. Moradian

    2006-11-01

    Full Text Available Abstract- The syndrome of diabetes mellitus, sensorineural deafness and megaloblastic anemia dose not result from thiamine deficiency. The previous reported patients had no sign of beriberi, had normal nutrition, and had no evidence of malabsorption. The features of this syndrome with apparent inheritance of autosomal recessive trait may define this puzzling syndrome as a true thiamine dependency state. The first Iranian patient was described by Vossough et al. in 1995. We found nine new cases with diagnostic criteria of thiamine responsive megaloblastic anemia during eight years of our study. In two patients, presentation of diabetes and anemia was concomitant. All of them were deaf with sensorineural hearing loss which was detected in infancy up to two years of age. The presence of congenital valvular heart disease was eliminated by normal echocardiography, but cardiomyopathy was discovered in two. Nonspecific amino-aciduria was discovered in three but urinary screening tests for hereditary orotic aciduria were negative. Ox-Phos biochemistry of muscle mitochondria which demonstrates severe defect in complexes I, III, IV in diabetes mellitus associated with deafness, were done but was unremarkable in our patients. Urinary methylmalonic acid and methyl malonyl carnitine by GS/MS and TMS was done in our patients and showed abnormal results in six patients. Thiamine gene, SLC 19A2, was detected in four patients.

  9. Allan-Herndon-Dudley syndrome with unusual profound sensorineural hearing loss.

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    Gagliardi, Lucia; Nataren, Nathalie; Feng, Jinghua; Schreiber, Andreas W; Hahn, Christopher N; Conwell, Louise S; Coman, David; Scott, Hamish S

    2015-08-01

    The Allan-Herndon-Dudley syndrome is caused by mutations in the thyroid hormone transporter, Monocarboxylate transporter 8 (MCT8). It is characterized by profound intellectual disability and abnormal thyroid function. We report on a patient with Allan-Herndon-Dudley syndrome (AHDS) with profound sensorineural hearing loss which is not usually a feature of AHDS and which may have been due to a coexisting nonsense mutation in Microphthalmia-associated transcription factor (MITF). © 2015 Wiley Periodicals, Inc.

  10. [The very severe sensorineural deafness patients caused by rubella virus infection: two cases report].

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    Ma, Jing; Wan, Lang; Xu, Fen

    2015-09-01

    To explore the audiological features in children who were sever sensorineural hearing loss infected with rubella virus. There were two cases of rubella virus infection in children who were deaf, they conducted the distortion product otoacoustic emission, ABR and auditory steady-state evoked response (ASSR) examination, then analyzed the results comprehensively. Two patients' mothers were prompted to have infected rubella virus during the early three months pregnant period by history and laboratory tests. The two patients were not detected deafness gene mutation. Audiology results implied the two patients were very severe binaural sensorineural deafness, so they were recommended to equipped with hearing aids and cochlear implant surgery. Early pregnancy women infected with rubella virus can cause very severe offspring sensorineural deafness. The crowd whose mother were suspected to infect with rubella virus in early pregnancy, that should be tracked and detected hearing in order to achieve early detection, early intervention and early treatment.

  11. Bilateral sensorineural deafness and hydrocephalus due to foramen of Monro obstruction in sibs: A newly described autosomal recessive disorder

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    Chudley, A.E.; McCullough, C.; McCullough, D.W. [Univ. of Manitoba, Winnipeg (Canada)

    1997-01-31

    We identified a Canadian-Mennonite family in which a brother and sister have hydrocephalus due to obstruction at the foramen of Monro and profound bilateral sensorineural deafness. This appears to be a unique combination of anomalies and, to our knowledge, has not been reported previously. Both parents and a brother are phenotypically normal. The parents are second cousins. Thus, on the basis of consanguinity, affected sibs of both sexes, and in the absence of evidence for intrauterine infections or other adverse perinatal events, this syndrome is likely inherited in an autosomal recessive fashion. 37 refs., 5 figs.

  12. Foveal Processing Under Concurrent Peripheral Load in Profoundly Deaf Adults

    Science.gov (United States)

    2016-01-01

    Development of the visual system typically proceeds in concert with the development of audition. One result is that the visual system of profoundly deaf individuals differs from that of those with typical auditory systems. While past research has suggested deaf people have enhanced attention in the visual periphery, it is still unclear whether or not this enhancement entails deficits in central vision. Profoundly deaf and typically hearing adults were administered a variant of the useful field of view task that independently assessed performance on concurrent central and peripheral tasks. Identification of a foveated target was impaired by a concurrent selective peripheral attention task, more so in profoundly deaf adults than in the typically hearing. Previous findings of enhanced performance on the peripheral task were not replicated. These data are discussed in terms of flexible allocation of spatial attention targeted towards perceived task demands, and support a modified “division of labor” hypothesis whereby attentional resources co-opted to process peripheral space result in reduced resources in the central visual field. PMID:26657078

  13. Refinement of the locus for non-syndromic sensorineural deafness ...

    Indian Academy of Sciences (India)

    Non-syndromic X-linked deafness is a rare form of genetic deafness in humans accounting for a small proportion of all hereditary hearing loss. Different clinical forms of non-syndromic X-linked deafness have been described, and most of these have been mapped. Here, we report a Chinese family affected by a congenital ...

  14. Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome

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    Abramowicz Marc

    2008-10-01

    Full Text Available Abstract Harboyan syndrome is a degenerative corneal disorder defined as congenital hereditary endothelial dystrophy (CHED accompanied by progressive, postlingual sensorineural hearing loss. To date, 24 cases from 11 families of various origin (Asian Indian, South American Indian, Sephardi Jewish, Brazilian Portuguese, Dutch, Gypsy, Moroccan, Dominican have been reported. More than 50% of the reported cases have been associated with parental consanguinity. The ocular manifestations in Harboyan syndrome include diffuse bilateral corneal edema occurring with severe corneal clouding, blurred vision, visual loss and nystagmus. They are apparent at birth or within the neonatal period and are indistinguishable from those characteristic of the autosomal recessive CHED (CHED2. Hearing deficit in Harboyan is slowly progressive and typically found in patients 10–25 years old. There are no reported cases with prelinglual deafness, however, a significant hearing loss in children as young as 4 years old has been detected by audiometry, suggesting that hearing may be affected earlier, even at birth. Harboyan syndrome is caused by mutations in the SLC4A11 gene located at the CHED2 locus on chromosome 20p13-p12, indicating that CHED2 and Harboyan syndrome are allelic disorders. A total of 62 different SLC4A11 mutations have been reported in 98 families (92 CHED2 and 6 Harboyan. All reported cases have been consistent with autosomal recessive transmission. Diagnosis is based on clinical criteria, detailed ophthalmological assessment and audiometry. A molecular confirmation of the clinical diagnosis is feasible. A variety of genetic, metabolic, developmental and acquired diseases presenting with clouding of the cornea should be considered in the differential diagnosis (Peters anomaly, sclerocornea, limbal dermoids, congenital glaucoma. Audiometry must be performed to differentiate Harboyan syndrome from CHED2. Autosomal recessive types of CHED (CHED2 and

  15. Sensorineural deafness, abnormal genitalia, synostosis of metacarpals and metatarsals 4 and 5, and mental retardation

    DEFF Research Database (Denmark)

    Mendioroz, Jacobo; Fernández-Toral, Joaquín; Suárez, Etelvina

    2005-01-01

    In 1988 Pfeiffer and Kapferer reported on a patient with sensorineural deafness, psychomotor delay, hypospadias, cerebral manifestations, and bilateral synostosis of the 4th and 5th metacarpals and metatarsals. Synostosis of the 4th and 5th metacarpals and metatarsals is a very rare defect that has...

  16. Noonan Syndrome: An Underestimated Cause of Severe to Profound Sensorineural Hearing Impairment. Which Clues to Suspect the Diagnosis?

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    Ziegler, Alban; Loundon, Natalie; Jonard, Laurence; Cavé, Hélène; Baujat, Geneviève; Gherbi, Souad; Couloigner, Vincent; Marlin, Sandrine

    2017-09-01

    To highlight Noonan syndrome as a clinically recognizable cause of severe to profound sensorineural hearing impairment. New clinical cases and review. Patients evaluated for etiological diagnosis by a medical geneticist in a reference center for hearing impairment. Five patients presenting with confirmed Noonan syndrome and profound sensorineural hearing impairment. Diagnostic and review of the literature. Five patients presented with profound sensorineural hearing impairment and molecularly confirmed Noonan syndrome. Sensorineural hearing impairment has been progressive for three patients. Cardiac echography identified pulmonary stenosis in two patients and was normal for the three other patients. Short stature was found in two patients. Mild intellectual disability was found in one patient. Inconspicuous clinical features as facial dysmorphism, cryptorchidism, or easy bruising were of peculiar interest to reach the diagnosis of Noonan syndrome. Profound sensorineural hearing impairment can be the main feature of Noonan syndrome. Associated features are highly variable; thus, detailed medical history and careful physical examination are mandatory to consider the diagnosis in case of a sensorineural hearing impairment.

  17. Contiguous deletion of SLC6A8 and BAP31 in a patient with severe dystonia and sensorineural deafness.

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    Osaka, Hitoshi; Takagi, Atsushi; Tsuyusaki, Yu; Wada, Takahito; Iai, Mizue; Yamashita, Sumimasa; Shimbo, Hiroko; Saitsu, Hirotomo; Salomons, Gajja S; Jakobs, Cornelis; Aida, Noriko; Toshihiro, Shinka; Kuhara, Tomiko; Matsumoto, Naomichi

    2012-05-01

    We report here a 6-year-old boy exhibiting severe dystonia, profound intellectual and developmental disability with liver disease, and sensorineural deafness. A deficient creatine peak in brain (1)H-MR spectroscopy and high ratio of creatine/creatinine concentration in his urine lead us to suspect a creatine transporter (solute carrier family 6, member 8; SLC6A8) deficiency, which was confirmed by the inability to take up creatine into fibroblasts. We found a large ~19 kb deletion encompassing exons 5-13 of SLC6A8 and exons 5-8 of the B-cell receptor-associated protein (BAP31) gene. This case is the first report in which the SLC6A8 and BAP31 genes are both deleted. The phenotype of BAP31 mutations has been reported only as a part of Xq28 deletion syndrome or contiguous ATP-binding cassette, sub-family D, member 1 (ABCD1)/DXS1375E (BAP31) deletion syndrome [MIM ID #300475], where liver dysfunction and sensorineural deafness have been suggested to be attributed to the loss of function of BAP31. Our case supports the idea that the loss of BAP31 is related to liver dysfunction and hearing loss. Copyright © 2012 Elsevier Inc. All rights reserved.

  18. Transient evoked otoacoustic emissions testing for screening of sensorineural deafness in puppies.

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    McBrearty, A; Penderis, J

    2011-01-01

    Transient evoked otoacoustic emissions (TEOAE) are widely used for human neonatal deafness screening, but have not been reported for clinical use in dogs. To investigate the feasibility of TEOAE testing in conscious puppies and the ability of TEOAE testing to correctly identify deaf and hearing ears, as defined by brainstem auditory evoked response (BAER). Forty puppies from 10 litters. Prospective study on puppies presented for hearing assessment as part of a congenital deafness BAER screening program. Hearing status was determined using BAER. TEOAE testing was performed after the BAER assessment and the results of the TEOAE testing were compared with the hearing status for each ear. Parameters were tested for normality using the D'Agostino Pearson test and comparisons between the deaf and hearing ears were made using Mann-Whitney tests. TEOAE testing was readily performed in puppies presented for congenital deafness screening. Using analysis parameters based on those used in human neonatal hearing screening, TEOAE testing correctly identified all deaf ears, as defined by BAER testing, with a sensitivity of 100% (95% CI: 56-100%) for diagnosing deafness and specificity of 78% (95% CI: 66-87%). TEOAE testing is an effective screening modality for identifying congenital sensorineural deafness in dogs. In light of the simpler and less expensive equipment, TEOAE testing has the potential to improve access to hearing screening and through this reduce the prevalence of congenital deafness in the dog. Copyright © 2011 by the American College of Veterinary Internal Medicine.

  19. Hearing Instruments for Unilateral Severe-to-Profound Sensorineural Hearing Loss in Adults: A Systematic Review and Meta-Analysis.

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    Kitterick, Pádraig Thomas; Smith, Sandra Nelson; Lucas, Laura

    2016-01-01

    A systematic review of the literature and meta-analysis was conducted to assess the nature and quality of the evidence for the use of hearing instruments in adults with a unilateral severe to profound sensorineural hearing loss. The PubMed, EMBASE, MEDLINE, Cochrane, CINAHL, and DARE databases were searched with no restrictions on language. The search included articles from the start of each database until February 11, 2015. Studies were included that (a) assessed the impact of any form of hearing instrument, including devices that reroute signals between the ears or restore aspects of hearing to a deaf ear, in adults with a sensorineural severe to profound loss in one ear and normal or near-normal hearing in the other ear; (b) compared different devices or compared a device with placebo or the unaided condition; (c) measured outcomes in terms of speech perception, spatial listening, or quality of life; (d) were prospective controlled or observational studies. Studies that met prospectively defined criteria were subjected to random effects meta-analyses. Twenty-seven studies reported in 30 articles were included. The evidence was graded as low-to-moderate quality having been obtained primarily from observational before-after comparisons. The meta-analysis identified statistically significant benefits to speech perception in noise for devices that rerouted the speech signals of interest from the worse ear to the better ear using either air or bone conduction (mean benefit, 2.5 dB). However, these devices also degraded speech understanding significantly and to a similar extent (mean deficit, 3.1 dB) when noise was rerouted to the better ear. Data on the effects of cochlear implantation on speech perception could not be pooled as the prospectively defined criteria for meta-analysis were not met. Inconsistency in the assessment of outcomes relating to sound localization also precluded the synthesis of evidence across studies. Evidence for the relative efficacy of

  20. Simultaneous Labyrinthectomy and Cochlear Implantation for Patients with Single-Sided Ménière’s Disease and Profound Sensorineural Hearing Loss

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    G. Doobe

    2015-01-01

    Full Text Available Objective. To investigate the treatment outcome of a simultaneous labyrinthectomy and cochlear implantation in patients with single-sided Ménière’s disease and profound sensorineural hearing loss. Study Design. Prospective study. Method. Five patients with single-sided Ménière’s disease with active vertigo and functional deafness were included. In all cases, simultaneous cochlear implantation combined with labyrinthectomy surgery was performed. The outcome has been evaluated by the Dizziness Handicap Inventory (DHI and speech recognition. Results. The combined labyrinthectomy and cochlear implantation led in all patients to a highly significant reduction of dizziness up to a restitutio ad integrum. After activation of the cochlear implant and rehabilitation, a mean monosyllabic speech understanding of 69% at 65 dB was observed. Conclusion. For patients with single-sided Ménière’s disease and profound sensorineural hearing loss the simultaneous labyrinthectomy and cochlear implantation are efficient method for the treatment of vertigo as well as the rehabilitation of the auditory system.

  1. Plasma antidiuretic hormone in cases with the early onset of profound unilateral deafness.

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    Takeda, Taizo; Kakigi, Akinobu; Nishioka, Rie; Taguchi, Daizo; Nishimura, Masahiko

    2008-12-01

    The p-ADH level in cases of juvenile unilateral profound deafness (JUPD) and the timecourse of the level were examined to investigate whether or not an increase of p-ADH is involved in the development of delayed endolymphatic hydrops (DEH) in JUPD. In 90 consecutive patients with unilateral profound or total sensorineural deafness with the onset in early childhood, pure-tone audiometric examination and the measurement of p-ADH and plasma osmolality (p-OSM) were followed up once or twice a year as far as possible. At every testing, we performed careful history-taking about episodic vertigo/dizziness, fluctuant hearing loss, and tinnitus in order to find out whether patients had experienced these clinical signs of the development of DEH. Means and standard deviation (S.D.) of p-ADH level and osmolality in all samples tested (n=368) were 7.3+/-7.0 pg/mL (0.7-52.0 pg/mL), and 288.6+/-4.4 mOsm/L (273-306 mOsm/L), respectively. The mean of p-ADH level was much higher than those previously reported in children and adolescents. High levels of p-ADH (over 5.0 pg/mL) were often observed in subjects between 6 and 19 years of age, but not so frequently in subjects of 20 years of age or older. Long-term follow-up of p-ADH levels revealed that DEH frequently developed in cases with persistent elevation of p-ADH. The elevation of p-ADH is likely to promote the development of DEH in cases of JUPD, although the underlying mechanism remains to be elucidated.

  2. Prevalence of the mitochondrial DNA A1555G mutation in sensorineural deafness patients in island Southeast Asia.

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    Malik, Safarina G; Pieter, Nova; Sudoyo, Herawati; Kadir, Abdul; Marzuki, Sangkot

    2003-01-01

    A mtDNA A1555G base substitution in a highly conserved region of the 12S rRNA gene has been reported to be the main cause of aminoglycoside induced deafness. This mutation is found in approximately 3% of Japanese and 0.5-2.4% of European sensorineural deafness patients. We report a high prevalence (5.3%) of the A1555G mutation in sensorineural deafness patients in Sulawesi (Indonesia). Our result confirms the importance of determining the prevalence of the mtDNA A1555G mutation in different populations, and the need for mutation detection before the administration of aminoglycoside antibiotics.

  3. X-linked ocular albinism and sensorineural deafness: Linkage to Xp22. 3

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    Winship, I.M.; Babaya, M.; Ramesar, R.S. (Univ. of Cape Town Medical School (South Africa))

    1993-11-01

    X-linked ocular albinism with late-onset sensorineural deafness (OASD) is an autonomous disorder that poses significant clinical problems, causing affected individuals to be blind and deaf by early middle age. Classical X-linked ocular albinism (without deafness; OA1) has recently been linked to markers in the Xp22.2-Xp22.3 region of the human genome. In the present report, a large South African family with OASD was investigated at the molecular level and tight linkage was found to the DXS452 locus at Xp22.3 using 25 informative meioses, with a maximum lod score of 7.1 at a recombination fraction of 0.00. These findings suggest that OA1 and OASD are allelic variants or that they may be due to contiguous gene defects. 12 refs., 1 fig.

  4. Congenital Sensorineural Deafness in Australian Stumpy-Tail Cattle Dogs Is an Autosomal Recessive Trait That Maps to CFA10

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    Sommerlad, Susan; McRae, Allan F.; McDonald, Brenda; Johnstone, Isobel; Cuttell, Leigh; Seddon, Jennifer M.; O'Leary, Caroline A.

    2010-01-01

    Background Congenital sensorineural deafness is an inherited condition found in many dog breeds, including Australian Stumpy-tail Cattle Dogs (ASCD). This deafness is evident in young pups and may affect one ear (unilateral) or both ears (bilateral). The genetic locus/loci involved is unknown for all dog breeds. The aims of this study were to determine incidence, inheritance mechanism, and possible association of congenital sensorineural deafness with coat colour in ASCD and to identify the genetic locus underpinning this disease. Methodology/Principal Findings A total of 315 ASCD were tested for sensorineural deafness using the brain stem auditory evoked response (BAER) test. Disease penetrance was estimated directly, using the ratio of unilaterally to bilaterally deaf dogs, and segregation analysis was performed using Mendel. A complete genome screen was undertaken using 325 microsatellites spread throughout the genome, on a pedigree of 50 BAER tested ASCD in which deafness was segregating. Fifty-six dogs (17.8%) were deaf, with 17 bilaterally and 39 unilaterally deaf. Unilaterally deaf dogs showed no significant left/right bias (p = 0.19) and no significant difference was observed in frequencies between the sexes (p = 0.18). Penetrance of deafness was estimated as 0.72. Testing the association of red/blue coat colour and deafness without accounting for pedigree structure showed that red dogs were 1.8 times more likely to be deaf (p = 0.045). The within family association between red/blue coat colour and deafness was strongly significant (p = 0.00036), with red coat colour segregating more frequently with deafness (COR = 0.48). The relationship between deafness and coat speckling approached significance (p = 0.07), with the lack of statistical significance possibly due to only four families co-segregating for both deafness and speckling. The deafness phenotype was mapped to CFA10 (maximum linkage peak on CFA10 −log10 p-value = 3

  5. Congenital sensorineural deafness in Australian stumpy-tail cattle dogs is an autosomal recessive trait that maps to CFA10.

    Directory of Open Access Journals (Sweden)

    Susan Sommerlad

    Full Text Available BACKGROUND: Congenital sensorineural deafness is an inherited condition found in many dog breeds, including Australian Stumpy-tail Cattle Dogs (ASCD. This deafness is evident in young pups and may affect one ear (unilateral or both ears (bilateral. The genetic locus/loci involved is unknown for all dog breeds. The aims of this study were to determine incidence, inheritance mechanism, and possible association of congenital sensorineural deafness with coat colour in ASCD and to identify the genetic locus underpinning this disease. METHODOLOGY/PRINCIPAL FINDINGS: A total of 315 ASCD were tested for sensorineural deafness using the brain stem auditory evoked response (BAER test. Disease penetrance was estimated directly, using the ratio of unilaterally to bilaterally deaf dogs, and segregation analysis was performed using Mendel. A complete genome screen was undertaken using 325 microsatellites spread throughout the genome, on a pedigree of 50 BAER tested ASCD in which deafness was segregating. Fifty-six dogs (17.8% were deaf, with 17 bilaterally and 39 unilaterally deaf. Unilaterally deaf dogs showed no significant left/right bias (p = 0.19 and no significant difference was observed in frequencies between the sexes (p = 0.18. Penetrance of deafness was estimated as 0.72. Testing the association of red/blue coat colour and deafness without accounting for pedigree structure showed that red dogs were 1.8 times more likely to be deaf (p = 0.045. The within family association between red/blue coat colour and deafness was strongly significant (p = 0.00036, with red coat colour segregating more frequently with deafness (COR = 0.48. The relationship between deafness and coat speckling approached significance (p = 0.07, with the lack of statistical significance possibly due to only four families co-segregating for both deafness and speckling. The deafness phenotype was mapped to CFA10 (maximum linkage peak on CFA10 -log10 p

  6. Congenital sensorineural deafness in Australian stumpy-tail cattle dogs is an autosomal recessive trait that maps to CFA10.

    Science.gov (United States)

    Sommerlad, Susan; McRae, Allan F; McDonald, Brenda; Johnstone, Isobel; Cuttell, Leigh; Seddon, Jennifer M; O'Leary, Caroline A

    2010-10-12

    Congenital sensorineural deafness is an inherited condition found in many dog breeds, including Australian Stumpy-tail Cattle Dogs (ASCD). This deafness is evident in young pups and may affect one ear (unilateral) or both ears (bilateral). The genetic locus/loci involved is unknown for all dog breeds. The aims of this study were to determine incidence, inheritance mechanism, and possible association of congenital sensorineural deafness with coat colour in ASCD and to identify the genetic locus underpinning this disease. A total of 315 ASCD were tested for sensorineural deafness using the brain stem auditory evoked response (BAER) test. Disease penetrance was estimated directly, using the ratio of unilaterally to bilaterally deaf dogs, and segregation analysis was performed using Mendel. A complete genome screen was undertaken using 325 microsatellites spread throughout the genome, on a pedigree of 50 BAER tested ASCD in which deafness was segregating. Fifty-six dogs (17.8%) were deaf, with 17 bilaterally and 39 unilaterally deaf. Unilaterally deaf dogs showed no significant left/right bias (p = 0.19) and no significant difference was observed in frequencies between the sexes (p = 0.18). Penetrance of deafness was estimated as 0.72. Testing the association of red/blue coat colour and deafness without accounting for pedigree structure showed that red dogs were 1.8 times more likely to be deaf (p = 0.045). The within family association between red/blue coat colour and deafness was strongly significant (p = 0.00036), with red coat colour segregating more frequently with deafness (COR = 0.48). The relationship between deafness and coat speckling approached significance (p = 0.07), with the lack of statistical significance possibly due to only four families co-segregating for both deafness and speckling. The deafness phenotype was mapped to CFA10 (maximum linkage peak on CFA10 -log10 p-value = 3.64), as was both coat colour and speckling. Fine

  7. UNILATERAL SENSORINEURAL DEAFNESS IN SCHOOL CHILDREN; A CLINICAL AND AUDIOLOGICAL EVALUATION AT A TERTIARY HOSPITAL OF TELANGANA

    Directory of Open Access Journals (Sweden)

    Harish Chandra Reddy

    2016-01-01

    Full Text Available INTRODUCTION Interpersonal communication, day to day activities, learning language and acquiring grades in the school are important parts in the life of school going children. Unilateral or bilateral Loss of hearing affects education and the child feels handicapped socially, emotionally, and scholastically. Such children lose grades and retained in the same class affecting them emotionally. The present study was conducted in order to find out the various causes of unilateral Sensorineural Hearing Loss (USNHL in children and to study role of various audiological findings in diagnosing the cause of impaired hearing in children. AIMS AND OBJECTIVES To find out the possible etiological causes and study the role of various audiological tests in finding the causes of unilateral impaired hearing in children. MATERIALS AND METHODS A prospective study was conducted in the Department of ENT of a tertiary Hospital attached to a Medical College in Warangal, Telangana. A total of 346 children aged between 4 and 15 years attended presenting with complaints of impaired hearing. Among them 73 children with unilateral sensorineural deafness were included in the study. After a thorough ENT examination subjective tests like pure tone audiometry and behavioral observation audiometry and objective tests like impedance audiometry, Oto-acoustic emissions (OAE, and brainstem evoked response audiometry (BERA were performed. RESULTS While identifying the possible etiological cause of USNHL, OAE revealed 56.16% REFER and 43.83% PASS results. Impedance audiometric results showed 71.22% as Type A Tympanogram. Pure tone audiometric results were mostly showed severe and profound hearing losses together accounting to 87.66%. BERA findings were mostly on severe and profound hearing loss (90.40%. No children with syndromic or cranio-facial abnormalities were found. CONCLUSIONS A prospective Hospital based study of school children with USNHL showed acquired nature of the

  8. Phonological Awareness and Reading Proficiency in Adults with Profound Deafness

    Science.gov (United States)

    Furlonger, Brett; Holmes, Virginia M.; Rickards, Field W.

    2014-01-01

    This study investigated differences in the phonological knowledge and reading skill of deaf adults using three experimental conditions that tested sensitivity to syllables, rhyme, and phonemes. Analysis of response latencies and accuracy in the three awareness tasks demonstrated that skilled deaf readers had superior phonological awareness skill…

  9. Possible mechanisms for sensorineural hearing loss and deafness in patients with propionic acidemia.

    Science.gov (United States)

    Grünert, S C; Bodi, I; Odening, K E

    2017-02-13

    Propionic acidemia is an inborn error of metabolism caused by deficiency of the mitochondrial enzyme propionyl-CoA carboxylase. Sensorineural deafness and severe hearing loss have been described as long-term complications of this disease, however, the mechanism has not yet been elucidated. We have recently shown by patch clamping experiments and Western blots that acute and chronic effects of accumulating metabolites such as propionic acid, propionylcarnitine and methylcitrate on the KvLQT1/KCNE1 channel complex cause long QT syndrome in patients with propionic acidemia by inhibition of K+ flow via this channel. The same KvLQT1/KCNE1 channel complex is expressed in the inner ear and essential for luminal potassium secretion into the endolymphatic space. A disruption of this K+ flow results in sensorineural hearing loss or deafness. It can be assumed that acute and chronic effects of accumulating metabolites on the KvLQT1/KCNE1 channel protein may similarly cause the hearing impairment of patients with propionic acidemia.

  10. The role of alternative GJB2 transcription in screening for neonatal sensorineural deafness in Austria.

    Science.gov (United States)

    Parzefall, Thomas; Lucas, Trevor; Koenighofer, Martin; Ramsebner, Reinhard; Frohne, Alexandra; Czeiger, Shelly; Baumgartner, Wolf-Dieter; Schoefer, Christian; Gstoettner, Wolfgang; Frei, Klemens

    2017-04-01

    Alterations within a novel putative Exon 1a within the gap junction beta 2 (GJB2) gene may play a role in the development of genetic hearing impairment in Austria. Mutations in the GJB2 gene are the most common cause of hereditary sensorineural deafness. Genome-wide screening for alternative transcriptional start sites in the human genome has revealed the presence of an additional GJB2 exon (E1a). This study tested the hypothesis of whether alternative GJB2 transcription involving E1a may play a role in the development of congenital sensorineural deafness in Austria. GJB2 E1a and flanking regions were sequenced in randomized normal hearing control subjects and three different patient groups with non-syndromic hearing impairment (NSHI), and bioinformatic analysis was performed. Statistical analysis of disease association was carried out using the Cochran-Armitage test for trend. A single change 2410 bp proximal to the translational start site (c.-2410T > C, rs7994748, NM_004004.5:c.-23 + 792T > C) was found to be significantly associated with the common c.35delG GJB2 mutation (p = .009). c.35delG in combination with c.-2410CC occurred at a 6.9-fold increased frequency compared to the control group. Additionally, one patient with idiopathic congenital hearing loss was found to be homozygous c.-2410CC.

  11. Neurodevelopmental Disorders in Children with Severe to Profound Sensorineural Hearing Loss: A Clinical Study

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    Chilosi, Anna M.; Comparini, Alessandro; Scusa, Maria F.; Berrettini, Stefano; Forli, Francesca; Battini, Roberta; Cipriani, Paola; Cioni, Giovanni

    2010-01-01

    Aim: The effects of sensorineural hearing loss (SNHL) are often complicated by additional disabilities, but the epidemiology of associated disorders is not clearly defined. The aim of this study was to evaluate the frequency and type of additional neurodevelopmental disabilities in a sample of children with SNHL and to investigate the relation…

  12. Sensorineural deafness

    Science.gov (United States)

    ... your hearing. The following may be helpful: Hearing aids Telephone amplifiers and other assistive devices Sign language (for those with severe hearing loss) Speech reading (such as lip reading and using visual cues to aid communication) A cochlear implant may be recommended for certain ...

  13. NLRP3 Is Expressed in the Spiral Ganglion Neurons and Associated with Both Syndromic and Nonsyndromic Sensorineural Deafness

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    Penghui Chen

    2016-01-01

    Full Text Available Nonsyndromic deafness is genetically heterogeneous but phenotypically similar among many cases. Though a variety of targeted next-generation sequencing (NGS panels has been recently developed to facilitate genetic screening of nonsyndromic deafness, some syndromic deafness genes outside the panels may lead to clinical phenotypes similar to nonsyndromic deafness. In this study, we performed comprehensive genetic screening in a dominant family in which the proband was initially diagnosed with nonsyndromic deafness. No pathogenic mutation was identified by targeted NGS in 72 nonsyndromic and another 72 syndromic deafness genes. Whole exome sequencing, however, identified a p.E313K mutation in NLRP3, a gene reported to cause syndromic deafness Muckle-Wells Syndrome (MWS but not included in any targeted NGS panels for deafness in previous reports. Follow-up clinical evaluation revealed only minor inflammatory symptoms in addition to deafness in six of the nine affected members, while the rest, three affected members, including the proband had no obvious MWS-related inflammatory symptoms. Immunostaining of the mouse cochlea showed a strong expression of NLRP3 in the spiral ganglion neurons. Our results suggested that NLRP3 may have specific function in the spiral ganglion neurons and can be associated with both syndromic and nonsyndromic sensorineural deafness.

  14. Long-term follow-up in distal renal tubular acidosis with sensorineural deafness.

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    Peces, R

    2000-11-01

    A 20-year-old man presented with failure to thrive and bilateral genu valgum. On the basis of growth failure, skeletal deformity, hyperchloremic metabolic acidosis with alkaline urine and hypokalemia, nephrocalcinosis, and hearing loss, a diagnosis of distal renal tubular acidosis (DRTA) with sensorineural deafness was made. The genu valgum was treated by corrective osteotomy. Skeletal deformity was corrected and impaired growth improved after sustained therapy of metabolic acidosis with alkali supplementation. During an 8-year follow-up period the patient's glomerular filtration rate remained stable, the nephrocalcinosis did not progress, and his height increased 10 cm. Although nephrolithiasis led to atrophy of the right kidney, at last follow-up, when the patient was 44 years old, his creatinine clearance was 50 ml/min per 1.73 m2 body surface.

  15. Labyrinthine window rupture as a cause of acute sensorineural hearing loss

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    Nagai, Tomoyuki; Nagai, Midori

    2011-01-01

    Labyrinthine window rupture (LWR) is one cause of acute sensorineural hearing loss and need for early exploration is clear for good improved hearing. Acute sensorineural hearing loss of 60?dB or more treated from May 2006 to May 2010 were retrospectively analyzed. There were 21 ears of severe deafness, 18 ears of profound deafness, and 10 ears of total deafness. All patients were examined with temporal bone CT. Space-occupying lesions around the labyrinthine windows were suggestive images of ...

  16. Speech timing and working memory in profoundly deaf children after cochlear implantation

    OpenAIRE

    Burkholder, Rose A.; Pisoni, David B.

    2003-01-01

    Thirty-seven profoundly deaf children between 8- and 9-years-old with cochlear implants and a comparison group of normal-hearing children were studied to measure speaking rates, digit spans, and speech timing during digit span recall. The deaf children displayed longer sentence durations and pauses during recall and shorter digit spans compared to the normal-hearing children. Articulation rates, measured from sentence durations, were strongly correlated with immediate memory span in both norm...

  17. Beneficios económicos del implante coclear para la hipoacusia sensorineural profunda Economic benefits of the cochlear implant for treating profound sensorineural hearing loss

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    Augusto Peñaranda

    2012-04-01

    Full Text Available OBJETIVO: Evaluar el costo-beneficio (CB, costo-utilidad (CU y costo-efectividad (CE de la implantación coclear, comparándola con el uso de audífonos en niños con hipoacusia sensorineural profunda bilateral. MÉTODOS: Se empleó la técnica no paramétrica Propensity Score Matching (PSM para realizar la evaluación de impacto económico del implante y así llevar a cabo los análisis CB, CU y CE. Se utilizó información primaria, tomada aleatoriamente a 100 pacientes: 62 intervenidos quirúrgicamente con el implante coclear (grupo de tratamiento y 38 pertenecientes al grupo de control o usuarios de audífono para tratar la hipoacusia sensorineural profunda. RESULTADOS: Se halló un diferencial de costos económicos -en beneficio del implante coclear- cercano a US$ 204 000 entre el implante y el uso de audífonos durante la esperanza de vida de los pacientes analizados. Dicha cifra indica los mayores gastos que deben cubrir los pacientes con audífono. Con este valor descontado, el indicador costo-beneficio señala que por cada dólar invertido en el implante coclear, para tratar al paciente, el retorno de la inversión es US$ 2,07. CONCLUSIONES: El implante coclear genera beneficios económicos para el paciente. También produce utilidades en salud dado que se encontró una relación positiva de CU (ganancia en decibeles y CE (ganancia en discriminación del lenguaje.OBJECTIVE: Evaluate the cost-benefit, cost-utility, and cost-effectiveness of cochlear implantation, comparing it to the use of hearing aids in children with profound bilateral sensorineural hearing loss. METHODS: The nonparametric propensity score matching method was used to carry out an economic and impact assessment of the cochlear implant and then perform cost-benefit, cost-utility, and cost-effectiveness analyses. Primary information was used, taken randomly from 100 patients: 62 who received cochlear implants (treatment group and 38 belonging to the control group who used

  18. Genetic frequencies related to severe or profound sensorineural hearing loss in Inner Mongolia Autonomous Region

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    Yongzhi Liu

    Full Text Available Abstract The aim was to study the frequencies of common deafness-related mutations and their contribution to hearing loss in different regions of Inner Mongolia. A total of 738 deaf children were recruited from five different ethnic groups of Inner Mongolia, including Han Chinese (n=486, Mongolian (n=216, Manchurian (n=24, Hui (n=6 and Daur (n=6. Nine common mutations in four genes (GJB2, SLC26A4, GJB3 and mitochondrial MT-RNR1 gene were detected by allele-specific PCR and universal array. At least one mutated allele was detected in 282 patients. Pathogenic mutations were detected in 168 patients: 114 were homozygotes and 54 were compound heterozygotes. The 114 patients were carriers of only one mutated allele. The frequency of GJB2 variants in Han Chinese (21.0% was higher than that in Mongolians (16.7%, but not significantly different. On the other hand, the frequency of SLC26A4 variants in Han Chinese (14.8% was lower than that in Mongolians (19.4%, but also not significantly different. The frequency of patients with pathogenic mutations was different in Ulanqab (21.4%, Xilingol (40.0%, Chifeng (40.0%, Hulunbeier (30.0%, Hohhot (26.3%, and in Baotou (0%. In conclusion, the frequency of mutated alleles in deafness-related genes did not differ between Han Chinese and Mongolians. However, differences in the distribution of common deafness-related mutations were found among the investigated areas of Inner Mongolia.

  19. The Hands with Eyes and Nose in the Palm: As Effective Communication Alternatives for Profoundly Deaf People in Zimbabwe

    Science.gov (United States)

    Mutswanga, Phillipa

    2017-01-01

    Drawing from the experiences and testimonies of people with profound deafness, the study qualitatively explored the use of the hands with eyes and nose in the palm as communication alternatives in the field of deafness. The study was prompted by the 27 year old lady, Leah Katz-Hernandez who is deaf who got engaged in March 2015 as the 2016…

  20. A role of the microphthalmia-associated transcription factor in congenital sensorineural deafness and eye pigmentation in Dalmatian dogs.

    Science.gov (United States)

    Stritzel, S; Wöhlke, A; Distl, O

    2009-02-01

    Microphthalmia-associated transcription factor (MITF) is involved in white spotting and deafness associated with lack of pigmentation in human and mice. In the present study, we employed MITF-associated markers to evaluate MITF as a candidate for canine congenital sensorineural deafness (CCSD) in Dalmatian dogs. We performed an association study using MITF flanking and intragenic markers for 88 Dalmatian dogs of different hearing and eye pigmentation status. A significant association was identified for MITF-related markers with CCSD and blue iris colour. We conclude that MITF might play a role in CCSD and blue eye colour in Dalmatian dogs.

  1. The Influence of Phonological Mechanisms in Written Spelling of Profoundly Deaf Children

    Science.gov (United States)

    Colombo, Lucia; Arfe, Barbara; Bronte, Tiziana

    2012-01-01

    In the present study, the effect of phonological and working memory mechanisms involved in spelling Italian single words was explored in two groups of children matched for grade level: a group of normally hearing children and a group of pre-verbally deaf children, with severe-to-profound hearing loss. Three-syllable and four-syllable familiar…

  2. Assessing the Efficacy of an Academic Hearing Peer Tutor for a Profoundly Deaf Student.

    Science.gov (United States)

    Burley, Suzanne; And Others

    1994-01-01

    Evaluation of using a hearing peer tutor to provide daily 20-minute math instruction for a profoundly deaf sixth-grade girl indicated that the peer tutoring intervention was highly successful, with the tutee meeting accuracy criteria for each of 4 curriculum objectives after only a brief period of intervention. (Author/DB)

  3. Multisensory Narrative Tracking by a Profoundly Deaf Subject Using an Electrocutaneous Vocoder and a Vibrotactile Aid.

    Science.gov (United States)

    Lynch, Michael P.; And Others

    1989-01-01

    The study assessed the ability to track connected discourse by a congenitally profoundly deaf adult using an electrocutaneous vocoder and/or a vibrotactile aid in conjunction with or without lipreading and aided hearing. Overall, improvement in tracking performance occurred within and across phases of the study. (Author/DB)

  4. Prevalence of congenital hereditary sensorineural deafness in Australian Cattle Dogs and associations with coat characteristics and sex

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    Sommerlad Susan F

    2012-10-01

    Full Text Available Abstract Background Congenital hereditary sensorineural deafness (CHSD occurs in many dog breeds, including Australian Cattle Dogs. In some breeds, CHSD is associated with a lack of cochlear melanocytes in the stria vascularis, certain coat characteristics, and potentially, abnormalities in neuroepithelial pigment production. This study investigates phenotypic markers for CHSD in 899 Australian Cattle Dogs. Results Auditory function was tested in 899 Australian Cattle Dogs in family groups using brainstem auditory evoked response testing. Coat colour and patterns, facial and body markings, gender and parental hearing status were recorded. Deafness prevalence among all 899 dogs was 10.8% with 7.5% unilaterally deaf, and 3.3% bilaterally deaf, and amongst pups from completely tested litters (n = 696 was 11.1%, with 7.5% unilaterally deaf, and 3.6% bilaterally deaf. Univariable and multivariable analyses revealed a negative association between deafness and bilateral facial masks (odds ratio 0.2; P ≤ 0.001. Using multivariable logistic animal modelling, the risk of deafness was lower in dogs with pigmented body spots (odds ratio 0.4; P = 0.050. No significant associations were found between deafness and coat colour. Within unilaterally deaf dogs with unilateral facial masks, no association was observed between the side of deafness and side of mask. The side of unilateral deafness was not significantly clustered amongst unilaterally deaf dogs from the same litter. Females were at increased risk of deafness (odds ratio from a logistic animal model 1.9; P = 0.034 after adjusting for any confounding by mask type and pigmented body spots. Conclusions Australian Cattle Dogs suffer from CHSD, and this disease is more common in dogs with mask-free faces, and in those without pigmented body patches. In unilaterally deaf dogs with unilateral masks, the lack of observed association between side of deafness and side of mask suggests that if CHSD is due to

  5. Prevalence of congenital hereditary sensorineural deafness in Australian Cattle Dogs and associations with coat characteristics and sex.

    Science.gov (United States)

    Sommerlad, Susan F; Morton, John M; Haile-Mariam, Mekonnen; Johnstone, Isobel; Seddon, Jennifer M; O'Leary, Caroline A

    2012-10-29

    Congenital hereditary sensorineural deafness (CHSD) occurs in many dog breeds, including Australian Cattle Dogs. In some breeds, CHSD is associated with a lack of cochlear melanocytes in the stria vascularis, certain coat characteristics, and potentially, abnormalities in neuroepithelial pigment production. This study investigates phenotypic markers for CHSD in 899 Australian Cattle Dogs. Auditory function was tested in 899 Australian Cattle Dogs in family groups using brainstem auditory evoked response testing. Coat colour and patterns, facial and body markings, gender and parental hearing status were recorded.Deafness prevalence among all 899 dogs was 10.8% with 7.5% unilaterally deaf, and 3.3% bilaterally deaf, and amongst pups from completely tested litters (n = 696) was 11.1%, with 7.5% unilaterally deaf, and 3.6% bilaterally deaf.Univariable and multivariable analyses revealed a negative association between deafness and bilateral facial masks (odds ratio 0.2; P ≤ 0.001). Using multivariable logistic animal modelling, the risk of deafness was lower in dogs with pigmented body spots (odds ratio 0.4; P = 0.050).No significant associations were found between deafness and coat colour.Within unilaterally deaf dogs with unilateral facial masks, no association was observed between the side of deafness and side of mask. The side of unilateral deafness was not significantly clustered amongst unilaterally deaf dogs from the same litter. Females were at increased risk of deafness (odds ratio from a logistic animal model 1.9; P = 0.034) after adjusting for any confounding by mask type and pigmented body spots. Australian Cattle Dogs suffer from CHSD, and this disease is more common in dogs with mask-free faces, and in those without pigmented body patches. In unilaterally deaf dogs with unilateral masks, the lack of observed association between side of deafness and side of mask suggests that if CHSD is due to defects in molecular pigment pathways, the molecular control

  6. Interaural comparison of spiral ganglion cell counts in profound deafness.

    Science.gov (United States)

    Seyyedi, Mohammad; Eddington, Donald K; Nadol, Joseph B

    2011-12-01

    This study is designed to measure the degree to which spiral ganglion cell (SGC) survival in the left and right ears is similar in profoundly hearing-impaired human patients with symmetric (right/left) etiology and sensitivity. This is of interest because a small difference between ears would imply that one ear could be used as a control ear in temporal bone studies evaluating the impact on SGC survival of a medical intervention in the other ear. Forty-two temporal bones from 21 individuals with bilaterally symmetric profound hearing impairment were studied. Both ears in each individual were impaired by the same etiology. Rosenthal's canal was reconstructed in two dimensions and segmental and total SGCs were counted. Correlation analysis and t-tests were used to compare segmental and total counts of left and right ears. Statistical power calculations illustrate how the results can be used to estimate the effect size (right/left difference in SGC count) that can be reliably identified as a function of sample size. Left counts (segmental and total) were significantly correlated with those in the right ears (p total count were respectively 0.64, 0.91, 0.93, 0.91 and 0.98. The hypothesis that mean segmental and total counts of right and left are the same could not be rejected by paired t-test. The variance in the between-ear difference across the temporal bones studied indicates that useful effect sizes can be reliably identified using subject numbers that are practical for temporal bone studies. For instance, there is 95% likelihood that an interaural difference in SGC count of approximately 1000 cells associated with a treatment/manipulation of one ear will be reliably detected in a bilaterally-symmetric profound hearing loss population of temporal bones from approximately 10 subjects. Copyright © 2011 Elsevier B.V. All rights reserved.

  7. Whole exome sequencing identifies mutations in Usher syndrome genes in profoundly deaf Tunisian patients.

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    Zied Riahi

    Full Text Available Usher syndrome (USH is an autosomal recessive disorder characterized by combined deafness-blindness. It accounts for about 50% of all hereditary deafness blindness cases. Three clinical subtypes (USH1, USH2, and USH3 are described, of which USH1 is the most severe form, characterized by congenital profound deafness, constant vestibular dysfunction, and a prepubertal onset of retinitis pigmentosa. We performed whole exome sequencing in four unrelated Tunisian patients affected by apparently isolated, congenital profound deafness, with reportedly normal ocular fundus examination. Four biallelic mutations were identified in two USH1 genes: a splice acceptor site mutation, c.2283-1G>T, and a novel missense mutation, c.5434G>A (p.Glu1812Lys, in MYO7A, and two previously unreported mutations in USH1G, i.e. a frameshift mutation, c.1195_1196delAG (p.Leu399Alafs*24, and a nonsense mutation, c.52A>T (p.Lys18*. Another ophthalmological examination including optical coherence tomography actually showed the presence of retinitis pigmentosa in all the patients. Our findings provide evidence that USH is under-diagnosed in Tunisian deaf patients. Yet, early diagnosis of USH is of utmost importance because these patients should undergo cochlear implant surgery in early childhood, in anticipation of the visual loss.

  8. Congenital sensorineural deafness in dalmatian dogs associated with quantitative trait loci.

    Science.gov (United States)

    Kluth, Susanne; Distl, Ottmar

    2013-01-01

    A genome-wide association study (GWAS) was performed for 235 Dalmatian dogs using the canine Illumina high density bead chip to identify quantitative trait loci (QTL) associated with canine congenital sensorineural deafness (CCSD). Data analysis was performed for all Dalmatian dogs and in addition, separately for brown-eyed and blue-eyed dogs because of the significant influence of eye colour on CCSD in Dalmatian dogs. Mixed linear model analysis (MLM) revealed seven QTL with experiment-wide significant associations (-log10P>5.0) for CCSD in all Dalmatian dogs. Six QTL with experiment-wide significant associations for CCSD were found in brown-eyed Dalmatian dogs and in blue-eyed Dalmatian dogs, four experiment-wide significant QTL were detected. The experiment-wide CCSD-associated SNPs explained 82% of the phenotypic variance of CCSD. Five CCSD-loci on dog chromosomes (CFA) 6, 14, 27, 29 and 31 were in close vicinity of genes shown as causative for hearing loss in human and/or mouse.

  9. Congenital sensorineural deafness in dalmatian dogs associated with quantitative trait loci.

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    Susanne Kluth

    Full Text Available A genome-wide association study (GWAS was performed for 235 Dalmatian dogs using the canine Illumina high density bead chip to identify quantitative trait loci (QTL associated with canine congenital sensorineural deafness (CCSD. Data analysis was performed for all Dalmatian dogs and in addition, separately for brown-eyed and blue-eyed dogs because of the significant influence of eye colour on CCSD in Dalmatian dogs. Mixed linear model analysis (MLM revealed seven QTL with experiment-wide significant associations (-log10P>5.0 for CCSD in all Dalmatian dogs. Six QTL with experiment-wide significant associations for CCSD were found in brown-eyed Dalmatian dogs and in blue-eyed Dalmatian dogs, four experiment-wide significant QTL were detected. The experiment-wide CCSD-associated SNPs explained 82% of the phenotypic variance of CCSD. Five CCSD-loci on dog chromosomes (CFA 6, 14, 27, 29 and 31 were in close vicinity of genes shown as causative for hearing loss in human and/or mouse.

  10. Syndrome of congenital cataracts, sensorineural deafness, Down syndrome-like facial appearance, short stature, and mental retardation: two additional cases.

    Science.gov (United States)

    Keppler-Noreuil, Kim; Welch, Judy; Baker-Lange, Katherine

    2007-11-01

    An apparently new syndrome of congenital cataracts, sensorineural deafness, Down syndrome-like facial appearance, short stature, and mental retardation was described by Gripp et al. 1996. The authors reported on two unrelated patients with congenital cataracts, sensorineural deafness, distinctive facial appearance, mental retardation, postnatal short stature, and skeletal changes. We report on two additional patients with findings most similar to the reported patients by Gripp et al. 1996, including bilateral congenital cataracts, hearing loss, craniofacial abnormalities, short stature, skeletal abnormalities, and developmental delay. Both of the patients reported herein had chromosome microarray analysis, which showed normal results in Patient 2 but abnormal results in Patient 1 and his mother who both had a chromosome 11q25 subtelomere deletion. Patient 1 and his mother's findings are atypical for the common findings reported in Jacobsen syndrome (11q terminal deletion syndrome), and consistent with the patients reported by Gripp et al. 1996. The etiology for these cases has been unknown. The microarray results on Patient 1 suggest that the other patients with findings of developmental delay, short stature, congenital cataracts, sensorineural hearing loss, and similar craniofacial features may have either a microdeletion of chromosome 11q terminal region or haploinsufficiency of a gene localized to this region. Copyright 2007 Wiley-Liss, Inc.

  11. [Speech perception test in Italian language for profoundly deaf children].

    Science.gov (United States)

    Genovese, E; Orzan, E; Turrini, M; Babighian, G; Arslan, E

    1995-10-01

    Speech perception tests are an important part of procedures for diagnosing pre-verbal hearing loss. Merely establishing a child's hearing threshold with and without a hearing aid is not sufficient to ensure an adequate evaluation with a view to selecting cases suitable for cochlear implants because it fails to indicate the real benefit obtained from using a conventional hearing aid reliably. Speech perception tests have proved useful not only for patient selection, but also for subsequent evaluation of the efficacy of new hearing aids, such as tactile devices and cochlear implants. In clinical practice, the tests most commonly adopted with small children are: The Auditory Comprehension Test (ACT), Discrimination after Training (DAT), Monosyllable, Trochee, Spondee tests (MTS), Glendonald Auditory Screening Priocedure (GASP), Early Speech Perception Test (ESP), Rather than considering specific results achieved in individual cases, reference is generally made to the four speech perception classes proposed by Moog and Geers of the CID of St. Louis. The purpose of this classification, made on the results obtained with suitably differentiated tests according to the child's age and language ability, is to detect differences in perception of a spoken message in ideal listening conditions. To date, no italian language speech perception test has been designed to establish the assessment of speech perception level in children with profound hearing impairment. We attempted, therefore, to adapt the existing English tests to the Italian language taking into consideration the differences between the two languages. Our attention focused on the ESP test since it can be applied to even very small children (2 years old). The ESP is proposed in a standard version for hearing-impaired children over the age of 6 years and in a simplified version for younger children. The rationale we used for selecting Italian words reflect the rationale established for the original version, but the

  12. Vestibular function of patients with profound deafness related to GJB2 mutation.

    Science.gov (United States)

    Kasai, Misato; Hayashi, Chieri; Iizuka, Takashi; Inoshita, Ayako; Kamiya, Kazusaku; Okada, Hiroko; Nakajima, Yukinori; Kaga, Kimitaka; Ikeda, Katsuhisa

    2010-09-01

    GJB2 mutations are responsible not only for deafness but also for the occurrence of vestibular dysfunction. However, vestibular dysfunction tends to be unilateral and less severe in comparison with that of bilateral deafness. The correlation between the cochlear and vestibular end-organs suggests that some children with congenital deafness may have vestibular impairments. On the other hand, GJB2 gene mutations are the most common cause of nonsyndromic deafness. The vestibular function of patients with congenital deafness (CD), which is related to GJB2 gene mutation, remains to be elucidated. The purpose of this study was to analyze the relationship between GJB2 gene mutation and vestibular dysfunction in adults with CD. A total of 31 subjects, including 10 healthy volunteers and 21 patients with CD, were enrolled in the study. A hearing test and genetic analysis were performed. The vestibular evoked myogenic potentials (VEMPs) were measured and a caloric test was performed to assess the vestibular function. The percentage of vestibular dysfunction was then statistically analyzed. The hearing level of all CD patients demonstrated a severe to profound impairment. In seven CD patients, their hearing impairment was related to GJB2 mutation. Five of the seven patients with CD related to GJB2 mutation demonstrated abnormalities in one or both of the two tests. The percentage of vestibular dysfunction of the patients with CD related to GJB2 mutation was statistically higher than in patients with CD unrelated to GJB2 mutation and in healthy controls.

  13. Evaluation of the occurrence of canine congenital sensorineural deafness in puppies of predisposed dog breeds using the brainstem auditory evoked response.

    Science.gov (United States)

    Płonek, Marta; Giza, Elżbieta; Niedźwiedź, Artur; Kubiak, Krzysztof; Nicpoń, Józef; Wrzosek, Marcin

    2016-12-01

    Canine congenital sensorineural deafness (CCSD) affects predisposed breeds of dogs and is primarily caused by an atrophy of the stria vascularis of the organ of Corti. The analysis of the brainstem auditory evoked response (BAER) is a reliable method for the evaluation of hearing in animals as it allows an accurate detection of unilateral or bilateral deafness. The occurrence of unilateral and bilateral deafness using the BAER was determined in a representative group of dogs in Poland, including Bull Terriers (n = 117), Australian Cattle Dogs (n = 62), English Setters (n = 32) and the Dogo Argentino (n = 32). Overall deafness, deafness in each dog breed and an association between deafness and phenotype were studied. Among the 243 dogs tested, 156 (81%) had a normal BAER, 27 (11%) were unilaterally deaf, and 12 (5%) were bilaterally deaf. The amplitudes and latencies of waves I, II, III, V, the V/I wave amplitude ratio, and wave I-V, I-III and III-V inter-peak intervals were recorded for each dog. Unilaterally and bilaterally deaf dogs were present in all the dog breeds studied. There were 17 (14.5%) deaf Bull Terriers, three (4.8%) deaf Australian Cattle Dogs, seven (21.9%) deaf English Setters, and 12 (37.5%) deaf Dogos Argentinos. Preventive BAER screening should be routinely performed in these four breeds to prevent the spread of genes responsible for deafness.

  14. KCNJ10 gene mutations causing EAST syndrome (epilepsy, ataxia, sensorineural deafness, and tubulopathy) disrupt channel function.

    Science.gov (United States)

    Reichold, Markus; Zdebik, Anselm A; Lieberer, Evelyn; Rapedius, Markus; Schmidt, Katharina; Bandulik, Sascha; Sterner, Christina; Tegtmeier, Ines; Penton, David; Baukrowitz, Thomas; Hulton, Sally-Anne; Witzgall, Ralph; Ben-Zeev, Bruria; Howie, Alexander J; Kleta, Robert; Bockenhauer, Detlef; Warth, Richard

    2010-08-10

    Mutations of the KCNJ10 (Kir4.1) K(+) channel underlie autosomal recessive epilepsy, ataxia, sensorineural deafness, and (a salt-wasting) renal tubulopathy (EAST) syndrome. We investigated the localization of KCNJ10 and the homologous KCNJ16 in kidney and the functional consequences of KCNJ10 mutations found in our patients with EAST syndrome. Kcnj10 and Kcnj16 were found in the basolateral membrane of mouse distal convoluted tubules, connecting tubules, and cortical collecting ducts. In the human kidney, KCNJ10 staining was additionally observed in the basolateral membrane of the cortical thick ascending limb of Henle's loop. EM of distal tubular cells of a patient with EAST syndrome showed reduced basal infoldings in this nephron segment, which likely reflects the morphological consequences of the impaired salt reabsorption capacity. When expressed in CHO and HEK293 cells, the KCNJ10 mutations R65P, G77R, and R175Q caused a marked impairment of channel function. R199X showed complete loss of function. Single-channel analysis revealed a strongly reduced mean open time. Qualitatively similar results were obtained with coexpression of KCNJ10/KCNJ16, suggesting a dominance of KCNJ10 function in native renal KCNJ10/KCNJ16 heteromers. The decrease in the current of R65P and R175Q was mainly caused by a remarkable shift of pH sensitivity to the alkaline range. In summary, EAST mutations of KCNJ10 lead to impaired channel function and structural changes in distal convoluted tubules. Intriguingly, the metabolic alkalosis present in patients carrying the R65P mutation possibly improves residual function of KCNJ10, which shows higher activity at alkaline pH.

  15. [Phenotypic trends and breeding values for canine congenital sensorineural deafness in Dalmatian dogs].

    Science.gov (United States)

    Blum, Meike; Distl, Ottmar

    2014-01-01

    In the present study, breeding values for canine congenital sensorineural deafness, the presence of blue eyes and patches have been predicted using multivariate animal models to test the reliability of the breeding values for planned matings. The dataset consisted of 6669 German Dalmatian dogs born between 1988 and 2009. Data were provided by the Dalmatian kennel clubs which are members of the German Association for Dog Breeding and Husbandry (VDH). The hearing status for all dogs was evaluated using brainstem auditory evoked potentials. The reliability using the prediction error variance of breeding values and the realized reliability of the prediction of the phenotype of future progeny born in each one year between 2006 and 2009 were used as parameters to evaluate the goodness of prediction through breeding values. All animals from the previous birth years were used for prediction of the breeding values of the progeny in each of the up-coming birth years. The breeding values based on pedigree records achieved an average reliability of 0.19 for the future 1951 progeny. The predictive accuracy (R2) for the hearing status of single future progeny was at 1.3%. Combining breeding values for littermates increased the predictive accuracy to 3.5%. Corresponding values for maternal and paternal half-sib groups were at 3.2 and 7.3%. The use of breeding values for planned matings increases the phenotypic selection response over mass selection. The breeding values of sires may be used for planned matings because reliabilities and predictive accuracies for future paternal progeny groups were highest.

  16. Lemierre’s Syndrome Associated with Mechanical Ventilation and Profound Deafness

    Directory of Open Access Journals (Sweden)

    Lukas Birkner

    2017-01-01

    Full Text Available Lemierre’s syndrome is a rare disorder that is characterized by anaerobic organisms inducing a thrombophlebitis of the internal jugular vein (IJV following a course of oropharyngeal infection. It often occurs in young and healthy patients. Clinicians continuously misinterpret early symptoms until infection disseminates systematically and life-threatening sepsis transpires. We report the case of a 58-year-old female developing Lemierre’s syndrome accompanied by invasive ventilation support and a profound deafness requiring the implementation of a cochlear implant. This is one of two reported cases of Lemierre’s syndrome associated with mechanical ventilation support and the only case associated with a cochlear implant.

  17. Clinical investigation on the beneficial effects of the Chinese medicinal herb Gushen Pian on sensorineural deafness and tinnitus.

    Science.gov (United States)

    Zhai, Suoqiang; Fang, Yaoyun; Yang, Weiyan; Gu, Rui; Han, Dongyi; Yang, Shiming

    2013-11-01

    The objective is to study the therapeutic effects of Gushen Pian on sensorineural deafness according to the Phase II clinical trial protocol, as approved for novel traditional Chinese medicines by Ministry of Health of PRC. This is a double blind study in which 120 patients were allocated randomly into treatment and control groups and an open treatment group (40 cases in each group). Patients in the treatment groups were administrated with Gushen Pian and controls received placebo. Routine examination of blood and urine, hepatic and renal function tests and pure tone audiometry were performed before and after treatment. Clinical symptoms and therapeutic outcomes were compared and evaluated. For double-blind treatment group, the total effective rate of deafness was 42.2% and total relieved rate of deafness was 4.6%; for double-blind control group, the total effective rate of deafness was 18.7% and total relieved rate of deafness was 0%; for simple treatment group, the total effective rate of deafness was 58.7% and total relieved rate of deafness was 6.3%. For double-blind treatment group, the total effective rate of tinnitus was 89.2% and total relieved rate of tinnitus was 59.5%; for double-blind control group, the total effective rate of tinnitus was 30.8% and total relieved rate of tinnitus was 5.1%; for simple treatment group, the total effective rate of tinnitus was 74.3% and total relieved rate of tinnitus was 57.1%. The double-blind treatment showed statistically significant differences from control group. The medication could effectively alleviate aural fullness, dizziness, lassitude of loins and knees, dysphoria with feverish sensation in chest, insomnia, and fatigue, etc. No adverse effect was reported during treatment; no abnormal results were reported in blood, urine, faces, heart function, liver function and kidney function examination. Gushen Pian had beneficial effect on deafness and tinnitus and could effectively alleviate aural fullness, insomnia

  18. Canine deafness.

    Science.gov (United States)

    Strain, George M

    2012-11-01

    Conductive deafness, caused by outer or middle ear obstruction, may be corrected, whereas sensorineural deafness cannot. Most deafness in dogs is congenital sensorineural hereditary deafness, associated with the genes for white pigment: piebald or merle. The genetic cause has not yet been identified. Dogs with blue eyes have a greater likelihood of hereditary deafness than brown-eyed dogs. Other common forms of sensorineural deafness include presbycusis, ototoxicity, noise-induced hearing loss, otitis interna, and anesthesia. Definitive diagnosis of deafness requires brainstem auditory evoked response testing. Copyright © 2012 Elsevier Inc. All rights reserved.

