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Sample records for profound congenital hearing

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

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    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.

  2. Speech perception, production and intelligibility in French-speaking children with profound hearing loss and early cochlear implantation after congenital cytomegalovirus infection.

    Science.gov (United States)

    Laccourreye, L; Ettienne, V; Prang, I; Couloigner, V; Garabedian, E-N; Loundon, N

    2015-12-01

    To analyze speech in children with profound hearing loss following congenital cytomegalovirus (cCMV) infection with cochlear implantation (CI) before the age of 3 years. In a cohort of 15 children with profound hearing loss, speech perception, production and intelligibility were assessed before and 3 years after CI; variables impacting results were explored. Post-CI, median word recognition was 74% on closed-list and 48% on open-list testing; 80% of children acquired speech production; and 60% were intelligible for all listeners or listeners attentive to lip-reading and/or aware of the child's hearing loss. Univariate analysis identified 3 variables (mean post-CI hearing threshold, bilateral vestibular areflexia, and brain abnormality on MRI) with significant negative impact on the development of speech perception, production and intelligibility. CI showed positive impact on hearing and speech in children with post-cCMV profound hearing loss. Our study demonstrated the key role of maximizing post-CI hearing gain. A few children had insufficient progress, especially in case of bilateral vestibular areflexia and/or brain abnormality on MRI. This led us to suggest that balance rehabilitation and speech therapy should be intensified in such cases. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  3. Congenital sensorineural hearing loss

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    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.

  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. 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.

  6. Multisensory Speech Perception by Profoundly Hearing-Impaired Children.

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    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…

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

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    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.

  8. Frequency compression hearing aid for severe-to-profound hearing impairments.

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    Sakamoto, S; Goto, K; Tateno, M; Kaga, K

    2000-10-01

    Objective of this study is to know how a frequency compression hearing aid with new concepts is beneficial for severe-to-profound hearing impairments. (2) Clinical trials of this hearing aid were conducted for 11 severe-to-profound hearing impaired listeners. These 11 wore the frequency compression hearing aid in their daily life and reported subjectively on its performance. Speech recognition tests with five listeners and audio-visual short sentence recognition tests with three listeners were also conducted. This hearing aid can separately adjust the fundamental frequency from the spectral envelope of input speech and can adjust frequency response by use of a post-processing digital filter. (3) Five listeners out of these 11 came to prefer this hearing aid in their daily life and are still wearing it. The results of the speech recognition tests show that the speech recognition scores were not improved for all listeners and the results of the audio-visual short sentence recognition tests do that the audio-visual recognition scores were improved for two listeners. (4) There were some severe-to-profound hearing impaired listeners who preferred the frequency compression hearing aid finally. It is also suggested that the benefits of this hearing aid may be evaluated correctly using not only speech but also visual materials.

  9. Changes in auditory perceptions and cortex resulting from hearing recovery after extended congenital unilateral hearing loss

    Directory of Open Access Journals (Sweden)

    Jill B Firszt

    2013-12-01

    Full Text Available Monaural hearing induces auditory system reorganization. Imbalanced input also degrades time-intensity cues for sound localization and signal segregation for listening in noise. While there have been studies of bilateral auditory deprivation and later hearing restoration (e.g. cochlear implants, less is known about unilateral auditory deprivation and subsequent hearing improvement. We investigated effects of long-term congenital unilateral hearing loss on localization, speech understanding, and cortical organization following hearing recovery. Hearing in the congenitally affected ear of a 41 year old female improved significantly after stapedotomy and reconstruction. Pre-operative hearing threshold levels showed unilateral, mixed, moderately-severe to profound hearing loss. The contralateral ear had hearing threshold levels within normal limits. Testing was completed prior to, and three and nine months after surgery. Measurements were of sound localization with intensity-roved stimuli and speech recognition in various noise conditions. We also evoked magnetic resonance signals with monaural stimulation to the unaffected ear. Activation magnitudes were determined in core, belt, and parabelt auditory cortex regions via an interrupted single event design. Hearing improvement following 40 years of congenital unilateral hearing loss resulted in substantially improved sound localization and speech recognition in noise. Auditory cortex also reorganized. Contralateral auditory cortex responses were increased after hearing recovery and the extent of activated cortex was bilateral, including a greater portion of the posterior superior temporal plane. Thus, prolonged predominant monaural stimulation did not prevent auditory system changes consequent to restored binaural hearing. Results support future research of unilateral auditory deprivation effects and plasticity, with consideration for length of deprivation, age at hearing correction, degree and type

  10. Congenital hearing loss. Is CT enough?

    African Journals Online (AJOL)

    Mahmoud Agha

    2014-01-24

    HRCT) in diag- nosis and management of congenital hearing loss. Patients and methods: This is a prospective study including 60 patients, 24 males and 36 females aged from 1 to 7years, who were presented by unilateral or ...

  11. Cancer Prevention Knowledge of People with Profound Hearing Loss

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    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

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

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    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...

  13. Prelinguistic Vocal Development in Infants with Typical Hearing and Infants with Severe-to-Profound Hearing Loss

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    Iyer, Suneeti Nathani; Oller, D. Kimbrough

    2008-01-01

    Delays in the onset of canonical babbling with hearing loss are extensively documented. Relatively little is known about other aspects of prelinguistic vocal development and hearing loss. Eight infants with typical hearing and eight with severe-to-profound hearing loss were matched with regard to a significant vocal development milestone, the…

  14. Multisensory speech perception of young children with profound hearing loss.

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    Kishon-Rabin, L; Haras, N; Bergman, M

    1997-10-01

    The contribution of a two-channel vibrotactile aid (Trill VTA 2/3, AVR Communications LTD) to the audiovisual perception of speech was evaluated in four young children with profound hearing loss using words and speech pattern contrasts. An intensive, hierarchical, and systematic training program was provided. The results show that the addition of the tactile (T) modality to the auditory and visual (A+V) modalities enhanced speech perception performance significantly on all tests. Specifically, at the end of the training sessions, the tactile supplementation increased word recognition scores in a 44-word, closed-set task by 12 percentage points; detection of consonant in final position by 50 percentage points; detection of sibilant in final position by 30 percentage points; and detection of voicing in final position by 25 percentage points. Significant learning over time was evident for all test materials, in all modalities. As expected, fastest learning (i.e., smallest time constants) was found for the AVT condition. The results of this study provide further evidence that sensory information provided by the tactile modality can enhance speech perception in young children.

  15. 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.

  16. 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

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

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    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.

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

    Directory of Open Access Journals (Sweden)

    Preben Homøe

    2013-08-01

    Full Text Available 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-language pathologists for evaluation, surgery and rehabilitation. In particular, children treated with CI are in need of long-term post-operative auditory training and other follow-up support. Design. The study is retrospective with updated information on present performance. Results. Since 2001, a total of 11 Greenlandic patients living in Greenland have been treated with CI, 7 children and 4 adults. Of these children, 4 use oral communication only and are full-time CI-users, 2 with full-time use of CI are still in progress with use of oral communication, and 1 has not acquired oral language yet, but has started auditory and speech training. Six children attend mainstream public school while one child is in kindergarten. Of the adults, only 1 has achieved good speech perception with full-time use of CI while 3 do not use the CI. Discussion. From an epidemiological point of view, approximately 1–3 children below 6 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 with the auditory and speech/language outcomes. Finally, we present a suggestion for the future CI treatment and recommendations for an increased effort in the

  19. Hearing Loss in Children With Asymptomatic Congenital Cytomegalovirus Infection.

    Science.gov (United States)

    Lanzieri, Tatiana M; Chung, Winnie; Flores, Marily; Blum, Peggy; Caviness, A Chantal; Bialek, Stephanie R; Grosse, Scott D; Miller, Jerry A; Demmler-Harrison, Gail

    2017-03-01

    To assess the prevalence, characteristics, and risk of sensorineural hearing loss (SNHL) in children with congenital cytomegalovirus infection identified through hospital-based newborn screening who were asymptomatic at birth compared with uninfected children. We included 92 case-patients and 51 controls assessed by using auditory brainstem response and behavioral audiometry. We used Kaplan-Meier survival analysis to estimate the prevalence of SNHL, defined as ≥25 dB hearing level at any frequency and Cox proportional hazards regression analyses to compare SNHL risk between groups. At age 18 years, SNHL prevalence was 25% (95% confidence interval [CI]: 17%-36%) among case-patients and 8% (95% CI: 3%-22%) in controls (hazard ratio [HR]: 4.0; 95% CI: 1.2-14.5; P = .02). Among children without SNHL by age 5 years, the risk of delayed-onset SNHL was not significantly greater for case-patients than for controls (HR: 1.6; 95% CI: 0.4-6.1; P = .5). Among case-patients, the risk of delayed-onset SNHL was significantly greater among those with unilateral congenital/early-onset hearing loss than those without (HR: 6.9; 95% CI: 2.5-19.1; P < .01). The prevalence of severe to profound bilateral SNHL among case-patients was 2% (95% CI: 1%-9%). Delayed-onset and progression of SNHL among children with asymptomatic congenital cytomegalovirus infection continued to occur throughout adolescence. However, the risk of developing SNHL after age 5 years among case-patients was not different than in uninfected children. Overall, 2% of case-patients developed SNHL that was severe enough for them to be candidates for cochlear implantation. Copyright © 2017 by the American Academy of Pediatrics.

  20. Congenitally deaf children's care trajectories in the context of universal neonatal hearing screening: a qualitative study of the parental experiences

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    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...

  1. Age of diagnosis for congenital hearing loss at Universitas Hospital ...

    African Journals Online (AJOL)

    the age of diagnosis of congenital hearing loss in the Free State. This study was undertaken to validate our perception that the current age of diagnosis of congenital hearing loss in our hospital was unacceptably high and to provide a benchmark against which improvements in healthcare delivery could be measured.

  2. 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.

  3. Bone-anchored hearing aid system application for unilateral congenital conductive hearing impairment: audiometric results.

    NARCIS (Netherlands)

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

    2008-01-01

    OBJECTIVE: To study the audiologic outcome of bone-anchored hearing aid (BAHA) application in patients with congenital unilateral conductive hearing impairment. STUDY DESIGN: Prospective audiometric evaluation on 20 patients. SETTING: Tertiary referral center. PATIENTS: The experimental group

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

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

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    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)

  6. Localization training results in individuals with unilateral severe to profound hearing loss.

    Science.gov (United States)

    Firszt, Jill B; Reeder, Ruth M; Dwyer, Noël Y; Burton, Harold; Holden, Laura K

    2015-01-01

    Adults with unilateral hearing loss often demonstrate decreased sound localization ability and report that situations requiring spatial hearing are especially challenging. Few studies have evaluated localization abilities combined with training in this population. The present pilot study examined whether localization of two sound types would improve after training, and explored the relation between localization ability or training benefit and demographic factors. Eleven participants with unilateral severe to profound hearing loss attended five training sessions; localization cues gradually decreased across sessions. Localization ability was assessed pre- and post-training. Assessment stimuli were monosyllabic words and spectral and temporal random spectrogram sounds. Root mean square errors for each participant and stimulus type were used in group and correlation analyses; individual data were examined with ordinary least squares regression. Mean pre-to post-training test results were significantly different for all stimulus types. Among the participants, eight significantly improved following training on at least one localization measure, whereas three did not. Participants with the poorest localization ability improved the most and likewise, those with the best pre-training ability showed the least training benefit. Correlation results suggested that test age, age at onset of severe to profound hearing loss and better ear high frequency audibility may contribute to localization ability. Results support the need for continued investigation of localization training efficacy and consideration of localization training within rehabilitation protocols for individuals with unilateral severe to profound hearing loss. Copyright © 2014 Elsevier B.V. All rights reserved.

  7. Preferred low- and high-frequency compression ratios among hearing aid users with moderately severe to profound hearing loss.

    Science.gov (United States)

    Keidser, Gitte; Dillon, Harvey; Dyrlund, Ole; Carter, Lyndal; Hartley, David

    2007-01-01

    This study aimed to determine the low- and high-frequency compression ratios of a fast-acting device that were preferred by people with moderately severe to profound hearing loss. Three compression ratios (1:1, 1.8:1, and 3:1) were combined in the low and high frequencies to produce nine schemes that were evaluated pair-wise for three weeks in the field using an adaptive procedure. The evaluation was performed by 21 experienced hearing aid users with a moderately severe to profound hearing loss. Diaries and an exit interview were used to monitor preferences. Generally, the subjects preferred lower compression ratios than are typically prescribed, especially in the low frequencies. Specifically, 11 subjects preferred linear amplification in the low frequencies, and 14 subjects preferred more compression in the high than in the low frequencies. Preferences could not be predicted from audiometric data, onset of loss, or past experience with amplification. The data suggest that clients with moderately severe to profound hearing loss should be fitted with low-frequency compression ratios in the range 1:1 to 2:1 and that fine-tuning is essential.

  8. Hearing loss in congenital toxoplasmosis detected by newborn screening.

    Science.gov (United States)

    Andrade, Gláucia Manzan Queiroz de; Resende, Luciana Macedo de; Goulart, Eugênio Marcos Andrade; Siqueira, Arminda Lucia; Vitor, Ricardo Wagner de Almeida; Januario, José Nelio

    2008-01-01

    Congenital toxoplasmosis may cause sensorineural deficit in up to 20% of the patients and proper treatment in the first year improves prognosis. In Brazil, this infections impact on hearing impairment is unknown. To evaluate hearing of newborns with congenital toxoplasmosis identified by the newborn screening service. This prospective study analyzed children with congenital toxoplasmosis identified by newborn screening (IgM anti-T.gondii) in Belo Horizonte during 2003/2004. The presence of IgM and/or IgA in the first 6 months or IgG at 12 months-of age in serology was used as case definition. Hearing tests were carried out at the time of diagnosis and 12 months later, including behavioral audiometry, evoked otoacoustic emission and brainstem evoked responses audiometry. Among 30.808 screened children (97% of live births), 20 had congenital toxoplasmosis, 15 (75%) were asymptomatic at birth. Nineteen children were evaluated by hearing tests. Four had sensorineural impairment (21.1%). One child had other risk factors for hearing impairment; the other three had no other risk factors but toxoplasmosis. Two properly children treated still had hearing loss, in disagreement with current literature. Results suggest that congenital toxoplasmosis, common in Brazil, is a risk factor for hearing impairment and its impact on hearing loss deserves further studies.

  9. 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

  10. Changes in auditory perceptions and cortex resulting from hearing recovery after extended congenital unilateral hearing loss

    OpenAIRE

    Firszt, Jill B.; Reeder, Ruth M.; Holden, Timothy A.; Harold eBurton; Chole, Richard A.

    2013-01-01

    Monaural hearing induces auditory system reorganization. Imbalanced input also degrades time-intensity cues for sound localization and signal segregation for listening in noise. While there have been studies of bilateral auditory deprivation and later hearing restoration (e.g. cochlear implants), less is known about unilateral auditory deprivation and subsequent hearing improvement. We investigated effects of long-term congenital unilateral hearing loss on localization, speech understanding, ...

  11. Profound mixed hearing loss: bone-anchored hearing aid system or cochlear implant?

    Science.gov (United States)

    Verhaegen, Veronique J O; Mulder, Jef J S; Mylanus, Emmanuel A M; Cremers, Cor W R J; Snik, Ad F M

    2009-10-01

    We evaluated 5 patients who were changed over from a Baha Cordelle to a cochlear implant (CI). Moreover, the level of phoneme score was determined at which a Baha Cordelle user was better helped with a CI. We offer descriptive case reports and a retrospective evaluation of speech recognition in Baha Cordelle users and CI users. In the CI users with noncompromised cochleas, the 10th percentile of the aided phoneme score in quiet at 65 dB sound pressure level (PS65) was 42%. We consider this PS65 as the cutoff level for switching from a Baha Cordelle to a CI. When patients with mixed hearing loss were using the Baha Cordelle, the PS65 of 42% was obtained at a mean sensorineural hearing loss component of about 70 dB hearing level (HL). This 70-dB HL component was used to consider Baha Cordelle users for cochlear implantation. The results of the 5 patients support these transition criteria. A CI is a valuable option in patients with mixed hearing loss when the sensorineural hearing loss component exceeds 70 dB HL or when the PS65 with a Baha Cordelle is less than about 40%. In such patients, the implantation procedure should be individualized on the basis of the clinical findings in the middle ear and mastoid cavity.

  12. 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.

  13. 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.

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

    Science.gov (United States)

    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

  15. 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.

  16. Associations and Dissociations between Psychoacoustic Abilities and Speech Perception in Adolescents with Severe-to-Profound Hearing Loss

    Science.gov (United States)

    Kishon-Rabin, Liat; Segal, Osnat; Algom, Daniel

    2009-01-01

    Purpose: To clarify the relationship between psychoacoustic capabilities and speech perception in adolescents with severe-to-profound hearing loss (SPHL). Method: Twenty-four adolescents with SPHL and young adults with normal hearing were assessed with psychoacoustic and speech perception tests. The psychoacoustic tests included gap detection…

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  19. Binaural auditory outcomes in patients with postlingual profound unilateral hearing loss: 3 years after cochlear implantation.

    Science.gov (United States)

    Mertens, Griet; Kleine Punte, Andrea; De Bodt, Marc; Van de Heyning, Paul

    2015-01-01

    The value of cochlear implants (CI) in patients with profound unilateral hearing loss (UHL) and tinnitus has recently been investigated. The authors previously demonstrated the feasibility of CI in a 12- month outcome study in a prospective UHL cohort. The aim of this study was to investigate the binaural auditory outcomes in this cohort 36 months after CI surgery. The 36-month outcome was evaluated in 22 CI users with postlingual UHL and severe tinnitus. Twelve subjects had contralateral normal hearing (single-sided deafness - SSD group) and 10 subjects had a contralateral, mild to moderate hearing loss and used a hearing aid (asymmetric hearing loss - AHL group). Speech perception in noise was assessed in two listening conditions: the CIoff and the CIon condition. The binaural summation effect (S0N0), binaural squelch effect (S0NCI) and the combined head shadow effect (SCIN0) were investigated. Subjective benefit in daily life was assessed by means of the Speech, Spatial and Qualities of Hearing Scale (SSQ). At 36 months, a significant binaural summation effect was observed for the study cohort (2.00, SD 3.82 dB; p binaural effect was not significant 12 months after CI surgery. A binaural squelch effect was significant for the AHL subgroup at 12 months (2.00, SD 4.38 dB; p < 0.05). A significant combined head shadow and squelch effect was also noted in the spatial configuration SCIN0 for the study cohort (4.00, SD 5.89 dB; p < 0.01) and for the AHL subgroup (5.67, SD 6.66 dB; p < 0.05). The SSQ data show that the perceived benefit in daily life after CI surgery remains stable up to 36 months at CIon. CI can significantly improve speech perception in noise in patients with UHL. The positive effects of CIon speech perception in noise increase over time up to 36 months after CI surgery. Improved subjective benefit in daily life was also shown to be sustained in these patients. © 2015 S. Karger AG, Basel.

  20. The experience of black parents/caretakers with the births and care of a child with profound congenital defects

    Directory of Open Access Journals (Sweden)

    M.S. Mabaso

    1990-09-01

    Full Text Available The reaction of Black families to the birth and care of a baby with profound congenital defects was researched using twenty case studies. It was found that the families went through stages of the grieving process, that they shifted from the Western/Christian viewpoint to the traditional viewpoint in their struggle to cope and that they find the existing services grossly inadequate.

  1. The Effect of Frequency Transposition on Speech Perception in Adolescents and Young Adults with Profound Hearing Loss

    Science.gov (United States)

    Gou, J.; Smith, J.; Valero, J.; Rubio, I.

    2011-01-01

    This paper reports on a clinical trial evaluating outcomes of a frequency-lowering technique for adolescents and young adults with severe to profound hearing impairment. Outcomes were defined by changes in aided thresholds, speech perception, and acceptance. The participants comprised seven young people aged between 13 and 25 years. They were…

  2. Murine model for congenital CMV infection and hearing impairment

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    Tao Liu

    2011-02-01

    Full Text Available Abstract Background Congenital cytomegalovirus (CMV infection is the leading cause of sensorineural hearing loss (SNHL, and SNHL is the most frequent sequela of congenital CMV infection. But the pathogenic mechanism remains unknown, and there is no ideal CMV intrauterine infection animal model to study the mechanisms by which SNHL develops. Methods We established the congenital murine cytomegalovirus (MCMV infection model by directly injecting the virus into the placenta on day 12.5 of gestation. Then, we observed the development and the MCMV congenital infection rate of the fetuses on the day they were born. Furthermore, we detected the auditory functions, the conditions of the MCMV infection, and the histological change of the inner ears of 28-day-old and 70-day-old offspring. Results Both the fetal loss rate and the teratism rate of offspring whose placentas were inoculated with MCMV increased, and their body length, head circumference, and weight decreased. The hearing level of offspring both decreased at both 28- and 70-days post birth; the 70-day-old mice developed lower hearing levels than did the 28-day old mice. No significant inflammatory changes in the cochleae of the mice were observed. MCMV DNA signals were mainly detected in the spiral ganglion neurons and the endolymph area, but not in the perilymph area. The number of neurons decreased, and their ultrastructures changed. Moreover, with age, the number of neurons dramatically decreased, and the ultrastructural lesions of neurons became much more severe. Conclusions The results suggest that the direct injection of MCMV into the placenta may efficiently cause fetal infection and disturb the intrauterine development of the fetus, and placental inoculation itself has no obvious adverse effects on offspring. The reduction in the number of spiral ganglion neurons and the ultrastructural lesions of the neurons may be the major cause of congenital CMV infection-induced progressive SNHL.

  3. 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

  4. SPEECH INTELLIGIBILITY DEVELOPMENT IN SEVERE TO PROFOUND HEARING-IMPAIRED CHILDREN ESTABLISHMENT OF A DATA COLLECTION FOR EARLY INTERVENTION IN HEARING-IMPAIRED CHILDREN IN IRAN

    Directory of Open Access Journals (Sweden)

    N. Daneshman P. Borghei

    2007-05-01

    Full Text Available The main goal of early detection of hearing impairment in children is early intervention. There is growing interest in early detection of hearing impairment in developing countries. The main purpose of this study was to investigate the spoken language development in severe to profound hearing impaired children and compared their speech intelligibility with normal hearing children at the same age. Nine severe to profound hearing impaired children below 2 years old out of the primer 42 cases were selected for this survey. They receive aural habilitation and also speech therapy after beginning the speech production. Speech intelligibility test of these children was recorded on audio-tape, when they read five questions which can be answered with one word only, at the age of 4, 5 and 6 in comparison with 27 normal hearing children at the same age. At the age of 4 the mean speech intelligibility score of the studied group was 31.77% (SD 12.17 and the control was %96 (SD 2.23. At the age of 5, this score was %51.22 (SD 14.42, the control one 97.85% (SD 1.93. Finally at age 6 it was 72% (SD 18.97 for hearing–impaired group and 99.22% (SD 1.18 in control one. Severe to profound hearing impaired children acquired spoken language but not at the same level. In general, their speech development showed about 2 to 3 years delay. Their speech intelligibility was acceptable for severe group around the age 6 but almost semi–intelligible for profound group at the same age.

  5. Comparison of Reading Comprehension Skill of Students with Severe to Profound Hearing Impairment from Second up to Fifth Grade of Exceptional Schools with Normal Hearing Students

    Directory of Open Access Journals (Sweden)

    Maryam Jalalipour

    2016-03-01

    Full Text Available Background: Reading is known as one of the most important learning tools. Research results consistently have shown that even a mild hearing impairment could affect the reading skills. Due to the reported differences in reading comprehension skills between hearing impaired students and their normal hearing peers, this research was conducted to compare the differences between the two groups. The other aim was to find any changes in the reading ability of hearing impaired group during elementary school. Methods: This study is a cross-sectional (descriptive–analytic one in which reading comprehension ability of 91 students with severe and profound hearing impairment (33 girls and 58 boys from 2nd up to 5th grade of exceptional schools were compared with 50 2nd grade normal hearing students in Ahvaz, Iran. The first section of Diagnostic Reading Test (Shirazi – Nilipour, 2004 was used in this study. Then the mean reading scores of hearing impaired students in each grade was compared with control group using SPSS 13 with Mann Whitney test. Results: There was a significant difference between average scores of hearing impaired students (boys and girls in 2nd to 5th grade with normal hearing students of 2nd grade (P<0.001. Reading comprehension scores of students with hearing impairment in higher grades had improved slightly, but it was still lower than that of the normal hearing students in the 2nd grade. Conclusion: It appears that reading comprehension skill of students with significant hearing impairment near the end of elementary school years becomes weaker than normal hearing students in the second grade. Therefore, it is essential to find and resolve the underlying reasons of this condition by all professionals who work in the field of education and rehabilitation of these students.

  6. Effects of early identification and intervention on language development in Japanese children with prelingual severe to profound hearing impairment.

    Science.gov (United States)

    Kasai, Norio; Fukushima, Kunihiro; Omori, Kana; Sugaya, Akiko; Ojima, Toshiyuki

    2012-04-01

    Early identification and intervention for prelingual bilateral severe to profound hearing loss is supposed to reduce the delay in language development. Many countries have implemented early detection and hearing intervention and conducted regional universal newborn hearing screening (UNHS). However, the benefits of UNHS in later childhood have not yet been confirmed, although language development at school age has a lifelong impact on children's future. Our Research on Sensory and Communicative Disorders project attempted to reveal the effects of UNHS and those of early intervention on the development of verbal communication in Japanese children. In this study, 319 children with prelingual bilateral severe to profound hearing loss, 4 to 10 years of age, were evaluated with the Test of Question-Answer Interaction Development used as an objective variable. Participation in UNHS and early intervention were used as explanatory variables. The adjusted odds ratio (AOR) was calculated after adjusting several confounding factors with use of logistic regression analysis. In addition, caregivers' answers were obtained by a questionnaire, and the process of diagnosis with and without UNHS was analyzed retrospectively. Early intervention was significantly associated with better language development (AOR, 3.23; p identification leads directly to early intervention.

  7. Subjective benefit after BAHA system application in patients with congenital unilateral conductive hearing impairment.

    NARCIS (Netherlands)

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

    2008-01-01

    OBJECTIVE: To study whether unilateral Bone-anchored Hearing Aid (BAHA) fitting led to subjective hearing benefit in patients with congenital unilateral conductive hearing impairment. STUDY DESIGN: Prospective evaluation on 20 patients. SETTING: Tertiary referral center. PATIENTS: Ten adults and 10

  8. 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...

  9. [Audiologic and molecular screening for hearing loss by 35delG mutation in connexin 26 gene and congenital cytomegalovirus infection].

    Science.gov (United States)

    Streitenberger, Edgardo Raúl; Suárez, Ariel Ignacio; Masciovecchio, María Verónica; Laurnagaray, Diana; Alda, Ernesto

    2011-12-01

    Hearing loss may be attributed to genetic and environmental factors. Mutations in the gene of the CX26 protein (connexin 26), are responsible for 30-80% of all cases of non-syndromic profound hearing loss. The 35delG is the most frequent variant in the caucasian population. As to environmental factors, the cytomegalovirus (CMV) is the main cause of congenital infection. To determine the prevalence of congenital CMV infection and the frequency of the 35delG mutation in newborns. To identify those at risk of suffering hearing loss in order to do an audiologic follow-up of detected cases. One thousand and twenty samples of dry blood spots corresponding to newborns were tested using conventional and real time PCR. Audiologic screening was performed to all newborns before hospital discharge. Fifteen out of 1020 subjects were heterozygous for the mutation. No homozygous patients were found. Six out of the samples tested positive for CMV (confirmed by a urine sample), out of which only one newborn was symptomatic. The auditory brainstem response was recorded in all these children. Hearing loss was found in three children with congenital CMV infection and two with 35delG mutation. The frecuency of 35delG mutation carriers in our population was 1.3% and the CMV congenital infection prevalence was 0.6%. Audiologic monitoring of these two populations allowed detection of hearing loss of late onset.

  10. Describing the trajectory of language development in the presence of severe-to-profound hearing loss: a closer look at children with cochlear implants versus hearing aids.

    Science.gov (United States)

    Yoshinaga-Itano, Christine; Baca, Rosalinda L; Sedey, Allison L

    2010-10-01

    The objective of this investigation was to describe the language growth of children with severe or profound hearing loss with cochlear implants versus those children with the same degree of hearing loss using hearing aids. A prospective longitudinal observation and analysis. University of Colorado Department of Speech Language and Hearing Sciences. There were 87 children with severe-to-profound hearing loss from 48 to 87 months of age. All children received early intervention services through the Colorado Home Intervention Program. Most children received intervention services from a certified auditory-verbal therapist or an auditory-oral therapist and weekly sign language instruction from an instructor who was deaf or hard of hearing and native or fluent in American Sign Language. The Test of Auditory Comprehension of Language, 3rd Edition, and the Expressive One Word Picture Vocabulary Test, 3rd Edition, were the assessment tools for children 4 to 7 years of age. The expressive language subscale of the Minnesota Child Development was used in the infant/toddler period (birth to 36 mo). Average language estimates at 84 months of age were nearly identical to the normative sample for receptive language and 7 months delayed for expressive vocabulary. Children demonstrated a mean rate of growth from 4 years through 7 years on these 2 assessments that was equivalent to their normal-hearing peers. As a group, children with hearing aids deviated more from the age equivalent trajectory on the Test of Auditory Comprehension of Language, 3rd Edition, and the Expressive One Word Picture Vocabulary Test, 3rd Edition, than children with cochlear implants. When a subset of children were divided into performance categories, we found that children with cochlear implants were more likely to be "gap closers" and less likely to be "gap openers," whereas the reverse was true for the children with hearing aids for both measures. Children who are educated through oral-aural combined with

  11. 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.

  12. Overview of Usher's Syndrome: Congenital Deafness and Progressive Loss of Vision

    Science.gov (United States)

    Vernon, McCay

    1974-01-01

    Usher's syndrome, a genetic condition causing congenital profound hearing loss and a progressive blindness due to retinitis pigmentosa, affects an estimated three to six percent of children in educational and rehabilitative programs for the hearing impaired. (Author)

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

    Science.gov (United States)

    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…

  14. 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.

  15. 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.

  16. The prevalence of congenital hearing loss in neonates with Down syndrome.

    Science.gov (United States)

    Tedeschi, Adrienne S; Roizen, Nancy J; Taylor, H Gerry; Murray, Gail; Curtis, Christine A; Parikh, Aditi Shah

    2015-01-01

    To determine the prevalence of hearing loss in newborns with Down syndrome. We performed a cross-sectional, retrospective chart review of all infants with Down syndrome born at a university-affiliated hospital (n = 77) or transferred in to the associated pediatric hospital (n = 32) following birth at an outlying hospital between 1995 and 2010. We determined the rate of failure of newborn hearing screens, the proportion of infants lost to follow-up, and the rate of confirmed hearing loss, as well as the associations of risk factors for hearing loss with confirmed hearing loss. Of the 109 patients with hearing screening data, 28 failed their newborn hearing screen. Twenty-seven infants were referred for audiologic evaluation, and 19 completed the evaluation. Fifteen of these 19 infants (79%) had confirmed hearing loss. The prevalence of congenital hearing loss in this sample of neonates with Down syndrome was 15%. Exposure to mechanical ventilation was the sole known risk factor associated with hearing loss. In this study, the loss to follow-up rate for infants with positive hearing screens was 32%. Newborns with Down syndrome have a higher prevalence of congenital hearing loss compared with the total neonatal population (15% vs 0.25%). Continued monitoring of hearing is needed in children with Down syndrome. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. The role of bone conduction hearing aids in congenital unilateral hearing loss: A systematic review.

    Science.gov (United States)

    Liu, C Carrie; Livingstone, Devon; Yunker, Warren K

    2017-03-01

    To systematically review the literature on the audiological and/or quality of life benefits of a bone conduction hearing aid (BCHA) in children with congenital unilateral conductive or sensorineural deafness. A systematic search was performed according to the PRISMA guidelines using the PubMed, Medline, and Embase databases. Data were collected on the following outcomes of interest: speech reception threshold, speech discrimination, sound localization, and quality of life measures. Given the heterogeneity of the data for quantitative analysis, the results are qualitatively summarized. Eight studies were included in the review. Four studies examined the audiological outcomes associated with bone conduction hearing aid implantation. There was a consistent gain in speech reception thresholds and speech discrimination, especially in noisy environments. Results pertaining to sound localization was inconsistent. The studies that examined quality of life measures reported a high usage rate of BCHAs among children. Quality of life improvements are reported with suggested benefit in the subdomain of learning. Given the potential benefits of a BCHA, along with the fact that it can be safely trialed using a headband, it is reasonable to trial a BCHA in children with congenital unilateral deafness. Should the trial offer audiological and/or quality of life benefits for the individual child, then BCHA implantation can be considered. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. 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

  19. Are Auditory Steady-State Responses Useful to Evaluate Severe-to-Profound Hearing Loss in Children?

    Directory of Open Access Journals (Sweden)

    Signe Schuster Grasel

    2015-01-01

    Full Text Available Objective. To evaluate Auditory Steady-State Responses (ASSR at high intensities in pediatric cochlear implant candidates and to compare the results to behavioral tests responses. Methods. This prospective study evaluated 42 children with suspected severe-to-profound hearing loss, aged from 3 to 72 months. All had absent ABR and OAE responses. ASSR were evoked using binaural single frequency stimuli at 110 dB HL with a 10 dB down-seeking procedure. ASSR and behavioral test results were compared. Results. Forty-two subjects completed both ASSR and behavioral evaluation. Eleven children (26.2% had bilateral responses. Four (9.5% showed unilateral responses in at least two frequencies, all confirmed by behavioral results. Overall 61 ASSR responses were obtained, most (37.7% in 500 Hz. Mean thresholds were between 101.3 and 104.2 dB HL. Among 27 subjects with absent ASSR, fifteen had no behavioral responses. Seven subjects showed behavioral responses with absent ASSR responses. No spurious ASSR responses were observed at 100 or 110 dB HL. Conclusion. ASSR is a valuable tool to detect residual hearing. No false-positive ASSR results were observed among 42 children, but in seven cases with absent ASSR, the test underestimated residual hearing as compared to the behavioral responses.

  20. Effectiveness of videos improving cancer prevention knowledge in people with profound hearing loss.

    Science.gov (United States)

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

    2012-06-01

    Deaf persons have a poorer understanding of cancer prevention, which is felt to be partly due to communication barriers. One hundred ninety-seven d/Deaf persons completed a survey and video on cancer prevention. Half viewed a spoken English program designed for hearing persons (control group); the other half viewed an amended program that had American Sign Language, captions, and printed English options added (experimental group). Knowledge was measured before and after the video, including 1 and 6 months later. Respondents were primarily Caucasian, had low incomes, lost hearing at young ages, and had d/Deaf spouses. Although overall knowledge improved after viewing the video, the presence of culture-specific communications (American Sign Language, captions) did not improve scores compared to the control group, either immediately after the intervention or over time. Moreover, percentage correct on all pretest, and almost all post-test, questions was <50% for both experimental and control groups. For all subjects, regardless of which group they were in, a hearing spouse (p  < 0.001) and more healthcare information sources (p = 0.001) improved knowledge, while African-Americans showed a trend to lesser improvement (p = 0.06). Using culture-specific language did not improve cancer prevention knowledge in this d/Deaf population, and overall knowledge remained low. More study is needed to determine the best way to increase cancer prevention knowledge in this population.

  1. Subjective benefit after BAHA system application in patients with congenital unilateral conductive hearing impairment.

    Science.gov (United States)

    Kunst, Sylvia J W; Hol, Myrthe K S; Mylanus, Emmanuel A M; Leijendeckers, Joop M; Snik, Ad F M; Cremers, Cor W R J

    2008-04-01

    To study whether unilateral Bone-anchored Hearing Aid (BAHA) fitting led to subjective hearing benefit in patients with congenital unilateral conductive hearing impairment. Prospective evaluation on 20 patients. Tertiary referral center. Ten adults and 10 children with congenital unilateral conductive hearing impairment, with a mean air-bone gap of 50 dB, were included. Subjective bilateral hearing benefit after BAHA fitting was measured using 2 disability-specific questionnaires: Chung and Stephens and the Speech, Spatial and Qualities of hearing profile (children's version in the patients aged BAHA fitting. Chung and Stephens' questionnaire showed an overall preference for the BAHA in several specific hearing situations. The Glasgow children's benefit inventory demonstrated an overall mean improvement of +34, which was the most prominent in the learning domain. The 10 adults showed an already good score on the Speech, Spatial and Qualities of hearing scale in the unaided situation. The BAHA was well accepted by most of the patients with congenital unilateral conductive hearing impairment. A preoperative trial of the BAHA system with the BAHA on a headband is part of the preoperative procedure. In children with unilateral conductive hearing loss, with regard to possible childs' development and communication difficulties, intervention with BAHA can be considered as an option.

  2. Long-term effects of congenital hearing impairment on language performance in adults

    NARCIS (Netherlands)

    Huysmans, E.; de Jong, J.; van Lanschot-Wery, J.H.; Festen, J.M.; Goverts, S.T.

    2014-01-01

    Aim The aim of this study was to examine long-term effects of moderate to severe congenital hearing impairment (MSCHI) at an age when language development is completed, i.e., in adults. Method We studied language performance in Dutch in 10 normal-hearing (NH) adults and 20 adults with MSCHI, using

  3. Congenital CMV infection: prevalence in newborns and the impact on hearing deficit.

    Science.gov (United States)

    Engman, Mona-Lisa; Malm, Gunilla; Engstrom, Lotta; Petersson, Karin; Karltorp, Eva; Tear Fahnehjelm, Kristina; Uhlen, Inger; Guthenberg, Claes; Lewensohn-Fuchs, Ilona

    2008-01-01

    Congenital cytomegalovirus (CMV) infection is asymptomatic in 90% of infected newborns but approximately 10-20% of these infants are at risk of developing sequelae later, mostly hearing deficit. The aims of the study were to investigate the prevalence of congenital CMV infection in a Swedish population of newborns and investigate the relative risk of hearing deficit in newborns with congenital CMV infection. The dried blood spot (DBS) samples of 6060 newborns in southern Stockholm during 12 months (October 2003-June 2004; August 2004-October 2004) were analysed for CMV DNA by TaqMan based real-time PCR. Hearing deficit was assessed by otoacoustic emission (OAE) within a newborn screening programme. 12 infants out of 6060 or 0.2% (95% CI 0.1-0.3%) had congenital CMV infection. One boy among the 12 infected infants had unilateral hearing loss, indicating that the risk of hearing loss is greatly increased (about 20 times) in CMV infected infants. No child developed ocular complications such as chorioretinopathy during 3 y of follow-up. Congenital CMV has an impact on child health but can easily be overlooked due to lack of signs in the neonatal period. Surveillance for congenital CMV is important in addition to programmes for prevention and treatment.

  4. New speech tests reveal benefit of wide-dynamic-range, fast-acting compression for consonant discrimination in children with moderate-to-profound hearing loss.

    Science.gov (United States)

    Marriage, Josephine E; Moore, Brian C J

    2003-10-01

    Fast-acting, wide-dynamic-range compression (WDRC) has been shown to give better discrimination of soft speech and shouted speech than linear amplification for moderately hearing-impaired young adults. For severe and profound hearing losses, higher compression ratios are needed. The resultant distortion of the temporal envelope and reduced modulation depth may offset improvements in audibility offered by WDRC. This study compares the effectiveness of WDRC and linear amplification for children with different degrees of hearing loss. Pre-recorded tests of closed-set consonant confusions and open-set word recognition were developed to assess performance. Three groups of subjects (aged 4-14 years) with moderate (51-70 dB), severe (71-90 dB) and profound (91-115 dB) hearing loss were fitted with hearing aids programmed with WDRC or linear amplification. The frequency response was adjusted to match each child's own hearing aid prescription. For each group, stimuli were presented both in quiet and in noise at levels chosen to avoid floor and ceiling effects. Consonant confusion scores for the profound and severe groups combined and for the moderate group were significantly better with WDRC than with linear amplification. Open-set test results showed greater variability. Although mean scores were higher for WDRC than for linear processing, the effects were of marginal statistical significance.

  5. 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.

  6. Hearing rehabilitation in congenital aural atresia using the bone-anchored hearing aid: audiological and satisfaction results.

    Science.gov (United States)

    Fuchsmann, Carine; Tringali, Stéphane; Disant, François; Buiret, Guillaume; Dubreuil, Christian; Froehlich, Patrick; Truy, Eric

    2010-12-01

    The BAHA (bone-anchored hearing aid) remains the most reliable method of auditory rehabilitation for patients with congenital aural atresia and improves hearing outcome and quality of life in these patients who are otherwise unable to benefit from traditional hearing aids. New techniques are being developed, such as fully implantable hearing devices, and will have to prove their efficacy and safety in this indication. To evaluate the audiological results and patient satisfaction after rehabilitation of congenital aural atresia using the BAHA. Sixteen patients suffering from bilateral congenital aural atresia were implanted with unilateral BAHA. Preoperative air- and bone-conduction thresholds and air-bone gap (ABG), preoperative sound reception threshold (SRT), postoperative aided thresholds, implantation complications, patient satisfaction, and the APHAB questionnaire were collected. After a mean follow-up period of 6.5 years, 15 patients were still using their device. Each patient had an average improvement of 33 ± 7 dB. Closure of the ABG within 15 dB of the preoperative bone-conduction thresholds occurred in 10 patients. Adverse skin reactions appeared in 50% of patients over 6.5 years of follow-up. Eleven of the 12 patients used their BAHA for more than 8 h per day.

  7. Hearing Screening in a School for the Severely-Profoundly Intellectually-Impaired and Multiply-Handicapped Students.

    Science.gov (United States)

    Yaffe, Linda

    1981-01-01

    Results were considered significant in that 11 percent of the students screened were identified as potentially hearing impaired as compared to 4 percent of the regular Baltimore City School student population who failed hearing screenings. (Author)

  8. A Comparison between Morphological and Syntactic Features of 4 to 5 Years Old in Education Severe to Profound Hearing Impaired and Normal Children

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    Leila Golpour

    2007-12-01

    Full Text Available Background and Aim: Learning Language is a skill which is acquired in early childhood. So, language gradually developed and new words and new structures slowly added to language knowledge. Hearing sense is the most important acquisition for of language and hearing disorder is a barrier for natural language acquiring .The purpose of this study is comparison between morphological and syntactic features of 4 to 5 years old severe to profound hearing impaired and normal children. Materials and Method: This cross-sectional study performed on 10 normal-hearing children with mean age of 4-5, from Gazvin kindergartens and 10 hearing impaired children with similar IQ and age from Nioosha Rehabilitation Center. The language and non language information was received by spontaneous and descriptive speech, and questionnaire, respectively and for comparing syntax comprehension, Specific language impairment test was used. Then these results were compared between two groups. Results: Difference between spontaneous speech and descriptive speech in hearing impaired child is just like normal child. These differences are that the number of utterance, the mean of lexical morpheme, functional morpheme in spontaneous speech is greater than descriptive speech but the mean length of utterance and richness of vocabulary in descriptive speech is greater than spontaneous speech. Mean of lexical morpheme, functional morpheme and richness of vocabulary related to morphological part and the number of utterance, the mean length of utterance and syntax comprehension related to syntax, in spontaneous and descriptive speech of normal children speech is greater than hearing impaireds`. Conclusion: According to recent researches, compared with normal child, the hearing impaired child nearly never to reach equal level, and for this reason, training for this group is necessary. It is concluded that although these children have severe to profound hearing loss they are developing their

  9. Neuroanatomical differences in visual, motor, and language cortices between congenitally deaf signers, hearing signers, and hearing non-signers

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    John S. Allen

    2013-08-01

    Full Text Available We investigated effects of sign language use and auditory deprivation from birth on the volumes of three cortical regions of the human brain: the visual cortex surrounding the calcarine sulcus in the occipital lobe; the language-related cortex in the inferior frontal gyrus (pars triangularis and pars opercularis; and the motor hand region in the precentral gyrus. The study included 25 congenitally deaf participants and 41 hearing participants (of which 16 were native sign language users; all were right-handed. Deaf participants exhibited a larger calcarine volume than hearing participants, which we interpret as the likely result of cross-modal compensation and/or dynamic interactions within sensory neural networks. Deaf participants also had increased volumes of the pars triangularis bilaterally compared to hearing signers and non-signers, which we interpret is related to the increased linguistic demands of speech processing and/or text reading for deaf individuals. Finally, although no statistically significant differences were found in the motor hand region for any of the groups, the deaf group was leftward asymmetric, the hearing signers essentially symmetric and the hearing non-signers were rightward asymmetric – results we interpret as the possible result of activity-dependent change due to life-long signing. The brain differences we observed in visual, motor, and language related areas in adult deaf native signers provide evidence for the plasticity available for cognitive adaptation to varied environments during development.

  10. A Narrow Internal Auditory Canal with Duplication in a Patient with Congenital Sensorineural Hearing Loss

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    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. Factors influencing speech and language outcomes of children with early identified severe/profound hearing loss: Clinician-identified facilitators and barriers.

    Science.gov (United States)

    Fulcher, Anne Nivelles; Purcell, Alison; Baker, Elise; Munro, Natalie

    2015-06-01

    Early identification of severe/profound childhood hearing loss (HL) gives these children access to hearing devices and early intervention to facilitate improved speech and language outcomes. Predicting which infants will go on to achieve such outcomes remains difficult. This study describes clinician identified malleable and non-malleable factors that may influence speech and language outcomes for children with severe/profound HL. Semi-structured interviews were conducted with six experienced auditory verbal clinicians. A collective case study design was implemented. The interviews were transcribed and coded into themes using constant comparative analysis. Clinicians identified that, for children with severe/profound HL, early identification, early amplification and commencing auditory-verbal intervention under 6 months of age may facilitate child progress. Possible barriers were living in rural/remote areas, the clinicians' lack of experience and confidence in providing intervention for infants under age 6-months and belonging to a family with a culturally and linguistically diverse (CALD) background. The results indicate that multiple factors need to be considered by clinicians working with children with HL and their families to determine how each child functions within their own environment and personal contexts, consistent with the International Classification of Functioning, Disability and Health (ICF) framework. Such an approach is likely to empower clinicians to carefully balance potential barriers to, and facilitators of, optimal speech and language outcomes for all children with HL.

  12. Early language development in children with profound hearing loss fitted with a device at a young age: part II--content of the first lexicon.

