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

Science.gov (United States)

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

2015-03-01

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

Small-Group Phonological Awareness Training for Pre-Kindergarten Children with Hearing Loss Who Wear Cochlear Implants and/or Hearing Aids

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Werfel, Krystal L.; Douglas, Michael; Ackal, Leigh

2016-01-01

This case report details a year-long phonological awareness (PA) intervention for pre-kindergarten children with hearing loss (CHL) who use listening and spoken language. All children wore cochlear implants and/or hearing aids. Intervention occurred for 15 min/day, 4 days per week across the pre-kindergarten school year and was delivered by…

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...

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

OpenAIRE

Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. F...

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...

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

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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.

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.

Cytomegalovirus (CMV) Infection Causes Degeneration of Cochlear Vasculature and Hearing Loss in a Mouse Model.

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Carraro, Mattia; Almishaal, Ali; Hillas, Elaine; Firpo, Matthew; Park, Albert; Harrison, Robert V

2017-04-01

Cytomegalovirus (CMV) infection is one of the most common causes of congenital hearing loss in children. We have used a murine model of CMV infection to reveal functional and structural cochlear pathogenesis. The cerebral cortex of Balb/c mice (Mus musculus) was inoculated with 2000 pfu (plaque forming units) of murine CMV on postnatal day 3. At 6 weeks of age, cochlear function was monitored using auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) measures. Histological assessment of cochlear vasculature using a corrosion cast technique was made at 8 weeks. Vascular casts of mCMV-damaged cochleas, and those of untreated control animals, were examined using scanning electron microscopy. We find very large variations in the degree of vascular damage in animals given identical viral injections (2000 pfu). The primary lesion caused by CMV infection is to the stria vascularis and to the adjacent spiral limbus capillary network. Capillary beds of the spiral ligament are generally less affected. The initial vascular damage is found in the mid-apical turn and appears to progress to more basal cochlear regions. After viral migration to the inner ear, the stria vascularis is the primary affected structure. We suggest that initial auditory threshold losses may relate to the poor development or maintenance of the endocochlear potential caused by strial dysfunction. Our increased understanding of the pathogenesis of CMV-related hearing loss is important for defining methods for early detection and treatment.

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

Science.gov (United States)

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 implantation in autistic children with profound sensorineural hearing loss.

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

2016-11-19

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

Progression of Unilateral Hearing Loss in Children With and Without Ipsilateral Cochlear Nerve Canal Stenosis: A Hazard Analysis.

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Purcell, Patricia L; Shinn, Justin R; Coggeshall, Scott S; Phillips, Grace; Paladin, Angelisa; Sie, Kathleen C Y; Horn, David L

2017-07-01

To investigate the risk of hearing loss progression in each ear among children with unilateral hearing loss associated with ipsilateral bony cochlear nerve canal (BCNC) stenosis. Tertiary pediatric referral center. Children diagnosed with unilateral hearing loss who had undergone temporal bone computed tomography imaging and had at least 6 months of follow-up audiometric testing were identified from a prospective audiological database. Two pediatric radiologists blinded to affected ear evaluated imaging for temporal bone anomalies and measured bony cochlear canal width independently. All available audiograms were reviewed, and air conduction thresholds were documented. Progression of hearing loss was defined by a 10 dB increase in air conduction pure-tone average. One hundred twenty eight children met inclusion criteria. Of these, 54 (42%) had a temporal bone anomaly, and 22 (17%) had ipsilateral BCNC stenosis. At 12 months, rates of progression in the ipsilateral ear were as follows: 12% among those without a temporal bone anomaly, 13% among those with a temporal bone anomaly, and 17% among those with BCNC stenosis. Children with BCNC stenosis had a significantly greater risk of progression in their ipsilateral ear compared with children with no stenosis: hazard ratio (HR) 2.17, 95% confidence interval (CI) (1.01, 4.66), p value 0.046. When we compared children with BCNC stenosis to those with normal temporal bone imaging, we found that the children with stenosis had nearly two times greater risk estimate for progression, but this difference did not reach significance, HR 1.9, CI (0.8, 4.3), p = 0.1. No children with BCNC stenosis developed hearing loss in their contralateral year by 12 months of follow-up. Children with bony cochlear nerve canal stenosis may be at increased risk for progression in their ipsilateral ear. Audiometric and medical follow-up for these children should be considered.

Beyond hearing : social-emotional outcomes following cochlear implantation in young children

NARCIS (Netherlands)

Ketelaar, Lizet

2014-01-01

In comparison to hearing children, children with hearing loss more often experience social-emotional problems. This thesis aimed to assess whether this was also true for 1-to-5-year-old children who had received a cochlear implant (CI) to remediate their hearing loss. In comparison to hearing peers,

The effect of cochlear implantation on tinnitus in patients with bilateral hearing loss : A systematic review

NARCIS (Netherlands)

Ramakers, Geerte G J; van Zon, Alice; Stegeman, Inge; Grolman, Wilko

2015-01-01

OBJECTIVES: To present an overview of the effect of cochlear implantation on tinnitus in adults with bilateral sensorineural hearing loss. DATA SOURCES: PubMed, Cochrane Library, CINAHL, and Embase databases were searched for articles from database inception up to January 13, 2015. METHODS: A

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

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

2017-01-01

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

Mechanisms of hearing loss in neurofibromatosis type 2.

Directory of Open Access Journals (Sweden)

Ashok R Asthagiri

Full Text Available Patients with neurofibromatosis type 2 (NF2 develop bilateral cochleovestibular schwannomas (CVSs that cause binaural deafness in most individuals. Hearing loss occurs in an unpredictable manner and the underlying mechanisms are not known. To gain insight into the pathophysiologic basis for hearing loss in NF2, we performed a prospective cross-sectional study of untreated ears in NF2 patients.One hundred consecutive NF2 patients in a prospective natural history study were included. Clinical and audiometric data were analyzed for treatment naïve ears. In addition to standard MR-imaging sequences, alterations in intralabyrinthine protein content were determined utilizing high resolution FLAIR, the presence of cochlear aperture obstruction was determined by examining 3D T2 sequences, and endolymphatic hydrops was identified on delayed post-contrast FLAIR sequences.Eighty-nine ears harboring 84 untreated CVSs in 56 consecutive NF2 patients (age 30 ± 16 years were analyzed. Thirty-four (38% ears had varying degrees of hearing loss. Elevated intralabyrinthine protein was identified in 70 (75% ears by FLAIR MR-imaging and was strongly associated with the presence of hearing loss (32/34 hearing loss ears; 94%(Fisher's exact test; P= .005. Elevated intralabyrinthine protein was associated with the presence of CVS-associated cochlear aperture obstruction (64 of 67 ears with elevated protein; 96%(Fisher's exact test; P<0.0001 in both normal and hearing loss ears. Elevated intralabyrinthine protein was not identified in ears without CVS (5 ears. While larger tumor size was associated with hearing loss (P=0.006, 16 hearing loss ears (47% harbored CVSs less than 0.5 cm(3, including 14 ears (88% with block of the cochlear aperture and elevated protein.These findings are consistent with a model in which hearing loss develops as a result of cochlear aperture obstruction and accumulation of intralabyrinthine protein. MRI based identification of elevated

Conductive Hearing Loss Has Long-Lasting Structural and Molecular Effects on Presynaptic and Postsynaptic Structures of Auditory Nerve Synapses in the Cochlear Nucleus.

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Clarkson, Cheryl; Antunes, Flora M; Rubio, Maria E

2016-09-28

Sound deprivation by conductive hearing loss increases hearing thresholds, but little is known about the response of the auditory brainstem during and after conductive hearing loss. Here, we show in young adult rats that 10 d of monaural conductive hearing loss (i.e., earplugging) leads to hearing deficits that persist after sound levels are restored. Hearing thresholds in response to clicks and frequencies higher than 8 kHz remain increased after a 10 d recovery period. Neural output from the cochlear nucleus measured at 10 dB above threshold is reduced and followed by an overcompensation at the level of the lateral lemniscus. We assessed whether structural and molecular substrates at auditory nerve (endbulb of Held) synapses in the cochlear nucleus could explain these long-lasting changes in hearing processing. During earplugging, vGluT1 expression in the presynaptic terminal decreased and synaptic vesicles were smaller. Together, there was an increase in postsynaptic density (PSD) thickness and an upregulation of GluA3 AMPA receptor subunits on bushy cells. After earplug removal and a 10 d recovery period, the density of synaptic vesicles increased, vesicles were also larger, and the PSD of endbulb synapses was larger and thicker. The upregulation of the GluA3 AMPAR subunit observed during earplugging was maintained after the recovery period. This suggests that GluA3 plays a role in plasticity in the cochlear nucleus. Our study demonstrates that sound deprivation has long-lasting alterations on structural and molecular presynaptic and postsynaptic components at the level of the first auditory nerve synapse in the auditory brainstem. Despite being the second most prevalent form of hearing loss, conductive hearing loss and its effects on central synapses have received relatively little attention. Here, we show that 10 d of monaural conductive hearing loss leads to an increase in hearing thresholds, to an increased central gain upstream of the cochlear nucleus at

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

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

2010-10-01

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

Hearing Preservation after Cochlear Implantation: UNICAMP Outcomes

Directory of Open Access Journals (Sweden)

Guilherme Machado de Carvalho

2013-01-01

Full Text Available Background. Electric-acoustic stimulation (EAS is an excellent choice for people with residual hearing in low frequencies but not high frequencies and who derive insufficient benefit from hearing aids. For EAS to be effective, subjects' residual hearing must be preserved during cochlear implant (CI surgery. Methods. We implanted 6 subjects with a CI. We used a special surgical technique and an electrode designed to be atraumatic. Subjects' rates of residual hearing preservation were measured 3 times postoperatively, lastly after at least a year of implant experience. Subjects' aided speech perception was tested pre- and postoperatively with a sentence test in quiet. Subjects' subjective responses assessed after a year of EAS or CI experience. Results. 4 subjects had total or partial residual hearing preservation; 2 subjects had total residual hearing loss. All subjects' hearing and speech perception benefited from cochlear implantation. CI diminished or eliminated tinnitus in all 4 subjects who had it preoperatively. 5 subjects reported great satisfaction with their new device. Conclusions. When we have more experience with our surgical technique we are confident we will be able to report increased rates of residual hearing preservation. Hopefully, our study will raise the profile of EAS in Brazil and Latin/South America.

Progressive age-dependence and frequency difference in the effect of gap junctions on active cochlear amplification and hearing.

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Zong, Liang; Chen, Jin; Zhu, Yan; Zhao, Hong-Bo

2017-07-22

Mutations of Connexin 26 (Cx26, GJB2), which is a predominant gap junction isoform in the cochlea, can induce high incidence of nonsyndromic hearing loss. We previously found that targeted-deletion of Cx26 in supporting Deiters cells and outer pillar cells in the cochlea can influence outer hair cell (OHC) electromotility and reduce active cochlear amplification leading to hearing loss, even though there are no gap junction connexin expressions in the auditory sensory hair cells. Here, we further report that hearing loss and the reduction of active amplification in the Cx26 targeted-deletion mice are progressive and different at high and low frequency regions, first occurring in the high frequency region and then progressively extending to the middle and low frequency regions with mouse age increased. The speed of hearing loss extending was fast in the basal high frequency region and slow in the apical low frequency region, showing a logarithmic function with mouse age. Before postnatal day 25, there were no significant hearing loss and the reduction of active cochlear amplification in the low frequency region. Hearing loss and the reduction of active cochlear amplification also had frequency difference, severe and large in the high frequency regions. These new data indicate that the effect of gap junction on active cochlear amplification is progressive, but, consistent with our previous report, exists in both high and low frequency regions in adulthood. These new data also suggest that cochlear gap junctions may have an important role in age-related hearing loss. Copyright © 2017 Elsevier Inc. All rights reserved.

Self-Esteem in Children and Adolescents With Hearing Loss

OpenAIRE

Warner-Czyz, Andrea D.; Loy, Betty A.; Evans, Christine; Wetsel, Ashton; Tobey, Emily A.

2015-01-01

Children with hearing loss are at risk for lower self-esteem due to differences from hearing peers relative to communication skills, physical appearance, and social maturity. This study examines the influence of generic factors unrelated to hearing loss (e.g., age, gender, temperament) and specific factors associated with hearing loss (e.g., age at identification, communication skills) on how children with hearing loss wearing cochlear implants or hearing aids appraise self-esteem. Fifty chil...

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...

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.

[Effect of rehabilitation for prelingual deaf children who use cochlear implants in conjunction with hearing aids in the opposite ears].

Science.gov (United States)

Tian, Yanjing; Zhou, Huifang; Zhang, Jing; Yang, Dong; Xu, Yi; Guo, Yuxi

2012-10-01

To compare the effect of rehabilitation of prelingual deaf children who used a cochlear implant (CI) in one ear and a hearing aids in the opposite ear while the hearing level of the opposite ears are different. Hearing ability, language ability and learning ability was included in the content. The aim of this research is to investigate better style of rehabilitation, and to offer the best help to the prelingual deaf children. Accord ing to the hearing level of the ear opposite to the one wearing a cochlear implant and whether the opposite ear wear a hearing aid or not, 30 prelingual deaf children were divided into three groups, including cochlear implant with opposite severe hearing loss and hearing aid ear (CI+SHA), cochlear implant with opposite profound hearing loss and hearing aid ear (CI+PHA), cochlear implant only (CI). The effect of rehabilitation was assessed in six different times (3,6,9,12,15 and 18 months after the cochlear implants and hearing aids began to work). The longer time the rehabilitation spends, the better the hearing ability,language ability and the learning ability were. The hearing ability of CI+SHA was better than those of CI+PHA (Pdeaf children should take much more time on rehabilitation. The effect of rehabilitation for prelingual deaf children who used cochlear implant in one ear and hearing aid in the other depend on the residual hearing level of the other ear. If a prelingual deaf children still has any residual hearing level in the ear opposite to the cochlear implant ear, it is better for him/her to wear a hearing aid in the ear.

Scalar position in cochlear implant surgery and outcome in residual hearing and the vestibular system.

Science.gov (United States)

Nordfalk, Karl Fredrik; Rasmussen, Kjell; Hopp, Einar; Greisiger, Ralf; Jablonski, Greg Eigner

2014-02-01

To evaluate the effect of the intracochlear electrode position on the residual hearing and VNG- and cVEMP responses. Prospective pilot study. Thirteen adult patients who underwent unilateral cochlear implant surgery were examined with high-resolution rotational tomography after cochlear implantation. All subjects were also tested with VNG, and 12 of the subjects were tested with cVEMP and audiometry before and after surgery. We found that although the electrode was originally planned to be positioned inside the scala tympani, only 8 of 13 had full insertion into the scala tympani. Loss of cVEMP response occurred to the same extent in the group with full scala tympani positioning and the group with scala vestibuli involvement. There was a non-significant difference in the loss of caloric response and residual hearing between the two groups. Interscalar dislocation of the electrode inside the cochlea was observed in two patients. A higher loss of residual hearing could be seen in the group with electrode dislocation between the scalae. Our findings indicate that intracochlear electrode dislocation is a possible cause to loss of residual hearing during cochlear implantation but cannot be the sole cause of postoperative vestibular loss.

Inter-trial coherence as a marker of cortical phase synchrony in children with sensorineural hearing loss and auditory neuropathy spectrum disorder fitted with hearing aids and cochlear implants

Science.gov (United States)

Nash-Kille, Amy; Sharma, Anu

2014-01-01

Objective Although brainstem dys-synchrony is a hallmark of children with auditory neuropathy spectrum disorder (ANSD), little is known about how the lack of neural synchrony manifests at more central levels. We used time-frequency single-trial EEG analyses (i.e., inter-trial coherence; ITC), to examine cortical phase synchrony in children with normal hearing (NH), sensorineural hearing loss (SNHL) and ANSD. Methods Single trial time-frequency analyses were performed on cortical auditory evoked responses from 41 NH children, 91 children with ANSD and 50 children with SNHL. The latter two groups included children who received intervention via hearing aids and cochlear implants. ITC measures were compared between groups as a function of hearing loss, intervention type, and cortical maturational status. Results In children with SNHL, ITC decreased as severity of hearing loss increased. Children with ANSD revealed lower levels of ITC relative to children with NH or SNHL, regardless of intervention. Children with ANSD who received cochlear implants showed significant improvements in ITC with increasing experience with their implants. Conclusions Cortical phase coherence is significantly reduced as a result of both severe-to-profound SNHL and ANSD. Significance ITC provides a window into the brain oscillations underlying the averaged cortical auditory evoked response. Our results provide a first description of deficits in cortical phase synchrony in children with SNHL and ANSD. PMID:24360131

Spiral ligament and stria vascularis changes in cochlear otosclerosis: effect on hearing level.

Science.gov (United States)

Doherty, Joni K; Linthicum, Fred H

2004-07-01

To investigate the effect of changes within the spiral ligament and stria vascularis on hearing in cochlear otosclerosis, we examined spiral ligament hyalinization, stria vascularis atrophy, and sensory hearing loss in cochlear otosclerosis and described changes in ion transport molecule expression. Retrospective. Tertiary referral center. Thirty-two cochleae from 24 temporal bone donors with histologic evidence of cochlear otosclerosis, including spiral ligament hyalinization. Audiography. Measurements of spiral ligament width, stria vascularis, and bone-conduction thresholds were compared by the amount of hyalinization. Expression of the ion transport molecules Na,K-ATPase, connexin 26, and carbonic anhydrase II were assessed by immunohistochemical techniques. Hyalinization most often involved the posterior basal turn (88%) and the posterior middle turn (27%). Spiral ligament hyalinization correlated significantly with stria vascularis atrophy in the posterior middle turn of the cochlea (rho = -0.63, p Bone-conduction thresholds at 2,000 and 4,000 Hz were significantly associated with the amount of stria vascularis atrophy (rho = -0.44, -0.40, p recycling, resulting in loss of endocochlear potential and sensory hearing loss.

Self-esteem in children and adolescents with hearing loss.

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Warner-Czyz, Andrea D; Loy, Betty A; Evans, Christine; Wetsel, Ashton; Tobey, Emily A

2015-03-09

Children with hearing loss are at risk for lower self-esteem due to differences from hearing peers relative to communication skills, physical appearance, and social maturity. This study examines the influence of generic factors unrelated to hearing loss (e.g., age, gender, temperament) and specific factors associated with hearing loss (e.g., age at identification, communication skills) on how children with hearing loss wearing cochlear implants or hearing aids appraise self-esteem. Fifty children with hearing loss wearing cochlear implants or hearing aids participated (Mean age: 12.88 years; mean duration of device use: 3.43 years). Participants independently completed online questionnaires to assess communication skills, social engagement, self-esteem, and temperament. Children with hearing loss rated global self-esteem significantly more positively than hearing peers, t = 2.38, p = .02. Self-esteem ratings attained significant positive correlations with affiliation (r = .42, p = .002) and attention (r = .45, p = .001) temperaments and a significant negative association with depressive mood (r = - .60, p self-esteem and demographic factors, communication skills, or social engagement. Because successful communication abilities do not always co-occur with excellent quality of life, clinicians and professionals working with children with hearing loss need to understand components contributing to self-esteem to improve identification, counseling, and external referrals for children in this population. © The Author(s) 2015.

Self-Esteem in Children and Adolescents With Hearing Loss

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Loy, Betty A.; Evans, Christine; Wetsel, Ashton; Tobey, Emily A.

2015-01-01

Children with hearing loss are at risk for lower self-esteem due to differences from hearing peers relative to communication skills, physical appearance, and social maturity. This study examines the influence of generic factors unrelated to hearing loss (e.g., age, gender, temperament) and specific factors associated with hearing loss (e.g., age at identification, communication skills) on how children with hearing loss wearing cochlear implants or hearing aids appraise self-esteem. Fifty children with hearing loss wearing cochlear implants or hearing aids participated (Mean age: 12.88 years; mean duration of device use: 3.43 years). Participants independently completed online questionnaires to assess communication skills, social engagement, self-esteem, and temperament. Children with hearing loss rated global self-esteem significantly more positively than hearing peers, t = 2.38, p = .02. Self-esteem ratings attained significant positive correlations with affiliation (r = .42, p = .002) and attention (r = .45, p = .001) temperaments and a significant negative association with depressive mood (r = − .60, p self-esteem and demographic factors, communication skills, or social engagement. Because successful communication abilities do not always co-occur with excellent quality of life, clinicians and professionals working with children with hearing loss need to understand components contributing to self-esteem to improve identification, counseling, and external referrals for children in this population. PMID:25755025

Establishment of a model of cochlear lesions in rats to study potential gene therapy for sensorineural hearing loss.

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Wang, Ye; Qiao, Li; Chen, Yang; Wen, Liting; Yue, Bo; Qiu, Jianhua; Wu, Shengxi

2015-12-01

Sensorineural hearing loss seriously influences a patient's daily life, and no effective treatments exist to date. Gene therapy is a potential treatment for regenerating hair cells to restore hearing. In this study, we established a cochlear lesions model to study hair cell regeneration by co-administration of kanamycin and furosemide. After the injections, we assessed the survival of outer hair cells (OHC), inner hair cells (IHC), supporting cells (SC), spiral ganglion neurons (SGN) and peripheral axons. Moreover, we used two viral vectors to detect the transgene distribution. Our results showed at 12h post-treatment, numerous OHC were missing in the basal turn. At 24h post-treatment, all OHCs in basal half of the cochlea were lost, and by 48h, OHC loss had spread to the apical coil. Four days after the injections, all OHCs were absent. At 1mo post-treatment, the organ of Corti had collapsed. In contrast, most of the SC remained 4d after the injections. The loss of SGN and peripheral axons was consistent with this time course post-treatment. The results of transgene distribution suggested the correlative gene can be transferred into the organ of Corti using adenoviruses (AdV) vectors and lentiviruses (LV) vectors in our cochlear lesion model. We assessed the details of HC death at more time point and chosen the time point for gene transfer in this model. We conclude that this cochlear lesion model would be suitable for the study of hair cell regeneration. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Self-Esteem in Children and Adolescents With Hearing Loss

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Andrea D. Warner-Czyz

2015-03-01

Full Text Available Children with hearing loss are at risk for lower self-esteem due to differences from hearing peers relative to communication skills, physical appearance, and social maturity. This study examines the influence of generic factors unrelated to hearing loss (e.g., age, gender, temperament and specific factors associated with hearing loss (e.g., age at identification, communication skills on how children with hearing loss wearing cochlear implants or hearing aids appraise self-esteem. Fifty children with hearing loss wearing cochlear implants or hearing aids participated (Mean age: 12.88 years; mean duration of device use: 3.43 years. Participants independently completed online questionnaires to assess communication skills, social engagement, self-esteem, and temperament. Children with hearing loss rated global self-esteem significantly more positively than hearing peers, t = 2.38, p = .02. Self-esteem ratings attained significant positive correlations with affiliation (r = .42, p = .002 and attention (r = .45, p = .001 temperaments and a significant negative association with depressive mood (r = − .60, p < .0001. No significant correlations emerged between self-esteem and demographic factors, communication skills, or social engagement. Because successful communication abilities do not always co-occur with excellent quality of life, clinicians and professionals working with children with hearing loss need to understand components contributing to self-esteem to improve identification, counseling, and external referrals for children in this population.

Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss

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Isabel Varela-Nieto

2017-04-01

Full Text Available Hearing loss (HL is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO. HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA, have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies.

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.

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

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Segal, Osnat; Kishon-Rabin, Liat

2017-12-20

The stressed word in a sentence (narrow focus [NF]) conveys information about the intent of the speaker and is therefore important for processing spoken language and in social interactions. The ability of participants with severe-to-profound prelingual hearing loss to comprehend NF has rarely been investigated. The purpose of this study was to assess the recognition and comprehension of NF by young adults with prelingual hearing loss compared with those of participants with normal hearing (NH). The participants included young adults with hearing aids (HA; n = 10), cochlear implants (CI; n = 12), and NH (n = 18). The test material included the Hebrew Narrow Focus Test (Segal, Kaplan, Patael, & Kishon-Rabin, in press), with 3 subtests, which was used to assess the recognition and comprehension of NF in different contexts. The following results were obtained: (a) CI and HA users successfully recognized the stressed word, with the worst performance for CI; (b) HA and CI comprehended NF less well than NH; and (c) the comprehension of NF was associated with verbal working memory and expressive vocabulary in CI users. Most CI and HA users were able to recognize the stressed word in a sentence but had considerable difficulty understanding it. Different factors may contribute to this difficulty, including the memory load during the task itself and linguistic and pragmatic abilities. https://doi.org/10.23641/asha.5572792.

[Strategy for minimally invasive cochlear implantation and residual hearing preservation].

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Huang, Y Y; Chen, J Y; Shen, M; Yang, J

2018-01-07

In the past few decades, considerable development was achieved in the cochlear implantation following the emergence of innovative electrode array and advances in minimally invasive surgery. Minimally invasive technique led to a better preservation of residual low-frequency hearing. The loss of residual hearing was caused by complicated factors. According to previous studies, a slower and stable speed of electrode insertion and the use of perioperative steroids were demonstrated to have a positive impact on hearing preservation. The selection of electrode array or its insertion approaches didn't show any distinctive benefits in hearing preservation.

Transforming growth factor β1 inhibition protects from noise-induced hearing loss

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Silvia eMurillo-Cuesta

2015-03-01

Full Text Available Excessive exposure to noise damages the principal cochlear structures leading to hearing impairment. Inflammatory and immune responses are central mechanisms in cochlear defensive response to noise but, if unregulated, they contribute to inner ear damage and hearing loss. Transforming growth factor ß (TGF-ß is a key regulator of both responses and high levels of this factor have been associated with cochlear injury in hearing loss animal models. To evaluate the potential of targeting TGF-ß as a therapeutic strategy for preventing or ameliorating noise-induced hearing loss, we studied the auditory function, cochlear morphology, gene expression and oxidative stress markers in mice exposed to noise and treated with TGF-ß1 peptidic inhibitors P17 and P144, just before or immediately after noise insult. Our results indicate that systemic administration of both peptides significantly improved both the evolution of hearing thresholds and the degenerative changes induced by noise-exposure in lateral wall structures. Moreover, treatments ameliorated the inflammatory state and redox balance. These therapeutic effects were dose-dependent and more effective if the TGF-ß1 inhibitors were administered prior to inducing the injury. In conclusion, inhibition of TGF-ß1 actions with antagonistic peptides represents a new, promising therapeutic strategy for the prevention and repair of noise-induced cochlear damage.

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.

Systemic steroid reduces long-term hearing loss in experimental pneumococcal meningitis

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Worsøe, Lise Lotte; Brandt, C.T.; Lund, S.P.

2010-01-01

Sensorineural hearing loss is a common complication of pneumococcal meningitis. Treatment with corticosteroids reduces inflammatory response and may thereby reduce hearing loss. However, both experimental studies and clinical trials investigating the effect of corticosteroids on hearing loss have...... generated conflicting results. The objective of the present study was to determine whether systemic steroid treatment had an effect on hearing loss and cochlear damage in a rat model of pneumococcal meningitis....

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

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Jepsen, Morten Løve; Dau, Torsten

2011-01-01

–438 (2008)] was used as a framework. The parameters of the cochlear processing stage of the model were adjusted to account for behaviorally estimated individual basilar-membrane inputoutput functions and the audiogram, from which the amounts of inner hair-cell and outer hair-cell losses were estimated......This study considered consequences of sensorineural hearing loss in ten listeners. The characterization of individual hearing loss was based on psychoacoustic data addressing audiometric pure-tone sensitivity, cochlear compression, frequency selectivity, temporal resolution, and intensity...

Clinical Application of Screening for GJB2 Mutations before Cochlear Implantation in a Heterogeneous Population with High Rate of Autosomal Recessive Nonsyndromic Hearing Loss

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Masoud Motasaddi Zarandy

2011-01-01

Full Text Available Clinical application of mutation screening and its effect on the outcome of cochlear implantation is widely debated. We investigated the effect of mutations in GJB2 gene on the outcome of cochlear implantation in a population with a high rate of consanguineous marriage and autosomal recessive nonsyndromic hearing loss. Two hundred and one children with profound prelingual sensorineural hearing loss were included. Forty-six patients had 35delG in GJB2. Speech awareness thresholds (SATs and speech recognition thresholds (SRTs improved following implantation, but there was no difference in performance between patients with GJB2-related deafness versus control (all >0.10. Both groups had produced their first comprehensible words within the same period of time following implantation (2.27 months in GJB2-related deaf versus 2.62 months in controls, =0.22. Although our findings demonstrate the need to uncover unidentified genetic causes of hereditary deafness, they do not support the current policy for genetic screening before cochlear implantation, nor prove a prognostic value.

Gd enhanced MRI in sensorineural hearing loss

International Nuclear Information System (INIS)

Takenaka, Mika; Tono, Tetsuya; Toyama, Katsuhiro; Kano, Kiyo; Morimitsu, Tamotsu

1996-01-01

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

Systemic steroid reduces long-term hearing loss in experimental pneumococcal meningitis

DEFF Research Database (Denmark)

Worsøe, Lise Lotte; Brandt, C.T.; Lund, S.P.

2010-01-01

Sensorineural hearing loss is a common complication of pneumococcal meningitis. Treatment with corticosteroids reduces inflammatory response and may thereby reduce hearing loss. However, both experimental studies and clinical trials investigating the effect of corticosteroids on hearing loss have...... generated conflicting results. The objective of the present study was to determine whether systemic steroid treatment had an effect on hearing loss and cochlear damage in a rat model of pneumococcal meningitis.......Sensorineural hearing loss is a common complication of pneumococcal meningitis. Treatment with corticosteroids reduces inflammatory response and may thereby reduce hearing loss. However, both experimental studies and clinical trials investigating the effect of corticosteroids on hearing loss have...

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

Science.gov (United States)

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

2015-12-01

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

Toward a Differential Diagnosis of Hidden Hearing Loss in Humans.

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M Charles Liberman

Full Text Available Recent work suggests that hair cells are not the most vulnerable elements in the inner ear; rather, it is the synapses between hair cells and cochlear nerve terminals that degenerate first in the aging or noise-exposed ear. This primary neural degeneration does not affect hearing thresholds, but likely contributes to problems understanding speech in difficult listening environments, and may be important in the generation of tinnitus and/or hyperacusis. To look for signs of cochlear synaptopathy in humans, we recruited college students and divided them into low-risk and high-risk groups based on self-report of noise exposure and use of hearing protection. Cochlear function was assessed by otoacoustic emissions and click-evoked electrocochleography; hearing was assessed by behavioral audiometry and word recognition with or without noise or time compression and reverberation. Both groups had normal thresholds at standard audiometric frequencies, however, the high-risk group showed significant threshold elevation at high frequencies (10-16 kHz, consistent with early stages of noise damage. Electrocochleography showed a significant difference in the ratio between the waveform peaks generated by hair cells (Summating Potential; SP vs. cochlear neurons (Action Potential; AP, i.e. the SP/AP ratio, consistent with selective neural loss. The high-risk group also showed significantly poorer performance on word recognition in noise or with time compression and reverberation, and reported heightened reactions to sound consistent with hyperacusis. These results suggest that the SP/AP ratio may be useful in the diagnosis of "hidden hearing loss" and that, as suggested by animal models, the noise-induced loss of cochlear nerve synapses leads to deficits in hearing abilities in difficult listening situations, despite the presence of normal thresholds at standard audiometric frequencies.