  19. Transient evoked otoacoustic emissions testing for screening of sensorineural deafness in puppies

    OpenAIRE

    McBrearty, A; Penderis, J

    2011-01-01

    Background: Transient evoked otoacoustic emissions (TEOAE) are widely used for human neonatal deafness screening, but have not been reported for clinical use in dogs. \\ud \\ud Hypothesis/Objectives: To investigate the feasibility of TEOAE testing in conscious puppies and the ability of TEOAE testing to correctly identify deaf and hearing ears, as defined by brainstem auditory evoked response (BAER). \\ud \\ud Animals: Forty puppies from 10 litters. \\ud \\ud Methods: Prospective study on puppies p...

  20. The False-Friend Effect in Three Profoundly Deaf Learners of French: Disentangling Morphology, Phonology and Orthography

    Science.gov (United States)

    Janke, Vikki; Kolokonte, Marina

    2015-01-01

    Three profoundly deaf individuals undertook a low-frequency backward lexical translation task (French/English), where morphological structure was manipulated and orthographic distance between test items was measured. Conditions included monomorphemic items (simplex), polymorphemic items (complex), items whose French morphological structure…

  1. Progressive sensorineural hearing impairment in maternally inherited diabetes mellitus and deafness (MIDD).

    NARCIS (Netherlands)

    Hendrickx, J.J.; Mudde, A.H.; Hart, L.M. 't; Huygen, P.L.M.; Cremers, C.W.R.J.

    2006-01-01

    OBJECTIVE: To study the progression of hearing impairment (HI) and audiological features in patients with the mitochondrial A to G mutation in the tRNA(LEU(RUU)) gene at position 3,243 associated with maternally inherited diabetes and deafness. DESIGN: Retrospective phenotype genotype family study.

  2. Dominant ER Stress-Inducing WFS1 Mutations Underlie a Genetic Syndrome of Neonatal/Infancy-Onset Diabetes, Congenital Sensorineural Deafness, and Congenital Cataracts.

    Science.gov (United States)

    De Franco, Elisa; Flanagan, Sarah E; Yagi, Takuya; Abreu, Damien; Mahadevan, Jana; Johnson, Matthew B; Jones, Garan; Acosta, Fernanda; Mulaudzi, Mphele; Lek, Ngee; Oh, Vera; Petz, Oliver; Caswell, Richard; Ellard, Sian; Urano, Fumihiko; Hattersley, Andrew T

    2017-07-01

    Neonatal diabetes is frequently part of a complex syndrome with extrapancreatic features: 18 genes causing syndromic neonatal diabetes have been identified to date. There are still patients with neonatal diabetes who have novel genetic syndromes. We performed exome sequencing in a patient and his unrelated, unaffected parents to identify the genetic etiology of a syndrome characterized by neonatal diabetes, sensorineural deafness, and congenital cataracts. Further testing was performed in 311 patients with diabetes diagnosed before 1 year of age in whom all known genetic causes had been excluded. We identified 5 patients, including the initial case, with three heterozygous missense mutations in WFS1 (4/5 confirmed de novo). They had diabetes diagnosed before 12 months (2 before 6 months) (5/5), sensorineural deafness diagnosed soon after birth (5/5), congenital cataracts (4/5), and hypotonia (4/5). In vitro studies showed that these WFS1 mutations are functionally different from the known recessive Wolfram syndrome-causing mutations, as they tend to aggregate and induce robust endoplasmic reticulum stress. Our results establish specific dominant WFS1 mutations as a cause of a novel syndrome including neonatal/infancy-onset diabetes, congenital cataracts, and sensorineural deafness. This syndrome has a discrete pathophysiology and differs genetically and clinically from recessive Wolfram syndrome. © 2017 by the American Diabetes Association.

  3. A gene responsible for profound congenital nonsyndromal recessive deafness maps to the pericentromeric region of chromosome 17

    Energy Technology Data Exchange (ETDEWEB)

    Friedman, T.B.; Liang, Y.; Asher, J.H. Jr. [and others

    1994-09-01

    Autosomal recessive deafness is the most common form of human hereditary hearing loss. Two percent of the 2,185 residents of Bengkala, Bali, Indonesia have profound congenital neurosensory nonsyndromal hereditary deafness due to a fully penetrant autosomal recessive mutation (NARD1). Families, identified through children with profound congenital deafness having hearing parents, give the expected 25% deaf progeny when corrected for ascertainment bias. Congenitally deaf individuals from Bengkala show no response to pure tone audiological examination. Obligate heterozygotes for autosomal recessive deafness in Bengkala have normal or borderline normal hearing. A chromosomal location for NARD1 was assigned directly using a linkage strategy that combines allele-frequency dependent homozygosity mapping (AHM) followed by an analysis of historical recombinants to position NARD1 relative to flanking markers. Thirteen deaf Bengkala villagers of hearing parents were typed initially for 148 STRPs distributed across the human genome and a cluster of tightly linked 17p markers with a significantly higher number of homozygotes than expected under Hardy-Weinberg and linkage equilibrium were identified. NARD1 maps closest to STRPs for D17S261 (Mfd41) and D17S805 (AFM234ta1) that are 3.2 cM apart. Recombinant genotypes for the flanking markers, D17S122 (VAW409) and D17S783 (AFM026vh7), in individuals homozygous for NARD1 place NARD1 in a 5.3 cM interval of the pericentromeric region of chromosome 17 on a refined 17p-17q12 genetic map.

  4. Cortical activation in profoundly deaf patients during cochlear implant stimulation demonstrated by H sub 2 (15)O PET

    Energy Technology Data Exchange (ETDEWEB)

    Herzog, H.; Lamprecht, A.; Kuehn, A.R.; Roden, W.; Vosteen, K.H.; Feinendegen, L.E. (Institute of Medicine, Juelich, (West Germany))

    1991-05-01

    Cochlear implants (CIs) are used to provide sensations of sound to profoundly deaf patients. The performance of the CI is assessed mainly by the subjective reports of patients. The aim of this study was to look for objective cortical responses to the stimulation of the CI. Two postlingually and two prelingually deaf patients were investigated by positron emission tomography (PET) using {sup 15}O-labeled water (H{sub 2}{sup 15}O) to determine the regional cerebral blood flow (rCBF). Instead of quantifying rCBF in absolute terms, it was estimated by referring the regional tissue concentration of H{sub 2}{sup 15}O to the mean whole brain concentration. CI stimulation encoded from white noise and sequential words led to an increased rCBF in the primary and secondary (Wernicke) auditory cortex. Relative elevations of up to 33% were observed bilaterally, although they were higher contralateral to the CI. These results were obtained not only in the postlingually deaf patients but also in two patients who had never been able to hear. Thus, it could be demonstrated that PET measurements of cerebral H{sub 2}{sup 15}O distribution yield objective responses of the central auditory system during electrical stimulation by CIs in profoundly deaf patients.

  5. Family influences on the cognitive development of profoundly deaf children: exploring the effects of socioeconomic status and siblings.

    Science.gov (United States)

    Macaulay, Catrin E; Ford, Ruth M

    2013-10-01

    We evaluated the cognitive development of 48 profoundly deaf children from hearing families (born 1994-2002, mean age M = 8.0 years at time of test, none of whom had received early auditory-verbal therapy) as a function of family socioeconomic status and number of siblings. Overall, the deaf children matched a younger group of 47 hearing controls (M = 4.6 years) on verbal ability, theory of mind, and cognitive inhibition. Partial correlations (controlling for age) revealed positive relations in the hearing group between maternal education and inhibition, between number of younger siblings and references to emotions, and between number of close-in-age siblings and references to desires and false beliefs. In the deaf group, there were positive relations between household income and memory span, between maternal education and references to false beliefs, and between number of younger siblings and nonverbal ability. In contrast, deaf children with a greater number of older siblings aged ≤12 years showed inferior memory span, inhibition, belief understanding, picture-sequencing accuracy, and mental-state language, suggesting that they failed to compete successfully with older siblings for their parents' attention and material resources. We consider the implications of the findings for understanding birth-order effects on deaf and language-impaired children.

  6. Consequences of a screening programme on the prevalence of congenital hereditary sensorineural deafness in the Australian Cattle Dog.

    Science.gov (United States)

    Sommerlad, S F; Morton, J M; Johnstone, I; O'Leary, C A; Seddon, J M

    2014-12-01

    Genetic disease testing programmes are used in domestic animal breeds to guide selective breeding with the aim of reducing disease prevalence. We assessed the change in the prevalence of canine congenital hereditary sensorineural deafness (CHSD) in litters of Australian Cattle Dogs following the introduction of a brainstem auditory evoked response (BAER) testing programme. We studied 608 pups from 122 litters from 10 breeding kennels. Despite 10 years of testing (1998-2008), no substantial reduction in prevalence of CHSD was evident in these 10 breeding kennels. Even for the subset of litters in which both parents were BAER tested as normal hearing (305 pups from 58 litters), there was no evidence of substantial reduction in prevalence. Odds ratios for CHSD in pups for each extra year since testing in the kennel commenced were 1.01 (95% CI, 0.88-1.17) and 1.03 (95% CI, 0.82-1.30) respectively for these populations. Amongst 284 dogs from 54 litters with extended pedigrees and both parents BAER-tested normal hearing, observed prevalences of CHSD were highest in pups with no BAER-tested normal grandparents (17% or 5/29) and lowest in pups with all four grandparents tested normal (0% or 0/9). In pups for which one, two and three grandparents tested negative, prevalences of CHSD were 12% (9/74), 9% (9/101) and 8% (6/71) respectively. Hence, testing programmes based on phenotypic screening may not lead to a substantial reduction in recessive genetic disease prevalence over the medium term, even when only tested normal parents are used. Exclusive breeding of litters in which both parents and all four grandparents are BAER-tested normal is expected to reduce CHSD prevalence in pups to the greatest extent over the long term. © 2014 Stichting International Foundation for Animal Genetics.

  7. Very good performance with bimodal stimulation in a like-hybrid modality in a patient with profound bilateral sensorineural hearing loss with low-frequencies preservation.

    Science.gov (United States)

    Laria, Carla; Auletta, Gennaro; Riccardi, Pasquale; Papa, Carmine; Malesci, Rita; Franzé, Annamaria; Marciano, Elio

    2014-01-01

    In this work we describe the experimental protocol set up to obtain very good results in speech performance and in time course, with a subject presenting profound bilateral sensorineural hearing loss with low-frequencies preservation. We used a bimodal stimulation with a like-hybrid modality. Auditory functions have been analyzed by standard tonal and speech audiometry tests, and verbal perception test. The fitting protocol permitted the subject to reach a perception at 65 dB of 100% in a very short time. The subject showed a sufficient recovery of the language spectral information and a good integration of verbal information with high consonantal recognition is present. This case report shows the importance to realize a correct cochlear implant fitting and that, in the case of bimodal stimulation, it is very important to obtain the mutual adjustment of the two hearing aids. Moreover, this study enhances the importance of realizing a preservative surgery to make the most of cochlear implants capacity. © 2013.

  8. Surgery to improve hearing of a preschool child with profound bilateral deafness

    OpenAIRE

    井口,郁雄; 増田,游; 高塚,ま由; 小倉, 義郎; 藤本, 明子

    1987-01-01

    Hearing loss in children under school age adversely effects speech and personality development. It is possible to improve conductive hearing loss by surgery, but difficult to improve combined hearing loss. The authors succeeded surgically improving the hearing of a 5 year-old boy suffering from speech retardation due to bilateral congenital combined hearing loss. The improvement in hearing aided speech training. He has graduated from schools for the deaf (primary, middle and senior high schoo...

  9. Comparison of speech discrimination in noise and directional hearing with 2 different sound processors of a bone-anchored hearing system in adults with unilateral severe or profound sensorineural hearing loss.

    Science.gov (United States)

    Wesarg, Thomas; Aschendorff, Antje; Laszig, Roland; Beck, Rainer; Schild, Christian; Hassepass, Frederike; Kroeger, Stefanie; Hocke, Thomas; Arndt, Susan

    2013-08-01

    To evaluate and compare the benefit of a bone-anchored hearing implant with 2 different sound processors in adult patients with unilateral severe to profound sensorineural hearing loss (UHL). Prospective crossover design. Tertiary referral center. Eleven adults with UHL and normal hearing in the contralateral ear were assigned to 2 groups. All subjects were unilaterally implanted with a bone-anchored hearing implant and were initially fitted with 2 different sound processors (SP-1 and SP-2). SP-1 is a multichannel device equipped with an omnidirectional microphone and relatively simple digital signal-processing technology and provides a user-adjustable overall gain and tone control with compression limiting. SP-2 is a fully channel-by-channel programmable device, which can be set with nonlinear dynamic range compression or linear amplification. In addition, SP-2 features automatic noise management, an automatic multichannel directional microphone, microphone position compensation, and an implementation of prescription rules for different types of hearing losses, one of them unilateral deafness. After at least 1-month use of the initial processor, both groups were fitted with the alternative processor. Speech discrimination in noise and localization tests were performed at baseline visit before surgery, after at least 1-month use of the initial processor, and after at least 2-week use of the alternative processor. Relative to unaided baseline, SP-2 enabled significantly better overall speech discrimination results, whereas there was no overall improvement with SP-1. There was no difference in speech discrimination between SP-1 and SP-2 in all spatial settings. Sound localization was comparably poor at baseline and with both processors but significantly better than chance level for all 3 conditions. Patients with UHL have an overall objective benefit for speech discrimination in noise using a bone-anchored hearing implant with SP-2. In contrast, there is no overall

  10. Smartphone speech-to-text applications for communication with profoundly deaf patients.

    Science.gov (United States)

    Lyall, F C; Clamp, P J; Hajioff, D

    2016-01-01

    Visual communication aids, such as handwriting or typing, are often used to communicate with deaf patients in the clinic. This study aimed to establish the feasibility of communicating through smartphone speech recognition software compared with writing or typing. Thirty doctors and medical students were timed writing, typing and dictating a standard set of six sentences appropriate for a post-operative consultation, and the results were assessed for accuracy and legibility. The mean time for smartphone dictation (17.8 seconds, 95 per cent confidence interval = 17.0-18.7) was significantly faster than writing (59.2 seconds, 95 per cent confidence interval = 56.6-61.7) or typing (44 seconds, 95 per cent confidence interval = 41.0-47.1) (p < 0.001). Speech recognition was slightly less accurate, but accuracy increased with time spent dictating. Smartphone dictation is a feasible alternative to typing and handwriting. Slow speech may improve accuracy. Early clinical experience has been promising.

  11. The effectiveness of bilateral cochlear implants for severe-to-profound deafness in adults: a systematic review.

    Science.gov (United States)

    van Schoonhoven, Jelmer; Sparreboom, Marloes; van Zanten, Bert G A; Scholten, Rob J P M; Mylanus, Emmanuel A M; Dreschler, Wouter A; Grolman, Wilko; Maat, Bert

    2013-02-01

    Assessment of the clinical effectiveness of bilateral cochlear implantation compared with unilateral cochlear implantation or bimodal stimulation, in adults with severe-to-profound hearing loss. In 2007, the National Institute for Health and Clinical Excellence (NICE) in the U.K. conducted a systematic review on cochlear implantation. This study forms an update of the adult part of the NICE review. The electronic databases MEDLINE and Embase were searched for English language studies published between October 2006 and March 2011. Studies were included that compared bilateral cochlear implantation with unilateral cochlear implantation and/or with bimodal stimulation, in adults with severe-to-profound sensorineural hearing loss. Speech perception in quiet and in noise, sound localization and lateralization, speech production, health-related quality of life, and functional outcomes were analyzed. Data extraction forms were used to describe study characteristics and the level of evidence. The effect size was calculated to compare different outcome measures. Pooling of data was not possible because of the heterogeneity of the studies. As in the NICE review, the level of evidence of the included studies was low, although some of the additional studies showed less risk of bias. All studies showed a significant bilateral benefit in localization over unilateral cochlear implantation. Bilateral cochlear implants were beneficial for speech perception in noise under certain conditions and several self-reported measures. Most speech perception in quiet outcomes did not show a bilateral benefit. The current review provides additional evidence in favor of bilateral cochlear implantation, even in complex listening situations.

  12. Rehabilitation and outcome of severe profound deafness in a group of 16 infants affected by congenital cytomegalovirus infection.

    Science.gov (United States)

    Ciorba, Andrea; Bovo, Roberto; Trevisi, Patrizia; Bianchini, Chiara; Arboretti, Rosa; Martini, Alessandro

    2009-10-01

    The aim of the study was to characterize the audiological consequences of congenital cytomegalovirus infection (CMV) and to evaluate the outcome of rehabilitation with hearing aids and/or cochlear implant (CI), associated with an adequate speech-language therapy. A retrospective review of data was made from a total of 16 infants, affected by severe to profound hearing loss from congenital CMV infection, referred to a tertiary audiological center for rehabilitation. Audiological evaluation was performed using behavioral audiometry, auditory brainstem responses (ABR) and/or electrocochleography (ECochG). Of the 16 children (median age at diagnosis of hearing loss: 21.33 +/- 0.7 months) with CMV hearing loss, 14 were affected by profound bilateral hearing loss and received a CI, while 2 were affected by bilateral severe hearing loss and received hearing aids. Cochlear implants can provide useful speech comprehension to patients with CMV-related deafness, even if language development is lower when compared to a group of Connexin (Cx) 26+ cochlear-implanted children (eight subjects), matched for age. Congenital CMV infection still represents a serious clinical condition, as well as an important cause of hearing loss in children. More studies have claimed to identify the pathophysiological mechanisms of damage and thus to ensure a better therapeutic approach. Nonetheless, in cases of CMV-deafened babies, the overall outcome of cochlear implantation is good.

  13. Ahsa1 and Hsp90 activity confers more severe craniofacial phenotypes in a zebrafish model of hypoparathyroidism, sensorineural deafness and renal dysplasia (HDR

    Directory of Open Access Journals (Sweden)

    Kelly Sheehan-Rooney

    2013-09-01

    The severity of most human birth defects is highly variable. Our ability to diagnose, treat and prevent defects relies on our understanding of this variability. Mutation of the transcription factor GATA3 in humans causes the highly variable hypoparathyroidism, sensorineural deafness and renal dysplasia (HDR syndrome. Although named for a triad of defects, individuals with HDR can also exhibit craniofacial defects. Through a forward genetic screen for craniofacial mutants, we isolated a zebrafish mutant in which the first cysteine of the second zinc finger of Gata3 is mutated. Because mutation of the homologous cysteine causes HDR in humans, these zebrafish mutants could be a quick and effective animal model for understanding the role of gata3 in the HDR disease spectrum. We demonstrate that, unexpectedly, the chaperone proteins Ahsa1 and Hsp90 promote severe craniofacial phenotypes in our zebrafish model of HDR syndrome. The strengths of the zebrafish system, including rapid development, genetic tractability and live imaging, make this an important model for variability.

  14. A comparative historical and demographic study of the neuromodulation management techniques of deep brain stimulation for dystonia and cochlear implantation for sensorineural deafness in children.

    Science.gov (United States)

    Hudson, V E; Elniel, A; Ughratdar, I; Zebian, B; Selway, R; Lin, J P

    2017-01-01

    Cochlear implants for sensorineural deafness in children is one of the most successful neuromodulation techniques known to relieve early chronic neurodisability, improving activity and participation. In 2012 there were 324,000 recipients of cochlear implants globally. To compare cochlear implant (CI) neuromodulation with deep brain stimulation (DBS) for dystonia in childhood and explore relations between age and duration of symptoms at implantation and outcome. Comparison of published annual UK CI figures for 1985-2009 with a retrospective cohort of the first 9 years of DBS for dystonia in children at a single-site Functional Neurosurgery unit from 2006 to 14. From 2006 to 14, DBS neuromodulation of childhood dystonia increased by a factor of 3.8 to a total of 126 cases over the first 9 years, similar to the growth in cochlear implants which increased by a factor of 4.1 over a similar period in the 1980s rising to 527 children in 2009. The CI saw a dramatic shift in practice from implantation at >5 years of age at the start of the programme towards earlier implantation by the mid-1990s. Best language results were seen for implantation 4 years, hence implantation deaf, pre-lingual children could benefit from cochlear neuromodulation if implanted early. Similar to initial CI use, the majority of children receiving DBS for dystonia in the first 9 years were 5-15 years of age, when the proportion of life lived with dystonia exceeds 90% thus limiting benefits. Early DBS neuromodulation for acquired motor disorders should be explored to maximise the benefits of dystonia reduction in a period of maximal developmental plasticity before the onset of disability. Learning from cochlear implantation, DBS can become an accepted management option in children under the age of 5 years who have a reduced proportion of life lived with dystonia, and not viewed as a last resort reserved for only the most severe cases where benefits may be at their most limited. Copyright © 2016

  15. Clinical need for a Baha trial in patients with single-sided sensorineural deafness. Analysis of a Baha database of 196 patients.

    Science.gov (United States)

    Desmet, Jolien; Bouzegta, Rajae; Hofkens, Anouk; De Backer, Annemarie; Lambrechts, Peggy; Wouters, Kristien; Claes, Jozef; De Bodt, Marc; Van de Heyning, Paul

    2012-03-01

    A bone-anchored hearing aid (Baha) is used in patients with single-sided sensorineural deafness (SSD) to overcome the head shadow effect. Of all the patients with SSD, treated at our hospital, 196 patients used a Baha on trial between November 2001 and April 2010. The objective of this study is to evaluate what factors determine the decision of a SSD patient whether or not to opt for a Baha device following a Baha trial period. 196 patients with SSD were enrolled for a trial period of 2 weeks at the Antwerp University Hospital, a tertiary referral centre. 93% of these patients suffered from an acquired hearing loss. 44% of all the patients (87/196) chose to wear a Baha device after the trail period, either on an abutment or on a headband. The collected data were analysed to determine correlations between the decision of a patient following a Baha trial period on the one hand, and Fletcher Index ipsi- and contralaterally, bone conduction hearing thresholds at the better hearing ear, aetiology, age at the start of the trial period, duration of hearing loss at the start of the trial period and the type of device used during the trial period, on the other hand. Although 66% of all the patients (109/196) declined the Baha after a trial, reasons not to choose a Baha were diverse and no crucial factors could be found that determine the success of a Baha trial period. Lack of improvement concerning speech understanding in noise was the most important reason mentioned by patients who declined the Baha. The authors advocate that all patients, suffering from SSD, should be offered the opportunity to try a Baha device as no factors could be found that determine the decision of a patient following the trial period.

  16. Rare cause of bilateral sudden deafness.

    Science.gov (United States)

    Vos, F I; Merkus, P; van Nieuwkerk, E B J; Hensen, E F

    2016-10-08

    In this paper, we describe the case of a 62-year-old female with recurring episodes of sudden deafness with vertigo and facial paresis. Within a month's time, this resulted in bilateral deafness and vestibular areflexia. Erroneously, the patient was diagnosed with sudden deafness of unknown origin and subsequently with neuroborreliosis (Lyme disease). The true diagnosis of relapsing polychondritis (RP) was revealed 9 months after initial presentation. The diagnostic delay is in part explained by the fact that, by definition, the disease has to relapse before the diagnosis can be made, but also by its pluriform clinical presentation. Timely identification of RP as the cause of this profound sensorineural hearing loss proved to be important. It was key in instigating adequate follow-up, and allowed for cochlear implantation before total cochlear obliteration, which might have hampered optimal hearing rehabilitation. 2016 BMJ Publishing Group Ltd.

  17. Speech reading and learning to read: a comparison of 8-year-old profoundly deaf children with good and poor reading ability.

    Science.gov (United States)

    Harris, Margaret; Moreno, Constanza

    2006-01-01

    Nine children with severe-profound prelingual hearing loss and single-word reading scores not more than 10 months behind chronological age (Good Readers) were matched with 9 children whose reading lag was at least 15 months (Poor Readers). Good Readers had significantly higher spelling and reading comprehension scores. They produced significantly more phonetic errors (indicating the use of phonological coding) and more often correctly represented the number of syllables in spelling than Poor Readers. They also scored more highly on orthographic awareness and were better at speech reading. Speech intelligibility was the same in the two groups. Cluster analysis revealed that only three Good Readers showed strong evidence of phonetic coding in spelling although seven had good representation of syllables; only four had high orthographic awareness scores. However, all 9 children were good speech readers, suggesting that a phonological code derived through speech reading may underpin reading success for deaf children.

  18. Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness.

    Science.gov (United States)

    Kim, Sang Hoon; Jung, A Ra; Kim, Su Il; Yeo, Seung Geun

    2016-04-01

    Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

  19. Cortical perfusion response to an electrical stimulation of the auditory nerve in profoundly deaf patients: Study with technetium-99m hexamethylpropylene amine oxime single photon emission tomography

    Energy Technology Data Exchange (ETDEWEB)

    Le Scao, Y.; Robier, A.; Beuter, P. (Centre Hospitalier Universitaire, 37 - Tours (France). Dept. of Otorhinolaryngology); Baulieu, J.L.; Pourcelot, L. (Centre Hospitalier Universitaire, 37 - Tours (France). Dept. of Nuclear Medicine)

    1992-04-01

    Brain activation procedures associated with single photon emission tomography (SPET) have recently been developed in healthy controls and diseased patients in order to help in their diagnosis and treatment. We investigated the effects of a promontory test (PT) on the cerebral distribution of technetium-99m hexamethyl-propylene amine oxime ({sup 99m}Tc-HMPAO) in 7 profoundly deaf patients, 6 PT+ and PT-. The count variation in the temporal lobe was calculated on 6 coronal slices using the ratio (R{sub stimulation}-R{sub deprivation})/R{sub deprivation} where R=counts in the temporal lobe was observed in all patients and was higher in all patients with PT+ than in the patient with PT-. The problems of head positioning and resolution of the system were taken into account, and we considered that the maximal count increment was related to the auditory cortex response to the stimulus. Further clinical investigations with high-resolution systems have to be performed in order to validate this presurgery test in cochlear implant assessment. (orig.).

  20. Prospective mutation screening of three common deafness genes in a large Taiwanese Cohort with idiopathic bilateral sensorineural hearing impairment reveals a difference in the results between families from hospitals and those from rehabilitation facilities.

    Science.gov (United States)

    Wu, Chen-Chi; Chen, Pei-Jer; Chiu, Yu-Hsun; Lu, Ying-Chang; Wu, Ming-Chueh; Hsu, Chuan-Jen

    2008-01-01

    Accurate epidemiological data on common deafness genes are essential to improve the efficiency and to reduce the cost of molecular diagnosis. They may depend on several factors, including a clear delineation of the source of patients being studied. In the present study, we hypothesize that patients with idiopathic sensorineural hearing loss recruited from different sources might reveal discrepancies in the epidemiological results of genetic screening, because patients from different sources might demonstrate distinct clinical or audiologic features and thus result in biased selection of subjects. To elucidate the relative importance of common deafness genes in Taiwanese and to verify our hypothesis, we conducted a prospective project screening mutations in GJB2, SLC26A4 and mitochondrial 12S rRNA gene in a total of 420 Taiwanese families with idiopathic bilateral sensorineural hearing loss, of which 325 families were recruited from hospitals and 95 from hearing rehabilitation facilities. Allele frequencies of common mutations in these three genes and distributions of the corresponding genotypes were then compared between the two groups. The allele frequencies of mutations in SLC26A4, GJB2 and mitochondrial 12S rRNA in the probands of the 420 families were 14.4, 21.7 and 3.8%, respectively. The allele frequency of SLC26A4 mutations in the hospital group was significantly higher than that in the rehabilitation facility group (16.2 vs. 8.4%, chi(2)-test, p < 0.05), whereas no difference in the frequencies of GJB2 mutations and mitochondrial 12S rRNA mutations was found between the two groups. Distributions of probands classified by SLC26A4 genotypes were also different between the two groups (chi(2)-test, p < 0.05). Accordingly, a discrepancy in the genetic screening results might exist between different sources of idiopathic hearing-impaired patients. Further analysis of audiological results and construction of a logistic regression model showed that different

  1. Localization to Xq22 and clinical update of a family with X-linked recessive mental retardation with progression sensorineural deafness, progressive tapeto-retinal degeneration and dystonia

    Energy Technology Data Exchange (ETDEWEB)

    Tranebjaerg, L.; Schwartz, C.; Huggins, K.; Barker, D.; Stevenson, R.; Arena, J.F.; Gedde-Dahl, T.; Mikkelsen, M.; Mellgren, S.; Anderson, K. [Greenwood Genetic Center, SC (United States)]|[Univ. of Utah, Salt Lake City, UT (United States)]|[Univ. of Miami, FL (United States)]|[University Hospital, Oslo (Norway)]|[University Hospital, S-Lund (Sweden)

    1994-07-15

    In a reinvestigation of a six-generation Norwegian family, originally reported with non-syndromic X-linked recessive deafness by Mohr and Mageroy, we have demonstrated several syndromic manifestations. The 10 clinically characterized affected males range in age from 14-61 years, and show progressive mental deterioration and visual disability. Ophthalmological and electrophysiological studies showed myopia, decreased visual acuity, combined cone-rod dystrophy as well as central areolar dystrophy by means of ERG. Brain CT-scans showed cortical and central atrophy without predilection to specific areas. Linkage analysis, using X-chromosomal RFLPs and CA-repeats, yielded a maximum LOD score of 4.37 with linkage to DXS17. DXS17 is localized to Xq22. One recombinant with COL4A5 (deficient in Alport syndrome) was observed. Results from the studies of this family will be important in reclassification of non-syndromic X-linked deafness since the family now represents syndromic deafness and XLMR with a specific phenotype.

  2. Infantile mitochondrial hepatopathy is a cardinal feature of MEGDEL syndrome (3-methylglutaconic aciduria type IV with sensorineural deafness, encephalopathy and Leigh-like syndrome) caused by novel mutations in SERAC1.

    Science.gov (United States)

    Sarig, Ofer; Goldsher, Dorit; Nousbeck, Janna; Fuchs-Telem, Dana; Cohen-Katsenelson, Ksenya; Iancu, Theodore C; Manov, Irena; Saada, Ann; Sprecher, Eli; Mandel, Hanna

    2013-09-01

    3-Methylglutaconic aciduria (3-MGCA) type IV is defined as a heterogeneous group of inborn errors featuring in common 3-MGCA and associated with primary mitochondrial dysfunction leading to a spectrum of multisystem conditions. We studied four patients who presented at birth with a clinical picture simulating a primary mitochondrial hepatic disorder consistent with the MEGDEL syndrome including 3-MGCA, sensorineural deafness, encephalopathy and a brain magnetic resonance imaging with signs of Leigh disease. All affected children displayed biochemical features consistent with mitochondrial OXPHOS dysfunction including hepatic mitochondrial DNA depletion in one patient. Homozygosity mapping identified a candidate locus on 6q25.2-6q26. Using whole exome sequencing, we identified two novel homozygous mutations in SERAC1 recently reported to harbor mutations in MEGDEL syndrome. Both mutations were found to lead to decreased or absent expression of SERAC1. The present findings indicate that infantile hepatopathy is a cardinal feature of MEGDEL syndrome. We thus propose to rename the disease MEGDHEL syndrome. Copyright © 2013 Wiley Periodicals, Inc.

  3. Causes of childhood deafness in Pukhtoonkhwa Province of Pakistan and the role of consanguinity.

    Science.gov (United States)

    Sajjad, M; Khattak, A A; Bunn, J E G; Mackenzie, I

    2008-10-01

    Deafness is the hidden disability of childhood, and leads to poor educational and employment prospects. There is little published information on deafness in Pakistan. Profound hearing impairment is more prevalent in countries where consanguineous marriages are common, such as Pakistan. This study aimed to assess causes of childhood deafness and association with parental consanguinity, within deaf and hearing children in the Peshawar district of Pukhtoonkhwa Province, Pakistan. One hundred and forty deaf children were identified from two schools for deaf children within the Peshawar district. These children were assessed via audiology, otoscopic examination, case note review and parental history, in order to attempt to ascertain the cause of their deafness. Two hundred and twenty-one attendees at a local immunisation clinic (taken as representative of the local childhood population) were also screened for hearing impairment. Parents of both groups of children were assessed by interview and questionnaire in order to ascertain the mother and father's family relationship (i.e. whether cousins or unrelated). Of the 140 deaf school pupils, 92.1 per cent were profoundly hearing impaired and 7.9 per cent were severely hearing impaired. All these children had bilateral sensorineural hearing loss. A possible cause of deafness was identified in only six of these children. Parental consanguinity (i.e. first or second cousins) was established for 86.4 per cent of deaf school pupils and 59.7 per cent of immunisation clinic attendees. None of the control children were identified as having a hearing problem. The prevalence of parental consanguinity was significantly higher in deaf children compared with non-hearing impaired children. However, the study also confirmed a high rate of consanguinity within the general Peshawar community. In this setting, prevention of consanguineous unions is the only means of reducing levels of congenital hearing impairment. The current levels of

  4. Postural Control in Deaf Children

    Directory of Open Access Journals (Sweden)

    Amir-Abbas Ebrahimi

    2017-02-01

    Full Text Available This cross-sectional study aimed to determine the reliability of static control evaluation with Synapsys Posturography System (SPS, Marseille, France and to compare the static postural control of deaf children with typically developing children. This study was conducted in 2 phases on 81 children of 7 to 12 years old in Tehran schools. The first phase examined the reliability of static balance evaluation with SPS. In this phase, a total of 12 children with typical development were evaluated and then do a re-test 1 week later. In the second phase, 30 children with profound sensorineural hearing loss (SNHL and high risk in their balance (selected from Baghcheban Schools for the Deaf as the experimental group, and 37 children with typical development (selected randomly from 2 primary schools for girls and boys in District 12 of Tehran Department of Education as control group were enrolled in the study. They were all placed under sensory organization test evaluation. Based on the results of intraclass correlation coefficient (ICC, the unilateral random effects model, test-retest reliability in different sensory conditions, the moderate to excellent results were obtained (ICC between 0.68 and 0.94. Also, the mean displacement of pressure center in all sensory conditions, the limits of stability (LOS area, the overall balance scores, and scores for balance sensory ratio (except the somatosensory ratio of children with typical development were better than the deaf peers (P˂0.05. The SPS has acceptable reliability to evaluate static posture in children between the ages of 7 and 12 years. Furthermore, deaf children as compared to children with typical development had a lower static postural control in all sensory conditions. This finding confirms the need to examine the postural control for identifying the extent of sensory deficit that has caused poor balance function, and also the need for early intervention to address the balance deficit in deaf

  5. Gd enhanced MRI in sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Takenaka, Mika; Tono, Tetsuya; Toyama, Katsuhiro; Kano, Kiyo; Morimitsu, Tamotsu [Miyazaki Medical Coll., Kiyotake (Japan)

    1996-09-01

    The enhanced MRI hearing findings of the inner ear in 124 patients with sensorineural hearing loss were evaluated. MR images were obtained before and after the intravenous administration of gadolinium (0.1 mmol/kg). In three out of seventy-nine patients with unilateral healing loss, cochlear and/or the vestibular enhancement was noted on the symptomatic side. The positive cases included those with Ramsay-Hunt syndrome, mumps and so-called sudden deafness. Forty-five patients with bilateral hearing loss showed no enhancement within the inner ear. Although positive gadolinium enhancement of the inner ear may detect inflammatory lesions due to a viral infection, its incidence in sensorineural hearing loss, including cases of sudden deafness. seems to be extremely rare. (author)

  6. A new nonsyndromic X-linked sensorineural hearing impairment linked to Xp21.2

    Energy Technology Data Exchange (ETDEWEB)

    Lalwani, A.K.; Brister, J.R.; Fex, J.; Grundfast, K.M.; Pikus, A.T.; Ploplis, B.; San Agustin, T.; Skarka, H.; Wilcox, E.R. [National Institutes of Health, Bethesda, MD (United States)

    1994-10-01

    X-linked deafness is a rare cause of hereditary hearing impairment. We have identified a family with X-linked dominant sensorineural hearing impairment, characterized by incomplete penetrance and variable expressivity in carrier females, that is linked to the Xp21.2, which contains the Duchenne muscular dystrophy (DMD) locus. The auditory impairment in affected males was congenital, bilateral, profound, sensorineural, affecting all frequencies, and without evidence of radiographic abnormality of the temporal bone. Adult carrier females manifested bilateral, mild-to-moderate high-frequency sensorineural hearing impairment of delayed onset during adulthood. Eighteen commercially available polymorphic markers from the X chromosome, generating a 10-15-cM map, were initially used for identification of a candidate region. DXS997, located within the DMD gene, generated a two-point LOD score of 2.91 at {theta} = 0, with every carrier mother heterozygous at this locus. Recombination events at DXS992 (located within the DMD locus, 3{prime} to exon 50 of the dystrophin gene) and at DXS1068 (5{prime} to the brain promoter of the dystrophin gene) were observed. No recombination events were noted with the following markers within the DMD locus: 5{prime}DYS II, intron 44, DXS997, and intron 50. There was no clinical evidence of Duchenne or Becker muscular dystrophy in any family member. It is likely that this family represents a new locus on the X chromosome, which when mutated results in nonsyndromic sensorineural hearing loss and is distinct from the heterogeneous group of X-linked hearing losses that have been previously described. 57 refs., 6 figs., 1 tab.

  7. A new nonsyndromic X-linked sensorineural hearing impairment linked to Xp21.2.

    Science.gov (United States)

    Lalwani, A. K.; Brister, J. R.; Fex, J.; Grundfast, K. M.; Pikus, A. T.; Ploplis, B.; San Agustin, T.; Skarka, H.; Wilcox, E. R.

    1994-01-01

    X-linked deafness is a rare cause of hereditary hearing impairment. We have identified a family with X-linked dominant sensorineural hearing impairment, characterized by incomplete penetrance and variable expressivity in carrier females, that is linked to the Xp21.2, which contains the Duchenne muscular dystrophy (DMD) locus. The auditory impairment in affected males was congenital, bilateral, profound, sensorineural, affecting all frequencies, and without evidence of radiographic abnormality of the temporal bone. Adult carrier females manifested bilateral, mild-to-moderate high-frequency sensorineural hearing impairment of delayed onset during adulthood. Eighteen commercially available, polymorphic markers from the X chromosome, generating a 10-15-cM map, were initially used for identification of a candidate region. DXS997, located within the DMD gene, generated a two-point LOD score of 2.91 at theta = 0, with every carrier mother heterozygous at this locus. Recombination events at DXS992 (located within the DMD locus, 3' to exon 50 of the dystrophin gene) and at DXS1068 (5' to the brain promoter of the dystrophin gene) were observed. No recombination events were noted with the following markers within the DMD locus: 5'DYS II, intron 44, DXS997, and intron 50. There was no clinical evidence of Duchenne or Becker muscular dystrophy in any family member. It is likely that this family represents a new locus on the X chromosome, which when mutated results in nonsyndromic sensorineural hearing loss and is distinct from the heterogeneous group of X-linked hearing losses that have been previously described. PMID:7942846

  8. Identification of acoustically similar and dissimilar vowels in profoundly deaf adults who use hearing aids and/or cochlear implants: some preliminary findings.

    Science.gov (United States)

    Hay-McCutcheon, Marcia J; Peterson, Nathaniel R; Rosado, Christian A; Pisoni, David B

    2014-03-01

    In this study, the authors examined the effects of aging and residual hearing on the identification of acoustically similar and dissimilar vowels in adults with postlingual deafness who use hearing aids (HAs) and/or cochlear implants (CIs). The authors used two groups of acoustically similar and dissimilar vowels to assess vowel identification. Also, the Consonant-Nucleus-Consonant Word Recognition Test (Peterson & Lehiste, 1962) and sentences from the Hearing in Noise Test (Nilsson, Soli, & Sullivan, 1994) were administered. Forty CI recipients with postlingual deafness (ages 31-81 years) participated in the study. Acoustically similar vowels were more difficult to identify than acoustically dissimilar vowels. With increasing age, performance deteriorated when identifying acoustically similar vowels. Vowel identification was also affected by the use of a contralateral HA and the degree of residual hearing prior to implantation. Moderate correlations were found between speech perception and vowel identification performance. Identification performance was affected by the acoustic similarity of the vowels. Older adults experienced more difficulty identifying acoustically similar confusable vowels than did younger adults. The findings might lend support to the ease of language understanding model (Ronnberg, Rudner, Foo, & Lunner, 2008), which proposes that the quality and perceptual robustness of acoustic input affects speech perception.

  9. Sensorineural hearing loss in children.

    LENUS (Irish Health Repository)

    Wormald, R

    2010-02-01

    The objective of the study was to examine the aetiology of sensorineural hearing loss (SNHL) in a paediatric population presenting to the National Centre of Medical Genetics. A retrospective chart review from 1998 to 2006. One hundred and twenty nine children were investigated for SNHL. The average age of diagnosis of hearing loss was 36 months. The degree of hearing loss was mild in 8 children, moderate in 33 children, severe in 31 children and profound in 57 children. Eighty-five children (66%) were diagnosed with a hereditary hearing loss, 11 (8%) children had an acquired hearing loss and no cause found in 33 (26%) children. This is the first report of the causes of hearing loss in Irish children. The mean age of diagnosis in our cohort is high and emphasises the need for a neonatal screening programme. There remains a number of children for whom the cause of hearing loss remains unknown.

  10. Congenital sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.

  11. GJB2 mutations in Mongolia: complex alleles, low frequency, and reduced fitness of the deaf.

    Science.gov (United States)

    Tekin, Mustafa; Xia, Xia-Juan; Erdenetungalag, Radnaabazar; Cengiz, Filiz Basak; White, Thomas W; Radnaabazar, Janchiv; Dangaasuren, Begzsuren; Tastan, Hakki; Nance, Walter E; Pandya, Arti

    2010-03-01

    We screened the GJB2 gene for mutations in 534 (108 multiplex and 426 simplex) probands with non-syndromic sensorineural deafness, who were ascertained through the only residential school for the deaf in Mongolia, and in 217 hearing controls. Twenty different alleles, including four novel changes, were identified. Biallelic GJB2 mutations were found in 4.5% of the deaf probands (8.3% in multiplex, 3.5% in simplex). The most common mutations were c.IVS1 + 1G > A (c.-3201G > A) and c.235delC with allele frequencies of 3.5% and 1.5%, respectively. The c.IVS1 + 1G > A mutation appears to have diverse origins based on associated multiple haplotypes. The p.V27I and p.E114G variants were frequently detected in both deaf probands and hearing controls. The p.E114G variant was always in cis with the p.V27I variant. Although in vitro experiments using Xenopus oocytes have suggested that p.[V27I;E114G] disturbs the gap junction function of Cx26, the equal distribution of this complex allele in both deaf probands and hearing controls makes it a less likely cause of profound congenital deafness. We found a lower frequency of assortative mating (37.5%) and decreased genetic fitness (62%) of the deaf in Mongolia as compared to the western populations, which provides an explanation for lower frequency of GJB2 deafness in Mongolia.

  12. Sensori-neural hearing loss following radiotherapy to the nasopharynx

    Energy Technology Data Exchange (ETDEWEB)

    Moretti, J.A.

    1976-04-01

    A retrospective study was done to ascertain the risks of cochlear damage from radiotherapy of the nasopharynx. Audiometric evaluation, pre- and post-radiotherapy, revealed that 7 out of 13 patients had sustained sensori-neural deafness. Contrary to what is generally believed of the resistance of the cochlea to radiotherapeutic damage, eventual loss of hearing can occasionally be expected in patients undergoing radiation therapy for head and neck tumors.

  13. Visual stimuli and written production of deaf signers.

    Science.gov (United States)

    Jacinto, Laís Alves; Ribeiro, Karen Barros; Soares, Aparecido José Couto; Cárnio, Maria Silvia

    2012-01-01

    To verify the interference of visual stimuli in written production of deaf signers with no complaints regarding reading and writing. The research group consisted of 12 students with education between the 4th and 5th grade of elementary school, with severe or profound sensorineural hearing loss, users of LIBRAS and with alphabetical writing level. The evaluation was performed with pictures in a logical sequence and an action picture. The analysis used the communicative competence criteria. There were no differences in the writing production of the subjects for both stimuli. In all texts there was no title and punctuation, verbs were in the infinitive mode, there was lack of cohesive links and inclusion of created words. The different visual stimuli did not affect the production of texts.

  14. KRITERIA DIAGNOSIS DAN DIAGNOSIS BANDING SUDDEN DEAFNESS (SSNHL

    Directory of Open Access Journals (Sweden)

    Arvindan Subramaniam

    2016-03-01

    Full Text Available Introduction: Sudden deafness or sudden sensorineural hearing loss (SSNHL is defined as sensorineural hearing loss of more than 30 dB at three consecutive frequencies within 3 days of onset, often unilateral and idiopathic. Etiology of sudden deafness is still not known, but there are many theories put forward by the experts as a risk factor for sudden deafness. The prevalence of sudden deafness 5-30 per 100,000 people per year. Distribution of men and women almost equally, with the peak age of 50-60 years. Sudden deafness diagnosis is made based on history, physical examination and audiometry. Sudden deafness has three characteristics; acute, sensorineural hearing loss and unknown etiology. Additional characteristics may include vertigo, tinnitus and the absence of cranial nerve involvement. Management of sudden deafness include conservative therapy with multiple modalities. Case: Patient male, 40 years, Bali, Hindu present with hearing loss since ± 2 weeks ago. Patients previously complained of heat in the ear ± 2 days ago accompanied by a downward hearing and ears. A history of vomiting, coughs and colds denied. History of treatment at the hospital and was hospitalized for ± 2 weeks. Patients had never suffered from the same disease. No history of sinusitis, allergy, anemia, autoimmune and other systemic diseases. Patients also had never experienced trauma and underwent nasal surgery before. Keywords:sudden deafness, sensorineural, audiometry.

  15. Longitudinal benefit from and satisfaction with the Baha system for patients with acquired unilateral sensorineural hearing loss.

    Science.gov (United States)

    Newman, Craig W; Sandridge, Sharon A; Wodzisz, Lisa M

    2008-12-01

    To analyze the short-, medium-, and long-term benefits from and satisfaction with the Baha for patients with profound unilateral sensorineural hearing loss (SNHL). Prospective clinical study. Head and Neck Institute at the Cleveland Clinic. Eight patients with acquired profound unilateral SNHL. Patients underwent unaided baseline testing and aided Baha testing using laboratory and self-report measures at 6 postfitting intervals. Laboratory measures included the revised Speech Perception in Noise test, Hearing in Noise Test, and localization testing. Disease-specific self-report measures included the Abbreviated Profile of Hearing Aid Benefit, Hearing Handicap Inventory for Adults, and Single-Sided Deafness Questionnaire. The Medical Outcomes Study SF-36 Health Survey was used to assess health-related quality of life. Based on 95% confidence intervals for unaided testing, significant improvements on aided revised Speech Perception in Noise, Abbreviated Profile of Hearing Aid Benefit, and Hearing Handicap Inventory for Adults scores were sustained during the 18-month test interval. Variable performance was observed for the Hearing in Noise Test over time and patients. No acclimatization effects were shown for localization testing or the generic health-related quality-of-life measure. Patients reported satisfaction in a variety of situations as quantified by the Single-Sided Deafness Questionnaire in the long-term. The Baha is effective in reducing psychosocial consequences of unilateral profound SNHL for the long-term. Improvement in speech understanding occurred when the primary signal was spatially separated from background noise. Localization performance did not improve with Baha use. Overall, patients were satisfied with their Baha and would still elect to have this procedure if given a second chance.

  16. The combination of vestibular impairment and congenital sensorineural hearing loss predisposes patients to ocular anomalies, including Usher syndrome.

    Science.gov (United States)

    Kletke, S; Batmanabane, V; Dai, T; Vincent, A; Li, S; Gordon, K A; Papsin, B C; Cushing, S L; Héon, E

    2017-07-01

    The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having USH. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was performed to determine the ocular phenotype. All the cases had undergone ocular examination and electroretinogram (ERG). Patients with an abnormal ERG underwent genetic testing for USH. We found an underlying ocular abnormality in 81.81% (27/33) of cases; of which 75% had refractive errors, and 50% of those patients showed visual improvement with refractive correction. A total of 14 cases (42.42%; 14/33) had generalized rod-cone dysfunction on ERG suggestive of Usher syndrome type 1, confirmed by mutational analysis. This work shows that adding vestibular impairment as a criterion for requesting an eye exam and adding the ERG to detect USH increases the chances of detecting ocular anomalies, when compared with previous literature focusing only on congenital SNHL. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  17. Molecular epidemiology of DFNB1 deafness in France

    Directory of Open Access Journals (Sweden)

    Molinari Nicolas

    2004-03-01

    Full Text Available Abstract Background Mutations in the GJB2 gene have been established as a major cause of inherited non syndromic deafness in different populations. A high number of sequence variations have been described in the GJB2 gene and the associated pathogenic effects are not always clearly established. The prevalence of a number of mutations is known to be population specific, and therefore population specific testing should be a prerequisite step when molecular diagnosis is offered. Moreover, population studies are needed to determine the contribution of GJB2 variants to deafness. We present our findings from the molecular diagnostic screening of the GJB2 and GJB6 genes over a three year period, together with a population-based study of GJB2 variants. Methods and results Molecular studies were performed using denaturing High Performance Liquid Chromatograghy (DHPLC and sequencing of the GJB2 gene. Over the last 3 years we have studied 159 families presenting sensorineural hearing loss, including 84 with non syndromic, stable, bilateral deafness. Thirty families were genotyped with causative mutations. In parallel, we have performed a molecular epidemiology study on more than 3000 dried blood spots and established the frequency of the GJB2 variants in our population. Finally, we have compared the prevalence of the variants in the hearing impaired population with the general population. Conclusion Although a high heterogeneity of sequence variation was observed in patients and controls, the 35delG mutation remains the most common pathogenic mutation in our population. Genetic counseling is dependent on the knowledge of the pathogenicity of the mutations and remains difficult in a number of cases. By comparing the sequence variations observed in hearing impaired patients with those sequence variants observed in general population, from the same ethnic background, we show that the M34T, V37I and R127H variants can not be responsible for profound or severe

  18. Resolution of sudden sensorineural hearing loss following a roller coaster ride.

    Science.gov (United States)

    Kumar, Aman; Sinha, Amrita; Al-Waa, Ahmad M

    2011-07-01

    We report a case of sudden unilateral sensorineural hearing loss of sudden onset during an aeroplane flight, which completely resolved during a roller coaster ride at Alton Towers theme park. A review of the literature concerning sudden idiopathic sensorineural hearing loss and spontaneous resolution are discussed. Initially, pure-tone audiometry showed a profound sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear (of note, the hearing was normal prior to the episode). Following resolution of the patient's symptoms during a roller coaster ride, pure-tone audiometry showed normal hearing thresholds in both ears. Sudden sensorineural hearing loss is a symptom of cochlear injury and the mechanism of the patient's symptoms was attributed to a patent cochlear aqueduct.

  19. A systematic search for linkage with nonsyndromic recessive deafness in two large Middle Eastern inbred kindreds excludes more than 30% of the genome

    Energy Technology Data Exchange (ETDEWEB)

    Weiss, S.; Korostishevsky, M. [Sackler Faculty of Medicine, Ramat-Aviv (Israel); Frydman, M. [Haim Sheba Medical Center, Tel-Hashomer (Israel)] [and others

    1994-09-01

    It has been estimated that as many as 35 loci may individually cause autosomal recessive non-syndromic deafness. The extreme genetic heterogeneity, limited clinical differentiation and phenotypic assortative mating in many western countries make many families unsuitable for genetic linkage studies. Recently the first of those loci was mapped (to 13q) in two consanguineous families from northern Tunisia. We are studying two large highly consanguineous Middle Eastern kindreds (a total of 26 deaf in 98 sampled individuals). Examination in each family showed no evidence of clinical heterogeneity and indicated an uncomplicated profound bilateral sensorineural deafness. We have been able to exclude the 13q locus as the cause of deafness in each kindred and have also excluded such `candidate` loci as regions as those causing Usher`s syndrome type 1 (11q13)(11p), Usher`s syndrome type II (1q32-q41), Waardenburg syndrome type I (2q37), branchio-oto-renal syndrome (8q12-q13), Monge`s deafness (5q31), and Treacher Collins syndrome (5q31.3-q33.3). To date, no lod scores greater than 1 have been obtained in either kindred using 150 RFLT`s, VNTR`s and highly polymorphic microsatellite markers (CA repeats and tetranucleotides). By Morton`s criterion a minimum of 30% of the autosomal genome can be excluded for each kindred separately.

  20. Should All Deaf Children Learn Sign Language?

    Science.gov (United States)

    Napoli, Donna Jo; Mellon, Nancy K; Niparko, John K; Rathmann, Christian; Mathur, Gaurav; Humphries, Tom; Handley, Theresa; Scambler, Sasha; Lantos, John D

    2015-07-01

    Every year, 10,000 infants are born in the United States with sensorineural deafness. Deaf children of hearing (and nonsigning) parents are unique among all children in the world in that they cannot easily or naturally learn the language that their parents speak. These parents face tough choices. Should they seek a cochlear implant for their child? If so, should they also learn to sign? As pediatricians, we need to help parents understand the risks and benefits of different approaches to parent-child communication when the child is deaf [corrected]. Copyright © 2015 by the American Academy of Pediatrics.

  1. Diffusion tensor imaging of the auditory nerve in patients with acquired single-sided deafness

    DEFF Research Database (Denmark)

    Vos, Sjoerd; Haakma, Wieke; Versnel, Huib

    2015-01-01

    following cochlear hair cell loss, and the amount of degeneration may considerably differ between the two ears, also in patients with bilateral deafness. A measure that reflects the nerve's condition would help to assess the best of both nerves and decide accordingly which ear should be implanted...... for optimal benefit from a CI. Diffusion tensor MRI (DTI) may provide such a measure, by allowing noninvasive investigations of the nerve's microstructure. In this pilot study, we show the first use of DTI to image the auditory nerve in five normal-hearing subjects and five patients with long-term profound...... single-sided sensorineural hearing loss. A specialized acquisition protocol was designed for a 3 T MRI scanner to image the small nerve bundle. The nerve was reconstructed using fiber tractography and DTI metrics - which reflect the nerve's microstructural properties - were computed per tract. Comparing...

  2. Pediatric Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Kizilay, Ahmet; Koca, Çiğdem Firat

    2016-06-01

    Sudden sensorineural hearing loss is defined as sudden unilateral or bilateral sensorineural hearing loss with at least 30 dB decrease in threshold in 3 contiguous test frequencies occurring over 72 hours or less. It is rare among children. The mechanism of the process and prognosis of the disorder remains unclear. The current incidence of sudden sensorineural hearing loss among pediatric population is unknown. The authors carried out a retrospective chart analysis of patients under 15 years of age from 2004 to 2015, who consulted to the Otolaryngology Head and Neck Surgery Department of Inonu University Medical Faculty. Age, sex, number of affected ear and side, audiometric evaluations, medical follow-up, treatment method, duration of treatment recovery, associated complaints; tinnitus and/or vertigo, presence of mumps disease were recorded for each patient. A 4-frequency pure-tone average (500, 1000, 2000, and 4000 Hz) was calculated for each ear. Complete recovery, defined as some hearing level compared with the nonaffected ear, was observed in 3 patients (21.4 %) and there was no partial hearing recovery. The hearing loss of 11 patient remained unchanged after prednisolone treatment. Two of the 11 patients had bilaterally total sensorineural hearing loss and evaluated as appropriate for cochlear implantation. Sex of patient and laterality of hearing loss were not correlated with hearing recovery. Sensorineural hearing loss among pediatrics has been the issue of otolaryngologists. The incidence, etiology, and treatment methods should be more studied.

  3. Children, Deaf, of Deaf Parents

    NARCIS (Netherlands)

    Baker, A.E.; van den Bogaerde, B.; Gertz, G.; Boudreault, P.

    2016-01-01

    Deaf children with Deaf parents usually grow up in the Deaf community, that is if their parents offer them a sign language and are active members of the community. These Deaf children are similar to other children of linguistic and cultural minorities in many ways. They are also different in that

  4. Deaf Epistemology: Deafhood and Deafness

    Science.gov (United States)

    Hauser, Peter C.; O'Hearn, Amanda; McKee, Michael; Steider, Anne; Thew, Denise

    2010-01-01

    Deaf epistemology constitutes the nature and extent of the knowledge that deaf individuals acquire growing up in a society that relies primarily on audition to navigate life. Deafness creates beings who are more visually oriented compared to their auditorily oriented peers. How hearing individuals interact with deaf individuals shapes how deaf…

  5. Mitochondrial Sensorineural Hearing Loss: A Retrospective Study and a Description of Cochlear Implantation in a MELAS Patient

    Directory of Open Access Journals (Sweden)

    Mauro Scarpelli

    2012-01-01

    Full Text Available Hearing impairment is common in patients with mitochondrial disorders, affecting over half of all cases at some time in the course of the disease. In some patients, deafness is only part of a multisystem disorder. By contrast, there are also a number of “pure” mitochondrial deafness disorders, the most common probably being maternally inherited. We retrospectively analyzed the last 60 genetically confirmed mitochondrial disorders diagnosed in our Department: 28 had bilateral sensorineural hearing loss, whereas 32 didn't present ear's abnormalities, without difference about sex and age of onset between each single group of diseases. We reported also a case of MELAS patient with sensorineural hearing loss, in which cochlear implantation greatly contributed to the patient's quality of life. Our study suggests that sensorineural hearing loss is an important feature in mitochondrial disorders and indicated that cochlear implantation can be recommended for patients with MELAS syndrome and others mitochondrial disorders.

  6. Deaf and Hearing Children: A Comparison of Peripheral Vision Development

    Science.gov (United States)

    Codina, Charlotte; Buckley, David; Port, Michael; Pascalis, Olivier

    2011-01-01

    This study investigated peripheral vision (at least 30[degrees] eccentric to fixation) development in profoundly deaf children without cochlear implantation, and compared this to age-matched hearing controls as well as to deaf and hearing adult data. Deaf and hearing children between the ages of 5 and 15 years were assessed using a new,…

  7. Deaf Children's Understanding of Other People's Thought Processes

    Science.gov (United States)

    Edmondson, Peter

    2006-01-01

    This study focuses on the ability of deaf children to predict the behaviours of other people, based on an understanding of their beliefs. An unexpected transfer task and a deceptive box task were used with a group of 55 severely/profoundly deaf children. Results reiterate the findings of other studies that many deaf children are grossly delayed in…

  8. Metaphor Comprehension by Deaf Young Adults

    Science.gov (United States)

    Gold, Rinat; Segal, Osnat

    2017-01-01

    In the present study, we compared the processing of both conventional and novel metaphors by deaf versus hearing young adults. Eighteen deaf participants with severe-to-profound hearing loss and 18 controls matched for age, sex, and years of education were presented with word pairs of 4 types (literal, conventional metaphors, novel metaphors, and…

  9. Development of Implanted Deaf Children's Conversational Skills

    Science.gov (United States)

    Le Maner-Idrissi, Gaid; Dardier, Virginie; Pajon, Cecile; Tan-Bescond, Geraldine; David, Kristell; Deleau, Michel; Godey, Benoit

    2010-01-01

    Previous studies of preverbal development have highlighted the recurrent difficulties experienced by deaf children in acquiring knowledge of the social rules and social skills pertaining to discourse. We expected cochlear implants in children with bilateral profound deafness to improve their use of verbal language, so that their communication…

  10. Feelings and Emotions in Deaf Adolescents

    Science.gov (United States)

    Cambra, Cristina

    2005-01-01

    The objective of this study is to explore the feelings and emotions of deaf adolescents. The study examines the emotional domain of 34 prelingual severely and profoundly deaf adolescents, matched by sex and age with hearing class peers. A sentence completion task (Loeb and Sarigiani, 1986) is employed to assess such feelings as happiness, sadness,…

  11. EEG Alpha and Beta Activity in Normal and Deaf Subjects.

    Science.gov (United States)

    Waldron, Manjula; And Others

    Electroencephalogram and task performance data were collected from three groups of young adult males: profoundly deaf Ss who signed from an early age, profoundly deaf Ss who only used oral (speech and speedreading) methods of communication, and normal hearing Ss. Alpha and Beta brain wave patterns over the Wernicke's area were compared across…

  12. What is profound?

    DEFF Research Database (Denmark)

    Sobisch, Jan-Ulrich

    2013-01-01

    Discussing the question, which elements on the path are to be considered profound. While a general view is that the most subtle practises are also the most profound, 'Jig-rten-mgon-po maintains that the most fundamental one's are to be considered the most profound.......Discussing the question, which elements on the path are to be considered profound. While a general view is that the most subtle practises are also the most profound, 'Jig-rten-mgon-po maintains that the most fundamental one's are to be considered the most profound....

