Usher syndrome and cochlear implantation.

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Loundon, Natalie; Marlin, Sandrine; Busquet, Denise; Denoyelle, Françoise; Roger, Gilles; Renaud, Francis; Garabedian, Erea Noel

2003-03-01

To evaluate the symptoms leading to diagnosis and the quality of rehabilitation after cochlear implantation in Usher syndrome. Retrospective cohort study. ENT department of a tertiary referral hospital. Among 210 patients given an implantation in the Ear, Nose, and Throat department, 185 were congenitally deaf and 13 had Usher syndrome (7.0%). Five had a family history of Usher, and eight were sporadic cases. Eleven cases were Usher type I, one was Usher type III, and one was not classified. The age at implantation ranged from 18 months to 44 years (mean, 6 years 1 month). The mean follow-up was 52 months (range, 9 months to 9 years). All patients had audiophonological and clinical examination, computed tomography scan of the temporal bones, ophthalmologic examination with fundoscopy, and an electroretinogram. Cerebral magnetic resonance imaging and vestibular examination were performed in 9 of 13 and 10 of 13 cases, respectively. Logopedic outcome measured preimplant and postimplant closed- and open-set word recognition and oral expression at follow-up. The most frequent initial sign of Usher syndrome was delayed walking, with a mean age of 20 months. Among the 172 other congenitally deaf children with implants, when deafness was not associated with other neurologic disorders, the mean age at walking was 14 months (p < 0.001). The fundoscopy was always abnormal after the age of 5 years, and the electroretinogram was abnormal in all cases. Vestibular function was abnormal in all but one case (nonclassified). The computed tomography scan and the magnetic resonance imaging were always normal. Logopedic results with cochlear implants showed good perception skills in all but one case. The best perceptive results were obtained in children implanted before the age of 9 years. Oral language had significantly progressed in 9 of 13 at follow-up. There was no relation between the visual acuity and the logopedic results. The earliest clinical sign associated with deafness

Cochlear Implantation in Siblings With Refsum's Disease.

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Stähr, Kerstin; Kuechler, Alma; Gencik, Martin; Arnolds, Judith; Dendy, Meaghan; Lang, Stephan; Arweiler-Harbeck, Diana

2017-08-01

Whether the origin of severe hearing loss in Refsum's syndrome is caused by cochlear impairment or retrocochlear degeneration remains unclear. This case report aims to investigate hearing performance before and after cochlear implantation to shed light on this question. Also, identification of new mutations causing Refsum's syndrome would be helpful in generating additional means of diagnosis. A family of 4 individuals was subjected to genetic testing. Two siblings (56 and 61 years old) suffered from severe hearing and vision loss and received bilateral cochlear implants. Genetic analysis, audiological outcome, and clinical examinations were performed. One new mutation in the PHYH gene (c.768del63bp) causing Refsum's disease was found. Preoperative distortion product otoacoustic emissions (DPAOEs) were absent. Postoperative speech perception in Freiburger speech test was 100% for bisyllabic words and 85% (patient No. 1) and 65% (patient No. 2), respectively, for monosyllabic words. Five years after implantation, speech perception remained stable for bisyllabic words but showed decreasing capabilities for monosyllabic words. A new mutation causing Refsum's disease is presented. Cochlear implantation in case of severe hearing loss leads to an improvement in speech perception and should be recommended for patients with Refsum's disease, especially when the hearing loss is combined with a severe loss of vision. Decrease of speech perception in the long-term follow-up could indicate an additional retrocochlear degeneration.

CT-Scans of Cochlear Implant Patients with Characteristics of Pendred Syndrome

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Sebastian Roesch

2013-12-01

Full Text Available Background: Sensorineural hearing loss (SNHL in newborns is estimated with an incidence around 1:10,000 per year and is divided into syndromic and non-syndromic forms. In case of present retrocochlear function‚ cochlear implantation allows speech and cognitive development in affected children, comparable to that of normal hearing children. Pathogenesis of SNHL remains unclear in many cases. Imaging of the temporal bone, such as computed tomography (CT and magnetic resonance imaging (MRI, can reveal conspicuous findings, e.g. enlarged vestibular aqueduct (EVA and Mondini malformation (MM of the cochlea. These malformations can be a clinical sign for Pendred syndrome. Methods: We screened CT scans of 75 cochlear implant patients for EVA and MM. Results: Six patients were observed to have either EVA alone (n=3, or MM alone (n=2, or a combination of both (n=1. Further malformations of the temporal bone could be found within the whole group, as well. Conclusion: Our results confirm the general opinion on EVA and MM, being commonly found in patients with SNHL. A possible association with Pendred syndrome needs to be confirmed by genetic investigations with search for mutations in the SLC26A4 gene and further clinical tests, such as Perchlorate test for surveillance of thyroid function.

Outcomes and Time to Emergence of Auditory Skills After Cochlear Implantation of Children With Charge Syndrome.

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Young, Nancy M; Tournis, Elizabeth; Sandy, Jenelle; Hoff, Stephen R; Ryan, Maura

2017-09-01

Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. Tertiary care children's hospital. Twelve children, seven males and five females. Mean age implant = 3.5 years (1.7-8.2 yr); mean duration follow-up = 4.7 years (1.5-10.1 yr). Cochlear implantation. Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7 dB SAT preoperative and 27.1 dB with the CI (p ≤ 0.002). Overall, four children improved to auditory Level 2 (improved detection), three obtained Level 3 (closed-set speech perception), and three had open-set speech perception with their CIs (Level 4) that was first evident at 3.5, 3.3, and 0.8 years postimplant testing. Two children had minimal or limited improvement. One child with hypoplasia of the cochlear nerve obtained open-set levels. Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.

Outcomes and special considerations of cochlear implantation in waardenburg syndrome.

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Kontorinis, Georgios; Lenarz, Thomas; Giourgas, Alexandros; Durisin, Martin; Lesinski-Schiedat, Anke

2011-08-01

The objective of this study was a state-of-the-art analysis of cochlear implantation in patients with Waardenburg syndrome (WS). Twenty-five patients with WS treated with cochlear implants in our department from 1990 to 2010. The 25 patients with WS underwent 35 cochlear implantations. Hearing outcome was evaluated using HSM sentence test in 65 dB in quiet, Freiburg Monosyllabic Test, and categories of auditory performance for children and compared with that of a control group. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging and computed tomography of the temporal bones. The mean follow-up time was 8.3 years (range, 0.3-18.3 yr). The majority achieved favorable postimplantation performance with mean HSM scores of 75.3% (range, 22.6%-99%) and Freiburg Monosyllabic Test scores of 67.8% (range, 14%-95%). However, in 4 cases, the results were less satisfactory. The comparison with the control group did not reveal any statistical significance (p = 0.56). In 6 patients (24%), behavioral disorders caused temporary difficulties during the rehabilitation procedure. Except of isolated large vestibule in 1 patient, the radiological assessment of the 50 temporal bones did not reveal any temporal bone abnormalities. Most patients with WS performed well with cochlear implants. However, WS is related to behavioral disorders that may cause temporary rehabilitation difficulties. Finally, temporal bone malformations that could affect cochlear implantation are notcharacteristic of WS.

Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

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Birgul Gumus

2017-01-01

Full Text Available Background. Keratitis-ichthyosis-deafness (KID syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature. Case Report. This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR, and the postoperative follow-up was conducted using pure-tone audiometry. Conclusion. Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications.

Relating the absence of binaural pitch percept to retro-cochlear impairment

DEFF Research Database (Denmark)

Santurette, Sébastien; Dau, Torsten

Binaural pitch stimuli, created by introducing an interaural phase difference over a narrow band of otherwise diotic white noise, produce an immediate tonal sensation with a pitch close to the centre of the phase-shifted band. In Santurette and Dau [Hear. Res. 223(1-2):29-47, 2007], it was shown...... that the salience of binaural pitch was affected by hearing impairment. Specifically, for subjects with a sensorineural impairment, binaural pitch perception was weaker than the normal-hearing average but the pitch sensation was immediately present. In contrast, no binaural pitch sensation at all was found...... for the (only) two subjects with damage at central stages. The aim of the present study is to clarify whether such a sharp distinction between levels of impairment can be made using binaural pitch stimuli. A pitch detection test was performed by three groups of subjects with: 1) normal hearing; 2) a cochlear...

The Influence of Cochlear Mechanical Dysfunction, Temporal Processing Deficits, and Age on the Intelligibility of Audible Speech in Noise for Hearing-Impaired Listeners

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Peter T. Johannesen

2016-05-01

Full Text Available The aim of this study was to assess the relative importance of cochlear mechanical dysfunction, temporal processing deficits, and age on the ability of hearing-impaired listeners to understand speech in noisy backgrounds. Sixty-eight listeners took part in the study. They were provided with linear, frequency-specific amplification to compensate for their audiometric losses, and intelligibility was assessed for speech-shaped noise (SSN and a time-reversed two-talker masker (R2TM. Behavioral estimates of cochlear gain loss and residual compression were available from a previous study and were used as indicators of cochlear mechanical dysfunction. Temporal processing abilities were assessed using frequency modulation detection thresholds. Age, audiometric thresholds, and the difference between audiometric threshold and cochlear gain loss were also included in the analyses. Stepwise multiple linear regression models were used to assess the relative importance of the various factors for intelligibility. Results showed that (a cochlear gain loss was unrelated to intelligibility, (b residual cochlear compression was related to intelligibility in SSN but not in a R2TM, (c temporal processing was strongly related to intelligibility in a R2TM and much less so in SSN, and (d age per se impaired intelligibility. In summary, all factors affected intelligibility, but their relative importance varied across maskers.

Speech recognition and communication outcomes with cochlear implantation in Usher syndrome type 3.

Science.gov (United States)

Pietola, Laura; Aarnisalo, Antti A; Abdel-Rahman, Akram; Västinsalo, Hanna; Isosomppi, Juha; Löppönen, Heikki; Kentala, Erna; Johansson, Reijo; Valtonen, Hannu; Vasama, Juha-Pekka; Sankila, Eeva-Marja; Jero, Jussi

2012-01-01

Usher syndrome Type 3 (USH3) is an autosomal recessive disorder characterized by variable type and degree of progressive sensorineural hearing loss and retinitis pigmentosa. Cochlear implants are widely used among these patients. To evaluate the results and benefits of cochlear implantation in patients with USH3. A nationwide multicenter retrospective review. During the years 1995-2005, in 5 Finnish university hospitals, 19 patients with USH3 received a cochlear implant. Saliva samples were collected to verify the USH3 genotype. Patients answered to 3 questionnaires: Glasgow Benefit Inventory, Glasgow Health Status Inventory, and a self-made questionnaire. Audiological data were collected from patient records. All the patients with USH3 in the study were homozygous for the Finnish major mutation (p.Y176X). Either they had severe sensorineural hearing loss or they were profoundly deaf. The mean preoperative hearing level (pure-tone average, 0.5-4 kHz) was 110 ± 8 dB hearing loss (HL) and the mean aided hearing level was 58 ± 11 dB HL. The postoperative hearing level (34 ± 9 dB HL) and word recognition scores were significantly better than before surgery. According to the Glasgow Benefit Inventory scores and Glasgow Health Status Inventory data related to hearing, the cochlear implantation was beneficial to patients with USH3. Cochlear implantation is beneficial to patients with USH3, and patients learn to use the implant without assistance.

Outcomes and benefits of pediatric cochlear implantation in children with additional disabilities: a review and report of family influences on outcomes.

Science.gov (United States)

Cejas, Ivette; Hoffman, Michael F; Quittner, Alexandra L

2015-01-01

The number of children with hearing loss with additional disabilities receiving cochlear implantation has increased dramatically over the past decade. However, little is known about their auditory and speech and language development following implantation. The purpose of this review is to evaluate the effects of cochlear implantation on the most common genetic and developmental disorders in children with hearing loss. Benefits of cochlear implantation for children with autism spectrum disorder, developmental delay, CHARGE syndrome, cerebral palsy, learning disorders, Usher syndrome, Waardenburg syndrome, and attention deficit/hyperactivity disorder are reviewed. Our review indicates that children with hearing loss and additional disabilities benefit from cochlear implantation, especially when implanted early. Thus, early interventions seem as important for these children as for deaf children without additional disabilities. Comparisons of outcomes across these disabilities indicate that children with little to no cognitive impairment (eg, Waardenburg sydrome, attention deficit hyperactivity disorder) have better outcomes than those with greater deficits in intellectual functioning (eg, autism, CHARGE syndrome). In addition, parents of children with hearing loss and additional disabilities report higher levels of parenting stress and greater child behavior problems than those without comorbid diagnoses. However, these parents are as sensitive when interacting with their children as parents with typically developing children using cochlear implantation. Given these results, it is critical to evaluate these children's developmental milestones to provide early implantation and intervention, appropriately counsel families regarding realistic expectations for the implant, and facilitate family adaptation.

Cochlear Implants

Science.gov (United States)

... NIDCD A cochlear implant is a small, complex electronic device that can help to provide a sense ... Hearing Aids Retinitis Pigmentosa - National Eye Institute Telecommunications Relay Services Usher Syndrome Your Baby's Hearing Screening News ...

Cochlear vertebral entrapment syndrome: a case report

Energy Technology Data Exchange (ETDEWEB)

Liu Chinghsiung; Lin Shinnkuang E-mail: sk1943@adm.cgmh.org.tw; Chang Yeujhy

2001-11-01

The authors describe a patient with isolated involvement of vestibulocochlear nerve by a huge vascular loop from vertebral dolichoectasia. No other neurological deficit was found except for unilateral hearing loss. Abnormal brainstem auditory evoked potential study indicated a retrocochlear lesion. The brain computed tomography (CT) and magnetic resonance imaging (MRI) studies demonstrated an abnormally enhanced vascular lesion impinged on the left porus acusticus with a displacement of the brainstem to the right. There was no infarction in the brainstem. A cerebral angiography demonstrated a megadolichoectatic horizontal loop at the intracranial portion of the left vertebral artery. There was no thrombus or atherosclerosis in the vertebrobasilar system. A mechanical compression by a vascular loop is the only possible pathogenesis for hearing loss. The authors diagnose this condition as cochlear vertebral entrapment syndrome.

Hearing-impaired children in the United Kingdom, III : cochlear implantation and the economic costs incurred by families

OpenAIRE

Barton, GR; Fortnum, HM; Stacey, PC; Summerfield, AQ

2006-01-01

Objectives: This article addresses two questions. First, are there differences in the economic costs incurred by families of hearing-impaired children depending on whether or not children have cochlear implants? Second, are these differences important when assessed from the perspective of society? Methods: In a cross-sectional survey, parents of a representative sample of hearing-impaired children provided data about annual resources used by the family because of their child’s hearing impairm...

[Cochlear implant treatment in Germany].

Science.gov (United States)

Jacob, R; Stelzig, Y

2013-01-01

Restoration of impaired auditory function through cochlear implant is possible, with high reliably and great success. Nevertheless, there are regular disputes between patients and insurance companies due to high costs. In Germany, approx. 1.9 Mio. people are severely hearing impaired. It can be estimated that for adequate hearing rehabilitation about 30,000 cochlear implants/year are necessary. Currently, less than 10% of those affected are offered cochlear implant. A handicap is defined if there is deviation from normal hearing for more than 6 months. This sets a time frame for the supply with cochlear implant after sudden deafness. The professional code requires to advice all medical options to a person seeking help for hearing loss. This includes benefit-risk consideration. At this point, the economic aspect plays no role. The indication for medical treatment is only subject to the treating physician and should not be modified by non-physicians or organizations. It should be noted that a supply of hearing aids is qualitatively different to the help from a cochlear implant, which provides a restoration of lost function. In social law (SGB V and IX) doctors are requested to advise and recommend all measures which contribute to normal hearing (both sides). This indicates that doctors may be prosecuted for not offering help when medically possible, just because health insurance employees did not approve the cost balance. The current situation, with insufficient medical care for the hearing impaired, needs clarifying. To do this, patients, health insurance companies, the political institutions, legislation and professional societies need to accept their responsibilities.

Outcomes and benefits of pediatric cochlear implantation in children with additional disabilities: a review and report of family influences on outcomes

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Cejas I

2015-05-01

Full Text Available Ivette Cejas,1 Michael F Hoffman,2 Alexandra L Quittner21Department of Otolaryngology, University of Miami Miller School of Medicine, 2Department of Psychology, University of Miami, Miami, FL, USAAbstract: The number of children with hearing loss with additional disabilities receiving cochlear implantation has increased dramatically over the past decade. However, little is known about their auditory and speech and language development following implantation. The purpose of this review is to evaluate the effects of cochlear implantation on the most common genetic and developmental disorders in children with hearing loss. Benefits of cochlear implantation for children with autism spectrum disorder, developmental delay, CHARGE syndrome, cerebral palsy, learning disorders, Usher syndrome, Waardenburg syndrome, and attention deficit/hyperactivity disorder are reviewed. Our review indicates that children with hearing loss and additional disabilities benefit from cochlear implantation, especially when implanted early. Thus, early interventions seem as important for these children as for deaf children without additional disabilities. Comparisons of outcomes across these disabilities indicate that children with little to no cognitive impairment (eg, Waardenburg sydrome, attention deficit hyperactivity disorder have better outcomes than those with greater deficits in intellectual functioning (eg, autism, CHARGE syndrome. In addition, parents of children with hearing loss and additional disabilities report higher levels of parenting stress and greater child behavior problems than those without comorbid diagnoses. However, these parents are as sensitive when interacting with their children as parents with typically developing children using cochlear implantation. Given these results, it is critical to evaluate these children's developmental milestones to provide early implantation and intervention, appropriately counsel families regarding realistic

Behavioral measures of cochlear compression and temporal resolution as predictors of speech masking release in hearing-impaired listeners

Science.gov (United States)

Gregan, Melanie J.; Nelson, Peggy B.; Oxenham, Andrew J.

2013-01-01

Hearing-impaired (HI) listeners often show less masking release (MR) than normal-hearing listeners when temporal fluctuations are imposed on a steady-state masker, even when accounting for overall audibility differences. This difference may be related to a loss of cochlear compression in HI listeners. Behavioral estimates of compression, using temporal masking curves (TMCs), were compared with MR for band-limited (500–4000 Hz) speech and pure tones in HI listeners and age-matched, noise-masked normal-hearing (NMNH) listeners. Compression and pure-tone MR estimates were made at 500, 1500, and 4000 Hz. The amount of MR was defined as the difference in performance between steady-state and 10-Hz square-wave-gated speech-shaped noise. In addition, temporal resolution was estimated from the slope of the off-frequency TMC. No significant relationship was found between estimated cochlear compression and MR for either speech or pure tones. NMNH listeners had significantly steeper off-frequency temporal masking recovery slopes than did HI listeners, and a small but significant correlation was observed between poorer temporal resolution and reduced MR for speech. The results suggest either that the effects of hearing impairment on MR are not determined primarily by changes in peripheral compression, or that the TMC does not provide a sufficiently reliable measure of cochlear compression. PMID:24116426

Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life.

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Hartel, Bas P; van Nierop, Josephine W I; Huinck, Wendy J; Rotteveel, Liselotte J C; Mylanus, Emmanuel A M; Snik, Ad F; Kunst, Henricus P M; Pennings, Ronald J E

2017-07-01

Usher syndrome type IIa (USH2a) is characterized by congenital moderate to severe hearing impairment and retinitis pigmentosa. Hearing rehabilitation starts in early childhood with the application of hearing aids. In some patients with USH2a, severe progression of hearing impairment leads to insufficient speech intelligibility with hearing aids and issues with adequate communication and safety. Cochlear implantation (CI) is the next step in rehabilitation of such patients. This study evaluates the performance and benefit of CI in patients with USH2a. Retrospective case-control study to evaluate the performance and benefit of CI in 16 postlingually deaf adults (eight patients with USH2a and eight matched controls). Performance and benefit were evaluated by a speech intelligibility test and three quality-of-life questionnaires. Patients with USH2a with a mean age of 59 years at implantation exhibited good performance after CI. The phoneme scores improved significantly from 41 to 87% in patients with USH2a (p = 0.02) and from 30 to 86% in the control group (p = 0.001). The results of the questionnaire survey demonstrated a clear benefit from CI. There were no differences in performance or benefit between patients with USH2a and control patients before and after CI. CI increases speech intelligibility and improves quality of life in patients with USH2a.

Importance of cochlear health for implant function.

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Pfingst, Bryan E; Zhou, Ning; Colesa, Deborah J; Watts, Melissa M; Strahl, Stefan B; Garadat, Soha N; Schvartz-Leyzac, Kara C; Budenz, Cameron L; Raphael, Yehoash; Zwolan, Teresa A

2015-04-01

Amazing progress has been made in providing useful hearing to hearing-impaired individuals using cochlear implants, but challenges remain. One such challenge is understanding the effects of partial degeneration of the auditory nerve, the target of cochlear implant stimulation. Here we review studies from our human and animal laboratories aimed at characterizing the health of the implanted cochlea and the auditory nerve. We use the data on cochlear and neural health to guide rehabilitation strategies. The data also motivate the development of tissue-engineering procedures to preserve or build a healthy cochlea and improve performance obtained by cochlear implant recipients or eventually replace the need for a cochlear implant. This article is part of a Special Issue entitled . Copyright © 2014 Elsevier B.V. All rights reserved.

CT evaluation of preoperative cochlear implantation cochlear implantation

International Nuclear Information System (INIS)

Tan Xiuzhong; Zhong Lansheng; Lan Bowen; Huang Yaosheng; Du Baowen; Zhu Jian

2004-01-01

Objective: To evaluate CT scan as a preoperative evaluation for cochlear implantation candidates. Methods: Axial high-resolution temporal bone CT and three-dimensional reconstruction of inner ear were performed in 93 patients with sensorineural hearing loss. results: Among 81 patients with congenital sensorineural deafness, Mondini malformation was seen in 7 case (13 ears); large vestibular aqueduct syndrome (LVAS) was revealed in 5 cases (8 ears); and inner ear ossification was found in 1 case (2 ears). In 1 case (2 ears) of inner ear fibrosis, reduced cochlear signal was noted on MRI but no unremarkable findings was shown on CT scan, however, in the operation, the device could not inserted into the basal circle of the cochlea, due to fibrous obliteration. In 12 patients with post-speech deafness, chronic suppurative tympanitis was seen in 2 cases (4 ears), and inner ear ossification was revealed in 1 case (2 ears). Conclusion: CT plays an indispensable role in the pre-operative evaluation of cochlear implantation. T 2 -weighted FSE-MRI of the inner ear is a useful complementary to CT scan. (authors)

The Repeatable Battery for the Assessment of Neuropsychological Status for Hearing Impaired Individuals (RBANS-H) before and after Cochlear Implantation: A Protocol for a Prospective, Longitudinal Cohort Study.

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Claes, Annes J; Mertens, Griet; Gilles, Annick; Hofkens-Van den Brandt, Anouk; Fransen, Erik; Van Rompaey, Vincent; Van de Heyning, Paul

2016-01-01

Background: Currently, an independent relationship between hearing loss and cognitive decline in older adults is suggested by large prospective studies. In general, cochlear implants improve hearing and the quality of life in severely to profoundly hearing impaired older persons. However, little is known about the effects of cochlear implantation on the cognitive evolution in this population. Aim of the study: The primary goal of this prospective, longitudinal cohort study is to explore the cognitive profile of severely to profoundly postlingually hearing impaired subjects before and after cochlear implantation. In addition, the current study aims to investigate the relationship between the cognitive function, audiometric performances, quality of life, and self-reliance in these patients. Methods: Twenty-five patients aged 55 or older, scheduled for cochlear implantation, will be enrolled in the study. They will be examined prior to implantation, at 6 and 12 months after implantation and annually thereafter. The test battery consists of (1) a cognitive examination, using the Repeatable Battery for the Assessment of Neuropsychological Status adapted for Hearing impaired persons (RBANS-H), (2) an audiological examination, including unaided and aided pure tone audiometry, speech audiometry in quiet and speech audiometry in noise, (3) the administration of four questionnaires evaluating quality of life and subjective hearing benefit and (4) a semi-structured interview about the self-reliance of the participant. Discussion: Up until now only one study has been conducted on this topic, focusing on the short-term effects of cochlear implantation on cognition in older adults. The present study is the first study to apply a comprehensive neuropsychological assessment adapted for severely to profoundly hearing impaired subjects in order to investigate the cognitive capabilities before and after cochlear implantation. Trial registration: The present protocol is

Psycholinguistic abilities in cochlear implant and hearing impaired children

OpenAIRE

Hassan, Hatem Ezzeldin; Eldin, Sally Taher Kheir; Al Kasaby, Rasha Mohamed

2014-01-01

Background: Many congenitally sensorineural hearing loss (SNHL) children and cochlear implant (CI) recipients develop near-normal language skills. However, there is a wide variation in individual outcomes following cochlear implantation, or using hearing aids. Some CI recipients or Hearing aids users never develop useable speech and oral language skills. The causes of this enormous variation in outcomes are only partly understood at the present time. So, the aim of this study was to assess th...

[Cochlear implant in patients with congenital malformation of inner ear].

Science.gov (United States)

Han, Dong-yi; Wu, Wen-ming; Xi, Xin; Huang, De-liang; Yang, Wei-yan

2004-02-01

To study surgical difficulty and key of the cochlear implant in patients with congenital malformation of inner ear. The cochlear implantations were performed in our department from Jan. 2001 to Apr. 2003 for 18 patients with the malformation of inner ear. In this series, there were 11 cases of large vestibular aqueduct syndrome (LVAS), 3 cases of Waardenberg syndrome, 3 cases of Mondini malformation, and 1 case of Usher syndrome. All 18 patients accepted the Nucleus 24-channel cochlear implantations, including Nucleus straight electrode in 13 cases but Contour implantation in 5 cases of LVAS. During operations, leakage of perilymph but not cerebrospinal fluid (CSF) from the open of scala tympani occurred in 11 cases of LVAS, however, the electrode was inserted successfully. The abnormalities of round window occurred in one of 3 cases of Waardenberg syndrome and 3 cases of Mondini malformation, respectively. The cochlear implant could be conducted successfully for the LVAS, and the postoperative effect was same as the ones for the deafness persons with normal development of inner ear. However, for the patients with Mondini syndrome and common cavity, it is important to accurately assess the extent of abnormalities in the inner ear and accompanied malformation before operation, and to evaluate the full extent of difficulties of the operation in order to minimize the risk of CSF leakage and meningitis.

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.

Hearing impairment in genotyped Wolfram syndrome patients.

NARCIS (Netherlands)

Plantinga, R.F.; Pennings, R.J.E.; Huygen, P.L.M.; Bruno, R.; Eller, P.; Barrett, T.G.; Vialettes, B.; Paquis-Fluklinger, V.; Lombardo, F.; Cremers, C.W.R.J.

2008-01-01

OBJECTIVES: Wolfram syndrome is a progressive neurodegenerative syndrome characterized by the features "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). We sought to study the audiometric data of genotyped Wolfram syndrome patients with sensorineural hearing impairment.

Are parents of children with cochlear implants coping?: research ...

African Journals Online (AJOL)

Many variables must be considered during the evaluation and rehabilitation of children for cochlear implantation, one of which is parental influence (for the duration of this report the parents, caregivers and guardians of children with cochlear implants and / or hearing impairments will be referred to as 'parents'). The aim of ...

Hearing impairment in genotyped Wolfram syndrome patients.

Science.gov (United States)

Plantinga, Rutger F; Pennings, Ronald J E; Huygen, Patrick L M; Bruno, Rocco; Eller, Philipp; Barrett, Timothy G; Vialettes, Bernard; Paquis-Fluklinger, Veronique; Lombardo, Fortunato; Cremers, Cor W R J

2008-07-01

Wolfram syndrome is a progressive neurodegenerative syndrome characterized by the features "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). We sought to study the audiometric data of genotyped Wolfram syndrome patients with sensorineural hearing impairment. Pure tone threshold data of 23 Wolfram syndrome patients were used for cross-sectional analysis in subgroups (age less than 16 years or between 19 and 25 years, gender, and origin). All subgroups, with 1 exception, showed a fairly similar type of hearing impairment with, on average, thresholds of about 25 dB (range, 0 to 65 dB) at 0.25 to 1 kHz, gently sloping downward to about 60 dB (range, 25 to 95 dB) at 8 kHz. The subgroup of Dutch women, which was excluded from the calculations of the average hearing thresholds, showed a higher degree of hearing impairment. Only the latter subgroup showed progression; however, contrary to the previous longitudinal analysis, progression was not significant in the present cross-sectional analysis, presumably because of the high degree of cross-subject variability. This unique collection of audiometric data from genotyped Wolfram syndrome patients shows no substantial progression in sensorineural hearing impairment with advancing age, no relation to the types of WFS1 mutations identified, and, with exclusion of the subgroup of Dutch female patients, no significant sex-related differences.

Motor Skills in Hearing Impaired Children with or without Cochlear Implant--A Systematic Review.

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Vidranski, Tihomir; Farkaš, Daria

2015-07-01

Hearing impairment is a major limitation in communication, and it can obstruct psychological development, development of social skills and motor development. Hearing impairment is the third most common contemporary chronic health condition, and it has become a public health problem. The effectiveness of problem solving in everyday life and in emergency situations depends greatly on the amount and quality of the motor programs. Therefore, it is evident that the normal motor development in persons with hearing impairment is essential for everyday life. The aim of this research is to analyze the available information pertaining to motor skills of hearing impaired children both with and without a cochlear implant (CI) and to analyze possibilities of influencing their motor skills. The relevant studies on motor skills of hearing impaired children both with and without CI were obtained by an extensive computer search of various databases using special keywords and extraction with respect to certain criteria, resulting in 22 studies. The overall results of this systematic review indicate that the children with hearing impairment exhibit suboptimal levels of motor skills especially balance. Very few studies compared children with hearing impairment with CI units and without CI units and the results of those studies are quite contradictory. Numerous studies have confirmed that the regular and appropriate physical exercise can improve motor skills of children with hearing impairment, especially balance. The fact that the development of motor skills is crucial for the child's interaction with the outside world, action, perception and acquisition of academic skills and other skills necessary for life shows the importance of motor skills development for children with hearing impairment.

The comparison of balance performance among children with cochlear implantation, post-aural aid and normal children

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Arezoo Ahmad pour

2014-11-01

Full Text Available Background: This cross-sectional study was carried out to compare balance performance between children with cochlear implants and post-aural aid and normal children. Methods: The present study was done on 67 severe to profound hearing impaired children. Of these, 21 children with an average age of 7 years and 4 months (±1.7 wore cochlear implant, 46 children with an average age of 7 years and 7 months (±1.7 wore post-aural aid and 60 children with an average 8 years and one month (±11 months were considered as a control group. All of the children were tested with the 9-stage balance subtest of Bruininks-Oseretsky Test of Motor Proficiency2 (BOT2. Results: The mean total BOT2 score of hearing impaired children was significantly lower than the normal group (P≤0.001. The mean total BOT2 score among children with cochlear implant, post-aural aids and normal group showed that cochlear implant group significantly performed weaker than the other two groups (P≤0.001. Conclusion: Hearing impaired children particularly children with cochlear implant are exposed to the risk of balance deficit. Hearing impaired children specially those who wear cochlear implants must be screened for vestibular hypofunction.

Cochlear implantation and clinical features in patients with Noonan syndrome and Noonan syndrome with multiple lentigines caused by a mutation in PTPN11.

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van Nierop, Josephine W I; van Trier, Dorothée C; van der Burgt, Ineke; Draaisma, Jos M T; Mylanus, Emmanuel A M; Snik, Ad F; Admiraal, Ronald J C; Kunst, Henricus P M

2017-06-01

Existing literature only reports a few patients with Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NSML) who underwent cochlear implantation (CI). The present study describes four NS patients and one NSML patient with a PTPN11 mutation. They all had severe to profound hearing loss, and they received a CI. The age at which the CI surgery occurred ranged from 1 to 13 years old, and the audiological results in all five patients improved after the CI. Otological and audiological examinations in NS and NSML are important, and for those with severe hearing loss, the CI surgery improved the audiological outcome regardless of age. Published by Elsevier B.V.

Deletion of PDZD7 disrupts the Usher syndrome type 2 protein complex in cochlear hair cells and causes hearing loss in mice.

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Zou, Junhuang; Zheng, Tihua; Ren, Chongyu; Askew, Charles; Liu, Xiao-Ping; Pan, Bifeng; Holt, Jeffrey R; Wang, Yong; Yang, Jun

2014-05-01

Usher syndrome type 2 (USH2) is the predominant form of USH, a leading genetic cause of combined deafness and blindness. PDZD7, a paralog of two USH causative genes, USH1C and USH2D (WHRN), was recently reported to be implicated in USH2 and non-syndromic deafness. It encodes a protein with multiple PDZ domains. To understand the biological function of PDZD7 and the pathogenic mechanism caused by PDZD7 mutations, we generated and thoroughly characterized a Pdzd7 knockout mouse model. The Pdzd7 knockout mice exhibit congenital profound deafness, as assessed by auditory brainstem response, distortion product otoacoustic emission and cochlear microphonics tests, and normal vestibular function, as assessed by their behaviors. Lack of PDZD7 leads to the disorganization of stereocilia bundles and a reduction in mechanotransduction currents and sensitivity in cochlear outer hair cells. At the molecular level, PDZD7 determines the localization of the USH2 protein complex, composed of USH2A, GPR98 and WHRN, to ankle links in developing cochlear hair cells, likely through its direct interactions with these three proteins. The localization of PDZD7 to the ankle links of cochlear hair bundles also relies on USH2 proteins. In photoreceptors of Pdzd7 knockout mice, the three USH2 proteins largely remain unchanged at the periciliary membrane complex. The electroretinogram responses of both rod and cone photoreceptors are normal in knockout mice at 1 month of age. Therefore, although the organization of the USH2 complex appears different in photoreceptors, it is clear that PDZD7 plays an essential role in organizing the USH2 complex at ankle links in developing cochlear hair cells. GenBank accession numbers: KF041446, KF041447, KF041448, KF041449, KF041450, KF041451.

Cochlear implant: Speech and language development in deaf and hard of hearing children following implantation

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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.

Tinnitus and Sleep Difficulties After Cochlear Implantation.

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Pierzycki, Robert H; Edmondson-Jones, Mark; Dawes, Piers; Munro, Kevin J; Moore, David R; Kitterick, Pádraig T

To estimate and compare the prevalence of and associations between tinnitus and sleep difficulties in a sample of UK adult cochlear implant users and those identified as potential candidates for cochlear implantation. The study was conducted using the UK Biobank resource, a population-based cohort of 40- to 69-year olds. Self-report data on hearing, tinnitus, sleep difficulties, and demographic variables were collected from cochlear implant users (n = 194) and individuals identified as potential candidates for cochlear implantation (n = 211). These "candidates" were selected based on (i) impaired hearing sensitivity, inferred from self-reported hearing aid use and (ii) impaired hearing function, inferred from an inability to report words accurately at negative signal to noise ratios on an unaided closed-set test of speech perception. Data on tinnitus (presence, persistence, and related distress) and on sleep difficulties were analyzed using logistic regression models controlling for gender, age, deprivation, and neuroticism. The prevalence of tinnitus was similar among implant users (50%) and candidates (52%; p = 0.39). However, implant users were less likely to report that their tinnitus was distressing at its worst (41%) compared with candidates (63%; p = 0.02). The logistic regression model suggested that this difference between the two groups could be explained by the fact that tinnitus was less persistent in implant users (46%) compared with candidates (72%; p reported difficulties with sleep were similar among implant users (75%) and candidates (82%; p = 0.28), but participants with tinnitus were more likely to report sleep difficulties than those without (p explanation is supported by the similar prevalence of sleep problems among implant users and potential candidates for cochlear implantation, despite differences between the groups in tinnitus persistence and related emotional distress. Cochlear implantation may therefore not be an appropriate intervention

Visual impairment in Finnish Usher syndrome type III.

NARCIS (Netherlands)

Plantinga, R.F.; Pennings, R.J.E.; Huygen, P.L.M.; Sankila, E.M.; Tuppurainen, K.; Kleemola, L.; Cremers, C.W.R.J.; Deutman, A.F.

2006-01-01

PURPOSE: To evaluate visual impairment in Finnish Usher syndrome type 3 (USH3) and compare this with visual impairment in Usher syndrome types 1b (USH1b) and 2a (USH2a). METHODS: We carried out a retrospective study of 28 Finnish USH3 patients, 24 Dutch USH2a patients and 17 Dutch USH1b patients.

Waardenburg Syndrome: An Unusual Indication of Cochlear Implantation Experienced in 11 Patients.

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Bayrak, Feda; Çatlı, Tolgahan; Atsal, Görkem; Tokat, Taşkın; Olgun, Levent

2017-08-01

The aim of this study was to present the surgical findings of children with Waardenburg syndrome (WS) and investigate speech development after cochlear implantation in this unique group of patients. A retrospective chart review of the patients diagnosed with WS and implanted between 1998 and 2015 was performed. Categories of auditory performance (CAP) test were used to assess the auditory skills of these patients. CAP is a nonlinear hierarchical scale used to rate a child's developing auditory abilities. Preoperative test results and intraoperative surgical findings of these patients have been presented. In total, 1835 cases were implanted a tour institution, and 1210 of these were children. Among these implantees, 11 were diagnosed with WS (0.59% of all implantees). Four of the 11 patients showed incomplete partition type 2bony labyrinth abnormality (Mondini deformity) and all patients showed intraoperative gusher during cochleostomy, which was subsided through routine interventions. No other complications occurred during surgery, and all patients showed satisfactory CAP results in the late postoperative period. Our experiences with cochlear implantation in patients with WS showed that the procedure is safe and effective in this group of patients. Surgeons should be aware of possible labyrinth malformations and intraoperative problems such as gusher in these patients. In long term, auditory performances may exhibit satisfactory results with optimal postoperative educational and supportive measures.

Demographic factors influencing educational placement of the hearing-impaired child with a cochlear implant.

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Yehudai, Noam; Tzach, Naama; Shpak, Talma; Most, Tova; Luntz, Michal

2011-08-01

To analyze educational placement settings of Israeli children with cochlear implants (CIs) and evaluate the prognostic influence of the following demographic variables on mainstreaming: age at implantation, experience with CI, socioeconomic status, ethnicity, and parents' educational level. Retrospective review. Tertiary referral center. The study population comprised 245 children with severe-to-profound hearing impairment and at least 1 year of experience with a unilateral CI. Mean age at implantation was 4.5 ± 3.9 years, and mean duration of CI use was 5.4 ± 2.8 years. Follow-up review and statistical analysis of available data on educational placement after cochlear implantation. Placement in mainstream education. Regular schools were attended by 89 children (36.3%) and special education schools by 156 (63.7%). Variables found to be significantly associated with mainstream educational placement were younger age at implantation, higher level of parental education, higher socioeconomic status, and ethnicity. Multivariate analysis using a logistic regression model revealed that the factor with the highest positive correlation with mainstreaming was parental education level. Our results show that parental education, a variable that the health system cannot control, significantly influences postimplantation results in term of educational placement and can thus limit the chances of implanted children to achieve mainstream placement even when identified and implanted at an early age.

A phone-assistive device based on Bluetooth technology for cochlear implant users.

Science.gov (United States)

Qian, Haifeng; Loizou, Philipos C; Dorman, Michael F

2003-09-01

Hearing-impaired people, and particularly hearing-aid and cochlear-implant users, often have difficulty communicating over the telephone. The intelligibility of telephone speech is considerably lower than the intelligibility of face-to-face speech. This is partly because of lack of visual cues, limited telephone bandwidth, and background noise. In addition, cellphones may cause interference with the hearing aid or cochlear implant. To address these problems that hearing-impaired people experience with telephones, this paper proposes a wireless phone adapter that can be used to route the audio signal directly to the hearing aid or cochlear implant processor. This adapter is based on Bluetooth technology. The favorable features of this new wireless technology make the adapter superior to traditional assistive listening devices. A hardware prototype was built and software programs were written to implement the headset profile in the Bluetooth specification. Three cochlear implant users were tested with the proposed phone-adapter and reported good speech quality.

Preliminary evaluation of child self-rating using the Child Tourette Syndrome Impairment Scale.

Science.gov (United States)

Cloes, Kelly Isaacs; Barfell, Kara S Francis; Horn, Paul S; Wu, Steve W; Jacobson, Sarah E; Hart, Kathleen J; Gilbert, Donald L

2017-03-01

To evaluate and compare how children with Tourette syndrome and parents rate tic and non-tic behavioral related impairment in home, school, and social domains; to compare these with clinician tic ratings; and to identify factors that may predict greater impairment. In a sample of 85 Tourette syndrome and 92 healthy control families, the Child Tourette Syndrome Impairment Scale, designed for parent-report and which includes 37 items rated for tic and non-tic impairment, was administered to parents and, with the referent modified, to children ages 9 to 17 years. Tic severity was rated using the Yale Global Tic Severity Scale (YGTSS). Analyses utilized descriptive and multivariate statistics. Tourette syndrome children's and parents' impairment ratings were higher than HC (ptic impairment ratings correlated with YGTSS (r=0.36 to 0.37; ptic and all 37 non-tic impairment items. For 29 items, children self-rated impairment higher for tics than non-tics. Diagnoses of attention-deficit-hyperactivity disorder and obsessive-compulsive disorder had larger effects on parent impairment ratings. The Child Tourette Syndrome Impairment Scale appears informative for child self-rating in Tourette syndrome. © 2016 Mac Keith Press.

[Cognitive impairments accompanying the burnout syndrome - a review].

Science.gov (United States)

Riedrich, Karin; Weiss, Elisabeth M; Dalkner, Nina; Reininghaus, Eva; Papousek, Ilona; Schwerdtfeger, Andreas; Lackner, Helmut K; Reininghaus, Bernd

2017-03-01

The rising prevalence of the burnout syndrome has increasingly moved it into the focus of scientific interest. In addition to emotional exhaustion and depersonalization, particularly reduced personal accomplishment has strong societal and economic effects. In recent years reduced personal accomplishment has increasingly been linked to cognitive impairment. However, up to now only a few studies have objectively assessed cognitive deficits in burnout patients. This article gives an overview of 16 studies which examined cognitive abilities in burnout patients. The findings are partly contradictory, probably due to methodical differences. Consensus has emerged concerning impairments of executive functions, i.a. vigilance, and memory updating and monitoring. Multifactorial causation may underlie the cognitive impairments. Targeted longitudinal studies are necessary in order to identify the affected cognitive functions and be able to make causal inferences on links between the burnout syndrome and specific cognitive impairments.

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

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Mauro Scarpelli

2012-01-01

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

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

Science.gov (United States)

Hassanzadeh, S

2012-10-01

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

Influence of cochlear implantation on peripheral vestibular receptor function.

Science.gov (United States)

Krause, Eike; Louza, Julia P R; Wechtenbruch, Juliane; Gürkov, Robert

2010-06-01

The objectives of this study were 1) to assess the influence of a cochlear implantation on peripheral vestibular receptor function in the inner ear in the implant and in the nonimplant side, and 2) to analyze a possible correlation with resulting vertigo symptoms. Prospective clinical study. Cochlear implant center at tertiary referral hospital. A total of 32 patients, aged 15 to 83 years, undergoing cochlear implantation were assessed pre- and postoperatively for caloric horizontal semicircular canal response and vestibular-evoked myogenic potentials of the sacculus, and postoperatively for subjective vertigo symptoms. Patients with vertigo were compared with patients without symptoms with regard to the findings of the vestibular function tests. Cochlear implantation represents a significant risk factor for horizontal semicircular canal impairment (P 0.05). Cochlear implantation is a relevant risk factor for damage of peripheral vestibular receptor function. Therefore, preservation not only of residual hearing function but also of vestibular function should be aimed for, by using minimally invasive surgical techniques. Copyright 2010 American Academy of Otolaryngology-Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.

Cochlear Implants in Children Diagnosed with CHARGE Syndrome

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Cardoso, Carolina Costa

2013-09-01

Full Text Available Introduction: The CHARGE association (coloboma of the eyes; heart disease; atresia of the choanae; retarded growth and development; genital hypoplasia/genitourinary anomalies; ear anomalies and/or hearing loss was first described in 1979 by Hall, and among its main features is hearing loss. This study presents a case aiming to establish relationships between performance on Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS and Meaningful Use of Speech Scales (MUSS tests and the analysis of hearing and language categories of a patient diagnosed with CHARGE syndrome, before and after cochlear implant (CI surgery. Case Report: A 7-year-old girl was diagnosed with CHARGE. She had severe sensorineural hearing loss and was a prelingual unilateral CI user. We analyzed data from the patient's medical records regarding therapies and video recordings. Results: The patient showed positive results in all evaluations after CI. IT-MAIS rose from 5 to 90% following the use of CI. MUSS also rose, from 75 to 72.5%, after use of CI. Classification of Auditory Skills changed from category 1 before use of CI to category 6 after use of CI. Classification of Language Skills changed from category 1 before use of CI to category 3 after use of CI. The CI is an aid but there are many factors in the therapeutic process, and great heterogeneity in individuals diagnosed with CHARGE should be investigated. Conclusion: The development of listening and language skills after CI use was demonstrated by IT-MAIS and MUSS tests, and categorization of speech and hearing in this child with a diagnosis of CHARGE syndrome shows that CI can be an effective technological resource to provide information on hearing as one source for language construction.

Impaired Perception of Sensory Consonance and Dissonance in Cochlear Implant Users.

Science.gov (United States)

Caldwell, Meredith T; Jiradejvong, Patpong; Limb, Charles J

2016-03-01

In light of previous research demonstrating poor pitch perception in cochlear implant (CI) users, we hypothesized that the presence of consonant versus dissonant chord accompaniment in real-world musical stimuli would not impact subjective assessment of degree of pleasantness in CI users. Consonance/dissonance are perceptual features of harmony resulting from pitch relationships between simultaneously presented musical notes. Generally, consonant sounds are perceived as pleasant and dissonant ones as unpleasant. CI users exhibit impairments in pitch perception, making music listening difficult and often unenjoyable. To our knowledge, consonance/dissonance perception has not been studied in the CI population. Twelve novel melodies were created for this study. By altering the harmonic structures of the accompanying chords, we created three permutations of varying dissonance for each melody (36 stimuli in all). Ten CI users and 12 NH listeners provided Likert scale ratings from -5 (very unpleasant) to +5 (very pleasant) for each of the stimuli. A two-way ANOVA showed main effects for Dissonance Level and Subject Type as well as a two-way interaction between the two. Pairwise comparisons indicated that NH stimuli pleasantness ratings decreased with increasing dissonance, whereas CI ratings did not. NH pleasantness ratings were consistently lower than CI ratings. For CI users, consonant versus dissonant chord accompaniment had no significant impact on whether a melody was considered pleasant or unpleasant. This finding may be partially responsible for the decreased enjoyment of many CI users during music perception and is another manifestation of impaired pitch perception in CI users.

Renal Impairment in Cirrhosis Unrelated to Hepatorenal Syndrome

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Gavin Low

2015-01-01

Full Text Available Renal impairment is common in liver disease and may occur as a consequence of the pathophysiological changes that underpin cirrhosis or secondary to a pre-existing unrelated insult. Nevertheless, the onset of renal impairment often portends a worsening prognosis. Hepatorenal syndrome remains one of the most recognized and reported causes of renal impairment in cirrhosis. However, other causes of renal impairment occur and can be classified into prerenal, intrinsic or postrenal, which are the subjects of the present review.

The prevalence of Usher syndrome and other retinal dystrophy-hearing impairment associations.

Science.gov (United States)

Rosenberg, T; Haim, M; Hauch, A M; Parving, A

1997-05-01

The study was undertaken to procure population-based prevalence data on the various types of Usher syndrome and other retinal dystrophy-hearing impairment associations. The medical files on 646 patients with a panretinal pigmentary dystrophy aged 20-49 years derived from the Danish Retinitis Pigmentosa (RP) register were scrutinised. The data were supplemented by a prior investigation on hearing ability in a part of the study population. After exclusion of patients with possibly extrinsic causes of hearing impairments, 118 patients, including 89 cases of Usher syndrome were allocated to one of five clinically defined groups. We calculated the following prevalence rates: Usher syndrome type I: 1.5/100,000, Usher syndrome type II: 2.2/100,000, and Usher syndrome type III: 0.1/100,000 corresponding to a 2:3 ratio between Usher syndrome type I and II. The overall prevalence rate of Usher syndrome was estimated to 5/100,000 in the Danish population, devoid of genetic isolates. The material comprised 11 cases with retinal dystrophy, hearing impairment, and additional syndromic features. Finally, 18 subjects with various retinal dystrophy-hearing impairment associations without syndromic features were identified, corresponding to a prevalence rate of 0.8/100,000. This group had a significant overrepresentation of X-linked RP, including two persons harboring a mutation in the retinitis pigmentosa GTP-ase regulator (RPGR) gene.

Association of metabolic syndrome and 25-hydroxyvitamin D with cognitive impairment among elderly Koreans.

Science.gov (United States)

Lee, Eun Young; Lee, Su Jin; Kim, Kyoung Min; Yun, Young Mi; Song, Bo Mi; Kim, Jong Eun; Kim, Hyeon Chang; Rhee, Yumie; Youm, Yoosik; Kim, Chang Oh

2017-07-01

Metabolic syndrome and vitamin D deficiency are prevalent in older adults, and are considered risk factors for cognitive impairment. We investigated the combined effects of MetS and serum 25-hydroxyvitamin D (25[OH]D) levels on cognitive function in older adults. We studied 2940 participants aged ≥65 years from the Korean Urban Rural Elderly cohort study. Metabolic syndrome was defined according to the updated Adult Treatment Panel III criteria. Serum 25(OH)D levels were categorized into four groups: metabolic syndrome prevalence and lower serum 25(OH)D levels than those without cognitive impairment. In univariate analysis, both metabolic syndrome and low 25(OH)D levels were associated with cognitive impairment. These associations remained unchanged after adjusting for potential confounders including age, sex, season and education. In addition, participants with metabolic syndrome and low 25(OH)D had significantly increased odds for cognitive impairment (odds ratio 3.06, 95% CI 1.61-5.80) when compared with those with no metabolic syndrome and high 25(OH)D. Metabolic syndrome was associated with cognitive impairment, and this risk was synergistically increased when metabolic syndrome was combined with low 25(OH)D. A focus on individuals with metabolic syndrome and low 25(OH)D might be helpful to identify older adults who are at risk of cognitive impairment. Geriatr Gerontol Int 2017; 17: 1069-1075. © 2016 Japan Geriatrics Society.

Investigation of the vestibular aqueduct and the cochlear aqueduct by temboral bone CT scan

International Nuclear Information System (INIS)

Shimizu, Ryuichi; Kamei, Tamio; Ito, Fumihide

1984-01-01

The visualization of the vestibular aqueduct and the cochlear aqueduct was investigated by temporal bone CT scan. The vestibular aqueduct was visualized in horizontal CT sections of 70.0% of normal ears, 61.5% of ears with chronic otitis media, 58.3% of ears with combined hearing impairment, 66.7% of ears in cases of sudden deafness, 70.8% of ears of patients with sensorineural hearing impairment without sudden deafness, 71.4% of cases of vertigo without hearing impairment and 12.5% of both diseased and contralateral ears of patients with Meniere's disease. Only in Meniere's disease was the vestibular aqueduct less visible in the diseased than in the normal ear (P<0.01). The cochlear aqueduct was visible in coronal sections of 50.0% of normal ears, 76.9% of those with chronic otitis media, 58.3% of those with combined hearing impairment, 66.7% of those with sudden deafness 41.7% of those with sensorineural hearing impairment without sudden deafness, 50.0% of the diseased ears of patients with Meniere's disease, 37.5% of the contralateral ears of those with Meniere's disease and 64.3% of those with vertigo without hearing impairment. Although the cochlear aqueduct was thus highly demonstrable in patients with chronic otitis media or sudden deafness, the difference between the percentage of visualization in these diseased and in normal ears was not statistically significant. (author)

Productivity of lexical categories in French-speaking children with cochlear implants.

Science.gov (United States)

Le Normand, M-T; Ouellet, C; Cohen, H

2003-11-01

The productivity of lexical categories was studied longitudinally in a sample of 17 young hearing-impaired French-speaking children with cochlear implants. Age of implantation ranged from 22 months to 76 months. Spontaneous speech samples were collected at six-month intervals over a period of 36 months, starting at the one-word stage. Four general measures of their linguistic production (number of utterances, verbal fluency, vocabulary, and grammatical production) as well as 36 specific lexical categories, according to the CHILDES codes, were computed in terms of tokens, i.e., total number of words. Cochlear-implanted children (CI) were compared to a French database of normally hearing children aged 2-4 compiled by the first author. Follow-up results indicate that, at the two-year post-implantation follow-up, noun, and verb morphology was significantly impaired. At the three-year follow-up, the cochlear-implanted group had recovered on adjectives, determiners and nouns, main verbs, and auxiliaries. The two groups differed significantly in processing locative adverbs, prepositions, pronouns, and verbs (infinitive verb, modal, and modal lexical), but individual variability within the cochlear-implanted group was substantial. Results are discussed in terms of recovery and developmental trends and variability in the acquisition of lexical categories by French children two years and three years post-implantation.

Cochlear implantation in patients with bilateral cochlear trauma.

Science.gov (United States)

Serin, Gediz Murat; Derinsu, Ufuk; Sari, Murat; Gergin, Ozgül; Ciprut, Ayça; Akdaş, Ferda; Batman, Cağlar

2010-01-01

Temporal bone fracture, which involves the otic capsule, can lead to complete loss of auditory and vestibular functions, whereas the patients without fractures may experience profound sensorineural hearing loss due to cochlear concussion. Cochlear implant is indicated in profound sensorineural hearing loss due to cochlear trauma but who still have an intact auditory nerve. This is a retrospective review study. We report 5 cases of postlingually deafened patients caused by cochlear trauma, who underwent cochlear implantation. Preoperative and postoperative hearing performance will be presented. These patients are cochlear implanted after the cochlear trauma in our department between 2001 and 2006. All patients performed very well with their implants, obtained open-set speech understanding. They all became good telephone users after implantation. Their performance in speech understanding was comparable to standard postlingual adult patients implanted. Cochlear implantation is an effective aural rehabilitation in profound sensorineural hearing loss caused by temporal bone trauma. Preoperative temporal bone computed tomography, magnetic resonance imaging, and promontorium stimulation testing are necessary to make decision for the surgery and to determine the side to be implanted. Surgery could be challenging and complicated because of anatomical irregularity. Moreover, fibrosis and partial or total ossification within the cochlea must be expected. Copyright 2010. Published by Elsevier Inc.

Surgical complications following cochlear implantation in adults based on a proposed reporting consensus

DEFF Research Database (Denmark)

Jeppesen, Jonas; Faber, Christian Emil

2013-01-01

Abstract Conclusion: The rate of severe complications was low and cochlear implantation is a relatively safe procedure. Standardization is crucial when reporting on cochlear implant complications to ensure comparability between studies. A consensus on the reporting of complications proposed by a ...... occurred following one implantation (0.3%). Transient chorda tympani syndrome (30.8%), vertigo/dizziness (29.5%) and tinnitus (4.9%) were the most frequent minor complications....

[Cochlear implant in patients with congenital malformation of the inner ear].

Science.gov (United States)

Wan, Liang-cai; Guo, Meng-he; Qian, Yu-hong; Liu, Shuang-xiu; Zhang, Hong-zheng; Chen, Shuai-jun; Chen, Hao; Gong, Jian

2009-10-01

To summarize the clinical experience with multi-channel cochlear implantation in patients with inner ear malformations and evaluate and the outcomes of speech rehabilitation. A retrospective study was conducted in 295 patients receiving cochlear implantation from 1998 to 2007, including 25 patients with large vestibular aqueduct syndrome (LVAS), 9 with Modini malformation, and 5 with common cavity deformity. All the patients received the Nucleus24 cochlear implants. In LVAS cases, 4 had Nucleus 24R (ST) implants, 8 had Contuor implants, 10 had Contuor Advance, and the remaining cases used Nucleus24(M) straight-electrode implants. Severe gusher appeared in 3 cases of LVAS, and perilymph fluctuation were seen in other 15 cases. Four patients with Mondini malformation and 2 with common cavity malformation also experienced severe gusher, but the electrodes were inserted smoothly in all the patients without postoperative facial paralysis or cerebrospinal fluid leakage. The hearing threshold in these patients was similar to that in patients with normal cochlear structure. After speech rehabilitation for over 6 months, the abilities of speech discrimination and spoken language improved in all the cases in comparison with the preoperative lingual functions. Multi-channel cochlear implantation can be performed in patients with inner ear malformation, but should not be attempted in patients with poor cochlear and cochlear nerve development. A comprehensive pre-operative radiographic and audiological evaluation is essential.

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

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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.

Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life

NARCIS (Netherlands)

Hartel, B.P.; Nierop, J.W.I. van; Huinck, W.J.; Rotteveel, L.J.C.; Mylanus, E.A.M.; Snik, A.F.M.; Kunst, H.P.M.; Pennings, R.J.E.

2017-01-01

OBJECTIVES: Usher syndrome type IIa (USH2a) is characterized by congenital moderate to severe hearing impairment and retinitis pigmentosa. Hearing rehabilitation starts in early childhood with the application of hearing aids. In some patients with USH2a, severe progression of hearing impairment

Demographic Characteristics and Impairments of Louisiana Students with Usher's Syndrome.

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Brown, S. C.

1987-01-01

Of 51 Louisiana students with Usher's Syndrome (a genetic condition characterized by hearing loss and progressive blindness), 71 percent manifested visual impairment and hearing loss, 9 percent had neither, 10 percent had visual impairments but a less-than-profound hearing loss, and 10 percent had profound hearing loss and no visual impairment.…

Predicting effects of impaired cochlear processing on consonant discrimination in stationary noise

DEFF Research Database (Denmark)

Jepsen, Morten Løve; Dau, Torsten; Ghitza, Oded

Cochlear hearing loss is typically associated with reduced sensitivity due to inner hair-cell (IHC) and outer hair-cell (OHC) dysfunction. OHC dysfunction also leads to supra-threshold deficits, such as reduced basilar-membrane (BM) compression as well as reduced frequency selectivity and temporal...... patterns from a Diagnostic Rhyme Test (DRT) were measured and analyzed in terms of acoustic-phonetic features. This was done for three listeners with cochlear hearing loss and at two signal-to-noise ratios. It is shown that the predicted errors patterns matched the measured patterns in most conditions......, such as the evaluation of hearing-instrument signal processing, where the effects of specific processing strategies can be simulated for individual hearing losses....

Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations.

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Elmaleh-Bergès, M; Baumann, C; Noël-Pétroff, N; Sekkal, A; Couloigner, V; Devriendt, K; Wilson, M; Marlin, S; Sebag, G; Pingault, V

2013-01-01

Waardenburg syndrome, characterized by deafness and pigmentation abnormalities, is clinically and genetically heterogeneous, consisting of 4 distinct subtypes and involving several genes. SOX10 mutations have been found both in types 2 and 4 Waardenburg syndrome and neurologic variants. The purpose of this study was to evaluate both the full spectrum and relative frequencies of inner ear malformations in these patients. Fifteen patients with Waardenburg syndrome and different SOX10 mutations were studied retrospectively. Imaging was performed between February 2000 and March 2010 for cochlear implant work-up, diagnosis of hearing loss, and/or evaluation of neurologic impairment. Eleven patients had both CT and MR imaging examinations, 3 had MR imaging only, and 1 had CT only. Temporal bone abnormalities were bilateral. The most frequent pattern associated agenesis or hypoplasia of ≥1 semicircular canal, an enlarged vestibule, and a cochlea with a reduced size and occasionally an abnormal shape, but with normal partition in the 13/15 cases that could be analyzed. Three patients lacked a cochlear nerve, bilaterally in 2 patients. In addition, associated abnormalities were found when adequate MR imaging sequences were available: agenesis of the olfactory bulbs (7/8), hypoplastic or absent lacrimal glands (11/14), hypoplastic parotid glands (12/14), and white matter signal anomalies (7/13). In the appropriate clinical context, bilateral agenesis or hypoplasia of the semicircular canals or both, associated with an enlarged vestibule and a cochlear deformity, strongly suggests a diagnosis of Waardenburg syndrome linked to a SOX10 mutation.

Evidence of across-channel processing for spectral-ripple discrimination in cochlear implant listeners.

Science.gov (United States)

Won, Jong Ho; Jones, Gary L; Drennan, Ward R; Jameyson, Elyse M; Rubinstein, Jay T

2011-10-01

Spectral-ripple discrimination has been used widely for psychoacoustical studies in normal-hearing, hearing-impaired, and cochlear implant listeners. The present study investigated the perceptual mechanism for spectral-ripple discrimination in cochlear implant listeners. The main goal of this study was to determine whether cochlear implant listeners use a local intensity cue or global spectral shape for spectral-ripple discrimination. The effect of electrode separation on spectral-ripple discrimination was also evaluated. Results showed that it is highly unlikely that cochlear implant listeners depend on a local intensity cue for spectral-ripple discrimination. A phenomenological model of spectral-ripple discrimination, as an "ideal observer," showed that a perceptual mechanism based on discrimination of a single intensity difference cannot account for performance of cochlear implant listeners. Spectral modulation depth and electrode separation were found to significantly affect spectral-ripple discrimination. The evidence supports the hypothesis that spectral-ripple discrimination involves integrating information from multiple channels. © 2011 Acoustical Society of America

Hearing Preservation in Cochlear Implant Surgery

Directory of Open Access Journals (Sweden)

Priscila Carvalho Miranda

2014-01-01

Full Text Available In the past, it was thought that hearing loss patients with residual low-frequency hearing would not be good candidates for cochlear implantation since insertion was expected to induce inner ear trauma. Recent advances in electrode design and surgical techniques have made the preservation of residual low-frequency hearing achievable and desirable. The importance of preserving residual low-frequency hearing cannot be underestimated in light of the added benefit of hearing in noisy atmospheres and in music quality. The concept of electrical and acoustic stimulation involves electrically stimulating the nonfunctional, high-frequency region of the cochlea with a cochlear implant and applying a hearing aid in the low-frequency range. The principle of preserving low-frequency hearing by a “soft surgery” cochlear implantation could also be useful to the population of children who might profit from regenerative hair cell therapy in the future. Main aspects of low-frequency hearing preservation surgery are discussed in this review: its brief history, electrode design, principles and advantages of electric-acoustic stimulation, surgical technique, and further implications of this new treatment possibility for hearing impaired patients.

Effect of cochlear nerve electrocautery on the adult cochlear nucleus.

Science.gov (United States)

Iseli, Claire E; Merwin, William H; Klatt-Cromwell, Cristine; Hutson, Kendall A; Ewend, Matthew G; Adunka, Oliver F; Fitzpatrick, Douglas C; Buchman, Craig A

2015-04-01

Electrocauterization and subsequent transection of the cochlear nerve induce greater injury to the cochlear nucleus than sharp transection alone. Some studies show that neurofibromatosis Type 2 (NF2) patients fit with auditory brainstem implants (ABIs) fail to achieve speech perception abilities similar to ABI recipients without NF2. Reasons for these differences remain speculative. One hypothesis posits poorer performance to surgically induced trauma to the cochlear nucleus from electrocautery. Sustained electrosurgical depolarization of the cochlear nerve may cause excitotoxic-induced postsynaptic nuclear injury. Equally plausible is that cautery in the vicinity of the cochlear nucleus induces necrosis. The cochlear nerve was transected in anesthetized adult gerbils sharply with or without bipolar electrocautery at varying intensities. Gerbils were perfused at 1, 3, 5, and 7 days postoperatively; their brainstem and cochleas were embedded in paraffin and sectioned at 10 μm. Alternate sections were stained with flourescent markers for neuronal injury or Nissl substance. In additional experiments, anterograde tracers were applied directly to a sectioned eighth nerve to verify that fluorescent-labeled profiles seen were terminating auditory nerve fibers. Cochlear nerve injury was observed from 72 hours postoperatively and was identical across cases regardless of surgical technique. Postsynaptic cochlear nucleus injury was not seen after distal transection of the nerve. By contrast, proximal transection was associated with trauma to the cochlear nucleus. Distal application of bipolar electrocautery seems safe for the cochlear nucleus. Application near the root entry zone must be used cautiously because this may compromise nuclear viability needed to support ABI stimulation.

Evaluation of visual impairment in Usher syndrome 1b and Usher syndrome 2a.

NARCIS (Netherlands)

Pennings, R.J.E.; Huygen, P.L.M.; Orten, D.J.; Wagenaar, M.; Aarem, A. van; Kremer, J.M.J.; Kimberling, W.J.; Cremers, C.W.R.J.; Deutman, A.F.