Age-related Hearing Impairment and the Triad of Acquired Hearing Loss

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Chao-Hui eYang

2015-07-01

Full Text Available Understanding underlying pathological mechanisms is prerequisite for a sensible design of protective therapies against hearing loss. The triad of age-related, noise-generated, and drug-induced hearing loss ¬¬displays intriguing similarities in some cellular responses of cochlear sensory cells such as a potential involvement of reactive oxygen species and apoptotic and necrotic cell death. On the other hand, detailed studies have revealed that molecular pathways are considerably complex and, importantly, it has become clear that pharmacological protection successful against one form of hearing loss will not necessarily protect against another. This review will summarize pathological and pathophysiological features of age-related hearing impairment (ARHI in human and animal models and address selected aspects of the commonality (or lack thereof of cellular responses in ARHI to drugs and noise.

Persistent conductive or mixed hearing loss after the placement of tympanostomy tubes.

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Whittemore, Kenneth R; Dornan, Briana K; Lally, Tara; Dargie, Jenna M

2012-10-01

Described is a case series of clinical findings in children with persistent conductive or mixed hearing loss following tympanostomy tube placement for serous otitis media. Retrospective chart review. Tertiary pediatric hospital. Medical records of thirty-nine children who were referred for either conductive or mixed hearing loss post-tympanostomy tube placement were reviewed for clinical histories, physical examinations, audiological evaluations, diagnostic studies, consultations, and surgical findings. Approval was obtained from the Boston Children's Hospital Institutional Review Board. Causes of hearing loss included ossicular abnormalities, cochlear abnormalities, 'third window' effects, cholesteatomas, genetic syndromes, and unknown causes. In four patients with isolated mild low-frequency conductive hearing loss, the cause was the presence of functional tubes. All patients diagnosed with a genetic syndrome had bilateral hearing loss. Patients with mixed hearing loss were diagnosed with cochlear abnormalities, 'third window' effects, or genetic syndromes. Computed tomography led to diagnosis in sixteen of twenty-five patients. Vestibular-evoked myogenic potential testing suggested a diagnosis in three of four patients. In children with persistent hearing loss following tympanostomy tube placement, identifying the laterality and type of hearing loss appears to be of importance in diagnosis. Patients with bilateral hearing loss should be considered for genetic testing, given the possibility of a syndrome. Patients identified with a mixed hearing loss should be evaluated for inner ear anomalies. Patients with mild, low-frequency hearing losses should be monitored audiologically and investigated further only if the hearing loss progresses and/or there is no resolution following tube extrusion. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Hearing loss and enlarged internal auditory canal in children.

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

2014-01-01

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

Cochlear implantation in children with unilateral hearing loss: A systematic review.

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Peters, Jeroen P M; Ramakers, Geerte G J; Smit, Adriana L; Grolman, Wilko

2016-03-01

To systematically review the literature on cochlear implantation (CI) for children with unilateral hearing loss (UHL). PubMed, Cochrane, CINAHL, and Embase databases were searched for articles up to June 29, 2015 for UHL, children and CI, and all of their synonyms. After screening of titles, abstracts, and full texts for eligible articles, directness of evidence (DoE) and risk of bias (RoB) were assessed for the included articles. Study characteristics and data on our outcomes of interest (speech perception in noise, sound localization, quality of life, and speech and language development) were extracted. In total, 296 unique articles were retrieved, of which five articles satisfied the eligibility criteria. All of these articles were case series or case reports and had a low to moderate DoE and a high RoB. In these studies, heterogeneous findings were reported in small patient samples. Speech perception in noise and localization ability improved in most patients. Although only measured in one study each, quality of life and speech and language development improved. Most of these results were not statistically significant. No firm conclusions can be drawn on the effectiveness of CI in children with UHL, due to heterogeneous findings, small sample sizes, and the lack of high Level of Evidence studies. Based on the findings of this systematic review, cochlear implantation may be an effective treatment option in children with UHL. Laryngoscope, 126:713-721, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Drug-induced Defibrinogenation as New Treatment Approach of Acute Hearing Loss in an Animal Model for Inner Ear Vascular Impairment.

Science.gov (United States)

Weiss, Bernhard G; Bertlich, Mattis; Bettag, Stephan A; Desinger, Hendrik; Ihler, Friedrich; Canis, Martin

2017-06-01

Disturbance of cochlear microcirculation is considered to be the final common pathway of various inner ear diseases. Hyperfibrinogenemia causing increased plasma viscosity is a known risk factor for sudden sensorineural hearing loss and may lead to a critical reduction of cochlear blood flow. The aim of this study was to evaluate the effect of a substantial reduction of plasma fibrinogen levels by drug-induced defibrinogenation for the treatment of acute hearing loss in vivo. Acute hearing loss was induced by hyperfibrinogenemia (i.v. injection of 330 mg/kg BW fibrinogen), using a guinea pig animal model. Parameters of cochlear microcirculation and hearing thresholds were quantified by intravital microscopy and evoked response audiometry. After obtaining baseline values, the course of hearing loss and disturbances of microcirculation were investigated under influence of intravenous defibrinogenation therapy (ancrod), corticosteroid, or placebo treatment, using 5 animals/group. Acute hyperfibrinogenemia caused hearing loss from 10 ± 7 to 26 ± 10 dB SPL at baseline. Drug-induced reduction of fibrinogen levels showed a significant increase of cochlear microcirculation (1.6-fold) and recovered hearing threshold (11 ± 6 dB SPL). Placebo or corticosteroid treatment had no effect on hearing loss (35 ± 7 dB SPL and 32 ± 18 dB SPL, respectively). Acute hyperfibrinogenemia resulted in hearing loss. Drug-induced reduction of elevated fibrinogen levels caused an increase in cochlear blood flow and a decrease in hearing thresholds. Placebo or corticosteroid treatment had no effect. Reduction of plasma fibrinogen levels could serve as a clinical treatment option for acute hearing loss.

Disrupted bone remodeling leads to cochlear overgrowth and hearing loss in a mouse model of fibrous dysplasia.

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Omar Akil

Full Text Available Normal hearing requires exquisite cooperation between bony and sensorineural structures within the cochlea. For example, the inner ear secretes proteins such as osteoprotegrin (OPG that can prevent cochlear bone remodeling. Accordingly, diseases that affect bone regulation can also result in hearing loss. Patients with fibrous dysplasia develop trabecular bone overgrowth resulting in hearing loss if the lesions affect the temporal bones. Unfortunately, the mechanisms responsible for this hearing loss, which could be sensorineural and/or conductive, remain unclear. In this study, we used a unique transgenic mouse model of increased Gs G-protein coupled receptor (GPCR signaling induced by expression of an engineered receptor, Rs1, in osteoblastic cells. These ColI(2.3+/Rs1+ mice showed dramatic bone lesions that histologically and radiologically resembled fibrous dysplasia. We found that ColI(2.3+/Rs1+ mice showed progressive and severe conductive hearing loss. Ossicular chain impingement increased with the size and number of dysplastic lesions. While sensorineural structures were unaffected, ColI(2.3+/Rs1+ cochleae had abnormally high osteoclast activity, together with elevated tartrate resistant acid phosphatase (TRAP activity and receptor activator of nuclear factor kappa-B ligand (Rankl mRNA expression. ColI(2.3+/Rs1+ cochleae also showed decreased expression of Sclerostin (Sost, an antagonist of the Wnt signaling pathway that normally increases bone formation. The osteocyte canalicular networks of ColI(2.3+/Rs1+ cochleae were disrupted and showed abnormal osteocyte morphology. The osteocytes in the ColI(2.3+/Rs1+ cochleae showed increased expression of matrix metalloproteinase 13 (MMP-13 and TRAP, both of which can support osteocyte-mediated peri-lacunar remodeling. Thus, while the ossicular chain impingement is sufficient to account for the progressive hearing loss in fibrous dysplasia, the deregulation of bone remodeling extends to the

Vowel production of Mandarin-speaking hearing aid users with different types of hearing loss.

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Yu-Chen Hung

Full Text Available In contrast with previous research focusing on cochlear implants, this study examined the speech performance of hearing aid users with conductive (n = 11, mixed (n = 10, and sensorineural hearing loss (n = 7 and compared it with the speech of hearing control. Speech intelligibility was evaluated by computing the vowel space area defined by the Mandarin Chinese corner vowels /a, u, i/. The acoustic differences between the vowels were assessed using the Euclidean distance. The results revealed that both the conductive and mixed hearing loss groups exhibited a reduced vowel working space, but no significant difference was found between the sensorineural hearing loss and normal hearing groups. An analysis using the Euclidean distance further showed that the compression of vowel space area in conductive hearing loss can be attributed to the substantial lowering of the second formant of /i/. The differences in vowel production between groups are discussed in terms of the occlusion effect and the signal transmission media of various hearing devices.

Swept-sine noise-induced damage as a hearing loss model for preclinical assays

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Lorena eSanz

2015-02-01

Full Text Available Mouse models are key tools for studying cochlear alterations in noise-induced hearing loss and for evaluating new therapies. Stimuli used to induce deafness in mice are usually white and octave band noises that include very low frequencies, considering the large mouse auditory range. We designed different sound stimuli, enriched in frequencies up to 20 kHz (violet noises to examine their impact on hearing thresholds and cochlear cytoarchitecture after short exposure. In addition, we developed a cytocochleogram to quantitatively assess the ensuing structural degeneration and its functional correlation. Finally, we used this mouse model and cochleogram procedure to evaluate the potential therapeutic effect of transforming growth factor β1 inhibitors P17 and P144 on noise-induced hearing loss. CBA mice were exposed to violet swept-sine noise with different frequency ranges (2-20 or 9-13 kHz and levels (105 or 120 dB SPL for 30 minutes. Mice were evaluated by auditory brainstem response and otoacoustic emission tests prior to and 2, 14 and 28 days after noise exposure. Cochlear pathology was assessed with gross histology; hair cell number was estimated by a stereological counting method. Our results indicate that functional and morphological changes induced by violet swept-sine noise depend on the sound level and frequency composition. Partial hearing recovery followed the exposure to 105 dB SPL, whereas permanent cochlear damage resulted from the exposure to 120 dB SPL. Exposure to 9-13 kHz noise caused an auditory threshold shift in those frequencies that correlated with hair cell loss in the corresponding areas of the cochlea that were spotted on the cytocochleogram. In summary, we present mouse models of noise-induced hearing loss, which depending on the sound properties of the noise, cause different degrees of cochlear damage, and could therefore be used to study molecules which are potential players in hearing loss protection and repair.

Autism and peripheral hearing loss: a systematic review.

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Beers, Alison N; McBoyle, Melanie; Kakande, Emily; Dar Santos, Rachelle C; Kozak, Frederick K

2014-01-01

To systematically review the literature describing the relationship between autism spectrum disorder (ASD) and peripheral hearing loss including literature recommendations for audiological assessment and auditory habilitation in cases where peripheral hearing loss and ASD coexist. Published studies indexed in MEDLINE (1948-2011). The search strategy identified 595 potential studies. After a review of the titles, 115 abstracts were reviewed and 39 articles were retrieved and assessed independently by at least two authors for possible inclusion. 22 articles pertained to children with ASD and peripheral hearing loss, hearing assessment in children with ASD, audiological habilitation for children with ASD or hyper-responsiveness in children with ASD. 17 further studies were garnered from the reference section of the 22 papers. Controversy exists in the literature regarding prevalence of hearing impairment among individuals with ASD. In cases where ASD and hearing impairment co-exist, diagnosis of one condition often leads to a delay in diagnosing the other. Audiological assessment can be difficult in children with ASD and test-retest reliability of behavioural thresholds can be poor. In cases where hearing impairment exists and hearing aids or cochlear implantation are recommended, devices are often fit with special considerations for the child with ASD. Hyper-responsiveness to auditory stimuli may be displayed by individuals with ASD. Evidence or the suspicion of hyper-responsiveness may be taken into consideration when fitting amplification and planning behavioural intervention. Prevalence rates of hearing impairment among individuals with ASD continue to be debated. At present there is no conclusive evidence that children with ASD are at increased risk of peripheral hearing loss. A complete audiological assessment is recommended in all cases where ASD is suspected so as not to delay the diagnosis of hearing impairment in the event that hearing loss and ASD co

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

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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.

Renexin as a rescue regimen for noise-induced hearing loss

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So Young Park

2014-01-01

Full Text Available Renexin, a compound of cilostazol and ginkgo biloba extract, has been reported to produce neuroprotective effects through antioxidant, antiplatelet, and vasodilatory mechanisms. This study was designed to investigate the protective effects of renexin on hearing, the organ of Corti (OC, and medial olivocochlear efferents against noise-induced damage. C57BL/6 mice were exposed to 110 dB SPL white noise for 60 min and then randomly divided into three groups: high- and low-dose renexin-treated groups and noise only group. Renexin were administered for 7 days: 90 mg/kg to the low-dose, and 180 mg/kg to the high-dose groups. All mice, including the controls underwent hearing tests on postnoise day 8 and were killed for cochlear harvest. We compared the hearing thresholds and morphology of the OC and cochlear efferents across the groups. The renexin-treated groups recovered from the immediate threshold shifts in a dose-dependent manner, while the noise group showed a permanent hearing loss. The renexin-treated ears demonstrated less degeneration of the OC. The diameters of the efferent terminals labeled with α-synuclein were preserved in the high-dose renexin-treated group. In the western blot assay of the cochlear homogenates, the treated groups displayed stronger expressions of α-synuclein than the noise and control groups, which may indicate that noise-induced enhanced activity of the cochlear efferent system was protected by renexin. Our results suggest that pharmacologic treatment with renexin is hopeful to reduce or prevent noise-induced hearing loss as a rescue regimen after noise exposure.

Curing hearing loss: Patient expectations, health care practitioners, and basic science.

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Oshima, Kazuo; Suchert, Steffen; Blevins, Nikolas H; Heller, Stefan

2010-01-01

Millions of patients are debilitated by hearing loss, mainly caused by degeneration of sensory hair cells in the cochlea. The underlying reasons for hair cell loss are highly diverse, ranging from genetic disposition, drug side effects, traumatic noise exposure, to the effects of aging. Whereas modern hearing aids offer some relief of the symptoms of mild hearing loss, the only viable option for patients suffering from profound hearing loss is the cochlear implant. Despite their successes, hearing aids and cochlear implants are not perfect. Particularly frequency discrimination and performance in noisy environments and general efficacy of the devises vary among individual patients. The advent of regenerative medicine, the publicity of stem cells and gene therapy, and recent scientific achievements in inner ear cell regeneration have generated an emerging spirit of optimism among scientists, health care practitioners, and patients. In this review, we place the different points of view of these three groups in perspective with the goal of providing an assessment of patient expectations, health care reality, and potential future treatment options for hearing disorders. (1) Readers will be encouraged to put themselves in the position of a hearing impaired patient or family member of a hearing impaired person. (2) Readers will be able to explain why diagnosis of the underlying pathology of hearing loss is difficult. (3) Readers will be able to list the main directions of current research aimed to cure hearing loss. (4) Readers will be able to understand the different viewpoints of patients and their relatives, health care providers, and scientists with respect to finding novel treatments for hearing loss. Copyright 2010 Elsevier Inc. All rights reserved.

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

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Augusto Peñaranda

2012-04-01

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

Current amplification models of sensorineurall and conductive hearing loss

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Ostojić Sanja

2012-01-01

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

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.

Experience Changes How Emotion in Music Is Judged: Evidence from Children Listening with Bilateral Cochlear Implants, Bimodal Devices, and Normal Hearing.

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Sara Giannantonio

Full Text Available Children using unilateral cochlear implants abnormally rely on tempo rather than mode cues to distinguish whether a musical piece is happy or sad. This led us to question how this judgment is affected by the type of experience in early auditory development. We hypothesized that judgments of the emotional content of music would vary by the type and duration of access to sound in early life due to deafness, altered perception of musical cues through new ways of using auditory prostheses bilaterally, and formal music training during childhood. Seventy-five participants completed the Montreal Emotion Identification Test. Thirty-three had normal hearing (aged 6.6 to 40.0 years and 42 children had hearing loss and used bilateral auditory prostheses (31 bilaterally implanted and 11 unilaterally implanted with contralateral hearing aid use. Reaction time and accuracy were measured. Accurate judgment of emotion in music was achieved across ages and musical experience. Musical training accentuated the reliance on mode cues which developed with age in the normal hearing group. Degrading pitch cues through cochlear implant-mediated hearing induced greater reliance on tempo cues, but mode cues grew in salience when at least partial acoustic information was available through some residual hearing in the contralateral ear. Finally, when pitch cues were experimentally distorted to represent cochlear implant hearing, individuals with normal hearing (including those with musical training switched to an abnormal dependence on tempo cues. The data indicate that, in a western culture, access to acoustic hearing in early life promotes a preference for mode rather than tempo cues which is enhanced by musical training. The challenge to these preferred strategies during cochlear implant hearing (simulated and real, regardless of musical training, suggests that access to pitch cues for children with hearing loss must be improved by preservation of residual hearing and

Experience Changes How Emotion in Music Is Judged: Evidence from Children Listening with Bilateral Cochlear Implants, Bimodal Devices, and Normal Hearing

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Papsin, Blake C.; Paludetti, Gaetano; Gordon, Karen A.

2015-01-01

Children using unilateral cochlear implants abnormally rely on tempo rather than mode cues to distinguish whether a musical piece is happy or sad. This led us to question how this judgment is affected by the type of experience in early auditory development. We hypothesized that judgments of the emotional content of music would vary by the type and duration of access to sound in early life due to deafness, altered perception of musical cues through new ways of using auditory prostheses bilaterally, and formal music training during childhood. Seventy-five participants completed the Montreal Emotion Identification Test. Thirty-three had normal hearing (aged 6.6 to 40.0 years) and 42 children had hearing loss and used bilateral auditory prostheses (31 bilaterally implanted and 11 unilaterally implanted with contralateral hearing aid use). Reaction time and accuracy were measured. Accurate judgment of emotion in music was achieved across ages and musical experience. Musical training accentuated the reliance on mode cues which developed with age in the normal hearing group. Degrading pitch cues through cochlear implant-mediated hearing induced greater reliance on tempo cues, but mode cues grew in salience when at least partial acoustic information was available through some residual hearing in the contralateral ear. Finally, when pitch cues were experimentally distorted to represent cochlear implant hearing, individuals with normal hearing (including those with musical training) switched to an abnormal dependence on tempo cues. The data indicate that, in a western culture, access to acoustic hearing in early life promotes a preference for mode rather than tempo cues which is enhanced by musical training. The challenge to these preferred strategies during cochlear implant hearing (simulated and real), regardless of musical training, suggests that access to pitch cues for children with hearing loss must be improved by preservation of residual hearing and improvements in

A Micro-Ethnographic Study of the Communication/Language Development in a Japanese Child with Profound Hearing Loss before and after Cochlear Implantation

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Kretschmer, Richard R.; Kretschmer, Laura; Kuwahara, Katsura; Truax, Roberta

2010-01-01

This study described the communication and spoken language development of a Japanese girl with profound hearing loss who used a cochlear implant from 19 months of age. The girl, Akiko, was born in Belgium where her family was living at that time. After she was identified as deaf at birth, she and her parents were provided with support services.…

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

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

2015-01-01

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

Coarticulation in Early Vocalizations by Children with Hearing Loss: A Locus Perspective

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Morrison, Helen Mccaffrey

2012-01-01

Locus equations derived from productions by three children with hearing loss revealed sensory and motor influences on anticipatory coarticulation. Participants who received auditory access to speech via hearing aids and cochlear implants at different ages (5-39 months) were recorded at approximately 6 and 12 months after hearing technology…

Hearing preservation cochlear implantation in children: The HEARRING Group consensus and practice guide.

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Rajan, Gunesh; Tavora-Vieira, Dayse; Baumgartner, Wolf-Dieter; Godey, Benoit; Müller, Joachim; O'Driscoll, Martin; Skarzynski, Henryk; Skarzynski, Piotr; Usami, Shin-Ichi; Adunka, Oliver; Agrawal, Sumit; Bruce, Iain; De Bodt, Marc; Caversaccio, Marco; Pilsbury, Harold; Gavilán, Javier; Hagen, Rudolf; Hagr, Abdulrahman; Kameswaran, Mohan; Karltorp, Eva; Kompis, Martin; Kuzovkov, Vlad; Lassaletta, Luis; Yongxin, Li; Lorens, Artur; Manoj, Manikoth; Martin, Jane; Mertens, Griet; Mlynski, Robert; Parnes, Lorne; Pulibalathingal, Sasidharan; Radeloff, Andreas; Raine, Christopher H; Rajeswaran, Ranjith; Schmutzhard, Joachim; Sprinzl, Georg; Staecker, Hinrich; Stephan, Kurt; Sugarova, Serafima; Zernotti, Mario; Zorowka, Patrick; Van de Heyning, Paul

2018-01-01

To provide multidisciplinary cochlear implant teams with a current consensus statement to support hearing preservation cochlear implantation (HPCI) in children, including those children with symptomatic partial deafness (PD) where the intention is to use electric-acoustic stimulation (EAS). The main objectives are to provide guidelines on who is a candidate, how to assess these children and when to implant if Med-El Flex electrode arrays are chosen for implantation. The HEARRING group reviewed the current evidence and practice regarding the management of children to be considered for HPCI surgery emphasizing the assessment needed prior to implantation in order to demonstrate the benefits in these children over time. The consensus statement addresses following three key questions: (1) Should these children be treated? (2) How to identify these children? (3) How to manage these children? The HEARRING group concludes that irrespective of the degree of residual hearing present, the concepts of hearing and structure preservation should be applied in every child undergoing cochlear implantation and that HPCI is a safe and reliable treatment option. Early detection and multidisciplinary assessment are key to the identification of children with symptomatic PD, these children should undergo HPCI as early as possible.

The significance of a hypoplastic bony canal for the cochlear nerve in patients with sensorineural hearing loss: CT and MRI findings

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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

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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.

A guinea pig model of selective severe high-frequency hearing loss.

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Havenith, Sarah; Klis, Sjaak F L; Versnel, Huib; Grolman, Wilko

2013-10-01

Using an appropriate dose of an aminoglycoside antibiotic cotreated with a loop diuretic a guinea pig model of high-frequency loss can be obtained mimicking cochlear implant candidates with low-frequency residual hearing. We examined the stability of this model over time. A well-established method to create an animal model for profound deafness is cotreatment with an aminoglycoside antibiotic and a loop diuretic. Recent data indicated that reduction of the aminoglycoside dose might yield selective high-frequency hearing loss. Such a model is relevant for studies related to hybrid cochlear implant devices, for example, with respect to preservation of residual hearing. Guinea pigs received an electrode for chronic recording of compound action potentials to tones to assess thresholds. They were treated with a coadministration of kanamycin (200 mg/kg) and furosemide (100 mg/kg), after which, the animals were sacrificed for histologic analysis at 2, 4, or 7 weeks. After 2 to 7 weeks threshold shifts were greater than 50 dB for 8 to 16 kHz in 15 of 17 animals, whereas threshold shifts at 2 kHz or lower were less than 50 dB in 13 animals. Major threshold shifts occurred the first 2 to 4 days; subsequently, some spontaneous recovery occurred and, after 2-3 weeks thresholds, remained stable. Inner hair cell loss still progressed between 2 and 4 weeks in the most basal cochlear region; thereafter, hair cell loss was stable. An appropriate animal model for selective severe high-frequency hearing loss was obtained, which is stable at 4 weeks after ototoxic treatment.

Recognition of "real-world" musical excerpts by cochlear implant recipients and normal-hearing adults.

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Gfeller, Kate; Olszewski, Carol; Rychener, Marly; Sena, Kimberly; Knutson, John F; Witt, Shelley; Macpherson, Beth

2005-06-01

The purposes of this study were (a) to compare recognition of "real-world" music excerpts by postlingually deafened adults using cochlear implants and normal-hearing adults; (b) to compare the performance of cochlear implant recipients using different devices and processing strategies; and (c) to examine the variability among implant recipients in recognition of musical selections in relation to performance on speech perception tests, performance on cognitive tests, and demographic variables. Seventy-nine cochlear implant users and 30 normal-hearing adults were tested on open-set recognition of systematically selected excerpts from musical recordings heard in real life. The recognition accuracy of the two groups was compared for three musical genre: classical, country, and pop. Recognition accuracy was correlated with speech recognition scores, cognitive measures, and demographic measures, including musical background. Cochlear implant recipients were significantly less accurate in recognition of previously familiar (known before hearing loss) musical excerpts than normal-hearing adults (p genre. Implant recipients were most accurate in the recognition of country items and least accurate in the recognition of classical items. There were no significant differences among implant recipients due to implant type (Nucleus, Clarion, or Ineraid), or programming strategy (SPEAK, CIS, or ACE). For cochlear implant recipients, correlations between melody recognition and other measures were moderate to weak in strength; those with statistically significant correlations included age at time of testing (negatively correlated), performance on selected speech perception tests, and the amount of focused music listening following implantation. Current-day cochlear implants are not effective in transmitting several key structural features (i.e., pitch, harmony, timbral blends) of music essential to open-set recognition of well-known musical selections. Consequently, implant

Computed tomographic features of the bony canal of the cochlear nerve in pediatric patients with unilateral sensorineural hearing loss

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Kono, Tatsuo

2008-01-01

The aim of this study was to evaluate the diameters of the various bony canals of the inner ear in patients with sensorineural hearing loss (SNHL) and establish criteria for detecting hypoplasia of the bony canal of the cochlear nerve. Measurements obtained in 118 patients without inner ear malformations among 160 patients with unilateral SNHL were analyzed. The diameters of the internal auditory canal and the bony canals of the cochlear, vestibular, and facial nerves were measured on transverse or coronal computed tomographic images. Mean values (±standard deviation (SD)) were compared between the affected and unaffected ears, and statistical analysis was done. The diameter of the bony canal of the cochlear nerve was significantly smaller in affected ears than in unaffected ears (P<0.01). The affected ears could be divided into groups with (72 ears) and without (46 ears) bony canal stenosis. Most (60%) of the patients with unilateral SNHL showed a significant difference in the diameters of the bony canals of the cochlear nerve between the affected and unaffected sides; moreover, the mean value was significantly smaller in affected ears. The diameter of <1.7 mm on transverse images or <1.8 mm on coronal images suggests hypoplasia. (author)

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

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

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.

Assessing Speech Intelligibility in Children with Hearing Loss: Toward Revitalizing a Valuable Clinical Tool

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Ertmer, David J.

2011-01-01

Background: Newborn hearing screening, early intervention programs, and advancements in cochlear implant and hearing aid technology have greatly increased opportunities for children with hearing loss to become intelligible talkers. Optimizing speech intelligibility requires that progress be monitored closely. Although direct assessment of…

New Criteria of Indication and Selection of Patients to Cochlear Implant

Directory of Open Access Journals (Sweden)

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.

Dual Language versus English-Only Support for Bilingual Children with Hearing Loss Who Use Cochlear Implants and Hearing Aids

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Bunta, Ferenc; Douglas, Michael; Dickson, Hanna; Cantu, Amy; Wickesberg, Jennifer; Gifford, René H.

2016-01-01

Background: There is a critical need to understand better speech and language development in bilingual children learning two spoken languages who use cochlear implants (CIs) and hearing aids (HAs). The paucity of knowledge in this area poses a significant barrier to providing maximal communicative outcomes to a growing number of children who have…

Hearing loss in enlarged vestibular aqueduct and incomplete partition type II.

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Ahadizadeh, Emily; Ascha, Mustafa; Manzoor, Nauman; Gupta, Amit; Semaan, Maroun; Megerian, Cliff; Otteson, Todd

The purpose of this work is to identify the role of incomplete partition type II on hearing loss among patients with enlarged vestibular aqueduct (EVA). EVA is a common congenital inner ear malformation among children with hearing loss, where vestibular aqueduct morphology in this population has been shown to correlate to hearing loss. However, the impact of incomplete partition between cochlear turns on hearing loss has not been, despite meaningful implications for EVA pathophysiology. A retrospective review of radiology reports for patients who had computed tomography (CT) scans with diagnoses of hearing loss at a tertiary medical center between January 2000 and June 2016 were screened for EVA. CT scans of the internal auditory canal (IAC) for those patients with EVA were examined for evidence of incomplete partition type II (IP-II), measurements of midpoint width and operculum width a second time, and patients meeting Cincinnati criteria for EVA selected for analysis. Statistical analysis including chi-square, Wilcoxon rank-sum, and t-tests were used to identify differences in outcomes and clinical predictors, as appropriate for the distribution of the data. Linear mixed models of hearing test results for all available tests were constructed, both univariable and adjusting for vestibular aqueduct morphometric features, with ear-specific intercepts and slopes over time. There were no statistically significant differences in any hearing test results or vestibular aqueduct midpoint and operculum widths. Linear mixed models, both univariable and those adjusting for midpoint and operculum widths, did not indicate a statistically significant effect of incomplete partition type II on hearing test results. Hearing loss due to enlarged vestibular aqueduct does not appear to be affected by the presence of incomplete partition type II. Our results suggest that the pathophysiological processes underlying hearing loss in enlarged vestibular aqueduct may not be a result of

Auditory brainstem responses of CBA/J mice with neonatal conductive hearing losses and treatment with GM1 ganglioside.

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Money, M K; Pippin, G W; Weaver, K E; Kirsch, J P; Webster, D B

1995-07-01

Exogenous administration of GM1 ganglioside to CBA/J mice with a neonatal conductive hearing loss ameliorates the atrophy of spiral ganglion neurons, ventral cochlear nucleus neurons, and ventral cochlear nucleus volume. The present investigation demonstrates the extent of a conductive loss caused by atresia and tests the hypothesis that GM1 ganglioside treatment will ameliorate the conductive hearing loss. Auditory brainstem responses were recorded from four groups of seven mice each: two groups received daily subcutaneous injections of saline (one group had normal hearing; the other had a conductive hearing loss); the other two groups received daily subcutaneous injections of GM1 ganglioside (one group had normal hearing; the other had a conductive hearing loss). In mice with a conductive loss, decreases in hearing sensitivity were greatest at high frequencies. The decreases were determined by comparing mean ABR thresholds of the conductive loss mice with those of normal hearing mice. The conductive hearing loss induced in the mice in this study was similar to that seen in humans with congenital aural atresias. GM1 ganglioside treatment had no significant effect on ABR wave I thresholds or latencies in either group.

Musical hallucination associated with hearing loss.

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Sanchez, Tanit Ganz; Rocha, Savya Cybelle Milhomem; Knobel, Keila Alessandra Baraldi; Kii, Márcia Akemi; Santos, Rosa Maria Rodrigues dos; Pereira, Cristiana Borges

2011-01-01

In spite of the fact that musical hallucination have a significant impact on patients' lives, they have received very little attention of experts. Some researchers agree on a combination of peripheral and central dysfunctions as the mechanism that causes hallucination. The most accepted physiopathology of musical hallucination associated to hearing loss (caused by cochlear lesion, cochlear nerve lesion or by interruption of mesencephalon or pontine auditory information) is the disinhibition of auditory memory circuits due to sensory deprivation. Concerning the cortical area involved in musical hallucination, there is evidence that the excitatory mechanism of the superior temporal gyrus, as in epilepsies, is responsible for musical hallucination. In musical release hallucination there is also activation of the auditory association cortex. Finally, considering the laterality, functional studies with musical perception and imagery in normal individuals showed that songs with words cause bilateral temporal activation and melodies activate only the right lobe. The effect of hearing aids on the improvement of musical hallucination as a result of the hearing loss improvement is well documented. It happens because auditory hallucination may be influenced by the external acoustical environment. Neuroleptics, antidepressants and anticonvulsants have been used in the treatment of musical hallucination. Cases of improvement with the administration of carbamazepine, meclobemide and donepezil were reported, but the results obtained were not consistent.