    Science.gov (United States)

    Nott, Pauline; Cowan, Robert; Brown, P Margaret; Wigglesworth, Gillian

    2009-10-01

    Lexical content is commonly understood to refer to the various categories of words that children produce and has been studied extensively in children with normal hearing. Unlike the hearing child, however, little is known about the word categories that make up the first lexicon of children with hearing loss (HL). Knowledge of the first lexicon is increasingly important, as infants with HL are now being detected through universal newborn hearing screening programs and fitted with hearing aids and cochlear implants in before 12 months of age. For these children, emergence of the first spoken words is a major milestone eagerly awaited by parents and one of the first verbal language goals of teachers and therapists working with such children. The purpose of this study was to evaluate the lexical content of the first 50 and 100 words produced by children with HL and to contrast this with that of a group of hearing children. Lexical content was compared in two groups of children: one group composed of 24 participants with severe profound or profound HL and a second group composed of 16 participants with normal hearing. Twenty-three participants in the HL group were fitted with a cochlear implant and one with bilateral hearing aids. All were "switched on" or fitted before 30 months of age. The Diary of Early Language (Di-EL) was used to collect a 100-word lexicon from each participant. All single word and frozen phrase data from each child's Di-EL were allocated to 1 of 15 word types grouped into four word categories (noun, predicate, grammatical, and paralexical), and the results were compared for both groups. The hearing and HL groups showed similar distributions of word categories, with nouns constituting the largest portion of the lexicon followed by predicates and paralexicals. Grammaticals made up the smallest portion of the lexicon. However, several significant differences were evident between the two groups. In both the 50- and 100-word lexicons, the hearing group

  13. 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)

  14. Bone-anchored hearing aids (Baha) in congenital aural atresia: personal experience.

    Science.gov (United States)

    Ricci, Giampietro; Volpe, Antonio Della; Faralli, Mario; Longari, Fabrizio; Lancione, Carla; Varricchio, Alfonso Maria; Frenguelli, Antonio

    2011-03-01

    Aural atresia is a congenital disease constituted by partial or complete lack of development of the external auditory canal, which is generally associated with malformations of the auricle and middle ear. Reconstruction of the auditory canal and correction of any deformities of the middle ear have yielded unpredictable results and variable functional outcomes, and there is a high rate of complications. Therefore, the use of bone-conduction hearing aids, such as the Baha, may represent a valid alternative for subjects who have conductive hearing loss with cochlear reserve that, as a rule, is fully conserved. The aim of this work is to reexamine our experience with the management of conductive and mixed hearing loss using the Baha system in children with bilateral aural atresia. We examined 31 patients with bilateral congenital aural atresia in whom a Baha system had been implanted. The patients, 16 males and 15 females, were between 5 and 14 years of age (mean 8.7). The following parameters were assessed for each patient: mean preoperative air and bone conduction for frequencies between 0.5 and 4kHz; mean preoperative threshold with conventional bone-conduction hearing aids; mean postoperative threshold with the Baha system; improvement in quality of life evaluated with the Glasgow Children's Benefit Inventory; rate and type of surgical complications. The mean preoperative air- and bone-conduction thresholds were 51.2±12.5 and 14.1±6.3dB HL, respectively. The mean preoperative threshold with a conventional bone-conduction hearing aid was 29.3±7.2dB HL, and the mean postoperative threshold with the Baha system was 18.1±7.5dB HL. Quality of life improved for all operated patients. The results of our study of the Baha system to treat patients with bilateral aural atresia were extremely satisfactory compared both with those of surgical reconstruction of the auditory canal and those of traditional bone-conduction hearing aids. Furthermore, great improvement was noted

  15. 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.

  16. Inner ear anomalies causing congenital sensorineural hearing loss: CT and MR imaging findings

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    Hong, Hyun Sook; Paik, Sang Hyun; Cha, Jang Gyu; Park, Seong Jin; Joh, Joon Hee; Park, Jai Soung; Kim, Dae Ho; Lee, Hae Kyung; Kim, Shi Chan [Soonchunhyang University Bucheon Hospital, Buchon(Korea, Republic of)

    2005-07-15

    Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cased had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss.

  17. 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

  18. Comparing NAL-NL1 and DSL v5 in Hearing Aids Fit to Children with Severe or Profound Hearing Loss: Goodness of Fit-to-Targets, Impacts on Predicted Loudness and Speech Intelligibility.

    Science.gov (United States)

    Ching, Teresa Y C; Quar, Tian Kar; Johnson, Earl E; Newall, Philip; Sharma, Mridula

    2015-03-01

    An important goal of providing amplification to children with hearing loss is to ensure that hearing aids are adjusted to match targets of prescriptive procedures as closely as possible. The Desired Sensation Level (DSL) v5 and the National Acoustic Laboratories' prescription for nonlinear hearing aids, version 1 (NAL-NL1) procedures are widely used in fitting hearing aids to children. Little is known about hearing aid fitting outcomes for children with severe or profound hearing loss. The purpose of this study was to investigate the prescribed and measured gain of hearing aids fit according to the NAL-NL1 and the DSL v5 procedure for children with moderately severe to profound hearing loss; and to examine the impact of choice of prescription on predicted speech intelligibility and loudness. Participants were fit with Phonak Naida V SP hearing aids according to the NAL-NL1 and DSL v5 procedures. The Speech Intelligibility Index (SII) and estimated loudness were calculated using published models. The sample consisted of 16 children (30 ears) aged between 7 and 17 yr old. The measured hearing aid gains were compared with the prescribed gains at 50 (low), 65 (medium), and 80 dB SPL (high) input levels. The goodness of fit-to-targets was quantified by calculating the average root-mean-square (RMS) error of the measured gain compared with prescriptive gain targets for 0.5, 1, 2, and 4 kHz. The significance of difference between prescriptions for hearing aid gains, SII, and loudness was examined by performing analyses of variance. Correlation analyses were used to examine the relationship between measures. The DSL v5 prescribed significantly higher overall gain than the NAL-NL1 procedure for the same audiograms. For low and medium input levels, the hearing aids of all children fit with NAL-NL1 were within 5 dB RMS of prescribed targets, but 33% (10 ears) deviated from the DSL v5 targets by more than 5 dB RMS on average. For high input level, the hearing aid fittings of

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

    Science.gov (United States)

    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.

  20. Hearing impairment in children with congenital cytomegalovirus (CMV) infection based on distortion product otoacoustic emissions (DPOAE) and brain evoked response audiometry stimulus click (BERA Click) examinations

    Science.gov (United States)

    Airlangga, T. J.; Mangunatmadja, I.; Prihartono, J.; Zizlavsky, S.

    2017-08-01

    Congenital cytomegalovirus (congenital CMV) infection is a leading factor of nongenetic sensorineural hearing loss in children. Hearing loss caused by CMV infection does not have a pathognomonic configuration hence further research is needed. The development of knowledge on hearing loss caused by congenital CMV infection is progressing in many countries. Due to a lack of research in the context of Indonesia, this study assesses hearing impairment in children with congenital CMV infection in Indonesia, more specifically in the Cipto Mangunkusumo Hospital. Our objective was to profile hearing impairment in children 0-5 years of age with congenital CMV infection using Distortion Product Otoacoustic Emissions (DPOAE) and Brain Evoked Response Audiometry Stimulus Click (BERA Click) examinations. This cross-sectional study was conducted in the Cipto Mangunkusum Hospital from November, 2015 to May 2016 with 27 children 0-5 years of age with congenital CMV infection. Of individual ears studied, 58.0% exhibited sensorineural hearing loss. There was a significant relationship between developmental delay and incidence of sensorineural hearing loss. Subjects with a developmental delay were 6.57 times more likely (CI 95%; 1.88-22.87) to experience sensorineural hearing loss. Congenital CMV infection has an important role in causing sensorineural hearing loss in children.

  1. Hearing loss in adult women with Turner's syndrome and other congenital hypogonadisms.

    Science.gov (United States)

    Ros, Cristina; Tercero, Anna; Alobid, Isam; Balasch, Juan; Santamaria, Joan; Mullol, Joaquim; Castelo-Branco, Camil

    2014-02-01

    To define the patterns and causes of hearing decline associated to Turner's syndrome (TS). An observational study with three cohorts was designed: 31 TS patients, 15 women with other congenital hypogonadims (OCH) and 41 healthy age-matched women taking contraception. Microotoscopy, standard pure-tone audiometry brain auditory evoked potentials (BAEP) were performed to study hearing function. Up to 87% of TS subjects suffered from some degree of hearing loss (HL) in the audiograms, compared with 20% OCH and 27% controls. Sensorineural hearing loss (SNHL) was the most frequent type of hypoacusia found in TS group. BAEP study demonstrated that 61% of TS women showed HL compared to 20% in OCH patients. No significant differences in latencies, amplitudes, and interpeaks of waves I, III and V were found between TS and OCH, nor when compared to reference population. Worse results were observed among the oldest TS patients, those with pure monosomy or isochromosome, and those with a history of recurrent otitis. More than a half of TS females presented HL. SNHL is the most frequent pattern among middle-aged women with TS. Old age, karyotype and recurrent otitis are predisposition factors to produce HL, while oestrogens play a minor role.

  2. 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.

  3. 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.

  4. Comparison of Gap in Noise Test Results Between Congenital Blind and Sighted Subjects With Normal Hearing

    Directory of Open Access Journals (Sweden)

    Soghrat Faghihzadeh

    2011-09-01

    Full Text Available Background and Aims: Main feature of auditory processing abilities is temporal processing including temporal resolution, temporal ordering, temporal integration and temporal masking. Many studies have shown the superiority of blinds in temporal discrimination over sighted subjects. In this study, temporal processing was compared in congenital blind subjects with sighted controls via gap in noise test (GIN.Methods: This analytic-prescriptive non-invasive cohort study was conducted on 22 congenital blinds (11 males and 11 females with a mean age of 26.22 years and 22 sighted control subjects (11 males and 11 females with a mean age of 24.04 years with normal hearing in faculty of Rehabilitation Tehran University of Medical Sciences. Gap in noise test results, approximate threshold and percent of corrected answers, were obtained and then, were analyzed by Mann-Whitney non-parametric statistical test.Results: There was a significant difference in the approximate threshold and the percent of corrected answers between congenital blinds and sighted control subjects (p<0.05. However, there was no significant difference between males and females in this regard (p>0.05.Conclusion: Auditory temporal resolution ability, the lower approximate threshold and the more corrected answers in gap in noise, in blind subjects is better than the sighted control group and it might be related to the compensative neuroplasticity after visual deprivation.

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

    Directory of Open Access Journals (Sweden)

    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

  6. Effects of congenital hearing loss and cochlear implantation on audiovisual speech perception in infants and children.

    Science.gov (United States)

    Bergeson, Tonya R; Houston, Derek M; Miyamoto, Richard T

    2010-01-01

    Cochlear implantation has recently become available as an intervention strategy for young children with profound hearing impairment. In fact, infants as young as 6 months are now receiving cochlear implants (CIs), and even younger infants are being fitted with hearing aids (HAs). Because early audiovisual experience may be important for normal development of speech perception, it is important to investigate the effects of a period of auditory deprivation and amplification type on multimodal perceptual processes of infants and children. The purpose of this study was to investigate audiovisual perception skills in normal-hearing (NH) infants and children and deaf infants and children with CIs and HAs of similar chronological ages. We used an Intermodal Preferential Looking Paradigm to present the same woman's face articulating two words ("judge" and "back") in temporal synchrony on two sides of a TV monitor, along with an auditory presentation of one of the words. The results showed that NH infants and children spontaneously matched auditory and visual information in spoken words; deaf infants and children with HAs did not integrate the audiovisual information; and deaf infants and children with CIs initially did not initially integrate the audiovisual information but gradually matched the auditory and visual information in spoken words. These results suggest that a period of auditory deprivation affects multimodal perceptual processes that may begin to develop normally after several months of auditory experience.

  7. Audiological evaluation and self-assessed hearing problems in subjects with single-sided congenital external ear malformations and associated conductive hearing loss.

    Science.gov (United States)

    Priwin, Claudia; Jönsson, Radi; Magnusson, Lennart; Hultcrantz, Malou; Granström, Gösta

    2007-04-01

    Previously, unilateral hearing impairment (UHI) has been considered of little consequence. However, a recent meta-analysis of children with UHI displayed educational and behavioural problems and possible delays of speech and language development. Further, patients with UHI consequently report hearing difficulties. Our study investigated hearing function, possible inner ear protection, and self-assessed hearing problems in 57 subjects aged between 3-80 years with single-sided congenital ear malformations and conductive UHI. Pure-tone thresholds and speech recognition (quiet, noise) were measured, and all patients completed a self-assessment questionnaire. Pure-tone thresholds corresponding to sensorineural function did not significantly differ between the normal (air conduction) and affected ear (bone conduction). However, speech recognition in both quiet and in noise was normal on the non-affected side but significantly worse on the malformed side. A moderate to high degree of self-assessed hearing problems were reported. In conclusion, hearing function in the affected ear was found to be subnormal in terms of supra threshold signal processing. Furthermore, a high degree of hearing difficulty was reported. Therefore, active treatment, surgery, or hearing amplification, might be considered.

  8. Long-term Compliance and Satisfaction With Percutaneous Bone Conduction Devices in Patients With Congenital Unilateral Conductive Hearing Loss

    NARCIS (Netherlands)

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

    2015-01-01

    OBJECTIVE: Patients with congenital unilateral conductive hearing loss (UCHL) can either be watchful monitored or treated surgically through the fitting of a percutaneous bone conduction device (BCD) or, in some cases, atresia repair. The current study evaluated the long-term compliance and

  9. Contribution of targeted saliva screening for congenital CMV-related hearing loss in newborns who fail hearing screening.

    Science.gov (United States)

    Ari-Even Roth, Daphne; Lubin, Daniel; Kuint, Jacob; Teperberg-Oikawa, Michal; Mendelson, Ella; Strauss, Tzipora; Barkai, Galia

    2017-11-01

    We previously reported a 2.2% rate of infants born with sensorineural hearing loss (SNHL) due to congenital cytomegalovirus (cCMV) infection identified by universal neonatal screen for cCMV using saliva. To evaluate the contribution of targeted saliva screening for cCMV to the detection of infants born with cCMV-related SNHL who failed universal newborn hearing screening (UNHS). We retrospectively reviewed the audiological and medical records of infants who failed UNHS and were tested for cCMV using saliva sample prior to discharge at Sheba Medical Center between 2014 and 2015. Positive cases were confirmed by urine sample. Two hundred (1%) of the 19 830 infants tested during the study period failed in-hospital hearing screening. A saliva specimen was obtained prior to discharge in 187 infants (93.5% of those who failed UNHS). In 178 infants saliva testing was performed at ≤21 days of chronological age and yielded results. cCMV infection was identified in 4/178 tested infants (2.25%, 95% CI 0.8% to 5.3%), of whom three were diagnosed with SNHL (1.7%, 95% CI 0.5% to 4.4%) and offered antiviral treatment. Two of the tested infants (1.12%, 95% CI 0.2% to 3.6%) were diagnosed with cCMV solely due to failure in UNHS. Occult central nervous system (CNS) symptoms of cCMV infection were detected in 2/4 infants following targeted investigation. Targeted cCMV screening in newborns who failed UNHS contributed to the early detection of infants born with cCMV-related isolated SNHL or with occult CNS symptoms who could potentially benefit from antiviral treatment. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  10. A Micro-Ethnographic Study of the Communication/Language Development in a Japanese Child with Profound Hearing Loss Before and After Cochlear Implantation

    Directory of Open Access Journals (Sweden)

    Richard R. Kretschmer

    2010-02-01

    Full Text Available This study described the communication and spoken language development of a Japanese girl with profound hearing loss who used a cochlear implant from 19 months of age. The girl, Akiko, was born in Belgium where her family was living at that time. After she was identified as deaf at birth, she and her parents were provided with support services. The family relocated to Japan when Akiko was 1 year 5 months of age. When she was 1 year 6 months of age Akiko underwent cochlear implantation. The cochlear implant device was activated when Akiko was 1 year 7 months of age. The parents routinely made video recordings of Akiko interacting with family members and teachers at home and at school. The video recordings taken by the parents used as the data for this study contained scenes of Akiko from the time she was 3 months of age until she was 4 years 11 months of age. Micro-ethnographic methods were used to analyze the dynamics and development of selected communicative interactions over this age span of Şfty-six months. The original pool of video recordings contained 213 scenes. As a result of video viewing and editing, Akiko’s communication development was found to follow expected patterns of development as described by other child language researchers of children with normal hearing. There were seven demarcations that represent Akiko’s communication and spoken language development: 1 perlocutionary, 2 transition of perlocutionary to illocutionary, 3 illocutionary, 4 transition of illocutionary to locutionary, 5 locutionary, 6 dialogue, and 7 narrative

  11. A Micro-Ethnographic Study of the Communication/Language Development in a Japanese Child with Profound Hearing Loss Before and After Cochlear Implantation

    Directory of Open Access Journals (Sweden)

    Richard R. Kretschmer

    2010-10-01

    Full Text Available This study described the communication and spoken language development of a Japanese girl with profound hearing loss who used a cochlear implant from 19 months of age. The girl, Akiko, was born in Belgium where her family was living at that time. After she was identified as deaf at birth, she and her parents were provided with support services. The family relocated to Japan when Akiko was 1 year 5 months of age. When she was 1 year 6 months of age Akiko underwent cochlear implantation. The cochlear implant device was activated when Akiko was 1 year 7 months of age. The parents routinely made video recordings of Akiko interacting with family members and teachers at home and at school. The video recordings taken by the parents used as the data for this study contained scenes of Akiko from the time she was 3 months of age until she was 4 years 11 months of age. Micro-ethnographic methods were used to analyze the dynamics and development of selected communicative interactions over this age span of fifty-six months. The original pool of video recordings contained 213 scenes.As a result of video viewing and editing, Akiko’s communication development was found to follow expected patterns of development as described by other child language researchers of children with normal hearing. There were seven demarcations that represent Akiko’s communication and spoken language development: 1 perlocutionary, 2 transition of perlocutionary to illocutionary, 3 illocutionary, 4 transition of illocutionary to locutionary, 5 locutionary, 6 dialogue, and 7 narrative.

  12. Three-year experience with the Sophono in children with congenital conductive unilateral hearing loss: tolerability, audiometry, and sound localization compared to a bone-anchored hearing aid.

    Science.gov (United States)

    Nelissen, Rik C; Agterberg, Martijn J H; Hol, Myrthe K S; Snik, Ad F M

    2016-10-01

    Bone conduction devices (BCDs) are advocated as an amplification option for patients with congenital conductive unilateral hearing loss (UHL), while other treatment options could also be considered. The current study compared a transcutaneous BCD (Sophono) with a percutaneous BCD (bone-anchored hearing aid, BAHA) in 12 children with congenital conductive UHL. Tolerability, audiometry, and sound localization abilities with both types of BCD were studied retrospectively. The mean follow-up was 3.6 years for the Sophono users (n = 6) and 4.7 years for the BAHA users (n = 6). In each group, two patients had stopped using their BCD. Tolerability was favorable for the Sophono. Aided thresholds with the Sophono were unsatisfactory, as they did not reach under a mean pure tone average of 30 dB HL. Sound localization generally improved with both the Sophono and the BAHA, although localization abilities did not reach the level of normal hearing children. These findings, together with previously reported outcomes, are important to take into account when counseling patients and their caretakers. The selection of a suitable amplification option should always be made deliberately and on individual basis for each patient in this diverse group of children with congenital conductive UHL.

  13. Surgery or implantable hearing devices in children with congenital aural atresia: 25 years of our experience.

    Science.gov (United States)

    Jovankovičová, Andrea; Staník, Roman; Kunzo, Samuel; Majáková, Lucia; Profant, Milan

    2015-07-01

    Congenital aural atresia and ear deformities have been the subject of serious discussions for centuries. These malformations are associated with significant aesthetic and functional problems. Outcome of the surgical solution is rarely optimal. Despite the gradual improvement of surgical techniques the surgery still remains associated with very limited short-term and mainly long-term functional outcome. Therefore, the priority treatment in modern otology becomes implantable devices--BAHA, Bonebridge and active middle ear implants. The functional and aesthetic outcomes of aural atresia reconstruction performed at Pediatric ENT Department of Children's University Hospital were retrospectively evaluated and compared with the results prospectively obtained from implantable hearing devices (BAHA, Vibrant Soundbridge, Bonebridge), which have been implanted in patients with aural atresia at Department of ORL HNS, University Hospital Bratislava. Aural atresia reconstruction has been performed in 34 patients during last 25 years. Results of the surgery could be viewed as excellent only in three patients (gain above 30 dB). Air conduction threshold has decreased after the surgery in seven patients, and in two cases total deafness occurred after the surgery. Patients gain on average 12 dB in auditory threshold after surgery. Hearing devices were implanted to the group of 11 children in order to improve their hearing. All of them were the patients with bilateral aural atresia. After implantation a significant improvement in hearing threshold occurred in all children (30-35 dB on average). Together with results of air conduction threshold in patient with aural atresia before and after surgery and implantation we also present a standard deviation. The functional outcome of implantable hearing devices in patients with bilateral aural atresia clearly dominates over the traditional reconstructive surgery. Aesthetic results in pinna deformity management remain a major concern for

  14. Controversies in the Field of Hearing Impairment: Early Identification, Educational Methods, and Cochlear Implants.

    Science.gov (United States)

    Samson-Fang, Lisa; Simons-McCandless, Marsha; Shelton, Clough

    2000-01-01

    This article discusses the incidence of infant hearing impairments, auditory screening tests, and available intervention choices for infants with severe to profound congenital hearing loss, including methods for communication and cochlear implantation. American Sign language, manually coded English, fingerspelling, total communication, and…

  15. 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

  16. Congenital aural atresia: bone-anchored hearing aid vs. external auditory canal reconstruction.

    Science.gov (United States)

    Bouhabel, Sarah; Arcand, Pierre; Saliba, Issam

    2012-02-01

    To compare the audiologic outcome and feasibility of bone-anchored hearing aid (BAHA) and external auditory canal reconstruction (EACR) surgeries in pediatric patients presenting a congenital aural atresia (CAA). A retrospective chart review of 40 patients operated in our tertiary pediatric care center between 2002 and 2010 was performed. 20 patients underwent EACR, whereas another 20 patients were implanted with a BAHA device. Air conduction (AC), bone conduction (BC), pure tone average (PTA) and speech discrimination score (SDS) were compared preoperatively, and hearing gain (HG) postoperatively at 6 and at 12 months at frequencies of 500, 1000, 2000 and 4000 Hz. Operative time, complications and associated microtia were documented as well. EACR patients were graded retrospectively upon Jahrsdoerfer's classification. Preoperative AC were significantly different between groups, at 500, 1000 and 2000 Hz but not at 4000 Hz. BAHA group compared postoperatively to EACR group showed significantly a superior HG of 46.9 ± 7.0 dB (paided air thresholds from the EACR group revealed an audiologic status similar to those of the BAHA group patients, at 6 months and one year postoperatively. Both groups had a similar evolution of their BC, as well as of the incidence of complications. We report one case of transient facial paralysis in the EACR group. Total operative time is significantly lower (pBAHA implantation (56 ± 21 min) than for EACR surgery (216 ± 174 min). No preoperative or postoperative correlation (Pearson correlation test; p>0.05) was found between patient's Jahrsdoerfer's score and their audiologic outcome. HG does not seem to be influenced by the presence of microtia. EACR, although constituting an attractive option, does not give acceptable results alone. It can however, when combined to conventional air conduction hearing aids, provide excellent audiologic outcomes comparable to BAHA. BAHA implantation is a reliable, safe and efficient therapeutic option

  17. Heterogeneity in Phenotype of Usher-Congenital Hyperinsulinism Syndrome

    DEFF Research Database (Denmark)

    Al Mutair, Angham N; Brusgaard, Klaus; Bin-Abbas, Bassam

    2013-01-01

    OBJECTIVETo evaluate the phenotype of 15 children with congenital hyperinsulinism (CHI) and profound hearing loss, known as homozygous 11p15-p14 deletion syndrome (MIM #606528).METHODSProspective clinical follow-up and genetic analysis by direct sequencing, Multiplex Ligation-dependent Probe.......CONCLUSIONSThe phenotype of homozygous 11p15-p14 deletion syndrome, or Usher-CHI syndrome, includes any severity of neonatal-onset CHI and severe, sensorineural hearing loss. Retinitis pigmentosa and nonautoimmune diabetes may occur in adolescence....

  18. 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.

  19. Concerns of Indian Mothers with Children Having Severe-to-Profound Hearing Impairment at Diagnosis and after 1–3 Years of Therapy

    Directory of Open Access Journals (Sweden)

    Nachiketa Rout

    2012-01-01

    Full Text Available Counseling training in graduate programs continues to be underrepresented. If parental queries are not addressed adequately, they keep visiting one doctor after another. Objective. The aim of the study is to identify maternal needs of children with hearing impairment at two stages of habilitation, that is, just after diagnosis (group I and after receiving 1 to 3 years of language therapy (group II. Methods. Two groups of mothers were asked to speak their queries about aural habilitation of their children. Queries were recorded, summarized, and categorized as per their priorities. Results. Group I mothers wanted to know about how the child would learn to listen and speak (45%, causes of hearing loss (33.7%, understanding the ear and hearing (10.2%, understanding the audiogram (7%, and coping with emotional aspects of hearing loss (5%, while group II parents had priorities concerning speech development (24.5% followed by child independence and employment (17.3%, schooling (15.6%, problem behaviors (11%, amplification device (9.4%, duration of therapy (8%, future of the child (8%, and questions about how can my child get adjusted to the “normal” world (6%. Conclusions. Culture- and language-specific materials to explain these issues need to be developed.

  20. Genes and Hearing Loss

    Science.gov (United States)

    ... Find an ENT Doctor Near You Genes and Hearing Loss Genes and Hearing Loss Patient Health Information News media interested in ... One of the most common birth defects is hearing loss or deafness (congenital), which can affect as ...

  1. Working Memory Capacity as a Factor Influencing the Relationship between Language Outcome and Rehabilitation in Mandarin-Speaking Preschoolers with Congenital Hearing Impairment

    Science.gov (United States)

    Lo, Ming; Chen, Pei-Hua

    2017-01-01

    Memory processes could account for a significant part of the variance in language performances of hearing-impaired children. However, the circumstance in which the performance of hearing-impaired children can be nearly the same as the performance of hearing children remains relatively little studied. Thus, a group of pre-school children with congenital, bilateral hearing loss and a group of pre-school children with normal hearing were invited to participate in this study. In addition, the hearing-impaired participants were divided into two groups according to their working memory span. A language disorder assessment test for Mandarin-speaking preschoolers was used to measure the outcomes of receptive and expressive language of the two groups of children. The results showed that the high-span group performed as good as the hearing group, while the low-span group showed lower accuracy than the hearing group. A linear mixed-effects analysis showed that not only length of rehabilitation but also the memory span affected the measure of language outcome. Furthermore, the rehabilitation length positively correlated with the measure of expressive language only among the participants of the high-span group. The pattern of the results indicates that working memory capacity is one of the factors that could support the children to acquire age-equivalent language skills. PMID:28337168

  2. Working Memory Capacity as a Factor Influencing the Relationship between Language Outcome and Rehabilitation in Mandarin-Speaking Preschoolers with Congenital Hearing Impairment.

    Science.gov (United States)

    Lo, Ming; Chen, Pei-Hua

    2017-01-01

    Memory processes could account for a significant part of the variance in language performances of hearing-impaired children. However, the circumstance in which the performance of hearing-impaired children can be nearly the same as the performance of hearing children remains relatively little studied. Thus, a group of pre-school children with congenital, bilateral hearing loss and a group of pre-school children with normal hearing were invited to participate in this study. In addition, the hearing-impaired participants were divided into two groups according to their working memory span. A language disorder assessment test for Mandarin-speaking preschoolers was used to measure the outcomes of receptive and expressive language of the two groups of children. The results showed that the high-span group performed as good as the hearing group, while the low-span group showed lower accuracy than the hearing group. A linear mixed-effects analysis showed that not only length of rehabilitation but also the memory span affected the measure of language outcome. Furthermore, the rehabilitation length positively correlated with the measure of expressive language only among the participants of the high-span group. The pattern of the results indicates that working memory capacity is one of the factors that could support the children to acquire age-equivalent language skills.

  3. Connexin 26 and 30 mutations in paediatric patients with congenital, non-syndromic hearing loss treated with cochlear implantation in Mediterranean Turkey.

    Science.gov (United States)

    Tarkan, Ö; Sari, P; Demirhan, O; Kiroğlu, M; Tuncer, Ü; Sürmelioğlu, Ö; Ozdemir, S; Yilmaz, M B; Kara, K

    2013-01-01

    Mutations in the genes for connexin 26 (GJB2) and connexin 30 (GJB6) play an important role in autosomal recessive, non-syndromic hearing loss. This study aimed to detect the 35delG and 167delT mutations of the GJB2 gene and the del(GJB6-D13S1830) mutation of the GJB6 gene in paediatric patients diagnosed with congenital, non-syndromic hearing loss and treated with cochlear implantation in Mediterranean Turkey. We included 94 children diagnosed with congenital, non-syndromic hearing loss and treated with cochlear implantation. Blood samples were collected, DNA extracted and an enzyme-linked immunosorbent assay performed to enable molecular diagnosis of mutations. Of the 94 children analysed, the 35delG mutation was detected in 12 (12.7 per cent): 10 (83.3 per cent) were homozygous and 2 (16.7 per cent) heterozygous mutant. The 167delT and del(GJB6-D13S1830) mutations were not detected. The GJB2-35delG mutation is a major cause of congenital, non-syndromic hearing loss in this study population.

  4. The Influence of Linguistic Proficiency on Masked Text Recognition Performance in Adults With and Without Congenital Hearing Impairment.

    Science.gov (United States)

    Huysmans, Elke; Bolk, Elske; Zekveld, Adriana A; Festen, Joost M; de Groot, Annette M B; Goverts, S Theo

    2016-01-01

    The authors first examined the influence of moderate to severe congenital hearing impairment (CHI) on the correctness of samples of elicited spoken language. Then, the authors used this measure as an indicator of linguistic proficiency and examined its effect on performance in language reception, independent of bottom-up auditory processing. In groups of adults with normal hearing (NH, n = 22), acquired hearing impairment (AHI, n = 22), and moderate to severe CHI (n = 21), the authors assessed linguistic proficiency by analyzing the morphosyntactic correctness of their spoken language production. Language reception skills were examined with a task for masked sentence recognition in the visual domain (text), at a readability level of 50%, using grammatically correct sentences and sentences with distorted morphosyntactic cues. The actual performance on the tasks was compared between groups. Adults with CHI made more morphosyntactic errors in spoken language production than adults with NH, while no differences were observed between the AHI and NH group. This outcome pattern sustained when comparisons were restricted to subgroups of AHI and CHI adults, matched for current auditory speech reception abilities. The data yielded no differences between groups in performance in masked text recognition of grammatically correct sentences in a test condition in which subjects could fully take advantage of their linguistic knowledge. Also, no difference between groups was found in the sensitivity to morphosyntactic distortions when processing short masked sentences, presented visually. These data showed that problems with the correct use of specific morphosyntactic knowledge in spoken language production are a long-term effect of moderate to severe CHI, independent of current auditory processing abilities. However, moderate to severe CHI generally does not impede performance in masked language reception in the visual modality, as measured in this study with short, degraded

  5. The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

    Directory of Open Access Journals (Sweden)

    Hajime Koyama

    2016-01-01

    Full Text Available Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months. Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.

  6. Delayed diagnosis of a patient with Usher syndrome 1C in a Louisiana Acadian family highlights the necessity of timely genetic testing for the diagnosis and management of congenital hearing loss.

    Science.gov (United States)

    Umrigar, Ayesha; Musso, Amanda; Mercer, Danielle; Hurley, Annette; Glausier, Cassondra; Bakeer, Mona; Marble, Michael; Hicks, Chindo; Tsien, Fern

    2017-01-01

    Advances in sequencing technologies and increased understanding of the contribution of genetics to congenital sensorineural hearing loss have led to vastly improved outcomes for patients and their families. Next-generation sequencing and diagnostic panels have become increasingly reliable and less expensive for clinical use. Despite these developments, the diagnosis of genetic sensorineural hearing loss still presents challenges for healthcare providers. Inherited sensorineural hearing loss has high levels of genetic heterogeneity and variable expressivity. Additionally, syndromic hearing loss (hearing loss and additional clinical abnormalities) should be distinguished from non-syndromic (hearing loss is the only clinical symptom). Although the diagnosis of genetic sensorineural hearing loss can be challenging, the patient's family history and ethnicity may provide critical information, as certain genetic mutations are more common in specific ethnic populations. The early identification of the cause of deafness can benefit patients and their families by estimating recurrence risks for future family planning and offering the proper interventions to improve their quality of life. Collaboration between pediatricians, audiologists, otolaryngologists, geneticists, and other specialists are essential in the diagnosis and management of patients with hearing disorders. An early diagnosis is vital for proper management and care, as some clinical manifestations of syndromic sensorineural hearing loss are not apparent at birth and have a delayed age of onset. We present a case of Usher syndrome (congenital deafness and childhood-onset blindness) illustrating the challenges encountered in the diagnosis and management of children presenting with congenital genetic sensorineural hearing loss, along with helpful resources for clinicians and families.

  7. 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....

  8. Bone-Anchored Hearing Aid vs. Reconstruction of the External Auditory Canal in Children and Adolescents with Congenital Aural Atresia: A Comparison Study of Outcomes.

    Science.gov (United States)

    Farnoosh, Soroush; Mitsinikos, F Tania; Maceri, Dennis; Don, Debra M

    2014-01-01

    Congenital aural atresia is a rare condition affecting 1 in 10,000-20,000 children a year. Surgery is required to restore hearing to facilitate normal development. The objective of this study was to compare outcomes in hearing, complications, and quality of life of surgical reconstruction of the external auditory canal reconstruction (EACR) and bone-anchored hearing aid (BAHA) in a pediatric population with congenital aural atresia. Subjects were children who had a diagnosis of congenital aural atresia or stenosis and who received either BAHA or EACR. The medical records of 68 children were reviewed for operative complications and audiometric results. A quality of life questionnaire was prospectively administered to a subset of subjects. Pre-operatively, air conduction threshold was not significantly different between groups at 500, 1000, 2000, and 4000 Hz (p > 0.05). Post-operatively, the BAHA group (44.3 ± 14.3 and 44.5 ± 11.3) demonstrated a significantly larger hearing gain than the EACR group (20.0 ± 18.9 and 15.3 ± 19.9) in both the short and long-term periods (p  0.05). Quality of life assessment revealed no statistical significance between the two groups (p > 0.05). Although the quality of life and incidence of surgical complications between the two interventions was not significantly different, BAHA implantation appears to provide a better, more reliable audiologic outcome than EACR.

  9. Bone -Anchored Hearing Aid versus Reconstruction of the External Auditory Canal in Children and Adolescents with Congenital Aural Atresia: A Comparison Study of Outcomes

    Directory of Open Access Journals (Sweden)

    Debra Mei Don

    2014-01-01

    Full Text Available Objectives/Hypothesis: Congenital aural atresia is a rare condition in children affecting 1 in 10,000 to 20,000 children a year. Surgery is required to restore hearing to facilitate normal development. The objective of this study was to compare outcomes in hearing, complications and quality of life of surgical reconstruction of the external auditory canal (EACR and bone-anchored hearing aid (BAHA in a pediatric population with congenital aural atresia. Study Design: Subjects were children who had a diagnosis of congenital aural atresia or stenosis and who received either BAHA or EACR. Methods: The medical records of 68 children were reviewed for operative complications and audiometric results. A quality of life questionnaire was prospectively administered to a subset of subjects. Results: Pre-operatively, air conduction threshold were not significantly different between groups at 500, 1000, 2000 and 4000 Hz (p>0.05. Post-operatively, the BAHA group (44.3 ± 14.3 and 44.5 ± 11.3 demonstrated a significantly larger hearing gain than the EACR group (20.0 ± 18.9 and 15.3 ± 19.9 in both the short and long-term periods (p<0.001. Overall the incidence of complications and need for revision surgery were comparable between groups (p>0.05. Quality of life assessment revealed no statistical significance between the two groups (p>0.05 Conclusion: Although the quality of life and incidence of surgical complications between the two interventions was not significantly different, BAHA implantation appears to provide a better, more reliable audiologic outcome than EACR.

  10. Deficiência auditiva na toxoplasmose congênita detectada pela triagem neonatal Hearing loss in congenital toxoplasmosis detected by newborn screening

    Directory of Open Access Journals (Sweden)

    Gláucia Manzan Queiroz de Andrade

    2008-02-01

    Full Text Available A toxoplasmose congênita pode causar déficit neurossensorial em até 20% dos casos e o tratamento no primeiro ano de vida melhora o prognóstico. No Brasil, desconhece-se o impacto da infecção na hipoacusia. OBJETIVO: Avaliar a audição de crianças com toxoplasmose congênita identificadas pela triagem neonatal. MATERIAL E MÉTODO: Estudo prospectivo de crianças com toxoplasmose congênita identificadas pela triagem neonatal (IgM anti-T. gondii em Belo Horizonte, durante 2003/2004. Realizada sorologia confirmatória (mãe/filho e consideradas positivas as crianças apresentando IgM e/ou IgA nos primeiros seis meses ou IgG aos 12 meses de vida. Avaliações auditivas ao diagnóstico e após 12 meses incluíram Audiometria Comportamental, Emissões Otoacústicas, Imitanciometria, Audiometria de Tronco Encefálico. RESULTADOS: Dentre 30.808 crianças triadas (97% dos nascidos vivos, 20 apresentavam toxoplasmose congênita, 15 (75% com infecção subclínica. Dezenove crianças realizaram avaliação auditiva. Quatro apresentaram déficit neurossensorial (21,1%. Uma criança apresentou outros fatores de risco para hipoacusia; nas outras três, a toxoplasmose foi o único fator observado. Duas crianças, tratadas adequadamente com antiparasitários, apresentaram déficit auditivo, em desacordo com a literatura. CONCLUSÃO: Os achados sugerem que a toxoplasmose congênita, prevalente no Brasil, é um fator de risco para hipoacusia e o impacto dessa infecção nas perdas auditivas deve ser estudado.Congenital toxoplasmosis may cause sensorineural deficit in up to 20% of the patients and proper treatment in the first year improves prognosis. In Brazil, this infection’s impact on hearing impairment is unknown. AIM: To evaluate hearing of newborns with congenital toxoplasmosis identified by the newborn screening service. METHOD: This prospective study analyzed children with congenital toxoplasmosis identified by newborn screening (IgM anti

  11. 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.

  12. [Genetic hearing loss].

    Science.gov (United States)

    Tanaka-Ouyang, Lei; Marlin, Sandrine; Nevoux, Jérôme

    2017-11-01

    Deafness is the most common sensory disability in developed countries affecting more than 2 births in 1000. Eighty percent of congenital deafness is genetic. Universal newborn hearing screening has been in place since 2012 in France. All genetic hearing losses are not congenital and all congenital hearing losses are not genetic. Genetic hearing loss may be syndromic (associated with other symptoms) (10 %) or non-syndromic (isolated) (90 %). Hearing loss may initially be the only symptom of syndromic deafness. A genetic origin can be diagnosed and must therefore be evoked systematically even in the adult. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  13. Study the Age of Hearing Loss Diagnosis and the Related Factors in a Group of School-Age Children in Baghch-e-Ban School of Hard of Hearing

    Directory of Open Access Journals (Sweden)

    Mahnaz Ahmadi

    1996-04-01

    Full Text Available Background: Estimation of the age of hearing loss diagnosis in every society is recommended to be compared with standard criteria to establish common dirth in management and rehabilitation. This study was conducted in order to determine the age of hearing loss diagnosis with respect to common criteria in Baghche-Ban school of hard of hearing children in Tehran.Methods: In this retrospective cross-sectional study, 445 children in stages 2, 6 and 10 of Baghche-Ban school of hard of hearing participated. the research was performed by means of the three-stages questionaires that children filled with help of their parents.Results: The mean age of detection of hearing loss in both acquired and congenital hearing loss in children was 3.5 year-old. while the age in which hearing loss was doubted was 1.8 year-old. In another word the mean age of hearing loss detection decreased as the hearing loss increases(in moderate- profound hearing loss(P<0.01. Conclusion: among the refrence sources for refering children with hearing loss the least refering was related to physicians. Adversly the mean age of doubting and detection of hearing loss increased in children with family history of hearing loss.(P<0.01. Meanwhile no relationship was detected between the age of diagnosis of hearing loss and the number of deaf members in the family(P<0.02.

  14. Novel missense mutation in the bZIP transcription factor, MAF, associated with congenital cataract, developmental delay, seizures and hearing loss (Aymé-Gripp syndrome).

    Science.gov (United States)

    Javadiyan, Shari; Craig, Jamie E; Sharma, Shiwani; Lower, Karen M; Casey, Theresa; Haan, Eric; Souzeau, Emmanuelle; Burdon, Kathryn P

    2017-05-08

    Cataract is a major cause of severe visual impairment in childhood. The purpose of this study was to determine the genetic cause of syndromic congenital cataract in an Australian mother and son. Fifty-one genes associated with congenital cataract were sequenced in the proband using a custom Ampliseq library on the Ion Torrent Personal Genome Machine (PGM). Reads were aligned against the human genome (hg19) and variants were annotated. Variants were prioritised for validation by Sanger sequencing if they were novel, rare or previously reported to be associated with paediatric cataract and were predicted to be protein changing. Variants were assessed for segregation with the phenotype in the affected mother. A novel likely pathogenic variant was identified in the transactivation domain of the MAF gene (c.176C > G, p.(Pro59Arg)) in the proband and his affected mother., but was absent in 326 unrelated controls and absent from public variant databases. The MAF variant is the likely cause of the congenital cataract, Asperger syndrome, seizures, hearing loss and facial characteristics in the proband, providinga diagnosis of Aymé-Gripp syndrome for the family.

  15. Hearing handicap in adults with unilateral deafness and bilateral hearing loss.

    Science.gov (United States)

    Iwasaki, Satoshi; Sano, Hajime; Nishio, Shinya; Takumi, Yutaka; Okamoto, Makito; Usami, Shin-ichi; Ogawa, Kaoru

    2013-06-01

    To assess the perception of hearing handicap in adult patients with unilateral sudden sensorineural hearing loss (SNHL) compared with those with bilateral SNHL or unilateral congenital SNHL. Retrospective chart review. Multicenter department of otolaryngology referrals. Seventy-one subjects in the unilateral severe-profound (>70 dB) sudden SNHL group (Group 1), 17 subjects in the unilateral prelingual or congenital SNHL group (Group 2), and 121 subjects in the bilateral SNHL group (Group 3). Questionnaire. Hearing Handicap Inventory for Adults (HHIA) and visual analogue scale (VAS) measurements of hearing handicap. Average levels of hearing loss were 92 dB in Group 1, 109 dB in Group 2, and 67 dB in Group 3. The relative percentage scores of HHIA and VAS compared with Group 3 were 72.6% and 81.0% in Group 1 and 25.4% and 50.3% in Group 2, respectively. A mild correlation between the HHIA subscale or VAS scores and degree of hearing loss could be found in Group 3. No significant correlation was found between the HHIA subscale or VAS scores and duration of hearing loss in Group 1 or Group 3. Higher scores were obtained in male subjects than in female subjects. Patients in Group 1 who were troubled by tinnitus scored significantly higher in the HHIA. In multiple logistic regression analysis, presence of tinnitus, older age, higher average hearing loss level, and group (bilateral SNHL>unilateral sudden SNHL>unilateral precongenital SNHL) revealed a significant positive association with high score (>42) of HHIA (odds ratio, 3.171, 1.021, 1.031, and 6.690, respectively). The results of HHIA and VAS suggest that not only patients with bilateral SNHL but also those with unilateral sudden SNHL, particularly those who have tinnitus, experience a hearing handicap.

  16. 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.

  17. Mutations in SLC33A1 cause a lethal autosomal-recessive disorder with congenital cataracts, hearing loss, and low serum copper and ceruloplasmin

    DEFF Research Database (Denmark)

    Huppke, Peter; Brendel, Cornelia; Kalscheuer, Vera

    2012-01-01

    , hearing loss, and severe developmental delay. Cerebral MRI showed pronounced cerebellar hypoplasia and hypomyelination. Homozygosity mapping was performed and displayed a region of commonality among three families at chromosome 3q25. Deep sequencing and conventional sequencing disclosed homozygous...... posttranslational modification of numerous proteins, without which normal lens and brain development is interrupted. Furthermore, AT-1 defects are a new and important differential diagnosis in patients with low copper and ceruloplasmin in serum.......Low copper and ceruloplasmin in serum are the diagnostic hallmarks for Menkes disease, Wilson disease, and aceruloplasminemia. We report on five patients from four unrelated families with these biochemical findings who presented with a lethal autosomal-recessive syndrome of congenital cataracts...

  18. Bone-anchored hearing aids in patients with acquired and congenital unilateral inner ear deafness (Baha CROS): clinical evaluation of 56 cases.

    Science.gov (United States)

    Hol, Myrthe K S; Kunst, Sylvia J W; Snik, Ad F M; Bosman, Arjan J; Mylanus, Emmanuel A M; Cremers, Cor W R J

    2010-07-01

    We performed an evaluation of the audiological and subjective benefits of the bone-anchored hearing aid (Baha) as a device for transcranial routing of sound (Baha CROS) in 56 patients with unilateral inner ear deafness. We performed a prospective clinical follow-up study in a tertiary referral center. Previously reported results of 29 patients were supplemented with a second series of 30 patients with unilateral inner ear deafness; 3 patients dropped out during the evaluation. Audiometric measurements were taken before and after Baha CROS fitting. Subjective benefits were quantified with 4 different patient questionnaires. The sound localization results in a well-structured test setting were not differentiable from chance. The 5 patients with congenital hearing loss showed better scores in the unaided sound localization measurements. Overall, most patients reported some subjective improvement in their capacity to localize sounds with the Baha CROS in daily life. The main effect of the Baha CROS was to alleviate the head shadow effect during the speech-in-noise test. Poor sound localization in this larger series of patients confirms the findings of previous studies. Improvements in the speech-in-noise scores corroborated the efficacy of the Baha CROS in alleviating the head shadow effect. The 4 different patient questionnaires revealed subjective benefit and satisfaction in various domains.