2004-01-01

PURPOSE: To evaluate visual impairment in Usher syndrome 1b (USH1b) and Usher syndrome 2a (USH2a). METHODS: We carried out a retrospective study of 19 USH1b patients and 40 USH2a patients. Cross-sectional regression analyses of the functional acuity score (FAS), functional field score (FFS) and

Can cochlear implantation improve neurocognition in the aging population?

Directory of Open Access Journals (Sweden)

Völter C

2018-04-01

Full Text Available Christiane Völter,1 Lisa Götze,1 Stefan Dazert,1 Michael Falkenstein,2,3 Jan Peter Thomas1 1Department of Otorhinolaryngology, Head and Neck Surgery, Ruhr University Bochum, St. Elisabeth-Hospital, Bochum, Germany; 2Institute for Work, Learning and Ageing (ALA, Bochum, Germany; 3Leibniz Research Centre for Working Environment and Human Factors, Dortmund, Germany Introduction: The relationship between cognition and the ability to hear is well known. Due to changes in demographics, the number of people with sensorineural hearing loss and cognitive impairment is increasing. The aim of this study was to identify the impact of hearing rehabilitation via cochlear implantation on cognitive decline among the aging population. Patients and methods: This prospective study included 60 subjects aged between 50 and 84 years (mean 65.8 years, SD=8.9 with a severe to profound bilateral hearing impairment. A computer-based evaluation of short- and long-term memory, processing speed, attention, working memory and inhibition was performed prior to surgery as well as 6 and 12 months after cochlear implantation. Additionally, speech perception at 65 and 80 dB (Freiburger monosyllabic speech test as well as disease-related (Nijmegen Cochlear Implant Questionnaire and general (WHOQOL-OLD quality of life were assessed. Results: Six months postimplantation, speech perception, quality of life and also neurocognitive abilities significantly increased. The most remarkable improvement after 6 months was detected in executive functions such as attention (p<0.001, inhibition (p=0.025 and working memory (n-back: p=0.002; operation span task: p=0.008, followed by delayed recall (p=0.03. In contrast, long-term memory showed a significant change of performance only after 12 months (p=0.021. After 6 months, most cognitive domains remained stable, except working memory assessed by the operation span task, which significantly improved between 6 and 12 months (p<0.001. No

Important Factors in the Cognitive Development of Children with Hearing Impairment: Case Studies of Candidates for Cochlear Implants

Directory of Open Access Journals (Sweden)

Nasralla, Heloisa Romeiro

2014-06-01

Full Text Available Introduction The factors that affect the development of children with and without hearing disabilities are similar, provided their innate communication abilities are taken into account. Parents need to mourn the loss of the expected normally hearing child, and it is important that parents create bonds of affection with their child. Objective To conduct a postevaluation of the development and cognition of 20 candidates for cochlear implants between 1 and 13 years of age and to observe important factors in their development. Methods The following instruments were used in accordance with their individual merits: interviews with parents; the Vineland Social Maturity Scale; the Columbia Maturity Scale; free drawings; Bender and Pre-Bender testing; and pedagogical tests. Results The results are described. Conclusion Parental acceptance of a child's deafness proved to be the starting point for the child's verbal or gestural communication development, as well as for cognitive, motor, and emotional development. If the association between deafness and fine motor skills (with or without multiple disabilities undermines the development of a child's speech, it does not greatly affect communication when the child interacts with his or her peers and receives maternal stimulation. Overprotection and poor sociability make children less independent, impairs their development, and causes low self-esteem. Further observational studies are warranted to determine how cochlear implants contribute to patient recovery.

Important factors in the cognitive development of children with hearing impairment: case studies of candidates for cochlear implants.

Science.gov (United States)

Nasralla, Heloisa Romeiro; Goffi Gomez, Maria Valéria Schimidt; Magalhaes, Ana Tereza; Bento, Ricardo Ferreira

2014-10-01

Introduction The factors that affect the development of children with and without hearing disabilities are similar, provided their innate communication abilities are taken into account. Parents need to mourn the loss of the expected normally hearing child, and it is important that parents create bonds of affection with their child. Objective To conduct a postevaluation of the development and cognition of 20 candidates for cochlear implants between 1 and 13 years of age and to observe important factors in their development. Methods The following instruments were used in accordance with their individual merits: interviews with parents; the Vineland Social Maturity Scale; the Columbia Maturity Scale; free drawings; Bender and Pre-Bender testing; and pedagogical tests. Results The results are described. Conclusion Parental acceptance of a child's deafness proved to be the starting point for the child's verbal or gestural communication development, as well as for cognitive, motor, and emotional development. If the association between deafness and fine motor skills (with or without multiple disabilities) undermines the development of a child's speech, it does not greatly affect communication when the child interacts with his or her peers and receives maternal stimulation. Overprotection and poor sociability make children less independent, impairs their development, and causes low self-esteem. Further observational studies are warranted to determine how cochlear implants contribute to patient recovery.

Evidence of across-channel processing for spectral-ripple discrimination in cochlear implant listeners a

Science.gov (United States)

Ho Won, Jong; Jones, Gary L.; Drennan, Ward R.; Jameyson, Elyse M.; Rubinstein, Jay T.

2011-01-01

Spectral-ripple discrimination has been used widely for psychoacoustical studies in normal-hearing, hearing-impaired, and cochlear implant listeners. The present study investigated the perceptual mechanism for spectral-ripple discrimination in cochlear implant listeners. The main goal of this study was to determine whether cochlear implant listeners use a local intensity cue or global spectral shape for spectral-ripple discrimination. The effect of electrode separation on spectral-ripple discrimination was also evaluated. Results showed that it is highly unlikely that cochlear implant listeners depend on a local intensity cue for spectral-ripple discrimination. A phenomenological model of spectral-ripple discrimination, as an “ideal observer,” showed that a perceptual mechanism based on discrimination of a single intensity difference cannot account for performance of cochlear implant listeners. Spectral modulation depth and electrode separation were found to significantly affect spectral-ripple discrimination. The evidence supports the hypothesis that spectral-ripple discrimination involves integrating information from multiple channels. PMID:21973363

Visual impairment in Finnish Usher syndrome type III.

Science.gov (United States)

Plantinga, Rutger F; Pennings, Ronald J E; Huygen, Patrick L M; Sankila, Eeva-Marja; Tuppurainen, Kaija; Kleemola, Leenamaija; Cremers, Cor W R J; Deutman, August F

2006-02-01

To evaluate visual impairment in Finnish Usher syndrome type 3 (USH3) and compare this with visual impairment in Usher syndrome types 1b (USH1b) and 2a (USH2a). We carried out a retrospective study of 28 Finnish USH3 patients, 24 Dutch USH2a patients and 17 Dutch USH1b patients. Cross-sectional regression analyses of the functional acuity score (FAS), functional field score (FFS*) and functional vision score (FVS*) related to age were performed for all patients. The FFS* and FVS* were calculated using the isoptre V-4 test target instead of the usual III-4 target. Statistical tests relating to regression lines and Student's t-test were used to compare between USH3 patients and the other genetic subtypes of Usher syndrome. Cross-sectional analyses revealed significant deterioration in the FAS (1.3% per year), FFS* (1.4% per year) and FVS* (1.8% per year) with advancing age in the USH3 patient group. At a given age the USH3 patients showed significantly poorer visual field function than the USH2a patients. The rate of deterioration in visual function in Finnish USH3 patients was fairly similar to that in Dutch USH1b or USH2a patients. At a given age, visual field impairment in USH3 patients was similar to that in USH1b patients but poorer than in USH2a patients.

Parent and Patient Perceptions of Functional Impairment Due to Tourette Syndrome: Development of a Shortened Version of the Child Tourette Syndrome Impairment Scale.

Science.gov (United States)

Barfell, Kara S Francis; Snyder, Ryan R; Isaacs-Cloes, Kelly M; Garris, Jordan F; Roeckner, Alyssa R; Horn, Paul S; Guthrie, Michael D; Wu, Steve W; Gilbert, Donald L

2017-07-01

The Child Tourette Syndrome Impairment Scale (CTIM) rates 37 problems in school, social, and home domains separately for tics and for comorbid diagnoses. However, a shorter version would be easier to implement in busy clinics. Using published data from 85 children with Tourette syndrome, 92 controls, and parents, factor analysis was used to generate a "mini-CTIM" composed of 12 items applied to tic and comorbid diagnoses. Child- and parent-rated mini-CTIM scores were compared and correlated across raters and accounting for clinician-rated tic severity and presence of attention-deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD). The mini-CTIM achieved domain Cronbach alphas ranging from 0.71 to 0.94 and intra-item correlation coefficients ranging from 0.84 to 0.96. The resulting scale correlated with clinician-rated tic severity and reflected the presence of ADHD and OCD. The mini-CTIM appears promising as a practical assessment tool for tic- and non-tic-related impairment in children with Tourette syndrome.

Technical devices for hearing-impaired individuals: cochlear implants and brain stem implants - developments of the last decade.

Science.gov (United States)

Müller, Joachim

2005-01-01

Over the past two decades, the fascinating possibilities of cochlear implants for congenitally deaf or deafened children and adults developed tremendously and created a rapidly developing interdisciplinary research field.The main advancements of cochlear implantation in the past decade are marked by significant improvement of hearing and speech understanding in CI users. These improvements are attributed to the enhancement of speech coding strategies.The Implantation of more (and increasingly younger) children as well as the possibilities of the restoration of binaural hearing abilities with cochlear implants reflect the high standards reached by this development. Despite this progress, modern cochlear implants do not yet enable normal speech understanding, not even for the best patients. In particular speech understanding in noise remains problematic [1]. Until the mid 1990ies research concentrated on unilateral implantation. Remarkable and effective improvements have been made with bilateral implantation since 1996. Nowadays an increasing numbers of patients enjoy these benefits.

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.

The Coxsackievirus and Adenovirus Receptor: a new adhesion protein in cochlear development.

Science.gov (United States)

Excoffon, Katherine J D A; Avenarius, Matthew R; Hansen, Marlan R; Kimberling, William J; Najmabadi, Hossein; Smith, Richard J H; Zabner, Joseph

2006-05-01

The Coxsackievirus and Adenovirus Receptor (CAR) is an essential regulator of cell growth and adhesion during development. The gene for CAR, CXADR, is located within the genomic locus for Usher syndrome type 1E (USH1E). Based on this and a physical interaction with harmonin, the protein responsible for USH1C, we hypothesized that CAR may be involved in cochlear development and that mutations in CXADR may be responsible for USH1E. The expression of CAR in the cochlea was determined by PCR and immunofluorescence microscopy. We found that CAR expression is highly regulated during development. In neonatal mice, CAR is localized to the junctions of most cochlear cell types but is restricted to the supporting and strial cells in adult cochlea. A screen of two populations consisting of non-syndromic deaf and Usher 1 patients for mutations in CXADR revealed one haploid mutation (P356S). Cell surface expression, viral receptor activity, and localization of the mutant form of CAR were indistinguishable from wild-type CAR. Although we were unable to confirm a role for CAR in autosomal recessive, non-syndromic deafness, or Usher syndrome type 1, based on its regulation, localization, and molecular interactions, CAR remains an attractive candidate for genetic deafness.

Diversity in cochlear morphology and its influence on cochlear implant electrode position

NARCIS (Netherlands)

Marel, K.S. van der; Briaire, J.J.; Wolterbeek, R..; Snel-Bongers, J.; Verbist, B.M.; Frijns, J.H.

2014-01-01

To define a minimal set of descriptive parameters for cochlear morphology and study its influence on the cochlear implant electrode position in relation to surgical insertion distance.Cochlear morphology and electrode position were analyzed using multiplanar reconstructions of the pre- and

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.

Screening for Vision Problems, Including Usher's Syndrome, among Hearing Impaired Students.

Science.gov (United States)

Fillman, Robyn D.; And Others

1987-01-01

A screening program for vision problems and Usher's Syndrome (a common cause of deaf-blindness) among 210 hearing-impaired students found 44 percent had significant vision problems and 1 percent had Usher's Syndrome. The program involved an interagency network of school, health care, and support personnel and utilized a dilated ophathalmological…

Audiological follow-up of 24 patients affected by Williams syndrome.

Science.gov (United States)

Barozzi, Stefania; Soi, Daniela; Spreafico, Emanuela; Borghi, Anna; Comiotto, Elisabetta; Gagliardi, Chiara; Selicorni, Angelo; Forti, Stella; Cesarani, Antonio; Brambilla, Daniele

2013-09-01

Williams syndrome is a neurodevelopmental disorder associated with cardiovascular problems, facial abnormalities and several behavioural and neurological disabilities. It is also characterized by some typical audiological features including abnormal sensitivity to sounds, cochlear impairment related to the outer hair cells of the basal turn of the cochlea, and sensorineural or mixed hearing loss, predominantly in the high frequency range. The aim of this report is to describe a follow-up study of auditory function in a cohort of children affected by this syndrome. 24 patients, aged 5-14 years, were tested by means of air/bone conduction pure-tone audiometry, immittance test and transient evoked otoacoustic emissions. They were evaluated again 5 years after the first assessment, and 10 of them underwent a second follow-up examination after a further 5 years. The audiometric results showed hearing loss, defined by a pure tone average >15 dB HL, in 12.5% of the participants. The incidence of hearing loss did not change over the 5-year period and increased to 30% in the patients who underwent the 10-year follow-up. Progressive sensorineural hearing loss was detected in 20% of the patients. A remarkable finding of our study regarded sensorineural hearing impairment in the high frequency range, which increased significantly from 25% to 50% of the participants over the 5-year period. The increase became even more significant in the group of patients who underwent the 10-year follow-up, by which time the majority of them (80%) had developed sensorineural hearing loss. Otoacoustic emissions were found to be absent in a high percentage of patients, thus confirming the cochlear fragility of individuals with Williams syndrome. Our study verified that most of the young Williams syndrome patients had normal hearing sensitivity within the low-middle frequency range, but showed a weakness regarding the high frequencies, the threshold of which worsened significantly over time in

Benefits of simultaneous bilateral cochlear implantation on verbal reasoning skills in prelingually deaf children

NARCIS (Netherlands)

Jacobs, Evi; Langereis, Margreet C.; Frijns, Johan H. M.; Free, Rolien H.; Goedegebure, Andre; Smits, Cas; Stokroos, Robert J.; Ariens-Meijer, Saskia A. M.; Mylanus, Emmanuel A. M.; Vermeulen, Anneke M.

2016-01-01

Background: Impaired auditory speech perception abilities in deaf children with hearing aids compromised their verbal intelligence enormously. The availability of unilateral cochlear implantation (Cl) auditory speech perception and spoken vocabulary enabled them to reach near ageappropriate levels.

Benefits of simultaneous bilateral cochlear implantation on verbal reasoning skills in prelingually deaf children

NARCIS (Netherlands)

Jacobs, E.; Langereis, M.C.; Frijns, J.H.; Free, R.H.; Goedegebure, A.; Smits, C.; Stokroos, R.J.; Ariens-Meijer, S.A.; Mylanus, E.A.M.; Vermeulen, A.M.J.

2016-01-01

BACKGROUND: Impaired auditory speech perception abilities in deaf children with hearing aids compromised their verbal intelligence enormously. The availability of unilateral cochlear implantation (CI) auditory speech perception and spoken vocabulary enabled them to reach near ageappropriate levels.

Ethical considerations in resource allocation in a cochlear implant program.

Science.gov (United States)

Westerberg, Brian D; Pijl, Sipke; McDonald, Michael

2008-04-01

To review processes of resource allocation and the ethical considerations relevant to the fair allocation of a limited number of cochlear implants to increasing numbers of potential recipients. Review of relevant considerations. Tertiary referral hospital. Editorial discussion of the ethical issues of resource allocation. Heterogeneity of audiometric thresholds, self-reported disability of hearing loss, age of the potential cochlear implant recipient, cost-effectiveness, access to resources, compliance with follow-up, social support available to the recipient, social consequences of hearing impairment, and other recipient-related factors. In a publicly funded health care system, there will always be a need for decision-making processes for allocation of finite fiscal resources. All candidates for cochlear implantation deserve fair consideration. However, they are a heterogeneous group in terms of needs and expected outcomes consisting of traditional and marginal candidates, with a wide range of benefit from acoustic amplification. We argue that implant programs should thoughtfully prioritize treatment on the basis of need and potential benefit. We reject queuing on the basis of "first-come, first-served" or on the basis of perceived social worth.

Temporal integration of loudness in listeners with hearing losses of primarily cochlear origin

DEFF Research Database (Denmark)

Buus, Søren; Florentine, Mary; Poulsen, Torben

1999-01-01

To investigate how hearing loss of primarily cochlear origin affects the loudness of brief tones, loudness matches between 5- and 200-ms tones were obtained as a function of level for 15 listeners with cochlear impairments and for seven age-matched controls. Three frequencies, usually 0.5, 1, and 4...... of temporal integration—defined as the level difference between equally loud short and long tones—varied nonmonotonically with level and was largest at moderate levels. No consistent effect of frequency was apparent. The impaired listeners varied widely, but most showed a clear effect of level on the amount...... of temporal integration. Overall, their results appear consistent with expectations based on knowledge of the general properties of their loudness-growth functions and the equal-loudness-ratio hypothesis, which states that the loudness ratio between equal-SPL long and brief tones is the same at all SPLs...

Speech and language development in cognitively delayed children with cochlear implants.

Science.gov (United States)

Holt, Rachael Frush; Kirk, Karen Iler

2005-04-01

The primary goals of this investigation were to examine the speech and language development of deaf children with cochlear implants and mild cognitive delay and to compare their gains with those of children with cochlear implants who do not have this additional impairment. We retrospectively examined the speech and language development of 69 children with pre-lingual deafness. The experimental group consisted of 19 children with cognitive delays and no other disabilities (mean age at implantation = 38 months). The control group consisted of 50 children who did not have cognitive delays or any other identified disability. The control group was stratified by primary communication mode: half used total communication (mean age at implantation = 32 months) and the other half used oral communication (mean age at implantation = 26 months). Children were tested on a variety of standard speech and language measures and one test of auditory skill development at 6-month intervals. The results from each test were collapsed from blocks of two consecutive 6-month intervals to calculate group mean scores before implantation and at 1-year intervals after implantation. The children with cognitive delays and those without such delays demonstrated significant improvement in their speech and language skills over time on every test administered. Children with cognitive delays had significantly lower scores than typically developing children on two of the three measures of receptive and expressive language and had significantly slower rates of auditory-only sentence recognition development. Finally, there were no significant group differences in auditory skill development based on parental reports or in auditory-only or multimodal word recognition. The results suggest that deaf children with mild cognitive impairments benefit from cochlear implantation. Specifically, improvements are evident in their ability to perceive speech and in their reception and use of language. However, it may

Anatomic and Quantitative Temporal Bone CT for Preoperative Assessment of Branchio-Oto-Renal Syndrome.

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Ginat, D T; Ferro, L; Gluth, M B

2016-12-01

We describe the temporal bone computed tomography (CT) findings of an unusual case of branchio-oto-renal syndrome with ectopic ossicles that are partially located in the middle cranial fossa. We also describe quantitative temporal bone CT assessment pertaining to cochlear implantation in the setting of anomalous cochlear anatomy associated with this syndrome.

Relationship between changes in the cochlear blood flow and disorder of hearing function induced by blast injury in guinea pigs.

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Chen, Wei; Wang, Jianmin; Chen, Jing; Chen, Jichuan; Chen, Zhiqiang

2013-01-01

The auditory system is the most susceptible to damages from blast waves. Blast injuries always lead to varying degrees of hearing impairment. Although a disorder of the cochlear blood flow (CoBF) has been considered to be related to many pathological processes of the auditory system and to contribute to various types of hearing loss, changes in the CoBF induced by blast waves and the relationship between such changes and hearing impairment are undefined. To observe the changes in the cochlear microcirculation after exposure to an explosion blast, investigate the relationship between changes in the CoBF and hearing impairment and subsequently explore the mechanism responsible for the changes in the CoBF, we detected the perfusion of the cochlear microcirculation and hearing threshold shift after exposure to an explosion blast. Then, an N-nitro-L-arginine-methyl ester (L-NAME, NO synthase inhibitor) solution and artificial perilymph were applied to the round window (RW) of the cochlea before the blast exposure, followed by an evaluation of the CoBF and hearing function. The results indicated that the changes in the CoBF were correlated to the strength of the blast wave. The cochlear blood flow significantly increased when the peak value of the blast overpressure was greater than approximately 45 kPa, and there was no significant change in the cochlear blood flow when the peak value of the blast overpressure was less than approximately 35 kPa. Following local administration of the NO synthase inhibitor L-NAME, the increase in the CoBF induced by the blast was inhibited, and this reduction was significantly associated with the hearing threshold.

Cochlear function tests in estimation of speech dynamic range.

Science.gov (United States)

Han, Jung Ju; Park, So Young; Park, Shi Nae; Na, Mi Sun; Lee, Philip; Han, Jae Sang

2016-10-01

The loss of active cochlear mechanics causes elevated thresholds, loudness recruitment, and reduced frequency selectivity. The problems faced by hearing-impaired listeners are largely related with reduced dynamic range (DR). The aim of this study was to determine which index of the cochlear function tests correlates best with the DR to speech stimuli. Audiological data on 516 ears with pure tone average (PTA) of ≤55 dB and word recognition score of ≥70% were analyzed. PTA, speech recognition threshold (SRT), uncomfortable loudness (UCL), and distortion product otoacoustic emission (DPOAE) were explored as the indices of cochlear function. Audiometric configurations were classified. Correlation between each index and the DR was assessed and multiple regression analysis was done. PTA and SRT demonstrated strong negative correlations with the DR (r = -0.788 and -0.860, respectively), while DPOAE sum was moderately correlated (r = 0.587). UCLs remained quite constant for the total range of the DR. The regression equation was Y (DR) = 75.238 - 0.719 × SRT (R(2 )=( )0.721, p equation.

Benefits and Risks of Cochlear Implants

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... and Medical Procedures Implants and Prosthetics Cochlear Implants Benefits and Risks of Cochlear Implants Share Tweet Linkedin ... the Use of Cochlear Implants What are the Benefits of Cochlear Implants? For people with implants: Hearing ...

Experiences with bimodal hearing and bilateral cochlear implantation in the elderly

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E.M. Schedlbauer

2011-03-01

Full Text Available With the geriatric population being the fastest growing segment of our society the number of elderly people with different levels of hearing impairments is increasing. According to the German Society of Hearing Impaired there are concerned 37% of the 60-69-year-old and 54% of the over 70-year-old. Furthermore the needs of the elderly are changing with augmented social activity and agility compared to former times: 80 is the new 70. After initial concerns about poor audiologic performance and added peri- and postoperative risks because of comorbidities, cochlear implantation is becoming more and more the method of choice also for postlingually deaf people of higher age. With the demographic shift there can be observed a trend reversal: the proportion of implantees is sliding from the young to the senior and duration of deafness at the time of implantation is declining (Battmer 2010. It is therefore necessary to investigate the impact of cochlear implantation on audiologic performance and quality of life in this growing age group.

Assessment of Cochlear Function during Cochlear Implantation by Extra- and Intracochlear Electrocochleography

OpenAIRE

Dalbert, Adrian; Pfiffner, Flurin; Hoesli, Marco; Koka, Kanthaiah; Veraguth, Dorothe; Roosli, Christof; Huber, Alexander

2018-01-01

Objective: The aims of this study were: (1) To investigate the correlation between electrophysiological changes during cochlear implantation and postoperative hearing loss, and (2) to detect the time points that electrophysiological changes occur during cochlear implantation. Material and Methods: Extra- and intracochlear electrocochleography (ECoG) were used to detect electrophysiological changes during cochlear implantation. Extracochlear ECoG recordings were conducted through a needle elec...

Systemic Inflammation and Lung Function Impairment in Morbidly Obese Subjects with the Metabolic Syndrome

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Astrid van Huisstede

2013-01-01

Full Text Available Background. Obesity and asthma are associated. There is a relationship between lung function impairment and the metabolic syndrome. Whether this relationship also exists in the morbidly obese patients is still unknown. Hypothesis. Low-grade systemic inflammation associated with the metabolic syndrome causes inflammation in the lungs and, hence, lung function impairment. Methods. This is cross-sectional study of morbidly obese patients undergoing preoperative screening for bariatric surgery. Metabolic syndrome was assessed according to the revised NCEP-ATP III criteria. Results. A total of 452 patients were included. Patients with the metabolic syndrome (n=293 had significantly higher blood monocyte (mean 5.3 versus 4.9, P=0.044 and eosinophil percentages (median 1.0 versus 0.8, P=0.002, while the total leukocyte count did not differ between the groups. The FEV1/FVC ratio was significantly lower in patients with the metabolic syndrome (76.7% versus 78.2%, P=0.032. Blood eosinophils were associated with FEV1/FVC ratio (adj. B −0.113, P=0.018. Conclusion. Although the difference in FEV1/FVC ratio between the groups is relatively small, in this cross-sectional study, and its clinical relevance may be limited, these data indicate that the presence of the metabolic syndrome may influence lung function impairment, through the induction of relative eosinophilia.

Neurotrophic treatment of the degenerating auditory nerve; cochlear implants in deafened guinea pigs

NARCIS (Netherlands)

Agterberg, M.J.H.

2009-01-01

To date, the cochlear implant is the most successful sensorineural prosthesis. The device consists of a small array with a number of electrodes implanted in the cochlea of profoundly hearing impaired people. Some people with an implant are able to use the telephone. Unfortunately, others hardly

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

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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…

[Cochlear implant in children: rational, indications and cost/efficacy].

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Martini, A; Bovo, R; Trevisi, P; Forli, F; Berrettini, S

2013-06-01

A cochlear implant (CI) is a partially implanted electronic device that can help to provide a sense of sound and support speech to severely to profoundly hearing impaired patients. It is constituted by an external portion, that usually sits behind the ear and an internal portion surgically placed under the skin. The external components include a microphone connected to a speech processor that selects and arranges sounds pucked up by the microphone. This is connected to a transmitter coil, worn on the side of the head, which transmits data to an internal receiver coil placed under the skin. The received data are delivered to an array of electrodes that are surgically implanted within the cochlea. The primary neural targets of the electrodes are the spiral ganglion cells which innervate fibers of the auditory nerve. When the electrodes are activated by the signal, they send a current along the auditory nerve and auditory pathways to the auditory cortex. Children and adults who are profoundly or severely hearing impaired can be fitted with cochlear implants. According to the Food and Drug Administration, approximately 188,000 people worldwide have received implants. In Italy it is extimated that there are about 6-7000 implanted patients, with an average of 700 CI surgeries per year. Cochlear implantation, followed by intensive postimplantation speech therapy, can help young children to acquire speech, language, and social skills. Early implantation provides exposure to sounds that can be helpful during the critical period when children learn speech and language skills. In 2000, the Food and Drug Administration lowered the age of eligibility to 12 months for one type of CI. With regard to the results after cochlear implantation in relation to early implantation, better linguistic results are reported in children implanted before 12 months of life, even if no sufficient data exist regarding the relation between this advantage and the duration of implant use and how long

Specific Language Impairment, Nonverbal IQ, Attention-Deficit/Hyperactivity Disorder, Autism Spectrum Disorder, Cochlear Implants, Bilingualism, and Dialectal Variants: Defining the Boundaries, Clarifying Clinical Conditions, and Sorting Out Causes.

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Rice, Mabel L

2016-02-01

The purpose of this research forum article is to provide an overview of a collection of invited articles on the topic "specific language impairment (SLI) in children with concomitant health conditions or nonmainstream language backgrounds." Topics include SLI, attention-deficit/hyperactivity disorder, autism spectrum disorder, cochlear implants, bilingualism, and dialectal language learning contexts. The topic is timely due to current debates about the diagnosis of SLI. An overarching comparative conceptual framework is provided for comparisons of SLI with other clinical conditions. Comparisons of SLI in children with low-normal or normal nonverbal IQ illustrate the unexpected outcomes of 2 × 2 comparison designs. Comparative studies reveal unexpected relationships among speech, language, cognitive, and social dimensions of children's development as well as precise ways to identify children with SLI who are bilingual or dialect speakers. The diagnosis of SLI is essential for elucidating possible causal pathways of language impairments, risks for language impairments, assessments for identification of language impairments, linguistic dimensions of language impairments, and long-term outcomes. Although children's language acquisition is robust under high levels of risk, unexplained individual variations in language acquisition lead to persistent language impairments.

Cognitive impairment, clinical severity and MRI changes in MELAS syndrome.

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Kraya, Torsten; Neumann, Lena; Paelecke-Habermann, Yvonne; Deschauer, Marcus; Stoevesandt, Dietrich; Zierz, Stephan; Watzke, Stefan

2017-12-29

To examine clinical severity, cognitive impairment, and MRI changes in patients with MELAS syndrome. Cognitive-mnestic functions, brain MRI (lesion load, cella media index) and clinical severity of ten patients with MELAS syndrome were examined. All patients carried the m.3243A>G mutation. The detailed neuropsychological assessment revealed cognitive deficits in attention, executive function, visuoperception, and -construction. There were significant correlations between these cognitive changes, lesion load in MRI, disturbances in everyday life (clinical scale), and high scores in NMDAS. Patients with MELAS syndrome showed no global neuropsychological deficit, but rather distinct cognitive deficits. Copyright © 2018 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

Influence of hearing age and understanding verbal instructions in children with cochlear implants

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Đoković Sanja

2013-01-01

Full Text Available Hearing age is defined as a period of using any amplification. Most researches indicate that hearing age influences the developmental rate of auditory and speech-language abilities in deaf children, especially when cochlear implantation was performed before the age of three. This research is aimed at analyzing the influence of hearing age on understanding verbal instructions in children with cochlear implants. The sample consists of 23 children with cochlear implants and 21 children with normal hearing, aged between 4 and 10. Hearing age of children with cochlear implants was between 2 and 7 years. Token Test with toys, adapted for children with hearing impairments, was used to analyze understanding verbal instructions. The results indicate that there are statistically significant differences between children with cochlear implants and children with normal hearing, aged between 4 and 7, on all subtests and the total score regardless of the hearing age (sub1 p<0.001, sub2 p<0.000, sub3 p<0.001, total score p<0.000. No statistically significant differences were determined on any of the subtests in children aged between 7.1 and 10, regardless of the hearing age. Comparative results analysis within the experimental group of children with different hearing age indicates that the difference in understanding verbal instructions between these two groups is not statistically significant.

Trends in cochlear implants.

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Zeng, Fan-Gang

2004-01-01

More than 60,000 people worldwide use cochlear implants as a means to restore functional hearing. Although individual performance variability is still high, an average implant user can talk on the phone in a quiet environment. Cochlear-implant research has also matured as a field, as evidenced by the exponential growth in both the patient population and scientific publication. The present report examines current issues related to audiologic, clinical, engineering, anatomic, and physiologic aspects of cochlear implants, focusing on their psychophysical, speech, music, and cognitive performance. This report also forecasts clinical and research trends related to presurgical evaluation, fitting protocols, signal processing, and postsurgical rehabilitation in cochlear implants. Finally, a future landscape in amplification is presented that requires a unique, yet complementary, contribution from hearing aids, middle ear implants, and cochlear implants to achieve a total solution to the entire spectrum of hearing loss treatment and management.

Congenitally Deafblind Children and Cochlear Implants

DEFF Research Database (Denmark)

Dammeyer, Jesper Herup

2008-01-01

There has been much research conducted demonstrating the positive benefits of cochlear implantation (CI) in children who are deaf. Research on cochlear implantation in children who are both deaf and blind, however, is lacking. The purpose of this article is to present a study of 5 congenitally...... deafblind children who received cochlear implants between 2.2 and 4.2 years of age.  Ratings of video observations were used to measure the children's early communication development with and without the use of their cochlear implants. In addition, parental interviews were used to assess the benefits...... parents perceived regarding their children's cochlear implants. Two examples are included in this article to illustrate the parents' perspectives about cochlear implantation in their deafblind children. Benefits of cochlear implantation in this cohort of children included improved attention and emotional...

Anaesthesia Management in a Patient with Waardenburg Syndrome and Review of the Literature.

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Peker, Kevser; Ergil, Julide; Öztürk, İbrahim

2015-10-01

Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism. Incidence is estimated as 2%-3% among the cases of congenital deafness and 1/42,000 of the general population. Children with Waardenburg syndrome usually require anaesthesia for the cochlear implant operation in early age. The features of the syndrome that may bear importance for anaesthetic management are laryngomalacia, multiple muscle contractures, limited neck movements, cyanotic cardiopathy and electrolyte imbalance. Patients with Waardenburg syndrome stand for difficult airway. We aimed to report anaesthetic management of a child with Waardenburg syndrome who underwent surgery for cochlear implantation.

Characterization of Cochlear, Vestibular and Cochlear-Vestibular Electrically Evoked Compound Action Potentials in Patients with a Vestibulo-Cochlear Implant

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T. A. K. Nguyen

2017-11-01

Full Text Available The peripheral vestibular system is critical for the execution of activities of daily life as it provides movement and orientation information to motor and sensory systems. Patients with bilateral vestibular hypofunction experience a significant decrease in quality of life and have currently no viable treatment option. Vestibular implants could eventually restore vestibular function. Most vestibular implant prototypes to date are modified cochlear implants to fast-track development. These use various objective measurements, such as the electrically evoked compound action potential (eCAP, to supplement behavioral information. We investigated whether eCAPs could be recorded in patients with a vestibulo-cochlear implant. Specifically, eCAPs were successfully recorded for cochlear and vestibular setups, as well as for mixed cochlear-vestibular setups. Similarities and slight differences were found for the recordings of the three setups. These findings demonstrated the feasibility of eCAP recording with a vestibulo-cochlear implant. They could be used in the short term to reduce current spread and avoid activation of non-targeted neurons. More research is warranted to better understand the neural origin of vestibular eCAPs and to utilize them for clinical applications.

Visual acuity and visual field impairment in Usher syndrome.

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Edwards, A; Fishman, G A; Anderson, R J; Grover, S; Derlacki, D J

1998-02-01

To determine the extent of visual acuity and visual field impairment in patients with types 1 and 2 Usher syndrome. The records of 53 patients with type 1 and 120 patients with type 2 Usher syndrome were reviewed for visual acuity and visual field area at their most recent visit. Visual field areas were determined by planimetry of the II4e and V4e isopters obtained with a Goldmann perimeter. Both ordinary and logistic regression models were used to evaluate differences in visual acuity and visual field impairment between patients with type 1 and type 2 Usher syndrome. The difference in visual acuity of the better eye between patients with type 1 and type 2 varied by patient age (P=.01, based on a multiple regression model). The maximum difference in visual acuity between the 2 groups occurred during the third and fourth decades of life (with the type 1 patients being more impaired), while more similar acuities were seen in both younger and older patients. Fifty-one percent (n=27) of the type 1 patients had a visual acuity of 20/40 or better in at least 1 eye compared with 72% (n=87) of the type 2 patients (age-adjusted odds ratio, 3.9). Visual field area to both the II4e (P=.001) and V4e (Ptype 1 patients than type 2 patients. A concentric central visual field greater than 20 degrees in at least 1 eye was present in 20 (59%) of the available 34 visual fields of type 1 patients compared with 70 (67%) of the available 104 visual fields of type 2 patients (age-adjusted odds ratio, 2.9) with the V4e target and in 6 (21%) of the available 29 visual fields of type 1 patients compared with 36 (38%) of the available 94 visual fields of type 2 patients (age-adjusted odds ratio, 4.9) with the II4e target. The fraction of patients who had a visual acuity of 20/40 or better and a concentric central visual field greater than 20 degrees to the II4e target in at least 1 eye was 17% (n=5) in the type 1 patients and 35% (n=33) in the type 2 patients (age-adjusted odds ratio, 3

Retrolabyrinthine approach for cochlear nerve preservation in neurofibromatosis type 2 and simultaneous cochlear implantation

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Bento, Ricardo Ferreira

2014-01-01

Full Text Available Introduction: Few cases of cochlear implantation (CI in neurofibromatosis type 2 (NF2 patients had been reported in the literature. The approaches described were translabyrinthine, retrosigmoid or middle cranial fossa. Objectives: To describe a case of a NF2- deafened-patient who underwent to vestibular schwannoma resection via RLA with cochlear nerve preservation and CI through the round window, at the same surgical time. Resumed Report: A 36-year-old woman with severe bilateral hearing loss due to NF2 was submitted to vestibular schwannoma resection and simultaneous CI. Functional assessment of cochlear nerve was performed by electrical promontory stimulation. Complete tumor removal was accomplishment via RLA with anatomic and functional cochlear and facial nerve preservation. Cochlear electrode array was partially inserted via round window. Sound field hearing threshold improvement was achieved. Mean tonal threshold was 46.2 dB HL. The patient could only detect environmental sounds and human voice but cannot discriminate vowels, words nor do sentences at 2 years of follow-up. Conclusion: Cochlear implantation is a feasible auditory restoration option in NF2 when cochlear anatomic and functional nerve preservation is achieved. The RLA is adequate for this purpose and features as an option for hearing preservation in NF2 patients.

Cochlear implant revision surgeries in children.

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Amaral, Maria Stella Arantes do; Reis, Ana Cláudia Mirândola B; Massuda, Eduardo T; Hyppolito, Miguel Angelo

2018-02-16

The surgery during which the cochlear implant internal device is implanted is not entirely free of risks and may produce problems that will require revision surgeries. To verify the indications for cochlear implantation revision surgery for the cochlear implant internal device, its effectiveness and its correlation with certain variables related to language and hearing. A retrospective study of patients under 18 years submitted to cochlear implant Surgery from 2004 to 2015 in a public hospital in Brazil. Data collected were: age at the time of implantation, gender, etiology of the hearing loss, audiological and oral language characteristics of each patient before and after Cochlear Implant surgery and any need for surgical revision and the reason for it. Two hundred and sixty-five surgeries were performed in 236 patients. Eight patients received a bilateral cochlear implant and 10 patients required revision surgery. Thirty-two surgeries were necessary for these 10 children (1 bilateral cochlear implant), of which 21 were revision surgeries. In 2 children, cochlear implant removal was necessary, without reimplantation, one with cochlear malformation due to incomplete partition type I and another due to trauma. With respect to the cause for revision surgery, of the 8 children who were successfully reimplanted, four had cochlear calcification following meningitis, one followed trauma, one exhibited a facial nerve malformation, one experienced a failure of the cochlear implant internal device and one revision surgery was necessary because the electrode was twisted. The incidence of the cochlear implant revision surgery was 4.23%. The period following the revision surgeries revealed an improvement in the subject's hearing and language performance, indicating that these surgeries are valid in most cases. Copyright © 2018 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

A Longitudinal Study of Pragmatic Language Development in Three Children with Cochlear Implants

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Dammeyer, Jesper

2012-01-01

Research has shown how cochlear implants (CIs), in children with hearing impairments, have improved speech perception and production, but very little is known about the children's pragmatic language development. During a 4-year longitudinal study of three children with CIs, certain aspects of pragmatic language development were observed in free…

Cochlear implantation in Mondini dysplasia.

Science.gov (United States)

Daneshi, Ahmad; Hassanzadeh, Saeid; Abasalipour, Parvaneh; Emamdjomeh, Hessamaddin; Farhadi, Mohammad

2003-01-01

The use of cochlear implantation to treat patients with inner ear malformations such as Mondini dysplasia has been increasingly successful. Until now, conventional hearing aids in these patients have not performed well. Consequently, the hearing problem for patients with this condition has been somewhat improved with the use of cochlear implants. Various results of cochlear implantation have been reported in these patients so far. This is a report of 5 patients with Mondini malformation who have undergone cochlear implant surgery. Copyright 2003 S. Karger AG, Basel

Wired to freedom: Life science, public politics, and the case of Cochlear Implantation.

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Jepsen, Kim Sune; Bertilsson, T Margareta

2017-02-01

Cochlear Implantation is now regarded as the most successful medical technology. It carries promises to provide deaf/hearing impaired individuals with a technological sense of hearing and an access to participate on a more equal level in social life. In this article, we explore the adoption of cochlear implantations among Danish users in order to shed more light on their social and political implications. We situate cochlear implantation in a framework of new life science advances, politics, and user experiences. Analytically, we draw upon the notion of social imaginary and explore the social dimension of life science through a notion of public politics adopted from the political theory of John Dewey. We show how cochlear implantation engages different social imaginaries on the collective and individual levels and we suggest that users share an imaginary of being "wired to freedom" that involves new access to social life, continuous communicative challenges, common practices, and experiences. In looking at their lives as "wired to freedom," we hope to promote a wider spectrum of civic participation in the benefit of future life science developments within and beyond the field of Cochlear Implantation. As our empirical observations are largely based in the Scandinavian countries (notably Denmark), we also provide some reflections on the character of the technology-friendly Scandinavian welfare states and the unintended consequences that may follow in the wake of rapid technology implementation of life science in society.

[Morphologic feature and cochlear implant surgical approach for cochlear modiolus deficiency].

Science.gov (United States)

Zhang, Daoxing

2014-09-01

To review the classification of cochlear modiolus deficiency and decision on surgical approach for above case,in order to provide mastery for cochlear implant (CI) indication. Basing on temporal bone HRCT pre-operation, CI subjects with modiolus deficiency were defined as following groups: (1) deficiency caused by cochlear dysplasia (Mondini malformation); (2) deficiency caused by dysplasia of cochlear and vestibule (Common cavity malformation); (3) deficiency caused by absence of internal acoustic meatus fundus (IP-III malformation). Three types of surgical approach were utilized: type I, electrode array was introduced through facial recess, enlarged the round window, type II, opened the surface of chchlea, electrode array was introduced through facial recess, fenestration on posterior promontory and then inserted around lateral wall of inner-cochlear cavity. type III, electrode array was introduce through fenestration of lateral semicircular canal and then placed close to the bony wall of common cavity. One hundred and sixty-six cochlear modiolus deficiency cases were identified into 3 groups as following: 135 Mondini malformation cases into group a, 18 common cavity malformation cases into group b, and 13 IP-III malformation cases into group c. Surgical approach: type I were used in 136 cases (123 Mondini cases and 13 IP-III cases), while approach type II in 12 cases (12 Mondini cases), and approach type III in 18 cases (18 common cavity cases). Income post-operation of CI: For group a (Mondini malformation), post-activation mean hearing threshold in sound field was 65 dB, speech recognition score is 95% (single finals test) and 25% (signal initials test), while it was 80 dB, 60% and 0 for group b (Conmon cavity malformation), and it was 55 dB, 100% and 45% for group c (IP-III malformation). The income of speech recognition score for cochlear modiolus deficiency was relatively poor, group b was worst and group c was best, while group a moderate.

Constitutional bone impairment in Noonan syndrome.

Science.gov (United States)

Baldassarre, Giuseppina; Mussa, Alessandro; Carli, Diana; Molinatto, Cristina; Ferrero, Giovanni Battista

2017-03-01

Noonan syndrome (NS) is an autosomal dominant trait characterized by genotypic and phenotypic variability. It belongs to the Ras/MAPK pathway disorders collectively named Rasopathies or neurocardiofaciocutaneous syndromes. Phenotype is characterized by short stature, congenital heart defects, facial dysmorphisms, skeletal and ectodermal anomalies, cryptorchidism, mild to moderate developmental delay/learning disability, and tumor predisposition. Short stature and skeletal dysmorphisms are almost constant and several studies hypothesized a role for the RAS pathway in regulating bone metabolism. In this study, we investigated the bone quality assessed by phalangeal quantitative ultrasound (QUS) and the metabolic bone profiling in a group of patients with NS, to determine whether low bone mineralization is primary or secondary to NS characteristics. Thirty-five patients were enrolled, including 20 males (55.6%) and 15 females (44.5%) aged 1.0-17.8 years (mean 6.4 ± 4.5, median 4.9 years). Each patients was submitted to clinical examination, estimation of the bone age, laboratory assays, and QUS assessment. Twenty-five percent of the cohort shows reduced QUS values for their age based on bone transmission time. Bone measurement were adjusted for multiple factors frequently observed in NS patients, such as growth retardation, delayed bone age, retarded puberty, and reduced body mass index, potentially affecting bone quality or its appraisal. In spite of the correction attempts, QUS measurement indicates that bone impairment persists in nearly 15% of the cohort studied. Our results indicate that bone impairment in NS is likely primary and not secondary to any of the phenotypic traits of NS, nor consistent with metabolic disturbances. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

Cockayne syndrome group B (Csb) and group a (Csa) deficiencies predispose to hearing loss and cochlear hair cell degeneration in mice.

Science.gov (United States)

Nagtegaal, A Paul; Rainey, Robert N; van der Pluijm, Ingrid; Brandt, Renata M C; van der Horst, Gijsbertus T J; Borst, J Gerard G; Segil, Neil

2015-03-11

Sensory hair cells in the cochlea, like most neuronal populations that are postmitotic, terminally differentiated, and non-regenerating, depend on robust mechanisms of self-renewal for lifelong survival. We report that hair cell homeostasis requires a specific sub-branch of the DNA damage nucleotide excision repair pathway, termed transcription-coupled repair (TCR). Cockayne syndrome (CS), caused by defects in TCR, is a rare DNA repair disorder with a broad clinical spectrum that includes sensorineural hearing loss. We tested hearing and analyzed the cellular integrity of the organ of Corti in two mouse models of this disease with mutations in the Csb gene (CSB(m/m) mice) and Csa gene (Csa(-/-) mice), respectively. Csb(m/m) and Csa(-/-) mice manifested progressive hearing loss, as measured by an increase in auditory brainstem response thresholds. In contrast to wild-type mice, mutant mice showed reduced or absent otoacoustic emissions, suggesting cochlear outer hair cell impairment. Hearing loss in Csb(m/m) and Csa(-/-) mice correlated with progressive hair cell loss in the base of the organ of Corti, starting between 6 and 13 weeks of age, which increased by 16 weeks of age in a basal-to-apical gradient, with outer hair cells more severely affected than inner hair cells. Our data indicate that the hearing loss observed in CS patients is reproduced in mouse models of this disease. We hypothesize that accumulating DNA damage, secondary to the loss of TCR, contributes to susceptibility to hearing loss. Copyright © 2015 the authors 0270-6474/15/354280-07$15.00/0.

COCHLEAR IMPLANTATION PREVALENCE IN ELDERLY

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A. V. Starokha

2014-01-01

Full Text Available Current paper describes an experience of cochlear implantation in elderly. Cochlear implantation has become a widely accepted intervention in the treatment of individuals with severe-to-profound sensorineural hearing loss. Cochlear implants are now accepted as a standard of care to optimize hearing and subsequent speech development in children and adults with deafness. But cochlear implantation affects not only hearing abilities, speech perception and speech production; it also has an outstanding impact on the social life, activities and self-esteem of each patient. The aim of this study was to evaluate the cochlear implantation efficacy in elderly with severe to profound sensorineural hearing loss. There were 5 patients under our observation. Surgery was performed according to traditional posterior tympanotomy and cochleostomy for cochlear implant electrode insertion for all observed patients. The study was conducted in two stages: before speech processor’s activation and 3 months later. Pure tone free field audiometry was performed to each patient to assess the efficiency of cochlear implantation in dynamics. The aim of the study was also to evaluate quality of life in elderly with severe to profound sensorineural hearing loss after unilateral cochlear implantation. Each patient underwent questioning with 36 Item Short Form Health Survey (SF-36. SF-36 is a set of generic, coherent, and easily administered quality-of-life measures. The SF-36 consists of eight scaled scores, which are the weighted sums of the questions in their section. Each scale is directly transformed into a 0-100 scale on the assumption that each question carries equal weight. The eight sections are: physical functioning; physical role functioning; emotional role functioning; vitality; emotional well-being; social role functioning; bodily pain; general health perceptions. Our results demonstrate that cochlear implantation in elderly consistently improved quality of life

Korsakoff Syndrome in Non-alcoholic Psychiatric Patients. Variable Cognitive Presentation and Impaired Frontotemporal Connectivity

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Georgios Nikolakaros

2018-05-01

Full Text Available Background: Non-alcoholic Wernicke's encephalopathy and Korsakoff syndrome are greatly underdiagnosed. There are very few reported cases of neuropsychologically documented non-alcoholic Korsakoff syndrome, and diffusion tensor imaging (DTI data are scarce.Methods: We report clinical characteristics and neuropsychological as well as radiological findings from three psychiatric patients (one woman and two men with a history of probable undiagnosed non-alcoholic Wernicke's encephalopathy and subsequent chronic memory problems.Results: All patients had abnormal neuropsychological test results, predominantly in memory. Thus, the neuropsychological findings were compatible with Korsakoff syndrome. However, the neuropsychological findings were not uniform. The impairment of delayed verbal memory of the first patient was evident only when the results of the memory tests were compared to her general cognitive level. In addition, the logical memory test and the verbal working memory test were abnormal, but the word list memory test was normal. The second patient had impaired attention and psychomotor speed in addition to impaired memory. In the third patient, the word list memory test was abnormal, but the logical memory test was normal. All patients had intrusions in the neuropsychological examination. Executive functions were preserved, except for planning and foresight, which were impaired in two patients. Conventional MRI examination was normal. DTI showed reduced fractional anisotropy values in the uncinate fasciculus in two patients, and in the corpus callosum and in the subgenual cingulum in one patient.Conclusions: Non-alcoholic Korsakoff syndrome can have diverse neuropsychological findings. This may partly explain its marked underdiagnosis. Therefore, a strong index of suspicion is needed. The presence of intrusions in the neuropsychological examination supports the diagnosis. Damage in frontotemporal white matter tracts, particularly in the

Korsakoff Syndrome in Non-alcoholic Psychiatric Patients. Variable Cognitive Presentation and Impaired Frontotemporal Connectivity.

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Nikolakaros, Georgios; Kurki, Timo; Paju, Janina; Papageorgiou, Sokratis G; Vataja, Risto; Ilonen, Tuula

2018-01-01

Background: Non-alcoholic Wernicke's encephalopathy and Korsakoff syndrome are greatly underdiagnosed. There are very few reported cases of neuropsychologically documented non-alcoholic Korsakoff syndrome, and diffusion tensor imaging (DTI) data are scarce. Methods: We report clinical characteristics and neuropsychological as well as radiological findings from three psychiatric patients (one woman and two men) with a history of probable undiagnosed non-alcoholic Wernicke's encephalopathy and subsequent chronic memory problems. Results: All patients had abnormal neuropsychological test results, predominantly in memory. Thus, the neuropsychological findings were compatible with Korsakoff syndrome. However, the neuropsychological findings were not uniform. The impairment of delayed verbal memory of the first patient was evident only when the results of the memory tests were compared to her general cognitive level. In addition, the logical memory test and the verbal working memory test were abnormal, but the word list memory test was normal. The second patient had impaired attention and psychomotor speed in addition to impaired memory. In the third patient, the word list memory test was abnormal, but the logical memory test was normal. All patients had intrusions in the neuropsychological examination. Executive functions were preserved, except for planning and foresight, which were impaired in two patients. Conventional MRI examination was normal. DTI showed reduced fractional anisotropy values in the uncinate fasciculus in two patients, and in the corpus callosum and in the subgenual cingulum in one patient. Conclusions: Non-alcoholic Korsakoff syndrome can have diverse neuropsychological findings. This may partly explain its marked underdiagnosis. Therefore, a strong index of suspicion is needed. The presence of intrusions in the neuropsychological examination supports the diagnosis. Damage in frontotemporal white matter tracts, particularly in the uncinate

Audiometric Characteristics of a Dutch DFNA10 Family With Mid-Frequency Hearing Impairment.

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van Beelen, Eline; Oonk, Anne M M; Leijendeckers, Joop M; Hoefsloot, Elisabeth H; Pennings, Ronald J E; Feenstra, Ilse; Dieker, Hendrik-Jan; Huygen, Patrick L M; Snik, Ad F M; Kremer, Hannie; Kunst, Henricus P M

2016-01-01

Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the hearing impairment in this family. Whole exome sequencing was performed in the proband. In addition, peripheral blood samples were collected from 23 family members, and segregation analyses were performed. All participants underwent otorhinolaryngological examinations and pure-tone audiometry, and 12 participants underwent speech audiometry. In addition, an extended set of audiometric measurements was performed in five family members to evaluate the functional status of the cochlea. Vestibular testing was performed in three family members. Two individuals underwent echocardiography to evaluate the nonsyndromic phenotype. The authors present a Dutch family with a truncating mutation in EYA4 causing a mid-frequency hearing impairment. This mutation (c.464del) leads to a frameshift and a premature stop codon (p.Pro155fsX). This mutation is the most N-terminal mutation in EYA4 found to date. In addition, a missense mutation, predicted to be deleterious, was found in EYA4 in two family members. Echocardiography in two family members revealed no signs of dilated cardiomyopathy. Results of caloric and velocity step tests in three family members showed no abnormalities. Hearing impairment was found to be symmetric and progressive, beginning as a mid-frequency hearing impairment in childhood and developing into a high-frequency, moderate hearing impairment later in life. Furthermore, an extended set of audiometric measurements was performed in five family members. The results were comparable to those obtained in patients with other sensory types of hearing impairments, such as patients with Usher syndrome type IIA and presbyacusis, and not to those obtained in patients with (cochlear

Neurocognitive impairment in childhood chronic fatigue syndrome

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Kei eMizuno

2013-04-01

Full Text Available Neurocognitive impairment is a feature of childhood chronic fatigue syndrome (CCFS. Several studies have demonstrated reduced attention control in CCFS patients in switching and divided attention tasks. In students, the extent of deterioration in task performance depends on the level of fatigue. Poor performance in switching and divided attention is common in both fatigued students and CCFS patients. Additionally, attentional functions show dramatic development from childhood to adolescence, suggesting that abnormal development of switching and divided attention may be induced by chronic fatigue. The brain structures associated with attentional control are situated in the frontal and parietal cortices, which are the last to mature, suggesting that severe fatigue in CCFS patients and students may inhibit normal structural and functional development in these regions. A combination of treatment with cognitive behavioral therapy and antidepressant medication is effective to improve attentional control processing in CCFS patients. Studies identifying the features of neurocognitive impairment in CCFS have improved our current understanding of the neurophysiological mechanisms of CCFS.

Cochlear implant users' spectral ripple resolution.

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Jeon, Eun Kyung; Turner, Christopher W; Karsten, Sue A; Henry, Belinda A; Gantz, Bruce J

2015-10-01

This study revisits the issue of the spectral ripple resolution abilities of cochlear implant (CI) users. The spectral ripple resolution of recently implanted CI recipients (implanted during the last 10 years) were compared to those of CI recipients implanted 15 to 20 years ago, as well as those of normal-hearing and hearing-impaired listeners from previously published data from Henry, Turner, and Behrens [J. Acoust. Soc. Am. 118, 1111-1121 (2005)]. More recently, implanted CI recipients showed significantly better spectral ripple resolution. There is no significant difference in spectral ripple resolution for these recently implanted subjects compared to hearing-impaired (acoustic) listeners. The more recently implanted CI users had significantly better pre-operative speech perception than previously reported CI users. These better pre-operative speech perception scores in CI users from the current study may be related to better performance on the spectral ripple discrimination task; however, other possible factors such as improvements in internal and external devices cannot be excluded.

Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

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Micol Busi

2015-01-01

Full Text Available Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT and magnetic resonance imaging (MRI in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years. Among these, 143 patients (64 females and 79 males presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children ranged from 9 months and 16 years (average = 4.4; median = 3.0. Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities

Cochlear implant users move in time to the beat of drum music.

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Phillips-Silver, Jessica; Toiviainen, Petri; Gosselin, Nathalie; Turgeon, Christine; Lepore, Franco; Peretz, Isabelle

2015-03-01

Cochlear implant users show a profile of residual, yet poorly understood, musical abilities. An ability that has received little to no attention in this population is entrainment to a musical beat. We show for the first time that a heterogeneous group of cochlear implant users is able to find the beat and move their bodies in time to Latin Merengue music, especially when the music is presented in unpitched drum tones. These findings not only reveal a hidden capacity for feeling musical rhythm through the body in the deaf and hearing impaired population, but illuminate promising avenues for designing early childhood musical training that can engage implanted children in social musical activities with benefits potentially extending to non-musical domains. Copyright © 2015 Elsevier B.V. All rights reserved.

Comparison of phonological awareness between children with cochlear implants and children with hearing aids

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Farzad Weisi

2013-02-01

Full Text Available Introduction: Advanced phonological skills are important for the acquisition of reading skills. Children with hearing impairment have reading skills are weaker than others because of auditory inputs and due to the defect in phonological skills. The use of hearing aids and cochlear implants help to collect information on people who are hard of hearing.Material and Methods: This descriptive - analytic study was done on 12 children with cochlear implant and 12 children with hearing aids that was selected from second grades students of Tehran primary schools. Children's phonological performance was assessed by phonological subtests of Nama reading test and the data were analyzed using SPSS 16.Results: The results showed that the means of scores of children with cochlear implants in Rhyme task were significantly greater than the children with hearing aids (P=0.034. But in means of scores of Phone deletion and Nonword reading tasks were not significant different between two groups (P=0.919, P=0.670.Discussion: Cochlear implant with accessibility auditory inputs can facilitated the acquisition of phonological awareness skills in hearing loss children. But whereas the other language inputs such as sight and touch input helped to developing these skills, children with hearing aids too also can acquisition these skills.

Serotonin projection patterns to the cochlear nucleus.

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Thompson, A M; Thompson, G C

2001-07-13

The cochlear nucleus is well known as an obligatory relay center for primary auditory nerve fibers. Perhaps not so well known is the neural input to the cochlear nucleus from cells containing serotonin that reside near the midline in the midbrain raphe region. Although the specific locations of the main, if not sole, sources of serotonin within the dorsal cochlear nucleus subdivision are known to be the dorsal and median raphe nuclei, sources of serotonin located within other cochlear nucleus subdivisions are not currently known. Anterograde tract tracing was used to label fibers originating from the dorsal and median raphe nuclei while fluorescence immunohistochemistry was used to simultaneously label specific serotonin fibers in cat. Biotinylated dextran amine was injected into the dorsal and median raphe nuclei and was visualized with Texas Red, while serotonin was visualized with fluorescein. Thus, double-labeled fibers were unequivocally identified as serotoninergic and originating from one of the labeled neurons within the dorsal and median raphe nuclei. Double-labeled fiber segments, typically of fine caliber with oval varicosities, were observed in many areas of the cochlear nucleus. They were found in the molecular layer of the dorsal cochlear nucleus, in the small cell cap region, and in the granule cell and external regions of the cochlear nuclei, bilaterally, of all cats. However, the density of these double-labeled fiber segments varied considerably depending upon the exact region in which they were found. Fiber segments were most dense in the dorsal cochlear nucleus (especially in the molecular layer) and the large spherical cell area of the anteroventral cochlear nucleus; they were moderately dense in the small cell cap region; and fiber segments were least dense in the octopus and multipolar cell regions of the posteroventral cochlear nucleus. Because of the presence of labeled fiber segments in subdivisions of the cochlear nucleus other than the

Elevated 20-HETE impairs coronary collateral growth in metabolic syndrome via endothelial dysfunction.

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Joseph, Gregory; Soler, Amanda; Hutcheson, Rebecca; Hunter, Ian; Bradford, Chastity; Hutcheson, Brenda; Gotlinger, Katherine H; Jiang, Houli; Falck, John R; Proctor, Spencer; Schwartzman, Michal Laniado; Rocic, Petra

2017-03-01

Coronary collateral growth (CCG) is impaired in metabolic syndrome (MetS). microRNA-145 (miR-145-Adv) delivery to our rat model of MetS (JCR) completely restored and neutrophil depletion significantly improved CCG. We determined whether low endogenous levels of miR-145 in MetS allowed for elevated production of 20-hydroxyeicosatetraenoic acid (20-HETE), which, in turn, resulted in excessive neutrophil accumulation and endothelial dysfunction leading to impaired CCG. Rats underwent 0-9 days of repetitive ischemia (RI). RI-induced cardiac CYP4F (neutrophil-specific 20-HETE synthase) expression and 20-HETE levels were increased (4-fold) in JCR vs. normal rats. miR-145-Adv and 20-HETE antagonists abolished and neutrophil depletion (blocking antibodies) reduced (~60%) RI-induced increases in CYP4F expression and 20-HETE production in JCR rats. Impaired CCG in JCR rats (collateral-dependent blood flow using microspheres) was completely restored by 20-HETE antagonists [collateral-dependent zone (CZ)/normal zone (NZ) flow ratio was 0.76 ± 0.07 in JCR + 20-SOLA, 0.84 ± 0.05 in JCR + 20-HEDGE vs. 0.11 ± 0.02 in JCR vs. 0.84 ± 0.03 in normal rats]. In JCR rats, elevated 20-HETE was associated with excessive expression of endothelial adhesion molecules and neutrophil infiltration, which were reversed by miR-145-Adv. Endothelium-dependent vasodilation of coronary arteries, endothelial nitric oxide synthase (eNOS) Ser1179 phosphorylation, eNOS-dependent NO ·- production and endothelial cell survival were compromised in JCR rats. These parameters of endothelial dysfunction were completely reversed by 20-HETE antagonism or miR-145-Adv delivery, whereas neutrophil depletion resulted in partial reversal (~70%). We conclude that low miR-145 in MetS allows for increased 20-HETE, mainly from neutrophils, which compromises endothelial cell survival and function leading to impaired CCG. 20-HETE antagonists could provide viable therapy for restoration of CCG in MetS. NEW & NOTEWORTHY

Does Differential Visual Exploration Contribute to Visual Memory Impairments in 22Q11.2 Microdeletion Syndrome?

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Bostelmann, M.; Glaser, B.; Zaharia, A.; Eliez, S.; Schneider, M.

2017-01-01

Background: Chromosome 22q11.2 microdeletion syndrome (22q11.2DS) is a genetic syndrome characterised by a unique cognitive profile. Individuals with the syndrome present several non-verbal deficits, including visual memory impairments and atypical exploration of visual information. In this study, we seek to understand how visual attention may…

Noise-induced cochlear synaptopathy in rhesus monkeys (Macaca mulatta).

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Valero, M D; Burton, J A; Hauser, S N; Hackett, T A; Ramachandran, R; Liberman, M C

2017-09-01

Cochlear synaptopathy can result from various insults, including acoustic trauma, aging, ototoxicity, or chronic conductive hearing loss. For example, moderate noise exposure in mice can destroy up to ∼50% of synapses between auditory nerve fibers (ANFs) and inner hair cells (IHCs) without affecting outer hair cells (OHCs) or thresholds, because the synaptopathy occurs first in high-threshold ANFs. However, the fiber loss likely impairs temporal processing and hearing-in-noise, a classic complaint of those with sensorineural hearing loss. Non-human primates appear to be less vulnerable to noise-induced hair-cell loss than rodents, but their susceptibility to synaptopathy has not been studied. Because establishing a non-human primate model may be important in the development of diagnostics and therapeutics, we examined cochlear innervation and the damaging effects of acoustic overexposure in young adult rhesus macaques. Anesthetized animals were exposed bilaterally to narrow-band noise centered at 2 kHz at various sound-pressure levels for 4 h. Cochlear function was assayed for up to 8 weeks following exposure via auditory brainstem responses (ABRs) and otoacoustic emissions (OAEs). A moderate loss of synaptic connections (mean of 12-27% in the basal half of the cochlea) followed temporary threshold shifts (TTS), despite minimal hair-cell loss. A dramatic loss of synapses (mean of 50-75% in the basal half of the cochlea) was seen on IHCs surviving noise exposures that produced permanent threshold shifts (PTS) and widespread hair-cell loss. Higher noise levels were required to produce PTS in macaques compared to rodents, suggesting that primates are less vulnerable to hair-cell loss. However, the phenomenon of noise-induced cochlear synaptopathy in primates is similar to that seen in rodents. Copyright © 2017 Elsevier B.V. All rights reserved.

Follow-up of cochlear implant use in patients who developed bacterial meningitis following cochlear implantation.