Effects of musical training and hearing loss on pitch discrimination

DEFF Research Database (Denmark)

Santurette, Sébastien; Bianchi, Federica; Dau, Torsten

2018-01-01

content of the sound and whether the harmonics are resolved by the auditory frequency analysis operated by cochlear processing. F0DLs are also heavily influenced by the amount of musical training received by the listener and by the spectrotemporal auditory processing deficits that often accompany...... sensorineural hearing loss. This paper reviews the latest evidence for how musical training and hearing loss affect pitch discrimination performance, based on behavioral F0DL experiments with complex tones containing either resolved or unresolved harmonics, carried out in listeners with different degrees...... of hearing loss and musicianship. A better understanding of the interaction between these two factors is crucial to determine whether auditory training based on musical tasks or targeted towards specific auditory cues may be useful to hearing-impaired patients undergoing hearing rehabilitation....

Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

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Pillion, Joseph P.; Vernick, David; Shapiro, Jay

2011-01-01

Osteogenesis imperfecta (OI) is the most common heritable disorder of connective tissue. It is associated with fractures following relatively minor injury, blue sclerae, dentinogenesis imperfecta, increased joint mobility, short stature, and hearing loss. Structures in the otic capsule and inner ear share in the histologic features common to other skeletal tissues. OI is due to mutations involving several genes, the most commonly involved are the COL1A1 or COL1A2 genes which are responsible for the synthesis of the proalpha-1 and proalpha-2 polypeptide chains that form the type I collagen triple helix. A genotype/phenotype relationship to hearing loss has not been established in OI. Hearing loss is commonly found in OI with prevalence rates ranging from 50 to 92% in some studies. Hearing loss in OI may be conductive, mixed, or sensorineural and is more common by the second or third decade. Treatment options such as hearing aids, stapes surgery, and cochlear implants are discussed. PMID:22567374

The cochlear implant and possibilities for narrowing the remaining gaps between prosthetic and normal hearing

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Blake S. Wilson

2017-12-01

Full Text Available Background: The cochlear implant has become the standard of care for severe or worse losses in hearing and indeed has produced the first substantial restoration of a lost or absent human sense using a medical intervention. However, the devices are not perfect and many efforts to narrow the remaining gaps between prosthetic and normal hearing are underway. Objective: To assess the present status of cochlear implants and to describe possibilities for improving them. Results: The present-day devices work well in quiet conditions for the great majority of users. However, not all users have high levels of speech reception in quiet and nearly all users struggle with speech reception in typically noisy acoustic environments. In addition, perception of sounds more complex than speech, such as most music, is generally poor unless residual hearing at low frequencies can be stimulated acoustically in conjunction with the electrical stimuli provided by the implant. Possibilities for improving the present devices include increasing the spatial specificity of neural excitation by reducing masking effects or with new stimulus modes; prudent pruning of interfering or otherwise detrimental electrodes from the stimulation map; a further relaxation in the criteria for implant candidacy, based on recent evidence from persons with high levels of residual hearing and to allow many more people to benefit from cochlear implants; and “top down” or “brain centric” approaches to implant designs and applications. Conclusions: Progress in the development of the cochlear implant and related treatments has been remarkable but room remains for improvements. The future looks bright as there are multiple promising possibilities for improvements and many talented teams are pursuing them. Keywords: Auditory prosthesis, Cochlear implant, Cochlear prosthesis, Deafness, Neural prosthesis

Hearing Preservation Outcomes With a Mid-Scala Electrode in Cochlear Implantation.

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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.

Children using Cochlear Implants Capitalize on Acoustical Hearing for Music Perception

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Talar eHopyan

2012-10-01

Full Text Available Cochlear implants (CIs electrically stimulate the auditory nerve providing children who are deaf with access to speech and music. Because of device limitations, it was hypothesized that children using CIs develop abnormal perception of musical cues. Perception of pitch and rhythm as well as memory for music was measured by the children’s version of the Montreal Battery of Amusia (MBEA in 23 unilateral CI users and 22 age-matched children with normal hearing. Children with CIs were less accurate than their normal hearing peers (p<0.05. CI users were best able to discern rhythm changes (p < .01 and to remember musical pieces (p < .01. Contrary to expectations, abilities to hear cues in music improved as the age at implantation increased (p < .01. Further analyses revealed that this was because the children implanted at older ages also had better low frequency hearing prior to cochlear implantation and were able to use this hearing prior to cochlear implantation by wearing hearing aids. Access to early acoustical hearing in the lower frequency ranges appears to establish a base for music perception, which can be accessed with later electrical CI hearing.

Use of Otoacoustic emissions in noise induced hearing loss prevention

CSIR Research Space (South Africa)

Edwards, A

2010-10-01

Full Text Available in laboratory- main clinical use on identification of hearing loss in newborn babies • Clinically sensitive tool for assessing NIHL and the outer hair cells (OHC) • repeatable results • identify cochlear damage before evidenced on an audiogram – normal... audiogram but evidence of OHC loss • Feasible method of evaluating HPD effectiveness using temporary emission shift (TES) • CSIR research developed a prediction model for Hearing Threshold Levels Copy of dancing hair cell.wm © CSIR 2010 Slide 6...

Delayed changes in auditory status in cochlear implant users with preserved acoustic hearing.

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Scheperle, Rachel A; Tejani, Viral D; Omtvedt, Julia K; Brown, Carolyn J; Abbas, Paul J; Hansen, Marlan R; Gantz, Bruce J; Oleson, Jacob J; Ozanne, Marie V

2017-07-01

This retrospective review explores delayed-onset hearing loss in 85 individuals receiving cochlear implants designed to preserve acoustic hearing at the University of Iowa Hospitals and Clinics between 2001 and 2015. Repeated measures of unaided behavioral audiometric thresholds, electrode impedance, and electrically evoked compound action potential (ECAP) amplitude growth functions were used to characterize longitudinal changes in auditory status. Participants were grouped into two primary categories according to changes in unaided behavioral thresholds: (1) stable hearing or symmetrical hearing loss and (2) delayed loss of hearing in the implanted ear. Thirty-eight percent of this sample presented with delayed-onset hearing loss of various degrees and rates of change. Neither array type nor insertion approach (round window or cochleostomy) had a significant effect on prevalence. Electrode impedance increased abruptly for many individuals exhibiting precipitous hearing loss; the increase was often transient. The impedance increases were significantly larger than the impedance changes observed for individuals with stable or symmetrical hearing loss. Moreover, the impedance changes were associated with changes in behavioral thresholds for individuals with a precipitous drop in behavioral thresholds. These findings suggest a change in the electrode environment coincident with the change in auditory status. Changes in ECAP thresholds, growth function slopes, and suprathreshold amplitudes were not correlated with changes in behavioral thresholds, suggesting that neural responsiveness in the region excited by the implant is relatively stable. Further exploration into etiology of delayed-onset hearing loss post implantation is needed, with particular interest in mechanisms associated with changes in the intracochlear environment. Copyright © 2017 Elsevier B.V. All rights reserved.

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.

The current status of audiologic rehabilitation for profound unilateral sensorineural hearing loss.

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Bishop, Charles E; Eby, Thomas L

2010-03-01

Audiologic rehabilitation of individuals with profound unilateral sensorineural hearing loss (USNHL) has traditionally been limited to the use of air-conduction contralateral routing of sound (CROS) hearing aids. Treatment for these individuals has expanded with new applications of the bone-anchored hearing aid (BAHA), transcranial hearing aid (t-CROS), and the cochlear implant. In this article, the authors review the literature that addresses these various treatment options. Contemporary review Historical information is available that describes the limited efficacy of air-conduction CROS hearing aids in lifting hearing handicap associated with USNHL. Current investigations on providing cross hearing are generally focused on use of the BAHA. Little is known at present whether new developments in hearing aid technology can improve on conventional air-conduction CROS or t-CROS approaches. Interestingly, the cochlear implant seems to be a viable option for individuals with USNHL and tinnitus who also have intact auditory nerve pathways. There is indication in the literature that BAHA provides greater relief of hearing handicap associated with USNHL than CROS hearing aids; however, both have been found to provide limited patient satisfaction and seemingly fall short of restoring true sound localization. Adequate trials have not been performed comparing BAHA with the best CROS hearing aid technology. Transcranial hearing aids and cochlear implants are experimental methods to treat USNHL and hold promise, although there remains a lack of studies available to fully support this.

Classification of Hearing Loss Disorders Using Teoae-Based Descriptors

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Hatzopoulos, Stavros Dimitris

Transiently Evoked Otoacoustic Emissions (TEOAE) are signals produced by the cochlea upon stimulation by an acoustic click. Within the context of this dissertation, it was hypothesized that the relationship between the TEOAEs and the functional status of the OHCs provided an opportunity for designing a TEOAE-based clinical procedure that could be used to assess cochlear function. To understand the nature of the TEOAE signals in the time and the frequency domain several different analyses were performed. Using normative Input-Output (IO) curves, short-time FFT analyses and cochlear computer simulations, it was found that for optimization of the hearing loss classification it is necessary to use a complete 20 ms TEOAE segment. It was also determined that various 2-D filtering methods (median and averaging filtering masks, LP-FFT) used to enhance of the TEOAE S/N offered minimal improvement (less than 6 dB per stimulus level). Higher S/N improvements resulted in TEOAE sequences that were over-smoothed. The final classification algorithm was based on a statistical analysis of raw FFT data and when applied to a sample set of clinically obtained TEOAE recordings (from 56 normal and 66 hearing-loss subjects) correctly identified 94.3% of the normal and 90% of the hearing loss subjects, at the 80 dB SPL stimulus level. To enhance the discrimination between the conductive and the sensorineural populations, data from the 68 dB SPL stimulus level were used, which yielded a normal classification of 90.2%, a hearing loss classification of 87.5% and a conductive-sensorineural classification of 87%. Among the hearing-loss populations the best discrimination was obtained in the group of otosclerosis and the worst in the group of acute acoustic trauma.

Weighting of Acoustic Cues to a Manner Distinction by Children with and without Hearing Loss

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Nittrouer, Susan; Lowenstein, Joanna H.

2015-01-01

Purpose: Children must develop optimal perceptual weighting strategies for processing speech in their first language. Hearing loss can interfere with that development, especially if cochlear implants are required. The three goals of this study were to measure, for children with and without hearing loss: (a) cue weighting for a manner distinction,…

Cellular mechanisms of noise-induced hearing loss.

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Kurabi, Arwa; Keithley, Elizabeth M; Housley, Gary D; Ryan, Allen F; Wong, Ann C-Y

2017-06-01

Exposure to intense sound or noise can result in purely temporary threshold shift (TTS), or leave a residual permanent threshold shift (PTS) along with alterations in growth functions of auditory nerve output. Recent research has revealed a number of mechanisms that contribute to noise-induced hearing loss (NIHL). The principle cause of NIHL is damage to cochlear hair cells and associated synaptopathy. Contributions to TTS include reversible damage to hair cell (HC) stereocilia or synapses, while moderate TTS reflects protective purinergic hearing adaptation. PTS represents permanent damage to or loss of HCs and synapses. While the substrates of HC damage are complex, they include the accumulation of reactive oxygen species and the active stimulation of intracellular stress pathways, leading to programmed and/or necrotic cell death. Permanent damage to cochlear neurons can also contribute to the effects of NIHL, in addition to HC damage. These mechanisms have translational potential for pharmacological intervention and provide multiple opportunities to prevent HC damage or to rescue HCs and spiral ganglion neurons that have suffered injury. This paper reviews advances in our understanding of cellular mechanisms that contribute to NIHL and their potential for therapeutic manipulation. Published by Elsevier B.V.

Is All Human Hearing Cochlear?

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Seyede Faranak Emami

2013-01-01

Full Text Available The objective of this cross-sectional study was to investigate the possibility that the saccule may contribute to human hearing. The forty participants included twenty healthy people and twenty other subjects selected from patients who presented with benign paroxysmal positional vertigo to Audiology Department of Hazrat Rasoul Akram hospital (Tehran, Iran. Assessments comprised of audiological evaluations, cervical vestibular evoked myogenic potentials (cVEMPs, recognition of spoken phonemes in white noise (Rsp in wn, and auditory brainstem response to 500 Hz tone burst (ABR500 HZ. Twenty affected ears with decreased vestibular excitability as detected by abnormal cVEMPs revealed decreased scores of Rsp in wn and abnormal findings of ABR500 HZ. Both unaffected and normal ears had normal results. Multiple comparisons of mean values of cVEMPs and ABR500 HZ between three groups were significant (P<0.05, ANOVA. The correlation between RSP in wn and p13 latencies was significant. The peak-to-peak amplitudes showed significant correlation to RSP in wn. The correlation between RSP in wn and the latencies of n23 was significant. In high-level of noisy competing situations, healthy human saccular sensation can mediate the detection of low frequencies and possibly help in cochlear hearing for frequency and intensity discrimination. So, all human hearing is not cochlear.

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.

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.

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.

[Hearing loss and idoneity--the segnalation of noise-induced hearing loss hearing Loss].

Science.gov (United States)

Albera, Roberto; Dagna, Federico; Cassandro, Claudia; Canale, Andrea

2011-01-01

Work idoneity in hearing loss must be related to working ability and evolution risks. Working ability is referred to the difficulties found in speech comprehension and in signals perception. As regards hearing loss evolution it is necessary to define if the subject is affected by conductive or neurosensorial hearing loss. In conductive hearing loss it is necessary to evaluate entity and frequential distribution of the deficit. In neurosensorial hearing loss it is necessary to distinguish between noise-induced hearing loss and extraprofessional hearing loss. In noise-induced hearing loss the evolution risk is high if the noise exposure is less than 10-15 years or the actual noise exposure is louder than the former. In case of extraprofessional hearing loss the evolution risk is higher in presbycusis, endolymphatic hydrops and toxic hearing loss. The necessity to report the presence on professionale noise-induced hearing loss arises if audiometric threshold is more than 25 dB at 0.5-1-2-3-4 kHz and if it is verified the professional origine of hearing loss.

Mouse Models for Pendrin-Associated Loss of Cochlear and Vestibular Function

Directory of Open Access Journals (Sweden)

Philine Wangemann

2013-12-01

Full Text Available The human gene SLC26A4 and the mouse ortholog Slc26a4 code for the protein pendrin, which is an anion exchanger expressed in apical membranes of selected epithelia. In the inner ear, pendrin is expressed in the cochlea, the vestibular labyrinth and the endolymphatic sac. Loss-of-function and hypo-functional mutations cause an enlargement of the vestibular aqueduct (EVA and sensorineural hearing loss. The relatively high prevalence of SLC26A4 mutations provides a strong imperative to develop rational interventions that delay, ameliorate or prevent pendrin-associated loss of cochlear and vestibular function. This review summarizes recent studies in mouse models that have been developed to delineate the role of pendrin in the physiology of hearing and balance and that have brought forward the concept that a temporally and spatially limited therapy may be sufficient to secure a life-time of normal hearing in children bearing mutations of SLC26A4.

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.

Presbycusis: a human temporal bone study of individuals with downward sloping audiometric patterns of hearing loss and review of the literature.

Science.gov (United States)

Nelson, Erik G; Hinojosa, Raul

2006-09-01

The purpose of this retrospective case review was to identify patterns of cochlear element degeneration in individuals with presbycusis exhibiting downward sloping audiometric patterns of hearing loss and to correlate these findings with those reported in the literature to clarify conflicting concepts regarding the association between hearing loss and morphologic abnormalities. Archival human temporal bones from individuals with presbycusis were selected on the basis of strict audiometric criteria for downward-sloping audiometric thresholds. Twenty-one temporal bones that met these criteria were identified and compared with 10 temporal bones from individuals with normal hearing. The stria vascularis volumes, spiral ganglion cell populations, inner hair cells, and outer hair cells were quantitatively evaluated. The relationship between the severity of hearing loss and the degeneration of cochlear elements was analyzed using univariate linear regression models. Outer hair cell loss and ganglion cell loss was observed in all individuals with presbycusis. Inner hair cell loss was observed in 18 of the 21 individuals with presbycusis and stria vascularis loss was observed in 10 of the 21 individuals with presbycusis. The extent of degeneration of all four of the cochlear elements evaluated was highly associated with the severity of hearing loss based on audiometric thresholds at 8,000 Hz and the pure-tone average at 500, 1,000, and 2,000 Hz. The extent of ganglion cell degeneration was associated with the slope of the audiogram. Individuals with downward-sloping audiometric patterns of presbycusis exhibit degeneration of the stria vascularis, spiral ganglion cells, inner hair cells, and outer hair cells that is associated with the severity of hearing loss. This association has not been previously reported in studies that did not use quantitative methodologies for evaluating the cochlear elements and strict audiometric criteria for selecting cases.

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.

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...

Otolithic organ function in patients with profound sensorineural hearing loss

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Yujuan Zhou

2016-06-01

Full Text Available Profound sensorineural hearing loss (PSHL is not uncommonly encountered in otology. In clinics, there is a high incidence of otolithic damage in patients with PSHL, but relevant reports are few. Sharing a continuous membranous structure and similar receptor cell ultrastructures, the cochlea and vestibule may be susceptible to the same harmful factors. Disorders of the inner ear may result in a variety of manifestations, including vertigo, spatial disorientation, blurred vision, impaired articulation, and hearing impairment. Considering the diversity of clinical symptoms associated with PSHL with otolithic dysfunction, it may be frequently misdiagnosed, and objective means of testing the function of otolithic organs should be recommended for hearing-impaired patients. Vestibular-evoked myogenic potentials (VEMPs via air-conducted sound are of great importance for the diagnosis of otolithic function. Hearing devices such as cochlear implants are commonly accepted treatments for PSHL, and early identification and treatment of vestibular disorders may increase the success rate of cochlear implantation. Therefore, it is necessary to increase awareness of otolithic functional states in patients with PSHL.

Revisiting the relationship of three-dimensional fluid attenuation inversion recovery imaging and hearing outcomes in adults with idiopathic unilateral sudden sensorineural hearing loss

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Liao, Wen-Huei [School of Medicine, National Yang Ming University, Taipei, 11221, Taiwan (China); Department of Otolaryngology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Wu, Hsiu-Mei [School of Medicine, National Yang Ming University, Taipei, 11221, Taiwan (China); Department of Radiology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Wu, Hung-Yi [Department of Radiology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Tu, Tzong-Yang; Shiao, An-Suey [School of Medicine, National Yang Ming University, Taipei, 11221, Taiwan (China); Department of Otolaryngology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Castillo, Mauricio [Department of Radiology, University of North Carolina, Chapel Hill, NC, 27599-7510 (United States); Hung, Sheng-Che, E-mail: hsz829@gmail.com [School of Medicine, National Yang Ming University, Taipei, 11221, Taiwan (China); Department of Radiology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Department of Biomedical Imaging and Radiological Sciences, National Yang Ming University, Taipei, 11221, Taiwan (China)

2016-12-15

Background and purpose: Three-dimensional fluid attenuation inversion recovery (3D FLAIR) may demonstrate high signal in the inner ears of patients with idiopathic sudden sensorineural hearing loss (ISSNHL), but the correlations of this finding with outcomes are still controversial. Here we compared 4 3D MRI sequences with the outcomes of patients with ISSNHL. Materials and methods: 77 adult patients with ISSNHL underwent MRI with pre contrast FLAIR, fast imaging employing steady-state acquisition images (FIESTA-C), post contrast T1WI and post contrast FLAIR. The extent and degree of high signal in both cochleas were evaluated in all patients, and asymmetry ratios between the affected ears and the normal ones were calculated. The relationships among MRI findings, including extent and asymmetry of abnormal cochlear high signals, degree of FLAIR enhancement, and clinical information, including age, vestibular symptoms, baseline hearing loss, and final hearing outcomes were analyzed. Results: 54 patients (28 men; age, 52.1 ± 15.5 years) were included in our study. Asymmetric cochlear signal intensities were more frequently observed in pre contrast and post contrast FLAIR (79.6% and 68.5%) than in FIESTA-C (61.1%) and T1WI (51.9%) (p < 0.001). Age, baseline hearing loss, extent of high signal and asymmetry ratios of pre contrast and post contrast FLAIR were all correlated with final hearing outcomes. In multivariate analysis, age and the extent of high signals were the most significant predictors of final hearing outcomes. Conclusion: 3D FLAIR provides a higher sensitivity in detecting the asymmetric cochlear signal abnormality. The more asymmetric FLAIR signals and presence of high signals beyond cochlea indicated a poorer prognosis.

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...

Recent advances in the study of age-related hearing loss - A Mini-Review

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Kidd, Ambrose R; Bao, Jianxin

2013-01-01

Hearing loss is a common age-associated affliction that can result from the loss of hair cells and spiral ganglion neurons (SGNs) in the cochlea. Although hair cells and SGNs are typically lost in the same cochlea, recent analysis suggests that they can occur independently, via unique mechanisms. Research has identified both environmental and genetic factors that contribute to degeneration of cochlear cells. Additionally, molecular analysis has identified multiple cell signaling mechanisms that likely contribute to pathological changes that result in hearing deficiencies. These analyses should serve as useful primers for future work, including genomic and proteomic analysis, to elucidate the mechanisms driving cell loss in the aging cochlea. Significant progress in this field has occurred in the past decade. As our understanding of aging-induced cochlear changes continues to improve, our ability to offer medical intervention will surely benefit the growing elderly population. PMID:22710288

Auditory Brainstem Responses to Bone-Conducted Brief Tones in Young Children with Conductive or Sensorineural Hearing Loss

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Jennifer L. Hatton

2012-01-01

Full Text Available The bone-conduction (BC tone ABR has been used clinically for over 20 years. The current study formally evaluated the test performance of the BC tone-evoked ABR in infants with hearing loss. Method. By comparing BC-ABR results to follow-up behavioural results, this study addressed two questions: (i whether the BC tone ABR was successful in differentiating children with conductive versus sensorineural hearing loss (Study A; conductive: 68 ears; SNHL: 129 ears and (ii the relationship between BC ABR and behavioural hearing loss severity (Study B: 2000 Hz: 104 ears; 500 Hz: 47 ears. Results. Results demonstrate that the “normal” BC-ABR levels accurately differentiated normal versus elevated cochlear sensitivity (accuracy: 98% for 2000 Hz; 98% for 500 Hz. A subset of infants in Study A with elevated BC-ABR (i.e., no response at normal level had additional testing at higher intensities, which allowed for categorization of the degree of cochlear impairment. Study B results indicate that the BC ABR accurately categorizes the degree of cochlear hearing loss for 2000 Hz (accuracy = 95.2%. A preliminary dBnHL-to-dBHL correction factor of “0 dB” was determined for 2000 Hz BC ABR. Conclusions. These findings further support the use of BC tone ABR for diagnostic ABR testing.

Auditory Brainstem Responses to Bone-Conducted Brief Tones in Young Children with Conductive or Sensorineural Hearing Loss

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Hatton, Jennifer L.; Janssen, Renée M.; Stapells, David R.

2012-01-01

The bone-conduction (BC) tone ABR has been used clinically for over 20 years. The current study formally evaluated the test performance of the BC tone-evoked ABR in infants with hearing loss. Method. By comparing BC-ABR results to follow-up behavioural results, this study addressed two questions: (i) whether the BC tone ABR was successful in differentiating children with conductive versus sensorineural hearing loss (Study A; conductive: 68 ears; SNHL: 129 ears) and (ii) the relationship between BC ABR and behavioural hearing loss severity (Study B: 2000 Hz: 104 ears; 500 Hz: 47 ears). Results. Results demonstrate that the “normal” BC-ABR levels accurately differentiated normal versus elevated cochlear sensitivity (accuracy: 98% for 2000 Hz; 98% for 500 Hz). A subset of infants in Study A with elevated BC-ABR (i.e., no response at normal level) had additional testing at higher intensities, which allowed for categorization of the degree of cochlear impairment. Study B results indicate that the BC ABR accurately categorizes the degree of cochlear hearing loss for 2000 Hz (accuracy = 95.2%). A preliminary dBnHL-to-dBHL correction factor of “0 dB” was determined for 2000 Hz BC ABR. Conclusions. These findings further support the use of BC tone ABR for diagnostic ABR testing. PMID:22988461

Genetic mutation susceptibility of hearing loss in child with severe neonatal jaundice

International Nuclear Information System (INIS)

Zahedi, F.D.; Rahman, R.A.; Abdullah, A.

2015-01-01

This case report demonstrates a case of 5-year-old non-syndromic Malay boy who passed the hearing screening test however he was confirmed has bilateral profound sensorineural hearing loss diagnosed at 3 months of age by brain stem evoked response (BSER). He has background history of severe neonatal jaundice and male siblings of hearing impairment. The antenatal and birth history was uneventful apart from maternal hypothyroidism. His other two elder brothers have bilateral sensorineural hearing loss and history of severe neonatal jaundice as well. The ear examinations, computed tomography scan and magnetic resonance imaging revealed normal findings. Right sided cochlear implantation was done at the age of 3 years old and he is still under audiology follow-up. Conclusion: Genetic studies are important to determine the cause of genetic mutation in susceptibility to hearing impairment that run in his family after severe neonatal jaundice. Those baby with risk of developing hearing loss required diagnostic hearing assessment. (author)

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

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Richard R. Kretschmer

2010-10-01

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

Prosody Perception and Production in Children with Hearing Loss and Age- and Gender-Matched Controls.

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Kalathottukaren, Rose Thomas; Purdy, Suzanne C; Ballard, Elaine

2017-04-01

Auditory development in children with hearing loss, including the perception of prosody, depends on having adequate input from cochlear implants and/or hearing aids. Lack of adequate auditory stimulation can lead to delayed speech and language development. Nevertheless, prosody perception and production in people with hearing loss have received less attention than other aspects of language. The perception of auditory information conveyed through prosody using variations in the pitch, amplitude, and duration of speech is not usually evaluated clinically. This study (1) compared prosody perception and production abilities in children with hearing loss and children with normal hearing; and (2) investigated the effect of age, hearing level, and musicality on prosody perception. Participants were 16 children with hearing loss and 16 typically developing controls matched for age and gender. Fifteen of the children with hearing loss were tested while using amplification (n = 9 hearing aids, n = 6 cochlear implants). Six receptive subtests of the Profiling Elements of Prosody in Speech-Communication (PEPS-C), the Child Paralanguage subtest of Diagnostic Analysis of Nonverbal Accuracy 2 (DANVA 2), and Contour and Interval subtests of the Montreal Battery of Evaluation of Amusia (MBEA) were used. Audio recordings of the children's reading samples were rated using a perceptual prosody rating scale by nine experienced listeners who were blinded to the children's hearing status. Thirty two children, 16 with hearing loss (mean age = 8.71 yr) and 16 age- and gender-matched typically developing children with normal hearing (mean age = 8.87 yr). Assessments were completed in one session lasting 1-2 hours in a quiet room. Test items were presented using a laptop computer through loudspeaker at a comfortable listening level. For children with hearing loss using hearing instruments, all tests were completed with hearing devices set at their everyday listening setting. All PEPS

COCHLEAR IMPLANTATION IN A PATIENT WITH USHER'S SYNDROME

OpenAIRE

Derinsu, Ufuk; Ciprut, Ayca

2016-01-01

Usher's Syndrome is an autosomal recessive disorder characterized by congenital hearing loss and retinitis pigmentosa. Usher’s Syndrome patients with severe to profound sensorineural hearing loss can be considered as candidates for cochlear implantation.This case study reports a deaf-blind with Usher's Syndrome who received a cochlear implant, the audiological evaluation is presented and the therapy sessions are discussed. The patient demonstrated good performance overtime after the...

Arctigenin protects against neuronal hearing loss by promoting neural stem cell survival and differentiation.

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Huang, Xinghua; Chen, Mo; Ding, Yan; Wang, Qin

2017-03-01

Neuronal hearing loss has become a prevalent health problem. This study focused on the function of arctigenin (ARC) in promoting survival and neuronal differentiation of mouse cochlear neural stem cells (NSCs), and its protection against gentamicin (GMC) induced neuronal hearing loss. Mouse cochlea was used to isolate NSCs, which were subsequently cultured in vitro. The effects of ARC on NSC survival, neurosphere formation, differentiation of NSCs, neurite outgrowth, and neural excitability in neuronal network in vitro were examined. Mechanotransduction ability demonstrated by intact cochlea, auditory brainstem response (ABR), and distortion product optoacoustic emissions (DPOAE) amplitude in mice were measured to evaluate effects of ARC on GMC-induced neuronal hearing loss. ARC increased survival, neurosphere formation, neuron differentiation of NSCs in mouse cochlear in vitro. ARC also promoted the outgrowth of neurites, as well as neural excitability of the NSC-differentiated neuron culture. Additionally, ARC rescued mechanotransduction capacity, restored the threshold shifts of ABR and DPOAE in our GMC ototoxicity murine model. This study supports the potential therapeutic role of ARC in promoting both NSCs proliferation and differentiation in vitro to functional neurons, thus supporting its protective function in the therapeutic treatment of neuropathic hearing loss in vivo. © 2017 Wiley Periodicals, Inc.

Decline of Low-Frequency Hearing in People With Ski-Slope Hearing Loss; Implications for Electrode Array Insertion.

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Schuurbiers, Jasper; Dingemanse, Gertjan; Metselaar, Mick

2017-12-01

The decline of low-frequency hearing in people with ski-slope hearing loss varies and might depend on etiology. People with ski-sloping hearing loss might benefit from cochlear implantation with preservation of residual hearing. To reduce the risk of losing low-frequency hearing after implantation, the electrode-array can be inserted partially up to the desired frequency. That, however, obstructs electrical stimulation of lower frequencies. To decide between complete or partial insertion, knowledge regarding the natural decline of low-frequency hearing is helpful. Patients with at least two ski-slope audiograms over time were selected. We calculated progression at lower frequencies for 320 patients. Etiologies for hearing loss were retrieved from medical records. Progression of hearing loss was analyzed separately for patients with uni- and bilateral hearing losses. Relative progression of hearing loss was obtained by comparing progression to a reference group. Average progression of PTA was 1.73 dB/yr and was not significantly different in the bilateral and unilateral group. Etiologies that did not show significantly more progression compared with the reference group could be identified as single or short-lasting pathologic events, whereas long-lasting conditions had significant more progression of PTA. Patients with a ski-slope hearing loss that was caused by a single or short-lasting event have low progression rate and are viable for partial insertion to minimize the risk of damaging residual low-frequency hearing. In the absence of such an event, complete insertion should be considered because faster than normal deterioration of low-frequency hearing over time will probably limit the advantage of preservation of residual hearing.

Evidence for the distortion product frequency place as a source of distortion product otoacoustic emission (DPOAE) fine structure in humans : II. Fine structure for different shapes of cochlear hearing loss

NARCIS (Netherlands)

Mauermann, M; Uppenkamp, S; van Hengel, P.W.J.; Kollmeier, B

1999-01-01

Distortion product otoacoustic emissions (DPOAE) were recorded from eight human subjects with mild to moderate cochlear hearing loss, using a frequency spacing of 48 primary pairs per octave and at a level L(1) = L(2) = 60 dBSPL and with a fixed ratio f(2)/f(1). Subjects with different shapes of

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.

Audiovisual sentence repetition as a clinical criterion for auditory development in Persian-language children with hearing loss.