  19. Etiology of hearing loss in children.

    Directory of Open Access Journals (Sweden)

    José Ignacio BENITO-OREJAS

    2017-06-01

    Full Text Available Introduction and objective: The neonatal hearing loss is one of the most common disabilities, with lifelong implications for the child and his family. The implementation of the universal newborn hearing screening and the development in molecular medicine, genetic and integrative neuroscience has perfected the early diagnosis of the hearing loss children and consequently its intervention. With this work, we want to clarify the audiological aspects and causes of the permanent hearing loss diagnosed during the past 20 years. Method: We reviewed retrospectively the records of the children diagnosed with less than 3 years of age of permanent hearing loss, during the period 1994-2015, in a tertiary center. Evaluate the time of home, laterality, type and degree of hearing loss. Depending on the background, genetic testing and other complementary explorations, we present the results of our diagnostic study. Results: In the study-population (n = 183, 71% of the permanent hearing loss > 30 dB HL was diagnosed at birth (congenital. Its main features are the bilaterality (81%, the predominance sensorineural (85% and the grade profound (42% or moderate (30%, more prevalent in the unilateral forms. About the etiologic diagnosis, a 47% of the cases are of origin genetic (29% of which are syndromic, a 25% of cause environmental and a 28% unknown. Discussion: Our results are consistent for the generally accepted distribution of causes, but there are discrepancies in the literature. Despite the different tests used, we had to infer the etiology in 62% of children with hearing loss, finally unknown by 28%. Conclusions: We consider fundamental the monitoring for a consensus standardized etiological protocol that orient in the diagnostic process of hearing loss in children.

  20. Hearing Loss and Cytomegalovirus.

    Science.gov (United States)

    Strauss, Melvin

    1997-01-01

    Cytomegalovirus is the most common cause of congenital virally induced hearing loss. Maternal infection is most often asymptomatic as is the infection in the newborn. Hearing loss occurs in both clinically apparent infection and in the asymptomatic infection. Current methods of detection, treatment, and prevention and research efforts are…

  1. Discovering the Unexpected with the Utilization of NGS in Diagnostics of Non-syndromic Hearing Loss Disorders: The Family Case of ILDR1-Dependent Hearing Loss Disorder

    Directory of Open Access Journals (Sweden)

    Jernej Kovač

    2017-06-01

    Full Text Available Sensorineural hearing loss (SNHL is a heterogeneous family of hearing disabilities with congenital (including genetic as well as acquired etiology. Congenital SNHL of genetic etiology is further sub-divided into autosomal dominant, autosomal recessive and X-linked SNHL. More than 60 genes are involved in the etiology of autosomal recessive non-syndromic hearing loss (ARNSHL commonly manifesting as heterogeneous pre-lingual profound to severe non-progressive clinical phenotype. ILDR1-dependent ARNSHL (DFNB42, OMIM: # 609646 is a very rare sub-type of hearing disability, with unknown prevalence, caused by function-damaging genetic variants in ILDR1 gene reported in families of Middle-Eastern origin. ILDR1 (Immunoglobulin-Like Domain-containing Receptor 1 is involved in the development of semicircular canal, tricellular tight junction and auditory hair cells. An apparently non-consanguineous family of European ancestry with two affected siblings with profound progressive hearing loss characterized in their infancy and successfully treated with cochlear implants (CI is presented. Genetic analysis of common ARNSHL genetic causes in the population of origin was negative, thus the next-generation sequencing (NGS and family segregation analysis to identify underlying causative genetic variant was performed. Unexpectedly and atypical for the population of origin a homozygous non-sense variant ILDR1 c.942C > A (p.Cys314Ter inherited from both heterozygous parents was identified in both patients. Contrary to the commonly reported phenotype, indices of a progressive hearing loss and potential compensatory mechanism of vestibular function were revealed with the analysis of clinical data. The utilization of NGS was demonstrated as an invaluable tool for the detection of atypical rare variants in diagnostics of unidentified hearing loss disorders.

  2. COXPD9 an Evolving Multisystem Disease; Congenital Lactic Acidosis, Sensorineural Hearing Loss, Hypertrophic Cardiomyopathy, Cirrhosis and Interstitial Nephritis.

    Science.gov (United States)

    Bursle, C; Narendra, A; Chuk, R; Cardinal, J; Justo, R; Lewis, B; Coman, D

    2017-01-01

    We present the second report of combined oxidative phosphorylation deficiency-9. The infant presented in the neonatal period with poor feeding, lactic acidosis and sensorineural hearing loss. He subsequently developed a lethal hypertrophic cardiomyopathy during infancy. Cirrhosis and interstitial nephritis were identified at autopsy. Exome sequencing has detected compound heterozygous mutations in the MRPL3 gene which encodes a large mitochondrial ribosome subunit protein. We identified a known heterozygous variant NM_007208 c.950>G (Pro317Arg) in the MRPL3 gene and a novel heterozygous mutation NM_007208 c.49delC p.(Arg17Aspfs*57). Mutations in MRPL3 have previously been shown to alter ribosome assembly and cause abnormal function of multiple respiratory chain complexes. Our case adds to the evolving knowledge of disorders of mitochondrial translation.

  3. Hearing loss and enlarged internal auditory canal in children.

    Science.gov (United States)

    Santos, Saturnino; Domínguez, M Jesús; Cervera, Javier; Suárez, Alicia; Bueno, Antonio; Bartolomé, Margarita; López, Rafael

    2014-01-01

    Among the temporal bone abnormalities that can be found in the etiological study of paediatric sensorineural hearing loss (SNHL) by imaging techniques, those related to the internal auditory canal (IAC) are the least frequent. The most prevalent of these abnormalities that is associated with SNHL is stenotic IAC due to its association with cochlear nerve deficiencies. Less frequent and less concomitant with SNHL is the finding of an enlarged IAC (>8mm). Retrospective and descriptive review of clinical associations, imaging, audiological patterns and treatment of 9 children with hearing loss and enlarged IAC in the period 1999 to 2012. Two groups of patients are described. The first, without association with vestibulocochlear dysplasias, consisted of: 2 patients with SNHL without other temporal bone or systemic abnormalities, one with bilateral mixed HL from chromosome 18q deletion, one with a genetic X-linked DFN3 hearing loss, one with unilateral hearing loss in neurofibromatosis type 2 with bilateral acoustic neuroma, and one with unilateral hearing loss with cochlear nerve deficiency. The second group, with association with vestibulocochlear dysplasias, was comprised of: one patient with moderate bilateral mixed hearing loss in branchio-oto-renal syndrome, one with profound unilateral SNHL with recurrent meningitis, and another with profound bilateral SNHL with congenital hypothyroidism. The presence of an enlarged IAC in children can be found in different clinical and audiological settings with relevancies that can range from life-threatening situations, such as recurrent meningitis, to isolated hearing loss with no other associations. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  4. Congenitally blind humans use different stimulus selection strategies in hearing: an ERP study of spatial and temporal attention.

    Science.gov (United States)

    Röder, Brigitte; Krämer, Ulrike M; Lange, Kathrin

    2007-01-01

    Superior temporal processing skills, both in the auditory and tactile system have been reported in blind as compared to sighted humans. The present experiment tested whether blind people prefer, as a possible consequence, temporal rather than spatial stimulus selection strategies. Eight congenitally blind adults were tested in a selective attention experiment that simultaneously manipulated spatial and temporal attention. Participants had to attend to an auditory offset stimulus demarcating the end of a short (600 ms) or long (1200 ms) interval. They had to detect slightly less intense offset markers at the attended point in time presented in the left (half of the trials) or right (other half of the trials) hemifield. Event-related potentials (ERPs) were recorded throughout the experiment. Whereas the N1 was significantly enhanced by both spatial and temporal attention in the sighted (see Lange, Kramer, & Röder, 2006), only a temporal attention effect was found in the blind. Moreover, in both groups a second, longer lasting negativity was observed for offset markers presented at the attended as compared to the unattended spatial location. This negativity was modulated by temporal attention only in the blind. These results are consistent with the assumption of a higher priority of time for stimulus selection in the absence of vision from birth.

  5. Language Characteristics of Preschool Children with Hearing Loss in Tehran, Iran

    Directory of Open Access Journals (Sweden)

    Dr. Younes Lotfi

    2010-06-01

    Full Text Available Background and Aim: Hearing impairment affects all aspect of individual life, specially language and communication skills. When hearing impairment is congenital or occurs early in life, the child’s ability to learn optimally through audition, will be affected. The aim of this study was to evaluate linguistic skills of preschool hearing impaired children and compare these skills with normal peers.Methods: This descriptive cross-sectional study was performed on 38 preschool hearing impaired children that the main handicap was severe to profound hearing loss with ability to communicate orally and 28 normal children with the same cultural and social context. Twenty four non linguistic variables including age, gender, the age of entrance of preschool center, number of hearing aids, etc. were obtained by filling a questionnaire and fifteen linguistics variables including number of utterance, morphemes, correct utterance, noun phrase, ambiguous utterance, correct sentences, compound sentences, etc. were collected by some part of TOLD-P-3 test and three complementary questions. Then we compared the data from two groups.Results: There were significant differences between number of utterance, number of correct mean length utterance, number of well-formed sentences in normal and hearing impaired group (p0.05.Conclusion: This study showed a severe deficit in linguistic skills in preschool hearing impaired children.

  6. 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…

  7. Identification of congenital deafblindness

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2012-01-01

    . The study evaluated the assessment procedure of 190 children and adults found to be congenitally deafblind. Among the 190 individuals 76 percent were determined using functional assessment in addition to medical examination. A case example involving a 12-year-old child is also presented to illustrate...... the complexity in identifying congenital deafblindness. It is concluded that determining deafblindness should not be limited to medical procedures (vision and hearing tests) alone, but may also involve a lengthy process to assess the level of sense functioning the individual possesses....

  8. 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.

  9. 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

  10. Etiology and associated GJB2 mutations in Mauritanian children with non-syndromic hearing loss.

    Science.gov (United States)

    Moctar, Ely Cheikh Mohamed; Riahi, Zied; El Hachmi, Hala; Veten, Fatimetou; Meiloud, Ghlana; Bonnet, Christine; Abdelhak, Sonia; Errami, Mohammed; Houmeida, Ahmed

    2016-11-01

    Origins of all hearing impairment forms may be divided into genetic mutations and acquired influence. Both carry damage to the inner ear structure resulting in a mild to profound dysfunction of the auditory system. The purpose of this study was to assess the different etiologies of deafness in two reference centers for hearing-impaired children in Nouakchott/Mauritania. Data on gender, age, consanguinity, etiology and family history of deafness were gathered by interviewing the custodians of 139 children with hearing loss. DNA of pupils with hereditary non-syndromic deafness was then screened for GJB2 mutations by sequencing methods. Postnatal hearing loss was found in 36 (25.8 %) out of the 139 children surveyed. The main etiologies of this group were infections caused by meningitis (12.9 %) and measles (2.8 %). Unknown and ototoxic origins accounted for, respectively, 5.7 and 3.5 %. In 103 (74.1 %) children, deafness was identified near after the time of birth and, therefore, presumed as congenital. 56.8 % of deaf children had consanguineous parents. Two GJB2 mutations, c.del35G with an allele frequency of 4.7 % and R32C (3.7 %) were detected. Infections such as meningitis and measles were the most prevalent causes of postnatal deafness. In cases of congenital hearing impairment, two GJB2 allele variants, i.e., del35G and R32C (3.7 %) were detected. Extended genetic testing is recommended for a more comprehensive determination of congenital causes.

  11. Hearing loss

    Science.gov (United States)

    Decreased hearing; Deafness; Loss of hearing; Conductive hearing loss; Sensorineural hearing loss; Presbycusis ... Symptoms of hearing loss may include: Certain sounds seeming too loud Difficulty following conversations when two or more people are talking ...

  12. Early Diagnosis and Intervention for Hearing Loss in Newborns Discharged from Intensive Care Units: a Four-year Follow-up Study in North of Iran

    Directory of Open Access Journals (Sweden)

    Sima Tajik

    2016-08-01

    Full Text Available Background Hearing loss is the most common congenital disorder the incidence of which is further increased in the presence of risk factors for hearing loss among newborns admitted to the neonatal intensive care unit (NICU. The aim of this study was early diagnosis and intervention for hearing loss in newborns discharged from NICU. Materials and Methods This prospective cohort study was conducted on 3,362 newborns discharged from the NICU in several hospitals in Babol, Iran. Each newborn was evaluated through the transient evoked otoacoustic emission (T EOAE test. In the absence of any result, retests including TEOAE and diagnostic auditory brainstem response (ABR were conducted. In case of hearing loss, intervention programs including hearing aids fitting and cochlear implant were considered for infants. Each newborn infant was follow-up for four years. The infant’s age was also calculated during the hearing loss diagnosis and the intervention program. Results Sensorineural hearing loss (SNHL was diagnosed in 35 (1.04% of the infants at an average age of 105.65 + 96.72 days. Most of hearing loss diagnosis (51.43% was before the age of 3 months. Hearing aids were fitted for 25 infants (80.64% with a mean age of 9.61 + 7.64 months. Cochlear implants were done for two (8% children. At the end of the follow up, all of the children except one case (3.22% were able to use verbal communication. Conclusion Hearing screening of the high risk NICU graduate babies has reduced the age of hearing loss diagnosis to 3 months. The presence of severe to profound hearing loss in this population highlights the importance of early diagnosis and intervention.

  13. 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.

  14. 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...

  15. 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.

  16. Genome wide analysis in a family with sensorineural hearing loss, autism and mental retardation.

    Science.gov (United States)

    Mosrati, Mohamed Ali; Schrauwen, Isabelle; Kamoun, Hassen; Charfeddine, Ilhem; Fransen, Erik; Ghorbel, Abdelmonem; Van Camp, Guy; Masmoudi, Saber

    2012-12-01

    Hearing loss is a common congenital anomaly with an incidence of 1 in 1000 live births. It has been described together with several other clinical features as fortuitous association or commune genetic syndrome. In this study, we investigated a consanguineous Tunisian family with moderate to profound congenital hearing loss, mental retardation and autistic behaviors. We performed a genome wide microarray analysis study using approximately 300,000 SNPs in a common set of 7 invidious of this family. We identified regions of suggestive linkage with hearing loss on chromosomes 6p12 and 7q34. In addition, we identified a deletion on chromosome 8p in the two autistic individuals. This report presents an illustration of how consanguinity could increase familial clustering of multiple hereditary diseases within the same family. The application of next generation sequencing for this family seems to be a good strategy for further analysis leading to the identification of candidate genes. Copyright © 2012 Elsevier B.V. All rights reserved.

  17. Congenital and perinatal cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Chun Soo Kim

    2010-01-01

    Full Text Available Cytomegalovirus (CMV is currently the most common agent of congenital infection and the leading infectious cause of brain damage and hearing loss in children. Symptomatic congenital CMV infections usually result from maternal primary infection during early pregnancy. One half of symptomatic infants have cytomegalic inclusion disease (CID, which is characterized by involvement of multiple organs, in particular, the reticuloendothelial and central nervous system (CNS. Moreover, such involvement may or may not include ocular and auditory damage. Approximately 90% of infants with congenital infection are asymptomatic at birth. Preterm infants with perinatal CMV infection can have symptomatic diseases such as pneumonia, hepatitis, and thrombocytopenia. Microcephaly and abnormal neuroradiologic imaging are associated with a poor prognosis. Hearing loss may occur in both symptomatic and asymptomatic infants with congenital infection and may progress through childhood. Congenital infection is defined by the isolation of CMV from infants within the first 3 weeks of life. Ganciclovir therapy can be considered for infants with symptomatic congenital CMV infection involving the CNS. Pregnant women of seronegative state should be counseled on the importance of good hand washing and other control measures to prevent CMV infection. Heat treatment of infected breast milk at 72?#608;for 5 seconds can eliminate CMV completely.

  18. Congenital Hypothyroidism

    Science.gov (United States)

    ... Disease Featured Resource Find an Endocrinologist Search Congenital Hypothyroidism March 2012 Download PDFs English Espanol Editors Rosalind S. ... Resources MedlinePlus (NIH) Mayo Clinic What is congenital hypothyroidism? Newborn babies who are unable to make enough ...

  19. Congenital toxoplasmosis

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/001360.htm Congenital toxoplasmosis To use the sharing features on this page, please enable JavaScript. Congenital toxoplasmosis is a group of symptoms that occur when ...

  20. [Study on multi-area universal newborn hearing screening in countryside of China].

    Science.gov (United States)

    Huang, Lihui; Cai, Zhenghua; Zhang, Hua; Peng, Shichun; Wu, Dongsheng; Wang, Lei; Fan, Weiping; Yang, Rulan; Huang, Yan; Luo, Xia; Tu, Lin; En, Hui; Qi, Beier; Zhen, Yong; Han, Demin

    2009-08-01

    To investigate the feasibility of universal newborn hearing screening in countryside in order to provide reliable evidence in launching this program all over the countryside of China. Subjects were 12,638 infants who were born in 9 counties from Jan 2004 to Dec 2005. TEOAE was used for the fast hearing screening. Infants were screened on the 2-7 days after the birth. The re-screening was conducted in 4-6 weeks if failed in the initial screening, and follow-up were provided continually if they also failed in the re-screening. Ten thousand eight hundred and forty-five of 12,638 (85.8%) were screened including 9,963 (91.9%) normal newborns and 882 (8.1%) newborns with high-risk. Seven thousand four hundred and fifty (68.7%) newborns passed the initial screening, and 3,395 (31.3%) people failed. One thousand seven hundred and ninety-three (14.2%) infants were refused to be screened. Only 2,536 (74.7%) were re-screened on time, and 859 (25.3%) did not receive re-screening. One hundred and twenty were failed in the re-screening or first screening, and 79 (65.8%) of them received diagnostic assessment. Among the infants received diagnostic assessment, 6 (7.6%) cases were found to have profound hearing loss in both ears, 9 (11.4%) cases were found to be severe hearing loss (7 in both ears and 2 in single ear), 11 (13.9%) cases were found to be moderate hearing loss (5 in both ear and 6 in single ear), 26 (32.9%) were found to have slight hearing loss (11 in both ear and 15 in single ears), and 27 (34.2%) were normal. Fifty-two infants were diagnosed as hearing loss with a prevalence of congenital hearing loss (in binaural and monaural) of 0.5% (52/10845) and a prevalence of bilateral hearing loss of 0.3% (29/10845). A prevalence of congenital hearing loss was 0.2% (22/9,963) in well infants and 3.4% (30/882) in high risk infants. Among the 13 cases of children with severe and profound hearing loss in both ears children, 8 (61.5%) cases were fitted with hearing aids and 1

  1. [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

  2. 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,…

  3. Identifying Hearing Loss By Means Of Iridology | Stearn | African ...

    African Journals Online (AJOL)

    Isolated reports of hearing loss presenting as markings on the iris exist, but to date the effectiveness of iridology to identify hearing loss has not been investigated. This study therefore aimed to determine the efficacy of iridological analysis in the identification of moderate to profound sensorineural hearing loss in adolescents ...

  4. 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.

  5. The BAHA Softband. A new treatment for young children with bilateral congenital aural atresia.

    NARCIS (Netherlands)

    Hol, M.K.S.; Cremers, C.W.R.J.; Coppens-Schellekens, W.; Snik, A.F.M.

    2005-01-01

    OBJECTIVE: To evaluate the validity of a bone-anchored hearing aid (BAHA) Softband (fitted unilaterally and bilaterally) in young children with bilateral congenital aural atresia. SUBJECTS: Two children with severe bilateral congenital conductive hearing loss, who had been fitted with a

  6. Profound mixed hearing loss: bone-anchored hearing aid system or cochlear implant?

    NARCIS (Netherlands)

    Verhaegen, V.J.O.; Mulder, J.J.S.; Mylanus, E.A.M.; Cremers, C.W.R.J.; Snik, A.F.M.

    2009-01-01

    OBJECTIVES: We evaluated 5 patients who were changed over from a Baha Cordelle to a cochlear implant (CI). Moreover, the level of phoneme score was determined at which a Baha Cordelle user was better helped with a CI. METHODS: We offer descriptive case reports and a retrospective evaluation of

  7. Influence of Implantable Hearing Aids and Neuroprosthesison Music Perception

    OpenAIRE

    Rahne, Torsten; Böhme, Lars; Götze, Gerrit

    2012-01-01

    The identification and discrimination of timbre are essential features of music perception. One dominating parameter within the multidimensional timbre space is the spectral shape of complex sounds. As hearing loss interferes with the perception and enjoyment of music, we approach the individual timbre discrimination skills in individuals with severe to profound hearing loss using a cochlear implant (CI) and normal hearing individuals using a bone-anchored hearing aid (Baha). With a recent de...

  8. From Individualism to Co-Construction and Back Again: Rethinking Research Methodology for Children with Profound and Multiple Learning Disabilities

    Science.gov (United States)

    Simmons, Ben; Watson, Debbie

    2015-01-01

    Children with profound and multiple learning disabilities (PMLD) are said to experience severe congenital impairments to consciousness and cognition stemming from neurological damage. Such children are understood as operating at the pre-verbal stages of development, and research in the field typically draws conceptual resources from psychology to…

  9. Hearing Screening

    Science.gov (United States)

    Johnson-Curiskis, Nanette

    2012-01-01

    Hearing levels are threatened by modern life--headsets for music, rock concerts, traffic noises, etc. It is crucial we know our hearing levels so that we can draw attention to potential problems. This exercise requires that students receive a hearing screening for their benefit as well as for making the connection of hearing to listening.

  10. About Hearing

    Science.gov (United States)

    ... with unilateral hearing should be monitored closely for linguistic, educational, or social gaps. For more information about ... see: Info to Go/Hearing Aids For more information about cochlear implants, see: Info ... resources about hearing, see: Hearing and Amplification (My Baby's ...

  11. 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

  12. 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.

  13. Advances in congenital and postnatal cytomegalovirus infections

    NARCIS (Netherlands)

    Gunkel, J.

    2017-01-01

    Congenital CMV infection (cCMV) is the most prevalent viral infection worldwide and the leading cause of non-genetic sensorineural hearing loss. Early diagnosis of cCMV infection is advantageous as it allows for regular follow-up and timely intervention in case of late-onset symptoms among

  14. Development of Joint Engagement in Young Deaf and Hearing Children: Effects of Chronological Age and Language Skills

    Science.gov (United States)

    Cejas, Ivette; Barker, David H.; Quittner, Alexandra L.; Niparko, John K.

    2014-01-01

    Purpose: To evaluate joint engagement (JE) in age-matched children with and without hearing and its relationship to oral language skills. Method: Participants were 180 children with severe-to-profound hearing loss prior to cochlear implant surgery, and 96 age-matched children with normal hearing; all parents were hearing. JE was evaluated in a…

  15. The economics of screening infants at risk of hearing impairment: an international analysis.

    Science.gov (United States)

    Burke, Martyn J; Shenton, Ruth C; Taylor, Matthew J

    2012-02-01

    Hearing impairment in children across the world constitutes a particularly serious obstacle to their optimal development and education, including language acquisition. Around 0.5-6 in every 1000 neonates and infants have congenital or early childhood onset sensorineural deafness or severe-to-profound hearing impairment, with significant consequences. Therefore, early detection is a vitally important element in providing appropriate support for deaf and hearing-impaired babies that will help them enjoy equal opportunities in society alongside all other children. This analysis estimates the costs and effectiveness of various interventions to screen infants at risk of hearing impairment. The economic analysis used a decision tree approach to determine the cost-effectiveness of newborn hearing screening strategies. Two unique models were built to capture different strategic screening decisions. Firstly, the cost-effectiveness of universal newborn hearing screening (UNHS) was compared to selective screening of newborns with risk factors. Secondly, the cost-effectiveness of providing a one-stage screening process vs. a two-stage screening process was investigated. Two countries, the United Kingdom and India, were used as case studies to illustrate the likely cost outcomes associated with the various strategies to diagnose hearing loss in infants. In the UK, the universal strategy incurs a further cost of approximately £2.3 million but detected an extra 63 cases. An incremental cost per case detected of £36,181 was estimated. The estimated economic burden was substantially higher in India when adopting a universal strategy due to the higher baseline prevalence of hearing loss. The one-stage screening strategy accumulated an additional 13,480 and 13,432 extra cases of false-positives, in the UK and India respectively when compared to a two-stage screening strategy. This represented increased costs by approximately £1.3 million and INR 34.6 million. The cost

  16. 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.

  17. 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

  18. Relationship between hearing complaint and hearing loss among older people

    Directory of Open Access Journals (Sweden)

    Teixeira, Adriane Ribeiro

    2009-03-01

    Full Text Available Introduction: Presbycusis is a public health problem. Despite its high prevalence, many elders do not have their hearing ability investigated periodically, because they do not have a specific complaint. Objective: To check whether there is a relationship between the complaint and the presence of hearing loss in elder people. Method: Transversal study in elders from a neighborhood in the city of Canoas, Rio Grande do Sul. After the definition of the neighborhood's geographic boundaries, all houses were visited, the older people's addresses were ascertained and the invitations to take part in the research were provided. A questionnaire survey was applied which had a question about hearing loss complaint and air-conducted hearing thresholds were obtained and studied. Out of the 72 identified elders 50 elders agreed to participate, 35 (70% women, and 15 (30% men. Results: It was confirmed that only 12 (24% elders showed a specific complaint of hearing loss, although 33 (66% elders showed slight, moderate, severe and profound hearing losses. Conclusion: Data analysis confirmed there was no relationship between the complaint and the presence of hearing loss in the assessed group, and demonstrated the need to forward the elders for audiological evaluation even without any specific complaint.

  19. 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...

  20. Congenital Scoliosis.

    Science.gov (United States)

    Moramarco, M; Weiss, H R

    2015-11-17

    Congenital scoliosis is a lateral deformity of the spine with a disturbance of the sagittal profile caused by malformations of vertebra and ribs. Typically, early surgical intervention is the suggested treatment (before three-years-old) for young patients with congenital scoliosis. While a previous study was conducted in 2011 to investigate long-term studies supporting the necessity for this recommendation and no evidence was found , this current review, is an updated search for evidence published from 2011 through March 2015. This also failed to find any prospective or randomized controlled studies to support the hypothesis that spinal fusion surgery in patients with congenital scoliosis should be considered as evidence-based treatment. Contradictory results exist on the safety of hemivertebra resection and segmental fusion using pedicle screw fixation. When using the VEPTR (vertical expandable prosthetic titanium rib) device, studies show a high rate of complications exist. It is difficult to predict the final outcome for patients with congenital scoliosis. However, it is possible that many patients with congenital scoliosis may be able to avoid spinal surgery with the application of advanced bracing technology [5]. Therefore, it is only prudent to advocate for conservative management first before spinal surgery is considered.

  1. Homozygosity Mapping Reveals Mutations of GRXCR1 as a Cause of Autosomal-Recessive Nonsyndromic Hearing Impairment

    Science.gov (United States)

    Schraders, Margit; Lee, Kwanghyuk; Oostrik, Jaap; Huygen, Patrick L.M.; Ali, Ghazanfar; Hoefsloot, Lies H.; Veltman, Joris A.; Cremers, Frans P.M.; Basit, Sulman; Ansar, Muhammad; Cremers, Cor W.R.J.; Kunst, Henricus P.M.; Ahmad, Wasim; Admiraal, Ronald J.C.; Leal, Suzanne M.; Kremer, Hannie

    2010-01-01

    We identified overlapping homozygous regions within the DFNB25 locus in two Dutch and ten Pakistani families with sensorineural autosomal-recessive nonsyndromic hearing impairment (arNSHI). Only one of the families, W98-053, was not consanguineous, and its sibship pointed toward a reduced critical region of 0.9 Mb. This region contained the GRXCR1 gene, and the orthologous mouse gene was described to be mutated in the pirouette (pi) mutant with resulting hearing loss and circling behavior. Sequence analysis of the GRXCR1 gene in hearing-impaired family members revealed splice-site mutations in two Dutch families and a missense and nonsense mutation, respectively, in two Pakistani families. The splice-site mutations are predicted to cause frameshifts and premature stop codons. In family W98-053, this could be confirmed by cDNA analysis. GRXCR1 is predicted to contain a GRX-like domain. GRX domains are involved in reversible S-glutathionylation of proteins and thereby in the modulation of activity and/or localization of these proteins. The missense mutation is located in this domain, whereas the nonsense and splice-site mutations may result in complete or partial absence of the GRX-like domain or of the complete protein. Hearing loss in patients with GRXCR1 mutations is congenital and is moderate to profound. Progression of the hearing loss was observed in family W98-053. Vestibular dysfunction was observed in some but not all affected individuals. Quantitative analysis of GRXCR1 transcripts in fetal and adult human tissues revealed a preferential expression of the gene in fetal cochlea, which may explain the nonsyndromic nature of the hearing impairment. PMID:20137778

  2. Hearing Problems

    Science.gov (United States)

    ... Read MoreDepression in Children and TeensRead MoreBMI Calculator Hearing ProblemsLoss in the ability to hear or discriminate ... This flow chart will help direct you if hearing loss is a problem for you or a ...

  3. 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

  4. Effects of diving on hearing and balance

    OpenAIRE

    Goplen, Frederik Kragerud

    2011-01-01

    Diving has profound effects on the human body including the inner ear, which contains the organs of hearing and balance. Ear injury is the most common medical problem in diving. The aim of this work was to explore the short and long-term effects of diving on hearing and balance through a simulated deep saturation dive and two epidemiological studies on commercial divers. The first study explored short-term effects of absolute pressure on the body. The high-pressure neurologi...

  5. Does hearing aid use affect audiovisual integration in mild hearing impairment?

    Science.gov (United States)

    Gieseler, Anja; Tahden, Maike A S; Thiel, Christiane M; Colonius, Hans

    2018-02-16

    There is converging evidence for altered audiovisual integration abilities in hearing-impaired individuals and those with profound hearing loss who are provided with cochlear implants, compared to normal-hearing adults. Still, little is known on the effects of hearing aid use on audiovisual integration in mild hearing loss, although this constitutes one of the most prevalent conditions in the elderly and, yet, often remains untreated in its early stages. This study investigated differences in the strength of audiovisual integration between elderly hearing aid users and those with the same degree of mild hearing loss who were not using hearing aids, the non-users, by measuring their susceptibility to the sound-induced flash illusion. We also explored the corresponding window of integration by varying the stimulus onset asynchronies. To examine general group differences that are not attributable to specific hearing aid settings but rather reflect overall changes associated with habitual hearing aid use, the group of hearing aid users was tested unaided while individually controlling for audibility. We found greater audiovisual integration together with a wider window of integration in hearing aid users compared to their age-matched untreated peers. Signal detection analyses indicate that a change in perceptual sensitivity as well as in bias may underlie the observed effects. Our results and comparisons with other studies in normal-hearing older adults suggest that both mild hearing impairment and hearing aid use seem to affect audiovisual integration, possibly in the sense that hearing aid use may reverse the effects of hearing loss on audiovisual integration. We suggest that these findings may be particularly important for auditory rehabilitation and call for a longitudinal study.

  6. [The effects of hearing level on auditory performance in infants with hearing aids].

    Science.gov (United States)

    Chen, Xueqing; Li, Jing; Wu, Yanjun; Zhang, Hua; Liu, Haihong; Wang, Shuo; Kong, Ying; Liu, Sha; Liu, Bo; Mo, Lingyan; Qi, Beier

    2012-12-01

    The purpose of this study is to investigate the effects of hearing level on auditory performance in infants with hearing aids and provide a clinical database for their hearing and speech habilitation. A total of 32 infants with prelingual hearing loss participated in this study, ranging in age at hearing aid fitting from 3 to 34 months with a mean of 16 months. According to their hearing level, they were divided into three groups. Infants in group A were with moderate hearing loss (41-60 dB HL). Infants in group B were with severe hearing loss (61-80 dB HL). Infants in group C were with profound hearing loss (>81 dB HL). The infant-toddler meaningful auditory integration scale (IT-MAIS) was used to evaluate their auditory performance. The evaluation was performed before hearing aid fitting and 1, 3, 6, 9, 12 months after fitting. The mean scores of auditory performance showed significant improvements with time of hearing aid use for the three groups of infants (P hearing aid fitting and at 1 month after fitting (P hearing aid fitting and at 1, 3 months after fitting (P hearing aid fitting and at 1, 3, 6 months after fitting (P 0.05). However, significant differences were observed between group A and C at 3, 6, 9, 12 months after fitting (P hearing loss developed significantly with the use of hearing aids within the first year after fitting. The degree of hearing loss in infants had significant influence on the development of auditory performance.

  7. Rehabilitation of patients with conductive hearing loss and moderate mental retardation by means of a bone-anchored hearing aid.

    NARCIS (Netherlands)

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

    2006-01-01

    OBJECTIVE: To evaluate whether the bone-anchored hearing aid (BAHA) can be applied successfully to patients with conductive hearing loss and moderate mental retardation. STUDY DESIGN: Retrospective clinical evaluation. SETTING: Tertiary referral center. PATIENTS: Twenty-two patients with congenital

  8. Congenital hemangioma.

    Science.gov (United States)

    Amouri, Meriem; Mesrati, Hela; Chaaben, Hend; Masmoudi, Abderrahmen; Mseddi, Madiha; Turki, Hamida

    2017-01-01

    Congenital hemangiomas (CHs) are fully developed hemangiomas that are present at birth. There are 2 possible types: rapidly involuting CHs (RICHs) and noninvoluting CHs (NICHs). We conducted a retrospective study (2008-2012) of 6 patients (2 females, 4 males) with CHs (mean age, 16 days). We analyzed the epidemiology, clinical characteristics, and clinical outcome of CHs over this 5-year period.

  9. Congenital Defects.

    Science.gov (United States)

    Goldman, Allen S.; And Others

    There are two general categories (not necessarily mutually exclusive) of congenital defects: (1) abnormalities that have an hereditary basis, such as single and multiple genes, or chromosomal abberration; and (2) abnormalities that are caused by nonhereditary factors, such as malnutrition, maternal disease, radiation, infections, drugs, or…

  10. Congenital amusias.

    Science.gov (United States)

    Tillmann, B; Albouy, P; Caclin, A

    2015-01-01

    In contrast to the sophisticated music processing reported in the general population, individuals with congenital amusia show deficits in music perception and production. Congenital amusia occurs without brain damage, sensory or cognitive deficits, and has been suggested as a lifelong deficit with genetic origin. Even though recognized for a long time, this disorder has been systematically studied only relatively recently for its behavioral and neural correlates. The currently most investigated hypothesis about the underlying deficits concerns the pitch dimension, notably with impaired pitch discrimination and memory. Anatomic and functional investigations of pitch processing revealed that the amusic brain presents abnormalities in the auditory and inferior frontal cortices, associated with decreased connectivity between these structures. The deficit also impairs processing of pitch in speech material and processing of the time dimension in music for some of the amusic individuals, but does not seem to affect spatial processing. Some studies suggest at least partial dissociation in the disorder between perception and production. Recent studies revealed spared implicit pitch perception in congenital amusia, supporting the power of implicit cognition in the music domain. Current challenges consist in defining different subtypes of congenital amusia as well as developing rehabilitation programs for this "musical handicap." © 2015 Elsevier B.V. All rights reserved.

  11. Auditory, Visual, and Auditory-Visual Perceptions of Emotions by Young Children with Hearing Loss versus Children with Normal Hearing

    Science.gov (United States)

    Most, Tova; Michaelis, Hilit

    2012-01-01

    Purpose: This study aimed to investigate the effect of hearing loss (HL) on emotion-perception ability among young children with and without HL. Method: A total of 26 children 4.0-6.6 years of age with prelingual sensory-neural HL ranging from moderate to profound and 14 children with normal hearing (NH) participated. They were asked to identify…

  12. 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.

  13. An economic model of adult hearing screening

    Directory of Open Access Journals (Sweden)

    A. Morris

    2011-03-01

    Full Text Available Populations are ageing and older adults make an increasing contribution to society, yet uncorrected hearing loss is common over the age of 50 years, increasing in prevalence and severity with age. The consequences of uncorrected hearing loss can be profound for hearing-impaired individuals and their communication partners but there is evidence that adults commonly delay 10-15 years before seeking help for hearing difficulty (Stephens et al., 1990; Davis et al., 2007 and the most common reason is the belief that their hearing is not bad enough (Ipsos-Mori/RNID survey, 2005. Hearing aids are currently the mainstay of intervention for hearing loss; evidence shows benefit to social functioning and quality of life even for mild hearing loss (Mulrow et al., 1990; Chisolm et al., 2007 and long term outcomes are better when they are obtained early (Davis et al., 2007. Screening adults for hearing loss would expedite intervention and reduce unmet need, leading to improved quality of life for many older adults. Previous work suggests adult hearing screening (AHS should target adults aged 50-65 years, old enough for prevalence to justify screening but young enough to gain from early intervention...

  14. Comparison of Performance of Transcranial Contralateral Routing of Signal, Pre-Implanted Trimmer Digital and Digital Bone Anchored Hearing Aid in Adults with Unilateral Hearing Loss

    OpenAIRE

    Devi, Neelamegarajan; Chatni, Suma; Ramadevi, Kasetty Jagannathaiah S.; Fakruddin, Darga Baba

    2015-01-01

    Individuals with unilateral hearing loss of severe-profound degree face listening difficulties while localizing a sound source and while perceiving speech in the presence of noise. The objective was to compare the efficacy of the digitally programmable bone anchored hearing aid (BAHA), trimmer digital BAHA and the transcranial contralateral routing of signal (T-CROS) in improving the listening performance in adults with unilateral hearing loss. Twenty-four adults with unilateral hearing loss ...

  15. 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.

  16. Music: A Tool for Expressive and Receptive Vocabulary for Children Who are Deaf or Hard of Hearing

    OpenAIRE

    Smith, Lauren

    2015-01-01

    Children with congenital hearing loss are at risk for speech, language, and academic delays. Early identification of hearing loss provides the opportunity for children who are deaf or hard of hearing (DHH) to obtain appropriate technology, such as hearing aids or cochlear implants, and to receive early intervention services to optimize development of listening and spoken language. Almost all industrialized countries have adopted policies for universal newborn hearing screening (National Cente...

  17. Pregnancy in a Woman With Congenital Generalized Lipodystrophy

    Science.gov (United States)

    Maguire, Marcy; Lungu, Andrea; Gorden, Phillip; Cochran, Elaine; Stratton, Pamela

    2012-01-01

    BACKGROUND Congenital generalized lipodystrophy is a rare disorder characterized by scant adipose tissue, profound leptin deficiency, and severe insulin resistance, resulting in multiple metabolic derangements, including hyperandrogenism, anovulation, and impaired fecundity. CASE A young woman with congenital generalized lipodystrophy receiving leptin therapy experienced menarche, conceived spontaneously, and delivered a liveborn male neonate. CONCLUSION Adipose tissue is important to normal female reproductive function. Leptin in particular appears to play a key role in adipose-mediated regulation of fertility. PMID:22270436

  18. [Congenital hydrocephalus].

    Science.gov (United States)

    Malagón-Valdez, J

    2006-04-10

    Congenital hydrocephalus or ventriculomegaly is a disorder that now can be diagnosed in uterus with ultrasonography, this gives the chance of being able to give a treatment the earliest as possible. The clinical manifestations are reviewed, the diagnosis, the frequent treatment and causes of congenital hydrocephalus, being the first agenesis of the Sylvius' aqueduct, followed by Arnold-Chiari's malformations with mielomeningocele. In most of the cases the peritoneal-ventricle shunt is the best surgery treatment and now, the treatment with ventriculostomy of third ventricle by endoscopy has fewer complications apparently and in several cases it is the definitive treatment. The evolution of the diagnosis with the support of specific therapies is effective and the early treatment is good, of course taking into account the etiology.

  19. Congenital rubella syndrome in Iran

    Directory of Open Access Journals (Sweden)

    Eftekhar Hasan

    2005-06-01

    Full Text Available Abstract Background Congenital rubella syndrome (CRS can be prevented with appropriate vaccination programs. The prevalence rates of rubella and CRS in Iran are unknown; therefore, the risk of exposure in pregnant women is not clear. The prevalence of CRS in the pre-vaccine period can be estimated by evaluating the proportion of children in the population with sensorineural hearing loss attributable to rubella. Methods This was a case-control study to estimate prevalence of CRS in Tehran (Iran by evaluating the proportion of children with sensorineural hearing loss attributable to rubella. The study used rubella antibody titer as an indicator, and compared the prevalence of rubella antibody between children with and without sensorineural hearing loss. Using these findings, the proportion of cases of sensorineural hearing loss attributable to rubella was estimated. Results A total of 225 children aged 1 to 4 years were entered into the study (113 cases and 112 controls. There was a significant difference between cases and controls with regard to rubella antibody seropositivity (19.5% vs. 8.9%, respectively, odds ratio = 2.47, 95% CI = 1.04–5.97. The proportion of sensorineural hearing loss cases attributable to rubella was found to be 12%, corresponding to a CRS prevalence of 0.2/1000. Conclusion The prevalence of CRS was approximately 0.2/1000 before rubella vaccination in Iran, Moreover; the results suggest that implementation of appropriate rubella vaccination programs could potentially prevent about 12% of cases of sensorineural hearing loss in Iranian children. This data could potentially be used as baseline data, which in conjunction with an appropriate method, to establish a surveillance system for rubella vaccination in Iran. An appropriate surveillance system is needed, because the introduction of a rubella vaccine without epidemiological data and an adequate monitoring program could result in the shifting of rubella cases to higher

  20. [Congenital aniridia].

    Science.gov (United States)

    Chiruţa, Daria; Stan, Cristina

    2014-01-01

    Aniridia is a rare congenital, hereditary, bilateral disease which is associated with various systemic and ocular defects. We present the case of a 61 year old patient who was admitted in the hospital of ophthalmology Cluj Napoca, for the symptoms caused by the ocular defects associated with aniridia. In this case, aniridia is autosomal dominant transmitted with incomplete penetrance and it is not accompanied by any systemic defects. The disease also affects three of her sons and two nephews of the patient.

  1. Congenital diplopodia

    Energy Technology Data Exchange (ETDEWEB)

    Brower, Jason S.; Wootton-Gorges, Sandra L.; Costouros, John G.; Boakes, Jennette; Greenspan, Adam [University of California, Davis, Department of Radiology, 4860 Y. Street, Suite 3100, CA 95817, Davis (United States)

    2003-11-01

    Diplopodia, or duplicated foot, is a rare congenital anomaly. It differs from polydactyly in that supernumerary metatarsal and tarsal bones are present as well as extra digits. Only a few cases of this anomaly have been reported in the literature to date. We present a newborn male without intrauterine teratogen exposure who was born with a duplicate foot of the left lower extremity and imperforate anus. (orig.)

  2. Types of Hearing Aids

    Science.gov (United States)

    ... Consumer Devices Consumer Products Hearing Aids Types of Hearing Aids Share Tweet Linkedin Pin it More sharing ... are some features for hearing aids? What are hearing aids? Hearing aids are sound-amplifying devices designed ...

  3. Congenital oval or round window malformations in children : Surgical findings and results in 17 cases

    NARCIS (Netherlands)

    Vincent, Robert; Wegner, Inge; Derks, Laura S M; Grolman, Wilko

    2016-01-01

    OBJECTIVES/HYPOTHESIS: To prospectively evaluate surgical findings and hearing results in children undergoing surgery for congenital oval or round window malformations (class IV malformations). STUDY DESIGN: A nonrandomized, nonblinded, case series of prospectively collected data. METHODS: Fourteen

  4. [The assessment of hearing impairment in patients over 60 years of age using hearing aids].

    Science.gov (United States)

    Skrzypek, Aleksandra; Sekula, Alicja; Deryło, Maria Bratumiła; Kuśmierczyk, Joanna; Talar, Marcin

    2014-01-01

    To assess the hearing impairment in people over 60 years old using hearing aids. This was a single-center study, but it is planned to extend it further to the whole country. The study was focused on patients with hearing aids. During the assessment 57 people were included in the observation in order to control the status of their hearing loss and benefit from traditional hearing aids as well as the possibility to apply the auditory implants in case of a little benefit from hearing aids. The otoscopy and pure tone audiometry were performed as well as the questionnaires on demographic and epidemiological data of patients were collected as well as the quality of their life with hearing aids was subjectively assessed. The results show that 91% of patients have sensorineural hearing loss (SHL), the remaining 9%--severe mixed hearing loss. Severe SHL was found in 22 patients, the moderate hearing loss was observed in 37%, and the profound SHL was the case in 5 patients. Minimal SHL was observed in 7% of patients (n=4). More than 73% of the study subjects were male (n=38). The average age of the patients who completed the survey was 74 years old. Thirty-five patients used their hearing aid over 3 years and less than 70% of them used it every day all day. Hearing aid was not actively used by 10 patients. Over the last year 51.92% of the patients underwent a hearing examination. The bone anchored hearing aid was suggested to 2% of subjects and the cochlear implant was offered to 10 patients. The data analysis shows the need to educate and inform the elderly about alternative methods of hearing loss treatment. Copyright © 2013 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z.o.o. All rights reserved.

  5. A Study of Rate of Speech and Intelligibility of Speech in Hearing impaired Pupils of Baghcheban School of Zahedan, Iran

    Directory of Open Access Journals (Sweden)

    Mahla SAEDI

    2013-12-01

    Full Text Available Hearing loss results in disruption of coordination in muscles and structures engaged in speech timing tasks and consequently acquisition of speech sounds is affected. The purpose of this study is to investigate and compare the speed and intelligibility of speech in hearing impaired and normal hearing Persian speaking elementary school boys and girls. 33 hearing impaired students are randomly selected from three hearing impaired groups (profound, severe and moderately-severe and compared with 60 normal hearing students. The speed of speech was measured according to reading Words Per Minute (WPM, and speech intelligibility was tested by a 5-rank scale method. As expected, the normal hearing had more speed and intelligibility of speech in contrast to the hearing impaired group. Also hearing impaired boys had a better speed and intelligibility of speech compared to hearing impaired girls but in normal hearing group, girls had better speed. The amount of P-value for moderately-severe and sever hearing impaired was 0.006 and this amount for profound and moderately-severe hearing impaired was 0.002, so p-value is <0.05 and significantly important. Profound hearing impaired group read the text more slowly and had lower speech intelligibility than other two hearing impaired groups.

  6. Language Characteristics of Preschool Children with Hearing Loss in Tehran, Iran

    OpenAIRE

    Dr. Younes Lotfi; Talieh Zarifian; Saeideh Mehrkian; Dr. Mehdi Rahgozar

    2010-01-01

    Background and Aim: Hearing impairment affects all aspect of individual life, specially language and communication skills. When hearing impairment is congenital or occurs early in life, the child’s ability to learn optimally through audition, will be affected. The aim of this study was to evaluate linguistic skills of preschool hearing impaired children and compare these skills with normal peers.Methods: This descriptive cross-sectional study was performed on 38 preschool hearing impaired chi...