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Mancini, Patrizia; D'Elia, Chiara; Bosco, Ersilia; De Seta, Elio; Panebianco, Valeria; Vergari, Valeria; Filipo, Roberto

2008-08-01

The present study is a long-term follow-up of speech perception outcomes and cochlear implant use in three cases of meningitis that occurred after cochlear implantation. Case series study. Study was performed on three children implanted with different models of Clarion devices, two of them with positioner. Recognition and comprehension were assessed via the Italian adaptation of GASP (TAP) test, and phonetically balanced bi-syllabic words in open-set. High resolution computed tomography scan acquisition was performed to obtain axial coronal and oblique multiplanar reconstructions of the cochlea. Two patients were affected by enlarged cochlear acqueduct and Mondini malformation the first carrying positioner. One patient had a normal cochlea, and the positioner could have been the main cause of bacterial spread. As a consequence of meningitis the child with normal cochlea and the other with enlarged vestibular acqueduct developed cochlear ossification, increased M-level and worsening of hearing outcomes. The child with Mondini malformation developed facial nerve stimulation. Contralateral implantation was performed in the first two patients. Bacterial meningitis occurring after cochlear implantation may induce cochlear ossification, facial nerve stimulation, and permanent or temporary loss of implant use. Planned follow-up with high resolution computed tomography and evaluation of M-levels could be useful prognostic tools in the management of these patients.

Audiovisual segregation in cochlear implant users.

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Simon Landry

Full Text Available It has traditionally been assumed that cochlear implant users de facto perform atypically in audiovisual tasks. However, a recent study that combined an auditory task with visual distractors suggests that only those cochlear implant users that are not proficient at recognizing speech sounds might show abnormal audiovisual interactions. The present study aims at reinforcing this notion by investigating the audiovisual segregation abilities of cochlear implant users in a visual task with auditory distractors. Speechreading was assessed in two groups of cochlear implant users (proficient and non-proficient at sound recognition, as well as in normal controls. A visual speech recognition task (i.e. speechreading was administered either in silence or in combination with three types of auditory distractors: i noise ii reverse speech sound and iii non-altered speech sound. Cochlear implant users proficient at speech recognition performed like normal controls in all conditions, whereas non-proficient users showed significantly different audiovisual segregation patterns in both speech conditions. These results confirm that normal-like audiovisual segregation is possible in highly skilled cochlear implant users and, consequently, that proficient and non-proficient CI users cannot be lumped into a single group. This important feature must be taken into account in further studies of audiovisual interactions in cochlear implant users.

Outcomes of Late Implantation in Usher Syndrome Patients.

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Hoshino, Ana Cristina H; Echegoyen, Agustina; Goffi-Gomez, Maria Valéria Schmidt; Tsuji, Robinson Koji; Bento, Ricardo Ferreira

2017-04-01

Introduction  Usher syndrome (US) is an autosomal recessive disorder characterized by hearing loss and progressive visual impairment. Some deaf Usher syndrome patients learn to communicate using sign language. During adolescence, as they start losing vision, they are usually referred to cochlear implantation as a salvage for their new condition. Is a late implantation beneficial to these children? Objective  The objective of this study is to describe the outcomes of US patients who received cochlear implants at a later age. Methods  This is a retrospective study of ten patients diagnosed with US1. We collected pure-tone thresholds and speech perception tests from pre and one-year post implant. Results  Average age at implantation was 18.9 years (5-49). Aided average thresholds were 103 dB HL and 35 dB HL pre and one-year post implant, respectively. Speech perception was only possible to be measured in four patients preoperatively, who scored 13.3; 26.67; 46% vowels and 56% 4-choice. All patients except one had some kind of communication. Two were bilingual. After one year of using the device, seven patients were able to perform the speech tests (from four-choice to close set sentences) and three patients abandoned the use of the implant. Conclusion  We observed that detection of sounds can be achieved with late implantation, but speech recognition is only possible in patients with previous hearing stimulation, since it depends on the development of hearing skills and the maturation of the auditory pathways.

Children with Chromosome 22q11.2 Deletion Syndrome Exhibit Impaired Spatial Working Memory

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Wong, Ling M.; Riggins, Tracy; Harvey, Danielle; Cabaral, Margarita; Simon, Tony J.

2014-01-01

Individuals with chromosome 22q11.2 deletion syndrome (22q11.2DS) have been shown to have impairments in processing spatiotemporal information. The authors examined whether children with 22q11.2DS exhibit impairments in spatial working memory performance due to these weaknesses, even when controlling for maintenance of attention. Children with…

"COCHLEAR IMPLANTATION IN PATIENTS WITH INNER EAR MALFORMATIONS"

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P. Borghei S. Abdi

2004-08-01

Full Text Available Performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute. This study was designed to analyze the operative findings,complications, and postoperative performance of patients with inner ear anomalies who underwent cochlear implantation. Six patients with inner ear malformations underwent implantation in our academic tertiary referral center from 1997 to 2002. The average follow-up period was 27 months. Malformations included one incomplete partition, one common cavity, one narrow internal acoustic canal (IAC in a patient with Riley-Day syndrome and 3 cases of large vestibular aqueduct. All received multi-channel implants either Nucleus 22 or Clarion device. Facial nerve was anomalous in 2 cases. CSF gusher occurred in 4 patients, which was controlled with packing the cochleostomy site. In all cases, the full length of electrode array was inserted, except one with Mondini's dysplasia where insertion failed in the first operation and was referred to another center for a successful surgery on the opposite ear. No other surgical complications were encountered. In 4 cases, all the 22 electrodes could be activated. All patients showed improved hearing performance after implantation. Four showed open-set speech recognition. The one with narrow IAC showed improved awareness to environmental sounds. In the other case (common cavity, the perception tests could not be performed because of very young age. Cochlear implantation in patients with inner ear malformations is a successful way of rehabilitation, although complications should be expected and auditory responses may be highly variable and relatively moderate.

Reactive neurogenesis and down-regulation of the potassium-chloride cotransporter KCC2 in the cochlear nuclei after cochlear deafferentation

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Brahim Tighilet

2016-08-01

Full Text Available While many studies have been devoted to investigating the homeostatic plasticity triggered by cochlear hearing loss, the cellular and molecular mechanisms involved in these central changes remain elusive. In the present study, we investigated the possibility of reactive neurogenesis after unilateral cochlear nerve section in the cochlear nucleus of cats. We found a strong cell proliferation in all the cochlear nucleus sub-divisions ipsilateral to the lesion. Most of the newly generated cells survive up to one month after cochlear deafferentation in all cochlear nuclei (except the dorsal cochlear nucleus and give rise to a variety of cell types, i.e. microglial cells, astrocytes and neurons. Interestingly, many of the newborn neurons had an inhibitory (GABAergic phenotype. This result is intriguing since sensory deafferentation is usually accompanied by enhanced excitation, consistent with a reduction in central inhibition. The membrane potential effect of GABA depends, however, on the intra-cellular chloride concentration, which is maintained at low levels in adults by the potassium chloride co-transporter KCC2. The KCC2 density on the plasma membrane of neurons was then assessed after cochlear deafferentation in the cochlear nuclei ipsilateral and contralateral to the lesion. Cochlear deafferentation is accompanied by a strong down-regulation of KCC2 ipsilateral to the lesion at 3 and 30 days post-lesion. This study suggests that reactive neurogenesis and downregulation of KCC2 is part of the vast repertoire involved in homeostatic plasticity triggered by hearing loss. These central changes may also play a role in the generation of tinnitus and hyperacusis.

Cochlear implants and medical tourism.

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McKinnon, Brian J; Bhatt, Nishant

2010-09-01

To compare the costs of medical tourism in cochlear implant surgery performed in India as compared to the United States. In addition, the cost savings of obtaining cochlear implant surgery in India were compare d to those of other surgical interventions obtained as a medical tourist. Searches were conducted on Medline and Google using the search terms: 'medical tourism', 'medical offshoring', 'medical outsourcing', 'cochlear implants' and 'cochlear implantation'. The information regarding cost of medical treatment was obtained from personal communication with individuals familiar with India's cochlear implantation medical tourism industry. The range of cost depended on length of stay as well as the device chosen. Generally the cost, inclusive of travel, surgery and device, was in the range of $21,000-30,000, as compared to a cost range of $40,000-$60,000 in the US. With the escalating cost of healthcare in the United States, it is not surprising that some patients would seek to obtain surgical care overseas at a fraction of the cost. Participants in medical tourism often have financial resources, but lack health insurance coverage. While cardiovascular and orthopedic surgery performed outside the United States in India at centers that cater to medical tourists are often performed at one-quarter to one-third of the cost that would have been paid in the United States, the cost differential for cochlear implants is not nearly as favorable.

Visual impairment in children with congenital Zika syndrome.

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Ventura, Liana O; Ventura, Camila V; Lawrence, Linda; van der Linden, Vanessa; van der Linden, Ana; Gois, Adriana L; Cavalcanti, Milena M; Barros, Eveline A; Dias, Natalia C; Berrocal, Audina M; Miller, Marilyn T

2017-08-01

To describe the visual impairment associated with ocular and neurological abnormalities in a cohort of children with congenital Zika syndrome (CZS). This cross-sectional study included infants with microcephaly born in Pernambuco, Brazil, from May to December 2015. Immunoglobulin M antibody capture enzyme-linked immunosorbent assay for the Zika virus on the cerebrospinal fluid samples was positive for all infants. Clinical evaluation consisted of comprehensive ophthalmologic examination including visual acuity, visual function assessment, visual developmental milestone, neurologic examination, and neuroimaging. A total of 32 infants (18 males [56%]) were included. Mean age at examination was 5.7 ± 0.9 months (range, 4-7 months). Visual function and visual developmental milestone could not be tested in 1 child (3%). Visual impairment was detected in 32 infants (100%). Retinal and/or optic nerve findings were observed in 14 patients (44%). There was no statistical difference between the patients with ocular findings and those without (P = 0.180). All patients (100%) demonstrated neurological and neuroimaging abnormalities; 3 (9%) presented with late-onset of microcephaly. Children with CZS demonstrated visual impairment regardless of retina and/or optic nerve abnormalities. This finding suggests that cortical/cerebral visual impairment may be the most common cause of blindness identified in children with CZS. Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

Processing of Binaural Pitch Stimuli in Hearing-Impaired Listeners

DEFF Research Database (Denmark)

Santurette, Sébastien; Dau, Torsten

2009-01-01

Binaural pitch is a tonal sensation produced by introducing a frequency-dependent interaural phase shift in binaurally presented white noise. As no spectral cues are present in the physical stimulus, binaural pitch perception is assumed to rely on accurate temporal fine structure coding and intact...... binaural integration mechanisms. This study investigated to what extent basic auditory measures of binaural processing as well as cognitive abilities are correlated with the ability of hearing-impaired listeners to perceive binaural pitch. Subjects from three groups (1: normal-hearing; 2: cochlear...... hearingloss; 3: retro-cochlear impairment) were asked to identify the pitch contour of series of five notes of equal duration, ranging from 523 to 784 Hz, played either with Huggins’ binaural pitch stimuli (BP) or perceptually similar, but monaurally detectable, pitches (MP). All subjects from groups 1 and 2...

Comparison of Social Interaction between Cochlear-Implanted Children with Normal Intelligence Undergoing Auditory Verbal Therapy and Normal-Hearing Children: A Pilot Study.

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Monshizadeh, Leila; Vameghi, Roshanak; Sajedi, Firoozeh; Yadegari, Fariba; Hashemi, Seyed Basir; Kirchem, Petra; Kasbi, Fatemeh

2018-04-01

A cochlear implant is a device that helps hearing-impaired children by transmitting sound signals to the brain and helping them improve their speech, language, and social interaction. Although various studies have investigated the different aspects of speech perception and language acquisition in cochlear-implanted children, little is known about their social skills, particularly Persian-speaking cochlear-implanted children. Considering the growing number of cochlear implants being performed in Iran and the increasing importance of developing near-normal social skills as one of the ultimate goals of cochlear implantation, this study was performed to compare the social interaction between Iranian cochlear-implanted children who have undergone rehabilitation (auditory verbal therapy) after surgery and normal-hearing children. This descriptive-analytical study compared the social interaction level of 30 children with normal hearing and 30 with cochlear implants who were conveniently selected. The Raven test was administered to the both groups to ensure normal intelligence quotient. The social interaction status of both groups was evaluated using the Vineland Adaptive Behavior Scale, and statistical analysis was performed using Statistical Package for Social Sciences (SPSS) version 21. After controlling age as a covariate variable, no significant difference was observed between the social interaction scores of both the groups (p > 0.05). In addition, social interaction had no correlation with sex in either group. Cochlear implantation followed by auditory verbal rehabilitation helps children with sensorineural hearing loss to have normal social interactions, regardless of their sex.

Prediction of consonant recognition in quiet for listeners with normal and impaired hearing using an auditory model.

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Jürgens, Tim; Ewert, Stephan D; Kollmeier, Birger; Brand, Thomas

2014-03-01

Consonant recognition was assessed in normal-hearing (NH) and hearing-impaired (HI) listeners in quiet as a function of speech level using a nonsense logatome test. Average recognition scores were analyzed and compared to recognition scores of a speech recognition model. In contrast to commonly used spectral speech recognition models operating on long-term spectra, a "microscopic" model operating in the time domain was used. Variations of the model (accounting for hearing impairment) and different model parameters (reflecting cochlear compression) were tested. Using these model variations this study examined whether speech recognition performance in quiet is affected by changes in cochlear compression, namely, a linearization, which is often observed in HI listeners. Consonant recognition scores for HI listeners were poorer than for NH listeners. The model accurately predicted the speech reception thresholds of the NH and most HI listeners. A partial linearization of the cochlear compression in the auditory model, while keeping audibility constant, produced higher recognition scores and improved the prediction accuracy. However, including listener-specific information about the exact form of the cochlear compression did not improve the prediction further.

Impairment of circulating endothelial progenitors in Down syndrome

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Costa Valerio

2010-09-01

Full Text Available Abstract Background Pathological angiogenesis represents a critical issue in the progression of many diseases. Down syndrome is postulated to be a systemic anti-angiogenesis disease model, possibly due to increased expression of anti-angiogenic regulators on chromosome 21. The aim of our study was to elucidate some features of circulating endothelial progenitor cells in the context of this syndrome. Methods Circulating endothelial progenitors of Down syndrome affected individuals were isolated, in vitro cultured and analyzed by confocal and transmission electron microscopy. ELISA was performed to measure SDF-1α plasma levels in Down syndrome and euploid individuals. Moreover, qRT-PCR was used to quantify expression levels of CXCL12 gene and of its receptor in progenitor cells. The functional impairment of Down progenitors was evaluated through their susceptibility to hydroperoxide-induced oxidative stress with BODIPY assay and the major vulnerability to the infection with human pathogens. The differential expression of crucial genes in Down progenitor cells was evaluated by microarray analysis. Results We detected a marked decrease of progenitors' number in young Down individuals compared to euploid, cell size increase and some major detrimental morphological changes. Moreover, Down syndrome patients also exhibited decreased SDF-1α plasma levels and their progenitors had a reduced expression of SDF-1α encoding gene and of its membrane receptor. We further demonstrated that their progenitor cells are more susceptible to hydroperoxide-induced oxidative stress and infection with Bartonella henselae. Further, we observed that most of the differentially expressed genes belong to angiogenesis, immune response and inflammation pathways, and that infected progenitors with trisomy 21 have a more pronounced perturbation of immune response genes than infected euploid cells. Conclusions Our data provide evidences for a reduced number and altered

Estrogen and hearing from a clinical point of view; characteristics of auditory function in women with Turner syndrome.

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Hederstierna, Christina; Hultcrantz, Malou; Rosenhall, Ulf

2009-06-01

Turner syndrome is a chromosomal aberration affecting 1:2000 newborn girls, in which all or part of one X chromosome is absent. This leads to ovarial dysgenesis and little or no endogenous estrogen production. These women have, among many other syndromal features, a high occurrence of ear and hearing problems, and neurocognitive dysfunctions, including reduced visual-spatial abilities; it is assumed that estrogen deficiency is at least partially responsible for these problems. In this, study 30 Turner women aged 40-67, with mild to moderate hearing loss, performed a battery of hearing tests aimed at localizing the lesion causing the sensorineural hearing impairment and assessing central auditory function, primarily sound localization. The results of TEOAE, ABR and speech recognition scores in noise were all indicative of cochlear dysfunction as the cause of the sensorineural impairment. Phase audiometry, a test for sound localization, showed mild disturbances in the Turner women compared to the reference group, suggesting that auditory-spatial dysfunction is another facet of the recognized neurocognitive phenotype in Turner women.

Parental expectations and outcomes of pediatric cochlear implantation.

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Piazza, Elizabeth; Kandathil, Cherian; Carron, Jeffrey D

2009-10-01

Cochlear implants have been used with increasing frequency over the past twenty years, including very young patients. To determine if parents are satisfied with their children's performance after cochlear implantation. Survey mailed to parents of children receiving cochlear implants. 31 questionnaires were returned out of 69 mailed (45 %). The vast majority of responding parents felt that their children benefited substantially from cochlear implant surgery. Cochlear implantation is effective in helping children develop auditory-oral communication skills. Access to auditory/oral communication programs in this state remains an obstacle in postoperative habilitation.

Bilateral Cochlear Implants: Maximizing Expected Outcomes.

Science.gov (United States)

Wallis, Kate E; Blum, Nathan J; Waryasz, Stephanie A; Augustyn, Marilyn

Sonia is a 4 years 1 month-year-old girl with Waardenburg syndrome and bilateral sensorineural hearing loss who had bilateral cochlear implants at 2 years 7 months years of age. She is referred to Developmental-Behavioral Pediatrics by her speech/language pathologist because of concerns that her language skills are not progressing as expected after the cochlear implant. At the time of the implant, she communicated using approximately 20 signs and 1 spoken word (mama). At the time of the evaluation (18 months after the implant) she had approximately 70 spoken words (English and Spanish) and innumerable signs that she used to communicate. She could follow 1-step directions in English but had more difficulty after 2-step directions.Sonia was born in Puerto Rico at 40 weeks gestation after an uncomplicated pregnancy. She failed her newborn hearing test and was given hearing aids that did not seem to help.At age 2 years, Sonia, her mother, and younger sister moved to the United States where she was diagnosed with bilateral severe-to-profound hearing loss. Genetic testing led to a diagnosis of Waardenburg syndrome (group of genetic conditions that can cause hearing loss and changes in coloring [pigmentation] of the hair, skin, and eyes). She received bilateral cochlear implants 6 months later.Sonia's mother is primarily Spanish-speaking and mostly communicates with her in Spanish or with gestures but has recently begun to learn American Sign Language (ASL). In a preschool program at a specialized school for the deaf, Sonia is learning both English and ASL. Sonia seems to prefer to use ASL to communicate.Sonia receives speech and language therapy (SLT) 3 times per week (90 minutes total) individually in school and once per week within a group. She is also receiving outpatient SLT once per week. Therapy sessions are completed in English, with the aid of an ASL interpreter. Sonia's language scores remain low, with her receptive skills in the first percentile, and her

Beyond Thiamine: Treatment for Cognitive Impairment in Korsakoff's Syndrome.

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Johnson, Justin M; Fox, Valerie

2018-03-27

Wernicke's encephalopathy is a condition whose treatment many consultation-liaison psychiatrists know quite well. Less clear, however, is the treatment of its dementia disorder descendent, the Korsakoff's syndrome (KS). This article seeks to review treatment options and provide recommendations for consultation-liaison psychiatrists treating cognitive impairment in KS. In this nonsystematic review, we reviewed PubMed, CINAHL Plus, and Google Scholar for published reports and studies regarding treatment of KS. The literature revealed case reports and placebo-controlled trials of various medications for treatment of KS, though the samples sizes were small and were mostly case reports. There is more attention devoted toward medications used in other dementia disorders, such as donepezil and memantine. The literature revealed more studies around behavioral interventions recommended for treatment of memory impairment in KS and they focused on cognitive remediation and environmental adaptation, such as the use of PDAs or alarms. There is no single, well-studied intervention proven effective as a primary treatment for cognitive impairment in KS. An approach of using environmental modifications in a well-structured living environment, combined with various cognitive interventions, such as pictorial associations, and perhaps a trial of donepezil or memantine, likely represents the best strategy for treating long-term cognitive impairment in KS. Published by Elsevier Inc.

[Meta-analysis of the efficacy of cochlear implantation in deaf patients with inner ear malformation].

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Xu, Qingqing; Zhai, Suoqiang; Han, Dongyi; Yang, Shiming; Shen, Weidong

2014-04-01

Assess and compare the clinical efficacy and safety of cochlear implantation in deaf patients with inner ear malformation and in the ones with normal inner ear structure, so that to clarify whether it is effective to restore hearing for the deaf patients with inner ear malformation. The literature with relevant key words were retrieved in the databases including PubMed, YZ365. com, WANFANG data, CMJD, CHKD and CNKI with language limited to Chinese and English. Extracted data included author, year of publication, diagnosis, treatment, et al. The clinical efficacy of cochlear implantation was assessed by the complications, electrode impedance, behavior T-level, hearing abilities and speech discrimination. Meta-analysis was performed using random or fixed effects model according to the heterogeneity of data. There were 11 randomized control studies involving 655 patients included in this study. There was no statistically significant difference among the deaf patients in mixed inner ear-malformation group, Mondini group and large vestibular aqueduct syndrome group in the aspects of postoperative complications, electrode impedance, behavior T-level, hearing abilities and speech discrimination. Cochlear implantation could be the way of treatment and rehabilitation for deaf patients with inner ear malformation. Further controlled studies with longer follow-up periods and more multiracial cases included may help to evaluate the efficacy of cochlear implantation for deaf patients with inner ear malformation more reliably.

The Visual Impairment Intracranial Pressure Syndrome in Long Duration NASA Astronauts: An Integrated Approach

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Otto, C. A.; Norsk, P.; Shelhamer, M. J.; Davis, J. R.

2015-01-01

The Visual Impairment Intracranial Pressure (VIIP) syndrome is currently NASA's number one human space flight risk. The syndrome, which is related to microgravity exposure, manifests with changes in visual acuity (hyperopic shifts, scotomas), changes in eye structure (optic disc edema, choroidal folds, cotton wool spots, globe flattening, and distended optic nerve sheaths). In some cases, elevated cerebrospinal fluid pressure has been documented postflight reflecting increased intracranial pressure (ICP). While the eye appears to be the main affected end organ of this syndrome, the ocular affects are thought to be related to the effect of cephalad fluid shift on the vascular system and the central nervous system. The leading hypotheses for the development of VIIP involve microgravity induced head-ward fluid shifts along with a loss of gravity-assisted drainage of venous blood from the brain, both leading to cephalic congestion and increased ICP. Although not all crewmembers have manifested clinical signs or symptoms of the VIIP syndrome, it is assumed that all astronauts exposed to microgravity have some degree of ICP elevation in-flight. Prolonged elevations of ICP can cause long-term reduced visual acuity and loss of peripheral visual fields, and has been reported to cause mild cognitive impairment in the analog terrestrial population of Idiopathic Intracranial Hypertension (IIH). These potentially irreversible health consequences underscore the importance of identifying the factors that lead to this syndrome and mitigating them.

Impaired perception of temporal fine structure and musical timbre in cochlear implant users.

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Heng, Joseph; Cantarero, Gabriela; Elhilali, Mounya; Limb, Charles J

2011-10-01

Cochlear implant (CI) users demonstrate severe limitations in perceiving musical timbre, a psychoacoustic feature of sound responsible for 'tone color' and one's ability to identify a musical instrument. The reasons for this limitation remain poorly understood. In this study, we sought to examine the relative contributions of temporal envelope and fine structure for timbre judgments, in light of the fact that speech processing strategies employed by CI systems typically employ envelope extraction algorithms. We synthesized "instrumental chimeras" that systematically combined variable amounts of envelope and fine structure in 25% increments from two different source instruments with either sustained or percussive envelopes. CI users and normal hearing (NH) subjects were presented with 150 chimeras and asked to determine which instrument the chimera more closely resembled in a single-interval two-alternative forced choice task. By combining instruments with similar and dissimilar envelopes, we controlled the valence of envelope for timbre identification and compensated for envelope reconstruction from fine structure information. Our results show that NH subjects utilize envelope and fine structure interchangeably, whereas CI subjects demonstrate overwhelming reliance on temporal envelope. When chimeras were created from dissimilar envelope instrument pairs, NH subjects utilized a combination of envelope (p = 0.008) and fine structure information (p = 0.009) to make timbre judgments. In contrast, CI users utilized envelope information almost exclusively to make timbre judgments (p < 0.001) and ignored fine structure information (p = 0.908). Interestingly, when the value of envelope as a cue was reduced, both NH subjects and CI users utilized fine structure information to make timbre judgments (p < 0.001), although the effect was quite weak in CI users. Our findings confirm that impairments in fine structure processing underlie poor perception of musical timbre in CI

The cochlear nerve canal and internal auditory canal in children with normal cochlea but cochlear nerve deficiency

International Nuclear Information System (INIS)

Yan, Fei; Li, Jianhong; Xian, Junfang; Wang, Zhenchang; Mo, Lingyan

2013-01-01

Background: There is an increasing frequency of requests for cochlear implantation (CI) in deaf children and more detailed image information is necessary for selecting appropriate candidates. Cochlear nerve deficiency (CND) is a contraindication to CI. Magnetic resonance imaging (MRI) has been used to evaluate the integrity of the cochlear nerve. The abnormalities of the cochlear nerve canal (CNC) and internal auditory canal (IAC) have been reported to be associated with CND. Purpose: To correlate CNC manifestation, size, and IAC diameter on high-resolution CT (HRCT) with CND diagnosed by MRI in children. Material and Methods: HRCT images from 35 sensorineurally deaf children who had normal cochlea but bilateral or unilateral CND diagnosed by MRI were studied retrospectively. The CNC and IAC manifestation and size were assessed and correlated with CND. Results: CND was diagnosed by MRI in 54/70 ears (77.1%). Thirty-two ears had an absent cochlear nerve (59.3%), while 22 ears had a small cochlear nerve (40.7%). The CNC diameter was 2.0 mm in 11 ears (20.4%). The IAC diameter was 3.0 mm in 29 ears (53.7%). Conclusion: The hypoplastic CNC might be more highly indicative of CND than that of a narrow IAC

Communication between cochlear perilymph and cerebrospinal fluid through the cochlear modiolus visualized after intratympanic administration of Gd-DTPA

International Nuclear Information System (INIS)

Naganawa, Shinji; Satake, Hiroko; Iwano, Shingo; Sone, Michihiko; Nakashima, Tsutomu

2008-01-01

Intratympanic injection of gadolinium diethylenetriaminepentaacetic acid (Gd-DTPA) has been reported as a procedure to visualize endolymphatic hydrops of Meniere's disease. We frequently noted that cerebrospinal fluid (CSF) in the internal auditory canal (IAC) was also enhanced after this procedure. The purpose of this study was to evaluate how frequently this occurs and to investigate the specific features of patients who lack this communication. A total of 25 patients with clinically suspected endolymphatic hydrops underwent the procedure. After 24 h, three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) and 3D constructive interference in steady state (3D-CISS) were performed. The presence of contrast enhancement in the CSF space of the fundus of the IAC was evaluated. The contrast ratio between CSF of the IAC fundus and cerebellar white matter on the injected side was 1.49±0.65, and that of the noninjected side was 0.32±0.16 (P<0.01). Enhancement of the CSF space in the IAC fundus was seen in all but two subjects: one had enlarged endolymphatic duct and sac syndrome (EEDS), and the other had cochlear nerve agenesis. In these two patients, the cochlear modiolus seemed to be normal. Intratympanic Gd-DTPA administration can reveal permeability of the modiolus and might facilitate evaluation of functional abnormalities of the modiolus not detected by conventional imaging tests. (author)

[Cochlear implantation through the middle fossa approach].

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Szyfter, W; Colletti, V; Pruszewicz, A; Kopeć, T; Szymiec, E; Kawczyński, M; Karlik, M

2001-01-01

The inner part of cochlear implant is inserted into inner ear during surgery through mastoid and middle ear. It is a classical method, used in the majority cochlear centers in the world. This is not a suitable method in case of chronic otitis media and middle ear malformation. In these cases Colletti proposed the middle fossa approach and cochlear implant insertion omitting middle ear structures. In patient with bilateral chronic otitis media underwent a few ears operations without obtaining dry postoperative cavity. Cochlear implantation through the middle fossa approach was performed in this patient. The bone fenster was cut, temporal lobe was bent and petrosus pyramid upper surface was exposed. When the superficial petrosal greater nerve, facial nerve and arcuate eminence were localised, the cochlear was open in the basal turn and electrode were inserted. The patient achieves good results in the postoperative speech rehabilitation. It confirmed Colletti tesis that deeper electrode insertion in the cochlear implantation through the middle fossa approach enable use of low and middle frequencies, which are very important in speech understanding.

[Multi-channel cochlear implants in patients with Mondini malformation].

Science.gov (United States)

Li, Yong-xin; Han, De-min; Zhao, Xiao-tian; Chen, Xue-qing; Kong, Ying; Zheng, Jun; Liu, Bo; Liu, Sha; Mo, Ling-yan; Zhang, Hua; Wang, Shuo

2004-02-01

To describe clinical experiences with multi-channel cochlear implantation in patients with Mondini malformation. Among 300 patients who received multi-channel cochlear implants from 1996 to 2002 in Beijing Tongren Hospital, 15 patients were diagnosed with Mondini malformation. A retrospective analysis was performed dealing with the surgical techniques, mapping and rehabilitations characteristics after surgery. 15 patients with normal cochlear structure are consider as control group. Gusher is found more common than the normal cochlear implantation, most of them are serious. The electrodes are inserted in the "cochleostomy" in full length of 13 Patients, 2 pairs of electrodes remains outside of "cochleostomy" in 2 patients. No serious complications occurred after implantation. All patients have auditory sensations. The impedance of the electrodes, the T level, C level and the hearing threshold are similar with the normal cochlear implantation group. The results have no significant difference in compare with normal cochlear group(P > 0.05). Multi-channel cochlear implantation could be performed safely in patients with Mondini malformation. The primary outcome for patients with Mondini malformation are similar to those with normal cochlear structure following the multi-channel cochlear implantation.

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.

Enhanced MRI in patients with Ramsay-Hunt's syndrome

International Nuclear Information System (INIS)

Yanagida, Masahiro; Ushiro, Koichi; Yamashita, Toshio; Kumazawa, Tadami; Katoh, Tsutomu

1993-01-01

Enhanced MRI was performed in 14 patients with Ramsay-Hunt,s syndrome to investigate the pathogenesis of this syndrome. All MRI studies were performed on a 0.5T superconductivity MRI system using a head coil with Gd-DTPA. Enhancement was observed in the areas of the distal internal auditory canal and labyrinthine segment in many patients, and was especially prominent in patients suffering from vertigo, tinnitus, and hearing loss. In some patients it involved not only the facial nerve of the internal auditory canal but also the cochlear nerve and vestibular nerves. Since histological changes of the facial nerve in patients with Ramsay-Hunt's syndrome are assumed to occur in the distal internal auditory canal and labyrinthine segment, which is more proximal than the geniculate ganglion, and the possibility is suggested that inflammation may be spread to the vestibular and cochlear nerve via the internal auditory canal. (14 refs., 2 figs.)

Enhanced MRI in patients with Ramsay-Hunt's syndrome

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Yanagida, Masahiro; Ushiro, Koichi; Yamashita, Toshio; Kumazawa, Tadami [Kansai Medical Univ., Osaka (Japan). Dept. of Otolaryngology; Katoh, Tsutomu [Kansai Medical Univ., Osaka (Japan). Dept. of Radiology

1993-01-01

Enhanced MRI was performed in 14 patients with Ramsay-Hunt,s syndrome to investigate the pathogenesis of this syndrome. All MRI studies were performed on a 0.5T superconductivity MRI system using a head coil with Gd-DTPA. Enhancement was observed in the areas of the distal internal auditory canal and labyrinthine segment in many patients, and was especially prominent in patients suffering from vertigo, tinnitus, and hearing loss. In some patients it involved not only the facial nerve of the internal auditory canal but also the cochlear nerve and vestibular nerves. Since histological changes of the facial nerve in patients with Ramsay-Hunt's syndrome are assumed to occur in the distal internal auditory canal and labyrinthine segment, which is more proximal than the geniculate ganglion, and the possibility is suggested that inflammation may be spread to the vestibular and cochlear nerve via the internal auditory canal. (14 refs., 2 figs.).

Cochlear implantation in a bilateral Mondini dysplasia.

Science.gov (United States)

Turrini, M; Orzan, E; Gabana, M; Genovese, E; Arslan, E; Fisch, U

1997-01-01

We report the speech perception progress and programming procedures of a case of congenital profound deafness and bilateral Mondini dysplasia implanted with a Nucleus 20 + 2 cochlear implant at the age of six. Unclear relations between electrodes array and cochlear partition made implant programming difficult and non-standard procedures were set. Cochlear implantation may give excellent rehabilitative results also in cochleae with malformation.

Assessment of Early Cognitive Impairment in Patients with Clinically Isolated Syndromes and Multiple Sclerosis

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Leyla Baysal Kıraç

2014-01-01

Full Text Available Objective. The aim of our study was to investigate the frequency and pattern of cognitive impairment in patients with clinically isolated syndromes and definite diagnosis of multiple sclerosis within the last 2 years. Methods. We assessed the cognitive status of 46 patients aged 18–49 years with clinically isolated syndromes or definite diagnosis of multiple sclerosis who have onset of their symptoms within the last 2 years. Patients were matched with 40 healthy participants for age, sex, and educational level. Neuropsychological assessment was performed by stroop test, paced auditory serial addition test (PASAT, controlled oral word association test (COWAT, clock drawing test, trail making test (TMT, faces symbol test (FST. Hamilton Depression Scale and Modified Fatigue Impact Scale were used to quantify the severity of any depression and fatigue the subjects might suffer. Results. 19.6% of early MS/CIS group failed at 4 and more tests and had significant cognitive impairment focused on attention, executive functions, memory, and learning. No significant relationship was found between cognitive impairment and disability and fatigue scores. Discussion. Cognitive impairment can be present from the earliest stage of multiple sclerosis. It should be considered among the main manifestations of MS even in the earliest stages of the disease.

Aspects of temporal bone anatomy and pathology in conjunction with cochlear implant surgery

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Stjernholm, Christina [Karolinska Inst., Stockholm (Sweden). Soedersjukhuset

2003-07-01

Cochlear implantation is a treatment for patients with severe sensorineural hearing loss/deafness, who get no help from ordinary hearing aids. The cochlear implant is surgically placed under the skin near the ear and a very thin electrode array is introduced into the cochlea of the inner ear, where it stimulates the remaining nerve fibers. The operation is complicated; it is performed with the aid of a microscope, and involves drilling very close to vital vessels and important nerves. High resolution computed tomography (CT) of the temporal bone is a part of the preoperative evaluation preceding cochlear implantation. It is a method for visualizing the bony structures of the middle and inner ear - to diagnose pathology and to describe the anatomy. The first work concerns CT of the temporal bone and cochlear implant surgery in children with CHARGE association. This is a rare condition with multiple congenital abnormalities, sometimes lethal. Children with CHARGE have different combinations of disabilities, of which impairments of vision and hearing, as well as balance problems and facial palsy can lead to developmental delay. There have been few reports of radiological temporal bone changes and none of cochlear implant surgery for this group. The work includes a report of the findings on preoperative CT and at surgery, as well as postimplant results in two children. A review of the latest diagnostic criteria of CHARGE and the temporal bone changes found in international literature is also included. The conclusion was that certain combinations of temporal bone changes in CHARGE are, if not specific, at least extremely rare in other materials. CT can visualize these changes and be used as a diagnostic tool. This is important, since some of the associated disabilities are not so obvious from the start. Early treatment is vital for the child's development. This work also shows that cochlear implantation may help some of these often very isolated children to

Aspects of temporal bone anatomy and pathology in conjunction with cochlear implant surgery

International Nuclear Information System (INIS)

Stjernholm, Christina

2003-01-01

Cochlear implantation is a treatment for patients with severe sensorineural hearing loss/deafness, who get no help from ordinary hearing aids. The cochlear implant is surgically placed under the skin near the ear and a very thin electrode array is introduced into the cochlea of the inner ear, where it stimulates the remaining nerve fibers. The operation is complicated; it is performed with the aid of a microscope, and involves drilling very close to vital vessels and important nerves. High resolution computed tomography (CT) of the temporal bone is a part of the preoperative evaluation preceding cochlear implantation. It is a method for visualizing the bony structures of the middle and inner ear - to diagnose pathology and to describe the anatomy. The first work concerns CT of the temporal bone and cochlear implant surgery in children with CHARGE association. This is a rare condition with multiple congenital abnormalities, sometimes lethal. Children with CHARGE have different combinations of disabilities, of which impairments of vision and hearing, as well as balance problems and facial palsy can lead to developmental delay. There have been few reports of radiological temporal bone changes and none of cochlear implant surgery for this group. The work includes a report of the findings on preoperative CT and at surgery, as well as postimplant results in two children. A review of the latest diagnostic criteria of CHARGE and the temporal bone changes found in international literature is also included. The conclusion was that certain combinations of temporal bone changes in CHARGE are, if not specific, at least extremely rare in other materials. CT can visualize these changes and be used as a diagnostic tool. This is important, since some of the associated disabilities are not so obvious from the start. Early treatment is vital for the child's development. This work also shows that cochlear implantation may help some of these often very isolated children to communicate

Cochlear hearing loss in patients with Laron syndrome.

Science.gov (United States)

Attias, Joseph; Zarchi, Omer; Nageris, Ben I; Laron, Zvi

2012-02-01

The aim of this prospective clinical study was to test auditory function in patients with Laron syndrome, either untreated or treated with insulin-like growth factor I (IGF-I). The study group consisted of 11 patients with Laron syndrome: 5 untreated adults, 5 children and young adults treated with replacement IGF-I starting at bone age Laron syndrome and may be prevented by starting treatment with IGF-I at an early developmental age.

The Mass1frings mutation underlies early onset hearing impairment in BUB/BnJ mice, a model for the auditory pathology of Usher syndrome IIC

Science.gov (United States)

Johnson, K.R.; Zheng, Q.Y.; Weston, M.D.; Ptacek, L.J.; Noben-Trauth, K.

2010-01-01

The human ortholog of the gene responsible for audiogenic seizure susceptibility in Frings and BUB/BnJ mice (mouse gene symbol Mass1) recently was shown to underlie Usher syndrome type IIC (USH2C). Here we report that the Mass1frings mutation is responsible for the early onset hearing impairment of BUB/BnJ mice. We found highly significant linkage of Mass1 with ABR threshold variation among mice from two backcrosses involving BUB/BnJ mice with mice of strains CAST/EiJ and MOLD/RkJ. We also show an additive effect of the Cdh23 locus in modulating the progression of hearing loss in backcross mice. Together, these two loci account for more than 70% of the total ABR threshold variation among the backcross mice at all ages. The modifying effect of the strain-specific Cdh23ahl variant may account for the hearing and audiogenic seizure differences observed between Frings and BUB/BnJ mice, which share the Mass1frings mutation. During postnatal cochlear development in BUB/BnJ mice, stereocilia bundles develop abnormally and remain immature and splayed into adulthood, corresponding with the early onset hearing impairment associated with Mass1frings. Progressive base–apex hair cell degeneration occurs at older ages, corresponding with the age-related hearing loss associated with Cdh23ahl. The molecular basis and pathophysiology of hearing loss suggest BUB/BnJ and Frings mice as models to study cellular and molecular mechanisms underlying USH2C auditory pathology. PMID:15820310

Selective Vulnerability of the Cochlear Basal Turn to Acrylonitrile and Noise

International Nuclear Information System (INIS)

Pouyatos, B.; Gearhart, C.A.; Miller, A.N.; Fulton, S.; Fechter, L.D.; Pouyatos, B.

2009-01-01

Exposure to acrylonitrile, a high-production industrial chemical, can promote noise-induced hearing loss (NIHL) in the rat even though this agent does not itself produce permanent hearing loss. The mechanism by which acrylonitrile promotes NIHL includes oxidative stress as antioxidant drugs can partially protect the cochlea from acrylonitrile + noise. Acrylonitrile depletes glutathione levels while noise can increase the formation of reactive oxygen species. It was previously noted that the high-frequency or basal turn of the cochlea was particularly vulnerable to the combined effects of acrylonitrile and noise when the octave band noise (OBN) was centered at 8 k Hz. Normally, such a noise would be expected to yield damage at a more apical region of the cochlea. The present study was designed to determine whether the basal cochlea is selectively sensitive to acrylonitrile or whether, by adjusting the frequency of the noise band, it would be possible to control the region of the auditory impairment. Rats were exposed to one of three different OBNs centered at different frequencies (4 k Hz, 110 dB and 8 or 16 k Hz at 97 dB) for 5 days, with and without administration of acrylonitrile (50 mg/kg/day). The noise was set to cause limited NIHL by itself. Auditory function was monitored by recording distortion products, by compound action potentials, and by performing cochlear histology. While the ACN-only and noise-only exposures induced no or little permanent auditory loss, the three exposures to acrylonitrile + noise produced similar auditory and cochlear impairments above 16 k Hz, despite the fact that the noise exposures covered 2 octaves. These observations show that the basal cochlea is much more sensitive to acrylonitrile + noise than the apical partition. They provide an initial basis for distinguishing the pattern of cochlear injury that results from noise exposure from that which occurs due to the combined effects of noise and a chemical contaminant.

Congenital malformations of the ear and cochlear implantation in children: review and temporal bone report of common cavity.

Science.gov (United States)

Graham, J M; Phelps, P D; Michaels, L

2000-01-01

The objective of this review is to analyze aspects of congenital malformation of the ear in relation to cochlear implantation in children. Having briefly described the in utero development of the ear and the classification of types of external, middle and inner ear malformation, five practical aspects of these malformations are discussed. It seems likely that the combination of bilateral profound sensorineural deafness with bilateral microtia severe enough to make a surgical approach to the cochlea difficult will be extremely uncommon. No such cases have been reported, although Klippel-Feil deformity seems the syndrome most likely to produce this set of circumstances. Abnormalities in the intratympanic course of the facial nerve have been associated with cochlear malformation, emphasizing the benefit of intra-operative facial nerve monitoring, and a technique suggested for safely avoiding an abnormally placed nerve. Fistulae of cerebrospinal fluid (CSF) and perilymph can complicate surgery and are relatively common in common cavity and Mondini malformations. Strategies for facilitating surgery in the presence of 'gushers', for measuring the pressure of a gusher and for placement of the cochlear implant electrode array are reviewed, with reports of fluctuating levels of electric current when implants lie in dysplastic cochleas. The relationship of implant performance to VIIIth nerve tissue in malformed cochleas is discussed, with a description of the histological findings in a common cavity cochlea. Techniques for identifying the absence of the cochlear nerve are reviewed. Stimulation of the facial nerve by cochlear implants has been described in cases of congenital malformation of the labyrinth but is relatively uncommon. Case reports of the benefit received by implanted children with congenital cochlear malformation have appeared since 1988. Most cases reported have not yet been followed for long enough to establish a clear picture of the outcome following

Trends in Cochlear Implants

OpenAIRE

Zeng, Fan-Gang

2004-01-01

More than 60,000 people worldwide use cochlear implants as a means to restore functional hearing. Although individual performance variability is still high, an average implant user can talk on the phone in a quiet environment. Cochlear-implant research has also matured as a field, as evidenced by the exponential growth in both the patient population and scientific publication. The present report examines current issues related to audiologic, clinical, engineering, anatomic, and physiologic as...

Severe visual impairment and retinal changes in a boy with a deletion of the gene for Nance-Horan syndrome.

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Mathys, R; Deconinck, H; Keymolen, K; Jansen, A; Van Esch, H

2007-01-01

We present the ophthalmologic findings in a boy with a deletion of Xp22 comprising the gene for Nance-Horan syndrome. Different mechanisms underlying the visual impairment in Nance-Horan syndrome are discussed.

What Does Music Sound Like for a Cochlear Implant User?

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Jiam, Nicole T; Caldwell, Meredith T; Limb, Charles J

2017-09-01

Cochlear implant research and product development over the past 40 years have been heavily focused on speech comprehension with little emphasis on music listening and enjoyment. The relatively little understanding of how music sounds in a cochlear implant user stands in stark contrast to the overall degree of importance the public places on music and quality of life. The purpose of this article is to describe what music sounds like to cochlear implant users, using a combination of existing research studies and listener descriptions. We examined the published literature on music perception in cochlear implant users, particularly postlingual cochlear implant users, with an emphasis on the primary elements of music and recorded music. Additionally, we administered an informal survey to cochlear implant users to gather first-hand descriptions of music listening experience and satisfaction from the cochlear implant population. Limitations in cochlear implant technology lead to a music listening experience that is significantly distorted compared with that of normal hearing listeners. On the basis of many studies and sources, we describe how music is frequently perceived as out-of-tune, dissonant, indistinct, emotionless, and weak in bass frequencies, especially for postlingual cochlear implant users-which may in part explain why music enjoyment and participation levels are lower after implantation. Additionally, cochlear implant users report difficulty in specific musical contexts based on factors including but not limited to genre, presence of lyrics, timbres (woodwinds, brass, instrument families), and complexity of the perceived music. Future research and cochlear implant development should target these areas as parameters for improvement in cochlear implant-mediated music perception.

[The development of musicality in children after cochlear implantation].

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Zheng, Yan; Liu, Bo; Dong, Ruijuan; Xu, Tianqiu; Chen, Jing; Chen, Xuejing; Zhong, Yan; Meng, Chao; Wang, Hong; Chen, Xueqing

2014-08-01

The purpose of this study is to analyze the development of musicality in children after cochlear implantation, and provide a clinical database for the evaluation of their musicality. Twenty-six children with cochlear implants (CI group) participated in this research. They received cochlear implants at the age of 11 to 68 months with a mean of 35.6 months. Seventy-six infants as a control group aged from 1 to 24 months with a mean of 6.1 months participated in this study, whose hearing were considered normal by passing the case history collection, high-risk registers for hearing loss and hearing screening using DPOAE. The music and young children with CIs: Musicality Rating Scale was used to evaluate their musicality. The evaluation was performed before cochlear implantation and 1, 3, 6, 9, 12, 24 months after cochlear implantation for children with cochlear implants. The evaluation was also performed at 1, 3, 6, 9, 12, 24 months for children with normal hearing. The mean scores of musicality showed significant improvements with time of CI use for CI group (Pmusicality also showed significant improvements with time for control group (P0.05). Significant difference was noted between the two groups at 24 months (Pmusicality of children with cochlear implants improved significantly with time after cochlear implantation. The most rapid growth was found in the first year after cochlear implantation.

The cochlear nerve canal and internal auditory canal in children with normal cochlea but cochlear nerve deficiency

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Yan, Fei; Li, Jianhong; Xian, Junfang; Wang, Zhenchang [Dept. of Radiology, Beijing Tongren Hospital, Capital Medical Univ., Beijing (China)], e-mail: cjr.wzhch@vip.163.com; Mo, Lingyan [Dept. of Otorhinolaryngology, Beijing Tongren Hospital, Capital Medical Univ., Beijing (China)

2013-04-15

Background: There is an increasing frequency of requests for cochlear implantation (CI) in deaf children and more detailed image information is necessary for selecting appropriate candidates. Cochlear nerve deficiency (CND) is a contraindication to CI. Magnetic resonance imaging (MRI) has been used to evaluate the integrity of the cochlear nerve. The abnormalities of the cochlear nerve canal (CNC) and internal auditory canal (IAC) have been reported to be associated with CND. Purpose: To correlate CNC manifestation, size, and IAC diameter on high-resolution CT (HRCT) with CND diagnosed by MRI in children. Material and Methods: HRCT images from 35 sensorineurally deaf children who had normal cochlea but bilateral or unilateral CND diagnosed by MRI were studied retrospectively. The CNC and IAC manifestation and size were assessed and correlated with CND. Results: CND was diagnosed by MRI in 54/70 ears (77.1%). Thirty-two ears had an absent cochlear nerve (59.3%), while 22 ears had a small cochlear nerve (40.7%). The CNC diameter was <1.5 mm in 36 ears (66.7%). The CNC diameter ranged between 1.5 and 2.0 mm in seven ears (13.0%) and was >2.0 mm in 11 ears (20.4%). The IAC diameter was <3.0 mm in 25 ears (46.3%) and >3.0 mm in 29 ears (53.7%). Conclusion: The hypoplastic CNC might be more highly indicative of CND than that of a narrow IAC.

New Criteria of Indication and Selection of Patients to Cochlear Implant

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André L. L. Sampaio

2011-01-01

Full Text Available Numerous changes continue to occur in cochlear implant candidacy. In general, these have been accompanied by concomitant and satisfactory changes in surgical techniques. Together, this has advanced the utility and safety of cochlear implantation. Most devices are now approved for use in patients with severe to profound unilateral hearing loss rather then the prior requirement of a bilateral profound loss. Furthermore, studies have begun utilizing short electrode arrays for shallow insertion in patients with considerable low-frequency residual hearing. This technique will allow the recipient to continue to use acoustically amplified hearing for the low frequencies simultaneously with a cochlear implant for the high frequencies. The advances in design of, and indications for, cochlear implants have been matched by improvements in surgical techniques and decrease in complications. The resulting improvements in safety and efficacy have further encouraged the use of these devices. This paper will review the new concepts in the candidacy of cochlear implant. Medline data base was used to search articles dealing with the following topics: cochlear implant in younger children, cochlear implant and hearing preservation, cochlear implant for unilateral deafness and tinnitus, genetic hearing loss and cochlear implant, bilateral cochlear implant, neuropathy and cochlear implant and neural plasticity, and the selection of patients for cochlear implant.

Remote programming of cochlear implants: a telecommunications model.

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McElveen, John T; Blackburn, Erin L; Green, J Douglas; McLear, Patrick W; Thimsen, Donald J; Wilson, Blake S

2010-09-01

Evaluate the effectiveness of remote programming for cochlear implants. Retrospective review of the cochlear implant performance for patients who had undergone mapping and programming of their cochlear implant via remote connection through the Internet. Postoperative Hearing in Noise Test and Consonant/Nucleus/Consonant word scores for 7 patients who had undergone remote mapping and programming of their cochlear implant were compared with the mean scores of 7 patients who had been programmed by the same audiologist over a 12-month period. Times required for remote and direct programming were also compared. The quality of the Internet connection was assessed using standardized measures. Remote programming was performed via a virtual private network with a separate software program used for video and audio linkage. All 7 patients were programmed successfully via remote connectivity. No untoward patient experiences were encountered. No statistically significant differences could be found in comparing postoperative Hearing in Noise Test and Consonant/Nucleus/Consonant word scores for patients who had undergone remote programming versus a similar group of patients who had their cochlear implant programmed directly. Remote programming did not require a significantly longer programming time for the audiologist with these 7 patients. Remote programming of a cochlear implant can be performed safely without any deterioration in the quality of the programming. This ability to remotely program cochlear implant patients gives the potential to extend cochlear implantation to underserved areas in the United States and elsewhere.

[Cochlear implantation in a child with congenital sensorineural deafness due to 35 DELG mutation in GJB2 (connexin 26) gene].

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Teriutin, F M; Barashkov, N A; Dzhemileva, L U; Posukh, O L; Fedotova, E E; Gurinova, E E; Fedorova, S A; Tavartkiladze, G A; Khusnutdinova, E K

2009-01-01

This paper reports the first case of cochlear implantation performed in this country in a child with congenital non-syndromic sensorineural loss of hearing having hereditary etiology and attributable to autosomal-recessive 35 delG mutation in locus DFNB1 (13q.11-q12) of GJB2 (connexin 26) gene.

Intraoperative cochlear nerve mapping with the mobile cochlear nerve compound action potential tracer in vestibular schwannoma surgery.

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Watanabe, Nobuyuki; Ishii, Takuya; Fujitsu, Kazuhiko; Kaku, Shogo; Ichikawa, Teruo; Miyahara, Kosuke; Okada, Tomu; Tanino, Shin; Uriu, Yasuhiro; Murayama, Yuichi

2018-05-18

OBJECTIVE The authors describe the usefulness and limitations of the cochlear nerve compound action potential (CNAP) mobile tracer (MCT) that they developed to aid in cochlear nerve mapping during vestibular schwannoma surgery (VSS) for hearing preservation. METHODS This MCT device requires no more than 2 seconds for stable placement on the nerve to obtain the CNAP and thus is able to trace the cochlear nerve instantaneously. Simultaneous bipolar and monopolar recording is possible. The authors present the outcomes of 18 consecutive patients who underwent preoperative useful hearing (defined as class I or II of the Gardner-Robertson classification system) and underwent hearing-preservation VSS with the use of the MCT. Mapping was considered successful when it was possible to detect and trace the cochlear nerve. RESULTS Mapping of the cochlear nerve was successful in 13 of 18 patients (72.2%), and useful hearing was preserved in 11 patients (61.1%). Among 8 patients with large tumors (Koos grade 3 or 4), the rate of successful mapping was 62.5% (5 patients). The rate of hearing preservation in patients with large tumors was 50% (4 patients). CONCLUSIONS In addition to microsurgical presumption of the arrangement of each nerve, frequent probing on and around an unidentified nerve and comparison of each waveform are advisable with the use of both more sensitive monopolar and more location-specific bipolar MCT. MCT proved to be useful in cochlear nerve mapping and may consequently be helpful in hearing preservation. The authors discuss some limitations and problems with this device.

Frequency of Depressive Syndromes in Elderly Individuals with No Cognitive Impairment, Mild Cognitive Impairment, and Alzheimer's Disease Dementia in a Memory Clinic Setting.

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Lee, Jun Ho; Byun, Min Soo; Yi, Dahyun; Choe, Young Min; Choi, Hyo Jung; Baek, Hyewon; Sohn, Bo Kyung; Kim, Hyun Jung; Lee, Younghwa; Woo, Jong Inn; Lee, Dong Young

2016-01-01

The aims of this study were to investigate the frequency of various depressive syndromes in elderly individuals with no cognitive impairment (NC), mild cognitive impairment (MCI), and Alzheimer's disease dementia (AD) in a memory clinic setting, and then to test whether severe and milder forms of depressive syndromes are differentially associated with the cognitive groups. For 216 NC, 478 MCI, and 316 AD subjects, we investigated the frequency of depressive syndromes, defined by three different categories: major and minor depressive disorder (MaDD and MiDD) according to the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, as well as depression according to the National Institute of Mental Health provisional diagnostic criteria for depression in Alzheimer's disease (NIMH-dAD). The frequency of MaDD did not show any significant difference among NC, MCI, and AD. In contrast, the frequencies of MiDD and NIMH-dAD were higher than those of MaDD and showed significant group differences with a gradual increase from NC to AD. The findings suggest that the degenerative process of Alzheimer's disease contributes to the occurrence of mild depressive conditions, but not to severe depression. © 2016 S. Karger AG, Basel.

Deafblind People's Experiences of Cochlear Implantation

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Soper, Janet

2006-01-01

Cochlear implants are electronic devices that create the sensation of hearing in those who cannot obtain any benefit from conventional hearing aids. This article examines the experience of cochlear implantation in a select group of individuals with acquired deafblindness, focusing on three key themes: access to communication, information and…

Enhanced MRI in patients with Ramsay-Hunt's syndrome

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Yanagida, Masahiro; Ushiro, Koichi; Yamashita, Toshio; Kumazawa, Tadami (Kansai Medical Univ., Osaka (Japan). Dept. of Otolaryngology); Katoh, Tsutomu (Kansai Medical Univ., Osaka (Japan). Dept. of Radiology)

1993-01-01

Enhanced MRI was performed in 14 patients with Ramsay-Hunt,s syndrome to investigate the pathogenesis of this syndrome. All MRI studies were performed on a 0.5T superconductivity MRI system using a head coil with Gd-DTPA. Enhancement was observed in the areas of the distal internal auditory canal and labyrinthine segment in many patients, and was especially prominent in patients suffering from vertigo, tinnitus, and hearing loss. In some patients it involved not only the facial nerve of the internal auditory canal but also the cochlear nerve and vestibular nerves. Since histological changes of the facial nerve in patients with Ramsay-Hunt's syndrome are assumed to occur in the distal internal auditory canal and labyrinthine segment, which is more proximal than the geniculate ganglion, and the possibility is suggested that inflammation may be spread to the vestibular and cochlear nerve via the internal auditory canal. (14 refs., 2 figs.).

STATIC AND DYNAMIC POSTURE CONTROL IN POSTLINGUAL COCHLEAR IMPLANTED PATIENTS: Effects of dual-tasking, visual and auditory inputs suppression

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BERNARD DEMANZE eLaurence

2014-01-01

Full Text Available Posture control is based on central integration of multisensory inputs, and on internal representation of body orientation in space. This multisensory feedback regulates posture control and continuously updates the internal model of body’s position which in turn forwards motor commands adapted to the environmental context and constraints. The peripheral localization of the vestibular system, close to the cochlea, makes vestibular damage possible following cochlear implant (CI surgery. Impaired vestibular function in CI patients, if any, may have a strong impact on posture stability. The simple postural task of quiet standing is generally paired with cognitive activity in most day life conditions, leading therefore to competition for attentional resources in dual-tasking, and increased risk of fall particularly in patients with impaired vestibular function. This study was aimed at evaluating the effects of post-lingual cochlear implantation on posture control in adult deaf patients. Possible impairment of vestibular function was assessed by comparing the postural performance of patients to that of age-matched healthy subjects during a simple postural task performed in static and dynamic conditions, and during dual-tasking with a visual or auditory memory task. Postural tests were done in eyes open (EO and eyes closed (EC conditions, with the cochlear implant activated (ON or not (OFF. Results showed that the CI patients significantly reduced limits of stability and increased postural instability in static conditions. In dynamic conditions, they spent considerably more energy to maintain equilibrium, and their head was stabilized neither in space nor on trunk while the controls showed a whole body rigidification strategy. Hearing (prosthesis on as well as dual-tasking did not really improve the dynamic postural performance of the CI patients. We conclude that CI patients become strongly visual dependent mainly in challenging postural conditions.

Obstructive sleep apnea-hypopnea syndrome and cognitive impairments in the elderly

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Song Shuling

2017-01-01

Full Text Available Obstructive sleep apnea-hypopnea syndrome (OSAHS is a common sleep-related breathing disorder that is associated with significant morbidity and mortality. It has received increasing attention that neurocognitive deficits occur with a high frequency in OSAHS. However, it is rarely known that OSAHS impacts on cognition in the elderly in whom an increased prevalence of OSAHS is present. In this review we consider recent studies in the association between OSAHS and cognitive impairments, with specific interest in the older population. Firstly, we elucidate the characteristics of OSAHS and OSAHS-related cognitive impairments in the older patients. Many studies have showed that the prevalence of OSAHS increases with age and it is higher in the elderly than other population. Moreover, OSAHS is associated with higher incidence of comorbidities and increased risk of clinical deterioration in the elderly, especially the neurocognitive impairments which even can develop dementia. Subsequently, we discuss the possible reasons of cognitive impairments that caused or aggravated by OSAHS in the elderly. The intermittent hypoxia (IH-related disturbances of homeostasis such as oxidative stress, inflammation, and age-related changes such as the changes of sleep architecture, the declined expression level of anti-aging gene, medical comorbidities and polypharmacy, may be both contribute to the increased risk of cognitive impairments in the older patients with OSAHS.

Chronic Conductive Hearing Loss Leads to Cochlear Degeneration.

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Liberman, M Charles; Liberman, Leslie D; Maison, Stéphane F

2015-01-01

Synapses between cochlear nerve terminals and hair cells are the most vulnerable elements in the inner ear in both noise-induced and age-related hearing loss, and this neuropathy is exacerbated in the absence of efferent feedback from the olivocochlear bundle. If age-related loss is dominated by a lifetime of exposure to environmental sounds, reduction of acoustic drive to the inner ear might improve cochlear preservation throughout life. To test this, we removed the tympanic membrane unilaterally in one group of young adult mice, removed the olivocochlear bundle in another group and compared their cochlear function and innervation to age-matched controls one year later. Results showed that tympanic membrane removal, and the associated threshold elevation, was counterproductive: cochlear efferent innervation was dramatically reduced, especially the lateral olivocochlear terminals to the inner hair cell area, and there was a corresponding reduction in the number of cochlear nerve synapses. This loss led to a decrease in the amplitude of the suprathreshold cochlear neural responses. Similar results were seen in two cases with conductive hearing loss due to chronic otitis media. Outer hair cell death was increased only in ears lacking medial olivocochlear innervation following olivocochlear bundle cuts. Results suggest the novel ideas that 1) the olivocochlear efferent pathway has a dramatic use-dependent plasticity even in the adult ear and 2) a component of the lingering auditory processing disorder seen in humans after persistent middle-ear infections is cochlear in origin.

Efter cochlear implant

DEFF Research Database (Denmark)

Højen, Anders

Dit barn har netop fået et cochlear implant. Hvad nu? Skal barnet fokusere udelukkende på at lære talt sprog, eller skal det også lære/fortsætte med tegnsprog eller støttetegn? Det er et vanskeligt spørgsmål, og før valget foretages, er det vigtigt at vurdere hvilke konsekvenser valget har, dels...... for den sproglige udvikling isoleret set, og dels for barnets udvikling ud fra en helhedsbetragtning. Dette indlæg fokuserer på, hvilke forventninger man kan have til cochlear implant-brugeres sproglige udvikling med talt sprog alene, hhv. med to sprog (tale og tegn). Disse forventninger er baseret på...

Functional Near-Infrared Spectroscopy Brain Imaging Investigation of Phonological Awareness and Passage Comprehension Abilities in Adult Recipients of Cochlear Implants

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Bisconti, Silvia; Shulkin, Masha; Hu, Xiaosu; Basura, Gregory J.; Kileny, Paul R.; Kovelman, Ioulia

2016-01-01

Purpose: The aim of this study was to examine how the brains of individuals with cochlear implants (CIs) respond to spoken language tasks that underlie successful language acquisition and processing. Method: During functional near-infrared spectroscopy imaging, CI recipients with hearing impairment (n = 10, mean age: 52.7 ± 17.3 years) and…

Impaired affective prosody decoding in severe alcohol use disorder and Korsakoff syndrome.

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Brion, Mélanie; de Timary, Philippe; Mertens de Wilmars, Serge; Maurage, Pierre

2018-06-01

Recognizing others' emotions is a fundamental social skill, widely impaired in psychiatric populations. These emotional dysfunctions are involved in the development and maintenance of alcohol-related disorders, but their differential intensity across emotions and their modifications during disease evolution remain underexplored. Affective prosody decoding was assessed through a vocalization task using six emotions, among 17 patients with severe alcohol use disorder, 16 Korsakoff syndrome patients (diagnosed following DSM-V criteria) and 19 controls. Significant disturbances in emotional decoding, particularly for negative emotions, were found in alcohol-related disorders. These impairments, identical for both experimental groups, constitute a core deficit in excessive alcohol use. Copyright © 2018 Elsevier B.V. All rights reserved.

Cognitive impairment associated with locomotive syndrome in community-dwelling elderly women in Japan

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Nakamura M

2017-09-01

Full Text Available Misa Nakamura,1 Fumie Tazaki,1 Kazuki Nomura,1 Taeko Takano,1 Masashi Hashimoto,1 Hiroshi Hashizume,2 Ichiro Kamei1 1Department of Rehabilitation, Osaka Kawasaki Rehabilitation University, Kaizuka, Osaka, Japan; 2Department of Orthopaedic Surgery, Wakayama Medical University, Wakayama, Japan Abstract: In our worldwide aging society, elderly people should maintain cognitive and physical function to help avoid health problems. Dementia is a major brain disease among elderly people, and is caused by cognitive impairment. The locomotive syndrome (LS refers to a condition in which people require healthcare services because of problems associated with locomotion. The purpose of this study was to determine the association between cognitive impairment and LS. Study participants were 142 healthy elderly female volunteers living in a rural area in Japan. Cognitive function was assessed using the Mini-Mental State Examination (MMSE. A score of ≤26 points on the MMSE was used to indicate categorically defined poor cognitive performance (cognitive impairment. The LS was defined by a score ≥16 points, and non-LS as <16 points, on the 25-question Geriatric Locomotive Function Scale (GLFS-25. Twenty-one participants (14.8% had an MMSE score ≤26, and 19.0% were found to have LS. Compared with the MMSE >26 group, the ≤26 group was significantly older, had a higher percentage of body fat, and a higher GLFS-25 score. Those with LS were significantly older, had a higher body mass index, a higher percentage of body fat, and a lower MMSE score. Participants in the LS group had higher odds of cognitive impairment than those without LS [odds ratio (OR =3.08] by logistic regression analysis adjusted for age. Furthermore, participants with GLFS-25 scores ≥6 had higher odds of cognitive impairment than those with a GLFS-25 score <6 by logistic regression analysis adjusted for both age (OR =4.44, and age and percent body fat (OR =4.12. These findings

Evaluating cochlear implant trauma to the scala vestibuli.