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Oryadi-Zanjani, Mohammad Majid; Vahab, Maryam; Rahimi, Zahra; Mayahi, Anis

2017-02-01

It is important for clinician such as speech-language pathologists and audiologists to develop more efficient procedures to assess the development of auditory, speech and language skills in children using hearing aid and/or cochlear implant compared to their peers with normal hearing. So, the aim of study was the comparison of the performance of 5-to-7-year-old Persian-language children with and without hearing loss in visual-only, auditory-only, and audiovisual presentation of sentence repetition task. The research was administered as a cross-sectional study. The sample size was 92 Persian 5-7 year old children including: 60 with normal hearing and 32 with hearing loss. The children with hearing loss were recruited from Soroush rehabilitation center for Persian-language children with hearing loss in Shiraz, Iran, through consecutive sampling method. All the children had unilateral cochlear implant or bilateral hearing aid. The assessment tool was the Sentence Repetition Test. The study included three computer-based experiments including visual-only, auditory-only, and audiovisual. The scores were compared within and among the three groups through statistical tests in α = 0.05. The score of sentence repetition task between V-only, A-only, and AV presentation was significantly different in the three groups; in other words, the highest to lowest scores belonged respectively to audiovisual, auditory-only, and visual-only format in the children with normal hearing (P audiovisual sentence repetition scores in all the 5-to-7-year-old children (r = 0.179, n = 92, P = 0.088), but audiovisual sentence repetition scores were found to be strongly correlated with auditory-only scores in all the 5-to-7-year-old children (r = 0.943, n = 92, P = 0.000). According to the study's findings, audiovisual integration occurs in the 5-to-7-year-old Persian children using hearing aid or cochlear implant during sentence repetition similar to their peers with normal hearing

Complex-Tone Pitch Discrimination in Listeners With Sensorineural Hearing Loss

DEFF Research Database (Denmark)

Bianchi, Federica; Fereczkowski, Michal; Zaar, Johannes

2016-01-01

estimated in the same listeners. The estimated reduction of cochlear compression was significantly correlated with the increase in the F0DL ratio, while no correlation was found with filter bandwidth. The effects of degraded frequency selectivity and loss of compression were considered in a simplified......-discrimination performance in listeners with SNHL. Pitch-discrimination thresholds were obtained for 14 normal-hearing (NH) and 10 hearing-impaired (HI) listeners for sine-phase (SP) and random-phase (RP) complex tones. When all harmonics were unresolved, the HI listeners performed, on average, worse than NH listeners...... in the RP condition but similarly to NH listeners in the SP condition. The increase in pitch-discrimination performance for the SP relative to the RP condition (F0DL ratio) was significantly larger in the HI as compared with the NH listeners. Cochlear compression and auditory-filter bandwidths were...

Hearing loss

Science.gov (United States)

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

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

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Preben Homøe

2013-08-01

Full Text Available Objective. Cochlear implant (CI treatment was introduced to the world in the 1980s and has become a routine treatment for congenital or acquired severe-to-profound hearing loss. CI treatment requires access to a highly skilled team of ear, nose and throat specialists, audiologists and speech-language pathologists for evaluation, surgery and rehabilitation. In particular, children treated with CI are in need of long-term post-operative auditory training and other follow-up support. Design. The study is retrospective with updated information on present performance. Results. Since 2001, a total of 11 Greenlandic patients living in Greenland have been treated with CI, 7 children and 4 adults. Of these children, 4 use oral communication only and are full-time CI-users, 2 with full-time use of CI are still in progress with use of oral communication, and 1 has not acquired oral language yet, but has started auditory and speech training. Six children attend mainstream public school while one child is in kindergarten. Of the adults, only 1 has achieved good speech perception with full-time use of CI while 3 do not use the CI. Discussion. From an epidemiological point of view, approximately 1–3 children below 6 years are in need of a CI every second year in Greenland often due to sequelae from meningitis, which may cause postinfectious deafness. Screening of new-borns for hearing has been started in Greenland establishing the basis for early diagnosis of congenital hearing impairment and subsequent intervention. The logistics and lack of availability of speech therapists in Greenland hampers possibilities for optimal language and speech therapy of CI patients in Greenland. This study aims at describing the results of CI treatment in Greenlanders and the outcome of the CI operations along with the auditory and speech/language outcomes. Finally, we present a suggestion for the future CI treatment and recommendations for an increased effort in the

Hearing loss alters serotonergic modulation of intrinsic excitability in auditory cortex.

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Rao, Deepti; Basura, Gregory J; Roche, Joseph; Daniels, Scott; Mancilla, Jaime G; Manis, Paul B

2010-11-01

Sensorineural hearing loss during early childhood alters auditory cortical evoked potentials in humans and profoundly changes auditory processing in hearing-impaired animals. Multiple mechanisms underlie the early postnatal establishment of cortical circuits, but one important set of developmental mechanisms relies on the neuromodulator serotonin (5-hydroxytryptamine [5-HT]). On the other hand, early sensory activity may also regulate the establishment of adultlike 5-HT receptor expression and function. We examined the role of 5-HT in auditory cortex by first investigating how 5-HT neurotransmission and 5-HT(2) receptors influence the intrinsic excitability of layer II/III pyramidal neurons in brain slices of primary auditory cortex (A1). A brief application of 5-HT (50 μM) transiently and reversibly decreased firing rates, input resistance, and spike rate adaptation in normal postnatal day 12 (P12) to P21 rats. Compared with sham-operated animals, cochlear ablation increased excitability at P12-P21, but all the effects of 5-HT, except for the decrease in adaptation, were eliminated in both sham-operated and cochlear-ablated rats. At P30-P35, cochlear ablation did not increase intrinsic excitability compared with shams, but it did prevent a pronounced decrease in excitability that appeared 10 min after 5-HT application. We also tested whether the effects on excitability were mediated by 5-HT(2) receptors. In the presence of the 5-HT(2)-receptor antagonist, ketanserin, 5-HT significantly decreased excitability compared with 5-HT or ketanserin alone in both sham-operated and cochlear-ablated P12-P21 rats. However, at P30-P35, ketanserin had no effect in sham-operated and only a modest effect cochlear-ablated animals. The 5-HT(2)-specific agonist 5-methoxy-N,N-dimethyltryptamine also had no effect at P12-P21. These results suggest that 5-HT likely regulates pyramidal cell excitability via multiple receptor subtypes with opposing effects. These data also show that

Hybrid cochlear implantation: quality of life, quality of hearing, and working performance compared to patients with conventional unilateral or bilateral cochlear implantation.

Science.gov (United States)

Härkönen, Kati; Kivekäs, Ilkka; Kotti, Voitto; Sivonen, Ville; Vasama, Juha-Pekka

2017-10-01

The objective of the present study is to evaluate the effect of hybrid cochlear implantation (hCI) on quality of life (QoL), quality of hearing (QoH), and working performance in adult patients, and to compare the long-term results of patients with hCI to those of patients with conventional unilateral cochlear implantation (CI), bilateral CI, and single-sided deafness (SSD) with CI. Sound localization accuracy and speech-in-noise test were also compared between these groups. Eight patients with high-frequency sensorineural hearing loss of unknown etiology were selected in the study. Patients with hCI had better long-term speech perception in noise than uni- or bilateral CI patients, but the difference was not statistically significant. The sound localization accuracy was equal in the hCI, bilateral CI, and SSD patients. QoH was statistically significantly better in bilateral CI patients than in the others. In hCI patients, residual hearing was preserved in all patients after the surgery. During the 3.6-year follow-up, the mean hearing threshold at 125-500 Hz decreased on average by 15 dB HL in the implanted ear. QoL and working performance improved significantly in all CI patients. Hearing outcomes with hCI are comparable to the results of bilateral CI or CI with SSD, but hearing in noise and sound localization are statistically significantly better than with unilateral CI. Interestingly, the impact of CI on QoL, QoH, and working performance was similar in all groups.

Animal Model of Sensorineural Hearing Loss Associated with Lassa Virus Infection.

Science.gov (United States)

Yun, Nadezhda E; Ronca, Shannon; Tamura, Atsushi; Koma, Takaaki; Seregin, Alexey V; Dineley, Kelly T; Miller, Milagros; Cook, Rebecca; Shimizu, Naoki; Walker, Aida G; Smith, Jeanon N; Fair, Joseph N; Wauquier, Nadia; Bockarie, Bayon; Khan, Sheik Humarr; Makishima, Tomoko; Paessler, Slobodan

2015-12-30

Approximately one-third of Lassa virus (LASV)-infected patients develop sensorineural hearing loss (SNHL) in the late stages of acute disease or in early convalescence. With 500,000 annual cases of Lassa fever (LF), LASV is a major cause of hearing loss in regions of West Africa where LF is endemic. To date, no animal models exist that depict the human pathology of LF with associated hearing loss. Here, we aimed to develop an animal model to study LASV-induced hearing loss using human isolates from a 2012 Sierra Leone outbreak. We have recently established a murine model for LF that closely mimics many features of human disease. In this model, LASV isolated from a lethal human case was highly virulent, while the virus isolated from a nonlethal case elicited mostly mild disease with moderate mortality. More importantly, both viruses were able to induce SNHL in surviving animals. However, utilization of the nonlethal, human LASV isolate allowed us to consistently produce large numbers of survivors with hearing loss. Surviving mice developed permanent hearing loss associated with mild damage to the cochlear hair cells and, strikingly, significant degeneration of the spiral ganglion cells of the auditory nerve. Therefore, the pathological changes in the inner ear of the mice with SNHL supported the phenotypic loss of hearing and provided further insights into the mechanistic cause of LF-associated hearing loss. Sensorineural hearing loss is a major complication for LF survivors. The development of a small-animal model of LASV infection that replicates hearing loss and the clinical and pathological features of LF will significantly increase knowledge of pathogenesis and vaccine studies. In addition, such a model will permit detailed characterization of the hearing loss mechanism and allow for the development of appropriate diagnostic approaches and medical care for LF patients with hearing impairment. Copyright © 2016, American Society for Microbiology. All Rights

Self-esteem and social well-being of children with cochlear implant compared to normal-hearing children

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Percy-Smith, L.; Caye-Thomasen, P.; Gudman, M.

2008-01-01

Objective: The purpose of this study was to make a quantitative comparison of parameters of self-esteem and social well-being between children with cochlear implants and normal-hearing children. Material and methods: Data were obtained from 164 children with cochlear implant (CI) and 2169 normal......-hearing children (NH). Parental questionnaires, used in a national survey assessing the self-esteem and well-being of normal-hearing children, were applied to the cochlear implanted group, in order to allow direct comparisons. Results: The children in the CI group rated significantly higher on questions about well...... overall self-esteem or number of friends. The two groups of children scored similarly on being confident, independent, social, not worried and happy. Conclusion: Children with cochlear implant score equal to or better than their normal-hearing peers on matters of self-esteem and social well-being. (C...

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

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Jill B Firszt

2013-12-01

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

Cochlear Synaptopathy and Noise-Induced Hidden Hearing Loss

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Lijuan Shi

2016-01-01

Full Text Available Recent studies on animal models have shown that noise exposure that does not lead to permanent threshold shift (PTS can cause considerable damage around the synapses between inner hair cells (IHCs and type-I afferent auditory nerve fibers (ANFs. Disruption of these synapses not only disables the innervated ANFs but also results in the slow degeneration of spiral ganglion neurons if the synapses are not reestablished. Such a loss of ANFs should result in signal coding deficits, which are exacerbated by the bias of the damage toward synapses connecting low-spontaneous-rate (SR ANFs, which are known to be vital for signal coding in noisy background. As there is no PTS, these functional deficits cannot be detected using routine audiological evaluations and may be unknown to subjects who have them. Such functional deficits in hearing without changes in sensitivity are generally called “noise-induced hidden hearing loss (NIHHL.” Here, we provide a brief review to address several critical issues related to NIHHL: (1 the mechanism of noise induced synaptic damage, (2 reversibility of the synaptic damage, (3 the functional deficits as the nature of NIHHL in animal studies, (4 evidence of NIHHL in human subjects, and (5 peripheral and central contribution of NIHHL.

Spatial Release From Masking in Simulated Cochlear Implant Users With and Without Access to Low-Frequency Acoustic Hearing

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Ben Williges

2015-12-01

Full Text Available For normal-hearing listeners, speech intelligibility improves if speech and noise are spatially separated. While this spatial release from masking has already been quantified in normal-hearing listeners in many studies, it is less clear how spatial release from masking changes in cochlear implant listeners with and without access to low-frequency acoustic hearing. Spatial release from masking depends on differences in access to speech cues due to hearing status and hearing device. To investigate the influence of these factors on speech intelligibility, the present study measured speech reception thresholds in spatially separated speech and noise for 10 different listener types. A vocoder was used to simulate cochlear implant processing and low-frequency filtering was used to simulate residual low-frequency hearing. These forms of processing were combined to simulate cochlear implant listening, listening based on low-frequency residual hearing, and combinations thereof. Simulated cochlear implant users with additional low-frequency acoustic hearing showed better speech intelligibility in noise than simulated cochlear implant users without acoustic hearing and had access to more spatial speech cues (e.g., higher binaural squelch. Cochlear implant listener types showed higher spatial release from masking with bilateral access to low-frequency acoustic hearing than without. A binaural speech intelligibility model with normal binaural processing showed overall good agreement with measured speech reception thresholds, spatial release from masking, and spatial speech cues. This indicates that differences in speech cues available to listener types are sufficient to explain the changes of spatial release from masking across these simulated listener types.

Hearing Aids: MedlinePlus Health Topic

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... for hearing loss (Medical Encyclopedia) Also in Spanish Topic Image MedlinePlus Email Updates Get Hearing Aids updates ... MEDICAL ENCYCLOPEDIA Devices for hearing loss Related Health Topics Cochlear Implants Hearing Disorders and Deafness National Institutes ...

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.

Effect of gender on the hearing performance of adult cochlear implant patients.

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Lenarz, Minoo; Sönmez, Hasibe; Joseph, Gert; Büchner, Andreas; Lenarz, Thomas

2012-05-01

To evaluate the role of gender on the hearing performance of postlingually deafened adult patients with cochlear implants. Individual retrospective cohort study. There were 638 postlingually deafened adults (280 men and 358 women) selected for a retrospective evaluation of their hearing performance with cochlear implants. Both genders underwent the same surgical and rehabilitative procedures and benefited from the latest technological advances available. There was no significant difference in the age, duration of deafness, and preoperative hearing performance between the genders. The test battery was composed of the Freiburger Monosyllabic Test, Speech Tracking, and the Hochmair-Schulz-Moser (HSM) sentence test in quiet and in 10-dB noise. The results of 5 years of follow-up are presented here. Genders showed a similar performance in Freiburger Monosyllabic Test and Speech Tracking Test. However, in the HSM test in noise, men performed slightly better than women in all of the follow-up sessions, which was statistically significant at 2 and 4 years after implantation. Although normal-hearing women use more predictive cognitive strategies in speech comprehension and are supposed to have a more efficient declarative memory system, this may not necessarily lead to a better adaptation to the altered auditory information delivered by a cochlear implant. Our study showed that in more complex listening situations such as speech tests in noise, men tend to perform slightly better than women. Gender may have an influence on the hearing performance of postlingually deafened adults with cochlear implants. Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.

Electrode Impedance Fluctuations as a Biomarker for Inner Ear Pathology After Cochlear Implantation.

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Choi, June; Payne, Matthew R; Campbell, Luke J; Bester, Christo W; Newbold, Carrie; Eastwood, Hayden; O'Leary, Stephen J

2017-12-01

Cochlear implant surgery now aims to preserve residual low frequency hearing. The current research explores whether fluctuations in the electrical impedance of cochlear implant electrodes may act as a biomarker for pathological changes that lead to the delayed loss of residual hearing. Secondary analysis of a double-blinded randomized trial, where methylprednisolone was administered intravenously before cochlear implantation with a view to preserving residual hearing. Seventy-four patients with residual hearing after cochlear implant surgery were investigated for an impedance "spike," defined as a median rise of ≥4 kΩ across all electrodes from the baseline measurements. Spikes were related to objective and subjective hearing loss, dizziness, and tinnitus. An impedance spike occurred in 14% (10/74) of enrolled patients. Three months after surgery, five patients exhibited spikes and three of these patients had a total loss of their residual hearing. 4.3% of the 69 patients without spikes lost residual hearing. At 1 year, 9 of 10 patients who exhibited spikes had lost all their residual hearing. 8.1% of the 37 patients who did not experience a spike lost their residual hearing. Seventy percent of patients exhibiting a spike also experienced vertigo. The administration of steroids at the time of surgery did not influence the occurrence of spikes. Our results suggest that there is a relationship between a spike and the loss of residual hearing. It seems that rises in impedance can reflect pathology within the inner ear and predict the future loss of residual hearing.

Drug discovery for hearing loss: Phenotypic screening of chemical compounds on primary cultures of the spiral ganglion.

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Whitlon, Donna S

2017-06-01

In the United States there are, at present, no drugs that are specifically FDA approved to treat hearing loss. Although several clinical trials are ongoing, including one testing D-methionine that is supported by the US Army, none of these trials directly address the effect of noise exposure on cochlear spiral ganglion neurons. We recently published the first report of a systematic chemical compound screen using primary, mammalian spiral ganglion cultures in which we were able to detect a compound and others in its class that increased neurite elongation, a critical step in restoring cochlear synapses after noise induced hearing loss. Here we discuss the issues, both pro and con, that influenced the development of our approach. These considerations may be useful for future compound screens that target the same or other attributes of cochlear spiral ganglion neurons. Copyright © 2016 Elsevier B.V. All rights reserved.

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

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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 implant treatment in Germany].

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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.

An evaluation of preservation of residual hearing using the suprameatal approach for cochlear implantation: can this implantation technique be used for preservation of residual hearing?

NARCIS (Netherlands)

Postelmans, Job T. F.; van Spronsen, Erik; Grolman, Wilko; Stokroos, Robert J.; Tange, Rinze A.; Maré, Marcel J.; Dreschler, Wouter A.

2011-01-01

The preservation of residual hearing has become a high priority in cochlear implant surgery. This study was designed to substantiate whether conservation of residual hearing can be preserved after cochlear implantation using the suprameatal approach. Retrospective chart review. Retrospective chart

Newborn hearing screening and strategy for early detection of hearing loss in infants.

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Jakubíková, Janka; Kabátová, Zuzana; Pavlovcinová, Gabriela; Profant, Milan

2009-04-01

More than 80% of permanent hearing losses (HL) in children are congenital. Newborn hearing screening (NHS) is the best method for early detection of suspected hearing loss. If the NHS is not universal more than 30% permanent hearing losses are not identified. There are various methods of NHS: otoacoustic emissions (TEOAE, DPOAE) and automatic auditory brainstem response (AABR). After hearing screening, and when hearing loss is suspected, tympanometry and audiological methods then used for determination of hearing threshold; these include ABR, ASSR or/and behavioral methods. The goal of this study is to evaluate the influence of UNHS on the early detection of hearing loss in children before and after the implementation of obligatory universal newborn hearing screening in Slovakia, and also on the etiologic evaluation of hearing impaired infants identified by screening. In Slovakia NHS started in 1998 and was provided in ENT departments. From May 1, 2006 UNHS has been mandatory in Slovakia, using two stages TEOAE in all newborn departments in Slovakia (64 newborn departments). In year 2005--42% of newborns in Slovakia were screened, in 2006--66% newborns and in 2007--94, 99% (three small newborn departments do not yet have equipment for OAE screening). For determination of hearing thresholds ASSR are used in two ENT departments and ABR in the other four ENT departments. Comparing the number of identified cases with bilateral severe permanent HL or deafness before and after UNHS, 22.8% more cases of PHL were identified in the first year of UNHS. Also the average age of diagnosis of PHL was lower. In the year 2007, 94% of newborns were screened. We found 0.947/1000 newborns with bilateral severe PHL (35.9%) more than before UNHS). After audiologic and etiologic assessment of the 76 infants who failed screening, 5 (6.58%) were found to have normal hearing, 16 (22.54%) had unilateral and 55 (77.46%) had bilateral SNHL. A non-syndromic genetic cause was present in 25

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

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

2015-01-01

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

Cochlear Implant

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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.

[A comparative study on efficacy of glucocorticoids, mineralocorticoids and vasoactive drugs on reversing hearing loss in patients suffering idiopathic sensorineural cochlear hypoacusis. A preliminary clinical trial].

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Campos-Bañales, Eugenia María; López-Campos, Daniel; de Serdio-Arias, José Luis; Esteban-Rodriguez, J; García-Sáinz, Mar; Muñoz-Cortés, Álvaro; López-Aguado, Daniel

2015-01-01

Sensory neural hearing loss (SNHL) is a disorder characterised by an important deterioration of the auditory function. Re-establishing normal ion homeostasis of the endolymph could be related to hearing recovery and it might be mediated by mineralocorticoids. The main purpose of this preliminary, randomized controlled clinical trial was assessing the recovery of idiopathic sensory neural cochlear hearing loss (SNHL) by comparing the efficacy of 2 types of steroids versus vasodilators. The 3-month intervention involved 70 patients, allocated into 4 different groups: a control with no medication, consisting of 14 patients (8 men and 6 women); a vasodilator group of 21 patients (11 men and 10 women); a glucocorticoid group with 16 patients (10 men and 6 women); and a mineralocorticoid therapy group, consisting of 19 patients (11 men and 8 women). The level of hearing loss and its topography were estimated using Liminal Tone Audiometry (LTA) and Auditory Brainstem Response (ABR). Our research found overall greater efficacy of mineralocorticoids versus glucocorticoids and vasodilators. There was better response in women than in men and it was higher from the left ear, regardless of patient gender. The hearing gain was significantly superior in the mineralocorticoid group, followed by the glucocorticoid group. However, the responses to vasodilators were lesser and of low statistical significance. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

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

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Yanfei Wang

2017-12-01

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

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

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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.

Language structures used by kindergartners with cochlear implants: relationship to phonological awareness, lexical knowledge and hearing loss.

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Nittrouer, Susan; Sansom, Emily; Low, Keri; Rice, Caitlin; Caldwell-Tarr, Amanda

2014-01-01

Listeners use their knowledge of how language is structured to aid speech recognition in everyday communication. When it comes to children with congenital hearing loss severe enough to warrant cochlear implants (CIs), the question arises of whether these children can acquire the language knowledge needed to aid speech recognition, in spite of only having spectrally degraded signals available to them. That question was addressed in the present study. Specifically, there were three goals: (1) to compare the language structures used by children with CIs to those of children with normal hearing (NH); (2) to assess the amount of variance in the language measures explained by phonological awareness and lexical knowledge; and (3) to assess the amount of variance in the language measures explained by factors related to the hearing loss itself and subsequent treatment. Language samples were obtained and transcribed for 40 children who had just completed kindergarten: 19 with NH and 21 with CIs. Five measures were derived from Systematic Analysis of Language Transcripts: (1) mean length of utterance in morphemes, (2) number of conjunctions, excluding and, (3) number of personal pronouns, (4) number of bound morphemes, and (5) number of different words. Measures were also collected on phonological awareness and lexical knowledge. Statistics examined group differences, as well as the amount of variance in the language measures explained by phonological awareness, lexical knowledge, and factors related to hearing loss and its treatment for children with CIs. Mean scores of children with CIs were roughly one standard deviation below those of children with NH on all language measures, including lexical knowledge, matching outcomes of other studies. Mean scores of children with CIs were closer to two standard deviations below those of children with NH on two out of three measures of phonological awareness (specifically those related to phonemic structure). Lexical knowledge

Cortical Plasticity after Cochlear Implantation

DEFF Research Database (Denmark)

Petersen, Bjørn; Gjedde, Albert; Wallentin, Mikkel

2013-01-01

recently implanted adult implant recipients listened to running speech or speech-like noise in four sequential PET sessions at each milestone. CI listeners with postlingual hearing loss showed differential activation of left superior temporal gyrus during speech and speech-like stimuli, unlike CI listeners...... with prelingual hearing loss. Furthermore, Broca's area was activated as an effect of time, but only in CI listeners with postlingual hearing loss. The study demonstrates that adaptation to the cochlear implant is highly related to the history of hearing loss. Speech processing in patients whose hearing loss...... occurred after the acquisition of language involves brain areas associated with speech comprehension, which is not the case for patients whose hearing loss occurred before the acquisition of language. Finally, the findings confirm the key role of Broca's area in restoration of speech perception, but only...

Ipsilateral distortion product otoacoustic emission (2 f1-f2) suppression in children with sensorineural hearing loss

Science.gov (United States)

Abdala, Carolina; Fitzgerald, Tracy S.

2003-08-01

Distortion product otoacoustic emission (DPOAE) ipsilateral suppression has been applied to study cochlear function and maturation in laboratory animals and humans. Although DPOAE suppression appears to be sensitive to regions of specialized cochlear function and to cochlear immaturity, it is not known whether it reflects permanent cochlear damage, i.e., sensorineural hearing loss (SNHL), in a reliable and systematic manner in humans. Eight school-aged children with mild-moderate SNHL and 20 normal-hearing children served as subjects in this study. DPOAE (2 f1-f2) suppression data were collected at four f2 frequencies (1500, 3000, 4000, and 6000 Hz) using moderate-level primary tones. Features of the DPOAE iso-suppression tuning curves and suppression growth were analyzed for both subject groups. Results show that DPOAE suppression tuning curves from hearing-impaired subjects can be reliably recorded. DPOAE suppression tuning curves were generally normal in appearance and shape for six out of eight hearing-impaired subjects but showed subtle abnormalities in at least one feature. There was not one single trend or pattern of abnormality that characterized all hearing-impaired subjects. The most prominent patterns of abnormality included: broadened tuning, elevated tip, and downward shift of tip frequency. The unique patterns of atypical DPOAE suppression in subjects with similar audiograms may suggest different patterns of underlying sensory cell damage. This speculation warrants further investigation.

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.

Noise-Induced Loss of Hair Cells and Cochlear Synaptopathy Are Mediated by the Activation of AMPK

Science.gov (United States)

Hill, Kayla; Yuan, Hu; Wang, Xianren

2016-01-01

Noise-induced hearing loss (NIHL) is a major unresolved public health problem. Here, we investigate pathomechanisms of sensory hair cell death and suggest a novel target for protective intervention. Cellular survival depends upon maintenance of energy homeostasis, largely by AMP-activated protein kinase (AMPK). In response to a noise exposure in CBA/J mice, the levels of phosphorylated AMPKα increased in hair cells in a noise intensity-dependent manner. Inhibition of AMPK via siRNA or the pharmacological inhibitor compound C attenuated noise-induced loss of outer hair cells (OHCs) and synaptic ribbons, and preserved auditory function. Additionally, noise exposure increased the activity of the upstream AMPK kinase liver kinase B1 (LKB1) in cochlear tissues. The inhibition of LKB1 by siRNA attenuated the noise-increased phosphorylation of AMPKα in OHCs, reduced the loss of inner hair cell synaptic ribbons and OHCs, and protected against NIHL. These results indicate that noise exposure induces hair cell death and synaptopathy by activating AMPK via LKB1-mediated pathways. Targeting these pathways may provide a novel route to prevent NIHL. SIGNIFICANCE STATEMENT Our results demonstrate for the first time that the activation of AMP-activated protein kinase (AMPK) α in sensory hair cells is noise intensity dependent and contributes to noise-induced hearing loss by mediating the loss of inner hair cell synaptic ribbons and outer hair cells. Noise induces the phosphorylation of AMPKα1 by liver kinase B1 (LKB1), triggered by changes in intracellular ATP levels. The inhibition of AMPK activation by silencing AMPK or LKB1, or with the pharmacological inhibitor compound C, reduced outer hair cell and synaptic ribbon loss as well as noise-induced hearing loss. This study provides new insights into mechanisms of noise-induced hearing loss and suggests novel interventions for the prevention of the loss of sensory hair cells and cochlear synaptopathy. PMID:27413159

Noise-Induced Loss of Hair Cells and Cochlear Synaptopathy Are Mediated by the Activation of AMPK.

Science.gov (United States)

Hill, Kayla; Yuan, Hu; Wang, Xianren; Sha, Su-Hua

2016-07-13

Noise-induced hearing loss (NIHL) is a major unresolved public health problem. Here, we investigate pathomechanisms of sensory hair cell death and suggest a novel target for protective intervention. Cellular survival depends upon maintenance of energy homeostasis, largely by AMP-activated protein kinase (AMPK). In response to a noise exposure in CBA/J mice, the levels of phosphorylated AMPKα increased in hair cells in a noise intensity-dependent manner. Inhibition of AMPK via siRNA or the pharmacological inhibitor compound C attenuated noise-induced loss of outer hair cells (OHCs) and synaptic ribbons, and preserved auditory function. Additionally, noise exposure increased the activity of the upstream AMPK kinase liver kinase B1 (LKB1) in cochlear tissues. The inhibition of LKB1 by siRNA attenuated the noise-increased phosphorylation of AMPKα in OHCs, reduced the loss of inner hair cell synaptic ribbons and OHCs, and protected against NIHL. These results indicate that noise exposure induces hair cell death and synaptopathy by activating AMPK via LKB1-mediated pathways. Targeting these pathways may provide a novel route to prevent NIHL. Our results demonstrate for the first time that the activation of AMP-activated protein kinase (AMPK) α in sensory hair cells is noise intensity dependent and contributes to noise-induced hearing loss by mediating the loss of inner hair cell synaptic ribbons and outer hair cells. Noise induces the phosphorylation of AMPKα1 by liver kinase B1 (LKB1), triggered by changes in intracellular ATP levels. The inhibition of AMPK activation by silencing AMPK or LKB1, or with the pharmacological inhibitor compound C, reduced outer hair cell and synaptic ribbon loss as well as noise-induced hearing loss. This study provides new insights into mechanisms of noise-induced hearing loss and suggests novel interventions for the prevention of the loss of sensory hair cells and cochlear synaptopathy. Copyright © 2016 the authors 0270-6474/16/367497-14$15.00/0.

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.

Ampleness of head movements of children and adolescents with sensorineural hearing loss.

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de Souza Melo, Renato

2017-02-01

Head movements are controlled by the vestibular system. Children with sensorineural hearing loss can present restrictions in ampleness of head movements due to damage in the vestibule-cochlear systems, resulting from injury in the inner ear. To evaluate the ampleness of head movements of children with normal hearing and children with sensorineural hearing loss and compare data between groups. Cross-sectional study that evaluated the ampleness of head movements of 96 students, being 48 with normal hearing and 48 with sensorineural hearing loss, of both sexes, with aged between 7 and 18 years old. The performance of ampleness of head movements was analyzed by a manual goniometric evaluation, according the references proposed by Marques. To the statistical analysis we used the t-Student test in case of normality of the data or the Mann-Whitney test when did not applied the suppositions of normality. Hearing loss children showed less mean in ampleness of all movements of head compared to normal hearing children, pointing difference to movements of flexion (p = 0,001), lateral inclination to the right (p = 0,025) and lateral rotation to the left (p = 0,021). Hearing loss children showed reduction in the ampleness of these head movements: flexion, lateral inclination to the right and lateral rotation to the left compared to normal hearing children. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Cochlear Implantation in Patients with Neurofibromatosis Type 2 and Patients with Vestibular Schwannoma in the Only Hearing Ear

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Erika Celis-Aguilar

2012-01-01

Full Text Available Cochlear implants are a new surgical option in the hearing rehabilitation of patients with neurofibromatosis type 2 (NF2 and patients with vestibular schwannoma (VS in the only hearing ear. Auditory brainstem implant (ABI has been the standard surgical treatment for these patients. We performed a literature review of patients with NF2 and patients with VS in the only hearing ear. Cochlear implantation (CI provided some auditory benefit in all patients. Preservation of cochlear nerve integrity is crucial after VS resection. Results ranged from environmental sound awareness to excellent benefit with telephone use. Promontory stimulation is recommended although not crucial. MRI can be performed safely in cochlear implanted patients.