  13. Unusual magnetic resonance findings in two children with sudden sensorineural hearing loss

    OpenAIRE

    Gasparetto,Emerson L.; Arnolfo de Carvalho Neto; Danny Warszawiak; Isac Bruck; Sérgio Antoniuk; Lúcia H.C. dos Santos

    2005-01-01

    OBJECTIVE: To describe the MRI findings of two pediatric patients with sudden sensorineural hearing loss (SSHL). CASE REPORTS: Two male patients (two-year and three-months-old, and one year and four-months-old) presented with sudden dumbness. Physical and neurological examinations were unremarkable besides bilateral hypoacusia. All the laboratory investigation was negative, and brain stem auditory evoked potentials showed deep bilateral deafness in both cases. MRI studies revealed normal inne...

  14. Moving Beyond GDP: Cost Effectiveness of Cochlear Implantation and Deaf Education in Latin America.

    Science.gov (United States)

    Emmett, Susan D; Tucci, Debara L; Bento, Ricardo F; Garcia, Juan M; Juman, Solaiman; Chiossone-Kerdel, Juan A; Liu, Ta J; de Muñoz, Patricia Castellanos; Ullauri, Alejandra; Letort, Jose J; Mansilla, Teresita; Urquijo, Diana P; Aparicio, Maria L; Gong, Wenfeng; Francis, Howard W; Saunders, James E

    2016-09-01

    Cochlear implantation (CI) and deaf education are cost effective management strategies of childhood profound sensorineural hearing loss in Latin America. CI has been widely established as cost effective in North America and Europe and is considered standard of care in those regions, yet cost effectiveness in other economic environments has not been explored. With 80% of the global hearing loss burden existing in low- and middle-income countries, developing cost effective management strategies in these settings is essential. This analysis represents the continuation of a global assessment of CI and deaf education cost effectiveness. Brazil, Colombia, Ecuador, Guatemala, Paraguay, Trinidad and Tobago, and Venezuela participated in the study. A Disability Adjusted Life Years model was applied with 3% discounting and 10-year length of analysis. Experts from each country supplied cost estimates from known costs and published data. Sensitivity analysis was performed to evaluate the effect of device cost, professional salaries, annual number of implants, and probability of device failure. Cost effectiveness was determined using the World Health Organization standard of cost effectiveness ratio/gross domestic product per capita (CER/GDP)<3. Deaf education was very cost effective in all countries (CER/GDP 0.07-0.93). CI was cost effective in all countries (CER/GDP 0.69-2.96), with borderline cost effectiveness in the Guatemalan sensitivity analysis (Max CER/GDP 3.21). Both cochlear implantation and deaf education are widely cost effective in Latin America. In the lower-middle income economy of Guatemala, implant cost may have a larger impact. GDP is less influential in the middle- and high-income economies included in this study.

  15. Prevalence of epileptiform discharges in children with sensori-neural hearing loss and behavioral problems compared to their normal hearing peers.

    Science.gov (United States)

    Amirsalari, Susan; Radfar, Shokoufeh; Ajallouyean, Mohammad; Saburi, Amin; Yousefi, Jaleh; Noohi, Sima; Tavallaie, Seyed Abbas; Hassanalifard, Mahdieh; Ghazavi, Yasaman

    2014-01-01

    Overactivity and behavioral problems are common problems in children with prelingually profound sensorineural hearing loss (SNHL). Data on epileptiform electroencephalography (EEG) discharges in deaf children with psychological disorders are so limited. The primary focus of this study was to determine the prevalence of epileptiform discharges (EDs) in children with SNHL and overactivity or behavioral problems. A total of 262 patients with prelingually profound SNHL who were referred to our cochlear implantation center between 2008 and 2010 were enrolled in this study. Children with SNHL who had diagnosis of overactivity and/or behavioral problems by a pediatric psychiatrist, underwent electroencephalography (EEG). EEG analysis was carried out by a board-certified pediatric neurologist. The control group consisted of 45 cases with overactivity or behavioral problems and normal hearing. One hundred thirty-eight children with mean age of 3.5±1.23 year were enrolled in the case group, of whom 88 cases (63.7%) were boy. The control group consisted of 45 cases with mean age of 3.2±1.53 years, of whom 30 (66.6%) cases were male. EDs were detected in 28 (20.02%) children of the case group (with SNHL) in comparison with 4 (8.88%) in the control group (without SNHL), which was statistically significantly different. In this study, we obtained higher frequency of EDs in deaf children with overactivity and/or behavioral problem compared to the children without SNHL. Further studies are required to evaluate the possible association of SNHL with EDs in overactive children.

  16. Deaf epistemology: Deafhood and Deafness.

    Science.gov (United States)

    Hauser, Peter C; O'Hearn, Amanda; McKee, Michael; Steider, Anne; Thew, Denise

    2010-01-01

    Deaf epistemology constitutes the nature and extent of the knowledge that deaf individuals acquire growing up in a society that relies primarily on audition to navigate life. Deafness creates beings who are more visually oriented compared to their auditorily oriented peers. How hearing individuals interact with deaf individuals shapes how deaf individuals acquire knowledge and how they learn. Aspects of the Deaf episteme, not caused by deafness but by Deafhood, have a positive impact on how deaf individuals learn, resist audism, stay healthy, and navigate the world. Research on psychology, health, and education are reviewed to illustrate how visually oriented beings think and view the world differently from the majority. The article provides support to the theory of multiple epistemologies,and has implications for families, teachers, and researchers.

  17. Sudden sensorineural hearing loss following intramuscular administration of penicillin.

    Science.gov (United States)

    Escada, Pedro Alberto; Capucho, Clara; Madeira da Silva, José Francisco

    2004-02-01

    We report a case of sudden hearing loss in a patient with acute exudative tonsillitis, occurring 15 minutes after the intramuscular administration of penicillin. Audiological evaluation documented a profound sensorineural hearing loss of the cochlear type. The mechanism of the hearing loss was probably an immediate hypersensitivity (type I) allergic drug reaction. Penicillin is used frequently for the treatment of several infections. Allergic reactions to penicillin are well known and include urticaria, maculopapular exanthems, angio-oedema, bronchospasm and anaphylaxis, but sudden hearing loss has never been recorded.

  18. Methadone Induced Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    Chadi Saifan

    2013-01-01

    Full Text Available Background. Sudden sensorineural hearing loss (SSHL caused by opiate abuse or overuse has been well documented in the medical literature. Most documented case reports have involved either heroin or hydrocodone/acetaminophen. Recently, case reposts of methadone induced SSHL have been published. Case Report. We present the case of a 31-year-old man who developed SSHL after a methadone overdose induced stupor. He was subsequently restarted on methadone at his regular dose. On follow-up audiometry exams, he displayed persistent moderately severe sensorineural hearing loss bilaterally. Discussion. This case is notable because unlike all but one previously reported case, the patient—who was restated on methadone—did not make a complete recovery. Conclusion. Methadone overuse in rare cases causes SSHL.

  19. Disacusia neurossensorial imunomediada Immunomediated sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Norma de Oliveira Penido

    2002-10-01

    evolution. They presented whether positive response to immunosuppressive therapy or positiviness to Western Blot test for hsp 70-68kD. Two patients presented rapidly progressive sensorineural hearing loss, associated to vestibular symptoms and the other presented unilateral sudden deafness. No patient reacted to rheumatological testes, and one presented increased erythrocyte sedimentation rate. None satisfactorily responded to corticotherapy, but two presented clinical improvement with other immunosuppressive therapies. The ISHL diagnosis is based on clinical aspects and on the positive response to therapeutic testes with immunosupressor drugs. The Western Blot test for hsp 70-68 kD, with 42% sensitivity and 90% specificity, is the only specific laboratorial exam for ISHL. One patient presented positiviness to this exam and did not responded to immunosuppressive therapy. Two patients with negative tests satisfactorily responded to immunosuppressive therapy. The low sensitivity and high costs of Western Blot test represent difficulties to the spread use of it. The early introduction of treatment has a major importance in the diagnosis of ISHL and to increase the auditory prognosis.

  20. [Study of generational risk in deafness inflicted couples using deafness gene microarray technique].

    Science.gov (United States)

    Wang, Ping; Zhao, Jia; Yu, Shu-yuan; Jin, Peng; Zhu, Wei; DU, Bo

    2011-06-01

    To explored the significance of screening the gene mutations of deafness related in deaf-mute (deaf & dumb) family using DNA microarray. Total of 52 couples of deaf-mute were recruited from Changchun deaf-mute community. With an average age of (58.3 ± 6.7) years old (x(-) ± s). Blood samples were obtained with informed consent. Their genomic DNA was extracted from peripheral blood and PCR was performed. Nine of hot spot mutations in four most common deafness pathologic gene were examined with the DNA microarray, including GJB2, GJB3, PDS and mtDNA 12S rRNA genes. At the same time, the results were verified with the traditional methods of sequencing. Fifty of normal people served as a control group. All patients were diagnosed non-syndromic sensorineural hearing loss by subjective pure tone audiometry. Thirty-two of 104 cases appeared GJB2 gene mutation (30.7%), the mutation sites included 35delG, 176del16, 235delC and 299delAT. Eighteen of 32 cases of GJB2 mutations were 235delC (59.1%). Seven of 104 cases appeared SLC26A4 gene IVS7-2 A > G mutation. Questionnaire survey and gene diagnosis revealed that four of 52 families have deaf offspring (7.6%). When a couple carries the same gene mutation, the risk of their children deafness was 100%. The results were confirmed with the traditional methods of sequencing. There is a high risk of deafness if a deaf-mute family is planning to have a new baby. It is very important and helpful to avoid deaf newborns again in deaf-mute family by DNA microarray.

  1. Refinement of the locus for non-syndromic sensorineural deafness ...

    Indian Academy of Sciences (India)

    Unknown

    2004-04-05

    Bergstrom et al. 1971). Several X-linked forms of isolated bilateral hearing impairment have been reported and their classification is based on the mode of onset and audiogram data (Gorlin 1995). Genetically, the locus DFN1 had been ...

  2. Genetics Home Reference: sensorineural deafness and male infertility

    Science.gov (United States)

    ... and CRISPR-Cas9? What is direct-to-consumer genetic testing? What is precision medicine? What is newborn screening? New Pages Leprosy Polycystic ovary syndrome Gorlin-Chaudhry-Moss syndrome All New & Updated Pages ...

  3. [The result of BAHA fitting in single sided deafness].

    Science.gov (United States)

    Tao, Yong; Li, Gang; Zheng, Yun; Wang, Kai; Meng, Zhaoli; Xu, Ke

    2013-01-01

    To learn the benefit of the unilateral profound sensorineural hearing loss patients wear bone conduction hearing aid BAHA. Sixteen unilateral profound sensorineural hearing loss patients done BAHA aided pure tone test and speech recognition threshold test in the quiet environment and MHINT test respectively. We can learn the effect of wearing BAHA by doing the unaided and aided speech recognition threshold test in different environment. BAHA had good compensation of aided threshold but have no obvious im prove of speech recognition threshold both in quiet and noise environment. According to the research and feedback of patients, BAHA have no obvious benefit to unilateral profound hearing loss. The related benefit test should he done first before deciding whether or not to implant BAHA. The long-term BAHA wearing benefit still need to further understand with unilateral profound sensorineural hearing loss patients.

  4. The Relationship between Communication Problems and Psychological Difficulties in Persons with Profound Acquired Hearing Loss.

    Science.gov (United States)

    Knutson, John F.; Lansing, Charissa R.

    1990-01-01

    Twenty-seven adults with postlingually acquired profound deafness were administered the Communication Profile for the Hearing Impaired and several tests of psychological functioning and adjustment. Inadequate communication strategies and poor accommodations to deafness were associated with depression, social introversion, loneliness, and social…

  5. [Deafness in the dog and cat: aetiology, diagnostics and treatment].

    Science.gov (United States)

    Bach, J-P; Lüpke, M; Wefstaedt, P

    2013-01-01

    Deafness is one of the most common sensory deficits in cats and dogs. Determining the scope and the cause of deafness is important for a possible therapy and can enable the handling of the animal by its owner as well as influence the decision about breeding with the affected animal. Electrodiagnostic testing using the brainstem auditory evoked response (BAER) enables an objective evaluation of the hearing function. Additionally, otoscopy and various diagnostic imaging techniques, including computed tomography, can help in determining the cause of deafness. While conductive deafness can often be treated, there is normally no satisfactory treatment for sensorineural deafness. In such cases, an important task of the attending veterinarian is to advise the owner on handling his/her animal.

  6. Causes of deafness in British Bangladeshi children: a prevalence twice that of the UK population cannot be accounted for by consanguinity alone.

    Science.gov (United States)

    Bajaj, Y; Sirimanna, T; Albert, D M; Qadir, P; Jenkins, L; Cortina-Borja, M; Bitner-Glindzicz, M

    2009-04-01

    To study the causes and prevalence of sensorineural deafness in Bangladeshi children resident in East London. This was a cross sectional survey of children of Bangladeshi origin living in East London with bilateral sensorineural hearing loss of 40 db HL or more. In this study, 134 patients were included. The study looked primarily at the causes of sensorineural hearing loss in this population. The prevalence of deafness in Bangladeshi children in East London is approximately 3.86 per 1000 [95% confidence intervals (CI) 3.20, 4.65] which is significantly greater than the average UK prevalence of 1.65 per 1000. The prevalence of deafness in these Bangladeshi children belonging to non-consanguineous families only, the prevalence falls to 2.73 per 1000 (95% CI 2.19, 3.41). In 60% cases the cause of deafness was genetic. The single most common cause of sensorineural hearing loss in this population was mutations in the GJB2 gene (Connexin 26) in 20 of these patients (17%). Parents were consanguineous in 33% of the families. This study concludes that prevalence of sensorineural deafness in Bangladeshi children is at least 2.3 times the national average. This study also concludes that genetic causes are the common cause of deafness in this ethnic group, with nearly 30% of children with non-syndromic deafness having mutations in GJB2. Although parental consanguinity was very high in this population it did not account for the whole increase in prevalence.

  7. GDP Matters: Cost Effectiveness of Cochlear Implantation and Deaf Education in Sub-Saharan Africa.

    Science.gov (United States)

    Emmett, Susan D; Tucci, Debara L; Smith, Magteld; Macharia, Isaac M; Ndegwa, Serah N; Nakku, Doreen; Mukara, Kaitesi B; Kaitesi, Mukara B; Ibekwe, Titus S; Mulwafu, Wakisa; Gong, Wenfeng; Francis, Howard W; Saunders, James E

    2015-09-01

    Cochlear implantation and deaf education are cost effective in Sub-Saharan Africa. Cost effectiveness of pediatric cochlear implantation has been well established in developed countries but is unknown in low resource settings, where access to the technology has traditionally been limited. With incidence of severe-to-profound congenital sensorineural hearing loss 5 to 6 times higher in low/middle-income countries than the United States and Europe, developing cost-effective management strategies in these settings is critical. Costs were obtained from experts in Nigeria, South Africa, Kenya, Rwanda, Uganda, and Malawi using known costs and published data, with estimation when necessary. A disability adjusted life years (DALY) model was applied using 3% discounting and 10-year length of analysis. Sensitivity analysis was performed to evaluate the effect of device cost, professional salaries, annual number of implants, and probability of device failure. Cost effectiveness was determined using the WHO standard of cost-effectiveness ratio/gross domestic product per capita (CER/GDP) less than 3. Cochlear implantation was cost effective in South Africa and Nigeria, with CER/GDP of 1.03 and 2.05, respectively. Deaf education was cost effective in all countries investigated, with CER/GDP ranging from 0.55 to 1.56. The most influential factor in the sensitivity analysis was device cost, with the cost-effective threshold reached in all countries using discounted device costs that varied directly with GDP. Cochlear implantation and deaf education are equally cost effective in lower-middle and upper-middle income economies of Nigeria and South Africa. Device cost may have greater impact in the emerging economies of Kenya, Uganda, Rwanda, and Malawi.

  8. The Impact of Cochlear Implant in the Oral Language of Children with Congenital Deafness.

    Science.gov (United States)

    Ramos, Daniela; Jorge, João Xavier; Teixeira, António; Ribeiro, Carlos; Paiva, António

    2015-01-01

    Children with severe to profound sensorineural deafness can acquire vocabulary and syntactic structures to communicate by oral language, after cochlear implant. Identify the linguistic skills of children with cochlear implant. Eighteen children of both gender, between 9 and 10 years, with congenital bilateral deafness, using cochlear implant, were studied. The evaluation instrument used was Observation Chart of Language-School Level. The results were compared with standard of normal-hearing children with the same hearing age. The scores registered in the linguistics structures studied, comparing implanted children and standard, was: phonology, 29.44 ± 8.4 vs. 29.68 ± 5.90, p = 0.91; semantics, 18.55 ± 8.89 vs. 19.20 ± 4.85, p = 0.76; morpho-syntax 21.89 ± 12.85 vs. 26.35 ± 10.36, p = 0.159. Regarding the tests of semantics, there was no significant difference. Concerning the tests of morpho-syntactic structure, the difference was significant in the derivation of words, 2.83 ± 2.81 vs. 4.65 ± 1.64, p = 0.014. In the phonology, a significant difference was found comparing implanted children and standard, in the discrimination of pseudo words, 6.6 ± 2.8 vs. 8.37 ± 2.32, p = 0.023. However, in syllabic segmentation, implanted children had a mean score 8.56 ± 1.6 significantly higher than standard, 5.9 ± 1.58, p language components studied confirms that cochlear implant promotes the development of oral verbal language in children with congenital deafness. Implanted children had acquired language skills similar to normal-hearing children with the same hearing age.

  9. Regenerative efficacy of mesenchymal stromal cells from human placenta in sensorineural hearing loss.

    Science.gov (United States)

    Kil, Kicheol; Choi, Mi Young; Kong, Ji Sun; Kim, Woo Jin; Park, Kyoung Ho

    2016-12-01

    Hearing loss is a common chronic disorder characterized by decline of auditory function. The global population have suffered from deafness and the transplantation of stem cells is regarded as a therapeutic strategy for this disease. We collected placenta from a total of 13 samples of full term pregnant women and isolated MSCs derived from human placenta and transplanted MSCs on deaf animal model. The normal group and the sensorineural hearing loss (SNHL) group and the experimental (transplanted MSCs) group were compared and estimated hearing level using auditory brainstem response (ABR) recordings and the otoacoustic emission (OAE) test. ABR threshold value and DPOAE level showed that MSCs transplantation groups was improved than the SNHL group. And the number of spiral ganglion neurons were increased in all turn of the cochlea. And there was no evidence of acute immunological rejection and inflammation response was not observed. This study is to evaluate regenerative efficacy of hearing loss by transplanting mesenchymal stromal cells (MSCs) derived from human placenta (amnion and chorion) in deaf animal model. We identified that MSCs transplantation restored auditory impairment and promoted cell regeneration. We hope to overcome sensorineural hearing loss by transplanting stem cells such as mesenchymal stromal cells (MSCs) from easily accessible adult stem cell source in placenta. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  10. Reduced procedural motor learning in deaf individuals

    Directory of Open Access Journals (Sweden)

    Justine eLévesque

    2014-05-01

    Full Text Available Studies in the deaf suggest that cross-modal neuroplastic changes may vary across modalities. Only a handful of studies have examined motor capacities in the profoundly deaf. These studies suggest the presence of deficits in manual dexterity and delays in movement production. As of yet, the ability to learn complex sequential motor patterns has not been explored in deaf populations. The aim of the present study was to investigate the procedural learning skills of deaf adults. A serial reaction-time task (SRTT was performed by 18 deaf subjects and 18 matched controls to investigate possible motor alteration subsequent to auditory deprivation. Deaf participants had various degrees of hearing loss. Half of the experimental group were early-deaf adults mostly using hearing aids, the remaining half were late-deaf adults using a cochlear implant. Participants carried out a repeating 12-item sequence of key presses along with random blocks containing no repeating sequence. Non-specific and sequence-specific learning was analyzed in relation to individual features related to the hearing loss. The results revealed significant differences between groups in sequence-specific learning, with deaf subjects being less efficient than controls in acquiring sequence-specific knowledge. We interpret the results in light of cross-modal plasticity and the auditory scaffolding hypothesis.

  11. Using Scaffolded Self-Editing to Improve the Writing of Signing Adolescent Deaf Students

    Science.gov (United States)

    Appanah, Thangi M.; Hoffman, Nancy

    2014-01-01

    The authors investigated the impact of the Deaf Student Editing Rubric (DSER) as a self-editing tool on the writing performance of prelingually profoundly deaf adolescent students whose first language is American Sign Language. The DSER was developed by the first author. The study participants included 15 Deaf students in 4 classrooms in grades…

  12. An Examination of Home, School, and Community Experiences of High-Achieving Deaf Adults

    Science.gov (United States)

    Tanner, Kara Kunst

    2017-01-01

    This qualitative study investigated the academic, community, and family experiences of adults who are profoundly deaf. The deaf adults were categorized as high-achieving by having attended college post-high school. The intent of this study is to give teachers, parents, and other deaf students, insight into the factors responsible for contributing…

  13. Not hearing us: An exploration of the experience of deaf prisoners in English and Welsh prisons

    OpenAIRE

    McCulloch, Daniel

    2012-01-01

    This research explores the experiences of profoundly deaf prisoners in England and Wales. It analyses current provisions for deaf prisoners and uses interviews and case studies to investigate the manner in which provision is delivered in order to make a set of recommendations for improving the treatment of deaf people in prison.

  14. EEG activity as an objective measure of cognitive load during effortful listening: A study on pediatric subjects with bilateral, asymmetric sensorineural hearing loss.

    Science.gov (United States)

    Marsella, Pasquale; Scorpecci, Alessandro; Cartocci, Giulia; Giannantonio, Sara; Maglione, Anton Giulio; Venuti, Isotta; Brizi, Ambra; Babiloni, Fabio

    2017-08-01

    Deaf subjects with hearing aids or cochlear implants generally find it challenging to understand speech in noisy environments where a great deal of listening effort and cognitive load are invested. In prelingually deaf children, such difficulties may have detrimental consequences on the learning process and, later in life, on academic performance. Despite the importance of such a topic, currently, there is no validated test for the assessment of cognitive load during audiological tasks. Recently, alpha and theta EEG rhythm variations in the parietal and frontal areas, respectively, have been used as indicators of cognitive load in adult subjects. The aim of the present study was to investigate, by means of EEG, the cognitive load of pediatric subjects affected by asymmetric sensorineural hearing loss as they were engaged in a speech-in-noise identification task. Seven children (4F and 3M, age range = 8-16 years) affected by asymmetric sensorineural hearing loss (i.e. profound degree on one side, mild-to-severe degree on the other side) and using a hearing aid only in their better ear, were included in the study. All of them underwent EEG recording during a speech-in-noise identification task: the experimental conditions were quiet, binaural noise, noise to the better hearing ear and noise to the poorer hearing ear. The subjects' Speech Recognition Thresholds (SRT) were also measured in each test condition. The primary outcome measures were: frontal EEG Power Spectral Density (PSD) in the theta band and parietal EEG PSD in the alpha band, as assessed before stimulus (word) onset. No statistically significant differences were noted among frontal theta power levels in the four test conditions. However, parietal alpha power levels were significantly higher in the "binaural noise" and in the "noise to worse hearing ear" conditions than in the "quiet" and "noise to better hearing ear" conditions (p hearing loss with the purpose of studying the cognitive load during

  15. The relationship between neonatal hyperbilirubinemia and sensorineural hearing loss.

    Science.gov (United States)

    Corujo-Santana, Cándido; Falcón-González, Juan Carlos; Borkoski-Barreiro, Silvia Andrea; Pérez-Plasencia, Daniel; Ramos-Macías, Ángel

    2015-01-01

    Severe jaundice that requires exchange transfusion has become a relatively rare situation today. About 60% of full term neonates and 80% of premature ones will suffer from jaundice within the first week of life. Hyperbilirubinemia at birth is a risk factor associated with hearing loss that is usually further linked to other factors that might have an effect on hearing synergistically. This study aimed to identify the relationship between hyperbilirubinemia at birth as a risk factor for sensorineural hearing loss in children born at Complejo Hospitalario Universitario Insular Materno-Infantil de Gran Canaria, in the 2007-2011 period. This was a retrospective study of 796 newborns that had hyperbilirubinemia at birth, using transient evoked otoacoustic emissions and evoked auditory brainstem response. Hundred eighty-five newborns (23.24%) were referred for evoked auditory brainstem response. Hearing loss was diagnosed for 35 (4.39%): 18 neonates (51.43%) with conductive hearing loss and 17 (48.57%) with sensorineural hearing loss, 3 of which were diagnosed as bilateral profound hearing loss. Half of the children had other risk factors associated, the most frequent being exposure to ototoxic medications. The percentage of children diagnosed with sensorineural hearing loss that suffered hyperbilirubinemia at birth is higher than for the general population. Of those diagnosed, none had levels of indirect bilirubin≥20mg/dl, only 47% had hyperbilirubinemia at birth as a risk factor and 53% had another auditory risk factor associated. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

  16. A study of the incidence and causation of deafness among the children in the tribal population of Manipur and its prevention

    OpenAIRE

    Das, C.; Sanasam, J. C.; Chukhu, N.; Bimol, N.

    1999-01-01

    In a clinico-audiological study of 6,674 children in 24 tribal villages in the hills of Manipur in a total population of 18,565, the incidence of deafness is found to be 6.62% The incidence of the conductive deafness is 98.64% and that of the sensori-neural is 1.35%. Episodes of cold attacks and running nose are found to be precursurs of the prevalent conductive deafness. Preventive measures are worked out.

  17. The role of music in deaf culture: deaf students' perception of emotion in music.

    Science.gov (United States)

    Darrow, Alice-Ann

    2006-01-01

    Although emotional interpretation of music is an individual and variable experience, researchers have found that typical listeners are quite consistent in associating basic or primary emotions such as happiness, sadness, fear, and anger to musical compositions. It has been suggested that an individual with a sensorineural hearing loss, or any lesion in auditory perceptors in the brain may have trouble perceiving music emotionally. The purpose of the present study was to investigate whether students with a hearing loss who associate with the deaf culture, assign the same emotions to music as students without a hearing loss. Sixty-two elementary and junior high students at a Midwestern state school for the deaf and students at neighboring elementary and junior high schools served as participants. Participants at the state school for the deaf had hearing losses ranging from moderate to severe. Twelve film score excerpts, composed to depict the primary emotions-happiness, sadness, and fear, were used as the musical stimuli. Participants were asked to assign an emotion to each excerpt. Results indicated a significant difference between the Deaf and typical hearing participants' responses, with hearing participants' responses more in agreement with the composers' intent. No significant differences were found for age or gender. Analyses of the Deaf participants' responses indicate that timbre, texture, and rhythm are perhaps the musical elements most influential in transmitting emotion to persons with a hearing loss. Adaptive strategies are suggested for assisting children who are deaf in accessing the elements of music intended to portray emotion.

  18. Postural control in children with typical development and children with profound hearing loss

    Directory of Open Access Journals (Sweden)

    Monteiro de Sousa AM

    2012-05-01

    Full Text Available Aneliza Maria Monteiro de Sousa,1 Jônatas de França Barros,2 Brígido Martins de Sousa Neto31Faculty of Health Sciences, University of Brasilia, Brasilia, Federal District, Brazil; 2Department of Physical Education at the Federal University of Rio Grande do Norte, Natal, Rio Grande do Norte, Brazil; 3University Center UNIEURO, Brasilia, Federal District, BrazilPurpose: To describe the behavior of the postural control in children with profound sensorineural hearing loss and compare the results of experimental tests with hearing children aged 7 to 10 years.Patients and methods: This is a cross-sectional study where 100 children were divided into experimental and control groups. We used a force platform, AccuSway Plus, where the tests were conducted under the experimental conditions: open base, eyes open (OBEO; open base, eyes closed (OBEC; closed base, eyes open (CBEO; closed base, eyes closed (CBEC. The body sway velocity (V of the center of pressure, the displacement in the anteroposterior direction (COPap and mediolateral (COPml of the center of pressure were the parameters to evaluate the postural control. For statistical analysis we used the nonparametric Mann–Whitney U test, with a significance level of 5%.Results: In comparisons of variables between the groups, the experimental group outperformed by at least 75% of the control group values. In terms of global trends, the experimental group shows higher values of body oscillations in all experimental conditions and variables evaluated. Children with hearing loss had poorer balance performance compared to the group of hearing. The inferential analysis revealed a statistically significant difference in the balance between deaf and hearing children in the OBEC experimental condition in relation to the COPml parameter (P = 0.04. There were no statistically significant differences in comparisons between the sexes when the groups were analyzed separately. The prevalence of unknown etiology

  19. Hearing Disorders and Sensorineural Aging

    Directory of Open Access Journals (Sweden)

    Alessandra Fioretti

    2014-01-01

    Full Text Available The physiological age-related hearing loss is defined as presbycusis and it is characterized by reduced hearing sensitivity and problems in understanding spoken language especially in a noisy environment. In elderly the reduced speech recognition is generally caused by a reduction of the cochlear cells in the organ of Corti and degeneration of the central auditory pathways. In order to have a complete management strategy of central and peripheral presbycusis the diagnostic evaluation should include clinical ENT examination, standard audiological tests, and tests of central auditory function. Treatment should include not only the appropriate instruments for peripheral compensation but also auditory rehabilitative training and counseling to prevent social isolation and loss of autonomy. Other common hearing disorders in elderly are tinnitus and hyperacusis which are often undervalued. Tinnitus is characterized by the perception of a “phantom” sound due to abnormal auditory perception. Hyperacusis is defined as a reduced tolerance to ordinary environmental sounds. Furthermore auditory, visual, nociceptive, and proprioceptive systems may be involved together in a possible context of “sensorineural aging.” The aim of this review is to underline the presence of hearing disorders like tinnitus and hyperacusis which in many cases coexist with hearing loss in elderly.

  20. Deaf Epistemology: The Deaf Way of Knowing

    Science.gov (United States)

    Holcomb, Thomas K.

    2010-01-01

    The standard epistemology requires the use of hard science to gain knowledge and discover the truth. In contrast, Deaf epistemology relies heavily on personal testimonies, personal experiences, and personal accounts to document knowledge. In recent years, a number of deaf schools have adopted deaf-centric policies shaped by Deaf epistemology in an…

  1. Dystonia and deafness due to SUCLA2 defect; Clinical course and biochemical markers in 16 children.

    NARCIS (Netherlands)

    Morava, E.; Steuerwald, U.; Carrozzo, R.; Kluijtmans, L.A.J.; Joensen, F.; Santer, R.; Dionisi-Vici, C.; Wevers, R.A.

    2009-01-01

    Patients with SUCLA2 gene defects characteristically develop the trias of early hypotonia, progressive dystonia and sensori-neural deafness. We describe the clinical course and biochemical phenotype in 16 children from the Faroe Islands with a homozygous SUCLA2 splice site mutation. Elevated urinary

  2. Tactile perception by the profoundly deaf. Speech and environmental sounds.

    Science.gov (United States)

    Plant, G L

    1982-11-01

    Four subjects fitted with single-channel vibrotactile aids and provided with training in their use took part in a testing programme aimed at assessing their aided and unaided lipreading performance, their ability to detect segmental and suprasegmental features of speech, and the discrimination of common environmental sounds. The results showed that the vibrotactile aid provided very useful information as to speech and non-speech stimuli with the subjects performing best on those tasks where time/intensity cues provided sufficient information to enable identification. The implications of the study are discussed and a comparison made with those results reported for subjects using cochlear implants.

  3. Ellis-van Creveld Syndrome and Profound Deafness Resulted by ...

    Indian Academy of Sciences (India)

    Navya

    2017-04-04

    Apr 4, 2017 ... Otolaryngol.96, 39–47. Butterfield R. J., Ramachandran D., Hasstedt S. J., Otterud B. E., Leppert M. F., Swoboda K. J. et al. 2009 A novel form of juvenile recessive ALS maps to loci on 6p25 and. 21q22.Neuromuscul.19, 279–287. Cahuana A., Palma C., Gonzáles W. and Geán E. 2004 Oral manifestations in ...

  4. Ellis–van Creveld syndrome and profound deafness resulted by ...

    Indian Academy of Sciences (India)

    Muhammad Umair

    2017-12-18

    Dec 18, 2017 ... as disproportionate dwarfism, short ribs, short limbs, dysplastic nails, cardiovascular malformations, post-axial polydactyly (PAP). (bilateral) of hands and feet. ... have congenital heart defects, usually an atrial or atrioven- tricular septal defect ... cardiac malformations and polydactyly (Ruiz-Perez et al. 2007).

  5. Ellis–van Creveld syndrome and profound deafness resulted by ...

    Indian Academy of Sciences (India)

    2017-12-18

    Dec 18, 2017 ... Abstract. Ellis–van Creveld syndrome is an autosomal recessive skeletal dysplasia primarily characterized by the features such as disproportionate dwarfism, short ribs, short limbs, dysplastic nails, cardiovascular malformations, post-axial polydactyly (PAP)(bilateral) of hands and feet. EVC/EVC2 located in ...

  6. Hereditary congenital unilateral deafness : A new disorder?

    NARCIS (Netherlands)

    Dikkers, FG; Verheij, JBGM; van Mechelen, M

    Congenital unilateral deafness is a rare disorder. The prevalence rates are unknown. The prevalence of children with severe to profound hearing losses that are congenital (or acquired before the development of speech and language) is 0.5 to 3 per 1,000 live births. Evidently, congenital unilateral

  7. Perception of persons with severe or profound deafness about the communication process during health care Percepción de la persona con sordera severa y/o profunda acerca del proceso de comunicación durante su atención de salud Percepção da pessoa com surdez severa e/ou profunda acerca do processo de comunicação durante seu atendimento de saúde

    Directory of Open Access Journals (Sweden)

    Adriane Helena Alves Cardoso

    2006-08-01

    Full Text Available This study describes a qualitative approach with the objective of characterizing the perceptions of people with severe or profound deafness about the communication process in the context of health care services. Study participants were 11 people with severe and/or profound deafness, who were interviewed using Brazilian sign language (LIBRAS. The interactions were videotaped and then transcribed. Care was taken to maintain the grammatical construction which was characteristic in the expression of each person. Three categories emerged from thematic analysis: Understanding, Need for Mediation, Feelings. Deaf persons do not achieve effective communication in health care, during which they experience negative feelings. Hence, the presence of a professional interpreter is needed.La finalidad de este estudio descriptivo con aproximación cualitativa fue caracterizar las percepciones de personas con sordera severa o profunda sobre el proceso de la comunicación en el contexto de su atención por profesionales de salud. Participaron once personas con sordera severa y/o profunda, que fueron entrevistadas mediante el lenguaje brasileño de signos (LIBRAS. Las interacciones fueron grabadas en cintas VHS y transcritas, manteniéndose la construcción gramatical característica de su expresión. Mediante análisis temático emergieron tres categorías: Entendimiento, Necesidad de Intermediación y sentimientos. El sordo no alcanza una comunicación eficaz durante la atención de salud, en la cual experimenta sentimientos negativos, necesitando la presencia de un profesional intérprete.Este estudo descritivo de abordagem qualitativa teve o objetivo de caracterizar as percepções da pessoa com surdez severa ou profunda sobre o processo de comunicação no contexto do seu atendimento por profissionais de saúde. Participaram 11 pessoas com surdez severa e/ou profunda, as quais foram entrevistadas usando-se LIBRAS. As interações foram gravadas em fitas VHS e

  8. Connexin 26 (GJB2) mutation in an Argentinean patient with keratitis-ichthyosis-deafness (KID) syndrome: a case report.

    Science.gov (United States)

    Dalamón, Viviana Karina; Buonfiglio, Paula; Larralde, Margarita; Craig, Patricio; Lotersztein, Vanesa; Choate, Keith; Pallares, Norma; Diamante, Vicente; Elgoyhen, Ana Belén

    2016-05-04

    Keratitis-Ichthyosis-Deafness (KID) syndrome is a rare condition characterized by pre-lingual sensorineural deafness with skin hyperkeratinization. The primary cause of the disease is a loss-of-function mutation in the GJB2 gene. Mutations in Argentinean patients have not been described. We studied a 2 year-old boy with bilateral congenital sensorineural deafness with dry skin over the entire body, hypotrichosis of the scalp, thin and light-blond hair. Analysis of the GJB2 gene nucleotide sequence revealed the substitution of guanine-148 by adenine predicted to result in an Asp50Asn amino acid substitution. This is the first KID report in a patient from Argentina. This de novo mutation proved to be the cause of keratitis-ichthyosis-deafness syndrome (KID-syndrome) in the patient, and has implications in medical genetic practice.

  9. Inner ear morphology is perturbed in two novel mouse models of recessive deafness.

    Directory of Open Access Journals (Sweden)

    Kerry A Miller

    Full Text Available Human MYO7A mutations can cause a variety of conditions involving the inner ear. These include dominant and recessive non-syndromic hearing loss and syndromic conditions such as Usher syndrome. Mouse models of deafness allow us to investigate functional pathways involved in normal and abnormal hearing processes. We present two novel mouse models with mutations in the Myo7a gene with distinct phenotypes. The mutation in Myo7a(I487N/I487N ewaso is located within the head motor domain of Myo7a. Mice exhibit a profound hearing loss and manifest behaviour associated with a vestibular defect. A mutation located in the linker region between the coiled-coil and the first MyTH4 domains of the protein is responsible in Myo7a(F947I/F947I dumbo. These mice show a less severe hearing loss than in Myo7a(I487N/I487N ewaso; their hearing loss threshold is elevated at 4 weeks old, and progressively worsens with age. These mice show no obvious signs of vestibular dysfunction, although scanning electron microscopy reveals a mild phenotype in vestibular stereocilia bundles. The Myo7a(F947I/F947I dumbo strain is therefore the first reported Myo7a mouse model without an overt vestibular phenotype; a possible model for human DFNB2 deafness. Understanding the molecular basis of these newly identified mutations will provide knowledge into the complex genetic pathways involved in the maintenance of hearing, and will provide insight into recessively inherited sensorineural hearing loss in humans.

  10. Sudden (reversible) sensorineural hearing loss in pregnancy.

    LENUS (Irish Health Repository)

    Kenny, R

    2011-03-01

    Sudden hearing loss directly associated with pregnancy or birth is a little known and rare occurrence. The temporary, unilateral, low-frequency sensorineural hearing loss in this case was reported after the birth of the patient\\'s first child, and again during the third trimester of her second pregnancy.

  11. Bringing Hearing to the Deaf

    Energy Technology Data Exchange (ETDEWEB)

    Shipsey, Ian (Purdue)

    2006-06-12

    In his talk, Shipsey will discuss the cochlear implant, the first device to successfully allow the profoundly deaf to regain some sense of hearing. A cochlear implant is a small electronic apparatus. Unlike a normal hearing aid, which amplifies sound, a cochlear implant is surgically implanted behind the ear where it converts sound waves into electrical impulses. These implants have instigated a popular but controversial revolution in the treatment of deafness, and they serve as a model for research in neuroscience and biomedical engineering. Shipsey will discuss the physiology of natural hearing from the perspective of a physicist. He will also touch on the function of cochlear implants in the context of historical treatments, electrical engineering, psychophysics, clinical evaluation of efficacy and personal experience. Finally, Shipsey will address the social implications of cochlear implantation and the future outlook for auditory prostheses.

  12. Deaf People Can!

    Science.gov (United States)

    Wixtrom, Christine

    1987-01-01

    Activities designed to increase deaf students' self-esteem and positive attitudes toward their deafness included a bulletin board displaying the different things deaf children and adults could do; and a trip to see actors from the National Theater of the Deaf. (CB)

  13. Combined examination of sequence and copy number variations in human deafness genes improves diagnosis for cases of genetic deafness.

    Science.gov (United States)

    Ji, Haiting; Lu, Jingqiao; Wang, Jianjun; Li, Huawei; Lin, Xi

    2014-01-01

    Copy number variations (CNVs) are the major type of structural variation in the human genome, and are more common than DNA sequence variations in populations. CNVs are important factors for human genetic and phenotypic diversity. Many CNVs have been associated with either resistance to diseases or identified as the cause of diseases. Currently little is known about the role of CNVs in causing deafness. CNVs are currently not analyzed by conventional genetic analysis methods to study deafness. Here we detected both DNA sequence variations and CNVs affecting 80 genes known to be required for normal hearing. Coding regions of the deafness genes were captured by a hybridization-based method and processed through the standard next-generation sequencing (NGS) protocol using the Illumina platform. Samples hybridized together in the same reaction were analyzed to obtain CNVs. A read depth based method was used to measure CNVs at the resolution of a single exon. Results were validated by the quantitative PCR (qPCR) based method. Among 79 sporadic cases clinically diagnosed with sensorineural hearing loss, we identified previously-reported disease-causing sequence mutations in 16 cases. In addition, we identified a total of 97 CNVs (72 CNV gains and 25 CNV losses) in 27 deafness genes. The CNVs included homozygous deletions which may directly give rise to deleterious effects on protein functions known to be essential for hearing, as well as heterozygous deletions and CNV gains compounded with sequence mutations in deafness genes that could potentially harm gene functions. We studied how CNVs in known deafness genes may result in deafness. Data provided here served as a basis to explain how CNVs disrupt normal functions of deafness genes. These results may significantly expand our understanding about how various types of genetic mutations cause deafness in humans.

  14. Imaging Keratitis-Icthyosis-Deafness (KID) syndrome with FDG-PET (F18-fluorodeoxiglucose-Positron Emission Tomography).

    Science.gov (United States)

    Aparici, Carina Mari; Arcienega, Daniela; Cho, Eric; Hawkins, Randy

    2010-01-01

    Keratitis-Icthyosis-Deafness (KID) syndrome is a rare dysplasia characterized by vascularizing keratitis, congenital sensorineural hearing-loss, and progressive erythrokeratoderma. To our knowledge, this is the first KID syndrome imaged with FDG-PET in the literature. This paper is intended to help familiarize with the FDG abnormalities related to this rare entity.

  15. Congenital deafness in Jack Russell terriers: prevalence and association with phenotype.

    Science.gov (United States)

    Comito, B; Knowles, K E; Strain, G M

    2012-08-01

    Congenital hereditary sensorineural deafness is the most common form of deafness in dogs. The objectives of this study were to determine a reliable measure of the prevalence of deafness in Jack Russell terriers, an affected breed, and associations between deafness and phenotypic characteristics. Brainstem auditory evoked response recordings and phenotypic parameters (coat color, coat texture, sex, eye color, sire and dam hearing status) were recorded for 1009 Jack Russell terriers. The prevalence of unilateral and bilateral deafness was 3.57% and 0.50%, respectively, lower by a factor of three to four than in earlier reports based on smaller and closely related kindreds. Significant association with deafness was identified with white coat color and parental hearing status, but not with sex or coat type. Lack of significant sex or coat type associations and the significant association with white coat color are consistent with previous reports. In conclusion the prevalence of deafness in Jack Russell terriers is lower than initially reported. Deafness was associated with white coat color and parental hearing status. The association with parental hearing status supports this form of deafness being a heritable trait in the breed and the association with white coat color supports an inheritance linked to pigmentation genes. Copyright © 2012 Elsevier Ltd. All rights reserved.

  16. Distal renal tubular acidosis with nerve deafness secondary to ATP6B1 gene mutation

    Directory of Open Access Journals (Sweden)

    Parvathina Sriram Naveen

    2015-01-01

    Full Text Available Autosomal recessive distal renal tubular acidosis (dRTA is associated with mutation in the ATP6B1 gene encoding the B1 subunit of H + -ATPase, one of the key membrane transporters for net acid excretion of α-intercalated cells of medullary collecting ducts. Sensori-neural deafness frequently accompanies this type of dRTA. We herewith describe a patient who had distinct features of dRTA with bilateral sensori-neural hearing loss and ATP6B1 mutation. This is a rare entity.

  17. Complex Treatment of Sensorineural Hearing Loss

    OpenAIRE

    Aleksandruk, N. V.

    2014-01-01

    Recent data on use of Ginkgo Biloba extract in otorhinolaryngological practice were presented. The mechanism of the curative action of Ginkgo Biloba extract (vasoprotective, antioxidative, rheological, and edematous) was described. Effectiveness of Ginkgo Biloba as a part of complex treatment of sensorineural hearing loss in children was elucidated. Results of the research proved effectiveness of treatment with Ginkgo Biloba and showed perspectives of Ginkgo Biloba use in treatment programs f...

  18. Overview on Deaf-Blindness

    Science.gov (United States)

    ... Topics About Deaf-Blindness > What is Deaf-Blindness Definitions of Deaf-Blindness Causes of Deaf-Blindness National Child Count & Demographics Communication > Communication Overview Early Communication Prelinguistic Communication Object Communication ...

  19. Adaptive crossmodal plasticity in deaf auditory cortex: areal and laminar contributions to supranormal vision in the deaf.

    Science.gov (United States)

    Lomber, Stephen G; Meredith, M Alex; Kral, Andrej

    2011-01-01

    This chapter is a summary of three interdigitated investigations to identify the neural substrate underlying supranormal vision in the congenitally deaf. In the first study, we tested both congenitally deaf and hearing cats on a battery of visual psychophysical tasks to identify those visual functions that are enhanced in the congenitally deaf. From this investigation, we found that congenitally deaf, compared to hearing, cats have superior visual localization in the peripheral field and lower visual movement detection thresholds. In the second study, we examined the role of "deaf" auditory cortex in mediating the supranormal visual abilities by reversibly deactivating specific cortical loci with cooling. We identified that in deaf cats, reversible deactivation of a region of cortex typically identified as the posterior auditory field (PAF) in hearing cats selectively eliminated superior visual localization abilities. It was also found that deactivation of the dorsal zone (DZ) of "auditory" cortex eliminated the superior visual motion detection abilities of deaf cats. In the third study, graded cooling was applied to deaf PAF and deaf DZ to examine the laminar contributions to the superior visual abilities of the deaf. Graded cooling of deaf PAF revealed that deactivation of the superficial layers alone does not cause significant visual localization deficits. Profound deficits were identified only when cooling extended through all six layers of deaf PAF. In contrast, graded cooling of deaf DZ showed that deactivation of only the superficial layers was required to elicit increased visual motion detection thresholds. Collectively, these three studies show that the superficial layers of deaf DZ mediate the enhanced visual motion detection of the deaf, while the full thickness of deaf PAF must be deactivated in order to eliminate the superior visual localization abilities of the congenitally deaf. Taken together, this combination of experimental approaches has

  20. Heritability and complex segregation analysis of deafness in Jack Russell Terriers.

    Science.gov (United States)

    Famula, Thomas R; Cargill, Edward J; Strain, George M

    2007-11-13

    The association between patterns of pigmentation and deafness in the dog has a long-documented history, with reports dating back over one hundred years. Long suspected of having a genetic basis, the search for loci with a pronounced influence in the expression of hearing loss in the dog has yet to be successful. No studies in the dog to date have found a possible influence of a specific colour locus associated with deafness. The present study is intended to evaluate the heritability of deafness in the Jack Russell Terrier (JRT), characterize the mode of inheritance, and evaluate the existence of a sex, coat colour, or coat texture influence on the expression of sensorineural deafness. The estimation of heritability of deafness in the JRT was 0.22 when deafness was considered a binary (normal/deaf) trait and 0.31 when deafness was considered a three-category (normal/unilateral/bilateral deafness). The influence of coat colour in the incidence of JRT deafness was statistically significant, indicating that dogs with more white are more likely to be deaf. The influence of sex or coat texture was not statistically significant in the incidence of JRT deafness. Complex segregation analysis revealed a model of a single locus with a large effect on the binary measure of hearing loss is not supported. This is the first attempt, to our knowledge, to characterize a genetic component responsible for deafness in the JRT. The heritability of deafness in the JRT was found to be 0.22 and 0.31 considering deafness to be a two-category or three-category trait, respectively. There appears to be an influence of coat colour on the expression of deafness. In an attempt to characterize the mode of inheritance of deafness in the JRT, a model of a single locus with a large effect on hearing loss is not supported with this data. Further study is needed to determine if a single locus may be influencing deafness in the JRT. While the absence of a clear mode of inheritance complicates genetic

  1. Heritability and complex segregation analysis of deafness in Jack Russell Terriers

    Directory of Open Access Journals (Sweden)

    Strain George M

    2007-11-01

    Full Text Available Abstract Background The association between patterns of pigmentation and deafness in the dog has a long-documented history, with reports dating back over one hundred years. Long suspected of having a genetic basis, the search for loci with a pronounced influence in the expression of hearing loss in the dog has yet to be successful. No studies in the dog to date have found a possible influence of a specific colour locus associated with deafness. The present study is intended to evaluate the heritability of deafness in the Jack Russell Terrier (JRT, characterize the mode of inheritance, and evaluate the existence of a sex, coat colour, or coat texture influence on the expression of sensorineural deafness. Results The estimation of heritability of deafness in the JRT was 0.22 when deafness was considered a binary (normal/deaf trait and 0.31 when deafness was considered a three-category (normal/unilateral/bilateral deafness. The influence of coat colour in the incidence of JRT deafness was statistically significant, indicating that dogs with more white are more likely to be deaf. The influence of sex or coat texture was not statistically significant in the incidence of JRT deafness. Complex segregation analysis revealed a model of a single locus with a large effect on the binary measure of hearing loss is not supported. Conclusion This is the first attempt, to our knowledge, to characterize a genetic component responsible for deafness in the JRT. The heritability of deafness in the JRT was found to be 0.22 and 0.31 considering deafness to be a two-category or three-category trait, respectively. There appears to be an influence of coat colour on the expression of deafness. In an attempt to characterize the mode of inheritance of deafness in the JRT, a model of a single locus with a large effect on hearing loss is not supported with this data. Further study is needed to determine if a single locus may be influencing deafness in the JRT. While the

  2. ASL Handshape Stories, Word Recognition and Signing Deaf Readers: An Exploratory Study

    Science.gov (United States)

    Gietz, Merrilee R.

    2013-01-01

    The effectiveness of using American Sign Language (ASL) handshape stories to teach word recognition in whole stories using a descriptive case study approach was explored. Four profoundly deaf children ages 7 to 8, enrolled in a self-contained deaf education classroom in a public school in the south participated in the story time five-week…

  3. Developing Communication Skills in Deaf Primary School Pupils: Introducing and Evaluating the SmiLE Approach

    Science.gov (United States)

    Alton, Sherryn; Herman, Rosalind; Pring, Tim

    2011-01-01

    Many profoundly deaf signers have difficulty communicating with hearing people. This article describes a therapy called "Strategies and Measurable Interaction in Live English" (smiLE; Schamroth and Threadgill, 2007a), an approach used to teach deaf children skills to become successful communicators in real-life situations. This study…

  4. Decoding Actions and Emotions in Deaf Children: Evidence from a Biological Motion Task

    Science.gov (United States)

    Ludlow, Amanda Katherine; Heaton, Pamela; Deruelle, Christine

    2013-01-01

    This study aimed to explore the recognition of emotional and non-emotional biological movements in children with severe and profound deafness. Twenty-four deaf children, together with 24 control children matched on mental age and 24 control children matched on chronological age, were asked to identify a person's actions, subjective states,…

  5. Parents' Experiences with Childhood Deafness: Implications for Family-Centered Services

    Science.gov (United States)

    Jackson, Carla Wood; Traub, Randi J.; Turnbull, Ann P.

    2008-01-01

    In response to the need for family-centered follow-up, this study examined parents' experiences with deafness after early identification. Qualitative inquiry methods were used to explore and describe the perceptions and experiences of nine parents of children identified with severe to profound deafness. Parents participated in face-to-face…

  6. The Contribution of Verbal Working Memory to Deaf Children's Oral and Written Production

    Science.gov (United States)

    Arfé, Barbara; Rossi, Cristina; Sicoli, Silvia

    2015-01-01

    This study investigated the contribution of verbal working memory to the oral and written story production of deaf children. Participants were 29 severely to profoundly deaf children aged 8-13 years and 29 hearing controls, matched for grade level. The children narrated a picture story orally and in writing and performed a reading comprehension…

  7. Theory of Mind: Deaf and Hearing Children's Comprehension of Picture Stories and Judgments of Social Situations.

    Science.gov (United States)

    Rhys-Jones, Sara L.; Ellis, Haydn D.

    2000-01-01

    This study compared 20 prelingually profoundly deaf adolescents (age 11-16 years) and 20 matched, hearing adolescents on a picture-sequencing task and a social judgment test. Results did not support the hypothesis that deaf adolescents possess significantly poorer knowledge about social reasoning than hearing peers but it did support the…

  8. Visual advantage in deaf adults linked to retinal changes.

    Directory of Open Access Journals (Sweden)

    Charlotte Codina

    Full Text Available The altered sensory experience of profound early onset deafness provokes sometimes large scale neural reorganisations. In particular, auditory-visual cross-modal plasticity occurs, wherein redundant auditory cortex becomes recruited to vision. However, the effect of human deafness on neural structures involved in visual processing prior to the visual cortex has never been investigated, either in humans or animals. We investigated neural changes at the retina and optic nerve head in profoundly deaf (N = 14 and hearing (N = 15 adults using Optical Coherence Tomography (OCT, an in-vivo light interference method of quantifying retinal micro-structure. We compared retinal changes with behavioural results from the same deaf and hearing adults, measuring sensitivity in the peripheral visual field using Goldmann perimetry. Deaf adults had significantly larger neural rim areas, within the optic nerve head in comparison to hearing controls suggesting greater retinal ganglion cell number. Deaf adults also demonstrated significantly larger visual field areas (indicating greater peripheral sensitivity than controls. Furthermore, neural rim area was significantly correlated with visual field area in both deaf and hearing adults. Deaf adults also showed a significantly different pattern of retinal nerve fibre layer (RNFL distribution compared to controls. Significant correlations between the depth of the RNFL at the inferior-nasal peripapillary retina and the corresponding far temporal and superior temporal visual field areas (sensitivity were found. Our results show that cross-modal plasticity after early onset deafness may not be limited to the sensory cortices, noting specific retinal adaptations in early onset deaf adults which are significantly correlated with peripheral vision sensitivity.

  9. Cochlear implantation versus auditory brainstem implantation in bilateral total deafness after head trauma: personal experience and review of the literature.

    Science.gov (United States)

    Medina, Marimar; Di Lella, Filippo; Di Trapani, Giuseppe; Prasad, Sampath Chandra; Bacciu, Andrea; Aristegui, Miguel; Russo, Alessandra; Sanna, Mario

    2014-02-01

    To determine the effectiveness of cochlear implant (CI) in hearing restoration after temporal bone (TB) fractures and investigate the adequacy of auditory brainstem implant (ABI) indication for TB fractures. Retrospective clinical study; a systematic review of the literature in PubMed was also performed to identify all published cases of bilateral TB fractures or bilateral deafness after head trauma treated by means of CI or ABI. Quaternary otology and skull base surgery referral center. Eleven consecutive patients presented with bilateral severe-to-profound sensorineural hearing loss after head trauma. CI as primary intervention or following a previous treatment. CI performances were evaluated in the auditory-only condition in both closed-set and open-set formats. Fourteen CI were placed, 11 as primary treatment and 3 after ABI failure. At the last follow-up, all patients gained useful open-set speech perception. In secondary CI, all patients obtained better auditory results with the CI if compared with ABI. CI performance did not decrease with time in any case. Cochlear implantation after TB fractures has proved to have excellent audiometric results. The aim of the initial evaluation of a patient with bilateral anacoustic ears from head trauma should always be to rehabilitate their hearing with a CI. The incidence of labyrinthitis ossificans, negative electrophysiologic testing, the risk of postoperative meningitis or facial nerve stimulation should not be the determinant factors that favor ABI placement.

  10. Saccular dysfunction in children with sensorineural hearing loss and auditory neuropathy/auditory dys-synchrony.

    Science.gov (United States)

    Emami, Seyede Faranak; Farahani, Farhad

    2015-01-01

    There is a correlation between the AN/AD disorder and the saccular dysfunction in children with severe sensorineural hearing loss, which means that saccular dysfunction can be a concomitant sign of AN/AD. In conclusion, the term of audio-vestibular dys-synchrony (AVS) is a more suitable description for this condition. Patients with auditory neuropathy/auditory dys-synchrony (AN/AD) characteristically demonstrate poor neural responses from the vestibulocochlear nerve and brainstem while displaying evidence of intact outer hair cells function. Therefore, the objective of this study is studying of the relationship of the saccular dysfunction with AN/AD disorder in children with sensorineural hearing loss. In this cross-sectional study, 100 children with bilateral severe-to-profound sensorineural hearing losses underwent audiologic tests and cervical vestibular-evoked myogenic potentials (cVEMPs) at the Audiology Department of Hamadan University of Medical Sciences (Hamadan, Iran). Eleven children with bilateral severe sensorineural hearing loss were given to unilateral AN/AD disorder (11 ears), and two children (4 ears) had bilateral AN/AD (total = 13 children). The ears with AN/AD took the form of unrepeatable or absent waves of ABR and presence of OAEs. The statistical analysis of an independent t-test between AN/AD ears as compared to non-AN/AD ears of these 13 children showed that the mean latencies of p13 and the mean latencies of n23 and the mean peak-to-peak amplitude had significant differences.

  11. The Effect of Vestibular Rehabilitation Therapy Program on Sensory Organization of Deaf Children With Bilateral Vestibular Dysfunction

    Directory of Open Access Journals (Sweden)

    Amir Abbas Ebrahimi

    2018-01-01

    Full Text Available The purpose of this study was to determine the effect of vestibular rehabilitation therapy program on the sensory organization of deaf children with bilateral vestibular dysfunction. This cross-sectional and analytic study was conducted on 24 students between the age of 7 and 12 years (6 girls and 18 boys with the profound sensorineural hearing loss (PTA>90 dB. They were assessed through the balance subtest in Bruininks-Oseretsky test of motor proficiency (BOTMP. For children which the total score of the balance subtest was 3 standard deviation lower than their peers with typical development, vestibular function testing was completed pre-intervention. Posturography Sensory organization testing (SOT was completed pre- and post-intervention with SPS (Synapsys, Marseille, France. Children with bilateral vestibular impairment were randomly assigned to either the exercise or control group. Exercise intervention consisted of compensatory training, emphasizing enhancement of visual and somatosensory function, and balance training. The exercise group entered in vestibular rehabilitation therapy program for 8 weeks. The children initially participating in the control group were provided the exercise intervention following the post-test. Based on the results there was significant difference in condition 5 and 6, areas of limits of stability (LOS, vestibular ratio and global score in posturography at the end of the intervention, but there was no significant difference in the control group in posturography (P<0.05. The results indicated that testing of vestibular, and postural control function, as well as intervention for deficiencies identified, should be included in deaf children rehabilitation program.

  12. The Effect of Vestibular Rehabilitation Therapy Program on Sensory Organization of Deaf Children With Bilateral Vestibular Dysfunction.

    Science.gov (United States)

    Ebrahimi, Amir Abbas; Jamshidi, Ali Ashraf; Movallali, Guita; Rahgozar, Mehdi; Haghgoo, Hojjat Allah

    2017-11-01

    The purpose of this study was to determine the effect of vestibular rehabilitation therapy program on the sensory organization of deaf children with bilateral vestibular dysfunction. This cross-sectional and analytic study was conducted on 24 students between the age of 7 and 12 years (6 girls and 18 boys) with the profound sensorineural hearing loss (PTA>90 dB). They were assessed through the balance subtest in Bruininks-Oseretsky test of motor proficiency (BOTMP). For children which the total score of the balance subtest was 3 standard deviation lower than their peers with typical development, vestibular function testing was completed pre-intervention. Posturography Sensory organization testing (SOT) was completed pre- and post-intervention with SPS (Synapsys, Marseille, France). Children with bilateral vestibular impairment were randomly assigned to either the exercise or control group. Exercise intervention consisted of compensatory training, emphasizing enhancement of visual and somatosensory function, and balance training. The exercise group entered in vestibular rehabilitation therapy program for 8 weeks. The children initially participating in the control group were provided the exercise intervention following the post-test. Based on the results there was significant difference in condition 5 and 6, areas of limits of stability (LOS), vestibular ratio and global score in posturography at the end of the intervention, but there was no significant difference in the control group in posturography (P<0.05). The results indicated that testing of vestibular, and postural control function, as well as intervention for deficiencies identified, should be included in deaf children rehabilitation program.

  13. [Iatrogenic and non-iatrogenic factors as causes of progressive sensorineural hearing loss].