  7. How Well Can Centenarians Hear?

    Science.gov (United States)

    Mao, Zhongping; Zhao, Lijun; Pu, Lichun; Wang, Mingxiao; Zhang, Qian; He, David Z. Z.

    2013-01-01

    With advancements in modern medicine and significant improvements in life conditions in the past four decades, the elderly population is rapidly expanding. There is a growing number of those aged 100 years and older. While many changes in the human body occur with physiological aging, as many as 35% to 50% of the population aged 65 to 75 years have presbycusis. Presbycusis is a progressive sensorineural hearing loss that occurs as people get older. There are many studies of the prevalence of age-related hearing loss in the United States, Europe, and Asia. However, no audiological assessment of the population aged 100 years and older has been done. Therefore, it is not clear how well centenarians can hear. We measured middle ear impedance, pure-tone behavioral thresholds, and distortion-product otoacoustic emission from 74 centenarians living in the city of Shaoxing, China, to evaluate their middle and inner ear functions. We show that most centenarian listeners had an “As” type tympanogram, suggesting reduced static compliance of the tympanic membrane. Hearing threshold tests using pure-tone audiometry show that all centenarian subjects had varying degrees of hearing loss. More than 90% suffered from moderate to severe (41 to 80 dB) hearing loss below 2,000 Hz, and profound (>81 dB) hearing loss at 4,000 and 8,000 Hz. Otoacoustic emission, which is generated by the active process of cochlear outer hair cells, was undetectable in the majority of listeners. Our study shows the extent and severity of hearing loss in the centenarian population and represents the first audiological assessment of their middle and inner ear functions. PMID:23755251

  8. How well can centenarians hear?

    Directory of Open Access Journals (Sweden)

    Zhongping Mao

    Full Text Available With advancements in modern medicine and significant improvements in life conditions in the past four decades, the elderly population is rapidly expanding. There is a growing number of those aged 100 years and older. While many changes in the human body occur with physiological aging, as many as 35% to 50% of the population aged 65 to 75 years have presbycusis. Presbycusis is a progressive sensorineural hearing loss that occurs as people get older. There are many studies of the prevalence of age-related hearing loss in the United States, Europe, and Asia. However, no audiological assessment of the population aged 100 years and older has been done. Therefore, it is not clear how well centenarians can hear. We measured middle ear impedance, pure-tone behavioral thresholds, and distortion-product otoacoustic emission from 74 centenarians living in the city of Shaoxing, China, to evaluate their middle and inner ear functions. We show that most centenarian listeners had an "As" type tympanogram, suggesting reduced static compliance of the tympanic membrane. Hearing threshold tests using pure-tone audiometry show that all centenarian subjects had varying degrees of hearing loss. More than 90% suffered from moderate to severe (41 to 80 dB hearing loss below 2,000 Hz, and profound (>81 dB hearing loss at 4,000 and 8,000 Hz. Otoacoustic emission, which is generated by the active process of cochlear outer hair cells, was undetectable in the majority of listeners. Our study shows the extent and severity of hearing loss in the centenarian population and represents the first audiological assessment of their middle and inner ear functions.

  9. A preliminary study of some pragmatic skills of hearing and hearing-impaired children by story retelling test

    Directory of Open Access Journals (Sweden)

    Farnoush Jarollahi

    2013-03-01

    Full Text Available Background and Aim: Pragmatics refers to speech interactions and the social aspect of communication in language contexts. Due to the crucial role of hearing in language skill development, hearing-impaired children have problems with all aspects of language, including pragmatics. These skills are crucial in children's daily life. There is a lack of Persian studies on hearing-impaired children. Therefore, the purpose of the present study is to describe some pragmatic skills of hearing and hearing-impaired children.Methods: This descriptive case study was conducted on five hearing-impaired and two normal-hearing 6 year old children. Their pragmatic language skills were studied by the Persian story retelling test. in a quiet room. The children's re-told story was recorded, analyzed, and scored.Results: There was no difference between the scores of hearing and severe hearing-impaired children. However, children with severe hearing loss and cochlear implant, and hearing children were different in maintaining the subject and sequence of events. Children with profound hearing loss and hearing aid, and hearing children had a significant difference in all aspects except main information. All subjects used conjunctions correctly.Conclusion: The pragmatic skills of hearing-impaired children are weaker than hearing children. There are also differences between hearing-impaired children's abilities. This difference in pragmatic skills shows the difference in amount of hearing loss, kind of assistive device, effective use of remaining hearing, onset and quality of aural rehabilitation program, and other factors. Therefore, a research with a greater sample size is necessary to explain these differences.

  10. Reversible induction of phantom auditory sensations through simulated unilateral hearing loss.

    OpenAIRE

    Schaette, R.; Turtle, C.; Munro, K. J.

    2012-01-01

    Tinnitus, a phantom auditory sensation, is associated with hearing loss in most cases, but it is unclear if hearing loss causes tinnitus. Phantom auditory sensations can be induced in normal hearing listeners when they experience severe auditory deprivation such as confinement in an anechoic chamber, which can be regarded as somewhat analogous to a profound bilateral hearing loss. As this condition is relatively uncommon among tinnitus patients, induction of phantom sounds by a lesser degree ...

  11. Reversible Induction of Phantom Auditory Sensations through Simulated Unilateral Hearing Loss

    OpenAIRE

    Roland Schaette; Charlotte Turtle; Munro, Kevin J.

    2012-01-01

    Tinnitus, a phantom auditory sensation, is associated with hearing loss in most cases, but it is unclear if hearing loss causes tinnitus. Phantom auditory sensations can be induced in normal hearing listeners when they experience severe auditory deprivation such as confinement in an anechoic chamber, which can be regarded as somewhat analogous to a profound bilateral hearing loss. As this condition is relatively uncommon among tinnitus patients, induction of phantom sounds by a lesser degree ...

  12. Hearing Aids

    Science.gov (United States)

    ... of hearing loss. Hearing loss can have a negative effect on communication, relationships, school/work performance, and emotional ... the President's Council of Advisors on Science and Technology (PCAST), and the ... in Effect Guidance Document: Conditions for Sale for Air-Conduction ...

  13. Lungfish Hearing

    DEFF Research Database (Denmark)

    Christensen, Christian Bech; Madsen, Peter Teglberg; Christensen-Dalsgaard, Jakob

    Recent research has shown that tympanic middle ears evolved independently in the major vertebrate groups and represent independent experiments in terrestrial hearing. Furthermore, the tympanic ear emerged quite late – ap - proximately 120 mya after the origin of the tetrapods and approximately 70...... my after the first truly terrestrial tetrapods emerged. One of the major challenges is to understand the transitional stages from tetrapod ancestors to the tympanic tetrapod ear, for example how a non-tympanic ear functions in terrestrial hearing. Lungfish are the closest living relatives...... and urodeles. Based on ABR and vibration measurements also on amphib - ians, lizards, snakes and alligators we can outline scenarios for the initial adaptations of the middle ear to non-tympanic hearing and assess the selection pressures later adapting the middle ear for tympanic hearing. Hearing by bone...

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

    Directory of Open Access Journals (Sweden)

    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.

  15. Analysis of caloric test responses in sudden hearing loss.

    Science.gov (United States)

    Shih, Cheng-Ping; Chou, Yu-Ching; Chen, Hsin-Chien; Lee, Jih-Chin; Chu, Yueng-Hsiang; Wang, Chih-Hung

    2017-02-01

    Sudden sensorineural hearing loss is characterized by a rapid-onset hearing loss that develops within 3 days. Vertigo may also be present. We conducted a retrospective study to investigate whether the severity of a loss of caloric function is associated with the initial hearing loss and with hearing recovery. Our study population was made up of 135 patients-67 men and 68 women, aged 25 to 71 years (mean: 50.9)-with sudden sensorineural hearing loss who had undergone bithermal caloric testing. We compared various patient factors according to patients' hearing level and their response to caloric testing. We also analyzed the canal paresis (CP) value in patients with an abnormal caloric response according to three factors: disease severity, vertigo, and hearing recovery, and we evaluated the correlation between the loss of caloric function and hearing outcomes. We found that an abnormal caloric response was significantly associated with a profound hearing loss at presentation, the presence of vertigo, and poor hearing recovery. Among patients with an abnormal caloric response, the CP value was significantly correlated with hearing recovery (r = 0.503, p = 0.001). Poor hearing recovery was seen in 80% of patients with a CP value of ≥40% but in only 25% of patients with a value of hearing recovery was worse in the patients with a CP value of ≥40% (p = 0.002). We conclude that a CP value of ≥40% is a significant prognostic factor for an unfavorable treatment outcome.

  16. Congenital syphilis

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang Wook; Kim, Kyung Soo; Hur, Don [Chosun University College of Medicine, Kwangju (Korea, Republic of)

    1983-12-15

    In recent years, marked increase in incidence of congenital syphilis has occurred throughout the world due to changes in social norms and development of penicillin-resistant strains. Early diagnosis plays an important role in congenital syphilis as the clinical manifestations may simulate many other conditions in the paediatric age group. The authors analyzed 52 cases of congenital syphilis admitted to the department of paediatrics, Chosun University Hospital, clinically and radiologically. Among them, 18 cases were born in this hospital and 34 cases were admitted from OPD, during the period of 8 years from January, 1975 to December, 1982. The results obtained were as follows; 1. In 28 of 34 cases (82%), the first clinical manifestations were below the age of 3 months. 2. Among the 52 cases, a male predominance was observed with a male to female ratio of 2 : 1. 3. The serologic test (VDRL) of the 52 studied cases showed reactive response in 49 cases (94%), and that of syphilitic mothers except 6 cases, reactive in all studied cases. 4. The major manifestations of the 52 cases were bone tenderness (12%) and swelling of the joints (7%) in skeletal system, hepatosplenomegaly (79%) and skin lesions (73%) in extraskeletal one. 5. The radiological skeletal changes were detected in 45 of 52 cases (87%), and the commonest findings were detected in 45 of 52 cases (87%), and the commonest findings were metaphysitis (83%) and periostitis (81%). The most characteristic type of metaphysitis were transverse trophic line (74%) and zone of rarefaction (65%). 6. The commonest bones to be affected were growing metaphyses of the long bones, particulary about the wrist and the knee. The order of frequency were radius (80%), uina (80%), tibia (77%), femur (69%) and humerus (40%)

  17. Valganciclovir for symptomatic congenital cytomegalovirus disease.

    Science.gov (United States)

    Kimberlin, David W; Jester, Penelope M; Sánchez, Pablo J; Ahmed, Amina; Arav-Boger, Ravit; Michaels, Marian G; Ashouri, Negar; Englund, Janet A; Estrada, Benjamin; Jacobs, Richard F; Romero, José R; Sood, Sunil K; Whitworth, M Suzanne; Abzug, Mark J; Caserta, Mary T; Fowler, Sandra; Lujan-Zilbermann, Jorge; Storch, Gregory A; DeBiasi, Roberta L; Han, Jin-Young; Palmer, April; Weiner, Leonard B; Bocchini, Joseph A; Dennehy, Penelope H; Finn, Adam; Griffiths, Paul D; Luck, Suzanne; Gutierrez, Kathleen; Halasa, Natasha; Homans, James; Shane, Andi L; Sharland, Michael; Simonsen, Kari; Vanchiere, John A; Woods, Charles R; Sabo, Diane L; Aban, Inmaculada; Kuo, Huichien; James, Scott H; Prichard, Mark N; Griffin, Jill; Giles, Dusty; Acosta, Edward P; Whitley, Richard J

    2015-03-05

    The treatment of symptomatic congenital cytomegalovirus (CMV) disease with intravenous ganciclovir for 6 weeks has been shown to improve audiologic outcomes at 6 months, but the benefits wane over time. We conducted a randomized, placebo-controlled trial of valganciclovir therapy in neonates with symptomatic congenital CMV disease, comparing 6 months of therapy with 6 weeks of therapy. The primary end point was the change in hearing in the better ear ("best-ear" hearing) from baseline to 6 months. Secondary end points included the change in hearing from baseline to follow-up at 12 and 24 months and neurodevelopmental outcomes, with each end point adjusted for central nervous system involvement at baseline. A total of 96 neonates underwent randomization, of whom 86 had follow-up data at 6 months that could be evaluated. Best-ear hearing at 6 months was similar in the 6-month group and the 6-week group (2 and 3 participants, respectively, had improvement; 36 and 37 had no change; and 5 and 3 had worsening; P=0.41). Total-ear hearing (hearing in one or both ears that could be evaluated) was more likely to be improved or to remain normal at 12 months in the 6-month group than in the 6-week group (73% vs. 57%, P=0.01). The benefit in total-ear hearing was maintained at 24 months (77% vs. 64%, P=0.04). At 24 months, the 6-month group, as compared with the 6-week group, had better neurodevelopmental scores on the Bayley Scales of Infant and Toddler Development, third edition, on the language-composite component (P=0.004) and on the receptive-communication scale (P=0.003). Grade 3 or 4 neutropenia occurred in 19% of the participants during the first 6 weeks. During the next 4.5 months of the study, grade 3 or 4 neutropenia occurred in 21% of the participants in the 6-month group and in 27% of those in the 6-week group (P=0.64). Treating symptomatic congenital CMV disease with valganciclovir for 6 months, as compared with 6 weeks, did not improve hearing in the short term

  18. 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

  19. [Aftereffects of congenital infections in infants].

    Science.gov (United States)

    Burdzenidze, E; Zhvania, M

    2006-12-01

    Congenital infections are among the most pressing health care problems. Congenital infections are not reason of congenital malformation and perinatal mortality only, but also pathologies that can be revealed during first year of life. Frequency for congenital viral infection displayed from birth varies between 23% and 92%. The aim of the study was the investigation of inherent infection consequences (citomegaloviral infection, herpes infection and chlamidia) in children in different age groups. Under our observation were 81 children with congenital infections. Among them 29 were with citomegaloviral infection, 17 with herpes infection; 15 chlamidia infection and 22 infections mix (citomegalovirus + herpes, citomegalovirus + chlamidia and chlamidia + herpes). In all observed children neurological simptomatic such as neuro-reflectory hyperexcitability syndrom, hypertension-hydrocephalic syndrom, musculary dystonia syndrom, hydrocephaly, retardation of psychomotor development etc. were present. After birth the worst prevalent are pathologies of cardiovascular system: functional cardiopathy, carditis, congenital heart disease (among them multivalvular disease), affection of hepatobilliar system, organs of vision and hearing etc are present also.

  20. Connecxin 26 Gene Mutations in Non-Syndromic Hearing Loss in Hamadan Province

    Directory of Open Access Journals (Sweden)

    Y. Shafeghati

    2006-01-01

    Full Text Available Introduction & Objective : Hearing loss is the most prevalent form of sensory impairment in humans, affecting approximately one in 1000 infants. In more than half of the cases, the deafness is inherited, and about 80% of hereditary deafness transmitted by autosomal recessive pattern. In hereditary congenital deafness, numerous mutations in GJB2 make the largest fractional contribution in many world populations. The GJB2 gene locus was identified in long arm of chromosome 13 (13q11-12. Materials & Methods : In this study, we investigated 76 probands (152 chromosomes of 76 families from Hamadan Province, suffering from severe to profound non-syndromic deafness, to determine the frequencies of GJB2 mutations. All samples were screened for 35delG mutation in GJB2 gene by allele-specific PCR (ARMS-PCR. Study was terminated for those who were homozygote for 35delG mutation. DHPLC analysis had been done on others to find abnormal elution profiles, followed by direct sequencing on samples with abnormal elution profiles. Results : We identified allelic variants and mutations in exons 1 & 2 of CX26 ( 35delG, R127H, V27I, V153I, E114G, -3170G>A. Interestingly we found a novel frame-shift mutation (507insAACG, that was not reported before in related medical literature. Also we detected a previously reported complex allelic variant "V27I;E114G/wt. 11 persons carried the 35delG allele (8 homozygote, 3 heterozygote, 2 cases were compound heterozygote for 35delG/wt and 3170G>A/wt and 1 proband showed the V27I;E114G/wt mutation.Conclusion : Based on these data, GJB2 mutations account for approximately 18.1% of severe-to-profound congenital deafness in the Hamadan population, and the most common of this mutation is 35delG.

  1. Congenital Toxoplasmosis

    Science.gov (United States)

    McAuley, James B.

    2014-01-01

    Toxoplasmosis is caused by infection with the parasite Toxoplasma gondii. It is one of the most common parasitic infections in humans and is most typically asymptomatic. However, primary infection in a pregnant woman can cause severe and disabling disease in the developing fetus. Recent developments have included increased understanding of the role of parasite genotype in determining infectivity and disease severity. Risk factors for acquisition of infection have been better defined, and the important role of foodborne transmission has been further delineated. In addition, strategies have emerged to decrease mother-to-child transmission through prompt identification of acutely infected pregnant women followed by appropriate treatment. Refined diagnostic tools, particularly the addition of immunoglobulin G avidity testing, allow for more accurate timing of maternal infection and hence better decision making during pregnancy. Congenitally infected children can be treated, beginning in utero and continuing through the first year of life, to ameliorate the severity of disease. However, despite these many advances in our understanding of congenital toxoplasmosis prevention and treatment, significant areas of study remain: we need better drugs, well defined strategies for screening of pregnant women, improved food safety, and improved diagnostic tests. PMID:25232475

  2. Auditory, Visual, and Auditory-Visual Speech Perception by Individuals with Cochlear Implants versus Individuals with Hearing Aids

    Science.gov (United States)

    Most, Tova; Rothem, Hilla; Luntz, Michal

    2009-01-01

    The researchers evaluated the contribution of cochlear implants (CIs) to speech perception by a sample of prelingually deaf individuals implanted after age 8 years. This group was compared with a group with profound hearing impairment (HA-P), and with a group with severe hearing impairment (HA-S), both of which used hearing aids. Words and…

  3. 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.

  4. Hearing Impairments

    Science.gov (United States)

    Cavender, Anna; Ladner, Richard E.

    For many people with hearing impairments, the degree of hearing loss is only a small aspect of their disability and does not necessarily determine the types of accessibility solutions or accommodations that may be required. For some people, the ability to adjust the audio volume may be sufficient. For others, translation to a signed language may be more appropriate. For still others, access to text alternatives may be the best solution. Because of these differences, it is important for researchers in Web accessibility to understand that people with hearing impairments may have very different cultural-linguistic traditions and personal backgrounds.

  5. 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

  6. Characteristics of children with unilateral hearing loss.

    Science.gov (United States)

    Fitzpatrick, Elizabeth M; Al-Essa, Rakan S; Whittingham, JoAnne; Fitzpatrick, Jessica

    2017-11-01

    The purpose of this study was to describe the clinical characteristics of children with unilateral hearing loss (UHL), examine deterioration in hearing, and explore amplification decisions. Population-based data were collected prospectively from time of diagnosis. Serial audiograms and amplification details were retrospectively extracted from clinical charts to document the trajectory and management of hearing loss. The study included all children identified with UHL in one region of Canada over a 13-year period (2003-2015) after implementation of universal newborn hearing screening. Of 537 children with permanent hearing loss, 20.1% (108) presented with UHL at diagnosis. They were identified at a median age of 13.9 months (IQR: 2.8, 49.0). Children with congenital loss were identified at 2.8 months (IQR: 2.0, 3.6) and made up 47.2% (n = 51), reflecting that a substantial portion had late-onset, acquired or late-identified loss. A total of 42.4% (n = 39) showed deterioration in hearing, including 16 (17.4%) who developed bilateral loss. By study end, 73.1% (79/108) of children had received amplification recommendations. Up to 20% of children with permanent HL are first diagnosed with UHL. About 40% are at risk for deterioration in hearing either in the impaired ear and/or in the normal hearing ear.

  7. The differential diagnosis of hearing loss.

    Science.gov (United States)

    Zahnert, Thomas

    2011-06-01

    According to the World Health Organization, hearing loss is one of the six leading contributors to the global burden of disease. It is becoming an ever more important problem in society at large, not just because the population is aging, but also because young people increasingly spend their leisure time in activities that expose them to excessive noise. On the other hand, the treatment of hearing loss is improving, as the result of technical developments in otological surgery, hearing aids, and cochlear implants. For nearly every type of hearing loss, there is now some type of rehabilitative treatment. The prerequisite to effective care is timely and accurate diagnosis. Review of the pertinent literature and national guidelines. The available epidemiological data on hearing loss in Germany are inadequate. It is roughly estimated that 13 to 14 million people in Germany are in need of treatment for hearing loss. The most common types of permanent hearing loss are those associated with old age, chronic otitis media, and acoustic trauma. Transient hearing loss is particularly common in childhood as a result of inadequate ventilation of the middle ear. The further technical development of cochlear implants has now widened their indications to include severe congenital deafness and presbycusis.

  8. Epidemiology of adult congenital heart disease: demographic variations worldwide

    NARCIS (Netherlands)

    Mulder, B. J. M.

    2012-01-01

    The population of adults with a congenital heart defect (CHD) is increasing, due to improved survival after cardiac surgery. To accommodate the specialised care for these patients, a profound interest in the epidemiology of CHD is required. The exact size of the current population of adults with CHD

  9. Hearing Impairment

    Science.gov (United States)

    ... Fitness Diseases & Conditions Infections Drugs & Alcohol School & Jobs Sports Expert Answers (Q&A) Staying Safe Videos for ... known as noise-induced hearing loss (NIHL) . Personal music players are among the chief culprits of NIHL ...

  10. Hearing Conservation

    Science.gov (United States)

    1995-01-01

    the federal standard. Footnote** See Title 29 Code of Federal Regulations (CFR) 1910.95 "Occupational Noise Exposure." (Back to text) | USDOL | CONTACT INFORMATION | DISCLAIMER | 15 of 15 OSHA 3074 - Hearing Conservation

  11. 2 SISTERS WITH MENTAL-RETARDATION, CATARACT, ATAXIA, PROGRESSIVE HEARING-LOSS, AND POLYNEUROPATHY

    NARCIS (Netherlands)

    BEGEER, JH; SCHOLTE, FA; VANESSEN, AJ

    1991-01-01

    Two sisters are described with a disorder characterised by mental retardation, congenital cataract, progressive spinocerebellar ataxia, sensorineural deafness, and signs of peripheral neuropathy. Progressive hearing loss, ataxia, and polyneuropathy became evident in the third decade. The

  12. Differentiating characteristics of deafblindness and autism in people with congenital deafblindness and profound intellectual disability

    NARCIS (Netherlands)

    Hoevenaars-van den Boom, M.A.A.; Antonissen, A.C.F.M.; Knoors, H.E.T.; Vervloed, M.P.J.

    2009-01-01

    In persons with deafblindness, it is hard to distinguish autism spectrum disorders from several deafblind specific behaviours caused by the dual sensory impairments, especially when these persons are also intellectually disabled. As a result, there is an over-diagnosis of autism in persons who are

  13. Challenges of Developing Communicative Interaction in Individuals with Congenital Profound Intellectual and Multiple Disabilities

    Science.gov (United States)

    Blain-Moraes, Stefanie; Chau, Tom

    2012-01-01

    Background: Physiological responses have been used in individuals with acquired disability to enable communicative interaction without motor movement. This study explored four autonomic nervous system (ANS) signals--electrodermal activity, skin temperature, cardiac patterns and respiratory patterns--to enable interaction with individuals born with…

  14. Hearing Loss in Children

    Science.gov (United States)

    ... Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2015 ...

  15. Genetics of Hearing Loss

    Science.gov (United States)

    ... Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2015 ...

  16. Managing Hearing Loss

    Science.gov (United States)

    ... page please turn JavaScript on. Feature: Hearing Loss Managing Hearing Loss Past Issues / Spring 2015 Table of ... not a cure. Read More "Hearing Loss" Articles Managing Hearing Loss / Symptoms, Devices, Prevention & Research / Screening Newborns / ...

  17. What's Hearing Loss?

    Science.gov (United States)

    ... Weight for Me? Your Teeth Heart Murmurs What's Hearing Loss? KidsHealth > For Kids > What's Hearing Loss? Print ... problem can also develop later in life. How Hearing Works To understand how and why hearing loss ...

  18. 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

  19. Laboratory-confirmed Congenital Rubella Syndrome at the ...

    African Journals Online (AJOL)

    Background: Congenital rubella syndrome (CRS) caused by rubella infection in uterine, is a major public health problem among women of child bearing age as it causes serious complications including foetal death or abnormalities including cardiovascular, ophthalmologic, respiratory and hearing impairment. Though there ...

  20. Hearing loss and renal syndromes.

    Science.gov (United States)

    Phelan, Paul J; Rheault, Michelle N

    2017-11-12

    The association between ear and kidney abnormalities has long been recognized; however, the connection between these two disparate organs is not always straightforward. Although Alport syndrome is the most well-known, there are over 20 disorders that need to be considered in the differential diagnosis of patients with both ear and kidney abnormalities. Commonalities are present between the kidney and ear in a number of structural proteins, developmentally important transcription factors, ciliary proteins, and channel proteins, and mutations in these pathways can lead to disease in both organ systems. This manuscript reviews the congenital disorders with both hearing and kidney manifestations.

  1. Current amplification models of sensorineurall and conductive hearing loss

    Directory of Open Access Journals (Sweden)

    Ostojić Sanja

    2012-01-01

    Full Text Available The main function of a hearing aid is to improve auditory and language abilities of hearing impaired users. The amplification model has to be adapted according to age, degree and type of hearing loss. The goal of this paper is to analyze the current amplification models of sensorineural and conductive hearing loss which can provide a high quality of speech perception and sounds at any degree of hearing loss. The BAHA is a surgically implantable system for treatment of conductive hearing loss that works through direct bone conduction. BAHA is used to help people with chronic ear infections, congenital external auditory canal atresia and single sided deafness who cannot benefit from conventional hearing aids. The last generation of hearing aid for sensorineural hearing loss is cochlear implant. Bimodal amplification improves binaural hearing. Hearing aids alone do not make listening easier in all situations. The things that can interfere with listening are background noises, distance from a sound and reverberation or echo. The device used most often today is the Frequency Modulated (FM system.

  2. [Congenital hypothyroidism].

    Science.gov (United States)

    Castilla Peón, María Fernanda

    Congenital hypothyroidism (CH) is a cause of preventable mental retardation; therefore, timely diagnosis and treatment by the primary care physician is very important. CH screening must be performed between the second and fifth days of life with capillary blood done with a heel prick and must be confirmed by measurement of thyroid hormones in venous blood. The most common cause of CH is thyroid dysgenesis, which may be identified by a thyroid scan carried out before initiating treatment. Treatment should be with levothyroxine (10-15μg/kg/day) and should not be delayed or suspended during the first 3 years of life due to the deleterious effect on neurodevelopment in case of low thyroid hormones during this time. Preterm or sick infants or those with Down syndrome require special consideration. This article provides diagnostic and therapeutic algorithms for CH. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  3. Congenital deafness is associated with specific somatosensory deficits in adolescents.

    Science.gov (United States)

    Moshourab, Rabih; Bégay, Valérie; Wetzel, Christiane; Walcher, Jan; Middleton, Steven; Gross, Manfred; Lewin, Gary R

    2017-06-26

    Hearing and touch represent two distinct sensory systems that both rely on the transformation of mechanical force into electrical signals. Here we used a battery of quantitative sensory tests to probe touch, thermal and pain sensitivity in a young control population (14-20 years old) compared to age-matched individuals with congenital hearing loss. Sensory testing was performed on the dominant hand of 111 individuals with normal hearing and 36 with congenital hearing loss. Subjects with congenital deafness were characterized by significantly higher vibration detection thresholds at 10 Hz (2-fold increase, P cooling which was not accompanied by changes in warm detection. At least 60% of children with non-syndromic hearing loss showed very significant loss of vibration detection ability (at 10 Hz) compared to age-matched controls. We thus propose that many pathogenic mutations that cause childhood onset deafness may also play a role in the development or functional maintenance of somatic mechanoreceptors.

  4. Auditory assessment of children with severe hearing loss using behavioural observation audiometry and brainstem evoked response audiometry

    OpenAIRE

    Rakhi Kumari; Priyanko Chakraborty; Jain, R K; Dhananjay Kumar

    2016-01-01

    Background: Early detection of hearing loss has been a long-standing priority in the field of audiology. Currently available auditory testing methods include both behavioural and non-behavioural or objective tests of hearing. This study was planned with an objective to assess hearing loss in children using behavioural observation audiometry and brain stem evoked response audiometry. Methods: A total of 105 cases suffering from severe to profound hearing loss were registered. After proper h...

  5. Hearing impairment caused by mutations in two different genes responsible for nonsyndromic and syndromic hearing loss within a single family.

    Science.gov (United States)

    Niepokój, Katarzyna; Rygiel, Agnieszka M; Jurczak, Piotr; Kujko, Aleksandra A; Śniegórska, Dominika; Sawicka, Justyna; Grabarczyk, Alicja; Bal, Jerzy; Wertheim-Tysarowska, Katarzyna

    2017-11-18

    Usher syndrome is rare genetic disorder impairing two human senses, hearing and vision, with the characteristic late onset of vision loss. This syndrome is divided into three types. In all cases, the vision loss is postlingual, while loss of hearing is usually prelingual. The vestibular functions may also be disturbed in Usher type 1 and sometimes in type 3. Vestibular areflexia is helpful in making a proper diagnosis of the syndrome, but, often, the syndrome is misdiagnosed as a nonsyndromic hearing loss. Here, we present a Polish family with hearing loss, which was clinically classified as nonsyndromic. After excluding mutations in the DFNB1 locus, we implemented the next-generation sequencing method and revealed that hearing loss was syndromic and mutations in the USH2A gene indicate Usher syndrome. This research highlights the importance of molecular analysis in establishing a clinical diagnosis of congenital hearing loss.

  6. A boy with homozygous microdeletion of NEUROG1 presents with a congenital cranial dysinnervation disorder [Moebius syndrome variant].

    Science.gov (United States)

    Schröder, Julia C; Läßig, Anne K; Galetzka, Danuta; Peters, Angelika; Castle, John C; Diederich, Stefan; Zechner, Ulrich; Müller-Forell, Wibke; Keilmann, Annerose; Bartsch, Oliver

    2013-02-18

    We report on a 6-year-old Turkish boy with profound sensorineural deafness, balance disorder, severe disorder of oral motor function, and mild developmental delay. Further findings included scaphocephaly, plagiocephaly, long palpebral fissures, high narrow palate, low-set posteriorly rotated ears, torticollis, hypoplastic genitalia and faulty foot posture. Parents were consanguineous. Computed tomography and magnetic resonance imaging showed bilateral single widened cochlear turn, narrowing of the internal auditory canal, and bilateral truncation of the vestibulo-cochlear nerve. Microarray analysis and next generation sequencing showed a homozygous deletion of chromosome 5q31.1 spanning 115.3 kb and including three genes: NEUROG1 (encoding neurogenin 1), DCNP1 (dendritic cell nuclear protein 1, C5ORF20) and TIFAB (TIFA-related protein). The inability to chew and swallow, deafness and balance disorder represented congenital palsies of cranial nerves V (trigeminal nerve) and VIII (vestibulo-cochlear nerve) and thus a congenital cranial dysinnervation disorder. Based on reported phenotypes of neurog1 null mutant mice and other vertebrates, we strongly propose NEUROG1 as the causative gene in this boy. The human NEUROG1 resides within the DFNB60 locus for non-syndromic autosomal recessive deafness on chromosome 5q22-q31, but linkage data have excluded it from being causative in the DFNB60 patients. Given its large size (35 Mb, >100 genes), the 5q22-q31 area could harbor more than one deafness gene. We propose NEUROG1 as a new gene for syndromic autosomal recessive hearing loss and congenital cranial dysinnervation disorder including cranial nerves V and VIII.

  7. The bone-anchored hearing aid for children: recent developments.

    Science.gov (United States)

    Snik, Ad; Leijendeckers, Joop; Hol, Myrthe; Mylanus, Emmanuel; Cremers, Cor

    2008-09-01

    In 1984 the Bone-Anchored Hearing Aid, or BAHA, system was introduced. Its transducer is coupled directly to the skull percutaneously to form a highly effective bone-conduction hearing device. Clinical studies on adults with conductive hearing loss have shown that the BAHA system outperforms conventional bone-conduction hearing aids. Therefore, the next step was to apply the BAHA system in children with congenital or acquired conductive hearing loss. Reviewed data showed that, on average, such children benefited significantly more from the BAHA than from reconstructive surgery. Thus, BAHA application appears to be the best option to achieve normal communication and speech and language development in children with bilateral conductive hearing loss. However, in children under the age of three to four years, a conventional solution must be applied, e.g. a bone conductor with a transcutaneous coupling, because they are too young to undergo BAHA implant surgery. In the case of unilateral congenital conductive hearing loss, there is no convincing evidence in the clinical literature for early intervention. In summary, the BAHA system can be considered a new, indispensable tool for children with bilateral conductive hearing loss.

  8. Congenital adrenal hyperplasia

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000411.htm Congenital adrenal hyperplasia To use the sharing features on this page, please enable JavaScript. Congenital adrenal hyperplasia is the name given to a group of ...

  9. Congenital Heart Information Network

    Science.gov (United States)

    ... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

  10. Gestational and congenital syphilis.

    Science.gov (United States)

    Wendel, G D

    1988-06-01

    The frequency of congenital syphilis continues to increase throughout the United States during the 1980s. Untreated maternal infection can lead to stillbirth, premature labor, congenital infection, and neonatal death. Preventive measures, based on control of early syphilis in women, prenatal care, improved diagnosis and diligent followup, can help to decrease the incidence of congenital syphilis. The clinical presentation and recommended regimens for therapy of the gravida with syphilis and neonates with suspected congenital syphilis are reviewed.

  11. 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

    2017-01-01

    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…

  12. 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

  13. Dutch Cochlear Implant Group (CI-ON) Consensus Protocol on Postmeningitis Hearing Evaluation and Treatment

    NARCIS (Netherlands)

    Merkus, Paul; Free, Rolien H.; Mylanus, Emmanuel A. M.; Stokroos, Robbert; Metselaar, Mick; van Spronsen, Erik; Grolman, Wilko; Frijns, Johan H. M.

    2010-01-01

    Objective: One of the most devastating sequelae of bacterial meningitis is profound hearing loss or even deafness. Although cochlear implantation is able to restore (some) hearing abilities, obliteration due to fibrosis and especially calcification of the cochlea in the postmeningitis period is

  14. 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…

  15. Cochlear ossification in patients with profound hearing loss following bacterial meningitis

    DEFF Research Database (Denmark)

    Caye-Thomasen, Per; Dam, Mikkel Seidelin; Omland, Silje Haukali

    2012-01-01

    Cochlear ossification following bacterial meningitis is related to causative pathogen, but not age at disease or time point of evaluation. However, progression may occur over time, especially in case of primary signs of ossification.......Cochlear ossification following bacterial meningitis is related to causative pathogen, but not age at disease or time point of evaluation. However, progression may occur over time, especially in case of primary signs of ossification....

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

    OpenAIRE

    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 ...

  17. Identifying perception behaviours in people with profound intellectual and multiple disabilities.

    Science.gov (United States)

    Roemer, Miriam; Verheul, Ellen; Velthausz, Frank

    2017-12-21

    To support people with profound intellectual and multiple disabilities (PIMD), it is essential to understand how they experience their environment. Insight into perception behaviour may provide an entry point for improved understanding. A random sample of a 30-min video registration of five participants with PIMD was used to code behaviours per second based on an ethogram containing 157 different perception behaviours in nine categories. Eighty-nine different perception behaviours were observed, of which movements with eyes, head and arms were most common. The senses used most were seeing, hearing and touching. Finally, the function of five perception patterns was established in relation to their function:awareness, focusing attention and tension regulation. Close observation using an observation ethogram provides insight into how people with PIMD perceive their environment. © 2017 John Wiley & Sons Ltd.

  18. Auditory and language skills of children using hearing aids.

    Science.gov (United States)

    Penna, Leticia Macedo; Lemos, Stela Maris Aguiar; Alves, Cláudia Regina Lindgren

    2015-01-01

    Hearing loss may impair the development of a child. The rehabilitation process for individuals with hearing loss depends on effective interventions. To describe the linguistic profile and the hearing skills of children using hearing aids, to characterize the rehabilitation process and to analyze its association with the children's degree of hearing loss. Cross-sectional study with a non-probabilistic sample of 110 children using hearing aids (6-10 years of age) for mild to profound hearing loss. Tests of language, speech perception, phonemic discrimination, and school performance were performed. The associations were verified by the following tests: chi-squared for linear trend and Kruskal-Wallis. About 65% of the children had altered vocabulary, whereas 89% and 94% had altered phonology and inferior school performance, respectively. The degree of hearing loss was associated with differences in the median age of diagnosis; the age at which the hearing aids were adapted and at which speech therapy was started; and the performance on auditory tests and the type of communication used. The diagnosis of hearing loss and the clinical interventions occurred late, contributing to impairments in auditory and language development. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  19. Auditory and language skills of children using hearing aids

    Directory of Open Access Journals (Sweden)

    Leticia Macedo Penna

    2015-04-01

    Full Text Available INTRODUCTION: Hearing loss may impair the development of a child. The rehabilitation process for individuals with hearing loss depends on effective interventions.OBJECTIVE: To describe the linguistic profile and the hearing skills of children using hearing aids, to characterize the rehabilitation process and to analyze its association with the children's degree of hearing loss.METHODS: Cross-sectional study with a non-probabilistic sample of 110 children using hearing aids (6-10 years of age for mild to profound hearing loss. Tests of language, speech perception, phonemic discrimination, and school performance were performed. The associations were verified by the following tests: chi-squared for linear trend and Kruskal-Wallis.RESULTS: About 65% of the children had altered vocabulary, whereas 89% and 94% had altered phonology and inferior school performance, respectively. The degree of hearing loss was associated with differences in the median age of diagnosis; the age at which the hearing aids were adapted and at which speech therapy was started; and the performance on auditory tests and the type of communication used.CONCLUSION: The diagnosis of hearing loss and the clinical interventions occurred late, contributing to impairments in auditory and language development.

  20. The impact of verbal capacity on theory of mind in deaf and hard of hearing children.

    Science.gov (United States)

    Levrez, Clovis; Bourdin, Beatrice; Le Driant, Barbara; D'Arc, Baudouin Forgeot; Vandromme, Luc

    2012-01-01

    Even when they have good language skills, many children with hearing loss lag several years behind hearing children in the ability to grasp beliefs of others. The researchers sought to determine whether this lag results from difficulty with the verbal demands of tasks or from conceptual delays. The researchers related children's performance on a nonverbal theory of mind task to their scores on verbal aptitude tests. Twelve French children (average age about 10 years) with severe to profound hearing loss and 12 French hearing children (average about 7 years) were evaluated. The children with hearing loss showed persistent difficulty with theory of mind tasks, even a nonverbal task, presenting results similar to those of hearing 6-year-olds. Also, the children with hearing loss showed a correlation between language level (lexical and morphosyntactic) and understanding of false beliefs. No such correlation was found in the hearing children.

  1. 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...

  2. Virtual Surgery in Congenital Heart Disease

    DEFF Research Database (Denmark)

    Sørensen, Thomas Sangild; Mosegaard, Jesper; Kislinskiy, Stefan

    2014-01-01

     Teaching, diagnosing, and planning of therapy in patients with complex structural cardiovascular heart disease require profound understanding of the three-dimensional (3D) nature of cardiovascular structures in these patients. To obtain such understanding, modern imaging modalities provide high...... et al., Cardiol Young 13:451–460, 2003). In combination with the availability of virtual models of congenital heart disease (CHD), techniques for computer- based simulation of cardiac interventions have enabled early clinical exploration of the emerging concept of virtual surgery (Sorensen et al...

  3. Is Congenital Syphilis Really Congenital Syphilis?

    Directory of Open Access Journals (Sweden)

    Yi Li

    2006-01-01

    Full Text Available Detroit has recently been distinguished as having the highest congenital syphilis rate in the United States (250.3 cases per 100 000 live births in Detroit versus 10.3 in the US. However, depending on each health department's followup and CDC reporting, these data may not accurately reflect the true congenital syphilis rate. This study examines the reported cases over a three-year time period with focus on the criteria used for diagnosis. All local health department congenital syphilis CDC collection forms (form 73.126 were reviewed for the years in question. The reported congenital syphilis cases in the year 2002–2004 in Detroit were reviewed. No cases met confirmed case criteria and few probable cases were based on neonatal evaluations. The majority of “congenital syphilis” cases were established based on incomplete maternal data such as missing followup serologic titers in the absence of complete neonatal information. In conclusion, although the reported congenital syphilis rate in Detroit is alarmingly high, the true occurrence of congenital syphilis is likely to have been overstated. A health department reporting program that includes more diligent neonatal followup would allow for a more accurate representation of this public health concern.

  4. Hearing loss caused by progressive degeneration of cochlear hair cells in mice deficient for the Barhl1 homeobox gene

    National Research Council Canada - National Science Library

    Shengguo Li; Sandy M. Price; Hugh Cahill; David K. Ryugo; Michael M. Shen; Mengqing Xiang

    2002-01-01

    .... We report that targeted disruption of Barhl1 , a mouse homolog of the Drosophila BarH homeobox genes, results in severe to profound hearing loss, providing a unique model for the study of age-related...

  5. Is Congenital Syphilis Really Congenital Syphilis?

    OpenAIRE

    Yi Li; Bernard Gonik

    2006-01-01

    Detroit has recently been distinguished as having the highest congenital syphilis rate in the United States (250.3 cases per 100 000 live births in Detroit versus 10.3 in the US). However, depending on each health department's followup and CDC reporting, these data may not accurately reflect the true congenital syphilis rate. This study examines the reported cases over a three-year time period with focus on the criteria used for diagnosis. All local health department congenital syphilis C...

  6. Experiences of adult patients hearing loss postlingually with Cochlear Implant

    Directory of Open Access Journals (Sweden)

    Teresa María Lizcano Tejado

    2013-09-01

    Full Text Available Hearing loss is a significant public health problem. The incidence is difficult to establish because of the lack of data in people under age three, but is estimated about 1 per thousand for severe and profound hearing loss.A cochlear implant (CI is a device that converts sounds into electrical energy that triggers a sensation of hearing. The IC is indicated in patients with severe bilateral sensorineural hearing loss with null or poor benefit use of hearing aids.The general objective of this project is to understand the experiences of adult patients with severe-profound sensorineural hearing loss with IC postlingually throughout the implementation process.A personal vision of those implemented will allow us to learn how to face the possibility to hear and interact with their environment, applying this information to improve health care provided to them and identifying those areas where such assistance should be improved. Also allow us to compare the initial expectations and have been achieved, creating realistic expectations for future candidates.For its development we have designed a qualitative study, based on the principles and procedures of grounded theory, semistructured interviews, participant observation and discussion groups.The data will be analyzed using the software Nudist ViVo 9.

  7. Spectrum of GJB2 mutations in Cypriot nonsyndromic hearing loss ...

    Indian Academy of Sciences (India)

    2001 Connexin 26 gene mutations in congenitally deaf children: pitfalls for genetic counselling. Arch. Otolaryngol. Head Neck Surg. 127, 927–933. Mese G., Londin E., Mui R., Brink P. R. and White T. W. 2004. Altered gating properties of functional Cx26 mutants associated with recessive non-syndromic hearing loss. Hum.

  8. 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 ...

  9. Measurements on Hearing

    DEFF Research Database (Denmark)

    Poulsen, Torben

    1996-01-01

    Background material for measurements of hearing for grammar school pupils. The note gives the necessary background for the exercise 'Measurement on Hearing'. The topics comprise sound and decibel, the ear, basic psychoacoustics, hearing threshold, audiometric measurement methods, speech and speech...

  10. Hearing Problems in Children

    Science.gov (United States)

    ... learning speech and language long before they talk. Hearing problems can be temporary or permanent. Sometimes, ear infections, injuries or diseases affect hearing. If your child does not hear well, get ...

  11. Syntactic Movement in Orally Trained Children with Hearing Impairment

    Science.gov (United States)

    Friedmann, Naama; Szterman, Ronit

    2006-01-01

    This study explored the comprehension and production of sentences derived by syntactic movement, in orally trained school-age Hebrew-speaking children with moderate to profound hearing impairment, aged 7;8?9;9 years. Experiments 1 and 2 tested the comprehension of relative clauses and topicalization sentences (with word orders of OVS [object,…

  12. Persistence--A Father's Response to His Son's Hearing Impairment.

    Science.gov (United States)

    Kulakowski, Joseph J.

    1984-01-01

    The father of a profoundly hearing impaired 4-year-old describes his initial anger upon discovering his child's problem and ways in which he translated those feelings to action on his child's behalf. He describes a program of intensive speech therapy and parent training that has resulted in progress in speech. (CL)

  13. Compression Amplification in Hearing Aids.

    Science.gov (United States)

    Hickson, L M

    1994-11-01

    In this tutorial, the characteristics of compression amplification in analog hearing aids and the experimental results obtained with single- and multichannel compression amplification systems are reviewed. Single-channel compression systems are classified into four broad groups on the basis of their static and dynamic characteristics: compression limiters, syllabic compressors, automatic volume control (AVC) systems, and "others," those with adaptive recovery time (aRT) and dual frontend automatic gain control (AGC) (Moore, 1990; Moore & Glasberg, 1988). Multichannel compression devices have, to date, used a variety of different types of compression, with syllabic compression in each frequency channel being the most popular. Experimental evidence suggests that compression limiting is generally superior to peak clipping as a means of controlling output, except for some individuals with severe to profound hearing loss. There is some evidence that syllabic compression and AVC systems enhance speech perception in quiet compared to conventional linear amplification; however, this improvement is not consistently maintained in the presence of background noise. The majority of recent research has focused on multichannel compression, and favorable results have been obtained with some systems. Research findings about the efficacy of using compression amplification for people with hearing loss have been extremely variable, and possible reasons for this are discussed.

  14. Recognition and Comprehension of "Narrow Focus" by Young Adults with Prelingual Hearing Loss Using Hearing Aids or Cochlear Implants

    Science.gov (United States)

    Segal, Osnat; Kishon-Rabin, Liat

    2017-01-01

    Purpose: The stressed word in a sentence (narrow focus [NF]) conveys information about the intent of the speaker and is therefore important for processing spoken language and in social interactions. The ability of participants with severe-to-profound prelingual hearing loss to comprehend NF has rarely been investigated. The purpose of this study…

  15. [Directional hearing of unilaterally hearing impaired--especially sense of sound direction in monaural hearing impairment and monaural deafness].