The effect of different cochlear implant microphones on acoustic hearing individuals’ binaural benefits for speech perception in noise

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Aronoff, Justin M.; Freed, Daniel J.; Fisher, Laurel M.; Pal, Ivan; Soli, Sigfrid D.

2011-01-01

Objectives Cochlear implant microphones differ in placement, frequency response, and other characteristics such as whether they are directional. Although normal hearing individuals are often used as controls in studies examining cochlear implant users’ binaural benefits, the considerable differences across cochlear implant microphones make such comparisons potentially misleading. The goal of this study was to examine binaural benefits for speech perception in noise for normal hearing individuals using stimuli processed by head-related transfer functions (HRTFs) based on the different cochlear implant microphones. Design HRTFs were created for different cochlear implant microphones and used to test participants on the Hearing in Noise Test. Experiment 1 tested cochlear implant users and normal hearing individuals with HRTF-processed stimuli and with sound field testing to determine whether the HRTFs adequately simulated sound field testing. Experiment 2 determined the measurement error and performance-intensity function for the Hearing in Noise Test with normal hearing individuals listening to stimuli processed with the various HRTFs. Experiment 3 compared normal hearing listeners’ performance across HRTFs to determine how the HRTFs affected performance. Experiment 4 evaluated binaural benefits for normal hearing listeners using the various HRTFs, including ones that were modified to investigate the contributions of interaural time and level cues. Results The results indicated that the HRTFs adequately simulated sound field testing for the Hearing in Noise Test. They also demonstrated that the test-retest reliability and performance-intensity function were consistent across HRTFs, and that the measurement error for the test was 1.3 dB, with a change in signal-to-noise ratio of 1 dB reflecting a 10% change in intelligibility. There were significant differences in performance when using the various HRTFs, with particularly good thresholds for the HRTF based on the

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....

Conditional deletion of pejvakin in adult outer hair cells causes progressive hearing loss in mice.

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Harris, Suzan L; Kazmierczak, Marcin; Pangršič, Tina; Shah, Prahar; Chuchvara, Nadiya; Barrantes-Freer, Alonso; Moser, Tobias; Schwander, Martin

2017-03-06

Mutations in the Pejvakin (Pjvk) gene cause autosomal recessive hearing loss DFNB59 with audiological features of auditory neuropathy spectrum disorder (ANSD) or cochlear dysfunction. The precise mechanisms underlying the variable clinical phenotypes of DFNB59 remain unclear. Here, we demonstrate that mice with conditional ablation of the Pjvk gene in all sensory hair cells or only in outer hair cells (OHCs) show similar auditory phenotypes with early-onset profound hearing loss. By contrast, loss of Pjvk in adult OHCs causes a slowly progressive hearing loss associated with OHC degeneration and delayed loss of inner hair cells (IHCs), indicating a primary role for pejvakin in regulating OHC function and survival. Consistent with this model, synaptic transmission at the IHC ribbon synapse is largely unaffected in sirtaki mice that carry a C-terminal deletion mutation in Pjvk. Using the C-terminal domain of pejvakin as bait, we identified in a cochlear cDNA library ROCK2, an effector for the small GTPase Rho, and the scaffold protein IQGAP1, involved in modulating actin dynamics. Both ROCK2 and IQGAP1 associate via their coiled-coil domains with pejvakin. We conclude that pejvakin is required to sustain OHC activity and survival in a cell-autonomous manner likely involving regulation of Rho signaling. Copyright © 2017 IBRO. Published by Elsevier Ltd. All rights reserved.

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 implants in Waardenburg syndrome.

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Cullen, Robert D; Zdanski, Carlton; Roush, Patricia; Brown, Carolyn; Teagle, Holly; Pillsbury, Harold C; Buchman, Craig

2006-07-01

Waardenburg syndrome is an autosomal-dominant syndrome characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia irides, a white forelock, and sensorineural hearing loss in 20% to 55% of patients. This patient population accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with Waardenburg syndrome who have undergone cochlear implantation. Pediatric cochlear implant recipients with documented evidence of Waardenburg syndrome underwent retrospective case review. All patients received their cochlear implants at the study institution followed by outpatient auditory habilitation. Charts were reviewed for etiology and duration of deafness, age at time of cochlear implantation, perioperative complications, duration of use, and performance outcomes. Results of standard tests batteries for speech perception and production administered as a part of the patients' auditory habilitation were reviewed. Seven patients with Waardenburg syndrome and cochlear implants were identified. The average age at implantation was 37 months (range, 18-64 months) and the average duration of use was 69 months (range, 12-143 months). All of these patients are active users of their devices and perform very well after implantation. There were no major complications in this small group of patients. Children with congenital sensorineural hearing loss without other comorbidities (e.g., developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. Patients with Waardenburg syndrome can be expected to have above-average performance after cochlear implantation.

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

Science.gov (United States)

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.

Correlation between subjective and objective hearing tests after unilateral and bilateral cochlear implantation

NARCIS (Netherlands)

Ramakers, Geerte G.J.; Smulders, Yvette E.; Van Zon, Alice; van Zanten, Gijsbert A.; Grolman, Wilko; Stegeman, Inge

2017-01-01

Background: There are many methods for assessing hearing performance after cochlear implantation. Standard evaluations often encompass objective hearing tests only, while patients' subjective experiences gain importance in today's healthcare. The aim of the current study was to analyze the

Cultural Identity of Young Deaf Adults with Cochlear Implants in Comparison to Deaf without Cochlear Implants and Hard-of-Hearing Young Adults.

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Goldblat, Ester; Most, Tova

2018-07-01

This study examined the relationships between cultural identity, severity of hearing loss (HL), and the use of a cochlear implant (CI). One hundred and forty-one adolescents and young adults divided into three groups (deaf with CI, deaf without CI, and hard-of-hearing (HH)) and 134 parents participated. Adolescents and young adults completed questionnaires on cultural identity (hearing, Deaf, marginal, bicultural-hearing, and bicultural-deaf) and communication proficiencies (hearing, spoken language, and sign language). Parents completed a speech quality questionnaire. Deaf participants without CI and those with CI differed in all identities except marginal identity. CI users and HH participants had similar identities except for a stronger bicultural-deaf identity among CI users. Three clusters of participants evolved: participants with a dominant bicultural-deaf identity, participants with a dominant bicultural-hearing identity and participants without a formed cultural identity. Adolescents and young adults who were proficient in one of the modes of communication developed well-established bicultural identities. Adolescents and young adults who were not proficient in one of the modes of communication did not develop a distinguished cultural identity. These results suggest that communication proficiencies are crucial for developing defined identities.

Literacy Skills among Deaf and Hard of Hearing Students and Students with Cochlear Implants in Bilingual/Bicultural Education

DEFF Research Database (Denmark)

Dammeyer, Jesper Herup

2014-01-01

Research has shown that many deaf students do not develop age-appropriate reading and writing abilities. This study evaluates the literacy skills of deaf students, hard of hearing students, and students with cochlear implants in bilingual/bicultural schools in Denmark. The results show that 45 per...... cent of the students did not have any reading and writing difficulties (i.e. they were no more than 1 year behind in school). Regression analysis models show that language abilities (either aural-oral or signed) and additional disabilities were explaining factors. Neither the level of hearing loss nor...

Auditory, Visual, and Auditory-Visual Perception of Emotions by Individuals with Cochlear Implants, Hearing Aids, and Normal Hearing

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Most, Tova; Aviner, Chen

2009-01-01

This study evaluated the benefits of cochlear implant (CI) with regard to emotion perception of participants differing in their age of implantation, in comparison to hearing aid users and adolescents with normal hearing (NH). Emotion perception was examined by having the participants identify happiness, anger, surprise, sadness, fear, and disgust.…

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.

A lack of immune system genes causes loss in high frequency hearing but does not disrupt cochlear synapse maturation in mice.

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Calton, Melissa A; Lee, Dasom; Sundaresan, Srividya; Mendus, Diana; Leu, Rose; Wangsawihardja, Felix; Johnson, Kenneth R; Mustapha, Mirna

2014-01-01

Early cochlear development is marked by an exuberant outgrowth of neurites that innervate multiple targets. The establishment of mature cochlear neural circuits is, however, dependent on the pruning of inappropriate axons and synaptic connections. Such refinement also occurs in the central nervous system (CNS), and recently, genes ordinarily associated with immune and inflammatory processes have been shown to play roles in synaptic pruning in the brain. These molecules include the major histocompatibility complex class I (MHCI) genes, H2-K(b) and H2-D(b), and the complement cascade gene, C1qa. Since the mechanisms involved in synaptic refinement in the cochlea are not well understood, we investigated whether these immune system genes may be involved in this process and whether they are required for normal hearing function. Here we report that these genes are not necessary for normal synapse formation and refinement in the mouse cochlea. We further demonstrate that C1qa expression is not necessary for normal hearing in mice but the lack of expression of H2-K(b) and H2-D(b) causes hearing impairment. These data underscore the importance of the highly polymorphic family of MHCI genes in hearing in mice and also suggest that factors and mechanisms regulating synaptic refinement in the cochlea may be distinct from those in the CNS.

High prevalence of abnormalities on CT and MR imaging in children with unilateral sensorineural hearing loss irrespective of age or degree of hearing loss.

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van Beeck Calkoen, E A; Sanchez Aliaga, E; Merkus, P; Smit, C F; van de Kamp, J M; Mulder, M F; Goverts, S T; Hensen, E F

2017-06-01

Evaluation of causal abnormalities identified on CT and MR imaging in children with unilateral sensorineural hearing loss (USNHL), and the association with age and severity of hearing loss. Retrospective cohort study. Tertiary referral otology/audiology center. 102 children diagnosed with USNHL between 2006 and 2016 were included. They underwent CT and/or MR imaging for the evaluation of the etiology of their hearing loss. Radiologic abnormalities of the inner ear and brain associated with USNHL. Using CT and/or MR imaging, causal abnormalities were identified in 49%, which is higher than previously reported (25-40%). The most frequently affected site was the labyrinth (29%), followed by the cochlear nerve (9%) and brain (7%). No significant difference in the number or type of abnormalities was found for the degree of hearing loss or age categories. Imaging is essential in the etiologic analysis of USNHL because of the high prevalence of causative abnormalities that can be identified with radiology, irrespective of the patients' age or degree of hearing loss. CT and MR imaging are complementary imaging options. The ideal imaging algorithm is controversial. Based on our findings, we conclude that there is limited additional diagnostic value of simultaneous dual modality imaging over sequential diagnostics. We therefore perform a stepwise radiological workup in order to maximize the diagnostic yield while minimizing impact and costs. If the primary imaging modality does not identify a cause for USNHL, performing the alternative imaging modality should be considered. Retrospective cohort study 2b. Copyright © 2017 Elsevier B.V. All rights reserved.

Classification of voice disorder in children with cochlear implantation and hearing aid using multiple classifier fusion

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Tayarani Hamid

2011-01-01

Full Text Available Abstract Background Speech production and speech phonetic features gradually improve in children by obtaining audio feedback after cochlear implantation or using hearing aids. The aim of this study was to develop and evaluate automated classification of voice disorder in children with cochlear implantation and hearing aids. Methods We considered 4 disorder categories in children's voice using the following definitions: Level_1: Children who produce spontaneous phonation and use words spontaneously and imitatively. Level_2: Children, who produce spontaneous phonation, use words spontaneously and make short sentences imitatively. Level_3: Children, who produce spontaneous phonations, use words and arbitrary sentences spontaneously. Level_4: Normal children without any hearing loss background. Thirty Persian children participated in the study, including six children in each level from one to three and 12 children in level four. Voice samples of five isolated Persian words "mashin", "mar", "moosh", "gav" and "mouz" were analyzed. Four levels of the voice quality were considered, the higher the level the less significant the speech disorder. "Frame-based" and "word-based" features were extracted from voice signals. The frame-based features include intensity, fundamental frequency, formants, nasality and approximate entropy and word-based features include phase space features and wavelet coefficients. For frame-based features, hidden Markov models were used as classifiers and for word-based features, neural network was used. Results After Classifiers fusion with three methods: Majority Voting Rule, Linear Combination and Stacked fusion, the best classification rates were obtained using frame-based and word-based features with MVR rule (level 1:100%, level 2: 93.75%, level 3: 100%, level 4: 94%. Conclusions Result of this study may help speech pathologists follow up voice disorder recovery in children with cochlear implantation or hearing aid who are

Pediatric tinnitus: Incidence of imaging anomalies and the impact of hearing loss.

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Kerr, Rhorie; Kang, Elise; Hopkins, Brandon; Anne, Samantha

2017-12-01

Guidelines exist for evaluation and management of tinnitus in adults; however lack of evidence in children limits applicability of these guidelines to pediatric patients. Objective of this study is to determine the incidence of inner ear anomalies detected on imaging studies within the pediatric population with tinnitus and evaluate if presence of hearing loss increases the rate of detection of anomalies in comparison to normal hearing patients. Retrospective review of all children with diagnosis of tinnitus from 2010 to 2015 ;at a tertiary care academic center. 102 pediatric patients with tinnitus were identified. Overall, 53 patients had imaging studies with 6 abnormal findings (11.3%). 51/102 patients had hearing loss of which 33 had imaging studies demonstrating 6 inner ear anomalies detected. This is an incidence of 18.2% for inner ear anomalies identified in patients with hearing loss (95% confidence interval (CI) of 7.0-35.5%). 4 of these 6 inner ear anomalies detected were vestibular aqueduct abnormalities. The other two anomalies were cochlear hypoplasia and bilateral semicircular canal dysmorphism. 51 patients had no hearing loss and of these patients, 20 had imaging studies with no inner ear abnormalities detected. There was no statistical difference in incidence of abnormal imaging findings in patients with and without hearing loss (Fisher's exact test, p ;= ;0.072.) CONCLUSION: There is a high incidence of anomalies detected in imaging studies done in pediatric patients with tinnitus, especially in the presence of hearing loss. Copyright © 2017 Elsevier B.V. All rights reserved.

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

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

2008-06-01

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

Parents' preferences for services for children with hearing loss: a conjoint analysis study.

Science.gov (United States)

Fitzpatrick, Elizabeth; Coyle, Douglas E; Durieux-Smith, Andrée; Graham, Ian D; Angus, Douglas E; Gaboury, Isabelle

2007-12-01

Early identification of permanent childhood hearing loss through universal newborn hearing screening is rapidly becoming a standard of care. However, it is well recognized that hearing screening must be embedded within a comprehensive system of rehabilitation and parent support services. This study was undertaken with parents of young children with permanent hearing loss to examine their preferences for characteristics associated with intervention services. A secondary goal was to explore whether preferences may differ according to patient subgroups. Conjoint analysis, a preference-based economic technique, was used to investigate parents' strength of preferences. A cross-sectional survey that consisted of hypothetical clinic scenarios was developed based on information from qualitative interviews with parents. The questionnaire was administered to parents receiving intervention services in the province of Ontario, Canada, shortly after the implementation of a universal hearing screening program. The sample was recruited from three different clinical programs. A total of 48 of 75 respondents completed the questionnaire, a response rate of 64%. The participants varied by screening status of the child (25 screened, 23 not screened), type of device (23 hearing aids, 25 cochlear implants), and region. All five characteristics of care that were selected for inclusion in the survey were found to be statistically significant attributes of services: coordinated services, access to parent support, access to information, frequency of services, and location of services. Parents showed a preference for clinic-based rather than home-based services. Preferences toward once a week therapy services rather than services two to three times weekly were also found. In particular, parents valued service models that consisted of well-coordinated care with access to support from other parents. Differences in respondents according to hearing screening status (screened or unscreened), type

Durability of Hearing Preservation after Cochlear Implantation with Conventional-Length Electrodes and Scala Tympani Insertion.

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Sweeney, Alex D; Hunter, Jacob B; Carlson, Matthew L; Rivas, Alejandro; Bennett, Marc L; Gifford, Rene H; Noble, Jack H; Haynes, David S; Labadie, Robert F; Wanna, George B

2016-05-01

To analyze factors that influence hearing preservation over time in cochlear implant recipients with conventional-length electrode arrays located entirely within the scala tympani. Case series with planned chart review. Single tertiary academic referral center. A retrospective review was performed to analyze a subgroup of cochlear implant recipients with residual acoustic hearing. Patients were included in the study only if their electrode arrays remained fully in the scala tympani after insertion and serviceable acoustic hearing (≤80 dB at 250 Hz) was preserved. Electrode array location was verified through a validated radiographic assessment tool. Patients with scala tympani. In this group, the style of electrode array may influence residual hearing preservation over time. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.

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.

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)

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.

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

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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.

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.

Congenital abnormality of the inner ear and internal auditory canal in a patient with deep sensorineural hearing loss: A case report

International Nuclear Information System (INIS)

Rivera, Diego; Saab, Said; Cordoba, Claudia; Montes, Guillermo; Barreto, Tatiana

2010-01-01

CT and MRI are complementary studies that have proven to be the best radiological tools in screening of children with unilateral or bilateral sensorineural hearing loss. Only about a 20% of the patients with congenital sensor neural hearing loss have manifestations in images. Due to the fact that most of these manifestations initiate in the bone, the CT is the first line of image study. MRI is indicated in the evaluation of suspected agenesis, neuropathy, aplasia or hypoplasia of the vestibulocochlear nerve, often associated with this type of hearing loss. We present a case of a 6 year old patient with deep bilateral sensor neural hearing loss, with radiological studies to determine a potential candidate for a cochlear or auditory brainstem implant as hearing rehabilitation.

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

Science.gov (United States)

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 injury and adaptive plasticity of the auditory cortex

Directory of Open Access Journals (Sweden)

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.

The Antioxidant Effect of Rosmarinic Acid by Different Delivery Routes in the Animal Model of Noise-Induced Hearing Loss.

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Fetoni, Anna R; Eramo, Sara Letizia Maria; Di Pino, Antonella; Rolesi, Rolando; Paciello, Fabiola; Grassi, Claudio; Troiani, Diana; Paludetti, Gaetano

2018-03-01

Trans-tympanic Rosmarinic Acid (RA), as compared with the systemic administration, protects against noise-induced auditory hair cell and hearing losses in rats in vivo. ROS production, lipoperoxidative damage, and an imbalance of antioxidant defences play a significant role in noise-induced hearing loss. Several molecules with antioxidant properties have been tested to restore redox homeostasis; however, drug delivery system represents a challenge for their effectiveness. In our model, acute and intense noise exposure induces hearing loss, hair cell death, and oxidative stress, with an increase in superoxide production and over-expression of lipid peroxidation in cochlear structures. RA was administrated in male Wistar rats by trans-tympanic (20 μl) and systemic (10 mg/kg) modality. In systemic administration, RA was injected 1 hour before noise exposure and once daily for the following 3 days. ABRs were measured before and at days 1, 3, 7, and 30 after noise exposure. Rhodamine-phalloidin staining, dihydroethidium and 8-isoprostane immunostainings were performed to assess and quantify outer hair cells loss, superoxide production, and lipid peroxidation in the different experimental groups. Systemic RA administration significantly decreased noise-induced hearing loss and the improvement of auditory function was paralleled by a significant reduction in cochlear oxidative stress. The trans-tympanic modality of drug administration showed a similar degree of protection both at the functional and morphological levels. The effectiveness of RA given via trans-tympanic injection could be interesting for the future application of this minimally-invasive procedure in the treatment of ROS-induced hearing loss.

Screening Newborns' Hearing Now Standard | NIH MedlinePlus the Magazine

Science.gov (United States)

... Possible Hearing Problem Your Baby's Hearing and Communicative Development Checklist Communication Considerations —for parents of children with hearing loss Cochlear Implants —surgically implanted hearing ...

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.

Science.gov (United States)

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

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

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Snik, Ad; Agterberg, Martijn; Bosman, Arjan

2015-01-01

Application of bilateral hearing devices in bilateral hearing loss and unilateral application in unilateral hearing loss (second ear with normal hearing) does not a priori lead to binaural hearing. An overview is presented on several measures of binaural benefits that have been used in patients with unilateral or bilateral deafness using one or two cochlear implants, respectively, and in patients with unilateral or bilateral conductive/mixed hearing loss using one or two percutaneous bone conduction implants (BCDs), respectively. Overall, according to this overview, the most significant and sensitive measure is the benefit in directional hearing. Measures using speech (viz. binaural summation, binaural squelch or use of the head shadow effect) showed minor benefits, except for patients with bilateral conductive/mixed hearing loss using two BCDs. Although less feasible in daily practise, the binaural masking level difference test seems to be a promising option in the assessment of binaural function. © 2015 S. Karger AG, Basel.

Toluene-induced hearing loss in the guinea pig.

Science.gov (United States)

Waniusiow, Delphine; Campo, Pierre; Venet, Thomas; Cossec, Benoît; Cosnier, Frédéric; Beydon, Dominique; Rieger, Benoît; Burgart, Manuella; Ferrari, Luc; Parietti-Winkler, Cécile

2009-10-01

Toluene is a high-production industrial solvent, which can disrupt the auditory system in rats. However, toluene-induced hearing loss is species dependent. For instance, despite long-lasting exposures to high concentrations of aromatic solvent, no study has yet succeeded in causing convincing hearing loss in the guinea pig. This latter species can be characterized by two metabolic particularities: a high amount of hepatic cytochrome P-450s (P-450s) and a high concentration of glutathione in the cochlea. It is therefore likely that the efficiency of both the hepatic and cochlear metabolisms plays a key role in the innocuousness of the hearing of guinea pigs to exposure to solvent. The present study was carried out to test the auditory resistance to toluene in glutathione-depleted guinea pigs whose the P-450 activity was partly inhibited. To this end, animals on a low-protein diet received a general P-450 inhibitor, namely SKF525-A. Meanwhile, they were exposed to 1750 ppm toluene for 4 weeks, 5 days/week, 6 h/day. Auditory function was tested by electrocochleography and completed by histological analyses. For the first time, a significant toluene-induced hearing loss was provoked in the P-450-inhibited guinea pigs. However, the ototoxic process caused by the solvent exposure was different from that observed in the rat. Only the stria vascularis and the spiral fibers were disrupted in the apical coil of the cochlea. The protective mechanisms developed by guinea pigs are discussed in the present publication.

Presença de microfonismo coclear no peate-clique: diagnóstico diferencial entre espectro da neuropatia auditiva e perdas auditivas cocleares descendentes em crianças Presence of cochlear microphonics in click-ABR: differential diagnosis between auditory neuropathy spectrum disorder and steeply sloping cochlear hearing loss in children

Directory of Open Access Journals (Sweden)

Gabriela Ribeiro Ivo Rodrigues

2010-12-01

Full Text Available TEMA: diagnóstico diferencial entre espectro da neuropatia auditiva e perdas auditivas cocleares descendentes em crianças com presença de microfonismo coclear no PEATE-clique. PROCEDIMENTOS: este relato de caso descreve os resultados da avaliação audiológica de duas crianças atendidas no Centro "Audição na Criança" da Divisão de Educação e Reabilitação dos Distúrbios da Comunicação da Pontifícia Universidade Católica de São Paulo (CeAC/DERDIC/PUCSP que apresentaram microfonismo coclear no registro do PEATE-clique. As crianças foram submetidas às avaliações utilizando-se o PEATE-clique, o registro das emissões otoacústicas e a avaliação audiológica tonal, com a técnica da Audiometria de Reforço Visual. RESULTADOS: as avaliações comportamental, eletroacústica e eletrofisiológica revelaram que as crianças apresentam perda auditiva sensorioneural (coclear com configuração descendente, de modo que a presença do microfonismo coclear no registro do PEATE-clique era provavelmente gerada pela preservação da cóclea nas frequências baixas. CONCLUSÃO: os casos apresentados mostram que na ausência das emissões otoacústicas e presença do microfonismo coclear, não se deve interpretar isoladamente cada exame, para que não ocorram equívocos no diagnóstico, que pode ser confundido com o Espectro da Neuropatia Auditiva. O microfonismo coclear pode aparecer em outras condições, tais como em perdas auditivas cocleares descendentes.BACKGROUND: differential diagnosis between auditory neuropathy spectrum disorder andsteeply sloping cochlear hearing loss in children with presence of cochlear microphonics for click-ABR. PROCEDURES: this case report describes the results of the audiological evaluation for two children assisted at Centro "Audição na Criança" of Divisão de Educação e Reabilitação dos Distúrbios da Comunicação of Pontifícia Universidade Católica of São Paulo (CeAC/DERDIC/PUCSP with presence

Mutations in cockayne syndrome-associated genes (Csa and Csb) predispose to cisplatin-induced hearing loss in mice

NARCIS (Netherlands)

R.N. Rainey (Robert N.); S.-Y. Ng (Sum-Yan); J. Llamas (Juan); G.T.J. van der Horst (Gijsbertus); N. Segil (Neil)

2016-01-01

textabstractCisplatin is a common and effective chemotherapeutic agent, yet it often causes permanent hearing loss as a result of sensory hair cell death. The causes of sensitivity to DNA-damaging agents in nondividing cell populations, such as cochlear hair and supporting cells, are poorly

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

International Nuclear Information System (INIS)

Hong, Hyun Sook; Paik, Sang Hyun; Cha, Jang Gyu; Park, Seong Jin; Joh, Joon Hee; Park, Jai Soung; Kim, Dae Ho; Lee, Hae Kyung; Kim, Shi Chan

2005-01-01

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

The cochlear implant as a tinnitus treatment.

Science.gov (United States)

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.

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

Science.gov (United States)

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.

Improving Spoken Language Outcomes for Children With Hearing Loss: Data-driven Instruction.

Science.gov (United States)

Douglas, Michael

2016-02-01

To assess the effects of data-driven instruction (DDI) on spoken language outcomes of children with cochlear implants and hearing aids. Retrospective, matched-pairs comparison of post-treatment speech/language data of children who did and did not receive DDI. Private, spoken-language preschool for children with hearing loss. Eleven matched pairs of children with cochlear implants who attended the same spoken language preschool. Groups were matched for age of hearing device fitting, time in the program, degree of predevice fitting hearing loss, sex, and age at testing. Daily informal language samples were collected and analyzed over a 2-year period, per preschool protocol. Annual informal and formal spoken language assessments in articulation, vocabulary, and omnibus language were administered at the end of three time intervals: baseline, end of year one, and end of year two. The primary outcome measures were total raw score performance of spontaneous utterance sentence types and syntax element use as measured by the Teacher Assessment of Spoken Language (TASL). In addition, standardized assessments (the Clinical Evaluation of Language Fundamentals--Preschool Version 2 (CELF-P2), the Expressive One-Word Picture Vocabulary Test (EOWPVT), the Receptive One-Word Picture Vocabulary Test (ROWPVT), and the Goldman-Fristoe Test of Articulation 2 (GFTA2)) were also administered and compared with the control group. The DDI group demonstrated significantly higher raw scores on the TASL each year of the study. The DDI group also achieved statistically significant higher scores for total language on the CELF-P and expressive vocabulary on the EOWPVT, but not for articulation nor receptive vocabulary. Post-hoc assessment revealed that 78% of the students in the DDI group achieved scores in the average range compared with 59% in the control group. The preliminary results of this study support further investigation regarding DDI to investigate whether this method can consistently

Auditory development in early amplified children: factors influencing auditory-based communication outcomes in children with hearing loss.

Science.gov (United States)

Sininger, Yvonne S; Grimes, Alison; Christensen, Elizabeth

2010-04-01

The purpose of this study was to determine the influence of selected predictive factors, primarily age at fitting of amplification and degree of hearing loss, on auditory-based outcomes in young children with bilateral sensorineural hearing loss. Forty-four infants and toddlers, first identified with mild to profound bilateral hearing loss, who were being fitted with amplification were enrolled in the study and followed longitudinally. Subjects were otherwise typically developing with no evidence of cognitive, motor, or visual impairment. A variety of subject factors were measured or documented and used as predictor variables, including age at fitting of amplification, degree of hearing loss in the better hearing ear, cochlear implant status, intensity of oral education, parent-child interaction, and the number of languages spoken in the home. These factors were used in a linear multiple regression analysis to assess their contribution to auditory-based communication outcomes. Five outcome measures, evaluated at regular intervals in children starting at age 3, included measures of speech perception (Pediatric Speech Intelligibility and Online Imitative Test of Speech Pattern Contrast Perception), speech production (Arizona-3), and spoken language (Reynell Expressive and Receptive Language). The age at fitting of amplification ranged from 1 to 72 mo, and the degree of hearing loss ranged from mild to profound. Age at fitting of amplification showed the largest influence and was a significant factor in all outcome models. The degree of hearing loss was an important factor in the modeling of speech production and spoken language outcomes. Cochlear implant use was the other factor that contributed significantly to speech perception, speech production, and language outcomes. Other factors contributed sparsely to the models. Prospective longitudinal studies of children are important to establish relationships between subject factors and outcomes. This study clearly

Cochlear ımplantatıon ın a patıent wıth usher's syndrome

OpenAIRE

Derinsu, Ufuk; Ciprut, Ayca

2002-01-01

Usher's Syndrome is an autosomal recessive disorder characterized by congenital hearing loss and retinitis pigmentosa. Usher’s Syndrome patients with severe to profound sensorineural hearing loss can be considered as candidates for cochlear implantation. This case study reports a deaf-blind with Usher's Syndrome who received a cochlear implant, the audiological evaluation is presented and the therapy sessions are discussed. The patient demonstrated good performance overtime after the impla...

Contemporary noise-induced hearing loss (NIHL) prevention.

Science.gov (United States)

Sułkowski, Wiesław; Owczarek, Kalina; Olszewski, Jurek

2017-08-31

Hearing impairment caused by noise, traditionally called - depending on the duration of exposure - acute or chronic acoustic trauma, includes, in addition to presbyacusis, the most common adult population of hearing impaired. In Poland - according to the report of the Central Statistical Office (GUS, 2011), the number of workers employed in NDN exceeded the noise level (85 dB) is about 200 thousand, the highest in the mining, metal and metal products production, textiles and wood production. According to the Regulation of the Council of Ministers of on June 30, 2009, on the list of occupational diseases (Journal of Laws No. 132, item 1115), it is defined as "bilateral permanent hearing loss of the cochlear or sensory-nerve type, expressed as an increase in hearing threshold of at least 45 dB in the ear better heard, calculated as an arithmetic mean for frequencies 1,2 and 3 kHz. Hearing impairments also occur in the military and police during field training and in combat where the source of acoustic injuries are firearms and pulse-inducing explosions (as in some industries) with high C peak levels (Lc peak) Time to rise to a maximum of <1 ms. The prevalence of loud music listening, particularly by personal stereo players, is also affecting children and adolescents with audiometric hearing loss, according to the World Health Organization (WHO) estimates of around 15-20%. The preventive action strategy is defined by the European Union legislation and the national implementing legislation that reduces or eliminates the risk and reduces (if not eliminated), taking into account available technical and organizational solutions to minimize the risk of hearing damage. If you can not reduce the noise levels with technical and organizational methods, you need individual hearing protectors. Ear protectors may be equipped with electronic systems with active noise reduction (which can improve low and medium frequency performance), adjustable attenuation (improves speech

Auditory preferences of young children with and without hearing loss for meaningful auditory-visual compound stimuli.