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Adunka, O; Kiefer, J; Unkelbach, M H; Radeloff, A; Gstoettner, W

2005-04-01

Placement of cochlear implant electrodes into the scala vestibuli may be intentional, e.g. in case of blocked scala tympani or unintentional as a result of trauma to the basilar membrane or erroneous location of the cochieostomy. The aim of this study was to evaluate the morphological consequences and cochlear trauma after implantation of different cochlear implant electrode arrays in the scala vestibuli. Human temporal bone study with histological and radiological evaluation. Twelve human cadaver temporal bones were implanted with different cochlear implant electrodes. Implanted bones were processed using a special method to section undecalcified bone. Cochlear trauma and intracochlear positions. All implanted electrodes were implanted into the scala vestibuli using a special approach that allows direct scala vestibuli insertions. Fractures of the osseous spiral lamina were evaluated in some bones in the basal cochlear regions. In most electrodes, delicate structures of the organ of Corti were left intact, however, Reissner's membrane was destroyed in all specimens and the electrode lay upon the tectorial membrane. In some bones the organ of Corti was destroyed. Scala vestibuli insertions did not cause severe trauma to osseous or neural structures, thus preserving the basis for electrostimulation of the cochlea. However, destruction of Reissner's membrane and impact on the Organ of Corti can be assumed to destroy residual hearing.

The quality of life in cochlear implant children after two years from surgery and its impact on the family

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Seyed Basir Hashemi

2010-06-01

Full Text Available Introduction: Cochlear implant has been established as effective option in rehabilitation of individuals with profound hearing impairment. As much of the studies about cochlear implants concentrated on aspects of speech perception and production, so we decided to study the quality of life of pre-lingual deaf children after at least 2 years of implantation. Materials and Methods: Twenty four patients’ parents in Fars Center were selected that had been implanted for at least 2 years and they filled the quality of life questionnaire. Three months later this questionnaire was filled again and results of two stages were analyzed. Results: In part of assessments p-value shows significant change in parent’s satisfaction. They believe that they can be beneficial for their children and in another part of this study, parents believe that the children communicate better, but still they need special care to do school homework and they have some difficulties in articulation. Conclusion: cochlear implantation is associated with improvement in quality of life. The improvement is significant in aspects of social communications and happiness.   

Biochemical alteration in children with idiopathic nephrotic syndrome associated with an increased risk of sensorineural hearing loss; additional insights in cochlear renal relationship.

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El Mashad, Ghada Mohamed; Abo El Fotoh, Wafaa Moustafa M; Zein El Abedein, Ahmed Mahmoud; Abd El Sadek, Fatma Abd El Raoof

2017-06-01

Children with Idiopathic Nephrotic Syndrome (INS) are at risk of hearing loss due to the adverse impact of medications and related immunological and genetic factors on both cochlea and kidney. So this work was planned to evaluate hearing status in children with INS and to clarify the possible associated risk factors by interpreting the clinical and laboratory profiles of those children. Ninety children with INS aged 5-14 years [30 patients with steroid-sensitive nephrotic syndrome (SSNS), 30 patients with steroid dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS), and 30 patients with steroid-resistant nephrotic syndrome (SRNS)], and 90 age and sex matched normal controls were enrolled into this study. Laboratory measurements of serum calcium, creatinine, cholesterol, blood urea and other relevant investigations were done. Pure tone audiometry was done with the sensory-neural hearing loss (SNHL) diagnosed when the level bone conduction was >20 dB and the difference in air to the bone gap was children with INS had SNHL, mostly of mild degree HL and primarily occurred at the lower frequencies. A highly significant statistical difference between controls and various types of nephrotic syndrome regarding pure tone audiometry measurements at frequencies 250, 500, 1000 Hz, whereas insignificant difference interpreting pure tone audiometry measurements in 2000, 4000 and 8000 Hz. Children with different phenotypes of nephrotic syndrome are at risk of sensorineural hearing impairment. The hazards associated with this impairment were higher blood pressure, hypercholesterolemia, hypoalbuminemia, and hypocalcemia. Copyright © 2017 Elsevier B.V. All rights reserved.

A clinical study on cognitive impairment in post-ischemic stroke patients with metabolic syndrome

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LI Chen

2012-02-01

Full Text Available Objective To explore the relation between metabolic syndrome (MetS and cognitive impairment after ischemic stroke. Methods Ninety-four cases of first ischemic stroke patients were divided into stroke without MetS group (n = 54 and stroke with MetS group (n = 40 according to the diagnostic criteria for MetS defined by Metabolic Syndrome Researching Group of Chinese Diabetes Society. All patients underwent Mini-Mental State Examination (MMSE, Clock Drawing Test (CDT, animal Verbal Fluency Test (aVFT, Trial Making Test-A (TMT-A at 2 weeks and 3 months after stroke to evaluate mental state such as verbal learning memory, and executive, attentional and visuospatial abilities. The incidence and development of cognitive impairment were also assessed. Results At 2 weeks and 3 months after stroke, the incidence of cognitive impairment were 24.47% (23/94 and 22.34% (21/94, respectively, and in the cognitive impairment patients the incidence of non-dementia were 21.28% (20/94 and 19.15% (18/94, while the incidence of dementia were 3.19% (3/94 and 3.19% (3/94, respectively. The incidence of cognitive impairment was higher in the stroke patients with MetS than the stroke patients without MetS, 37.50% (15/40 vs 14.81% (8/54 (Z = 2.500, P = 0.012 at 2 weeks after stroke and 35.00% (14/40 vs 12.96% (7/54 (Z = 2.513, P = 0.012 at 3 months after stroke. In the scores of MMSE, delay recall and CDT of the stroke patients with MetS were all lower than those without MetS at 2 weeks after stroke and at 3 months after stroke (P < 0.05, for all. The stroke patients with MetS had more cognition deterioration than the stroke patients without MetS at 3 months after stroke, the difference was significant (Z = 2.134, P = 0.033. Conclusion MetS can increase the incidence of cognitive impairment, especially non-dementia cognitive impairment in post ischemic stroke. Executive dysfunction and hypomnesis are often seen. The development of cognitive impairment in stroke patients

Effects of chronic furosemide on central neural hyperactivity and cochlear thresholds after cochlear trauma in guinea pig

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Wilhelmina eMulders

2014-08-01

Full Text Available Increased neuronal spontaneous firing rates have been observed throughout the central auditory system after trauma to the cochlea and this hyperactivity is believed to be associated with the phantom perception of tinnitus. Previously we have shown in an animal model of hearing loss, that an acute injection with furosemide can significantly decrease hyperactivity after cochlear trauma and eliminate behavioural evidence of tinnitus of early onset. However, furosemide also has the potential to affect cochlear thresholds. In this paper we measured the effects of a chronic (daily injections for 7 days furosemide treatment on the spontaneous firing rate of inferior colliculus neurons and on cochlear thresholds in order to establish whether a beneficial effect on hyperactivity can be obtained without causing additional hearing loss. Guinea pigs were exposed to a 10 kHz, 124dB, 2 hour acoustic trauma, and after 5 days of recovery, were given daily i.p. injections of 80mg/kg furosemide or an equivalent amount of saline. The activity of single IC neurons was recorded 24 hours following the last injection. The furosemide treatment had no effect on cochlear thresholds compared to saline injections but did result in significant reductions in spontaneous firing rates recorded in inferior colliculus. These results that suggest a long term beneficial effect of furosemide on hyperactivity after cochlear trauma may be achievable without detrimental effects on hearing, which is important when considering therapeutic potential.

The cochlear implant as a tinnitus treatment.

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Vallés-Varela, Héctor; Royo-López, Juan; Carmen-Sampériz, Luis; Sebastián-Cortés, José M; Alfonso-Collado, Ignacio

2013-01-01

Tinnitus is a symptom of high prevalence in patients with cochlear pathology. We studied the evolution of tinnitus in patients undergoing unilateral cochlear implantation for treatment of profound hearing loss. This was a longitudinal, retrospective study of patients that underwent unilateral cochlear implantation and who had bilateral tinnitus. Tinnitus was assessed quantitatively and qualitatively before surgery and at 6 and 12 months after surgery. We evaluated 20 patients that underwent unilateral cochlear implantation with a Nucleus(®) CI24RE Contour Advance™ electrode device. During the periods in which the device was in operation, improvement or disappearance of tinnitus was evidenced in the ipsilateral ear in 65% of patients, and in the contralateral ear, in 50%. In periods in which the device was disconnected, improvement or disappearance of tinnitus was found in the ipsilateral ear in 50% of patients, and in the ear contralateral to the implant in 45% of the patients. In 10% of the patients, a new tinnitus appeared in the ipsilateral ear. The patients with profound hearing loss and bilateral tinnitus treated with unilateral cochlear implantation improved in a high percentage of cases, in the ipsilateral ear and in the contralateral ear. Copyright © 2012 Elsevier España, S.L. All rights reserved.

Cochlear implants in children implanted in Jordan: A parental overview.

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Alkhamra, Rana A

2015-07-01

Exploring the perspective of parents on the cochlear implant process in Jordan. Sixty parents of deaf children were surveyed on the information gathering process prior to cochlear implant surgery, and their implant outcome expectations post-surgery. Whether child or parent characteristics may impact parents' post-surgical expectations was explored. Although parents used a variety of information sources when considering a cochlear implant, the ear, nose and throat doctor comprised their major source of information (60%). Parents received a range of information prior to cochlear implant but agreed (93.3%) on the need for a multidisciplinary team approach. Post-surgically, parents' expected major developments in the areas of spoken language (97%), and auditory skills (100%). Receiving education in mainstream schools (92%) was expected too. Parents perceived the cochlear implant decision as the best decision they can make for their child (98.3%). A significant correlation was found between parents contentment with the cochlear implant decision and expecting developments in the area of reading and writing (r=0.7). Child's age at implantation and age at hearing loss diagnosis significantly affected parents' post-implant outcome expectations (pparents agree on the need for a comprehensive multidisciplinary team approach during the different stages of the cochlear implant process. Parents' education about cochlear implants prior to the surgery can affect their post-surgical outcome expectations. The parental perspective presented in this study can help professionals develop better understanding of parents' needs and expectations and henceforth improve their services and support during the different stages of the cochlear implant process. Copyright © 2015. Published by Elsevier Ireland Ltd.

Efficient estimates of cochlear hearing loss parameters in individual listeners

DEFF Research Database (Denmark)

Fereczkowski, Michal; Jepsen, Morten Løve; Dau, Torsten

2013-01-01

It has been suggested that the level corresponding to the knee-point of the basilar membrane (BM) input/output (I/O) function can be used to estimate the amount of inner- and outer hair-cell loss (IHL, OHL) in listeners with a moderate cochlear hearing impairment Plack et al. (2004). According...... to Jepsen and Dau (2011) IHL + OHL = HLT [dB], where HLT stands for total hearing loss. Hence having estimates of the total hearing loss and OHC loss, one can estimate the IHL. In the present study, results from forward masking experiments based on temporal masking curves (TMC; Nelson et al., 2001...... estimates of the knee-point level. Further, it is explored whether it is possible to estimate the compression ratio using only on-frequency TMCs. 10 normal-hearing and 10 hearing-impaired listeners (with mild-to-moderate sensorineural hearing loss) were tested at 1, 2 and 4 kHz. The results showed...

Comparison of outcomes in a case of bilateral cochlear implantation using devices manufactured by two different implant companies (Cochlear Corporation and Med-El).

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Withers, S J; Gibson, W P; Greenberg, S L; Bray, M

2011-05-01

This paper reports a case of a patient who has had bilateral cochlear implants that have been manufactured by different cochlear implant companies (Cochlear Corporation and Med-El). Comparison of speech perception tests following single implant insertion and bilateral insertion (3 and 12 months). The patient was also interviewed to obtain a subjective opinion on their quality of hearing. The patient reported that their Med-El implant had better sound quality than their Cochlear Corporation implant. The speech perception tests however failed to show any difference. Despite no difference found with the objective tests hearing is very subjective and therefore the patient's opinion on the quality of sound is important. It is only a matter of time before other patients are fitted with bilateral cochlear implants from different companies and this information should be collated to allow comparison between manufacturers.

[Inspecting the cochlear scala tympanic with flexible and semi-flexible micro-endoscope].

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Zhang, Daoxcing; Zhang, Yankun

2006-02-01

Flexible and semi-flexible micro-endoscopes were used in cochlear scala tympani inspection , to explore their application in inner ear examination. Fifteen profound hearing loss patients preparing for cochlear implant were included in this study. During the operation, micro-endoscopy was performed after opening the cochlear scala tympani. And 1 mm diameter semi-flexible micro-endoscope could go as deep as 9 mm into the cochlear scala tympani, while 0. 5 mm diameter flexible micro-endoscope could go as deep as 25 mm. The inspecting results were compared with video recording. Using 0.5 mm flexible micro-endoscope, we canould check cochlear scala tympani with depth range of 15-25 mm, but the video imaging was not clear enough to examine the microstructure in the cochlear. With 1 mm diameter semi-flexible micro-endoscope, we could reach 9 mm deep into the cochlear. During the examination, we found 3 cases with calcification deposit in osseous spiral lamina, l case with granulation tissue in the lateral wall of scala tympani, no abnormal findings in the other 11 cases. Inspecting the cochlear scala tympani with 0.5 mm flexible micro-endoscope, even though we can reach the second circuit of the cochlear, it is difficult to find the pathology in the cochlear because of the poor video imaging. With 1 mm semi-flexible micro-endoscope, we can identify the microstructure of the cochlear clearly and find the pathologic changes, but the inserting depth was limited to 9 mm with limitation to examine the whole cochlear.

Cochlear implant: what the radiologist should know

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Natalia Delage Gomes

2013-06-01

Full Text Available Cochlear implant is the method of choice in the treatment of deep sensorineural hypoacusis, particularly in patients where conventional amplification devices do not imply noticeable clinical improvement. Imaging findings are crucial in the indication or contraindication for such surgical procedure. In the assessment of the temporal bone, radiologists should be familiar with relative or absolute contraindication factors, as well as with factors that might significantly complicate the implantation. Some criteria such as cochlear nerve aplasia, labyrinthine and/or cochlear aplasia are still considered as absolute contraindications, in spite of studies bringing such criteria into question. Cochlear dysplasias constitute relative contraindications, among them labyrinthitis ossificans is highlighted. Other alterations may be mentioned as complicating agents in the temporal bone assessment, namely, hypoplasia of the mastoid process, aberrant facial nerve, otomastoiditis, otosclerosis, dehiscent jugular bulb, enlarged endolymphatic duct and sac. The experienced radiologist assumes an important role in the evaluation of this condition.

Cochlear implant: what the radiologist should know

International Nuclear Information System (INIS)

Gomes, Natalia Delage; Couto, Caroline Laurita Batista; Gaiotti, Juliana Oggioni; Costa, Ana Maria Doffemond; Ribeiro, Marcelo Almeida; Diniz, Renata Lopes Furletti Caldeira

2013-01-01

Cochlear implant is the method of choice in the treatment of deep sensorineural hypoacusis, particularly in patients where conventional amplification devices do not imply noticeable clinical improvement. Imaging findings are crucial in the indication or contraindication for such surgical procedure. In the assessment of the temporal bone, radiologists should be familiar with relative or absolute contraindication factors, as well as with factors that might significantly complicate the implantation. Some criteria such as cochlear nerve aplasia, labyrinthine and/or cochlear aplasia are still considered as absolute contraindications, in spite of studies bringing such criteria into question. Cochlear dysplasias constitute relative contraindications, among them labyrinthitis ossificans is highlighted. Other alterations may be mentioned as complicating agents in the temporal bone assessment, namely, hypoplasia of the mastoid process, aberrant facial nerve, otomastoiditis, otosclerosis, dehiscent jugular bulb, enlarged endolymphatic duct and sac. The experienced radiologist assumes an important role in the evaluation of this condition. (author)

Chronic lead exposure induces cochlear oxidative stress and potentiates noise-induced hearing loss.

Science.gov (United States)

Jamesdaniel, Samson; Rosati, Rita; Westrick, Judy; Ruden, Douglas M

2018-08-01

Acquired hearing loss is caused by complex interactions of multiple environmental risk factors, such as elevated levels of lead and noise, which are prevalent in urban communities. This study delineates the mechanism underlying lead-induced auditory dysfunction and its potential interaction with noise exposure. Young-adult C57BL/6 mice were exposed to: 1) control conditions; 2) 2 mM lead acetate in drinking water for 28 days; 3) 90 dB broadband noise 2 h/day for two weeks; and 4) both lead and noise. Blood lead levels were measured by inductively coupled plasma mass spectrometry analysis (ICP-MS) lead-induced cochlear oxidative stress signaling was assessed using targeted gene arrays, and the hearing thresholds were assessed by recording auditory brainstem responses. Chronic lead exposure downregulated cochlear Sod1, Gpx1, and Gstk1, which encode critical antioxidant enzymes, and upregulated ApoE, Hspa1a, Ercc2, Prnp, Ccl5, and Sqstm1, which are indicative of cellular apoptosis. Isolated exposure to lead or noise induced 8-12 dB and 11-25 dB shifts in hearing thresholds, respectively. Combined exposure induced 18-30 dB shifts, which was significantly higher than that observed with isolated exposures. This study suggests that chronic exposure to lead induces cochlear oxidative stress and potentiates noise-induced hearing impairment, possibly through parallel pathways. Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.

Tinnitus after Simultaneous and Sequential Bilateral Cochlear Implantation.

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Ramakers, Geerte G J; Kraaijenga, Véronique J C; Smulders, Yvette E; van Zon, Alice; Stegeman, Inge; Stokroos, Robert J; Free, Rolien H; Frijns, Johan H M; Huinck, Wendy J; Van Zanten, Gijsbert A; Grolman, Wilko

2017-01-01

There is an ongoing global discussion on whether or not bilateral cochlear implantation should be standard care for bilateral deafness. Contrary to unilateral cochlear implantation, however, little is known about the effect of bilateral cochlear implantation on tinnitus. To investigate tinnitus outcomes 1 year after bilateral cochlear implantation. Secondarily, to compare tinnitus outcomes between simultaneous and sequential bilateral cochlear implantation and to investigate long-term follow-up (3 years). This study is a secondary analysis as part of a multicenter randomized controlled trial. Thirty-eight postlingually deafened adults were included in the original trial, in which the presence of tinnitus was not an inclusion criterion. All participants received cochlear implants (CIs) because of profound hearing loss. Nineteen participants received bilateral CIs simultaneously and 19 participants received bilateral CIs sequentially with an inter-implant interval of 2 years. The prevalence and severity of tinnitus before and after simultaneous and sequential bilateral cochlear implantation were measured preoperatively and each year after implantation with the Tinnitus Handicap Inventory (THI) and Tinnitus Questionnaire (TQ). The prevalence of preoperative tinnitus was 42% (16/38). One year after bilateral implantation, there was a median difference of -8 (inter-quartile range (IQR): -28 to 4) in THI score and -9 (IQR: -17 to -9) in TQ score in the participants with preoperative tinnitus. Induction of tinnitus occurred in five participants, all in the simultaneous group, in the year after bilateral implantation. Although the preoperative and also the postoperative median THI and TQ scores were higher in the simultaneous group, the median difference scores were equal in both groups. In the simultaneous group, tinnitus scores fluctuated in the 3 years after implantation. In the sequential group, four patients had an additional benefit of the second CI: a total

Balance impairment in individuals with Wolfram syndrome.

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Pickett, Kristen A; Duncan, Ryan P; Paciorkowski, Alex R; Permutt, M Alan; Marshall, Bess; Hershey, Tamara; Earhart, Gammon M

2012-07-01

Wolfram syndrome (WFS), a rare neurodegenerative disorder, is characterized by early onset insulin-dependent diabetes mellitus, optic atrophy, deafness, diabetes insipidus, and neurological abnormalities. Although previously unreported, we hypothesized that neurological complications may be detectable in relatively early stages of the disease. As the cerebellum and brainstem seem particularly vulnerable in WFS, we focused on balance functions critically dependent on these regions. The primary goal of this investigation was to compare balance in young individuals with WFS, in relatively early stages of the disease, to an age-matched cohort using a clinically applicable test. Balance was assessed via the mini-BESTest in 13 children, adolescents and young adults with WFS and 30 typically developing age-matched individuals. A significant difference was observed between groups in balance as well as in three of four subcomponents of the mini-BESTest and in two timed tasks related to balance. Mini-BESTest scores were correlated with age among typically developing individuals. In the WFS group, mini-BESTest scores were related to overall motor dysfunction, but not age. Impairments in balance in WFS may occur earlier in the disease process than previously recognized and appear to be related to overall neurological progression rather than chronological age. Recognizing balance impairments and understanding which balance systems contribute to balance deficits in those with WFS may allow for development of effective patient-centered treatment paradigms. Copyright © 2012 Elsevier B.V. All rights reserved.

Reversible brain atrophy and cognitive impairment in an adolescent Japanese patient with primary adrenal Cushing’s syndrome

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Ohara N

2014-09-01

Full Text Available Nobumasa Ohara,1 Hiroshi Suzuki,1 Akiko Suzuki,1 Masanori Kaneko,1 Masahiro Ishizawa,1 Kazuo Furukawa,1 Takahiro Abe,1 Yasuhiro Matsubayashi,1 Takaho Yamada,1 Osamu Hanyu,1 Takayoshi Shimohata,2 Hirohito Sone1 1Department of Hematology, Endocrinology and Metabolism, Faculty of Medicine, Niigata University, Niigata, Japan; 2Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan Abstract: Endogenous Cushing’s syndrome is an endocrine disease resulting from chronic exposure to excessive glucocorticoids produced in the adrenal cortex. Although the ultimate outcome remains uncertain, functional and morphological brain changes are not uncommon in patients with this syndrome, and generally persist even after resolution of hypercortisolemia. We present an adolescent patient with Cushing’s syndrome who exhibited cognitive impairment with brain atrophy. A 19-year-old Japanese male visited a local hospital following 5 days of behavioral abnormalities, such as money wasting or nighttime wandering. He had hypertension and a 1-year history of a rounded face. Magnetic resonance imaging (MRI revealed apparently diffuse brain atrophy. Because of high random plasma cortisol levels (28.7 µg/dL at 10 AM, he was referred to our hospital in August 2011. Endocrinological testing showed adrenocorticotropic hormone-independent hypercortisolemia, and abdominal computed tomography demonstrated a 2.7 cm tumor in the left adrenal gland. The patient underwent left adrenalectomy in September 2011, and the diagnosis of cortisol-secreting adenoma was confirmed histologically. His hypertension and Cushingoid features regressed. Behavioral abnormalities were no longer observed, and he was classified as cured of his cognitive disturbance caused by Cushing’s syndrome in February 2012. MRI performed 8 months after surgery revealed reversal of brain atrophy, and his subsequent course has been uneventful. In summary, the young age at onset and the

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...

Neurocognitive testing and cochlear implantation: insights into performance in older adults

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Cosetti MK

2016-05-01

compared to a patient’s own performance before implantation. Of these, nine (45% showed moderate or pronounced improvement. Overall, improvements were largest in the verbal and memory domains. Logistic regression demonstrated a significant relationship between speech perception and cognitive function over time. Five neurocognitive tests were predictive of improved speech perception following implantation. Conclusion: Comprehensive neurocognitive testing of elderly women demonstrated areas of improvement in cognitive function and auditory perception following cochlear implantation. Multiple neurocognitive tests were strongly associated with current speech perception measures. While these data shed light on the complex relationship between hearing and cognition by showing that CI may slow the expected age-related cognitive decline, further research is needed to examine the impact of hearing rehabilitation on cognitive decline. Keywords: cognitive function, cognitive impairment, dementia, aging, hearing loss, cochlear implant, neuropsychological testing

Reversible brain atrophy and cognitive impairment in an adolescent Japanese patient with primary adrenal Cushing's syndrome.

Science.gov (United States)

Ohara, Nobumasa; Suzuki, Hiroshi; Suzuki, Akiko; Kaneko, Masanori; Ishizawa, Masahiro; Furukawa, Kazuo; Abe, Takahiro; Matsubayashi, Yasuhiro; Yamada, Takaho; Hanyu, Osamu; Shimohata, Takayoshi; Sone, Hirohito

2014-01-01

Endogenous Cushing's syndrome is an endocrine disease resulting from chronic exposure to excessive glucocorticoids produced in the adrenal cortex. Although the ultimate outcome remains uncertain, functional and morphological brain changes are not uncommon in patients with this syndrome, and generally persist even after resolution of hypercortisolemia. We present an adolescent patient with Cushing's syndrome who exhibited cognitive impairment with brain atrophy. A 19-year-old Japanese male visited a local hospital following 5 days of behavioral abnormalities, such as money wasting or nighttime wandering. He had hypertension and a 1-year history of a rounded face. Magnetic resonance imaging (MRI) revealed apparently diffuse brain atrophy. Because of high random plasma cortisol levels (28.7 μg/dL) at 10 AM, he was referred to our hospital in August 2011. Endocrinological testing showed adrenocorticotropic hormone-independent hypercortisolemia, and abdominal computed tomography demonstrated a 2.7 cm tumor in the left adrenal gland. The patient underwent left adrenalectomy in September 2011, and the diagnosis of cortisol-secreting adenoma was confirmed histologically. His hypertension and Cushingoid features regressed. Behavioral abnormalities were no longer observed, and he was classified as cured of his cognitive disturbance caused by Cushing's syndrome in February 2012. MRI performed 8 months after surgery revealed reversal of brain atrophy, and his subsequent course has been uneventful. In summary, the young age at onset and the short duration of Cushing's syndrome probably contributed to the rapid recovery of both cognitive dysfunction and brain atrophy in our patient. Cushing's syndrome should be considered as a possible etiological factor in patients with cognitive impairment and brain atrophy that is atypical for their age.

Prevention and management of cochlear implant infections.

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Gluth, Michael B; Singh, Rajesh; Atlas, Marcus D

2011-11-01

Understanding the issues of infection related to an implantable medical device is crucial to all cochlear implant teams. Furthermore, given the risk of central nervous system complications and the relatively high quantity of underlying resource investment associated with cochlear implantation, the stakes of infection are high. The optimal strategies to prevent and manage such infections are still evolving as good-quality prospective data to guide such management decisions are not yet abundant within the medical literature and many recommendations are based on retrospective reviews or anecdotal evidence. We will outline a general strategy to deal with cochlear implant-related infection based on both the authors' experience and the published literature.

Lexical and semantic ability in groups of children with cochlear implants, language impairment and autism spectrum disorder.

Science.gov (United States)

Löfkvist, Ulrika; Almkvist, Ove; Lyxell, Björn; Tallberg, Ing-Mari

2014-02-01

Lexical-semantic ability was investigated among children aged 6-9 years with cochlear implants (CI) and compared to clinical groups of children with language impairment (LI) and autism spectrum disorder (ASD) as well as to age-matched children with normal hearing (NH). In addition, the influence of age at implantation on lexical-semantic ability was investigated among children with CI. 97 children divided into four groups participated, CI (n=34), LI (n=12), ASD (n=12), and NH (n=39). A battery of tests, including picture naming, receptive vocabulary and knowledge of semantic features, was used for assessment. A semantic response analysis of the erroneous responses on the picture-naming test was also performed. The group of children with CI exhibited a naming ability comparable to that of the age-matched children with NH, and they also possessed a relevant semantic knowledge of certain words that they were unable to name correctly. Children with CI had a significantly better understanding of words compared to the children with LI and ASD, but a worse understanding than those with NH. The significant differences between groups remained after controlling for age and non-verbal cognitive ability. The children with CI demonstrated lexical-semantic abilities comparable to age-matched children with NH, while children with LI and ASD had a more atypical lexical-semantic profile and poorer sizes of expressive and receptive vocabularies. Dissimilar causes of neurodevelopmental processes seemingly affected lexical-semantic abilities in different ways in the clinical groups. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

The association of cognitive impairment with gray matter atrophy and cortical lesion load in clinically isolated syndrome.

Science.gov (United States)

Diker, Sevda; Has, Arzu Ceylan; Kurne, Aslı; Göçmen, Rahşan; Oğuz, Kader Karlı; Karabudak, Rana

2016-11-01

Multiple sclerosis can impair cognition from the early stages and has been shown to be associated with gray matter damage in addition to white matter pathology. To investigate the profile of cognitive impairment in clinically isolated syndrome (CIS), and the contribution of cortical inflammation, cortical and deep gray matter atrophy, and white matter lesions to cognitive decline. Thirty patients with clinically isolated syndrome and twenty demographically- matched healthy controls underwent neuropsychologic assessment through the Rao Brief Repeatable Battery, and brain magnetic resonance imaging with double inversion recovery using a 3T scanner. Patients with clinically isolated syndrome performed significantly worse than healthy controls on tests that evaluated verbal memory, visuospatial learning and memory, and verbal fluency. Significant deep gray matter atrophy was found in the patients but cortical volume was not lower than the controls. Visual memory tests correlated with the volume of the hippocampus, cerebral white matter and deep gray matter structures and with cerebellar cortical atrophy. Cortical or white matter lesion load did not affect cognitive test results. In our patients with CIS, it was shown that cognitive impairment was mainly related to cerebral white matter, cerebellar cortical and deep gray matter atrophy, but not with cortical inflammation, at least in the early stage of disease. Copyright © 2016 Elsevier B.V. All rights reserved.

Depression, anxiety and somatization in primary care: syndrome overlap and functional impairment.

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Löwe, Bernd; Spitzer, Robert L; Williams, Janet B W; Mussell, Monika; Schellberg, Dieter; Kroenke, Kurt

2008-01-01

To determine diagnostic overlap of depression, anxiety and somatization as well as their unique and overlapping contribution to functional impairment. Two thousand ninety-one consecutive primary care clinic patients participated in a multicenter cross-sectional survey in 15 primary care clinics in the United States (participation rate, 92%). Depression, anxiety, somatization and functional impairment were assessed using validated scales from the Patient Health Questionnaire (PHQ) (PHQ-8, eight-item depression module; GAD-7, seven-item Generalized Anxiety Disorder Scale; and PHQ-15, 15-item somatic symptom scale) and the Short-Form General Health Survey (SF-20). Multiple linear regression analyses were used to investigate unique and overlapping associations of depression, anxiety and somatization with functional impairment. In over 50% of cases, comorbidities existed between depression, anxiety and somatization. The contribution of the commonalities of depression, anxiety and somatization to functional impairment substantially exceeded the contribution of their independent parts. Nevertheless, depression, anxiety and somatization did have important and individual effects (i.e., separate from their overlap effect) on certain areas of functional impairment. Given the large syndrome overlap, a potential consideration for future diagnostic classification would be to describe basic diagnostic criteria for a single overarching disorder and to optionally code additional diagnostic features that allow a more detailed classification into specific depressive, anxiety and somatoform subtypes.

Effects of NSAIDs on the Inner Ear: Possible Involvement in Cochlear Protection

Directory of Open Access Journals (Sweden)

Akira Hara

2010-04-01

Full Text Available Cyclooxygenase and lipoxygenase, two important enzymes involved in arachidonic acid metabolism, are major targets of non-steroidal anti-inflammatory drugs (NSAIDs. Recent investigations suggest that arachidonic cascades and their metabolites may be involved in maintaining inner ear functions. The excessive use of aspirin may cause tinnitus in humans and impairment of the outer hair cell functions in experimental animals. On the other hand, NSAIDs reportedly exhibit protective effects against various kinds of inner ear disorder. The present review summarizes the effects of NSAIDs on cochlear pathophysiology. NSAIDs are a useful ameliorative adjunct in the management of inner ear disorders.

Gain and frequency tuning within the mouse cochlear apex

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Oghalai, John S.; Raphael, Patrick D. [Department of Otolaryngology, Stanford University School of Medicine, Stanford, California (United States); Gao, Simon [Department of Otolaryngology, Stanford University School of Medicine, Stanford, California (United States); Department of Bioengineering, Rice University, Houston, Texas (United States); Lee, Hee Yoon [Department of Otolaryngology, Stanford University School of Medicine, Stanford, California (United States); Department of Electrical Engineering, Stanford University, Stanford, California (United States); Groves, Andrew K. [Department of Neuroscience, Department of Molecular and Human Genetics, and Program in Developmental Biology, Baylor College of Medicine, Houston, Texas (United States); Zuo, Jian [Department of Developmental Neurobiology, St. Jude Children’s Research Hospital, Memphis, Tennessee (United States); Applegate, Brian E. [Department of Biomedical Engineering, Texas A& M University, College Station, Texas (United States)

2015-12-31

Normal mammalian hearing requires cochlear outer hair cell active processes that amplify the traveling wave with high gain and sharp tuning, termed cochlear amplification. We have used optical coherence tomography to study cochlear amplification within the apical turn of the mouse cochlea. We measured not only classical basilar membrane vibratory tuning curves but also vibratory responses from the rest of the tissues that compose the organ of Corti. Basilar membrane tuning was sharp in live mice and broad in dead mice, whereas other regions of the organ of Corti demonstrated phase shifts consistent with additional filtering beyond that provided by basilar membrane mechanics. We use these experimental data to support a conceptual framework of how cochlear amplification is tuned within the mouse cochlear apex. We will also study transgenic mice with targeted mutations that affect different biomechanical aspects of the organ of Corti in an effort to localize the underlying processes that produce this additional filtering.

Gain and frequency tuning within the mouse cochlear apex

International Nuclear Information System (INIS)

Oghalai, John S.; Raphael, Patrick D.; Gao, Simon; Lee, Hee Yoon; Groves, Andrew K.; Zuo, Jian; Applegate, Brian E.

2015-01-01

Normal mammalian hearing requires cochlear outer hair cell active processes that amplify the traveling wave with high gain and sharp tuning, termed cochlear amplification. We have used optical coherence tomography to study cochlear amplification within the apical turn of the mouse cochlea. We measured not only classical basilar membrane vibratory tuning curves but also vibratory responses from the rest of the tissues that compose the organ of Corti. Basilar membrane tuning was sharp in live mice and broad in dead mice, whereas other regions of the organ of Corti demonstrated phase shifts consistent with additional filtering beyond that provided by basilar membrane mechanics. We use these experimental data to support a conceptual framework of how cochlear amplification is tuned within the mouse cochlear apex. We will also study transgenic mice with targeted mutations that affect different biomechanical aspects of the organ of Corti in an effort to localize the underlying processes that produce this additional filtering

Impaired nitric oxide production in children with MELAS syndrome and the effect of arginine and citrulline supplementation.

Science.gov (United States)

El-Hattab, Ayman W; Emrick, Lisa T; Hsu, Jean W; Chanprasert, Sirisak; Almannai, Mohammed; Craigen, William J; Jahoor, Farook; Scaglia, Fernando

2016-04-01

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is one of the most frequent maternally inherited mitochondrial disorders. The pathogenesis of this syndrome is not fully understood and believed to result from several interacting mechanisms including impaired mitochondrial energy production, microvasculature angiopathy, and nitric oxide (NO) deficiency. NO deficiency in MELAS syndrome is likely to be multifactorial in origin with the decreased availability of the NO precursors, arginine and citrulline, playing a major role. In this study we used stable isotope infusion techniques to assess NO production in children with MELAS syndrome and healthy pediatric controls. We also assessed the effect of oral arginine and citrulline supplementations on NO production in children with MELAS syndrome. When compared to control subjects, children with MELAS syndrome were found to have lower NO production, arginine flux, plasma arginine, and citrulline flux. In children with MELAS syndrome, arginine supplementation resulted in increased NO production, arginine flux, and arginine concentration. Citrulline supplementation resulted in a greater increase of these parameters. Additionally, citrulline supplementation was associated with a robust increase in citrulline concentration and flux and de novo arginine synthesis rate. The greater effect of citrulline in increasing NO production is due to its greater ability to increase arginine availability particularly in the intracellular compartment in which NO synthesis takes place. This study, which is the first one to assess NO metabolism in children with mitochondrial diseases, adds more evidence to the notion that NO deficiency occurs in MELAS syndrome, suggests a better effect for citrulline because of its greater role as NO precursor, and indicates that impaired NO production occurs in children as well as adults with MELAS syndrome. Thus, the initiation of treatment with NO precursors may be

[Emotional response to music by postlingually-deafened adult cochlear implant users].

Science.gov (United States)

Wang, Shuo; Dong, Ruijuan; Zhou, Yun; Li, Jing; Qi, Beier; Liu, Bo

2012-10-01

To assess the emotional response to music by postlingually-deafened adult cochlear implant users. Munich music questionnaire (MUMU) was used to match the music experience and the motivation of use of music between 12 normal-hearing and 12 cochlear implant subjects. Emotion rating test in Musical Sounds in Cochlear Implants (MuSIC) test battery was used to assess the emotion perception ability for both normal-hearing and cochlear implant subjects. A total of 15 pieces of music phases were used. Responses were given by selecting the rating scales from 1 to 10. "1" represents "very sad" feeling, and "10" represents "very happy feeling. In comparison with normal-hearing subjects, 12 cochlear implant subjects made less active use of music for emotional purpose. The emotion ratings for cochlear implant subjects were similar to normal-hearing subjects, but with large variability. Post-lingually deafened cochlear implant subjects on average performed similarly in emotion rating tasks relative to normal-hearing subjects, but their active use of music for emotional purpose was obviously less than normal-hearing subjects.

United Kingdom national paediatric bilateral cochlear implant audit: preliminary results.

Science.gov (United States)

Cullington, Helen; Bele, Devyanee; Brinton, Julie; Lutman, Mark

2013-11-01

Prior to 2009, United Kingdom (UK) public funding was mainly only available for children to receive unilateral cochlear implants. In 2009, the National Institute for Health and Care Excellence published guidance for cochlear implantation following their review. According to these guidelines, all suitable children are eligible to have simultaneous bilateral cochlear implants or a sequential bilateral cochlear implant if they had received the first before the guidelines were published. Fifteen UK cochlear implant centres formed a consortium to carry out a multi-centre audit. The audit involves collecting data from simultaneously and sequentially implanted children at four intervals: before bilateral cochlear implants or before the sequential implant, 1, 2, and 3 years after bilateral implants. The measures include localization, speech recognition in quiet and background noise, speech production, listening, vocabulary, parental perception, quality of life, and surgical data including complications. The audit has now passed the 2-year point, and data have been received on 850 children. This article provides a first view of some data received up until March 2012.

The preoperative imaging evaluation for cochlear implantation

International Nuclear Information System (INIS)

Liu Zhonglin; Wang Zhenchang; Fu Lin; Li Yong; Xian Junfang; Yang Bentao; Lan Baosen; Li Yongxin; Zheng Jun; Song Yan; Liu Bo; Chen Xueqing; He Haili

2006-01-01

Objective: To analyze CT and MRI findings of temporal bone and to evaluate preoperative diagnostic value for cochlear implantation. Methods: One hundred and sixty candidates for cochlear implantation were examined with axial CT scan, 64 of them also with coronal CT scan, and 119 patients with MRI. Results: All of 320 ears were well-aerated, and 206 ears had mastoid cavities extended posteriorly to the sigmoid sinus. The length from posterior-lateral tympanic wall to the outer cortex was (2.34±0.42) mm (left side) and (2.25±0.40) mm (right side) (U=1.887, P 1 and T 2 signal on MRI. The congenital malformations of inner ear occurred in 67 ears, including complete dysplasia in 1 ear, cochlear hypodysplasia in 6 ears, Mondini deformation in 5 ears, enlarged vestibular aqueduct in 40 ears, dysplastic semicircular canal and the vestibulae in 10 ears, and narrowing of internal auditory canal in 5 ears. Conclusion: Preoperative imaging examinations can provide critical information to ensure successful cochlear' implantation. (authors)

Tinnitus after Simultaneous and Sequential Bilateral Cochlear Implantation

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Geerte G. J. Ramakers

2017-11-01

Full Text Available ImportanceThere is an ongoing global discussion on whether or not bilateral cochlear implantation should be standard care for bilateral deafness. Contrary to unilateral cochlear implantation, however, little is known about the effect of bilateral cochlear implantation on tinnitus.ObjectiveTo investigate tinnitus outcomes 1 year after bilateral cochlear implantation. Secondarily, to compare tinnitus outcomes between simultaneous and sequential bilateral cochlear implantation and to investigate long-term follow-up (3 years.Study designThis study is a secondary analysis as part of a multicenter randomized controlled trial.MethodsThirty-eight postlingually deafened adults were included in the original trial, in which the presence of tinnitus was not an inclusion criterion. All participants received cochlear implants (CIs because of profound hearing loss. Nineteen participants received bilateral CIs simultaneously and 19 participants received bilateral CIs sequentially with an inter-implant interval of 2 years. The prevalence and severity of tinnitus before and after simultaneous and sequential bilateral cochlear implantation were measured preoperatively and each year after implantation with the Tinnitus Handicap Inventory (THI and Tinnitus Questionnaire (TQ.ResultsThe prevalence of preoperative tinnitus was 42% (16/38. One year after bilateral implantation, there was a median difference of −8 (inter-quartile range (IQR: −28 to 4 in THI score and −9 (IQR: −17 to −9 in TQ score in the participants with preoperative tinnitus. Induction of tinnitus occurred in five participants, all in the simultaneous group, in the year after bilateral implantation. Although the preoperative and also the postoperative median THI and TQ scores were higher in the simultaneous group, the median difference scores were equal in both groups. In the simultaneous group, tinnitus scores fluctuated in the 3 years after implantation. In the sequential group

Anaesthesia Management in a Patient with Waardenburg Syndrome and Review of the Literature

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Peker, Kevser; Ergil, Julide; Öztürk, İbrahim

2015-01-01

Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism. Incidence is estimated as 2%–3% among the cases of congenital deafness and 1/42,000 of the general population. Children with Waardenburg syndrome usually require anaesthesia for the cochlear implant operation in early age. The features of the syndrome that may bear importance for anaesthetic management are laryngomalacia, multiple muscle contr...

A prospective study evaluating cochlear implant management skills: development and validation of the Cochlear Implant Management Skills survey.

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Bennett, R J; Jayakody, D M P; Eikelboom, R H; Taljaard, D S; Atlas, M D

2016-02-01

To investigate the ability of cochlear implant (CI) recipients to physically handle and care for their hearing implant device(s) and to identify factors that may influence skills. To assess device management skills, a clinical survey was developed and validated on a clinical cohort of CI recipients. Survey development and validation. A prospective convenience cohort design study. Specialist hearing implant clinic. Forty-nine post-lingually deafened, adult CI recipients, at least 12 months postoperative. Survey test-retest reliability, interobserver reliability and responsiveness. Correlations between management skills and participant demographic, audiometric, clinical outcomes and device factors. The Cochlear Implant Management Skills survey was developed, demonstrating high test-retest reliability (0.878), interobserver reliability (0.972) and responsiveness to intervention (skills training) [t(20) = -3.913, P = 0.001]. Cochlear Implant Management Skills survey scores range from 54.69% to 100% (mean: 83.45%, sd: 12.47). No associations were found between handling skills and participant factors. This is the first study to demonstrate a range in cochlear implant device handling skills in CI recipients and offers clinicians and researchers a tool to systematically and objectively identify shortcomings in CI recipients' device handling skills. © 2015 John Wiley & Sons Ltd.

Low Proportion of Dietary Plant Protein among Athletes with Premenstrual Syndrome-Related Performance Impairment.

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Yamada, Keiko; Takeda, Takashi

2018-02-01

Premenstrual syndrome (PMS) is psychosomatic disorder that are limited to the late luteal phase in the menstrual cycle. PMS could impair athletic performance. To investigate associations between proportions of dietary plant and animal protein and PMS-related impairment of athletic performance, we surveyed 135 female athletes aged 18-23 years attending Kindai University. Participants belonged to authorized university clubs, all of which have high rankings in Japanese university sports. Participants completed self-administered questionnaires on diet history, demographics, and PMS-related impairment of athletic performance. Total protein, animal protein, and plant protein intake were examined, and the proportion of dietary plant protein was calculated for each participant. We divided athletes into two groups: those without PMS-related impairment of athletic performance (n = 117) and those with PMS-related performance impairment (n = 18). A t-test was used to compare mean values and multivariable adjusted mean values between groups; adjustment variables were energy intake, body mass index, and daily training duration. Total protein intake was not significantly different between the groups. However, athletes whose performance was affected by PMS reported higher intake of animal protein (mean 50.6 g) than athletes whose performance was unaffected by PMS (mean 34.9 g). Plant protein intake was lower among athletes with PMS-related impairment (mean 25.4 g) than among athletes without impairment (mean 26.9 g). The proportion of dietary plant protein was lower among athletes with PMS-related impairment (39.3%) than those without impairment (45.9%). A low proportion of dietary plant protein may cause PMS-related athletic impairment among athletes.

The Human Cochlear Mechanical Nonlinearity Inferred via Psychometric Functions

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Nizami Lance

2013-12-01

Extension of the model of Schairer and colleagues results in credible cochlear nonlinearities in man, suggesting that forward-masking provides a non-invasive way to infer the human mechanical cochlear nonlinearity.

Noise alters guinea pig's blood-labyrinth barrier ultrastructure and permeability along with a decrease of cochlear Claudin-5 and Occludin.

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Wu, Yong-Xiang; Zhu, Guo-Xia; Liu, Xin-Qin; Sun, Fei; Zhou, Ke; Wang, Shuang; Wang, Chun-Mei; Jia, Jin-Wen; Song, Jian-Tao; Lu, Lian-Jun

2014-12-24

Noise exposure (NE) is a severe modern health hazard that induces hearing impairment. However, the noise-induced ultrastructural changes of blood-labyrinth barrier (BLB) and the potential involvements of tight junction proteins (TJP) remain inconclusive. We investigated the effects of NE on not only the ultrastructure of cochlea and permeability of BLB but also the expression of TJP within the guinea pig cochlea. Male albino guinea pigs were exposed to white noise for 4 h or 2 consecutive days (115 dB sound pressure level, 6 hours per day) and the hearing impairments and light microscopic change of BLB were evaluated with auditory brainstem responses (ABR) and the cochlear sensory epithelia surface preparation, respectively. The cochlear ultrastructure and BLB permeability after NE 2d were revealed with transmission electron microscope (TEM) and lanthanum nitrate-tracing techniques, respectively. The potential alterations of TJPs Claudin-5 and Occludin were quantified with immunohistochemistry and western blot. NE induced significant hearing impairment and NE 2d contributed to significant outer hair cell (OHC) loss that is most severe in the first row of outer hair cells. Furthermore, the loosen TJ and an obvious leakage of lanthanum nitrate particles beneath the basal lamina were revealed with TEM. Moreover, a dose-dependent decrease of Claudin-5 and Occludin was observed in the cochlea after NE. All these findings suggest that both decrease of Claudin-5 and Occludin and increased BLB permeability are involved in the pathologic process of noise-induced hearing impairment; however, the causal relationship and underlying mechanisms should be further investigated.

Transcanal labyrinthectomy for intractable vertigo after unilateral cochlear implantation.

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Heidenreich, Katherine D; Basura, Gregory J; Zwolan, Teresa A; El-Kashlan, Hussam K; Telian, Steven A

2011-10-01

Document the use of transcanal labyrinthectomy to treat disabling attacks of vertigo after unilateral cochlear implantation. A 46-year-old woman with severe-profound bilateral sensorineural hearing loss secondary to enlarged vestibular aqueducts underwent cochlear implantation for her right ear with a Nucleus Freedom device. The surgery was uneventful, and postoperative imaging confirmed that the electrode was positioned properly. She developed episodic vertigo 10 to 14 days after the implant surgery, which failed to improve with aggressive vestibular rehabilitation therapy. Plugging of the round window for possible perilymphatic fistula did not relieve her symptoms. Right transcanal labyrinthectomy supplemented by filling the vestibule with gentamicin-soaked Gelfoam and then a customized vestibular rehabilitation program. Comparison of vestibular symptoms and cochlear implant performance before and after transcanal labyrinthectomy. The patient had immediate relief of symptoms, and the function of the cochlear implant was not adversely affected. Transcanal labyrinthectomy may be an effective method to ablate the vestibular end organ after unilateral cochlear implantation. It can offer relief of disabling vertigo without adversely affecting the performance of the implant.

Fourteen-years experience with cochlear implantation in Ramathibodi Hospital.

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Kasemsuwan, Lalida; Cheewaruangroj, Wichit; Tungkeeratichai, Jumroon; Bhongmakapat, Thongchai; Thawin, Cheamchit; Lertsukprasert, Krisna; Tiravanitchakul, Rattinan; Dara, Rada; Laothamatas, Jiraporn

2010-12-01

To review the cochlear implant program in Ramathibodi Hospital and share experience of cochlear implantation emphasized on clinical and surgical outcomes. Retrospective review of 143 ears (140 patients) operated with cochlear implant between 1995 and 2009. The demographic data including etiology of deafness and findings from temporal bone CTscans were reviewed. The authors' experience with cochlear implant surgery in terms of patient selection, patient advisory clinic, necessary equipment, pre- and postoperative evaluations, surgical techniques and complications were discussed. Most congenital origin was unknown etiology and congenital rubella was the most common known cause. From the CT scans of congenital deafness, vestibular aqueduct dilatation was the most common and found in 29.31% while Mondini malformation was shown to be 16.37%. The authors' surgical technique of using the pocket method and designed bony ridge at cortical mastoid rim had helped stabilizing the implant and electrode fancoil. During the last two years, no complication or revision surgery was detected. Cochlear implant surgery in both children and adults can result in good surgical outcome and fewer complications under experienced surgeons and a good team.

Cochlear injury and adaptive plasticity of the auditory cortex

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ANNA R. eFETONI

2015-02-01

Full Text Available Growing evidence suggests that cochlear stressors as noise exposure and aging can induce homeostatic/maladaptive changes in the central auditory system from the brainstem to the cortex. Studies centered on such changes have revealed several mechanisms that operate in the context of sensory disruption after insult (noise trauma, drug- or age-related injury. The oxidative stress is central to current theories of induced sensory neural hearing loss and aging, and interventions to attenuate the hearing loss are based on antioxidant agent. The present review addresses the recent literature on the alterations in hair cells and spiral ganglion neurons due to noise-induced oxidative stress in the cochlea, as well on the impact of cochlear damage on the auditory cortex neurons. The emerging image emphasizes that noise-induced deafferentation and upward spread of cochlear damage is associated with the altered dendritic architecture of auditory pyramidal neurons. The cortical modifications may be reversed by treatment with antioxidants counteracting the cochlear redox imbalance. These findings open new therapeutic approaches to treat the functional consequences of the cortical reorganization following cochlear damage.

Verbal Working Memory in Children With Cochlear Implants

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Caldwell-Tarr, Amanda; Low, Keri E.; Lowenstein, Joanna H.

2017-01-01

Purpose Verbal working memory in children with cochlear implants and children with normal hearing was examined. Participants Ninety-three fourth graders (47 with normal hearing, 46 with cochlear implants) participated, all of whom were in a longitudinal study and had working memory assessed 2 years earlier. Method A dual-component model of working memory was adopted, and a serial recall task measured storage and processing. Potential predictor variables were phonological awareness, vocabulary knowledge, nonverbal IQ, and several treatment variables. Potential dependent functions were literacy, expressive language, and speech-in-noise recognition. Results Children with cochlear implants showed deficits in storage and processing, similar in size to those at second grade. Predictors of verbal working memory differed across groups: Phonological awareness explained the most variance in children with normal hearing; vocabulary explained the most variance in children with cochlear implants. Treatment variables explained little of the variance. Where potentially dependent functions were concerned, verbal working memory accounted for little variance once the variance explained by other predictors was removed. Conclusions The verbal working memory deficits of children with cochlear implants arise due to signal degradation, which limits their abilities to acquire phonological awareness. That hinders their abilities to store items using a phonological code. PMID:29075747

Cochlear implantation for single-sided deafness and tinnitus suppression.

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Holder, Jourdan T; O'Connell, Brendan; Hedley-Williams, Andrea; Wanna, George

To quantify the potential effectiveness of cochlear implantation for tinnitus suppression in patients with single-sided deafness using the Tinnitus Handicap Inventory. The study included 12 patients with unilateral tinnitus who were undergoing cochlear implantation for single-sided deafness. The Tinnitus Handicap Inventory was administered at the patient's cochlear implant candidacy evaluation appointment prior to implantation and every cochlear implant follow-up appointment, except activation, following implantation. Patient demographics and speech recognition scores were also retrospectively recorded using the electronic medical record. A significant reduction was found when comparing Tinnitus Handicap Inventory score preoperatively (61.2±27.5) to the Tinnitus Handicap Inventory score after three months of cochlear implant use (24.6±28.2, p=0.004) and the Tinnitus Handicap Inventory score beyond 6months of CI use (13.3±18.9, p=0.008). Further, 45% of patients reported total tinnitus suppression. Mean CNC word recognition score improved from 2.9% (SD 9.4) pre-operatively to 40.8% (SD 31.7) by 6months post-activation, which was significantly improved from pre-operative scores (p=0.008). The present data is in agreement with previously published studies that have shown an improvement in tinnitus following cochlear implantation for the large majority of patients with single-sided deafness. Copyright © 2017 Elsevier Inc. All rights reserved.

Impedance and electrically evoked compound action potential (ECAP drop within 24 hours after cochlear implantation.

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Joshua Kuang-Chao Chen

Full Text Available Previous animal study revealed that post-implantation electrical detection levels significantly declined within days. The impact of cochlear implant (CI insertion on human auditory pathway in terms of impedance and electrically evoked compound action potential (ECAP variation within hours after surgery remains unclear, since at this time frequency mapping can only commence weeks after implantation due to factors associated with wound conditions. The study presented our experiences with regards to initial switch-on within 24 hours, and thus the findings about the milieus inside cochlea within the first few hours after cochlear implantation in terms of impedance/ECAP fluctuations. The charts of fifty-four subjects with profound hearing impairment were studied. A minimal invasive approach was used for cochlear implantation, characterized by a small skin incision (≈ 2.5 cm and soft techniques for cochleostomy. Impedance/ECAP was measured intro-operatively and within 24 hours post-operatively. Initial mapping within 24 hours post-operatively was performed in all patients without major complications. Impedance/ECAP became significantly lower measured within 24 hours post-operatively as compared with intra-operatively (p<0.001. There were no differences between pre-operative and post-operative threshold for air-conduction hearing. A significant drop of impedance/ECAP in one day after cochlear implantation was revealed for the first time in human beings. Mechanisms could be related to the restoration of neuronal sensitivity to the electrical stimulation, and/or the interaction between the matrix enveloping the electrodes and the electrical stimulation of the initial switch-on. Less wound pain/swelling and soft techniques both contributed to the success of immediate initial mapping, which implied a stable micro-environment inside the cochlea despite electrodes insertion. Our research invites further studies to correlate initial impedance/ECAP changes

Image Registration of Cochlear µCT Data Using Heat Distribution Similarity

DEFF Research Database (Denmark)

Kjer, Hans Martin; Vera, Sergio; Fagertun, Jens

2015-01-01

Better understanding of the anatomical variability of the human cochlear is important for the design and function of Cochlear Implants. Good non-rigid alignment of high-resolution cochlear μCT data is a challenging task. In this paper we study the use of heat distribution similarity between sampl...

Family factors, emotional functioning, and functional impairment in juvenile fibromyalgia syndrome.

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Kashikar-Zuck, Susmita; Lynch, Anne M; Slater, Shalonda; Graham, T Brent; Swain, Nicole F; Noll, Robert B

2008-10-15

Family factors and emotional functioning can play an important role in the ability of adolescents with juvenile primary fibromyalgia syndrome (JPFS) to cope with their condition and function in their everyday lives. The primary objectives of this study were to determine 1) whether adolescents with JPFS and their caregivers differed from healthy age-matched comparison peers and their caregivers in terms of emotional distress and functional impairment; 2) whether there were any differences in the family environment of adolescents with JPFS compared with healthy comparison peers; and 3) which individual-, caregiver-, and family-level variables were associated with functional impairment in adolescents with JPFS. Participants were 47 adolescents with JPFS recruited from a pediatric rheumatology clinic and 46 comparison peers without chronic illness matched for age, sex, and race. Participants and their caregivers (all mothers) completed a battery of standardized measures administered in their homes. Adolescents with JPFS had greater internalizing and externalizing symptoms than healthy comparison peers. Mothers of adolescents with JPFS reported twice as many pain conditions and significantly greater depressive symptoms than mothers of comparison peers. The JPFS group also had poorer overall family functioning and more conflicted family relationships. In adolescents with JPFS, maternal pain history was associated with significantly higher functional impairment. Increased distress and chronic pain are evident in families of adolescents with JPFS, and family relationships are also impacted. Implications for child functional impairment and the need for inclusion of caregivers in treatment are discussed.

The localization of facial motor impairment in sporadic Möbius syndrome.

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Cattaneo, L; Chierici, E; Bianchi, B; Sesenna, E; Pavesi, G

2006-06-27

To investigate the neurophysiologic aspects of facial motor control in patients with sporadic Möbius syndrome defined as nonprogressive congenital facial and abducens palsy. The authors assessed 24 patients with sporadic Möbius syndrome by performing a complete clinical examination and neurophysiologic tests including facial nerve conduction studies, needle electromyography examination of facial muscles, and recording of the blink reflex and of the trigeminofacial inhibitory reflex. Two distinct groups of patients were identified according to neurophysiologic testing. The first group was characterized by increased facial distal motor latencies (DMLs) and poor recruitment of small and polyphasic motor unit action potentials (MUAPs). The second group was characterized by normal facial DMLs and neuropathic MUAPs. It is hypothesized that in the first group, the disorder is due to a rhombencephalic maldevelopment with selective sparing of small-size MUs, and in the second group, the disorder is related to an acquired nervous injury during intrauterine life, with subsequent neurogenic remodeling of MUs. The trigeminofacial reflexes showed that in most subjects of both groups, the functional impairment of facial movements was caused by a nuclear or peripheral site of lesion, with little evidence of brainstem interneuronal involvement. Two different neurophysiologically defined phenotypes can be distinguished in sporadic Möbius syndrome, with different pathogenetic implications.

The lifestyle of patients with chronic fatigue syndrome and the effect on fatigue and functional impairments

NARCIS (Netherlands)

Goedendorp, M. M.; Knoop, H.; Schippers, G. M.; Bleijenberg, G.

2009-01-01

Background: Little is known about the lifestyle of patients with chronic fatigue syndrome (CFS) and its influence on symptoms of CFS. The present study aimed to investigate the lifestyle of patients with CFS, and to assess whether lifestyle factors are related to fatigue and functional impairments.

The lifestyle of patients with chronic fatigue syndrome and the effect on fatigue and functional impairments.

NARCIS (Netherlands)

Goedendorp, M.M.; Knoop, H.; Schippers, G.M.; Bleijenberg, G.

2009-01-01

BACKGROUND: Little is known about the lifestyle of patients with chronic fatigue syndrome (CFS) and its influence on symptoms of CFS. The present study aimed to investigate the lifestyle of patients with CFS, and to assess whether lifestyle factors are related to fatigue and functional impairments.

Modelling Cochlear Mechanics

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Guangjian Ni

2014-01-01

Full Text Available The cochlea plays a crucial role in mammal hearing. The basic function of the cochlea is to map sounds of different frequencies onto corresponding characteristic positions on the basilar membrane (BM. Sounds enter the fluid-filled cochlea and cause deflection of the BM due to pressure differences between the cochlear fluid chambers. These deflections travel along the cochlea, increasing in amplitude, until a frequency-dependent characteristic position and then decay away rapidly. The hair cells can detect these deflections and encode them as neural signals. Modelling the mechanics of the cochlea is of help in interpreting experimental observations and also can provide predictions of the results of experiments that cannot currently be performed due to technical limitations. This paper focuses on reviewing the numerical modelling of the mechanical and electrical processes in the cochlea, which include fluid coupling, micromechanics, the cochlear amplifier, nonlinearity, and electrical coupling.

Impaired driving simulation in patients with Periodic Limb Movement Disorder and patients with Obstructive Sleep Apnea Syndrome

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Gieteling, Esther W.; Bakker, Marije S.; Hoekema, Aarnoud; Maurits, Natasha M.; Brouwer, Wiebo H.; van der Hoeven, Johannes H.

Background: Excessive daytime sleepiness (EDS) is considered to be responsible for increased collision rate and impaired driving simulator performance in Obstructive Sleep Apnea Syndrome (OSAS) patients. Periodic Limb Movement Disorder (PLMD) patients also frequently report EDS and may also have

Bilateral cochlear implantation in a patient with bilateral temporal bone fractures.

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Chung, Jae Ho; Shin, Myung Chul; Min, Hyun Jung; Park, Chul Won; Lee, Seung Hwan

2011-01-01

With the emphasis on bilateral hearing nowadays, bilateral cochlear implantation has been tried out for bilateral aural rehabilitation. Bilateral sensorineural hearing loss caused by head trauma can get help from cochlear implantation. We present the case of a 44-year-old man with bilateral otic capsule violating temporal bone fractures due to head trauma. The patient demonstrated much improved audiometric and psychoacoustic performance after bilateral cochlear implantation. We believe bilateral cochlear implantation in such patient can be a very effective tool for rehabilitation. Copyright © 2011 Elsevier Inc. All rights reserved.

Cochlear nucleus neuron analysis in individuals with presbycusis.

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Hinojosa, Raul; Nelson, Erik G

2011-12-01

The aim of this study was to analyze the cochlear nucleus neuron population in individuals with normal hearing and presbycusis. Retrospective study of archival human temporal bone and brain stem tissues. Using strict inclusion criteria, the temporal bones and cochlear nuclei from six normal hearing individuals and four individuals with presbycusis were selected for analysis. The spiral ganglion cell population, the cochlear nucleus neuron population, and the cell body size of the neurons were quantified in these cases. A relationship was not observed between age and the spiral ganglion cell population in the normal hearing group. Presbycusis subjects exhibited a reduced spiral ganglion cell population. The mean cochlear nucleus neuron population was observed to be significantly higher in the presbycusis group (mean ± standard deviation: 114,170 ± 10,570) compared to the normal hearing group (91,470 ± 9,510) (P = .019). This difference was predominantly the result of greater multipolar and granule cell neuron populations. Only the fusiform neuron type exhibited a significantly different mean cell body cross-sectional area between the normal hearing group (242 ± 27) and the presbycusis group (300 ± 37) (P = .033). This investigation is the first time, to our knowledge, that the populations of the eight neuron types in the cochlear nucleus have been quantified in both normal hearing individuals and individuals with presbycusis. The data support the concept that presbycusis is not an effect of aging alone but instead may be a condition that predisposes one to hearing loss with advancing age and is characterized by a congenitally elevated cochlear nucleus neuron population. Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.

Threshold shift: effects of cochlear implantation on the risk of pneumococcal meningitis.

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Wei, Benjamin P C; Shepherd, Robert K; Robins-Browne, Roy M; Clark, Graeme M; O'Leary, Stephen J

2007-04-01

The study goals were to examine whether cochlear implantation increases the risk of meningitis in the absence of other risk factors and to understand the pathogenesis of pneumococcal meningitis post cochlear implantation. Four weeks following surgery, 54 rats (18 of which received a cochleostomy alone, 18 of which received a cochleostomy and acute cochlear implantation using standard surgical techniques, and 18 of which received a cochlear implant) were infected with Streptococcus pneumoniae via three different routes of bacterial inoculation (middle ear, inner ear, and intraperitoneal) to represent all potential routes of bacterial infection from the upper respiratory tract to the meninges. The presence of a cochlear implant reduced the threshold of bacteria required to cause pneumococcal meningitis from all routes of infection in healthy animals. The presence of a cochlear implant increases the risk of pneumococcal meningitis regardless of the route of bacterial infection. Early detection and treatment of pneumococcal infection such as otitis media may be required, as cochlear implantation may lead to a reduction of infectious threshold for meningitis.

Testicular dysgenesis syndrome comprises some but not all cases of hypospadias and impaired spermatogenesis

DEFF Research Database (Denmark)

Jørgensen, N; Rajpert-De Meyts, Ewa; Main, K M

2010-01-01

In 2001, when the testicular dysgenesis syndrome (TDS) concept was proposed, it suggested that impaired development of foetal testes could lead to increased risks of cryptorchidism, hypospadias, decreased spermatogenesis or testis cancer. The TDS concept links the pathogenesis of the four disorders...... with cryptorchidism or TGCC. By contrast, recent studies demonstrated that among men with isolated hypospadias, only a fraction of cases are linked to TDS. There is no doubt that TDS contributes to impaired semen quality. This is most obvious for cases with visible dysgenetic features in testis histology......, but in the majority of men with impaired semen quality as the only symptom, an association with TDS is less clear. Such cases have a very heterogeneous aetiology and may be caused by a host of other - often post-natal-factors. In conclusion, the TDS as a holistic concept has inspired new research activities and led...