    Science.gov (United States)

    Oikawa, T

    1990-03-01

    I. OBJECTIVE. The present study was undertaken in order to determine the onset of monaural deafness, especially whether it is congenital or acquired, making an investigation into the sense of sound direction in monaural hearing impairment and monaural deafness and comparing them between hearing-impaired patients and normal hearers. II. SUBJECTS. This study was carried out on 26 patients with monaural hearing impairment, 22 patients with monaural deafness and 10 normal hearers. III. METHODS. A circle with a radius of 1.3m was drawn around a fixed patient's position in a sound proof room, and the circle was divided into 16 directions at an equal angle of 22.5 degrees. A blindfolded hearer was instructed to listen to a speaker for white noise of 60dB (A) for one second and verbally answer in which direction he heard the noise. The normal hearers were tested in 4-, 8-, and 16-directions, and the hearing-impaired patients were tested in only 8-directions. IV. RESULTS. 1. Normal Hearers The rate of correct answers decreased with increasing directions of sound. The incorrect answers in 4-direction testing were only confusion between forward and backward directions, and similar incorrect answers were made in 8- and 16-direction testing. All other incorrect answers were errors of less than 45 degrees. 2. Monaurally Hearing-impaired Patients The rate of correct answers on the whole was low. There was such a relationship between the rate of correct answers and the mean hearing level of patients that the total rate of correct answers decreased with increasing hearing impairment. This correlation was statistically significant, and there was a still more significant correlation between the degree of hearing impairment and the rate of correct answers as to the right and left directions. Incorrect answers were errors of 90 degrees or less on the healthy side, while errors were made for all directions on the affected side. 3. Monaurally Deaf Patients The rate of correct answers was

  16. Leber congenital amaurosis, from darkness to light: An ode to Irene Maumenee

    OpenAIRE

    Coussa, Razek Georges; Solache, Irma Lopez; Koenekoop, Robert K.

    2017-01-01

    This article is dedicated to Irene Hussels-Maumenee, Professor of Human Genetics and Ophthalmology, Johns Hopkins’ Wilmer Eye Institute, Ocular Genetics Fellowship director in 1994–1995. Leber congenital amaurosis (LCA) has almost come full circle, from a profound and molecularly uncharacterized form of congenital retinal blindness to one in which a large number of causative genes and disease pathways are known; and the world’s first human retinal disease to be treated by gene therapy. Dr. Ma...

  17. The factors associated with a self-perceived hearing handicap in elderly people with hearing impairment--results from a community-based study.

    Science.gov (United States)

    Chang, Hsin-Pin; Ho, Chin-Yu; Chou, Pesus

    2009-10-01

    Elderly persons with a physiologic hearing deficit (hearing impairment) are not necessarily socially or emotionally disturbed by the deficit in everyday life (hearing handicap). The self-perception of a hearing handicap in elderly people is a key element in seeking consultation for a hearing impairment or using hearing aids. Thus, it is important to determine the factors associated with the self-perception of a hearing handicap. The aims of the present study were to report the relation between a hearing impairment and the self-perception of a hearing handicap, and the factors associated with a self-perceived hearing handicap among a group of randomly recruited, community-dwelling elderly persons, aged 65 yr and older, in Taipei, Taiwan. A cross-sectional survey of community-dwelling elderly persons aged 65 yr and older (N = 1220) participating in an annual general purpose geriatric health examination in 2005 in Taipei. Pure-tone audiometry and a questionnaire including the Hearing Handicap Inventory for the Elderly-Screening Version (HHIE-S) were administered, after obtaining the subject's consent to participate in the study. Demographic information, lifestyle, self-report health status, and biochemical data were also collected. There was a moderate association (gamma(s) = 0.52) between hearing impairment and self-perceived handicap. Only 21.4% of the study subjects with moderate to profound hearing impairment (M4 >or=41 dB HL, N = 555) perceived themselves as hearing-handicapped (HHIE-S total score >or=10). Besides hearing level, marital status (widowed) and self-perceived general health (bad or neutral) were factors that are significantly associated with a self-perceived hearing handicap among elderly subjects with moderate to profound hearing impairment. For study subjects with moderate to profound hearing impairment (M4 >or=41 dB HL), 5.0% of those with HHIE-S or=10 used or felt that they required hearing aids (chi2 test, p handicap (HHIE-S >or=10) is an

  18. Impact of low-frequency hearing.

    Science.gov (United States)

    Büchner, A; Schüssler, M; Battmer, R D; Stöver, T; Lesinski-Schiedat, A; Lenarz, T

    2009-01-01

    Today, cochlear implantation is the treatment of choice in the case of severe to profound hearing loss, but the speech perception abilities of many recipients in noisy conditions are still poor and the overall sound quality and ease of listening still require improvement. Residual low-frequency hearing has been associated with improved hearing performance in cochlear implant patients, especially in difficult listening environments (i.e. cocktail party effect). It seems that low-frequency information can enhance the segregation of competing voices, which leads to better speech understanding in noise. We investigated the effect of low-frequency hearing on speech perception performance in a group of subjects being implanted with the Nucleus Hybrid-L device. The Hybrid-L device is a modified Nucleus Freedom implant, which instead of the standard electrode carries a very delicate electrode array that allows preservation of residual hearing to a great extent. Sentence test results from 22 subjects are presented here. Additionally, for 8 subjects, the acoustically presented frequency range was limited to 300, 500 and 700 Hz, and speech perception tests with a single competing talker were conducted. The Hybrid-L study group achieved a speech reception threshold of 15.9 dB in the hearing aid alone condition, 10.8 dB in the cochlear implant alone condition, and 3.9 dB when using the combination of cochlear implant and hearing aid. Differences between the 3 conditions are statistically significant. Results from the additional experiment on the acoustically presented frequency range suggest that very limited residual hearing below 500 Hz is already sufficient to produce a significant improvement in speech perception performance in conjunction with a cochlear implant. (c) 2009 S. Karger AG, Basel.

  19. Hearing configuration in children with cCMV infection and proposal of a flow chart for hearing evaluation.

    Science.gov (United States)

    Foulon, Ina; Vleurinck, Leen; Kerkhofs, Kristin; Gordts, Frans

    2015-01-01

    This study had three main goals: (1) to determine the hearing configuration in hearing-impaired children born with a congenital CMV (cCMV) infection, (2) to see whether auditory neuropathy spectrum disorder (ANSD) was present, and (3) to propose a flow chart for the follow-up of hearing in children with cCMV. Hearing configuration and the presence of ANSD in cCMV infected children was analysed. Selection criteria were: hearing-impaired children with a regular audiometric follow-up for at least 36 months, no other major risk factors for hearing loss, a normal middle-ear status, and an appropriate behavioral response to the given pure-tone stimuli. Out of a cohort of 206 cCMV infected children, 18 hearing-impaired children were selected. Audiograms of all children showed a flat configuration of SNHL: the slope between octave bands was never greater than 10 decibels. None of the 18 children were found to have ANSD. Hearing impairment in cCMV infants affected all frequencies equally and ANSD does not appear to be a feature of cCMV infection. A flow chart for hearing follow-up in children with cCMV infection was suggested in order to provide guidance, improve uniformity in follow-up, and to make results easier to compare.

  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. Intelligence and Academic Achievement With Asymptomatic Congenital Cytomegalovirus Infection.

    Science.gov (United States)

    Lopez, Adriana S; Lanzieri, Tatiana M; Claussen, Angelika H; Vinson, Sherry S; Turcich, Marie R; Iovino, Isabella R; Voigt, Robert G; Caviness, A Chantal; Miller, Jerry A; Williamson, W Daniel; Hales, Craig M; Bialek, Stephanie R; Demmler-Harrison, Gail

    2017-11-01

    To examine intelligence, language, and academic achievement through 18 years of age among children with congenital cytomegalovirus infection identified through hospital-based newborn screening who were asymptomatic at birth compared with uninfected infants. We used growth curve modeling to analyze trends in IQ (full-scale, verbal, and nonverbal intelligence), receptive and expressive vocabulary, and academic achievement in math and reading. Separate models were fit for each outcome, modeling the change in overall scores with increasing age for patients with normal hearing ( n = 78) or with sensorineural hearing loss (SNHL) diagnosed by 2 years of age ( n = 11) and controls ( n = 40). Patients with SNHL had full-scale intelligence and receptive vocabulary scores that were 7.0 and 13.1 points lower, respectively, compared with controls, but no significant differences were noted in these scores among patients with normal hearing and controls. No significant differences were noted in scores for verbal and nonverbal intelligence, expressive vocabulary, and academic achievement in math and reading among patients with normal hearing or with SNHL and controls. Infants with asymptomatic congenital cytomegalovirus infection identified through newborn screening with normal hearing by age 2 years do not appear to have differences in IQ, vocabulary or academic achievement scores during childhood, or adolescence compared with uninfected children. Copyright © 2017 by the American Academy of Pediatrics.

  2. Nonlinear spectrum compression for the hearing impaired via a frequency-domain processing algorithm.

    Science.gov (United States)

    Paarmann, Larry D

    2006-01-01

    In this paper, the results of both normal-hearing, and profoundly hearing-impaired adults, tested with spectrum compressed speech via the modified chirp-z algorithm, with and without visual stimuli, are reported. Ten normal-hearing adult listeners and five profoundly hearing-impaired adult listeners were asked to identify nonsense syllables presented auditorily and bimodally (audition and vision) via video tape in two conditions: lowpass filtered or unprocessed, and spectrum compressed. The lowpass filtered and spectrum compressed speech occupies the same spectrum width of 840 Hz; at 900 Hz and above, the attenuation is at least 60 dB. The spectrum compression is performed by means of a modified chirp-z algorithm, and is described in this paper. The testing results are significant and are reported in this paper. While the signal processing approach is somewhat intensive, the realtime throughput delay is small. Recent advances in hardware speed suggest that realization in a hearing aid is feasible.

  3. 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.

  4. Bone Anchored Hearing Aid

    Science.gov (United States)

    2002-01-01

    conditions were successfully treated with antibiotics, and only 1% to 2% required surgical revision. Less than 1% required removal of the fixture. Other complications included failure to osseointegrate and loss of fixture and/or abutment due to trauma or infection. Effectiveness Studies showed that BAHAs were implanted in people who have conduction or mixed hearing loss, congenital atresia or suppurative otitis media who were not candidates for surgical repair, and who cannot use conventional bone conduction hearing aids. The need for BAHA is not age- related. Objective audiometric measures and subjective patient satisfaction surveys showed that BAHA significantly improved the unaided and aided free field and sound field thresholds as well as speech discrimination in quiet and in noise for former users of conventional bone conduction hearing aids. The outcomes were ambiguous for former users of air conduction hearing aids. BAHA has been shown to reduce the frequency of ear infection and reduce the discharge particularly among patients with suppurative otitis media. Patients have reported that BAHA improved their quality of life. Reported benefits were improved speech intelligibility, better sound comfort, less pressure on the head, less skin irritation, greater cosmetic acceptance and increase in confidence. Main reported shortcomings were wind noise, feedback and difficulty in using the telephone. Experts and the BAHA manufacturer recommended that recipients of a BAHA implant be at least 5 years old. Challenges associated with the implantation of BAHA in pediatric patients include thin bone, soft bone, higher rates of fixture loss due to trauma, psychological problems, and higher revision rates due to rapid bone growth. The overall outcomes are comparable to adult BAHA. The benefits of pediatric BAHA (e.g. on speech development) appear to outweigh the disadvantages. Screening according to strict eligibility criteria, preoperative counselling, close monitoring by a physician

  5. Mitochondrial disorders in congenital myopathies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of congenital myopathies: congenital muscular dystrophies and congenital structural myopathies. It describes changes in congenital muscular dystrophies with type VI collagen, in myodystrophy with giant mitochondria, in congenital central core myopathies, myotubular myopathy, etc. Clinical and experimental findings are presented. Approaches to therapy for energy disorders in congenital myopathies are depicted.

  6. Hearing preservation with a hybrid cochlear implant (electro-acoustic stimulation – case report

    Directory of Open Access Journals (Sweden)

    Janez Rebol

    2014-01-01

    Full Text Available Typical preoperative hearing loss of candidates for a hybrid cochlear implant ranges from mild to moderate hearing loss in the low frequencies (up to 500 Hz and severe to profound hearing loss in the mid and high frequencies (above 1500 Hz. Usually, the patients have limited or no benefit from hearing aids and their word discrimination score is between 10–60 %. These patients often do not use the hearing aids at all, because they do not hear well in the middle and high frequencies.This 8-year old boy presented with symetrical hearing loss, which was profound in middle and high frequencies and moderate in low frequencies. Because of that, his speech development was impaired. We inserted a hybrid cochlear implant with a hearing preservation electrode by atraumatic insertion technique through the round window and succeeded to preserve his residual hearing. He adapted well to the use of hearing aid and cochlear implant and significantly improved understanding of the high frequency words and sentences.

  7. Bilateral sequential cochlear implantation in the congenitally deaf child: evidence to support the concept of a 'critical age' after which the second ear is less likely to provide an adequate level of speech perception on its own.

    Science.gov (United States)

    Graham, John; Vickers, Debi; Eyles, Julie; Brinton, Julie; Al Malky, Ghada; Aleksy, Wanda; Martin, Jane; Henderson, Lise; Mawman, Deborah; Robinson, Philip; Midgley, Elizabeth; Hanvey, Kate; Twomey, Tracey; Johnson, Susan; Vanat, Zebunnisa; Broxholme, Cath; McAnallen, Cecilia; Allen, Agnes; Bray, Monica

    2009-09-01

    This study attempts to answer the question of whether there is a 'critical age' after which a second contralateral cochlear implant is less likely to provide enough speech perception to be of practical use. The study was not designed to predict factors that determine successful binaural implant use, but to see if there was evidence to help determine the latest age at which the second ear can usefully be implanted, should the first side fail and become unusable.Outcome data, in the form of speech perception test results, were collected from 11 cochlear implant programmes in the UK and one centre in Australia. Forty-seven congenitally bilaterally deaf subjects who received bilateral sequential implants were recruited to the study. The study also included four subjects with congenital unilateral profound deafness who had lost all hearing in their only hearing ear and received a cochlear implant in their unilaterally congenitally deaf ear. Of those 34 subjects for whom complete sets of data were available, the majority (72%) of those receiving their second (or unilateral) implant up to the age of 13 years scored 60 per cent or above in the Bamford Kowal Bench (BKB) sentence test, or equivalent. In contrast, of those nine receiving their second or unilateral implant at the age of 15 or above, none achieved adequate levels of speech perception on formal testing: two scored 29 per cent and 30 per cent, respectively, and the rest seven per cent or less.A discriminant function analysis performed on the data suggests that it is unlikely that a second contralateral implant received after the age of 16 to 18 years will, on its own, provide adequate levels of speech perception. As more children receive sequential bilateral cochlear implants and the pool of data enlarges the situation is likely to become clearer.The results provide support for the concept of a 'critical age' for implanting the second ear in successful congenitally deaf unilateral cochlear implant users. This

  8. 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.

  9. Hard of Hearing?

    DEFF Research Database (Denmark)

    T Christensen, Vibeke

    This summary presents the results of a study of the impact of reduced hearing in relation to labour-market attachment and working life. Reduced hearing contributes to early retirement. Many people with impaired hearing are not aware of the impact of their hearing problems on their working life an...

  10. 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).

  11. CERN hearing day

    CERN Multimedia

    2005-01-01

    1 in 10 people suffer from hearing loss - do you? The Medical Service invites everyone working on CERN premises to participate in the National Hearing Day on: Thursday 10th March From 9am to 4pm The Infirmary, Blg. 57, Gr.Fl. We will be offering hearing tests (audiogram); information, advice on hearing loss, tinnitus and more. Deafness does not just affect the elderly: in Europe, 50% the hearing-impaired are under the age of 55. Exposure to excessive noise is one of the main reasons for hearing loss. But PREVENTION IS POSSIBLE AND EFFECTIVE: for example, Hearing protection devices could reduce tinnitus cases by 80%.

  12. CERN hearing day

    CERN Multimedia

    2005-01-01

    1 in 10 people suffer from hearing loss - do you? The Medical Service invites everyone working on the CERN site to participate in the NATIONAL HEARING DAY on: Thursday 10th March 2005 From 9am to 4pm The Infirmary, Blg. 57, Ground Floor We will be offering hearing tests (audiograms), as well as information and advice on hearing loss, tinnitus, etc. Deafness does not just affect the elderly: in Europe, 50% of the hearing-impaired are under the age of 55. Exposure to excessive noise is one of the main reasons for hearing problems but PREVENTION IS POSSIBLE. For example, hearing protection devices can prevent 80% of tinnitus cases.

  13. CERN hearing day

    CERN Multimedia

    2005-01-01

    1 in 10 people suffer from hearing loss ? do you? The Medical Service invites everyone working on the CERN site to participate in the NATIONAL HEARING DAY on: Thursday 10th March 2005 From 9am to 4pm The Infirmary, Blg. 57, Ground Floor We will be offering hearing tests (audiograms), as well as information and advice on hearing loss, tinnitus, etc. Deafness does not just affect the elderly: in Europe, 50% of the hearing-impaired are under the age of 55. Exposure to excessive noise is one of the main reasons for hearing problems but prevention is possible. For example, hearing protection devices can prevent 80% of tinnitus cases.

  14. CERN hearing day

    CERN Multimedia

    2005-01-01

    1 in 10 people suffer from hearing loss - do you? The Medical Service invites everyone working on CERN premises to participate in the National Hearing Day on: Thursday 10th March From 9am to 4pm The Infirmary, Blg. 57, Gr.Fl. We will be offering hearing tests (audiogram); information, advice on hearing loss, tinnitus and more. Deafness does not just affect the elderly: in Europe, 50% the hearing-impaired are under the age of 55. Exposure to excessive noise is one of the main reasons for hearing loss. But prevention is possible and effective: for example, Hearing protection devices could reduce tinnitus cases by 80%.

  15. Prevalence of visual and hearing impairment in a Dutch institutionalized population with intellectual disability.

    Science.gov (United States)

    Evenhuis, H M; Theunissen, M; Denkers, I; Verschuure, H; Kemme, H

    2001-10-01

    A screening of hearing and visual function was performed using clinical assessment methods in a Dutch institutionalized population of 672 people with mild to profound intellectual disability (ID). Because the studied population was not comparable to the total Dutch population with ID, subgroups were distinguished according to level of ID, age younger and older than 50 years, and the presence or absence of Down's syndrome (DS). The prevalences of both hearing and visual impairment were considerably increased in all subgroups, as compared with the general population. In the least affected group, i.e. those or = 50 years. To a lesser extent, young adults with severe or profound ID had an increased risk of hearing impairment. Visual impairment and blindness were specifically highly prevalent in people with severe or profound ID (51% or = 50 years were also significant risk factors for visual impairment. There was an alarmingly high prevalence of combined sensory impairment, especially in those with severe or profound ID (20%). Although hearing impairment had been diagnosed prior to this screen in 138 people and visual impairment in 65 individuals, a first diagnosis of hearing impairment was made in 128 subjects and of visual impairment in 90 cases. This highlights the tendency for sensory impairments to go unnoticed in people with ID, which is not restricted to those with severe or profound ID. Therefore, the present authors stress the importance of regular screening as outlined in the existing IASSID international consensus statement.

  16. Hearing loss in stranded odontocete dolphins and whales.

    Directory of Open Access Journals (Sweden)

    David Mann

    Full Text Available The causes of dolphin and whale stranding can often be difficult to determine. Because toothed whales rely on echolocation for orientation and feeding, hearing deficits could lead to stranding. We report on the results of auditory evoked potential measurements from eight species of odontocete cetaceans that were found stranded or severely entangled in fishing gear during the period 2004 through 2009. Approximately 57% of the bottlenose dolphins and 36% of the rough-toothed dolphins had significant hearing deficits with a reduction in sensitivity equivalent to severe (70-90 dB or profound (>90 dB hearing loss in humans. The only stranded short-finned pilot whale examined had profound hearing loss. No impairments were detected in seven Risso's dolphins from three different stranding events, two pygmy killer whales, one Atlantic spotted dolphin, one spinner dolphin, or a juvenile Gervais' beaked whale. Hearing impairment could play a significant role in some cetacean stranding events, and the hearing of all cetaceans in rehabilitation should be tested.

  17. The Master Hearing Aid

    Science.gov (United States)

    Curran, James R.

    2013-01-01

    As early as the 1930s the term Master Hearing Aid (MHA) described a device used in the fitting of hearing aids. In their original form, the MHA was a desktop system that allowed for simulated or actual adjustment of hearing aid components that resulted in a changed hearing aid response. Over the years the MHA saw many embodiments and contributed to a number of rationales for the fitting of hearing aids. During these same years, the MHA was viewed by many as an inappropriate means of demonstrating hearing aids; the audio quality of the desktop systems was often superior to the hearing aids themselves. These opinions and the evolution of the MHA have molded the modern perception of hearing aids and the techniques used in the fitting of hearing aids. This article reports on a history of the MHA and its influence on the fitting of hearing aids. PMID:23686682

  18. 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…

  19. 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.

  20. 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…

  1. 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 ...

  2. Chronic Hypoxemia in Children With Congenital Heart Defect Impairs Airway Epithelial Sodium Transport.

    Science.gov (United States)

    Kaskinen, Anu K; Helve, Otto; Andersson, Sture; Kirjavainen, Turkka; Martelius, Laura; Mattila, Ilkka P; Rautiainen, Paula; Pitkänen, Olli M

    2016-01-01

    Ambient hypoxia impairs the airway epithelial Na transport, which is crucial in lung edema reabsorption. Whether chronic systemic hypoxemia affects airway Na transport has remained largely unknown. We have therefore investigated whether chronic systemic hypoxemia in children with congenital heart defect affects airway epithelial Na transport, Na transporter-gene expression, and short-term lung edema accumulation. Prospective, observational study. Tertiary care medical center responsible for nationwide pediatric cardiac surgery. Ninety-nine children with congenital heart defect or acquired heart disease (age range, 6 d to 14.8 yr) were divided into three groups based on their level of preoperative systemic hypoxemia: 1) normoxemic patients (SpO2% ≥ 95%; n = 44), 2) patients with cyanotic congenital heart defect and moderate hypoxemia (SpO2 86-94%; n = 16), and 3) patients with cyanotic congenital heart defect and profound systemic hypoxemia (SpO2 ≤ 85%; n = 39). Nasal transepithelial potential difference served as a surrogate measure for epithelial Na transport of the respiratory tract. Profoundly hypoxemic patients had 29% lower basal nasal transepithelial potential difference (p = 0.02) and 55% lower amiloride-sensitive nasal transepithelial potential difference (p = 0.0003) than normoxemic patients. In profoundly hypoxemic patients, nasal epithelial messenger RNA expressions of two airway Na transporters (amiloride-sensitive epithelial Na channel and β1- Na-K-ATPase) were not attenuated, but instead α1-Na-K-ATPase messenger RNA levels were higher (p = 0.03) than in the normoxemic patients, indicating that posttranscriptional factors may impair airway Na transport. The chest radiograph lung edema score increased after congenital cardiac surgery in profoundly hypoxemic patients (p = 0.0004) but not in patients with normoxemia or moderate hypoxemia. The impaired airway epithelial amiloride-sensitive Na transport activity in profoundly hypoxemic children with

  3. Congenital symmastia revisited

    DEFF Research Database (Denmark)

    Sillesen, Nanna H; Hölmich, Lisbeth R; Siersen, Hans Erik

    2012-01-01

    Symmastia is defined as medial confluence of the breast. The term 'symmastia' is modified from Greek (syn meaning 'together', and mastos meaning 'breast') and was first presented by Spence et al. in 1983. Two forms of symmastia exist: an iatrogenic and a congenital version. Congenital symmastia...

  4. Factors associated with the occurrence of hearing loss after pneumococcal meningitis

    DEFF Research Database (Denmark)

    Worsøe, Lise Lotte; Caye-Thomasen, P.; Brandt, C.T.

    2010-01-01

    -tone hearing threshold levels were compared with normative data. Results. Of 240 patients examined by use of audiometry, 129 (54%) had a hearing deficit, and 50 (39%) of these 129 patients were not suspected of hearing loss at discharge from hospital. Of the 240 patients, 16 (7%) had profound unilateral...... is common after pneumococcal meningitis, and audiometry should be performed on all those who survive pneumococcal meningitis. Important risk factors for hearing loss are advanced age, female sex, severity of meningitis, and bacterial serotype...

  5. AIR TRAVEL FOR INFANTS WITH CONGENITAL HEART DISEASE

    Directory of Open Access Journals (Sweden)

    Adarsh Patil

    2015-01-01

    Full Text Available INTRODUCTION : It is well documented that, healthy person can tolerate the cabin environment of a commercial airline which is pressurized to the level of 5000 ft. however this environment brings profound physiological changes in patients with cardiovascular dise a se. With rise in number of patients travelling internationally for treatment of cardiac problems especially infants travelling by air for congenital heart disease treatment has increased in the recent time and is evident by the reports of medical incidents involving infants with congenital heart disease onboard . (1 Though the IATA medical manual mentions that adult patients with Esenminger Syndrome should not undertake air travel . (2 This article examines the case for infants with congenital heart diseases as there have been no previous studies reported

  6. Neural correlates of taste perception in congenital olfactory impairment

    DEFF Research Database (Denmark)

    Gagnon, Léa; Vestergaard, Martin; Madsen, Kristoffer

    2014-01-01

    Olfaction and gustation contribute both to the appreciation of food flavours. Although acquired loss of smell has profound consequences on the pleasure of eating, food habits and body weight, less is known about the impact of congenital olfactory impairment on gustatory processing. Here we examined...... taste identification accuracy and its neural correlates using functional magnetic resonance imaging (fMRI) in 12 congenitally olfactory impaired individuals and 8 normosmic controls. Results showed that taste identification was worse in congenitally olfactory impaired compared to control subjects. The f......MRI results demonstrated that olfactory impaired individuals had reduced activation in medial orbitofrontal cortex (mOFC) relative to normosmic subjects while tasting. In addition, olfactory performance as measured with the Sniffin׳ Sticks correlated positively with taste-induced BOLD signal increases...

  7. Hearing loss in pregnancy

    OpenAIRE

    Ashok Murthy, V.; Krishna, Kirtan

    2011-01-01

    To study hearing loss in healthy pregnant women. Tertiary care hospital. Prospective study. We screened fifty healthy, non-complicated pregnant women (study group) in the third trimester for hearing loss who had no previous history for the same. Fifty healthy, non-pregnant women (control group) were also screened for hearing loss with a normal pure tone audiogram (PTA) for evidence of hearing loss. Thirteen women in the study group had evidence of hearing loss, in the form of absence of disto...

  8. Canine hearing loss management.

    Science.gov (United States)

    Scheifele, Lesa; Clark, John Greer; Scheifele, Peter M

    2012-11-01

    Dog owners and handlers are naturally concerned when suspicion of hearing loss arises for their dogs. Questions frequently asked of the veterinarian center on warning signs of canine hearing loss and what can be done for the dog if hearing loss is confirmed. This article addresses warning signs of canine hearing loss, communication training and safety awareness issues, and the feasibility of hearing aid amplification for dogs. Copyright © 2012 Elsevier Inc. All rights reserved.

  9. [One case of a pregnant femal taking realgar induceing congenital inner ear malformation].

    Science.gov (United States)

    Diao, Mingfang; Sun, Jianjun; Liu, Yang

    2015-08-01

    A pregnant female taken realgar because of superstition, which caused the baby congenital deafness. Auditory test indicated that bilateral auditory brainstem response (ABR) hearing threshold level was greater than 90 dB nHL and auditory steady state response (ASSR) hearing level ranging from 0.5 kHz to 4 kHz was beyond 110 dB HL. Temporal bone CT showed that bilateral cochlear and semicircular canal malformations, with internal auditory canal broadened.

  10. Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review,

    OpenAIRE

    Fernandes, Nayara Freitas; Morettin,Marina; Yamaguti, Elisabete Honda; Costa, Orozimbo Alves; BEVILACQUA, Maria Cecilia

    2015-01-01

    Introduction: Currently, there are no doubts about the benefits of cochlear implants for the development of children with severe or profound hearing loss. However, there is still no consensus among researchers and professionals regarding the benefits for the improvement of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants. Objective: Review the available evidence in the literature to answer the following: "What is the performance of hearing skills i...

  11. Improving Art Museum Accessibility for Adults With Acquired Hearing Loss.

    Science.gov (United States)

    Meyer, Susanna; Larrivee, Linda; Veneziano-Korzec, Ann; Stacy, Katrina

    2017-03-01

    Adults with hearing loss rated the accessibility of guided or docent-led art museum tours with and without hearing assistive technology (HAT). Nineteen individuals (average age 64 years, range 35-87 years) with acquired hearing loss participated. All participants had a bilateral hearing loss (mild to profound) using hearing aids (n = 12), cochlear implants (n = 5), or no technology (n = 2). Two docents who were previously trained to modify their presentations and use clear speech led the tours. Participants experienced a tour with and without the museum's HAT and rated its effectiveness using a rating scale. The study used a pre-post test design. The docent-led tours with HAT were rated significantly higher (p = .003) than the tours without HAT. Participants made several suggestions on improving museum accessibility for individuals with hearing loss. The use of HAT during a museum tour was beneficial for individuals with hearing loss. Training docents to modify their presentations, use clear speech, and HAT improved the accessibility of docent-led tours for individuals with hearing loss.

  12. The intervention model for affective involvement and its effectiveness: Fostering affective involvement between persons who are congenitally deafblind and their communication partners

    NARCIS (Netherlands)

    Martens, M.A.W.

    2014-01-01

    Can one share emotions with someone who can’t hear and see well? This dissertation addresses the effectiveness of a training for professionals to foster affective involvement or the mutual sharing of emotions with people who are congenitally deafblind. People with congenital deafblindness are

  13. Hearing Loss in Children: Types of Hearing Loss

    Science.gov (United States)

    ... Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2015 ...

  14. Drug Induced Hearing Loss: Researchers Study Strategies to Preserve Hearing

    Science.gov (United States)

    ... JavaScript on. Feature: Drug-Induced Hearing Loss Researchers Study Strategies to Preserve Hearing Past Issues / Spring 2016 Table ... Read More "Drug Induced Hearing Loss" Articles Researchers Study Strategies to Preserve Hearing / What Is Ototoxicity? Spring 2016 ...

  15. Universal newborn hearing screening: preliminary experience at the University Hospital of Cagliari

    Directory of Open Access Journals (Sweden)

    Giulia Pinna

    2012-10-01

    Full Text Available Bilateral congenital or acquired sensorineural hearing loss is a pathological condition affecting 1-2 children per 1,000 live births; it represents a major issue in public health because its late identification can negatively affect speech and language development. The aim of hearing screening is to obtain diagnosis and management of hearing loss as soon as possible; in fact early diagnosis and treatment allow children with congenital hearing impairment to acquire adequate linguistic competence. The present study reports our preliminary experience in newborn hearing screening at Neonatology services of University of Cagliari (Italy. During the first semester of surveillance, between January 2012 and June 2012, hearing screening was performed on a total of 901 babies using two different methods, TEOAEs in healthy neonates and automated ABR in high-risk babies. All infants were screened prior to hospital discharge; in some cases, especially for preterm infants of Neonatal Intensive Care Unit and Puericulture Institute, the screening was performed after discharge, to achieve a possible better global and acoustic maturation; 5 cases of hearing impairment were found. In the present study the Authors confirmed that it is possible to start a universal hearing screening in a relatively short time reaching the percentages suggested by Joint Committee on Infant Hearing.

  16. Congenital cholesteatoma of the middle ear - uncommon clinical presentation

    Directory of Open Access Journals (Sweden)

    Bukurov Bojana

    2014-01-01

    Full Text Available Introduction. Congenital cholesteatoma of the middle ear is un uncommon and yet not well-defined disease. Only few cases of cholesteatoma in the fossa ovalis with unusual clinical presentation have been reported in medical literature. Case report. We reported a 16-year-old girl with congenital cholesteatoma in the fossa ovalis with minimal clinical presentation. A small mass was found occluding the fossa ovalis and mimicking otosclerotic process within tympanic cavity. The operation started as stapedotomy, and when the process was confirmed it converted to mastoidectomy via the retroauricular approach. Conclusion. The diagnosis of congenital cholesteatoma in children should always be considered, even if the clinical symptoms imitate other ear disorders, in our case otosclerosis. [Projekat Ministarstva nauke Republike Srbije, br. 179055: Cochlear implantation impact on education of deaf and hearing-impaired

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

    Science.gov (United States)

    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.

  18. Efficacy of multimodality approach to sudden hearing loss.

    Science.gov (United States)

    Naiboğllu, Bariş; Külekçi, Semra; Sürmeli, Mehmet; Verim, Ayşegül; Kalaycik Ertugay, Çiğdem; İhvan, Önder; Şeneldir, Lütfü; Zer Toros, Sema

    2015-01-01

    This study aims to investigate whether addition of intratympanic steroid (ITS) to systemic steroid and hyperbaric oxygen (HBO) is effective in the treatment of sudden hearing loss (SHL). Between January 2008 and October 2011, 58 patients diagnosed with SHL were enrolled in the study. Twenty patients (11 males, 9 females; mean age 45.3±21 years; range 24 to 66 years) who received systemic steroid and HBO composed group 1, while 38 patients (19 males, 19 females; mean age 41.6±16 years; range 25 to 61 years) who received ITS in addition to systemic steroid and HBO composed group 2. Post-treatment hearing improvement was statistically significant in both groups in terms of the mean pure tone according to the Siegel's criteria (p0.05). However, there was a strong statistically significant difference in terms of profound hearing loss over 90 dB (phearing loss in group 1 benefited treatment, while addition of ITS to the treatment yielded success in six of 12 patients with profound hearing loss (50%) in group 2 (p<0.05). Addition of ITS to systemic steroid and HBO treatment may yield better results in patients with SHL. However, ITS injection seems beneficial for patients with profound SHL.

  19. Congenital Hypothyroidism in a Five Year Old Nigerian Girl: A Case ...

    African Journals Online (AJOL)

    Congenital hypothyroidism (CH) is a condition of thyroid hormone deficiency present at birth, with an incidence of 1: 3000 - 4000 infants worldwide. It usually manifest in neonatal period or early infancy. The diagnosis can be delayed where routine screening is not done. Untreated infants have profound mental retardation ...

  20. Story retelling skills in Persian speaking hearing-impaired children.

    Science.gov (United States)

    Jarollahi, Farnoush; Mohamadi, Reyhane; Modarresi, Yahya; Agharasouli, Zahra; Rahimzadeh, Shadi; Ahmadi, Tayebeh; Keyhani, Mohammad-Reza

    2017-05-01

    Since the pragmatic skills of hearing-impaired Persian-speaking children have not yet been investigated particularly through story retelling, this study aimed to evaluate some pragmatic abilities of normal-hearing and hearing-impaired children using a story retelling test. 15 normal-hearing and 15 profound hearing-impaired 7-year-old children were evaluated using the story retelling test with the content validity of 89%, construct validity of 85%, and reliability of 83%. Three macro structure criteria including topic maintenance, event sequencing, explicitness, and four macro structure criteria including referencing, conjunctive cohesion, syntax complexity, and utterance length were assessed. The test was performed with live voice in a quiet room where children were then asked to retell the story. The tasks of the children were recorded on a tape, transcribed, scored and analyzed. In the macro structure criteria, utterances of hearing-impaired students were less consistent, enough information was not given to listeners to have a full understanding of the subject, and the story events were less frequently expressed in a rational order than those of normal-hearing group (P < 0.0001). Regarding the macro structure criteria of the test, unlike the normal-hearing students who obtained high scores, hearing-impaired students failed to gain any scores on the items of this section. These results suggest that Hearing-impaired children were not able to use language as effectively as their hearing peers, and they utilized quite different pragmatic functions. Copyright © 2017 Elsevier B.V. All rights reserved.

  1. The relation of hearing loss degrees and oral stereognosis in 5-year-old children

    Directory of Open Access Journals (Sweden)

    Seyede Zohre Mousavi

    2012-12-01

    Full Text Available Background and Aim: Oral stereognosis is the ability to recognize the objects placed in the mouth; this plays a significant role in speech sounds production. Since the children with hearing loss have articulation disorders, this study aimed to clear the relation of hearing loss degrees and oral stereognosis in 5-year-old children.Methods: In this cross-sectional non-invasive study, 40 children of 5-year-old (30 children with different degrees of hearing loss and 10 normal children were involved. Oral steriognostic test was done for all of them and the Mann-Whitney U was used for statistical analysis.Results: There were significant differences between the mean of oral stereognostic ability between the normal children and the children with severe (p<0.01 or profound hearing loss (p=0.05. There was no significant difference between the mean of oral stereognostic ability among the children with moderate, severe and profound hearing loss compared with together. Besides, there was no significant difference between the mean of the time of diagnosis among all of hearing loss and normal children.Conclusion: The study shows that the children with moderate, severe and profound hearing loss have inefficient oral stereognosis in comparison with the normal children.

  2. 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.

  3. Negotiating hearing disability and hearing disabled identities

    DEFF Research Database (Denmark)

    Lykke Hindhede, Anette

    2012-01-01

        Using disability theory as a framework and social science theories of identity to strengthen the arguments, this paper explores empirically how working-age adults confront the medical diagnosis of hearing impairment. For most participants hearing impairment threatens the stability of social...... interaction and the construction of hearing disabled identities is seen as shaped in the interaction with the hearing impaired person‟s surroundings. In order to overcome the potential stigmatisation the „passing‟ as normal becomes predominant. For many the diagnosis provokes radical redefinitions of the self....... The discursively produced categorisation and subjectivity of senescence mean that rehabilitation technologies such as hearing aids identify a particular life-style (disabled) which determines their social significance. Thus wearing a hearing aid works against the contemporary attempt to create socially ideal...

  4. Congenital Cytomegalovirus infection: advances and challenges in diagnosis, prevention and treatment.

    Science.gov (United States)

    Marsico, Concetta; Kimberlin, David W

    2017-04-17

    Cytomegalovirus (CMV) is the most frequent cause of congenital infection worldwide, with an estimated incidence in developing countries of 0.6-0.7% of all live births. The burden of disease related to congenital CMV in substantial, as it is the leading non-genetic cause of sensorineural hearing loss and an important cause of neurodevelopmental disabilities in children. Despite its clinical significance, congenital CMV infection often goes undetected because the majority of infected infants are asymptomatic at birth and screening programs have not been substantially implemented. Other than behavioral measures, effective interventions aimed at the prevention of maternal infection and of mother-to-child transmission are lacking. Due to a convergence of recent advances in both diagnostic and therapeutic strategies in infants with congenital CMV, though, the field likely will be changing rapidly over just the next few years. Specifically, a highly-sensitive screening test with high throughput potential has been developed, and treatment of infants symptomatically infected with congenital CMV has proven to be well-tolerated and effective in improving long-term hearing and neurodevelopmental outcomes.This review highlights the clinical importance of congenital CMV infection, the developments in laboratory diagnostics, and the benefits of antiviral therapy. It also identifies the global efforts still required in the prevention of maternal infection and in the optimization of antiviral therapy to further reduce the burden of congenital CMV disease.

  5. The “Silent” Global Burden of Congenital Cytomegalovirus

    Science.gov (United States)

    Emery, Vincent C.; Lazzarotto, Tiziana; Gupta, Ravindra K.

    2013-01-01

    Human cytomegalovirus (CMV) is a leading cause of congenital infections worldwide. In the developed world, following the virtual elimination of circulating rubella, it is the commonest nongenetic cause of childhood hearing loss and an important cause of neurodevelopmental delay. The seroprevalence of CMV in adults and the incidence of congenital CMV infection are highest in developing countries (1 to 5% of births) and are most likely driven by nonprimary maternal infections. However, reliable estimates of prevalence and outcome from developing countries are not available. This is largely due to the dogma that maternal preexisting seroimmunity virtually eliminates the risk for sequelae. However, recent data demonstrating similar rates of sequelae, especially hearing loss, following primary and nonprimary maternal infection have underscored the importance of congenital CMV infection in resource-poor settings. Although a significant proportion of congenital CMV infections are attributable to maternal primary infection in well-resourced settings, the absence of specific interventions for seronegative mothers and uncertainty about fetal prognosis have discouraged routine maternal antibody screening. Despite these challenges, encouraging results from prototype vaccines have been reported, and the first randomized phase III trials of prenatal interventions and prolonged postnatal antiviral therapy are under way. Successful implementation of strategies to prevent or reduce the burden of congenital CMV infection will require heightened global awareness among clinicians and the general population. In this review, we highlight the global epidemiology of congenital CMV and the implications of growing knowledge in areas of prevention, diagnosis, prognosis, and management for both low (50 to 70%)- and high (>70%)-seroprevalence settings. PMID:23297260

  6. 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...

  7. 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.

  8. 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...

  9. 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

  10. 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.

  11. Noise-Induced Hearing Loss

    Science.gov (United States)

    ... Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2015 ...

  12. Eldercare at Home: Hearing Problems

    Science.gov (United States)

    ... Join our e-newsletter! Resources Eldercare at Home: Hearing Problems Caregiving How Tos Understanding the Problem Fifty percent ... at all). Unfortunately, not many older people with hearing problems visit a hearing specialist or wear a hearing ...

  13. Hearing Aid and children

    Directory of Open Access Journals (Sweden)

    Jamileh Fatahi

    2002-07-01

    Full Text Available In order to develop oral communication, hearing impaired infants and young children must be able to hear speech comfortably and consistently. To day children with all degrees of hearing loss may be condidates for some kinds of amlification. As children differ from adults, many Factors should be consider in hearing aid selection, evaluation and fitting. For example the child age when he or she is candidate for custom instruments? Do we consider programmable Hearing aid? Are multi memory instruments appropriate for them? What about directional microphones? What style of hearing aid do we select? In this paper such questions are Answered.

  14. Delayed loss of hearing after hearing preservation cochlear implantation: Human temporal bone pathology and implications for etiology.

    Science.gov (United States)

    Quesnel, Alicia M; Nakajima, Hideko Heidi; Rosowski, John J; Hansen, Marlan R; Gantz, Bruce J; Nadol, Joseph B

    2016-03-01

    After initially successful preservation of residual hearing with cochlear implantation, some patients experience subsequent delayed hearing loss. The etiology of such delayed hearing loss is unknown. Human temporal bone pathology is critically important in investigating the etiology, and directing future efforts to maximize long term hearing preservation in cochlear implant patients. Here we present the temporal bone pathology from a patient implanted during life with an Iowa/Nucleus Hybrid S8 implant, with initially preserved residual hearing and subsequent hearing loss. Both temporal bones were removed for histologic processing and evaluated. Complete clinical and audiologic records were available. He had bilateral symmetric high frequency severe to profound hearing loss prior to implantation. Since he was implanted unilaterally, the unimplanted ear was presumed to be representative of the pre-implantation pathology related to his hearing loss. The implanted and contralateral unimplanted temporal bones both showed complete degeneration of inner hair cells and outer hair cells in the basal half of the cochleae, and only mild patchy loss of inner hair cells and outer hair cells in the apical half. The total spiral ganglion neuron counts were similar in both ears: 15,138 (56% of normal for age) in the unimplanted right ear and 13,722 (51% of normal for age) in the implanted left ear. In the basal turn of the implanted left cochlea, loose fibrous tissue and new bone formation filled the scala tympani, and part of the scala vestibuli. Delayed loss of initially preserved hearing after cochlear implantation was not explained by additional post-implantation degeneration of hair cells or spiral ganglion neurons in this patient. Decreased compliance at the round window and increased damping in the scala tympani due to intracochlear fibrosis and new bone formation might explain part of the post-implantation hearing loss. Reduction of the inflammatory and immune response to

  15. 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

  16. Congenital orbital teratoma

    Directory of Open Access Journals (Sweden)

    Shereen Aiyub

    2013-01-01

    Full Text Available We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  17. Congenital Adrenal Hyperplasia

    National Research Council Canada - National Science Library

    Speiser, Phyllis W

    2015-01-01

    Congenital adrenal hyperplasia associated with deficiency of steroid 21-hydroxylase is the most common inborn error in adrenal function and the most common cause of adrenal insufficiency in the pediatric age group...

  18. Contemporary Hearing Rehabilitation Options in Patients with Aural Atresia

    Directory of Open Access Journals (Sweden)

    Jacky F. W. Lo

    2014-01-01

    Full Text Available Congenital aural atresia is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is the malformation of the auricle due to a failure of development of the external ear. Aural atresia, with or without microtia, may significantly affect the hearing and social life of the patients. It is important for every medical practitioner to be aware of the possible treatment options for hearing rehabilitation in this group of patients. In the era of modern technology, new choices, including Bone-Anchored Hearing Aid (BAHA (Cochlear Ltd. and Oticon Medical, Vibrant Soundbridge (VSB (MED-EL, Innsbruck, Austria, and Bonebridge system (BB (MED-EL, Innsbruck, Austria, provide high-end alternatives to traditional Bone Conduction Hearing Aid and Auditory Canal Reconstruction. All these options have advantages and disadvantages, and they are appropriate for different patients and/or at different ages. This paper aims to provide an overview of the management of hearing rehabilitation in congenital aural atresia patients and a discussion of each treatment option.

  19. Contemporary Hearing Rehabilitation Options in Patients with Aural Atresia

    Science.gov (United States)

    Lo, Jacky F. W.; Tsang, Willis S. S.; Yu, Joannie Y. K.; Ho, Osan Y. M.; Ku, Peter K. M.; Tong, Michael C. F.

    2014-01-01

    Congenital aural atresia is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is the malformation of the auricle due to a failure of development of the external ear. Aural atresia, with or without microtia, may significantly affect the hearing and social life of the patients. It is important for every medical practitioner to be aware of the possible treatment options for hearing rehabilitation in this group of patients. In the era of modern technology, new choices, including Bone-Anchored Hearing Aid (BAHA) (Cochlear Ltd. and Oticon Medical), Vibrant Soundbridge (VSB) (MED-EL, Innsbruck, Austria), and Bonebridge system (BB) (MED-EL, Innsbruck, Austria), provide high-end alternatives to traditional Bone Conduction Hearing Aid and Auditory Canal Reconstruction. All these options have advantages and disadvantages, and they are appropriate for different patients and/or at different ages. This paper aims to provide an overview of the management of hearing rehabilitation in congenital aural atresia patients and a discussion of each treatment option. PMID:24883324

  20. Congenital cytomegalovirus infection

    OpenAIRE

    D'Oronzio, U; Arlettaz, R.; Hagmann, C.