Science.gov (United States)

Zupan, Barbra; Sussman, Joan E

2009-01-01

Experiment 1 examined modality preferences in children and adults with normal hearing to combined auditory-visual stimuli. Experiment 2 compared modality preferences in children using cochlear implants participating in an auditory emphasized therapy approach to the children with normal hearing from Experiment 1. A second objective in both experiments was to evaluate the role of familiarity in these preferences. Participants were exposed to randomized blocks of photographs and sounds of ten familiar and ten unfamiliar animals in auditory-only, visual-only and auditory-visual trials. Results indicated an overall auditory preference in children, regardless of hearing status, and a visual preference in adults. Familiarity only affected modality preferences in adults who showed a strong visual preference to unfamiliar stimuli only. The similar degree of auditory responses in children with hearing loss to those from children with normal hearing is an original finding and lends support to an auditory emphasis for habilitation. Readers will be able to (1) Describe the pattern of modality preferences reported in young children without hearing loss; (2) Recognize that differences in communication mode may affect modality preferences in young children with hearing loss; and (3) Understand the role of familiarity in modality preferences in children with and without hearing loss.

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.

Balance Performance of Deaf Children With and Without Cochlear Implants

Directory of Open Access Journals (Sweden)

Amir-Abbas Ebrahimi

2016-12-01

Full Text Available  The aim of this study was to compare the static and dynamic balance performance of deaf children with and without cochlear implants. This is a cross-sectional study of 145 school children, aged between 7 and 12 years comprising 85 children with congenital or early acquired bilateral profound sensorineural hearing loss (the hearing loss group and 60 normal hearing aged-matched control counterparts were assessed using the balance subtest of Bruininks-Oseretsky test of Motor Proficiency (BOTMP. The hearing loss group, 50 without cochlear implants (the non-implant group and 35 of them with unilateral cochlear implants (the implant group were recruited from schools for the deaf and normal hearing children (the control group randomly selected from two randomly selected elementary schools of Tehran city. The scores were analyzed using one-way ANOVA. The total score of deaf children especially the implant group were significantly lower than the control group P<0.001. The balance performance of the control group was better than the implant group in all of the items as well as the non-implant group except the fourth tested item (walking forward on a line (P<0.05. The balance score of the implant group was significantly lower than the non-implant group except for the third tested item (standing on the preferred leg on a balance beam with eyes closed. The findings suggested that deaf children, specifically those with cochlear implants are at risk for motor and balance deficits. Thus, vestibular and motor evaluations, as well as interventions to improve balance and motor skills, should be prioritized for this population.

Hidden Hearing Loss and Computational Models of the Auditory Pathway: Predicting Speech Intelligibility Decline

Science.gov (United States)

2016-11-28

Title: Hidden Hearing Loss and Computational Models of the Auditory Pathway: Predicting Speech Intelligibility Decline Christopher J. Smalt...representation of speech intelligibility in noise. The auditory-periphery model of Zilany et al. (JASA 2009,2014) is used to make predictions of...auditory nerve (AN) responses to speech stimuli under a variety of difficult listening conditions. The resulting cochlear neurogram, a spectrogram

Audiovisual spoken word recognition as a clinical criterion for sensory aids efficiency in Persian-language children with hearing loss.

Science.gov (United States)

Oryadi-Zanjani, Mohammad Majid; Vahab, Maryam; Bazrafkan, Mozhdeh; Haghjoo, Asghar

2015-12-01

The aim of this study was to examine the role of audiovisual speech recognition as a clinical criterion of cochlear implant or hearing aid efficiency in Persian-language children with severe-to-profound hearing loss. This research was administered as a cross-sectional study. The sample size was 60 Persian 5-7 year old children. The assessment tool was one of subtests of Persian version of the Test of Language Development-Primary 3. The study included two experiments: auditory-only and audiovisual presentation conditions. The test was a closed-set including 30 words which were orally presented by a speech-language pathologist. The scores of audiovisual word perception were significantly higher than auditory-only condition in the children with normal hearing (Paudiovisual presentation conditions (P>0.05). The audiovisual spoken word recognition can be applied as a clinical criterion to assess the children with severe to profound hearing loss in order to find whether cochlear implant or hearing aid has been efficient for them or not; i.e. if a child with hearing impairment who using CI or HA can obtain higher scores in audiovisual spoken word recognition than auditory-only condition, his/her auditory skills have appropriately developed due to effective CI or HA as one of the main factors of auditory habilitation. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Noise-induced and age-related hearing loss:  new perspectives and potential therapies [version 1; referees: 4 approved

Directory of Open Access Journals (Sweden)

M Charles Liberman

2017-06-01

Full Text Available The classic view of sensorineural hearing loss has been that the primary damage targets are hair cells and that auditory nerve loss is typically secondary to hair cell degeneration. Recent work has challenged that view. In noise-induced hearing loss, exposures causing only reversible threshold shifts (and no hair cell loss nevertheless cause permanent loss of >50% of the synaptic connections between hair cells and the auditory nerve. Similarly, in age-related hearing loss, degeneration of cochlear synapses precedes both hair cell loss and threshold elevation. This primary neural degeneration has remained a “hidden hearing loss” for two reasons: 1 the neuronal cell bodies survive for years despite loss of synaptic connection with hair cells, and 2 the degeneration is selective for auditory nerve fibers with high thresholds. Although not required for threshold detection when quiet, these high-threshold fibers are critical for hearing in noisy environments. Research suggests that primary neural degeneration is an important contributor to the perceptual handicap in sensorineural hearing loss, and it may be key to the generation of tinnitus and other associated perceptual anomalies. In cases where the hair cells survive, neurotrophin therapies can elicit neurite outgrowth from surviving auditory neurons and re-establishment of their peripheral synapses; thus, treatments may be on the horizon.

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.

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....

Pharmacokinetic Properties of Adenosine Amine Congener in Cochlear Perilymph after Systemic Administration

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Hao Chang

2017-01-01

Full Text Available Noise-induced hearing loss (NIHL is a global health problem affecting over 5% of the population worldwide. We have shown previously that acute noise-induced cochlear injury can be ameliorated by administration of drugs acting on adenosine receptors in the inner ear, and a selective A1 adenosine receptor agonist adenosine amine congener (ADAC has emerged as a potentially effective treatment for cochlear injury and resulting hearing loss. This study investigated pharmacokinetic properties of ADAC in rat perilymph after systemic (intravenous administration using a newly developed liquid chromatography-tandem mass spectrometry detection method. The method was developed and validated in accordance with the USA FDA guidelines including accuracy, precision, specificity, and linearity. Perilymph was sampled from the apical turn of the cochlea to prevent contamination with the cerebrospinal fluid. ADAC was detected in cochlear perilymph within two minutes following intravenous administration and remained in perilymph above its minimal effective concentration for at least two hours. The pharmacokinetic pattern of ADAC was significantly altered by exposure to noise, suggesting transient changes in permeability of the blood-labyrinth barrier and/or cochlear blood flow. This study supports ADAC development as a potential clinical otological treatment for acute sensorineural hearing loss caused by exposure to traumatic noise.

Speech recognition in normal hearing and sensorineural hearing loss as a function of the number of spectral channels

NARCIS (Netherlands)

Baskent, Deniz

Speech recognition by normal-hearing listeners improves as a function of the number of spectral channels when tested with a noiseband vocoder simulating cochlear implant signal processing. Speech recognition by the best cochlear implant users, however, saturates around eight channels and does not

Ferulic acid promotes survival and differentiation of neural stem cells to prevent gentamicin-induced neuronal hearing loss.

Science.gov (United States)

Gu, Lintao; Cui, Xinhua; Wei, Wei; Yang, Jia; Li, Xuezhong

2017-11-15

Neural stem cells (NSCs) have exhibited promising potential in therapies against neuronal hearing loss. Ferulic acid (FA) has been widely reported to enhance neurogenic differentiation of different stem cells. We investigated the role of FA in promoting NSC transplant therapy to prevent gentamicin-induced neuronal hearing loss. NSCs were isolated from mouse cochlear tissues to establish in vitro culture, which were then treated with FA. The survival and differentiation of NSCs were evaluated. Subsequently, neurite outgrowth and excitability of the in vitro neuronal network were assessed. Gentamicin was used to induce neuronal hearing loss in mice, in the presence and absence of FA, followed by assessments of auditory brainstem response (ABR) and distortion product optoacoustic emissions (DPOAE) amplitude. FA promoted survival, neurosphere formation and differentiation of NSCs, as well as neurite outgrowth and excitability of in vitro neuronal network. Furthermore, FA restored ABR threshold shifts and DPOAE in gentamicin-induced neuronal hearing loss mouse model in vivo. Our data, for the first time, support potential therapeutic efficacy of FA in promoting survival and differentiation of NSCs to prevent gentamicin-induced neuronal hearing loss. Copyright © 2017 Elsevier Inc. All rights reserved.

Current concepts in age-related hearing loss: Epidemiology and mechanistic pathways

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Yamasoba, Tatsuya; Lin, Frank R.; Someya, Shinichi; Kashio, Akinori; Sakamoto, Takashi; Kondo, Kenji

2013-01-01

Age-related hearing loss (AHL), also known as presbycusis, is a universal feature of mammalian aging and is characterized by a decline of auditory function, such as increased hearing thresholds and poor frequency resolution. The primary pathology of AHL includes the hair cells, stria vascularis, and afferent spiral ganglion neurons as well as the central auditory pathways. A growing body of evidence in animal studies has suggested that cumulative effect of oxidative stress could induce damage to macromolecules such as mitochondrial DNA (mtDNA) and that the resulting accumulation of mtDNA mutations/deletions and decline of mitochondrial function play an important role in inducing apoptosis of the cochlear cells, thereby the development of AHL. Epidemiological studies have demonstrated four categories of risk factors of AHL in humans: cochlear aging, environment such as noise exposure, genetic predisposition, and health co-morbidities such as cigarette smoking and atherosclerosis. Genetic investigation has identified several putative associating genes, including those related to antioxidant defense and atherosclerosis. Exposure to noise is known to induce excess generation of reactive oxygen species (ROS) in the cochlea, and cumulative oxidative stress can be enhanced by relatively hypoxic situations resulting from the impaired homeostasis of cochlear blood supply due to atherosclerosis, which could be accelerated by genetic and co-morbidity factors. Antioxidant defense system may also be influenced by genetic backgrounds. These may explain the large variations of the onset and extent of AHL among elderly subjects. PMID:23422312

Bisphosphonate-Linked TrkB Agonist: Cochlea-Targeted Delivery of a Neurotrophic Agent as a Strategy for the Treatment of Hearing Loss.

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Kempfle, Judith S; Nguyen, Kim; Hamadani, Christine; Koen, Nicholas; Edge, Albert S; Kashemirov, Boris A; Jung, David H; McKenna, Charles E

2018-04-18

Hearing loss affects more than two-thirds of the elderly population, and more than 17% of all adults in the U.S. Sensorineural hearing loss related to noise exposure or aging is associated with loss of inner ear sensory hair cells (HCs), cochlear spiral ganglion neurons (SGNs), and ribbon synapses between HCs and SGNs, stimulating intense interest in therapies to regenerate synaptic function. 7,8-Dihydroxyflavone (DHF) is a selective and potent agonist of tropomyosin receptor kinase B (TrkB) and protects the neuron from apoptosis. Despite evidence that TrkB agonists can promote survival of SGNs, local delivery of drugs such as DHF to the inner ear remains a challenge. We previously demonstrated in an animal model that a fluorescently labeled bisphosphonate, 6-FAM-Zol, administered to the round window membrane penetrated the membrane and diffused throughout the cochlea. Given their affinity for bone mineral, including cochlear bone, bisphosphonates offer an intriguing modality for targeted delivery of neurotrophic agents to the SGNs to promote survival, neurite outgrowth, and, potentially, regeneration of synapses between HCs and SGNs. The design and synthesis of a bisphosphonate conjugate of DHF (Ris-DHF) is presented, with a preliminary evaluation of its neurotrophic activity. Ris-DHF increases neurite outgrowth in vitro, maintains this ability after binding to hydroxyapatite, and regenerates synapses in kainic acid-damaged cochlear organ of Corti explants dissected in vitro with attached SGNs. The results suggest that bisphosphonate-TrkB agonist conjugates have promise as a novel approach to targeted delivery of drugs to treat sensorineural hearing loss.

Effect of low-level laser treatment on cochlea hair-cell recovery after ototoxic hearing loss

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Rhee, Chung-Ku; He, Peijie; Jung, Jae Yun; Ahn, Jin-Chul; Chung, Phil-Sang; Lee, Min Young; Suh, Myung-Whan

2013-12-01

The primary cause of hearing loss includes damage to cochlear hair cells. Low-level laser therapy (LLLT) has become a popular treatment for damaged nervous systems. Based on the idea that cochlea hair cells and neural cells are from same developmental origin, the effect of LLLT on hearing loss in animal models is evaluated. Hearing loss animal models were established, and the animals were irradiated by 830-nm diode laser once a day for 10 days. Power density of the laser treatment was 900 mW/cm2, and the fluence was 162 to 194 J. The tympanic membrane was evaluated after LLLT. Thresholds of auditory brainstem responses were evaluated before treatment, after gentamicin, and after 10 days of LLLT. Quantitative scanning electron microscopic (SEM) observations were done by counting remaining hair cells. Tympanic membranes were intact at the end of the experiment. No adverse tissue reaction was found. On SEM images, LLLT significantly increased the number of hair cells in middle and basal turns. Hearing was significantly improved by laser irradiation. After LLLT treatment, both the hearing threshold and hair-cell count significantly improved.

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.

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.

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.

Pre-enlistment hearing loss and hearing loss disability among US soldiers and marines

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Marlene E Gubata

2013-01-01

Full Text Available Hearing loss is a common condition among US adults, with some evidence of increasing prevalence in young adults. Noise-induced hearing loss attributable to employment is a significant source of preventable morbidity world-wide. The US military population is largely comprised of young adult males serving in a wide variety of occupations, many in high noise-level conditions, at least episodically. To identify accession and service-related risk factors for hearing-related disability, matched case-control study of US military personnel was conducted. Individuals evaluated for hearing loss disability in the US Army and Marine Corps were frequency matched to controls without history of disability evaluation on service and enlistment year. Conditional logistic regression was used to examine the association between accession and service-related factors and hearing-related disability evaluations between October 2002 and September 2010. Individuals with medically disqualifying audiograms or hearing loss diagnoses at application for military service were 8 and 4 times more likely, respectively, to have a disability evaluation related to hearing loss, after controlling for relevant accession, demographic, and service-related factors. Conservative hearing loss thresholds on pre-enlistment audiograms, stricter hearing loss medical waiver policies or qualified baseline audiograms pre-enlistment are needed in the U.S military. Industrial corporations or labor unions may also benefit from identifying individuals with moderate hearing loss at the time of employment to ensure use of personal protective equipment and engineer controls of noise.

An Investigation of Spatial Hearing in Children with Normal Hearing and with Cochlear Implants and the Impact of Executive Function

Science.gov (United States)

Misurelli, Sara M.

The ability to analyze an "auditory scene"---that is, to selectively attend to a target source while simultaneously segregating and ignoring distracting information---is one of the most important and complex skills utilized by normal hearing (NH) adults. The NH adult auditory system and brain work rather well to segregate auditory sources in adverse environments. However, for some children and individuals with hearing loss, selectively attending to one source in noisy environments can be extremely challenging. In a normal auditory system, information arriving at each ear is integrated, and thus these binaural cues aid in speech understanding in noise. A growing number of individuals who are deaf now receive cochlear implants (CIs), which supply hearing through electrical stimulation to the auditory nerve. In particular, bilateral cochlear implants (BICIs) are now becoming more prevalent, especially in children. However, because CI sound processing lacks both fine structure cues and coordination between stimulation at the two ears, binaural cues may either be absent or inconsistent. For children with NH and with BiCIs, this difficulty in segregating sources is of particular concern because their learning and development commonly occurs within the context of complex auditory environments. This dissertation intends to explore and understand the ability of children with NH and with BiCIs to function in everyday noisy environments. The goals of this work are to (1) Investigate source segregation abilities in children with NH and with BiCIs; (2) Examine the effect of target-interferer similarity and the benefits of source segregation for children with NH and with BiCIs; (3) Investigate measures of executive function that may predict performance in complex and realistic auditory tasks of source segregation for listeners with NH; and (4) Examine source segregation abilities in NH listeners, from school-age to adults.

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

Cochlear implantation in patient with Dandy-walker syndrome.

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de Oliveira, Adriana Kosma Pires; Hamerschmidt, Rogerio; Mocelin, Marcos; Rezende, Rodrigo K

2012-07-01

 Dandy Walker Syndrome is a congenital abnormality in the central nervous system, characterized by a deficiency in the development of middle cerebelar structures, cystic dilatation of the posterior pit communicating with the fourth ventricle and upward shift of the transverse sinuses, tentorium and dyes. Among the clinical signs are occipital protuberances, a progressive increase of the skull, bowing before the fontanels, papilledema, ataxia, gait disturbances, nystagmus, and intellectual impairment.  To describe a case of female patient, 13 years old with a diagnosis of this syndrome and bilateral hearing loss underwent cochlear implant surgery under local anesthesia and sedation.  CGS, 13 years old female was referred to the Otolaryngological Department of Otolaryngology Institute of Parana with a diagnosis of "Dandy-Walker syndrome" for Otolaryngological evaluation for bilateral hearing loss with no response to the use of hearing aids. Final Comments: The field of cochlear implants is growing rapidly. We believe that the presence of Dandy-Walker syndrome cannot be considered a contraindication to the performance of cochlear implant surgery, and there were no surgical complications due to neurological disorders with very favorable results for the patient who exhibits excellent discrimination. It has less need for lip reading with improvement in speech quality.

Cochlear implantation in patient with Dandy-walker syndrome

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Oliveira, Adriana Kosma Pires de

2012-01-01

Full Text Available Introduction: Dandy Walker Syndrome is a congenital abnormality in the central nervous system, characterized by a deficiency in the development of middle cerebelar structures, cystic dilatation of the posterior pit communicating with the fourth ventricle and upward shift of the transverse sinuses, tentorium and dyes. Among the clinical signs are occipital protuberances, a progressive increase of the skull, bowing before the fontanels, papilledema, ataxia, gait disturbances, nystagmus, and intellectual impairment. Objectives: To describe a case of female patient, 13 years old with a diagnosis of this syndrome and bilateral hearing loss underwent cochlear implant surgery under local anesthesia and sedation. Case Report: CGS, 13 years old female was referred to the Otolaryngological Department of Otolaryngology Institute of Parana with a diagnosis of "Dandy-Walker syndrome" for Otolaryngological evaluation for bilateral hearing loss with no response to the use of hearing aids. Final Comments: The field of cochlear implants is growing rapidly. We believe that the presence of Dandy-Walker syndrome cannot be considered a contraindication to the performance of cochlear implant surgery, and there were no surgical complications due to neurological disorders with very favorable results for the patient who exhibits excellent discrimination. It has less need for lip reading with improvement in speech quality.

Relationship between spectrotemporal modulation detection and music perception in normal-hearing, hearing-impaired, and cochlear implant listeners.

Science.gov (United States)

Choi, Ji Eun; Won, Jong Ho; Kim, Cheol Hee; Cho, Yang-Sun; Hong, Sung Hwa; Moon, Il Joon

2018-01-15

The objective of this study was to examine the relationship between spectrotemporal modulation (STM) sensitivity and the ability to perceive music. Ten normal-nearing (NH) listeners, ten hearing aid (HA) users with moderate hearing loss, and ten cochlear Implant (CI) users participated in this study. Three different types of psychoacoustic tests including spectral modulation detection (SMD), temporal modulation detection (TMD), and STM were administered. Performances on these psychoacoustic tests were compared to music perception abilities. In addition, psychoacoustic mechanisms involved in the improvement of music perception through HA were evaluated. Music perception abilities in unaided and aided conditions were measured for HA users. After that, HA benefit for music perception was correlated with aided psychoacoustic performance. STM detection study showed that a combination of spectral and temporal modulation cues were more strongly correlated with music perception abilities than spectral or temporal modulation cues measured separately. No correlation was found between music perception performance and SMD threshold or TMD threshold in each group. Also, HA benefits for melody and timbre identification were significantly correlated with a combination of spectral and temporal envelope cues though HA.

Simultaneous bilateral laser therapy accelerates recovery after noise-induced hearing loss in a rat model

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Jae-Hun Lee

2016-07-01

Full Text Available Noise-induced hearing loss is a common type of hearing loss. The effects of laser therapy have been investigated from various perspectives, including in wound healing, inflammation reduction, and nerve regeneration, as well as in hearing research. A promising feature of the laser is its capability to penetrate soft tissue; depending on the wavelength, laser energy can penetrate into the deepest part of the body without damaging non-target soft tissues. Based on this idea, we developed bilateral transtympanic laser therapy, which uses simultaneous laser irradiation in both ears, and evaluated the effects of bilateral laser therapy on cochlear damage caused by noise overexposure. Thus, the purpose of this research was to assess the benefits of simultaneous bilateral laser therapy compared with unilateral laser therapy and a control. Eighteen Sprague-Dawley rats were exposed to narrow-band noise at 115 dB SPL for 6 h. Multiple auditory brainstem responses were measured after each laser irradiation, and cochlear hair cells were counted after the 15th such irradiation. The penetration depth of the 808 nm laser was also measured after sacrifice. Approximately 5% of the laser energy reached the contralateral cochlea. Both bilateral and unilateral laser therapy decreased the hearing threshold after noise overstimulation in the rat model. The bilateral laser therapy group showed faster functional recovery at all tested frequencies compared with the unilateral laser therapy group. However, there was no difference in the endpoint ABR results or final hair cell survival, which was analyzed histologically.

Cochlear implantation in children and adults in Switzerland

OpenAIRE

Brand, Yves; Senn, Pascal; Kompis, Martin; Dillier, Norbert; Allum, John H. J.

2014-01-01

The cochlear implant (CI) is one of the most successful neural prostheses developed to date. It offers artificial hearing to individuals with profound sensorineural hearing loss and with insufficient benefit from conventional hearing aids. The first implants available some 30 years ago provided a limited sensation of sound. The benefit for users of these early systems was mostly a facilitation of lip-reading based communication rather than an understanding of speech. Considerable progress has...

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.

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.

Sensori-neural hearing loss in patients treated with irradiation for nasopharyngeal carcinoma

International Nuclear Information System (INIS)

Grau, C.; Moller, K.; Overgaard, M.; Overgaard, J.; Elbrond, O.

1991-01-01

The present investigation has been carried out to evaluate the sensitivity of the inner ear to irradiation. Cochlear function was tested in a cohort of 22 patients before and 7-84 months after receiving external irradiation for nasopharyngeal carcinoma. The pre-irradiation sensori-neural hearing threshold at 500, 1000, 2000, and 4000 Hz was used as a baseline for the individual patient, and the observed sensori-neural hearing loss (SNHL) was calculated as the difference between pre- and post-irradiation values. The pre-irradiation hearing level or patient age was not correlated with the actual SNHL. In contrast, there was a significant correlation between the total radiation dose to the inner ear and the observed hearing impairment. SNHL was most pronounced in the high frequencies, with values up to 35 dB (4000 Hz) and 25 dB (2000 Hz) in some patients. The latent period for the complication appeared to be 12 months or more. The deleterious effect of irradiation on the hearing should be kept in mind both in treatment planning and in the follow-up after radiotherapy

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

Science.gov (United States)

Most, Tova; Rothem, Hilla; Luntz, Michal

2009-01-01

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

Occupational hearing loss

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... this page: //medlineplus.gov/ency/article/001048.htm Occupational hearing loss To use the sharing features on this page, please enable JavaScript. Occupational hearing loss is damage to the inner ear from noise ...

Diffusion Tensor Imaging of Central Auditory Pathways in Patients with Sensorineural Hearing Loss: A Systematic Review.

Science.gov (United States)

Tarabichi, Osama; Kozin, Elliott D; Kanumuri, Vivek V; Barber, Samuel; Ghosh, Satra; Sitek, Kevin R; Reinshagen, Katherine; Herrmann, Barbara; Remenschneider, Aaron K; Lee, Daniel J

2018-03-01

Objective The radiologic evaluation of patients with hearing loss includes computed tomography and magnetic resonance imaging (MRI) to highlight temporal bone and cochlear nerve anatomy. The central auditory pathways are often not studied for routine clinical evaluation. Diffusion tensor imaging (DTI) is an emerging MRI-based modality that can reveal microstructural changes in white matter. In this systematic review, we summarize the value of DTI in the detection of structural changes of the central auditory pathways in patients with sensorineural hearing loss. Data Sources PubMed, Embase, and Cochrane. Review Methods We used the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement checklist for study design. All studies that included at least 1 sensorineural hearing loss patient with DTI outcome data were included. Results After inclusion and exclusion criteria were met, 20 articles were analyzed. Patients with bilateral hearing loss comprised 60.8% of all subjects. Patients with unilateral or progressive hearing loss and tinnitus made up the remaining studies. The auditory cortex and inferior colliculus (IC) were the most commonly studied regions using DTI, and most cases were found to have changes in diffusion metrics, such as fractional anisotropy, compared to normal hearing controls. Detectable changes in other auditory regions were reported, but there was a higher degree of variability. Conclusion White matter changes based on DTI metrics can be seen in patients with sensorineural hearing loss, but studies are few in number with modest sample sizes. Further standardization of DTI using a prospective study design with larger sample sizes is needed.

[Presbycusis - Age Related Hearing Loss].

Science.gov (United States)

Fischer, N; Weber, B; Riechelmann, H

2016-07-01

Presbycusis or age related hearing loss can be defined as a progressive, bilateral and symmetrical sensorineural hearing loss due to age related degeneration of inner ear structures. It can be considered a multifactorial complex disorder with environmental and genetic factors. The molecular, electrophysiological and histological damage at different levels of the inner ear cause a progressive hearing loss, which usually affects the high frequencies of hearing. The resulting poor speech recognition has a negative impact on cognitive, emotional and social function in older adults. Recent investigations revealed an association between hearing impairment and social isolation, anxiety, depression and cognitive decline in elderly. These findings emphasize the importance of diagnosis and treating hearing loss in the elderly population. Hearing aids are the most commonly used devices for treating presbycusis. The technical progress of implantable hearing devices allows an effective hearing rehabilitation even in elderly with severe hearing loss. However, most people with hearing impairments are not treated adequately. © Georg Thieme Verlag KG Stuttgart · New York.

Cardiac and renal dysfunction is associated with progressive hearing loss in patients with Fabry disease.

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Maria Köping

Full Text Available Fabry disease (FD is an X-linked recessive hereditary lysosomal storage disorder which results in the accumulation of globotriaosylceramid (Gb3 in tissues of kidney and heart as well as central and peripheral nervous system. Besides prominent renal and cardiac organ involvement, cochlear symptoms like high-frequency hearing loss and tinnitus are frequently found with yet no comprehensive data available in the literature.To examine hearing loss in patients with FD depending on cardiac and renal function.Single-center study with 68 FD patients enrolled between 2012 and 2016 at the Department of Oto-Rhino-Laryngology, Plastic, Aesthetic and Reconstructive Head and Neck Surgery of the University of Würzburg. Every subject underwent an oto-rhino-laryngological examination as well as behavioral, electrophysiological and electroacoustical audiological testing. High-frequency thresholds were evaluated by using a modified PTA6 (0.5, 1, 2, 4, 6, 8 and HF-PTA (6, 8 kHz. Renal function was measured by eGFR, cardiac impairment was graduated by NYHA class.Sensorineural hearing loss was detected in 58.8% of the cohort, which occurred typically in sudden episodes and affected especially high frequencies. Hearing loss is asymmetric, beginning unilaterally and affecting the contralateral ear later. Tinnitus was reported by 41.2%. Renal and cardiac impairment influenced the severity of hearing loss (p < 0.05.High frequency hearing loss is a common problem in patients with FD. Although not life-threatening, it can seriously reduce quality of life and should be taken into account in diagnosis and therapy. Optimized extensive hearing assessment including higher frequency thresholds should be used.

Musical background not associated with self-perceived hearing performance or speech perception in postlingual cochlear-implant users.

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Fuller, Christina; Free, Rolien; Maat, Bert; Başkent, Deniz

2012-08-01

In normal-hearing listeners, musical background has been observed to change the sound representation in the auditory system and produce enhanced performance in some speech perception tests. Based on these observations, it has been hypothesized that musical background can influence sound and speech perception, and as an extension also the quality of life, by cochlear-implant users. To test this hypothesis, this study explored musical background [using the Dutch Musical Background Questionnaire (DMBQ)], and self-perceived sound and speech perception and quality of life [using the Nijmegen Cochlear Implant Questionnaire (NCIQ) and the Speech Spatial and Qualities of Hearing Scale (SSQ)] in 98 postlingually deafened adult cochlear-implant recipients. In addition to self-perceived measures, speech perception scores (percentage of phonemes recognized in words presented in quiet) were obtained from patient records. The self-perceived hearing performance was associated with the objective speech perception. Forty-one respondents (44% of 94 respondents) indicated some form of formal musical training. Fifteen respondents (18% of 83 respondents) judged themselves as having musical training, experience, and knowledge. No association was observed between musical background (quantified by DMBQ), and self-perceived hearing-related performance or quality of life (quantified by NCIQ and SSQ), or speech perception in quiet.

Noise-Induced Hearing Loss

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... Home » Health Info » Hearing, Ear Infections, and Deafness Noise-Induced Hearing Loss On this page: What is ... I find additional information about NIHL? What is noise-induced hearing loss? Every day, we experience sound ...

Sensory-motor relationships in speech production in post-lingually deaf cochlear-implanted adults and normal-hearing seniors: Evidence from phonetic convergence and speech imitation.

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Scarbel, Lucie; Beautemps, Denis; Schwartz, Jean-Luc; Sato, Marc

2017-07-01

Speech communication can be viewed as an interactive process involving a functional coupling between sensory and motor systems. One striking example comes from phonetic convergence, when speakers automatically tend to mimic their interlocutor's speech during communicative interaction. The goal of this study was to investigate sensory-motor linkage in speech production in postlingually deaf cochlear implanted participants and normal hearing elderly adults through phonetic convergence and imitation. To this aim, two vowel production tasks, with or without instruction to imitate an acoustic vowel, were proposed to three groups of young adults with normal hearing, elderly adults with normal hearing and post-lingually deaf cochlear-implanted patients. Measure of the deviation of each participant's f 0 from their own mean f 0 was measured to evaluate the ability to converge to each acoustic target. showed that cochlear-implanted participants have the ability to converge to an acoustic target, both intentionally and unintentionally, albeit with a lower degree than young and elderly participants with normal hearing. By providing evidence for phonetic convergence and speech imitation, these results suggest that, as in young adults, perceptuo-motor relationships are efficient in elderly adults with normal hearing and that cochlear-implanted adults recovered significant perceptuo-motor abilities following cochlear implantation. Copyright © 2017 Elsevier Ltd. All rights reserved.

Outcomes of Children with Hearing Loss: Data Collection and Methods.