Scala vestibuli cochlear implantation in patients with partially ossified cochleas.

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Berrettini, Stefano; Forli, Francesca; Neri, Emanuele; Segnini, Giovanni; Franceschini, Stefano Sellari

2002-11-01

Partial cochlear obstruction is a relatively common finding in candidates for cochlear implants and frequently involves the inferior segment of the scala tympani in the basal turn of the cochlea. In such patients, the scala vestibuli is often patent and offers an alternative site for implantation. The current report describes two patients with such partial obstruction of the inferior segment of the basal cochlear turn, caused in one case by systemic vasculitis (Takayasu's disease) and in the other by obliterative otosclerosis. A scala vestibuli implantation allowed for complete insertion of the electrode array. No problems were encountered during the surgical procedures and the good post-operative hearing and communicative outcomes achieved were similar to those reported in patients without cochlear ossification. The importance of accurate pre-operative radiological study of the inner ear is underscored, to disclose the presence and define the features of the cochlear ossification and ultimately to properly plan the surgical approach.

Impaired spatial body representation in complex regional pain syndrome type 1 (CRPS I).

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Reinersmann, Annika; Landwehrt, Julia; Krumova, Elena K; Ocklenburg, Sebastian; Güntürkün, Onur; Maier, Christoph

2012-11-01

Recently, a shift of the visual subjective body midline (vSM), a correlate of the egocentric reference frame, towards the affected side was reported in patients with complex regional pain syndrome (CRPS). However, the specificity of this finding is as yet unclear. This study compares 24 CRPS patients to 21 patients with upper limb pain of other origin (pain control) and to 24 healthy subjects using a comprehensive test battery, including assessment of the vSM in light and dark, line bisection, hand laterality recognition, neglect-like severity symptoms, and motor impairment (disability of the arm, shoulder, and hand). 1-way analysis of variance, t-tests, significance level: 0.05. In the dark, CRPS patients displayed a significantly larger leftward spatial bias when estimating their vSM, compared to pain controls and healthy subjects, and also reported lower motor function than pain controls. For right-affected CRPS patients only, the deviation of the vSM correlated significantly with the severity of distorted body perception. Results confirm previous findings of impaired visuospatial perception in CRPS patients, which might be the result of the involvement of supraspinal mechanisms in this pain syndrome. These mechanisms might accentuate the leftward bias that results from a right-hemispheric dominance in visuospatial processing and is known as pseudoneglect. Pseudoneglect reveals itself in the tendency to perceive the midpoint of horizontal lines or the subjective body midline left of the centre. It was observable in all 3 groups, but most pronounced in CRPS patients, which might be due to the cortical reorganisation processes associated with this syndrome. Copyright © 2012 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.

Production of verb tenses in children with cochlear implants

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Sokolovac Ivana

2016-01-01

Full Text Available The production of verb tenses leads to better language development of children with cochlear implants. The aim of this study was to assess the acquisition of verb tenses in children with cochlear implants. The sample included 60 children, aged from 9 to 15, with average intellectual abilities. The study group consisted of 30 patients with cochlear implants, with no additional disabilities. The control group consisted of 30 subjects with typical speech - language development and preserved hearing. The acquisition of basic tenses was assessed by 'Corpus for the Assessment of the Use of Tenses' (Dimić, 2003. Significant statistical differences were found in the use of the present tense in children with cochlear implants and hearing children (t=-4.385; p<0.001 as well as in the use of the past tense (t=-4.650; p<0.001, and the future tense (t=-4.269; p<0.001. There was also a significant difference in the use of irregular verb 'go' (t=-3.958; p<0.001, as well as in the combination of the present and the past tense (t=-5.806; p<0.001. The present tense was used correctly by most children with cochlear implants (70%, followed by the past tense (53%, and finally the future tense (23%. Children with cochlear implants, even after several years of re/habilitation, do not reach the grammatical development of children with normal hearing.

Hair Barrette Induced Cochlear Implant Receiver Stimulator Site Infection with Extrusion

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Trung N. Le

2015-01-01

Full Text Available Background. Cochlear implant infections and extrusion are uncommon but potentially devastating complications. Recent literature suggests conservative management can be employed. Local measures inclusive of aggressive surgical debridement with vascularized flaps and parenteral antibiotics represent a viable option and often permit device salvage. However, explantation should be considered if there is evidence of systemic, intracranial, or intractable infection. Method. A Case report and literature review. Case Report. This case illustrates a complicated local wound infection associated with cochlear implantation due to transcutaneous adherence of a ferrous hair barrette to a cochlear implant magnet. Reconstruction of computed tomography (CT data with 3D volume rendering significantly improved the value of the images and facilitated patient counseling as well as operative planning. Conclusion. Cochlear implant infections can be associated with foreign bodies. CT images are beneficial in the evaluation of cochlear implant complications. 3D CT images provide a comprehensive view of the site of interest, displaying the relationship of the hardware to the skull and soft tissues, while minimizing associated artifacts. Cochlear implant patients should consider use of nonmetallic hair devices.

Prevalence of inner ear anomalies among cochlear implant candidates.

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Aldhafeeri, Ahmad M; Alsanosi, Abdulrahman A

2016-10-01

To determine the prevalence of inner ear anomalies and the frequency of different anomaly types among cochlear implant recipients. This study included a retrospective chart review of all patients who received cochlear implants between January 2009 and January 2013 in King Abdulaziz University Hospital cochlear implant program in Riyadh, Saudi Arabia. All subjects underwent thin-cut CT of the temporal bone and MRI. The collected data included age, gender, and CT and MRI findings regarding temporal bone anomalies. Patients with any identified congenital inner ear anomalies were included in the study.  In total, 316 patients' cases were reviewed. Inner ear malformations were identified in 24 patients, which represented a prevalence of 7.5%. Among these 24 patients, 8 (33.3%) presented with a large vestibular aqueduct (LVA), 8 (33.3%) semicircular canal (SCC) dysplasia, 7 (29.1%) classical Mondini deformity, and one (4.1%) cochlear hypoplasia. The prevalence of inner ear anomalies among cochlear implant recipients was 7.5%. This result is consistent with findings worldwide. The most common anomalies were LVA and SCC hypoplasia; by contrast, in other regions, the most common anomaly is either the Mondini deformity, or LVA.

Hyperbaric oxygen upregulates cochlear constitutive nitric oxide synthase

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Kao Ming-Ching

2011-02-01

Full Text Available Abstract Background Hyperbaric oxygen therapy (HBOT is a known adjuvant for treating ischemia-related inner ear diseases. Controversies still exist in the role of HBOT in cochlear diseases. Few studies to date have investigated the cellular changes that occur in inner ears after HBOT. Nitric oxide, which is synthesized by nitric oxide synthase (NOS, is an important signaling molecule in cochlear physiology and pathology. Here we investigated the effects of hyperbaric oxygen on eardrum morphology, cochlear function and expression of NOS isoforms in cochlear substructures after repetitive HBOT in guinea pigs. Results Minor changes in the eardrum were observed after repetitive HBOT, which did not result in a significant hearing threshold shift by tone burst auditory brainstem responses. A differential effect of HBOT on the expression of NOS isoforms was identified. Upregulation of constitutive NOS (nNOS and eNOS was found in the substructures of the cochlea after HBOT, but inducible NOS was not found in normal or HBOT animals, as shown by immunohistochemistry. There was no obvious DNA fragmentation present in this HBOT animal model. Conclusions The present evidence indicates that the customary HBOT protocol may increase constitutive NOS expression but such upregulation did not cause cell death in the treated cochlea. The cochlear morphology and auditory function are consequently not changed through the protocol.

Exploring the experiences of teenagers with cochlear implants.

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Watson, Victoria; Verschuur, Carl; Lathlean, Judith

2016-11-01

Teenage cochlear implant users' perceptions of deafness, surgery, fitting of the device and life as a cochlear implant wearer were explored in order to gain a more comprehensive understanding of teenagers' experiences of living with the device. Semi-structured in-depth interviews were undertaken and analysed using thematic analysis. Ten teenagers aged 14-16 years with at least one cochlear implant were interviewed. Seven teenagers experienced great pre-operative anxiety and two reported significant post-operative pain. Four of the teenagers described a mismatch between their expectations and the disappointing reality of adjusting to the device. However, all the teenagers reported an enhanced sense of well-being as a result of being able to interact more easily with their world around them. The teenagers differed in the extent to which they identified with the hearing and deaf world. Despite the early challenges, over time the teenagers experienced many functional and psychosocial benefits. Most felt their lives were now easier as a result of the cochlear implant(s). They described complex, flexible identities. By giving prominence to the teenagers' voices this study has added new knowledge concerning their experience of surgery. The findings also more fully revealed the challenges of adjusting to the device and the impact of having a cochlear implant on the teenagers' identities. Clinical recommendations are made to address the gaps in service highlighted by these findings.

Using Discrete Trial Training to Identify Specific Learning Impairments in Boys with Fragile X Syndrome

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Hall, Scott S.; Hustyi, Kristin M.; Hammond, Jennifer L.; Hirt, Melissa; Reiss, Allan L.

2014-01-01

We examined whether "discrete trial training" (DTT) could be used to identify learning impairments in mathematical reasoning in boys with fragile X syndrome (FXS). Boys with FXS, aged 10-23 years, and age and IQ-matched controls, were trained to match fractions to pie-charts and pie-charts to decimals either on a computer or with a…

Impaired glucose tolerance, type 2 diabetes and metabolic syndrome in polycystic ovary syndrome: a systematic review and meta-analysis.

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Moran, Lisa J; Misso, Marie L; Wild, Robert A; Norman, Robert J

2010-01-01

BACKGROUND Polycystic ovary syndrome (PCOS) is a common condition in reproductive-aged women associated with impaired glucose tolerance (IGT), type 2 diabetes mellitus (DM2) and the metabolic syndrome. METHODS A literature search was conducted (MEDLINE, CINAHL, EMBASE, clinical trial registries and hand-searching) identifying studies reporting prevalence or incidence of IGT, DM2 or metabolic syndrome in women with and without PCOS. Data were presented as odds ratio (OR) [95% confidence interval (CI)] with fixed- and random-effects meta-analysis by Mantel-Haenszel methods. Quality testing was based on Newcastle-Ottawa Scaling and The Cochrane Collaboration's risk of bias assessment tool. Literature searching, data abstraction and quality appraisal were performed by two investigators. RESULTS A total of 2192 studies were reviewed and 35 were selected for final analysis. Women with PCOS had increased prevalence of IGT (OR 2.48, 95% CI 1.63, 3.77; BMI-matched studies OR 2.54, 95% CI 1.44, 4.47), DM2 (OR 4.43, 95% CI 4.06, 4.82; BMI-matched studies OR 4.00, 95% CI 1.97, 8.10) and metabolic syndrome (OR 2.88, 95% CI 2.40, 3.45; BMI-matched studies OR 2.20, 95% CI 1.36, 3.56). One study assessed IGT/DM2 incidence and reported no significant differences in DM2 incidence (OR 2.07, 95% CI 0.68, 6.30). One study assessed conversion from normal glucose tolerance to IGT/DM2 (OR 2.4, 95% CI 0.7, 8.0). No studies reported metabolic syndrome incidence. CONCLUSIONS Women with PCOS had an elevated prevalence of IGT, DM2 and metabolic syndrome in both BMI and non-BMI-matched studies. Few studies have determined IGT/DM2 or metabolic syndrome incidence in women with and without PCOS and further research is required.

Coding strategies for cochlear implants under adverse environments

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Tahmina, Qudsia

Cochlear implants are electronic prosthetic devices that restores partial hearing in patients with severe to profound hearing loss. Although most coding strategies have significantly improved the perception of speech in quite listening conditions, there remains limitations on speech perception under adverse environments such as in background noise, reverberation and band-limited channels, and we propose strategies that improve the intelligibility of speech transmitted over the telephone networks, reverberated speech and speech in the presence of background noise. For telephone processed speech, we propose to examine the effects of adding low-frequency and high- frequency information to the band-limited telephone speech. Four listening conditions were designed to simulate the receiving frequency characteristics of telephone handsets. Results indicated improvement in cochlear implant and bimodal listening when telephone speech was augmented with high frequency information and therefore this study provides support for design of algorithms to extend the bandwidth towards higher frequencies. The results also indicated added benefit from hearing aids for bimodal listeners in all four types of listening conditions. Speech understanding in acoustically reverberant environments is always a difficult task for hearing impaired listeners. Reverberated sounds consists of direct sound, early reflections and late reflections. Late reflections are known to be detrimental to speech intelligibility. In this study, we propose a reverberation suppression strategy based on spectral subtraction to suppress the reverberant energies from late reflections. Results from listening tests for two reverberant conditions (RT60 = 0.3s and 1.0s) indicated significant improvement when stimuli was processed with SS strategy. The proposed strategy operates with little to no prior information on the signal and the room characteristics and therefore, can potentially be implemented in real-time CI

Phenotypic and molecular assessment of seven patients with 6p25 deletion syndrome: Relevance to ocular dysgenesis and hearing impairment

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Ritch Robert

2004-06-01

Full Text Available Abstract Background Thirty-nine patients have been described with deletions involving chromosome 6p25. However, relatively few of these deletions have had molecular characterization. Common phenotypes of 6p25 deletion syndrome patients include hydrocephalus, hearing loss, and ocular, craniofacial, skeletal, cardiac, and renal malformations. Molecular characterization of deletions can identify genes that are responsible for these phenotypes. Methods We report the clinical phenotype of seven patients with terminal deletions of chromosome 6p25 and compare them to previously reported patients. Molecular characterization of the deletions was performed using polymorphic marker analysis to determine the extents of the deletions in these seven 6p25 deletion syndrome patients. Results Our results, and previous data, show that ocular dysgenesis and hearing impairment are the two most highly penetrant phenotypes of the 6p25 deletion syndrome. While deletion of the forkhead box C1 gene (FOXC1 probably underlies the ocular dysgenesis, no gene in this region is known to be involved in hearing impairment. Conclusions Ocular dysgenesis and hearing impairment are the two most common phenotypes of 6p25 deletion syndrome. We conclude that a locus for dominant hearing loss is present at 6p25 and that this locus is restricted to a region distal to D6S1617. Molecular characterization of more 6p25 deletion patients will aid in refinement of this locus and the identification of a gene involved in dominant hearing loss.

Verbal Working Memory in Children with Cochlear Implants

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Nittrouer, Susan; Caldwell-Tarr, Amanda; Low, Keri E.; Lowenstein, Joanna H.

2017-01-01

Purpose: Verbal working memory in children with cochlear implants and children with normal hearing was examined. Participants: Ninety-three fourth graders (47 with normal hearing, 46 with cochlear implants) participated, all of whom were in a longitudinal study and had working memory assessed 2 years earlier. Method: A dual-component model of…

Working memory in Farsi-speaking children with normal development and cochlear implant.

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Soleymani, Zahra; Amidfar, Meysam; Dadgar, Hooshang; Jalaie, Shohre

2014-04-01

Working memory has an important role in language acquisition and development of cognition skills. The ability of encoding, storage and retrieval of phonological codes, as activities of working memory, acquired by audition sense. Children with cochlear implant experience a period that they are not able to perceive sounds. In order to assess the effect of hearing on working memory, we investigated working memory as a cognition skill in children with normal development and cochlear implant. Fifty students with normal hearing and 50 students with cochlear implant aged 5-7 years participated in this study. Children educated in the preschool, the first and second grades. Children with normal development were matched based on age, gender, and grade of education with cochlear implant. Two components of working memory including phonological loop and central executive were compared between two groups. Phonological loop assessed by nonword repetition task and forward digit span. To assess central executive component backward digit span was used. The developmental trend was studied in children with normal development and cochlear implant as well. The effect of age at implantation in children with cochlear implants on components of working memory was investigated. There are significant differences between children with normal development and cochlear implant in all tasks that assess working memory (p memory between different grades showed significant differences both in children with normal development and in children with cochlear implant (p implied that children with cochlear implant may experience difficulties in working memory. Therefore, these children have problems in encoding, practicing, and repeating phonological units. The results also suggested working memory develops when the child grows up. In cochlear implant children, with decreasing age at implantation and increasing their experience in perceiving sound, working memory skills improved. Copyright © 2014 Elsevier

Patient-specific estimation of detailed cochlear shape from clinical CT images

DEFF Research Database (Denmark)

Kjer, H Martin; Fagertun, Jens; Wimmer, Wilhelm

2018-01-01

of the detailed patient-specific cochlear shape from CT images. From a collection of temporal bone [Formula: see text]CT images, we build a cochlear statistical deformation model (SDM), which is a description of how a human cochlea deforms to represent the observed anatomical variability. The model is used...... for regularization of a non-rigid image registration procedure between a patient CT scan and a [Formula: see text]CT image, allowing us to estimate the detailed patient-specific cochlear shape. We test the accuracy and precision of the predicted cochlear shape using both [Formula: see text]CT and CT images...

Clinical peculiarities of antibiotic associated bowels impairment and its significance in irritable bowel syndrome appearance

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І. O. Pasichna

2016-06-01

Full Text Available Aim: the main objective of this study was to investigate bowels impairment due to treatment with antibiotics, its incidence and clinical peculiarities; to evaluate its role in appearance of in irritable bowel syndrome. Material and Methods. We studied 110 patients (33 males and 77 females, age range 16-83 years, who received treatment with antibiotic. We evaluated the function of the intestine before treatment with antibiotic, then in 1 week, 3 months after treatment finish (1, 2, 3, 4 visits respectively. Control group included 20 healthy persons, who haven't had antibiotics administered during recent two years. Results. We revealed that the signs of bowel function impairment took place at the first visit in 18.2% of patients, at the second visit – in 60.0% of patients, at the third visit – in 45.5% of patients and at the fourth visit – in 41.1% of patients. At the second, third and fourth visits the signs of bowels function impairment were observed reliably more often then at the first visit (before antibiotic administration, p<0.001. At the second visit the signs bowels function disorders were the most prominent: abdominal pain – in 44.5%, distention – in 46.4%, diarrhea – in 29.1%, constipation – in 18.2%, presence of both (diarrhea and periodically constipation manifestations – in 2.7%; and extraintestinal manifestations (depression. depressed mood, sorrow, apathy, decreased stamina, sleep disturbances – in 29.1% of patients. Clinical manifestations of irritable bowel syndrome occured in 6 months of observation in 32.2% of patients. Conclusions. The signs of bowel function impairment were observed in 60.0% of patients after finishing treatment with antibiotic. This incidence is much higher than in control group (р<0.001. Bowel disorders mostly manifested as the changes in quantity and consistency of feaces, pain, abdominal distention and extraintestinal manifestations. In 32.2% of patients clinical manifestations of

Mondini deformity in a case of Turner syndrome. A radiological finding.

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Bodet Agustí, Eduard; Galido Ortego, Xavier; Ghani Martínez, Fares; García González, Begoña; Borràs Perera, Montserrat; Seara Gil, Angel

2012-01-01

Turner syndrome (TS) is the human being's most frequent sex chromosome abnormality. Progressive sensorineural hearing loss is documented in more than 50% of the women affected by this syndrome. Although Mondini defect is the cochlear congenital malformation most frequently identified in other polymalformative syndromes, it has rarely been reported in TS. We describe the case of a 32-year-old woman with TS who presented progressive sensorineural hearing loss. The computed tomography of the ears showed bilateral Mondini deformity. Copyright © 2010 Elsevier España, S.L. All rights reserved.

A combination of two truncating mutations in USH2A causes more severe and progressive hearing impairment in Usher syndrome type IIa

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Hartel, B.P.; Lofgren, M.; Huygen, P.L.; Guchelaar, I.; Lo, A.N.K.N.; Sadeghi, A.M.; van Wijk, E.; Tranebjaerg, L.; Kremer, H.; Kimberling, W.J.; Cremers, C.W.R.J.; Moller, C.; Pennings, R.J.

2016-01-01

OBJECTIVES: Usher syndrome is an inherited disorder that is characterized by hearing impairment (HI), retinitis pigmentosa, and in some cases vestibular dysfunction. Usher syndrome type IIa is caused by mutations in USH2A. HI in these patients is highly heterogeneous and the present study evaluates

Challenges in Improving Cochlear Implant Performance and Accessibility.

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Zeng, Fan-Gang

2017-08-01

Here I identify two gaps in cochlear implants that have been limiting their performance and acceptance. First, cochlear implant performance has remained largely unchanged, despite the number of publications tripling per decade in the last 30 years. Little has been done so far to address a fundamental limitation in the electrode-to-neuron interface, with the electrode size being a thousand times larger than the neuron diameter while the number of electrodes being a thousand times less. Both the small number and the large size of electrodes produce broad spatial activation and poor frequency resolution that limit current cochlear implant performance. Second, a similarly rapid growth in cochlear implant volume has not produced an expected decrease in unit price in the same period. The high cost contributes to low market penetration rate, which is about 20% in developed countries and less than 1% in developing countries. I will discuss changes needed in both research strategy and business practice to close the gap between prosthetic and normal hearing as well as that between haves and have-nots.

Systematic review: Radiological and histological evidence of cochlear implant insertion trauma in adult patients.

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Hoskison, Emma; Mitchell, Scott; Coulson, Chris

2017-07-01

Cochlear implantation (CI) has developed from its origins in the 1980s. Initially, CI was for profound bilateral hearing impairment. However, candidacy for CI have become more widespread in recent years with unilateral implantation and an emphasis on hearing preservation. Evidence supports full electrode insertion in an atraumatic fashion into the scala tympani (ST) provides optimal hearing outcomes. The main aim of this systematic review was to elucidate the degree of trauma associated with CI insertion. A systematic literature search was undertaken using PubMed Medline. A grading system described by Eshraghi was used to classify cochlear trauma. Both radiological and histological studies were included. Twenty one papers were identified which were relevant to our search. In total, 653 implants were inserted and 115 (17.6%) showed evidence of trauma. The cochleas with trauma had basilar membrane elevation in 5.2%, ruptured in 5.2%, the electrode passed from the ST to the SV in 84.4% and there was grade 4 trauma in 5.2%. The studies used a variety of histological and radiological methods to assess for evidence of trauma in both cadaveric temporal bones and live recipients. Minimizing cochlear trauma during implant insertion is important to preserve residual hearing and optimize audiological performance. An overall 17.6% trauma rate suggests that CI insertion could be improved with more accurate and consistent electrode insertion such as in the form of robotic guidance. The correlation of cochlea trauma with post-operative hearing has yet to be determined.

Visual impairment evaluation in 119 children with congenital Zika syndrome.

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Ventura, Liana O; Ventura, Camila V; Dias, Natália de C; Vilar, Isabelle G; Gois, Adriana L; Arantes, Tiago E; Fernandes, Luciene C; Chiang, Michael F; Miller, Marilyn T; Lawrence, Linda

2018-06-01

To assess visual impairment in a large sample of infants with congenital Zika syndrome (CZS) and to compare with a control group using the same assessment protocol. The study group was composed of infants with confirmed diagnosis of CZS. Controls were healthy infants matched for age, sex, and socioeconomic status. All infants underwent comprehensive ophthalmologic evaluation including visual acuity, visual function assessment, and visual developmental milestones. The CZS group included 119 infants; the control group, 85 infants. At examination, the mean age of the CZS group was 8.5 ± 1.2 months (range, 6-13 months); of the controls, 8.4 ± 1.8 months (range, 5-12 months; P = 0.598). Binocular Teller Acuity Card (TAC) testing was abnormal in 107 CZS infants and in 4 controls (89.9% versus 5% [P visual development milestones were less achieved by infants with CZS compared to controls (P visual impairment. A protocol for assessment of the ocular findings, visual acuity, and visual developmental milestones tested against age-matched controls is suggested. Copyright © 2018 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

The role of temporomandibular joint dysfunction and occlusal disorders in the pathophysiology of somatogenic cochlear and vestibular syndrome

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A. V. Boldin

2016-01-01

Full Text Available Rationale: Temporomandibular joint (TMJ dysfunction and occlusion abnormalities can cause cochlear and vestibular disorders. This issue is at the crossroads of several disciplines: otoneurology, physiotherapy, dentistry, medical rehabilitation and posturology, which often makes it difficult to timely diagnose them and delays the onset of treatment. Aim: To assess the role of abnormal dental occlusion and TMJ disorders in the pathophysiology and clinical manifestation of cochleovestibular syndrome. Materials and methods: We examined 300 subjects with clinical signs of cochleovestibular syndrome, asymmetry of occlusion and/or TMJ dysfunction (the main group, 55 patients with signs of TMJ structural and functional disorders and occlusal disorders without a cochleovestibular syndrome (the reference group, and 35 healthy volunteers (the control group. All patients were examined by a neurologist, an ENT specialist, a dentist and a physiotherapist. A series of additional investigations of the brachiocephalic vessels, cervical spine, TMJ, auditory and vestibular function, premature tooth contacts were performed. Results: The main group patients had high values of TMJ dysfunction in the Hamburg test (5.85 vs 2.2 in the reference group and higher proportions of patients with moderate and severe TMJ dysfunction (n = 243, 81% and n = 13, 23.7%, respectively. The functional muscle test parameters and the results of manual muscle testing in the main group patients were significantly different from those in the control group (р < 0.05, whereas most values obtained in the reference group did not differ significantly (р > 0.05. Patients with cochleoves-tibular syndrome had 2 to 3-fold higher rates of vertebrogenic dysfunctions than those from the reference group. The video nystamography technique detected the positional cervical nystagmus in 100% (n = 300 of patients from the main group, whereas there were no nystagmus in those from the reference group

Pneumococcal meningitis post-cochlear implantation: preventative measures.

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Wei, Benjamin P C; Shepherd, Robert K; Robins-Browne, Roy M; Clark, Graeme M; O'Leary, Stephen J

2010-11-01

Both clinical data and laboratory studies demonstrated the risk of pneumococcal meningitis post-cochlear implantation. This review examines strategies to prevent post-implant meningitis. Medline/PubMed database; English articles after 1980. Search terms: cochlear implants, pneumococcus meningitis, streptococcus pneumonia, immunization, prevention. Narrative review. All articles relating to post-implant meningitis without any restriction in study designs were assessed and information extracted. The presence of inner ear trauma as a result of surgical technique or cochlear implant electrode array design was associated with a higher risk of post-implant meningitis. Laboratory data demonstrated the effectiveness of pneumococcal vaccination in preventing meningitis induced via the hematogenous route of infection. Fibrous sealing around the electrode array at the cochleostomy site, and the use of antibiotic-coated electrode array reduced the risk of meningitis induced via an otogenic route. The recent scientific data support the U.S. Food and Drug Administration recommendation of pneumococcal vaccination for the prevention of meningitis in implant recipients. Nontraumatic cochlear implant design, surgical technique, and an adequate fibrous seal around the cochleostomy site further reduce the risk of meningitis. Copyright © 2010 American Academy of Otolaryngology–Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.

Multichannel cochlear implantation in the scala vestibuli.

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Lin, Karen; Marrinan, Michelle S; Waltzman, Susan B; Roland, J Thomas

2006-08-01

Sensorineural hearing loss resulting from otosclerosis, meningitis, chronic otitis media, autoimmune ear disease, and trauma can be associated with partial or total obstruction of the cochlear scalae. Multichannel cochlear implantation may be difficult in a cochlea with an obstructed scala tympani. The purpose of this study is to determine the safety and efficacy of scala tympani electrode insertion. Retrospective chart review. Academic medical center. Eight children and adults with profound sensorineural hearing loss who underwent cochlear implantation with known scala vestibuli electrode array insertion were subjects for this study. Eight study subjects underwent implantation: five with the Nucleus 24RCS (Contour) device and three with the Nucleus 24M device. Imaging findings, operative findings, and age-appropriate speech perception testing. All patients had full electrode insertion. Various obstructive patterns on computed tomography and magnetic resonance imaging were found, and there was a range of speech perception results. All but one patient improved based on age-appropriate monosyllabic word and sentence tests. Scala vestibuli multielectrode insertion is a viable alternative when scala tympani insertion is not possible because of abnormal anatomy or anatomical changes secondary to disease or previous implantation. We will also present an algorithm of options for decision making for implantation when encountering cochlear obstruction and difficult electrode insertion.

Hearing aid and cochlear implant use in children with hearing loss at three years of age: Predictors of use and predictors of changes in use

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Marnane, Vivienne; Ching, Teresa YC

2015-01-01

Objective To examine usage patterns of hearing aids and cochlear implants in children up to three years of age, how usage changes longitudinally, and factors associated with device usage. Design Parent report and Parent’s Evaluation of Aural/oral performance of Children (PEACH) data were obtained at six and twelve months after hearing-aid fitting or cochlear implant switch-on, and again at three years of age. The effect of device use on auditory functional performance was investigated using the PEACH questionnaire. Study sample Four hundred and thirteen participants from the Longitudinal Outcomes of Children with Hearing Impairment (LOCHI) study were included for analysis. Result For users of hearing aids, higher usage at three years was associated with higher maternal education, and more severe hearing loss. For users of cochlear implants, higher usage was associated with higher maternal education and the absence of additional disabilities. Higher PEACH scores was associated with higher usage scores. After allowing for the effects of demographic characteristics, device use was not a significant predictor of functional performance. Conclusions Sixty-two percent of children achieved consistent use (>75% of waking hours) within the first year of receiving a hearing aid or a cochlear implant, and 71% by three years of age. PMID:25816866

Cochlear Implants and Psychiatric Assessments: a Norrie Disease Case Report.

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Jacques, Denis; Dubois, Thomas; Zdanowicz, Nicolas; Gilain, Chantal; Garin, Pierre

2017-09-01

It is important to perform psychiatric assessments of adult patients who are candidates for cochlear implants both to screen them for psychiatric disorders and to assess their understanding and compliance with the procedure. Deafness is a factor of difficulty for conducting in-depth psychiatric interviews, but concomitant blindness may make it impossible. After a description of Norrie disease, a rare disease in which blindness and deafness may occur together, we propose a case report of a patient suffering from the disease and who consulted in view of a cochlear implant. Early information on cochlear implants appears to be necessary before total deafness occurs in patients suffering from Norrie disease. An inventory of digital communication tools that can be used by the patient is also highly valuable. Research should be supported for a more systematic use of psychiatric assessments prior to cochlear implants. In the special case of Norrie disease, we recommend early screening for mental retardation and related psychotic disorders and, depending on the patient's level of understanding, preventive information on the benefits and limits of cochlear implants before total deafness occurs.

Quality of life and adjustment in children and adolescents with Moebius syndrome: Evidence for specific impairments in social functioning.

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Strobel, Linda; Renner, Gerolf

2016-01-01

Moebius syndrome is a rare congenital disorder characterized by congenital facial paralysis and impairment of ocular abduction. The absence of facial expression in people with Moebius syndrome may impair social interaction. The present study aims at replicating prior findings on psychological adjustment in children and adolescents with Moebius syndrome and providing first data on quality of life. Parents (n=26) and children and adolescents (n=14) with Moebius-Syndrome completed the KINDL(R) (Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents), the SDQ (Strengths and Difficulties Questionnaire), and a newly devised questionnaire on the global, social, and emotional impact of Moebius syndrome, including a scale that addressed what conjectures were made concerning the thoughts of other people about a child with Moebius syndrome. In comparison with normative data, quality of life was reduced for the subscale Friends (large effect for both parental ratings and self-report) and the Total Score of the KINDL(R). Parents reported elevated levels of peer problems on the respective subscale of the SDQ. In SDQ self-report data, cases classified as abnormal were observed on Peer Problems only. Moebius-specific scales showed adequate reliabilities, and were related, most notably for parent-report, to quality of life. 29% of children reported feelings of anger when being stared at, and wished that they were not affected by Moebius syndrome. Although most children and adolescents in our sample did not show behavioral problems or reduced quality of life, our results indicate that their emotional and social development should be closely monitored. Maintaining satisfying peer relationships seems to be a special challenge for children and adolescents with Moebius syndrome. Copyright © 2016 Elsevier Ltd. All rights reserved.

Examining the Language Phenotype in Children with Typical Development, Specific Language Impairment, and Fragile X Syndrome

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Haebig, Eileen; Sterling, Audra; Hoover, Jill

2016-01-01

Purpose: One aspect of morphosyntax, finiteness marking, was compared in children with fragile X syndrome (FXS), specific language impairment (SLI), and typical development matched on mean length of utterance (MLU). Method: Nineteen children with typical development (mean age = 3.3 years), 20 children with SLI (mean age = 4.9 years), and 17 boys…

Peripheral auditory processing and speech reception in impaired hearing

DEFF Research Database (Denmark)

Strelcyk, Olaf

One of the most common complaints of people with impaired hearing concerns their difficulty with understanding speech. Particularly in the presence of background noise, hearing-impaired people often encounter great difficulties with speech communication. In most cases, the problem persists even...... if reduced audibility has been compensated for by hearing aids. It has been hypothesized that part of the difficulty arises from changes in the perception of sounds that are well above hearing threshold, such as reduced frequency selectivity and deficits in the processing of temporal fine structure (TFS......) at the output of the inner-ear (cochlear) filters. The purpose of this work was to investigate these aspects in detail. One chapter studies relations between frequency selectivity, TFS processing, and speech reception in listeners with normal and impaired hearing, using behavioral listening experiments. While...

Costs involved in using a cochlear implant in South Africa

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Gillian Robyn Kerr

2012-12-01

Full Text Available Cochlear implantation is an expensive but effective lifelong intervention for individuals with a severe-to-profound hearing loss. The primary aim of this study was to survey the short- and long-term costs of cochlear implantation. Individuals (N=154 using cochlear implants obtained from the University of Stellenbosch-Tygerberg Hospital Cochlear Implant Unit in Cape Town, South Africa were surveyed using a questionnaire and patient record review. The questionnaire used a combination of closed and open-ended questions to gather both quantitative and qualitative information. Costs were categorised as short- and long-term costs. All costs were converted to constant rands (June 2010 using the Consumer Price Index to allow for comparison in real terms over time. In the first 10 years of implantation the average estimated costs incurred by adults totalled R379 626, and by children R455 225. The initial purchase of the implant system was the most substantial cost, followed by upgrading of the processor. Travel and accommodation costs peaked in the first 2 years. On average the participants spent R2 550 per year on batteries and spares. Rehabilitation for children cost an average of R7 200. Insurance costs averaged R4 040 per year, and processor repairs R3 000 each. In addition to the upfront expense of obtaining the cochlear implant system, individuals using a cochlear implant in South Africa should be prepared for the long-term costs of maintenance, accessing the unit, support services and additional costs associated with use. Knowledge of these costs is important to ensure that individuals are successful users of their cochlear implants in the long term.

Metabolic syndrome, impaired fasting glucose and obesity, as predictors of incident diabetes in 14 120 hypertensive patients of ASCOT-BPLA: comparison of their relative predictability using a novel approach.

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Gupta, A K; Prieto-Merino, D; Dahlöf, B; Sever, P S; Poulter, N R

2011-08-01

To evaluate, in hypertensive patients, whether the metabolic syndrome is a better predictor of new-onset diabetes compared with impaired fasting glucose, obesity or its other individual components alone, or collectively. Cox models were developed to assess the risk of new-onset diabetes associated with the metabolic syndrome after adjusting for a priori confounders (age, sex, ethnicity and concomitant use of non-cardiovascular medications), its individual components and other determinants of new-onset diabetes. Area under receiver operator curves using the metabolic syndrome or models of impaired fasting glucose were compared, and the ability of these models to correctly identify those who (after 5-years of follow-up) would or would not develop diabetes was assessed. The metabolic syndrome adjusted for a priori confounders and its individual components, and further adjusted for other determinants, was associated with significantly increased risk of new-onset diabetes [1.19 (1.00-1.40), P = 0.05 and 1.22 (1.03-1.44), P = 0.02, respectively]. The discriminative ability of the metabolic syndrome model [area under receiver operating curve: 0.764 (0.750-0.778)] was significantly better than the model of impaired fasting glucose [0.742 (0.727-0.757)] (P fasting glucose status (37.7%) (P fasting glucose were associated with an approximately 9-fold (7.47-10.45) increased risk of new-onset diabetes. Among normoglycaemic patients, the metabolic syndrome was also associated with significantly increased risk of new-onset diabetes, after adjusting for BMI and a priori confounders [1.66 (1.29-2.13)]. Both impaired fasting glucose and the metabolic syndrome predict the risk of new-onset diabetes; however, the metabolic syndrome is a better predictor than impaired fasting glucose in assigning the risk of new-onset diabetes in hypertensive patients, and among those with normoglycaemia. © 2011 The Authors. Diabetic Medicine © 2011 Diabetes UK.

Intraoperative Electrocochleographic Characteristics of Auditory Neuropathy Spectrum Disorder in Cochlear Implant Subjects

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William J. Riggs

2017-07-01

Full Text Available Auditory neuropathy spectrum disorder (ANSD is characterized by an apparent discrepancy between measures of cochlear and neural function based on auditory brainstem response (ABR testing. Clinical indicators of ANSD are a present cochlear microphonic (CM with small or absent wave V. Many identified ANSD patients have speech impairment severe enough that cochlear implantation (CI is indicated. To better understand the cochleae identified with ANSD that lead to a CI, we performed intraoperative round window electrocochleography (ECochG to tone bursts in children (n = 167 and adults (n = 163. Magnitudes of the responses to tones of different frequencies were summed to measure the “total response” (ECochG-TR, a metric often dominated by hair cell activity, and auditory nerve activity was estimated visually from the compound action potential (CAP and auditory nerve neurophonic (ANN as a ranked “Nerve Score”. Subjects identified as ANSD (45 ears in children, 3 in adults had higher values of ECochG-TR than adult and pediatric subjects also receiving CIs not identified as ANSD. However, nerve scores of the ANSD group were similar to the other cohorts, although dominated by the ANN to low frequencies more than in the non-ANSD groups. To high frequencies, the common morphology of ANSD cases was a large CM and summating potential, and small or absent CAP. Common morphologies in other groups were either only a CM, or a combination of CM and CAP. These results indicate that responses to high frequencies, derived primarily from hair cells, are the main source of the CM used to evaluate ANSD in the clinical setting. However, the clinical tests do not capture the wide range of neural activity seen to low frequency sounds.

Is cochlear implantation a good treatment method for profoundly deafened elderly?

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Lachowska M

2013-10-01

Full Text Available Magdalena Lachowska, Agnieszka Pastuszka, Paulina Glinka, Kazimierz Niemczyk Department of Otolaryngology, Hearing Implant Center, Medical University of Warsaw, Warsaw, Poland Purpose: To assess the benefits of cochlear implantation in the elderly. Patients and methods: A retrospective analysis of 31 postlingually deafened elderly (≥60 years of age with unilateral cochlear implants was conducted. Audiological testing included preoperative and postoperative pure-tone audiometry and a monosyllabic word recognition test presented from recorded material in free field. Speech perception tests included Ling's six sound test (sound detection, discrimination, and identification, syllable discrimination, and monosyllabic and multisyllabic word recognition (open set without lip-reading. Everyday life benefits from cochlear implantation were also evaluated. Results: The mean age at the time of cochlear implantation was 72.4 years old. The mean post-implantation follow-up time was 2.34 years. All patients significantly improved their audiological and speech understanding performances. The preoperative mean pure-tone average threshold for 500 Hz, 1,000 Hz, 2,000 Hz, and 4,000 Hz was 110.17 dB HL. Before cochlear implantation, all patients scored 0% on the monosyllabic word recognition test in free field at 70 dB SPL intensity level. The postoperative pure-tone average was 37.14 dB HL (the best mean threshold was 17.50 dB HL, the worst was 58.75 dB HL. After the surgery, mean monosyllabic word recognition reached 47.25%. Speech perception tests showed statistically significant improvement in speech recognition. Conclusion: The results of this study showed that cochlear implantation is indeed a successful treatment for improving speech recognition and offers a great help in everyday life to deafened elderly patients. Therefore, they can be good candidates for cochlear implantation and their age alone should not be a relevant or excluding factor when choosing

The long-term concerns post cochlear implantation as experienced ...

African Journals Online (AJOL)

Background. Cochlear implantation aims to provide an effective means of spoken communication for prelingually deaf children. However, studies in this field are mostly clinically orientated, with little focus on the experiences and long-term concerns of families post cochlear implantation (CI). Objective. To describe the ...

A software tool for analyzing multichannel cochlear implant signals.

Science.gov (United States)

Lai, Wai Kong; Bögli, Hans; Dillier, Norbert

2003-10-01

A useful and convenient means to analyze the radio frequency (RF) signals being sent by a speech processor to a cochlear implant would be to actually capture and display them with appropriate software. This is particularly useful for development or diagnostic purposes. sCILab (Swiss Cochlear Implant Laboratory) is such a PC-based software tool intended for the Nucleus family of Multichannel Cochlear Implants. Its graphical user interface provides a convenient and intuitive means for visualizing and analyzing the signals encoding speech information. Both numerical and graphic displays are available for detailed examination of the captured CI signals, as well as an acoustic simulation of these CI signals. sCILab has been used in the design and verification of new speech coding strategies, and has also been applied as an analytical tool in studies of how different parameter settings of existing speech coding strategies affect speech perception. As a diagnostic tool, it is also useful for troubleshooting problems with the external equipment of the cochlear implant systems.

Libyan cochlear implant programme: achievements, difficulties, and future goals

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Samya El-Ogbi

2011-05-01

Full Text Available Cochlear implantation has become established worldwide as a safe and effective method of auditory rehabilitation of selected severely and profound deaf children and adults. Over 100,000 patients have received cochlear implants worldwide with the paediatric population proving to be the main beneficiaries. The Libyan cochlear implant programme was set up in 2004. Data relating to the patients who received cochlear implantation at Tripoli Medical Centre between October 2007 and February 2010 were analysed. Implant operations were performed on 37 patients. All patients received Med-El SONATATI 100 devices. Thirty-four (91.9% of these patients were children, whilst three (8.1% were adults. Combined, congenital hearing loss (56.8% and perinatal/neonatal (29.7% were the two main aetiological factors in children. Seventeen patients (45.9% had a positive family history of deafness. Sixteen patients (43.2% were born to blood-related parents. The overall rate of minor and major complications was 16.2%, which is comparable to previous studies.

Libyan cochlear implant programme: achievements, difficulties, and future goals.

Science.gov (United States)

Salamat, Ali; Esriti, Anwer; Ehtuish, Asia; El-Ogbi, Samya

2011-01-01

Cochlear implantation has become established worldwide as a safe and effective method of auditory rehabilitation of selected severely and profound deaf children and adults. Over 100,000 patients have received cochlear implants worldwide with the paediatric population proving to be the main beneficiaries. The Libyan cochlear implant programme was set up in 2004. Data relating to the patients who received cochlear implantation at Tripoli Medical Centre between October 2007 and February 2010 were analysed. Implant operations were performed on 37 patients. All patients received Med-El SONATA(TI) (100) devices. Thirty-four (91.9%) of these patients were children, whilst three (8.1%) were adults. Combined, congenital hearing loss (56.8%) and perinatal/neonatal (29.7%) were the two main aetiological factors in children. Seventeen patients (45.9%) had a positive family history of deafness. Sixteen patients (43.2%) were born to blood-related parents. The overall rate of minor and major complications was 16.2%, which is comparable to previous studies.

Facial nerve stimulation outcomes after cochlear implantation with cochlear-facial dehiscence

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Christina H. Fang, MD

2017-06-01

Conclusion: Cochlear-facial dehiscence can predispose patients to post-implant FNS. Prior temporal bone irradiation may carry a higher risk of FNS. We recommend scrutiny for CFD in CTs of CI candidates and appropriate risk counseling for FNS if CFD is discovered and more frequent monitoring for FNS by audiology.

Anomalous facial nerve canal with cochlear malformations.

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Romo, L V; Curtin, H D

2001-05-01

Anteromedial "migration" of the first segment of the facial nerve canal has been previously identified in a patient with a non-Mondini-type cochlear malformation. In this study, several patients with the same facial nerve canal anomaly were reviewed to assess for the association and type of cochlear malformation. CT scans of the temporal bone of 15 patients with anteromedial migration of the first segment of the facial nerve canal were collected from routine departmental examinations. In seven patients, the anomalous course was bilateral, for a total of 22 cases. The migration was graded relative to normal as either mild/moderate or pronounced. The cochlea in each of these cases was examined for the presence and size of the basilar, second, and apical turns. The turns were either absent, small, normal, or enlarged. The CT scans of five patients with eight Mondini malformations were examined for comparison. The degree of the facial nerve migration was pronounced in nine cases and mild/moderate in 13. All 22 of these cases had associated cochlear abnormalities of the non-Mondini variety. These included common cavity anomalies with lack of definition between the cochlea and vestibule (five cases), cochleae with enlarged basilar turns and absent second or third turns (five cases), and cochleae with small or normal basilar turns with small or absent second or third turns (12 cases). None of the patients with Mondini-type cochlear malformations had anteromedial migration of the facial nerve canal. Anteromedial migration of the facial nerve canal occurs in association with some cochlear malformations. It did not occur in association with the Mondini malformations. A cochlea with a Mondini malformation, being similar in size to a normal cochlea, may physically prohibit such a deviation in course.

Microphone directionality, pre-emphasis filter, and wind noise in cochlear implants.

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Chung, King; McKibben, Nicholas

2011-10-01

Wind noise can be a nuisance or a debilitating masker for cochlear implant users in outdoor environments. Previous studies indicated that wind noise at the microphone/hearing aid output had high levels of low-frequency energy and the amount of noise generated is related to the microphone directionality. Currently, cochlear implants only offer either directional microphones or omnidirectional microphones for users at-large. As all cochlear implants utilize pre-emphasis filters to reduce low-frequency energy before the signal is encoded, effective wind noise reduction algorithms for hearing aids might not be applicable for cochlear implants. The purposes of this study were to investigate the effect of microphone directionality on speech recognition and perceived sound quality of cochlear implant users in wind noise and to derive effective wind noise reduction strategies for cochlear implants. A repeated-measure design was used to examine the effects of spectral and temporal masking created by wind noise recorded through directional and omnidirectional microphones and the effects of pre-emphasis filters on cochlear implant performance. A digital hearing aid was programmed to have linear amplification and relatively flat in-situ frequency responses for the directional and omnidirectional modes. The hearing aid output was then recorded from 0 to 360° at flow velocities of 4.5 and 13.5 m/sec in a quiet wind tunnel. Sixteen postlingually deafened adult cochlear implant listeners who reported to be able to communicate on the phone with friends and family without text messages participated in the study. Cochlear implant users listened to speech in wind noise recorded at locations that the directional and omnidirectional microphones yielded the lowest noise levels. Cochlear implant listeners repeated the sentences and rated the sound quality of the testing materials. Spectral and temporal characteristics of flow noise, as well as speech and/or noise characteristics before

Parents' views on the quality of life of their children 2-3 years after cochlear implantation.

Science.gov (United States)

Huttunen, K; Rimmanen, S; Vikman, S; Virokannas, N; Sorri, M; Archbold, S; Lutman, M E

2009-12-01

Cochlear implants for children are known to have impact on the lives of recipients and their families in a variety of ways. To obtain a clearer picture of these benefits, we explored the quality of life of 36 Finnish children and their families 2-3 years after unilateral cochlear implantation. The studied children were, on average 5 years old, and had received their implant at the median age of 2 years:5 months (range 1:6 to 12:3). Most (67%) of the children used speech, eight (22%) used speech and signs, and four (11%) used sign language as their main communication mode. A third of the children had concomitant problems in addition to their profound hearing impairment. A validated closed-set questionnaire "Children with cochlear implants: parental perspectives" (available, e.g., at http://www.earfoundation.org.uk/research/questionnaires.html) was used to find out parents' views and experiences on implantation and explore life after it. Parents were most satisfied with improved/expanded social relations, improved communication (the development of spoken language), general functioning with the help of hearing and improved self-reliance of the child. Benefit of cochlear implantation was also detected with the Categories of Auditory Performance (CAP), which was concordant with views of the parents on the progress of their child in the areas of communication and education. When deciding on implantation, the parents particularly expected auditory information to enhance their child's safety in traffic, joining socially the hearing world, and better employment prospects as adults. Concerning the process of implantation, parents especially valued the know-how and fluent services of the implant centre, positive attitude within the family and information received from other families during the time they were considering the implant decision. Parents also found it important that they have the possibility to influence the communication mode that is used in their child

Mutational spectrum of the WFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric disease

NARCIS (Netherlands)

Cryns, K; Sivakumaran, TA; Van den Ouweland, JMW; Pennings, RJE; Cremers, CWRJ; Flothmann, K; Young, TL; Smith, RJH; Lesperance, MM; Van Camp, G

2003-01-01

WFS1 is a novel gene and encodes an 890 amino-acid glycoprotein (wolframin), predominantly localized in the endoplasmic reticulum. Mutations in WFS1 underlie autosomal recessive Wolfram syndrome and autosomal dominant low frequency sensorineural hearing impairment (LFSNHI) DFNA6/14. In addition,

Mutational spectrum of the WFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric disease.

NARCIS (Netherlands)

Cryns, K.; Sivakumaran, T.A.; Ouweland, J.M.W. van den; Pennings, R.J.E.; Cremers, C.W.R.J.; Flothmann, K.; Young, T.L.; Smith, R.J.H.; Lesperance, M.M.; Camp, G. van

2003-01-01

WFS1 is a novel gene and encodes an 890 amino-acid glycoprotein (wolframin), predominantly localized in the endoplasmic reticulum. Mutations in WFS1 underlie autosomal recessive Wolfram syndrome and autosomal dominant low frequency sensorineural hearing impairment (LFSNHI) DFNA6/14. In addition,

Cochlear implantation in autistic children with profound sensorineural hearing loss.

Science.gov (United States)

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.

Conductive component after cochlear implantation in patients with residual hearing conservation.

Science.gov (United States)

Chole, Richard A; Hullar, Timothy E; Potts, Lisa G

2014-12-01

Changes in auditory thresholds following cochlear implantation are generally assumed to be due to damage to neural elements. Theoretical studies have suggested that placement of a cochlear implant can cause a conductive hearing loss. Identification of a conductive component following cochlear implantation could guide improvements in surgical techniques or device designs. The purpose of this study is to characterize new-onset conductive hearing losses after cochlear implantation. In a prospective study, air- and bone-conduction audiometric testing were completed on cochlear implant recipients. An air-bone gap equal to or greater than 15 dB HL at 2 frequencies determined the presence of a conductive component. Of the 32 patients with preoperative bone-conduction hearing, 4 patients had a new-onset conductive component resulting in a mixed hearing loss, with air-conduction thresholds ranging from moderate to profound and an average air-bone gap of 30 dB HL. One had been implanted through the round window, 2 had an extended round window, and 1 had a separate cochleostomy. Loss of residual hearing following cochlear implantation may be due in part to a conductive component. Identifying the mechanism for this conductive component may help minimize hearing loss. Postoperative hearing evaluation should measure both air- and bone-conduction thresholds.

[Applied anatomy of scala tympani inlet related to cochlear implantation].

Science.gov (United States)

Zou, Tuanming; Guo, Menghe; Zhang, Hongzheng; Shu, Fan; Xie, Nanping

2012-06-01

To investigate the related parameters of the temporal bone structure for determining the position of implanting electrode into the scala tympani in cochlear implantation surgery through the facial recess and epitympanum approach. In a surgical simulation experiment, 20 human temporal bones were studied and measured to determine the related parameters of the temporal bone structure. The distance 5.91∓0.29 mm between the short process of the incus and the round window niche, 2.11∓0.18 mm between the stapes and the round window niche, 6.70∓0.19 mm between the facial nerve in the perpendicular paragraph and the round window niche, 2.22∓0.21 mm from the pyramidal eminence to the round window, and 2.16∓0.14 mm between the stapes and the round window. The minimal distance between the implanting electrode and the vestibular window was 2.12∓0.19 mm. The distance between the cochleariform process and the round window niche was 3.79∓0.17 mm. The position of the cochlear electrode array insertion into the second cochlear turn was 2.25∓0.13 mm under the stapes. The location of the cochlear electrode array insertion into the second cochlear turn was 2.28∓0.20 mm inferior to the pyramidal eminence. These parameters provide a reference value to determine the different positions of cochlear electrode array insertion into the scale tympani in different patients.

Usher syndrome type III can mimic other types of Usher syndrome.

Science.gov (United States)

Pennings, Ronald J E; Fields, Randall R; Huygen, Patrick L M; Deutman, August F; Kimberling, William J; Cremers, Cor W R J

2003-06-01

Clinical and genetic characteristics are presented of 2 patients from a Dutch Usher syndrome type III family who have a new homozygous USH3 gene mutation: 149-152delCAGG + insTGTCCAAT. One individual (IV:1) is profoundly hearing impaired and has normal vestibular function and retinitis punctata albescens (RPA). The other individual is also profoundly hearing impaired, but has well-developed speech, vestibular areflexia, and retinitis pigmentosa sine pigmento (RPSP). These findings suggest that Usher syndrome type III can be clinically misdiagnosed as either Usher type I or II; that Usher syndrome patients who are profoundly hearing impaired and have normal vestibular function should be tested for USH3 mutations; and that RPA and RPSP can occur as fundoscopic manifestations of pigmentary retinopathy in Usher syndrome.

Deficiency in origin licensing proteins impairs cilia formation: implications for the aetiology of Meier-Gorlin syndrome.

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Tom Stiff

Full Text Available Mutations in ORC1, ORC4, ORC6, CDT1, and CDC6, which encode proteins required for DNA replication origin licensing, cause Meier-Gorlin syndrome (MGS, a disorder conferring microcephaly, primordial dwarfism, underdeveloped ears, and skeletal abnormalities. Mutations in ATR, which also functions during replication, can cause Seckel syndrome, a clinically related disorder. These findings suggest that impaired DNA replication could underlie the developmental defects characteristic of these disorders. Here, we show that although origin licensing capacity is impaired in all patient cells with mutations in origin licensing component proteins, this does not correlate with the rate of progression through S phase. Thus, the replicative capacity in MGS patient cells does not correlate with clinical manifestation. However, ORC1-deficient cells from MGS patients and siRNA-mediated depletion of origin licensing proteins also have impaired centrosome and centriole copy number. As a novel and unexpected finding, we show that they also display a striking defect in the rate of formation of primary cilia. We demonstrate that this impacts sonic hedgehog signalling in ORC1-deficient primary fibroblasts. Additionally, reduced growth factor-dependent signaling via primary cilia affects the kinetics of cell cycle progression following cell cycle exit and re-entry, highlighting an unexpected mechanism whereby origin licensing components can influence cell cycle progression. Finally, using a cell-based model, we show that defects in cilia function impair chondroinduction. Our findings raise the possibility that a reduced efficiency in forming cilia could contribute to the clinical features of MGS, particularly the bone development abnormalities, and could provide a new dimension for considering developmental impacts of licensing deficiency.

A case report: the first successful cochlear implant in Uganda.

African Journals Online (AJOL)

A case report: the first successful cochlear implant in Uganda. Richard Byaruhanga1, J. ... The patient was a 23 year old male whose presenting com- plaint was inability to .... Custom Sound by Cochlear (the company that manu- factures the ...

Multimodal MRI reveals structural connectivity differences in 22q11 deletion syndrome related to impaired spatial working memory

NARCIS (Netherlands)

O'Hanlon, Erik; Howley, Sarah; Prasad, Sarah; McGrath, Jane; Leemans, Alexander; McDonald, Colm; Garavan, Hugh; Murphy, Kieran C

2016-01-01

INTRODUCTION: Impaired spatial working memory is a core cognitive deficit observed in people with 22q11 Deletion syndrome (22q11DS) and has been suggested as a candidate endophenotype for schizophrenia. However, to date, the neuroanatomical mechanisms describing its structural and functional

Cochlear pathology in chronic suppurative otitis media.

Science.gov (United States)

Walby, A P; Barrera, A; Schuknecht, H F

1983-01-01

Chronic suppurative otitis media (COM) is reported to cause elevation of bone-conduction thresholds either by damage to cochlear sensorineural structures or by alteration in the mechanics of sound transmission in the ear. A retrospective study was made of the medical records of 87 patients with unilateral uncomplicated COM to document that abnormality in bone conduction does exist. In a separate study the cochlear pathology in 12 pairs of temporal bones with unilateral COM was studied by light microscopy. Infected ears showed higher than normal mean bone-conduction thresholds by amounts ranging from 1 dB at 500 Hz to 9.5 dB at 4,000 Hz. The temporal bones showed no greater loss of specialized sensorineural structures in infected ears than in normal control ears. Because there is no evidence that COM caused destruction of hair cells or cochlear neurons, alteration in the mechanics of sound transmission becomes a more plausible explanation for the hearing losses.

Why is coronary collateral growth impaired in type II diabetes and the metabolic syndrome?

Science.gov (United States)

Rocic, Petra

2012-01-01

Type II diabetes and the metabolic syndrome are strong predictors of severity of occlusive coronary disease and poorer outcomes of coronary revascularization therapies. Coronary collateral growth can provide an alternative or accessory pathway of revascularization. However, collateral growth is impaired in type II diabetes and the metabolic syndrome. Although many factors necessary for collateral growth are known and many interventions have shown promising results in animal studies, not a single attempt to induce coronary collateral growth in human clinical trials has led to satisfactory results. Accordingly, the first part of this review outlines the known deleterious effects of diabetes and the metabolic syndrome on factors necessary for collateral growth, including pro-angiogenic growth factors, endothelial function, the redox state of the coronary circulation, intracellular signaling, leukocytes and bone marrow-derived progenitors cells. The second section highlights the gaps in our current knowledge of how these factors interact with the radically altered environment of the coronary circulation in diabetes and the metabolic syndrome. The interplay between these pathologies and inadequately explored areas related to the temporal regulation of collateral remodeling and the roles of the extracellular matrix, vascular cell phenotype and pro-inflammatory cytokines are emphasized with implications to development of efficient therapies. PMID:22342811

Speech perception benefits of internet versus conventional telephony for hearing-impaired individuals.

Science.gov (United States)

Mantokoudis, Georgios; Dubach, Patrick; Pfiffner, Flurin; Kompis, Martin; Caversaccio, Marco; Senn, Pascal

2012-07-16

Telephone communication is a challenge for many hearing-impaired individuals. One important technical reason for this difficulty is the restricted frequency range (0.3-3.4 kHz) of conventional landline telephones. Internet telephony (voice over Internet protocol [VoIP]) is transmitted with a larger frequency range (0.1-8 kHz) and therefore includes more frequencies relevant to speech perception. According to a recently published, laboratory-based study, the theoretical advantage of ideal VoIP conditions over conventional telephone quality has translated into improved speech perception by hearing-impaired individuals. However, the speech perception benefits of nonideal VoIP network conditions, which may occur in daily life, have not been explored. VoIP use cannot be recommended to hearing-impaired individuals before its potential under more realistic conditions has been examined. To compare realistic VoIP network conditions, under which digital data packets may be lost, with ideal conventional telephone quality with respect to their impact on speech perception by hearing-impaired individuals. We assessed speech perception using standardized test material presented under simulated VoIP conditions with increasing digital data packet loss (from 0% to 20%) and compared with simulated ideal conventional telephone quality. We monaurally tested 10 adult users of cochlear implants, 10 adult users of hearing aids, and 10 normal-hearing adults in the free sound field, both in quiet and with background noise. Across all participant groups, mean speech perception scores using VoIP with 0%, 5%, and 10% packet loss were 15.2% (range 0%-53%), 10.6% (4%-46%), and 8.8% (7%-33%) higher, respectively, than with ideal conventional telephone quality. Speech perception did not differ between VoIP with 20% packet loss and conventional telephone quality. The maximum benefits were observed under ideal VoIP conditions without packet loss and were 36% (P = .001) for cochlear implant users, 18

Microanatomy of the cochlear hook

Science.gov (United States)

Kwan, Changyow Claire; Tan, Xiaodong; Stock, Stuart R.; Soriano, Carmen; Xiao, Xianghui; Richter, Claus-Peter

2017-09-01

Communication among humans occurs through coding and decoding of acoustic information. The inner ear or cochlea acts as a frequency analyzer and divides the acoustic signal into small frequency bands, which are processed at different sites along the cochlea. The mechano-electrical conversion is accomplished by the soft tissue structures in the cochlea. While the anatomy for most of the cochlea has been well described, a detailed description of the very high frequency and vulnerable cochlear hook region is missing. To study the cochlear hook, mice cochleae were imaged with synchrotron radiation and high-resolution reconstructions have been made from the tomographic scans. This is the first detailed description of the bony and soft tissues of the hook region of the mammalian cochlea.

Hearing dysfunction in heterozygous Mitf(Mi-wh) /+ mice, a model for Waardenburg syndrome type 2 and Tietz syndrome.

Science.gov (United States)

Ni, Christina; Zhang, Deming; Beyer, Lisa A; Halsey, Karin E; Fukui, Hideto; Raphael, Yehoash; Dolan, David F; Hornyak, Thomas J

2013-01-01

The human deafness-pigmentation syndromes, Waardenburg syndrome (WS) type 2a, and Tietz syndrome are characterized by profound deafness but only partial cutaneous pigmentary abnormalities. Both syndromes are caused by mutations in MITF. To illuminate differences between cutaneous and otic melanocytes in these syndromes, their development and survival in heterozygous Microphthalmia-White (Mitf(Mi-wh) /+) mice were studied and hearing function of these mice characterized. Mitf(Mi-wh) /+ mice have a profound hearing deficit, characterized by elevated auditory brainstem response thresholds, reduced distortion product otoacoustic emissions, absent endocochlear potential, loss of outer hair cells, and stria vascularis abnormalities. Mitf(Mi-wh) /+ embryos have fewer melanoblasts during embryonic development than their wild-type littermates. Although cochlear melanocytes are present at birth, they disappear from the Mitf(Mi-wh) /+ cochlea between P1 and P7. These findings may provide insight into the mechanism of melanocyte and hearing loss in human deafness-pigmentation syndromes such as WS and Tietz syndrome and illustrate differences between otic and follicular melanocytes. © 2012 John Wiley & Sons A/S.

Cochlear implantation in branchio-oto-renal syndrome — A surgical challenge

OpenAIRE

Kameswaran, Mohan; Kumar, R. S. Anand; Murali, Sathiya; Raghunandhan, S.; Karthikeyan, K.

2007-01-01

Branchio-oto-renal syndrome (Melnick-Fraser Syndrome) is a rare Autosomal Dominant disorder characterized by the syndromic association of branchial cysts or fistulae along with external, middle & inner malformations and renal anomalies. Incomplete penetrance and variable expressivity are common with the phenotypic variation ranging from mild to severe forms & consisting of various eye, ear, oral and craniofacial abnormalities. Mutations in the EYA1 gene on chromosomal site 8q13.3 are identifi...

Parental Expectation from Children with Cochlear Implants in Indian Context: a Questionnaire Based Study.

Science.gov (United States)

Kumar, Prawin; Sanju, Himanshu Kumar; Mishra, Rajkishor; Singh, Varun; Mohan, Priyanka

2017-04-01

Introduction  Parental support is important in the habilitation/rehabilitation of children using cochlear implant devices. Hence, it is important for families to know the realistic expectations regarding outcomes from CIs. Objective  The objective of the present study is to know the parents' expectation from children using CIs. Methods  For this study, we recruited 23 parents of children using CIs. We administered 15 questions translated in to Hindi related to communication abilities, social skills, academic achievement, change in future life, rehabilitation demand, and stress due to hearing impairment. Results  The response of the questions (5-point rating scale) related to communication abilities showed that parents were expecting children using CIs to use the telephone (95%), to be able to detect soft sounds (99%), to listen in crowds (86%), to be able to easily understand others (76%), and to show improvement in communication skills (78%). Similarly, for questions related to social skills showed 90% of the parents expecting that their children with CIs should be able to easily make friends with normal hearing peers, and 80% of the parents were expecting the children to achieve high standards in their reading and writing skills. Questions related to change in future life showed 86% of the parents expecting their children with CIs to act like normal hearing children. Further, 78% parents showed positive response regarding importance of intensive training. However, 70% of the parents reported stress in the family due to the existence of the hearing impaired child. Conclusion  Overall, the existing questionnaire-based study showed that parents have high expectations from their children with cochlear implant.

Inactivation of STAT3 Signaling Impairs Hair Cell Differentiation in the Developing Mouse Cochlea.