    2015-01-01

    Clinical details of 50 infants with congenital cytomegalovirus infection identified in a prospective study are reported. The mean birthweight, gestational age, and head circumference of children with congenital cytomegalovirus infection were not significantly different from those of controls. Three (6%) had symptoms at birth--two neurological and one pneumonitis. In the first four months of life transient hepatosplenomegaly occurred in two infected children and six suffered interstitial pneum...

  1. Multifocal Congenital Hemangiopericytoma.

    Science.gov (United States)

    Robl, Renata; Carvalho, Vânia Oliveira; Abagge, Kerstin Taniguchi; Uber, Marjorie; Lichtvan, Leniza Costa Lima; Werner, Betina; Mehrdad Nadji, Mehrdad

    2017-01-01

    Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported. © 2016 Wiley Periodicals, Inc.

  2. [Research progress on the etiology of delayed-onset hearing loss in children].

    Science.gov (United States)

    Wang, X Y; Huang, L H; Du, Y T

    2017-10-07

    Newborn hearing screening is an effective method for early detection of hearing loss, however, it is not able to detect delayed-onset hearing loss. By exploring the etiology of delayed-onset hearing loss in children, it can provide a clinical basis for early detection of delayed-onset hearing loss. Mutations in SLC26A4, mitochondrial, GJB2 and other genes, enlarged vestibular aqueduct, congenital cytomegalovirus infection, extracorporeal membrane oxygenation, and auditory neuropathy et al were more commonly reported risk factors. In this paper, the risk factors related to delayed-onset hearing loss, which are divided into 5 categories: genetic mutation, abnormal inner ear malformation, perinatal factors, auditory neuropathy and no identifiable cause, are reviewed and analyzed.

  3. Does Acquired Hypothyroidism Affect the Hearing Functions?

    Directory of Open Access Journals (Sweden)

    Ayşe Arduç

    2015-12-01

    Full Text Available Purpose: It is well known that congenital hypothyroidism can cause hearing loss. However, conflicting results were found in studies investigating hearing functions in acquired hypothyroidism. Therefore, we evaluated the audiometric findings in patients with acquired hypothyroidism. Material and Method: The study included 58 patients with hypothyroidism and age- and gender-matched 34 healthy controls. Twenty eight (48.27% patients had subclinical hypothyroidism, and 30 (51.73% had obvious hypothyroidism. All subjects had a normal otoscopic examination and tympanometry. Pure tone audiometry at 250, 500, 1000, 2000, 4000, 6000, and 8000 Hertz (Hz was performed in both groups. Blood pressure measurements and the levels of plasma electrolytes, lipids and vitamin B12 were available in all subjects. Results: Hypothyroidism group and control group were similar with respect to systolic and diastolic blood pressures and plasma glucose, lipid, vitamin B12, calcium, sodium, potassium, and chloride levels. Significantly higher audiometric thresholds (dB at 250 (10 (0-45 vs. 5 (0-15, p<0.001 and 500 Hz (10 (0-40 vs. 10 (-5-15, p=0.003 were recorded in hypothyroid patients compared to that in healthy controls. Hearing thresholds at 250 and 500 Hz correlated positively with thyroid-stimulating hormone (TSH, and negatively with free triiodothyronine and free thyroxine. Subclinical hypothyroid patients had a higher hearing threshold at 250 Hz than healthy controls (p=0.001. Discussion: Our study demonstrated that hearing ability decreases in hypothyroidism, even in subclinical hypothyroidism. The changes in TSH and thyroid hormone levels seem to be directly related to the hearing loss in this population of patients.

  4. Hearing Loss: Screening Newborns

    Science.gov (United States)

    ... of this page please turn JavaScript on. Feature: Hearing Loss Screening Newborns Past Issues / Spring 2015 Table ... deafness, which account for most cases. Screening Newborns' Hearing Now Standard In 1993, children born in the ...

  5. OI Issues: Hearing Loss

    Science.gov (United States)

    Hearing Loss and Osteogenesis Imperfecta Introduction Significant hearing loss has been reported in approximately 50% of people with osteogenesis imperfecta (OI) beginning any time from childhood into middle age. While not ...

  6. Hearing Aid Assembly

    Science.gov (United States)

    Grugel, Richard N. (Inventor)

    2002-01-01

    Progress in hearing aids has come a long way. Yet despite such progress hearing aids are not the perfect answer to many hearing problems. Some adult ears cannot accommodate tightly fitting hearing aids. Mouth movements such as chewing, talking, and athletic or other active endeavors also lead to loosely fitting ear molds. It is well accepted that loosely fitting hearing aids are the cause of feedback noise. Since feedback noise is the most common complaint of hearing aid wearers it has been the subject of various patents. Herein a hearing aid assembly is provided eliminating feedback noise. The assembly includes the combination of a hearing aid with a headset developed to constrict feedback noise.

  7. Hearing loss and music

    Science.gov (United States)

    ... and how often you are exposed to loud music Headphone use Family history of hearing loss Jobs or activities that increase your chance of hearing loss from music are: Being a musician, sound crew member, or ...

  8. Factors associated with the occurrence of hearing loss after pneumococcal meningitis

    DEFF Research Database (Denmark)

    Worsøe, Lise Lotte; Caye-Thomasen, P.; Brandt, C.T.

    2010-01-01

    Background. On the basis of a nationwide registration during a 5-year period (1999-2003), the frequency and severity of hearing loss was investigated retrospectively in 343 consecutive Danish patients who survived pneumococcal meningitis, to identify important risk factors (including the pneumoco......Background. On the basis of a nationwide registration during a 5-year period (1999-2003), the frequency and severity of hearing loss was investigated retrospectively in 343 consecutive Danish patients who survived pneumococcal meningitis, to identify important risk factors (including...... hearing loss, and another 16 (7%) had bilateral profound hearing loss. Significant risk factors for hearing loss were advanced age, the presence of comorbidity, severity of meningitis, a low CSF glucose level, a high CSF protein level, and a certain pneumococcal serotype (P ... is common after pneumococcal meningitis, and audiometry should be performed on all those who survive pneumococcal meningitis. Important risk factors for hearing loss are advanced age, female sex, severity of meningitis, and bacterial serotype...

  9. [Presbyacusis--hearing loss in old age].

    Science.gov (United States)

    Rosenhall, U

    2001-06-06

    Presbyacusis is a very common type of hearing loss, often having profound effects on the quality of life in old age. Since the number of elderly persons is increasing, the incidence of presbyacusis is also expected to increase in the future. Presbyacusis is caused by cochlear degeneration, most pronounced in the basal cochlear coil. The most common audiometric configuration is a gently sloping audiogram, above all affecting the high frequencies. Efforts to improve auditory communication in old age are important, and can be expected to result in improved quality of life for elderly persons and in more efficient use of public resources. The alleviation of age-related hearing handicap includes aural rehabilitation with hearing aid fitting and training programs, specially designed for elderly people. Hearing loss is often combined with other handicaps, such as dementia, immobility and poor vision. The synergistic effects of multiple handicaps can be extensive. Prevention is an issue which is both challenging and problematic. The most important preventive measure is noise reduction, which must start early in life and not shortly before retirement. Inner ear treatment programs, currently under development, might possibly be suitable for treatment of inner ear disorders in the future. Considerable gains can be achieved with respect to resources both human and economical through rehabilitation and suitable preventive measures.

  10. Balance assessment in hearing-impaired children.

    Science.gov (United States)

    Walicka-Cupryś, Katarzyna; Przygoda, Łukasz; Czenczek, Ewelina; Truszczyńska, Aleksandra; Drzał-Grabiec, Justyna; Zbigniew, Trzaskoma; Tarnowski, Adam

    2014-11-01

    According to the scientific reports the postural stability is inseparably associated with hearing organ's correct functioning. The aim of the study was to evaluate the degree of disorders occurring in balance reactions in this group of children with profound hearing loss compared to their healthy peers. The study worked with a total of 228 children, including 65 who are deaf (DCH) and 163 subjects without any hearing deficits (CON) in the control group. Stabilometric measurements were performed with the use of a force distribution platform. The results indicate statistically significant differences in terms of one parameter (the total path length) recorded in the test with the eyes open and a whole range of parameters recorded when the subjects had their eyes closed (the width, height, and area of the ellipse, the total path length, and the horizontal and vertical sway). The study results showed better values of the static balance parameters in deaf children as compared to their peers without hearing disorders and the differences were particularly evident in the test with the subject's eyes closed. The results suggest significantly better processing of sensory stimuli in postural reactions particularly from propioception, and to a lesser extent, from the vision system observed in the subjects as compared to their peers in the control group. Copyright © 2014 Elsevier Ltd. All rights reserved.

  11. Congenital stapes ankylosis in children : Surgical findings and results in 35 cases

    NARCIS (Netherlands)

    Vincent, Robert; Wegner, Inge; Kamalski, Digna M A|info:eu-repo/dai/nl/314096477; Bittermann, Arnold J N|info:eu-repo/dai/nl/357805461; Grolman, Wilko

    2016-01-01

    Objective: To evaluate surgical findings and hearing results in children undergoing middle ear surgery for congenital stapes ankylosis with or without other ossicular malformations (Teunissen and Cremers class I and class II malformations). Study Design: A nonrandomized, nonblinded case series of

  12. Goldenhar syndrome and other congenital disorders of the ear by HR-PCT

    Energy Technology Data Exchange (ETDEWEB)

    Grobovschek, M.; Oberascher, G.

    1987-09-01

    The use of CT in hospitals in which also ENT departments are located will be useful in cases of osseous congenital disorders of the ear. In combination with the clinical and audiometric investigations CT of the temporal bone will be helpful for the indication of the operations to ameliorate the hearing.

  13. Noise Induced Hearing Loss with Tinnitus: Does TRT Help?

    Directory of Open Access Journals (Sweden)

    Nidhi Vohra Maggon

    2017-04-01

    Full Text Available Introduction To determine if hearing augmentation and tinnitus retraining therapy (TRT helps in cases of Tinnitus with Noise induced hearing loss (NIHL and does degree of hearing loss, severity or duration of tinnitus affect recovery  Materials and Methods A prospective study was done on 100 patients of NIHL with tinnitus from Jan 14-Jul 15. Degree of hearing loss was assessed. Tinnitus severity was scored on Tinnitus handicap inventory (THI scale as Slight, Mild, Moderate, severe or catastrophic and patients were subjected to TRT. Patients scored after 1 year of TRT. A relation between tinnitus severity, duration and degree of hearing loss on recovery from tinnitus was analysed. Result 62 of the 100 patients improved following TRT. Discussion In 100 patients THI scores improved from a mean of 63.12 (SD-21.12 to 38.16 (SD-18.21. Mean difference between pre and post-intervention THI scores was 24.96 (SD-17.97. Improvement was significant in severe or profound hearing loss (P12 months. All groups showed improvement. Reduction in Post-TRT THI was significant but did not show any difference among groups. Conclusion TRT helps in tinnitus with NIHL particularly if hearing loss is severe. Severe or catastrophic tinnitus patients experience greater improvement. Duration of tinnitus has no impact.

  14. Middle ear implants: functional gain in mixed hearing loss.

    Science.gov (United States)

    Zernotti, Mario Emilio; Gregorio, Maria Fernanda Di; Sarasty, Andrea C Bravo

    2012-02-01

    Osseous atresia and chronic otitis media are diseases benefit with middle ear implants. Surgery for atresia is technically complicated, has significant number of complications and functional results are often poor. The osseointegrated hearing aids are an alternative. They provide a very good functional gain, but have many problems with the skin and osseointegration. In chronic otitis media, the ossiculoplasty solved partially the hearing problem. Unfortunately in some cases of otitis media and in open cavities fitted with conventional hearing aids the gain is unsatisfactory. To determine the usefulness of an active middle ear implant. Longitudinal Study. Vibrant-Soundbrigde was implanted in eight patients with severe mixed hearing loss. Four patients had chronic otitis media and four had unilateral atresia. The placement of the stimulator (FMT or Floating Mass Transducer) was in five patients on round window, two in stapes and one in the oval window. Functional gain was 35 dB, 40 dB, 48.7 dB and 50 dB for the frequencies 500, 1000, 2000 and 4000 Hz, respectively. Vibrant-Soundbrigde is an excellent option in hearing recovery in severe and profound mixed hearing loss. It also provides an excellent functional gain in diseases difficult to treat with conventional hearing aids.

  15. A Patient with Interstitial 5q21 Deletion, Familial Adenomatous Polyposis, Dysmorphic Features, and Profound Neurologic Dysfunction

    Directory of Open Access Journals (Sweden)

    Manoochehr Karjoo

    2017-01-01

    Full Text Available Familial adenomatous polyposis (FAP is a hereditary autosomal dominant cancer syndrome, results from germ line mutation or deletion of the Adenomatous Polyposis Coli (APC gene on chromosome 5q21. Patients with FAP suffer from multiple polyps mainly at the colorectal region as well as other parts of the gastrointestinal tract, which has propensity to transform into carcinoma. FAP has also been well described in association with various syndromic extra-gastrointestinal manifestations. Less commonly, patients with FAP present with varying degrees of cognitive dysfunction and developmental delay, though the reason for the association is unclear. Herein, we report the case of a male patient born with an interstitial deletion of chromosome 5q, 46,XY, del(5 (q14q23, presenting with familial adenomatous polyposis (FAP, profound developmental delay, cognitive dysfunction, and multiple congenital anomalies including talipes equinovarus, agenesis of the corpus callosum, and dysmorphic facial features.

  16. Hearing poorly with skill

    DEFF Research Database (Denmark)

    Day, Dennis

    2012-01-01

    This paper offers an account of ongoing research into hearing. I offer a characterization of 'skil- led practitioners' from an Ethnomethodological perspective. The skilled practitioner in question is a generic 'hard of hearing' person. The ambition is that such a characterization, both in its...... making and its final state, may be an intrinsic part of design practices concerning the development of hearing aids....

  17. Hearing-aid tester

    Science.gov (United States)

    Kessinger, R.; Polhemus, J. T.; Waring, J. G.

    1977-01-01

    Hearing aids are automatically checked by circuit that applies half-second test signal every thirty minutes. If hearing-aid output is distorted, too small, or if battery is too low, a warning lamp is activated. Test circuit is incorporated directly into hearing-aid package.

  18. Frequency of Congenital Heart Diseases in Prelingual Sensory-Neural Deaf Children

    Directory of Open Access Journals (Sweden)

    Masoud Motasaddi Zarandy

    2016-03-01

    Full Text Available Introduction: Hearing impairment is the most frequent sensorial congenital defect in newborns and has increased to 2–4 cases per 1,000 live births. Sensory-neural hearing loss (SNHL accounts for more than 90% of all hearing loss. This disorder is associated with other congenital disorders such as renal, skeletal, ocular, and cardiac disorders. Given that congenital heart diseases are life-threatening, we decided to study the frequency of congenital heart diseases in children with congenital sensory-neural deafness.  Materials and Methods: All children who had undergone cochlear implantation surgery due to SNHL and who had attended our hospital for speech therapy during 2008–2011 were evaluated by Doppler echocardiography.  Results: Thirty-one children (15 boys and 16 girls with a mean age of 55.70 months were examined, and underwent electrocardiography (ECG and echocardiography. None of the children had any signs of heart problems in their medical records. Most of their heart examinations were normal, one patient had expiratory wheeze, four (12% had mid-systolic click, and four (12% had an intensified S1 sound. In echocardiography, 15 children (46% had mitral valve prolapse (MVP and two (6% had minimal mitral regurgitation (MR. Mean ejection fraction (EF was 69% and the mean fractional shortening (FS was 38%.  Conclusion:  This study indicates the need for echocardiography and heart examinations in children with SNHL.

  19. Profound Muscle Weakness and Pain after One Dose of Actonel

    Directory of Open Access Journals (Sweden)

    Irina Badayan

    2009-01-01

    Full Text Available The World Health Organization (WHO defines osteopenia as a bone density between 1 and 2.5 standard deviation (SD below the bone density of a normal young adult Iqbal 2000. Osteoporosis is defined as 2.5 SD or more below that reference point Iqbal 2000. Bisphosphonates are a group of medications used to treat osteoporosis, Padget's disease of bone, and osteopenia. We report a woman who developed profound muscle weakness and pain after one dose of Risedronate (Actonel.

  20. Congenital Heart Diseases associated with Identified Syndromes ...

    African Journals Online (AJOL)

    BACKGROUND: Congenital heart diseases are commonly associated with other extra cardiac congenital malformations. OBJECTIVE: To identify congenital heart diseases associated with identified syndromes and other extra cardiac congenital malformations in children in our hospital. METHODS: A prospective descriptive ...

  1. Narrative competence among hearing-impaired and normal-hearing children: analytical cross-sectional study

    Directory of Open Access Journals (Sweden)

    Alexandra Dezani Soares

    Full Text Available CONTEXT AND OBJECTIVE: Oral narrative is a means of language development assessment. However, standardized data for deaf patients are scarce. The aim here was to compare the use of narrative competence between hearing-impaired and normal-hearing children. DESIGN AND SETTING: Analytical cross-sectional study at the Department of Speech-Language and Hearing Sciences, Universidade Federal de São Paulo. METHODS: Twenty-one moderately to profoundly bilaterally hearing-impaired children (cases and 21 normal-hearing children without language abnormalities (controls, matched according to sex, age, schooling level and school type, were studied. A board showing pictures in a temporally logical sequence was presented to each child, to elicit a narrative, and the child's performance relating to narrative structure and cohesion was measured. The frequencies of variables, their associations (Mann-Whitney test and their 95% confidence intervals was analyzed. RESULTS: The deaf subjects showed poorer performance regarding narrative structure, use of connectives, cohesion measurements and general punctuation (P < 0.05. There were no differences in the number of propositions elaborated or in referent specification between the two groups. The deaf children produced a higher proportion of orientation-related propositions (P = 0.001 and lower proportions of propositions relating to complicating actions (P = 0.015 and character reactions (P = 0.005. CONCLUSION: Hearing-impaired children have abnormalities in different aspects of language, involving form, content and use, in relation to their normal-hearing peers. Narrative competence was also associated with the children's ages and the school type.

  2. Diphtheria and hearing loss.

    OpenAIRE

    Schubert, C. R.; Cruickshanks, K. J.; Wiley, T. L.; Klein, R.; Klein, B E; Tweed, T. S.

    2001-01-01

    OBJECTIVE: To determine if infectious diseases usually experienced in childhood have an effect on hearing ability later in life. METHODS: The Epidemiology of Hearing Loss Study (N = 3,753) is a population-based study of age-related hearing loss in adults aged 48 to 92 years in Beaver Dam, Wisconsin. As part of this study, infectious disease history was obtained and hearing was tested using pure-tone audiometry. Hearing loss was defined as a pure-tone average of thresholds at 500 Hz, 1,000 Hz,...

  3. Eptifibatide-induced acute profound thrombocytopenia: a case report.

    Science.gov (United States)

    Graidis, Christos; Golias, Christos; Dimitriadis, Dimokritos; Dimitriadis, Georgios; Bitsis, Theodosis; Dimitrelos, Ilias; Tsiakou, Afroditi; Charalabopoulos, Konstantinos

    2014-02-25

    The interactions among cells or among cells and components of the extracellular matrix, is a crucial pathophysiological process involving some molecules collectively known as adhesion molecules (CAMs). Glycoprotein IIb / IIIa receptors are only restricted to blood platelets and they bind fibrinogen and adhesion proteins such as fibronectin, vitronectin, von Willebrand factor to form cross bridges between adjacent platelets. IIb/IIIa receptor antagonists are an object of intense research activity for target therapy worldwide during the last decades. Three GPIIb/IIIa inhibitors, abciximab, tirofiban, and eptifibatide, have been approved for clinical use. Profound thrombocytopenia is an uncommon but clinically important complication of glycoprotein IIb/IIIa inhibitors. This case report discusses a forty-four-year-old male patient with acute coronary syndrome who underwent percutaneous coronary intervention and developed profound thrombocytopenia within 4 hours of first administration of eptifibatide. This report adds another case of eptifibatide-induced thrombocytopenia to the medical literature and endorses the importance of platelet count monitoring after initiating therapy with this agent.

  4. Congenital Malaria in China

    Science.gov (United States)

    Liu, Xue; Culleton, Richard; Tao, Li; Xia, Hui; Gao, Qi

    2014-01-01

    Abstract Background Congenital malaria, in which infants are directly infected with malaria parasites from their mother prior to or during birth, is a potentially life-threatening condition that occurs at relatively low rates in malaria-endemic regions. It is recognized as a serious problem in Plasmodium falciparum–endemic sub-Saharan Africa, where recent data suggests that it is more common than previously believed. In such regions where malaria transmission is high, neonates may be protected from disease caused by congenital malaria through the transfer of maternal antibodies against the parasite. However, in low P. vivax–endemic regions, immunity to vivax malaria is low; thus, there is the likelihood that congenital vivax malaria poses a more significant threat to newborn health. Malaria had previously been a major parasitic disease in China, and congenital malaria case reports in Chinese offer valuable information for understanding the risks posed by congenital malaria to neonatal health. As most of the literature documenting congenital malaria cases in China are written in Chinese and therefore are not easily accessible to the global malaria research community, we have undertaken an extensive review of the Chinese literature on this subject. Methods/Principal Findings Here, we reviewed congenital malaria cases from three major searchable Chinese journal databases, concentrating on data from 1915 through 2011. Following extensive screening, a total of 104 cases of congenital malaria were identified. These cases were distributed mainly in the eastern, central, and southern regions of China, as well as in the low-lying region of southwest China. The dominant species was P. vivax (92.50%), reflecting the malaria parasite species distribution in China. The leading clinical presentation was fever, and other clinical presentations were anaemia, jaundice, paleness, diarrhoea, vomiting, and general weakness. With the exception of two cases, all patients were cured

  5. Congenital cranial dysinnervation disorders.

    Science.gov (United States)

    Singh, Anupam; Pandey, P K; Agrawal, Ajai; Mittal, Sanjeev Kumar; Rana, Kartik Maheshbhai; Bahuguna, Chirag

    2017-12-01

    The European Neuromuscular Centre (ENMC) derived the term Congenital Cranial Dysinnervation Disorders in 2002 at an international workshop for a group of congenital neuromuscular diseases. CCDDs are congenital, non-progressive ophthalmoplegia with restriction of globe movement in one or more fields of gaze. This group of sporadic and familial strabismus syndromes was initially referred to as the 'congenital fibrosis syndromes' because it was assumed that the primary pathologic process starts in the muscles of eye motility. Over the last few decades, evidence has accumulated to support that the primary pathologic process of these disorders is neuropathic rather than myopathic. This is believed that for normal development of extra ocular muscles and for preservation of muscle fiber anatomy, normal intra-uterine development of the innervation to these muscles is essential. Congenital dysinnervation to these EOMs can lead to abnormal muscle structure depending upon the stage and the extent of such innervational defects. Over last few years new genes responsible for CCDD have been identified, permitting a better understanding of associated phenotypes, which can further lead to better classification of these disorders. Introduction of high-resolution MRI has led to detailed study of cranial nerves courses and muscles supplied by them. Thus, due to better understanding of pathophysiology and genetics of CCDDs, various treatment modalities can be developed to ensure good ocular alignment and better quality of life for patients suffering from the same.

  6. Congenital Diseases of the Intestine

    NARCIS (Netherlands)

    D. Halim (Danny)

    2016-01-01

    markdownabstractAll research described in this dissertation is focused on understanding the pathophysiology of three rare congenital diseases of the intestine, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), congenital short bowel syndrome (CSBS), and hereditary multiple

  7. Is There a Right Ear Advantage in Congenital Aural Atresia?

    Science.gov (United States)

    Reed, Robert; Hubbard, Matthew; Kesser, Bradley W

    2016-12-01

    To compare speech/language development and academic progress between children with right versus left congenital aural atresia (CAA). Case control survey and review of audiometric data. Tertiary care academic practice. Children with unilateral CAA. Demographic and audiometric data; rates of grade retention, use of any hearing or learning resource, and behavioral problems. No significant differences in grade retention rate, utilization of amplification, speech language therapy, use of an individualized education program, or frequency modulated system were found between children with right versus left CAA. Children with left CAA were significantly more likely to be enrolled in special education programs (p = 0.026). Differences in reported communication problems approached significance with more difficulty noted in the right ear group (p = 0.059). Left CAA patients were also more likely to have reported behavioral problems (p = 0.0039). Contrary to the hypothesis that a normal hearing right ear confers a language advantage in patients with unilateral hearing loss, children with left CAA (normal right ear) were statistically more likely to be enrolled in a special education program and have behavioral problems. Reported communication problems were more common in right CAA patients, but this did not reach statistical significance. No differences were found in use of amplification, frequency modulated system, individualized education program, or grade retention. Further investigation of both the clinical implications and underlying psychoacoustics of unilateral hearing loss and the identification and habilitation of "at risk" unilateral hearing loss children is warranted.

  8. Atypical white-matter microstructure in congenitally deaf adults: A region of interest and tractography study using diffusion-tensor imaging.

    Science.gov (United States)

    Karns, Christina M; Stevens, Courtney; Dow, Mark W; Schorr, Emily M; Neville, Helen J

    2017-01-01

    Considerable research documents the cross-modal reorganization of auditory cortices as a consequence of congenital deafness, with remapped functions that include visual and somatosensory processing of both linguistic and nonlinguistic information. Structural changes accompany this cross-modal neuroplasticity, but precisely which specific structural changes accompany congenital and early deafness and whether there are group differences in hemispheric asymmetries remain to be established. Here, we used diffusion tensor imaging (DTI) to examine microstructural white matter changes accompanying cross-modal reorganization in 23 deaf adults who were genetically, profoundly, and congenitally deaf, having learned sign language from infancy with 26 hearing controls who participated in our previous fMRI studies of cross-modal neuroplasticity. In contrast to prior literature using a whole-brain approach, we introduce a semiautomatic method for demarcating auditory regions in which regions of interest (ROIs) are defined on the normalized white matter skeleton for all participants, projected into each participants native space, and manually constrained to anatomical boundaries. White-matter ROIs were left and right Heschl's gyrus (HG), left and right anterior superior temporal gyrus (aSTG), left and right posterior superior temporal gyrus (pSTG), as well as one tractography-defined region in the splenium of the corpus callosum connecting homologous left and right superior temporal regions (pCC). Within these regions, we measured fractional anisotropy (FA), radial diffusivity (RD), axial diffusivity (AD), and white-matter volume. Congenitally deaf adults had reduced FA and volume in white matter structures underlying bilateral HG, aSTG, pSTG, and reduced FA in pCC. In HG and pCC, this reduction in FA corresponded with increased RD, but differences in aSTG and pSTG could not be localized to alterations in RD or AD. Direct statistical tests of hemispheric asymmetries in these

  9. 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.

  10. Congenital cataract screening

    Directory of Open Access Journals (Sweden)

    Zhale Rajavi

    2016-01-01

    Full Text Available Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender.

  11. Congenital optic nerve anomalies.

    Science.gov (United States)

    Martín-Begué, N; Saint-Gerons, M

    2016-12-01

    To update the current knowledge about congenital optic disc anomalies. A comprehensive literature search was performed in the major biomedical databases. Patients with these anomalies usually have poor vision in infancy. Refractive errors are common, and serous retinal detachment may develop in some of these anomalies. It is critically important to clinically differentiate between these congenital optic disc anomalies, as central nervous system malformations are common in some, whereas others may be associated with systemic anomalies. Congenital optic disc anomalies are a heterogeneous group of pathologies with characteristic fundus appearance and systemic associations. We should always try to make a correct diagnosis, in order to ask for specific tests, as well as to provide an adequate follow-up. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  12. Congenital syphilis: literature review

    Directory of Open Access Journals (Sweden)

    Eduardo Chaida Sonda

    2013-01-01

    Full Text Available Syphilis is an infectious disease caused by Treponema pallidum and has high rates of vertical transmission, which can reach 100% depending on the maternal disease and stage of pregnancy. The diagnosis of gestational syphilis is simple and its screening is required during the prenatal period. However, this disease still has a high prevalence, affecting two million pregnant women worldwide. The procedures performed in newborns with congenital syphilis represent costs that are three-fold higher than the ones spent with a baby without this infection. The treatment is generally carried out with penicillin and must be extended to sexual partners. Inadequate or lack of treatment of congenital syphilis can result in miscarriage, premature birth, acute complications and other fetal sequelae. KEYWORDS: Congenital syphilis. Treponema pallidum. Vertical transmission.

  13. 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.

  14. Auditory maturation and congenital hearing loss in NICU infants

    NARCIS (Netherlands)

    S. Coenraad (Saskia)

    2011-01-01

    textabstractThe number of preterm births has increased over the past decades as a result of increasing maternal age and in vitro fertilization (1). At the same time the survival of preterm infants has increased due to advances in perinatal and neonatal care. For example, antenatal corticosteroids

  15. Congenital syphilis in the newborn.

    OpenAIRE

    V; Chawla; Pandit, P B; Nkrumah, F K

    1988-01-01

    We studied 53 newborn babies with congenital syphilis. The common clinical features seen were low birth weight, hepatosplenomegaly, anaemia, jaundice, and symmetrical superficial desquamation of the skin affecting palms and soles. The presence of these clinical signs is highly suggestive of early congenital syphilis. Hydrops fetalis without rhesus or ABO isoimmunisation should always arouse the suspicion of congenital syphilis.

  16. Congenital Toxoplasmosis: A Review.

    Science.gov (United States)

    Hampton, Marissa Martinez

    2015-01-01

    Acute infection of toxoplasmosis during pregnancy is detrimental to the developing fetus. In the United States, approximately 1 in 10,000 live births are affected by congenital toxoplasmosis. Although multifactorial in etiology, maternal infection is primarily attributed to the consumption of contaminated meat or water. Infection and transmission to the fetus may result in devastating neurologic impairment. Screening methods for all pregnant women should be implemented in routine prenatal care. This article will highlight the inherent dangers of congenital toxoplasmosis, while including general care of the fetus for prevention of transmission, medical management, and long-term outcomes.

  17. Congenital Hemolytic Anemia.

    Science.gov (United States)

    Haley, Kristina

    2017-03-01

    Red blood cell (RBC) destruction can be secondary to intrinsic disorders of the RBC or to extrinsic causes. In the congenital hemolytic anemias, intrinsic RBC enzyme, RBC membrane, and hemoglobin disorders result in hemolysis. The typical clinical presentation is a patient with pallor, anemia, jaundice, and often splenomegaly. The laboratory features include anemia, hyperbilirubinemia, and reticulocytosis. For some congenital hemolytic anemias, splenectomy is curative. However, in other diseases, avoidance of drugs and toxins is the best therapy. Supportive care with transfusions are also mainstays of therapy. Chronic hemolysis often results in the formation of gallstones, and cholecystectomy is often indicated. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Congenital maxillary double lip

    Directory of Open Access Journals (Sweden)

    Dinesh Singh Chauhan

    2012-01-01

    Full Text Available Double lip, also referred to as "macrocheilia," is a rare anomaly which affects the upper lip more commonly than the lower lip. It consists of a fold of excess or redundant hypertrophic tissue on the mucosal side of the lip. The congenital double lip is believed to be present at birth and becomes more prominent after eruption of teeth. It affects esthetics and also interferes with speech and mastication. Simple surgical excision produces good functional and cosmetic results. We report a case of a non-syndromic congenital maxillary double lip in a 21-year-old male patient.

  19. Congenital laryngeal anomalies,

    Directory of Open Access Journals (Sweden)

    Michael J. Rutter

    2014-12-01

    Full Text Available Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants.

  20. Congenital preduodenal portal vein

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang Jin; Hwang, Mi Soo; Huh, Young Soo; Park, Bok Hwan [College of Medicine, Youngnam University, Gyeongsan (Korea, Republic of)

    1991-03-15

    Congenital preduodenal portal vein, first reported by Knight in 1921, is an extremely rare congenital anomaly in which the portal vein passes anteriorly to the duodenum rather than posteriorly in its normal location. It is of surgical significance because it may cause difficulties in operations involving the gall bladder, biliary duct, or duodenum. Recently, we experienced 2 cases of preduodenal portal vein. One was found during surgical exploration for the diagnosis and correction of malrotation of the bowels and the other in a 3 day-old male newborn associated with dextrocardia, situs inversus, and duodenal obstruction by diaphragm. We report these 2 cases with a review of the literature.

  1. Music and hearing aids.

    Science.gov (United States)

    Madsen, Sara M K; Moore, Brian C J

    2014-10-31

    The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. © The Author(s) 2014.

  2. Music and Hearing Aids

    Science.gov (United States)

    Moore, Brian C. J.

    2014-01-01

    The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. PMID:25361601

  3. Hearing: Noise-Induced Hearing Loss

    Science.gov (United States)

    ... is not hearing high-pitched sounds, like the singing of birds, or not understanding speech when in ... radio. Music, the sounds of nature, and the voices of loved ones can bring you pleasure; sirens ...

  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. Bone-anchored hearing aid implantation in a patient with Goldenhar syndrome.

    Science.gov (United States)

    Santarelli, Griffin; Redfern, Roberta E; Benson, Aaron G

    2015-12-01

    Patients with Goldenhar syndrome exhibit a number of characteristic symptoms, including middle and internal ear malformations that may cause profound hearing loss. Bone-anchored hearing aids have been used to treat these patients in the past, although complications may arise due to the nature of the disease. Herein we present the case of a pediatric patient with Goldenhar syndrome whose hearing aid abutment extruded spontaneously because of poor bone quality, despite adequate thickness. We provide a brief review of the literature and suggest a flexible surgical plan for any syndromic pediatric patient.

  6. Early motor development of children with a congenital cytomegalovirus infection.

    Science.gov (United States)

    De Kegel, Alexandra; Maes, Leen; Dhooge, Ingeborg; van Hoecke, Helen; De Leenheer, Els; Van Waelvelde, Hilde

    2016-01-01

    Congenital cytomegalovirus (cCMV) infection is the most important etiology of non-hereditary childhood hearing loss and an important cause of neurodevelopmental delay. The current study aimed to investigate the early motor development of symptomatic and asymptomatic cCMV infected children with and without sensorineural hearing loss (SNHL). Sixty-four children with a cCMV infection, without cerebral palsy, were compared to a control group of 107 normal hearing children. They were assessed around the ages of 6, 12, and 24 months with the Peabody Developmental Motor Scales-2 (PDMS-2), Alberta Infant Motor Scales (AIMS), and Ghent Developmental Balance Test (GDBT). The cCMV infected children were subdivided into a symptomatic (n=26) and asymptomatic cCMV group (n=38) but also into a cCMV group with SNHL (n=19) and without SNHL (n=45). Symptomatic cCMV infected children and cCMV infected children with SNHL performed significantly weaker for all gross motor outcome measures. A congenital CMV infection is a risk factor for a delay in the early motor development. Follow-up will be necessary to gain insight into the exact cause of this motor delay and to define the predictive value of early motor assessment of cCMV infected children. Copyright © 2015 Elsevier Ltd. All rights reserved.

  7. Children with profound intellectual and multiple disabilities : the effects of functional movement activities

    NARCIS (Netherlands)

    van der Putten, A; Vlaskamp, C; Reynders, K; Nakken, H

    Objective: To determine the effect of functional movement activities within the MOVE ( Mobility Opportunities Via Education) curriculum on the independence of children with profound intellectual and multiple disabilities. Subjects: Forty-four children with profound intellectual and multiple

  8. Do You Hear What Horton Hears?

    Science.gov (United States)

    Snyder, Robert; Johnson, Jordan

    2010-01-01

    "I've never heard of a small speck of dust that is able to yell" says Horton of a sound he hears well (Geisel 1954). It is always valuable to connect science to student's interests and their everyday world--so what better way to teach concepts relating to sound than to read "Horton Hears a Who" by Dr. Seuss? Here the authors present several…

  9. [Semi-implantable transcutaneous bone conduction hearing devices].

    Science.gov (United States)

    Siegert, R; Kanderske, J

    2014-07-01

    Patients with air-bone gaps or combined hearing loss that cannot be corrected by tympanoplasty can be treated with bone conduction hearing aids. The disadvantages of conventional and percutaneous systems are, on one hand, the obvious external fixation components and on the other hand, the biological and psychosocial problems of open implants. This project was therefore set up to develop a semi-implantable, magnetically anchored transcutaneous bone conduction device, introduce it into clinical application and follow-up the results. The principle of this bone conduction device is the magnetic coupling of an external vibrator via implanted double magnets. After extensive laboratory tests, this method was introduced into the clinic in 2006. Following the initial pilot study, 184 implantations in 143 patients have been performed in Recklinghausen since 2008. Long-term evaluation of 20 congenital atresia patients treated with these devices was possible. Worldwide, more than 3000 of these devices have been implanted. The operative implantation technique is relatively simple. With the new "upside down technique", bone removal is no longer necessary. The 2.6-mm thin implants are hardly noticeable. The hearing improvement is similar to that of other bone conduction hearing aids. This semi-implantable transcutaneous bone conduction hearing device is another option for patients with air-bone gaps, combined hearing loss or single-sided deafness.

  10. Long-term asymmetric hearing affects cochlear implantation outcomes differently in adults with pre- and postlingual hearing loss.

    Science.gov (United States)

    Boisvert, Isabelle; McMahon, Catherine M; Dowell, Richard C; Lyxell, Björn

    2015-01-01

    In many countries, a single cochlear implant is offered as a treatment for a bilateral hearing loss. In cases where there is asymmetry in the amount of sound deprivation between the ears, there is a dilemma in choosing which ear should be implanted. In many clinics, the choice of ear has been guided by an assumption that the reorganisation of the auditory pathways caused by longer duration of deafness in one ear is associated with poorer implantation outcomes for that ear. This assumption, however, is mainly derived from studies of early childhood deafness. This study compared outcomes following implantation of the better or poorer ear in cases of long-term hearing asymmetries. Audiological records of 146 adults with bilateral hearing loss using a single hearing aid were reviewed. The unaided ear had 15 to 72 years of unaided severe to profound hearing loss before unilateral cochlear implantation. 98 received the implant in their long-term sound-deprived ear. A multiple regression analysis was conducted to assess the relative contribution of potential predictors to speech recognition performance after implantation. Duration of bilateral significant hearing loss and the presence of a prelingual hearing loss explained the majority of variance in speech recognition performance following cochlear implantation. For participants with postlingual hearing loss, similar outcomes were obtained by implanting either ear. With prelingual hearing loss, poorer outcomes were obtained when implanting the long-term sound-deprived ear, but the duration of the sound deprivation in the implanted ear did not reliably predict outcomes. Contrary to an apparent clinical consensus, duration of sound deprivation in one ear has limited value in predicting speech recognition outcomes of cochlear implantation in that ear. Outcomes of cochlear implantation are more closely related to the period of time for which the brain is deprived of auditory stimulation from both ears.

  11. Influence of Implantable Hearing Aids and Neuroprosthesison Music Perception

    Directory of Open Access Journals (Sweden)

    Torsten Rahne

    2012-01-01

    Full Text Available The identification and discrimination of timbre are essential features of music perception. One dominating parameter within the multidimensional timbre space is the spectral shape of complex sounds. As hearing loss interferes with the perception and enjoyment of music, we approach the individual timbre discrimination skills in individuals with severe to profound hearing loss using a cochlear implant (CI and normal hearing individuals using a bone-anchored hearing aid (Baha. With a recent developed behavioral test relying on synthetically sounds forming a spectral continuum, the timbre difference was changed adaptively to measure the individual just noticeable difference (JND in a forced-choice paradigm. To explore the differences in timbre perception abilities caused by the hearing mode, the sound stimuli were varied in their fundamental frequency, thus generating different spectra which are not completely covered by a CI or Baha system. The resulting JNDs demonstrate differences in timbre perception between normal hearing individuals, Baha users, and CI users. Beside the physiological reasons, also technical limitations appear as the main contributing factors.

  12. Influence of Implantable Hearing Aids and Neuroprosthesison Music Perception

    Science.gov (United States)

    Rahne, Torsten; Böhme, Lars; Götze, Gerrit

    2012-01-01

    The identification and discrimination of timbre are essential features of music perception. One dominating parameter within the multidimensional timbre space is the spectral shape of complex sounds. As hearing loss interferes with the perception and enjoyment of music, we approach the individual timbre discrimination skills in individuals with severe to profound hearing loss using a cochlear implant (CI) and normal hearing individuals using a bone-anchored hearing aid (Baha). With a recent developed behavioral test relying on synthetically sounds forming a spectral continuum, the timbre difference was changed adaptively to measure the individual just noticeable difference (JND) in a forced-choice paradigm. To explore the differences in timbre perception abilities caused by the hearing mode, the sound stimuli were varied in their fundamental frequency, thus generating different spectra which are not completely covered by a CI or Baha system. The resulting JNDs demonstrate differences in timbre perception between normal hearing individuals, Baha users, and CI users. Beside the physiological reasons, also technical limitations appear as the main contributing factors. PMID:22645422

  13. The congenital cranial dysinnervation disorders.

    Science.gov (United States)

    Gutowski, N J; Chilton, J K

    2015-07-01

    Congenital cranial dysinnervation disorders (CCDD) encompass a number of related conditions and includes Duane syndrome, congenital fibrosis of the external ocular muscles, Möbius syndrome, congenital ptosis and hereditary congenital facial paresis. These are congenital disorders where the primary findings are non-progressive and are caused by developmental abnormalities of cranial nerves/nuclei with primary or secondary dysinnervation. Several CCDD genes have been found, which enhance our understanding of the mechanisms involved in brain stem development and axonal guidance. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  14. Behavioral effects of congenital ventromedial prefrontal cortex malformation

    Directory of Open Access Journals (Sweden)

    Boes Aaron D

    2011-12-01

    Full Text Available Abstract Background A detailed behavioral profile associated with focal congenital malformation of the ventromedial prefrontal cortex (vmPFC has not been reported previously. Here we describe a 14 year-old boy, B.W., with neurological and psychiatric sequelae stemming from focal cortical malformation of the left vmPFC. Case Presentation B.W.'s behavior has been characterized through extensive review Patience of clinical and personal records along with behavioral and neuropsychological testing. A central feature of the behavioral profile is severe antisocial behavior. He is aggressive, manipulative, and callous; features consistent with psychopathy. Other problems include: egocentricity, impulsivity, hyperactivity, lack of empathy, lack of respect for authority, impaired moral judgment, an inability to plan ahead, and poor frustration tolerance. Conclusions The vmPFC has a profound contribution to the development of human prosocial behavior. B.W. demonstrates how a congenital lesion to this cortical region severely disrupts this process.

  15. The comparative study of reading comprehension in normal-hearing and hearing-loss student

    Directory of Open Access Journals (Sweden)

    Gholamali Afrooz

    2012-06-01

    Full Text Available Background and Aim: In different language approaches, reading is considered as a major skill. Language skills comprising listening, speaking, reading and writing are completely interlinked with each other. Thus, providing active application of listening and speaking skills, children will learn reading and writing skills easily. The aim of this research was to compare reading skills of hearing-loss students studying at exceptional schools with normal students of different degrees.Methods: The research was conducted post-traumatically using standardized 2006 PIRLS test. 80 students in fifth grade of exceptional primary schools, and first and fourth grade of exceptional high schools in Tehran City who had severe and or profound hearing loss were randomly selected along with 80 normal students in ordinary schools who were selected using available sampling method.Results: There were significant differences between reading comprehension of hearing-loss and normal students of all three grades (p<0.001. Reading comprehension in female students was significantly higher than the male ones (p<0.001. There were not any significant differences in mean reading comprehension scores of hearing-loss students in different grades which demonstrate that their reading skills had not developed in tandem with their age and school educations.Conclusion: This study shows that one of the significant concerns regarding hearing-loss students is their reading comprehension ability. Considering reading ability as one of the most important acquisitive abilities which students learn at school, evaluation of reading skills of hearing loss students as well as recognition of their strengths and weaknesses in this field is indispensable.

  16. How to quantify binaural hearing in patients with unilateral hearing using hearing implants

    NARCIS (Netherlands)

    Snik, A.F.M.; Agterberg, M.J.H.; Bosman, A.

    2015-01-01

    Application of bilateral hearing devices in bilateral hearing loss and unilateral application in unilateral hearing loss (second ear with normal hearing) does not a priori lead to binaural hearing. An overview is presented on several measures of binaural benefits that have been used in patients with

  17. [Inner Ear Hearing Loss].

    Science.gov (United States)

    Hesse, G

    2016-06-01

    Hearing loss is one of the most dominant handicaps in modern societies, which additionally very often is not realized or not admitted. About one quarter of the general population suffers from inner ear hearing loss and is therefore restricted in communicational skills. Demographic factors like increasing age play an important role as well as environmental influences and an increasing sound and noise exposure especially in leisure activities. Thus borders between a "classical" presbyacusis - if it ever existed - and envirionmentally induced hearing loss disappear. Today restrictions in hearing ability develop earlier in age but at the same time they are detected and diagnosed earlier. This paper can eventually enlighten the wide field of inner ear hearing loss only fragmentarily; therefore mainly new research, findings and developments are reviewed. The first part discusses new aspects of diagnostics of inner ear hearing loss and different etiologies. © Georg Thieme Verlag KG Stuttgart · New York.

  18. Congenital Lumbar Hernia

    OpenAIRE

    Sanjay Sharma; Gagan Bali; Satish Parihar; Neeraj Koul

    2008-01-01

    Lumbar hernia is a rare hernia. It constitutes less than one percent of all abdominal hernias. It can becongenital or acquired. Acquired can occur either spontaneously or after surgery or trauma. Only 300cases of lumbar hernia are reported till date. We report a case of congenital lumbar hernia in one month oldmale baby

  19. CONGENITAL URETHROPERINEAL FISTULA: REPORT

    African Journals Online (AJOL)

    CONGENiTAL URETHROPERINEAL FISTULA l8 A'DISTINCT TYPE OF URETHRAL DUPLlCATION. gram revealed a normal dorsal urethra and failed to opacity the fistulous tract, but the fis- tula was demonstrated by injection of contrast through the perineal orifice (tistulogram). Cystourethroscopy revealed a normal dor~.

  20. Congenital heart disease

    Science.gov (United States)

    ... for acne, chemicals, alcohol, and infections (such as rubella ) during pregnancy can contribute to some congenital heart problems. Poorly ... medicines. Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible ...

  1. EAMJ March -Congenital

    African Journals Online (AJOL)

    iMac User

    INTRODUCTION. Mild degrees of congenital lymphoedema are considered common in the normal population, reflecting normal developmental variability in the regression of the lymphoedema present in every fetus before birth (1). The most common clinical causes of lymphedema are generally not inherited, occurring as.