    Science.gov (United States)

    De Capua, B; Barbieri, M T; Tozzi, A; Passàli, D

    1998-08-01

    The aim of our study was to analyze factors such as noise, chemical drugs, industrial solvents and radiotherapy, which can cause cochlear lesions with progressive sensorineural hearing loss. Although an acute overstimulation by acoustic energy may induce an irreversible hearing loss, in most cases the noise-induced deafness is related to the duration of the exposure and to the level of the acoustic stimulation. A permanent hearing deficit occurs when the acoustic level exceeds 85 dBs. Also several classes of drugs are described as having ototoxic potential: aminoglycoside antibiotics, loop diuretics, antimalarial drugs such as quinine, salicylates, some chemotherapeutic antineoplastic agents. Their potential ototoxic effect seems to be related not only to the molecule, but also to individual predisposition, dose and route of administration. Regarding the benzene derivatives, there is a relationship between their ototoxicity and factors such as duration of exposure and concentration in the local environment. Finally, radiotherapy to areas near the temporal bone may produce a degenerative insult to the vascular stria and the hair cell causing a progressive sensorineural hearing loss.

  14. Mutations in MYH9 exons 1, 16, 26, and 30 are infrequently found in Japanese patients with nonsyndromic deafness.

    Science.gov (United States)

    Kunishima, Shinji; Matsunaga, Tatsuo; Ito, Yoshimi; Saito, Hidehiko

    2009-10-01

    Mutations in MYH9 result in the autosomal dominant giant platelet disorders with leukocyte inclusion bodies with varying degrees of Alport manifestations, including nephritis, deafness, and cataracts. A specific MYH9 mutation in exon 16, R705H, causes nonsyndromic deafness DFNA17. We searched for mutations in MYH9 exons 1, 16, 26, and 30 in a total of 157 Japanese patients with nonsyndromic deafness without known cause of hearing loss, but no mutations were found. We conclude that mutations in MYH9 are infrequently found in patients with nonsyndromic deafness and suggest that MYH9 mutations infrequently cause isolated sensorineural hearing loss. Thus, MYH9 may not currently be a good candidate gene for efficient screening of genetic causes in nonsyndromic deafness.

  15. Sensorineural hearing loss in hemorrhagic dengue?

    Science.gov (United States)

    Ribeiro, Bruna Natália Freire; Guimarães, Alexandre Caixeta; Yazawa, Felipe; Takara, Tammy Fumiko Messias; de Carvalho, Guilherme Machado; Zappelini, Carlos Eduardo Monteiro

    2015-01-01

    Dengue is an acute febrile infectious disease, with high fever followed by symptoms flu-like. Dengue hemorrhagic fever (DHF) is a vascular leak syndrome and could present spontaneous bleeding and worsening of symptoms after some days. Dengue could have some ENT manifestations, however hearing loss is not one of them. Sudden hearing loss is considered as sensorineural or perceptual hearing loss with a sudden onset in a person without other prior otological history. The relation between infectious diseases and sudden hearing are been investigated, some viruses were already linked, but the relation between dengue virus and sudden hearing still remains unknown. This article has the goal of presenting a case of DHF that evolved with SSHL in his hospitalization process. We report a 60 years-male patient of with DHF who developed bilateral secretory otitis media and sensorineural hearing loss after the fifth day of onset of symptoms. His hearing loss remained even after 7 months and the patient was referred for hearing aid fitting. This is the first case report that brings together DHF and sudden hearing loss. In the development of this case no other cause to sudden hearing loss was found and the correlation between dengue and hearing loss was questioned. In the literature review was found that some viruses, as mumps virus, varicella-zoster virus and HSV-1 and HSV-2 are related to sudden hearing loss, all of them fit in the viral theory. Besides the viral theory of sudden hearing loss, there is the vascular theory that is the occlusion of the end artery that supplies the cochlea. DHF has a vascular commitment, and the hypothesis of a vascular cause could be elicited in this case. Many studies in this area are needed and this article has the objective of elicit the discussion about the subject. Could dengue be associated with sensorineural hearing loss? Copyright © 2015. Published by Elsevier Ltd.

  16. Sociological Aspects of Deafness.

    Science.gov (United States)

    World Federation of the Deaf, Rome (Italy).

    Nine conference papers treat the sociological aspects of deafness. Included are "Individuals Being Deaf and Blind and Living with a Well Hearing Society" by A. Marx (German Federal Republic), "A Deaf Man's Experiences in a Hearing World" by A. B. Simon(U.S.A.), "Problem of Text Books and School Appliances for Vocational…

  17. Two new cases with microdeletion of 17q23.2 suggest presence of a candidate gene for sensorineural hearing loss within this region

    DEFF Research Database (Denmark)

    Schönewolf-Greulich, Bitten; Ronan, Anne; Ravn, Kristine

    2011-01-01

    . In this report, we describe two new 17q23.2 deletion patients with mild intellectual disability and sensorineural hearing loss. They both had submicroscopic deletions smaller than the common deleted region for the 8 previously described 17q23.2 microdeletion cases. TBX4 was previously suggested...... as the responsible gene for the heart or limb defects observed in 17q23.2 deletion patients, but the present cases do not have these features despite deletion of this gene. The finding of sensorineural hearing loss in 5 of the 10 cases, including the present cases, with a microdeletion at17q23.2, strongly suggests...... the presence of a candidate gene for hearing loss within this region. We screened 41 patients with profound sensorineural hearing loss for mutations of TBX2 and detected no mutations....

  18. X-linked albinism-deafness syndrome and Waardenburg syndrome type II: A hypothesis

    Energy Technology Data Exchange (ETDEWEB)

    Zlotogora, J. [Hadassah Univ. Hospital, Jerusalem (Israel)

    1995-11-20

    Margolis reported on a large pedigree with a {open_quotes}new{close_quotes} X-linked syndrome of profound deafness and albinism (MIM 300700, albinism-deafness syndrome). The affected males presented with profound deafness and severe pigmentary abnormalities of the skin. At birth the skin appeared as almost albinotic except for areas of light pigmentation over the gluteal and scrotal areas, and thereafter pigmentation gradually increased over the body. Skin changes ultimately included areas of hypopigmentation and spots of hyperpigmentation. Some of the affected males also had blue irides, heterochromia, or segmental color iris changes. In carrier females, variable hearing impairment was documented without any pigmentary changes. 9 refs., 1 fig.

  19. 38 CFR 17.149 - Sensori-neural aids.

    Science.gov (United States)

    2010-07-01

    ... medical treatment. (c) VA will furnish needed hearing aids to those veterans who have service-connected... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other...

  20. A honey bee can threat ear: Sudden sensorineural hearing loss.

    Science.gov (United States)

    Düzenli, Ufuk; Bozan, Nazım; Ayral, Abdurrahman; Yalınkılıç, Abdülaziz; Kıroğlu, Ahmet Faruk

    2017-11-01

    Sudden sensorineural hearing loss is an otologic emergency. Many etiological factors can lead to this pathology. Honey bee (Apis mellifera) sting may lead to local and systemic reactions due to sensitization of the patient. In this paper we described a sudden sensorineural hearing loss occurred after honey bee sting. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Ataxia with vitamin E deficiency associated with deafness.

    Science.gov (United States)

    Kara, Bülent; Uzümcü, Abdullah; Uyguner, Oya; Rosti, Rasim Ozgür; Koçbaş, Ayça; Ozmen, Meral; Kayserili, Hülya

    2008-01-01

    Ataxia with vitamin E deficiency (AVED) is a rare autosomal recessive disorder, usually with a phenotype resembling Friedreich ataxia, caused by selective impairment of gastrointestinal vitamin E absorption. Vitamin E supplementation improves symptoms and prevents disease progress. In North Africa and Southern Europe, AVED is as common as Friedreich ataxia. There are no reported cases from Turkey. We herein report a 16-year-old Turkish girl with AVED, who was found to have total deletion of the TTPA gene as well as sensorineural deafness, and we present her follow-up data after vitamin E therapy.

  2. Marinesco-Sjogren Syndrome With Sensori Neural Deafness And Primary Optic Atrophy

    Directory of Open Access Journals (Sweden)

    Aleem M A

    1999-01-01

    Full Text Available Marinesco-Sjogren syndrome (MSS is a rare genetically determined disorder characterised by bilateral cataract, cerebellar ataxia and mental deficiency. The pattern of inheritance is autosomal recessive but it may be variable. In MSS association of hyperlactacidaemia and hypopyruvicaemia, a defective oxidative phosphorylation in mitochondria, is supposed. We are reporting three patients of MSS along with sensorineural deafness and optic atrophy from a single Indian family.

  3. Mobile phone usage does not affect sudden sensorineural hearing loss.

    Science.gov (United States)

    Sagiv, D; Migirov, L; Madgar, O; Nakache, G; Wolf, M; Shapira, Y

    2018-01-01

    Recent studies found that mobile phone users had a significantly greater risk of having elevated thresholds in speech frequencies. This study investigated the correlation between the laterality of sudden sensorineural hearing loss, handedness and the preferred ear for mobile phone use. The study included all patients who presented with sudden sensorineural hearing loss to the Department of Otolaryngology - Head and Neck Surgery in our tertiary referral medical centre between 2014 and 2016. Patients were asked to indicate their dominant hand and preferred ear for mobile phone use. The study comprised 160 patients. No correlation was found between the dominant hand or preferred ear for mobile phone use and the side of sudden sensorineural hearing loss. There was no correlation between the side of the sudden sensorineural hearing loss (preferable or non-preferable for mobile phone use) and audiometric characteristics. No correlation was found between the laterality of ears used for mobile phone and sudden sensorineural hearing loss.

  4. Does Deafness Affect Resilience?

    Directory of Open Access Journals (Sweden)

    Ahmadi

    2015-10-01

    Full Text Available Background Resilience is a positive psychological characteristic that contributes to mental health and adjustment under challenging conditions, such as deafness. Deafness is a traumatic experience and causes communication disorders; it may also affect resilience. Objectives We compared the resilience of deaf signers to that of a matched group of hearing individuals. Materials and Methods This comparative study was performed to assess self-evaluated resilience in 45 deaf signers and in 76 matched hearing subjects from Hamadan, Iran. Resilience scores were measured using a modified connor-davidson resilience scale. Results The average resilience score was 61.20 in deaf signers and 62.8 in hearing subjects; however, this difference was not statistically significant. The resilience score was different in female and male deaf participants. It was 65.22 for male deaf participants (SD = 10.4 and 55.17 for female deaf participants (SD = 16.1, and this difference was statistically significant (P = 0.014. The gender difference between the resilience scores of hearing participants (male, 66.24 [SD = 16.7] and female, 59.36 [SD = 13.9] was not significant (P = 0.057. Discussion Similar resilience scores in deaf and hearing participants may be due to appropriate interaction of deaf signers with family members and society. Male deaf subjects were more resilient than female ones; studies should be done to examine the effects of cultural characteristics that may provide females with less communication opportunities than males.

  5. The Application of PECS in a Deaf Child with Autism: A Case Study

    Science.gov (United States)

    Malandraki, Georgia A.; Okalidou, Areti

    2007-01-01

    A 10-year-old nonverbal Greek boy, C.Z., who had been diagnosed with both bilateral sensorineural profound hearing loss and autism, was taught to use the Picture Exchange Communication System (PECS), with some modifications and extensions, over a 4-month intensive intervention period. C.Z.'s original communication and behavioral status as well as…

  6. Developing Written Literacy in Deaf Children through Analyzing Sign Language.

    Science.gov (United States)

    Akamatsu, Carol Tane; Armour, Victoria A.

    1987-01-01

    The effects of complementary sign language instruction on three measures of literacy were evaluated with six severely to profoundly deaf high school residential students. Instruction involved comparing American Sign Language and English, literal and figurative translation, and editing and grammatical English skills. Positive results have…

  7. Spelling of Deaf Children Who Use Cochlear Implants

    Science.gov (United States)

    Hayes, Heather; Kessler, Brett; Treiman, Rebecca

    2011-01-01

    The spellings of 39 profoundly deaf users of cochlear implants, aged 6 to 12 years, were compared with those of 39 hearing peers. When controlled for age and reading ability, the error rates of the 2 groups were not significantly different. Both groups evinced phonological spelling strategies, performing better on words with more typical…

  8. Factors Affecting Auditory Performance of Postlinguistically Deaf Adults Using Cochlear Implants : An Update with 2251 Patients

    NARCIS (Netherlands)

    Blamey, Peter; Artieres, Franoise; Başkent, Deniz; Bergeron, Francois; Beynon, Andy; Burke, Elaine; Dillier, Norbert; Dowell, Richard; Fraysse, Bernard; Gallego, Stephane; Govaerts, Paul J.; Green, Kevin; Huber, Alexander M.; Kleine-Punte, Andrea; Maat, Bert; Marx, Mathieu; Mawman, Deborah; Mosnier, Isabelle; O'Connor, Alec Fitzgerald; O'Leary, Stephen; Rousset, Alexandra; Schauwers, Karen; Skarzynski, Henryk; Skarzynski, Piotr H.; Sterkers, Olivier; Terranti, Assia; Truy, Eric; Van de Heyning, Paul; Venail, Frederic; Vincent, Christophe; Lazard, Diane S.

    2013-01-01

    Objective: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome. Study Design:

  9. THE DEAFNESS, THE DEAF AND HIS DISCURSE

    Directory of Open Access Journals (Sweden)

    Neuma Chaveiro

    2006-12-01

    Full Text Available ABSTRACT: The knowledge of LIBRAS – Brazilian Sign Language – is determining to the process of structuring the discursive formations and to the constitution of the deaf one. The present work aims to discuss the LIBRAS as an important tool of structuring the discursive of the deaf individual an as a facilitating factor in the description of his health problems. The data constitute two texts, one of them wrote by a deaf skilled in LIBRAS and the other wrote by a deaf who does not express himself in this kind of language. It was verified that the first text’s author is consistent, has mobility and moves easily through the discursive formations, but otherwise is the text belonging to the other patient. It can be stated that the Brazilian sign language – LIBRAS – is a decisive tool in the working out of the deaf discursive formations and the comprehension of his discourse by the health area professionals provide a larger understanding of the constitution of the deaf identity, a fundamental aspect for an improvement in the services offered in the health area. KEYWORDS: Deafness; Communication; Sign Language.

  10. Aspects of the linguistic competence of deaf children.

    Science.gov (United States)

    Wood, D

    1984-02-01

    In this paper the following three issues are considered in the light of recent research: Do severely/profoundly deaf children develop a grammar for English? Evidence is presented which suggests that they do and that this grammar displays a number of general characteristics some of which parallel the language of younger hearing children, others that do not. The limitations of this grammar will be discussed and hypotheses about the nature, origins and inevitability of such limitations explored. In what ways do the linguistic experiences of deaf children differ from that of hearing children? Some aspects of the deaf child's experience of language will be explored and their linguistic and psychological implications discussed. The relationships between current research findings on the linguistic development of the deaf and the possibility of improved educational methods will also be explored to consider the various different philosophies currently being debated in this field.

  11. Sensorineural hearing loss in hemorrhagic dengue?

    Directory of Open Access Journals (Sweden)

    Bruna Natália Freire Ribeiro

    2015-01-01

    Discussion and conclusion: This is the first case report that brings together DHF and sudden hearing loss. In the development of this case no other cause to sudden hearing loss was found and the correlation between dengue and hearing loss was questioned. In the literature review was found that some viruses, as mumps virus, varicella-zoster virus and HSV-1 and HSV-2 are related to sudden hearing loss, all of them fit in the viral theory. Besides the viral theory of sudden hearing loss, there is the vascular theory that is the occlusion of the end artery that supplies the cochlea. DHF has a vascular commitment, and the hypothesis of a vascular cause could be elicited in this case. Many studies in this area are needed and this article has the objective of elicit the discussion about the subject. Could dengue be associated with sensorineural hearing loss?

  12. The relative contributions of speechreading and vocabulary to deaf and hearing children's reading ability.

    Science.gov (United States)

    Kyle, Fiona Elizabeth; Campbell, Ruth; MacSweeney, Mairéad

    2016-01-01

    Vocabulary knowledge and speechreading are important for deaf children's reading development but it is unknown whether they are independent predictors of reading ability. This study investigated the relationships between reading, speechreading and vocabulary in a large cohort of deaf and hearing children aged 5 to 14 years. 86 severely and profoundly deaf children and 91 hearing children participated in this study. All children completed assessments of reading comprehension, word reading accuracy, speechreading and vocabulary. Regression analyses showed that vocabulary and speechreading accounted for unique variance in both reading accuracy and comprehension for deaf children. For hearing children, vocabulary was an independent predictor of both reading accuracy and comprehension skills but speechreading only accounted for unique variance in reading accuracy. Speechreading and vocabulary are important for reading development in deaf children. The results are interpreted within the Simple View of Reading framework and the theoretical implications for deaf children's reading are discussed. Copyright © 2015 Elsevier Ltd. All rights reserved.

  13. Sensorineural hearing loss in Lassa fever: two case reports

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    Okokhere Peter O

    2009-01-01

    Full Text Available Abstract Introduction Lassa fever is an acute arena viral haemorrhagic fever with varied neurological sequelae. Sensorineural hearing loss is one of the rare complications which occur usually during the convalescent stage of the infection. Case presentation The cases of two female patients aged 19 and 43 years old, respectively, with clinical features suggestive of Lassa fever and confirmed by immunoserological/Lassa-virus-specific reverse transcriptase polymerase chain reaction are presented. Both patients developed severe sensorineural hearing loss at acute phases of the infections. Conclusion Sensorineural hearing loss from Lassa fever infections can occur in both acute and convalescent stages and is probably induced by an immune response.

  14. Libraryservice for the deaf

    OpenAIRE

    Forssell, Beatrice

    2011-01-01

    The aim of this two years master thesis is to explore the relationship between public libraries and deaf adults. I want to know if deaf adults are a prioritized group in Swedish public libraries. Deaf adults use Swedish sign language witch is a visual language and differs from Swedish. I discuss the difference in Swedish Sign Language and Swedish and the importance of Sign Language literature. I also explore what methods can be used for mak-ing the library service towards the deaf group more ...

  15. Perspectives for the treatment of sensorineural hearing loss by cellular regeneration of the inner ear.

    Science.gov (United States)

    Almeida-Branco, Mario S; Cabrera, Sonia; Lopez-Escamez, Jose A

    2015-01-01

    Sensorineural hearing loss is a caused by the loss of the cochlear hair cells with the consequent deafferentation of spiral ganglion neurons. Humans do not show endogenous cellular regeneration in the inner ear and there is no exogenous therapy that allows the replacement of the damaged hair cells. Currently, treatment is based on the use of hearing aids and cochlear implants that present different outcomes, some difficulties in auditory discrimination and a limited useful life. More advanced technology is hindered by the functional capacity of the remaining spiral ganglion neurons. The latest advances with stem cell therapy and cellular reprogramming have developed several possibilities to induce endogenous regeneration or stem cell transplantation to replace damaged inner ear hair cells and restore hearing function. With further knowledge of the cellular and molecular biology of the inner ear and its embryonic development, it will be possible to use induced stem cells as in vitro models of disease and as replacement cellular therapy. Investigation in this area is focused on generating cellular therapy with clinical use for the treatment of profound sensorineural hearing loss. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

  16. Variables with prognostic value in the onset of idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Bogaz, Eduardo Amaro; Maranhão, André Souza de Albuquerque; Inoue, Daniel Paganini; Suzuki, Flavia Alencar de Barros; Penido, Norma de Oliveira

    2015-01-01

    The establishment of an individualized prognostic evaluation in patients with a diagnosis of idiopathic sudden sensorineural hearing loss (ISSHL) remains a difficult and imprecise task, due mostly to the variety of etiologies. Determining which variables have prognostic value in the initial assessment of the patient would be extremely useful in clinical practice. To establish which variables identifiable at the onset of idiopathic sudden sensorineural hearing loss have prognostic value in the final hearing recovery. Prospective, longitudinal cohort study. Patients with ISSHL followed by the Department of Otology-Neurotology of a quaternary hospital were included. The following variables were evaluated and correlated with final hearing recovery: age, gender, vertigo, tinnitus, initial degree of hearing loss, contralateral ear hearing, and elapsed time to treatment. 127 patients with ISSHL were evaluated. Rates of absolute and relative recovery were 23.6dB and 37.2% respectively. Complete hearing improvement was observed in 15.7% patients; 27.6% demonstrated significant improvement and improvement was noted in 57.5%. During the onset of ISSHL, the following variables were correlated with a worse prognosis: dizziness, profound hearing loss, impaired hearing in the contralateral ear, and delay to start treatment. Tinnitus at the onset of ISSHL correlated with a better prognosis. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  17. When Lightning Strikes Twice: Profoundly Gifted, Profoundly Accomplished.

    Science.gov (United States)

    Makel, Matthew C; Kell, Harrison J; Lubinski, David; Putallaz, Martha; Benbow, Camilla P

    2016-07-01

    The educational, occupational, and creative accomplishments of the profoundly gifted participants (IQs ⩾ 160) in the Study of Mathematically Precocious Youth (SMPY) are astounding, but are they representative of equally able 12-year-olds? Duke University's Talent Identification Program (TIP) identified 259 young adolescents who were equally gifted. By age 40, their life accomplishments also were extraordinary: Thirty-seven percent had earned doctorates, 7.5% had achieved academic tenure (4.3% at research-intensive universities), and 9% held patents; many were high-level leaders in major organizations. As was the case for the SMPY sample before them, differential ability strengths predicted their contrasting and eventual developmental trajectories-even though essentially all participants possessed both mathematical and verbal reasoning abilities far superior to those of typical Ph.D. recipients. Individuals, even profoundly gifted ones, primarily do what they are best at. Differences in ability patterns, like differences in interests, guide development along different paths, but ability level, coupled with commitment, determines whether and the extent to which noteworthy accomplishments are reached if opportunity presents itself. © The Author(s) 2016.

  18. Googling "Deaf": Deafness in the World's English-Language Press

    Science.gov (United States)

    Power, Des

    2007-01-01

    An Internet search tool, Google Alert, was used to survey the global English-language press July-December 2005 for references to deaf people. The survey found that such references focus on people who are deaf rather than the disability itself, thus demonstrating how well deaf people fit into the mainstream. Derogatory terminology such as "deaf and…

  19. Intratympanic Methylprednisolone Injection as First Line Therapy for Idiopathic Sudden Sensorineural Hearing Loss

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    Mukul Patar

    2017-08-01

    Full Text Available Introduction Steroid therapy is considered to be the gold standard for sudden sensorineural hearing loss (SSNHL. Delivering steroids by intratympanic injection is more efficient than systemic injections with minimum or no side effects. The present study was aimed to evaluate the efficacy and safety of intratympanic methylprednisolone injections as initial first line therapy for unilateral idiopathic SSNHL and the ease of giving it by otoendoscopy. Materials and Methods A prospective analysis was performed for the patients diagnosed as unilateral idiopathic SSNHL from April 2014 to April 2016 and receiving intratympanic steroids injections as first line therapy. Patients with unilateral sensorineural hearing loss of at least 30 dB at 3 contiguous frequencies occurring within a period of not more than 3 days are only included. All of the intratympanic steroid (ITS injections were administered as OPD procedures. Each patient was treated by 3 injections given at 3 days interval.  Results A total of 22 patients who underwent primary intratympanic steroid (ITS injection for unilateral SSNHL during the study period were included in the study. The mean age was 42.22 years (+ 9.79 and age ranged from 27 to 68 years. Patients included in our study came within 2nd to 27th day of occurrence of deafness and the mean duration (days from onset of disease to start of ITS was 7.86 days. The average hearing gain in our study was 44.22 dB. In the present study 11 patients (50% showed complete hearing improvement and 10 cases (45.45% had partial and one (4.54% showed no hearing recovery at 3 weeks follow up period. Conclusion Minimal systemic absorption with minimum or no systemic effects and high percentage of success rate encouraged the surgeons to prefer ITS as primary therapy for idiopathic unilateral SSNHL. It is effective, cheap, well-tolerated and can be performed as OPD procedure.

  20. Negotiating Deaf Bodies and Corporeal Experiences: The Cybernetic Deaf Subject

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    Thomas P. Horejes

    2013-04-01

    Full Text Available Deaf people negotiate their embodiment through corporeal experiences to provide a perception of what it means to be human. Some deaf people search for a framework where being deaf is human, not a disability. Other deaf people experience their deafness as a disability and use technology as a means to negotiate their embodiment and experiences. The role of technology or cybernetics, particularly cochlear implants, for the deaf will be examined as a way to understand cultural identities and diverse ideological perspectives concerning what it means to be deaf and normal. Then, this paper focuses on social constructed ‘bodies’ for the deaf using embodied theory and action as a part of a theoretical framework to showcase theoretical ideas and actualities of some deaf people’s lives and experiences. These discussions are ways to open dialogues and collaborative inquiries on larger important issues such as what it means to be deaf and, in essence, human.

  1. Sensorineural hearing loss in Kawasaki disease.

    Science.gov (United States)

    Aggarwal, Varun; Etinger, Veronica; Orjuela, Andres F

    2016-01-01

    Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2(nd) week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well.

  2. Relation between Glaucoma and Sensorineural Hearing Loss

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    A Mollasadeghi

    2008-01-01

    Full Text Available Introduction: Glaucoma is one of the leading causes of blindness throughout the world. Some studies have suggested a relationship between glaucoma and sensorineural hearing loss, while others have found no evidence of an association. We performed a study to determine whether there is a significant difference in hearing of patients with glaucoma and a match control population. Methods: In this cross-sectional study, from February, 2005 till April, 2006, 44 patients with glaucoma were studied. The age range was between 15 to 60 years. After taking a complete medical history, those suffering from presbycusis, history of exposure to ototoxic drugs and substances and history of ear surgery were excluded from the study. All of the patients were cases of open-angle glaucoma, and were surveyed separately for normal-pressure glaucoma. Then complete audiometric tests (PTA, SDS, SRT, Impedance were conducted for all of them, and the results compared with a control group. Results: There was no statistically significant difference between the case group and control group in PTA, SDS, and SRT, except for Normal Tension Glaucoma (NTG. There wasn't any statistically significant difference between two groups with respect to age, gender, and history of diseases. In the NTG group, significant difference was seen only in high frequencies. Conclusion: As mentioned, there was a statistically significant difference between NTG group and control group. It is therefore recommended to conduct complete audiometric tests and histopathologic examinations in this group for early detection of hearing loss and application of rehabilitative measures.

  3. [Bilateral versus unilateral sudden sensorineural hearing loss].

    Science.gov (United States)

    Ni, Maomei; Li, Dehong; Peng, Weihui; Peng, Yikun; Ren, Juanjuan

    2010-01-01

    To analyze the clinical characteristics and treatment effect between bilateral (bi-) and unilateral (uni-) sudden sensorineural hearing loss (SSNHL). Four hundred and eighty cases of SSNHL were retrospective study, which were divided into two groups of bi-SSNHL (n = 40) and uni-SSNHL (n = 440). Clinical characteristics and treatment effects were compared of the two groups. The incidence rate of bi-SSNHL was 8.3 percent and uni-SSNHL was 91.7 percent of all patients with SSNHL. Bi-SSNHL occurs more commonly in patients of old age, diabetes mellitus, and lipid panes abnormalities compared to uni-SSNHL. Twenty-eight ears in the bi-SSNHL group showed hearing recovery (35%), compared with 56.4 percent of patients with uni-SSNHL. Bi-SSNHL and uni-SSNHL may have a completely different clinical characteristics and treatment effect, that implies a different pathophysiology and prognosis. Recognition their different clinical characteristics and treatment effect between bilateral and unilateral SSNHL can help in counseling and managing the patients and correctly evaluate the prognosis.

  4. Cancer Prevention Knowledge of People with Profound Hearing Loss

    Science.gov (United States)

    Meador, Helen E.; Reed, Barbara D.; Sen, Ananda; Gorenflo, Daniel W.

    2009-01-01

    BACKGROUND Deaf persons, a documented minority population, have low reading levels and difficulty communicating with physicians. The effect of these on their knowledge of cancer prevention recommendations is unknown. METHODS A cross-sectional study of 222 d/Deaf persons in Michigan, age 18 and older, chose one of four ways (voice, video of a certified American Sign Language interpreter, captions, or printed English) to complete a self-administered computer video questionnaire about demographics, hearing loss, language history, health-care utilization, and health-care information sources, as well as family and social variables. Twelve questions tested their knowledge of cancer prevention recommendations. The outcome measures were the percentage of correct answers to the questions and the association of multiple variables with these responses. RESULTS Participants averaged 22.9% correct answers with no gender difference. Univariate analysis revealed that smoking history, types of medical problems, last physician visit, and women having previous cancer preventive tests did not affect scores. Improved scores occurred with computer use (p = 0.05), higher education (p English in multiple situations (p English use (p = 0.01) and believing that smoking was bad (p = 0.05) were associated with improved scores. CONCLUSION Persons with profound hearing loss have poor knowledge of recommended cancer prevention interventions. English use in multiple settings was strongly associated with increased knowledge. PMID:19132325

  5. Changes of the directional brain networks related with brain plasticity in patients with long-term unilateral sensorineural hearing loss.

    Science.gov (United States)

    Zhang, G-Y; Yang, M; Liu, B; Huang, Z-C; Li, J; Chen, J-Y; Chen, H; Zhang, P-P; Liu, L-J; Wang, J; Teng, G-J

    2016-01-28

    Previous studies often report that early auditory deprivation or congenital deafness contributes to cross-modal reorganization in the auditory-deprived cortex, and this cross-modal reorganization limits clinical benefit from cochlear prosthetics. However, there are inconsistencies among study results on cortical reorganization in those subjects with long-term unilateral sensorineural hearing loss (USNHL). It is also unclear whether there exists a similar cross-modal plasticity of the auditory cortex for acquired monaural deafness and early or congenital deafness. To address this issue, we constructed the directional brain functional networks based on entropy connectivity of resting-state functional MRI and researched changes of the networks. Thirty-four long-term USNHL individuals and seventeen normally hearing individuals participated in the test, and all USNHL patients had acquired deafness. We found that certain brain regions of the sensorimotor and visual networks presented enhanced synchronous output entropy connectivity with the left primary auditory cortex in the left long-term USNHL individuals as compared with normally hearing individuals. Especially, the left USNHL showed more significant changes of entropy connectivity than the right USNHL. No significant plastic changes were observed in the right USNHL. Our results indicate that the left primary auditory cortex (non-auditory-deprived cortex) in patients with left USNHL has been reorganized by visual and sensorimotor modalities through cross-modal plasticity. Furthermore, the cross-modal reorganization also alters the directional brain functional networks. The auditory deprivation from the left or right side generates different influences on the human brain. Copyright © 2015 IBRO. Published by Elsevier Ltd. All rights reserved.

  6. Detection of Deafness-Causing Mutations in the Greek Mitochondrial Genome

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    Haris Kokotas

    2011-01-01

    Full Text Available Mitochondrion harbors its own DNA, known as mtDNA, encoding certain essential components of the mitochondrial respiratory chain and protein synthesis apparatus. mtDNA mutations have an impact on cellular ATP production and many of them are undoubtedly a factor that contributes to sensorineural deafness, including both syndromic and non-syndromic forms. Hot spot regions for deafness mutations are the MTRNR1 gene, encoding the 12S rRNA, the MTTS1 gene, encoding the tRNA for Ser(UCN, and the MTTL1 gene, encoding the tRNA for Leu(UUR. We investigated the impact of mtDNA mutations in the Greek hearing impaired population, by testing a cohort of 513 patients suffering from childhood onset prelingual or postlingual, bilateral, sensorineural, syndromic or non-syndromic hearing loss of any degree for six mitochondrial variants previously associated with deafness. Screening involved the MTRNR1 961delT/insC and A1555G mutations, the MTTL1 A3243G mutation, and the MTTS1 A7445G, 7472insC and T7510C mutations. Although two patients were tested positive for the A1555G mutation, we failed to identify any subject carrying the 961delT/insC, A3243G, A7445G, 7472insC, or T7510C mutations. Our findings strongly support our previously raised conclusion that mtDNA mutations are not a major risk factor for sensorineural deafness in the Greek population.

  7. Enhancement of visual motion detection thresholds in early deaf people.

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    Martha M Shiell

    Full Text Available In deaf people, the auditory cortex can reorganize to support visual motion processing. Although this cross-modal reorganization has long been thought to subserve enhanced visual abilities, previous research has been unsuccessful at identifying behavioural enhancements specific to motion processing. Recently, research with congenitally deaf cats has uncovered an enhancement for visual motion detection. Our goal was to test for a similar difference between deaf and hearing people. We tested 16 early and profoundly deaf participants and 20 hearing controls. Participants completed a visual motion detection task, in which they were asked to determine which of two sinusoidal gratings was moving. The speed of the moving grating varied according to an adaptive staircase procedure, allowing us to determine the lowest speed necessary for participants to detect motion. Consistent with previous research in deaf cats, the deaf group had lower motion detection thresholds than the hearing. This finding supports the proposal that cross-modal reorganization after sensory deprivation will occur for supramodal sensory features and preserve the output functions.

  8. Comparing Motor Development of Deaf Children of Deaf Parents and Deaf Children of Hearing Parents

    Science.gov (United States)

    Lieberman, Lauren J.; Volding, Lori; Winnick, Joseph P.

    2004-01-01

    Deaf children of Deaf parents perform better academically (Ritter-Brinton & Stewart, 1992), linguistically (Courtin, 2000; M. Harris, 2001; Vaccari & Marschark, 1997), and socially (Hadadian & Rose, 1991; M. Harris, 2001) than Deaf children of hearing parents. Twenty-nine Deaf children in residential schools were assessed to determine if a…

  9. Language and cognitive development in deaf children: deaf children with deaf and deaf children with hearing parents

    OpenAIRE

    Ajda Pfifer

    2011-01-01

    The article reviews the current studies regarding language and cognitive development in children who are deaf. Deaf communicate orally and with sign language. 90 % of deaf children are born into hearing families and hearing parents in most cases do not know the sign language. Besides, hearing parents usually want for their child to become "normally" speaking. Most of the deaf children born into hearing families have very poor early communication. It is now well established that deaf children ...

  10. Sustained attention, selective attention and cognitive control in deaf and hearing children.

    Science.gov (United States)

    Dye, Matthew W G; Hauser, Peter C

    2014-03-01

    Deaf children have been characterized as being impulsive, distractible, and unable to sustain attention. However, past research has tested deaf children born to hearing parents who are likely to have experienced language delays. The purpose of this study was to determine whether an absence of auditory input modulates attentional problems in deaf children with no delayed exposure to language. Two versions of a continuous performance test were administered to 37 deaf children born to Deaf parents and 60 hearing children, all aged 6-13 years. A vigilance task was used to measure sustained attention over the course of several minutes, and a distractibility test provided a measure of the ability to ignore task irrelevant information - selective attention. Both tasks provided assessments of cognitive control through analysis of commission errors. The deaf and hearing children did not differ on measures of sustained attention. However, younger deaf children were more distracted by task-irrelevant information in their peripheral visual field, and deaf children produced a higher number of commission errors in the selective attention task. It is argued that this is not likely to be an effect of audition on cognitive processing, but may rather reflect difficulty in endogenous control of reallocated visual attention resources stemming from early profound deafness. Copyright © 2013 Elsevier B.V. All rights reserved.

  11. Young Skilled Deaf Readers Have an Enhanced Perceptual Span in Reading.

    Science.gov (United States)

    Bélanger, Nathalie N; Lee, Michelle; Schotter, Elizabeth R

    2017-04-27

    Recently, Bélanger, Slattery, Mayberry and Rayner (2012) showed, using the moving window paradigm, that profoundly deaf adults have a wider perceptual span during reading relative to hearing adults matched on reading level. This difference might be related to the fact that deaf adults allocate more visual attention to simple stimuli in the parafovea (Bavelier, Dye & Hauser, 2006). Importantly, this reorganization of visual attention in deaf individuals is already manifesting in deaf children (Dye, Hauser & Bavelier, 2009). This leads to questions about the time course of the emergence of an enhanced perceptual span (which is under attentional control; Rayner, 2014; Miellet, O'Donnell, & Sereno, 2009) in young deaf readers. The present research addressed this question by comparing the perceptual spans of young deaf readers (age 7-15) and young hearing children (age 7-15). Young deaf readers, like deaf adults, were found to have a wider perceptual span relative to their hearing peers matched on reading level, suggesting that strong and early reorganization of visual attention in deaf individuals goes beyond the processing of simple visual stimuli and emerges into more cognitively complex tasks, such as reading.

  12. [Acute low tone sensorineural hearing loss caused by inner ear barotrauma].

    Science.gov (United States)

    Abe, T; Sasamori, S; Oikawa, T; Aigami, T; Endo, Y; Matsuki, C

    1989-09-01

    Three cases of inner ear barotrauma with subjective symptoms and hearing impairment which were similar to the low tone sudden deafness were reported. Case 1 was a 34-year-old man who developed a hearing loss in the next morning of taking an airplane and recovered four days after. Case 2 was a 42-year-old man who developed a hearing loss 2 days after flying in an airplane and hearing loss have recurred 4 times in his right ear for 3 months. Eight months after recovery of previous recurrent attack, a hearing loss occurred in his left ear without flying and recurred twice for 3 weeks. Case 3 was a 25-year-old woman who developed a hearing loss in the right ear after 24 meter depth scuba diving and recurred 4 times for 40 days. Those three patients complained of no vertigo at any attacks and were treated conservatively. From previous reports and the onset and the course of hearing disturbance, acute low tone sensorineural hearing loss in case 1 and case 2 was thought to be caused by circulatory disturbance of the inner ear and in case 3 thought to be caused by inner ear window rupture. But, endolymphatic hydrops was also needed to be take into account in those three cases as a common possible cause. Inner ear barotrauma and so called labyrinthine window rupture were considered to be one of the diseases needed to differentiate from low tone sudden deafness without reference to mono-attack type or recurrent type.

  13. [Analysis of sensorineural hearing loss in chronic suppurative otitis media].

    Science.gov (United States)

    Feng, Hongyun; Chen, Ying

    2004-10-01

    To explore the association between chronic suppurative otitis media and sensorineural hearing loss. The files of 147 patients with unilateral chronic suppurative media were reviewed in a retrospective study. Differences between diseased and control ear bone conduction thresholds were analysed by the paired student's t-test over the four frequencies (0.5 kHz, 1.0 kHz, 2.0 kHz, 4.0 kHz). The effect of the presence of cholesteatoma and ossicular erosion on sensorineural hearing loss over the speech frequencies (0.5 kHz, 1.0 kHz, 2.0 kHz) and 4.0 kHz were analysed by one-way ANOVA. Linear regression models were used to clarify the relationships between sensorineural hearing loss and chronic suppurative otitis media. The differences in bone conduction threshold between diseased and control ear were statistically significant. Bone conduction threshold shift at speech frequency was associated with the ages. The presence of cholesteatoma and ossicular erosion was not associated with a significantly increased risk of sensorineural hearing loss. Chronic suppurative otitis media can result in sensorineural hearing loss. The higher frequencies are easier affected than the lower frequencies.

  14. Binaural integration of periodically alternating speech following cochlear implantation in subjects with profound sensorineural unilateral hearing loss.

    Science.gov (United States)

    Wesarg, Thomas; Richter, Nicole; Hessel, Horst; Günther, Stefanie; Arndt, Susan; Aschendorff, Antje; Laszig, Roland; Hassepass, Frederike

    2015-01-01

    In cochlear implant (CI) recipients with unilateral hearing loss (UHL) and normal hearing (NH) in the contralateral ear, the central auditory system receives signals of different auditory modalities, i.e. electrically via the CI ear as well as acoustically via the NH ear. The present study investigates binaural integration of bimodal stimulation in the central auditory system of 10 CI subjects with UHL by applying a modified version of the Rapidly Alternating Speech Perception (RASP) test to characterise speech recognition ability under monotic and dichotic listening arrangements. Subsequently, the results for each monotic and dichotic test condition were compared to quantify the binaural benefit from CI usage. The study results demonstrate significantly improved speech recognition under dichotic compared to monotic listening conditions, providing evidence that there is binaural integration of acoustically and electrically transmitted speech segments in the central nervous system at brainstem and cortical levels. In contrast to more commonly used tests of binaural integration, such as localisation, the RASP test provides the clinical option to investigate binaural integration involving structures at the cortical level. © 2015 S. Karger AG, Basel.

  15. Hyperbaric oxygen for sudden sensorineural deafness: when guideline development goes astray

    NARCIS (Netherlands)

    De Ru, J. A.; Koole, A.; Bol Raap, R. D.; Teguh, D. N.; van Hulst, R. A.

    2017-01-01

    Problem: This article uses a clinical example to describe the need, when formulating a guideline, to include all evidence levels in the deliberations leading to the recommendations. Methodology: A narrative review, in which the different viewpoints on a recent guideline from the Dutch Society of

  16. Deafness: Cross-modal plasticity and cochlear implants

    Science.gov (United States)

    Lee, Dong Soo; Lee, Jae Sung; Oh, Seung Ha; Kim, Seok-Ki; Kim, Jeung-Whoon; Chung, June-Key; Lee, Myung Chul; Kim, Chong Sun

    2001-01-01

    Hearing in profoundly deaf people can be helped by inserting an implant into the inner ear to stimulate the cochlear nerve. This also boosts the low metabolic activity of the auditory cortex, the region of the brain normally used for hearing. Other sensory modalities, such as sign language, can also activate the auditory cortex, a phenomenon known as cross-modal plasticity. Here we show that when metabolism in the auditory cortex of prelingually deaf children (whose hearing was lost before they learned to talk) has been restored by cross-modal plasticity, the auditory cortex can no longer respond to signals from a cochlear implant installed afterwards. Neural substrates in the auditory cortex might therefore be routed permanently to other cognitive processes in prelingually deaf patients.

  17. Novel approaches to treating sensorineural hearing loss. Auditory genetics and necessary factors for stem cell transplant.

    Science.gov (United States)

    Vlastarakos, Petros V; Nikolopoulos, Thomas P; Tavoulari, Evangelia; Kiprouli, Catherine; Ferekidis, Eleftherios

    2008-08-01

    Sensorineural hearing loss is a chronic disease, with a serious impact on human communication and quality of life. Exposure to various factors can lead to irreversible hearing impairment, as the auditory epithelium in humans comprises terminally differentiated cells. By contrast, the inner ear of lower vertebrates and invertebrates shows regenerative capacity. Efforts to regenerate the damaged human inner ear may involve renewed cell proliferation, or transplanting cells that can differentiate into sensory cells. Literature review. Animal studies, in vitro studies, retrospective-cohort studies, community-based case-controls, clinical guidelines, and review articles. Embryonic stem cells, inner ear stem cells, and stem cells from other tissues (i.e., neural tissue, hematopoietic system) may be candidates for restoring the auditory epithelium. Transcriptional regulation of p27kip1 is the primary determinant of terminal mitosis and the final number of postmitotic progenitors of hair and supporting cells. Basic helix-loop-helix transcription factor Math1 was found to be necessary and sufficient for the production of auditory hair cells. Notch signaling seems to play a major role in the regulation of Math1, through lateral inhibition. Brn3c, Gfi1, and Barhl1 are also specific transcription factors that have been implicated in hair cell maintenance and consequent survival. Evidence concerning development, maintenance, and regeneration of hair cells is still at an embryonic stage. Combined data, as attempted in the present study, will lead to a more successful management of deafness.

  18. Comprehensive Genetic Analysis of Japanese Autosomal Dominant Sensorineural Hearing Loss Patients.

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    Yoh-Ichiro Iwasa

    Full Text Available In general, autosomal dominant inherited hearing loss does not have a founder mutation, with the causative mutation different in each family. For this reason, there has been a strong need for efficient diagnosis methods for autosomal dominant sensorineural hearing loss (ADSNHL patients. This study sought to verify the effectiveness of our analysis algorithm for the screening of ADSNHL patients as well as the usefulness of the massively parallel DNA sequencing (MPS.Seventy-five Japanese ADSNHL patients from 53 ENT departments nationwide participated in this study. We conducted genetic analysis of 75 ADSNHL patients using the Invader assay, TaqMan genotyping assay and MPS-based genetic screening.A total of 46 (61.3% ADSNHL patients were found to have at least one candidate gene variant.We were able to achieve a high mutation detection rate through the combination of the Invader assay, TaqMan genotyping assay and MPS. MPS could be used to successfully identify mutations in rare deafness genes.

  19. Congenitally deaf children following cochlear implantation.

    Science.gov (United States)

    O'Donoghue, G M; Nikolopoulos, T; Archbold, S M; Tait, M

    1998-01-01

    The aim of this study is to determine the auditory performance of congenitally deaf children following cochlear implantation. A prospective study is undertaken of 71 such children who have been implanted in a dedicated paediatric cochlear implant centre and who have been followed up to 3 years following implantation. All children are aged less than 8 years at the time of implantation and all receive a multichannel cochlear implant system. No child meeting these criteria has been excluded from the study. The average age at implantation is 56.5 months (range 27 to 93 months, standard deviation 15.9 months). Auditory performance is assessed by using the Categories of Auditory Perception (CAP) scale which is developed primarily as a clinical tool for evaluating profoundly deaf young children following cochlear implantation. The median score prior to implantation on this scale is Category 0 (no awareness of environmental sound), at the 1 year interval is Category 4 (discrimination some speech sounds without lip-reading), and at the 2 and 3 year interval, the median score on the CAP scale is Category 5 (understanding of common phrases without lip-reading). These results indicate the ability of cochlear implants to provide significant auditory receptive skills to young congenitally deaf children.

  20. Deafness--the neglected and hidden disability.

    Science.gov (United States)

    Mackenzie, I; Smith, A

    2009-10-01

    The problem of deafness or hearing loss is increasing world-wide. In countries rich and poor, people are living longer, and presbyacusis, the deafness of old age, is becoming more frequent. Hearing loss is a chronic and often life-long disability that, depending on the severity and the frequencies affected, can cause profound damage to the development of speech, language, and cognitive skills in children, especially if commencing prelingually. That damage, in turn, affects the child's progress in school and, later, his or her ability to obtain, keep, and perform an occupation. For all ages and for both sexes, hearing loss causes difficulties with interpersonal communication and leads to significant individual social problems, especially isolation and stigmatization. All of these difficulties are much magnified in developing countries, where there are generally limited services for the hard of hearing, few people trained to help those with hearing loss, and little awareness about how to deal with the difficulties associated with such loss. Although deafness and hearing impairment are likely to have huge economic effects in such countries, in most areas these effects remain to be quantified.

  1. Hearing Disorders and Deafness

    Science.gov (United States)

    ... enough to enjoy talking with friends or family. Hearing disorders make it hard, but not impossible, to ... often be helped. Deafness can keep you from hearing sound at all. What causes hearing loss? Some ...

  2. Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis.

    Science.gov (United States)

    Hall, A C; Leong, A C; Jiang, D; Fitzgerald-O'Connor, A

    2013-07-01

    Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle-Wells syndrome. Previous reports have described the hearing loss to be progressive in nature. To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle-Wells syndrome. The patient underwent a cochlear implantation with a modest outcome. Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

  3. Inattentional deafness in music.

    Science.gov (United States)

    Koreimann, Sabrina; Gula, Bartosz; Vitouch, Oliver

    2014-01-01

    While inattentional blindness is a modern classic in attention and perception research, analogous phenomena of inattentional deafness have been widely neglected. We here present the first investigation of inattentional deafness in and with music under controlled experimental conditions. Inattentional deafness in music is defined as the inability to consciously perceive an unexpected musical stimulus when attention is focused on a certain facet of the piece. Participants listened to a modification of the first 1'50″ of Richard Strauss' Thus Spake Zarathustra; while the control group just listened, the experimental group had to count the number of timpani beats. An e-guitar solo served as the unexpected event. In Study 1, experimental data from n = 115 participants were analyzed. Non-musicians were compared with musicians to investigate the impact of expertise. In Study 2 (n = 47), the scope of the inattentional deafness effect was investigated with a more salient unexpected stimulus. Results demonstrate an inattentional deafness effect under dynamic musical conditions. Quite unexpectedly, the effect was structurally equivalent even for musicians. Our findings clearly show that sustained inattentional deafness exists in the musical realm, in close correspondence to inattentional blindness with dynamic visual stimuli.

  4. Persistent Thalamic Sound Processing Despite Profound Cochlear Denervation

    Directory of Open Access Journals (Sweden)

    Anna R. Chambers

    2016-08-01

    Full Text Available Neurons at higher stages of sensory processing can partially compensate for a sudden drop in input from the periphery through a homeostatic plasticity process that increases the gain on weak afferent inputs. Even after a profound unilateral auditory neuropathy where > 95% of synapses between auditory nerve fibers and inner hair cells have been eliminated with ouabain, central gain can restore the cortical processing and perceptual detection of basic sounds delivered to the denervated ear. In this model of profound auditory neuropathy, cortical processing and perception recover despite the absence of an auditory brainstem response (ABR or brainstem acoustic reflexes, and only a partial recovery of sound processing at the level of the inferior colliculus (IC, an auditory midbrain nucleus. In this study, we induced a profound cochlear neuropathy with ouabain and asked whether central gain enabled a compensatory plasticity in the auditory thalamus comparable to the full recovery of function previously observed in the auditory cortex (ACtx, the partial recovery observed in the IC, or something different entirely. Unilateral ouabain treatment in adult mice effectively eliminated the ABR, yet robust sound-evoked activity persisted in a minority of units recorded from the contralateral medial geniculate body (MGB of awake mice. Sound-driven MGB units could decode moderate and high-intensity sounds with accuracies comparable to sham-treated control mice, but low-intensity classification was near chance. Pure tone receptive fields and synchronization to broadband pulse trains also persisted, albeit with significantly reduced quality and precision, respectively. MGB decoding of temporally modulated pulse trains and speech tokens were both greatly impaired in ouabain-treated mice. Taken together, the absence of an ABR belied a persistent auditory processing at the level of the MGB that was likely enabled through increased central gain. Compensatory

  5. [Multi-center study on the treatment of sudden deafness accompanied with tinnitus].

    Science.gov (United States)

    Zhao, Hui; Dong, Hong; Cheng, Yan; Ma, Yuanxu; Lin, Peng; Zhong, Shixun; Kang, Houyong; Qian, Yi; Hu, Guohua

    2015-06-01

    To investigate the clinical characteristics and the effects of different drug therapies in patients of sudden deafness accompanied with tinnitus. The international standardized clinical research methods, the unified design and program were used in the study. The patients of sudden deafness accompanied with tinnitus, aged between 18 to 65 years old, were recruited, whose duration was less than two weeks with no medication. The patients were divided into four types according to the hearing curve: type A was acute sensorineural hearing loss in low tone frequencies, type B was acute sensorineural hearing loss in high tone frequencies, type C was acute sensorineural hearing loss in all frequencies and type D was total deafness. Each type had four different treatment programs, based on the unified designed randomized table. A total of 1024 cases with single side sudden deafness were recruited in the study by 33 hospitals in China from August 2007 to October 2011. Among the 1024 cases, 922 cases were accompanied with tinnitus (90.04%). By classification of audiogram, among the 922 cases, 169 cases were type A (82.44%), 127 cases were type B (90.07%), 370 cases were type C (92.04%), and 256 cases were type D (92.75%). The tinnitus mostly was persistent and low tone tinnitus. The degree of the tinnitus was mostly 2-3 grade. The curative effects of different types were analyzed, type A had the highest rate of 96.18%, type C was 87.75%, type B was 81.51%, and type D had the lowest rate of 75.32%. Significant difference of curative rate between different types was detected (χ² = 125.33, P = 0.000). There had no significant difference between the four different treatment groups (all P > 0.05). In the cases with single side sudden deafness accompanied with tinnitus, the type in low tone frequencies has the best curative effect, followed by the type in all frequencies. The type in high tone frequencies and the total deafness type have poor curative results. The steroid plays a

  6. Language and cognitive development in deaf children: deaf children with deaf and deaf children with hearing parents

    Directory of Open Access Journals (Sweden)

    Ajda Pfifer

    2011-01-01

    Full Text Available The article reviews the current studies regarding language and cognitive development in children who are deaf. Deaf communicate orally and with sign language. 90 % of deaf children are born into hearing families and hearing parents in most cases do not know the sign language. Besides, hearing parents usually want for their child to become "normally" speaking. Most of the deaf children born into hearing families have very poor early communication. It is now well established that deaf children of deaf parents generally exhibit normal patterns of development in social, linguistic and cognitive domains relative to their hearing peers. One of the longest-running debates in the field of deaf education was whether introducing young deaf children to sign language impairs their ability and motivation for learning spoken language. Today we have no evidence supporting the hypothesis of a negative effect of sign language on development of spoken langugage.

  7. Steroid Use in Idiopathic Sudden Sensorineural Hearing Loss: What ...

    African Journals Online (AJOL)

    Idiopathic sudden sensorineural hearing loss is a disease of unknown etiology. Controversy in the literature argues whether the condition should be treated by steroid therapy. In this case study, a Medline literature search was completed to find out if there is any evidence to support its use in this condition.

  8. Ipsilateral café-au-lait macules, deafness, and pituitary microadenoma in a 35-year-old woman.

    Science.gov (United States)

    Valdivieso, Rommel

    2008-02-01

    The association between ipsilateral café-au-lait macules, deafness, and pituitary adenoma has not been described previously. A 35-year-old woman affected since birth with two café-au-lait macules, one measuring 15 x 20 cm, with an irregular shape, covering almost all of the left buttock, and the other measuring 6 x 13 cm on the front side of the left thigh, is described. For as long as the patient can remember, she has been totally deaf in the left ear, diagnosed as 100% congenital sensorineural deafness. In addition, a left-side pituitary microadenoma was detected at the age of 31 years. She was also found to have a transitional lumbosacral vertebra, and multiple dermatofibromas and nevomelanocytic nevi. It is suggested that this association is not a coincidence, but is part of a neurocutaneous syndrome which has not been described previously.

  9. Cortical deafness in multiple sclerosis

    OpenAIRE

    Tabira, T; Tsuji, S; Nagashima, T.; Nakajima, T.; Kuroiwa, Y

    1981-01-01

    Cortical deafness in a patient with multiple sclerosis is reported. Complete recovery from total deafness was seen following stages of auditory agnosia and pure word deafness. The otological and neurophysiological studies suggested lesions in subcortical white matter. This report stresses the rarity of the condition, its subcortical origin and good prognosis.

  10. Meeting global deaf peers, visiting ideal deaf places: deaf ways of education leading to empowerment, an exploratory case study.

    Science.gov (United States)

    De Clerck, Goedele A M

    2007-01-01

    In a flemish case study, deaf role models revealed a moment of awakening, indicated by the Flemish sign WAKE-UP Contact with deaf cultural rhetoric made them wake up, and deconstruct and reconstruct their lives, a process represented by a circle of deaf empowerment. Flemish deaf leaders mentioned acquiring this rhetoric during visits to deaf dream worlds (in Flemish Sign Language, WORLD DREAM): places with ideal conditions for deaf people. Such global deaf encounters (Breivik, Haualand, & Solvang, 2002) lead to the "insurrection of subjugated [deaf] knowledges" (Pease, 2002, p. 33). Whereas deaf education had never provided them with deaf cultural rhetoric and was depositing upon them oppressive societal conventions (Jankowski, 1997), a common sign language (Mottez, 1993) and global deaf experience (Breivik et al., 2002; Murray, in press) in barrier-free environments (Jankowski, 1997) provided deaf ways of deaf education (Erting, 1996; Reilly, 1995).

  11. Ectodermal dysplasia with alopecia, onychodysplasia, hypohidrosis, keratoderma, abnormal teeth and deafness

    Directory of Open Access Journals (Sweden)

    Akhyani Maryam

    2007-01-01

    Full Text Available The ectodermal dysplasias are a heterogeneous group of disorders with primary defect in hair, teeth, nail and sweat gland function. Numerous types have been described and several classifications exist. Here, we present a patient with ectodermal dysplasia with alopecia, dysplastic nails, hypohidrosis, sensorineural deafness, palmoplantar keratoderma, abnormal teeth and dry skin. To our knowledge, combination of all these features in ectodermal dysplasia has not been reported in the past. The etiology is unknown, but consanguinity of parents points to an autosomal recessive inheritance.

  12. Evaluation of Long-Term Cochlear Implant Use in Subjects With Acquired Unilateral Profound Hearing Loss: Focus on Binaural Auditory Outcomes.

    Science.gov (United States)

    Mertens, Griet; De Bodt, Marc; Van de Heyning, Paul

    Cochlear implantation (CI) in subjects with unilateral profound sensorineural hearing loss was investigated. The authors of the present study demonstrated the binaural auditory outcomes in a 12- and 36-month prospective cohort outcome study. The present study aimed to do a long-term (LT) evaluation of the auditory outcomes in an analogous study group. LT evaluation was derived from 12 single-sided deaf (SSD) CI recipients and from 11 CI recipients with asymmetric hearing loss (AHL). A structured interview was conducted with each subjects. Speech perception in noise and sound localization were assessed in a CIOFF and in a CION condition. Four binaural effects were calculated: summation effect (S0N0), squelch effect (S0NCI), combined head shadow effect (SCIN0), and spatial release from masking (SRM). At the LT evaluation, the contribution of a CI or a bone conduction device on speech perception in noise was investigated in two challenging spatial configurations in the SSD group. All (23/23) subjects wore their CI 7 days a week at LT follow-up evaluation, which ranged from 3 to 10 years after implantation. In the SSD group, a significant combined head shadow effect of 3.17 dB and an SRM benefit of 4.33 dB were found. In the AHL group, on the other hand, the summation effect (2.00 dB), the squelch effect (2.67 dB), the combined head shadow effect (3.67 dB), and SRM benefit (2.00 dB) were significant at LT testing. In both the spatial challenging configurations, the speech in noise results was significantly worse in the condition with the bone conduction device compared with the unaided condition. No negative effect was found for the CION condition. A significant benefit in the CION condition was found for sound localization compared with the CIOFF condition in the SSD group and in the AHL group. All subjects wore their CI 7 days a week at LT follow-up evaluation. The presence of binaural effects has been demonstrated with speech in noise testing, sound localization

  13. Diversity of deaf identities.

    Science.gov (United States)

    Bat-Chava, Y

    2000-12-01

    Social Identity Theory (Tajfel, 1981) posits that members of minority groups achieve positive social identity by (a) attempting to gain access to the mainstream through individual mobility or (b) working with other group members to bring about social change. Some people may use a combination of both strategies. Through the use of cluster analysis, the existence of three identities associated with these strategies was discerned in a sample of 267 deaf adults: culturally hearing identity, culturally deaf identity, and bicultural identity, each comprising about a third of the sample. A subset of 56 people were interviewed in depth; excerpts are presented to illustrate the identity types. Qualified support was found for the prediction that people with culturally deaf and bicultural identities would have higher self-esteem.

  14. Developmental dysgraphia with profound hearing impairment: intervention by auditory methods enabled by cochlear implant.

    Science.gov (United States)

    Fukushima, Kunihiro; Kawasaki, Akihiro; Nagayasu, Rie; Kunisue, Kazuya; Maeda, Yukihide; Kariya, Shin; Kataoka, Yuko; Nishizaki, Kazunori

    2008-06-01

    Learning disability combined with hearing impairment (LDHI) is a poor prognostic factor for the language development of hearing impaired children after educational intervention. A typical example of a child with LDHI and effective interventions provided by cochlear implants are presented in this report. A case of congenital cytomegaloviral infection that showed dysgraphia as well as profound deafness was reported and an underlying visual processing problem diagnosed in the present case caused the patient's dysgraphia. The dysgraphia could be circumvented by the use of auditory memory fairly established by a cochlear implant.

  15. [Prospective clinical multi-center study on the treatment of sudden deafness with different typings in China].

    Science.gov (United States)

    2013-05-01

    To investigate the necessity of subtyping for the treatment of sudden deafness. The international standardized clinical research methods, the unified design and program were used in the study. The sudden deafness patients between 18 to 65 years old were recruited, whose duration was less than two weeks with no medication. The patients were divided into four types according to the hearing curve: type A was acute sensorineural hearing loss in low tone frequencies, type B was acute sensorineural hearing loss in high tone frequencies, type C was acute sensorineural hearing loss in all frequencies and type D was total deafness. Each type had four different treatment programs, based on the unified designed randomized table. Total of 1024 cases with single side sudden deafness were recruited in the study by 33 hospitals in China from August 2007 to October 2011, including 492 male cases (48.05%), and 532 females (51.95%). The average age was (41.2 ± 12.8) years old. By classification of audiogram, among the 1024 cases, 205 cases were type A (20.20%), 141 cases were type B (13.77%), 402 cases were type C (39.26%), 276 cases were type D (26.95%). The curative effects of different types were analyzed, type A had the highest rate of 90.73%, type C was 82.59%, type D was 70.29% and type B had the lowest rate of 65.96%. Significant difference of curative rate between different types was detected (χ(2) = 231.58, P = 0.000). Regarding the curative results of the 1024 cases, 378 cases were recovery (36.91%), 229 cases were excellent better (22.36%), 198 cases were better (19.34%) and 219 cases were poor (21.39%). Glucocorticoid was effective for each type and the combined treatment was better than any single medication. Different types of the hearing curves of sudden deafness have different curing effect, it indicates that different types should use different treatments. It'is important that sudden deafness should be treated according to the types of the hearing curve. The type

  16. Literacy and linguistic development in bilingual deaf children: implications of the "and" for phonological processing.