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Chen, Qianqian; Quan, Yizhou; Wang, Naitao; Xie, Chengying; Ji, Zhongzhong; He, Hao; Chai, Renjie; Li, Huawei; Yin, Shankai; Chin, Y Eugene; Wei, Xunbin; Gao, Wei-Qiang

2017-07-11

Although STAT3 signaling is demonstrated to regulate sensory cell differentiation and regeneration in the zebrafish, its exact role is still unclear in mammalian cochleae. Here, we report that STAT3 and its activated form are specifically expressed in hair cells during mouse cochlear development. Importantly, conditional cochlear deletion of Stat3 leads to an inhibition on hair cell differentiation in mice in vivo and in vitro. By cell fate analysis, inactivation of STAT3 signaling shifts the cell division modes from asymmetric to symmetric divisions from supporting cells. Moreover, inhibition of Notch signaling stimulates STAT3 phosphorylation, and inactivation of STAT3 signaling attenuates production of supernumerary hair cells induced by a Notch pathway inhibitor. Our findings highlight an important role of the STAT3 signaling during mouse cochlear hair cell differentiation and may have clinical implications for the recovery of hair cell loss-induced hearing impairment. Copyright © 2017 International Society for Stem Cell Research. Published by Elsevier Inc. All rights reserved.

Inactivation of STAT3 Signaling Impairs Hair Cell Differentiation in the Developing Mouse Cochlea

Directory of Open Access Journals (Sweden)

Qianqian Chen

2017-07-01

Full Text Available Although STAT3 signaling is demonstrated to regulate sensory cell differentiation and regeneration in the zebrafish, its exact role is still unclear in mammalian cochleae. Here, we report that STAT3 and its activated form are specifically expressed in hair cells during mouse cochlear development. Importantly, conditional cochlear deletion of Stat3 leads to an inhibition on hair cell differentiation in mice in vivo and in vitro. By cell fate analysis, inactivation of STAT3 signaling shifts the cell division modes from asymmetric to symmetric divisions from supporting cells. Moreover, inhibition of Notch signaling stimulates STAT3 phosphorylation, and inactivation of STAT3 signaling attenuates production of supernumerary hair cells induced by a Notch pathway inhibitor. Our findings highlight an important role of the STAT3 signaling during mouse cochlear hair cell differentiation and may have clinical implications for the recovery of hair cell loss-induced hearing impairment.

Emotion Understanding in Deaf Children with a Cochlear Implant

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Wiefferink, Carin H.; Rieffe, Carolien; Ketelaar, Lizet; De Raeve, Leo; Frijns, Johan H. M.

2013-01-01

It is still largely unknown how receiving a cochlear implant affects the emotion understanding in deaf children. We examined indices for emotion understanding and their associations with communication skills in children aged 2.5-5 years, both hearing children (n = 52) and deaf children with a cochlear implant (n = 57). 2 aspects of emotion…

Auditory Mechanics of the Tectorial Membrane and the Cochlear Spiral

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Gavara, Núria; Manoussaki, Daphne; Chadwick, Richard S.

2012-01-01

Purpose of review This review is timely and relevant since new experimental and theoretical findings suggest that cochlear mechanics from the nanoscale to the macroscale are affected by mechanical properties of the tectorial membrane and the spiral shape. Recent findings Main tectorial membrane themes covered are i) composition and morphology, ii) nanoscale mechanical interactions with the outer hair cell bundle, iii) macroscale longitudinal coupling, iv) fluid interaction with inner hair cell bundles, v) macroscale dynamics and waves. Main cochlear spiral themes are macroscale low-frequency energy focusing and microscale organ of Corti shear gain. Implications Findings from new experimental and theoretical models reveal exquisite sensitivity of cochlear mechanical performance to tectorial membrane structural organization, mechanics, and its positioning with respect to hair bundles. The cochlear spiral geometry is a major determinant of low frequency hearing. Suggestions are made for future research directions. PMID:21785353

[Pre-operation evaluation and intra-operation management of cochlear implantation].

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Zhang, Dao-xing; Hu, Bao-hua; Xiao, Yu-li; Shi, Bo-ning

2004-10-01

To summarize pre-operation evaluation experiences in cochlear implantation. Performing auditory evaluation and image analysis seriously in 158 severe hearing loss or total deaf cases before cochlear implantation, comparing their performance with the findings during and post operation. Among the total 158 cases, 116 cases with normal structure, 42 cases with the abnormal findings of the inner or middle ear. Stapedial gusher happened in 6 cases, 1 case was not predicted before operation. Except 1 case with serious malformation, the findings of other 157 cases in operation were consistent with the pre-operation evaluation. We helped all patients reconstruct auditory conduction with cochlear implantation, and the average hearing level up to 37.6 dB SPL. Performing image analysis seriously before operation and planning for operation according to HRCT can do great help to cochlear implantation. The operation under the HRCT instruction has less complications.

Music Therapy for Preschool Cochlear Implant Recipients

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Gfeller, Kate; Driscoll, Virginia; Kenworthy, Maura; Van Voorst, Tanya

2010-01-01

This paper provides research and clinical information relevant to music therapy for preschool children who use cochlear implants (CI). It consolidates information from various disciplinary sources regarding (a) cochlear implantation of young prelingually-deaf children (~age 2-5), (b) patterns of auditory and speech-language development, and (c) research regarding music perception of children with CIs. This information serves as a foundation for the final portion of the article, which describes typical music therapy goals and examples of interventions suitable for preschool children. PMID:23904691

Effects of residual hearing on cochlear implant outcomes in children: A systematic-review.

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Chiossi, Julia Santos Costa; Hyppolito, Miguel Angelo

2017-09-01

to investigate if preoperative residual hearing in prelingually deafened children can interfere on cochlear implant indication and outcomes. a systematic-review was conducted in five international databases up to November-2016, to locate articles that evaluated cochlear implantation in children with some degree of preoperative residual hearing. Outcomes were auditory, language and cognition performances after cochlear implant. The quality of the studies was assessed and classified according to the Oxford Levels of Evidence table - 2011. Risk of biases were also described. From the 30 articles reviewed, two types of questions were identified: (a) what are the benefits of cochlear implantation in children with residual hearing? (b) is the preoperative residual hearing a predictor of cochlear implant outcome? Studies ranged from 04 to 188 subjects, evaluating populations between 1.8 and 10.3 years old. The definition of residual hearing varied between studies. The majority of articles (n = 22) evaluated speech perception as the outcome and 14 also assessed language and speech production. There is evidence that cochlear implant is beneficial to children with residual hearing. Preoperative residual hearing seems to be valuable to predict speech perception outcomes after cochlear implantation, even though the mechanism of how it happens is not clear. More extensive researches must be conducted in order to make recommendations and to set prognosis for cochlear implants based on children preoperative residual hearing. Copyright © 2017 Elsevier B.V. All rights reserved.

Congenitally Deafblind Children and Cochlear Implants: Effects on Communication

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Dammeyer, Jesper

2009-01-01

There has been much research conducted demonstrating the positive benefits of cochlear implantation (CI) in children who are deaf. Research on CI in children who are both deaf and blind, however, is lacking. The purpose of this article is to present a study of five congenitally deafblind children who received cochlear implants between 2.2 and 4.2…

Reduction in spread of excitation from current focusing at multiple cochlear locations in cochlear implant users.

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Padilla, Monica; Landsberger, David M

2016-03-01

Channel interaction from a broad spread of excitation is likely to be a limiting factor in performance by cochlear implant users. Although partial tripolar stimulation has been shown to reduce spread of excitation, the magnitude of the reduction is highly variable across subjects. Because the reduction in spread of excitation is typically only measured at one electrode for a given subject, the degree of variability across cochlear locations is unknown. The first goal of the present study was to determine if the reduction in spread of excitation observed from partial tripolar current focusing systematically varies across the cochlea. The second goal was to measure the variability in reduction of spread of excitation relative to monopolar stimulation across the cochlea. The third goal was to expand upon previous results that suggest that scaling of verbal descriptors can be used to predict the reduction in spread of excitation, by increasing the limited number of sites previously evaluated and verify the relationships remain with the larger dataset. The spread of excitation for monopolar and partial tripolar stimulation was measured at 5 cochlear locations using a psychophysical forward masking task. Results of the present study suggest that although partial tripolar stimulation typically reduces spread of excitation, the degree of reduction in spread of excitation was found to be highly variable and no effect of cochlear location was found. Additionally, subjective scaling of certain verbal descriptors (Clean/Dirty, Pure/Noisy) correlated with the reduction in spread of excitation suggesting sound quality scaling might be used as a quick clinical estimate of channels providing a reduction in spread of excitation. This quick scaling technique might help clinicians determine which patients would be most likely to benefit from a focused strategy. Copyright © 2016 Elsevier B.V. All rights reserved.

Barriers to pediatric cochlear implantation: A parental survey.

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Yang, Charles Q; Reilly, Brian K; Preciado, Diego A

2018-01-01

This study aims to (1) determine barriers in the pediatric cochlear implantation process specific to publicly insured patients, wherein delayed implantation has been reported, and (2) compare the perceived barriers between publicly and privately insured patients. Tertiary care cochlear implantation center at academic pediatric hospital. Cross-sectional survey, retrospective chart review. The validated, 39 item Barriers to Care Questionnaire was administered to the parents of 80 recipients of cochlear implantation by two surgeons between 2013 and 2016. Survey results and diagnosis to implant interval were compared based on public or private insurance status. Two-tailed Mann-Whitney and Fisher's exact test was used for statistical analysis. Of 110 cochlear implants, 27 of 80 (34%) English-speaking parents completed the survey. 15 were privately insured and 12 were publicly insured. 23 of 27 respondents received cochlear implantation for pre-lingual sensorineural hearing loss. Publicly insured patients had significantly longer median time from diagnosis to implant than privately insured (19 vs. 8 mo, p = 0.01). The three worst scoring barrier categories for privately insured families in order were Pragmatics, Expectations, and Marginalization, whereas for publicly insured families it was Pragmatics, Skills, and Expectations. The worst scoring question for privately insured patients was "Having to take time off work". For the publicly insured, it was "Lack of communication." Privately insured patients reported more barriers on the Barriers to Care Questionnaire than publicly insured patients did. Although pragmatics was the worst-scoring barrier category for both groups, difficulties found on the survey ranked differently for each group. This information can help providers address disparities and access barriers for vulnerable patients. Published by Elsevier B.V.

Intranasal siRNA administration reveals IGF2 deficiency contributes to impaired cognition in Fragile X syndrome mice.

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Pardo, Marta; Cheng, Yuyan; Velmeshev, Dmitry; Magistri, Marco; Eldar-Finkelman, Hagit; Martinez, Ana; Faghihi, Mohammad A; Jope, Richard S; Beurel, Eleonore

2017-03-23

Molecular mechanisms underlying learning and memory remain imprecisely understood, and restorative interventions are lacking. We report that intranasal administration of siRNAs can be used to identify targets important in cognitive processes and to improve genetically impaired learning and memory. In mice modeling the intellectual deficiency of Fragile X syndrome, intranasally administered siRNA targeting glycogen synthase kinase-3β (GSK3β), histone deacetylase-1 (HDAC1), HDAC2, or HDAC3 diminished cognitive impairments. In WT mice, intranasally administered brain-derived neurotrophic factor (BDNF) siRNA or HDAC4 siRNA impaired learning and memory, which was partially due to reduced insulin-like growth factor-2 (IGF2) levels because the BDNF siRNA- or HDAC4 siRNA-induced cognitive impairments were ameliorated by intranasal IGF2 administration. In Fmr1 -/- mice, hippocampal IGF2 was deficient, and learning and memory impairments were ameliorated by IGF2 intranasal administration. Therefore intranasal siRNA administration is an effective means to identify mechanisms regulating cognition and to modulate therapeutic targets.

Comparison of learning preferences of Turkish children who had been applied cochlear implantation in Turkey and Germany according to theory of multiple intelligence.

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Sahli, Sanem; Laszig, Roland; Aschendorff, Antje; Kroeger, Stefanie; Wesarg, Thomas; Belgin, Erol

2011-12-01

The aim of the study is to determinate the using dominant multiple intelligence types and compare the learning preferences of Turkish cochlear implanted children aged four to ten in Turkey and Germany according to Theory of multiple intelligence. The study has been conducted on a total of 80 children and four groups in Freiburg/Germany and Ankara/Turkey. The applications have been done in University of Freiburg, Cochlear Implant Center in Germany, and University of Hacettepe, ENT Department, Audiology and Speech Pathology Section in Turkey. In this study, the data have been collected by means of General Information Form and Cochlear Implant Information Form applied to parents. To determine the dominant multiple intelligence types of children, the TIMI (Teele Inventory of Multiple Intelligences) which was developed by Sue Teele have been used. The study results exposed that there was not a statistically significant difference on dominant intelligence areas and averages of scores of multiple intelligence types in control groups (p>0.05). Although, the dominant intelligence areas were different (except for first dominant intelligence) in cochlear implanted children in Turkey and Germany, there was not a statistically significant difference on averages of scores of dominant multiple intelligence types. Every hearing impaired child who started training, should be evaluated in terms of multiple intelligence areas and identified strengths and weaknesses. Multiple intelligence activities should be used in their educational programs. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Severe Health-Related Quality of Life Impairment in Active Primary Sjögren's Syndrome and Patient-Reported Outcomes: Data From a Large Therapeutic Trial.

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Cornec, Divi; Devauchelle-Pensec, Valérie; Mariette, Xavier; Jousse-Joulin, Sandrine; Berthelot, Jean-Marie; Perdriger, Aleth; Puéchal, Xavier; Le Guern, Véronique; Sibilia, Jean; Gottenberg, Jacques-Eric; Chiche, Laurent; Hachulla, Eric; Yves Hatron, Pierre; Goeb, Vincent; Hayem, Gilles; Morel, Jacques; Zarnitsky, Charles; Dubost, Jean Jacques; Saliou, Philippe; Pers, Jacques Olivier; Seror, Raphaèle; Saraux, Alain

2017-04-01

To identify the principal determinants of health-related quality of life (HRQOL) impairment in patients with active primary Sjögren's syndrome (SS) participating in a large therapeutic trial, Tolerance and Efficacy of Rituximab in Primary Sjögren's Syndrome (TEARS). At the inclusion visit for the TEARS trial, 120 patients with active primary SS completed the Short Form 36 health survey (SF-36), a validated HRQOL assessment tool. Univariate then multivariate linear regression analyses were used to assess associations linking SF-36 physical and mental components to demographic data, patient-reported outcomes (symptom intensity assessments for dryness, pain, and fatigue, including the European League Against Rheumatism [EULAR] Sjögren's Syndrome Patient Reported Index [ESSPRI]), objective measures of dryness and autoimmunity, and physician evaluation of systemic activity (using the EULAR Sjögren's Syndrome Disease Activity Index [ESSDAI]). SF-36 scores indicated marked HRQOL impairments in our population with active primary SS. Approximately one-third of the patients had low, moderate, and high systemic activity according to the ESSDAI. ESSPRI and ESSDAI scores were moderately but significantly correlated. The factors most strongly associated with HRQOL impairment were patient-reported symptoms, best assessed using the ESSPRI, with pain and ocular dryness intensity showing independent associations with HRQOL. Conversely, systemic activity level was not associated with HRQOL impairment in multivariate analyses, even in the patient subset with ESSDAI values indicating moderate-to-high systemic activity. The cardinal symptoms of primary SS (dryness, pain, and fatigue, best assessed using the ESSPRI) are stronger predictors of HRQOL impairment than systemic involvement (assessed by the ESSDAI) and should be used as end points in future therapeutic trials focusing on patients' well-being. New consensual and data-driven response criteria are needed for primary SS

Preoperative vestibular assessment protocol of cochlear implant surgery: an analytical descriptive study.

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Bittar, Roseli Saraiva Moreira; Sato, Eduardo Setsuo; Ribeiro, Douglas Jósimo Silva; Tsuji, Robinson Koji

Cochlear implants are undeniably an effective method for the recovery of hearing function in patients with hearing loss. To describe the preoperative vestibular assessment protocol in subjects who will be submitted to cochlear implants. Our institutional protocol provides the vestibular diagnosis through six simple tests: Romberg and Fukuda tests, assessment for spontaneous nystagmus, Head Impulse Test, evaluation for Head Shaking Nystagmus and caloric test. 21 patients were evaluated with a mean age of 42.75±14.38 years. Only 28% of the sample had all normal test results. The presence of asymmetric vestibular information was documented through the caloric test in 32% of the sample and spontaneous nystagmus was an important clue for the diagnosis. Bilateral vestibular areflexia was present in four subjects, unilateral arreflexia in three and bilateral hyporeflexia in two. The Head Impulse Test was a significant indicator for the diagnosis of areflexia in the tested ear (p=0.0001). The sensitized Romberg test using a foam pad was able to diagnose severe vestibular function impairment (p=0.003). The six clinical tests were able to identify the presence or absence of vestibular function and function asymmetry between the ears of the same individual. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Cardiorenal Syndrome in Acute Heart Failure Syndromes

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Mohammad Sarraf

2011-01-01

Full Text Available Impaired cardiac function leads to activation of the neurohumoral axis, sodium and water retention, congestion and ultimately impaired kidney function. This sequence of events has been termed the Cardiorenal Syndrome. This is different from the increase in cardiovascular complications which occur with primary kidney disease, that is, the so-called Renocardiac Syndrome. The present review discusses the pathogenesis of the Cardiorenal Syndrome followed by the benefits and potential deleterious effects of pharmacological agents that have been used in this setting. The agents discussed are diuretics, aquaretics, natriuretic peptides, vasodilators, inotropes and adenosine α1 receptor antagonists. The potential role of ultrafiltration is also briefly discussed.

MRI of the labyrinth with volume rendering for cochlear implants candidates

International Nuclear Information System (INIS)

Sakata, Motomichi; Harada, Kuniaki; Shirase, Ryuji; Suzuki, Junpei; Nagahama, Hiroshi

2009-01-01

We demonstrated three-dimensional models of the labyrinth by volume rendering (VR) in preoperative assessment for cochlear implantation. MRI data sets were acquired in selected subjects using three-dimensional-fast spin echo sequences (3D-FSE). We produced the three-dimensional models of the labyrinth from axial heavily T2-weighted images. The three-dimensional models distinguished the scala tympani and scala vestibuli and provided multidirectional images. The optimal threshold three-dimensional models clearly showed the focal region of signal loss in the cochlear turns (47.1%) and the presence of inner ear anomalies (17.3%) in our series of patients. This study was concluded that these three-dimensional models by VR provide the oto-surgeon with precise, detailed, and easily interpreted information about the cochlear turns for cochlear implants candidates. (author)

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

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

2018-02-01

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.

Selective deletion of cochlear hair cells causes rapid age-dependent changes in spiral ganglion and cochlear nucleus neurons.

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Tong, Ling; Strong, Melissa K; Kaur, Tejbeer; Juiz, Jose M; Oesterle, Elizabeth C; Hume, Clifford; Warchol, Mark E; Palmiter, Richard D; Rubel, Edwin W

2015-05-20

During nervous system development, critical periods are usually defined as early periods during which manipulations dramatically change neuronal structure or function, whereas the same manipulations in mature animals have little or no effect on the same property. Neurons in the ventral cochlear nucleus (CN) are dependent on excitatory afferent input for survival during a critical period of development. Cochlear removal in young mammals and birds results in rapid death of target neurons in the CN. Cochlear removal in older animals results in little or no neuron death. However, the extent to which hair-cell-specific afferent activity prevents neuronal death in the neonatal brain is unknown. We further explore this phenomenon using a new mouse model that allows temporal control of cochlear hair cell deletion. Hair cells express the human diphtheria toxin (DT) receptor behind the Pou4f3 promoter. Injections of DT resulted in nearly complete loss of organ of Corti hair cells within 1 week of injection regardless of the age of injection. Injection of DT did not influence surrounding supporting cells directly in the sensory epithelium or spiral ganglion neurons (SGNs). Loss of hair cells in neonates resulted in rapid and profound neuronal loss in the ventral CN, but not when hair cells were eliminated at a more mature age. In addition, normal survival of SGNs was dependent on hair cell integrity early in development and less so in mature animals. This defines a previously undocumented critical period for SGN survival. Copyright © 2015 the authors 0270-6474/15/357878-14$15.00/0.

Age at implantation and auditory memory in cochlear implanted children.

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Mikic, B; Miric, D; Nikolic-Mikic, M; Ostojic, S; Asanovic, M

2014-05-01

Early cochlear implantation, before the age of 3 years, provides the best outcome regarding listening, speech, cognition an memory due to maximal central nervous system plasticity. Intensive postoperative training improves not only auditory performance and language, but affects auditory memory as well. The aim of this study was to discover if the age at implantation affects auditory memory function in cochlear implanted children. A total of 50 cochlear implanted children aged 4 to 8 years were enrolled in this study: early implanted (1-3y) n = 27 and late implanted (4-6y) n = 23. Two types of memory tests were used: Immediate Verbal Memory Test and Forward and Backward Digit Span Test. Early implanted children performed better on both verbal and numeric tasks of auditory memory. The difference was statistically significant, especially on the complex tasks. Early cochlear implantation, before the age of 3 years, significantly improve auditory memory and contribute to better cognitive and education outcomes.

Labyrinthectomy with cochlear implantation.

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Zwolan, T A; Shepard, N T; Niparko, J K

1993-05-01

Numerous reports indicate that the cochlea remains responsive to electrical stimulation following labyrinthectomy. We report a case of a 47-year-old woman with a severe to profound sensorineural hearing loss from birth, who developed episodic vertigo with symptoms suggestive of delayed onset endolymphatic hydrops. Following 8 months of failed medical and vestibular rehabilitation management, a right-sided labyrinthectomy combined with cochlear implantation was performed without complication. Postoperatively the patient was free of vertigo. Attempts to activate the patient's device between 4 to 12 weeks after surgery were unsuccessful as stimulation of the electrodes resulted in discomfort. However, all 20 electrodes elicited comfortable hearing sensations 16 weeks postsurgery. One year after the successful activation, the patient demonstrated improved sound awareness and speech recognition with the implant when compared with preoperative performance with a hearing aid. This case study suggests that electrical detection thresholds with prosthetic stimulation may be unstable in the recently labyrinthectomized ear but supports and extends prior observations of preserved cochlear responsiveness after labyrinthectomy.

MR imaging of the cochlear modiolus after intratympanic administration of Gd-DTPA

International Nuclear Information System (INIS)

Kawai, Hisashi; Naganawa, Shinji; Ishihara, Shunichi; Sone, Michihiko; Nakashima, Tsutomu

2010-01-01

We evaluated whether enhancement of the cochlear modiolus could be visualized 24 hours after intratympanic injection of gadolinium diethylenetriamine penta-acetic acid (Gd-DTPA) using a 3-dimensional real inversion recovery sequence combined with a 32-channel head coil at 3 tesla. Intratympanic injection of Gd-DTPA has been reported for visualizing endolymphatic hydrops in Meniere's disease, and its use has shown communication between the cochlear perilymph and cerebrospinal fluid in the internal auditory canal. Although the cochlear modiolus has been considered the route for this communication, this has not been confirmed through direct visualization of its enhancement. We qualitatively and quantitatively evaluated the presence of contrast enhancement in the modiolus in 19 patients with clinically suspected endolymphatic hydrops or hearing loss who underwent imaging as described above. The contrast ratio (CR) between the cochlear modiolus and cerebellar white matter on the injected side was 1.09±1.23, and that on the non-injected side was -0.48±0.38 (P<0.01). In all subjects, the CR value was larger on the injected than non-injected side, and enhancement of the cochlear modiolus was also recognized visually. Intratympanic Gd-DTPA can be administered to visualize enhancement of the cochlear modiolus and may thereby reveal its functional anatomy. (author)

Impaired olfactory function in patients with polycystic ovary syndrome

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Sezen Bozkurt Koseoglu

2016-06-01

Full Text Available Polycystic ovary syndrome (PCOS is an endocrine disorder which affects 6.6% of women of child-bearing age. Although olfactory dysfunction is frequent in the population and it negatively affects quality of life, neither physicians or patients consider this important. This case-control study included 30 patients diagnosed with PCOS, and 25 healthy age-matched controls. Sniffin' sticks tests (BurghartGmbH, Wedel, Germany were used to analyze olfactory functions, and the Beck Depression Inventory was used to evaluate depressive symptoms. The total odor score was significantly lower in the PCOS group compared to the control group (p<0.005. The Beck depression score was higher in the PCOS group (p<0.005. There was a negative correlation between the total odor score and the Beck Depression Score. Patients with PCOS have impaired olfactory function. This might be related to depressive disorders that are also observed in those patients.

Effects of a Word-Learning Training on Children With Cochlear Implants

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Lund, Emily

2014-01-01

Preschool children with hearing loss who use cochlear implants demonstrate vocabulary delays when compared to their peers without hearing loss. These delays may be a result of deficient word-learning abilities; children with cochlear implants perform more poorly on rapid word-learning tasks than children with normal hearing. This study explored the malleability of rapid word learning of preschoolers with cochlear implants by evaluating the effects of a word-learning training on rapid word learning. A single-subject, multiple probe design across participants measured the impact of the training on children’s rapid word-learning performance. Participants included 5 preschool children with cochlear implants who had an expressive lexicon of less than 150 words. An investigator guided children to identify, repeat, and learn about unknown sets of words in 2-weekly sessions across 10 weeks. The probe measure, a rapid word-learning task with a different set of words than those taught during training, was collected in the baseline, training, and maintenance conditions. All participants improved their receptive rapid word-learning performance in the training condition. The functional relation indicates that the receptive rapid word-learning performance of children with cochlear implants is malleable. PMID:23981321

Cochlear implantation in the Mondini inner ear malformation.

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Miyamoto, R T; Robbins, A J; Myres, W A; Pope, M L

1986-07-01

We report the case of a profoundly deaf 4-year-old boy with congenital deafness as a result of Mondini's dysplasia. The Mondini inner ear malformation is the result of arrested labyrinthine development during embryogenesis and is characterized by both bony and membranous anomalies of the inner ear. The dysplastic cochlear anatomy does not preclude successful cochlear implantation, and electrical threshold measurements are similar to those recorded in pediatric subjects deafened as a result of other causes.

Spectral-Temporal Modulated Ripple Discrimination by Children With Cochlear Implants.

Science.gov (United States)

Landsberger, David M; Padilla, Monica; Martinez, Amy S; Eisenberg, Laurie S

A postlingually implanted adult typically develops hearing with an intact auditory system, followed by periods of deafness (or near deafness) and adaptation to the implant. For an early implanted child whose brain is highly plastic, the auditory system matures with consistent input from a cochlear implant. It is likely that the auditory system of early implanted cochlear implant users is fundamentally different than postlingually implanted adults. The purpose of this study is to compare the basic psychophysical capabilities and limitations of these two populations on a spectral resolution task to determine potential effects of early deprivation and plasticity. Performance on a spectral resolution task (Spectral-temporally Modulated Ripple Test [SMRT]) was measured for 20 bilaterally implanted, prelingually deafened children (between 5 and 13 years of age) and 20 hearing children within the same age range. Additionally, 15 bilaterally implanted, postlingually deafened adults, and 10 hearing adults were tested on the same task. Cochlear implant users (adults and children) were tested bilaterally, and with each ear alone. Hearing listeners (adults and children) were tested with the unprocessed SMRT and with a vocoded version that simulates an 8-channel cochlear implant. For children with normal hearing, a positive correlation was found between age and SMRT score for both the unprocessed and vocoded versions. Older hearing children performed similarly to hearing adults in both the unprocessed and vocoded test conditions. However, for children with cochlear implants, no significant relationship was found between SMRT score and chronological age, age at implantation, or years of implant experience. Performance by children with cochlear implants was poorer than performance by cochlear implanted adults. It was also found that children implanted sequentially tended to have better scores with the first implant compared with the second implant. This difference was not

Audiological manifestations in mitochondrial encephalomyopathy lactic acidosis and stroke like episodes (MELAS) syndrome.

Science.gov (United States)

Vandana, V P; Bindu, Parayil Sankaran; Sonam, Kothari; Govindaraj, Periyasamy; Taly, Arun B; Gayathri, Narayanappa; Chiplunkar, Shwetha; Govindaraju, Chikkanna; Arvinda, H R; Nagappa, Madhu; Sinha, Sanjib; Thangaraj, Kumarasamy

2016-09-01

Reports of audiological manifestations in specific subgroups of mitochondrial disorders are limited. This study aims to describe the audiological findings in patients with MELAS syndrome and m.3243A>G mutation. Audiological evaluation was carried out in eight patients with confirmed MELAS syndrome and m.3243A>G mutation. The evaluation included a complete neurological evaluation, pure tone audiometry (n=8), otoacoustic emissions (n=8) and brainstem evoked response audiometry (n=6), magnetic resonance imaging (n=8) and muscle biospy (n=6). Eight patients (Age range: 5-45 years; M:F-1:3) including six children and two adults underwent formal audiological evaluation. Five patients had hearing loss; of these two had "subclinical hearing loss", one had moderate and two had severe hearing loss. The abnormalities included abnormal audiometry (n=5), otoacoustic emission testing (n=7) and absent brainstem auditory evoked responses (n=1). The findings were suggestive of cochlear involvement in four and retrocochlear in one. This study shows that hearing loss of both cochlear and retrocochlear origin occurs in patients with MELAS and may be subclinical. Early referrals for audiological evaluation is warranted to recognize the subclinical hearing loss in these patients. The therapeutic implications include early interventions in the form of hearing aids, cochlear implants and cautioning the physicians for avoidance of aminoglycosides. Copyright © 2016 Elsevier B.V. All rights reserved.

Rehabilitation of deaf persons with cochlear implants

International Nuclear Information System (INIS)

Gstoettner, W.; Hamzavi, J.; Czerny, C.

1997-01-01

In the last decade, the rehabilitation of postlingually deaf adults and prelingually deaf children with cochlear implants has been established as a treatment of deafness. The technological development of the implant devices and improvement of the surgical technique have led to a considerable increase of hearing performance during the last years. The postlingually deaf adults are able to use the telephone and may be integrated in their original job. Prelingually deaf children can even visit normal schools after cochlear implantation and hearing rehabilitation training. In order to preoperatively establish the state of the cochlear, radiological diagnosis of the temporal bone is necessary. High resolution computerized tomography imaging of the temporal bone with coronar and axial 1 mm slices and MRI with thin slice technique (three dimensional, T2 weighted turbo-spinecho sequence with 0.7 mm slices) have proved to be valuable according to our experience. Furthermore a postoperative synoptical X-ray, in a modified Chausse III projection, offers good information about the position of the implant and insertion of the stimulating electrode into the cochlea. (orig.) [de

Audio-Tactile Integration in Congenitally and Late Deaf Cochlear Implant Users

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Nava, Elena; Bottari, Davide; Villwock, Agnes; Fengler, Ineke; Büchner, Andreas; Lenarz, Thomas; Röder, Brigitte

2014-01-01

Several studies conducted in mammals and humans have shown that multisensory processing may be impaired following congenital sensory loss and in particular if no experience is achieved within specific early developmental time windows known as sensitive periods. In this study we investigated whether basic multisensory abilities are impaired in hearing-restored individuals with deafness acquired at different stages of development. To this aim, we tested congenitally and late deaf cochlear implant (CI) recipients, age-matched with two groups of hearing controls, on an audio-tactile redundancy paradigm, in which reaction times to unimodal and crossmodal redundant signals were measured. Our results showed that both congenitally and late deaf CI recipients were able to integrate audio-tactile stimuli, suggesting that congenital and acquired deafness does not prevent the development and recovery of basic multisensory processing. However, we found that congenitally deaf CI recipients had a lower multisensory gain compared to their matched controls, which may be explained by their faster responses to tactile stimuli. We discuss this finding in the context of reorganisation of the sensory systems following sensory loss and the possibility that these changes cannot be “rewired” through auditory reafferentation. PMID:24918766

Audio-tactile integration in congenitally and late deaf cochlear implant users.

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Elena Nava

Full Text Available Several studies conducted in mammals and humans have shown that multisensory processing may be impaired following congenital sensory loss and in particular if no experience is achieved within specific early developmental time windows known as sensitive periods. In this study we investigated whether basic multisensory abilities are impaired in hearing-restored individuals with deafness acquired at different stages of development. To this aim, we tested congenitally and late deaf cochlear implant (CI recipients, age-matched with two groups of hearing controls, on an audio-tactile redundancy paradigm, in which reaction times to unimodal and crossmodal redundant signals were measured. Our results showed that both congenitally and late deaf CI recipients were able to integrate audio-tactile stimuli, suggesting that congenital and acquired deafness does not prevent the development and recovery of basic multisensory processing. However, we found that congenitally deaf CI recipients had a lower multisensory gain compared to their matched controls, which may be explained by their faster responses to tactile stimuli. We discuss this finding in the context of reorganisation of the sensory systems following sensory loss and the possibility that these changes cannot be "rewired" through auditory reafferentation.

Can Impairment Interfere with Performance by Women with Carpal Tunnel Syndrome According to International Classification of Function?

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Somayeh Kavousipor

2015-03-01

Full Text Available Background: Carpal tunnel syndrome is the most prevalent compression neuropathy of upper extremity which, two of the most important risk factors of that are the female sex and manual works. In the model of international classification of function, disability and health, disease is an impairment, results in functional limitation. The goal of this study is to compare hand function of participants between various severities of carpal tunnel syndrome. Methods: In a cross-sectional study, during 6 months period of time, 30 housekeeper women with carpal tunnel syndrome, with the mean age of 47.03 years, were selected through simple sampling. They were assessed for hand function, by Purdue peg board test and Boston questionnaire, after that a professional practitioner had performed Nerve Conductive Velocity (NCV test and identified the severity of their diseases. Then the data were analyzed with SPSS software, by Kruskal-Wallis test. Results: The mean of Purdue peg board test and Boston questionnaire scores in various clusters of carpal tunnel syndrome severity, were not different (P>0.05. Conclusion: In this research, severity of electrodiagnostic findings of participants, with carpal tunnel syndrome, is not related to their performance and functional limitations.

Obstructive sleep apnea syndrome and cognitive impairment: effects of CPAP

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Alessandra Giordano

2011-10-01

Full Text Available Obstructive Sleep Apnea Syndrome (OSAS is a sleep disorder characterised by repetitive episodes of upper airway obstruction (apnea or reduced airflow (hypopnoea despite persistent respiratory effort. Apnea is defined as the cessation of breathing for at least 10 seconds during sleep, while hypopnoea is defined as at least 30% reduction in airflow for 10 seconds associated with oxygen desaturation and sleep fragmentation. The presence in the general population is about 4%. The principal symptoms are: excessive daytime sleepiness (EDS, snoring, dry throat, morning headache, night sweats, gastro-esophageal reflux, and increased blood pressure.Long term complications can be: increased cardio-cerebrovascular risk and cognitive impairment such as deficiency in attention, vigilance, visual abilities, thought, speech, perception and short term memory.Continuous Positive Airway Pressure (CPAP is currently the best non-invasive therapy for OSAS.CPAP guarantees the opening of upper airways using pulmonary reflexive mechanisms increasing lung volume during exhalation and resistance reduction, decreasing electromyografical muscular activity around airways.The causes of cognitive impairments and their possible reversibility after CPAP treatment have been analysed in numerous studies. The findings, albeit controversial, show that memory, attention and executive functions are the most compromised cognitive functions.The necessity of increasing the patient compliance with ventilotherapy is evident, in order to prevent cognitive deterioration and, when possible, rehabilitate the compromised functions, a difficult task for executive functions.

Adult-Onset Fluoxetine Treatment Does Not Improve Behavioral Impairments and May Have Adverse Effects on the Ts65Dn Mouse Model of Down Syndrome

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Markus Heinen

2012-01-01

Full Text Available Down syndrome is caused by triplication of chromosome 21 and is associated with neurocognitive phenotypes ranging from severe intellectual disability to various patterns of more selective neuropsychological deficits, including memory impairments. In the Ts65Dn mouse model of Down syndrome, excessive GABAergic neurotransmission results in local over-inhibition of hippocampal circuits, which dampens hippocampal synaptic plasticity and contributes to cognitive impairments. Treatments with several GABAA receptor antagonists result in increased plasticity and improved memory deficits in Ts65Dn mice. These GABAA receptor antagonists are, however, not suitable for clinical applications. The selective serotonin reuptake inhibitor fluoxetine, in contrast, is a widely prescribed antidepressant that can also enhance plasticity in the adult rodent brain by lowering GABAergic inhibition. For these reasons, we wondered if an adult-onset 4-week oral fluoxetine treatment restores spatial learning and memory impairments in Ts65Dn mice. Fluoxetine did not measurably improve behavioral impairments of Ts65Dn mice. On the contrary, we observed seizures and mortality in fluoxetine-treated Ts65Dn mice, raising the possibility of a drug × genotype interaction with respect to these adverse treatment outcomes. Future studies should re-address this in larger animal cohorts and determine if fluoxetine treatment is associated with adverse treatment effects in individuals with Down syndrome.

Modeling auditory perception of individual hearing-impaired listeners

DEFF Research Database (Denmark)

Jepsen, Morten Løve; Dau, Torsten

showed that, in most cases, the reduced or absent cochlear compression, associated with outer hair-cell loss, quantitatively accounts for broadened auditory filters, while a combination of reduced compression and reduced inner hair-cell function accounts for decreased sensitivity and slower recovery from...... selectivity. Three groups of listeners were considered: (a) normal hearing listeners; (b) listeners with a mild-to-moderate sensorineural hearing loss; and (c) listeners with a severe sensorineural hearing loss. A fixed set of model parameters were derived for each hearing-impaired listener. The simulations...

The Phonemic Awareness Skills of Cochlear Implant Children and Children with Normal Hearing in Primary School

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Aliakbar Dashtelei

2015-12-01

Full Text Available Objectives: Phonemic awareness skills have a significant impact on children speech and language. The purpose of this study was investigating the phonemic awareness skills of children with cochlear implant and normal hearing peers in primary school. Methods: phonemic awareness subscales of phonological awareness test were administered to 30 children with cochlear implantation at the first to sixth grades of primary school and 30 children with normal hearing who were matched in age with cochlear implant group. All of children were between 6 to 11 years old. Children with cochlear implant had at least 1 to 2 years of implant experience and they were over 5 years when they receive implantation. Children with cochlear implant were selected from Special education centers in Tehran and children with normal hearing were recruited from primary schools in Tehran. The phonemic awareness skills were assessed in both groups. Results: The results showed that the Mean scores of phonemic awareness skills in cochlear implant children were significantly lower than children with normal hearing (P<.0001. Discussion: children with cochlear implant, despite Cochlear implantation prosthesis, had lower performance in phonemic awareness when compared with normal hearing children. Therefore, due to importance of phonemic awareness skills in learning of literacy skills, and defects of these skills in children with cochlear implant, these skills should be assessed carefully in children with cochlear implant and rehabilitative interventions should be considered.

Pre-, intra- and post-operative imaging of cochlear implants

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Vogl, T.J.; Naguib, N.N.N.; Burck, I. [University Hospital Frankfurt (Germany). Inst. of Diagnostic and Interventional Radiology; Tawfik, A. [Mansoura Univ. (Egypt). Dept. of Diagnostic and Interventional Radiology; Emam, A. [University Hospital Alexandria (Egypt). Dept. of Diagnostic and Interventional Radiology; Nour-Eldin, A. [University Hospital Cairo (Egypt). Dept. of Radiology; Stoever, T. [University Hospital of Frankfurt (Germany). Dept. of Otolaryngology

2015-11-15

The purpose of this review is to present essential imaging aspects in patients who are candidates for a possible cochlear implant as well as in postsurgical follow-up. Imaging plays a major role in providing information on preinterventional topography, variations and possible infections. Preoperative imaging using DVT, CT, MRI or CT and MRI together is essential for candidate selection, planning of surgical approach and exclusion of contraindications like the complete absence of the cochlea or cochlear nerve, or infection. Relative contraindications are variations of the cochlea and vestibulum. Intraoperative imaging can be performed by fluoroscopy, mobile radiography or DVT. Postoperative imaging is regularly performed by conventional X-ray, DVT, or CT. In summary, radiological imaging has its essential role in the pre- and post-interventional period for patients who are candidates for cochlear implants.

Is there a best side for cochlear implants in post-lingual patients?

Science.gov (United States)

Amaral, Maria Stella Arantes do; Damico, Thiago A; Gonçales, Alina S; Reis, Ana C M B; Isaac, Myriam de Lima; Massuda, Eduardo T; Hyppolito, Miguel Angelo

2017-07-29

Cochlear Implant is a sensory prosthesis capable of restoring hearing in patients with severe or profound bilateral sensorineural hearing loss. To evaluate if there is a better side to be implanted in post-lingual patients. Retrospective longitudinal study. Participants were 40 subjects, of both sex, mean age of 47 years, with post-lingual hearing loss, users of unilateral cochlear implant for more than 12 months and less than 24 months, with asymmetric auditor reserve between the ears (difference of 10dBNA, In at least one of the frequencies with a response, between the ears), divided into two groups. Group A was composed of individuals with cochlear implant in the ear with better auditory reserve and Group B with auditory reserve lower in relation to the contralateral side. There was no statistical difference for the tonal auditory threshold before and after cochlear implant. A better speech perception in pre-cochlear implant tests was present in B (20%), but the final results are similar in both groups. The cochlear implant in the ear with the worst auditory residue favors a bimodal hearing, which would allow the binaural summation, without compromising the improvement of the audiometric threshold and the speech perception. Copyright © 2017 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

A deleterious Nav1.1 mutation selectively impairs telencephalic inhibitory neurons derived from Dravet Syndrome patients

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Sun, Yishan; Paşca, Sergiu P; Portmann, Thomas; Goold, Carleton; Worringer, Kathleen A; Guan, Wendy; Chan, Karen C; Gai, Hui; Vogt, Daniel; Chen, Ying-Jiun J; Mao, Rong; Chan, Karrie; Rubenstein, John LR; Madison, Daniel V; Hallmayer, Joachim; Froehlich-Santino, Wendy M; Bernstein, Jonathan A; Dolmetsch, Ricardo E

2016-01-01

Dravet Syndrome is an intractable form of childhood epilepsy associated with deleterious mutations in SCN1A, the gene encoding neuronal sodium channel Nav1.1. Earlier studies using human induced pluripotent stem cells (iPSCs) have produced mixed results regarding the importance of Nav1.1 in human inhibitory versus excitatory neurons. We studied a Nav1.1 mutation (p.S1328P) identified in a pair of twins with Dravet Syndrome and generated iPSC-derived neurons from these patients. Characterization of the mutant channel revealed a decrease in current amplitude and hypersensitivity to steady-state inactivation. We then differentiated Dravet-Syndrome and control iPSCs into telencephalic excitatory neurons or medial ganglionic eminence (MGE)-like inhibitory neurons. Dravet inhibitory neurons showed deficits in sodium currents and action potential firing, which were rescued by a Nav1.1 transgene, whereas Dravet excitatory neurons were normal. Our study identifies biophysical impairments underlying a deleterious Nav1.1 mutation and supports the hypothesis that Dravet Syndrome arises from defective inhibitory neurons. DOI: http://dx.doi.org/10.7554/eLife.13073.001 PMID:27458797

Cochlear implantation in children with congenital cytomegalovirus infection accompanied by psycho-neurological disorders.

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Yamazaki, Hiroshi; Yamamoto, Rinko; Moroto, Saburo; Yamazaki, Tomoko; Fujiwara, Keizo; Nakai, Masako; Ito, Juichi; Naito, Yasushi

2012-04-01

Cochlear implantation was effective for deaf children with congenital cytomegalovirus (CMV) infection, but their cochlear implant (CI) outcomes were often impaired, depending on the types of CMV-associated psycho-neurological disorders. Evaluation of cognitive development and autistic tendency of implantees might be useful to predict their CI outcomes. To reveal the influence of CMV-associated psycho-neurological disorders on CI outcomes. This was a retrospective evaluation of 11 implantees with congenital CMV infection (CMV-CIs) and 14 implantees with autosomal recessive hearing loss (genetic-CIs). Nine of 11 CMV-CIs suffered from psycho-neurological disorders; one from attention deficit hyperactivity disorder, two from pervasive developmental disorder, and six from mental retardation. Aided hearing thresholds with CIs in the two groups did not differ, but two autistic and two mentally retarded CMV-CIs showed significantly low scores in speech discrimination tests. Language-Social (L-S) developmental quotients (DQs) evaluated by the Kyoto Scale of Psychological development were improved after the implantation in both groups, but the postoperative increase of L-S DQs was significantly smaller in the CMV-CIs than that of genetic-CIs. Interestingly, the postoperative L-S and Cognitive-Adaptive (C-A) DQs showed statistically significant correlation in all cases except for two autistic CMV-CIs whose L-S DQs were much lower than those expected from their C-A DQs.

STEREOLOGICAL ANALYSIS OF THE COCHLEAR NUCLEI OF MONKEY (MACACA FASCICULARIS AFTER DEAFFERENTATION

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Ana M Insausti

2011-05-01

Full Text Available The cochlear nuclei (CN in the brainstem receive the input signals from the inner ear through the cochlear nerve, and transmit these signals to higher auditory centres. A variety of lesions of the cochlear nerve cause deafness. As reported in the literature, artificial removal of auditive input, or 'deafferentation', induces structural alterations in the CN. The purpose of this study was to estimate a number of relevant stereological parameters of the CN in control and deafferented Macaca fascicularis monkeys.

Sequential bilateral cochlear implantation in children: parents' perspective and device use.

NARCIS (Netherlands)

Sparreboom, M.; Leeuw, A.R.; Snik, A.F.M.; Mylanus, E.A.M.

2012-01-01

OBJECTIVE: The purpose of this study was (1) to measure parental expectations before surgery of a sequentially placed second cochlear implant and compare these results with parental observations postoperatively and (2) to measure device use of the second cochlear implant and compare to unilateral

Individuals with 22q11.2 Deletion Syndrome Are Impaired at Explicit, but Not Implicit, Discrimination of Local Forms Embedded in Global Structures

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Giersch, Anne; Glaser, Bronwyn; Pasca, Catherine; Chabloz, Mélanie; Debbané, Martin; Eliez, Stephan

2014-01-01

Individuals with 22q11.2 deletion syndrome (22q11.2DS) are impaired at exploring visual information in space; however, not much is known about visual form discrimination in the syndrome. Thirty-five individuals with 22q11.2DS and 41 controls completed a form discrimination task with global forms made up of local elements. Affected individuals…

Visualization of spiral ganglion neurites within the scala tympani with a cochlear implant in situ.

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Chikar, Jennifer A; Batts, Shelley A; Pfingst, Bryan E; Raphael, Yehoash

2009-05-15

Current cochlear histology methods do not allow in situ processing of cochlear implants. The metal components of the implant preclude standard embedding and mid-modiolar sectioning, and whole mounts do not have the spatial resolution needed to view the implant within the scala tympani. One focus of recent auditory research is the regeneration of structures within the cochlea, particularly the ganglion cells and their processes, and there are multiple potential benefits to cochlear implant users from this work. To facilitate experimental investigations of auditory nerve regeneration performed in conjunction with cochlear implantation, it is critical to visualize the cochlear tissue and the implant together to determine if the nerve has made contact with the implant. This paper presents a novel histological technique that enables simultaneous visualization of the in situ cochlear implant and neurofilament-labeled nerve processes within the scala tympani, and the spatial relationship between them.

A Case of Cochlear Implantation in Bromate-Induced Bilateral Sudden Deafness.

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Eom, Tae-Ho; Lee, Sungsu; Cho, Hyong-Ho; Cho, Yong-Beom

2015-04-01

Despite the well-established nature of bromate-induced ototoxicity, cochlear implantation after bromate intoxication has been rarely documented. We hereby present a case of a 51-year-old female deafened completely after bromate ingestion. Her hearing was not restored by systemic steroid treatment and hearing aids were of no use. A cochlear implantation was performed on her right ear 3 months after the bromate ingestion. In bromate intoxication cases, early monitoring of hearing level is necessary and other drugs with potential ototoxicity should be avoided. The outcome of cochlear implantation was excellent in this case of bromate-induced deafness.

Topics in Modeling of Cochlear Dynamics: Computation, Response and Stability Analysis

Science.gov (United States)

Filo, Maurice G.

This thesis touches upon several topics in cochlear modeling. Throughout the literature, mathematical models of the cochlea vary according to the degree of biological realism to be incorporated. This thesis casts the cochlear model as a continuous space-time dynamical system using operator language. This framework encompasses a wider class of cochlear models and makes the dynamics more transparent and easier to analyze before applying any numerical method to discretize space. In fact, several numerical methods are investigated to study the computational efficiency of the finite dimensional realizations in space. Furthermore, we study the effects of the active gain perturbations on the stability of the linearized dynamics. The stability analysis is used to explain possible mechanisms underlying spontaneous otoacoustic emissions and tinnitus. Dynamic Mode Decomposition (DMD) is introduced as a useful tool to analyze the response of nonlinear cochlear models. Cochlear response features are illustrated using DMD which has the advantage of explicitly revealing the spatial modes of vibrations occurring in the Basilar Membrane (BM). Finally, we address the dynamic estimation problem of BM vibrations using Extended Kalman Filters (EKF). Due to the limitations of noninvasive sensing schemes, such algorithms are inevitable to estimate the dynamic behavior of a living cochlea.

Rapid learning of minimally different words in five- to six-year-old children : Effects of acoustic salience and hearing impairment

NARCIS (Netherlands)

Giezen, M.R.; Escudero, P.; Baker, A.E.

This study investigates the role of acoustic salience and hearing impairment in learning phonologically minimal pairs. Picture-matching and object-matching tasks were used to investigate the learning of consonant and vowel minimal pairs in five- to six-year-old deaf children with a cochlear implant

Protective role of hydrogen sulfide against noise-induced cochlear damage: a chronic intracochlear infusion model.

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Xu Li

Full Text Available BACKGROUND: A reduction in cochlear blood flow plays an essential role in noise-induced hearing loss (NIHL. The timely regulation of cochlear perfusion determines the progression and prognosis of NIHL. Hydrogen sulfide (H(2S has attracted increasing interest as a vasodilator in cardiovascular systems. This study identified the role of H(2S in cochlear blood flow regulation and noise protection. METHODOLOGY/PRINCIPAL FINDINGS: The gene and protein expression of the H(2S synthetase cystathionine-γ-lyase (CSE in the rat cochlea was examined using immunofluorescence and real-time PCR. Cochlear CSE mRNA levels varied according to the duration of noise exposure. A chronic intracochlear infusion model was built and artificial perilymph (AP, NaHS or DL-propargylglycine (PPG were locally administered. Local sodium hydrosulfide (NaHS significantly increased cochlear perfusion post-noise exposure. Cochlear morphological damage and hearing loss were alleviated in the NaHS group as measured by conventional auditory brainstem response (ABR, cochlear scanning electron microscope (SEM and outer hair cell (OHC count. The highest percentage of OHC loss occurred in the PPG group. CONCLUSIONS/SIGNIFICANCE: Our results suggest that H(2S plays an important role in the regulation of cochlear blood flow and the protection against noise. Further studies may identify a new preventive and therapeutic perspective on NIHL and other blood supply-related inner ear diseases.

Cochlear pathology: CT and MRI findings

International Nuclear Information System (INIS)

Martinez, Manuel; Bruno, Claudio; Martin, Eduardo; Canale, Nancy; De Luca, Laura; Spina, Juan C. h

2002-01-01

The authors present a retrospective analysis of 50 patients with perceptive hearing loss. The neuro physiological work-up (evoked potentials, audiometric tests and otoemissions) identified the cochlea as the site of origin of the hearing loss. The imaging studies (high resolution CT and MR, including 3D volumes) showed petrous bone trauma (n=5) infections (bacterial or viral origin) (n=12) otosclerosis (n=8), Paget disease (n=3), and labyrinthine neurinomas (n=2). The other 20 cases showed development-linked disorders (Mitchel's, Mondini, Scheibe, Alexander, and hypoplasia of the cochlear canal). Imaging with CT and MR can identify in detail the structural abnormality affecting the cochlea and occasionally the immediate peri-cochlear region (otic capsule); the affected portion of the cochlea may thus be determined. (author)

Speech Perception Benefits of Internet Versus Conventional Telephony for Hearing-Impaired Individuals

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Dubach, Patrick; Pfiffner, Flurin; Kompis, Martin; Caversaccio, Marco

2012-01-01

Background Telephone communication is a challenge for many hearing-impaired individuals. One important technical reason for this difficulty is the restricted frequency range (0.3–3.4 kHz) of conventional landline telephones. Internet telephony (voice over Internet protocol [VoIP]) is transmitted with a larger frequency range (0.1–8 kHz) and therefore includes more frequencies relevant to speech perception. According to a recently published, laboratory-based study, the theoretical advantage of ideal VoIP conditions over conventional telephone quality has translated into improved speech perception by hearing-impaired individuals. However, the speech perception benefits of nonideal VoIP network conditions, which may occur in daily life, have not been explored. VoIP use cannot be recommended to hearing-impaired individuals before its potential under more realistic conditions has been examined. Objective To compare realistic VoIP network conditions, under which digital data packets may be lost, with ideal conventional telephone quality with respect to their impact on speech perception by hearing-impaired individuals. Methods We assessed speech perception using standardized test material presented under simulated VoIP conditions with increasing digital data packet loss (from 0% to 20%) and compared with simulated ideal conventional telephone quality. We monaurally tested 10 adult users of cochlear implants, 10 adult users of hearing aids, and 10 normal-hearing adults in the free sound field, both in quiet and with background noise. Results Across all participant groups, mean speech perception scores using VoIP with 0%, 5%, and 10% packet loss were 15.2% (range 0%–53%), 10.6% (4%–46%), and 8.8% (7%–33%) higher, respectively, than with ideal conventional telephone quality. Speech perception did not differ between VoIP with 20% packet loss and conventional telephone quality. The maximum benefits were observed under ideal VoIP conditions without packet loss and

Reading skills after cochlear implantation

NARCIS (Netherlands)

Vermeulen, A.M.

2007-01-01

It has frequently been found that profoundly deaf children with conventional hearing aids have difficulties with the comprehension of written text. Cochlear Implants (CIs) were expected to enhance the reading comprehension of these profoundly deaf children because they provide auditory access to

Learning and Memory Processes Following Cochlear Implantation:The Missing Piece of the Puzzle

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David B. Pisoni

2016-04-01

Full Text Available At the present time, there is no question that cochlear implants work and often work very well in quiet listening conditions for many profoundly deaf children and adults. The speech and language outcomes data published over the last two decades document quite extensively the clinically significant benefits of cochlear implants. Although there now is a large body of evidence supporting the efficacy of cochlear implants as a medical intervention for profound hearing loss in both children and adults, there still remain a number of challenging unresolved clinical and theoretical issues that deal with the effectiveness of cochlear implants in individual patients that have not yet been successfully resolved. In this paper, we review recent findings on learning and memory, two central topics in the field of cognition that have been seriously neglected in research on cochlear implants. Our research findings on sequence learning, memory and organization processes, and retrieval strategies used in verbal learning and memory of categorized word lists suggests that basic domain-general learning abilities may be the missing piece of the puzzle in terms of understanding the cognitive factors that underlie the enormous individual differences and variability routinely observed in speech and language outcomes following cochlear implantation.

Language understanding and vocabulary of early cochlear implanted children

DEFF Research Database (Denmark)

Percy-Smith, L; Busch, GW; Sandahl, M

2013-01-01

The aim of the study was to identify factors associated with the level of language understanding, the level of receptive and active vocabulary, and to estimate effect-related odds ratios for cochlear implanted children's language level.......The aim of the study was to identify factors associated with the level of language understanding, the level of receptive and active vocabulary, and to estimate effect-related odds ratios for cochlear implanted children's language level....

Computational tool for postoperative evaluation of cochlear implant patients

International Nuclear Information System (INIS)

Giacomini, Guilherme; Pavan, Ana Luiza M.; Pina, Diana R. de; Altemani, Joao M.C.; Castilho, Arthur M.

2016-01-01

The aim of this study was to develop a tool to calculate the insertion depth angle of cochlear implants, from computed tomography exams. The tool uses different image processing techniques, such as thresholding and active contour. Then, we compared the average insertion depth angle of three different implant manufacturers. The developed tool can be used, in the future, to compare the insertion depth angle of the cochlear implant with postoperative response of patient's hearing. (author)

Staphylococcus lugdunensis: novel organism causing cochlear implant infection

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Samina Bhumbra

2014-06-01

Full Text Available A majority of cochlear implant infections are caused by Staphylococcus aureus or Pseudomonas aeruginosa. Reported here is a pediatric patient with a cochlear implant infection caused by methicillin-resistant Staphylococcus lugdunensis, a coagulase-negative Staphylococcus that has only recently been determined to be clinically relevant (1988. Unlike other coagulase-negative Staphylococcus, it is more aggressive, carrying a greater potential for tissue destruction. In pediatrics, the organism is uncommon, poorly described, and generally pan-susceptible. Described herein is the presentation and management of this unusual organism in a pediatric setting.

Hearing impairment related to age in Usher syndrome types 1B and 2A.

Science.gov (United States)

Wagenaar, M; van Aarem, A; Huygen, P; Pieke-Dahl, S; Kimberling, W; Cremers, C

1999-04-01

To evaluate hearing impairment in 2 common genetic subtypes of Usher syndrome, USH1B and USH2A. Cross-sectional analysis of hearing threshold related to age in patients with genotypes determined by linkage and mutation analysis. Otolaryngology department, university referral center. Nineteen patients with USH1B and 27 with USH2A were examined. All participants were living in the Netherlands and Belgium. Pure tone audiometry of the best ear at last visit. The patients with USH1B had residual hearing without age dependence, with minimum thresholds of 80, 95, and 120 dB at 0.25, 0.5, and 1 to 2 kHz, respectively. Mean thresholds of patients with USH2A were about 45 to 55 dB better than these minimum values. Distinctive audiographic features of patients with USH2A were maximum hearing thresholds of 70, 80, and 100 dB at 0.25, 0.5, and 1 kHz, respectively, only at younger than 40 years. Progression of hearing impairment in USH2A was 0.7 dB/y on average for 0.25 to 4 kHz and could not be explained by presbyacusis alone. The USH1B and USH2A can be easily distinguished by hearing impairment at younger than 40 years at the low frequencies. Hearing impairment in our patients with USH2A could be characterized as progressive.

Modeling and segmentation of intra-cochlear anatomy in conventional CT

Science.gov (United States)

Noble, Jack H.; Rutherford, Robert B.; Labadie, Robert F.; Majdani, Omid; Dawant, Benoit M.

2010-03-01

Cochlear implant surgery is a procedure performed to treat profound hearing loss. Since the cochlea is not visible in surgery, the physician uses anatomical landmarks to estimate the pose of the cochlea. Research has indicated that implanting the electrode in a particular cavity of the cochlea, the scala tympani, results in better hearing restoration. The success of the scala tympani implantation is largely dependent on the point of entry and angle of electrode insertion. Errors can occur due to the imprecise nature of landmark-based, manual navigation as well as inter-patient variations between scala tympani and the anatomical landmarks. In this work, we use point distribution models of the intra-cochlear anatomy to study the inter-patient variations between the cochlea and the typical anatomic landmarks, and we implement an active shape model technique to automatically localize intra-cochlear anatomy in conventional CT images, where intra-cochlear structures are not visible. This fully automatic segmentation could aid the surgeon to choose the point of entry and angle of approach to maximize the likelihood of scala tympani insertion, resulting in more substantial hearing restoration.

Cochlear Damage Affects Neurotransmitter Chemistry in the Central Auditory System

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Donald Albert Godfrey

2014-11-01

Full Text Available Tinnitus, the perception of a monotonous sound not actually present in the environment, affects nearly 20% of the population of the United States. Although there has been great progress in tinnitus research over the past 25 years, the neurochemical basis of tinnitus is still poorly understood. We review current research about the effects of various types of cochlear damage on the neurotransmitter chemistry in the central auditory system and document evidence that different changes in this chemistry can underlie similar behaviorally measured tinnitus symptoms. Most available data have been obtained from rodents following cochlear damage produced by cochlear ablation, loud sound, or ototoxic drugs. Effects on neurotransmitter systems have been measured as changes in neurotransmitter level, synthesis, release, uptake, and receptors. In this review, magnitudes of changes are presented for neurotransmitter-related amino acids, acetylcholine, and serotonin. A variety of effects have been found in these studies that may be related to animal model, survival time, type of cochlear damage, or methodology. The overall impression from the evidence presented is that any imbalance of neurotransmitter-related chemistry could disrupt auditory processing in such a way as to produce tinnitus.

Interaction of tinnitus suppression and hearing ability after cochlear implantation.

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Wang, Qian; Li, Jia-Nan; Lei, Guan-Xiong; Chen, Dai-Shi; Wang, Wei-Ze; Chen, Ai-Ting; Mong, Meng-Di; Li, Sun; Jiao, Qing-Shan; Yang, Shi-Ming

2017-10-01

To study the postoperative impact of cochlear implants (CIs) on tinnitus, as well as the impact of tinnitus on speech recognition with CI switched on. Fifty-two postlingual deafened CI recipients (21 males and 31 females) were assessed using an established Tinnitus Characteristics Questionnaire and Tinnitus Handicap Inventory (THI) before and after cochlear implantation. The tinnitus loudness was investigated when CI was switched on and off in CI recipients with persistent tinnitus. The relation between tinnitus loudness and recipients' satisfaction of cochlear implantation was analyzed by the visual analogue scale (VAS) score. With CI 'OFF', 42 CI recipients experienced tinnitus postimplant ipsilaterally and 44 contralaterally. Tinnitus was totally suppressed ipsilateral to the CI with CI 'ON' in 42.9%, partially suppressed in 42.9%, unchanged in 11.9% and aggravated in 2.4%. Tinnitus was totally suppressed contralaterally with CI 'ON' in 31.8% of CI recipients, partially suppressed in 47.7%, unchanged in 20.5%. Pearson correlation analysis showed that tinnitus loudness and the results of cochlear implant patients satisfaction was negatively correlated (r = .674, p tinnitus. The tinnitus loudness may affect patients' satisfaction with the use of CI.

Improving Access for Pediatric and Adult Cochlear Implant Candidates in Ontario

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Yvonne Emily James

2016-10-01

Full Text Available In 2011, the Ontario Ministry of Health and Long-Term Care announced the one-time allocation of $5.9 million to be shared by cochlear implant programs at five Ontario hospitals. The primary goal of this reform was to address cochlear implant wait times. More specifically, this funding was aimed at reducing adult wait times by 50% and to completely eliminate pediatric waiting lists. Prior to this funding, wait times for pediatric and adult cochlear implants were known to exceed four years. The funding was provided in response to a growing body of research that demonstrates increased speech perception and vocabulary among pediatric recipients, and pressure from parents of children on cochlear implant waiting lists, surgeons and other involved healthcare providers (e.g., auditory verbal therapists, audiologists, and speech language pathologists. The decision to increase funding was also influenced by government stakeholders who believed this one-time investment would be returned as pediatric patients reach adulthood and are better equipped to participate in mainstream (i.e., hearing society. While this one-time funding model has the potential to eliminate wait times for pediatric patients, thereby ensuring these children can access therapeutic services as early as possible, it does not address the future of cochlear implant waiting lists or the capacity of health human resources to absorb this sudden and unprecedented influx of pediatric patients.

Mothers' Talk to Children with Down Syndrome, Language Impairment, or Typical Development about Familiar and Unfamiliar Nouns and Verbs

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Bird, Elizabeth Kay-Raining; Cleave, Patricia

2016-01-01

This study investigated how forty-six mothers modified their talk about familiar and unfamiliar nouns and verbs when interacting with their children with Down Syndrome (DS), language impairment (LI), or typical development (TD). Children (MLUs < 2·7) were group-matched on expressive vocabulary size. Mother-child dyads were recorded playing with…

A new computed tomography method to identify meningitis-related cochlear ossification and fibrosis before cochlear implantation.

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Ichikawa, Kazunori; Kashio, Akinori; Mori, Harushi; Ochi, Atushi; Karino, Shotaro; Sakamoto, Takashi; Kakigi, Akinobu; Yamasoba, Tatsuya

2014-04-01

To develop a new method to determine the presence of intracochlear ossification and/or fibrosis in cochlear implantation candidates with bilateral profound deafness following meningitis. Diagnostic test assessment. A university hospital. This study involved 15 ears from 13 patients with profound deafness following meningitis who underwent cochlear implantation. These ears showed normal structures, soft tissue, partial bony occlusion, and complete bony occlusion in 4, 3, 2, and 6 ears, respectively. We measured radiodensity in Hounsfield units (HU) using 0.5-mm-thick axial high-resolution computed tomography image slices at 3 different levels in the basal turn, the fenestration, and inferior and ascending segment sites, located along the electrode-insertion path. Pixel-level analysis on the DICOM viewer yielded actual computed tomography values of intracochlear soft tissues by eliminating the partial volume effect. The values were compared with the intraoperative findings. Values for ossification (n = 12) ranged from +547 HU to +1137 HU; for fibrosis (n = 11), from +154 HU to +574 HU; and for fluid (n = 22), from -49 HU to +255 HU. From these values, we developed 2 presets of window width (WW) and window level (WL): (1) WW: 1800, WL: 1100 (200 HU to 2000 HU) and (2) WW: 1500, WL: 1250 (500 HU to 2000 HU). The results using these 2 presets corresponded well to the intraoperative findings. Our new method is easy and feasible for preoperative determination of the presence of cochlear ossification and/or fibrosis that develops following meningitis.