  2. Pseudoamblyopia in Congenital Cyclotropia

    Directory of Open Access Journals (Sweden)

    Antonio Frattolillo

    2017-01-01

    Full Text Available Purpose. To study the effect of surgery on amblyopia and suppression associated with congenital cyclovertical strabismus. Methods. The fixation pattern was investigated with microperimetry before and soon after surgery in ten consecutive children operated for congenital superior oblique palsy at the S. Martino Hospital, Belluno, Italy, between September 2014 and December 2015. Changes in visual performance in terms of best-corrected visual acuity (BCVA and stereopsis between the day before and one week after surgery were also evaluated. No other amblyopia treatment has been administered during the time study. Results. Surgical correction of the excyclodeviation in congenital SO palsy determined monocular and binocular sensory consequences: monocularly, in the cyclodeviated amblyopic eye, BCVA (0.46–0.03 LogMAR; p<0.0001 and the fixation pattern improved, as demonstrated by microperimetry examination. Binocularly, stereopsis improved or emerged while suppression at the Worth four-dot test disappeared. Conclusions. In the absence of further amblyopic factors such as coexisting constant vertical and/or horizontal deviation and anisometropia, the amblyopia encountered in congenital SO palsy may resolve soon after the surgical alignment. Therefore, it may be considered and defined “pseudoamblyopia.”

  3. Severe congenital neutropenia

    DEFF Research Database (Denmark)

    Borregaard, Niels

    2014-01-01

    In this issue of Blood, Tidwell et al1 demonstrate that mutations in the start codon (protein synthesis is initiated at the codon ATG) of neutrophil elastase (ELANE) result in the production of N-terminally truncated elastase, which mislocates to the nucleus and results in severe congenital neutr...... neutropenia (SCN)....

  4. Multiple congenital cranial hemangiomas

    Energy Technology Data Exchange (ETDEWEB)

    Koulouris, George [Alfred Hospital, Department of Radiology, Prahran, Victoria (Australia); Rao, Padma [Royal Children' s Hospital, Department of Radiology, Parkville, Victoria (Australia)

    2005-08-01

    Though cranial hemangiomas are second only to vertebral hemangiomas in frequency, such lesions are rarely congenital and multiple. It is probable that the true incidence of congenital calvarial hemangiomas is higher than that reported in the literature, as they are unlikely to undergo imaging, most being asymptomatic and without a significant soft tissue component. We present a case of multiple congenital calvarial and skull base cavernous-type hemangiomas, diagnosed in a 4-day-old female, involving the right zygoma, maxilla, frontal and petrous temporal bones and contralateral squamous temporal bone. Surgical biopsy confirmed the radiological diagnosis as well as the concomitant multiple subcutaneous capillary-type hemangiomas which were identified clinically. No specific clinical syndrome or chromosomal abnormality was identified and the underlying cerebral parenchyma was normal with no intra-axial involvement. With conservative treatment, two lesions completely resolved and a further two lesions subsequently decreased in both size and degree of enhancement. To the best of our knowledge, this is the first case of multiple congenital hemangiomas involving the calvarium and skull base. Despite this, the radiological features, combined with the clinical findings of multiple capillary hemangiomas, were characteristic enough to permit an accurate preoperative diagnosis. Osseous hemangiomas should feature prominently in any differential diagnosis of multiple hypervascular lesions, as they are common, more so when limited to an anatomical region, irrespective of site or age. (orig.)

  5. EAMJ March -Congenital

    African Journals Online (AJOL)

    iMac User

    the intestines, but were also found in the pleura, pericardium, thyroid gland, and kidney. Several patients demonstrated congenital cardiac and blood vessel anomalies, pointing to a disturbance of angiogenesis in at least some of the patients. Facial features were variable, and were chiefly characterised, in a typical patient, ...

  6. Congenital Heart Defects

    Science.gov (United States)

    ... of the heart. It is present at birth. Congenital heart defects are the most common type of birth defect. The defects can involve the ... and heart transplants. The treatment depends on the type of the defect, how ... and general health. NIH: National Heart, Lung, and Blood Institute

  7. Congenital aggressive lipomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Lachmann, R.S.; Mehringer, C.M.; Finklestein, J.; Maenza, R.

    1983-05-01

    Three cases of congenital lipomatosis involving the thoracic region posteriorly are presented delineating the natural history of the disease and depicting underlying bone and soft tissue changes. The rib widening and pleural thickening appear to be related to increased intercostal vascularity feeding the overlying tumor, as delineated by angiography. This entity is not described in the radiologic literature.

  8. EAMJ Congenital.indd

    African Journals Online (AJOL)

    2010-02-02

    Feb 2, 2010 ... Congenital afibrinogenaemia (CA), is a rare inherited bleeding disorder characterised by complete deficiency of fibrinogen in the plasma. Blood clotting tests are indefinitely prolonged in patients. The mode of inheritance is autosomal recessive. Typically patients present with excessive cord bleeding after ...

  9. Congenital syphilis who risk?

    African Journals Online (AJOL)

    1989-08-05

    Aug 5, 1989 ... The prevalence of syphilis (or positive serology) in pregnant mothers delivering at Baragwanath Hospital, Johannesburg, was assessed in order to try to establish the prevalence of congenital syphilis and possibly to identify a specific popula- tion at risk. From August 1985 to January 1986 all mothers.

  10. Congenital Short QT Syndrome

    Directory of Open Access Journals (Sweden)

    Johnson Francis

    2016-01-01

    Full Text Available Congenital short QT syndrome (SQTS is characterised by extremely short QT intervals, typically with QTc less than 330 ms and a propensity for life threatening ventricular arrhythmias and atrial fibrillation. The QT interval in SQTS does not change significantly with heart rate and the T waves have a narrow base and high voltage, similar to those in hyperkalemia.

  11. Classic congenital adrenal hyperplasia.

    Science.gov (United States)

    Nermoen, Ingrid; Husebye, Eystein S; Myhre, Anne Grethe; Løvås, Kristian

    2017-04-01

    Congenital adrenal hyperplasia is attributed to inherited enzyme defects in the adrenal cortex. The classical form results in reduced production of cortisol and aldosterone, accompanied by an increase in production of adrenal cortical androgens. This causes virilisation in girls, adrenocortical failure and early puberty in both sexes. This article describes the genetics, clinical picture, diagnostics and treatment.

  12. GJB2 mutations and degree of hearing loss: a multicenter study.

    NARCIS (Netherlands)

    Snoeckx, R.L.; Huygen, P.L.M.; Feldmann, D.; Marlin, S.; Denoyelle, F.; Waligora, J.; Mueller-Malesinska, M.; Pollak, A.; Ploski, R.; Murgia, A.; Orzan, E.; Castorina, P.; Ambrosetti, U.; Nowakowska-Szyrwinska, E.; Bal, J.; Wiszniewski, W.; Janecke, A.R.; Nekahm-Heis, D.; Seeman, P.; Bendova, O.; Kenna, M.A.; Frangulov, A.; Rehm, H.L.; Tekin, M.; Incesulu, A.; Dahl, H.H.; Sart, D. du; Jenkins, L.; Lucas, D.; Bitner-Glindzicz, M.; Avraham, K.B.; Brownstein, Z.; Castillo, I. del; Moreno, F.; Blin, N.; Pfister, M.; Sziklai, I.; Toth, T.; Kelley, P.M.; Cohn, E.S.; Maldergem, L. van; Hilbert, P.; Roux, A.F.; Mondain, M.; Hoefsloot, L.H.; Cremers, C.W.R.J.; Lopponen, T.; Lopponen, H.; Parving, A.; Gronskov, K.; Schrijver, I.; Roberson, J.; Gualandi, F.; Martini, A.; Lina-Granade, G.; Pallares-Ruiz, N.; Correia, C.; Fialho, G.; Cryns, K.; Hilgert, N.; Heyning, P. van de; Nishimura, C.J.; Smith, R.J.H.; Camp, G. van

    2005-01-01

    Hearing impairment (HI) affects 1 in 650 newborns, which makes it the most common congenital sensory impairment. Despite extraordinary genetic heterogeneity, mutations in one gene, GJB2, which encodes the connexin 26 protein and is involved in inner ear homeostasis, are found in up to 50% of

  13. Hearing aid adjustment

    DEFF Research Database (Denmark)

    Heinemann, Trine; Matthews, Ben; Raudaskoski, Pirkko Liisa

    2012-01-01

    to the interaction during hearing aid fitting. This report of a Danish pilot study describes two such problems. The first problem arises from the requirement that the audiologist needs to ‘translate’ the patient’s subjective hearing description for making technological decisions. The second problem is the way...... in which the hearing aid user’s implicit and often unrealistic expectations are handled. This kind of research has potential application for developing a model of best practices....

  14. Hearing and Speech at Seven

    Science.gov (United States)

    Sheridan, Mary D.; Peckham, Catherine S.

    1973-01-01

    Evaluated for social and educational aspects at 7 years of age were 133 children with moderate hearing loss, 46 children with severe unilateral hearing loss, and 215 children with normal hearing but with unintelligible speech. (DB)

  15. Noise and Hearing Loss Prevention

    Science.gov (United States)

    ... visit this page: About CDC.gov . NOISE AND HEARING LOSS PREVENTION Recommend on Facebook Tweet Share Compartir Hearing ... noise levels cannot be adequately reduced. Noise and Hearing Loss on the NIOSH Science Blog Read and comment ...

  16. International hearing protector standardization

    DEFF Research Database (Denmark)

    Poulsen, Torben

    2002-01-01

    Hearing protectors shall fulfill some minimum requirements to their performance. As hearing protector manufacturers sell the products all over the world, the testing and certification of hearing protectors has become an international issue. The ISO working group WG17 under the headlines Acoustics......, Noise, produce hearing protector standards to be used at an international level. The presentation will cover the ongoing work in WG17, including the revision of existing standards (ISO 4869-1, ISO 4869-3), upcoming new standards (ISO 4869-7) and the plans and status for future standards (performance...

  17. Hearing Conservation Live #2430

    Energy Technology Data Exchange (ETDEWEB)

    Chochoms, Michael [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2016-08-09

    Occupational hearing loss is one of the most common work-related illnesses in the United States (US). From 22 to 30 million US workers are exposed to hazardous noise levels at work, and 25% of these workers will develop permanent hearing loss. Hearing loss from noise is slow and painless, and you can have a disability before you notice it. This course presents the hazards associated with workplace noise, the purpose and elements of the Los Alamos National Laboratory (LANL) Hearing Conservation Program (HCP), and controls that are available to reduce your exposure to hazardous levels of noise.

  18. Linguistic Profiles of Children with CI as Compared with Children with Hearing or Specific Language Impairment

    Science.gov (United States)

    Hoog, Brigitte E.; Langereis, Margreet C.; Weerdenburg, Marjolijn; Knoors, Harry E. T.; Verhoeven, Ludo

    2016-01-01

    Background: The spoken language difficulties of children with moderate or severe to profound hearing loss are mainly related to limited auditory speech perception. However, degraded or filtered auditory input as evidenced in children with cochlear implants (CIs) may result in less efficient or slower language processing as well. To provide insight…

  19. Linguistic profiles of children with CI as compared with children with hearing or specific language impairment

    NARCIS (Netherlands)

    Hoog, B.E. de; Langereis, M.C.; Weerdenburg, M. van; Knoors, H.E.; Verhoeven, L.

    2016-01-01

    BACKGROUND: The spoken language difficulties of children with moderate or severe to profound hearing loss are mainly related to limited auditory speech perception. However, degraded or filtered auditory input as evidenced in children with cochlear implants (CIs) may result in less efficient or

  20. Content Analysis of Memory and Memory-Related Research Studies on Children with Hearing Loss

    Science.gov (United States)

    Dogan, Murat; Hasanoglu, Gülcihan

    2016-01-01

    Memory plays a profound role in explaining language development, academic learning, and learning disabilities. Even though there is a large body of research on language development, literacy skills, other academic skills, and intellectual characteristics of children with hearing loss, there is no holistic study on their memory processes.…

  1. Communicative Competence of Pre-School, Afrikaans-Speaking, Hearing-Impaired Children.

    Science.gov (United States)

    Rubin, Esther

    1988-01-01

    The communicative competence of 12 Afrikaans-speaking children (mean age 4 years), with severe to profound hearing, loss was evaluated. Among findings was that the subjects were resourceful in using the context effectively in conversational interactions and frequently used nonverbal cues such as gesture. (DB)

  2. Cross-Modal Re-Organization in Clinical Populations with Hearing Loss

    Directory of Open Access Journals (Sweden)

    Anu Sharma

    2016-01-01

    Full Text Available We review evidence for cross-modal cortical re-organization in clinical populations with hearing loss. Cross-modal plasticity refers to the ability for an intact sensory modality (e.g., vision or somatosensation to recruit cortical brain regions from a deprived sensory modality (e.g., audition to carry out sensory processing. We describe evidence for cross-modal changes in hearing loss across the age-spectrum and across different degrees of hearing impairment, including children with profound, bilateral deafness with cochlear implants, single-sided deafness before and after cochlear implantation, and adults with early-stage, mild-moderate, age-related hearing loss. Understanding cross-modal plasticity in the context of auditory deprivation, and the potential for reversal of these changes following intervention, may be vital in directing intervention and rehabilitation options for clinical populations with hearing loss.

  3. Screening of the hearing of newborns - Update

    Directory of Open Access Journals (Sweden)

    von Voß, Hubertus

    2006-11-01

    Full Text Available Introduction: Permanent congenital bilateral hearing loss (CHL of moderate or greater degree (≥40 dB HL is a rare disease, with a prevalence of about 1 to 3 per 1000 births. However, it is one of the most frequent congenital diseases. Reliance on physician observation and parental recognition has not been successful in the past in detecting significant hearing loss in the first year of life. With this strategy significant hearing losses have been detected in the second year of life. With two objective technologies based on physiologic response to sound, otoacoustic emissions (OAE and auditory brainstem response (ABR hearing screening in the first days of life is made possible. Objectives: The objective of this health technology assessment report is to update the evaluation on clinical effectiveness and cost-effectiveness of newborn hearing screening programs. Universal newborn hearing screening (UHNS (i, selective screening of high risk newborns (ii, and the absence of a systematic screening program are compared for age at identification and age at hearing aid fitting of children with hearing loss. Secondly the potential benefits of early intervention are analysed. Costs and cost-effectiveness of newborn hearing screening programs are determined. This report is intended to make a contribution to the decision making whether and under which conditions a newborn hearing screening program should be reimbursed by the statutory sickness funds in Germany. Methods: This health technology assessment report updates a former health technology assessment (Kunze et al. 2004 [1]. A systematic review of the literature was conducted, based on a documented search and selection of the literature using predefined inclusion and exclusion criteria and a documented extraction and appraisal of the included studies. To assess the cost-effectiveness of the different screening strategies in Germany the decision analytic Markov state model which had been developed in

  4. Hearing Evaluation in Children (For Parents)

    Science.gov (United States)

    ... of Braces Eating Disorders Mitral Valve Prolapse Arrhythmias Hearing Evaluation in Children KidsHealth > For Parents > Hearing Evaluation ... hearing screened early and checked regularly. Causes of Hearing Loss Hearing loss is a common birth defect, ...

  5. Genetics Home Reference: nonsyndromic hearing loss

    Science.gov (United States)

    ... factor for hearing loss. Age-related hearing loss (presbycusis) is thought to have both genetic and environmental ... Encyclopedia: Hearing or speech impairment - resources Health Topic: Hearing Disorders and Deafness Health Topic: Hearing Problems in Children Health Topic: ...

  6. [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.

  7. Congenital and compensated vestibular dysfunction in childhood: an overlooked entity.

    Science.gov (United States)

    Weiss, Avery H; Phillips, James O

    2006-07-01

    We report five children with previously unrecognized vestibular dysfunction detected by clinical examination and confirmed by quantitative vestibular testing. Patient 1 presented with fluctuating visual acuity and intermittent nystagmus. Patient 2 had congenital hearing loss associated with imbalance, delayed motor development, and cyclic vomiting. Patient 3 had neurotrophic keratitis with an intermittent head tilt, imbalance, and motor delays. Patient 4 showed ataxia and eye movement abnormalities following traumatic brain injury and had reading difficulties. Patient 5 had episodic vertigo and eye movement abnormalities from infancy. Clinical vestibular testing emphasized spontaneous nystagmus, rapid head thrust, and assessment of post-rotatory nystagmus. Quantitative vestibular testing included the sinusoidal chair rotation and velocity step tests, measurement of dynamic visual acuity, post-head-shake nystagmus, and computerized platform posturography. Pediatric neurologists encounter children with congenital and compensated vestibular dysfunction, which can be recognized on the basis of relevant history and clinical abnormalities of the ocular-ocular reflex.

  8. Neuroimaging findings of congenital Zika virus infection: a pictorial essay.

    Science.gov (United States)

    Zare Mehrjardi, Mohammad; Poretti, Andrea; Huisman, Thierry A G M; Werner, Heron; Keshavarz, Elham; Araujo Júnior, Edward

    2017-03-01

    Zika virus (ZIKV) is a mosquito-borne arbovirus from the Flaviviridae family. It had caused several epidemics since its discovery in 1947, but there was no significant attention to this virus until the recent outbreak in Brazil in 2015. The main concern is the causal relationship between prenatal ZIKV infection and congenital microcephaly, which has been confirmed recently. Moreover, ZIKV may cause other central nervous system abnormalities such as brain parenchymal atrophy with secondary ventriculomegaly, intracranial calcification, malformations of cortical development (such as polymicrogyria, and lissencephaly-pachygyria), agenesis/hypoplasia of the corpus callosum, cerebellar and brainstem hypoplasia, sensorineural hearing-loss, and ocular abnormalities as well as arthrogryposis in the infected fetuses. Postnatal (acquired) ZIKV infection usually has an asymptomatic or mildly symptomatic course, while prenatal (congenital) ZIKV infection has a more severe course and may cause severe brain anomalies that are described as congenital Zika syndrome. In this pictorial essay, we aim to illustrate the prenatal and postnatal neuroimaging findings that may be seen in fetuses and neonates with congenital Zika syndrome, and will discuss possible radiological differential diagnoses. A detailed knowledge of these findings is paramount for an early correct diagnosis, prognosis determination, and counseling of the affected children and families.

  9. Acoustic temporal modulation detection in normal-hearing and cochlear implanted listeners: effects of hearing mechanism and development.

    Science.gov (United States)

    Park, Min-Hyun; Won, Jong Ho; Horn, David L; Rubinstein, Jay T

    2015-06-01

    Temporal modulation detection ability matures over many years after birth and may be particularly sensitive to experience during this period. Profound hearing loss during early childhood might result in greater perceptual deficits than a similar loss beginning in adulthood. We tested this idea by measuring performance in temporal modulation detection in profoundly deaf children and adults fitted with cochlear implants (CIs). At least two independent variables could constrain temporal modulation detection performance in children with CIs: altered encoding of modulation information due to the CI-auditory nerve interface, and atypical development of central processing of sound information provided by CIs. The effect of altered encoding was investigated by testing subjects with one of two different hearing mechanisms (normal hearing vs. CI) and the effect of atypical development was studied by testing two different age groups. All subjects were tested for their ability to detect acoustic temporal modulations of sound amplitude. A comparison of the slope, or cutoff frequency, of the temporal modulation transfer functions (TMTFs) among the four subject groups revealed that temporal resolution was mainly constrained by hearing mechanism: normal-hearing listeners could detect smaller amplitude modulations at high modulation frequencies than CI users. In contrast, a comparison of the height of the TMTFs revealed a significant interaction between hearing mechanism and age group on overall sensitivity to temporal modulation: sensitivity was significantly poorer in children with CIs, relative to the other three groups. Results suggest that there is an age-specific vulnerability of intensity discrimination or non-sensory factors, which subsequently affects sensitivity to temporal modulation in prelingually deaf children who use CIs.

  10. Profound sedation with propofol modifies atrial fibrillation dynamics.

    Science.gov (United States)

    Cervigón, Raquel; Moreno, Javier; Pérez-Villacastín, Julián; Castells, Francisco

    2013-09-01

    During atrial fibrillation (AF), multiple wandering propagation wavelets at high rates drift around both atria under controversial hierarchical models. Antiarrhythmic drugs modify the cardiac ionic currents supporting the fibrillation process within the atria, and can alter AF propagation dynamics and even terminate the arrhythmia. However, some other drugs, theoretically nonantiarrhythmic, may slightly block particular cardiac ionic currents through uncertain mechanisms in such a subtle way at regular heart rates that may have been pharmacologically overlooked. These potential effects might be better exposed at much higher activation rates as in AF, where atrial cells depolarize over 400 times per second. In this review, we aimed to compile and discuss results from several studies evaluating the net effect of profound sedation with propofol on atrial cells and atrioventricular (AV) conduction. Propofol is a very commonly used anesthetic agent, and its possible effect on AF dynamics has systematically not been taken into account in the myriad of clinical studies dealing with AF intracardiac recordings. The possible effect of sedation with propofol on AF was evaluated through the analysis of AF propagation patterns before and after its infusion in a series of patients submitted to pulmonary vein ablation. Effect on AV conduction will be discussed as well. ©2013, The Authors. Journal compilation ©2013 Wiley Periodicals, Inc.

  11. EFFECT OF JUMPS IN PROFOUNDNESS ON THE FOOTBALLER REFLECTION

    Directory of Open Access Journals (Sweden)

    Nebojša Đošić

    2011-09-01

    Full Text Available The treatment endured one month. For this time 8 trainings and totally 322 jums in profoundness were done with differant altitudes from 42 cm to 105 cm. The complete time of work with time-out between sequences was about 120 minutes. The time-out between the sequences was from 2 ' til 6'. The pause between the training was from 2 to 5 days. The puls after 30 '' from the finishing of the jums in th sequence was from 100 to 140 pulsation in the minute measured by palpation. On the finaly measurement is constated that the two leg jump from place in height was better for 7 cm , and the one leg jump with three footsteps spring was better for 2 cm. This such result indicate on the assumption that the progress must bi greater if the program would be longer , for example two, three months and if this program should be done by footbalers which are active and in the best player years i.e. between 18 -30 year. The author was by this time 40 years old.

  12. Current controversies in diagnosis, management, and prevention of congenital cytomegalovirus: updates for the pediatric practitioner.

    Science.gov (United States)

    Harrison, Gail J

    2015-05-01

    Congenital cytomegalovirus (CMV) infection has been called "the elephant in our living room" because it is a major public health problem that for decades has been unrecognized and unaddressed. Congenital CMV infection is a common cause of sensorineural hearing loss, vision loss, neurodevelopment disabilities, liver disease, and growth failure. Diagnostic tests are now widely available to identify newborns with congenital CMV infection, congenitally infected newborns now can be easily assessed for evidence of organ involvement, and there are now antiviral treatments and other interventions available to improve the outcome in children with congenital CMV disease. A licensed vaccine to prevent CMV infection is not yet available; however, a "CMV knowledge vaccine," composed of "an ounce of CMV awareness and three simple precautions" and that is endorsed by the Centers for Disease Control and Prevention is available for pregnant women who wish to reduce their contact with potentially CMV-infected secretions and therefore reduce their risk of acquiring CMV during pregnancy. Medical experts in the field of congenital CMV have been called upon for a consensus statement for diagnosis and treatment, and nonprofit organizations of families affected by congenital CMV from around the world have formed a collaborative coalition to facilitate the spread of CMV knowledge and awareness. Copyright 2015, SLACK Incorporated.

  13. 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.

  14. 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.

  15. Hospital-based surveillance of congenital rubella syndrome in Indonesia.

    Science.gov (United States)

    Herini, Elisabeth Siti; Gunadi; Triono, Agung; Mulyadi, Asal Wahyuni Erlin; Mardin, Niprida; Rusipah; Soenarto, Yati; Reef, Susan E

    2017-03-01

    Congenital rubella syndrome (CRS) has serious consequences, such as miscarriage, stillbirth, and severe birth defects in infants, resulting from rubella virus infection during pregnancy. However, rubella vaccine has not yet been implemented in Indonesia. This study aimed (1) to estimate the incidence of CRS in Indonesia, (2) describe the clinical features of CRS at our referral hospital, and (3) pilot a CRS surveillance system to be extended to other hospitals. We conducted a 4-month prospective surveillance study of infants aged rubella-specific IgM antibody or rubella IgG antibody levels. Of 47 suspected cases of CRS, 11/47 (23.4%), 9/47 (19.1%), and 27/47 (57.5%) were diagnosed as laboratory-confirmed, clinically compatible, and discarded CRS, respectively. The most common defects among laboratory-confirmed CRS cases were hearing impairment (100%), congenital cataracts (72.7%), microcephaly (72.7%), and congenital heart defects (45.5%). The number of laboratory-confirmed CRS cases among Indonesian infants is high. Furthermore, hearing impairment is the most common clinical feature of CRS in infants. Our findings indicate the importance of implementation of rubella vaccine in Indonesia. Conducting hospital-based surveillance of CRS in other hospitals in Indonesia may be appropriate. What is Known: •Congenital rubella syndrome (CRS) has serious consequences in infants resulting from rubella virus infection during pregnancy. •The incidence of CRS in most developed countries has greatly decreased since implementation of rubella vaccination. •Rubella vaccine has not yet been implemented in many developing countries. What is New: •The number of laboratory-confirmed CRS cases among Indonesian infants was high. •Implementation of rubella vaccine into immunization programs in Indonesia is important because of the high number of CRS cases. •Our study highlights the need for ongoing prospective surveillance of CRS in Indonesia.

  16. Congenital intestinal lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Popović Dušan Đ.

    2011-01-01

    Full Text Available Background. Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. Case report. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and suportive therapy. Conclusion. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  17. Update on congenital glaucoma

    Directory of Open Access Journals (Sweden)

    Mandal Anil

    2011-12-01

    Full Text Available Congenital glaucoma is a global problem and poses a diagnostic and therapeutic challenge to the ophthalmologist. A detailed evaluation under general anesthesia is advisable to establish the diagnosis and plan for management. Medical therapy has a limited role and surgery remains the primary therapeutic modality. While goniotomy or trabeculotomy ab externo is valuable in the management of congenital glaucoma, primary combined trabeculotomy-trabeculectomy offers the best hope of success in advanced cases. Trabeculectomy with antifibrotic agent and glaucoma drainage devices has a role in the management of refractory cases, and cyclodestructive procedures should be reserved for patients where these procedures have failed. Early diagnosis, prompt therapeutic intervention and proper refractive correction are keys to success. Management of residual vision and visual rehabilitation should be an integral part of the management of children with low vision and lifelong follow-up is a must.

  18. Update on congenital glaucoma

    Science.gov (United States)

    Mandal, Anil K; Chakrabarti, Debasis

    2011-01-01

    Congenital glaucoma is a global problem and poses a diagnostic and therapeutic challenge to the ophthalmologist. A detailed evaluation under general anesthesia is advisable to establish the diagnosis and plan for management. Medical therapy has a limited role and surgery remains the primary therapeutic modality. While goniotomy or trabeculotomy ab externo is valuable in the management of congenital glaucoma, primary combined trabeculotomy–trabeculectomy offers the best hope of success in advanced cases. Trabeculectomy with antifibrotic agent and glaucoma drainage devices has a role in the management of refractory cases, and cyclodestructive procedures should be reserved for patients where these procedures have failed. Early diagnosis, prompt therapeutic intervention and proper refractive correction are keys to success. Management of residual vision and visual rehabilitation should be an integral part of the management of children with low vision and lifelong follow-up is a must. PMID:21150027

  19. Congenital diaphramatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    Kline-Fath, Beth M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Fetal Care Center of Cincinnati, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States)

    2012-01-15

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  20. Hearing in Insects.

    Science.gov (United States)

    Göpfert, Martin C; Hennig, R Matthias

    2016-01-01

    Insect hearing has independently evolved multiple times in the context of intraspecific communication and predator detection by transforming proprioceptive organs into ears. Research over the past decade, ranging from the biophysics of sound reception to molecular aspects of auditory transduction to the neuronal mechanisms of auditory signal processing, has greatly advanced our understanding of how insects hear. Apart from evolutionary innovations that seem unique to insect hearing, parallels between insect and vertebrate auditory systems have been uncovered, and the auditory sensory cells of insects and vertebrates turned out to be evolutionarily related. This review summarizes our current understanding of insect hearing. It also discusses recent advances in insect auditory research, which have put forward insect auditory systems for studying biological aspects that extend beyond hearing, such as cilium function, neuronal signal computation, and sensory system evolution.

  1. Hearing Aid Tester

    Science.gov (United States)

    1978-01-01

    Hearing aids often develop malfunctions that are not detected by the wearer. This is particularly true when the wearers are school-age children. Studies of selected groups showed that from 30 to more than 50 percent of school children were not getting adequate benefit from their hearing aids because of unrecognized malfunctions, usually low or dead batteries. This can be serious because hearing impairment retards a child's educational progress. NASA technology incorporated in the Hearing Aid Malfunction Detection Unit (HAMDU), the device pictured, is expected to provide an effective countermeasure to the childrens' hearing aid problem. A patent license has been awarded to a minority-owned firm, Hopkins International Company, a subsidiary of H. H. Aerospace Design Co., Inc., Elmford, New York. The company plans early commercial availability of its version of the device.

  2. Individual Hearing Loss

    Directory of Open Access Journals (Sweden)

    Sébastien Santurette

    2016-06-01

    Full Text Available It is well-established that hearing loss does not only lead to a reduction of hearing sensitivity. Large individual differences are typically observed among listeners with hearing impairment in a wide range of suprathreshold auditory measures. In many cases, audiometric thresholds cannot fully account for such individual differences, which make it challenging to find adequate compensation strategies in hearing devices. How to characterize, model, and compensate for individual hearing loss were the main topics of the fifth International Symposium on Auditory and Audiological Research (ISAAR, held in Nyborg, Denmark, in August 2015. The following collection of papers results from some of the work that was presented and discussed at the symposium.

  3. [Hearing impairment and dementia].

    Science.gov (United States)

    Kilimann, I; Óvari, A; Hermann, A; Witt, G; Pau, H W; Teipel, S

    2015-07-01

    The World Health Organization (WHO) burden of disease study identified dementia and hearing problems as leading causes of loss of quality of life in the industrial world. The prevalence of dementia and hearing problems increases in aging societies. Comorbidity of these two diseases causes increasing demands on healthcare systems. The similarity and possible interaction of symptoms renders diagnosis and therapy of dementia and hearing loss a challenge for neurologists, psychiatrists, ear, nose and throat (ENT) and hearing specialists. Knowledge of both diseases enables an early intervention and helps preserve participation in society and thereby reducing the risk of developing dementia. This paper focuses on the characteristics of the diagnosis and therapy of hearing problems and dementia.

  4. Possible rare congenital dysinnervation disorder: congenital ptosis associated with adduction.

    Science.gov (United States)

    Mendes, Sílvia; Beselga, Diana; Campos, Sónia; Neves, Arminda; Campos, Joana; Carvalho, Sílvia; Silva, Eduardo; Castro Sousa, João Paulo

    2015-01-01

    Ptosis is defined as an abnormally low position of the upper eyelid margin. It can be congenital or acquired, uni or bilateral, and isolated or associated with other ocular and nonocular defects. We report a case of a female child, aged 8 years, with congenital right ptosis increased on right adduction and with left ptosis on left adduction. There was no horizontal ocular movement limitation. Apparent underaction of the right inferior oblique muscle was also present. We believe that within the possible mechanisms it is more likely that it is a congenital innervation dysgenesis syndrome (CID)/congenital cranial dysinnervation disorder (CCDD).

  5. Adjustment Problems of Hearing and Hearing-Impaired Students in ...

    African Journals Online (AJOL)

    The study examined the social and academic adjustment problems of some mainstreamed hearing and hearing-impaired students who were randomly selected from two integrated schools in Ibadan metropolis. The sample consisted of 232 junior secondary school students. 125 of them are hearing while 107 are hearing ...

  6. HearCom: hearing in the communication society

    NARCIS (Netherlands)

    Vlaming, M.S.M.G.; Kollmeier, B.; Dreschler, W.A.; Martin, R.; Wouters, J.; Grover, B.; Mohammadh, Y.; Houtgast, T.

    2011-01-01

    A group of 28 research partners joined the EU-funded project HearCom with the overall aim to improve hearing communication. One of the main achievements has been the provision of advanced hearing screening tests by telephone and Internet. Next to that it was aimed to harmonize hearing diagnostic

  7. HearCom: Hearing in the Communication Society

    NARCIS (Netherlands)

    Vlaming, Marcel S. M. G.; Kollmeier, Birger; Dreschler, Wouter A.; Martin, Rainer; Wouters, Jan; Grover, Brian; Mohammadh, Yehya; Houtgast, Tammo

    2011-01-01

    A group of 28 research partners joined the EU-funded project HearCom with the overall aim to improve hearing communication. One of the main achievements has been the provision of advanced hearing screening tests by telephone and Internet. Next to that it was aimed to harmonize hearing diagnostic

  8. Other congenital abnormalities.

    Science.gov (United States)

    Cobbett, J R

    1974-06-29

    The plastic surgeon is not a miracle worker, as so many of his patients believe. Nevertheless, he can do much to minimize the functional and cosmetic effect of many congenital deformities. If a moral can be drawn from this article it must be that the plastic surgeon should be given an early opportunity to see and assess the patients described here, if only to ease the anxiety in the minds of their parents by appropriate reassurance and discussion.

  9. Congenital syphilis: literature review

    OpenAIRE

    Eduardo Chaida Sonda; Felipe Farias Richter; Graziela Boschetti; Marcela Pase Casasola; Candice Franke Krumel; Cristiane Pimentel Hernandes Machado

    2013-01-01

    Syphilis is an infectious disease caused by Treponema pallidum and has high rates of vertical transmission, which can reach 100% depending on the maternal disease and stage of pregnancy. The diagnosis of gestational syphilis is simple and its screening is required during the prenatal period. However, this disease still has a high prevalence, affecting two million pregnant women worldwide. The procedures performed in newborns with congenital syphilis represent costs that are three-fold higher ...

  10. The Case of Total Deafness II: Phrasing in the Prelinguistic Vocalizations of a Child with Congenital Absence of Cochleas.

    Science.gov (United States)

    Lynch, Michael P.

    1996-01-01

    Reports on the continuing study of a congenitally acochlear child using an analytical focus on the prelinguistic vocalizations involving the description of syllable groupings within a prosodic hierarchy. Results indicate that audition is not necessary for the formation of prelinguistic phrasing, but hearing does influence certain aspects of…

  11. Development of Brainstem-Evoked Responses in Congenital Auditory Deprivation

    Directory of Open Access Journals (Sweden)

    J. Tillein

    2012-01-01

    Full Text Available To compare the development of the auditory system in hearing and completely acoustically deprived animals, naive congenitally deaf white cats (CDCs and hearing controls (HCs were investigated at different developmental stages from birth till adulthood. The CDCs had no hearing experience before the acute experiment. In both groups of animals, responses to cochlear implant stimulation were acutely assessed. Electrically evoked auditory brainstem responses (E-ABRs were recorded with monopolar stimulation at different current levels. CDCs demonstrated extensive development of E-ABRs, from first signs of responses at postnatal (p.n. day 3 through appearance of all waves of brainstem response at day 8 p.n. to mature responses around day 90 p.n.. Wave I of E-ABRs could not be distinguished from the artifact in majority of CDCs, whereas in HCs, it was clearly separated from the stimulus artifact. Waves II, III, and IV demonstrated higher thresholds in CDCs, whereas this difference was not found for wave V. Amplitudes of wave III were significantly higher in HCs, whereas wave V amplitudes were significantly higher in CDCs. No differences in latencies were observed between the animal groups. These data demonstrate significant postnatal subcortical development in absence of hearing, and also divergent effects of deafness on early waves II–IV and wave V of the E-ABR.

  12. Congenital polycythemia in Chuvashia.

    Science.gov (United States)

    Sergeyeva, A; Gordeuk, V R; Tokarev, Y N; Sokol, L; Prchal, J F; Prchal, J T

    1997-03-15

    Familial and congenital polycythemia, not due to high oxygen affinity hemoglobin or reduced 2,3-diphosphoglycerate in erythrocytes, is common in the Chuvash population of the Russian Federation. Hundreds of individuals appear to be affected in an autosomal recessive pattern. We studied six polycythemic Chuvash patients <20 years of age from unrelated families and 12 first-degree family members. Hemoglobins were markedly elevated in the index subjects (mean +/- standard deviation [SD] of 22.6 +/- 1.4 g/dL), while platelet and white blood cell counts were normal. Although performed in only three of the index subjects, serum erythropoietin concentrations determined by both radioimmune and functional assays were significantly higher in polycythemic patients compared with first-degree family members with normal hemoglobin concentrations. Southern blot analysis of the Bgl 2 erythropoietin gene polymorphism showed that one polycythemic subject was a heterozygote, suggesting the absence of linkage of polycythemia with the erythropoietin gene, assuming autosomal recessive inheritance. Polymerase chain reaction (PCR) amplification of the GGAA and GA minisatellite polymorphic regions of the erythropoietin receptor gene showed no evidence of linkage of phenotype with this gene. We conclude that Chuvash polycythemia may represent a secondary form of familial and congenital polycythemia of as yet unknown etiology. This condition is the only endemic form of familial and congenital polycythemia described.

  13. Metabolic Surgery Profoundly Influences Gut Microbial-Host Metabolic Crosstalk

    Science.gov (United States)

    Li, Jia V.; Ashrafian, Hutan; Bueter, Marco; Kinross, James; Sands, Caroline; le Roux, Carel W; Bloom, Stephen R.; Darzi, Ara; Athanasiou, Thanos; Marchesi, Julian R.; Nicholson, Jeremy K.; Holmes, Elaine

    2013-01-01

    Background and Aims Bariatric surgery is increasingly performed worldwide to treat morbid obesity and is also known as metabolic surgery to reflect its beneficial metabolic effects especially with respect to improvement in type 2 diabetes. Understanding surgical weight loss mechanisms and metabolic modulation is required to enhance patient benefits and operative outcomes. Methods We apply a parallel and statistically integrated metagenomic and metabonomic approach to characterize Roux-en-Y gastric bypass (RYGB) effects in a rat model. Results We show substantial shifts of the main gut phyla towards higher levels of Proteobacteria (52-fold) specifically Enterobacter hormaechei. We also find low levels of Firmicutes (4.5-fold) and Bacteroidetes (2-fold) in comparison to sham-operated rats. Faecal extraction studies reveal a decrease in faecal bile acids and a shift from protein degradation to putrefaction through decreased faecal tyrosine with concomitant increases in faecal putrescine and diamnoethane. We find decreased urinary amines and cresols and demonstrate indices of modulated energy metabolism post-RYGB including decreased urinary succinate, 2-oxoglutarate, citrate and fumarate. These changes could also indicate renal tubular acidosis, which associates with increased flux of mitochondrial tricarboxylic acid cycle intermediates. A surgically-induced effect on the gut-brain-liver metabolic axis is inferred by increased neurotropic compounds; faecal γ-aminobutyric acid (GABA) and glutamate. Conclusion This profound co-dependence of mammalian and microbial metabolism, which is systematically altered following RYGB surgery, suggests that RYGB exerts local and global metabolic activities. The effect of RYGB surgery on the host metabolic-microbial crosstalk augments our understanding of the metabolic phenotype of bariatric procedures and can facilitate enhanced treatments for obesity-related diseases. PMID:21572120

  14. Predictors of Early Reading Skill in 5-Year-Old Children With Hearing Loss Who Use Spoken Language

    OpenAIRE

    Cupples, Linda; Ching, Teresa Y. C.; CROWE, KATHRYN; Day, Julia; Seeto, Mark

    2014-01-01

    This research investigated the concurrent association between early reading skills and phonological awareness (PA), print knowledge, language, cognitive, and demographic variables in 101 5-year-old children with prelingual hearing losses ranging from mild to profound who communicated primarily using spoken language. All participants were fitted with hearing aids (n = 71) or cochlear implants (n = 30). They completed standardized assessments of PA, receptive vocabulary, letter knowledge, word ...

  15. Congenital syphilis, still a reality

    OpenAIRE

    Rajat Gupta; Vora, Rita V.

    2013-01-01

    Congenital syphilis is a potentially serious pathology affecting newborns of infected mothers. Even one case of congenital syphilis is a sentinel public health event, since timely diagnosis and treatment of syphilis infected pregnant woman should prevent transmission almost entirely. Here, we are reporting a case of early symptomatic congenital syphilis presented with severe desquamating papulosquamous lesions over multiple body parts along with erosive lesions around oral cavity and nostrils.

  16. Identification of Ocular and Auditory Manifestations of Congenital Rubella Syndrome in Mbingo

    Directory of Open Access Journals (Sweden)

    Imran Jivraj

    2014-01-01

    Full Text Available Purpose. Congenital rubella syndrome (CRS is a global cause of preventable hearing impairment, blindness, and intellectual impairment. The present study sought to identify ocular and auditory manifestations of CRS in school-aged children in Mbingo, Cameroon. Design. Cross sectional study. Subjects. Students at two schools, one for children with hearing impairment, were screened for cataract, congenital glaucoma, and pigmentary retinopathy. Methods. Students underwent seven-field digital fundus photography through a dilated pupil using a Topcon NW200 nonmydriatic camera. Images were assessed by retina specialists in Canada via teleophthalmology. Clinical evidence was integrated to form case definitions for CRS based on Center for Disease Control and Prevention guidelines. Serological evidence of rubella infection was obtained using standardized IgG antibody titers. Main Outcome Measure. Number of probable and suspicious cases of CRS. Results. Between September 2009 and May 2010, 320 students participated. There were 28 (10.2% probable cases, 104 (37.8% suspects, and 143 (52.0% unaffected. Rubella IgG serology was positive in 79 (48.7% of children with hearing impairment and 11 (7.4% of children with normal hearing. Conclusions. The present study identified 28 probable cases of CRS. Furthermore, 92.6% of students with normal hearing did not possess rubella IgG antibodies making future cases of CRS likely without intervention.

  17. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... Health Conditions Fukuyama congenital muscular dystrophy Fukuyama congenital muscular dystrophy Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Fukuyama congenital muscular dystrophy is an inherited condition that predominantly affects the ...

  18. Genetics Home Reference: congenital dyserythropoietic anemia

    Science.gov (United States)

    ... blood cells. This disorder is one of many types of anemia , which is a condition characterized by a shortage ... link) PubMed OMIM (3 links) ANEMIA, CONGENITAL DYSERYTHROPOIETIC, TYPE Ia ANEMIA, CONGENITAL DYSERYTHROPOIETIC, TYPE II ANEMIA, CONGENITAL DYSERYTHROPOIETIC, TYPE ...

  19. Genetics Home Reference: congenital diaphragmatic hernia

    Science.gov (United States)

    ... Home Health Conditions Congenital diaphragmatic hernia Congenital diaphragmatic hernia Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Congenital diaphragmatic hernia is a defect in the diaphragm. The diaphragm, ...

  20. Binaural Hearing and Beamforming in Digital Hearing Aid

    Directory of Open Access Journals (Sweden)

    Ahmadreza Nazeri

    2001-05-01

    Full Text Available Binaural hearing is an important phenomenon in hearing for human being. Nowadays, the role of binaural hearing in the process of amplification has been focused. Since hearing aids act separately in the process of amplification and hearing, the attentions has been devoted to designing a system for binaural amplification by means of Beam forming which will be explained in more details in the current article.

  1. Early detection of congenital syphilis

    Directory of Open Access Journals (Sweden)

    Nagalakshmi Chowdhary

    2014-01-01

    Full Text Available Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson′s teeth.

  2. Early detection of congenital syphilis.

    Science.gov (United States)

    Chowdhary, Nagalakshmi; Rani, Bs Kavya; Mukunda, K S; Kiran, N K

    2014-01-01

    Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson's teeth.

  3. 77 FR 23319 - Public Hearing

    Science.gov (United States)

    2012-04-18

    ... COMMISSION Public Hearing AGENCY: Susquehanna River Basin Commission. ACTION: Notice. SUMMARY: The Susquehanna River Basin Commission will hold a public hearing on May 10, 2012, in Harrisburg, Pennsylvania. At this public hearing, the Commission will hear testimony on the projects listed in the Supplementary...

  4. 78 FR 64260 - Public Hearing

    Science.gov (United States)

    2013-10-28

    ... COMMISSION Public Hearing AGENCY: Susquehanna River Basin Commission. ACTION: Notice. SUMMARY: The Susquehanna River Basin Commission will hold a public hearing on November 13, 2013, in Harrisburg, Pennsylvania. At this public hearing, the Commission will hear testimony on the projects listed in the...

  5. 78 FR 5556 - Public Hearing

    Science.gov (United States)

    2013-01-25

    ... COMMISSION Public Hearing AGENCY: Susquehanna River Basin Commission. ACTION: Notice. SUMMARY: The Susquehanna River Basin Commission will hold a public hearing on February 14, 2013, in Harrisburg, Pennsylvania. At this public hearing, the Commission will hear testimony on the projects listed in the...

  6. 78 FR 24785 - Public Hearing

    Science.gov (United States)

    2013-04-26

    ... COMMISSION Public Hearing AGENCY: Susquehanna River Basin Commission. ACTION: Notice. SUMMARY: The Susquehanna River Basin Commission will hold a public hearing on May 23, 2013, in Harrisburg, Pennsylvania. At this public hearing, the Commission will hear testimony on the projects listed in the Supplementary...

  7. Patient satisfaction with correction of congenital penile curvature.

    Science.gov (United States)

    Mayer, M; Rey Valzacchi, G; Silva Garretón, A; Layus, O; Gueglio, G

    2017-12-29

    Congenital penile curvature is a relatively rare disease, resulting from a deviation of the penis from the body's straight axis when erect. The prevalence is difficult to determine. Although it has been suggested that the condition could affect up to 10% of the male population, most of these deviations are minor, with no clinical or psychological importance, which leads to its underdiagnosis. Effective correction of the deviation can markedly improve the quality of life of adolescents with this condition. To assess the quality of the sex life of patients diagnosed with congenital penile curvature who underwent surgical correction. Design Retrospective, observational cohort study. Data was collected from the medical records of patients who underwent surgical correction of congenital penile curvature from June 2004 to August 2016. The patients completed the following self-administered questionnaires: Sexual Quality of Life Questionnaire-Male (SQOL-M), International Index of Erectile Function 5 (IIEF 5) and "How satisfied are you with the results of the surgery? From 0 to 10". Twenty-two corporoplasties were performed to correct the patients' congenital penile curvature. The patients' average age was 23.4 years (range, 17-35). The mean deviation prior to surgery was 47.9° (range, 20°-90°). The average score on the SQOL-M was 52 points (range, 6-66). The average score on the IIEF 5 was 22.4 points. Congenital penile curvature profoundly decreases quality of life, and early surgery is fundamental for repairing the anatomical deformation and thereby significantly restores the patients' psychosocial and sexual wellbeing. Copyright © 2017 AEU. Publicado por Elsevier España, S.L.U. All rights reserved.