    Science.gov (United States)

    McQuarrie, Lynn; Parrila, Rauno

    2014-01-01

    Cumulating evidence suggests that the establishment of high-quality phonological representations is the cognitive precursor that facilitates the acquisition of language (spoken, signed, and written). The authors present two studies that contrast the nature of bilingual profoundly deaf children's phonological representations derived from a spoken language and from a signed language using the framework of "functional equivalence" as outlined in McQuarrie and Parilla (2009). The authors argue further that a signed-language phonological system is suited in establishing the "functional" representational base that will support reading acquisition for bilingual deaf learners. They highlight rapidly developing empirical research on dual-language interactions between signed language and written language is highlighted, and discuss the need to take such data into account in any discussion of fundamental skills necessary to support reading achievement in bilingual profoundly deaf learners.

  17. Implantes cocleares em crianças portadoras de deficiência auditiva decorrente de meningite Cochlear implant in deaf children due to meningites

    Directory of Open Access Journals (Sweden)

    Maria C. Bevilacqua

    2003-12-01

    and attention deficits as well as inability for learning. Characteristically the hearing loss due to meningitis is bilateral profound sensorineural symmetric and descendent and it is more common in males and in children under 5 years old and it brings severe implications in the emotional, social and development. AIM: To compare the performance of two groups of pre-lingually deaf implanted children, with age varying from 1 year and 10 months to 6 years, with hearing loss due to meningitis and other varied causes. STUDY DESIGN: Transversal cohorte. MATERIAL AND METHOD: 63 profoundly deaf implanted children were evaluated, 25 (40% were male and 38 (60% were females. In 12 cases (19% the hearing loss was caused by meningitis and in 51 cases (81% hearing loss was due to a variety of causes. All participants were submitted to ear nose throat examination and clinical and objective audiological evaluation. CONCLUSIONS: the occurrence of partial insertion was higher in the meningitis group. There were no statically significant differences on word and phoneme recognition as well as in the questionnaire for evaluation of the hearing (MAIS and language (MUSS skills between the groups.

  18. Auditory pathway functioning in prelingual deafness : The clinical consequences for cochlear implantation

    NARCIS (Netherlands)

    Lammers, M.J.W.

    2015-01-01

    Cochlear implantation has proven itself as a very successful treatment for both children and adults with severe to profound hearing loss. Although most patients obtain a high level of speech perception, performance is more variable in prelingually deaf patients, i.e., patients with an onset of

  19. Integrated Educational/Leisure Time Model for Deaf-Blind Children and Youth. Final Report.

    Science.gov (United States)

    Thompson, Marie

    The report describes the accomplishments of a swimming project to teach instructional objectives to deaf blind, severely-to-profoundly retarded students, using nonhandicapped high school and college students who were trained and paid as peer tutors. Tutors recieved hands-on as well as didactic training and were evaluated by means of pretests and…

  20. Literacy and Linguistic Development in Bilingual Deaf Children: Implications of the "and" for Phonological Processing

    Science.gov (United States)

    McQuarrie, Lynn; Parrila, Rauno

    2014-01-01

    Cumulating evidence suggests that the establishment of high-quality phonological representations is the "cognitive precursor" that facilitates the acquisition of language (spoken, signed, and written). The authors present two studies that contrast the nature of bilingual profoundly deaf children's phonological representations derived…

  1. Bone-anchored hearing aid in unilateral inner ear deafness: a study of 20 patients.

    NARCIS (Netherlands)

    Hol, M.K.S.; Bosman, A.J.; Snik, A.F.M.; Mylanus, E.A.M.; Cremers, C.W.R.J.

    2004-01-01

    OBJECTIVE: To evaluate the benefit of a bone-anchored hearing aid (BAHA) contralateral routing of sound (CROS) in 20 patients with unilateral inner ear deafness. SUBJECTS: 21 patients were recruited; 15 had undergone acoustic neuroma surgery and 6 patients had unilateral profound hearing loss due to

  2. Deaf Readers and Phrasal Verbs: Instructional Efficacy of Chunking as a Visual Tool

    Science.gov (United States)

    Atwell, William R.

    2013-01-01

    The purpose of this study was to examine the effectiveness of a visual strategy that of chunking or visually bracketing phrasal verbs in sentences in short stories. A descriptive case study design was used for this study to compare the two instructional strategies. In this study, stories were presented to 14 severely and profound deaf students…

  3. The Deaf Child and Solving Problems of Arithmetic: The Importance of Comprehensive Reading.

    Science.gov (United States)

    Pau, Carrasumada Serrano

    1995-01-01

    The influence of reading comprehension level on solving verbally expressed arithmetic problems was studied with 12 students (ages 8 to 12) who were prelingually and profoundly deaf. Reading comprehension level was related to students' problem-solving level. Verbal arithmetic problems contained linguistic forms that were difficult for deaf…

  4. Fontbonne University: Collaboration in Speech-Language Pathology and Early Intervention in Deaf Education

    Science.gov (United States)

    Rice, Gale; Lenihan, Susan

    2010-01-01

    Children with hearing loss are best served through the collaborative efforts of their parents and a team of professionals. For communication development and educational achievement, speech-language pathologists and teachers of the deaf working together can have a profound impact. A collaborative approach to assessment, direct instruction, and…

  5. Children of Deaf Adults

    NARCIS (Netherlands)

    van den Bogaerde, B.; Baker, A.E.; Gertz, G.; Boudreault, P.

    2016-01-01

    The hearing children of Deaf parents grow up in two cultures with two languages. They are similar to other bilingual, bicultural children in many ways but are special also. They can be in conflict between two worlds and often carry an extra burden of responsibility in functioning as a bridge between

  6. Research on Deafness.

    Science.gov (United States)

    American Annals of the Deaf, 1997

    1997-01-01

    Lists doctoral dissertations that were completed during 1996 that relate to children and adults with hearing impairments, including the education of children who are deaf or hard of hearing, early intervention, hearing loss, teacher inservice training, cochlear implants, substance abuse, sign language, and socialization. (CR)

  7. Sampling the Deaf Experience.

    Science.gov (United States)

    Teller, Henry E.; And Others

    1993-01-01

    Two graduate students in deaf education wore ear plugs for two months to simulate hearing loss, and recorded their experiences and feelings. Excerpts from their journals are presented, commenting on such daily activities as shopping at a mall, watching television, driving, babysitting, and attending a football game. (JDD)

  8. Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss.

    Science.gov (United States)

    Reitsma, Sietze; Stokroos, Robert; Weber, Jacobiene W; van Tongeren, Joost

    2015-12-01

    To present the rare case of a young boy with idiopathic intracranial hypertension presenting with bilateral sensorineural hearing loss developing over several months. This was accompanied by headaches, otalgia, tinnitus, and vertigo. Furthermore, we aim to provide a concise review on this matter, as this report represents the second case in literature of pediatric idiopathic intracranial hypertension presenting with hearing loss. Workup of a 9-year-old boy with bilateral sensorineural hearing loss, including (among others) physical examination, audiometry, diagnostic imaging, and lumbar puncture. Physical examination including fundoscopy as well as imaging showed no abnormalities. At presentation, pure tone audiometry revealed bone conduction thresholds of about 30 dB HL in both ears. Two months later, this declined to about 35 dB HL in both ears. Lumbar puncture revealed an increased intracranial pressure. The boy was thus diagnosed with idiopathic intracranial hypertension. After the lumbar puncture, the otological complaints gradually resolved, and the hearing normalized (bone conduction thresholds of 0-5 dB HL). Although rare, sensorineural hearing loss in the pediatric population together with otalgia, tinnitus, and vertigo can be due to idiopathic intracranial hypertension and as such can be reversible. © The Author(s) 2015.

  9. Current perception threshold and the HAVS Stockholm sensorineural scale.

    Science.gov (United States)

    House, Ron; Krajnak, Kristine; Manno, Michael; Lander, Lina

    2009-10-01

    It is important to determine which tests of sensorineural dysfunction identify the neurological damage from hand-arm vibration exposure. To examine the association between the hand-arm vibration syndrome (HAVS) Stockholm sensorineural scale stages and tests of peripheral neurological function including measurement of current perception threshold (CPT) and nerve conduction. All the subjects were men who were assessed for HAVS with a medical and occupational history and physical examination to determine the Stockholm stage, CPT testing at frequencies of 5, 250 and 2000 Hz for the median and ulnar nerves and measurement of nerve conduction carried out in a blinded fashion. A total of 155 of the 157 recruited subjects agreed to take part in the study, a 99% participation rate. CPT was statistically significantly increased (P Stockholm sensorineural Stages 1 and > or =2 in comparison to Stage 0 for every frequency and nerve combination. However, CPT could not discriminate well between Stages 1 and > or =2. There was no association between median or ulnar neuropathy measured by nerve conduction and the Stockholm stages. Polychotomous multinomial logistic regression indicated that the CPT measurements at 2000 Hz, corresponding to damage to large myelinated nerve fibres, were most predictive of both Stockholm Stages 1 and > or =2 in comparison to Stage 0. Neuropathy measured by nerve conduction was unrelated to the Stockholm scale stages. CPT was increased above Stage 0 but did not distinguish well between the higher stages of the Stockholm scale.

  10. Genetics Home Reference: deafness and myopia syndrome

    Science.gov (United States)

    ... Health Conditions Deafness and myopia syndrome Deafness and myopia syndrome Printable PDF Open All Close All Enable ... view the expand/collapse boxes. Description Deafness and myopia syndrome is a disorder that causes problems with ...

  11. [Clinical analysis of sudden deafness after radiotherapy and chemotherapy in nasopharyngeal carcinoma patients].

    Science.gov (United States)

    Yao, Liangzhong; Liu, Junjie; Pan, Zhiling; Yang, Xiangning; Zhu, Yanli

    2014-04-01

    To investigate the clinical features and therapeutic effects of sudden deafness after radiotherapy combined with chemotherapy in nasopharyngeal carcinoma patients. Clinical data of 42 nasopharyngeal carcinoma patients suffered from sudden deafness after radiotherapy combined with chemotherapy were analyzed retrospectively. Among the 42 patients, 2 showed moderate deafness, 4 presented excessive deafness, 30 suffered from severe deafness, and 6 exhibited profound deafness. The audiogram pattern of 33 patients met with the type of high tone frequencies hearing loss, and that of the rest 9 cases showed hearing loss at all frequencies. All patients received medical therapy combined with hyperbaric oxygen therapy. Of all the cases with hearing loss, 2 were cured, 2 showed excellent recovery, 9 came out partial recovery, and 29 showed no response to the treatment. The total effective rate was 30.95%. For the accompanied symptoms, none of the 30 cases of tinnitus were relieved, 3 out of 10 cases of aural fullness were cured, and the 5 cases of dizziness or vertigo were all improved. The sudden deafness after radiotherapy combined with chemotherapy in patients with nasopharyngeal carcinoma is closely related to radiotherapy. The hearing loss is serious, and the therapeutic effects are not satisfactory.

  12. The effect of side of implantation on unilateral cochlear implant performance in patients with prelingual and postlingual sensorineural hearing loss: A systematic review.

    Science.gov (United States)

    Kraaijenga, V J C; Derksen, T C; Stegeman, I; Smit, A L

    2017-09-25

    Cerebral lateralisation of language processing leads to a right ear advantage in normal hearing subjects. The aim of this study was to present a systematic overview of the effect of implantation side on postoperative cochlear implant performance in patients with symmetrical severe to profound sensorineural hearing loss. PubMed, Embase and The Cochrane Library databases. Databases were searched from database inception up to 9 January 2017 for cochlear implant and side and all synonyms. Title, abstract and full-text of retrieved articles were screened for eligibility. Then, directness of evidence and risk of bias were assessed. For the included articles, study characteristics and outcome data (hearing and language development) were extracted. 2541 unique articles were screened, of which twenty were eligible for critical appraisal. No randomised controlled trials were identified. Twelve studies with a high directness of evidence remained for data extraction. Four of six studies including children with pre-lingual sensorineural hearing loss and four of seven studies investigating adults with postlingual sensorineural hearing loss found a right ear advantage in at least one outcome measurement related to cochlear implant performance. The available evidence on the effect of side of implantation is of low quality, as study populations and outcome measures are heterogeneous. The majority of studies reveals evidence for a right ear advantage in prelingually deafened children as well as postlingually deafened adults. In view of the present evidence and as no left ear advantage was identified, we cautiously advise implanting the cochlear implant in the right ear when other prognostic factors do not favour the left ear and sensorineural hearing loss is symmetrical. © 2017 The Authors. Clinical Otolaryngology Published by John Wiley & Sons Ltd.

  13. Loss of CIB2 Causes Profound Hearing Loss and Abolishes Mechanoelectrical Transduction in Mice

    Directory of Open Access Journals (Sweden)

    Yanfei Wang

    2017-12-01

    Full Text Available Calcium and integrin-binding protein 2 (CIB2 belongs to a protein family with four known members, CIB1 through CIB4, which are characterized by multiple calcium-binding EF-hand domains. Among the family members, the Cib1 and Cib2 genes are expressed in mouse cochlear hair cells, and mutations in the human CIB2 gene have been associated with nonsyndromic deafness DFNB48 and syndromic deafness USH1J. To further explore the function of CIB1 and CIB2 in hearing, we established Cib1 and Cib2 knockout mice using the clustered regularly interspaced short palindromic repeat (CRISPR-associated Cas9 nuclease (CRISPR/Cas9 genome editing technique. We found that loss of CIB1 protein does not affect auditory function, whereas loss of CIB2 protein causes profound hearing loss in mice. Further investigation revealed that hair cell stereocilia development is affected in Cib2 knockout mice. Noticeably, loss of CIB2 abolishes mechanoelectrical transduction (MET currents in auditory hair cells. In conclusion, we show here that although both CIB1 and CIB2 are readily detected in the cochlea, only loss of CIB2 results in profound hearing loss, and that CIB2 is essential for auditory hair cell MET.

  14. Loss of CIB2 Causes Profound Hearing Loss and Abolishes Mechanoelectrical Transduction in Mice.

    Science.gov (United States)

    Wang, Yanfei; Li, Jie; Yao, Xuerui; Li, Wei; Du, Haibo; Tang, Mingliang; Xiong, Wei; Chai, Renjie; Xu, Zhigang

    2017-01-01

    Calcium and integrin-binding protein 2 (CIB2) belongs to a protein family with four known members, CIB1 through CIB4, which are characterized by multiple calcium-binding EF-hand domains. Among the family members, the Cib1 and Cib2 genes are expressed in mouse cochlear hair cells, and mutations in the human CIB2 gene have been associated with nonsyndromic deafness DFNB48 and syndromic deafness USH1J. To further explore the function of CIB1 and CIB2 in hearing, we established Cib1 and Cib2 knockout mice using the clustered regularly interspaced short palindromic repeat (CRISPR)-associated Cas9 nuclease (CRISPR/Cas9) genome editing technique. We found that loss of CIB1 protein does not affect auditory function, whereas loss of CIB2 protein causes profound hearing loss in mice. Further investigation revealed that hair cell stereocilia development is affected in Cib2 knockout mice. Noticeably, loss of CIB2 abolishes mechanoelectrical transduction (MET) currents in auditory hair cells. In conclusion, we show here that although both CIB1 and CIB2 are readily detected in the cochlea, only loss of CIB2 results in profound hearing loss, and that CIB2 is essential for auditory hair cell MET.

  15. Some Functions and Uses of Literacy in the Deaf Community.

    Science.gov (United States)

    Maxwell, Madeline

    1985-01-01

    Analyzes the ways writing is used among the deaf and between deaf and hearing communicators by four groups; deaf adults who sign, families in which parents are hearing and at least one child is deaf, families in which parents are deaf and children are hearing or deaf, and deaf and hearing schoolteachers. (SED)

  16. Constraints on communication in classrooms for the deaf.

    Science.gov (United States)

    Matthews, T J; Reich, C F

    1993-03-01

    One explanation for the relatively low scholastic achievement of deaf students is the character of communication in the classroom. Unlike aural communication methods, line-of-sight methods share the limitation that the receiver of the message must look at the sender. To assess the magnitude of this constraint, we measured the amount of time signers were looked at by potential receivers in typical secondary school classes for the deaf. Videotaped segments indicated that on average the messages sent by teachers and students were seen less than half the time. Students frequently engaged in collateral conversations. The constraints of line-of-sight communication are profound and should be addressed by teaching techniques, classroom layout, and possibly, the use of computer-communication technology.

  17. Profound thrombocytopenia after primary exposure to eptifibatide

    Directory of Open Access Journals (Sweden)

    Nicholas B Norgard

    2010-09-01

    Full Text Available Nicholas B Norgard, Brian T BadgleyUniversity at Buffalo, School of Pharmacy and Pharmaceutical Sciences, Buffalo, NY, USAAbstract: Eptifibatide is a glycoprotein IIb/IIIa receptor antagonist used to reduce the incidence of ischemic events in patients with acute coronary syndromes and those undergoing percutaneous coronary intervention. A minority of patients given eptifibatide develop acute, profound thrombocytopenia (<20,000 cells/mm3 within a few hours of receiving the drug. This case report discusses a patient who developed profound thrombocytopenia within hours of receiving eptifibatide for the first time. The Naranjo algorithm classified the likelihood that this patient’s thrombocytopenia was related to eptifibatide as probable. Profound thrombocytopenia is an uncommon but clinically important complication of eptifibatide. This case report emphasizes the importance of monitoring platelet counts routinely at baseline and within 2–6 hours of eptifibatide administration.Keywords: drug-induced thrombocytopenia, glycoprotein IIb/IIIa antagonists, eptifibatide, thrombocytopenia

  18. Music lessons improve auditory perceptual and cognitive performance in deaf children

    Directory of Open Access Journals (Sweden)

    Françoise eROCHETTE

    2014-07-01

    Full Text Available Despite advanced technologies in auditory rehabilitation of profound deafness, deaf children often exhibit delayed cognitive and linguistic development and auditory training remains a crucial element of their education. In the present cross-sectional study, we assess whether music would be a relevant tool for deaf children rehabilitation. In normal-hearing children, music lessons have been shown to improve cognitive and linguistic-related abilities, such as phonetic discrimination and reading. We compared auditory perception, auditory cognition, and phonetic discrimination between 14 profoundly deaf children who completed weekly music lessons for a period of 1.5 to 4 years and 14 deaf children who did not receive musical instruction. Children were assessed on perceptual and cognitive auditory tasks using environmental sounds: discrimination, identification, auditory scene analysis, auditory working memory. Transfer to the linguistic domain was tested with a phonetic discrimination task. Musically-trained children showed better performance in auditory scene analysis, auditory working memory and phonetic discrimination tasks, and multiple regressions showed that success on these tasks was at least partly driven by music lessons. We propose that musical education contributes to development of general processes such as auditory attention and perception, which, in turn, facilitate auditory-related cognitive and linguistic processes.

  19. Music lessons improve auditory perceptual and cognitive performance in deaf children.

    Science.gov (United States)

    Rochette, Françoise; Moussard, Aline; Bigand, Emmanuel

    2014-01-01

    Despite advanced technologies in auditory rehabilitation of profound deafness, deaf children often exhibit delayed cognitive and linguistic development and auditory training remains a crucial element of their education. In the present cross-sectional study, we assess whether music would be a relevant tool for deaf children rehabilitation. In normal-hearing children, music lessons have been shown to improve cognitive and linguistic-related abilities, such as phonetic discrimination and reading. We compared auditory perception, auditory cognition, and phonetic discrimination between 14 profoundly deaf children who completed weekly music lessons for a period of 1.5-4 years and 14 deaf children who did not receive musical instruction. Children were assessed on perceptual and cognitive auditory tasks using environmental sounds: discrimination, identification, auditory scene analysis, auditory working memory. Transfer to the linguistic domain was tested with a phonetic discrimination task. Musically trained children showed better performance in auditory scene analysis, auditory working memory and phonetic discrimination tasks, and multiple regressions showed that success on these tasks was at least partly driven by music lessons. We propose that musical education contributes to development of general processes such as auditory attention and perception, which, in turn, facilitate auditory-related cognitive and linguistic processes.

  20. Effects of a Wearable, Tactile Aid on Language Comprehension of Prelingual Profoundly Deaf Children.

    Science.gov (United States)

    Proctor, Adele

    Factors influencing the use of nonacoustic aids (such as visual displays and tactile devices) with the hearing impaired are reviewed. The benefits of tactile devices in improving speech reading/lipreading and speech are pointed out. Tactile aids which provide information on rhythm, rate, intensity, and duration of speech increase lipreading and…

  1. Expressive Vocabulary, Morphology, Syntax and Narrative Skills in Profoundly Deaf Children after Early Cochlear Implantation

    Science.gov (United States)

    Boons, Tinne; De Raeve, Leo; Langereis, Margreet; Peeraer, Louis; Wouters, Jan; van Wieringen, Astrid

    2013-01-01

    Practical experience and research reveal generic spoken language benefits after cochlear implantation. However, systematic research on specific language domains and error analyses are required to probe sub-skills. Moreover, the effect of predictive factors on distinct language domains is unknown. In this study, outcomes of 70 school-aged children…

  2. Expressive vocabulary, morphology, syntax and narrative skills in profoundly deaf children after early cochlear implantation

    NARCIS (Netherlands)

    Tinne Boons; Margreet Langereis; prof. Dr. Louis Peeraer; Jan Wouters; van Wieringen; Leo De Raeve

    2013-01-01

    Practical experience and research reveal generic spoken language benefits after cochlear implantation. However, systematic research on specific language domains and error analyses are required to probe sub-skills. Moreover, the effect of predictive factors on distinct language domains is unknown. In

  3. Tonotopic organisation of the auditory cortex in sloping sensorineural hearing loss.

    Science.gov (United States)

    Wolak, Tomasz; Cieśla, Katarzyna; Lorens, Artur; Kochanek, Krzysztof; Lewandowska, Monika; Rusiniak, Mateusz; Pluta, Agnieszka; Wójcik, Joanna; Skarżyński, Henryk

    2017-11-01

    Although the tonotopic organisation of the human primary auditory cortex (PAC) has already been studied, the question how its responses are affected in sensorineural hearing loss remains open. Twenty six patients (aged 38.1 ± 9.1 years; 12 men) with symmetrical sloping sensorineural hearing loss (SNHL) and 32 age- and gender-matched controls (NH) participated in an fMRI study using a sparse protocol. The stimuli were binaural 8s complex tones with central frequencies of 400 HzCF, 800 HzCF, 1600 HzCF, 3200 HzCF, or 6400 HzCF, presented at 80 dB(C). In NH responses to all frequency ranges were found in bilateral auditory cortices. The outcomes of a winnermap approach, showing a relative arrangement of active frequency-specific areas, was in line with the existing literature and revealed a V-shape high-frequency gradient surrounding areas that responded to low frequencies in the auditory cortex. In SNHL frequency-specific auditory cortex responses were observed only for sounds from 400 HzCF to 1600 HzCF, due to the severe or profound hearing loss in higher frequency ranges. Using a stringent statistical threshold (p < 0.05; FWE) significant differences between NH and SNHL were only revealed for mid and high-frequency sounds. At a more lenient statistical threshold (p < 0.001, FDRc), however, the size of activation induced by 400 HzCF in PAC was found statistically larger in patients with a prelingual, as compared to a postlingual onset of hearing loss. In addition, this low-frequency range was more extensively represented in the auditory cortex when outcomes obtained in all patients were contrasted with those revealed in normal hearing individuals (although statistically significant only for the secondary auditory cortex). The outcomes of the study suggest preserved patterns of large-scale tonotopic organisation in SNHL which can be further refined following auditory experience, especially when the hearing loss occurs prelingually. SNHL can induce both

  4. Profound thrombocytopenia after primary exposure to eptifibatide.

    Science.gov (United States)

    Norgard, Nicholas B; Badgley, Brian T

    2010-01-01

    Eptifibatide is a glycoprotein IIb/IIIa receptor antagonist used to reduce the incidence of ischemic events in patients with acute coronary syndromes and those undergoing percutaneous coronary intervention. A minority of patients given eptifibatide develop acute, profound thrombocytopenia (eptifibatide for the first time. The Naranjo algorithm classified the likelihood that this patient's thrombocytopenia was related to eptifibatide as probable. Profound thrombocytopenia is an uncommon but clinically important complication of eptifibatide. This case report emphasizes the importance of monitoring platelet counts routinely at baseline and within 2-6 hours of eptifibatide administration.

  5. Sensorineural hearing loss in insulin-dependent diabetic patients

    Directory of Open Access Journals (Sweden)

    Koosha A.

    2007-11-01

    Full Text Available Background: Among patients who have sensorineural hearing loss of unknown etiology, diabetes is one of the diseases to be routinely investigated. The relationship between diabetes mellitus and hearing loss is still controversial. The purpose of this study was to examine the prevalence of sensorineural hearing loss in patients with insulin-dependent diabetes mellitus (IDDM compared to control group.Methods: In a cross-sectional study pure tone audiometry (PTA and speech audiometry was performed in 62 patients with insulin-dependent diabetes mellitus (IDDM, aged under 40 years, and in 62 randomly selected age-matched non-diabetic control subjects. Subjects with otological and other metabolic diseases were excluded from the study. We applied the SPSS.10 statistical analysis software Chi-square and student's test. Results: Statistical analysis showed that the hearing of the diabetic patients were significantly worsen than the control subjects. The hearing level tended to be worsen in the diabetic patients than that in control subjects, but the differences were statistically significant only at frequencies of 250,500, 4000 and 8000 Hz p>0.05(. There wasn't statistical significant difference between sex in two study groups p>0.05(. The mean duration of diabetes was no statistically significant with hearing loss p>0.05(. The frequency of complications such as retinopathy, nephropathy, and neuropathy in the diabetic groups had no correlation with speech threshold (p>0.05(.‏ There were no significant differences between speech reception threshold, speech discrimination score and acoustic reflex in two groups.Conclusions: We conclude that type I diabetes mellitus can cause sensorineural hearing loss.

  6. [Subclinical sensorineural hearing loss in female patients with rheumatoid arthritis].

    Science.gov (United States)

    Treviño-González, José Luis; Villegas-González, Mario Jesús; Muñoz-Maldonado, Gerardo Enrique; Montero-Cantu, Carlos Alberto; Nava-Zavala, Arnulfo Hernán; Garza-Elizondo, Mario Alberto

    2015-01-01

    The rheumatoid arthritis is a clinical entity capable to cause hearing impairment that can be diagnosed promptly with high frequencies audiometry. To detect subclinical sensorineural hearing loss in patients with rheumatoid arthritis. Cross-sectional study on patients with rheumatoid arthritis performing high frequency audiometry 125Hz to 16,000Hz and tympanometry. The results were correlated with markers of disease activity and response to therapy. High frequency audiometry was performed in 117 female patients aged from 19 to 65 years. Sensorineural hearing loss was observed at a sensitivity of pure tones from 125 to 8,000 Hz in 43.59%, a tone threshold of 10,000 to 16,000Hz in 94.02% patients in the right ear and in 95.73% in the left ear. Hearing was normal in 8 (6.84%) patients. Hearing loss was observed in 109 (93.16%), and was asymmetric in 36 (30.77%), symmetric in 73 (62.37%), bilateral in 107 (91.45%), unilateral in 2 (1.71%), and no conduction and/or mixed hearing loss was encountered. Eight (6.83%) patients presented vertigo, 24 (20.51%) tinnitus. Tympanogram type A presented in 88.90% in the right ear and 91.46% in the left ear, with 5.98 to 10.25% type As. Stapedius reflex was present in 75.3 to 85.2%. Speech discrimination in the left ear was significantly different (p = 0.02)in the group older than 50 years. No association was found regarding markers of disease activity, but there was an association with the onset of rheumatoid arthritis disease. Patients with rheumatoid arthritis had a high prevalence of sensorineural hearing loss for high and very high frequencies. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

  7. Oral Communication Development in Severe to Profound Hearing Impaired Children After Receiving Aural Habilitation

    Directory of Open Access Journals (Sweden)

    Soleimani Farin

    2009-10-01

    Full Text Available Communication, cognition, language, and speech are interrelated and develop together. It should come as no surprise to us that the key to intervention with deaf children is to establish, as early as possible, a functional communication system for the child and the parents. Early intervention programs need to be multidisciplinary, technologically sound and most important, it should take cognizance of the specific context (community, country in which the child and family function. The main aim of this study was to obtain oral communication development regarding current status of the intervention (aural habilitation and speech therapyfor children with severe to profound hearing impairment in Iran. A prospective longitudinal study was undertaken on a consecutive group of children with severe to profound deafness. Nine severe to profound hearing-impaired children out of the primer 42 cases, who were detected below two years old, had been selected in the previous study to receive aural habilitation. The average of their speech intelligibility scores was near 70% at age 6, which was accounted as poor oral communication and only two of them were able to communicate by spoken language. An integrated intervention services continued again for one year and their oral communication skill was assessed by their speech intelligibility. The intelligibility test of children was recorded on audio-tape, when they read 10 questions such as where is your home. This can be answered only in one word. Each tape was presented to10 normal hearing listeners, and their task was to write down, the answers in Persian orthography. At the beginning (at age 6 the average speech intelligibility score of these children was 72% and only two of them had score of 90% and 100%. At age 7, all of the severe groups were over 90%, and only two profound ones achieved the score of 48% and 62%. All of severe groups develop oral communication, but profound ones had a semi-intelligible speech

  8. Sudden sensorineural hearing loss in human monocytic ehrlichiosis.

    Science.gov (United States)

    Bhalla, Vidur; Rodgers, Brian; Lin, James

    2017-08-01

    The hallmarks of symptomatic human monocytic ehrlichiosis (HME) include fever, headache, myalgia, nausea, malaise, transaminitis, and blood cell abnormalities. Previous case reports have described isolated cranial nerve palsies in infected patients but not hearing loss. We describe the onset of sudden sensorineural hearing loss in 2 patients with HME-a 31-year-old woman and an 82-year-old woman. The older patient experienced objective and subjective improvement in her hearing after treatment with an antibiotic and steroid taper; the younger patient was lost to audiologic follow-up. Additionally, we discuss the possible mechanisms of the hearing loss in these patients.

  9. Perceived Stress among Deaf Adults

    Science.gov (United States)

    Jones, Elaine G.; Ouellette, Sue E.; Kang, Youngmi

    2006-01-01

    The Present Article describes the effectiveness of stress management classes in decreasing perceived stress among Deaf adults. Deaf adults may experience unique stressors, in addition to circumstances associated with increased stress in the general population. The Perceived Stress Scale (S. Cohen, Kamarck, & Mermelstein, 1983) was used as a…

  10. Identity development in deaf adolescents

    NARCIS (Netherlands)

    Kunnen, E. Saskia

    2014-01-01

    We studied identity development during 5 years in 11 deaf adolescents who attend a school for deaf children in the highest level of regular secondary education (age between 14 and 19 years). Identity development is conceptualized by the processes of exploration and commitment formation, as

  11. Overview on Deaf-Blindness

    Science.gov (United States)

    Miles, Barbara

    2008-01-01

    It may seem that deaf-blindness refers to a total inability to see or hear. However, in reality deaf-blindness is a condition in which the combination of hearing and visual losses in children cause "such severe communication and other develop mental and educational needs that they cannot be accommodated in special education programs solely for…

  12. Anger communication in deaf children

    NARCIS (Netherlands)

    Rieffe, C.J.; Meerum Terwogt, M.

    2006-01-01

    In this study, we investigated how deaf children express their anger towards peers and with what intentions. Eleven-year-old deaf children (n = 21) and a hearing control group (n = 36) were offered four vignettes describing anger-evoking conflict situations with peers. Children were asked how they

  13. Social Connectedness of Deaf Retirees

    Science.gov (United States)

    Shaw, Sherry; Roberson, Len

    2013-01-01

    The intergenerational connectedness that has traditionally bound members of the Deaf community to each other is changing amidst the current technological and cultural landscape. This study explores perceptions of Deaf retirees concerning their usefulness to younger generations and their need to stay connected to each other despite increasing…

  14. Are Deaf Students Visual Learners?

    Science.gov (United States)

    Marschark, Marc; Morrison, Carolyn; Lukomski, Jennifer; Borgna, Georgianna; Convertino, Carol

    2013-01-01

    It is frequently assumed that by virtue of their hearing losses, deaf students are visual learners. Deaf individuals have some visual-spatial advantages relative to hearing individuals, but most have been linked to use of sign language rather than auditory deprivation. How such cognitive differences might affect academic performance has been…

  15. Evaluative expression in deaf children's written narratives

    NARCIS (Netherlands)

    Beijsterveldt, E.M. van; Hell, J.G. van

    2009-01-01

    Background: Deaf children vary in the use of and proficiency in signed language. The majority of studies on writing skills of children who are deaf did not assess deaf children's proficiency in signed language and/or grouped together deaf children with varying sign language skills. Aims: Adopting a

  16. Evaluative Expression in Deaf Children's Written Narratives

    Science.gov (United States)

    van Beijsterveldt, Liesbeth Maria; van Hell, Janet G.

    2009-01-01

    Background: Deaf children vary in the use of and proficiency in signed language. The majority of studies on writing skills of children who are deaf did not assess deaf children's proficiency in signed language and/or grouped together deaf children with varying sign language skills. Aims: Adopting a bimodal bilingual perspective, we examined…

  17. Programs for the Deaf-Blind.

    Science.gov (United States)

    American Annals of the Deaf, 1991

    1991-01-01

    This directory lists contact information for programs for the deaf-blind in the United States in 3 categories: (1) programs for deaf-blind children and youth (29 programs listed); (2) Helen Keller National Center for Deaf-Blind Youth and Adults (1 national and 10 regional offices); and (3) programs for training teachers of the deaf-blind (4…

  18. Are Deaf Students Visual Learners?

    Science.gov (United States)

    Marschark, Marc; Morrison, Carolyn; Lukomski, Jennifer; Borgna, Georgianna; Convertino, Carol

    2013-01-01

    It is frequently assumed that by virtue of their hearing losses, deaf students are visual learners. Deaf individuals have some visual-spatial advantages relative to hearing individuals, but most have been are linked to use of sign language rather than auditory deprivation. How such cognitive differences might affect academic performance has been investigated only rarely. This study examined relations among deaf college students’ language and visual-spatial abilities, mathematics problem solving, and hearing thresholds. Results extended some previous findings and clarified others. Contrary to what might be expected, hearing students exhibited visual-spatial skills equal to or better than deaf students. Scores on a Spatial Relations task were associated with better mathematics problem solving. Relations among the several variables, however, suggested that deaf students are no more likely to be visual learners than hearing students and that their visual-spatial skill may be related more to their hearing than to sign language skills. PMID:23750095

  19. Apartheid in deaf education: examining workforce diversity.

    Science.gov (United States)

    Simms, Laurene; Rusher, Melissa; Andrews, Jean F; Coryell, Judy

    2008-01-01

    A survey of 3,227 professionals in 313 deaf education programs found that 22.0% of teachers and 14.5% of administrators were deaf--a less than 10% increase in deaf professionals since 1993. Additionally, 21.7% of teachers and 6.1% of administrators were professionals of color. Of these minority teachers, only 2.5% were deaf persons of color. Only 3 deaf administrators of color were identified. The study describes how "apartheid" or "intellectual oppression" may result from unchanged hiring practices in K-12 programs for the deaf and in postsecondary institutions. Using a bottle metaphor, the researchers describe how deaf persons of color are often stuck in "a bottleneck on the highway to opportunity." Relevant data underscore that the field of deaf education must diversify its professional force in order to utilize the intellectual, linguistic, and multicultural proficiencies of hearing teachers of color, deaf teachers, and deaf teachers of color.

  20. An Eye Tracking Study on the Perception and Comprehension of Unimodal and Bimodal Linguistic Inputs by Deaf Adolescents.

    Science.gov (United States)

    Mastrantuono, Eliana; Saldaña, David; Rodríguez-Ortiz, Isabel R

    2017-01-01

    An eye tracking experiment explored the gaze behavior of deaf individuals when perceiving language in spoken and sign language only, and in sign-supported speech (SSS). Participants were deaf (n = 25) and hearing (n = 25) Spanish adolescents. Deaf students were prelingually profoundly deaf individuals with cochlear implants (CIs) used by age 5 or earlier, or prelingually profoundly deaf native signers with deaf parents. The effectiveness of SSS has rarely been tested within the same group of children for discourse-level comprehension. Here, video-recorded texts, including spatial descriptions, were alternately transmitted in spoken language, sign language and SSS. The capacity of these communicative systems to equalize comprehension in deaf participants with that of spoken language in hearing participants was tested. Within-group analyses of deaf participants tested if the bimodal linguistic input of SSS favored discourse comprehension compared to unimodal languages. Deaf participants with CIs achieved equal comprehension to hearing controls in all communicative systems while deaf native signers with no CIs achieved equal comprehension to hearing participants if tested in their native sign language. Comprehension of SSS was not increased compared to spoken language, even when spatial information was communicated. Eye movements of deaf and hearing participants were tracked and data of dwell times spent looking at the face or body area of the sign model were analyzed. Within-group analyses focused on differences between native and non-native signers. Dwell times of hearing participants were equally distributed across upper and lower areas of the face while deaf participants mainly looked at the mouth area; this could enable information to be obtained from mouthings in sign language and from lip-reading in SSS and spoken language. Few fixations were directed toward the signs, although these were more frequent when spatial language was transmitted. Both native and non

  1. An Eye Tracking Study on the Perception and Comprehension of Unimodal and Bimodal Linguistic Inputs by Deaf Adolescents

    Directory of Open Access Journals (Sweden)

    Eliana Mastrantuono

    2017-06-01

    Full Text Available An eye tracking experiment explored the gaze behavior of deaf individuals when perceiving language in spoken and sign language only, and in sign-supported speech (SSS. Participants were deaf (n = 25 and hearing (n = 25 Spanish adolescents. Deaf students were prelingually profoundly deaf individuals with cochlear implants (CIs used by age 5 or earlier, or prelingually profoundly deaf native signers with deaf parents. The effectiveness of SSS has rarely been tested within the same group of children for discourse-level comprehension. Here, video-recorded texts, including spatial descriptions, were alternately transmitted in spoken language, sign language and SSS. The capacity of these communicative systems to equalize comprehension in deaf participants with that of spoken language in hearing participants was tested. Within-group analyses of deaf participants tested if the bimodal linguistic input of SSS favored discourse comprehension compared to unimodal languages. Deaf participants with CIs achieved equal comprehension to hearing controls in all communicative systems while deaf native signers with no CIs achieved equal comprehension to hearing participants if tested in their native sign language. Comprehension of SSS was not increased compared to spoken language, even when spatial information was communicated. Eye movements of deaf and hearing participants were tracked and data of dwell times spent looking at the face or body area of the sign model were analyzed. Within-group analyses focused on differences between native and non-native signers. Dwell times of hearing participants were equally distributed across upper and lower areas of the face while deaf participants mainly looked at the mouth area; this could enable information to be obtained from mouthings in sign language and from lip-reading in SSS and spoken language. Few fixations were directed toward the signs, although these were more frequent when spatial language was transmitted. Both

  2. Endothelial dysfunction in idiopathic sudden sensorineural hearing loss: a review

    Directory of Open Access Journals (Sweden)

    Nicola Quaranta

    2016-07-01

    Full Text Available An endothelial dysfunction has been described in idiopathic sudden sensorineural hearing loss (ISSHL patients. The purpose of our review was to: i identify, evaluate and review recent research about cardiovascular risk factors involvement and signs of endothelial dysfunction in ISSHL; ii implication of these discovering in clinical practice and future research. A Medline literature search was conducted to identify any study on the involvement of endothelial dysfunction in ISSHL, published in the English language in the last decade. The following MEDLINE search terms were used: sudden sensorineural hearing loss (SSHL and endothelial dysfunction (text words. Additional studies were identified by hand searching the references of original articles and review articles. Studies were not excluded on the basis of the qualitative or quantitative definitions of SSHL, treatment regimens, or outcome measures. Data were extracted from included papers by a reviewer. Information on the patients, investigations, methods, interventions, and outcomes were systematically analyzed. Characteristics and results of all included studies were reviewed systematically. High levels of adhesion molecules, hyperhomocysteinemia and lower folate levels, unbalanced oxidative status, a lower value of flow-mediated dilatation of brachial artery and a reduced percentage of circulating endothelial progenitor cells in patients affected by ISSHL support the hypothesis that this syndrome should be considered as a microcirculation disorder based on endothelial dysfunction and drive clinicians to implement all the traditional strategies used for preventing cardiovascular events, to also reduce the likelihood of ISSHL occurrence.

  3. Adjunctive hyperbaric oxygen treatment for idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Chin, Chun-Shih; Lee, Tsai-Yun; Wu, Ming-Feng

    2017-01-01

    This study aims to evaluate the hearing gain efficacy from adjunctive hyperbaric oxygen (HBO₂) treatment in patients with idiopathic sudden sensorineural hearing loss (ISSHL). A retrospective analysis of chart reviews was performed on patients with ISSHL between January 2013 through December 2015. All patients were referred to us from our ENT Department for adjunctive hyperbaric oxygen treatment. The results were assessed through pure-tone audiometry (PTA) data change (hearing gain), both before and after HBO₂) treatment. Age, gender, affected ear side, HBO₂) treatment sessions, both before and after HBO₂ treatment PTA were all recorded. Ninety-three (93) patients with ISSHL were included in the study. The average hearing gain in this study was 17.9 dB (p=0.001), where a total of 46 (49.46%) patients showed an improvement (hearing gain≥dB) in response to HBO₂ treatment (p=0.002). Patients with the poorest initial severity of hearing loss who displayed a greater degree of hearing improvement after HBO₂ treatment were male and in the 40- to 59-year-old age group. This study found that adjunctive hyperbaric oxygen treatment was efficacious for patients with idiopathic sudden sensorineural hearing loss. The total average hearing gain was recorded to be 17.9 dB.

  4. Endothelial Dysfunction in Idiopathic Sudden Sensorineural Hearing Loss: A Review.

    Science.gov (United States)

    Quaranta, Nicola; De Ceglie, Vincenzo; D'Elia, Alessandra

    2016-04-20

    An endothelial dysfunction has been described in idiopathic sudden sensorineural hearing loss (ISSHL) patients. The purpose of our review was to: i) identify, evaluate and review recent research about cardiovascular risk factors involvement and signs of endothelial dysfunction in ISSHL; ii) implication of these discovering in clinical practice and future research. A Medline literature search was conducted to identify any study on the involvement of endothelial dysfunction in ISSHL, published in the English language in the last decade. The following MEDLINE search terms were used: sudden sensorineural hearing loss (SSHL) and endothelial dysfunction (text words). Additional studies were identified by hand searching the references of original articles and review articles. Studies were not excluded on the basis of the qualitative or quantitative definitions of SSHL, treatment regimens, or outcome measures. Data were extracted from included papers by a reviewer. Information on the patients, investigations, methods, interventions, and outcomes were systematically analyzed. Characteristics and results of all included studies were reviewed systematically. High levels of adhesion molecules, hyperhomocysteinemia and lower folate levels, unbalanced oxidative status, a lower value of flow-mediated dilatation of brachial artery and a reduced percentage of circulating endothelial progenitor cells in patients affected by ISSHL support the hypothesis that this syndrome should be considered as a microcirculation disorder based on endothelial dysfunction and drive clinicians to implement all the traditional strategies used for preventing cardiovascular events, to also reduce the likelihood of ISSHL occurrence.

  5. Fostering Positive Deaf Identity Development in a K-2 Deaf Classroom /

    OpenAIRE

    Hipskind, Courtney

    2014-01-01

    All Deaf children deserve to have opportunities to openly explore, examine, and affirm their own Deaf identities at school, yet there is a shortage of curricula and resources dedicated to this basic need. The aim of this thesis is to provide Deaf children with such opportunities. The curriculum within- Fostering Deaf Identity Development in a K-2 Deaf Classroom- consists of two units that address positive Deaf identity formation. The first unit focuses on the characterization and affirmation ...

  6. Early Interactions with Children Who Are Deaf-Blind

    Science.gov (United States)

    ... Topics About Deaf-Blindness > What is Deaf-Blindness Definitions of Deaf-Blindness Causes of Deaf-Blindness National Child Count & Demographics Communication > Communication Overview Early Communication Prelinguistic Communication Object Communication ...

  7. Establishment of joint attention in dyads involving hearing mothers of deaf and hearing children, and its relation to adaptive social behavior.

    Science.gov (United States)

    Nowakowski, Matilda E; Tasker, Susan L; Schmidt, Louis A

    2009-01-01

    Mounting evidence points to joint attention as a mediating variable in children's adaptive behavior development. Joint attention in interactions between hearing mothers and congenitally deaf (n = 27) and hearing (n = 29) children, ages 18-36 months, was examined. All deaf children had severe to profound hearing loss. Mother-child interactions were coded for maternally initiated and child-initiated success rates in establishing joint attention; mothers completed ratings of their children's adaptive behavior. Hearing mother-deaf child dyads had significantly lower maternally initiated success rates. No significant between-group differences on child-initiated success rates were shown. Maternal ratings of adaptive behavior were significantly lower for deaf children, and related positively and significantly to both child-initiated and maternally initiated success rates. The findings suggest that mother-child interactions that are low in successful establishment of joint attention might mediate the development of socioemotional problems evident in deaf children with hearing families.

  8. Profound thrombocytopenia after primary exposure to eptifibatide

    OpenAIRE

    Norgard, Nicholas; Badgley,Brian

    2010-01-01

    Nicholas B Norgard, Brian T BadgleyUniversity at Buffalo, School of Pharmacy and Pharmaceutical Sciences, Buffalo, NY, USAAbstract: Eptifibatide is a glycoprotein IIb/IIIa receptor antagonist used to reduce the incidence of ischemic events in patients with acute coronary syndromes and those undergoing percutaneous coronary intervention. A minority of patients given eptifibatide develop acute, profound thrombocytopenia (<20,000 cells/mm3) within a few hours of receiving the drug. This c...

  9. [Analysis of the hereditary etiology of 336 patients with non-syndromic sensorineural hearing loss from Ningxia Hui Autonomous Region of China].

    Science.gov (United States)

    Wang, Yan-li; Zhu, Yi-ming; Liu, Xiao-wen; Xu, Bai-cheng; Guo, Yu-fen; Wang, Qiu-ju

    2012-09-01

    To investigate the molecular genetic causes and their characteristics of deafness in Ningxia province, we established screening of three common hereditary deafness genes in 336 deaf and hard-of-hearing patients in this district. Peripheral blood samples were obtained from a total of 336 patients with non-syndromic sensorineural hearing loss in parts of special education schools in Ningxia province to extract genomic DNA. The mitochondrial DNA 12S rRNA m.1555A > G mutation was screened by PCR Alw26I digestion and sequence analysis PCR and direct sequencing were used to analyze the coding region of GJB2 and exons 8 and 19 of SLC26A4. Statistical analysis was performed by using SPSS 11.0 software. Frequencies of different GJB2 or SLC26A4 mutations were compared between Han and Hui people. Among these 336 patients, seven cases (2.08%, 7/336) were found to carry mtDNA 12S rRNA m.1555A > G homozygous mutation, 45 cases (13.39%) were caused by GJB2 mutations and 28 cases (8.33%) had two mutated alleles (homozygote and compound heterozygote) of SLC26A4. In detail, 16.67% (56/336) patients carried GJB2 mutations including 11 single mutant carriers. The allele frequency of c.235delC and c.299_300delAT were 9.52% (64/672) and 2.68% (18/672), respectively, making up 81.19% (82/101) of all pathogenic mutated alleles for GJB2. The single mutant allele carriers of SLC26A4 is 32, and two types (c.919-2A > G and c.2168A > G) accounted for 95.29% (24/27) mutations, totally. We also found that statistically significant differences in c.919-2A > G and c.2168A > G frequencies between Han and Hui people (c.919-2A > G, χ(2) = 8.229, P = 0.004; c.2168A > G, χ(2) = 5.277, P = 0.022). However, there was no statistically significant difference in GJB2 mutation between Han and Hui people. GJB2 mutation was a primary cause for non-syndromic sensorineural hearing loss in Ningxia province, and c.235delC was the most common mutant forms of GJB2. c.919-2A > G and c.2168A > G were common mutant

  10. Positional cloning of deafness genes

    NARCIS (Netherlands)

    Kremer, H.; Cremers, F.P.M.

    2009-01-01

    The identification of the majority of the known causative genes involved in nonsyndromic sensorineural hearing loss (NSHL) started with linkage analysis as part of a positional cloning procedure. The human and mouse genome projects in combination with technical developments on genotyping,

  11. Static and Dynamic Balance in Congenital Severe to Profound Hearing-Impaired Children

    Directory of Open Access Journals (Sweden)

    Farideh HajiHeydari

    2011-09-01

    Full Text Available Background and Aim: Research conducted since the early 1900s has consistently identified differences between deaf and hearing children on performance of a wide variety of motor tasks, most notably balance. Our study was performed to test static and dynamic balance skills in congenital severe to profound hearing impaired children in comparison with normal age-matched children.Methods: This cross-sectional study was conducted on 30 severe to profound hearing impaired and 40 normal children with age 6 to 10 years old. Bruininks-Oseretsky test of motor proficiency 2, balance subset with 9 parts was used for evaluation of balance skills.Results: Hearing-impaired children showed 16.7 to 100% fail results in 7 parts of the balance subset. In normal children fail result was revealed just in 3 parts of the balance subset from 2.5 to 57.5%, and differences between two groups were significant (p<0.0001. There was a significant difference between two groups in two static balance skills of standing on one leg on a line and standing on one leg on a balance beam with eyes closed (p<0.0001.conclusion: It seems that development of static balance skills are longer than dynamic ones. Because severe to profound hearing-impaired children showed more weakness than normal children in both static and dynamic balance abilities, functional tests of balance proficiency can help to identify balance disorders in these children.

  12. Epileptiform electroencephalogram abnormality in children with congenital sensorineural hearing loss.

    Science.gov (United States)

    El-Badry, Mohamed Mohamed; Hamdy, Nermin Aly; Sobhy, Sayed; Gamal, Reham

    2014-04-01

    This work was designed to study electroencephalogram findings in children with congenital sensorineural hearing loss and correlate these findings with the SNHL parameters as duration, etiology, severity, and type. Ninety children with bilateral congenital sensorineural hearing loss served as the study group. They were free from any neurological disorders or symptoms that are commonly associated with abnormal electroencephalogram as convulsions or loss of consciousness. Twenty children having normal hearing with no history of otological or neurological disorders served as the control group. All children participating in the study were subjected to full medical and audiological history, otological examination, neurological examination, audiological evaluation and electroencephalogram recording. Mean age of the children in the control group was 3.56 ± 2.1 years and mean age of the children in the study group was 3.8 ± 2.2 years. While none of the control children had abnormal electroencephalogram, 38 (42.2%) of children with congenital SNHL had epileptiform electroencephalogram abnormality. The epileptiform abnormality was generalized in 14 children (36.8%), focal temporal in 17 children (44.7%) and focal other than temporal in 7 children (18.4%). According to the hemispheric side affected, the abnormality was right in 14 children (36.8%), left in 10 children (26.3%) and bilateral in 14 children (36.8%). No statistically significant predominance of specific site or side of the epileptiform abnormality was found. Similarly, no statistical significant prevalent of the epileptiform abnormality was found in relation to the age or sex of children, duration of hearing loss or etiology of hearing loss (i.e., genetic vs. neonatal insults). On the other hand, the epileptiform abnormality was statistically prevalent in children with moderate degree of hearing loss, and in children with auditory neuropathy spectrum disorder. The epileptiform electroencephalogram abnormality is

  13. HSP and deafness

    Science.gov (United States)

    Donkervoort, Sandra; Bharucha-Goebel, Diana; Yun, Pomi; Hu, Ying; Mohassel, Payam; Hoke, Ahmet; Zein, Wadih M.; Ezzo, Daniel; Atherton, Andrea M.; Modrcin, Ann C.; Dasouki, Majed; Foley, A. Reghan

    2017-01-01

    Objective: To identify the underlying genetic cause in 2 sisters affected with progressive lower extremity spasticity, neuropathy, and early-onset deafness. Methods: Whole-exome sequencing was performed, and segregation testing of variants was investigated using targeted Sanger sequencing. An inherited paternal mosaic mutation was further evaluated through quantitative analysis of the ratio of mutant vs wild-type allele in genomic DNA from various tissues, including blood, dermal fibroblasts, and saliva. Results: A novel heterozygous nonsense mutation (c.1140C>A; p.Y380X) in SOX10 was identified in the affected sisters. Paternal mosaicism was suspected based on a small chromatogram peak, which was less than the heterozygous peak of the mutated allele. Consistent with mosaicism, the mosaic paternal samples had notable variability in the ratio of mutant vs wild-type allele in various tissues (compared with the fully heterozygous daughter), with the highest paternal mutant levels in saliva (32.7%) and lowest in dermal fibroblasts (13.9%). Targeted clinical re-examination of the father revealed a sensorimotor neuropathy that was previously clinically unrecognized. Conclusions: These findings expand the phenotypic spectrum of SOX10-related neurocristopathy. Mutations in SOX10 should be considered in patients presenting with a complicated form of hereditary spastic paraplegia that includes neuropathy and deafness. Diagnostic workup may be complicated, as SOX10 mutations can present in a mosaic state, with a mild clinical manifestation. PMID:28534044

  14. Deaf not Daft: The Deaf in Mental Subnormality Hospitals.

    Science.gov (United States)

    Williams, Chris

    1982-01-01

    Case studies of deaf or hearing impaired persons in institutions for the mentally retarded illustrate the ways in which the "invisible handicap" can mask cognitive ability, causing unnecessary institutionalization. (CL)

  15. Discovery of CDH23 as a Significant Contributor to Progressive Postlingual Sensorineural Hearing Loss in Koreans.

    Directory of Open Access Journals (Sweden)

    Bong Jik Kim

    Full Text Available CDH23 mutations have mostly been associated with prelingual severe-to-profound sensorineural hearing loss (SNHL in either syndromic or nonsyndromic SNHL (DFNB12. Herein, we demonstrate the contribution of CDH23 mutations to postlingual nonsyndromic SNHL (NS-SNHL. We screened 32 Korean adult probands with postlingual NS-SNHL sporadically or in autosomal recessive fashion using targeted panel or whole exome sequencing. We identified four (12.5%, 4/32 potential postlingual DFNB12 families that segregated the recessive CDH23 variants, qualifying for our criteria along with rapidly progressive SNHL. Three of the four families carried one definite pathogenic CDH23 variant previously known as the prelingual DFNB12 variant in a trans configuration with rare CDH23 variants. To determine the contribution of rare CDH23 variants to the postlingual NS-SNHL, we checked the minor allele frequency (MAF of CDH23 variants detected from our postlingual NS-SNHL cohort and prelingual NS-SNHL cohort, among the 2040 normal control chromosomes. The allele frequency of these CDH23 variants in our postlingual cohort was 12.5%, which was significantly higher than that of the 2040 control chromosomes (5.53%, confirming the contribution of these rare CDH23 variants to postlingual NS-SNHL. Furthermore, MAF of rare CDH23 variants from the postlingual NS-SNHL group was significantly higher than that from the prelingual NS-SNHL group. This study demonstrates an important contribution of CDH23 mutations to poslingual NS-SNHL and shows that the phenotypic spectrum of DFNB12 can be broadened even into the presbycusis, depending on the pathogenic potential of variants. We also propose that pathogenic potential of CDH23 variants and the clinical fate of DFNB12 may be predicted by MAF.

  16. Research progress in pathogenic genes of hereditary non-syndromic mid-frequency deafness.

    Science.gov (United States)

    Xia, Wenjun; Liu, Fei; Ma, Duan

    2016-06-01

    Hearing impairment is considered as the most prevalent impairment worldwide. Almost 600 million people in the world suffer from mild or moderate hearing impairment, an estimated 10% of the human population. Genetic factors play an important role in the pathogenesis of this disorder. Hereditary hearing loss is divided into syndromic hearing loss (associated with other anomalies) and non-syndromic hearing loss (not associated with other anomalies). Approximately 80% of genetic deafness is non-syndromic. On the basis of the frequency of hearing loss, hereditary non-syndromic hearing loss can be divided into high-, mid-, low-, and total-frequency hearing loss. An audiometric finding of mid-frequency sensorineural hearing loss, or a "bowl-shaped" audiogram, is uncommon. Up to now, merely 7 loci have been linked to mid-frequency hearing loss. Only four genetic midfrequency deafness genes, namely, DFNA10 (EYA4), DFNA8/12 (TECTA), DFNA13 (COL11A2), DFNA44 (CCDC50), have been reported to date. This review summarizes the research progress of the four genes to draw attention to mid-frequency deafness genes.

  17. Use of the ages and stages questionnaire in young children who are deaf/hard of hearing as a screening for additional disabilities.

    Science.gov (United States)

    Wiley, Susan; Meinzen-Derr, Jareen

    2013-05-01

    Children with sensorineural hearing loss have a high rate of additional disabilities beyond those expected by the impact of hearing loss on language and are identified later than children without hearing loss. Identifying these developmental delays and allowing earlier implementation of interventions specific to these concerns would improve care. To determine whether the Ages and Stages Questionnaire (ASQ) can identify unmet needs or unidentified developmental delays among young children with bilateral sensorineural hearing loss. Fifty children with any degree of bilateral sensorineural hearing loss, 6months to 36months of age recruited from a tertiary referral center and regional early intervention programs for children who are deaf/hard of hearing. The ASQ and comprehensive developmental evaluations with the Revised Gesell Developmental Schedules. Sensitivities and specificities of the ASQ were calculated. While 32% of our population had a developmental delay outside the domain of communication, the ASQ had poor sensitivity on the overall score without the communication domain as well as for fine motor, cognitive, personal-social domains. It had good sensitivity for the domains of communication and gross motor skills and good specificity (ranging 83-85%) on specific domains as well as for the overall score (70%). The ASQ does not provide an effective mean for identifying additional developmental concerns in young children with bilateral sensorineural hearing loss. Further studies are needed to determine how to identify additional disabilities in a timely manner among young children with hearing loss. Copyright © 2012 Elsevier Ltd. All rights reserved.

  18. Modified intratympanic treatment for idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Kara, Emrah; Cetik, Fikret; Tarkan, Ozgür; Sürmelioğlu, Ozgür

    2010-05-01

    Steroids are the only proven drugs in the treatment for idiopathic sudden sensorineural hearing loss. In the recent studies, it has been suggested that, steroids delivered through the intratympanic route obtained higher perilymph levels, resulting in better hearing outcomes. The purpose of this study is to compare the hearing outcomes of the two routes of steroid treatment: intratympanic route and systemic route. In this prospective study, 60 consecutive patients with idiopathic sensorineural hearing loss treated between January 2005 and September 2008 were enrolled: 29 were in the intratympanic steroid group (ITSG) and 31 were in the systemic steroid group (SSG). In the ITSG, 5 intratympanic injections of dexamethasone were performed with the dose of 4 mg/ml, consecutively. Oral methylprednisolone was given at the dose of 1 mg/kg, tapered every 2 days and stopped at 10 days, in the SSG. The pure tone averages (PTA), speech discrimination scores (SDS) and the percentage of the patients who made an improvement more than 10 dB were analyzed on the tenth day and 2 months after the treatment statistically. The improvement in PTA on tenth day and second month after treatment was 31.38 and 37.55 dB, in the ITSG and 19.35 and 20.68 dB in the SSG, respectively. The improvement in SDS in the same time period was 35.24 and 37.52% in the ITSG and 20.13 and 19.61% in the SSG, respectively. Also, 25 of the 29 patients (86.2%) in the ITSG and 16 of the 31 patients (51.6%) in the SSG made an improvement more than 10 dB on PTA in the second month control. Intratympanic steroids gave better hearing results than systemic steroids with no systemic side effects. Studies with more sample sizes will identify the best steroid for injection, application time, frequency and dose.

  19. Acceptability judgments still matter: Deafness and documentation.

    Science.gov (United States)

    Hall, Matthew L; Mayberry, Rachel I; Ferreira, Victor S

    2017-01-01

    The target article's call to end reliance on acceptability judgments is premature. First, it restricts syntactic inquiry to cases were a semantically equivalent alternative is available. Second, priming studies require groups of participants who are linguistically homogenous and whose grammar is known to the researcher. These requirements would eliminate two major research areas: syntactic competence in d/Deaf individuals, and language documentation. (We follow the convention of using deaf to describe hearing levels, Deaf to describe cultural identity, and d/Deaf to include both. Our own work has focused on Deaf signers, but the same concerns could apply to other deaf populations.).