Cochlear anatomy: CT and MR imaging

International Nuclear Information System (INIS)

Martinez, Manuel; Bruno, Claudio; Martin, Eduardo; Canale, Nancy; De Luca, Laura; Spina, Juan C. h

2002-01-01

The authors present a brief overview of the normal cochlear anatomy with CT and MR images in order to allow a more complete identification of the pathological findings in patients with perceptive hipoacusia. (author)

Spelling in Written Stories by School-Age Children with Cochlear Implants

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Straley, Sara G.; Werfel, Krystal L.; Hendricks, Alison Eisel

2016-01-01

This study evaluated the spelling of 3rd to 6th grade children with cochlear implants in written stories. Spelling was analysed using traditional correct/incorrect scoring as well as the Spelling Sensitivity Score, which provides linguistic information about spelling attempts. Children with cochlear implants spelled 86 per cent of words in stories…

Expressive Language Development in 45 Cochlear Implanted Children Following 2 Years of Implantation

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Seyed Basir Hashemi

2011-10-01

Full Text Available Objectives: Profound hearing loss encounters children with delay in speech and language. As it is known language acquisition in young deaf children is a lengthy process, but cochlear implanted children have better spoken language skills than if they had not received the device. According to the importance of cochlear implant in deaf child's language development, this study evaluates the effect of different variables on child's language performance. Methods: 45 cochlear implanted children were tested, all of whom had used the device for at least 2 years. In order to evaluate the children, the NEWSHA test which is fitted for Persian speaking children was performed and language development of the children was compared through stepwise discriminant analysis. Results: After evaluation of the effect of different variables like child's age of implantation, participating in rehabilitation classes, parent's cooperation and their level of education, we came to a conclusion that the child's age of implantation and rehabilitation program significantly develop the child's language performance. Discussion: The value of cochlear implant in improvement of deaf children in speech, language perception, production and comprehension is confirmed by different studies which have been done on cochlear implanted children. Also, the present study indicates that language development in cochlear implanted children is highly related to their age of implantation and rehabilitation program.

Deriving cochlear delays in humans using otoacoustic emissions and auditory evoked potentials

DEFF Research Database (Denmark)

Pigasse, Gilles

A great deal of the processing of incoming sounds to the auditory system occurs within the cochlear. The organ of Corti within the cochlea has differing mechanical properties along its length that broadly gives rise to frequency selectivity. Its stiffness is at maximum at the base and decreases...... relation between frequency and travel time in the cochlea defines the cochlear delay. This delay is directly associated with the signal analysis occurring in the inner ear and is therefore of primary interest to get a better knowledge of this organ. It is possible to estimate the cochlear delay by direct...... and invasive techniques, but these disrupt the normal functioning of the cochlea and are usually conducted in animals. In order to obtain an estimate of the cochlear delay that is closer to the normally functioning human cochlea, the present project investigates non-invasive methods in normal hearing adults...

Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness

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Marina Davcheva-Chakar

2014-03-01

Full Text Available Background: Cochlear implants (CI for the rehabilitation of patients with profound or total bilateral sensorineural hypoacusis represent the initial use of electrical fields to provide audibility in cases where the use of sound amplifiers does not provide satisfactory results. Aims: To compare speech perception performance after cochlear implantation in children with connexin 26-associated deafness with that of a control group of children with deafness of unknown etiology. Study Design: Retrospective comparative study. Methods: During the period from 2006 to , cochlear implantation was performed on 26 children. Eighteen of these children had undergone genetic tests for mutation of the Gap Junction Protein Beta 2 (GJB2 gene. Bi-allelic GJB2 mutations were confirmed in 7 out of 18 examined children. In order to confirm whether genetic factors have influence on speech perception after cochlear implantation, we compared the post-implantation speech performance of seven children with mutations of the GBJ2 (connexin 26 gene with seven other children who had the wild type version of this particular gene. The latter were carefully matched according to the age at cochlear implantation. Speech perception performance was measured before cochlear implantation, and one and two years after implantation. All the patients were arranged in line with the appropriate speech perception category (SPC. Non-parametric tests, Friedman ANOVA and Mann-Whitney’s U test were used for statistical analysis. Results: Both groups showed similar improvements in speech perception scores after cochlear implantation. Statistical analysis did not confirm significant differences between the groups 12 and 24 months after cochlear implantation. Conclusion: The results obtained in this study showed an absence of apparent distinctions in the scores of speech perception between the two examined groups and therefore might have significant implications in selecting prognostic indicators

Immediate and delayed cochlear neuropathy after noise exposure in pubescent mice.

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Jane Bjerg Jensen

Full Text Available Moderate acoustic overexposure in adult rodents is known to cause acute loss of synapses on sensory inner hair cells (IHCs and delayed degeneration of the auditory nerve, despite the completely reversible temporary threshold shift (TTS and morphologically intact hair cells. Our objective was to determine whether a cochlear synaptopathy followed by neuropathy occurs after noise exposure in pubescence, and to define neuropathic versus non-neuropathic noise levels for pubescent mice. While exposing 6 week old CBA/CaJ mice to 8-16 kHz bandpass noise for 2 hrs, we defined 97 dB sound pressure level (SPL as the threshold for this particular type of neuropathic exposure associated with TTS, and 94 dB SPL as the highest non-neuropathic noise level associated with TTS. Exposure to 100 dB SPL caused permanent threshold shift although exposure of 16 week old mice to the same noise is reported to cause only TTS. Amplitude of wave I of the auditory brainstem response, which reflects the summed activity of the cochlear nerve, was complemented by synaptic ribbon counts in IHCs using confocal microscopy, and by stereological counts of peripheral axons and cell bodies of the cochlear nerve from 24 hours to 16 months post exposure. Mice exposed to neuropathic noise demonstrated immediate cochlear synaptopathy by 24 hours post exposure, and delayed neurodegeneration characterized by axonal retraction at 8 months, and spiral ganglion cell loss at 8-16 months post exposure. Although the damage was initially limited to the cochlear base, it progressed to also involve the cochlear apex by 8 months post exposure. Our data demonstrate a fine line between neuropathic and non-neuropathic noise levels associated with TTS in the pubescent cochlea.

AUDITORY REHABILITATION AND BILATERAL COCHLEAR IMPLANT ON CHILD WHITH CEREBRAL PALSY

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Anacléia Melo da Silva Hilgenberg

2013-01-01

Full Text Available OBJECTIVE: To describe the hearing and language improvement in children with cerebral palsy submitted to cochlear implantation bilaterally. METHODS: Prospective, analytical descriptive. Study case and a broad bibliographical analysis in MEDLINE/PUBMED and EMBASE including detailed analysis of the rehabilitation techniques as serial phonoaudiological appointments; Sessions of phonoterapy recorded and criterious analysis of the tests such as IT-MAIS and MacArthur analysis. The teste were applied after Bilateral Cochlear Implantation: 15, 20, 22, 31 e 38 months.RESULTS: The results obtained and bibliographical analysis showed that cochlear implantation in children with cerebral palsy and profound hearing loss is the best option at the moment to perform hearing rehabilitation. The IT-MAIS and MacArthur tests showed a big improvement at 15 and 38 months after use of the cochlear implants. The IT-MAIS test showed 40% score in the beggining and 75% at the end. Mac Arthur test revealed a big evolution at vocabulary comprehension (58 to 342 and linguistic repertory (54 to 289.

Using the Positive and Negative Syndrome Scale (PANSS) to Define Different Domains of Negative Symptoms: Prediction of Everyday Functioning by Impairments in Emotional Expression and Emotional Experience.

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Harvey, Philip D; Khan, Anzalee; Keefe, Richard S E

2017-12-01

Background: Reduced emotional experience and expression are two domains of negative symptoms. The authors assessed these two domains of negative symptoms using previously developed Positive and Negative Syndrome Scale (PANSS) factors. Using an existing dataset, the authors predicted three different elements of everyday functioning (social, vocational, and everyday activities) with these two factors, as well as with performance on measures of functional capacity. Methods: A large (n=630) sample of people with schizophrenia was used as the data source of this study. Using regression analyses, the authors predicted the three different aspects of everyday functioning, first with just the two Positive and Negative Syndrome Scale factors and then with a global negative symptom factor. Finally, we added neurocognitive performance and functional capacity as predictors. Results: The Positive and Negative Syndrome Scale reduced emotional experience factor accounted for 21 percent of the variance in everyday social functioning, while reduced emotional expression accounted for no variance. The total Positive and Negative Syndrome Scale negative symptom factor accounted for less variance (19%) than the reduced experience factor alone. The Positive and Negative Syndrome Scale expression factor accounted for, at most, one percent of the variance in any of the functional outcomes, with or without the addition of other predictors. Implications: Reduced emotional experience measured with the Positive and Negative Syndrome Scale, often referred to as "avolition and anhedonia," specifically predicted impairments in social outcomes. Further, reduced experience predicted social impairments better than emotional expression or the total Positive and Negative Syndrome Scale negative symptom factor. In this cross-sectional study, reduced emotional experience was specifically related with social outcomes, accounting for essentially no variance in work or everyday activities, and being the

Speech Recognition in Adults With Cochlear Implants: The Effects of Working Memory, Phonological Sensitivity, and Aging.

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Moberly, Aaron C; Harris, Michael S; Boyce, Lauren; Nittrouer, Susan

2017-04-14

Models of speech recognition suggest that "top-down" linguistic and cognitive functions, such as use of phonotactic constraints and working memory, facilitate recognition under conditions of degradation, such as in noise. The question addressed in this study was what happens to these functions when a listener who has experienced years of hearing loss obtains a cochlear implant. Thirty adults with cochlear implants and 30 age-matched controls with age-normal hearing underwent testing of verbal working memory using digit span and serial recall of words. Phonological capacities were assessed using a lexical decision task and nonword repetition. Recognition of words in sentences in speech-shaped noise was measured. Implant users had only slightly poorer working memory accuracy than did controls and only on serial recall of words; however, phonological sensitivity was highly impaired. Working memory did not facilitate speech recognition in noise for either group. Phonological sensitivity predicted sentence recognition for implant users but not for listeners with normal hearing. Clinical speech recognition outcomes for adult implant users relate to the ability of these users to process phonological information. Results suggest that phonological capacities may serve as potential clinical targets through rehabilitative training. Such novel interventions may be particularly helpful for older adult implant users.

Cyborgization: Deaf Education for Young Children in the Cochlear Implantation Era

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Valente, Joseph Michael

2011-01-01

The author, who was raised oral deaf himself, recounts a visit to a school for young deaf children and discovers that young d/Deaf children and their rights are subverted by the cochlear implantation empire. The hypercapitalist, techno-manic times of cochlear implantation has wreaked havoc to the lives of not only young children with deafness but…

The scala vestibuli for cochlear implantation. An anatomic study.

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Gulya, A J; Steenerson, R L

1996-02-01

Traditionally, cochlear implantation has used the scala tympani (ST) for electrode insertion. When faced with ST ossification, the surgeon may elect to drill out the cochlea to accomplish partial electrode insertion. Theoretically, another option in this situation is to insert the electrode into the scala vestibuli (SV). To determine whether or not the dimensions of the SV are sufficient to accommodate an electrode array so as to assess the feasibility of SV cochlear implantation. The study of 20 normal human temporal bones, comparing the maximum diameter and surface area of the ST with those of the combined SV and scala media. The dimensions of the SV and scala media were comparable to those of the ST and appeared sufficient to accommodate a cochlear implant electrode array. It appears that the combination of SV and scala media is a viable alternative route for electrode insertion, at least on the basis of anatomic dimensions, in those cases in which the ST is obliterated.

Effect of unilateral and simultaneous bilateral cochlear implantation on tinnitus : A Prospective Study

NARCIS (Netherlands)

van Zon, Alice; Smulders, Yvette E.; Ramakers, Geerte G. J.; Stegeman, Inge; Smit, Adriana L.; Van Zanten, Gijsbert A.; Stokroos, Robert J.; Hendrice, Nadia; Free, Rolien H.; Maat, Bert; Frijns, Johan H. M.; Mylanus, Emmanuel A. M.; Huinck, Wendy J.; Topsakal, Vedat; Tange, Rinze A.; Grolman, Wilko

Objectives/HypothesisTo determine the effect of cochlear implantation on tinnitus perception in patients with severe bilateral postlingual sensorineural hearing loss and to demonstrate possible differences between unilateral and bilateral cochlear implantation. Study DesignProspective study.

Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome.

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Takata, Munenori; Amiya, Eisuke; Watanabe, Masafumi; Omori, Kazuko; Imai, Yasushi; Fujita, Daishi; Nishimura, Hiroshi; Kato, Masayoshi; Morota, Tetsuro; Nawata, Kan; Ozeki, Atsuko; Watanabe, Aya; Kawarasaki, Shuichi; Hosoya, Yumiko; Nakao, Tomoko; Maemura, Koji; Nagai, Ryozo; Hirata, Yasunobu; Komuro, Issei

2014-07-01

Marfan syndrome is an inherited disorder characterized by genetic abnormality of microfibrillar connective tissue proteins. Endothelial dysfunction is thought to cause aortic dilation in subjects with a bicuspid aortic valve; however, the role of endothelial dysfunction and endothelial damaging factors has not been elucidated in Marfan syndrome. Flow-mediated dilation, a noninvasive measurement of endothelial function, was evaluated in 39 patients with Marfan syndrome. Aortic diameter was measured at the aortic annulus, aortic root at the sinus of Valsalva, sinotubular junction and ascending aorta by echocardiography, and adjusted for body surface area (BSA). The mean value of flow-mediated dilation was 6.5 ± 2.4 %. Flow-mediated dilation had a negative correlation with the diameter of the ascending thoracic aorta (AscAd)/BSA (R = -0.39, p = 0.020) and multivariate analysis revealed that flow-mediated dilation was an independent factor predicting AscAd/BSA, whereas other segments of the aorta had no association. Furthermore, Brinkman index had a somewhat greater influence on flow-mediated dilation (R = -0.42, p = 0.008). Although subjects who smoked tended to have a larger AscAd compared with non-smokers (AscA/BSA: 17.3 ± 1.8 versus 15.2 ± 3.0 mm/m(2), p = 0.013), there was no significant change in flow-mediated dilation, suggesting that smoking might affect aortic dilation via an independent pathway. Common atherogenic risks, such as impairment of flow-mediated dilation and smoking status, affected aortic dilation in subjects with Marfan syndrome.

Free-form image registration of human cochlear μCT data using skeleton similarity as anatomical prior

DEFF Research Database (Denmark)

Kjer, Hans Martin; Fagertun, Jens; Vera, Sergio

2016-01-01

Better understanding of the anatomical variability of the human cochlear is important for the design and function of Cochlear Implants. Proper non-rigid alignment of high-resolution cochlear μCT data is a challenge for the typical cubic B-spline registration model. In this paper we study one way ...

Cochlear Implantation and Single-sided Deafness: A Systematic Review of the Literature

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Cabral Junior, Francisco

2015-07-01

Full Text Available Introduction Current data show that binaural hearing is superior to unilateral hearing, specifically in the understanding of speech in noisy environments. Furthermore, unilateral hearing reduce onés ability to localize sound. Objectives This study provides a systematic review of recent studies to evaluate the outcomes of cochlear implantation in patients with single-sided deafness (SSD with regards to speech discrimination, sound localization and tinnitus suppression. Data Synthesis We performed a search in the PubMed, Cochrane Library and Lilacs databases to assess studies related to cochlear implantation in patients with unilateral deafness. After critical appraisal, eleven studies were selected for data extraction and analysis of demographic, study design and outcome data. Conclusion Although some studies have shown encouraging results on cochlear implantation and SSD, all fail to provide a high level of evidence. Larger studies are necessary to define the tangible benefits of cochlear implantation in patients with SSD.

Long latency auditory evoked potentials in children with cochlear implants: systematic review.

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Silva, Liliane Aparecida Fagundes; Couto, Maria Inês Vieira; Matas, Carla Gentile; Carvalho, Ana Claudia Martinho de

2013-11-25

The aim of this study was to analyze the findings on Cortical Auditory Evoked Potentials in children with cochlear implant through a systematic literature review. After formulation of research question and search of studies in four data bases with the following descriptors: electrophysiology (eletrofisiologia), cochlear implantation (implante coclear), child (criança), neuronal plasticity (plasticidade neuronal) and audiology (audiologia), were selected articles (original and complete) published between 2002 and 2013 in Brazilian Portuguese or English. A total of 208 studies were found; however, only 13 contemplated the established criteria and were further analyzed; was made data extraction for analysis of methodology and content of the studies. The results described suggest rapid changes in P1 component of Cortical Auditory Evoked Potentials in children with cochlear implants. Although there are few studies on the theme, cochlear implant has been shown to produce effective changes in central auditory path ways especially in children implanted before 3 years and 6 months of age.

Intracochlear pressure measurements in scala media inform models of cochlear mechanics

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Kale, Sushrut; Olson, Elizabeth S.

2015-12-01

In the classic view of cochlear mechanics, the cochlea is comprised of two identical fluid chambers separated by the cochlear partition (CP). In this view the traveling wave pressures in the two chambers mirror each other; they are equal in magnitude and opposite in phase. A fast pressure mode adds approximately uniformly. More recent models of cochlear mechanics take into account the structural complexity of the CP and the resulting additional mechanical modes would lead to distinct (non-symmetric) patterns of pressure and motion on the two sides of the CP. However, there was little to no physiological data that explored these predictions. To this aim, we measured intracochlear fluid pressure in scala media (SM), including measurements close to the sensory tissue, using miniaturized pressure sensors (˜ 80 μm outer diameter). Measurements were made in-vivo from the basal cochlear turn in gerbils. SM pressure was measured at two longitudinal locations in different preparations. In a subset of the experiments SM and ST (scala tympani) pressures were measured at the same longitudinal location. Traveling wave pressures were observed in both SM and ST, and showed the relative phase predicted by the classical theory. In addition, SM pressure showed spatial variations that had not been observed in ST, which points to a relatively complex CP motion on the SM side. These data both underscore the first-order validity of the classic cochlear traveling wave model, and open a new view to CP mechanics.

Assessment of morphological-functional state of children with cochlear implants

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V.M. Pysanko

2016-10-01

Full Text Available Purpose: assessment of morphological-functional state of pre-school age children with cochlear implants and substantiation of need in post-operative rehabilitation in period of preparation for comprehensive school. Material: we tested weakly hearing children with cochlear implants (n=127, age - 5.6±0.6 years. They were the main group. Control group consisted of children with normal hearing (n=70, age - 5.7±0.4 years. Morphological-functional state was assessed by indicators of physical and biological condition, visual analyzer, posture parameters and foot arch, muscular system and level of coordination. We calculated index of integral morphological-functional state assessment. Results: Morphological functional state of most of children (with cochlear implants was characterized by low physical condition indicators and disharmony. We observed delay in biological development. Index of morphological-functional state integral assessment witnesses, that such child can not study in comprehensive school. Rehabilitation program can reduce the gap between children with normal hearing and those with cochlear implants. Conclusions: Rehabilitation program facilitates quicker domestic and social rehabilitation of children at the account of widening the circle of communication, learning new actions and conceptions. It can permit for such children to study at school together with their healthy peers.

Automatic Model Generation Framework for Computational Simulation of Cochlear Implantation.

Science.gov (United States)

Mangado, Nerea; Ceresa, Mario; Duchateau, Nicolas; Kjer, Hans Martin; Vera, Sergio; Dejea Velardo, Hector; Mistrik, Pavel; Paulsen, Rasmus R; Fagertun, Jens; Noailly, Jérôme; Piella, Gemma; González Ballester, Miguel Ángel

2016-08-01

Recent developments in computational modeling of cochlear implantation are promising to study in silico the performance of the implant before surgery. However, creating a complete computational model of the patient's anatomy while including an external device geometry remains challenging. To address such a challenge, we propose an automatic framework for the generation of patient-specific meshes for finite element modeling of the implanted cochlea. First, a statistical shape model is constructed from high-resolution anatomical μCT images. Then, by fitting the statistical model to a patient's CT image, an accurate model of the patient-specific cochlea anatomy is obtained. An algorithm based on the parallel transport frame is employed to perform the virtual insertion of the cochlear implant. Our automatic framework also incorporates the surrounding bone and nerve fibers and assigns constitutive parameters to all components of the finite element model. This model can then be used to study in silico the effects of the electrical stimulation of the cochlear implant. Results are shown on a total of 25 models of patients. In all cases, a final mesh suitable for finite element simulations was obtained, in an average time of 94 s. The framework has proven to be fast and robust, and is promising for a detailed prognosis of the cochlear implantation surgery.

Clinical outcomes of scala vestibuli cochlear implantation in children with partial labyrinthine ossification.

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Lin, Yung-Song

2009-03-01

Cochlear implantation via the scala vestibuli is a viable approach in those with ossification in the scala tympani. With extended cochlear implant experience, there is no significant difference in the mapping parameters and auditory performance between those implanted via scala vestibuli and via scala tympani. To assess the clinical outcomes of cochlear implantation via scala vestibuli. In a cohort follow-up study, 11 prelingually deafened children who received cochlear implantation between age 3 and 10 years through the scala vestibuli served as participants. The mapping parameters (i.e. comfortable level (C), threshold level (T), dynamic range) and auditory performance of each participant were evaluated following initial cochlear implant stimulation, then at 3 month intervals for 2 years, then semi-annually. The follow-up period lasted for 9 years 9 months on average, with a minimum of 8 years 3 months. The clinical results of the mapping parameters and auditory performance of children implanted via the scala vestibuli were comparative to those who were implanted via the scala tympani. No balance problem was reported by any of these patients. One child exhibited residual low frequency hearing after implantation.

Cochlear implants and spoken language processing abilities: review and assessment of the literature.

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Peterson, Nathaniel R; Pisoni, David B; Miyamoto, Richard T

2010-01-01

Cochlear implants (CIs) process sounds electronically and then transmit electric stimulation to the cochlea of individuals with sensorineural deafness, restoring some sensation of auditory perception. Many congenitally deaf CI recipients achieve a high degree of accuracy in speech perception and develop near-normal language skills. Post-lingually deafened implant recipients often regain the ability to understand and use spoken language with or without the aid of visual input (i.e. lip reading). However, there is wide variation in individual outcomes following cochlear implantation, and some CI recipients never develop useable speech and oral language skills. The causes of this enormous variation in outcomes are only partly understood at the present time. The variables most strongly associated with language outcomes are age at implantation and mode of communication in rehabilitation. Thus, some of the more important factors determining success of cochlear implantation are broadly related to neural plasticity that appears to be transiently present in deaf individuals. In this article we review the expected outcomes of cochlear implantation, potential predictors of those outcomes, the basic science regarding critical and sensitive periods, and several new research directions in the field of cochlear implantation.

The contribution of short-term memory capacity to reading ability in adolescents with cochlear implants.

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Edwards, Lindsey; Aitkenhead, Lynne; Langdon, Dawn

2016-11-01

This study aimed to establish the relationship between short-term memory capacity and reading skills in adolescents with cochlear implants. A between-groups design compared a group of young people with cochlear implants with a group of hearing peers on measures of reading, and auditory and visual short-term memory capacity. The groups were matched for non-verbal IQ and age. The adolescents with cochlear implants were recruited from the Cochlear Implant Programme at a specialist children's hospital. The hearing participants were recruited from the same schools as those attended by the implanted adolescents. Participants were 18 cochlear implant users and 14 hearing controls, aged between 12 and 18 years. All used English as their main language and had no significant learning disability or neuro-developmental disorder. Short-term memory capacity was assessed in the auditory modality using Forward and Reverse Digit Span from the WISC IV UK, and visually using Forward and Reverse Memory from the Leiter-R. Individual word reading, reading comprehension and pseudoword decoding were assessed using the WIAT II UK. A series of ANOVAs revealed that the adolescents with cochlear implants had significantly poorer auditory short-term memory capacity and reading skills (on all measures) compared with their hearing peers. However, when Forward Digit Span was entered into the analyses as a covariate, none of the differences remained statistically significant. Deficits in immediate auditory memory persist into adolescence in deaf children with cochlear implants. Short-term auditory memory capacity is an important neurocognitive process in the development of reading skills after cochlear implantation in childhood that remains evident in later adolescence. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Exercise reveals impairments in left ventricular systolic function in patients with metabolic syndrome.

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Fournier, Sara B; Reger, Brian L; Donley, David A; Bonner, Daniel E; Warden, Bradford E; Gharib, Wissam; Failinger, Conard F; Olfert, Melissa D; Frisbee, Jefferson C; Olfert, I Mark; Chantler, Paul D

2014-01-01

Metabolic syndrome (MetS) is the manifestation of a cluster of cardiovascular risk factors and is associated with a threefold increase in the risk of cardiovascular morbidity and mortality, which is suggested to be mediated, in part, by resting left ventricular (LV) systolic dysfunction. However, to what extent resting LV systolic function is impaired in MetS is controversial, and there are no data indicating whether LV systolic function is impaired during exercise. Accordingly, the objective of this study was to examine comprehensively the LV and arterial responses to exercise in individuals with MetS without diabetes and/or overt cardiovascular disease in comparison to a healthy control population. Cardiovascular function was characterized using Doppler echocardiography and gas exchange in individuals with MetS (n = 27) versus healthy control subjects (n = 20) at rest and during peak exercise. At rest, individuals with MetS displayed normal LV systolic function but reduced LV diastolic function compared with healthy control subjects. During peak exercise, individuals with MetS had impaired contractility, pump performance and vasodilator reserve capacity versus control subjects. A blunted contractile reserve response resulted in diminished arterial-ventricular coupling reserve and limited aerobic capacity in individuals with MetS versus control subjects. These findings are of clinical importance, because they provide insight into the pathophysiological changes in MetS that may predispose this population of individuals to an increased risk of cardiovascular morbidity and mortality.

Aspects of Music with Cochlear Implants – Music Listening Habits and Appreciation in Danish Cochlear Implant Users

DEFF Research Database (Denmark)

Petersen, Bjørn; Hansen, Mads; Sørensen, Stine Derdau

Cochlear implant users differ significantly from their normal hearing peers when it comes to perception of music. Several studies have shown that structural features – such as rhythm, timbre, and pitch – are transmitted less accurately through an implant. However, we cannot predict personal...... music less post-implantation than prior to their hearing loss. Nevertheless, a large majority of CI listeners either prefer music over not hearing music at all or find music as pleasant as they recall it before their hearing loss, or more so....... enjoyment of music solely as a function of accuracy of perception. But can music be pleasant with a cochlear implant at all? Our aim here was to gather information of both music enjoyment and listening habits before the onset of hearing loss and post-operation from a large, representative sample of Danish...

Listening Effort With Cochlear Implant Simulations

NARCIS (Netherlands)

Pals, Carina; Sarampalis, Anastasios; Başkent, Deniz

2013-01-01

Purpose: Fitting a cochlear implant (CI) for optimal speech perception does not necessarily optimize listening effort. This study aimed to show that listening effort may change between CI processing conditions for which speech intelligibility remains constant. Method: Nineteen normal-hearing

Identifying cochlear implant channels with poor electrode-neuron interface: electrically-evoked auditory brainstem responses measured with the partial tripolar configuration

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Bierer, Julie Arenberg; Faulkner, Kathleen F.; Tremblay, Kelly L.

2011-01-01

obtained with both the monopolar and partial tripolar configurations. The Wave V amplitude growth functions with increasing stimulus level showed the predicted effect of shallower growth for the partial tripolar than for the monopolar configuration, but this was observed only for the low threshold channel. In contrast, high-threshold channels showed the opposite effect; steeper growth functions were seen for the partial tripolar configuration. Conclusions These results suggest that behavioral thresholds or EABRs measured with a restricted stimulus can be used to identify potentially impaired cochlear implant channels. Channels having high thresholds and steep growth functions would likely not activate the appropriate spatially restricted region of the cochlea, leading to suboptimal perception. As a clinical tool, quick identification of impaired channels could lead to patient-specific mapping strategies and result in improved speech and music perception. PMID:21178633

Advancing Binaural Cochlear Implant Technology

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Mathias Dietz

2015-12-01

Full Text Available This special issue contains a collection of 13 papers highlighting the collaborative research and engineering project entitled Advancing Binaural Cochlear Implant Technology—ABCIT—as well as research spin-offs from the project. In this introductory editorial, a brief history of the project is provided, alongside an overview of the studies.

Meningitis after cochlear implantation in Mondini malformation.

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Page, E L; Eby, T L

1997-01-01

Although the potential for CSF leakage and subsequent meningitis after cochlear implantation in the malformed cochlea has been recognized, this complication has not been previously reported. We report a case of CSF otorhinorrhea and meningitis after minor head trauma developing 2 years after cochlear implantation in a child with Mondini malformation. Leakage of CSF was identified from the cochleostomy around the electrode of the implant, and this leak was sealed with a temporalis fascia and muscle plug. Although this complication appears to be rare, care must be taken to seal the cochleostomy in children with inner ear malformations at the initial surgery, and any episode of meningitis after surgery must be thoroughly investigated to rule out CSF leakage from the labyrinth.

The Role of MRI in Diagnosing Neurovascular Compression of the Cochlear Nerve Resulting in Typewriter Tinnitus.

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Bae, Y J; Jeon, Y J; Choi, B S; Koo, J-W; Song, J-J

2017-06-01

Typewriter tinnitus, a symptom characterized by paroxysmal attacks of staccato sounds, has been thought to be caused by neurovascular compression of the cochlear nerve, but the correlation between radiologic evidence of neurovascular compression of the cochlear nerve and symptom presentation has not been thoroughly investigated. The purpose of this study was to examine whether radiologic evidence of neurovascular compression of the cochlear nerve is pathognomonic in typewriter tinnitus. Fifteen carbamazepine-responding patients with typewriter tinnitus and 8 control subjects were evaluated with a 3D T2-weighted volume isotropic turbo spin-echo acquisition sequence. Groups 1 (16 symptomatic sides), 2 (14 asymptomatic sides), and 3 (16 control sides) were compared with regard to the anatomic relation between the vascular loop and the internal auditory canal and the presence of neurovascular compression of the cochlear nerve with/without angulation/indentation. The anatomic location of the vascular loop was not significantly different among the 3 groups (all, P > .05). Meanwhile, neurovascular compression of the cochlear nerve on MR imaging was significantly higher in group 1 than in group 3 ( P = .032). However, considerable false-positive (no symptoms with neurovascular compression of the cochlear nerve on MR imaging) and false-negative (typewriter tinnitus without demonstrable neurovascular compression of the cochlear nerve) findings were also observed. Neurovascular compression of the cochlear nerve was more frequently detected on the symptomatic side of patients with typewriter tinnitus compared with the asymptomatic side of these patients or on both sides of control subjects on MR imaging. However, considering false-positive and false-negative findings, meticulous history-taking and the response to the initial carbamazepine trial should be regarded as more reliable diagnostic clues than radiologic evidence of neurovascular compression of the cochlear nerve. �

Divergence of fine and gross motor skills in prelingually deaf children: implications for cochlear implantation.

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Horn, David L; Pisoni, David B; Miyamoto, Richard T

2006-08-01

The objective of this study was to assess relations between fine and gross motor development and spoken language processing skills in pediatric cochlear implant users. The authors conducted a retrospective analysis of longitudinal data. Prelingually deaf children who received a cochlear implant before age 5 and had no known developmental delay or cognitive impairment were included in the study. Fine and gross motor development were assessed before implantation using the Vineland Adaptive Behavioral Scales, a standardized parental report of adaptive behavior. Fine and gross motor scores reflected a given child's motor functioning with respect to a normative sample of typically developing, normal-hearing children. Relations between these preimplant scores and postimplant spoken language outcomes were assessed. In general, gross motor scores were found to be positively related to chronologic age, whereas the opposite trend was observed for fine motor scores. Fine motor scores were more strongly correlated with postimplant expressive and receptive language scores than gross motor scores. Our findings suggest a disassociation between fine and gross motor development in prelingually deaf children: fine motor skills, in contrast to gross motor skills, tend to be delayed as the prelingually deaf children get older. These findings provide new knowledge about the links between motor and spoken language development and suggest that auditory deprivation may lead to atypical development of certain motor and language skills that share common cortical processing resources.

A preliminary report of music-based training for adult cochlear implant users: rationales and development

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Gfeller, Kate; Guthe, Emily; Driscoll, Virginia; Brown, Carolyn J.

2015-01-01

Objective This paper provides a preliminary report of a music-based training program for adult cochlear implant (CI) recipients. Included in this report are descriptions of the rationale for music-based training, factors influencing program development, and the resulting program components. Methods Prior studies describing experience-based plasticity in response to music training, auditory training for persons with hearing impairment, and music training for cochlear implant recipients were reviewed. These sources revealed rationales for using music to enhance speech, factors associated with successful auditory training, relevant aspects of electric hearing and music perception, and extant evidence regarding limitations and advantages associated with parameters for music training with CI users. This information formed the development of a computer-based music training program designed specifically for adult CI users. Results Principles and parameters for perceptual training of music, such as stimulus choice, rehabilitation approach, and motivational concerns were developed in relation to the unique auditory characteristics of adults with electric hearing. An outline of the resulting program components and the outcome measures for evaluating program effectiveness are presented. Conclusions Music training can enhance the perceptual accuracy of music, but is also hypothesized to enhance several features of speech with similar processing requirements as music (e.g., pitch and timbre). However, additional evaluation of specific training parameters and the impact of music-based training on speech perception of CI users are required. PMID:26561884

Phonological working memory and its relationship with language abilities in children with cochlear implants

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Fatemeh Haresabadi

2014-12-01

Full Text Available Background and Aim: Many studies have demonstrated a close relationship between phonological working memory and language abilities in normal children and children with language developmental disorders, such as those with cochlear implants. A review of these studies would clarify communication and learning in such children and provide more comprehensive information regarding their education and treatment. In this study, the characteristics of phonological working memory and its relationship with language abilities in children with cochlear implants was examined.Recent Findings: In this study, the authors studied the characteristics of phonological working memory and its relationship with language abilities of children with cochlear implants. These studies showed that in addition to demographic variables, phonological working memory is a factor that affects language development in children with cochlear implants. Children with cochlear implants typically have a shorter memory span.Conclusion: It is thought that the deficiency in primary auditory sensory input and language stimulation caused by difficulties in the processing and rehearsal of auditory information in phonological working memory is the main cause of the short memory span in such children. Conversely, phonological working memory problems may have adverse effects on the language abilities in such children. Therefore, to provide comprehensive and appropriate treatment for children with cochlear implants, the reciprocal relationship between language abilities and phonological working memory should be considered.

Dialogue enabling speech-to-text user assistive agent system for hearing-impaired person.

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Lee, Seongjae; Kang, Sunmee; Han, David K; Ko, Hanseok

2016-06-01

A novel approach for assisting bidirectional communication between people of normal hearing and hearing-impaired is presented. While the existing hearing-impaired assistive devices such as hearing aids and cochlear implants are vulnerable in extreme noise conditions or post-surgery side effects, the proposed concept is an alternative approach wherein spoken dialogue is achieved by means of employing a robust speech recognition technique which takes into consideration of noisy environmental factors without any attachment into human body. The proposed system is a portable device with an acoustic beamformer for directional noise reduction and capable of performing speech-to-text transcription function, which adopts a keyword spotting method. It is also equipped with an optimized user interface for hearing-impaired people, rendering intuitive and natural device usage with diverse domain contexts. The relevant experimental results confirm that the proposed interface design is feasible for realizing an effective and efficient intelligent agent for hearing-impaired.

Spatial channel interactions in cochlear implants

Science.gov (United States)

Tang, Qing; Benítez, Raul; Zeng, Fan-Gang

2011-08-01

The modern multi-channel cochlear implant is widely considered to be the most successful neural prosthesis owing to its ability to restore partial hearing to post-lingually deafened adults and to allow essentially normal language development in pre-lingually deafened children. However, the implant performance varies greatly in individuals and is still limited in background noise, tonal language understanding, and music perception. One main cause for the individual variability and the limited performance in cochlear implants is spatial channel interaction from the stimulating electrodes to the auditory nerve and brain. Here we systematically examined spatial channel interactions at the physical, physiological, and perceptual levels in the same five modern cochlear implant subjects. The physical interaction was examined using an electric field imaging technique, which measured the voltage distribution as a function of the electrode position in the cochlea in response to the stimulation of a single electrode. The physiological interaction was examined by recording electrically evoked compound action potentials as a function of the electrode position in response to the stimulation of the same single electrode position. The perceptual interactions were characterized by changes in detection threshold as well as loudness summation in response to in-phase or out-of-phase dual-electrode stimulation. To minimize potentially confounding effects of temporal factors on spatial channel interactions, stimulus rates were limited to 100 Hz or less in all measurements. Several quantitative channel interaction indexes were developed to define and compare the width, slope and symmetry of the spatial excitation patterns derived from these physical, physiological and perceptual measures. The electric field imaging data revealed a broad but uniformly asymmetrical intracochlear electric field pattern, with the apical side producing a wider half-width and shallower slope than the basal

Cochlear Implant

Directory of Open Access Journals (Sweden)

Mehrnaz Karimi

1992-04-01

Full Text Available People with profound hearing loss are not able to use some kinds of conventional amplifiers due to the nature of their loss . In these people, hearing sense is stimulated only when the auditory nerve is activated via electrical stimulation. This stimulation is possible through cochlear implant. In fact, for the deaf people who have good mental health and can not use surgical and medical treatment and also can not benefit from air and bone conduction hearing aids, this device is used if they have normal central auditory system. The basic parts of the device included: Microphone, speech processor, transmitter, stimulator and receiver, and electrode array.

[Music therapy in adults with cochlear implants : Effects on music perception and subjective sound quality].

Science.gov (United States)

Hutter, E; Grapp, M; Argstatter, H

2016-12-01

People with severe hearing impairments and deafness can achieve good speech comprehension using a cochlear implant (CI), although music perception often remains impaired. A novel concept of music therapy for adults with CI was developed and evaluated in this study. This study included 30 adults with a unilateral CI following postlingual deafness. The subjective sound quality of the CI was rated using the hearing implant sound quality index (HISQUI) and musical tests for pitch discrimination, melody recognition and timbre identification were applied. As a control 55 normally hearing persons also completed the musical tests. In comparison to normally hearing subjects CI users showed deficits in the perception of pitch, melody and timbre. Specific effects of therapy were observed in the subjective sound quality of the CI, in pitch discrimination into a high and low pitch range and in timbre identification, while general learning effects were found in melody recognition. Music perception shows deficits in CI users compared to normally hearing persons. After individual music therapy in the rehabilitation process, improvements in this delicate area could be achieved.

Simultaneous Bilateral Cochlear Implantation for a 6-Month Child with a History of Meningitis

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Kh. M. Diab

2015-01-01

Full Text Available The article presents the first case of simultaneous bilateral cochlear implantation for a 6-month child with IV degree sensorineural hearing loss after meningocephalitis in Russia. Despite partial ossification of the cochlear basal turns, the early timing of implantation allowed to fully implant active electrodes to both ears. The simultaneous bilateral cochlear implantation in young children, who had meningitis, is a minimally invasive and highly efficient procedure with good long-term results of oral-aural after-care.

The intensity-pitch relation revisited: monopolar versus bipolar cochlear stimulation.

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Arnoldner, Christoph; Riss, Dominik; Kaider, Alexandra; Mair, Alois; Wagenblast, Jens; Baumgartner, Wolf-Dieter; Gstöttner, Wolfgang; Hamzavi, Jafar-Sasan

2008-09-01

The very high speech perception scores now being achieved with cochlear implants have led to demands for similar levels of achievement in music perception and perception in noisy environments. One of the crucial factors in these fields is pitch perception. The aim of the present study was to investigate the extent to which pitch perception is influenced by the intensity of the stimulus, through the use of different stimulation modes (monopolar, bipolar) and different electrodes (lateral and perimodiolar). Sixteen postlingually deafened patients with an average implant use of 3.1 years were included in this study. All patients were using a Cochlear (CI24M, CI24R, CI24RE) cochlear implant. Subjects were asked to compare the pitch of an intensity-constant reference tone with the pitch of a test tone of varying intensity. The test was repeated for apical, mediocochlear, and basal channel locations, and also for monopolar and bipolar stimulation. It was found that in monopolar stimulation 87.5% and in bipolar stimulation 85.7% of the patients perceived a clear pitch change with changing intensity of the stimulus (Spearman correlation coefficients r 0.3, respectively). A total of 73.1% of these patients perceived lower pitches with increasing intensity, 26.9% reported the opposite effect. No statistically significant difference in the intensity-pitch correlation could be found between mono- and bipolar stimulation. Neither the mean dynamic range nor the type of electrode used was found to be related to the correlation coefficient. Although the majority of today's cochlear implant recipients perform well and the intensity-pitch relation in cochlear implant recipients is still poorly understood, rising demands on speech-coding strategies may soon make a compensation of the pitch shifts desirable. Although the results of our study tend to argue against a peripheral mechanism, the exact origin of this phenomenon remains unclear.

Factors contributing to communication skills development in cochlear implanted children.

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Ostojić, Sanja; Djoković, Sanja; Radić-šestić, Marina; Nikolić, Mina; Mikić, Branka; Mirić, Danica

2015-08-01

Over the last 10 years more than 300 persons received cochlear implant in Serbia and more than 90% of the recipients were children under 10 years of age. The program of cochlear implantation includes postoperative rehabilitation in which cognitive, integrative and developmental methods are used. The study was conducted to reveal factors affecting communication performance (CP) of cochlear implanted (Cl) children. Special attention was focused on the influence of the duration and intensity of rehabilitation and hearing age on further development of communication skills. A group of 30 CI children (13 boys and 17 girls) aged 2 to 5 years was enrolled in the study. All of the children had average intelligence and no other developmental disorder. They lived in families and attended rehabilitative seances 3 to 5 times a week. Their parents/caregivers answered structured questionnaire about functioning after pediatric cochlear implantation (FAPCI) and the results were the subject of detailed statistical analysis. Analysis of variance did not show any difference between the boys and the girls regarding FAPCI achievements (F(1, 28) = 2.909; p = 0.099) and age aberration in CP score (F(1,28) = 0.114, p = 0.738). Correlation analysis showed a statistically significant difference in FAPCI scores related to hearing age and duration of rehabilitation. Regression analysis (enter method) showed that model consisting of indipendent variables significantly contributed to prediction of overall FAPCI scores and Adjusted R2 value could explain 32% difference in communication skills of participants in this study. Communication skills of CI children evaluated by FAPCI are falling behind normatives for normal hearing children 18.6 months on the average. Hearing age, duration and intensity of rehabilitation have positive predictive value for communication skills development. Later identification of hearing loss and later cochlear implantation lead to delayed development of communication

Impairment of different protein domains causes variable clinical presentation within Pitt-Hopkins syndrome and suggests intragenic molecular syndromology of TCF4.

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Bedeschi, Maria Francesca; Marangi, Giuseppe; Calvello, Maria Rosaria; Ricciardi, Stefania; Leone, Francesca Pia Chiara; Baccarin, Marco; Guerneri, Silvana; Orteschi, Daniela; Murdolo, Marina; Lattante, Serena; Frangella, Silvia; Keena, Beth; Harr, Margaret H; Zackai, Elaine; Zollino, Marcella

2017-11-01

Pitt-Hopkins syndrome is a neurodevelopmental disorder characterized by severe intellectual disability and a distinctive facial gestalt. It is caused by haploinsufficiency of the TCF4 gene. The TCF4 protein has different functional domains, with the NLS (nuclear localization signal) domain coded by exons 7-8 and the bHLH (basic Helix-Loop-Helix) domain coded by exon 18. Several alternatively spliced TCF4 variants have been described, allowing for translation of variable protein isoforms. Typical PTHS patients have impairment of at least the bHLH domain. To which extent impairment of the remaining domains contributes to the final phenotype is not clear. There is recent evidence that certain loss-of-function variants disrupting TCF4 are associated with mild ID, but not with typical PTHS. We describe a frameshift-causing partial gene deletion encompassing exons 4-6 of TCF4 in an adult patient with mild ID and nonspecific facial dysmorphisms but without the typical features of PTHS, and a c.520C > T nonsense variant within exon 8 in a child presenting with a severe phenotype largely mimicking PTHS, but lacking the typical facial dysmorphism. Investigation on mRNA, along with literature review, led us to suggest a preliminary phenotypic map of loss-of-function variants affecting TCF4. An intragenic phenotypic map of loss-of-function variants in TCF4 is suggested here for the first time: variants within exons 1-4 and exons 4-6 give rise to a recurrent phenotype with mild ID not in the spectrum of Pitt-Hopkins syndrome (biallelic preservation of both the NLS and bHLH domains); variants within exons 7-8 cause a severe phenotype resembling PTHS but in absence of the typical facial dysmorphism (impairment limited to the NLS domain); variants within exons 9-19 cause typical Pitt-Hopkins syndrome (impairment of at least the bHLH domain). Understanding the TCF4 molecular syndromology can allow for proper nosology in the current era of whole genomic investigations. Copyright �

Cochlear implant with a non-removable magnet: preliminary research at 3-T MRI.

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Dubrulle, F; Sufana Iancu, A; Vincent, C; Tourrel, G; Ernst, O

2013-06-01

To perform preliminary tests in vitro and with healthy volunteers to determine the 3-T MRI compatibility of a cochlear implant with a non-removable magnet. In the in vitro phase, we tested six implants for temperature changes and internal malfunctioning. We measured the demagnetisation of 65 internal magnets with different tilt angles between the implant's magnetic field (bi) and the main magnetic field (b0). In the in vivo phase, we tested 28 operational implants attached to the scalps of volunteers with the head in three different positions. The study did not find significant temperature changes or electronic malfunction in the implants tested in vitro. We found considerable demagnetisation of the cochlear implant magnets in the in vitro and in vivo testing influenced by the position of the magnet in the main magnetic field. We found that if the bi/b0 angle is 90°, there is demagnetisation in almost 60 % of the cases. When the angle is around 90°, the risk of demagnetisation is low (6.6 %). The preliminary results on cochlear implants with non-removable magnets indicate the need to maintain the contraindication of passage through 3-T MRI. • Magnetic resonance imaging can affect cochlear implants and vice versa. • Demagnetisation of cochlear implant correlates with the angle between bi and b0. • The position of the head in the MRI influences the demagnetisation. • Three-Tesla MRI for cochlear implants is still contraindicated. • However some future solutions are discussed.

Prevention and management of vascular complications in middle ear and cochlear implant surgery.

Science.gov (United States)

Di Lella, Filippo; Falcioni, Maurizio; Piccinini, Silvia; Iaccarino, Ilaria; Bacciu, Andrea; Pasanisi, Enrico; Cerasti, Davide; Vincenti, Vincenzo

2017-11-01

The objective of this study is to illustrate prevention strategies and management of vascular complications from the jugular bulb (JB) and internal carotid artery (ICA) during middle ear surgery or cochlear implantation. The study design is retrospective case series. The setting is tertiary referral university hospital. Patients were included if presented pre- or intraoperative evidence of high-risk anatomical anomalies of ICA or JB during middle ear or cochlear implant surgery, intraoperative vascular injury, or revision surgery after the previous iatrogenic vascular lesions. The main outcome measures are surgical outcomes and complications rate. Ten subjects were identified: three underwent cochlear implant surgery and seven underwent middle ear surgery. Among the cochlear implant patients, two presented with anomalies of the JB impeding access to the cochlear lumen and one underwent revision surgery for incorrect positioning of the array in the carotid canal. Subtotal petrosectomy was performed in all cases. Anomalies of the JB were preoperatively identified in two patients with attic and external auditory canal cholesteatoma, respectively. In a patient, a high and dehiscent JB was found during myringoplasty, while another underwent revision surgery after iatrogenic injury of the JB. A dehiscent ICA complicated middle ear effusion in one case, while in another case, a carotid aneurysm determined a cholesterol granuloma. Rupture of a pseudoaneurysm of the ICA occurred in a child during second-stage surgery and required permanent balloon occlusion without neurological complications. Knowledge of normal anatomy and its variants and preoperative imaging are the basis for prevention of vascular complications during middle ear or cochlear implant surgery.

Association of Branched and Aromatic Amino Acids Levels with Metabolic Syndrome and Impaired Fasting Glucose in Hypertensive Patients

OpenAIRE

Weng, Liming; Quinlivan, Eoin; Gong, Yan; Beitelshees, Amber L.; Shahin, Mohamed H.; Turner, Stephen T.; Chapman, Arlene B.; Gums, John G.; Johnson, Julie A.; Frye, Reginald F.; Garrett, Timothy J.; Cooper-DeHoff, Rhonda M.

2015-01-01

Background: The three branched amino acids (valine, leucine, and isoleucine) and two aromatic amino acids (tyrosine and phenylalanine) have been associated with many adverse metabolic pathways, including diabetes. However, these associations have been identified primarily in otherwise healthy Caucasian populations. We aimed to investigate the association of this five-amino-acid signature with metabolic syndrome and impaired fasting glucose (IFG) in a hypertensive cohort of Caucasian and Afric...

American parent perspectives on quality of life in pediatric cochlear implant recipients.

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Kumar, Roshini; Warner-Czyz, Andrea; Silver, Cheryl H; Loy, Betty; Tobey, Emily

2015-01-01

Cochlear implantation influences not only communication but also psychosocial outcomes in children with severe to profound hearing loss. Focusing on issues specific to cochlear implantation (e.g., self-reliance, social relations, education, effects of implantation, and supporting the child) may provide a more accurate and relative view of functional status of pediatric cochlear implant (CI) recipients. The present study analyzes parental perspectives of CI-specific health-related quality of life (HRQoL) in children with CIs to determine (a) if parents differentially rate their child's quality of life according to psychosocial domain (e.g., communication, self-reliance, education); (b) if associations exist between quality of life domains specific to cochlear implantation in pediatric implant recipients; and (c) if demographic variables (i.e., chronologic age, age at cochlear implantation, duration of device experience) mediate parent ratings of quality of life in pediatric CI recipients. Parents of 33 children with CIs (mean age, 9.85 years; mean age of CI activation, 2.47 years; mean device experience, 7.47 years) completed a validated condition-specific questionnaire, Children With Cochlear Implants: Parental Perspectives. Parents positively rated most HRQoL domains, although education and effects of implantation received significantly less positive ratings (p self-reliance, and well-being) significantly correlated with at least 5 other domains, suggesting that positivity in one domain co-occurs with positivity in other domains. Demographic variables (chronologic age, CI activation age, and duration of CI use) did not correlate significantly with psychosocial outcomes; rather, parents reported positive HRQoL and successful functional use of CI across demographic variables. Parents of children and adolescents with CIs rate overall HRQoL positively across psychosocial domains. Significantly less positive ratings of education and effects of implantation may result

Cognitive Impairment and Brain Imaging Characteristics of Patients with Congenital Cataracts, Facial Dysmorphism, Neuropathy Syndrome

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Teodora Chamova

2015-01-01

Full Text Available Congenital cataracts, facial dysmorphism, neuropathy (CCFDN syndrome is a complex autosomal recessive multisystem disorder. The aim of the current study is to evaluate the degree of cognitive impairment in a cohort of 22 CCFDN patients and its correlation with patients’ age, motor disability, ataxia, and neuroimaging changes. Twenty-two patients with genetically confirmed diagnosis of CCFDN underwent a detailed neurological examination. Verbal and nonverbal intelligence, memory, executive functions, and verbal fluency wеre assessed in all the patients aged 4 to 47 years. Brain magnetic resonance imaging was performed in 20 affected patients. Eighteen affected were classified as having mild intellectual deficit, whereas 4 had borderline intelligence. In all psychometric tests, evaluating different cognitive domains, CCFDN patients had statistically significant lower scores when compared to the healthy control group. All cognitive domains seemed equally affected. The main abnormalities on brain MRI found in 19/20 patients included diffuse cerebral atrophy, enlargement of the lateral ventricles, and focal lesions in the subcortical white matter, different in number and size, consistent with demyelination more pronounced in the older CCFDN patients. The correlation analysis of the structural brain changes and the cognitive impairment found a statistically significant correlation only between the impairment of short-term verbal memory and the MRI changes.

Assessment of auditory cortical function in cochlear implant patients using 15O PET

International Nuclear Information System (INIS)

Young, J.P.; O'Sullivan, B.T.; Gibson, W.P.; Sefton, A.E.; Mitchell, T.E.; Sanli, H.; Cervantes, R.; Withall, A.; Royal Prince Alfred Hospital, Sydney,

1998-01-01

Full text: Cochlear implantation has been an extraordinarily successful method of restoring hearing and the potential for full language development in pre-lingually and post-lingually deaf individuals (Gibson 1996). Post-lingually deaf patients, who develop their hearing loss later in life, respond best to cochlear implantation within the first few years of their deafness, but are less responsive to implantation after several years of deafness (Gibson 1996). In pre-lingually deaf children, cochlear implantation is most effect in allowing the full development language skills when performed within a critical period, in the first 8 years of life. These clinical observations suggest considerable neural plasticity of the human auditory cortex in acquiring and retaining language skills (Gibson 1996, Buchwald 1990). Currently, electrocochleography is used to determine the integrity of the auditory pathways to the auditory cortex. However, the functional integrity of the auditory cortex cannot be determined by this method. We have defined the extent of activation of the auditory cortex and auditory association cortex in 6 normal controls and 6 cochlear implant patients using 15 O PET functional brain imaging methods. Preliminary results have indicated the potential clinical utility of 15 O PET cortical mapping in the pre-surgical assessment and post-surgical follow up of cochlear implant patients. Copyright (1998) Australian Neuroscience Society

A longitudinal study of the bilateral benefit in children with bilateral cochlear implants.

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Asp, Filip; Mäki-Torkko, Elina; Karltorp, Eva; Harder, Henrik; Hergils, Leif; Eskilsson, Gunnar; Stenfelt, Stefan

2015-02-01

To study the development of the bilateral benefit in children using bilateral cochlear implants by measurements of speech recognition and sound localization. Bilateral and unilateral speech recognition in quiet, in multi-source noise, and horizontal sound localization was measured at three occasions during a two-year period, without controlling for age or implant experience. Longitudinal and cross-sectional analyses were performed. Results were compared to cross-sectional data from children with normal hearing. Seventy-eight children aged 5.1-11.9 years, with a mean bilateral cochlear implant experience of 3.3 years and a mean age of 7.8 years, at inclusion in the study. Thirty children with normal hearing aged 4.8-9.0 years provided normative data. For children with cochlear implants, bilateral and unilateral speech recognition in quiet was comparable whereas a bilateral benefit for speech recognition in noise and sound localization was found at all three test occasions. Absolute performance was lower than in children with normal hearing. Early bilateral implantation facilitated sound localization. A bilateral benefit for speech recognition in noise and sound localization continues to exist over time for children with bilateral cochlear implants, but no relative improvement is found after three years of bilateral cochlear implant experience.

Cochlear implantation in the world's largest medical device market: utilization and awareness of cochlear implants in the United States.

Science.gov (United States)

Sorkin, Donna L

2013-03-01

Provision of cochlear implants (CIs) for those within the criteria for implantation remains lower in the United States than in some other developed nations. When adults and children are grouped together, the rate of utilization/provision remains low at around 6%. For children, the provision rate is about 50% of those who could benefit from an implant, compared with figures of about 90% for the Flanders part of Belgium, the United Kingdom and other European countries. The probable reasons for this underprovision include: low awareness of the benefits of CIs among the population; low awareness among health-care professionals; the lack of specific referral pathways; some political issues relating to the Deaf Community; and financial issues related to health provision. Such financial issues result in situations which either fail to provide for access to implants or provide too low a level of the necessary funding, especially for low-income individuals covered by public health-care programs such as Medicaid. These issues might be mitigated by adoption and publication of standards for best clinical practices for CI provision, availability of current cost-effectiveness data, and the existence of an organization dedicated to cochlear implantation. Such an organization, the American Cochlear Implant Alliance (ACI Alliance), was recently organized and is described in the paper by Niparko et al. in this Supplement.

CT study of 17 cases of congenital cochlear abnormality. Embryologic and anatomo-functional relationship

OpenAIRE

Maurício, JC; Biscoito, L; Branco, G

1993-01-01

In this review of cochlear malformations, 17 cases were studied by CT scan (high resolution-target program) including 10 males and 7 females. The anomaly was bilateral in 12 cases and unilateral in 7 (the latter included 2 Mondini type aplasias and one Michel). The cochlear malformation (whose most frequent expression was the cochlear hypoplasia with 1 or 1 1/2 spires-65% of the cases) was associated to semicircular canals and/or vestibular aqueduct anomaly in 82% of the patients; it was excl...

Language development in Japanese children who receive cochlear implant and/or hearing aid.

Science.gov (United States)

Iwasaki, Satoshi; Nishio, Shinya; Moteki, Hideaki; Takumi, Yutaka; Fukushima, Kunihiro; Kasai, Norio; Usami, Shin-Ichi

2012-03-01

This study aimed to investigate a wide variety of factors that influence auditory, speech, and language development following pediatric cochlear implantation (CI). Prospective collection of language tested data in profound hearing-impaired children. Pediatric CI can potentially be effective to development of practical communication skills and early implantation is more effective. We proposed a set of language tests (assessment package of the language development for Japanese hearing-impaired children; ALADJIN) consisting of communication skills testing (test for question-answer interaction development; TQAID), comprehensive (Peabody Picture Vocabulary Test-Revised; PVT-R and Standardized Comprehension Test for Abstract Words; SCTAW) and productive vocabulary (Word Fluency Test; WFT), and comprehensive and productive syntax (Syntactic processing Test for Aphasia; STA). Of 638 hearing-impaired children recruited for this study, 282 (44.2%) with >70 dB hearing impairment had undergone CI. After excluding children with low birth weight (11 points on the Pervasive Developmental Disorder ASJ Rating Scale for the test of autistic tendency, and those better than those in HA-only users. The scores for PVT-R (pbetter than those in HA-only users. STA and TQAID scores in CI-HA users were significantly (pbetter than those in unilateral CI-only users. The high correlation (r=0.52) has been found between the age of CI and maximum speech discrimination score. The scores of speech and language tests in the implanted children before 24 months of age have been better than those in the implanted children after 24 months of age. We could indicate that CI was effective for language development in Japanese hearing-impaired children and early CI was more effective for productive vocabulary and syntax. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Identifying cochlear implant channels with poor electrode-neuron interfaces: electrically evoked auditory brain stem responses measured with the partial tripolar configuration.

Science.gov (United States)

Bierer, Julie Arenberg; Faulkner, Kathleen F; Tremblay, Kelly L

2011-01-01

tripolar configurations. The Wave V amplitude growth functions with increasing stimulus level showed the predicted effect of shallower growth for the partial tripolar than for the monopolar configuration, but this was observed only for the low-threshold channels. In contrast, high-threshold channels showed the opposite effect; steeper growth functions were seen for the partial tripolar configuration. These results suggest that behavioral thresholds or EABRs measured with a restricted stimulus can be used to identify potentially impaired cochlear implant channels. Channels having high thresholds and steep growth functions would likely not activate the appropriate spatially restricted region of the cochlea, leading to suboptimal perception. As a clinical tool, quick identification of impaired channels could lead to patient-specific mapping strategies and result in improved speech and music perception.

Acoustic properties of vocal singing in prelingually-deafened children with cochlear implants or hearing aids.

Science.gov (United States)

Mao, Yitao; Zhang, Mengchao; Nutter, Heather; Zhang, Yijing; Zhou, Qixin; Liu, Qiaoyun; Wu, Weijing; Xie, Dinghua; Xu, Li

2013-11-01

The purpose of the present study was to investigate vocal singing performance of hearing-impaired children with cochlear implants (CI) and hearing aids (HA) as well as to evaluate the relationship between demographic factors of those hearing-impaired children and their singing ability. Thirty-seven prelingually-deafened children with CIs and 31 prelingually-deafened children with HAs, and 37 normal-hearing (NH) children participated in the study. The fundamental frequencies (F0) of each note in the recorded songs were extracted and the duration of each sung note was measured. Five metrics were used to evaluate the pitch-related and rhythm-based aspects of singing accuracy. Children with CIs and HAs showed significantly poorer performance in either the pitch-based assessments or the rhythm-based measure than the NH children. No significant differences were seen between the CI and HA groups in all of these measures except for the mean deviation of the pitch intervals. For both hearing-impaired groups, length of device use was significantly correlated with singing accuracy. There is a marked deficit in vocal singing ability either in pitch or rhythm accuracy in a majority of prelingually-deafened children who have received CIs or fitted with HAs. Although an increased length of device use might facilitate singing performance to some extent, the chance for the hearing-impaired children fitted with either HAs or CIs to reach high proficiency in singing is quite slim. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

A combination of two truncating mutations in USH2A causes more severe and progressive hearing impairment in Usher syndrome type IIa

DEFF Research Database (Denmark)

Hartel, Bas P.; Lofgren, Maria; Huygen, Patrick L. M.

2016-01-01

Objectives Usher syndrome is an inherited disorder that is characterized by hearing impairment (HI), retinitis pigmentosa, and in some cases vestibular dysfunction. Usher syndrome type IIa is caused by mutations in USH2A. HI in these patients is highly heterogeneous and the present study evaluates...... the effects of different types of USH2A mutations on the audiometric phenotype. Data from two large centres of expertise on Usher Syndrome in the Netherlands and Sweden were combined in order to create a large combined sample of patients to identify possible genotype-phenotype correlations. Design...... A retrospective study on HI in 110 patients (65 Dutch and 45 Swedish) genetically diagnosed with Usher syndrome type IIa. We used methods especially designed for characterizing and testing differences in audiological phenotype between patient subgroups. These methods included Age Related Typical Audiograms (ARTA...

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....

Motor dysfunction of complex regional pain syndrome is related to impaired central processing of proprioceptive information.

Science.gov (United States)

Bank, Paulina J M; Peper, C Lieke E; Marinus, Johan; Beek, Peter J; van Hilten, Jacobus J

2013-11-01

Our understanding of proprioceptive deficits in complex regional pain syndrome (CRPS) and its potential contribution to impaired motor function is still limited. To gain more insight into these issues, we evaluated accuracy and precision of joint position sense over a range of flexion-extension angles of the wrist of the affected and unaffected sides in 25 chronic CRPS patients and in 50 healthy controls. The results revealed proprioceptive impairment at both the patients' affected and unaffected sides, characterized predominantly by overestimation of wrist extension angles. Precision of the position estimates was more prominently reduced at the affected side. Importantly, group differences in proprioceptive performance were observed not only for tests at identical percentages of each individual's range of wrist motion but also when controls were tested at wrist angles that corresponded to those of the patient's affected side. More severe motor impairment of the affected side was associated with poorer proprioceptive performance. Based on additional sensory tests, variations in proprioceptive performance over the range of wrist angles, and comparisons between active and passive displacements, the disturbances of proprioceptive performance most likely resulted from altered processing of afferent (and not efferent) information and its subsequent interpretation in the context of a distorted "body schema." The present results point at a significant role for impaired central processing of proprioceptive information in the motor dysfunction of CRPS and suggest that therapeutic strategies aimed at identification of proprioceptive impairments and their restoration may promote the recovery of motor function in CRPS patients. Copyright © 2013 American Pain Society. Published by Elsevier Inc. All rights reserved.

Hallucinations Experienced by Visually Impaired: Charles Bonnet Syndrome

OpenAIRE

Pang, Linda

2016-01-01

ABSTRACT Charles Bonnet Syndrome is a condition where visual hallucinations occur as a result of damage along the visual pathway. Patients with Charles Bonnet Syndrome maintain partial or full insight that the hallucinations are not real, absence of psychological conditions, and absence of hallucinations affecting other sensory modalities, while maintaining intact intellectual functioning. Charles Bonnet Syndrome has been well documented in neurologic, geriatric medicine, and psychiatric lite...

Comparison of self-esteem level of adolescents with cochlear implant and normal hearing.