  8. Congenital Chloride Diarrhea: Diagnosis by Easy-Accessible Chloride Measurement in Feces

    OpenAIRE

    van Gils, C.; M.-C. Eckhardt; Nielsen, P E; Nybo, M.

    2016-01-01

    Background. Congenital chloride diarrhea (CCD) is an autosomal recessive disorder caused by mutations in the genes encoding the intestinal Cl?/HCO3 ? exchanger and is clinically characterized by watery, profound diarrhea, electrolyte disturbances, and metabolic alkalosis. The CCD diagnosis is based on the clinical symptoms and measurement of high chloride concentration in feces (>90?mmol/L) and is confirmed by DNA testing. Untreated CCD is lethal, while long-term clinical outcome improves whe...

  9. Study of the hearing loss in children and adolescents, comparing the periods of 1990-1994 and 1994-2000.

    Science.gov (United States)

    de Nobrega, Manoel; Weckx, Luc Luis Maurice; Juliano, Yara

    2005-06-01

    In 1994, a study was performed with 200 children and adolescents suffering from hearing loss. It concluded that the diagnostic confirmation of hearing loss within 2 years of age occurred in just 13% of the cases, although 56% were suspected in that phase. The loss of time of over 2 years between suspicion and confirmation of hearing loss occurred in 42% of the cases. The comparison of the main hearing loss etiologies-genetic cause, consanguinity, congenital rubella, meningitis, perinatal events and unknown causes-in children and adolescents in the periods of 1990-1994 and 1994-2000; comparison of incidence, in males and females, for each etiology and among the others; comparison of age at the first consultation, for each and among them; and the investigation as to whether the time between suspicion and diagnosis of hearing loss was different for each etiology and among the others. During the period of 1990-2000, of the 519 children and adolescents with hearing loss, 442 individuals were selected, in the two moments of the study: 1990-1994 and 1994-2000. The variables used were: sex, age at first consultation, suspected etiology and time between suspicion and confirmation of hearing loss. Congenital rubella, genetic and perinatal causes, meningitis, consanguinity and unknown causes were responsible for over 80% of all etiologies, in both periods. There were no differences between the sexes in the periods studied. There was no relation among age, sex and etiology. Among the etiologies studied, there were no differences in the lengths of times between suspicion and confirmation of hearing loss, in each period separately. The comparative study showed that congenital rubella, genetic and unknown causes took longer times between suspicion and confirmation of hearing loss, for the period of 1990-1994, as compared with 1994-2000. Congenital rubella remains as an important etiology, as well post-meningitis deafness. Age at first consultation did not show relationship to the

  10. Prescribing and Verifying Hearing Aids Applying the American Academy of Audiology Pediatric Amplification Guideline: Protocols and Outcomes from the Ontario Infant Hearing Program.

    Science.gov (United States)

    Bagatto, Marlene; Moodie, Sheila; Brown, Christine; Malandrino, April; Richert, Frances; Clench, Debbie; Scollie, Susan

    2016-03-01

    Guidelines and protocols for pediatric hearing aid fitting are necessary to meet the goals of Early Hearing Detection and Intervention (EHDI) programs. The American Academy of Audiology published an update to their Pediatric Amplification Guideline in 2013. Ontario's Infant Hearing Program (IHP) offers specific protocols that aim to fulfill recommended guidelines. It has recently been updated to align with the American Academy of Audiology Guideline and other evidence. A summary of the updates to the Ontario IHP's Amplification Protocol is described. In addition, data illustrating hearing-related outcomes of the program are offered. The updated Ontario protocol is based on evidence, wherever possible. Where research is not yet available, clinical decision support has been described in a systematic way. Outcomes of the Ontario IHP were obtained through a longitudinal clinical observation study. One hundred and fifteen children with hearing loss, who wore hearing aids, were included in the outcome analyses (mean = 28.6 mo; range = 1.3-115.3 mo). Hearing losses ranged from mild to profound, unilateral or bilateral sensorineural (pure-tone average = 52.3 dB HL). They were recruited from four IHP clinics within Ontario. Children with complexities in addition to hearing loss were included. The children were fitted with hearing aids following Ontario's Amplification Protocol. During routine clinical appointments, IHP Audiologists administered questionnaires to the parents of their pediatric patients using a systematic outcome measurement protocol (University of Western Ontario Pediatric Audiological Monitoring Protocol). Hearing aid fitting details (e.g., speech intelligibility index) were also gathered to describe the quality of the hearing aid fittings in relation to the functional outcomes. Regression analyses were conducted to characterize scores on the questionnaires and the impact of important variables. Children with complexities were analyzed separately from those

  11. [Prevalence, Risk Factors and Diagnostics of Hearing Impairment in Preterm Infants].

    Science.gov (United States)

    Franck, C; Vorwerk, W; Köhn, A; Rißmann, A; Vorwerk, U

    2017-06-01

    Introduction: The preterm birth is clearly associated with increased risk of developing congenital hearing impairment. Therefore, special attention must be paid to the postnatal control of auditory function in all preterm infants. The present work investigates if the latest scientific findings regarding prevalence, clinical diagnostics, therapy and risk factors of hearing impairment in premature infants are regularly implemented in daily practice. Methods: At the department of phoniatrics and pediatric audiology of the University Hospital of Magdeburg, the treatment data of 126 preterm children born between 2006 and 2011 were evaluated retrospectively. The additional analysis of all records available at the screening center (n=67 640) covering this period enables drawing conclusions on the total number and prevalence of hearing impairment in preterm infants in Saxony-Anhalt. Results: Almost all premature babies, like mature newborns, underwent postnatal hearing screening of both ears. The data analysis shows that the practical implementation often does not comply with the guideline of the G-BA (Gemeinsamer Bundesausschuss) in all details. For example, the recommended screening method for preterm infants (AABR) or the screening and treatment timing are not always applied in accordance with the guidelines of the G-BA. Discussion: Assessment of the practical implementation of universal newborn hearing screening was planned at the time of the introduction of the hearing screening program by the G-BA. As a part of this investigation, the practical care of vulnerable groups such as preterm infants must be given special attention. Based on the collected data, the diagnostics and therapy should be unified. Regardless of the maternity clinic where the infants were born, there should be the same opportunity for early diagnosis and thus for prognostically better treatment of congenital hearing impairment. Rapid postnatal fitting with hearing aid can stimulate the maturation

  12. Nanotechnological Inventions and Nanomaterials Produce A Profound Effect

    Directory of Open Access Journals (Sweden)

    VLASOV Vladimir Alexeevich

    2015-02-01

    Full Text Available The inventions in the area of nanotechnologies and nanomaterials produce a profound effect in construction, housing and communal services and adjacent economic fields as they allow us: to increase mechanical strength, coefficient of elasticity, alkali resistance and temperature of products vitrification; to obtain nanostructured coatings with the property of shape memory on the steel; to raise the dynamics of coal burning and its full burnout in the boilers of thermoelectric power station; to produce metal nanopowders with increased stored energy 10–15% etc. For example, the invention «Epoxy composition for high strength, alkali resistant structures» refers to epoxy composition used as a binder for production of high strength, thermal- and alkali-resistant glass-fiber material which can be applied in the manufacture process of construction reinforcement to strengthen concrete structures. The invention «The method to produce nanostructured reaction foil» can be used to join different materials including metal alloys, ceramics, amorphous materials and elements of microelectronic devices that are sensible to the heating. This process provides decreased labour-output ratio and energy consumption as well as the condition to manufacture foil with specified stored energy and high mechanical properties. The invention «The method of intensification of burning lowreactionary coal in the boilers of thermoelectric power station» refers to the thermal energy and can be implemented at the thermal plants. The increased dynamics of inflaming and burning leads to full burnout of powdered-coal low-reactionary fuel and decreased mechanical underfiring. The specialists may be also interested in the following inventions: fine dispersed organic suspension of carbon metal-containing nanostructures and the method to produce it; the dispersion of carbon nanotubes; the composition for reinforcement of building structures; the reinforced plate element made of

  13. Apps for hearing healthcare.

    Science.gov (United States)

    Paglialonga, Alessia; Tognola, Gabriella; Pinciroli, Francesco

    2015-01-01

    The hearing healthcare scenario is rapidly evolving due to the pervasive use of m-Health solutions, in particular mobile apps. This brings along significant advantages and opportunities (e.g., accessibility, affordability, personalized healthcare, patient empowerment) as well as significant potential risks and threats (e.g., safety, misuse, quality issues, privacy). Our research aims at the identification and assessment of apps in the hearing healthcare domain. In this article we present an overview of the current availability, variety, and penetration of hearing-related apps.

  14. Hearing Aid Personalization

    DEFF Research Database (Denmark)

    Nielsen, Jens Brehm; Nielsen, Jakob; Jensen, Bjørn Sand

    2013-01-01

    Modern digital hearing aids require and offer a great level of personalization. Today, this personalization is not performed based directly on what the user actually perceives, but on a hearing-care professional’s interpretation of what the user explains about what is perceived. In this paper......, an interactive personalization system based on Gaussian process regression and active learning is proposed, which personalize the hearing aids based directly on what the user perceives. Preliminary results demonstrate a significant difference between a truly personalized setting obtained with the proposed system...

  15. Introduction to audiology: Some basics about hearing loss, hearing technologies and barriers to hearing aid use

    DEFF Research Database (Denmark)

    Mourtou, Eleni; Meis, Markus

    2012-01-01

    This chapter provides background information for researchers who wish to become familiar with some basic medical and audiological aspects of hearing loss and the technology of hearing aids. It introduces (1) the disciplines involved in research on hearing loss, (2) the medical categories of hearing...

  16. Hearing Aids: MedlinePlus Health Topic

    Science.gov (United States)

    ... National Library of Medicine) Article: Hearing Loss in Adults. Article: Bilateral versus unilateral hearing aids for bilateral hearing impairment in... Article: Preliminary audiologic and peri-operative outcomes ...

  17. Permanent Childhood Hearing Impairment: Aetiological Evaluation of Infants identified through the Irish Newborn Hearing Screening Programme

    LENUS (Irish Health Repository)

    Smith, A

    2017-11-01

    The Newborn Hearing Screening Programme (NHSP) was established in Cork University Maternity Hospital (CUMH) in April 2011. Between April 2011 and July 2014, 42 infants were identified with a Permanent Childhood Hearing Impairment (PCHI). Following this diagnosis, infants underwent a paediatric assessment according to recognised guidelines with the intention of identifying the underlying aetiology of the PCHI. The aim of this study was to assess the findings of this aetiological workup via retrospective chart review. PCHI data was obtained from the eSP database. This is a web based information system (eSP) used to track each baby through the screening and referral process A retrospective chart review of these patients was performed. Sixteen (38%) infants were diagnosed with a bilateral sensorineural hearing loss. Two infants had congenital CMV infection. A Connexin 26 gene mutation was detected in one infant. Two infants were diagnosed with Waardenburg syndrome, One with Pendred syndrome and one with Pfeiffer syndrome. Five babies underwent cochlear implantation. Through adherence to the recommended protocol a possible cause of PCHI may be determined. This study has identified areas of future improvement for this service in Ireland.

  18. Hearing Protection and Hearing Symptoms in Danish Symphony Orchestras

    DEFF Research Database (Denmark)

    Laitinen, Heli; Poulsen, Torben

    2006-01-01

    A study about hearing protectors, problems involving hearing protector usage, hearing problems and working surroundings of classical musicians was made in three Danish symphony orchestras. The questionnaire used in the study was based on a previous study, a study made in Sweden to rock musicians......, and a questionnaire used in researching occlusion effect with hearing aid users. Also a section from an EQ-5D- questionnaire (a standardised instrument for use as a measure of health outcome) was included to the study. Orchestras were visited by the authors and informed about hearing protection, hearing loss, and ear...... symptoms. 146 musicians filled out the questionnaire. Results show that musicians wear hearing protectors to some extent but their usage can be irregular and hearing protector is sometimes used in one ear only. Musicians worry about their hearing. The more ear symptoms musicians have, the more they use...

  19. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  20. Speech perception studies using a multichannel electrotactile speech processor, residual hearing, and lipreading.

    Science.gov (United States)

    Cowan, R S; Alcantara, J I; Whitford, L A; Blamey, P J; Clark, G M

    1989-06-01

    Three studies are reported on the speech perception of normally hearing and hearing-impaired adults using combinations of visual, auditory, and tactile input. In study 1, mean scores for four normally hearing subjects showed that addition of tactile information, provided through the multichannel electrotactile speech processor, to either audition alone (300-Hz low-pass-filtered speech) or lipreading plus audition resulted in significant improvements in phoneme and word discrimination scores. Information transmission analyses demonstrated the effectiveness of the tactile aid in providing cues to duration, F1 and F2 features for vowels, and manner of articulation features for consonants, especially features requiring detection and discrimination of high-frequency information. In study 2, six different cutoff frequencies were used for a low-pass-filtered auditory signal. Mean scores for vowel and consonant identification were significantly higher with the addition of tactile input to audition alone at each cutoff frequency up to 1500 Hz. The mean speechtracking rate was also significantly increased by the additional tactile input up to 1500 Hz. Study 3 examined speech discrimination of three hearing-impaired adults. Additional information available through the tactile aid was shown to improve speech discrimination scores; however, the degree of increase was inversely related to the level of residual hearing. Results indicate that the electrotactile aid may be useful for patients with little residual hearing and for the severely to profoundly hearing impaired, who could benefit from the high-frequency information presented through the tactile modality, but unavailable through hearing aids.

  1. Examination of characteristics and management of children with hearing loss and autism spectrum disorders.

    Science.gov (United States)

    Fitzpatrick, Elizabeth M; Lambert, Linda; Whittingham, JoAnne; Leblanc, Emma

    2014-09-01

    Up to 40% of children with hearing loss present with other developmental disabilities. The purpose of this study was to document the prevalence of autism spectrum disorders (ASD) in children with permanent hearing loss, to describe the audiologic characteristics, and to examine clinical management. Prospective data related to clinical characteristics of children identified with hearing loss and ASD were examined. A retrospective chart review was also conducted to explore clinical management and uptake of amplification. The study included all children in one Canadian region identified with permanent hearing loss and followed from 2002-2010. Of a total of 785 children with permanent hearing loss, 2.2% (n = 17) also received a diagnosis of ASD. The 13 boys and 4 girls presented with a range of audiologic profiles from unilateral to profound bilateral hearing loss. Four of five children with unilateral hearing loss experienced progression to bilateral loss. Amplification was recommended for all but one child and 9 of 16 children continued to use their hearing devices. The higher prevalence rate of ASD in this clinical population is consistent with previous reports. Our findings suggest that some children with autism can derive benefits from the use of amplification.

  2. Hearing rehabilitation in cerebral palsy: development of language and hearing after cochlear implantation

    Directory of Open Access Journals (Sweden)

    Anacléia Melo da Silva Hilgenberg

    2015-06-01

    Full Text Available INTRODUCTION: Auditory rehabilitation in children with bilateral severe-to-profound sensorineural hearing loss with cochlear implant has been developed in recent decades; however, the rehabilitation of children with cerebral palsy still remains a challenge to otolaryngology and speech therapy professionals. OBJECTIVE: To verify the effectiveness of cochlear implants in the development of auditory and language skills in children with cerebral palsy. METHODS: A prospective analytical study. The evaluation of auditory responses to speech test was applied to the children in this study at regular intervals following implantation. Standardized tests that assess and quantify the development of auditory and language skills were administered and speech therapy video records and speech therapy files were analyzed. All children went through individually tailored intensive audiological rehabilitation programs following cochlear implantation. RESULTS: Two participants had gradual auditory and language development when compared to other participants who reached advanced levels in hearing and oral language classifications. CONCLUSION: The use of the Cochlear implant enabled participants to reach advanced stages of hearing and language skills in three of the five participants with cerebral palsy in this study. This electronic device is a viable therapeutic option for children with cerebral palsy to help them achieve complex levels of auditory and language skills.

  3. Hearing rehabilitation in cerebral palsy: development of language and hearing after cochlear implantation.

    Science.gov (United States)

    Hilgenberg, Anacléia Melo da Silva; Cardoso, Carolina Costa; Caldas, Fernanda Ferreira; Tschiedel, Renata de Sousa; Deperon, Tatiana Medeiros; Bahmad Jr, Fayez

    2015-01-01

    Auditory rehabilitation in children with bilateral severe-to-profound sensorineural hearing loss with cochlear implant has been developed in recent decades; however, the rehabilitation of children with cerebral palsy still remains a challenge to otolaryngology and speech therapy professionals. To verify the effectiveness of cochlear implants in the development of auditory and language skills in children with cerebral palsy. A prospective analytical study. The evaluation of auditory responses to speech test was applied to the children in this study at regular intervals following implantation. Standardized tests that assess and quantify the development of auditory and language skills were administered and speech therapy video records and speech therapy files were analyzed. All children went through individually tailored intensive audiological rehabilitation programs following cochlear implantation. Two participants had gradual auditory and language development when compared to other participants who reached advanced levels in hearing and oral language classifications. The use of the Cochlear implant enabled participants to reach advanced stages of hearing and language skills in three of the five participants with cerebral palsy in this study. This electronic device is a viable therapeutic option for children with cerebral palsy to help them achieve complex levels of auditory and language skills. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  4. Microgastria: congenital microgastria

    Directory of Open Access Journals (Sweden)

    Nilton Crepaldi Vicente

    Full Text Available The authors report a case of an one-year-old girl with growth retardation, vomiting, aspiration pneumonias and malnutrition presenting gastroesopheal reflux and microgastria. The child was underwent a double lumen Roux-en-Y jejunal reservoir (Hunt-Lawrence pouch. This treatment improved nutritional status and growth. No others anomalies were detected. Congenital microgastria is a rare anomaly which is usually associated with other malformations. The authors reviewed the literature and recommend the gastric augmentation for the treatment for microgastria.

  5. Nonclassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  6. Current Concepts - Congenital Scoliosis

    Science.gov (United States)

    Tikoo, Agnivesh; Kothari, Manish K.; Shah, Kunal; Nene, Abhay

    2017-01-01

    Background: Congenital scoliosis is one of the ‘difficult to treat’ scenarios which a spine surgeon has to face. Multiple factors including the age of child at presentation, no definite pattern of deformity and associated anomalies hinder the execution of the ideal treatment plan. All patients of congenital scoliosis need to be investigated in detail. X rays and MRI of spine is usually ordered first. Screening investigations to rule out VACTERL (Visceral, Anorectal, Cardiac, Tracheo-esophageal fistula, Renal and Lung) abnormalities are required. They are cardiac echocardiography and ultrasonography of abdomen and pelvis. CT scan is required to understand the complex deformity and is helpful in surgical planning. Methods: A comprehensive medical literature review was done to understand the current surgical and non surgical treatment options available. An attempt was made to specifically study limitations and advantages of each procedure. Results: The treatment of congenital scoliosis differs with respect to the age of presentation. In adults with curves more than 50 degrees or spinal imbalance the preferred treatment is osteotomy and correction. In children the goals are different and treatment strategy has to be varied according to the age of patient. A single or two level hemivertebra can easily be treated with hemivertebra excision and short segment fusion. However, more than 3 levels or multiple fused ribs and chest wall abnormalities require a guided growth procedure to prevent thoracic insufficiency syndrome. The goal of management in childhood is to allow guided spine growth till the child reaches 10 - 12 years of age, when a definitive fusion can be done. The current research needs to be directed more at the prevention and understanding the etiology of the disease. Till that time, diagnosing the disease early and treating it before the sequels set in, is of paramount importance. Conclusion: The primary aim of treatment of congenital scoliosis is to allow

  7. Congenital Diaphragmatic Hernia

    Directory of Open Access Journals (Sweden)

    Tovar Juan A

    2012-01-01

    Full Text Available Abstract Congenital Diaphragmatic Hernia (CDH is defined by the presence of an orifice in the diaphragm, more often left and posterolateral that permits the herniation of abdominal contents into the thorax. The lungs are hypoplastic and have abnormal vessels that cause respiratory insufficiency and persistent pulmonary hypertension with high mortality. About one third of cases have cardiovascular malformations and lesser proportions have skeletal, neural, genitourinary, gastrointestinal or other defects. CDH can be a component of Pallister-Killian, Fryns, Ghersoni-Baruch, WAGR, Denys-Drash, Brachman-De Lange, Donnai-Barrow or Wolf-Hirschhorn syndromes. Some chromosomal anomalies involve CDH as well. The incidence is

  8. Congenital erythropoietic porphyria

    Directory of Open Access Journals (Sweden)

    Wen-Hao Lee

    2012-06-01

    Full Text Available Congenital erythropoietic porphyria (CEP, or “Günther disease”, is a rare variant of porphyria. It is an autosomal recessive disease caused by deficient uroporphyrinogen III synthase (URO-III-synthase, the fourth enzyme in the heme biosynthetic pathway. We herein report a case of a man with the typical clinical presentations of hyper- and hypo-pigmentation and blister formation over sun-exposed areas, mutilation of the fingers, dark-purple urine, and erythrodontia with pinkish fluorescence under a Wood’s lamp. The diagnosis was confirmed by decreased activity of URO-III-synthase in red blood cells (RBC and a porphyrin profile compatible with CEP.

  9. Congenital imprinting disorders

    DEFF Research Database (Denmark)

    Eggermann, Thomas; Netchine, Irène; Temple, I Karen

    2015-01-01

    Imprinting disorders (IDs) are a group of eight rare but probably underdiagnosed congenital diseases affecting growth, development and metabolism. They are caused by similar molecular changes affecting regulation, dosage or the genomic sequence of imprinted genes. Each ID is characterised...... their common underlying (epi)genetic aetiologies, and their basic pathogenesis and long-term clinical consequences remain largely unknown. Efforts to elucidate the aetiology of IDs are currently fragmented across Europe, and standardisation of diagnostic and clinical management is lacking. The new consortium...

  10. Surgery for Congenital Cataract

    Directory of Open Access Journals (Sweden)

    David Yorston FRCS FRCOphth

    2004-01-01

    Full Text Available The management of congenital cataract is very different to the treatment of a routine age-related cataract. In adults, surgery may be delayed for years without affecting the visual outcome. In infants, if the cataract is not removed during the first year of life, the vision will never be fully regained after surgery. In adults, if the aphakia is not corrected immediately, it can be corrected later. In young children, if the aphakia is not corrected, the vision will never develop normally.

  11. Congenital giant melanocytic nevi

    Directory of Open Access Journals (Sweden)

    Shahla Khan

    2009-07-01

    Full Text Available Nevi are common skin tumors caused by abnormal overgrowth of cells from the epidermal and dermal layers of the skin. Most nevi are benign, but some pre-cancerous nevi must be monitored or removed. The giant congenital nevus is greater than 10 cm in size, pigmented and often hairy. Between 4% and 6% of these lesions will develop into a malignant melanoma. Since approximately 50% of the melanoma develop by the age of two, and 80% by the age of seven, early removal is recommended. The objective of this paper is to present a unique case of giant nevi and their surgical management.

  12. Congenital indifference to pain

    Directory of Open Access Journals (Sweden)

    Kapasi A

    1992-01-01

    Full Text Available A 9-month old female infant developed a non-healing deep necrotic ulcer on the tongue in apposition with the central incisors. There was no response to painful stimuli. Investigations revealed normal blood biochemistry, sweat, histamine and capsaicin tests. Nerve conduction studies and light microscopy of sural nerve and skin were normal. The child was diagnosed as a case of congenital indifference to pain and was treated with teeth extraction and plastic repair of the tongue. In next 2 years the child had recurrent ulcers of the tongue leading to fibrosis and shortening of tongue hampering the speech.

  13. Systemic congenital lymphangiomatosis

    Directory of Open Access Journals (Sweden)

    Ligia Maria Suppo de Souza

    Full Text Available Systemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen, thyroid and kidneys. The severe and diffuse involvement with cysts in both lungs by lymphangiomatosis was associated with poor prognosis and death in our case.

  14. EAMJ Congenital Sep 09.indd

    African Journals Online (AJOL)

    2009-09-09

    Sep 9, 2009 ... Stocker, J.T., Madewell, J.E. and Drake, R.M.. Congenital cystic adenomatoid malformation of the lung: classification and morphologic spectrum. Hum. Pathol. 1977; 8:155-171. 6. Benjamin, D.R. and Cahill, J.L. Bronchioloalveolar carcinoma of the lung and congenital cystic adenomatoid malformation.

  15. Congenital Heart Defects (For Parents)

    Science.gov (United States)

    ... and related health problems. Common Heart Defects Common types of congenital heart defects, which can affect any part of the heart ... circulation. Echocardiograms are the primary tool for diagnosing congenital ... is a specialized type of ultrasound that allows diagnosis of heart problems ...

  16. Congenital Syphilis Masquerading as Leukemia.

    Science.gov (United States)

    Lee, Tiffany; Bell, Stephanie; Scimeme, Jason; Maraqa, Nizar

    2017-07-01

    As of late, the incidence of congenital syphilis in the United States is increasing. Each new case represents a failure of preventing, diagnosing, and treating syphilis in pregnant women. Pediatricians should confirm that all women have received adequate screening for and management of syphilis during pregnancy. Congenital syphilis is easily treatable but may be a diagnostic challenge with high morbidity and mortality.

  17. congenital epulis in a newborn

    African Journals Online (AJOL)

    GB

    BACKGROUND: Congenital epulis is a rare lesion of the newborn, presenting as mass in the oral cavity which can interfere with respiration and feeding. It should be distinguished from other lesions which can occur in newborns, both clinically and histopathologically. CASE DETAILS: Here, we report a case of congenital ...

  18. Placental histopathology of congenital syphilis.

    Science.gov (United States)

    Sheffield, Jeanne S; Sánchez, Pablo J; Wendel, George D; Fong, David W I; Margraf, Linda R; Zeray, Fiker; McIntire, Donald D; Barton Rogers, Beverly

    2002-07-01

    To evaluate the contribution of placental histopathology to the diagnosis of congenital syphilis. From January 1, 1986, through December 31, 1998, all pregnant women presenting to a large, urban Dallas County labor and delivery unit with untreated syphilis at delivery and who had placental evaluation performed were identified. Women were clinically staged, and the infants were evaluated for congenital syphilis using a standard protocol. Each placenta was evaluated by two independent pathologists. Histologic characteristics of the placenta related to congenital syphilis in live-born and stillborn infants were then analyzed. Sixty-seven women met the study criteria: 33 (49%) stillborn and 18 (27%) live-born infants with congenital syphilis, 15 (22%) uninfected live-born infants, and one uninfected stillborn fetus diagnosed by current criteria. There were no differences between the groups with regard to demographic characteristics, prenatal care, or stage of syphilis. Stillborn infants were more likely to deliver preterm (P gestational age, histopathology revealed necrotizing funisitis, villous enlargement, and acute villitis associated with congenital syphilis. Erythroblastosis was more common in stillborn infants with congenital syphilis than all live-born infants (odds ratio 16, 95% confidence interval 1, 370). The addition of histologic evaluation to conventional diagnostic evaluations improved the detection rate for congenital syphilis from 67% to 89% in live-born infants, and 91% to 97% in stillborn infants. Our results show that histopathologic examination of the placenta is a valuable adjunct to the contemporary diagnostic criteria used to diagnose congenital syphilis.

  19. Hearing loss - infants

    Science.gov (United States)

    ... brain after birth, such as meningitis or measles Problems with the structure of the inner ear Tumors Central hearing loss results from damage to the auditory nerve itself, or the brain pathways that lead ...

  20. Hearing and diabetes.

    Science.gov (United States)

    de España, R; Biurrun, O; Lorente, J; Traserra, J

    1995-01-01

    The results of the audiological evaluation of 47 diabetics are presented. The patients were divided into two groups: A (17/47), type I early diabetics, and B (30/47), type I chronic diabetics. The evaluation included puretone audiometry, high-frequency audiometry and auditory brainstem response. There was a control group consisting of 30 healthy subjects. In group 1 the audiological assessment was normal in all cases. In group 2 hearing loss was found in 30% of cases (9/30). Hearing loss was significantly correlated with age (p = 0.0019) and duration of diabetes (p = 0.0143), but not with diabetic microangiopathy (p = 0.1506). The authors conclude that hearing loss is not a usual feature in diabetic patients. When present, hearing loss should be attributed to the effect of diabetes on the age-related physiological impairment of the inner ear. The pathogenic mechanisms remain obscure.

  1. Hearing Conservation Team

    Data.gov (United States)

    Federal Laboratory Consortium — The Hearing Conservation Team focuses on ways to identify the early stages of noise-induced damage to the human ear.Our current research involves the evaluation of...

  2. Help with Hearing

    Science.gov (United States)

    ... observing their behavior in response to sounds. Today’s technology, however, allows even a sleeping baby to be evaluated. Many hospitals now screen newborns’ hearing before they even leave ...

  3. Psychosocial Development in 5-Year-Old Children With Hearing Loss Using Hearing Aids or Cochlear Implants

    Science.gov (United States)

    Ching, Teresa Y. C.; Cupples, Linda; Button, Laura; Leigh, Greg; Marnane, Vivienne; Whitfield, Jessica; Gunnourie, Miriam; Martin, Louise

    2017-01-01

    This article reports on the psychosocial development and factors influencing outcomes of 5-year-old children with cochlear implants (CIs) or hearing aids (HAs). It further examines differences between children with CIs and HAs with similar levels of hearing loss. Data were collected as part of the Longitudinal Outcomes of Children with Hearing Impairment study—a prospective, population-based study. Parents/caregivers of children completed the Strengths and Difficulties Questionnaire (n = 333), the Social Skills subscale from the Child Development Inventory (n = 317), and questionnaires on functional auditory behavior (Parents’ Evaluation of Aural/oral performance of Children), and demographics. Children completed assessments of nonverbal cognitive ability (Wechsler Non-verbal Scale of Ability) and language (Preschool Language Scale - fourth edition). On average, parent-rated Strengths and Difficulties Questionnaire scores on emotional or behavioral difficulties were within 1 SD of the normative mean; however, Child Development Inventory scores on social skills were more than 1 SD below the norm. Children with severe-to-profound hearing losses using HAs had significantly more behavioral problems than children with CIs. Regression analyses showed that non-verbal cognitive ability, language, and functional auditory behavior were significantly associated with psychosocial outcomes for children with HAs, whereas outcomes for children with CIs were associated with functional auditory behavior and the presence of additional disabilities. Age at hearing intervention, severity of hearing loss, and communication mode were not associated with outcomes. The results suggest that even children who develop good language ability with the help of a HA or CI may have psychosocial problems if they exhibit difficulties with listening and communicating in everyday environments. The findings have implications for developing interventions for young children with hearing

  4. Psychosocial Development in 5-Year-Old Children With Hearing Loss Using Hearing Aids or Cochlear Implants.

    Science.gov (United States)

    Wong, Cara L; Ching, Teresa Y C; Cupples, Linda; Button, Laura; Leigh, Greg; Marnane, Vivienne; Whitfield, Jessica; Gunnourie, Miriam; Martin, Louise

    2017-01-01

    This article reports on the psychosocial development and factors influencing outcomes of 5-year-old children with cochlear implants (CIs) or hearing aids (HAs). It further examines differences between children with CIs and HAs with similar levels of hearing loss. Data were collected as part of the Longitudinal Outcomes of Children with Hearing Impairment study-a prospective, population-based study. Parents/caregivers of children completed the Strengths and Difficulties Questionnaire ( n = 333), the Social Skills subscale from the Child Development Inventory ( n = 317), and questionnaires on functional auditory behavior (Parents' Evaluation of Aural/oral performance of Children), and demographics. Children completed assessments of nonverbal cognitive ability (Wechsler Non-verbal Scale of Ability) and language (Preschool Language Scale - fourth edition). On average, parent-rated Strengths and Difficulties Questionnaire scores on emotional or behavioral difficulties were within 1 SD of the normative mean; however, Child Development Inventory scores on social skills were more than 1 SD below the norm. Children with severe-to-profound hearing losses using HAs had significantly more behavioral problems than children with CIs. Regression analyses showed that non-verbal cognitive ability, language, and functional auditory behavior were significantly associated with psychosocial outcomes for children with HAs, whereas outcomes for children with CIs were associated with functional auditory behavior and the presence of additional disabilities. Age at hearing intervention, severity of hearing loss, and communication mode were not associated with outcomes. The results suggest that even children who develop good language ability with the help of a HA or CI may have psychosocial problems if they exhibit difficulties with listening and communicating in everyday environments. The findings have implications for developing interventions for young children with hearing

  5. Comparison of Speech-in-Noise and Localization Benefits in Unilateral Hearing Loss Subjects Using Contralateral Routing of Signal Hearing Aids or Bone-Anchored Implants.

    Science.gov (United States)

    Snapp, Hillary A; Holt, Fred D; Liu, Xuezhong; Rajguru, Suhrud M

    2017-01-01

    To compare the benefit of wireless contralateral routing of signal (CROS) technology to bone-anchored implant (BAI) technology in monaural listeners. Prospective, single-subject. Tertiary academic referral center. Adult English speaking subjects using either a CROS hearing aid or BAI as treatment for unilateral severe-profound hearing loss. Aided performance utilizing the subjects BAI or CROS hearing device. Outcome measures included speech-in-noise perception using the QuickSIN (Etymotic Research, Elkgrove Village, IL, 2001) speech-in-noise test and localization ability using narrow and broadband stimuli. Performance was measured in the unaided and aided condition and compared with normal hearing controls. Subjective outcomes measures included the Speech Spatial and Qualities hearing scale and the Glasgow Hearing Aid Benefit Profile. A significant improvement in speech-in-noise performance for monaural listeners (p noise performance. CROS and BAI hearing devices do not provide any localization benefit in the horizontal plane for monaural listeners and there is no significant difference between systems.

  6. Comparison of performance of transcranial contralateral routing of signal, pre-implanted trimmer digital and digital bone anchored hearing aid in adults with unilateral hearing loss

    Directory of Open Access Journals (Sweden)

    Neelamegarajan Devi

    2015-06-01

    Full Text Available Individuals with unilateral hearing loss of severe-profound degree face listening difficulties while localizing a sound source and while perceiving speech in the presence of noise. The objective was to compare the efficacy of the digitally programmable bone anchored hearing aid (BAHA, trimmer digital BAHA and the transcranial contralateral routing of signal (T-CROS in improving the listening performance in adults with unilateral hearing loss. Twenty-four adults with unilateral hearing loss was assessed for sound field thresholds, speech perception performance in quiet and noise (direct and indirect conditions and the subjective quality rating of speech in unaided and aided with either T-CROS or digitally programmable BAHA or trimmer digital BAHA attached to the headband. Results indicated that the participants performed better with both the digitally programmable and the trimmer digital BAHA than the T-CROS in both quiet and noise. However, the digitally programmable BAHA performed better when the speech arrived from the poorer ear side. The current study helps in prioritizing the hearing amplification devices for the trial and also helps in arriving at the appropriate hearing amplification device for the individuals with unilateral hearing loss.

  7. Comparison of Performance of Transcranial Contralateral Routing of Signal, Pre-Implanted Trimmer Digital and Digital Bone Anchored Hearing Aid in Adults with Unilateral Hearing Loss.

    Science.gov (United States)

    Devi, Neelamegarajan; Chatni, Suma; Ramadevi, Kasetty Jagannathaiah S; Fakruddin, Darga Baba

    2015-06-11

    Individuals with unilateral hearing loss of severe-profound degree face listening difficulties while localizing a sound source and while perceiving speech in the presence of noise. The objective was to compare the efficacy of the digitally programmable bone anchored hearing aid (BAHA), trimmer digital BAHA and the transcranial contralateral routing of signal (T-CROS) in improving the listening performance in adults with unilateral hearing loss. Twenty-four adults with unilateral hearing loss was assessed for sound field thresholds, speech perception performance in quiet and noise (direct and indirect conditions) and the subjective quality rating of speech in unaided and aided with either T-CROS or digitally programmable BAHA or trimmer digital BAHA attached to the headband. Results indicated that the participants performed better with both the digitally programmable and the trimmer digital BAHA than the T-CROS in both quiet and noise. However, the digitally programmable BAHA performed better when the speech arrived from the poorer ear side. The current study helps in prioritizing the hearing amplification devices for the trial and also helps in arriving at the appropriate hearing amplification device for the individuals with unilateral hearing loss.

  8. Human Hearing Loss

    OpenAIRE

    Ware, Stacy Lee

    2014-01-01

    Hearing loss affects as much as 5% of the global human population and its negative consequences, often exacerbated by cultural bias or distributive injustice, include delayed cognitive and language development, learning deficits and poor academic performance, chronic unemployment and dependency, poverty, elevated risk of harm and poor health. This paper is based on a review of the academic literature as well as other credible published resources to identify the principal causes of hearing los...

  9. Congenital nystagmus and negative electroretinography

    Directory of Open Access Journals (Sweden)

    Roussi M

    2011-04-01

    Full Text Available Mirella Roussi, Hélène Dalens, Jean Jacques Marcellier, Franck BacinDepartment of Ophthalmology, Clermont-Ferrand University, Clermont-Ferrand, FranceAbstract: Congenital nystagmus is a pathologic oculomotor state appearing at about three to four months of age. The precise diagnosis requires detailed clinical examination and electrophysiological findings. This case report presents two male patients with congenital nystagmus examined longitudinally from the age of six months until 17-18 years of age. Clinical and electrophysiological protocols were detailed. The first results showed electronegative electroretinography in the two cases and examination combined with electroretinographic findings helped us to make the diagnosis of Congenital Night Stationary Blindness (CSNB. This diagnosis was confirmed by genetic studies. CSNB is interesting to study because through electrophysiological findings, it enables a better understanding of the physiology of neural transmission in the outer part of the retina.Keywords: Congenital nystagmus, negative electroretinography, congenital night stationary blindness

  10. Congenital syphilis: early- and late-stage findings of rhagades and dental anomalies.

    Science.gov (United States)

    Khetarpal, Shilpi; Kempf, Ellen; Mostow, Eliot

    2011-01-01

    Congenital syphilis occurs via vertical transmission when Treponema pallidum crosses the placenta after the 16th week of gestation. Manifestations of congenital syphilis vary from asymptomatic infection to stillbirth. We present a case of congenital syphilis in a Vietnamese orphan who presented with rhagades, hearing deficit, and abnormal dentition after adoption in the United States. He demonstrated both early- and late-stage findings of congenital syphilis. He is presumed to be 5 years old, and it is uncertain if he received treatment. Upon arrival to the United States, his rapid plasma reagin test was negative, but his fluorescent treponemal antibody-absorption test was positive, so no further treatment was recommended. It is possible that other late congenital syphilis stigmata may develop in his later years. The Centers for Disease Control recommends screening all pregnant women for syphilis at the beginning of prenatal care. Clinicians should be made aware of the manifestations of congenital syphilis, especially when caring for international orphans and children born to mothers who lacked prenatal care. © 2010 Wiley Periodicals, Inc.

  11. Dietary habits and hearing.

    Science.gov (United States)

    Rosenhall, Ulf; Idrizbegovic, Esma; Hederstierna, Christina; Rothenberg, Elisabet

    2015-02-01

    Abstract Objective: Study groups from three age cohorts of 70-75 year-olds were investigated to search for possible correlations between dietary habits and auditory function. A cross-sectional, epidemiological study. A total number of 524 people (275 women, 249 men) were recruited from three age cohorts. The study sample was representative of the general population. All participants answered a diet history and were tested with pure-tone audiometry. Eleven categories of food consumption were related to pure-tone averages of low-mid frequency hearing, and high frequency hearing. Two consistent correlations between diet and hearing were observed. One was a correlation between good hearing and a high consumption of fish in the male group. The other was a correlation between poor high frequency hearing and a high consumption of food rich in low molecular carbohydrates in both genders; a larger effect size was seen in females. The study indicates that diet is important for aural health in aging. According to this study fish is beneficial to hearing, whereas consumption of "junk food", rich in low molecular carbohydrates, is detrimental. Other correlations, e.g. between high consumption of antioxidants, were not demonstrated here, but cannot be excluded.

  12. Exploring reasons for late identification of children with early-onset hearing loss.

    Science.gov (United States)

    Fitzpatrick, Elizabeth M; Dos Santos, Johnny Cesconetto; Grandpierre, Viviane; Whittingham, JoAnne

    2017-09-01

    Several studies have shown that early identification of childhood hearing loss leads to better language outcomes. However, delays in the confirmation of hearing loss persist even in the presence of well-established universal newborn hearing screening programs (UNHS). The objective of this population-based study was to document the proportion of children who experienced delayed confirmation of congenital and early onset hearing loss in a UNHS program in one region of Canada. The study also sought to determine the reasons for delayed confirmation of hearing loss in children. Population level data related to age of first assessment, age of identification and clinical characteristics were collected prospectively for all children identified through the UNHS program. We documented the number of children who experienced delay (defined as more than 3 months) from initial audiologic assessment to confirmation of hearing loss. A detailed chart review was subsequently performed to examine the reasons for delay to confirmation. Of 418 children identified from 2003 to 2013, 182 (43.5%) presented with congenital or early onset hearing loss, of whom 30 (16.5%) experienced more than 3 months delay from initial audiologic assessment to confirmation of their hearing disorder. The median age of first assessment and confirmation of hearing loss for these 30 children was 3.7 months (IQR: 2.0, 7.6) and 13.8 months (IQR: 9.7, 26.1) respectively. Close examination of the factors related to delay to confirmation revealed that for the overwhelming majority of children, a constellation of factors contributed to late diagnosis. Several children (n = 22; 73.3%) presented with developmental/medical issues, 15 of whom also had middle ear dysfunction at assessment, and 9 of whom had documented family follow-up concerns. For the remaining eight children, additional reasons included ongoing middle ear dysfunction for five children, complicated by family follow-up concerns (n = 3) and mild

  13. Enlarged Vestibular Aqueduct Syndrome: Sudden Hearing Loss in a Child with a Cerebral Shunt.

    Science.gov (United States)

    Wendt, Wendi-Jo; Hashikawa, Andrew

    2017-07-04

    Enlarged vestibular aqueduct syndrome (EVAS) is the most common congenital ear anomaly that causes sensorineural hearing loss in children and may predispose a child to sudden hearing loss from sudden pressure changes or minor head trauma. We report a case of a 4-year-old boy with a history of a ventriculoperitoneal shunt and migraines who presented to the emergency department with parental and child care provider reports of acute hearing loss, without a history of trauma, infection, or hardware malfunction, who was diagnosed with bilateral EVAS. Diagnosis of EVAS occurs with specific temporal bone imaging with either high-resolution, thin-cut computed tomography or magnetic resonance imaging scans. Enlarged vestibular aqueduct syndrome is typically refractory to medical treatment and often results in hearing loss that is too severe to benefit from amplification, requiring cochlear implantation.

  14. Congenital nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Claudia Fanni

    2014-06-01

    Full Text Available CNS (Congenital nephrotic syndrome is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary and secondary forms (acquired and associated with other syndromes. The most common form is the Finnish CNS (CNF, congenital nephrotic syndrome of the Finnish type, a hereditary form whose name derives from the fact that the highest incidence is described in that country (1.2:10,000. The pathogenesis, the clinical picture, the diagnostic criteria, the therapy and the outcome are described in details.  Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  15. Cartilage Conduction Hearing Aids for Severe Conduction Hearing Loss.

    Science.gov (United States)

    Nishimura, Tadashi; Hosoi, Hiroshi; Saito, Osamu; Shimokura, Ryota; Yamanaka, Toshiaki; Kitahara, Tadashi

    2018-01-01

    To assess the benefits of a new type of hearing aid using cartilage conduction (CC) in patients with severe conduction hearing loss and evaluate its potential for practical use. Consecutive, prospective case series. Forty-one subjects (21 with bilateral aural atresia; 15 with unilateral aural atresia; and 5 others) participated in this study. Fitting and gain adjustments of the CC hearing aids were performed to the ear(s) with conduction hearing loss. The function gains were measured. Evaluation of the measurements of speech performance-intensity functions, speech recognition scores, tolerance of environmental noise, and subject questionnaires were also performed, and judged according to the "Guidelines for the evaluation of hearing aid fitting" established by the Japan Audiological Society. The thresholds were significantly improved by CC hearing aids. The functional gains for CC hearing aids were nearly equivalent to that for their previously used hearing aids. The style of the transducer fixation and the type of aural atresia had no significant influence on the functional gains. Most of the assessment results were judged to be sufficient. Before the trial, bone conduction hearing aids had been used most frequently by bilateral aural atresia subjects. However, after the trial, most subjects continued to use CC hearing aids instead of reverting back to their original device. Overall, 39 subjects continued use of the CC hearing aids. No severe adverse effects were noted in the trial. Cartilage conduction hearing aids could be an additional and beneficial option for severe conduction hearing loss from aural atresia.

  16. Hearing Loss in Children: Data and Statistics

    Science.gov (United States)

    ... Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2015 ...

  17. Hearing Loss in Children: Screening and Diagnosis

    Science.gov (United States)

    ... Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2015 ...

  18. Healhy Ageing in People with Profound Intellectual and Multiple Disabilities : Promoting Physical Activity

    NARCIS (Netherlands)

    van Alphen, Helena; Bossink, Leontien; Schalen, Gertruud Henrike; van der Putten, Annette

    2016-01-01

    Physical activity is beneficial, also for people who are characterized by profound intellectual and severe motor disabilities. However, these people are totally dependent on others to participate in physical activities. To date, promoting physical activity in people with these profound disabilities

  19. Teaching Individuals with Profound Multiple Disabilities to Access Preferred Stimuli with Multiple Microswitches

    Science.gov (United States)

    Tam, Gee May; Phillips, Katrina J.; Mudford, Oliver C.

    2011-01-01

    We replicated and extended previous research on microswitch facilitated choice making by individuals with profound multiple disabilities. Following an assessment of stimulus preferences, we taught 6 adults with profound multiple disabilities to emit 2 different responses to activate highly preferred stimuli. All participants learnt to activate…

  20. Joint Attention Behaviours in People with Profound Intellectual and Multiple Disabilities: The Influence of the Context

    Science.gov (United States)

    Neerinckx, Heleen; Maes, Bea

    2016-01-01

    Background: In spite of the profound cognitive and physical problems, people with profound intellectual and multiple disabilities (PIMD) are able to develop joint attention behaviours (JAB) and benefit from positive interactions. Aims: To investigate which context factors influence the JAB of people with PIMD. Method: Based on video recordings of…