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Tomblin, J Bruce; Walker, Elizabeth A; McCreery, Ryan W; Arenas, Richard M; Harrison, Melody; Moeller, Mary Pat

2015-01-01

The primary objective of this article was to describe recruitment, data collection, and methods for a longitudinal, multicenter study involving children with bilateral mild to severe hearing loss. The goals of this research program were to characterize the developmental outcomes of children with mild to severe bilateral hearing loss during infancy and the preschool years. Furthermore, the researchers examined how these outcomes were associated with the child's hearing loss and how home background and clinical interventions mediated and moderated these outcomes. The participants in this study were children who are hard of hearing (CHH) and children with normal hearing (CNH) who provided comparison data. CHH were eligible for participation if (1) their chronological age was between 6 months and 7 years of age at the time of recruitment, (2) they had a better-ear pure-tone average of 25 to 75 dB HL, (3) they had not received a cochlear implant, (4) they were from homes where English was the primary language, and (5) they did not demonstrate significant cognitive or motor delays. Across the time span of recruitment, 430 parents of potential children with hearing loss made contact with the research group. This resulted in 317 CHH who qualified for enrollment. In addition, 117 CNH qualified for enrollment. An accelerated longitudinal design was used, in which multiple age cohorts were followed long enough to provide overlap. Specifically, children were recruited and enrolled continuously across an age span of 6.5 years and were followed for at least 3 years. This design allowed for tests of time (period) versus cohort age effects that could arise by changes in services and technology over time, yet still allowed for examination of important developmental relationships. The distribution of degree of hearing loss for the CHH showed that the majority of CHH had moderate or moderate-to-severe hearing losses, indicating that the sample undersampled children with mild HL. For

[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.

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.

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

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

observed for adults. An additional finding was that SMRT score was negatively correlated with age for adults with implants. Results from this study suggest that basic psychophysical capabilities of early implanted children and postlingually implanted adults differ when assessed in the sound field using their personal implant processors. Because spectral resolution does not improve with age for early implanted children, it seems likely that the sparse representation of the signal provided by a cochlear implant limits spectral resolution development. These results are supported by the finding that postlingually implanted adults, whose auditory systems matured before the onset of hearing loss, perform significantly better than early implanted children on the spectral resolution test.

Outcomes of 3-Year-Old Children With Hearing Loss and Different Types of Additional Disabilities

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Cupples, Linda

2014-01-01

This research investigated the speech, language, and functional auditory outcomes of 119 3-year-old children with hearing loss and additional disabilities. Outcomes were evaluated using direct assessment and caregiver report. Multiple regressions revealed that type of additional disability and level of maternal education were significant predictors of language outcomes. Poorer outcomes were achieved in a combined group of children with autism, cerebral palsy, and/or developmental delay (DD) (Group A), compared with children with vision or speech output impairments, syndromes not entailing DD, or medical disorders (Group B). Better outcomes were associated with higher levels of maternal education. The association between better language outcomes and earlier cochlear implant switch-on approached significance. Further regression analyses were conducted separately for children with different types of additional disabilities. Level of maternal education was the only significant predictor of outcomes for Group A children, whereas degree of hearing loss was the strongest predictor for children in Group B. The findings highlight the variable impact that different types of additional disabilities can have on language development in children with hearing loss. PMID:24150488

Sensorineural hearing loss in children.

LENUS (Irish Health Repository)

Wormald, R

2010-02-01

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

Phonological processes in the speech of school-age children with hearing loss: Comparisons with children with normal hearing.

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Asad, Areej Nimer; Purdy, Suzanne C; Ballard, Elaine; Fairgray, Liz; Bowen, Caroline

2018-04-27

In this descriptive study, phonological processes were examined in the speech of children aged 5;0-7;6 (years; months) with mild to profound hearing loss using hearing aids (HAs) and cochlear implants (CIs), in comparison to their peers. A second aim was to compare phonological processes of HA and CI users. Children with hearing loss (CWHL, N = 25) were compared to children with normal hearing (CWNH, N = 30) with similar age, gender, linguistic, and socioeconomic backgrounds. Speech samples obtained from a list of 88 words, derived from three standardized speech tests, were analyzed using the CASALA (Computer Aided Speech and Language Analysis) program to evaluate participants' phonological systems, based on lax (a process appeared at least twice in the speech of at least two children) and strict (a process appeared at least five times in the speech of at least two children) counting criteria. Developmental phonological processes were eliminated in the speech of younger and older CWNH while eleven developmental phonological processes persisted in the speech of both age groups of CWHL. CWHL showed a similar trend of age of elimination to CWNH, but at a slower rate. Children with HAs and CIs produced similar phonological processes. Final consonant deletion, weak syllable deletion, backing, and glottal replacement were present in the speech of HA users, affecting their overall speech intelligibility. Developmental and non-developmental phonological processes persist in the speech of children with mild to profound hearing loss compared to their peers with typical hearing. The findings indicate that it is important for clinicians to consider phonological assessment in pre-school CWHL and the use of evidence-based speech therapy in order to reduce non-developmental and non-age-appropriate developmental processes, thereby enhancing their speech intelligibility. Copyright © 2018 Elsevier Inc. All rights reserved.

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, 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.

Hearing: Noise-Induced Hearing Loss

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... reduce your exposure to noise by choosing quiet leisure activities rather than noisy ones. Develop the habit ... the degree of your hearing loss, you may benefit from using a hearing aid (a device you ...

Individual Hearing Loss

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Sébastien Santurette

2016-06-01

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

Unilateral hearing loss in children: a retrospective study and a review of the current literature.

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Rohlfs, Anna-Katharina; Friedhoff, Johannes; Bohnert, Andrea; Breitfuss, Achim; Hess, Markus; Müller, Frank; Strauch, Anke; Röhrs, Marianne; Wiesner, Thomas

2017-04-01

Despite the introduction of universal newborn hearing screening (UNHS), unilateral hearing loss (UHL) is sometimes recognized late. This diagnostic delay has adverse repercussions, given the importance of binaural hearing for the development of normal auditory processing. It is incorrect to maintain that unilateral hearing is the minimum requirement for adequate speech development and that hearing aid provision is consequently unnecessary. In our retrospective study, hearing aid provision resulted in improved directional and selective hearing (quiet and noisy environments) and, compared with their chronically ill counterparts, the children in our study displayed superior health-related quality of life (HRQoL) scores in all areas. On the basis of the results, the authors conclude that even mild hearing losses (from an auditory threshold of 30 to 40 dB) should have the opportunity for hearing aid provision. A selective literature review was conducted in PubMed and textbooks and with reference to national and international guidelines. Early diagnosis and treatment of UHL have a positive effect on verbal-cognitive, linguistic, communicative, and socio-emotional development, as demonstrated by neurophysiological studies. Among the treatment modalities with differing effects on the quality of binaural hearing, cochlear implants are now used increasingly in children with hearing loss bordering on deafness. Published evidence and clinical experience support early diagnosis and treatment. Wherever feasible, hearing aid provision before or at the end of the first year of life is recommended for children with UHL. What is Known: • Almost 30 years ago, poor academic performance was reported in children with unilateral hearing loss (UHL). • Despite improvements in treatment options, it is traditionally held that unilateral hearing is the minimum requirement for adequate speech development and hearing aid provision is unnecessary. What is New: • Academic and behavioral

Hereditary Hearing Loss.

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Tran, LenhAnh P.; Grundfast, Kenneth M.

1997-01-01

This article discusses inheritance patterns in hearing loss, epidemiology, clues to genetic causes, locating genes that cause hereditary disorders, genes related to hearing loss disorders in individuals with Usher syndrome, Waardenburg syndrome, Treacher-Collins syndrome, Branchio-oto-renal and Pendred syndromes, and the significance of finding…

Hearing loss associated with enlarged vestibular aqueduct and zero or one mutant allele of SLC26A4.

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Rose, Jane; Muskett, Julie A; King, Kelly A; Zalewski, Christopher K; Chattaraj, Parna; Butman, John A; Kenna, Margaret A; Chien, Wade W; Brewer, Carmen C; Griffith, Andrew J

2017-07-01

To characterize the severity and natural history of hearing loss, and the prevalence of having a cochlear implant in a maturing cohort of individuals with enlarged vestibular aqueduct (EVA) and zero or one mutant allele of SLC26A4. Prospective cohort study of subjects ascertained between 1998 and 2015 at the National Institutes of Health Clinical Center. Study subjects were 127 individuals (median age, 8 years; range, 0-59 years) with EVA in at least one ear. Ears with EVA and zero or one mutant allele of SLC26A4 had mean 0.5/1/2/4-kHz pure-tone averages of 62.6 and 52.9 dB HL, respectively, in contrast to EVA ears with two mutant alleles of SLC26A4 (88.1 dB HL; P zero, one, and two mutant alleles, respectively (P = .00833). This association was not independent (P = .534) but reflected underlying correlations with age at time of first audiogram (P = .003) or severity of hearing loss (P = .000). Ears with EVA and zero or one mutant allele of SLC26A4 have less severe hearing loss, no difference in prevalence of fluctuation, and a lower prevalence of cochlear implantation in comparison to ears with two mutant alleles of SLC26A4. NA Laryngoscope, 127:E238-E243, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

Direct Reprogramming of Spiral Ganglion Non-neuronal Cells into Neurons: Toward Ameliorating Sensorineural Hearing Loss by Gene Therapy

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Teppei Noda

2018-02-01

Full Text Available Primary auditory neurons (PANs play a critical role in hearing by transmitting sound information from the inner ear to the brain. Their progressive degeneration is associated with excessive noise, disease and aging. The loss of PANs leads to permanent hearing impairment since they are incapable of regenerating. Spiral ganglion non-neuronal cells (SGNNCs, comprised mainly of glia, are resident within the modiolus and continue to survive after PAN loss. These attributes make SGNNCs an excellent target for replacing damaged PANs through cellular reprogramming. We used the neurogenic pioneer transcription factor Ascl1 and the auditory neuron differentiation factor NeuroD1 to reprogram SGNNCs into induced neurons (iNs. The overexpression of both Ascl1 and NeuroD1 in vitro generated iNs at high efficiency. Transcriptome analyses revealed that iNs displayed a transcriptome profile resembling that of endogenous PANs, including expression of several key markers of neuronal identity: Tubb3, Map2, Prph, Snap25, and Prox1. Pathway analyses indicated that essential pathways in neuronal growth and maturation were activated in cells upon neuronal induction. Furthermore, iNs extended projections toward cochlear hair cells and cochlear nucleus neurons when cultured with each respective tissue. Taken together, our study demonstrates that PAN-like neurons can be generated from endogenous SGNNCs. This work suggests that gene therapy can be a viable strategy to treat sensorineural hearing loss caused by degeneration of PANs.

Cochlear implantation in Mondini dysplasia.

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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

Modeling and segmentation of intra-cochlear anatomy in conventional CT

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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.

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...

Acoustic reflex measurements and the loudness function in sensorineural hearing loss

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Sheila Uliel

1980-11-01

Full Text Available The suprathreshold acoustic reflex responses of forty two ears affected by sensorineural hearing loss of cochlear origin and fifty-eight ears demonstrating normal hearing, were recorded by means of an electro-acoustic impedance meter and attached X-Y recorder. The recordings were done in ascending and descending fashion,  at successively increasing and decreasing 5dB intensity levels from 90-120-90 dB HL respectively, for the individual pure-tone frequencies of 500, 1 000, 2 000 and 4 000 Hz. The contralateral mode of measurement was employed. Analysis of  these recordings indicated that the acoustic reflex  responses could be differentiated into five  characteristic patterns of  growth, which could be depicted upon a continuum of peaked, peaked-rounded, rounded, rounded-flat,  and flat  shapes. The peaked and peaked-rounded patterns were found  to predominate at all four pure-tone frequencies  in the normal ears, while the rounded-fiat  and flat  patterns were found  to predominate only at the higher pure-tone frequencies of 2 000 and 4 000 Hz in the ears affected  by sensorineural hearing loss. This latter relationship was also able to be applied to two disorders of  the loudness functio— loudness recruitment and hyperacusis. It was concluded that the flattened  acoustic reflex  patterns at the higher pure-tone frequencies  constituted a potential diagnostic cue related to the differential  diagnosis of sensorineural hearing loss, and to disorders of  the loudness function.

The benefits of Square dancing as a means of physical activity for Czech dancers with hearing loss

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Petra Kurková

2014-12-01

Full Text Available Background: Hearing, a strong line of communication that enables individuals to learn about the world around them, is a major factor contributing to the psychomotor development of every individual. Hearing loss can also affect the conception and perception of sounds and rhythm. Objective: The purpose of this study was to describe and analyse the benefits of Square and Round dancing for persons with hearing loss. Methods: The present study is an analytic-descriptive qualitative research. The sample was constituted non-probabilistically based on the following features: a a participant had to have hearing problems (hearing loss and b had to have participated regularly in Square dance for at least two years. Each participant was asked to name possible people to be interviewed (snowball technique. We analysed the data of 7 individuals (6 males and 1 female with hearing loss. The mean age of the dancers with hearing loss was 51.3 years. The participants had no cochlear implants or any other physical or vision related impairments. Results: The present findings constitute the first published survey regarding Czech Square dancers' status, their family's hearing status, hearing aid use, communication preference, education in integrated or segregated settings, the influence of family background on dance initiation, coach preference (hearing or deaf, and the environment for participation in Square dance as a mode of physical activity with regular dancers and with dancers with hearing loss as well. In the present sample of dancers with hearing loss, most were from hearing families and had hearing siblings. The degree to which individuals with hearing loss feel comfortable with the hearing world appears to influence their later preference for participating in regular, as opposed to segregated, physical activities. More than half of the dancers with hearing loss who participated in this research study would like to meet with the deaf minority. One of the

Audiological outcomes of cochlear implantation in Waardenburg Syndrome

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Magalhães, Ana Tereza de Matos

2014-01-01

Full Text Available Introduction: The most relevant clinical symptom in Waardenburg syndrome is profound bilateral sensorioneural hearing loss. Aim: To characterize and describe hearing outcomes after cochlear implantation in patients with Waardenburg syndrome to improve preoperative expectations. Method: This was an observational and retrospective study of a series of cases. Children who were diagnosed with Waardenburg syndrome and who received a multichannel cochlear implant between March 1999 and July 2012 were included in the study. Intraoperative neural response telemetry, hearing evaluation, speech perception, and speech production data before and after surgery were assessed. Results: During this period, 806 patients received a cochlear implant and 10 of these (1.2% were diagnosed with Waardenburg syndrome. Eight of the children received a Nucleus 24® implant and 1 child and 1 adult received a DigiSonic SP implant. The mean age at implantation was 44 months among the children. The average duration of use of a cochlear implant at the time of the study was 43 months. Intraoperative neural responses were present in all cases. Patients who could use the speech processor effectively had a pure tone average of 31 dB in free-field conditions. In addition, the MUSS and MAIS questionnaires revealed improvements in speech perception and production. Four patients did not have a good outcome, which might have been associated with ineffective use of the speech processor. Conclusion: Despite the heterogeneity of the group, patients with Waardenburg syndrome who received cochlear implants were found to have hearing thresholds that allowed access to speech sounds. However, patients who received early intervention and rehabilitation showed better evolution of auditory perception.

Audiological outcomes of cochlear implantation in Waardenburg Syndrome.

Science.gov (United States)

Magalhães, Ana Tereza de Matos; Samuel, Paola Angélica; Goffi-Gomez, Maria Valeria Schimdt; Tsuji, Robinson Koji; Brito, Rubens; Bento, Ricardo Ferreira

2013-07-01

 The most relevant clinical symptom in Waardenburg syndrome is profound bilateral sensorioneural hearing loss.  To characterize and describe hearing outcomes after cochlear implantation in patients with Waardenburg syndrome to improve preoperative expectations.  This was an observational and retrospective study of a series of cases. Children who were diagnosed with Waardenburg syndrome and who received a multichannel cochlear implant between March 1999 and July 2012 were included in the study. Intraoperative neural response telemetry, hearing evaluation, speech perception, and speech production data before and after surgery were assessed.  During this period, 806 patients received a cochlear implant and 10 of these (1.2%) were diagnosed with Waardenburg syndrome. Eight of the children received a Nucleus 24(®) implant and 1 child and 1 adult received a DigiSonic SP implant. The mean age at implantation was 44 months among the children. The average duration of use of a cochlear implant at the time of the study was 43 months. Intraoperative neural responses were present in all cases. Patients who could use the speech processor effectively had a pure tone average of 31 dB in free-field conditions. In addition, the MUSS and MAIS questionnaires revealed improvements in speech perception and production. Four patients did not have a good outcome, which might have been associated with ineffective use of the speech processor.  Despite the heterogeneity of the group, patients with Waardenburg syndrome who received cochlear implants were found to have hearing thresholds that allowed access to speech sounds. However, patients who received early intervention and rehabilitation showed better evolution of auditory perception.

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)

Characteristics and application of inner ear CT in 20 cases of sensorineural hearing loss in children.

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Huo, Liang; Wang, Hua

2012-12-01

This study shows that a number of children with sensorineural hearing loss (SNHL) have inner ear malformations demonstrated by multi-slice spiral computed tomography (MSCT). MSCT allows a comprehensive assessment of various congenital inner ear malformations through high quality multiplanar reformation (MPR) and can display the site and degree of the malformation three-dimensionally and intuitively. This is very useful for cochlear implantation. To evaluate the feasibility and usability of MSCT in pediatric SNHL with inner ear malformations. Sixty-five patients were diagnosed with SNHL by brainstem auditory evoked response (BAER). Inner ear MSCT scan and coronal MPR reconstruction were performed in all cases. This study demonstrated 20 cases (33 ears) with inner ear malformations, which included 10 ears with cochlear malformations, 7 with vestibular malformations, 5 with semicircular canal malformations, 8 with internal auditory canal (IAC) malformations, and 15 with vestibular malformations. Cochlear malformations included one ear with Michel deformity, two ears with common cavity deformity, one with cochlear aplasia, one with hypoplastic cochlea, two with incomplete partition type I, and three with incomplete partition type II (Mondini deformity).

Disruption of ion-trafficking system in the cochlear spiral ligament prior to permanent hearing loss induced by exposure to intense noise: possible involvement of 4-hydroxy-2-nonenal as a mediator of oxidative stress.

Directory of Open Access Journals (Sweden)

Taro Yamaguchi

Full Text Available Noise-induced hearing loss is at least in part due to disruption of endocochlear potential, which is maintained by various K(+ transport apparatuses including Na(+, K(+-ATPase and gap junction-mediated intercellular communication in the lateral wall structures. In this study, we examined the changes in the ion-trafficking-related proteins in the spiral ligament fibrocytes (SLFs following in vivo acoustic overstimulation or in vitro exposure of cultured SLFs to 4-hydroxy-2-nonenal, which is a mediator of oxidative stress. Connexin (Cx26 and Cx30 were ubiquitously expressed throughout the spiral ligament, whereas Na(+, K(+-ATPase α1 was predominantly detected in the stria vascularis and spiral prominence (type 2 SLFs. One-hour exposure of mice to 8 kHz octave band noise at a 110 dB sound pressure level produced an immediate and prolonged decrease in the Cx26 expression level and in Na+, K(+-ATPase activity, as well as a delayed decrease in Cx30 expression in the SLFs. The noise-induced hearing loss and decrease in the Cx26 protein level and Na(+, K(+-ATPase activity were abolished by a systemic treatment with a free radical-scavenging agent, 4-hydroxy-2,2,6,6-tetramethylpiperidine 1-oxyl, or with a nitric oxide synthase inhibitor, N(ω-nitro-L-arginine methyl ester hydrochloride. In vitro exposure of SLFs in primary culture to 4-hydroxy-2-nonenal produced a decrease in the protein levels of Cx26 and Na(+, K(+-ATPase α1, as well as Na(+, K(+-ATPase activity, and also resulted in dysfunction of the intercellular communication between the SLFs. Taken together, our data suggest that disruption of the ion-trafficking system in the cochlear SLFs is caused by the decrease in Cxs level and Na(+, K(+-ATPase activity, and at least in part involved in permanent hearing loss induced by intense noise. Oxidative stress-mediated products might contribute to the decrease in Cxs content and Na(+, K(+-ATPase activity in the cochlear lateral wall structures.

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

African Journals Online (AJOL)

quality of life of families with children who have a hearing loss or who are fitted with cochlear implants. .... term going into the work force with a severe disability will he be .... balance between professional caution and urgency of treatment.

Asymmetric hearing loss in a random population of patients with mild to moderate sensorineural hearing loss.

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Segal, Nili; Shkolnik, Mark; Kochba, Anat; Segal, Avichai; Kraus, Mordechai

2007-01-01

We evaluated the correlation of asymmetric hearing loss, in a random population of patients with mild to moderate sensorineural hearing loss, to several clinical factors such as age, sex, handedness, and noise exposure. We randomly selected, from 8 hearing institutes in Israel, 429 patients with sensorineural hearing loss of at least 30 dB at one frequency and a speech reception threshold not exceeding 30 dB. Patients with middle ear disease or retrocochlear disorders were excluded. The results of audiometric examinations were compared binaurally and in relation to the selected factors. The left ear's hearing threshold level was significantly higher than that of the right ear at all frequencies except 1.0 kHz (p < .05). One hundred fifty patients (35%) had asymmetric hearing loss (more than 10 dB difference between ears). In most of the patients (85%) the binaural difference in hearing threshold level, at any frequency, was less than 20 dB. Age, handedness, and sex were not found to be correlated to asymmetric hearing loss. Noise exposure was found to be correlated to asymmetric hearing loss.

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 ...

Mutations in Cockayne Syndrome-Associated Genes (Csa and Csb) Predispose to Cisplatin-Induced Hearing Loss in Mice

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Rainey, Robert N.; Ng, Sum-yan; Llamas, Juan; van der Horst, Gijsbertus T. J.

2016-01-01

Cisplatin is a common and effective chemotherapeutic agent, yet it often causes permanent hearing loss as a result of sensory hair cell death. The causes of sensitivity to DNA-damaging agents in nondividing cell populations, such as cochlear hair and supporting cells, are poorly understood, as are the specific DNA repair pathways that protect these cells. Nucleotide excision repair (NER) is a conserved and versatile DNA repair pathway for many DNA-distorting lesions, including cisplatin-DNA adducts. Progressive sensorineural hearing loss is observed in a subset of NER-associated DNA repair disorders including Cockayne syndrome and some forms of xeroderma pigmentosum. We investigated whether either of the two overlapping branches that encompass NER, transcription-coupled repair or global genome repair, which are implicated in Cockayne syndrome and xeroderma pigmentosum group C, respectively, modulates cisplatin-induced hearing loss and cell death in the organ of Corti, the auditory sensory epithelium of mammals. We report that cochlear hair cells and supporting cells in transcription-coupled repair-deficient Cockayne syndrome group A (Csa−/−) and group B (Csb−/−) mice are hypersensitive to cisplatin, in contrast to global genome repair-deficient Xpc−/− mice, both in vitro and in vivo. We show that sensory hair cells in Csa−/− and Csb−/− mice fail to remove cisplatin-DNA adducts efficiently in vitro; and unlike Xpc−/− mice, Csa−/− and Csb−/− mice lose hearing and manifest outer hair cell degeneration after systemic cisplatin treatment. Our results demonstrate that Csa and Csb deficiencies predispose to cisplatin-induced hearing loss and hair/supporting cell damage in the mammalian organ of Corti, and emphasize the importance of transcription-coupled DNA repair in the protection against cisplatin ototoxicity. SIGNIFICANCE STATEMENT The utility of cisplatin in chemotherapy remains limited due to serious side effects, including

Sign Language and Spoken Language for Children With Hearing Loss: A Systematic Review.

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Fitzpatrick, Elizabeth M; Hamel, Candyce; Stevens, Adrienne; Pratt, Misty; Moher, David; Doucet, Suzanne P; Neuss, Deirdre; Bernstein, Anita; Na, Eunjung

2016-01-01

Permanent hearing loss affects 1 to 3 per 1000 children and interferes with typical communication development. Early detection through newborn hearing screening and hearing technology provide most children with the option of spoken language acquisition. However, no consensus exists on optimal interventions for spoken language development. To conduct a systematic review of the effectiveness of early sign and oral language intervention compared with oral language intervention only for children with permanent hearing loss. An a priori protocol was developed. Electronic databases (eg, Medline, Embase, CINAHL) from 1995 to June 2013 and gray literature sources were searched. Studies in English and French were included. Two reviewers screened potentially relevant articles. Outcomes of interest were measures of auditory, vocabulary, language, and speech production skills. All data collection and risk of bias assessments were completed and then verified by a second person. Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) was used to judge the strength of evidence. Eleven cohort studies met inclusion criteria, of which 8 included only children with severe to profound hearing loss with cochlear implants. Language development was the most frequently reported outcome. Other reported outcomes included speech and speech perception. Several measures and metrics were reported across studies, and descriptions of interventions were sometimes unclear. Very limited, and hence insufficient, high-quality evidence exists to determine whether sign language in combination with oral language is more effective than oral language therapy alone. More research is needed to supplement the evidence base. Copyright © 2016 by the American Academy of Pediatrics.

Genes and Hearing Loss

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... ENTCareers Marketplace Find an ENT Doctor Near You Genes and Hearing Loss Genes and Hearing Loss Patient ... mutation may only have dystopia canthorum. How Do Genes Work? Genes are a road map for the ...

Cochlear Implants

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... 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 ...

OI Issues: Hearing Loss

Science.gov (United States)

... signals normally to the brain. In addition, hearing losses are classified according to the degree of severity: • Mild, • Moderate, • Severe, • Profound. Hearing losses are also classified according to the sound frequency ...

Audiological and surgical evidence for the presence of a third window effect for the conductive hearing loss in DFNX2 deafness irrespective of types of mutations.

Science.gov (United States)

Choi, Byung Yoon; An, Yong-Hwi; Park, Joo Hyun; Jang, Jeong Hun; Chung, Hyun Chung; Kim, Ah-Reum; Lee, Jun Ho; Kim, Chong-Sun; Oh, Seung Ha; Chang, Sun O

2013-11-01

The objective of this study was to clarify the cause of the air-bone gap in incomplete partition (IP) type III cases according to the POU3F4 gene (DFNX2) mutation type. A retrospective analysis of patient medical records was done in a tertiary referral medical center. Five IP type III patients proved to be carrying a mutation in or affecting POU3F4. The hearing and the middle ear status at either exploratory tympanotomy or cochlear implantation from these DFNX2 cases was reviewed. Four of five unrelated IP type III patients harbored a point mutation of POU3F4 and the fifth patient carried a large genomic deletion upstream to POU3F4. Two of the four DFNX2 patients carrying a point mutation had moderate to severe mixed hearing loss with a substantial amount of air-bone gap. These patients underwent exploratory tympanotomy to identify the cause of their hearing loss. The other three patients, including one carrying a large deletion, had profound hearing loss at presentation and received a cochlear implant. In the exploratory tympanotomy group with a substantial amount of air-bone gap and a point mutation (n = 2), one patient had a perfect ossicular chain with normal mobility, a positive ipsilateral stapedial reflex, and a positive round window reflex. In the cochlear implantation group (n = 3), we found a stapes with normal mobility and a positive round window reflex in one patient who harbored a large genomic deletion upstream to POU3F4. We concluded that the probable presence of the third window effect is not limited to the particular type of POU3F4 mutation.

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

Directory of Open Access Journals (Sweden)

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

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...

Age-related hearing loss

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... grow older. Your genes and loud noise (from rock concerts or music headphones) may play a large role. The following factors contribute to age-related hearing loss: Family history (age-related hearing loss tends to run in ...

The use of auditory and visual context in speech perception by listeners with normal hearing and listeners with cochlear implants

Directory of Open Access Journals (Sweden)

Matthew eWinn

2013-11-01

Full Text Available There is a wide range of acoustic and visual variability across different talkers and different speaking contexts. Listeners with normal hearing accommodate that variability in ways that facilitate efficient perception, but it is not known whether listeners with cochlear implants can do the same. In this study, listeners with normal hearing (NH and listeners with cochlear implants (CIs were tested for accommodation to auditory and visual phonetic contexts created by gender-driven speech differences as well as vowel coarticulation and lip rounding in both consonants and vowels. Accommodation was measured as the shifting of perceptual boundaries between /s/ and /ʃ/ sounds in various contexts, as modeled by mixed-effects logistic regression. Owing to the spectral contrasts thought to underlie these context effects, CI listeners were predicted to perform poorly, but showed considerable success. Listeners with cochlear implants not only showed sensitivity to auditory cues to gender, they were also able to use visual cues to gender (i.e. faces as a supplement or proxy for information in the acoustic domain, in a pattern that was not observed for listeners with normal hearing. Spectrally-degraded stimuli heard by listeners with normal hearing generally did not elicit strong context effects, underscoring the limitations of noise vocoders and/or the importance of experience with electric hearing. Visual cues for consonant lip rounding and vowel lip rounding were perceived in a manner consistent with coarticulation and were generally used more heavily by listeners with CIs. Results suggest that listeners with cochlear implants are able to accommodate various sources of acoustic variability either by attending to appropriate acoustic cues or by inferring them via the visual signal.

Risk Factors for Hearing Loss in Patients Treated With Intensity-Modulated Radiotherapy for Head-and-Neck Tumors

International Nuclear Information System (INIS)

Zuur, Charlotte L.; Simis, Yvonne J.; Lamers, Emmy A.; Hart, Augustinus A.; Dreschler, Wouter A.; Balm, Alfons J.; Rasch, Coen R.

2009-01-01

Purpose: Radiotherapy (RT) is a common treatment of head-and-neck carcinoma. The objective of this study was to perform a prospective multivariate assessment of the dose-effect relationship between intensity-modulated RT and hearing loss. Methods and Materials: Pure tone audiometry at 0.250-16 kHz was obtained before and after treatment in 101 patients (202 ears). All patients received full-course intensity-modulated RT (range, 56-70 Gy), with a median cochlear dose of 11.4 Gy (range, 0.2-69.7). Results: Audiometry was performed 1 week before and a median of 9 weeks (range, 1-112) after treatment. The mean hearing deterioration at pure tone average air-conduction 1-2-4 kHz was small (from 28.6 dB HL to 30.1 dB HL). However, individual patients showed clinically significant hearing loss, with 10-dB threshold shift incidences of 13% and 18% at pure tone averages air-conduction 1-2-4 kHz and 8-10-12.5 kHz, respectively. Post-treatment hearing capability was unfavorable in the case of greater inner ear radiation doses (p <0.0001), unfavorable baseline hearing capability (p <0.0001), green-eyed patients (p <0.0001), and older age (p <0.0001). Using multivariate analysis, a prediction of individual hearing capabiltity after treatment was made. Conclusion: RT-induced hearing loss in the mean population is modest. However, clinically significant hearing loss was observed in older patients with green eyes and unfavorable pretreatment hearing. In these patients, the intended radiation dose may be adjusted according to the proposed predictive model, aiming to decrease the risk of ototoxicity.

The role of spectral and temporal cues in voice gender discrimination by normal-hearing listeners and cochlear implant users.

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Fu, Qian-Jie; Chinchilla, Sherol; Galvin, John J

2004-09-01

The present study investigated the relative importance of temporal and spectral cues in voice gender discrimination and vowel recognition by normal-hearing subjects listening to an acoustic simulation of cochlear implant speech processing and by cochlear implant users. In the simulation, the number of speech processing channels ranged from 4 to 32, thereby varying the spectral resolution; the cutoff frequencies of the channels' envelope filters ranged from 20 to 320 Hz, thereby manipulating the available temporal cues. For normal-hearing subjects, results showed that both voice gender discrimination and vowel recognition scores improved as the number of spectral channels was increased. When only 4 spectral channels were available, voice gender discrimination significantly improved as the envelope filter cutoff frequency was increased from 20 to 320 Hz. For all spectral conditions, increasing the amount of temporal information had no significant effect on vowel recognition. Both voice gender discrimination and vowel recognition scores were highly variable among implant users. The performance of cochlear implant listeners was similar to that of normal-hearing subjects listening to comparable speech processing (4-8 spectral channels). The results suggest that both spectral and temporal cues contribute to voice gender discrimination and that temporal cues are especially important for cochlear implant users to identify the voice gender when there is reduced spectral resolution.