  20. Sensorineural hearing loss following irradiation to the malignant tumor of the head and neck

    Energy Technology Data Exchange (ETDEWEB)

    Murakami, Masafumi; Kobari, Hitomi; Kanno, Hidetaka; Aikawa, Tohru; Anzai, Tomohiro; Okamura, Hiro-oki; Ohtani, Iwao; Hoshino, Toshiaki

    1989-03-01

    We observed sensorineural hearing loss following X-ray irradiation to the malignant tumor of head and neck. There were 24 patients whose auditory organs lied within the irradiation field. Ten of these patients were affected by sensorineural hearing loss. Hearing loss occurred at a high frequency in elderly patients, epipharynx tumor and high dose of irradiation. Many cases revealed high tone hearing loss. Most cases showed about a 20/similar to/30 dB hearing loss, so their impediment seemed not severe in daily life. In some of these cases, we could have temporal bone findings, but there were no particular findings relevant to sensorineural hearing loss. (author).

  1. Asystole Following Profound Vagal Stimulation During Hepatectomy

    Directory of Open Access Journals (Sweden)

    Preeta John

    2008-01-01

    Full Text Available Asystole in a non laparoscopic upper abdominal surgery following intense vagal stimulation is a rare event. This case report highlights the need for awareness of such a complication when a thoracic epidural anaesthetic has been given in addition to a general anaesthetic for an upper abdominal procedure. A combined thoracic epidural and general anaesthetic was given. The anterior abdominal wall was retracted forty minutes after administration of the epidural bolus. This maneuver resulted in a profound vagal response with bradycardia and asystole. The patient was resuscitated successfully with a cardiac massage, atropine and adrenaline and the surgery was resumed. Surgery lasted eleven hours and was uneventful.

  2. Evaluation of cardiovascular risks and recovery of idiopathic sudden sensorineural hearing loss in hospitalised patients: comparison between complete and partial sudden sensorineural hearing loss.

    Science.gov (United States)

    Haremza, C; Klopp-Dutote, N; Strunski, V; Page, C

    2017-10-01

    To evaluate the presence of cardiovascular risk factors and recovery of idiopathic sudden sensorineural hearing loss in hospitalised patients. A single-centre retrospective study of 80 patients hospitalised for idiopathic sudden sensorineural hearing loss was conducted over a 6-year period. Mean pure tone hearing thresholds were assessed by pure tone audiometry. Twenty-three of 80 patients (28.75 per cent) initially had no cardiovascular risk factors. Forty-five patients had hyperlipidaemia, 22 patients had hypertension, 7 patients had diabetes mellitus and 7 patients were obese. No statistically significant difference was observed between patients with complete versus partial sudden sensorineural hearing loss (p = 0.0708) concerning the cardiovascular risk factors. At long-term follow up, the hearing recovery rate was not significantly different between the two groups of patients (p = 0.7541). The lack of a clear relationship between idiopathic sudden sensorineural hearing loss and cardiovascular risk factors suggests that sudden sensorineural hearing loss has a predominantly multifactorial disease profile regardless of hearing impairment severity.

  3. Hearing, Deaf, and Hard-of-Hearing Israeli Adolescents' Evaluations of Deaf Men and Deaf Women's Occupational Competence

    National Research Council Canada - National Science Library

    Amatzia Weisel; Rachel Gali Cinamon

    2005-01-01

    This study examined 74 deaf and hard-of-hearing (D/HH) and 91 hearing high school students regarding their own occupational aspirations and their evaluations of occupational competence (EOCs) for deaf adults...

  4. Experience with cochlear implants in Greenlanders with profound hearing loss living in Greenland

    DEFF Research Database (Denmark)

    Homøe, Preben; Andersen, Ture; Grøntved, Aksel

    2013-01-01

    OBJECTIVE: Cochlear implant (CI) treatment was introduced to the world in the 1980s and has become a routine treatment for congenital or acquired severe-to-profound hearing loss. CI treatment requires access to a highly skilled team of ear, nose and throat specialists, audiologists and speech...... years are in need of a CI every second year in Greenland often due to sequelae from meningitis, which may cause postinfectious deafness. Screening of new-borns for hearing has been started in Greenland establishing the basis for early diagnosis of congenital hearing impairment and subsequent...... intervention. The logistics and lack of availability of speech therapists in Greenland hampers possibilities for optimal language and speech therapy of CI patients in Greenland. This study aims at describing the results of CI treatment in Greenlanders and the outcome of the CI operations along...

  5. What We Should Teach Deaf Children: Deaf Teachers' Folk Models in Britain, the USA and Mexico

    Science.gov (United States)

    Sutton-Spence, Rachel; Ramsey, Claire

    2010-01-01

    Deaf teachers around the world have folk models and beliefs that reflect their understanding of what deaf children need to learn in order to develop healthy identities as deaf people. In this research we report what teachers from England, the USA and Mexico have told us about using creative signing with deaf children. Themes emerging from our data…

  6. Limited English Literacy Proficiency in Deaf People: A Review of Deafness and Hearing Perspectives.

    Science.gov (United States)

    Rodda, Michael; Eleweke, C. Jonah; Chapman, Jonathan

    1998-01-01

    This article examines the deafness and hearing perspectives concerning people with deafness and English literacy. Because literacy is important for people with deafness, it is suggested that carefully developed bilingual-bicultural programs could facilitate the development of English literacy skills in individuals who are deaf. (Contains…

  7. Should we teach thinking skills to deaf children?

    Directory of Open Access Journals (Sweden)

    Emma Tamsin Kelty

    2006-04-01

    Full Text Available This pilot study aimed to identify the benefits of developing thinking skills with KS1 deaf children who used British Sign Language (BSL. It arose as a response to the findings of a variety of researches who had reported a number of ‘failings’ apparent in the educational and learning activity of deaf children. It used a case study approach involving five profoundly deaf Key stage 1 children and explored the extent to which, using materials grounded in the Somerset Thinking Skills Course, the teaching of thinking skills in a supportive environment could remediate some of these issues. The strongly visual nature of the material supported pupil exchanges mediated by the use of sign language. Analysis of video film was used to plot individual pupil development of scanning skills, their use of nouns versus adjectives, micro-skills and macro-abilities. Pupil reasoning skills, how they were supported, their ownership and role of the facilitator were also examined. The results showed that within eight weeks (equivalent to four hours in total the children were more able to express their perceptions. They watched other children in order to access their signed information and appeared to use this to develop, elaborate, extend and provide reasons when it was their turn to present. There was also evidence of enhanced creativity and originality in their contributions. This pilot study urges the need for further research and suggests that a priority should be given to developing this approach in the teaching of deaf children. Due to the complexity of thinking skills it further recommends that this area should be taught as a separate topic that can inform other subjects.

  8. Deaf Liberation Theology and Social Justice

    Directory of Open Access Journals (Sweden)

    Hannah Lewis

    2017-10-01

    Full Text Available Deaf Liberation Theology is a branch of theology that has been developed over the past twenty years, with the book Deaf Liberation Theology published by Ashgate in 2007 (Lewis 2007 as a focal point of this development. This article briefly looks at the roots of Deaf Liberation Theology in both the concept of Deaf people as an oppressed linguistic minority and the principles of Liberation theology as an engaged contextual theology using the methodology of the hermeneutical circle. It then seeks to examine the impact of Deaf Liberation Theology in practice over the past decade, in particular the impact especially through increasing self-confidence and self-esteem so that deaf people themselves feel empowered to work for social justice. It will use personal reflections by a number of deaf individuals in the UK as source material, and look at how this experience and developments in Deaf studies might develop into the future to further develop social justice.

  9. Characterizing auditory processing and perception in individual listeners with sensorineural hearing loss

    DEFF Research Database (Denmark)

    Jepsen, Morten Løve; Dau, Torsten

    2011-01-01

    This study considered consequences of sensorineural hearing loss in ten listeners. The characterization of individual hearing loss was based on psychoacoustic data addressing audiometric pure-tone sensitivity, cochlear compression, frequency selectivity, temporal resolution, and intensity discrim...

  10. Films about the deaf: the representations of deaf and sign languages

    OpenAIRE

    Carolina Hessel Silveira

    2009-01-01

    This paper analyzes two films about deafness which have not been investigated in the Brazilian academic context. They are Mandy (directed by Alexander Mackendrick, 1952, England) and After the Silence (by Fred Gerber, 1996, USA). The analysis is supported by Cultural Studies and Deaf Studies, especially on the concepts of cultural pedagogies, deaf culture, deaf identities, sign language, as well as on the analysis of other films about deaf people conducted by Thoma (2004). Both films are clas...

  11. Acquiring English as a second language via print: the task for deaf children.

    Science.gov (United States)

    Hoffmeister, Robert J; Caldwell-Harris, Catherine L

    2014-08-01

    Only a minority of profoundly deaf children read at age-level. We contend this reflects cognitive and linguistic impediments from lack of exposure to a natural language in early childhood, as well as the inherent difficulty of learning English only through the written modality. Yet some deaf children do acquire English via print. The current paper describes a theoretical model of how children could, in principle, acquire a language via reading and writing. The model describes stages of learning which represent successive, conceptual insights necessary for second/foreign language learning via print. Our model highlights the logical difficulties present when one cannot practice a language outside of reading/writing, such as the necessity of translating to a first language, the need for explicit instruction, and difficulty that many deaf children experience in understanding figurative language. Our model explains why learning to read is often a protracted process for deaf children and why many fail to make progress after some initial success. Because language acquisition is thought to require social interaction, with meaning cued by extralinguistic context, the ability of some deaf individuals to acquire language through print represents an overlooked human achievement worthy of greater attention by cognitive scientists. Copyright © 2014 Elsevier B.V. All rights reserved.

  12. Concurrent and Longitudinal Predictors of Reading for Deaf and Hearing Children in Primary School.

    Science.gov (United States)

    Harris, Margaret; Terlektsi, Emmanouela; Kyle, Fiona Elizabeth

    2017-04-01

    Forty-one children with severe-profound prelingual hearing loss were assessed on single word reading, reading comprehension, English vocabulary, phonological awareness and speechreading at three time points, 1 year apart (T1-T3). Their progress was compared with that of a group of hearing children of similar nonverbal IQ, initially reading at the same level. Single word reading improved at each assessment point for the deaf children but there was no growth in reading comprehension from T2 to T3. There were no differences between children with cochlear implants and those with hearing aids on either reading measure but orally educated children had higher scores than children who signed in the classroom. English vocabulary and speechreading were the most consistent longitudinal predictors of reading for the deaf children. Phonological awareness was the most consistent longitudinal predictor for the hearing group and also a concurrent predictor of reading at T3 for both groups. There were many more significant correlations among the various measures for the deaf children than the hearing at both T1 and T3, suggesting that skills underpinning reading, including phonological awareness and vocabulary, are more closely related for deaf children. Implications of these findings for of deaf children's literacy are explored. © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  13. Ira Hirsh and oral deaf education: The role of audition in language development

    Science.gov (United States)

    Geers, Ann

    2002-05-01

    Prior to the 1960s, the teaching of speech to deaf children consisted primarily of instruction in lip reading and tactile perception accompanied by imitative exercises in speech sound production. Hirsh came to Central Institute for the Deaf with an interest in discovering the auditory capabilities of normal-hearing listeners. This interest led him to speculate that more normal speech development could be encouraged in deaf children by maximizing use of their limited residual hearing. Following the tradition of Max Goldstein, Edith Whetnall, and Dennis Fry, Hirsh gave scientific validity to the use of amplified speech as the primary avenue to oral language development in prelingually deaf children. This ``auditory approach,'' combined with an emphasis on early intervention, formed the basis for auditory-oral education as we know it today. This presentation will examine how the speech perception, language, and reading skills of prelingually deaf children have changed as a result of improvements in auditory technology that have occurred over the past 30 years. Current data from children using cochlear implants will be compared with data collected earlier from children with profound hearing loss who used hearing aids. [Work supported by NIH.

  14. The Contribution of Verbal Working Memory to Deaf Children’s Oral and Written Production

    Science.gov (United States)

    Arfé, Barbara; Rossi, Cristina; Sicoli, Silvia

    2015-01-01

    This study investigated the contribution of verbal working memory to the oral and written story production of deaf children. Participants were 29 severely to profoundly deaf children aged 8–13 years and 29 hearing controls, matched for grade level. The children narrated a picture story orally and in writing and performed a reading comprehension test, the Wechsler Intelligence Scale for Children-Fourth Edition forward digit span task, and a reading span task. Oral and written stories were analyzed at the microstructural (i.e., clause) and macrostructural (discourse) levels. Hearing children’s stories scored higher than deaf children’s at both levels. Verbal working memory skills contributed to deaf children’s oral and written production over and above age and reading comprehension skills. Verbal rehearsal skills (forward digit span) contributed significantly to deaf children’s ability to organize oral and written stories at the microstructural level; they also accounted for unique variance at the macrostructural level in writing. Written story production appeared to involve greater verbal working memory resources than oral story production. PMID:25802319

  15. Lateralization effects during semantic and rhyme judgement tasks in deaf and hearing subjects.

    Science.gov (United States)

    D'Hondt, Murielle; Leybaert, Jacqueline

    2003-11-01

    A visual hemifield experiment investigated hemispheric specialization among hearing children and adults and prelingually, profoundly deaf youngsters who were exposed intensively to Cued Speech (CS). Of interest was whether deaf CS users, who undergo a development of phonology and grammar of the spoken language similar to that of hearing youngsters, would display similar laterality patterns in the processing of written language. Semantic, rhyme, and visual judgement tasks were used. In the visual task no VF advantage was observed. A RVF (left hemisphere) advantage was obtained for both the deaf and the hearing subjects for the semantic task, supporting Neville's claim that the acquisition of competence in the grammar of language is critical in establishing the specialization of the left hemisphere for language. For the rhyme task, however, a RVF advantage was obtained for the hearing subjects, but not for the deaf ones, suggesting that different neural resources are recruited by deaf and hearing subjects. Hearing the sounds of language may be necessary to develop left lateralised processing of rhymes.

  16. The effect of social skills training on perceived competence of female adolescents with deafness.

    Science.gov (United States)

    Soleimanieh Naeini, Tahereh; Keshavarzi Arshadi, Farnaz; Hatamizadeh, Nikta; Bakhshi, Enayatollah

    2013-12-01

    Although there are considerable researches on effectiveness of social skills training, little information is available on the effects of such training on perceived competence of adolescents with deafness. This study was conducted in special school settings to determine the effects of social skills training on perceived competence of female adolescents with deafness. A prepost quasi-experimental design was used to perform the study. Sixty nine female students with deafness who were enrolled in all of the four different special secondary schools in Tehran, Iran, between 2010 and 2011 participated in this research. Two of four secondary schools were randomly allocated to the intervention group (33 students), and the other two to the control group (36 students). The participants were between 11 and 21 years (Mean = 15.43; SD = 1.89), and more than three fourth of each groups ( i.e. 28 students in each groups) were affected by profound hearing impairment . The intervention group participated in twelve bi-weekly sessions. Pretest and posttest data were collected using the 'Hearing Impaired Children Self-Image Test'. The questionnaire was filled by an interviewer. This questionnaire asks students about their feeling toward their own competence in domains of cognitive, physical, socio-emotional and communication competence and school adjustment. The data was analyzed by using SPSS software, version 16. The intervention led to significant improvement in total perceived competence scores of adolescents with deafness (P deafness would improve their sense of competence, and emotional well being.

  17. Previously Unreported Biallelic Mutation in DNAJC19: Are Sensorineural Hearing Loss and Basal Ganglia Lesions Additional Features of Dilated Cardiomyopathy and Ataxia (DCMA) Syndrome?

    Science.gov (United States)

    Ucar, Sema Kalkan; Mayr, Johannes A; Feichtinger, René G; Canda, Ebru; Çoker, Mahmut; Wortmann, Saskia B

    2017-01-01

    Dilated cardiomyopathy (DCM), non-progressive cerebellar ataxia (A), testicular dysgenesis, growth failure, and 3-methylglutaconic aciduria are the hallmarks of DNAJC19 defect (or DCMA syndrome) due to biallelic mutations in DNAJC19. To date DCMA syndrome has been reported in 19 patients from Canada and in two Finnish siblings. The underlying pathomechanism is unknown; however, DNAJC19 is presumed to be involved in mitochondrial membrane related processes (e.g., protein import and cardiolipin remodeling). Here, we report an additional patient with progressive cerebellar atrophy and white matter changes. A Turkish boy presented at age 2 months with dilated cardiomyopathy (initially worsening then stabilizing in the second year of life), growth failure, bilateral cryptorchidism, and facial dysmorphism. Mental and motor developmental were, respectively, moderately and severely delayed. Profound intentional tremor and dyskinesia, spasticity (particularly at the lower extremities), and dystonia were observed. Sensorineural hearing loss was also diagnosed. MRI showed bilateral basal ganglia signal alterations. Plasma lactate levels were increased, as was urinary excretion of 3-methylglutaconic acid. He deceased aged 3 years. Sanger Sequencing of DNAJC19 confirmed the clinical diagnosis of DNAJC19 defect by revealing the previously unreported homozygous stop mutation c.63delC (p.Tyr21*). Investigation of enzymes of mitochondrial energy metabolism revealed decreased activity of cytochrome c oxidase in muscle tissue. Sensorineural hearing loss and bilateral basal ganglia lesions are common symptoms of mitochondrial disorders. This is the first report of an association with DNAJC19 defect.

  18. Motor proficiency and dynamic visual acuity in children with bilateral sensorineural hearing loss.

    Science.gov (United States)

    Martin, Willemien; Jelsma, Jennifer; Rogers, Christine

    2012-10-01

    Due to the close relationship between the cochlea and the peripheral vestibular system, the function of the vestibular system may be impaired in children with sensorineural hearing loss. The aims of this study were to determine the prevalence of impairments of motor performance and dynamic visual acuity, and the nature and extent of interaction between these in children with sensorineural hearing loss between the ages of 4 and 14 years. This research utilized a correlational, cross-sectional, descriptive design. Thirty-two children with sensorineural hearing loss were matched according to age and gender with children with no hearing impairment. Motor performance was evaluated by means of the Movement Assessment Battery for Children-2 and dynamic visual acuity was evaluated with the dynamic visual acuity test. The performances of the two groups on the different tests were then compared. The one-sided chi-square test or Fisher's exact test was used to determine whether there was any association between sensorineural hearing loss, impaired motor performance and poor dynamic visual acuity. The Mann-Whitney U-test was used to determine the difference between children with sensorineural hearing loss and those with normal hearing on the Movement Assessment Battery for Children-2. Forward stepwise regression was used to establish the predictors of the Movement Assessment Battery for Children-2 total standard score. The Kruskal-Wallis test was used to compare scores of children with normal hearing and those with a mild to moderate sensorineural hearing loss on the Movement Assessment Battery for Children-2. Reduced dynamic visual acuity is associated with sensorineural hearing loss (p=0.026). Motor performance is dependent on dynamic visual acuity and severity of sensorineural hearing loss (r(2)=0.41, p=0.001). The results of this study indicate that in children with sensorineural hearing loss, the prevalence of reduced dynamic visual acuity is 15.6% and of motor impairment

  19. Static and dynamic balance of children and adolescents with sensorineural hearing loss

    OpenAIRE

    Melo, Renato de Souza; Marinho, Sônia Elvira dos Santos; Freire, Maryelly Evelly Araújo; Souza, Robson Arruda; Damasceno, Hélio Anderson Melo; Raposo, Maria Cristina Falcão

    2017-01-01

    ABSTRACT Objective To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. Methods A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Results Hearing loss students showed more changes in s...

  20. Isolated Contralateral Sudden Sensorineural Hearing Loss: An Unusual Manifestation of Pontine Infarct

    OpenAIRE

    Eluvathingal Muttikkal, Thomas Jose; Kesser, Bradley W.; Mukherjee, Sugoto

    2014-01-01

    Unilateral, acute onset sensorineural hearing loss (“sudden sensorineural hearing loss” [SSNHL]) as an isolated event without other associated neurological deficits usually results from a lesion of the cochlea. Lesions in the ascending central auditory pathways cranial to the cochlear nucleus seldom result in unilateral hearing loss due to decussation of the central auditory pathways at multiple levels. We describe a patient with a tiny acute infarct in the right pons resulting in isolated ac...

  1. Telehealth and the Deaf: A Comparison Study

    Science.gov (United States)

    Wilson, Jaime A. B.; Wells, M. Gawain

    2009-01-01

    Within the deaf population, an extreme mental health professional shortage exists that may be alleviated with videoconferencing technology--also known as telehealth. Moreover, much needed mental health education within the deaf population remains largely inaccessible. Researchers have warned that the deaf population may remain underserved if…

  2. Understanding Deaf Readers: An Interpretative Phenomenological Analysis

    Science.gov (United States)

    Kelstone, Aaron Weir

    2013-01-01

    The development of reading skills, beyond a functional level, is difficult for most deaf readers. Standardized testing demonstrates a median 4th grade reading level that remains consistent even after national norming of the Stanford Achievement test on the population of deaf school children. Deaf education continues to generate various educational…

  3. Deaf child sexual education and family leadership

    OpenAIRE

    García, Mirna Maura

    2010-01-01

    This paper is an approach to the study of the role of the family in sexual education of deaf children and adolescents. The difference between hearing and deaf families is taken into consideration. Likewise, hints that favor communication between deaf children and hearing parents are given.

  4. Deaf Parents and Their Hearing Children.

    Science.gov (United States)

    Singleton, Jenny L.; Tittle, Matthew D.

    2000-01-01

    This literature review begins with a description of the deaf community, their language and culture; then describes communication patterns and parenting issues in deaf-parented families; examines the role of the hearing child in a deaf family and how that affects their functioning in the hearing world; and finally discusses considerations and…

  5. Deaf child sexual education and family leadership

    Directory of Open Access Journals (Sweden)

    García, Mirna Maura

    2010-07-01

    Full Text Available This paper is an approach to the study of the role of the family in sexual education of deaf children and adolescents. The difference between hearing and deaf families is taken into consideration. Likewise, hints that favor communication between deaf children and hearing parents are given.

  6. Development of Deaf Identity: An Ethnographic Study

    Science.gov (United States)

    McIlroy, Guy; Storbeck, Claudine

    2011-01-01

    This ethnographic study explores the identity development of 9 deaf participants through the narratives of their educational experiences in either mainstream or special schools for the Deaf. This exploration goes beyond a binary conceptualization of deaf identity that allows for only the medical and social models and proposes a bicultural…

  7. Representations of Sound in American Deaf Literature

    Science.gov (United States)

    Rosen, Russell S.

    2007-01-01

    Sound plays a prominent role in narrative description of characters and environs in mainstream American literature. A review of American Deaf literature shows that the representations of sound held for deaf writers are in extensional and oppositional terms. American deaf writers, in their descriptions of entities, characters, functions, and…

  8. No Dummies: Deafness, Baseball, and American Culture

    Science.gov (United States)

    Edwards, R. A. R.

    2012-01-01

    This article begins by examining the historical and social factors that led to 1901 being the "deafest" year in major league baseball history with four deaf players. In particular, the author discusses the career of William Ellsworth "Dummy" Hoy, a deaf man from Ohio who became the most celebrated deaf player in history and…

  9. Teaching deaf learners. Psychological and developmental foundations

    NARCIS (Netherlands)

    Knoors, H.E.T.; Marschark, M.

    2014-01-01

    Teaching Deaf Learners: Psychological and Developmental Foundations explores how deaf students (children and adolescents) learn and the conditions that support their reaching their full cognitive potential -- or not. Beginning with an introduction to teaching and learning of both deaf and hearing

  10. Vocational Evaluation of Severely Disabled Deaf Clients.

    Science.gov (United States)

    Watson, Douglas

    Research and practice in deafness rehabilitation show that evaluation services for severely disabled deaf clients can best be provided within a "total adjustment environment" which incorporates a number of special program considerations associated with the evaluation of deaf clients. Four of these considerations are (1) a rehabilitation…

  11. Family Therapy with Deaf Member Families.

    Science.gov (United States)

    Sloman, Leon; And Others

    1987-01-01

    Examines how family therapists can be more responsive to the unique needs and problems of deaf family members. Compares methods of training in communication for deaf children, addressing the conflicts that may accompany the adoption of a given method. Stresses the pivotal role of communication problems between hearing and deaf family members in…

  12. Pages from the Past: Causes of Deafness.

    Science.gov (United States)

    Turner, W. W.

    1997-01-01

    This reprint of an historical article from 1847 discusses the principal causes of congenital deafness, including constitutional tendency to deafness in the parents, mental impressions of the mother previous to the birth of her child, and intermarriage of near relations. Causes of accidental deafness are also discussed, including injuries from…

  13. Novel subtype of congenital partial lipodystrophy with mandibular hypoplasia, sensorineural deafness and short stature of unknown genetic origin.

    Science.gov (United States)

    Sasaki, Haruka; Ohkubo, Kumiko; Kobayashi, Kunihisa; Tajiri, Yuji; Ugi, Satoshi; Kashwagi, Atsunori

    2017-01-01

    The unique clinical manifestations of congenital partial lipodystrophy are herein reported due to its rarity. © 2017 The Authors. Journal of Diabetes Investigation published by Asian Association for the Study of Diabetes (AASD) and John Wiley & Sons Australia, Ltd.

  14. Mutations in the gene encoding B1 subunit of H+-ATPase cause renal tubular acidosis with sensorineural deafness

    NARCIS (Netherlands)

    Karet, FE; Finberg, KE; Nelson, RD; Nayir, A; Mocan, H; Sanjad, SA; Rodriguez-Soriano, J; Santos, F; Cremers, CWRJ; di Pietro, A; Hoffbrand, BI; Winiarski, J; Bakkaloglu, A; Ozen, S; Dusunsel, R; Goodyer, P; Hulton, SA; Wu, DK; Skvorak, AB; Morton, CC; Cunningham, MJ; Jha, [No Value; Lifton, RP

    H+-ATPases are ubiquitous in nature; V-ATPases pump protons against an electrochemical gradient, whereas F-ATPases reverse the process, synthesizing ATP. We demonstrate here that mutations in ATP6B1, encoding the B-subunit of the apical proton pump mediating distal nephron acid secretion, cause

  15. Sensorineural hearing loss in patients with Kawasaki disease.

    Science.gov (United States)

    Park, Sun Young; Kim, Young Hyun; Kim, Yeo Hyang; Hyun, Myung Chul; Lee, Young Hwan

    2015-11-01

    Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.

  16. Mutations in TUBB4B Cause a Distinctive Sensorineural Disease.

    Science.gov (United States)

    Luscan, Romain; Mechaussier, Sabrina; Paul, Antoine; Tian, Guoling; Gérard, Xavier; Defoort-Dellhemmes, Sabine; Loundon, Natalie; Audo, Isabelle; Bonnin, Sophie; LeGargasson, Jean-François; Dumont, Julien; Goudin, Nicolas; Garfa-Traoré, Meriem; Bras, Marc; Pouliet, Aurore; Bessières, Bettina; Boddaert, Nathalie; Sahel, José-Alain; Lyonnet, Stanislas; Kaplan, Josseline; Cowan, Nicholas J; Rozet, Jean-Michel; Marlin, Sandrine; Perrault, Isabelle

    2017-12-07

    Leber congenital amaurosis (LCA) is a neurodegenerative disease of photoreceptor cells that causes blindness within the first year of life. It occasionally occurs in syndromic metabolic diseases and plurisystemic ciliopathies. Using exome sequencing in a multiplex family and three simplex case subjects with an atypical association of LCA with early-onset hearing loss, we identified two heterozygous mutations affecting Arg391 in β-tubulin 4B isotype-encoding (TUBB4B). Inspection of the atomic structure of the microtubule (MT) protofilament reveals that the β-tubulin Arg391 residue contributes to a binding pocket that interacts with α-tubulin contained in the longitudinally adjacent αβ-heterodimer, consistent with a role in maintaining MT stability. Functional analysis in cultured cells overexpressing FLAG-tagged wild-type or mutant TUBB4B as well as in primary skin-derived fibroblasts showed that the mutant TUBB4B is able to fold, form αβ-heterodimers, and co-assemble into the endogenous MT lattice. However, the dynamics of growing MTs were consistently altered, showing that the mutations have a significant dampening impact on normal MT growth. Our findings provide a link between sensorineural disease and anomalies in MT behavior and describe a syndromic LCA unrelated to ciliary dysfunction. Copyright © 2017 American Society of Human Genetics. All rights reserved.

  17. MicroRNAs in sensorineural diseases of the ear

    Directory of Open Access Journals (Sweden)

    Kathy eUshakov

    2013-12-01

    Full Text Available Non-coding microRNAs have a fundamental role in gene regulation and expression in almost every multicellular organism. Only discovered in the last decade, microRNAs are already known to play a leading role in many aspects of disease. In the vertebrate inner ear, microRNAs are essential for controlling development and survival of hair cells. Moreover, dysregulation of microRNAs has been implicated in sensorineural hearing impairment, as well as in other ear diseases such as cholesteatomas, vestibular schwannomas and otitis media. Due to the inaccessibility of the ear in humans, animal models have provided the optimal tools to study microRNA expression and function, in particular mice and zebrafish. A major focus of current research has been to discover the targets of the microRNAs expressed in the inner ear, in order to determine the regulatory pathways of the auditory and vestibular systems. The potential for microRNA manipulation in development of therapeutic tools for hearing impairment is as yet unexplored, paving the way for future work in the field.

  18. Endothelial progenitor cells in sudden sensorineural hearing loss.

    Science.gov (United States)

    Quaranta, Nicola; Ramunni, Alfonso; De Luca, Concetta; Brescia, Paola; Dambra, Porzia; De Tullio, Giacomina; Vacca, Angelo; Quaranta, Antonio

    2011-04-01

    Endothelial progenitor cells (EPCs) are a unique subtype of circulating cells with properties similar to those of embryonal angioblasts. They have the potential to proliferate and to differentiate into mature endothelial cells. EPCs are reduced in patients with vascular risk factors due to a decreased mobilization, an increased consumption at the site of damage or a reduced half-life. The results of this study confirm the existence of an endothelial dysfunction in patients with sudden sensorineural hearing loss (SSHL) and support the vascular involvement in the pathogenesis of the disease. The aim of this study was to evaluate the concentration of EPCs in patients affected by SSHL. Twenty-one patients affected by SSHL were evaluated. The number of EPCs was analyzed by flow cytometry analysis of peripheral blood CD34+KDR+CD133+ cells. Circulating levels of EPCs were significantly lower in SSHL patients compared with controls. In particular, CD34+KDR+ cells and CD34+CD133+KDR+ cells were significantly reduced (p < 0.05).

  19. The management of children with Down syndrome and profound hearing loss.

    Science.gov (United States)

    Phelan, E; Pal, R; Henderson, L; Green, K M J; Bruce, I A

    2016-01-01

    Although, the association between Down syndrome (DS) and conductive hearing loss is well recognized, the fact that a small proportion of these children may have a severe to profound sensorineural hearing loss that could benefit from cochlear implantation (CI) is less well understood. The management of significant co-morbidities in children with DS can delay initial diagnosis of hearing impairment and assessment of suitability for CI can likewise be challenging, due to difficulties conditioning to behavioural hearing tests. We performed a retrospective case note review of three children with DS referred to the Manchester Cochlear Implant Programme. Three illustrative cases are described including CI in a 4 years old. Using conventional outcome measurement instruments, the outcome could be considered to be suboptimal with a Categories of Auditory Performance score of 4 at 6 months post-op and at last follow up. In part, this is likely to reflect the delay in implantation, but the role of cognitive impairment must be considered. The cases described emphasize the importance of comprehensive radiological and audiological assessment in children with DS being considered for CI. The influence of cognitive impairment upon outcome of CI must be taken into account, but should not be considered a contra-indication to implantation in children with DS. Benefit that might be considered limited when quantified using existing general outcome measurement instruments, may have a significant impact upon psychosocial development and quality of life in children with significant cognitive impairment, or other additional needs.

  20. Deafness and motor abilities level

    Directory of Open Access Journals (Sweden)

    A Zwierzchowska

    2008-09-01

    Full Text Available The audition injury hinders some motor motions and the organised coordination at the higher level and may be a cause of disturbances and disorder in some motor abilities adoption. It was assumed that deafness including its aetiology and injury mechanism may significantly influence the motor development of human being. The study aimed in checking if the deafness, as a result of various unfavourable factors, determines the motor development of children and youngsters. Consequently the dependency between qualitative features i.e.: signed motor level and aetiology, audition injury mechanism and the deafness degree was examined. The mechanism and aetiology of hearing correlated with the motor abilities displayed statistically significant dependencies in few motor trials only. Revealed correlations regarded mostly the coordination trials excluding the flexibility one. Statistically significant dependencies between the audition diminution and the motor abilities level were not found.

  1. Deaf children learning to sign

    Directory of Open Access Journals (Sweden)

    Jim Kyle

    2001-01-01

    Full Text Available It used to be thought that deaf children had a language difficulty. Research we have carried out on deaf children in deaf families from the age of three months, indicates that deaf children learn sign language as effectively as hearing children learn to speak. In contrast, deaf children from hearing homes, even in signing programmes at school lag behind in the acquisition of sign language even up to the age of 11 years. Some initial intervention work has been carried out with families to introduce sign language earlier and several possible means of improving the language environment of deaf children are explored in this paper. Costumava-se pensar que as crianças surdas tinham dificuldade de linguagem. Uma pesquisa que realizamos com crianças surdas, de famílias surdas, a partir de 3 meses de idade, indica que crianças surdas aprendem a língua de sinais tão eficazmente quanto crianças ouvintes aprendem a falar. Em contraste, crianças surdas, de lares ouvintes, mesmo estando em programas para o aprendizado de sinais na escola, ficam atrás na aquisição da língua de sinais até a idade de 11 anos de idade. Alguns trabalhos iniciais de intervenção, que têm sido realizados com as famílias para introduzir a língua de sinais mais cedo, bem como vários meios possíveis de enriquecer o meio lingüístico de crianças surdas são explorados nesse artigo.

  2. Understanding minds: early cochlear implantation and the development of theory of mind in children with profound hearing impairment.

    Science.gov (United States)

    Sundqvist, Annette; Lyxell, Björn; Jönsson, Radoslava; Heimann, Mikael

    2014-03-01

    The present study investigates how auditory stimulation from cochlear implants (CI) is associated with the development of Theory of Mind (ToM) in severely and profoundly hearing impaired children with hearing parents. Previous research has shown that deaf children of hearing parents have a delayed ToM development. This is, however, not always the case with deaf children of deaf parents, who presumably are immersed in a more vivid signing environment. Sixteen children with CI (4.25 to 9.5 years of age) were tested on measures of cognitive and emotional ToM, language and cognition. Eight of the children received their first implant relatively early (before 27 months) and half of them late (after 27 months). The two groups did not differ in age, gender, language or cognition at entry of the study. ToM tests included the unexpected location task and a newly developed Swedish social-emotional ToM test. The tests aimed to test both cognitive and emotional ToM. A comparison group of typically developing hearing age matched children was also added (n=18). Compared to the comparison group, the early CI-group did not differ in emotional ToM. The late CI-group differed significantly from the comparison group on both the cognitive and emotional ToM tests. The results revealed that children with early cochlear implants solved ToM problems to a significantly higher degree than children with late implants, although the groups did not differ on language or cognitive measures at baseline. The outcome suggests that early cochlear implantation for deaf children in hearing families, in conjunction with early social and communicative stimulation in a language that is native to the parents, can provide a foundation for a more normalized ToM development. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  3. The impact of cochlear implantation on speech understanding, subjective hearing performance, and tinnitus perception in patients with unilateral severe to profound hearing loss.

    Science.gov (United States)

    Távora-Vieira, Dayse; Marino, Roberta; Acharya, Aanand; Rajan, Gunesh P

    2015-03-01

    This study aimed to determine the impact of cochlear implantation on speech understanding in noise, subjective perception of hearing, and tinnitus perception of adult patients with unilateral severe to profound hearing loss and to investigate whether duration of deafness and age at implantation would influence the outcomes. In addition, this article describes the auditory training protocol used for unilaterally deaf patients. This is a prospective study of subjects undergoing cochlear implantation for unilateral deafness with or without associated tinnitus. Speech perception in noise was tested using the Bamford-Kowal-Bench speech-in-noise test presented at 65 dB SPL. The Speech, Spatial, and Qualities of Hearing Scale and the Abbreviated Profile of Hearing Aid Benefit were used to evaluate the subjective perception of hearing with a cochlear implant and quality of life. Tinnitus disturbance was measured using the Tinnitus Reaction Questionnaire. Data were collected before cochlear implantation and 3, 6, 12, and 24 months after implantation. Twenty-eight postlingual unilaterally deaf adults with or without tinnitus were implanted. There was a significant improvement in speech perception in noise across time in all spatial configurations. There was an overall significant improvement on the subjective perception of hearing and quality of life. Tinnitus disturbance reduced significantly across time. Age at implantation and duration of deafness did not influence the outcomes significantly. Cochlear implantation provided significant improvement in speech understanding in challenging situations, subjective perception of hearing performance, and quality of life. Cochlear implantation also resulted in reduced tinnitus disturbance. Age at implantation and duration of deafness did not seem to influence the outcomes.

  4. Staffs' documentation of participation for adults with profound intellectual disability or profound intellectual and multiple disabilities.

    Science.gov (United States)

    Talman, Lena; Gustafsson, Christine; Stier, Jonas; Wilder, Jenny

    2017-06-21

    This study investigated what areas of International Classification of Functioning, Disability and Health were documented in implementation plans for adults with profound intellectual disability or profound intellectual and multiple disabilities with focus on participation. A document analysis of 17 implementation plans was performed and International Classification of Functioning, Disability and Health was used as an analytic tool. One hundred and sixty-three different codes were identified, especially in the components Activities and participation and Environmental factors. Participation was most frequently coded in the chapters Community, social and civic life and Self-care. Overall, the results showed that focus in the implementation plans concerned Self-care and Community, social and civic life. The other life areas in Activities and participation were seldom, or not at all, documented. A deeper focus on participation in the implementation plans and all life areas in the component Activities and participation is needed. It is important that the documentation clearly shows what the adult wants, wishes, and likes in everyday life. It is also important to ensure that the job description for staff contains both life areas and individual preferences so that staff have the possibility to work to fulfill social and individual participation for the target group. Implications for rehabilitation There is a need for functioning working models to increase participation significantly for adults with profound intellectual disability or profound intellectual and multiple disabilities. For these adults, participation is achieved through the assistance of others and support and services carried out must be documented in an implementation plan. The International Classification of Functioning, Disability and Health can be used to support staff and ensure that information about the most important factors in an individual's functioning in their environment is not omitted in

  5. Phonological processing of rhyme in spoken language and location in sign language by deaf and hearing participants: a neurophysiological study.

    Science.gov (United States)

    Colin, C; Zuinen, T; Bayard, C; Leybaert, J

    2013-06-01

    Sign languages (SL), like oral languages (OL), organize elementary, meaningless units into meaningful semantic units. Our aim was to compare, at behavioral and neurophysiological levels, the processing of the location parameter in French Belgian SL to that of the rhyme in oral French. Ten hearing and 10 profoundly deaf adults performed a rhyme judgment task in OL and a similarity judgment on location in SL. Stimuli were pairs of pictures. As regards OL, deaf subjects' performances, although above chance level, were significantly lower than that of hearing subjects, suggesting that a metaphonological analysis is possible for deaf people but rests on phonological representations that are less precise than in hearing people. As regards SL, deaf subjects scores indicated that a metaphonological judgment may be performed on location. The contingent negative variation (CNV) evoked by the first picture of a pair was similar in hearing subjects in OL and in deaf subjects in OL and SL. However, an N400 evoked by the second picture of the non-rhyming pairs was evidenced only in hearing subjects in OL. The absence of N400 in deaf subjects may be interpreted as the failure to associate two words according to their rhyme in OL or to their location in SL. Although deaf participants can perform metaphonological judgments in OL, they differ from hearing participants both behaviorally and in ERP. Judgment of location in SL is possible for deaf signers, but, contrary to rhyme judgment in hearing participants, does not elicit any N400. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  6. Outcomes of cochlear implantation in deaf children of deaf parents: comparative study.

    Science.gov (United States)

    Hassanzadeh, S

    2012-10-01

    This retrospective study compared the cochlear implantation outcomes of first- and second-generation deaf children. The study group consisted of seven deaf, cochlear-implanted children with deaf parents. An equal number of deaf children with normal-hearing parents were selected by matched sampling as a reference group. Participants were matched based on onset and severity of deafness, duration of deafness, age at cochlear implantation, duration of cochlear implantation, gender, and cochlear implant model. We used the Persian Auditory Perception Test for the Hearing Impaired, the Speech Intelligibility Rating scale, and the Sentence Imitation Test, in order to measure participants' speech perception, speech production and language development, respectively. Both groups of children showed auditory and speech development. However, the second-generation deaf children (i.e. deaf children of deaf parents) exceeded the cochlear implantation performance of the deaf children with hearing parents. This study confirms that second-generation deaf children exceed deaf children of hearing parents in terms of cochlear implantation performance. Encouraging deaf children to communicate in sign language from a very early age, before cochlear implantation, appears to improve their ability to learn spoken language after cochlear implantation.

  7. Profound Olfactory Dysfunction in Myasthenia Gravis

    Science.gov (United States)

    Leon-Sarmiento, Fidias E.; Bayona, Edgardo A.; Bayona-Prieto, Jaime; Osman, Allen; Doty, Richard L.

    2012-01-01

    In this study we demonstrate that myasthenia gravis, an autoimmune disease strongly identified with deficient acetylcholine receptor transmission at the post-synaptic neuromuscular junction, is accompanied by a profound loss of olfactory function. Twenty-seven MG patients, 27 matched healthy controls, and 11 patients with polymiositis, a disease with peripheral neuromuscular symptoms analogous to myasthenia gravis with no known central nervous system involvement, were tested. All were administered the University of Pennsylvania Smell Identification Test (UPSIT) and the Picture Identification Test (PIT), a test analogous in content and form to the UPSIT designed to control for non-olfactory cognitive confounds. The UPSIT scores of the myasthenia gravis patients were markedly lower than those of the age- and sex-matched normal controls [respective means (SDs) = 20.15 (6.40) & 35.67 (4.95); p<0.0001], as well as those of the polymiositis patients who scored slightly below the normal range [33.30 (1.42); p<0.0001]. The latter finding, along with direct monitoring of the inhalation of the patients during testing, implies that the MG-related olfactory deficit is unlikely due to difficulties sniffing, per se. All PIT scores were within or near the normal range, although subtle deficits were apparent in both the MG and PM patients, conceivably reflecting influences of mild cognitive impairment. No relationships between performance on the UPSIT and thymectomy, time since diagnosis, type of treatment regimen, or the presence or absence of serum anti-nicotinic or muscarinic antibodies were apparent. Our findings suggest that MG influences olfactory function to the same degree as observed in a number of neurodegenerative diseases in which central nervous system cholinergic dysfunction has been documented. PMID:23082113

  8. Deaf Community Mobilization in Uruguay

    Science.gov (United States)

    Lockwood, Elizabeth

    2010-01-01

    There are more than 50 million (or approximately 15%) persons with disabilities living in Latin America and the Caribbean: 80 percent live in impoverished conditions, lack employment, and encounter social exclusion. Deaf and hard of hearing persons are particularly impacted as they are frequently denied access to most sectors of society. This…

  9. Considerations on Deafness and Homosexuality.

    Science.gov (United States)

    Phaneuf, Jean

    1987-01-01

    The article reviews the literature and discusses emotions, attitudes, and general reactions toward homosexuality in deaf individuals. The "coming out" process is discussed, with related social, familial, and personal implications, as well as the role of professional counseling and intervention. (Author/DB)

  10. Deafness and Autistic Spectrum Disorders

    Science.gov (United States)

    Vernon, McCay; Rhodes, Anthony

    2009-01-01

    An orientation to autistic spectrum disorders (ASD), also known as autism, is provided, and the specific syndrome of autism and deafness is addressed. The two conditions have in common a major problem: communication. Case histories are provided, the development of treatment for autism is discussed, and the separate disorders that make up ASD are…

  11. Writing in Young Deaf Children

    Science.gov (United States)

    Williams, Cheri; Mayer, Connie

    2015-01-01

    The authors conducted an integrative review of the research literature on the writing development, writing instruction, and writing assessment of young deaf children ages 3 to 8 years (or preschool through third grade) published between 1990 and 2012. A total of 17 studies were identified that met inclusion criteria. The analysis examined research…

  12. Interpreting Hymns for Deaf Worshippers.

    Science.gov (United States)

    Maxwell, Madeline M.; Boster, Shirley

    1982-01-01

    Discusses the special problems of interpreting hymns written in archaic English and then matching words of a translation to music. Addresses the question of whether competence in ASL and knowledge of signs for religious terms are sufficient for hymns to be of value to deaf worshippers. (EKN)

  13. The Consequence Deafness has on the Psychological and ...

    African Journals Online (AJOL)

    The Consequence Deafness has on the Psychological and Academic Development of deaf students. The case of Alpha special school for the deaf in Addis Ababa, Hermata and Mendera Junior School at Jimma Town.

  14. Evaluative expression in deaf children's written narratives.

    Science.gov (United States)

    van Beijsterveldt, Liesbeth Maria; van Hell, Janet G

    2009-01-01

    Deaf children vary in the use of and proficiency in signed language. The majority of studies on writing skills of children who are deaf did not assess deaf children's proficiency in signed language and/or grouped together deaf children with varying sign language skills. Adopting a bimodal bilingual perspective, we examined evaluative expression, an important narrative tool in both oral/written languages and signed languages, in narratives written in Dutch by deaf children who are proficient in Sign Language of the Netherlands (SLN) and deaf children who are low-proficient in SLN, and hearing monolingual and bilingual children. We hypothesized that deaf children who are proficient in signed language use their knowledge of evaluative expression in signed language to enrich their narratives in written Dutch, and more so than deaf children who are low-proficient in signed language and hearing monolingual and bilingual children. We examined the use of eight different evaluative devices in narratives written by deaf proficiently and low-proficiently signing children, and hearing monolingual and bilingual children. Narratives were also examined for morpho-syntactic errors and use of complex sentences. The results show that proficiently signing deaf children's narratives contain more evaluative devices that enrich the referential structure of the narrative than narratives of low-proficiently signing deaf children, and hearing bilingual and monolingual children. We propose that proficiently signing deaf children use their knowledge of SLN to convey evaluation in their written narratives, and thus have an advantage in enriching their narratives. This study also shows that in order to gain insight into deaf people's writing, it is important to take variations in sign language proficiency into account.

  15. Toward a theory of deaf ethnos: deafnicity -- D/deaf (Homaemon - Homoglosson - Homothreskon).

    Science.gov (United States)

    Eckert, Richard Clark

    2010-01-01

    Should ethnicity be used to interpret relations between the Deaf community and the hearing people? Recent scholarship questioning the merits of Deaf ethnicity suggests a need to reexamine the use of ethnicity when describing Deaf identity and culture. This article provides an overview of key contributions to race and ethnicity discourse in the 20th century, identifies epistemological and ontological errors to avoid, suggests adherence to the classical Greek concept of ethnos as an alternative to ethnie, and argues for the continuing significance of Deaf ethnicity. Specifically, I propose that Deaf ethnicity is a triadic relational nexus that approximates communities of origin, language, and religion. This is expressed as Deafnicity approximately D/deaf (Hómaemon * Homóglosson * Homóthreskon). Deafnicity offers a promising alternative for examining relations between Deaf and hearing communities, exploring variance between nationalized Deaf communities, and expanding our understanding of audism.

  16. Suicide in deaf populations: a literature review.

    Science.gov (United States)

    Turner, Oliver; Windfuhr, Kirsten; Kapur, Navneet

    2007-10-08

    Studies have found that deaf individuals have higher rates of psychiatric disorder than those who are hearing, while at the same time encountering difficulties in accessing mental health services. These factors might increase the risk of suicide. However, the burden of suicidal behaviour in deaf people is currently unknown. The aim of the present review was to provide a summary of literature on suicidal behaviour with specific reference to deaf individuals. The objectives of the review were to establish the incidence and prevalence of suicidal behaviour in deaf populations; describe risk factors for suicidal behaviour in deaf populations; describe approaches to intervention and suicide prevention that have been used in deaf populations. A number of electronic databases (e.g. Medline, PsycINFO, CINAHL, EMBASE, Dissertation Abstracts International, Web of Science, ComDisDome, ASSIA, Education Sage Full Text, Google Scholar, and the grey literature databases FADE and SIGLE) were explored using a combination of key words and medical subject headings as search terms. Reference lists of papers were also searched. The Science and Social Sciences Citation Index electronic databases were used to identify studies that had cited key papers. We also contacted experts and organisations with an interest in the field. Very few studies focussed specifically on suicide in deaf populations. Those studies that were included (n = 13) generally involved small and unrepresentative samples. There were limited data on the rate of suicidal behaviour in deaf people. One study reported evidence of hearing impairment in 0.2% of all suicide deaths. Another found that individuals with tinnitus seen in specialist clinics had an elevated rate of suicide compared to the general population. The rates of attempted suicide in deaf school and college students during the previous year ranged from 1.7% to 18%, with lifetime rates as high as 30%. Little evidence was found to suggest that risk factors for

  17. Prognostic factors for idiopathic sudden sensorineural hearing loss treated with hyperbaric oxygen therapy and intravenous steroids.

    Science.gov (United States)

    Hosokawa, S; Sugiyama, K; Takahashi, G; Takebayashi, S; Mineta, H

    2017-01-01

    This study evaluated the prognosis of idiopathic sudden sensorineural hearing loss when treated with hyperbaric oxygen therapy and intravenous steroids. The clinical data for 334 patients with idiopathic sudden sensorineural hearing loss treated by hyperbaric oxygen therapy and intravenous steroids at our hospital were retrospectively reviewed. These data included the initial averaged five-frequency hearing level, patient age, interval between onset of symptoms and treatment, vertigo as a complication, and co-existence of diabetes mellitus. The overall improvement rate was 69.2 per cent, including better improvement (25.5 per cent), good improvement (21.0 per cent) and fair improvement (22.7 per cent). Hyperbaric oxygen therapy appears to confer a significant additional therapeutic benefit when used in combination with steroid therapy for idiopathic sudden sensorineural hearing loss. If performed early, hyperbaric oxygen therapy may bring about hearing improvement in many patients who are unresponsive to initial therapy.

  18. Hyperventilation-induced nystagmus in vestibular schwannoma and unilateral sensorineural hearing loss.

    Science.gov (United States)

    Mandalà, Marco; Giannuzzi, Annalisa; Astore, Serena; Trabalzini, Franco; Nuti, Daniele

    2013-07-01

    We evaluated the incidence and characteristics of hyperventilation-induced nystagmus (HVN) in 49 patients with gadolinium-enhanced magnetic resonance imaging evidence of vestibular schwannoma and 53 patients with idiopathic unilateral sensorineural hearing loss and normal radiological findings. The sensitivity and specificity of the hyperventilation test were compared with other audio-vestibular diagnostic tests (bedside examination of eye movements, caloric test, auditory brainstem responses) in the two groups of patients. The hyperventilation test scored the highest diagnostic efficiency (sensitivity 65.3 %; specificity 98.1 %) of the four tests in the differential diagnosis of vestibular schwannoma and idiopathic unilateral sensorineural hearing loss. Small tumors with a normal caloric response or caloric paresis were associated with ipsilateral HVN and larger tumors and severe caloric deficits with contralateral HVN. These results confirm that the hyperventilation test is a useful diagnostic test for predicting vestibular schwannoma in patients with unilateral sensorineural hearing loss.

  19. A Phenomenological Study of Online Learning for Deaf Students in Postsecondary Education: A Deaf Perspective

    Science.gov (United States)

    Wooten, Patricia Michelle

    2014-01-01

    This qualitative phenomenological study investigated the effects of online learning for deaf college students as opposed to the mainstream classroom setting. This study specifically analyzed the writing and reading skills of deaf students in general and the development of English literacy of prelingually deaf students and those from non-English…

  20. The Church of Deaf Sociality: Deaf Churchgoing Practices and "Sign Bread and Butter" in Bangalore, India

    Science.gov (United States)

    Friedner, Michele

    2014-01-01

    This article ethnographically analyzes the practices of deaf young adults in Bangalore, India. As sign language is not used by families, schools, or other institutions, the church is a crucial educational space. Churchgoing provides deaf young adults with opportunities to orient themselves toward other deaf young adults, to develop new ideas of…

  1. Toward ethical research practice with deaf participants.

    Science.gov (United States)

    Singleton, Jenny L; Jones, Gabrielle; Hanumantha, Shilpa

    2014-07-01

    In recent years, scholars have been critical of what they consider unethical conduct by researchers whose studies focus on members of the Deaf or signing communities. This is the first empirical study that investigates ethical concerns and recommendations from the perspective of three stakeholder groups (Deaf research participants, researchers, and Deaf studies experts). We analyzed focus group discussions using strategies from grounded theory and community-based participatory research. The themes we identified highlight the need for the broader scientific research community to include linguistically and culturally sensitive research procedures that more adequately protect the rights of Deaf research participants, as well as other marginalized groups. We address the need to increase the number of Deaf scientists and reconsider collaboration practices between Deaf and hearing researchers. © The Author(s) 2014.

  2. Synchronization to auditory and visual rhythms in hearing and deaf individuals

    Science.gov (United States)

    Iversen, John R.; Patel, Aniruddh D.; Nicodemus, Brenda; Emmorey, Karen

    2014-01-01

    A striking asymmetry in human sensorimotor processing is that humans synchronize movements to rhythmic sound with far greater precision than to temporally equivalent visual stimuli (e.g., to an auditory vs. a flashing visual metronome). Traditionally, this finding is thought to reflect a fundamental difference in auditory vs. visual processing, i.e., superior temporal processing by the auditory system and/or privileged coupling between the auditory and motor systems. It is unclear whether this asymmetry is an inevitable consequence of brain organization or whether it can be modified (or even eliminated) by stimulus characteristics or by experience. With respect to stimulus characteristics, we found that a moving, colliding visual stimulus (a silent image of a bouncing ball with a distinct collision point on the floor) was able to drive synchronization nearly as accurately as sound in hearing participants. To study the role of experience, we compared synchronization to flashing metronomes in hearing and profoundly deaf individuals. Deaf individuals performed better than hearing individuals when synchronizing with visual flashes, suggesting that cross-modal plasticity enhances the ability to synchronize with temporally discrete visual stimuli. Furthermore, when deaf (but not hearing) individuals synchronized with the bouncing ball, their tapping patterns suggest that visual timing may access higher-order beat perception mechanisms for deaf individuals. These results indicate that the auditory advantage in rhythmic synchronization is more experience- and stimulus-dependent than has been previously reported. PMID:25460395

  3. Inattentional deafness under dynamic musical conditions

    OpenAIRE

    Koreimann, Sabrina; Strauß, Sabine; Vitouch, Oliver

    2009-01-01

    While inattentional blindness is a modern classic in attention and perception research, analogous phenomena of inattentional deafness are less well-known. In music, inattentional deafness has never been demonstrated under controlled experimental conditions, despite of indirect evidence for related effects. We tested inattentional deafness with real music in both musicians and non-musicians. Participants listened to the first 1’50” of Richard Strauss’ Thus Spake Zarathustra, with the experimen...

  4. Suicide in deaf populations: a literature review

    OpenAIRE

    Kapur Navneet; Windfuhr Kirsten; Turner Oliver

    2007-01-01

    Abstract Background Studies have found that deaf individuals have higher rates of psychiatric disorder than those who are hearing, while at the same time encountering difficulties in accessing mental health services. These factors might increase the risk of suicide. However, the burden of suicidal behaviour in deaf people is currently unknown. The aim of the present review was to provide a summary of literature on suicidal behaviour with specific reference to deaf individuals. The objectives ...

  5. MULTIMEDIA BASED LEARNING MATERIALS FOR DEAF STUDENTS

    OpenAIRE

    Luqman Hidayat; Gunarhadi; Furqon Hidayatulloh

    2017-01-01

    Deaf students have different abilities from students who have the ability to hear a lesson at school. Barriers to hear experienced by students with hearing impairment can affect the language, academic, and social skills of deaf students. Deaf students can still obtain information from other senses that are still functioning, such as the senses of sight, touch, taste and smell or of residual hearing that still exist. In the world of education, one way to overcome this obstacle is by making mul...

  6. Stigma in Mothers of Deaf Children

    OpenAIRE

    Hossein Ebrahimi; Eissa Mohammadi; Mohammad Ali Mohammadi; Akbar Pirzadeh; Hamzeh Mahmoudi; Ismail Ansari

    2015-01-01

    Introduction: A deaf child creates a feeling of stigma in many hearing parents. Stigma in mothers can have a negative impact on a child?s treatment and rehabilitation process. Therefore, this study was conducted to evaluate the extent of stigma in mothers with deaf children. Materials and Methods: This descriptive, cross-sectional study was conducted in 2013 among 90 mothers with deaf children. The data-collection instrument included the stigma scale in the mothers of children with disabiliti...

  7. Profound Impacts of AN Arctic Face Lift

    Science.gov (United States)

    Nghiem, Son

    Son Nghiem, son.v.nghiem@jpl.nasa.gov Jet Propulsion Laboratory, California Institute of Technology, Pasadena, California, United States The ice cover on the Arctic Ocean has undergone a face lift that removes much of the older and thicker perennial ice and replaces it with the younger and thinner seasonal ice. Although the sea ice cover is a thin skin compared to the depth of the Arctic Ocean, this face lift exerts profound change in the Arctic environment. Here, we present scatterometer remote sensing of Arctic sea ice change and its implication on chemical processes from the ice surface to the troposphere extending into the internal continental land. In the context of a half century change, the extent of perennial ice declines at rate of 0.5 million km2 per decade in the 1970s-1990s while there is no discernable trend in the 1950s-1960s. Abruptly, the rate of decrease has tripled to 1.5 million km2 per decade in the 2000s. A record was set in the reduction of Arctic perennial ice extent in winter 2008. By 1 March 2008, perennial ice extent was reduced by one million km2 compared to that at the same time in 2007. On 1 May 2009, perennial ice extent was reduced to 2.1 million km2 , which is a virtual tie to 2.2 million km2 of perennial ice extent on 1 May 2008 given the uncertainty of ±0.2 million km2 . Although the extent of perennial ice extent is similar, its distribution is quite different, with a significant perennial ice pack in the Beaufort Sea in 2008, and in contrast a large expanse of perennial ice along the Transpolar Drift Stream in 2009. The continuing drastic reduction of perennial ice significantly decreases the overall surface albedo, resulting in enhanced solar heat absorption in spring and summer, which further decreases the Arctic ice pack through the ice-albedo feedback mechanism and ice melt from the underside due to oceanic thermodynamic interactions. Satellite maps of sea ice class distribution show the closely conformation with patterns of

  8. Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Lemmerling, M.; De Praeter, G.; Mollet, P.; Mortele, K.; Kunnen, M. [Univ. Hospital, Gent (Belgium). Dept. of Radiol.; Dhooge, I. [Department of Otorhinolaryngology, University Hospital Gent, Gent (Belgium); Mastenbroek, G. [Department of Neurology, Streekziekenhuis De Honte, Terneuzen (Netherlands)

    1998-05-01

    We present a 50-year-old man who was investigated for sensorineural hearing loss. On MRI of the brain superficial siderosis of the central nervous system was seen, while MRI of the spine revealed an ependymoma of the cauda equina. This case illustrates the importance of performing T2-weighted imaging of the brain and posterior fossa when sensorineural hearing loss is present. Spine imaging is mandatory when superficial siderosis of the brain is diagnosed without identification of a bleeding source in the brain. (orig.) With 5 figs., 10 refs.

  9. Foucault and deaf education in Finland

    Directory of Open Access Journals (Sweden)

    Lauri Siisiäinen

    2016-03-01

    Full Text Available The influence of Michel Foucault’s thinking in critical disability studies, and to social studies of deafness, can hardly be doubted. Foucault has offered valuable tools for the critical rethinking of deaf education and pedagogy with respect to normalization and disciplinary power, which are integrally related to the historical construction of deafness as deficiency and pathology by modern, medical, and psychological knowledge. This article explores the applicability and critical potential of the Foucauldian concepts of disciplinary power, surveillance, and normalization within the specific context of the history of deaf education in Finland. The article focuses on the modernization of the education of deaf children that began during the latter half of the nineteenth century in Finland, with the influence of oralism – a pedagogical discourse and deaf-education methods of German origin. Deafness was characterized as a pathology or abnormality of the most severe kind. When taken at the general level, Foucault’s well-known concepts are easily applicable to the analysis of deaf education, also in the Finnish context. However, it is argued that things become much more complex if we first examine more closely the roles played by the eye and the ear, by optic and aural experience, in these Foucauldian notions, and if we then relate this enquiry to our analysis of oralist pedagogy and deaf education.