Science.gov (United States)

Sahli, Sanem; Belgin, Erol

2006-09-01

The purpose of this study is to compare the levels of self-esteem of adolescents with cochlear implants (before and after cochlear implantation) and the ones who have normal hearing. For this purpose, Rosenberg self-esteem scale is applied upon the study group which consists of 30 adolescents with cochlear implant between the ages of 12-19 and upon the control group which consists of 60 adolescents having the similar characteristics. The scale is used to evaluate the level of self-esteem of adolescents with cochlear implant and with normal hearing. At the end of the application, the scores of these two groups which they got according to their answers were compared statistically. When the results were examined, there seemed to be no significant difference statistically between the self-esteem values of the cochlear implant group and the control group. Apart from this, there seemed to be significant difference statistically between the self-esteem values of the before cochlear implantation and control group. In this study, we examined changes in the level of self-esteem according to different variables. As a result, it was found out that in both groups levels of self-esteem was higher for adolescents who had had preschool education, had brothers/sisters, high level of income, whose mother was working and whose father and mother had higher levels of education. On the other hand, the birth sequence and the child's father's profession did not seem to have any effect on the child's level of self-esteem. As a result of these findings, it was thought that cochlear implantation had a positive effect on life quality and it was suggested that the adolescents and their families should get assistance from experts about the characteristics and principles of approaching the child in this period. The adolescent should be directed towards social activities and courses, their positive sides should be supported and further studies should be carried out with different case groups on

Technological, biological, and acoustical constraints to music perception in cochlear implant users.

Science.gov (United States)

Limb, Charles J; Roy, Alexis T

2014-02-01

Despite advances in technology, the ability to perceive music remains limited for many cochlear implant users. This paper reviews the technological, biological, and acoustical constraints that make music an especially challenging stimulus for cochlear implant users, while highlighting recent research efforts to overcome these shortcomings. The limitations of cochlear implant devices, which have been optimized for speech comprehension, become evident when applied to music, particularly with regards to inadequate spectral, fine-temporal, and dynamic range representation. Beyond the impoverished information transmitted by the device itself, both peripheral and central auditory nervous system deficits are seen in the presence of sensorineural hearing loss, such as auditory nerve degeneration and abnormal auditory cortex activation. These technological and biological constraints to effective music perception are further compounded by the complexity of the acoustical features of music itself that require the perceptual integration of varying rhythmic, melodic, harmonic, and timbral elements of sound. Cochlear implant users not only have difficulty perceiving spectral components individually (leading to fundamental disruptions in perception of pitch, melody, and harmony) but also display deficits with higher perceptual integration tasks required for music perception, such as auditory stream segregation. Despite these current limitations, focused musical training programs, new assessment methods, and improvements in the representation and transmission of the complex acoustical features of music through technological innovation offer the potential for significant advancements in cochlear implant-mediated music perception. Copyright © 2013 Elsevier B.V. All rights reserved.

Simplifying cochlear implant speech processor fitting

NARCIS (Netherlands)

Willeboer, C.

2008-01-01

Conventional fittings of the speech processor of a cochlear implant (CI) rely to a large extent on the implant recipient's subjective responses. For each of the 22 intracochlear electrodes the recipient has to indicate the threshold level (T-level) and comfortable loudness level (C-level) while

Static and dynamic posture control in postlingual cochlear implanted patients: effects of dual-tasking, visual and auditory inputs suppression.

Science.gov (United States)

Bernard-Demanze, Laurence; Léonard, Jacques; Dumitrescu, Michel; Meller, Renaud; Magnan, Jacques; Lacour, Michel

2013-01-01

Posture control is based on central integration of multisensory inputs, and on internal representation of body orientation in space. This multisensory feedback regulates posture control and continuously updates the internal model of body's position which in turn forwards motor commands adapted to the environmental context and constraints. The peripheral localization of the vestibular system, close to the cochlea, makes vestibular damage possible following cochlear implant (CI) surgery. Impaired vestibular function in CI patients, if any, may have a strong impact on posture stability. The simple postural task of quiet standing is generally paired with cognitive activity in most day life conditions, leading therefore to competition for attentional resources in dual-tasking, and increased risk of fall particularly in patients with impaired vestibular function. This study was aimed at evaluating the effects of postlingual cochlear implantation on posture control in adult deaf patients. Possible impairment of vestibular function was assessed by comparing the postural performance of patients to that of age-matched healthy subjects during a simple postural task performed in static (stable platform) and dynamic (platform in translation) conditions, and during dual-tasking with a visual or auditory memory task. Postural tests were done in eyes open (EO) and eyes closed (EC) conditions, with the CI activated (ON) or not (OFF). Results showed that the postural performance of the CI patients strongly differed from the controls, mainly in the EC condition. The CI patients showed significantly reduced limits of stability and increased postural instability in static conditions. In dynamic conditions, they spent considerably more energy to maintain equilibrium, and their head was stabilized neither in space nor on trunk: they behaved dynamically without vision like an inverted pendulum while the controls showed a whole body rigidification strategy. Hearing (prosthesis on) as well

Vestibular dysfunction in Turner syndrome: a case report.

Science.gov (United States)

Baxter, Michael; Agrawal, Yuri

2014-02-01

Turner syndrome is a well-known cause of sensorineural hearing loss, and the lack of estrogen has been implicated in cochlear dysfunction. It has never been associated with vestibular dysfunction. We report a case of a patient with Turner syndrome who was found to have bilateral vestibular dysfunction based on video-oculography (VOG) testing. A single patient with a history of Turner syndrome who was found to have significant bilateral vestibular dysfunction. After noticing a deficit in the vestibulo-ocular reflexes on qualitative horizontal head impulse examination, the patient underwent VOG testing. VOG testing quantatively measures angular vestibulo-ocular reflex (AVOR) gain in the horizontal semicircular canal plane. AVOR gain represents the eye movement response to a head movement; in normal individuals the eye movement is fully compensatory and gain values are close to unity. VOG results showed AVOR gains of 0.29 and 0.36 on the right and left sides, respectively. We have presented a case of a woman with Turner syndrome with asymptomatic vestibular dysfunction demonstrated with VOG testing. Although there is a documented relationship between Turner syndrome and sensorineural hearing loss, there are no previous studies or case reports linking Turner syndrome and vestibular dysfunction. Additional research and added vigilance in monitoring Turner syndrome patients may be warranted.

Comparison between bilateral cochlear implants and Neurelec Digisonic(®) SP Binaural cochlear implant: speech perception, sound localization and patient self-assessment.

Science.gov (United States)

Bonnard, Damien; Lautissier, Sylvie; Bosset-Audoit, Amélie; Coriat, Géraldine; Beraha, Max; Maunoury, Antoine; Martel, Jacques; Darrouzet, Vincent; Bébéar, Jean-Pierre; Dauman, René

2013-01-01

An alternative to bilateral cochlear implantation is offered by the Neurelec Digisonic(®) SP Binaural cochlear implant, which allows stimulation of both cochleae within a single device. The purpose of this prospective study was to compare a group of Neurelec Digisonic(®) SP Binaural implant users (denoted BINAURAL group, n = 7) with a group of bilateral adult cochlear implant users (denoted BILATERAL group, n = 6) in terms of speech perception, sound localization, and self-assessment of health status and hearing disability. Speech perception was assessed using word recognition at 60 dB SPL in quiet and in a 'cocktail party' noise delivered through five loudspeakers in the hemi-sound field facing the patient (signal-to-noise ratio = +10 dB). The sound localization task was to determine the source of a sound stimulus among five speakers positioned between -90° and +90° from midline. Change in health status was assessed using the Glasgow Benefit Inventory and hearing disability was evaluated with the Abbreviated Profile of Hearing Aid Benefit. Speech perception was not statistically different between the two groups, even though there was a trend in favor of the BINAURAL group (mean percent word recognition in the BINAURAL and BILATERAL groups: 70 vs. 56.7% in quiet, 55.7 vs. 43.3% in noise). There was also no significant difference with regard to performance in sound localization and self-assessment of health status and hearing disability. On the basis of the BINAURAL group's performance in hearing tasks involving the detection of interaural differences, implantation with the Neurelec Digisonic(®) SP Binaural implant may be considered to restore effective binaural hearing. Based on these first comparative results, this device seems to provide benefits similar to those of traditional bilateral cochlear implantation, with a new approach to stimulate both auditory nerves. Copyright © 2013 S. Karger AG, Basel.

Projections from the cochlear nuclei in the mustache bat, Pteronotus parnellii

International Nuclear Information System (INIS)

Zook, J.M.; Casseday, J.H.

1985-01-01

Ascending projections of the cochlear nuclei in the mustache bat were analyzed by anterograde transport of [ 3 H]-leucine and by retrograde transport of HRP. The authors were particularly interested in pathways to two parts of the system: (1) to the medial superior olive, because this nucleus is missing in most echolocating bats, but appears to be present in the mustache bat, and (2) to the intermediate and ventral nuclei of the lateral lemniscus, because these nuclei are hypertrophied and highly differentiated in all echolocating bats that we have examined. The results show a highly systematic projection from the anteroventral cochlear nucleus to all of the auditory nuclei in the brain stem. After an injection of [ 3 H]-leucine in the anterior and dorsal part of the anteroventral cochlear nucleus, presumably in a region sensitive to low frequencies, label is seen in the following locations: ipsilateral to the injection in the lateral part of the lateral superior olive; bilaterally in the dorsal part of the medial superior olive; contralateral to the injection in the dorsal parts of the intermediate and ventral nuclei of the lateral lemniscus; and in the anterolateral part of the central nucleus of the inferior colliculus. After an injection of [ 3 H]-leucine in a posterior part of the anteroventral cochlear nucleus, presumably in a region sensitive to high frequencies, labeling is in the same set of nuclei, but within each nucleus the label is now in a different location. Projections from the entire anteroventral cochlear nucleus to the inferior colliculus are confined to the ventral two-thirds of the central nucleus

Hearing Preservation Outcomes With a Mid-Scala Electrode in Cochlear Implantation.

Science.gov (United States)

Hunter, Jacob B; Gifford, René H; Wanna, George B; Labadie, Robert F; Bennett, Marc L; Haynes, David S; Rivas, Alejandro

2016-03-01

To evaluate hearing preservation (HP) outcomes in adult cochlear implant recipients with a mid-scala electrode. Tertiary academic center. Adult patients implanted with a mid-scala electrode between May 2013 and July 2015. Cochlear implantation. Age, sex, surgical approach, residual hearing changes post cochlear implantation, HP rates using different published classifications, and speech perception scores. Fifty ears for 47 patients (mean age, 58.2 yr; range, 23-86) were implanted with the electrode. Recognizing that not all patients were true HP candidates and/or underwent generally accepted HP surgical techniques, 39 ears had preoperative low-frequency hearing (audiometric threshold ≤ 85dB HL at 250Hz), 24 preserved acoustic hearing postoperatively (75.0%). Patients who had preserved acoustic hearing were implanted via round window (N = 18), extended round window (N = 4), or via cochleostomy (N = 2) approaches. Mean threshold elevation for low-frequency pure-tone average (125, 250, and 500  Hz) was 20.2  dB after surgery. 43.8% of patients had aidable low-frequency hearing at activation, 30.0% at 6-months postoperatively, and 30.8% 1-year postopera tively. Using a formula outlined by Skarzynski and colleagues, at 6-months postoperatively, 15.0% of patients had complete HP, whereas 40.0% had partial HP. At 1-year, these percentages decreased to 0% and 38.5%, respectively. Age, type of approach, and perioperative steroid use were not correlated with HP outcomes at activation and 6-months postoperatively (p > 0.05). The mid-scala electrode evaluated allows preservation of low-frequency hearing in patients undergoing cochlear implantation at rates and degrees of preservation close to other reports in the cochlear implant literature.

Morphological correlates of hearing loss after cochlear implantation and electro-acoustic stimulation in a hearing-impaired Guinea pig model.

Science.gov (United States)

Reiss, Lina A J; Stark, Gemaine; Nguyen-Huynh, Anh T; Spear, Kayce A; Zhang, Hongzheng; Tanaka, Chiemi; Li, Hongzhe

2015-09-01

Hybrid or electro-acoustic stimulation (EAS) cochlear implants (CIs) are designed to provide high-frequency electric hearing together with residual low-frequency acoustic hearing. However, 30-50% of EAS CI recipients lose residual hearing after implantation. The objective of this study was to determine the mechanisms of EAS-induced hearing loss in an animal model with high-frequency hearing loss. Guinea pigs were exposed to 24 h of noise (12-24 kHz at 116 dB) to induce a high-frequency hearing loss. After recovery, two groups of animals were implanted (n = 6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no stimulation during this time frame. A third group (n = 6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem responses were recorded biweekly to monitor changes in hearing. The organ of Corti was immunolabeled with phalloidin, anti-CtBP2, and anti-GluR2 to quantify hair cells, ribbons and post-synaptic receptors. The lateral wall was immunolabeled with phalloidin and lectin to quantify stria vascularis capillary diameters. Bimodal or trimodal diameter distributions were observed; the number and location of peaks were objectively determined using the Aikake Information Criterion and Expectation Maximization algorithm. Noise exposure led to immediate hearing loss at 16-32 kHz for all groups. Cochlear implantation led to additional hearing loss at 4-8 kHz; this hearing loss was negatively and positively correlated with minimum and maximum peaks of the bimodal or trimodal distributions of stria vascularis capillary diameters, respectively. After chronic stimulation, no significant group changes in thresholds were seen; however, elevated thresholds at 1 kHz in implanted, stimulated animals were significantly correlated with decreased presynaptic ribbon and postsynaptic receptor counts. Inner and outer hair cell counts did not differ between groups and

Audiological findings after multichannel cochlear implantation in patients with Mondini dysplasia.

Science.gov (United States)

Munro, K J; George, C R; Haacke, N P

1996-12-01

Mondini dysplasia is a congenital malformation of the inner ear. To date, five individuals with this malformation have received cochlear implants at the South of England Cochlear Implant Centre. The aim of this study was to review the audiological findings of these individuals after implantation. The soundfield thresholds after implantation are in the region of 30-40 dB (A). The results of suprathreshold speech recognition tasks show substantial variability in performance but this is no greater than that obtained from implant users with no malformation. All individuals were able to detect and recognize a variety of environmental sounds that would previously have been inaudible. These findings, along with the reported improvement in quality of life, mean that Mondini dysplasia is not a contra-indication for multichannel cochlear implantation. This information will be useful to other centres when considering implantation in similar patients.

Impaired Central Pulsatile Hemodynamics in Children and Adolescents With Marfan Syndrome.

Science.gov (United States)

Grillo, Andrea; Salvi, Paolo; Marelli, Susan; Gao, Lan; Salvi, Lucia; Faini, Andrea; Trifirò, Giuliana; Carretta, Renzo; Pini, Alessandro; Parati, Gianfranco

2017-11-07

Marfan syndrome is characterized by aortic root dilation, beginning in childhood. Data about aortic pulsatile hemodynamics and stiffness in pediatric age are currently lacking. In 51 young patients with Marfan syndrome (12.0±3.3 years), carotid tonometry was performed for the measurement of central pulse pressure, pulse pressure amplification, and aortic stiffness (carotid-femoral pulse wave velocity). Patients underwent an echocardiogram at baseline and at 1 year follow-up and a genetic evaluation. Pathogenetic fibrillin-1 mutations were classified between "dominant negative" and "haploinsufficient." The hemodynamic parameters of patients were compared with those of 80 sex, age, blood pressure, and heart-rate matched controls. Central pulse pressure was significantly higher (38.3±12.3 versus 33.6±7.8 mm Hg; P =0.009), and pulse pressure amplification was significantly reduced in Marfan than controls (17.9±15.3% versus 32.3±17.4%; P Marfan and controls (4.98±1.00 versus 4.75±0.67 m/s). In the Marfan group, central pulse pressure and pulse pressure amplification were independently associated with aortic diameter at the sinuses of Valsalva (respectively, β=0.371, P =0.010; β=-0.271, P =0.026). No significant difference in hemodynamic parameters was found according to fibrillin-1 genotype. Patients who increased aortic Z-scores at 1-year follow-up presented a higher central pulse pressure than the remaining (42.7±14.2 versus 32.3±5.9 mm Hg; P =0.004). Central pulse pressure and pulse pressure amplification were impaired in pediatric Marfan syndrome, and associated with aortic root diameters, whereas aortic pulse wave velocity was similar to that of a general pediatric population. An increased central pulse pressure was present among patients whose aortic dilatation worsened at 1-year follow-up. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

The significance of a hypoplastic bony canal for the cochlear nerve in patients with sensorineural hearing loss: CT and MRI findings

International Nuclear Information System (INIS)

Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun

2004-01-01

The purpose of this study is to evaluate the significance of the hypoplastic canal for the cochlear nerve in patients with sensorineural hearing loss (SNHL) and the relationship between the hypoplastic bony canal and aplasia or hypoplasia of the cochlear nerve. A retrospective review of high resolution temporal CT(HRCT) and MRI findings was conducted. The narrow bony canal of the cochlear nerve and the relative size of the internal auditory canal were correlated with the cochlear nerve deficiency on MRI. The comparative size of the component nerves (facial, cochlear, superior vestibular, inferior vestibular nerve), and the relative size of the internal auditory canal and the bony canal of the cochlear nerve were measured. The clinical history and the results of the clinical examination were reviewed for each patient. High resolution MRI showed aplasia of the common vestibulocochlear nerve in one patient and a deficiency of the cochlear nerve in 9 patients. These abnormalities occurred in association with a prominent narrowing of the canal for the cochlear nerve and a stenosis of the internal auditory canal, which was observed on temporal bone CT in 9 patients with congenital SNHL. Three patients had normal IAC, despite the presence of a hypoplastic cochlear nerve on the side on which they had SNHL. In one patient, the narrowing of the canal for the cochlear nerve and internal auditory canal were not found to be associated with acquired SNHL. The hypoplastic bony canal for the cochlear nerve might be more highly indicative of congenital cochlear nerve deficiency than that of the narrow internal auditory canal, and the position of the crista falciformis should also be carefully

The significance of a hypoplastic bony canal for the cochlear nerve in patients with sensorineural hearing loss: CT and MRI findings

Energy Technology Data Exchange (ETDEWEB)

Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun [College of Medicine, Yonsei Univ., Wonju (Korea, Republic of)

2004-04-01

The purpose of this study is to evaluate the significance of the hypoplastic canal for the cochlear nerve in patients with sensorineural hearing loss (SNHL) and the relationship between the hypoplastic bony canal and aplasia or hypoplasia of the cochlear nerve. A retrospective review of high resolution temporal CT(HRCT) and MRI findings was conducted. The narrow bony canal of the cochlear nerve and the relative size of the internal auditory canal were correlated with the cochlear nerve deficiency on MRI. The comparative size of the component nerves (facial, cochlear, superior vestibular, inferior vestibular nerve), and the relative size of the internal auditory canal and the bony canal of the cochlear nerve were measured. The clinical history and the results of the clinical examination were reviewed for each patient. High resolution MRI showed aplasia of the common vestibulocochlear nerve in one patient and a deficiency of the cochlear nerve in 9 patients. These abnormalities occurred in association with a prominent narrowing of the canal for the cochlear nerve and a stenosis of the internal auditory canal, which was observed on temporal bone CT in 9 patients with congenital SNHL. Three patients had normal IAC, despite the presence of a hypoplastic cochlear nerve on the side on which they had SNHL. In one patient, the narrowing of the canal for the cochlear nerve and internal auditory canal were not found to be associated with acquired SNHL. The hypoplastic bony canal for the cochlear nerve might be more highly indicative of congenital cochlear nerve deficiency than that of the narrow internal auditory canal, and the position of the crista falciformis should also be carefully.

Metabolic syndrome impairs notch signaling and promotes apoptosis in chronically ischemic myocardium.

Science.gov (United States)

Elmadhun, Nassrene Y; Sabe, Ashraf A; Lassaletta, Antonio D; Chu, Louis M; Kondra, Katelyn; Sturek, Michael; Sellke, Frank W

2014-09-01

Impaired angiogenesis is a known consequence of metabolic syndrome (MetS); however, the mechanism is not fully understood. Recent studies have shown that the notch signaling pathway is an integral component of cardiac angiogenesis. We tested, in a clinically relevant swine model, the effects of MetS on notch and apoptosis signaling in chronically ischemic myocardium. Ossabaw swine were fed either a regular diet (control [CTL], n = 8) or a high-cholesterol diet (MetS, n = 8) to induce MetS. An ameroid constrictor was placed to induce chronic myocardial ischemia. Eleven weeks later, the wine underwent cardiac harvest of the ischemic myocardium. Downregulation of pro-angiogenesis proteins notch2, notch4, jagged2, angiopoietin 1, and endothelial nitric oxide synthase were found in the MetS group compared with the CTL group. Also, upregulation of pro-apoptosis protein caspase 8 and downregulation of anti-angiogenesis protein phosphorylated forkhead box transcription factor 03 and pro-survival proteins phosphorylated P38 and heat shock protein 90 were present in the MetS group. Cell death was increased in the MetS group compared with the CTL group. Both CTL and MetS groups had a similar arteriolar count and capillary density, and notch3 and jagged1 were both similarly concentrated in the smooth muscle wall. MetS in chronic myocardial ischemia significantly impairs notch signaling by downregulating notch receptors, ligands, and pro-angiogenesis proteins. MetS also increases apoptosis signaling, decreases survival signaling, and increases cell death in chronically ischemic myocardium. Although short-term angiogenesis appears unaffected in this model of early MetS, the molecular signals for angiogenesis are impaired, suggesting that inhibition of notch signaling might underlie the decreased angiogenesis in later stages of MetS. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

Speech perception and production in children with inner ear malformations after cochlear implantation.

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Rachovitsas, Dimitrios; Psillas, George; Chatzigiannakidou, Vasiliki; Triaridis, Stefanos; Constantinidis, Jiannis; Vital, Victor

2012-09-01

The aim of this study was to assess the speech perception and speech intelligibility outcome after cochlear implantation in children with malformed inner ear and to compare them with a group of congenitally deaf children implantees without inner ear malformation. Six deaf children (five boys and one girl) with inner ear malformations who were implanted and followed in our clinic were included. These children were matched with six implanted children with normal cochlea for age at implantation and duration of cochlear implant use. All subjects were tested with the internationally used battery tests of listening progress profile (LiP), capacity of auditory performance (CAP), and speech intelligibility rating (SIR). A closed and open set word perception test adapted to the Modern Greek language was also used. In the dysplastic group, two children suffered from CHARGE syndrome, another two from mental retardation, and two children grew up in bilingual homes. At least two years after switch-on, the dysplastic group scored mean LiP 62%, CAP 3.8, SIR 2.1, closed-set 61%, and open-set 49%. The children without inner ear dysplasia achieved significantly better scores, except for CAP which this difference was marginally statistically significant (p=0.009 for LiP, p=0.080 for CAP, p=0.041 for SIR, p=0.011 for closed-set, and p=0.006 for open-set tests). All of the implanted children with malformed inner ear showed benefit of auditory perception and speech production. However, the children with inner ear malformation performed less well compared with the children without inner ear dysplasia. This was possibly due to the high proportion of disabilities detected in the dysplastic group, such as CHARGE syndrome and mental retardation. Bilingualism could also be considered as a factor which possibly affects the outcome of implanted children. Therefore, children with malformed inner ear should be preoperatively evaluated for cognitive and developmental delay. In this case

Advances in cochlear implant telemetry: evoked neural responses, electrical field imaging, and technical integrity.

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Mens, L.H.M.

2007-01-01

During the last decade, cochlear implantation has evolved into a well-established treatment of deafness, predominantly because of many improvements in speech processing and the controlled excitation of the auditory nerve. Cochlear implants now also feature telemetry, which is highly useful to

Rapid assessment of bilateral cochlear implantation for children in Kazakhstan.

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Kosherbayeva, Lyazzat; Hailey, David; Kozhageldiyeva, Laura

2014-10-01

The aim of this study was to evaluate the effectiveness of bilateral cochlear implantation (CI) compared with unilateral CI for deaf children in the context of the Republic of Kazakhstan health system. Methods. A literature search was conducted, using the PubMed, Cochrane, and Embase data bases for studies that compared the effectiveness of bilateral and unilateral CI in children. The search included English language, publications from 2002-2012. Two reviewers independently evaluated all relevant studies. Administrative data relevant to CI in Kazakhstan were obtained from the Ministry of Health. Three relevant systematic reviews and an health technology assessment report were found. There was evidence of incremental benefits from bilateral CI but the quality of the available studies was poor and there was little information on longer term outcomes. No conclusions could be drawn regarding later incremental improvements to speech perception, learning, and quality of life. To date, in the Republic of Kazakhstan there is not full coverage of audiological screening due to the lack of medical equipment. This leads to late detection of hearing-impaired children and a long rehabilitation period, requiring more resources. Age of implantation in children is late and only a small minority attend general schools. The clinical effectiveness of bilateral CI, an expensive health technology, requires further study. Given the current situation in Kazakhstan with audiological screening and access to unilateral CI, there appeared to be other priorities for improving services for children with profound hearing impairment.

Hearing experience and receptive vocabulary development in deaf children with cochlear implants.

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Fagan, Mary K; Pisoni, David B

2010-01-01

This study investigated receptive vocabulary delay in deaf children with cochlear implants. Participants were 23 children with profound hearing loss, ages 6-14 years, who received a cochlear implant between ages 1.4 and 6 years. Duration of cochlear implant use ranged from 3.7 to 11.8 years. Peabody Picture Vocabulary Test, Third Edition (PPVT-III) data were analyzed first by examining children's errors for evidence of difficulty in specific lexical content areas, and second by calculating standard scores with reference to hearing age (HA) (i.e., chronological age [CA]--age at implantation) rather than CA. Participants showed evidence of vocabulary understanding across all PPVT-III content categories with no strong evidence of disproportionate numbers of errors in any specific content area despite below-average mean standard scores. However, whereas mean standard scores were below the test mean established for hearing children when based on CA, they were within the average range for hearing children when calculated based on HA. Thus, children's vocabulary knowledge was commensurate with years of cochlear implant experience, providing support for the role of spoken language experience in vocabulary acquisition.

Early presentation of gait impairment in Wolfram Syndrome

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Pickett Kristen A

2012-12-01

Full Text Available Abstract Background Classically characterized by early onset insulin-dependent diabetes mellitus, optic atrophy, deafness, diabetes insipidus, and neurological abnormalities, Wolfram syndrome (WFS is also associated with atypical brainstem and cerebellar findings in the first decade of life. As such, we hypothesized that gait differences between individuals with WFS and typically developing (TD individuals may be detectable across the course of the disease. Methods Gait was assessed for 13 individuals with WFS (min 6.4 yrs, max 25.8 yrs and 29 age-matched, typically developing individuals (min 5.6 yrs, max 28.5 yrs using a GAITRite ® walkway system. Velocity, cadence, step length, base of support and double support time were compared between groups. Results Across all tasks, individuals with WFS walked slower (p = 0.03, took shorter (p ≤ 0.001 and wider (p ≤ 0.001 steps and spent a greater proportion of the gait cycle in double support (p = 0.03 compared to TD individuals. Cadence did not differ between groups (p = 0.62. Across all tasks, age was significantly correlated with cadence and double support time in the TD group but only double support time was correlated with age in the WFS group and only during preferred pace forward (rs= 0.564, p = 0.045 and dual task forward walking (rs= 0.720, p = 0.006 tasks. Individuals with WFS also had a greater number of missteps during tandem walking (p ≤ 0.001. Within the WFS group, spatiotemporal measures of gait did not correlate with measures of visual acuity. Balance measures negatively correlated with normalized gait velocity during fast forward walking (rs = −0.59, p = 0.03 and percent of gait cycle in double support during backward walking (rs = −0.64, p = 0.03. Conclusions Quantifiable gait impairments can be detected in individuals with WFS earlier than previous clinical observations suggested. These impairments are not fully accounted for by the visual or balance deficits

Early presentation of gait impairment in Wolfram Syndrome.

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Pickett, Kristen A; Duncan, Ryan P; Hoekel, James; Marshall, Bess; Hershey, Tamara; Earhart, Gammon M

2012-12-08

Classically characterized by early onset insulin-dependent diabetes mellitus, optic atrophy, deafness, diabetes insipidus, and neurological abnormalities, Wolfram syndrome (WFS) is also associated with atypical brainstem and cerebellar findings in the first decade of life. As such, we hypothesized that gait differences between individuals with WFS and typically developing (TD) individuals may be detectable across the course of the disease. Gait was assessed for 13 individuals with WFS (min 6.4 yrs, max 25.8 yrs) and 29 age-matched, typically developing individuals (min 5.6 yrs, max 28.5 yrs) using a GAITRite ® walkway system. Velocity, cadence, step length, base of support and double support time were compared between groups. Across all tasks, individuals with WFS walked slower (p = 0.03), took shorter (p ≤ 0.001) and wider (p ≤ 0.001) steps and spent a greater proportion of the gait cycle in double support (p = 0.03) compared to TD individuals. Cadence did not differ between groups (p = 0.62). Across all tasks, age was significantly correlated with cadence and double support time in the TD group but only double support time was correlated with age in the WFS group and only during preferred pace forward (rs = 0.564, p = 0.045) and dual task forward walking (rs = 0.720, p = 0.006) tasks. Individuals with WFS also had a greater number of missteps during tandem walking (p ≤ 0.001). Within the WFS group, spatiotemporal measures of gait did not correlate with measures of visual acuity. Balance measures negatively correlated with normalized gait velocity during fast forward walking (rs = -0.59, p = 0.03) and percent of gait cycle in double support during backward walking (rs = -0.64, p = 0.03). Quantifiable gait impairments can be detected in individuals with WFS earlier than previous clinical observations suggested. These impairments are not fully accounted for by the visual or balance deficits associated with WFS, and may be a reflection of early cerebellar and

Cochlear implant: the family's perspective.

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Vieira, Sheila de Souza; Dupas, Giselle; Chiari, Brasilia Maria

2018-07-01

To understand the family's experience of a child who uses a cochlear implant (CI). Specifically, to identify the difficulties, changes, and feelings entailed by deafness and the use of the CI; the coping strategies; and to understand the role of the family for the child with a CI. Qualitative research, using Symbolic Interactionism and Straussian Grounded Theory as the theoretical and methodological frameworks, respectively. Data collection instrument: semi-structured interview. A total of 9 families (32 individuals) participated in the study. The children's ages ranged from 6 to 11 years old (mean = 8.9 years old). Their experience is described in the following categories: Having to fight for results, Coping with difficult situations, Recognizing that you are not alone, Learning to overcome, and Having one's life restored by the implant. Cochlear implantation changes the direction of the child and the family's life by restoring the child's opportunity to hear and to obtain good results in her personal, social, and academic development. Even after implantation, the child continues to experience difficulties and requires the family's mobilization in order to be successful. The family is the principal actor in the process of the child's rehabilitation.

Dislocation of cochlear implant magnet as a complication following MRI.

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Murtojärvi, Sarita; Salonen, Jaakko

According to current best knowledge, an MRI scan can be performed for patients with cochlear implants. The warnings and recommendations of the implant manufacturers must be followed strictly to prevent complications, such as overheating, migration or demagnetization of the magnet in the implant. We report on a case of cochlear implant magnet dislocation as a complication for an MRI scan. The patient had a tight bandage around the head to hold the magnet in place as recommended by the manufacturer, but apparently the bandage was not in the correct place.

Continued expression of GATA3 is necessary for cochlear neurosensory development.

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Jeremy S Duncan

Full Text Available Hair cells of the developing mammalian inner ear are progressively defined through cell fate restriction. This process culminates in the expression of the bHLH transcription factor Atoh1, which is necessary for differentiation of hair cells, but not for their specification. Loss of several genes will disrupt ear morphogenesis or arrest of neurosensory epithelia development. We previously showed in null mutants that the loss of the transcription factor, Gata3, results specifically in the loss of all cochlear neurosensory development. Temporal expression of Gata3 is broad from the otic placode stage through the postnatal ear. It therefore remains unclear at which stage in development Gata3 exerts its effect. To better understand the stage specific effects of Gata3, we investigated the role of Gata3 in cochlear neurosensory specification and differentiation utilizing a LoxP targeted Gata3 line and two Cre lines. Foxg1(Cre∶Gata3(f/f mice show recombination of Gata3 around E8.5 but continue to develop a cochlear duct without differentiated hair cells and spiral ganglion neurons. qRT-PCR data show that Atoh1 was down-regulated but not absent in the duct whereas other hair cell specific genes such as Pou4f3 were completely absent. In addition, while Sox2 levels were lower in the Foxg1(Cre:Gata3(f/f cochlea, Eya1 levels remained normal. We conclude that Eya1 is unable to fully upregulate Atoh1 or Pou4f3, and drive differentiation of hair cells without Gata3. Pax2-Cre∶Gata3(f/f mice show a delayed recombination of Gata3 in the ear relative to Foxg1(Cre:Gata3(f/f . These mice exhibited a cochlear duct containing patches of partially differentiated hair cells and developed only few and incorrectly projecting spiral ganglion neurons. Our conditional deletion studies reveal a major role of Gata3 in the signaling of prosensory genes and in the differentiation of cochlear neurosenory cells. We suggest that Gata3 may act in combination with Eya1, Six1, and

Cochlear implant benefits in deafness rehabilitation: PET study of temporal voice Activations

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Coez, A.; Zilbovicius, M. [CEA, Serv Hosp Frederic Joliot, INSERM, Res Unit Neuroimaging and Psychiat, U797, IFR49, F-91406 Orsay (France); Zilbovicius, M.; Syrota, A.; Samson, Y. [CEA, DSV, DRM, Serv Hosp Frederic Joliot, F-91406 Orsay (France); Bizaguet, E. [Lab Correct Audit, Paris (France); Coez, A. [Univ Paris Sud 11, Paris (France); Ferrary, E.; Bouccara, D.; Mosnier, I.; Sterkers, O. [INSERM, Unit M 867, Paris (France); Ambert-Dahan, E. [Hop Beaujon, Serv ORL Chirurg Cervicofaciale, AP-HP, Clichy (France); Ferrary, E.; Bouccara, D.; Mosnier, I.; Sterkers, O. [Inst Fed Rech Claude Bernard Physiol et Pathol, IFR02, Paris (France); Samson, Y. [Hop La Pitie Salpetriere, Serv Urgences Cerebro-vasc, AP-HP, Paris (France); Samson, Y. [Univ Paris 06, Paris (France); Sterkers, O. [Univ Denis Diderot Paris 7, Paris (France)

2008-07-01

Cochlear implants may improve the medical and social prognosis of profound deafness. Nevertheless, some patients have experienced poor results without any clear explanations. One correlate may be an alteration in cortical voice processing. To test this hypothesis, we studied the activation of human temporal voice areas (TVA) using a well-standardized PET paradigm adapted from previous functional MRI (fMRI) studies. Methods: A PET H{sub 2}{sup 15}O activation study was performed on 3 groups of adult volunteers: normal-hearing control subjects (n 6) and cochlear-implanted post-lingually deaf patients with {>=}2 y of cochlear implant experience, with intelligibility scores in the 'Lafon monosyllabic task' {>=}80% (Good group; n 6) or {<=}20% (Poor group; n 6). Relative cerebral blood flow was measured in 3 conditions: rest, passive listening to human voice, and non-voice stimuli. Results: Compared with silence, the activations induced by non-voice stimuli were bilaterally located in the superior temporal regions in all groups. However these activations were significantly and similarly reduced in both cochlear implant groups, whereas control subjects showed supplementary activations. Compared with non-voice, the voice stimuli induced bilateral activation of the TVA along the superior temporal sulcus (STS) in both the control and the Good groups. In contrast, these activations were not detected in the Poor group, which showed only left unilateral middle STS activation. Conclusion: These results suggest that PET is an adequate method to explore cochlear implant benefits and that this benefit could be linked to the activation of the TVA. (authors)

Cochlear implant benefits in deafness rehabilitation: PET study of temporal voice Activations

International Nuclear Information System (INIS)

Coez, A.; Zilbovicius, M.; Zilbovicius, M.; Syrota, A.; Samson, Y.; Bizaguet, E.; Coez, A.; Ferrary, E.; Bouccara, D.; Mosnier, I.; Sterkers, O.; Ambert-Dahan, E.; Ferrary, E.; Bouccara, D.; Mosnier, I.; Sterkers, O.; Samson, Y.; Samson, Y.; Sterkers, O.

2008-01-01

Cochlear implants may improve the medical and social prognosis of profound deafness. Nevertheless, some patients have experienced poor results without any clear explanations. One correlate may be an alteration in cortical voice processing. To test this hypothesis, we studied the activation of human temporal voice areas (TVA) using a well-standardized PET paradigm adapted from previous functional MRI (fMRI) studies. Methods: A PET H 2 15 O activation study was performed on 3 groups of adult volunteers: normal-hearing control subjects (n 6) and cochlear-implanted post-lingually deaf patients with ≥2 y of cochlear implant experience, with intelligibility scores in the 'Lafon monosyllabic task' ≥80% (Good group; n 6) or ≤20% (Poor group; n 6). Relative cerebral blood flow was measured in 3 conditions: rest, passive listening to human voice, and non-voice stimuli. Results: Compared with silence, the activations induced by non-voice stimuli were bilaterally located in the superior temporal regions in all groups. However these activations were significantly and similarly reduced in both cochlear implant groups, whereas control subjects showed supplementary activations. Compared with non-voice, the voice stimuli induced bilateral activation of the TVA along the superior temporal sulcus (STS) in both the control and the Good groups. In contrast, these activations were not detected in the Poor group, which showed only left unilateral middle STS activation. Conclusion: These results suggest that PET is an adequate method to explore cochlear implant benefits and that this benefit could be linked to the activation of the TVA. (authors)

Nakalanga Syndrome: Clinical Characteristics, Potential Causes, and Its Relationship with Recently Described Nodding Syndrome.

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Kathrin Föger

2017-02-01

Full Text Available Nakalanga syndrome is a condition that was described in Uganda and various other African countries decades ago. Its features include growth retardation, physical deformities, endocrine dysfunction, mental impairment, and epilepsy, amongst others. Its cause remains obscure. Nodding syndrome is a neurological disorder with some features in common with Nakalanga syndrome, which has been described mainly in Uganda, South Sudan, and Tanzania. It has been considered an encephalopathy affecting children who, besides head nodding attacks, can also present with stunted growth, delayed puberty, and mental impairment, amongst other symptoms. Despite active research over the last years on the pathogenesis of Nodding syndrome, to date, no convincing single cause of Nodding syndrome has been reported. In this review, by means of a thorough literature search, we compare features of both disorders. We conclude that Nakalanga and Nodding syndromes are closely related and may represent the same condition. Our findings may provide new directions in research on the cause underlying this neurological disorder.

Evaluation of visual impairment in Usher syndrome 1b and Usher syndrome 2a.

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Pennings, Ronald J E; Huygen, Patrick L M; Orten, Dana J; Wagenaar, Mariette; van Aarem, Annelies; Kremer, Hannie; Kimberling, William J; Cremers, Cor W R J; Deutman, August F

2004-04-01

To evaluate visual impairment in Usher syndrome 1b (USH1b) and Usher syndrome 2a (USH2a). We carried out a retrospective study of 19 USH1b patients and 40 USH2a patients. Cross-sectional regression analyses of the functional acuity score (FAS), functional field score (FFS) and functional vision score (FVS) related to age were performed. Statistical tests relating to regression lines and Student's t-test were used to compare between (sub)groups of patients. Parts of the available individual longitudinal data were used to obtain individual estimates of progressive deterioration and compare these to those obtained with cross-sectional analysis. Results were compared between subgroups of USH2a patients pertaining to combinations of different types of mutations. Cross-sectional analyses revealed significant deterioration of the FAS (0.7% per year), FFS (1.0% per year) and FVS (1.5% per year) with advancing age in both patient groups, without a significant difference between the USH1b and USH2a patients. Individual estimates of the deterioration rates were substantially and significantly higher than the cross-sectional estimates in some USH2a cases, including values of about 5% per year (or even higher) for the FAS (age 35-50 years), 3-4% per year for the FFS and 4-5% per year for the FVS (age > 20 years). There was no difference in functional vision score behaviour detected between subgroups of patients pertaining to different biallelic combinations of specific types of mutations. The FAS, FFS and FVS deteriorated significantly by 0.7-1.5% per year according to cross-sectional linear regression analysis in both USH1b and USH2a patients. Higher deterioration rates (3-5% per year) in any of these scores were attained, according to longitudinal data collected from individual USH2a patients. Score behaviour was similar across the patient groups and across different biallelic combinations of various types of mutations. However, more elaborate studies, preferably covering

Cushing's syndrome: a model for sarcopenic obesity.

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Drey, Michael; Berr, Christina M; Reincke, Martin; Fazel, Julia; Seissler, Jochen; Schopohl, Jochen; Bidlingmaier, Martin; Zopp, Stefanie; Reisch, Nicole; Beuschlein, Felix; Osswald, Andrea; Schmidmaier, Ralf

2017-09-01

Obesity and its metabolic impairments are discussed as major risk factors for sarcopenia leading to sarcopenic obesity. Cushing's syndrome is known to be associated with obesity and muscle atrophy. We compared Cushing's syndrome with matched obese controls regarding body composition, physical performance, and biochemical markers to test the hypothesis that Cushing's syndrome could be a model for sarcopenic obesity. By propensity score matching, 47 controls were selected by body mass index and gender as obese controls. Fat mass and muscle mass were measured by bioelectrical impedance analysis. Muscle function was assessed by chair rising test and hand grip strength. Biochemical markers of glucose and lipid metabolism and inflammation (hsCRP) were measured in peripheral blood. Muscle mass did not differ between Cushing's syndrome and obese controls. However, Cushing's syndrome patients showed significantly greater chair rising time (9.5 s vs. 7.3 s, p = 0.008) and significantly lower hand grip strength (32.1 kg vs. 36.8 kg, p = 0.003). Cushing's syndrome patients with impaired fasting glucose have shown the highest limitations in hand grip strength and chair rising time. Similar to published data in ageing medicine, Cushing's syndrome patients show loss of muscle function that cannot be explained by loss of muscle mass. Impaired muscle quality due to fat infiltration may be the reason. This is supported by the observation that Cushing's syndrome patients with impaired glucose metabolism show strongest deterioration of muscle function. Research in sarcopenic obesity in elderly is hampered by confounding comorbidities and polypharmacy. As Cushing's syndrome patients are frequently free of comorbidities and as Cushing's syndrome is potentially curable we suggest Cushing's syndrome as a clinical model for further research in sarcopenic obesity.

Electroacoustic verification of frequency modulation systems in cochlear implant users.

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Fidêncio, Vanessa Luisa Destro; Jacob, Regina Tangerino de Souza; Tanamati, Liége Franzini; Bucuvic, Érika Cristina; Moret, Adriane Lima Mortari

2017-12-26

The frequency modulation system is a device that helps to improve speech perception in noise and is considered the most beneficial approach to improve speech recognition in noise in cochlear implant users. According to guidelines, there is a need to perform a check before fitting the frequency modulation system. Although there are recommendations regarding the behavioral tests that should be performed at the fitting of the frequency modulation system to cochlear implant users, there are no published recommendations regarding the electroacoustic test that should be performed. Perform and determine the validity of an electroacoustic verification test for frequency modulation systems coupled to different cochlear implant speech processors. The sample included 40 participants between 5 and 18 year's users of four different models of speech processors. For the electroacoustic evaluation, we used the Audioscan Verifit device with the HA-1 coupler and the listening check devices corresponding to each speech processor model. In cases where the transparency was not achieved, a modification was made in the frequency modulation gain adjustment and we used the Brazilian version of the "Phrases in Noise Test" to evaluate the speech perception in competitive noise. It was observed that there was transparency between the frequency modulation system and the cochlear implant in 85% of the participants evaluated. After adjusting the gain of the frequency modulation receiver in the other participants, the devices showed transparency when the electroacoustic verification test was repeated. It was also observed that patients demonstrated better performance in speech perception in noise after a new adjustment, that is, in these cases; the electroacoustic transparency caused behavioral transparency. The electroacoustic evaluation protocol suggested was effective in evaluation of transparency between the frequency modulation system and the cochlear implant. Performing the adjustment of

A case of improved hearing with cochlear implantation in Gaucher disease type 1.

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Endo, Shiori; Mizuta, Kunihiro; Yamatodani, Takashi; Nakanishi, Hiroshi; Hosokawa, Kumiko; Misawa, Kiyoshi; Hosokawa, Seiji; Mineta, Hiroyuki

2018-06-01

Gaucher disease is a lysosomal storage disorder that is caused by congenital defective function of the enzyme glucocerebrosidase. Glucocerebroside that is not hydrolyzed by glucocerebrosidase mainly accumulates in the reticular tissue. We describe a Japanese boy with Gaucher disease type 1 who developed bilateral profound sensorineural hearing loss within approximately 4years. We performed cochlear implantation initially on his right ear and again on his left ear 5 months later. The cochlear implants were successfully utilized with a speech discrimination score of 95% on a Japanese sentence recognition test. There are many reports of central hearing loss in Gaucher disease type 2 or 3. However, to the best of our knowledge, this is the first report of profound inner ear hearing loss with Gaucher disease. It also appears to be the first record of cochlear implantation for Gaucher disease. Cochlear implants may be useful for sensorineural hearing loss in patients with Gaucher disease without neurological symptoms other than hearing loss. Copyright © 2017 Elsevier B.V. All rights reserved.

Electrode selection for hearing preservation in cochlear implantation: A review of the evidence

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Jason A. Brant

2016-09-01

Full Text Available Objective: To review and assess the ideal length of electrode in cochlear implant patients for hearing preservation. Methods: The English language literature was reviewed for studies including hearing preservation and speech understanding for electrodes of different lengths. Results: One prospective trial was found, and there were no studies that randomized patients into different length electrodes with an intent to preserve hearing. Eight studies total included multiple length electrodes and contained data regarding hearing preservation. Conclusions: Although there is some evidence that indicates that shorter electrodes may improve both short and long-term hearing preservation rates in cochlear implant patients, no study has directly compared implant length on hearing preservation in a similar patient population. A randomized trial of short and standard length electrodes for hearing preservation is warranted. In the interim, utilization of current electrodes measuring 20–25 mm could seem to be a prudent approach when seeking to preserve residual hearing without unduly compromising cochlear coverage. Keywords: Electrode, Length, Hearing preservation, Cochlear implantation

Manifestations of Renal Impairment in Fructose-induced Metabolic Syndrome.

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Bratoeva, Kameliya; Stoyanov, George S; Merdzhanova, Albena; Radanova, Mariya

2017-11-07

Introduction International studies show an increased incidence of chronic kidney disease (CKD) in patients with metabolic syndrome (MS). It is assumed that the major components of MS - obesity, insulin resistance, dyslipidemia, and hypertension - are linked to renal damage through the systemic release of several pro-inflammatory mediators, such as uric acid (UA), C-reactive protein (CRP), and generalized oxidative stress. The aim of the present study was to investigate the extent of kidney impairment and manifestations of dysfunction in rats with fructose-induced MS. Methods We used a model of high-fructose diet in male Wistar rats with 35% glucose-fructose corn syrup in drinking water over a duration of 16 weeks. The experimental animals were divided into two groups: control and high-fructose drinking (HFD). Serum samples were obtained from both groups for laboratory study, and the kidneys were extracted for observation via light microscopy examination. Results All HFD rats developed obesity, hyperglycemia, hypertriglyceridemia, increased levels of CRP and UA (when compared to the control group), and oxidative stress with high levels of malondialdehyde and low levels of reduced glutathione. The kidneys of the HFD group revealed a significant increase in kidney weight in the absence of evidence of renal dysfunction and electrolyte disturbances. Under light microscopy, the kidneys of the HFD group revealed amyloid deposits in Kimmelstiel-Wilson-like nodules and the walls of the large caliber blood vessels, early-stage atherosclerosis with visible ruptures and scarring, hydropic change (vacuolar degeneration) in the epithelial cells covering the proximal tubules, and increased eosinophilia in the distant tubules when compared to the control group. Conclusion Under the conditions of a fructose-induced metabolic syndrome, high serum UA and CRP correlate to the development of early renal disorders without a clinical manifestation of renal dysfunction. These

EEG frontal asymmetry related to pleasantness of music perception in healthy children and cochlear implanted users.

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Vecchiato, G; Maglione, A G; Scorpecci, A; Malerba, P; Marsella, P; Di Francesco, G; Vitiello, S; Colosimo, A; Babiloni, Fabio

2012-01-01

Interestingly, the international debate about the quality of music fruition for cochlear implanted users does not take into account the hypothesis that bilateral users could perceive music in a more pleasant way with respect to monolateral users. In this scenario, the aim of the present study was to investigate if cerebral signs of pleasantness during music perception in healthy child are similar to those observed in monolateral and in bilateral cochlear implanted users. In fact, previous observations in literature on healthy subjects have indicated that variations of the frontal EEG alpha activity are correlated with the perceived pleasantness of the sensory stimulation received (approach-withdrawal theory). In particular, here we described differences between cortical activities estimated in the alpha frequency band for a healthy child and in patients having a monolateral or a bilateral cochlear implant during the fruition of a musical cartoon. The results of the present analysis showed that the alpha EEG asymmetry patterns observed in a healthy child and that of a bilateral cochlear implanted patient are congruent with the approach-withdrawal theory. Conversely, the scalp topographic distribution of EEG power spectra in the alpha band resulting from the monolateral cochlear user presents a different EEG pattern from the normal and bilateral implanted patients. Such differences could be explained at the light of the approach-withdrawal theory. In fact, the present findings support the hypothesis that a monolateral cochlear implanted user could perceive the music in a less pleasant way when compared to a healthy subject or to a bilateral cochlear user.

Degradation of labial information modifies audiovisual speech perception in cochlear-implanted children.

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Huyse, Aurélie; Berthommier, Frédéric; Leybaert, Jacqueline

2013-01-01

The aim of the present study was to examine audiovisual speech integration in cochlear-implanted children and in normally hearing children exposed to degraded auditory stimuli. Previous studies have shown that speech perception in cochlear-implanted users is biased toward the visual modality when audition and vision provide conflicting information. Our main question was whether an experimentally designed degradation of the visual speech cue would increase the importance of audition in the response pattern. The impact of auditory proficiency was also investigated. A group of 31 children with cochlear implants and a group of 31 normally hearing children matched for chronological age were recruited. All children with cochlear implants had profound congenital deafness and had used their implants for at least 2 years. Participants had to perform an /aCa/ consonant-identification task in which stimuli were presented randomly in three conditions: auditory only, visual only, and audiovisual (congruent and incongruent McGurk stimuli). In half of the experiment, the visual speech cue was normal; in the other half (visual reduction) a degraded visual signal was presented, aimed at preventing lipreading of good quality. The normally hearing children received a spectrally reduced speech signal (simulating the input delivered by the cochlear implant). First, performance in visual-only and in congruent audiovisual modalities were decreased, showing that the visual reduction technique used here was efficient at degrading lipreading. Second, in the incongruent audiovisual trials, visual reduction led to a major increase in the number of auditory based responses in both groups. Differences between proficient and nonproficient children were found in both groups, with nonproficient children's responses being more visual and less auditory than those of proficient children. Further analysis revealed that differences between visually clear and visually reduced conditions and between

Routes, dynamics, and correlates of cochlear inflammation in terminal and recovering experimental meningitis

DEFF Research Database (Denmark)

Cayé-Thomasen, Per; Worsøe, Lise; Brandt, Christian Thomas

2009-01-01

OBJECTIVES/HYPOTHESIS: To examine the routes, dynamics and correlates of cochlear inflammation in meningitis to provide information on the pathogenesis of the associated hearing loss and indications for rational pharmacotherapeutical intervention. STUDY DESIGN: A well-established rat model...... with inflammatory cells via cochlear aqueduct, whereas the endolymphatic space was infiltrated from the spiral ligament. Rosenthal's canal was infiltrated through osseous spiral lamina canaliculi. In the untreated group, the degree of inflammation correlated with time of death, whereas antibiotic treatment reversed...... this development. Perilymphatic inflammation correlated significantly with the CSF leukocyte count, whereas endolymphatic inflammation correlated with spiral ligament inflammation. CONCLUSIONS: Meningogenic inflammation of the rat cochlea occurs via the cochlear aqueduct and the spiral ligament capillary bed...

The perception of stress pattern in young cochlear implanted children: an EEG study

Directory of Open Access Journals (Sweden)

Niki Katerina Vavatzanidis

2016-03-01

Full Text Available Children with sensorineural hearing loss may (regain hearing with a cochlear implant – a device that transforms sounds into electric pulses and bypasses the dysfunctioning inner ear by stimulating the auditory nerve directly with an electrode array. Many implanted children master the acquisition of spoken language successfully, yet we still have little knowledge of the actual input they receive with the implant and specifically which language sensitive cues they hear. This would be important however, both for understanding the flexibility of the auditory system when presented with stimuli after a (life-long phase of deprivation and for planning therapeutic intervention. In rhythmic languages the general stress pattern conveys important information about word boundaries. Infant language acquisition relies on such cues and can be severely hampered when this information is missing, as seen for dyslexic children and children with specific language impairment. Here we ask whether children with a cochlear implant perceive differences in stress patterns during their language acquisition phase and if they do, whether it is present directly following implant stimulation or if and how much time is needed for the auditory system to adapt to the new sensory modality. We performed a longitudinal ERP study, testing in bimonthly intervals the stress pattern perception of 17 young hearing impaired children (age range: 9-50 months; mean: 22 months during their first 6 months of implant use. An additional session before the implantation served as control baseline. During a session they passively listened to an oddball paradigm featuring the disyllable baba, which was stressed either on the first or second syllable (trochaic vs. iambic stress pattern. A group of age-matched normal hearing children participated as controls.Our results show, that within the first 6 months of implant use the implanted children develop a negative mismatch response for iambic but not

The Perception of Stress Pattern in Young Cochlear Implanted Children: An EEG Study.

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Vavatzanidis, Niki K; Mürbe, Dirk; Friederici, Angela D; Hahne, Anja

2016-01-01

Children with sensorineural hearing loss may (re)gain hearing with a cochlear implant-a device that transforms sounds into electric pulses and bypasses the dysfunctioning inner ear by stimulating the auditory nerve directly with an electrode array. Many implanted children master the acquisition of spoken language successfully, yet we still have little knowledge of the actual input they receive with the implant and specifically which language sensitive cues they hear. This would be important however, both for understanding the flexibility of the auditory system when presented with stimuli after a (life-) long phase of deprivation and for planning therapeutic intervention. In rhythmic languages the general stress pattern conveys important information about word boundaries. Infant language acquisition relies on such cues and can be severely hampered when this information is missing, as seen for dyslexic children and children with specific language impairment. Here we ask whether children with a cochlear implant perceive differences in stress patterns during their language acquisition phase and if they do, whether it is present directly following implant stimulation or if and how much time is needed for the auditory system to adapt to the new sensory modality. We performed a longitudinal ERP study, testing in bimonthly intervals the stress pattern perception of 17 young hearing impaired children (age range: 9-50 months; mean: 22 months) during their first 6 months of implant use. An additional session before the implantation served as control baseline. During a session they passively listened to an oddball paradigm featuring the disyllable "baba," which was stressed either on the first or second syllable (trochaic vs. iambic stress pattern). A group of age-matched normal hearing children participated as controls. Our results show, that within the first 6 months of implant use the implanted children develop a negative mismatch response for iambic but not for trochaic

Predicting social functioning in children with a cochlear implant and in normal-hearing children: the role of emotion regulation.

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Wiefferink, Carin H; Rieffe, Carolien; Ketelaar, Lizet; Frijns, Johan H M

2012-06-01

The purpose of the present study was to compare children with a cochlear implant and normal hearing children on aspects of emotion regulation (emotion expression and coping strategies) and social functioning (social competence and externalizing behaviors) and the relation between emotion regulation and social functioning. Participants were 69 children with cochlear implants (CI children) and 67 normal hearing children (NH children) aged 1.5-5 years. Parents answered questionnaires about their children's language skills, social functioning, and emotion regulation. Children also completed simple tasks to measure their emotion regulation abilities. Cochlear implant children had fewer adequate emotion regulation strategies and were less socially competent than normal hearing children. The parents of cochlear implant children did not report fewer externalizing behaviors than those of normal hearing children. While social competence in normal hearing children was strongly related to emotion regulation, cochlear implant children regulated their emotions in ways that were unrelated with social competence. On the other hand, emotion regulation explained externalizing behaviors better in cochlear implant children than in normal hearing children. While better language skills were related to higher social competence in both groups, they were related to fewer externalizing behaviors only in cochlear implant children. Our results indicate that cochlear implant children have less adequate emotion-regulation strategies and less social competence than normal hearing children. Since they received their implants relatively recently, they might eventually catch up with their hearing peers. Longitudinal studies should further explore the development of emotion regulation and social functioning in cochlear implant children. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Electrophysiological evidence for altered visual, but not auditory, selective attention in adolescent cochlear implant users.

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Harris, Jill; Kamke, Marc R

2014-11-01

Selective attention fundamentally alters sensory perception, but little is known about the functioning of attention in individuals who use a cochlear implant. This study aimed to investigate visual and auditory attention in adolescent cochlear implant users. Event related potentials were used to investigate the influence of attention on visual and auditory evoked potentials in six cochlear implant users and age-matched normally-hearing children. Participants were presented with streams of alternating visual and auditory stimuli in an oddball paradigm: each modality contained frequently presented 'standard' and infrequent 'deviant' stimuli. Across different blocks attention was directed to either the visual or auditory modality. For the visual stimuli attention boosted the early N1 potential, but this effect was larger for cochlear implant users. Attention was also associated with a later P3 component for the visual deviant stimulus, but there was no difference between groups in the later attention effects. For the auditory stimuli, attention was associated with a decrease in N1 latency as well as a robust P3 for the deviant tone. Importantly, there was no difference between groups in these auditory attention effects. The results suggest that basic mechanisms of auditory attention are largely normal in children who are proficient cochlear implant users, but that visual attention may be altered. Ultimately, a better understanding of how selective attention influences sensory perception in cochlear implant users will be important for optimising habilitation strategies. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Cochlear neuropathy in human presbycusis: Confocal analysis of hidden hearing loss in post-mortem tissue.

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Viana, Lucas M; O'Malley, Jennifer T; Burgess, Barbara J; Jones, Dianne D; Oliveira, Carlos A C P; Santos, Felipe; Merchant, Saumil N; Liberman, Leslie D; Liberman, M Charles

2015-09-01

Recent animal work has suggested that cochlear synapses are more vulnerable than hair cells in both noise-induced and age-related hearing loss. This synaptopathy is invisible in conventional histopathological analysis, because cochlear nerve cell bodies in the spiral ganglion survive for years, and synaptic analysis requires special immunostaining or serial-section electron microscopy. Here, we show that the same quadruple-immunostaining protocols that allow synaptic counts, hair cell counts, neuronal counts and differentiation of afferent and efferent fibers in mouse can be applied to human temporal bones, when harvested within 9 h post-mortem and prepared as dissected whole mounts of the sensory epithelium and osseous spiral lamina. Quantitative analysis of five "normal" ears, aged 54-89 yrs, without any history of otologic disease, suggests that cochlear synaptopathy and the degeneration of cochlear nerve peripheral axons, despite a near-normal hair cell population, may be an important component of human presbycusis. Although primary cochlear nerve degeneration is not expected to affect audiometric thresholds, it may be key to problems with hearing in noise that are characteristic of declining hearing abilities in the aging ear. Copyright © 2015 Elsevier B.V. All rights reserved.

Four siblings with distal renal tubular acidosis and nephrocalcinosis, neurobehavioral impairment, short stature, and distinctive facial appearance: a possible new autosomal recessive syndrome.

Science.gov (United States)

Faqeih, Eissa; Al-Akash, Samhar I; Sakati, Nadia; Teebi, Prof Ahmad S

2007-09-01

We report on four siblings (three males, one female) born to first cousin Arab parents with the constellation of distal renal tubular acidosis (RTA), small kidneys, nephrocalcinosis, neurobehavioral impairment, short stature, and distinctive facial features. They presented with early developmental delay with subsequent severe mental, behavioral and social impairment and autistic-like features. Their facial features are unique with prominent cheeks, well-defined philtrum, large bulbous nose, V-shaped upper lip border, full lower lip, open mouth with protruded tongue, and pits on the ear lobule. All had proteinuria, hypercalciuria, hypercalcemia, and normal anion-gap metabolic acidosis. Renal ultrasound examinations revealed small kidneys, with varying degrees of hyperechogenicity and nephrocalcinosis. Additional findings included dilated ventricles and cerebral demyelination on brain imaging studies. Other than distal RTA, common causes of nephrocalcinosis were excluded. The constellation of features in this family currently likely represents a possibly new autosomal recessive syndrome providing further evidence of heterogeneity of nephrocalcinosis syndromes. Copyright 2007 Wiley-Liss, Inc.

Standardization of reliability reporting for cochlear implants: an interim report.

Science.gov (United States)

Backous, Douglas D; Watson, Stacey D

2007-04-01

To propose a standard definition of "out of specification" for cochlear implants and a paradigm for inclusion of category C of the ISO standard 5841-2:2000 for reporting in cumulative survival statistics. A standard definition of "out of specification" and consistent reporting by manufacturers of cochlear implants will create a fair and consistent representation of cumulative survival. This will allow discernment of differences between manufacturers for reliability and for detection of trends in reliability between model types from the same manufacturer. Three separate meetings with representatives of the three manufacturers of cochlear implants marketed in the United States were staged over a 13-mo period. Standard questions, created by the authors, were addressed by each representative to determine the current state of device reliability reporting. Results were presented to clinicians at the William House Cochlear Implant study Group and the Implantable devices sub-committee of the American Academy of Otolaryngology (2004, 2005) and at the 8th International Cochlear Implant Conference (2004) for feedback. After assimilation of feedback by all parties, the standard was written and reviewed by representatives from each manufacturer for accuracy of data. A complaint-driven standard was developed. A "cochlear implant" as an internal device placed and skin closed in surgery. An internal device is "out of specification" when one or more technical characteristics is outside the limits of normal function and results in explantation or non-use by the patient." Children will be reported separately from adults, each model of device will be reported on annually, a minimum of 200 devices must be in each model group for Cumulative Survival Reporting (CSR). Confidence limits are set at 95%. Explants will be determined to be "biological" or "technical." Technical explants are included in CSR reports. Devices failing to meet specifications set by the manufacturer, not in use but

The mechanosensory structure of the hair cell requires clarin-1, a protein encoded by Usher syndrome III causative gene.

Science.gov (United States)

Geng, Ruishuang; Melki, Sami; Chen, Daniel H-C; Tian, Guilian; Furness, David N; Oshima-Takago, Tomoko; Neef, Jakob; Moser, Tobias; Askew, Charles; Horwitz, Geoff; Holt, Jeffrey R; Imanishi, Yoshikazu; Alagramam, Kumar N

2012-07-11

Mutation in the clarin-1 gene (Clrn1) results in loss of hearing and vision in humans (Usher syndrome III), but the role of clarin-1 in the sensory hair cells is unknown. Clarin-1 is predicted to be a four transmembrane domain protein similar to members of the tetraspanin family. Mice carrying null mutation in the clarin-1 gene (Clrn1(-/-)) show loss of hair cell function and a possible defect in ribbon synapse. We investigated the role of clarin-1 using various in vitro and in vivo approaches. We show by immunohistochemistry and patch-clamp recordings of Ca(2+) currents and membrane capacitance from inner hair cells that clarin-1 is not essential for formation or function of ribbon synapse. However, reduced cochlear microphonic potentials, FM1-43 [N-(3-triethylammoniumpropyl)-4-(4-(dibutylamino)styryl) pyridinium dibromide] loading, and transduction currents pointed to diminished cochlear hair bundle function in Clrn1(-/-) mice. Electron microscopy of cochlear hair cells revealed loss of some tall stereocilia and gaps in the v-shaped bundle, although tip links and staircase arrangement of stereocilia were not primarily affected by Clrn1(-/-) mutation. Human clarin-1 protein expressed in transfected mouse cochlear hair cells localized to the bundle; however, the pathogenic variant p.N48K failed to localize to the bundle. The mouse model generated to study the in vivo consequence of p.N48K in clarin-1 (Clrn1(N48K)) supports our in vitro and Clrn1(-/-) mouse data and the conclusion that CLRN1 is an essential hair bundle protein. Furthermore, the ear phenotype in the Clrn1(N48K) mouse suggests that it is a valuable model for ear disease in CLRN1(N48K), the most prevalent Usher syndrome III mutation in North America.

Modifying cochlear implant design: advantages of placing a return electrode in the modiolus.

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