  10. Congenitally deaf children's care trajectories in the context of universal neonatal hearing screening: a qualitative study of the parental experiences

    OpenAIRE

    Hardonk, Stefan; Desnerck ., Greetje; Loots, Gerrit; van Hove, Geert; Van Kerschaver, Erwin; Sigurjónsdóttir, Hanna Björg; Vanroelen, Christophe; Louckx, Fred

    2011-01-01

    The objective of this study is to examine the early care trajectories of congenitally deaf children from a parental perspective, starting with universal neonatal hearing screenings. The analysis using a three-dimensional care trajectory concept is aimed at developing a basic typology of postscreening care trajectories. Children with severe/profound hearing loss, registered in the Flanders' (Belgium) universal neonatal hearing screening program, born between 1999 and 2001. Thematic content ana...

  11. Clinical and mutational spectrum of hypoparathyroidism, deafness and renal dysplasia syndrome.

    Science.gov (United States)

    Belge, Hendrica; Dahan, Karin; Cambier, Jean-François; Benoit, Valérie; Morelle, Johann; Bloch, Julie; Vanhille, Philippe; Pirson, Yves; Demoulin, Nathalie

    2017-05-01

    Hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome is a rare autosomal dominant disorder, secondary to mutations in the GATA-3 gene. Due to its wide range of penetrance and expressivity, the disease may not always be recognized. We herein describe clinical and genetic features of patients with HDR syndrome, highlighting diagnostic clues. Medical records of eight patients from five unrelated families exhibiting GATA-3 mutations were reviewed retrospectively, in conjunction with all previously reported cases. HDR syndrome was diagnosed in eight patients between the ages of 18 and 60 years. Sensorineural deafness was consistently diagnosed, ranging from clinical hearing loss since infancy in seven patients to deafness detected only by audiometry in adulthood in one single patient. Hypoparathyroidism was present in six patients (with hypocalcaemia and inaugural seizures in two out of six). Renal abnormalities observed in six patients were diverse and of dysplastic nature. Three patients displayed nephrotic-range proteinuria and reached end-stage renal disease (ESRD) between the ages of 19 and 61 years, whilst lesions of focal and segmental glomerulosclerosis were histologically demonstrated in one of them. Interestingly, phenotype severity differed significantly between a mother and son within one family. Five new mutations of GATA-3 were identified, including three missense mutations affecting zinc finger motifs [NM_001002295.1: c.856A>G (p.N286D) and c.1017C>G (p.C339W)] or the conserved linker region [c.896G>A (p.R299G)], and two splicing mutations (c.924+4_924+19del and c.1051-2A>G). Review of 115 previously reported cases of GATA-3 mutations showed hypoparathyroidism and deafness in 95% of patients, and renal abnormalities in only 60%. Overall, 10% of patients had reached ESRD. We herein expand the clinical and mutational spectrum of HDR syndrome, illustrating considerable inter- and intrafamilial phenotypic variability. Diagnosis of HDR should be

  12. [Maternally Inherited Diabetes and Deafness].

    Science.gov (United States)

    Sampedro, A; Barbón, J J; Alvarez, J A; Andrés, M A; Baldó, C

    2009-07-01

    We described the follow up of a patient with diabetes mellitus type 2 who had a macular pattern dystrophy and bilateral neurosensory hearing loss. Electrophysiological studies revealed abnormal pattern electroretinography and impaired electro-oculogram responses. Maternally Inherited Diabetes, neurosensory Deafness and generally macular pattern distrophy (MIDD syndrome), is a rare mitochondrial disease, responsible for approximately 0.5 to 2.8% of diabetes mellitus.

  13. Extremely low penetrance of deafness associated with the mitochondrial 12S rRNA mutation in 16 Chinese families: implication for early detection and prevention of deafness.

    Science.gov (United States)

    Dai, Pu; Liu, Xin; Han, Dongyi; Qian, Yaping; Huang, Deliang; Yuan, Huijun; Li, Weiming; Yu, Fei; Zhang, Ruining; Lin, Hongyan; He, Yong; Yu, Youjun; Sun, Quanzhu; Qin, Huaiyi; Li, Ronghua; Zhang, Xin; Kang, Dongyang; Cao, Juyang; Young, Wie-Yen; Guan, Min-Xin

    2006-02-03

    Mutations in mitochondrial DNA (mtDNA) have been found to be associated with sensorineural hearing loss. We report here the clinical, genetic, and molecular characterization of 16 Chinese pedigrees (a total of 246 matrilineal relatives) with aminoglycoside-induced impairment. Clinical evaluation revealed the variable phenotype of hearing impairment including audiometric configuration in these subjects, although these subjects share some common features: being bilateral and sensorineural hearing impairment. Strikingly, these Chinese pedigrees exhibited extremely low penetrance of hearing loss, ranging from 4% to 18%, with an average of 8%. In particular, nineteen of 246 matrilineal relatives in these pedigrees had aminoglycoside-induced hearing loss. Mutational analysis of the mtDNA in these pedigrees showed the presence of homoplasmic 12S rRNA A1555G mutation, which has been associated with hearing impairment in many families worldwide. The extremely low penetrance of hearing loss in these Chinese families carrying the A1555G mutation strongly supports the notion that the A1555G mutation itself is not sufficient to produce the clinical phenotype. Children carrying the A1555G mutation are susceptible to the exposure of aminoglycosides, thereby inducing or worsening hearing impairment, as in the case of these Chinese families. Using those genetic and molecular approaches, we are able to diagnose whether children carry the ototoxic mtDNA mutation. Therefore, these data have been providing valuable information and technology to predict which individuals are at risk for ototoxicity, to improve the safety of aminoglycoside therapy, and eventually to decrease the incidence of deafness.

  14. Cortical Deafness - A Clinical, Audiological, Electrophysiological, Radiological And Follow Up Study

    Directory of Open Access Journals (Sweden)

    Sinha S

    2003-01-01

    Full Text Available Cortical deafness occurs following lesions of the Heshcl′s gyri or the subcortical auditory pathways. Stroke is the commonest etiology. The clinical, audiological, electrophysiological and radiological observations of a patient with cortical deafness secondary to ischemic stroke involving bilateral Heschl′s gyri, with long term follow up is being reported. He had profound degree of hearing loss, poor auditory verbal comprehension, poor auditory repetition, inability to take dictation and poor response to other non-linguistic sounds despite having adequate hearing assessed by objective audiological tests. He was advised to adopt speech reading techniques and resort to gestural and written modalities to augment his communication skills. He has shown only a marginal improvement over the last six years.

  15. Films about the deaf: the representations of deaf and sign languages

    Directory of Open Access Journals (Sweden)

    Carolina Hessel Silveira

    2009-12-01

    Full Text Available This paper analyzes two films about deafness which have not been investigated in the Brazilian academic context. They are Mandy (directed by Alexander Mackendrick, 1952, England and After the Silence (by Fred Gerber, 1996, USA. The analysis is supported by Cultural Studies and Deaf Studies, especially on the concepts of cultural pedagogies, deaf culture, deaf identities, sign language, as well as on the analysis of other films about deaf people conducted by Thoma (2004. Both films are classified as drama, and particular attention was given to how deaf characters are represented, highlighting scenes showing the difficulties deaf people face in a hearing society. It is worth noting that in the end of both films the deaf characters manage to speak and hear. The pedagogical impact of these films is questioned as they show that the deaf may be able to speak and hear after using Sign Language. Deaf representations, deaf education and sign language are present in both films, although there is a difference in approach between them.

  16. Deaf education in China: history, current issues, and emerging deaf voices.

    Science.gov (United States)

    Lytle, Richard R; Johnson, Kathryn E; Hui, Yang Jun

    An overview is provided of (a) deaf education in China, (b) views of deaf Chinese, and (c) recent empowering international collaborations. China's national policy focuses on oral/aural education and hearing rehabilitation. However, everyday practice in schools for deaf children includes various forms of Chinese Sign Language. Early childhood education focuses on speech and hearing. Elementary and secondary school curricula reflect low expectations for deaf students and lack the same academic content provided to hearing students. There are limited higher education opportunities. There are no support services such as note takers or interpreters for mainstreamed students. There are no deaf teacher preparation or interpreter training programs. Jobs are few; the vast majority of deaf adults are unemployed. Deaf people interviewed for the article describe their needs, their dreams, and the changes they are witnessing, which result in part from recent empowering international collaborations.

  17. PERSONAL IDENTITY IN DEAF ADOLESCENTS

    Directory of Open Access Journals (Sweden)

    Joanna KOSSEWSKA

    2008-06-01

    Full Text Available The purpose of this study was to investigate the factors influencing the identity deaf adolescents. The study involved 67 deaf adolescents (38 boys and 29 girls aged 16 to 19 students of secondary school. Ninety-three hearing children constituted a comparison group. The structure of identity was explored on the basis of identification references given by the subjects who were to reply in writing, 20 times running, to the question: „Who Am I?” the test, adapted from M. H. Kuhn and T. S. McPartland by Martines and Silvestre (1995 given in written and signed mode.Results showed that the hearing status as well as mode of communication influence the description of personal identity. It was found that deaf adoles­cents used more descriptions especially in the fol­lowing categories: Civil Status, Body and Physical Appearance, Tastes and Activities, Friendship and Relationships, Personal and Social Situation, Negative Personal Traits, and Neutral Personality Traits. Although this study could demonstrate im­pact independent variables on identity, the data raise the need for further, preferably longitudinal, research. This complex phenomenon has to be examined more closely.Combined self-descriptive processes lead to the development of an organized, learned and dynamic identity, and subjective description of an individ­ual has strong emotional consequences for the in­dividual in question.

  18. Hyperbaric oxygen for idiopathic sudden sensorineural hearing loss and tinnitus.

    Science.gov (United States)

    Bennett, Michael H; Kertesz, Tom; Perleth, Matthias; Yeung, Philip; Lehm, Jan P

    2012-10-17

    This is an update of a Cochrane Review first published in The Cochrane Library in Issue 1, 2005 and previously updated in 2007 and 2009.Idiopathic sudden sensorineural hearing loss (ISSHL) is common and has a significant effect on quality of life. Hyperbaric oxygen therapy (HBOT) may improve oxygen supply to the inner ear and result in an improvement in hearing. To assess the benefits and harms of HBOT for treating ISSHL and/or tinnitus. We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; Database of Randomised Trials in Hyperbaric Medicine (DORCTHIM); CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ICTRP and additional sources for published and unpublished trials. The date of the most recent search was 2 May 2012, following previous searches in 2009, 2007 and 2004. Randomised studies comparing the effect on ISSHL and tinnitus of HBOT and alternative therapies. Three authors evaluated the quality of trials using the 'Risk of bias' tool and extracted data from the included trials. Seven trials contributed to this review (392 participants). The studies were small and of generally poor quality. Pooled data from two trials did not show any significant improvement in the chance of a 50% increase in hearing threshold on pure-tone average with HBOT (risk ratio (RR) with HBOT 1.53, 95% confidence interval (CI) 0.85 to 2.78, P = 0.16), but did show a significantly increased chance of a 25% increase in pure-tone average (RR 1.39, 95% CI 1.05 to 1.84, P = 0.02). There was a 22% greater chance of improvement with HBOT, and the number needed to treat (NNT) to achieve one extra good outcome was 5 (95% CI 3 to 20). There was also an absolute improvement in average pure-tone audiometric threshold following HBOT (mean difference (MD) 15.6 dB greater with HBOT, 95% CI 1.5 to 29.8, P = 0.03). The significance of any improvement in tinnitus could not be

  19. [Clinical comparison of idiopathic sudden deafness in children and the elderly].

    Science.gov (United States)

    Ao, Min; Deng, Jie; Qi, Xing; He, Gang

    2015-07-01

    This retrospective study compared clinical manifestations of idiopathic sudden hearing loss between children and the elderly. 44 pediatric patients and 76 elderly patients diagnosed with idiopathic sudden deafness in our clinic from December 2009 to September 2014 were enrolled. Different clinical parameters were compared. The incidence of initially profound hearing loss was highest and mild hearing loss was lowest in both groups (P 0.05). The number of patients was the most in initially profound type of audiogram pattern and the fewest in ascending type in both groups (P children (27/44, 61.4%) was higher than that in elderly patients (32/76, 42.1%) (P 0.05). The highest recovery rate in children was in those with descending type and the lowest was in those with profound type (P elderly patients with initially different types of audiogram pattern (P > 0.05). Hearing recovery rates of descending type in children were higher than that in elderly patients. Presence of tinnitus in pediatric patients was not relavent to the outcome (P > 0.05). Presence of tinnitus in elderly patients was associated with favorable outcomes. (P 0.05). Presence of dizziness in elderly patients was associated with poor outcomes (P elderly patients was not relavent to the outcome (P > 0.05). Presence of chronic diseases in elderly patients was not associated with the outcome (P > 0.05). The clinical manifestion of idiopathic sudden deafness is respective in Children and in elderly patients.

  20. Acute unilateral sensorineural hearing loss associated with anabolic steroids and polycythaemia: case report.

    Science.gov (United States)

    Tikka, T; Mistry, N; Janjua, A

    2016-03-01

    Unilateral sudden sensorineural hearing loss due to an infarct in the vertebrobasilar system has been widely reported. Most patients have a background of traditional coronary risk factors related to these cerebrovascular episodes. A 32-year-old male, a regular user of anabolic steroids, presented to the emergency department with unilateral sensorineural hearing loss and symptoms suggestive of an infarct of the anterior inferior cerebellar artery but in the absence of risk factors for ischaemic stroke. Magnetic resonance imaging confirmed the presence of infarction in the region supplied by the anterior inferior cerebellar artery. Polycythaemia was found on haematological analysis, which we believe was secondary to the use of anabolic steroids. The patient was commenced on aspirin as per the stroke management protocol. There was resolution of neurological symptomatology six weeks after the episode, but no improvement in hearing. To our knowledge, this is the first case report of unilateral sensorineural hearing loss secondary to the use of anabolic steroids causing polycythaemia. This cause should be considered in the differential diagnosis of patients presenting with sensorineural hearing loss, especially in young males, when no other risk factors can be identified.

  1. Audiovisual Asynchrony Detection and Speech Intelligibility in Noise With Moderate to Severe Sensorineural Hearing Impairment

    NARCIS (Netherlands)

    Baskent, Deniz; Bazo, Danny

    2011-01-01

    Objective: The objective of this study is to explore the sensitivity to intermodal asynchrony in audiovisual speech with moderate to severe sensorineural hearing loss. Based on previous studies, two opposing expectations were an increase in sensitivity, as hearing-impaired listeners heavily rely on

  2. Postural control assessment in students with normal hearing and sensorineural hearing loss.

    Science.gov (United States)

    Melo, Renato de Souza; Lemos, Andrea; Macky, Carla Fabiana da Silva Toscano; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

    2015-01-01

    Children with sensorineural hearing loss can present with instabilities in postural control, possibly as a consequence of hypoactivity of their vestibular system due to internal ear injury. To assess postural control stability in students with normal hearing (i.e., listeners) and with sensorineural hearing loss, and to compare data between groups, considering gender and age. This cross-sectional study evaluated the postural control of 96 students, 48 listeners and 48 with sensorineural hearing loss, aged between 7 and 18 years, of both genders, through the Balance Error Scoring Systems scale. This tool assesses postural control in two sensory conditions: stable surface and unstable surface. For statistical data analysis between groups, the Wilcoxon test for paired samples was used. Students with hearing loss showed more instability in postural control than those with normal hearing, with significant differences between groups (stable surface, unstable surface) (p<0.001). Students with sensorineural hearing loss showed greater instability in the postural control compared to normal hearing students of the same gender and age. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  3. Language Development and Impairment in Children with Mild to Moderate Sensorineural Hearing Loss

    Science.gov (United States)

    Halliday, Lorna F.; Tuomainen, Outi; Rosen, Stuart

    2017-01-01

    Purpose: The goal of this study was to examine language development and factors related to language impairments in children with mild to moderate sensorineural hearing loss (MMHL). Method: Ninety children, aged 8-16 years (46 children with MMHL; 44 aged-matched controls), were administered a battery of standardized language assessments, including…

  4. An update on drug design strategies to prevent acquired sensorineural hearing loss.

    Science.gov (United States)

    Bodmer, Daniel

    2017-11-01

    Acute sensorineural hearing loss is a dramatic event for the patient. Different pathologies might result in acute sensorineural hearing loss, such as sudden hearing loss, exposure to medications/drugs or loud sound. Current therapeutic approaches include steroids and hyperbaric oxygen in addition to other methods. Research activities of the past have shed light on the molecular mechanisms involved in damage to hair cells, the synapses at the hair cell spiral ganglion junction and the stria vascularis. Molecular events and signaling pathways which underlie damage to these structures have been discovered. Areas covered: This paper summarizes current research efforts involved in investigating the molecular mechanisms involved in acute sensorineural hearing loss. Expert opinion: While progress has been made in unraveling basic mechanisms involved in acute sensorineural hearing loss, it is difficult to translate basic concepts to the clinic. There are often conflicting data in animal and human studies on the effect of a given intervention. There is also a lack of high quality clinical trials (double blind, placebo controlled and high powered). However, this author is confident that research efforts will pay out and that some of these efforts will translate into new therapeutic options for patients with acute hearing loss.

  5. Identification of evidence for autoimmune pathology of bilateral sudden sensorineural hearing loss using proteomic analysis.

    Science.gov (United States)

    Lee, Jeon Mi; Kim, Jin Young; Bok, Jinwoong; Kim, Kyu-Sung; Choi, Jae Young; Kim, Sung Huhn

    2017-10-01

    Sudden sensorineural hearing loss (S-SNHL) is an inner ear disorder with an abrupt hearing loss occurring loss; LC-MS: liquid chromatography-mass spectrometry; MS: mass spectrometry; autoAb: autoantibody; 1-DE: one-dimensional electrophoresis. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. Risk factors for sensorineural hearing loss in NICU infants compared to normal hearing NICU controls

    NARCIS (Netherlands)

    Coenraad, S.; Goedegebure, A.; van Goudoever, J. B.; Hoeve, L. J.

    2010-01-01

    To evaluate independent etiologic factors associated with sensorineural hearing loss in infants who have been admitted to the neonatal intensive care unit compared to normal hearing controls. Between 2004 and 2009, 3366 infants were admitted to the neonatal intensive care unit of Sophia Children's

  7. Profile and Stability of Sensorineural Hearing Loss in Persistent Pulmonary Hypertension of the Newborn.

    Science.gov (United States)

    Walton, Joseph P.; Hendricks-Munoz, Karen

    1991-01-01

    This study found that 19 of 51 infants with persistent pulmonary hypertension of the newborn were diagnosed with sensorineural hearing loss, an incidence 25 times greater than that of intensive care unit infants in general. Treatment durations with mechanical ventilation were significantly longer for the hearing-impaired group compared to the…

  8. Progression of low-frequency sensorineural hearing loss (DFNA6/14-WFS1).

    NARCIS (Netherlands)

    Pennings, R.J.E.; Bom, S.J.H.; Cryns, K.; Flothmann, K.; Huygen, P.L.M.; Kremer, J.M.J.; Camp, G. van; Cremers, C.W.R.J.

    2003-01-01

    OBJECTIVE: To assess the audiometric profile and speech recognition characteristics in affected members of 2 families with DFNA6/14 harboring heterozygous mutations in the WFS1 gene that cause an autosomal dominant nonsyndromic sensorineural hearing impairment trait. DESIGN: Family study. SETTING:

  9. Megadolicho basilar artery as a cause of asymmetrical sensorineural hearing loss - case report

    Directory of Open Access Journals (Sweden)

    Melo, Antonio Antunes

    2011-07-01

    Full Text Available Introduction: At the differentiated diagnosis of asymmetrical sensorineural hearing losses, vascular disorders are present, one of which is megadolicho basilar artery. This disease is generally asymptomatic, and when symptoms are found, they can be caused by a compression or ischemia. Clinically, sensorineural hearing loss, tinnitus, headache, facial hypoesthesia, trigeminal neuralgia, vertigo, diplopia and facial palsy, among others, are likely to occur. The image examination of choice for its diagnosis is nuclear magnetic resonance. The megadolicho basilar artery therapy can be surgical or conservative, according to the associated findings. A multidisciplinary approach, including a neurologist, neurosurgeon and an otorhinolaryngologist is recommended for a proper administration of the case. Objective: Report the case of a patient with asymmetrical sensorineural hearing loss, diagnosed of megadolicho basilar artery. Case report: JBS, 57-year-old white male with a history of asymmetrical sensorineural hearing loss and bilateral whistle-like tinnitus for several years. The otorhinolaryngologic evaluation, including otoscopy, anterior rhinoscopy and oral pharynx, was normal. Final Comments: The treatment consisted in following up with the patient, controlling the tinnitus by drugs and using an individual sound amplification apparatus on the left ear.

  10. Antiviral treatment of idiopathic sudden sensorineural hearing loss : A prospective, randomized, double-blind clinical trial

    NARCIS (Netherlands)

    Stokroos, RJ; Albers, FWJ; Tenvergert, EM

    A subclinical viral labyrinthitis has been postulated in the literature to elicit Idiopathic Sudden Sensorineural Hearing Loss. An etiological role for the herpes virus family is assumed. Corticosteroids possess a limited beneficial effect on hearing recovery in ISSHL. In this study, the therapeutic

  11. Impact of Nonaspirin Nonsteroidal Anti-inflammatory Agents and Acetaminophen on Sensorineural Hearing Loss: A Systematic Review

    Science.gov (United States)

    Kyle, Meghann E.; Wang, James C.; Shin, Jennifer J.

    2015-01-01

    Objective To perform a systematic review evaluating the association between sensorineural hearing loss and (1) nonsteroidal anti-inflammatory drugs (NSAIDs) as a class, (2) NSAIDs available over the counter, (3) NSAIDs in short intravenous courses, (4) prescription NSAIDs utilized by patients without systemic inflammatory conditions, (5) prescription NSAIDs in patients with arthritides, and (6) acetaminophen with and without concomitant narcotic usage. Data Sources Computerized searches of PubMed, EMBASE, and the Cochrane Library were updated through May 2014, along with manual searches and inquiries to topic experts. Review Methods The systematic review was performed according to an a priori protocol. Data extraction was performed by 2 independent investigators, and it focused on relevant audiologic measurements, methodological elements related to risk of bias, and potential confounders. Results The 23 criterion-meeting studies included a total of 92,532 participants, with mixed results. Sulindac was the only specific agent to have been studied with formal audiometry in a randomized double-blind placebo-controlled trial in which hearing was the reported primary outcome: Although an effect was seen in the unadjusted analysis (pure tone threshold > 15 dB, 9.3% vs 2.9%; relative risk [RR], 3.2; confidence interval [CI], 1.09-9.55; P = .02), the effect dissipated in the adjusted analysis (P = .09). There was a significant effect on self-reported hearing loss from NSAIDs as a class (RR, 1.21; CI, 1.11-1.33), ibuprofen (RR, 1.13; CI, 1.06-1.19), and acetaminophen (RR, 1.21; CI, 1.11-1.33), but no formal audiometric data confirm or refute this suggested effect. Audiometry has demonstrated profound loss in some instances of acetaminophen-narcotic combination ingestions. Conclusions Data are varied regarding the impact of NSAIDs and acetaminophen on population hearing health. PMID:25560405

  12. Morphological sensitivity in deaf readers of Dutch

    NARCIS (Netherlands)

    Hoogmoed, A.H. van; Verhoeven, L.T.W.; Schreuder, R.; Knoors, H.E.T.

    2011-01-01

    Deaf children experience difficulties with reading comprehension. These difficulties are not completely explained by their difficulties with the reading of single short words. Whether deaf children and adults lag behind in the morphological processing of longer words is therefore examined in two

  13. Communities of Practice: Literacy and Deaf Children

    Science.gov (United States)

    Kristoffersen, Ann-Elise; Simonsen, Eva

    2016-01-01

    This article aims to discuss young deaf children's access to literacy within a sociocultural perspective. We introduce the concept of communities of practice as an aspect in early literacy development for young deaf children. Preschools are learning communities and thus constitute communities of practice. Our discussion on the use of communities…

  14. Deaf Teenagers and Family Alcohol Problems.

    Science.gov (United States)

    Stevens, Raymond P.

    1987-01-01

    Deaf teenagers have more trouble coping with the effects of parental alcohol abuse than do hearing teenagers. Suggestions are made for helping the deaf teenager and other family members deal with these problems, especially in potentially violent situations. Two short case studies are provided to illustrate intervention methods and outcomes.…

  15. Burnout in Professionals Working with Deaf Children.

    Science.gov (United States)

    Meadow, Kathryn P.

    1981-01-01

    Two hundred forty deaf education professionals completed an inventory on burnout, career motivation, and job satisfaction. Teachers of deaf students were more likely to experience burnout than teachers of nonhandicapped children and teachers aged 27 through 30 expressed the highest degree of emotional exhaustion. (CL)

  16. Depression and Suicide in Deaf Adolescents.

    Science.gov (United States)

    Messenger, Carolyn J.; Bibby, Mary Ann

    1998-01-01

    This article shares one mother's experiences with the depression of her son and his friend who are both deaf. Findings from interviews with the two adolescents are provided, along with recommendations for educators, peers, and professionals for helping teenagers with deafness coping with depression and thoughts of suicide. (Contains references.)…

  17. Critical Literacy: Deaf Adults Speak Out

    Science.gov (United States)

    Enns, Charlotte

    2009-01-01

    The purpose of this paper is to describe a variety of teaching and learning strategies that were used within a classroom of Deaf adults participating in a high school English course as part of an upgrading program. The class was conducted in a bilingual manner; that is, being Deaf and communicating with American Sign Language (ASL) was not…

  18. Genetics: advances in genetic testing for deafness.

    Science.gov (United States)

    Shearer, A Eliot; Smith, Richard J H

    2012-12-01

    To provide an update on recently discovered human deafness genes and to describe advances in comprehensive genetic testing platforms for deafness, both of which have been enabled by new massively parallel sequencing technologies. Over the review period, three syndromic and six nonsyndromic deafness genes have been discovered, bringing the total number of nonsyndromic deafness genes to 64. Four studies have shown the utility of massively parallel sequencing for comprehensive genetic testing for deafness. Three of these platforms have been released on a clinical or commercial basis. Deafness is the most common sensory deficit in humans. Genetic diagnosis has traditionally been difficult due to extreme genetic heterogeneity and a lack of phenotypic variability. For these reasons, comprehensive genetic screening platforms have been developed with the use of massively parallel sequencing. These technologies are also accelerating the pace of gene discovery for deafness. Because genetic diagnosis is the basis for molecular therapies, these advances lay the foundation for the clinical care of deaf and hard-of-hearing persons in the future.

  19. Literacy for Deaf People Seeking Employment.

    Science.gov (United States)

    Ryall, Gordon

    1993-01-01

    Focuses on the literacy problems of deaf people who are seeking employment. The article argues for the provision of adequate English-as-a-Second-Language (ESL) literacy skills training, makes programming recommendations, and suggests employment strategies that deaf people need. (12 references) (Author/CK)

  20. The Significance of Deaf Identity for Psychological Well-Being

    Science.gov (United States)

    Chapman, Madeleine; Dammeyer, Jesper

    2017-01-01

    Research has paid attention to how deaf identity affects life outcomes such as psychological well-being. However, studies are often carried out with small samples and without controlling for other variables. This study examined how different forms of identity--deaf, hearing, bicultural (deaf and hearing), and marginal (neither deaf nor…

  1. Violence against Deaf Women: Effect of Partner Hearing Status

    Science.gov (United States)

    Anderson, Melissa L.; Kobek Pezzarossi, Caroline M.

    2014-01-01

    Using a sample of Deaf female undergraduate students, the current study sought to investigate the prevalence, correlates, and characteristics of intimate partner violence victimization in hearing-Deaf and Deaf-Deaf relationships. Initial results suggest that similarities in hearing status and communication preference are associated with increased…

  2. Without Boundaries: An Inquiry into Deaf Epistemologies through a Metaparadigm

    Science.gov (United States)

    Wang, Ye

    2010-01-01

    The ongoing debate on Deaf epistemologies reflects two major paradigms in deaf education: positivism and constructivism. The present article investigates Deaf epistemologies through a metaparadigm, which should blur the boundaries among different paradigms and connect the epistemological inquiry to instructional practice for d/Deaf students. The…

  3. Programs for Deaf-Blind Children and Adults.

    Science.gov (United States)

    American Annals of the Deaf, 2000

    2000-01-01

    This annual directory lists programs for deaf-blind children and adults including programs for deaf-blind children and youth (national and state level), the Helen Keller Centers for deaf-blind youth and adults, and programs for training teachers of deaf-blind students. (DB)

  4. Social Maturity and Executive Function among Deaf Learners

    Science.gov (United States)

    Marschark, Marc; Kronenberger, William G.; Rosica, Mark; Borgna, Georgianna; Convertino, Carol; Durkin, Andreana; Machmer, Elizabeth; Schmitz, Kathryn L.

    2017-01-01

    Two experiments examined relations among social maturity, executive function, language, and cochlear implant (CI) use among deaf high school and college students. Experiment 1 revealed no differences between deaf CI users, deaf nonusers, and hearing college students in measures of social maturity. However, deaf students (both CI users and…

  5. Deaf Children and English: More Ways Parents Can Help.

    Science.gov (United States)

    Katasse, Constance; Cartwright, Daisy

    1997-01-01

    Ideas to help parents of deaf children foster English language development include communicating regularly in writing, handling public encounters the "deaf" way, interacting with deaf adults, reading books by/about deaf people, learning and maintaining sign language skills, joining local and state associations, and making sure the television has a…

  6. Cortical cross-modal plasticity following deafness measured using functional near-infrared spectroscopy.

    Science.gov (United States)

    Dewey, Rebecca S; Hartley, Douglas E H

    2015-07-01

    Evidence from functional neuroimaging studies suggests that the auditory cortex can become more responsive to visual and somatosensory stimulation following deafness, and that this occurs predominately in the right hemisphere. Extensive cross-modal plasticity in prospective cochlear implant recipients is correlated with poor speech outcomes following implantation, highlighting the potential impact of central auditory plasticity on subsequent aural rehabilitation. Conversely, the effects of hearing restoration with a cochlear implant on cortical plasticity are less well understood, since the use of most neuroimaging techniques in CI recipients is either unsafe or problematic due to the electromagnetic artefacts generated by CI stimulation. Additionally, techniques such as functional magnetic resonance imaging (fMRI) are confounded by acoustic noise produced by the scanner that will be perceived more by hearing than by deaf individuals. Subsequently it is conceivable that auditory responses to acoustic noise produced by the MR scanner may mask auditory cortical responses to non-auditory stimulation, and render inter-group comparisons less significant. Uniquely, functional near-infrared spectroscopy (fNIRS) is a silent neuroimaging technique that is non-invasive and completely unaffected by the presence of a CI. Here, we used fNIRS to study temporal-lobe responses to auditory, visual and somatosensory stimuli in thirty profoundly-deaf participants and thirty normally-hearing controls. Compared with silence, acoustic noise stimuli elicited a significant group fNIRS response in the temporal region of normally-hearing individuals, which was not seen in profoundly-deaf participants. Visual motion elicited a larger group response within the right temporal lobe of profoundly-deaf participants, compared with normally-hearing controls. However, bilateral temporal lobe fNIRS activation to somatosensory stimulation was comparable in both groups. Using fNIRS these results

  7. Body Perception and Action Following Deafness

    Directory of Open Access Journals (Sweden)

    M. S. Houde

    2016-01-01

    Full Text Available The effect of deafness on sensory abilities has been the topic of extensive investigation over the past decades. These investigations have mostly focused on visual capacities. We are only now starting to investigate how the deaf experience their own bodies and body-related abilities. Indeed, a growing corpus of research suggests that auditory input could play an important role in body-related processing. Deafness could therefore disturb such processes. It has also been suggested that many unexplained daily difficulties experienced by the deaf could be related to deficits in this underexplored field. In the present review, we propose an overview of the current state of knowledge on the effects of deafness on body-related processing.

  8. Numerical Estimation in Deaf and Hearing Adults.

    Science.gov (United States)

    Bull, Rebecca; Marschark, Marc; Sapere, Patty; Davidson, Wendy A; Murphy, Derek; Nordmann, Emily

    2011-08-01

    Deaf students often lag behind hearing peers in numerical and mathematical abilities. Studies of hearing children with mathematical difficulties highlight the importance of estimation skills as the foundation for formal mathematical abilities, but research with adults is limited. Deaf and hearing college students were assessed on the Number-to-Position task as a measure of estimation, and completed standardised assessments of arithmetical and mathematical reasoning. Deaf students performed significantly more poorly on all measures, including making less accurate number-line estimates. For deaf students, there was also a strong relationship showing that those more accurate in making number-line estimates achieved higher scores on the math achievement tests. No such relationship was apparent for hearing students. Further insights into the estimation abilities of deaf individuals should be made, including tasks that require symbolic and non-symbolic estimation and which address the quality of estimation strategies being used.

  9. Environmental assessment: Deaf Smith County site, Texas

    Energy Technology Data Exchange (ETDEWEB)

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified a location in Deaf Smith County, Texas, as one of nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Deaf Smith County site and the eight other potentially sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. The Deaf Smith County site is in the Permian Basin, which is one of five distinct geohydrologic settings considered for the first repository. On the basis of the evaluations reported in this EA, the DOE has found that the Deaf Smith County site is not disqualified under the guidelines. On the basis of these findings, the DOE is nominating the Deaf Smith County site as one of the five sites suitable for characterization. 591 refs., 147 figs., 173 tabs.

  10. BILINGUALISM: MULTICULTURALISM HOLOPRAXIOLOGY OF THE VENEZUELAN DEAF

    Directory of Open Access Journals (Sweden)

    Héctor Florencio Martínez Pérez

    2014-04-01

    Full Text Available The formation of the child has been made regularly and without many prejudices or tbacks,  until this had some physical characteristic or perceptual, who twisted his attention.  To those who were born with the inability to listen or hear properly, excluded in all respects. At the end of the 20th century, the deaf began to defend their identity and differed between Deafness (lack of hearing of deafness, with "S", which is a socio-anthropological perspective, which includes the use of sign language and the learning of reading and writing of the Spanish (bilingualism in their training. This research had as general objective to unveil bilingualism from an intercultural intersubjectivity of the deaf in Venezuela by applying a qualitative related paradigm with methodology fenomenologica-hermeneutica of Max Van Manen. The information collected observing and interviewing in depth (12 deaf students, parents or representatives (6, (3 researchers and educational specialists deaf and listeners (12. To analyze and triangulate information, obtained the following conclusions about the bilingual deaf: their physical and intellectual abilities are exactly the same to the listeners;  they can achieve the necessary qualification for any job; is required the language of signs so that you can put into practice the language; those who have the organizational capacity to develop oral language, it should not hinder him this opportunity, without detriment to the learning of the language of signs and the systematic training of the deaf teachers and deaf family, educational managers, political and employer of the deaf is essentially required.

  11. Computational solution for the auxiliary in the literacy of deaf

    Directory of Open Access Journals (Sweden)

    João Carlos Lopes Fernandes

    2016-11-01

    Full Text Available The learning of the deaf is a great challenge for educators, especially in Portuguese-speaking course. Brazilian schools are not prepared for suits with deaf, because they lack trained professionals. Current Brazilian educational policies, seeking socialize all deaf and not deaf students. The Brazilian deaf community uses LIBRAS, Brazilian sign language as their main form of communication between them. Integrating LBS and Portuguese is one of the main current challenges and the use of computers has helped a lot.

  12. On the possibilities and limits of "DEAF DEAF SAME": Tourism and empowerment camps in Adamorobe (Ghana, Bangalore and Mumbai (India.

    Directory of Open Access Journals (Sweden)

    Michele Ilana Friedner

    2014-06-01

    Full Text Available This article qualitatively analyzes the ways that the discourse of "deaf universalism" circulates within two common deaf practices: tourism and engaging in interventions. Arguing that the largely Northern-situated discipline of Deaf Studies does not adequately examine how deaf bodies and discourses travel, ethnographic data compiled in India and Ghana during transnational encounters is employed to examine how claims of "sameness" and "difference" are enacted and negotiated. Similarly, this article examines how deaf individuals and groups deploy the concepts of deaf "heavens" and "hells" to analyze their travel experiences and justify interventions. We argue that deaf travelers and those engaging in interventions, mostly from Northern countries, employ teleological concepts that they attempt to impose on deaf "others." Adopting a critical approach, this article argues for the importance of carving out a space within Deaf Studies for allowing non-Northern concepts to come to the fore. Keywords: Deaf, Development, Universalism, Discourse, India, Ghana

  13. Literatura Surda/Deaf Literature

    Directory of Open Access Journals (Sweden)

    Lodenir Becker Karnopp

    2006-01-01

    Full Text Available O objetivo do presente artigo é proceder a uma análise dos livros de literatura infantil Cinderela Surda e Rapunzel Surda, focalizando os sentidos produzidos sobre identidades e diferenças. As análises desses livros pretendem contribuir para a discussão da produção de uma literatura surda, que está vinculada às discussões sobre cultura e identidade. Na investigação desses materiais, os textos e as imagens produzidas evidenciam que os autores buscam o caminho da auto-representação do grupo de surdos, através da luta pelo estabelecimento do que reconhecem como suas identidades e suas diferenças. Tais evidências estão no uso da língua de sinais, em suas formas de narrar as histórias e/ou de adaptar histórias clássicas, tendo como base suas formas de existência, suas formas de ler, traduzir, conceber e julgar os produtos culturais que consomem e que produzem. This article aims to present an analysis of the fairy tales, Deaf Cinderella and Deaf Rapunzel, focusing on the meanings produced from identities and differences. The analyses of these two books intend to give a contribution to the discussion on the production of deaf literature, which is linked to the discussions on culture and identity. In the investigation of these books, the texts and the images produced show that the authors seek the path to self- representation of the deaf community, through the struggle for the establishment of what they recognize as their identities and differences. Such evidences are in the use of sign language, in their ways of narrating their stories and/or of adapting classic fairy tales, having as a basis their existential ways of being, their ways of reading, translating, conceiving and judging the cultural products which they consume and produce.

  14. Does deafness lead to enhancement of visual spatial cognition in children? Negative evidence from deaf nonsigners.

    Science.gov (United States)

    Parasnis, I; Samar, V; Bettger, J; Sathe, K

    1996-01-01

    This study investigated whether deafness contributes to enhancement of visual spatial cognition independent of knowledge of a sign language. Congenitally deaf school children in India who were born to hearing parents and were not exposed to any sign language, and matched hearing controls, were given a test of digit span and five tests that measured visual spatial skills. The deaf group showed shorter digit span than the hearing group, consistent with previous studies. Deaf and hearing children did not differ in their performance on the visual spatial skills test, suggesting that deafness per se may not be a sufficient factor for enhancement of visual spatial cognition. Early exposure to a sign language and fluent sign skills may be the critical factors that lead to differential development of visual spatial skills in deaf people.

  15. Adapting health education material for deaf audiences.

    Science.gov (United States)

    Pollard, Robert Q; Dean, Robyn K; O'Hearn, Amanda; Haynes, Sharon L

    2009-05-01

    The deaf population is an often-overlooked limited English proficiency (LEP) group at risk for health disparities associated with low health literacy. Lack of access to health information conveyed via radio, television, or ambient auditory sources such as public conversation further aggravates this population's low health literacy. Methods of adapting health education material for hearing LEP populations do not reach deaf audiences with equal effectiveness. We adapt health education material for deaf audiences by first determining the "learning points" contained in vetted source material. A dialog-based film script covering those learning points is created. Supplemental content addressing common deaf population knowledge gaps and sociocultural experiences is added. Deaf actors are filmed following the adapted American Sign Language (ASL) script. Their ASL is back-translated into English to yield vocal track and subtitle scripts. The source material author(s) are consulted throughout the process to assure the film's adherence to the learning point list. Users report that the adapted product is more relevant, engaging, and effective for deaf audiences. This adaptation approach may aid in reducing deaf population health disparities. (PsycINFO Database Record (c) 2009 APA, all rights reserved).

  16. Hearing loss without overt metabolic acidosis in ATP6V1B1 deficient MRL mice, a new genetic model for non-syndromic deafness with enlarged vestibular aqueducts.

    Science.gov (United States)

    Tian, Cong; Gagnon, Leona H; Longo-Guess, Chantal; Korstanje, Ron; Sheehan, Susan M; Ohlemiller, Kevin K; Schrader, Angela D; Lett, Jaclynn M; Johnson, Kenneth R

    2017-10-01

    Mutations of the human ATP6V1B1 gene cause distal renal tubular acidosis (dRTA; OMIM #267300) often associated with sensorineural hearing impairment; however, mice with a knockout mutation of Atp6v1b1 were reported to exhibit a compensated acidosis and normal hearing. We discovered a new spontaneous mutation (vortex, symbol vtx) of Atp6v1b1 in an MRL/MpJ (MRL) colony of mice. In contrast to the reported phenotype of the knockout mouse, which was developed on a primarily C57BL/6 (B6) strain background, MRL-Atp6v1b1vtx/vtx mutant mice exhibit profound hearing impairment, which is associated with enlarged endolymphatic compartments of the inner ear. Mutant mice have alkaline urine but do not exhibit overt metabolic acidosis, a renal phenotype similar to that of the Atpbv1b1 knockout mouse. The abnormal inner ear phenotype of MRL- Atp6v1b1vtx/vtx mice was lost when the mutation was transferred onto the C57BL/6J (B6) background, indicating the influence of strain-specific genetic modifiers. To genetically map modifier loci in Atp6v1b1vtx/vtx mice, we analysed ABR thresholds of progeny from a backcross segregating MRL and B6 alleles. We found statistically significant linkage with a locus on Chr 13 that accounts for about 20% of the hearing threshold variation in the backcross mice. The important effect that genetic background has on the inner ear phenotype of Atp6v1b1 mutant mice provides insight into the hearing loss variability associated with dRTA caused by ATP6V1B1 mutations. Because MRL-Atp6v1b1vxt/vtx mice do not recapitulate the metabolic acidosis of dRTA patients, they provide a new genetic model for nonsyndromic deafness with enlarged vestibular aqueduct (EVA; OMIM #600791). © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  17. Music and Deaf Culture: Images from the Media and Their Interpretation by Deaf and Hearing Students.

    Science.gov (United States)

    Darrow; Loomis

    1999-01-01

    The purpose of the study was threefold: (a) to examine how the visual media have portrayed the subject of music and the deaf, (b) to verify the validity of these portrayals with members of the deaf community, and (c) to compare and contrast deaf and hearing audiences' impressions of these portrayals. An additional purpose of the research was to examine the results in light of possible misconceptions that may be construed by music therapists and music educators based upon the media's representation of the relationship between music and deaf culture. Since music therapists and music educators are the primary persons responsible for the music instruction of students in school programs for deaf and hard-of-hearing students, it is particularly important that they receive accurate messages about the relationship of music to deaf culture. Fifty deaf (n = 25) and hearing (n = 25) undergraduate college students individually viewed motion picture and television excerpts related to music and the deaf. Subjects were instructed to take notes as needed regarding the content of each excerpt and their impressions. Students were then interviewed in their native language, English or American Sign Language, as to their interpretations and perceptions regarding these excerpts and their accuracy. Interviews of the deaf students were translated into English from American Sign Language by trained interpreters. Written transcriptions were then made of the interpreters' English translations of the interviews with deaf students and of the verbal interviews with hearing students. Interview transcripts from both groups were coded and analyzed for recurring themes and patterns using content analysis. Data analysis revealed cultural patterns for the two groups, impressions specific to individual subjects, and trends in communication style and content for the two groups. Implications for music therapists and music educators are given regarding the influence of the media, characteristics of deaf

  18. Peer to peer deaf literacy: working with young deaf people and peer tutors in India

    OpenAIRE

    Gillen, Julia; Panda, Sibaji; Papen, Uta; Zeshan, Ulrike

    2016-01-01

    This report of research in progress introduces the project: Literacy development with deaf communities using sign language, peer tuition, and learner-generated online content: Sustainable educational innovation. The rationale, aims, and participatory approach to learning and teaching English literacy to deaf learners in India are described. Deaf learners are particularly marginalised in the mainstream educational systems of developing countries. This project responds through designing a parti...

  19. Waardenburg syndrome in childhood deafness in Cameroon

    African Journals Online (AJOL)

    Waardenburg syndrome (WS) is an inherited disorder in which patients exhibit varying combinations of sensorineural hearing loss and abnormal pigmentation of the eyes, hair and skin.[1-4]. WS is clinically heterogeneous and has been classified into four major types. Type I (WS1,. MIM 193500) is characterised by ...

  20. Stigma in Mothers of Deaf Children

    Directory of Open Access Journals (Sweden)

    Hossein Ebrahimi

    2015-03-01

     Results: Results showed that most mothers suffer from stigma due to having a deaf child. The mean stigma score was 96.48 ±27.72. In total, 24.4% of mothers reported that they had received strange and mocking looks; 72.2% regarded child deafness as a sign of divine retribution; and 33.3% felt ashamed of their child’s deafness. There was an inverse relationship between the mother’s level of education and mean stigma scores (P

  1. Stress Deafness in Persian Speakers

    Directory of Open Access Journals (Sweden)

    Mehran Ghajargar

    2012-10-01

    Full Text Available Background and Aim: The current study has been designed based on the framework of the Stress Deafness Model (SDM, and aims at investigating native listeners’ general perceptual sensitivity to theacoustic correlates of stress in Persian, a language with predictable stress. According to SDM, more regularity in a language implies poorer perceptual sensitivity of its native speakers, as regular stress patterns will not require lexical encoding.Methods: The experiment was a modified method of adjustment task where subjects had to simulate stimuli played to them. A total of thirty five Persian speakers took part in the experiment and wereplaced in three groups based on their linguistic background. In addition to overall perceptual sensitivity, the effect of exposure to English and phonetic knowledge were also tested.Results: Persian speakers showed a weak perception of stress correlates. It was found, however, that exposure to English will improve stress deafness among Persian natives (p<0.0001. However, the results failed to show any significant effect by phonetic knowledge. It was also shown that the duration had the most erroneous perception by participants (p=0.0001, while there was no statistically significant difference between understanding fundamental frequency clues and intensity perception by listeners.Conclusion: Since Persian speakers showed an overall weak perception of stress correlates, the results support the predictions made by SDM.

  2. Suicide in deaf populations: a literature review

    Directory of Open Access Journals (Sweden)

    Kapur Navneet

    2007-10-01

    Full Text Available Abstract Background Studies have found that deaf individuals have higher rates of psychiatric disorder than those who are hearing, while at the same time encountering difficulties in accessing mental health services. These factors might increase the risk of suicide. However, the burden of suicidal behaviour in deaf people is currently unknown. The aim of the present review was to provide a summary of literature on suicidal behaviour with specific reference to deaf individuals. The objectives of the review were to establish the incidence and prevalence of suicidal behaviour in deaf populations; describe risk factors for suicidal behaviour in deaf populations; describe approaches to intervention and suicide prevention that have been used in deaf populations. Methods A number of electronic databases (e.g. Medline, PsycINFO, CINAHL, EMBASE, Dissertation Abstracts International, Web of Science, ComDisDome, ASSIA, Education Sage Full Text, Google Scholar, and the grey literature databases FADE and SIGLE were explored using a combination of key words and medical subject headings as search terms. Reference lists of papers were also searched. The Science and Social Sciences Citation Index electronic databases were used to identify studies that had cited key papers. We also contacted experts and organisations with an interest in the field. Results Very few studies focussed specifically on suicide in deaf populations. Those studies that were included (n = 13 generally involved small and unrepresentative samples. There were limited data on the rate of suicidal behaviour in deaf people. One study reported evidence of hearing impairment in 0.2% of all suicide deaths. Another found that individuals with tinnitus seen in specialist clinics had an elevated rate of suicide compared to the general population. The rates of attempted suicide in deaf school and college students during the previous year ranged from 1.7% to 18%, with lifetime rates as high as 30

  3. Silencing Deafness: Displacing Disability in the Nineteenth Century

    Directory of Open Access Journals (Sweden)

    Esme Cleall

    2015-03-01

    Full Text Available This article traces the way in which the language of displacement and silence were used in nineteenth-century discussions of deafness and connects this tendency to the marginalised place deaf experience occupies historically. Throughout the nineteenth century, a period which saw the consolidation of ‘the deaf and dumb’ as a social category, the word ‘forgetting’ crept into numerous discussions of deafness by both deaf and hearing commentators. Some, such as the educationalist Alexander Graeme Bell, were overt in their desire to forget deafness, demanding disability was ‘bred out’ and deaf culture condemned to the forgotten past. Others used the term ambivalently and sometimes metaphorically discussing the deaf as ‘forgotten’ by society, and ‘children of silence’. Some even pleaded that people who were deaf were not forgotten. But, though varied, the use of the imagery of forgetting and silence to evoke deafness is recurrent, and may, therefore, be seen to reveal something about how deaf experience can be approached as a displacement where deafness was spatially and imaginatively marginalised. I argue that one of the consequences of the conceptual framing of deafness through the language of forgetting was actively to silence deafness and to neutralise the idea that disability should be marginal and could be forgotten.

  4. Imaging of postoperative sensorineural complications of stapes surgery: a pictorial essay.

    Science.gov (United States)

    Ayache, Denis; Lejeune, Delphine; Williams, Marc T

    2007-01-01

    Sensorineural hearing loss and/or vertigo are rare but severe complications of stapes surgery for otosclerosis, ranging from 0.2 to 3%. Management of such complications depends on the underlying cause: intravestibular protrusion of the prosthesis, perilymph fistula, labyrinthitis, and reparative granuloma extending into the vestibule. Surgery is mandatory in cases of intravestibular prosthesis or of persistent perilymph fistula. In cases of suppurative labyrinthitis or reparative granuloma extending into the vestibule, prognosis is usually poor, despite aggressive medical therapy or revision surgery. CT scan or magnetic resonance imaging can frequently help to determine the cause of the inner ear complication of stapedectomy. Demonstrative cases are presented to illustrate the prominent place of imaging in managing sensorineural complications of stapes surgery.

  5. Mechanisms of Sensorineural Cell Damage, Death and Survival in the Cochlea

    Directory of Open Access Journals (Sweden)

    Allen Frederic Ryan

    2015-04-01

    Full Text Available The majority of acquired hearing loss, including presbycusis, is caused by irreversible damage to the sensorineural tissues of the cochlea. This article reviews the intracellular mechanisms that contribute to sensorineural damage in the cochlea, as well as the survival signaling pathways that can provide endogenous protection and tissue rescue. These data have primarily been generated in hearing loss not directly related to age. However, there is evidence that similar mechanisms operate in presbycusis. Moreover, accumulation of damage from other causes can contribute to age-related hearing loss. Potential therapeutic interventions to balance opposing but interconnected cell damage and survival pathways, such as antioxidants, anti-apoptotics, and pro-inflammatory cytokine inhibitors, are also discussed.

  6. [Hypoacusis-deafness related to perinatal adverse conditions. According to the register available in a specialized unit of Ciudad de Mexico. Analysis according to birth weight].

    Science.gov (United States)

    Peñazola-López, Y R; Castillo-Maya, G; García-Pedroza, F; Sánchez-López, H

    2004-01-01

    To analyse perinatal adverse conditions related with birth weight in 160 children with hearing impairment-deafness (prelingual sensorineural hearing loss) according to medical data of a specialized medical unit in Mexico City. The average age of children was 4 years old +/- 1.7; 61% were male. 47.5% of the cases had a birthweight between 1,500 and 2,900 g. 90 cases (56%) had an abnormal delivery and 68 (42.5%) were first pregnancy cases. A high number of cases had delayed breathing, cyanosis, jaundice or respiratory failure. 104 cases (65%) had deafness and 56 (35%) presented. hearing loss. Only 11% of the children had a birth-weight under 1,500 g. 58% of all cases had a birth weight lower than 3,000 g. The quality of medical assistance provided during the perinatal period has a very important role in origin of hearing impairment--deafness in this group of children. It is important to increase our knowledge about the perinatal adverse conditions in order to establish preventive programs.

  7. A Teacher Takes on the Challenges of Deaf Literacy: An Interview with Jennifer Herbold.

    Science.gov (United States)

    Milone, Michael

    2003-01-01

    Interviews Jennifer Herbold, a deaf teacher of deaf students at the New Mexico School for the Deaf. Discusses important factors in determining Deaf students' success at learning to read. Notes that technology has enormous potential with deaf students because it provides increased exposure to English, it is often fun to use for deaf students, and…

  8. Prothrombotic gene mutations in patients with sudden sensorineural hearing loss and cardiovascular thrombotic disease.

    Science.gov (United States)

    Capaccio, Pasquale; Cuccarini, Valeria; Ottaviani, Francesco; Fracchiolla, Nicola Stefano; Bossi, Anna; Pignataro, Lorenzo

    2009-03-01

    Impaired cochlear perfusion seems to be an important event in sudden sensorineural hearing loss. Prothrombotic gene mutations have been related to vascular disorders and sudden hearing loss. We assessed the prothrombotic risk in 10 patients with sudden sensorineural hearing loss who had previously experienced cardiovascular events to support its vascular pathogenesis. Ten patients underwent hematologic tests (MTHFR C677T/A1298C, prothrombin G20210A, platelet GlyIIIaA1/A2, and V Leiden G1691A genotyping; fibrinogenemia; cholesterolemia: homocysteinemia; folatemia). The results were compared with those of 100 previously investigated patients with sudden hearing loss alone and those of 200 healthy controls. DNA was isolated from peripheral blood leukocytes, and the gene mutations were investigated by polymerase chain reaction and a LightCycler DNA analyzer. Two patients had 2 mutant alleles, 6 had 3, and 2 had 4. The mean homocysteine, cholesterol, and fibrinogen levels were above the upper limit of normal; the mean folate levels were slightly above the lower limit of normal. Multiple mutations were more frequent in the patient group than in the previously analyzed patients and healthy controls. The association between inherited and acquired prothrombotic factors in patients with sudden sensorineural hearing loss and thrombotic diseases in other sites suggests that a multifactorial mechanism may underlie microvascular cochlear impairment. Hematologic investigation, including MTHFR, prothrombin, platelet, and V Leiden genotyping, may help to detect patients at potential risk of recurrent hearing loss and multiple microvascular diseases, and could be usefully performed in otherwise idiopathic sudden sensorineural hearing loss.

  9. Diminished temporal coding with sensorineural hearing loss emerges in background noise.

    Science.gov (United States)

    Henry, Kenneth S; Heinz, Michael G

    2012-10-01

    Behavioral studies in humans suggest that sensorineural hearing loss (SNHL) decreases sensitivity to the temporal structure of sound, but neurophysiological studies in mammals provide little evidence for diminished temporal coding. We found that SNHL in chinchillas degraded peripheral temporal coding in background noise substantially more than in quiet. These results resolve discrepancies between previous studies and help to explain why perceptual difficulties in hearing-impaired listeners often emerge in noisy situations.

  10. Language Development and Impairment in Children with Mild to Moderate Sensorineural Hearing Loss

    OpenAIRE

    Halliday, L. F.; Tuomainen, O.; Rosen, S.

    2017-01-01

    PURPOSE: The goal of this study was to examine language development and factors related to language impairments in children with mild to moderate sensorineural hearing loss (MMHL). METHOD: Ninety children, aged 8–16 years (46 children with MMHL; 44 aged-matched controls), were administered a battery of standardized language assessments, including measures of phonological processing, receptive and expressive vocabulary and grammar, word and nonword reading, and parental report of communica...

  11. Mutations in OTOGL, encoding the inner ear protein otogelin-like, cause moderate sensorineural hearing loss.

    Science.gov (United States)

    Yariz, Kemal O; Duman, Duygu; Zazo Seco, Celia; Dallman, Julia; Huang, Mingqian; Peters, Theo A; Sirmaci, Asli; Lu, Na; Schraders, Margit; Skromne, Isaac; Oostrik, Jaap; Diaz-Horta, Oscar; Young, Juan I; Tokgoz-Yilmaz, Suna; Konukseven, Ozlem; Shahin, Hashem; Hetterschijt, Lisette; Kanaan, Moien; Oonk, Anne M M; Edwards, Yvonne J K; Li, Huawei; Atalay, Semra; Blanton, Susan; Desmidt, Alexandra A; Liu, Xue-Zhong; Pennings, Ronald J E; Lu, Zhongmin; Chen, Zheng-Yi; Kremer, Hannie; Tekin, Mustafa

    2012-11-02

    Hereditary hearing loss is characterized by a high degree of genetic heterogeneity. Here we present OTOGL mutations, a homozygous one base pair deletion (c.1430 delT) causing a frameshift (p.Val477Glufs(∗)25) in a large consanguineous family and two compound heterozygous mutations, c.547C>T (p.Arg183(∗)) and c.5238+5G>A, in a nonconsanguineous family with moderate nonsyndromic sensorineural hearing loss. OTOGL maps to the DFNB84 locus at 12q21.31 and encodes otogelin-like, which has structural similarities to the epithelial-secreted mucin protein family. We demonstrate that Otogl is expressed in the inner ear of vertebrates with a transcription level that is high in embryonic, lower in neonatal, and much lower in adult stages. Otogelin-like is localized to the acellular membranes of the cochlea and the vestibular system and to a variety of inner ear cells located underneath these membranes. Knocking down of otogl with morpholinos in zebrafish leads to sensorineural hearing loss and anatomical changes in the inner ear, supporting that otogelin-like is essential for normal inner ear function. We propose that OTOGL mutations affect the production and/or function of acellular structures of the inner ear, which ultimately leads to sensorineural hearing loss. Copyright © 2012 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  12. Cochlear pathology of long term neomycin induced deafness in cats.

    Science.gov (United States)

    Leake, P A; Hradek, G T

    1988-04-01

    The long term sequelae of hair cell destruction consequent from administration of the ototoxic aminoglycoside antibiotic, neomycin sulfate, were evaluated in histological and ultrastructural studies of cochlear morphology in cats. Complete hearing loss, as defined by an absence of brainstem evoked responses to click stimulation at 120 dB peak SPL, was induced by intramuscular injections of neomycin at 50 mg/kg body weight/day, and cochlear pathology was studied at 6 months and 1, 3 and 4 years following onset of profound deafness. In these long term ototoxicity cases the organ of Corti was collapsed and resorbed over the basal one-quarter to three-quarters of the cochlear spiral, depending on duration of deafness. Significant progressive reduction in the spiral ganglion cell population and sequential degenerative alterations in the remaining neurons were observed with increasing time elapsed after induced hearing loss. The sequence of pathological alterations in spiral ganglion neurons appeared to be: a) swelling, demyelination and degeneration of the peripheral dendrites; b) demyelination and shrinkage of the cell soma with preservation of the central axon; and c) demyelination of the central axon and degeneration of the cell perikaryon. In apical cochlear regions, severe degeneration of the spiral ganglion preceded the collapse of the tunnel of Corti and regional loss of pillar cells. Residual populations of spiral ganglion neurons were as low as 1-2% of the normal values in the most severely degenerated cochleae in the series. Light microscopic and ultrastructural studies revealed a selective survival advantage for the unmyelinated type II neurons over the myelinated type I neurons with these long survival periods. The prolonged time course and atrophic nature of these pathological alterations suggests that degeneration of spiral ganglion neurons progresses continuously following drug-induced insult to the cochlea. Some possible factors contributing to this long

  13. Syllabic organization and deafness: orthographic structure or letter frequency in reading?

    Science.gov (United States)

    Olson, A C; Nickerson, J F

    2001-05-01

    For hearing people, structure given to orthographic information may be influenced by phonological structures that develop with experience of spoken language. In this study we examine whether profoundly deaf individuals structure orthographic representation differently. We ask "Would deaf students who are advanced readers show effects of syllable structure despite their altered experience of spoken language, or would they, because of reduced influence from speech, organize their orthographic knowledge according to groupings defined by letter frequency?" We used a task introduced by Prinzmetal (Prinzmetal, Treiman, & Rho, 1986) in which participants were asked to judge the colour of letters in briefly presented words. As with hearing participants, the number of errors made by deaf participants was influenced by syllable structure (Prinzmetal et al., 1986; Rapp, 1992). This effect could not be accounted for by letter frequency. Furthermore, there was no correlation between the strength of syllable effects and residual speech or hearing. Our results support the view that the syllable is a unit of linguistic organization that is abstract enough to apply to both spoken and written language.

  14. Deafness and permanently reduced potassium channel gene expression and function in hypothyroid Pit1dw mutants

    Science.gov (United States)

    Mustapha, Mirna; Fang, Qing; Gong, Tzy-Wen; Dolan, David F.; Raphael, Yehoash; Camper, Sally A.; Duncan, R. Keith

    2012-01-01

    The absence of thyroid hormone (TH) during late gestation and early infancy can cause irreparable deafness in both humans and rodents. A variety of rodent models have been utilized in an effort to identify the underlying molecular mechanism. Here, we characterize a mouse model of secondary hypothyroidism, pituitary transcription factor 1 (Pit1dw), which has profound, congenital deafness that is rescued by oral TH replacement. These mutants have tectorial membrane abnormalities, including a prominent Hensen's stripe, elevated β-tectorin composition, and disrupted striated-sheet matrix. They lack distortion product otoacoustic emissions and cochlear microphonic responses, and exhibit reduced endocochlear potentials, suggesting defects in outer hair cell function and potassium recycling. Auditory system and hair cell physiology, histology and anatomy studies reveal novel defects of hormone deficiency related to deafness: (1) permanently impaired expression of KCNJ10 in the stria vascularis of Pit1dw mice, which likely contributes to the reduced endocochlear potential, (2) significant outer hair cell loss in the mutants, which may result from cellular stress induced by the lower KCNQ4 expression and current levels in Pit1dw mutant outer hair cells and (3) sensory and strial cell deterioration, which may have implications for thyroid hormone dysregulation in age related hearing impairment. In summary, we suggest that these defects in outer hair cell and strial cell function are important contributors to the hearing impairment in Pit1dw mice. PMID:19176829

  15. Cochlear implant: Speech and language development in deaf and hard of hearing children following implantation

    Directory of Open Access Journals (Sweden)

    Ostojić Sanja

    2011-01-01

    Full Text Available Bacground/Aim. Almost 200 cochlear implantations were done in the four centers (two in Belgrade, per one in Novi Sad and Niš in Serbia from 2002 to 2009. Less than 10% of implantees were postlingually deaf adults. The vast majority, i.e. 90% were pre- and perilingually profoundly deaf children. The aim of this study was to assess the influence of improved auditory perception due to cochlear implantation on comprehension of abstract words in children as compared with hearing impaired children with conventional hearing aids and normal hearing children. Methods. Thirty children were enrolled in this study: 20 hearing impaired and 10 normal hearing. The vocabulary test was used. Results. The overall results for the whole test (100 words showed a significant difference in favor of the normal hearing as compared with hearing impaired children. The normal hearing children successfully described or defined 77.93% of a total of 100 words. Success rate for the cochlear implanted children was 26.87% and for the hearing impaired children with conventional hearing aids 20.32%. Conclusion. Testing for abstract words showed a statistically significant difference between the cochlear implanted and the hearing impaired children with hearing aids (Mann- Whitney U-test, p = 0.019 implying considerable advantage of cochlear implants over hearing aids regarding successful speech development in prelingually deaf children.

  16. Temporal entrainment of visual attention in children: effects of age and deafness.

    Science.gov (United States)

    Dye, Matthew W G

    2014-12-01

    The major documented effect of auditory deprivation on visual processing is enhanced spatial attention, in particular to the visual periphery and to moving stimuli. However, there is a parallel literature that has reported deficits in temporal aspects of visual processing in individuals with profound hearing losses. This study builds upon previous work showing possible deficits in processing of rapid serial visual presentation streams in deaf children [Restorative Neurology and Neuroscience (2010), 28, 181-192]. Deaf native signers of American Sign Language and hearing children and adults were asked to perform a 2-AFC identification task with a visual target embedded in a stream of visual stimuli presented at 6 Hz. Both children and adults displayed attentional awakening, whereby target identification accuracy improved as the number of stimuli preceding the target increased. For deaf children, however, this awakening effect was less pronounced than that observed in hearing children, interpreted as difficulty sustaining entrainment to the stimulus stream. The data provide the first account of attentional awakening in children, showing that it improves across the 6-13 year age range. They also provide additional support to the possibility of domain-general alterations in the processing of temporal information in the absence of auditory input. Copyright © 2014 Elsevier Ltd. All rights reserved.

  17. Environmental assessment, Deaf Smith County site, Texas

    Energy Technology Data Exchange (ETDEWEB)

    1986-05-01

    The Nuclear Waste Policy Act of 1982 (42 USC sections 10101-10226) requires the environmental assessment of a proposed site to include a statement of the basis for nominating a site as suitable for characterization. Volume 2 provides a detailed statement evaluating the site suitability of the Deaf Smith County Site under DOE siting guidelines, as well as a comparison of the Deaf Smith County Site to the other sites under consideration. The evaluation of the Deaf Smith County Site is based on the impacts associated with the reference repository design, but the evaluation will not change if based on the Mission Plan repository concept. The second part of this document compares the Deaf Smith County Site to Davis Canyon, Hanford, Richton Dome and Yucca Mountain. This comparison is required under DOE guidelines and is not intended to directly support subsequent recommendation of three sites for characterization as candidate sites. 259 refs., 29 figs., 66 refs. (MHB)

  18. Environmental assessment: Deaf Smith County site, Texas

    Energy Technology Data Exchange (ETDEWEB)

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified a location in Deaf Smith County, Texas, as one of the nine potentially acceptable sites for mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Deaf Smith County site and eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. The Deaf Smith County site is in the Permian Basin, which is one of five distinct geohydrologic settings considered for the first repository. On the basis of the evaluations reported in this EA, the DOE has found that the Deaf Smith County site is not disqualified under the guidelines.

  19. Health Care Access Among Deaf People

    National Research Council Canada - National Science Library

    Kuenburg, Alexa; Fellinger, Paul; Fellinger, Johannes

    2016-01-01

    .... The present study reviews literature from 2000 to 2015 on access to health care for deaf people and reveals significant challenges in communication with health providers and gaps in global health...

  20. Human dignity and the profoundly disabled: a theological perspective.

    Science.gov (United States)

    Matthews, Pia

    2011-01-01

    One challenge to the concept of human dignity is that it is a rootless notion invoked simply to mask inequalities that inevitably exist between human beings. This privileging of humans is speciesist and its weak point is the profoundly disabled human being. This article argues that far from being a weak point, the profoundly disabled person is a source of strength and witness to the intrinsic dignity that all human beings have by virtue of being human. The disabled represent the reality of human existence that is both strong and fragile. Although human dignity can be understood philosophically its depth is rooted in Christian theological insights. The profoundly disabled occupy a privileged position and share in a theology of mission since they testify to the interdependence of every human being and human dependence on God to a myopic world that only values strength, autonomy and independence.

  1. Stigma in Mothers of Deaf Children

    Directory of Open Access Journals (Sweden)

    Hossein Ebrahimi

    2015-03-01

    Full Text Available Introduction: A deaf child creates a feeling of stigma in many hearing parents. Stigma in mothers can have a negative impact on a child’s treatment and rehabilitation process. Therefore, this study was conducted to evaluate the extent of stigma in mothers with deaf children.  Materials and Methods: This descriptive, cross-sectional study was conducted in 2013 among 90 mothers with deaf children. The data-collection instrument included the stigma scale in the mothers of children with disabilities. The reliability and validity of the instrument were confirmed through content validity and Cronbach’s alpha coefficient (α=86%, respectively. Data were analyzed using SPSS-15 software.   Results: Results showed that most mothers suffer from stigma due to having a deaf child. The mean stigma score was 96.48 ±27.72. In total, 24.4% of mothers reported that they had received strange and mocking looks; 72.2% regarded child deafness as a sign of divine retribution; and 33.3% felt ashamed of their child’s deafness. There was an inverse relationship between the mother’s level of education and mean stigma scores (P

  2. Access to English Language Acquisition in Ghana Schools for the Deaf: Are the Deaf Students Handicapped?

    Science.gov (United States)

    Obosu, Gideon Kwesi; Opoku-Asare, Nana Afia; Deku, Prosper

    2016-01-01

    This paper primarily discusses the challenges deaf students in Ghana are likely to grapple with as they access education provided for them in English language. The arguments discussed in this paper are supported by findings from a multiple site case study of five Schools for the Deaf purposively sampled from four regions of Ghana. Observations…

  3. Objectification Theory and Deaf Cultural Identity Attitudes: Roles in Deaf Women's Eating Disorder Symptomatology

    Science.gov (United States)

    Moradi, Bonnie; Rottenstein, Adena

    2007-01-01

    This study examined the generalizability of direct and mediated links posited in objectification theory among internalization of sociocultural standards of beauty, body surveillance, body shame, and eating disorder symptoms with a sample of Deaf women. The study also examined the role of marginal Deaf cultural identity attitudes within this…

  4. "Deaf discourse": the social construction of deafness in a Bedouin community

    NARCIS (Netherlands)

    Kisch, S.

    2008-01-01

    Among the Al-Sayyid Arab-Bedouin, the use of an indigenous sign language is widespread and provides the foundation of a signing community shared by hearing and deaf people. Cases with comparable high incidences of deafness have in recent years stimulated debates in diverse academic disciplines.

  5. Changes in tonal audiometry in children with progressive sensorineural hearing loss and history of Neonatal Intensive Care Unit discharge. A 20 year long-term follow-up.

    Science.gov (United States)

    Martínez-Cruz, Carlos F; Poblano, Adrián; García-Alonso Themann, Patricia

    2017-10-01

    Newborns from Neonatal intensive care units (NICU) are at high-risk for sensorineural hearing loss (SNHL) a follow-up is needed for early diagnosis and intervention. Our objective here was to describe the features and changes of SNHL at different periods during a follow-up of almost 20 years. Risk factors for SNHL during development were analyzed. The audiological examination included: Brainstem auditory evoked potentials (BAEP), and Transient evoked otoacoustic emissions (TEOAE). At birth; tonal audiometry (between 125 and 8000 Hz), and tympanometry were performed at 5, 10, 15, and 20 years of age. Sixty-five percent of cases presented bilateral absence of BAEP. At 5 years of age, the most frequent SNHL level was severe (42.5%), followed by moderate (22.5%), and profound level (20%), in all cases, the SNHL was symmetrical with a predominance of lesion for the high frequencies. Exchange transfusion was associated with a higher degree of SNHL (OR = 6.00, CI = 1.11-32.28, p < 0.02). In 55%, SNHL remained stable, but in 40% of the cases it was progressive. At the end of the study six cases with moderate loss progressed to the severe level and seven cases with severe level progressed to profound. Forty percent of infants with SNHL discharged from NICU may present a progression in the hearing loss. Exchange transfusion was associated with a higher degree of SNHL. NICU graduates with SNHL merit a long-term audiological follow-up throughout their lifespan. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. Deafness and Diversity: Early Intervention.

    Science.gov (United States)

    Jackson, Rebecca L; Ammerman, Sarah B; Trautwein, Blane A

    2015-01-01

    earlier identification has increased the number of infants identified with hearing loss. A significant and growing proportion of children who are D/deaf or hard of hearing have a disability (DWD). Literature related to infants and toddlers who are DWD is scarce because of the heterogeneity of the population and because many disabilities may go undiagnosed until a child is older. Service availability, professional preparation, and use of evidence-based practices must improve to best meet the needs of these children and their families. An examination of theory, research, and practice in early intervention for children who are DWD revealed a lack of qualified professionals and a need for targeted instruction in teacher preparation programs and for technological advances paired with treatment (e.g., telepractice). Increased transdisciplinary collaboration and technology utilization in teacher preparation hold promise as ways of improving service provision to young children who are DWD.

  7. Headbobber: a combined morphogenetic and cochleosaccular mouse model to study 10qter deletions in human deafness.

    Directory of Open Access Journals (Sweden)

    Annalisa Buniello

    Full Text Available The recessive mouse mutant headbobber (hb displays the characteristic behavioural traits associated with vestibular defects including headbobbing, circling and deafness. This mutation was caused by the insertion of a transgene into distal chromosome 7 affecting expression of native genes. We show that the inner ear of hb/hb mutants lacks semicircular canals and cristae, and the saccule and utricle are fused together in a single utriculosaccular sac. Moreover, we detect severe abnormalities of the cochlear sensory hair cells, the stria vascularis looks severely disorganised, Reissner's membrane is collapsed and no endocochlear potential is detected. Myo7a and Kcnj10 expression analysis show a lack of the melanocyte-like intermediate cells in hb/hb stria vascularis, which can explain the absence of endocochlear potential. We use Trp2 as a marker of melanoblasts migrating from the neural crest at E12.5 and show that they do not interdigitate into the developing strial epithelium, associated with abnormal persistence of the basal lamina in the hb/hb cochlea. We perform array CGH, deep sequencing as well as an extensive expression analysis of candidate genes in the headbobber region of hb/hb and littermate controls, and conclude that the headbobber phenotype is caused by: 1 effect of a 648 kb deletion on distal Chr7, resulting in the loss of three protein coding genes (Gpr26, Cpmx2 and Chst15 with expression in the inner ear but unknown function; and 2 indirect, long range effect of the deletion on the expression of neighboring genes on Chr7, associated with downregulation of Hmx3, Hmx2 and Nkx1.2 homeobox transcription factors. Interestingly, deletions of the orthologous region in humans, affecting the same genes, have been reported in nineteen patients with common features including sensorineural hearing loss and vestibular problems. Therefore, we propose that headbobber is a useful model to gain insight into the mechanisms underlying deafness in

  8. Long-term subjective benefit with a bone conduction implant sound processor in 44 patients with single-sided deafness.

    Science.gov (United States)

    Desmet, Jolien; Wouters, Kristien; De Bodt, Marc; Van de Heyning, Paul

    2014-07-01

    Studies that investigate the subjective benefit from a bone conduction implant (BCI) sound processor in patients with single-sided sensorineural deafness (SSD) have been limited to examining short- and mid-term benefit. In the current study, we performed a survey among 44 SSD BCI users with a median follow-up time of 50 months. Forty-four experienced SSD BCI users participated in the survey, which consisted of the Abbreviated Profile of Hearing Aid Benefit, the Single-Sided Deafness Questionnaire, the Short Hearing Handicap Inventory for Adults, and a self-made user questionnaire. For patients with tinnitus, the Tinnitus Questionnaire was also completed. The results of the survey were correlated with contralateral hearing loss, age at implantation, duration of the hearing loss at the time of implantation, duration of BCI use, and the presence and burden of tinnitus. In total, 86% of the patients still used their sound processor. The Abbreviated Profile of Hearing Aid Benefit and the Short Hearing Handicap Inventory for Adults show a statistically significant overall improvement with the BCI. The Single-Sided Deafness Questionnaire and the user questionnaire showed that almost 40% of the patients reported daily use of the sound processor. However, the survey of daily use reveals benefit only in certain circumstances. Speech understanding in noisy situations is rated rather low, and 58% of all patients reported that their BCI benefit was less than expected. The majority of the patients reported an overall improvement from using their BCI. However, the number of users decreases during a longer follow-up time and patients get less enthusiastic about the device after an extended period of use, especially in noisy situations. However, diminished satisfaction because of time-related reductions in processor function could not be ruled out.

  9. Multisensory Speech Perception by Profoundly Hearing-Impaired Children.

    Science.gov (United States)

    Lynch, Michael P.; And Others

    1989-01-01

    Eight profoundly hearing-impaired children, aged 5-11, received tactual word recognition training with tactual speech perception aids. Following training, subjects were tested on trained words and new words. Performance was significantly better on both sets of words when words were presented with a combined condition of tactual aid and aided…

  10. Standing Ovations and Profound Learning: Cultural Diversity in Theatre.

    Science.gov (United States)

    Ellis, Roger

    2000-01-01

    Describes the profound learning that took place at the International Children's Theatre Festival in Toyama City, Japan in July 2000. Argues that participation by the Japanese-American Drama Ensemble, a youth group from the public schools in Lexington, Massachusetts, and more than 400 children from all over the planet, showcased the cultural…

  11. Teaching Profoundly Retarded Adults to Ascend Stairs Safely.

    Science.gov (United States)

    Cipani, Ennio; And Others

    1982-01-01

    The study was designed to modify the stair climbing behavior of two profoundly retarded residents through backward shaping with graduated guidance, edible rewards, a correction procedure, and a 30 second timeout. Both residents showed an increase in the number of correct steps used while ascending the stairs.

  12. Pre-Language Activities for the Profoundly Mentally Retarded.

    Science.gov (United States)

    Poole, Marilyn R.; And Others

    Provided are sample lesson plans for a program to develop pre-language skills in profoundly retarded children and adults. Characteristic of the suggested activities is the stimulation of all sensory channels through structured infant-like play activities in five general areas: oral stimulation, sensory arousal, motor stimulation, vocal play, and…

  13. Profound Haemaological Changes In Rats Fed On Different Diet ...

    African Journals Online (AJOL)

    At the end of six weeks feeding period, blood samples were obtained and total leukocyte count was done. The results of total court show that animals fed in protein supplemented diet had a profound increase in their leukocyte court when compered with the control. The study shows that specific dietary elements can induce ...

  14. "Deaf discourse": the social construction of deafness in a Bedouin community.

    Science.gov (United States)

    Kisch, Shifra

    2008-01-01

    Among the Al-Sayyid Arab-Bedouin, the use of an indigenous sign language is widespread and provides the foundation of a signing community shared by hearing and deaf people. Cases with comparable high incidences of deafness have in recent years stimulated debates in diverse academic disciplines. Lacking an accurate term, they are regularly referred to as "Martha's Vineyard situations" and have often been oversimplified and romanticized. This article provides an in-depth analysis of a Bedouin shared-signing community and advocates closer investigation of both facilitating and disabling social practices, which would also allow better examination of comparable cases. This article concentrates on the shared use of sign language, the asymmetry it entails, and the manifold forms of translation and mediation that take place. Whereas most hearing Al-Sayyid persons have access to both spoken and signed modes of communication, deaf people's communication remains largely restricted to the signed mode (hence, the asymmetry). However, in contrast to the common reduction of deafness to the disabling absence of speech or need for translation, deaf people's need for translation is not unusual among the Al-Sayyid; local communication patterns involve many different forms of translation between different spoken languages, written languages, discourses, and social domains. Additionally, ample translators are readily available. Moreover, the common familiarity with deaf people and sign language facilitates the production and sharing of a unique experiential knowledge, grounded in daily experiences and practices. In this context, deafness is not easily subjugated to its medical model. However, encounters with the medical and educational establishment present a series of challenges that may severely exacerbate deaf people's structure of opportunities. Finally, I consider the attempts made so far to classify comparable cases; unfortunately, these mostly attempt to classify deaf

  15. Impact of correcting visual impairment and low vision in deaf-mute students in Pune, India

    Directory of Open Access Journals (Sweden)

    Parikshit Gogate

    2016-01-01

    Full Text Available Aim: The aim of this study was to evaluate visual acuity and vision function before and after providing spectacles and low vision devices (LVDs in deaf-mute students. Settings: Schools for deaf-mute in West Maharashtra. Methods: Hearing-impaired children in all special schools in Pune district underwent detailed visual acuity testing (with teachers' help, refraction, external ocular examination, and fundoscopy. Students with refractive errors and low vision were provided with spectacles and LVD. The LV Prasad-Functional Vision Questionnaire consisting of twenty items was administered to each subject before and after providing spectacles, LVDs. Statistical Analysis: Wilcoxon matched-pairs signed-ranks test. Results: 252/929 (27.1% students had a refractive error. 794 (85.5% were profound deaf. Two-hundred and fifty students were dispensed spectacles and LVDs. Mean LogMAR visual acuity before introduction of spectacles and LVDs were 0.33 ± 0.36 which improved to 0.058 (P < 0.0001 after intervention. It was found that difference in functional vision pre- and post-intervention was statistically significant (P < 0.0001 for questions 1–19. The most commonly reported difficulties were for performing distance task like reading the bus destination (58.7%, making out the bus number (51.1%, copying from blackboard (47.7%, and seeing whether somebody is waving hand from across the road (45.5%. In response to question number 20, 57.4% of students felt that their vision was much worse than their friend's vision, which was reduced to 17.6% after dispensing spectacles and LVDs. Conclusion: Spectacle and LVD reduced visual impairment and improved vision function in deaf-mute students, augmenting their ability to negotiate in and out of school.

  16. Intergenerational Communication Modes in Deaf-Parented Families.

    Science.gov (United States)

    Mallory, Barbara L.; And Others

    1993-01-01

    This study focused on the intergenerational modes used in 15 family triads: hearing child, deaf parent, hearing grandparent. Results raise questions about the effect of mismatched language modes on intergenerational relationships in deaf-parented families. (19 references) (VWL)

  17. The Role of Parents in the Development of Deaf Children

    National Research Council Canada - National Science Library

    IWONA JAGOSZEWSKA

    2017-01-01

    Both the parents and the deaf children live with a disability. The specificity of deafness has a significant impact on the functioning of the families which often requires support of their care and educational functions...

  18. Genetics Home Reference: autosomal dominant cerebellar ataxia, deafness, and narcolepsy

    Science.gov (United States)

    ... Twitter Home Health Conditions ADCADN Autosomal dominant cerebellar ataxia, deafness, and narcolepsy Printable PDF Open All Close ... the expand/collapse boxes. Description Autosomal dominant cerebellar ataxia, deafness, and narcolepsy ( ADCADN ) is a nervous system ...

  19. Childhood Deafness: How Big a Problem In Malawi?

    African Journals Online (AJOL)

    the prevalence of childhood deafness in societies similar to Malawi's and extrapolates ... dice chances of admission (personal communication). For those becoming deaf ... people other than his I her immediate family)?. 23. 10, Compared with ...

  20. DeafSpace and the principles of universal design.

    Science.gov (United States)

    Edwards, Claire; Harold, Gill

    2014-01-01

    Recent debates about the epistemological origins of Universal Design (UD) have questioned how far universalist design approaches can address the particularities and diversities of the human form through a series of standardised, technical responses. This article contributes to these debates by discussing an emergent architectural paradigm known as DeafSpace, which articulates a set of design principles originating from the d/Deaf community in the US. Commentary. DeafSpace has emerged as a design paradigm rooted in an expression of d/Deaf cultural identity based around sign language, rather than as a response designed to compensate for, or minimise, impairment. It distinguishes itself from UD by articulating a more user-centred design process, but its principles are arguably rooted in notions of d/Deaf identity based around consensus and homogeneity, with less attention paid to the socio-political contexts which shape diverse experiences of d/Deafness and the exclusion(s) of d/Deaf people from the built environment. While proponents of DeafSpace argue that UD and DeafSpace are not mutually exclusive, nor DeafSpace principles applicable only to d/Deaf people, questions remain about the type of spaces DeafSpace creates, most notably whether they lead to the creation of particularist spaces of and for the d/Deaf community, or reflect a set of design principles which can be embedded across a range of different environments. Implications for Rehabilitation UD as a basis for rehabilitation has been critiqued on the basis that creates "standardised", or universal solutions, thus negating the particularities of the human form. DeafSpace is an architectural paradigm rooted in socio-linguistic understandings of Deafness and the cultural identity of the Deaf community. It challenges UD's technocratic emphasis on minimising impairment and asserts design which is rooted in a more qualitative understanding of individuals' relationship with their environment. DeafSpace seeks to

  1. Inbreeding as a cause for deafness: Dadhkai study

    OpenAIRE

    Sushil Razdan; Sunil Kumar Raina; Pandita, Kamal K.; Shiveta Razdan; Renu Nanda; Rajni Kaul; Sandeep Dogra

    2012-01-01

    Background: We report on the higher prevalence of deaf-mutes from a village in Jammu and Kashmir State of India. Materials and Methods: A cross-sectional study among 79 deaf mutes using pedigree analysis, audiometry, imaging and molecular analysis. Results: A high rate of hereditary deafness with 79 individuals diagnosed to be suffering from non-syndrome deafness in a total population of 2452 individuals residing in the village. Interpretation: Flourishing of intermarriages led t...

  2. 77 FR 42187 - Relay Services for Deaf-Blind Individuals

    Science.gov (United States)

    2012-07-18

    ... COMMISSION 47 CFR Part 64 Relay Services for Deaf-Blind Individuals AGENCY: Federal Communications Commission... Communications and Video Accessibility Act of 2010, Section 105, Relay Services for Deaf-Blind Individuals, Order... Services for Deaf-Blind Individuals, CG Docket No. 10-210. Form Number: N/A. Type of Review: Revision of a...

  3. Sensitivity to Conversational Maxims in Deaf and Hearing Children

    Science.gov (United States)

    Surian, Luca; Tedoldi, Mariantonia; Siegel, Michael

    2010-01-01

    We investigated whether access to a sign language affects the development of pragmatic competence in three groups of deaf children aged 6 to 11 years: native signers from deaf families receiving bimodal/bilingual instruction, native signers from deaf families receiving oralist instruction and late signers from hearing families receiving oralist…

  4. Reading and Deaf Individuals: Perspectives on the Qualitative Similarity Hypothesis

    Science.gov (United States)

    Wang, Ye; Andrews, Jean

    2014-01-01

    In this, the first article in the "American Annals of the Deaf" special issue on English reading development for individuals who are d/Deaf and hard of hearing, the coeditors aim to promote interdisciplinary dialogue among researchers regarding literacy research with d/Deaf and hard of hearing (d/Dhh) students by setting the tone for an…

  5. Education Reforms and English Teaching for the Deaf in Japan

    Science.gov (United States)

    Quay, Suzanne

    2005-01-01

    Deaf education is in a period of great transition in Japan as a result of the "Educational Reform Plan for the 21st Century" proposed by the Japanese education ministry. Unfortunately, the communication needs of deaf students have not been taken into account in the Plan's recommendations. One area where deaf students must attain the same…

  6. Deaf Children with Disabilities: Rights under the IDEA

    Science.gov (United States)

    Raimondo, Barbara

    2010-01-01

    Parents of children who are deaf and who have disabilities often face barriers in ensuring that their children receive the services they need. Some of these barriers include lack of awareness about deafness-disability constellations, shortages of professionals knowledgeable about how deaf children learn or the impact of the disability on deaf…

  7. The Early Years: Parents and Young Deaf Children Reading Together

    Science.gov (United States)

    Lutz, Lori

    2017-01-01

    Research is just beginning to describe the role of reading in the lives of families with deaf children. While the time that deaf children spend reading or being read to represents only a small part of their lives at home, research highlights its importance for young children--hearing as well as deaf. Children whose parents read to them at home…

  8. Influences on Facial Emotion Recognition in Deaf Children

    Science.gov (United States)

    Sidera, Francesc; Amadó, Anna; Martínez, Laura

    2017-01-01

    This exploratory research is aimed at studying facial emotion recognition abilities in deaf children and how they relate to linguistic skills and the characteristics of deafness. A total of 166 participants (75 deaf) aged 3-8 years were administered the following tasks: facial emotion recognition, naming vocabulary and cognitive ability. The…

  9. Reaching the Summit: Deaf Adults as Essential Partners in Education

    Science.gov (United States)

    Bourne-Firl, Bridgetta

    2016-01-01

    How do we reach the summit in terms of supporting the best transition possible for each young deaf or hard of hearing individual in the United States? Should professionals who are hearing work alone to succeed with deaf and hard of hearing students? No matter how good the intention, if we want deaf and hard of hearing students to transition from…

  10. Programs for Deaf-blind Children and Adults.

    Science.gov (United States)

    American Annals of the Deaf, 2001

    2001-01-01

    This directory provides information on programs for deaf-blind children and adults including national and state programs, the Helen Keller Centers for Deaf-Blind Youth and Adults, and programs for training teachers of deaf-blind students. Within each broad category, programs are listed alphabetically by state and provide detailed contact…

  11. Social Factors Influencing Participation in Sport for the Deaf.

    Science.gov (United States)

    Stewart, David A.

    1987-01-01

    The article looks at social factors influencing participation in sport by the deaf including communication mode and value orientations of community, family, school, and peers of both the hearing and deaf world. A model for integration of the deaf into sports is offered. (DB)

  12. Language policy and literacy among deaf people in Lesotho ...

    African Journals Online (AJOL)

    ... as yet, failed to change the trend whereby deaf learners remain the most discriminated against. The article further argues that denying deaf learners an opportunity to acquire literacy through the Lesotho Sign Language, which is their primary language, contributes to the unsatisfactory state of deaf education in Lesotho.

  13. Congenital non-syndromal deafness at Adamarobe, an isolated ...

    African Journals Online (AJOL)

    A study to determine the prevalence, incidence and audiometric characteristics of deafness was done at Adamarobe, an isolated deaf village in Ghana. The procedures adopted include collection of family histories, pedigree, otoscopy and audiometric evaluation. As it turned out, a total of 45 deaf persons were identified in ...

  14. The Use of Acquired Speech by Deaf Adults in Nigeria.

    Science.gov (United States)

    Mba, Peter O.

    The controversy between the oral and the total communication approach to deaf education is reviewed and a study of the use of acquired speech by 71 deaf adults in Nigeria is presented. Questionnaire results are discussed in terms of demographics, cause and age at onset of deafness, use of amplification, school achievement, type of employment,…

  15. Rubella Deaf-Blind Child: Implications of Psychological Assessment. Proceedings.

    Science.gov (United States)

    Rouin, Carole

    Presented are proceedings of a conference involving authorities in testing and evaluating the blind, deaf, and deaf-blind. In a paper titled "Psychological Implications of Assessing the Deaf", C. Goetzinger discusses references used in audiology, anatomy and physiology of the ear, degrees of hearing impairment, and implications of the various…

  16. The Impact of Single-Sided Deafness upon Music Appreciation.

    Science.gov (United States)

    Meehan, Sarah; Hough, Elizabeth A; Crundwell, Gemma; Knappett, Rachel; Smith, Mark; Baguley, David M

    2017-05-01

    Many of the world's population have hearing loss in one ear; current statistics indicate that up to 10% of the population may be affected. Although the detrimental impact of bilateral hearing loss, hearing aids, and cochlear implants upon music appreciation is well recognized, studies on the influence of single-sided deafness (SSD) are sparse. We sought to investigate whether a single-sided hearing loss can cause problems with music appreciation, despite normal hearing in the other ear. A tailored questionnaire was used to investigate music appreciation for those with SSD. We performed a retrospective survey of a population of 51 adults from a University Hospital Audiology Department SSD clinic. SSD was predominantly adult-onset sensorineural hearing loss, caused by a variety of etiologies. Analyses were performed to assess for statistical differences between groups, for example, comparing music appreciation before and after the onset of SSD, or before and after receiving hearing aid(s). Results demonstrated that a proportion of the population experienced significant changes to the way music sounded; music was found to sound more unnatural (75%), unpleasant (71%), and indistinct (81%) than before hearing loss. Music was reported to lack the perceptual qualities of stereo sound, and to be confounded by distortion effects and tinnitus. Such changes manifested in an altered music appreciation, with 44% of participants listening to music less often, 71% of participants enjoying music less, and 46% of participants reporting that music played a lesser role in their lives than pre-SSD. Negative effects surrounding social occasions with music were revealed, along with a strong preference for limiting background music. Hearing aids were not found to significantly ameliorate these effects. Results could be explained in part through considerations of psychoacoustic changes intrinsic to an asymmetric hearing loss and impaired auditory scene analysis. Given the prevalence of

  17. Autozygosity mapping of autosomal recessive non-syndromic sensorineural hearing loss (ARNSSNHL)

    Energy Technology Data Exchange (ETDEWEB)

    Brown, K.A.; Nobel, A.; Markham, A.F. [Univ. of Leeds (United Kingdom)] [and others

    1994-09-01

    Congenital deafness affects about 1 in 2000 persons and is of genetic origin in approximately half these cases. The majority of congenital deafness is non-syndromic and over 75% of cases are compatible with autosomal recessive inheritance. Mapping of the loci responsible for ARNSSNHL will be complicated by genetic heterogeneity. Our approach to isolating genes involved in ARNSSNHL is by autozygosity mapping which involves the genetic analysis of children resulting from consanguineous marriages with the aim of identifying regions of homozygosity unique to the genomes of affected individuals which have been inherited from a common ancestor. The population employed in this study is the Pakistani community of Leeds, Bradford and Manchester in the UK which originated from the Mirpur region of Pakistan. Microsatellite analysis of the genome with markers spaced, on average, 10 cM apart is in progress and the investigation of 15 consanguineous families has identified one family which shows linkage to human chromosome 13q. This family appears to be linked to the same autosomal recessive deafness locus as two Tunisian families recently described and confirms that this chromosome 13q locus is also responsible, although as a minor contributor, to the deafness observed in the Pakistani population.

  18. Gênero e surdez / Gender and deafness

    Directory of Open Access Journals (Sweden)

    Madalena Klein

    2007-01-01

    Full Text Available Este artigo propõe-se discutir a temática da surdez, articulando-a com discussões referentes a gênero e sexualidade, tomando por referência autores da perspectiva dosEstudos Culturais em Educação e dos Estudos Surdos. A surdez e os surdos, assim como o gênero, são entendidos a partir da diferença cultural. No mercado de trabalho em geral, as surdas são discriminadas, porém são a maioria no professorado, o que é uma conseqüência da feminização do trabalho docente. A crescente presença feminina na liderança dos movimentos surdos pode estar relacionada a essa maioria de professoras, que carregam para os movimentos características da organização escolar. Analisamos dois artigos sobre o tema, além de um encontro sobre mulheres surdas ocorrido em Pelotas – RS, onde as participantes destacaram seu papel na luta política da associação dos surdos, enquanto aos homens cabe o papel de organizar as atividades de lazer e esportes. A luta pelos direitos das mulheres surdas vem crescendo no Brasil e há necessidade de haver mais estudos sobre essa temática.Abstract This paper discusses the topic of deafness, articulating it with discussions referring to gender and sexuality, based on authors from the perspectives of Cultural Studies in Education and Deaf Studies. Deafness and deaf people, as the gender issue, are understood through a cultural difference perspective. Deaf women are discriminated inthe workplace in general, but they are the majority in the teaching profession, a consequence of the feminization of the teaching work. The increasing female presence as leaders of deaf movements may be related to this greater rate of women as teachers, who carry the features of the school organization onto the movements. Two papers onthe topic were examined, as well as a meeting on deaf women occurring in Pelotas – RS, whose participants highlighted their role in the political struggle by the deaf people’s association, while men take

  19. Stigma in Mothers of Deaf Children

    Science.gov (United States)

    Ebrahimi, Hossein; Mohammadi, Eissa; Mohammadi, Mohammad Ali; Pirzadeh, Akbar; Mahmoudi, Hamzeh; Ansari, Ismail

    2015-01-01

    Introduction: A deaf child creates a feeling of stigma in many hearing parents. Stigma in mothers can have a negative impact on a child’s treatment and rehabilitation process. Therefore, this study was conducted to evaluate the extent of stigma in mothers with deaf children. Materials and Methods: This descriptive, cross-sectional study was conducted in 2013 among 90 mothers with deaf children. The data-collection instrument included the stigma scale in the mothers of children with disabilities. The reliability and validity of the instrument were confirmed through content validity and Cronbach’s alpha coefficient (α=86%), respectively. Data were analyzed using SPSS-15 software. Results: Results showed that most mothers suffer from stigma due to having a deaf child. The mean stigma score was 96.48 ±27.72. In total, 24.4% of mothers reported that they had received strange and mocking looks; 72.2% regarded child deafness as a sign of divine retribution; and 33.3% felt ashamed of their child’s deafness. There was an inverse relationship between the mother’s level of education and mean stigma scores (P<0.033). The stigma score was higher in mothers who were living independently of their relatives (P<0.029). The mean stigma score in mothers of children with a cochlear implant was lower than that of mothers of children with earphones (86.70 vs. 99.64), and this difference tended towards significance (P=0.057). Conclusion: This study showed that half of all mothers with deaf children were scorned and felt ashamed of having a deaf child in the family because of the stigma. The majority of mothers with deaf children felt stigmatized, and only their education and residency status affected this issue. The mothers of cochlear-implanted children perceived less stigma. Due to the various social and psychological problems caused by hearing impairment, it is necessary to consider the emotional health and psychological state of the mothers in addition to rehabilitation

  20. [Evaluation of deaf-mute patients with sensitive deafness gene screening in Shandong province].

    Science.gov (United States)

    Ji, Yu-bin; Han, Dong-yi; Wang, Da-yong; Zhou, Yu; Zhao, Cui; Wang, Hui; Lan, Lan; Wang, Qiu-ju

    2009-09-29

    To discuss how to determine the number of samples in epidemiological study about deafness genes and reveal the characteristics of GJB2, SLC26A4 and mitochondrial DNA A1555G mutations in deaf-mute patients in schools for deaf-mutes in Shandong Province. A total of 485 subjects were collected from the different schools for deaf-mutes in Shandong province. Amplified target fragments included GJB2 coding sequence, mtDNA12SrRNA and exon 8, 10, 17, 19 of SLC26A4 gene. The amplicons of mtDNA 12S rRNA were subjected to restriction enzyme Alw26I. The amplicons of patients whose enzyme reaction highly indicating A1555G mutation, amplicons of GJB2 and those exons PCR products of SLC26A4 were directly sequenced. The study revealed that 36.29% patients had two mutated alleles (homozygote & compound heterozygote) of GJB2 (24.12%) and SLC26A4 (6.60%) and mtDNA12SrRNA A1555G (5.57%). The 235delC and IVS7-2A > G were still the mutational hot spot in GJB2 and SLC26A4 respectively. The method of determining the number of sample is very important in the epidemiological study. There were about 24 thousand deaf-mute patients who were caused by three sensitive deafness genes mutations in Shandong province. Screening the sensitive deafness genes in newborn is imminent.

  1. Health promotion of families of deaf children

    Directory of Open Access Journals (Sweden)

    Mirna Albuquerque Frota

    2012-06-01

    Full Text Available Objective: To investigate the impact of hearing loss in the family dynamics of the deaf child; identify the family’s knowledge about deafness, understand how parents experience the diagnosis and treatment of child with hearing impairment. Methods: The study has aqualitative approach developed at the Center for Integrated Medical Care - NAMI, attached to the University of Fortaleza - UNIFOR located in Fortaleza - CE, Brazil. The participants were six mothers of children with hearing impairment. Data collection was carried outthrough participant observation and semi-structured interview. The Thematic Analysis of Bardin was used for processing the data. Results: After coding, some categories emerged from the discourse: Misinformation of Hearing Loss; impact of the discovery of hearingloss, caregivers and facilitators of the development of the deaf children. Conclusion: The birth of a deaf child alters the previous family balance, causing specific problems, such as the communication barrier, whose solution is related to how to handle the situation. Itis necessary to promote changes, emphasizing the involvement of caregivers and loved as facilitators of deaf child’s development. In Phonoaudiology, this attitude represents discovering new ways to identify the need for the subject, which requires strategies thatvalue their opinion, allowing the expression of expectations, perceptions, representations and feelings.

  2. Consanguinity and deafness in Omani children.

    Science.gov (United States)

    Khabori, Mazin Al; Patton, Michael A

    2008-01-01

    This study was based on a national retrospective analysis of 1400 questionnaires on the causes of deafness in Omani children, collected from 1986 to 2000. It was found that 70% of the deaf children were from parents of consanguineous marriages, and 30% from non-consanguineous unions. In those with consanguineous families 70.16% were first cousin marriages, 17.54% were second cousins, and 10.86% were from the same tribe. The proportion arising from first cousin marriages was higher than the background rate of first cousin marriages in Oman. In the total cohort, 45% had other family members with hearing loss. There was a greater chance of other relatives being affected in the consanguineous group as opposed to the non-consanguineous group (29.7% versus 15.3%). In most cases the affected relative was a deaf sibling (67.8%). We have demonstrated a higher rate of consanguinity amongst parents of deaf children in Oman and suggest this is associated with a higher frequency of autosomal recessive deafness in this paediatric population.

  3. Deaf Culture and Competing Discourses in a Residential School for the Deaf: "Can Do" versus "Can't Do"

    Science.gov (United States)

    O'Brien, Catherine A.; Placier, Peggy

    2015-01-01

    From an ethnographic case study of a state-funded residential school for the Deaf, the authors employed Critical Discourse Analysis to identify competing discourses in the talk of educators. These discourses are embedded in the historical oppression and labeling of deaf people as disabled and the development of Deaf culture as a counter-discourse.…

  4. Sign-Language Theatre and Deaf Theatre: New Definitions and Directions. Center on Deafness Publication Series No. 2.

    Science.gov (United States)

    Miles, Dorothy S.; Fant, Louie J., Jr.

    Offered are guidelines to the development of theatre for, by, and about deaf persons. Various terms used for sign-language theatre and deaf theatre are defined and discussed in an introductory section, and the use of sign language as a theatrical medium is explained. The production of theatre is covered by sections on the history of deaf theatre,…

  5. Acoustic reflex measurements and the loudness function in sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Sheila Uliel

    1980-11-01

    Full Text Available The suprathreshold acoustic reflex responses of forty two ears affected by sensorineural hearing loss of cochlear origin and fifty-eight ears demonstrating normal hearing, were recorded by means of an electro-acoustic impedance meter and attached X-Y recorder. The recordings were done in ascending and descending fashion,  at successively increasing and decreasing 5dB intensity levels from 90-120-90 dB HL respectively, for the individual pure-tone frequencies of 500, 1 000, 2 000 and 4 000 Hz. The contralateral mode of measurement was employed. Analysis of  these recordings indicated that the acoustic reflex  responses could be differentiated into five  characteristic patterns of  growth, which could be depicted upon a continuum of peaked, peaked-rounded, rounded, rounded-flat,  and flat  shapes. The peaked and peaked-rounded patterns were found  to predominate at all four pure-tone frequencies  in the normal ears, while the rounded-fiat  and flat  patterns were found  to predominate only at the higher pure-tone frequencies of 2 000 and 4 000 Hz in the ears affected  by sensorineural hearing loss. This latter relationship was also able to be applied to two disorders of  the loudness functio— loudness recruitment and hyperacusis. It was concluded that the flattened  acoustic reflex  patterns at the higher pure-tone frequencies  constituted a potential diagnostic cue related to the differential  diagnosis of sensorineural hearing loss, and to disorders of  the loudness function.

  6. Diffusion Tensor Imaging of Central Auditory Pathways in Patients with Sensorineural Hearing Loss: A Systematic Review.

    Science.gov (United States)

    Tarabichi, Osama; Kozin, Elliott D; Kanumuri, Vivek V; Barber, Samuel; Ghosh, Satra; Sitek, Kevin R; Reinshagen, Katherine; Herrmann, Barbara; Remenschneider, Aaron K; Lee, Daniel J

    2017-11-01

    Objective The radiologic evaluation of patients with hearing loss includes computed tomography and magnetic resonance imaging (MRI) to highlight temporal bone and cochlear nerve anatomy. The central auditory pathways are often not studied for routine clinical evaluation. Diffusion tensor imaging (DTI) is an emerging MRI-based modality that can reveal microstructural changes in white matter. In this systematic review, we summarize the value of DTI in the detection of structural changes of the central auditory pathways in patients with sensorineural hearing loss. Data Sources PubMed, Embase, and Cochrane. Review Methods We used the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement checklist for study design. All studies that included at least 1 sensorineural hearing loss patient with DTI outcome data were included. Results After inclusion and exclusion criteria were met, 20 articles were analyzed. Patients with bilateral hearing loss comprised 60.8% of all subjects. Patients with unilateral or progressive hearing loss and tinnitus made up the remaining studies. The auditory cortex and inferior colliculus (IC) were the most commonly studied regions using DTI, and most cases were found to have changes in diffusion metrics, such as fractional anisotropy, compared to normal hearing controls. Detectable changes in other auditory regions were reported, but there was a higher degree of variability. Conclusion White matter changes based on DTI metrics can be seen in patients with sensorineural hearing loss, but studies are few in number with modest sample sizes. Further standardization of DTI using a prospective study design with larger sample sizes is needed.

  7. Effect of antioxidant supplementation on the auditory threshold in sensorineural hearing loss: a meta-analysis.

    Science.gov (United States)

    Souza, Maria Eduarda Di Cavalcanti Alves de; Costa, Klinger Vagner Teixeira da; Vitorino, Paulo Augusto; Bueno, Nassib Bezerra; Menezes, Pedro de Lemos

    2017-08-26

    Hearing loss is conceptualized as any impairment of the ability to hear and/or detect speech or environment sounds, regardless of cause, type, or degree. It may occur at different stages of life; during pregnancy or childbirth, in childhood, adulthood or old age. It should be noted that aging is the most common cause of sensorineural hearing loss followed by noise-induced hearing loss, and both are closely related to the formation of reactive oxygen species. Dietary antioxidant supplementation has been employed as a therapeutic strategy to prevent and/or delay the risks of major human diseases. To assess randomized clinical trials to determine the effect of antioxidant supplementation on the auditory thresholds in patients of different age groups with sensorineural hearing loss. This systematic review consisted of a search in the following databases: MEDLINE, CENTRAL, ScienceDirect, Scopus, Web of Science, LILACS, SciELO and ClinicalTrials.gov. Additionally, the gray literature was also searched. The search strategy included terms related to the intervention (antioxidant supplementation), primary outcome (sensorineural hearing loss), as well as terms related to randomized clinical trials to improve search sensitivity. Based on 977 potentially relevant records identified through the search in the databases, ten full-text publications were retrieved for further evaluation. The increase in threshold at the 4kHz frequency was statistically higher in the control group (1.89 [1.01-2.78], phearing loss caused by exposure to high sound pressure levels. There was no improvement in the thresholds with vitamin E supplementation. Copyright © 2017 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  8. Acoustic reflex measurements and the loudness function in sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Sheila Uliel

    1980-08-01

    Full Text Available The suprathreshold acoustic reflex responses of forty two ears affected by sensorineural hearing loss of cochlear origin and fifty-eight ears demonstrating normal hearing, were recorded by means of an electro-acoustic impedance meter and attached X-Y recorder. The recordings were done in ascending and descending fashion,  at successively increasing and decreasing 5dB intensity levels from 90-120-90 dB HL respectively, for the individual pure-tone frequencies of 500, 1 000, 2 000 and 4 000 Hz. The contralateral mode of measurement was employed. Analysis of  these recordings indicated that the acoustic reflex  responses could be differentiated into five  characteristic patterns of  growth, which could be depicted upon a continuum of peaked, peaked-rounded, rounded, rounded-flat,  and flat  shapes. The peaked and peaked-rounded patterns were found  to predominate at all four pure-tone frequencies  in the normal ears, while the rounded-fiat  and flat  patterns were found  to predominate only at the higher pure-tone frequencies of 2 000 and 4 000 Hz in the ears affected  by sensorineural hearing loss. This latter relationship was also able to be applied to two disorders of  the loudness functio— loudness recruitment and hyperacusis. It was concluded that the flattened  acoustic reflex  patterns at the higher pure-tone frequencies  constituted a potential diagnostic cue related to the differential  diagnosis of sensorineural hearing loss, and to disorders of  the loudness function.

  9. Long-term Incidence and Degree of Sensorineural Hearing Loss in Otosclerosis.

    Science.gov (United States)

    Ishai, Reuven; Halpin, Christopher F; Shin, Jennifer J; McKenna, Michael J; Quesnel, Alicia M

    2016-12-01

    1) To evaluate the long-term incidence and degree of the sensorineural component of hearing loss (SNHL) in patients with otosclerosis after accounting for expected age-related hearing loss. 2) To identify variables that might predict development of sensorineural hearing loss due to otosclerosis. Retrospective audiometric database and chart review. Tertiary referral center. Consecutive patients with otosclerosis observed between 1994 and 2004, with ≥10 years follow-up, excluding patients with postoperative hearing loss or surgery before the initial audiogram. Bone conduction (BC) thresholds at 0.5, 1, 2, and 4 kHz and Word Recognition. BC threshold change (BCTC) over ≥10 years minus estimated age-related threshold change (ARTC) specific to age and sex for each patient (based on ISO 7029 reference population). Three-hundred fifty-seven ears (290 patients) met study criteria, including 217 ears that had undergone stapedectomy during the study period. Mean follow-up was 14.0 years. The average BCTC after subtracting estimated ARTC was 4.6, 2.6, 3, and 2.7 dB for 0.5, 1, 2, and 4 kHz frequencies, respectively. However, 34% of ears (122 ears) had clinically significant progression of SNHL during the study period (>10 dB BCTC beyond expected ARTC at ≥2 frequencies). Multivariate analysis demonstrated that the probability of developing clinically significant SNHL was higher for women (odds ratio 1.86, p = 0.018) and lower for operated patients (odds ratio 0.46, p = 0.002). The average long-term sensorineural hearing loss due to otosclerosis was statistically significantly more than for age alone at each frequency, but these average values (from 2.6 to 4.6 dB for tested frequencies) were clinically insignificant. Approximately one-third of patients with otosclerosis demonstrated a clinically significant progression of the sensorineural component of hearing loss, with the average BCTC above expected age-related changes ranging from 10.2 to 14.6 dB for

  10. A Narrow Internal Auditory Canal with Duplication in a Patient with Congenital Sensorineural Hearing Loss

    Energy Technology Data Exchange (ETDEWEB)

    Baik, Hye Won; Yu, Hye On; Kim, Kyung Soo; Kim, Gi Hyeon [Chung-Ang University Hospital, College of Medicine, Seoul (Korea, Republic of)

    2008-07-15

    A narrow internal auditory canal (IAC) with duplication is a rare anomaly of the temporal bone. It is associated with congenital sensorineural hearing loss. Aplasia or hypoplasia of the vestibulocochlear nerve may cause the hearing loss. We present an unusual case of an isolated narrow IAC with duplication that was detected by a CT scan. In this case, the IAC was divided by a bony septum into an empty stenotic inferoposterior portion and a large anterosuperior portion containing the facial nerve that was clearly delineated on MRI.

  11. Imported Case of Lassa Fever in Sweden With Encephalopathy and Sensorineural Hearing Deficit.

    Science.gov (United States)

    Grahn, Anna; Bråve, Andreas; Lagging, Martin; Dotevall, Leif; Ekqvist, David; Hammarström, Helena; Karlberg, Helen; Lagerqvist, Nina; Sansone, Martina; Tegnell, Anders; Ulleryd, Peter; Studahl, Marie

    2016-10-01

    We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas.

  12. Coronary artery bypass grafting and sensorineural hearing loss, a cohort study

    Directory of Open Access Journals (Sweden)

    Ashraf Omer

    2005-12-01

    Full Text Available Abstract Background Sudden sensorineural hearing loss is routinely encountered by the otologist. The etiology is varied and often identifiable. One of the relatively less frequent causes is surgery. Apart from being an established entity with otological surgeries, sensorineural hearing loss has also been known to occur after non-otological procedures under general anesthesia. Commonest amongst these procedures is cardiopulmonary bypass, an association that has long been recognized. However, despite the proposition of diverse hypotheses in the past, the pathophysiology remains unclear. Methods The study is a prospective matched cohort study that will be carried out in Aga Khan University Hospital, Karachi, Pakistan. Participants among exposed would include all those patients who would be undergoing coronary artery bypass surgery in the hospital who fall under the criteria for inclusion. Unexposed group would comprise of patients undergoing a non-bypass procedure of similar duration under the same type of anesthesia who meet the selection criteria. Both these groups will undergo audiometric testing at our hospital on three different occasions during the course of this study. Initially before the procedure to test the baseline hearing capacity; then one week after the procedure to assess any changes in hearing ability following the surgery; and finally a third audiogram at six weeks follow-up to assess further changes in any hearing deficits noted during the second phase of testing. Certain variables including the subjects' demographics and those concerning the procedure itself will be noted and used later for risk factors analysis. A detailed past medical and surgical history will also be obtained. Data analysis would include calculation of relative risk and significance of the results, by running the chi-square test. Other statistical tests like Fisher exact test may then be employed to facilitate data interpretation. Continuous scale may then be

  13. Disaster Relief and Crisis Intervention with Deaf Communities: Lessons Learned from the Japanese Deaf Community.

    Science.gov (United States)

    Takayama, Kota

    2017-01-01

    During natural disasters and crises, the deaf and hard of hearing community might not have full accessibility to all of the information shared with the larger hearing community. This could be due to the lack of awareness among social work professionals about these cultural and linguistic needs of this minority population. The purpose of this article is to explore the challenges faced by the deaf community and to discuss culturally and linguistically appropriate crisis intervention and mobilization to natural disaster situations based on the experiences of the Japanese deaf communities affected by the Kobe and Tohoku earthquakes.

  14. Observations on Working Psychoanalytically with a Profoundly Amnesic Patient.

    Science.gov (United States)

    Moore, Paul A; Salas, Christian E; Dockree, Suvi; Turnbull, Oliver H

    2017-01-01

    Individuals with profound amnesia are markedly impaired in explicitly recalling new episodic events, but appear to preserve the capacity to use information from other sources. Amongst these preserved capacities is the ability to form new memories of an emotional nature - a skill at the heart of developing and sustaining interpersonal relationships. The psychoanalytic study of individuals with profound amnesia might contribute to the understanding the importance of each memory system, including effects on key analytic processes such as transference and countertransference. However, psychoanalytic work in the presence of profound amnesia might also require important technical modifications. In the first report of its kind, we describe observations from a long term psychoanalytic process (72 sessions) with an individual (JL) who has profound amnesia after an anoxic episode. The nature of therapy was shaped by JL's impairment in connecting elements that belong to distant (and even relatively close) moments in the therapeutic process. However, we were also able to document areas of preservation, in what appears to be a functioning therapeutic alliance. As regards transference, the relationship between JL and his analyst can be viewed as the evolution of a narcissistic transference, and case material is provided that maps this into three phases: (i) rejecting; (ii) starting to take in; and (iii) full use of the analytic space - where each phase exhibits differing degrees of permeability between JL and the analyst. This investigation appears to have important theoretical implications for psychoanalytic practice, and for psychotherapy in general - and not only with regard to brain injured populations. We especially note that it raises questions concerning the mechanism of therapeutic action in psychoanalysis and psychotherapy, and the apparent unimportance of episodic memory for many elements of therapeutic change.

  15. Profound hyperlipidaemia due to concomitant diabetes and hypothyroidism

    OpenAIRE

    Samaan, M Constantine; Murphy, Nuala; Costigan, Colm

    2010-01-01

    A previously well 5-year-old girl presented with new onset type 1 diabetes mellitus and diabetic ketoacidosis, and was found to be profoundly hyperlipidaemic. Further investigations showed that she had associated hypothyroidism. She responded to insulin and L-thyroxine treatments and her lipid profile returned to normal 2 months after diagnosis. Despite starting anticoagulant therapy early, she developed deep vein thrombosis of the lower limb. Her family screen did not demonstrate familial hy...

  16. Observations on Working Psychoanalytically with a Profoundly Amnesic Patient

    Science.gov (United States)

    Moore, Paul A.; Salas, Christian E.; Dockree, Suvi; Turnbull, Oliver H.

    2017-01-01

    Individuals with profound amnesia are markedly impaired in explicitly recalling new episodic events, but appear to preserve the capacity to use information from other sources. Amongst these preserved capacities is the ability to form new memories of an emotional nature – a skill at the heart of developing and sustaining interpersonal relationships. The psychoanalytic study of individuals with profound amnesia might contribute to the understanding the importance of each memory system, including effects on key analytic processes such as transference and countertransference. However, psychoanalytic work in the presence of profound amnesia might also require important technical modifications. In the first report of its kind, we describe observations from a long term psychoanalytic process (72 sessions) with an individual (JL) who has profound amnesia after an anoxic episode. The nature of therapy was shaped by JL’s impairment in connecting elements that belong to distant (and even relatively close) moments in the therapeutic process. However, we were also able to document areas of preservation, in what appears to be a functioning therapeutic alliance. As regards transference, the relationship between JL and his analyst can be viewed as the evolution of a narcissistic transference, and case material is provided that maps this into three phases: (i) rejecting; (ii) starting to take in; and (iii) full use of the analytic space – where each phase exhibits differing degrees of permeability between JL and the analyst. This investigation appears to have important theoretical implications for psychoanalytic practice, and for psychotherapy in general – and not only with regard to brain injured populations. We especially note that it raises questions concerning the mechanism of therapeutic action in psychoanalysis and psychotherapy, and the apparent unimportance of episodic memory for many elements of therapeutic change. PMID:28890703

  17. Observations on Working Psychoanalytically with a Profoundly Amnesic Patient

    Directory of Open Access Journals (Sweden)

    Paul A. Moore

    2017-08-01

    Full Text Available Individuals with profound amnesia are markedly impaired in explicitly recalling new episodic events, but appear to preserve the capacity to use information from other sources. Amongst these preserved capacities is the ability to form new memories of an emotional nature – a skill at the heart of developing and sustaining interpersonal relationships. The psychoanalytic study of individuals with profound amnesia might contribute to the understanding the importance of each memory system, including effects on key analytic processes such as transference and countertransference. However, psychoanalytic work in the presence of profound amnesia might also require important technical modifications. In the first report of its kind, we describe observations from a long term psychoanalytic process (72 sessions with an individual (JL who has profound amnesia after an anoxic episode. The nature of therapy was shaped by JL’s impairment in connecting elements that belong to distant (and even relatively close moments in the therapeutic process. However, we were also able to document areas of preservation, in what appears to be a functioning therapeutic alliance. As regards transference, the relationship between JL and his analyst can be viewed as the evolution of a narcissistic transference, and case material is provided that maps this into three phases: (i rejecting; (ii starting to take in; and (iii full use of the analytic space – where each phase exhibits differing degrees of permeability between JL and the analyst. This investigation appears to have important theoretical implications for psychoanalytic practice, and for psychotherapy in general – and not only with regard to brain injured populations. We especially note that it raises questions concerning the mechanism of therapeutic action in psychoanalysis and psychotherapy, and the apparent unimportance of episodic memory for many elements of therapeutic change.

  18. [Effect of rehabilitation for prelingual deaf children who use cochlear implants in conjunction with hearing aids in the opposite ears].

    Science.gov (United States)

    Tian, Yanjing; Zhou, Huifang; Zhang, Jing; Yang, Dong; Xu, Yi; Guo, Yuxi

    2012-10-01

    To compare the effect of rehabilitation of prelingual deaf children who used a cochlear implant (CI) in one ear and a hearing aids in the opposite ear while the hearing level of the opposite ears are different. Hearing ability, language ability and learning ability was included in the content. The aim of this research is to investigate better style of rehabilitation, and to offer the best help to the prelingual deaf children. Accord ing to the hearing level of the ear opposite to the one wearing a cochlear implant and whether the opposite ear wear a hearing aid or not, 30 prelingual deaf children were divided into three groups, including cochlear implant with opposite severe hearing loss and hearing aid ear (CI+SHA), cochlear implant with opposite profound hearing loss and hearing aid ear (CI+PHA), cochlear implant only (CI). The effect of rehabilitation was assessed in six different times (3,6,9,12,15 and 18 months after the cochlear implants and hearing aids began to work). The longer time the rehabilitation spends, the better the hearing ability,language ability and the learning ability were. The hearing ability of CI+SHA was better than those of CI+PHA (Pdeaf children should take much more time on rehabilitation. The effect of rehabilitation for prelingual deaf children who used cochlear implant in one ear and hearing aid in the other depend on the residual hearing level of the other ear. If a prelingual deaf children still has any residual hearing level in the ear opposite to the cochlear implant ear, it is better for him/her to wear a hearing aid in the ear.

  19. Tools Facilitating Communication for the Deaf

    Directory of Open Access Journals (Sweden)

    Luz Magnolia Tilano-Vega

    2014-12-01

    Full Text Available There is a great deal of interest worldwide in serving people with diverse abilities and needs, especially in the field of education. This implies a commitment to inclusion, empowerment and creating opportunities in a range of sectors. The deaf pose a considerable challenge to any proposal for inclusion and social integration, because their difficulty affects communication. The purpose of this study is to contribute to theoretical reflection on the development of communication tools that give the deaf access to education. It is based on a review of studies found in databases, on institutional websites and in journals on disciplines such as teaching, psychology and engineering. The various tools that have been created to help members of the deaf community strengthen the social, educational, recreational and work-related aspects of their lives are listed.

  20. Intubation of Profoundly Agitated Patients Treated with Prehospital Ketamine.

    Science.gov (United States)

    Olives, Travis D; Nystrom, Paul C; Cole, Jon B; Dodd, Kenneth W; Ho, Jeffrey D

    2016-12-01

    Profound agitation in the prehospital setting confers substantial risk to patients and providers. Optimal chemical sedation in this setting remains unclear. The goal of this study was to describe intubation rates among profoundly agitated patients treated with prehospital ketamine and to characterize clinically significant outcomes of a prehospital ketamine protocol. This was a retrospective cohort study of all patients who received prehospital ketamine, per a predefined protocol, for control of profound agitation and who subsequently were transported to an urban Level 1 trauma center from May 1, 2010 through August 31, 2013. Identified records were reviewed for basic ambulance run information, subject characteristics, ketamine dosing, and rate of intubation. Emergency Medical Services (EMS) ambulance run data were matched to hospital-based electronic medical records. Clinically significant outcomes are characterized, including unadjusted and adjusted rates of intubation. Overall, ketamine was administered 227 times in the prehospital setting with 135 cases meeting study criteria of use of ketamine for treatment of agitation. Endotracheal intubation was undertaken for 63% (85/135) of patients, including attempted prehospital intubation in four cases. Male gender and late night arrival were associated with intubation in univariate analyses (χ2=12.02; P=.001 and χ2=5.34; P=.021, respectively). Neither ketamine dose, co-administration of additional sedating medications, nor evidence of ethanol (ETOH) or sympathomimetic ingestion was associated with intubation. The association between intubation and both male gender and late night emergency department (ED) arrival persisted in multivariate analysis. Neither higher dose (>5mg/kg) ketamine nor co-administration of midazolam or haloperidol was associated with intubation in logistic regression modeling of the 120 subjects with weights recorded. Two deaths were observed. Post-hoc analysis of intubation rates suggested a