Alteration of frequency range for binaural beats in acute low-tone hearing loss.

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Karino, Shotaro; Yamasoba, Tatsuya; Ito, Ken; Kaga, Kimitaka

2005-01-01

The effect of acute low-tone sensorineural hearing loss (ALHL) on the interaural frequency difference (IFD) required for perception of binaural beats (BBs) was investigated in 12 patients with unilateral ALHL and 7 patients in whom ALHL had lessened. A continuous pure tone of 30 dB sensation level at 250 Hz was presented to the contralateral, normal-hearing ear. The presence of BBs was determined by a subjective yes-no procedure as the frequency of a loudness-balanced test tone was gradually adjusted around 250 Hz in the affected ear. The frequency range in which no BBs were perceived (FRNB) was significantly wider in the patients with ALHL than in the controls, and FRNBs became narrower in the recovered ALHL group. Specifically, detection of slow BBs with a small IFD was impaired in this limited (10 s) observation period. The significant correlation between the hearing level at 250 Hz and FRNBs suggests that FRNBs represent the degree of cochlear damage caused by ALHL.

Effects of Clinician-Guided Emergent Literacy Intervention Using Interactive Tablet Technology for Preschool Children with Cochlear Implants

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Brouwer, Kyle; Downing, Hannah; Westhoff, Sara; Wait, Ryann; Entwisle, Lavin K.; Messersmith, Jessica J.; Hanson, Elizabeth K.

2017-01-01

The purpose of this study was to investigate if intervention based on a mobile application would improve the print knowledge and vocabulary of preschool children with and without hearing loss. This was a multiple baseline study that included four preschool children. Two of the children had hearing loss and utilized cochlear implants, while the…

Strategies for Working with Children with Cochlear Implants

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Schraer-Joiner, Lyn; Prause-Weber, Manuela

2009-01-01

According to the National Institute on Deafness and Other Communication Disorders, 23,000 individuals in the United States, including 10,000 children, have a cochlear implant. This biomedical electronic device has been a breakthrough in the auditory rehabilitation of individuals diagnosed with severe or profound sensorineural hearing losses who…

Recovery of Abnormal ABR in Neonates and Infants at Risk of Hearing Loss.

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Psarommatis, Ioannis; Voudouris, Charalampos; Kapetanakis, Ioannis; Athanasiadi, Faselida; Douros, Konstantinos

2017-01-01

The purpose of this retrospective study is to present the clinical experience of a single institution on the recovery of ABR thresholds in a large population of neonates and infants at risk of hearing loss. Potential prognostic factors associated with this phenomenon were also investigated. Out of 2248 high risk infants, 384 had abnormal ABR at initial hearing evaluation and 168 of them had absent ABR or a threshold ≥80 dBnHL. From this subgroup, a significant percentage showed complete or partial recovery on reexamination (32.7% and 9.3%, resp.), performed 4-6 months later. The presence of normal otoacoustic emissions was associated with the ABR restoration on reexamination. Moreover, the very young age at the initial hearing screening seems to be related to higher probabilities of false positive ABR. The potential recovery of hearing in HR infants raises concerns about the very early cochlear implantation in HR infants less than one year. Such a treatment modality should be decided cautiously and only after obtaining valid and stable objective and subjective hearing thresholds. This holds especially true for infants showing an auditory neuropathy profile, as they presented a much greater probability of ABR recovery.

Disproportionate Cochlear Length in Genus Homo Shows a High Phylogenetic Signal during Apes’ Hearing Evolution

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Braga, J.; Loubes, J-M.; Descouens, D.; Dumoncel, J.; Thackeray, J. F.; Kahn, J-L.; de Beer, F.; Riberon, A.; Hoffman, K.; Balaresque, P.; Gilissen, E.

2015-01-01

Changes in lifestyles and body weight affected mammal life-history evolution but little is known about how they shaped species’ sensory systems. Since auditory sensitivity impacts communication tasks and environmental acoustic awareness, it may have represented a deciding factor during mammal evolution, including apes. Here, we statistically measure the influence of phylogeny and allometry on the variation of five cochlear morphological features associated with hearing capacities across 22 living and 5 fossil catarrhine species. We find high phylogenetic signals for absolute and relative cochlear length only. Comparisons between fossil cochleae and reconstructed ape ancestral morphotypes show that Australopithecus absolute and relative cochlear lengths are explicable by phylogeny and concordant with the hypothetized ((Pan,Homo),Gorilla) and (Pan,Homo) most recent common ancestors. Conversely, deviations of the Paranthropus oval window area from these most recent common ancestors are not explicable by phylogeny and body weight alone, but suggest instead rapid evolutionary changes (directional selection) of its hearing organ. Premodern (Homo erectus) and modern human cochleae set apart from living non-human catarrhines and australopiths. They show cochlear relative lengths and oval window areas larger than expected for their body mass, two features corresponding to increased low-frequency sensitivity more recent than 2 million years ago. The uniqueness of the “hypertrophied” cochlea in the genus Homo (as opposed to the australopiths) and the significantly high phylogenetic signal of this organ among apes indicate its usefulness to identify homologies and monophyletic groups in the hominid fossil record. PMID:26083484

Intratympanic steroid prevents long-term spiral ganglion neuron loss in experimental meningitis

DEFF Research Database (Denmark)

Worsøe, Lise Lotte; Brandt, C.T.; Lund, S.P.

2010-01-01

Hypothesis: Intratympanic steroid treatment prevents hearing loss and cochlear damage in a rat model of pneumococcal meningitis. Background: Sensorineural hearing loss is a long-term complication of meningitis affecting up to a third of survivors. Streptococcus pneumoniae is the bacterial species...... for 3 days. Hearing loss and cochlear damage were assessed by distortion product otoacoustic emissions, auditory brainstem response at 16 kHz, and spiral ganglion neuron density. Results: Fifty-six days after infection, auditory brainstem response showed no significant differences between groups...... in the spiral ganglion compared with both intratympanic and systemic saline (p = 0.0082 and p = 0.0089; Mann-Whitney test). Histology revealed fibrosis of the tympanic membrane and cavity in steroid-treated animals, which plausibly caused the low-frequency hearing loss. Conclusion: Intratympanic betamethasone...

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

Hearing loss at work? Hearing loss from leisure activities?

CERN Multimedia

2006-01-01

The nurses of the Medical Service would like invite all persons working on the CERN site to take part in a: HEARING LOSS DETECTION WEEK From 28 August to 1st September 2006 At the Infirmary, Building 57 from 9 a.m. to 4 p.m. Hearing tests - advice - information - documentation - protective equipment

Finite Verb Morphology in the Spontaneous Speech of Dutch-Speaking Children With Hearing Loss.

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Hammer, Annemiek; Coene, Martine

2016-01-01

In this study, the acquisition of Dutch finite verb morphology is investigated in children with cochlear implants (CIs) with profound hearing loss and in children with hearing aids (HAs) with moderate to severe hearing loss. Comparing these two groups of children increases our insight into how hearing experience and audibility affect the acquisition of morphosyntax. Spontaneous speech samples were analyzed of 48 children with CIs and 29 children with HAs, ages 4 to 7 years. These language samples were analyzed by means of standardized language analysis involving mean length of utterance, the number of finite verbs produced, and target-like subject-verb agreement. The outcomes were interpreted relative to expectations based on the performance of typically developing peers with normal hearing. Outcomes of all measures were correlated with hearing level in the group of HA users and age at implantation in the group of CI users. For both groups, the number of finite verbs that were produced in 50-utterance sample was on par with mean length of utterance and at the lower bound of the normal distribution. No significant differences were found between children with CIs and HAs on any of the measures under investigation. Yet, both groups produced more subject-verb agreement errors than are to be expected for typically developing hearing peers. No significant correlation was found between the hearing level of the children and the relevant measures of verb morphology, both with respect to the overall number of verbs that were used and the number of errors that children made. Within the group of CI users, the outcomes were significantly correlated with age at implantation. When producing finite verb morphology, profoundly deaf children wearing CIs perform similarly to their peers with moderate-to-severe hearing loss wearing HAs. Hearing loss negatively affects the acquisition of subject-verb agreement regardless of the hearing device (CI or HA) that the child is wearing. The

A comparative study of age-related hearing loss in wild type and insulin-like growth factor I deficient mice

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Raquel Riquelme

2010-06-01

Full Text Available Insulin-like growth factor-I (IGF-I belongs to the family of insulin-related peptides that fulfils a key role during the late development of the nervous system. Human IGF1 mutations cause profound deafness, poor growth and mental retardation. Accordingly, Igf1−/− null mice are dwarfs that have low survival rates, cochlear alterations and severe sensorineural deafness. Presbycusis (age-related hearing loss is a common disorder associated with aging that causes social and cognitive problems. Aging is also associated with a decrease in circulating IGF-I levels and this reduction has been related to cognitive and brain alterations, although there is no information as yet regarding the relationship between presbycusis and IGF-I biodisponibility. Here we present a longitudinal study of wild type Igf1+/+ and null Igf1−/− mice from 2 to 12 months of age comparing the temporal progression of several parameters: hearing, brain morphology, cochlear cytoarchitecture, insulin-related factors and IGF gene expression and IGF-I serum levels. Complementary invasive and non-invasive techniques were used, including auditory brainstem-evoked response (ABR recordings and in vivo MRI brain imaging. Igf1−/− null mice presented profound deafness at all the ages studied, without any obvious worsening of hearing parameters with aging. Igf1+/+ wild type mice suffered significant age-related hearing loss, their auditory thresholds and peak I latencies augmenting as they aged, in parallel with a decrease in the circulating levels of IGF-I. Accordingly, there was an age-related spiral ganglion degeneration in wild type mice that was not evident in the Igf1 null mice. However, the Igf1−/− null mice in turn developed a prematurely aged stria vascularis reminiscent of the diabetic strial phenotype. Our data indicate that IGF-I is required for the correct development and maintenance of hearing, supporting the idea that IGF-I-based therapies could contribute to

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

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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.

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

Science.gov (United States)

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.

[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.

Viral Causes of Hearing Loss: A Review for Hearing Health Professionals

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Cohen, Brandon E.; Durstenfeld, Anne

2014-01-01

A number of viral infections can cause hearing loss. Hearing loss induced by these viruses can be congenital or acquired, unilateral or bilateral. Certain viral infections can directly damage inner ear structures, others can induce inflammatory responses which then cause this damage, and still others can increase susceptibility or bacterial or fungal infection, leading to hearing loss. Typically, virus-induced hearing loss is sensorineural, although conductive and mixed hearing losses can be seen following infection with certain viruses. Occasionally, recovery of hearing after these infections can occur spontaneously. Most importantly, some of these viral infections can be prevented or treated. For many of these viruses, guidelines for their treatment or prevention have recently been revised. In this review, we outline many of the viruses that cause hearing loss, their epidemiology, course, prevention, and treatment. PMID:25080364

Causes of hearing impairment in the Norwegian paediatric cochlear implant program.

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Siem, Geir; Fagerheim, Toril; Jonsrud, Christoffer; Laurent, Claude; Teig, Erik; Harris, Sten; Leren, Trond P; Früh, Andreas; Heimdal, Ketil

2010-08-01

Severe to profound hearing impairment (HI) is estimated to affect around 1/2000 young children. Advances in genetics have made it possible to identify several genes related to HI. This information can cast light upon prognostic factors regarding the outcome in cochlear implantation, and provide information both for scientific and genetic counselling purposes. From 1992 to 2005, 273 children from 254 families (probands) were offered cochlear implants in Norway. An evaluation of the causes of HI, especially regarding the genes GJB2, GJB6, SLC26A4, KCNQ1, KCNE1, and the mutation A1555G in mitochondrial DNA was performed in 85% of the families. The number of probands with unknown cause of HI was thus reduced from 120 to 68 (43% reduction). Ninety-eight (46%) of the probands had an identified genetic etiology of their HI. A relatively high prevalence of Jervell and Lange-Nielsen syndrome was found. The main causes of severe and profound HI were similar to those found in other European countries. GJB2 mutations are a common cause of prelingual HI in Norwegian cochlear implanted children.

The effects of familiarity and complexity on appraisal of complex songs by cochlear implant recipients and normal hearing adults.

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Gfeller, Kate; Christ, Aaron; Knutson, John; Witt, Shelley; Mehr, Maureen

2003-01-01

The purposes of this study were (a) to develop a test of complex song appraisal that would be suitable for use with adults who use a cochlear implant (assistive hearing device) and (b) to compare the appraisal ratings (liking) of complex songs by adults who use cochlear implants (n = 66) with a comparison group of adults with normal hearing (n = 36). The article describes the development of a computerized test for appraisal, with emphasis on its theoretical basis and the process for item selection of naturalistic stimuli. The appraisal test was administered to the 2 groups to determine the effects of prior song familiarity and subjective complexity on complex song appraisal. Comparison of the 2 groups indicates that the implant users rate 2 of 3 musical genres (country western, pop) as significantly more complex than do normal hearing adults, and give significantly less positive ratings to classical music than do normal hearing adults. Appraisal responses of implant recipients were examined in relation to hearing history, age, performance on speech perception and cognitive tests, and musical background.

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

DEFF Research Database (Denmark)

Mourtou, Eleni; Meis, Markus

2012-01-01

This chapter provides background information for researchers who wish to become familiar with some basic medical and audiological aspects of hearing loss and the technology of hearing aids. It introduces (1) the disciplines involved in research on hearing loss, (2) the medical categories of hearing...... loss and their various effects on communication, (3) the different degrees of hearing loss as defined by different national and international organizations, (4) statistics on the preva- lence of hearing loss worldwide, (5) some technological aspects of hearing instruments, (6) sta- tistics on non...

Acoustic Trauma - Hearing Loss in Teenagers

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... I Can Hear It, It's Too Loud: Earbuds & Teen Hearing Loss Page Content ​Many parents will agree ... hearing loss." Recommended Hearing Screenings for Older Children & Teens Kids should be screened at three ages: 11- ...

A descriptive analysis of language and speech skills in 4- to 5-yr-old children with hearing loss.

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Fitzpatrick, Elizabeth M; Crawford, Leah; Ni, Andy; Durieux-Smith, Andrée

2011-01-01

Early intervention through hearing aids (HAs) and cochlear implants (CIs) aims to reduce the negative effects of childhood hearing loss and to promote optimal communication development over time. The primary goal of this study was to examine the communication outcomes of children with CIs and children with HAs at age 4 to 5 yrs and to consider their spoken language skills relative to a group of typically developing hearing peers. In this multicenter observational study, communication results were obtained for a total of 88 children at age 4 to 5 yrs. Participants were recruited from three clinical programs in two cities in the province of Ontario, Canada. This study was undertaken shortly after the introduction of a new provincial population screening initiative and included both children who were screened and not screened for hearing loss. The study sample comprised 51 children with sensorineural hearing loss and 37 children with normal hearing. Of the 51 children with hearing loss, 26 used CIs and 25 used HAs. The degree of hearing loss ranged from mild to profound. All children were enrolled in rehabilitation programs focused on oral language development. Children's language skills were assessed with an extensive battery of child- and parent-administered speech and language measures. Assessment of language skills showed no significant differences between the children with severe to profound hearing loss using CIs and children with varying degrees of hearing loss using HAs. However, children with HAs showed better articulation skills. Overall, both groups of children obtained scores on communication measures that were lower than their hearing peers. The number of children with hearing loss who obtained spoken speech-language scores within 1 SD of normative populations ranged from 65 to 86% depending on the test measure. Children with average hearing loss of 70 dB HL or better generally obtained scores on all measures in line with those of age-matched norms while

Passing otoacustic emissions as a complementar method in the topodiagnosis of the neurosensories hearing loss

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Franceschi, Cacineli Marion de

2010-03-01

Full Text Available Introduction: To evaluate the otoacustic emissions (EOAETs in patients with neurosensory hearing loss do not belong to the clinical routine. However it would obtain valuables information concerning the topodiagnosis. Objective: To identify signs of retro cochlear alteration in individuals with neurosensory dysacusis diagnosis. Method: A transversal, observational, quantitative and, prospective study. Were analyzed 34 patients' records of users of the Speech Therapy Attendance Service. In the study were included individuals with neurosensory hearing loss of moderate to deep degree. An evaluation of Passing Otoacustic Emissions (EOAETs was performed in all the individuals of the sample. Those that do not presented EOAETs had the external and middle ear' condition evaluates through meatoscopy and tympanometry to eliminate ears with sings of conductive alteration. Results: Before that the exclusion criteria were applied, they have remained 13 individuals, totalizing 26 ears: four with hearing loss of moderate degree (15%, four with moderately severe degree (15%, two with severe degree (8%, 15 with deep degree (58% and, one with deafness (4%. The tympanometric curves found were 22 (85% Type A and, four (15% Type C. It was verified the presence of EOAETs in only two ears (8% of a same individual. Conclusion: It was verified the predominance of the EOAETs absence in individuals with neurosensory hearing loss of moderate to deep degree. In one case the EOAETs were registered, that suggest retrocochlear alteration. Raising suspicion of retrocochlear alterations.

Cochlear pathology in chronic suppurative otitis media.

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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.

Triagem auditiva neonatal com emissões otoacusticas e reflexo cocleo-palpebral: estudo da sensibilidade e especificidade Newborn Hearing Screening with otoacoustic emissions and cochlear-palpebral reflex: sensitivity and specificity study

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Rosanna Mariangela Giaffredo Angrisani

2012-10-01

Full Text Available OBJETIVO: analisar a especificidade e sensibilidade da TAN com emissões otoacústicas evocadas por estimulo transiente (EOAT associadas à pesquisa do reflexo cócleo-palpebral (RCP, comparando-os aos resultados do Potencial Evocado Auditivo de Tronco Encefalico (PEATE. MÉTODO: a casuística do presente estudo foi composta por 369 RN de risco para deficiência auditiva que foram submetidos à triagem com a captação das emissões otoacústicas evocadas por estímulo transiente e pesquisa do reflexo cócleo- palpebral. Os resultados foram comparados aos resultados do Potencial Evocado Auditivo de Tronco Encefalico (PEATE. RESULTADOS: a incidência do tipo da perda auditiva na população geral foi de 4RN (1,1% com perda coclear, 22 (5,9% com perda condutiva, 2 (0,5% com espectro da neuropatia auditiva (ENA, 14 (3,8% com alteração central e 15 (4,1% com atraso de maturação da via auditiva. A TAN mostrou 100% de sensibilidade e 94,6% de especificidade na detecção de alterações cocleares e espectro da neuropatia auditiva; 77,3% de sensibilidade e 94,6% de especificidade na detecção de alterações condutivas e 42,9% de sensibilidade e 94,6% de especificidade na detecção de alterações centrais. Todos os achados evidenciaram diferenças estatisticamente significantes em relação aos neonatos auditivamente normais. CONCLUSÃO: este protocolo de TAN mostrou-se eficaz na detecção de RN com alterações de cocleares e espectro da neuropatia auditiva com sensibilidade e especificidade elevadas.PURPOSE: to study the specificity and sensitivity of NHS with otoacoustic emissions transient stimulus (TEOAE associated with cochlear-palpebral reflex (CPR, and comparing the results of Auditory Evoked Potential (BAEP. METHOD: three hundred and sixty-nine neonates under risk of hearing loss were evaluated. Evaluation procedures consisted of transient otocoustic emissions(TOAEs, cochlear-palpebral reflex (CPR, and ABR diagnostic carried out in the

Sudden bilateral hearing loss after organophosphate inhalation

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Mehmet Akif Dundar

2016-12-01

Full Text Available Sudden bilateral hearing loss are seen rarely and the toxic substance exposure constitutes a small part of etiology. A Fifty-eight-year-old woman admitted to our clinic with sudden bilateral hearing loss shortly after chlorpyrifos-ethyl exposure. Otolaryngologic examination findings were normal. The patient had 40 dB sensorineural hearing loss (SNHL on the right ear and 48 dB SNHL on the left ear. Additional diagnostic tests were normal. The conventional treatment for sudden hearing loss was performed. On the second week following organophosphate (OP exposure the patient's hearing loss almost completely resolved. OP's are heavily used in agriculture and should be taken into consideration as an etiologic factor in sudden hearing loss. Keywords: Organophosphates, Hearing loss, Sudden

The effect of music on auditory perception in cochlear-implant users and normal-hearing listeners

NARCIS (Netherlands)

Fuller, Christina Diechina

2016-01-01

Cochlear implants (CIs) are auditory prostheses for severely deaf people that do not benefit from conventional hearing aids. Speech perception is reasonably good with CIs; other signals such as music perception are challenging. First, the perception of music and music related perception in CI users

Dexamethasone loaded nanoparticles exert protective effects against Cisplatin-induced hearing loss by systemic administration.

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Sun, Changling; Wang, Xueling; Chen, Dongye; Lin, Xin; Yu, Dehong; Wu, Hao

2016-04-21

Ototoxicity is one of the most important adverse effects of cisplatin chemotherapy. As a common treatment of acute sensorineural hearing loss, systemic administration of steroids was demonstrated ineffective against cisplatin-induced hearing loss (CIHL) in published studies. The current study aimed to evaluate the potential protective effect of dexamethasone (DEX) encapsulated in polyethyleneglycol-coated polylactic acid (PEG-PLA) nanoparticles (DEX-NPs) against cisplatin-induced hearing loss following systemic administration. DEX was fabricated into PEG-PLA nanoparticles using emulsion and evaporation technique as previously reported. DEX or DEX-NPs was administered intraperitoneally to guinea pigs 1h before cisplatin administration. Auditory brainstem response (ABR) threshold shifts were measured at four frequencies (4, 8, 16, and 24kHz) 1 day before and three days after cisplatin injection. Cochlear morphology was examined to evaluate inner ear injury induced by cisplatin exposure. A single dose of DEX-NPs 1h before cisplatin treatment resulted in a significant preservation of the functional and structural properties of the cochlea, which was equivalent to the effect of multidose (3 days) DEX injection. In contrast, no significant protective effect was observed by single dose injection of DEX. The results of histological examination of the cochleae were consistent with the functional measurements. In conclusion, a single dose DEX-NPs significantly attenuated cisplatin ototoxicity in guinea pigs after systemic administration at both histological and functional levels indicating the potential therapeutic benefits of these nanoparticles for enhancing the delivery of DEX in acute sensorineural hearing loss. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

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…

Sensorineural hearing loss in hemorrhagic dengue?

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Bruna Natália Freire Ribeiro

2015-01-01

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

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.

Risk factors for hearing loss in neonates

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Ni Luh Putu Maharani

2016-11-01

Full Text Available Background An estimated 6 of 1,000 children with live births suffer from permanent hearing loss at birth or the neonatal period. At least 90% of cases occur in developing countries. Hearing loss should be diagnosed as early as possible so that intervention can be done before the age of 6 months. Objective To determine risk factors for hearing loss in neonates. Methods We performed a case-control study involving 100 neonates with and without hearing loss who were born at Sanglah Hospital, Denpasar from November 2012 to February 2013. Subjects were consisted of 2 groups, those with hearing loss (case group of 50 subjects and without hearing loss (control group of 50 subjects. The groups were matched for gender and birth weight. We assessed the following risk factors for hearing loss: severe neonatal asphyxia, hyperbilirubinemia, meningitis, history of aminoglycoside therapy, and mechanical ventilation by Chi-square analysis. The results were presented as odds ratio and its corresponding 95% confidence intervals. Results Seventy percent of neonates with hearing loss had history of aminoglycoside therapy. Multivariable analysis revealed that aminoglycoside therapy of 14 days or more was a significant risk factor for hearing loss (OR 2.7; 95%CI 1.1 to 6.8; P=0.040. There were no statistically significant associations between hearing loss and severe asphyxia, hyperbilirubinemia, meningitis, or mechanical ventilation. Conclusion Aminoglycoside therapy for >=14 days was identified as a risk factor for hearing loss in neonates.

An overview of hereditary hearing loss.

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Bayazit, Yildirim A; Yilmaz, Metin

2006-01-01

Understanding the genetic basis of hearing loss is important because almost 50% of profound hearing loss are caused by genetic factors and more than 120 independent genes have been identified. In this review, after a brief explanation of some genetic terms (allele, heterozygosis, homozygosis, polymorphism, genotype and phenotype), classification of genetic hearing loss (syndromic versus nonsyndromic, and recessive dominant, X-linked and mitochondrial) was performed. Some of the most common syndromes (Usher, Pendred, Jervell and Lange-Nielsen, Waardenburg, branchio-oto-renal, Stickler, Treacher Collins and Alport syndromes, biotinidase deficiency and Norrie disease) causing genetic hearing loss were also explained briefly. The genes involved in hearing loss and genetic heterogeneity were presented. Copyright 2006 S. Karger AG, Basel.

Improving speech-in-noise recognition for children with hearing loss: potential effects of language abilities, binaural summation, and head shadow.

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Nittrouer, Susan; Caldwell-Tarr, Amanda; Tarr, Eric; Lowenstein, Joanna H; Rice, Caitlin; Moberly, Aaron C

2013-08-01

This study examined speech recognition in noise for children with hearing loss, compared it to recognition for children with normal hearing, and examined mechanisms that might explain variance in children's abilities to recognize speech in noise. Word recognition was measured in two levels of noise, both when the speech and noise were co-located in front and when the noise came separately from one side. Four mechanisms were examined as factors possibly explaining variance: vocabulary knowledge, sensitivity to phonological structure, binaural summation, and head shadow. Participants were 113 eight-year-old children. Forty-eight had normal hearing (NH) and 65 had hearing loss: 18 with hearing aids (HAs), 19 with one cochlear implant (CI), and 28 with two CIs. Phonological sensitivity explained a significant amount of between-groups variance in speech-in-noise recognition. Little evidence of binaural summation was found. Head shadow was similar in magnitude for children with NH and with CIs, regardless of whether they wore one or two CIs. Children with HAs showed reduced head shadow effects. These outcomes suggest that in order to improve speech-in-noise recognition for children with hearing loss, intervention needs to be comprehensive, focusing on both language abilities and auditory mechanisms.

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

Science.gov (United States)

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.

Contrast enhancement of the cochlear aqueduct in MR imaging: its frequency and clinical significance

International Nuclear Information System (INIS)

Nakamura, T.; Naganawa, S.; Fukatsu, H.; Sakurai, Y.; Ishigaki, T.; Aoki, I.; Ninomiya, A.; Nakashima, T.

2003-01-01

There have been no previous reports on contrast enhancement of the cochlear aqueduct in magnetic resonance imaging. The purpose of the present study was to evaluate the frequency and significance of this finding. Thirty-one patients (15 men and 16 women; age range 18-81 years) with otologic symptoms (sudden sensorineural hearing loss, vertigo, or tinnitus) were examined using contrast-enhanced imaging on a 1.5-T MR scanner. The normal ear served as the control. Two radiologists evaluated contrast enhancement in the area of the cochlear aqueduct. Forty-eight of 62 ears (77.4%) showed contrast enhancement of the cochlear aqueduct, but no significant differences in the frequency of contrast enhancement were observed between patients with and patients without vertigo, tinnitus, sensorineural hearing loss, cerebellopontine angle tumors, or a high-riding jugular bulb. In addition, no gender- or age-related differences were noted. Contrast enhancement of the cochlear aqueduct was frequently observed, but the frequency of enhancement in symptomatic ears was not significantly higher than in control ears. The results of this study may prove helpful in avoiding unnecessary examinations and potential diagnostic confusion. (orig.)

Contrast enhancement of the cochlear aqueduct in MR imaging: its frequency and clinical significance

Energy Technology Data Exchange (ETDEWEB)

Nakamura, T; Naganawa, S; Fukatsu, H; Sakurai, Y; Ishigaki, T [Department of Radiology, Nagoya University School of Medicine, 65 Tsurumai-cho, 466-8550, Shouwa-ku, Nagoya (Japan); Aoki, I; Ninomiya, A [Medical Systems Company, Toshiba Corporation, Nasu Operations, Otawara-shi, Tochigi (Japan); Nakashima, T [Department of Otorhinolaryngology, Nagoya University School of Medicine, Shouwa-ku, Nagoya (Japan)

2003-09-01

There have been no previous reports on contrast enhancement of the cochlear aqueduct in magnetic resonance imaging. The purpose of the present study was to evaluate the frequency and significance of this finding. Thirty-one patients (15 men and 16 women; age range 18-81 years) with otologic symptoms (sudden sensorineural hearing loss, vertigo, or tinnitus) were examined using contrast-enhanced imaging on a 1.5-T MR scanner. The normal ear served as the control. Two radiologists evaluated contrast enhancement in the area of the cochlear aqueduct. Forty-eight of 62 ears (77.4%) showed contrast enhancement of the cochlear aqueduct, but no significant differences in the frequency of contrast enhancement were observed between patients with and patients without vertigo, tinnitus, sensorineural hearing loss, cerebellopontine angle tumors, or a high-riding jugular bulb. In addition, no gender- or age-related differences were noted. Contrast enhancement of the cochlear aqueduct was frequently observed, but the frequency of enhancement in symptomatic ears was not significantly higher than in control ears. The results of this study may prove helpful in avoiding unnecessary examinations and potential diagnostic confusion. (orig.)

Speak on time! Effects of a musical rhythmic training on children with hearing loss.

Science.gov (United States)

Hidalgo, Céline; Falk, Simone; Schön, Daniele

2017-08-01

This study investigates temporal adaptation in speech interaction in children with normal hearing and in children with cochlear implants (CIs) and/or hearing aids (HAs). We also address the question of whether musical rhythmic training can improve these skills in children with hearing loss (HL). Children named pictures presented on the screen in alternation with a virtual partner. Alternation rate (fast or slow) and the temporal predictability (match vs mismatch of stress occurrences) were manipulated. One group of children with normal hearing (NH) and one with HL were tested. The latter group was tested twice: once after 30 min of speech therapy and once after 30 min of musical rhythmic training. Both groups of children (NH and with HL) can adjust their speech production to the rate of alternation of the virtual partner. Moreover, while children with normal hearing benefit from the temporal regularity of stress occurrences, children with HL become sensitive to this manipulation only after rhythmic training. Rhythmic training may help children with HL to structure the temporal flow of their verbal interactions. Copyright © 2017 Elsevier B.V. All rights reserved.

Theory-of-mind development in oral deaf children with cochlear implants or conventional hearing aids.

Science.gov (United States)

Peterson, Candida C

2004-09-01

In the context of the established finding that theory-of-mind (ToM) growth is seriously delayed in late-signing deaf children, and some evidence of equivalent delays in those learning speech with conventional hearing aids, this study's novel contribution was to explore ToM development in deaf children with cochlear implants. Implants can substantially boost auditory acuity and rates of language growth. Despite the implant, there are often problems socialising with hearing peers and some language difficulties, lending special theoretical interest to the present comparative design. A total of 52 children aged 4 to 12 years took a battery of false belief tests of ToM. There were 26 oral deaf children, half with implants and half with hearing aids, evenly divided between oral-only versus sign-plus-oral schools. Comparison groups of age-matched high-functioning children with autism and younger hearing children were also included. No significant ToM differences emerged between deaf children with implants and those with hearing aids, nor between those in oral-only versus sign-plus-oral schools. Nor did the deaf children perform any better on the ToM tasks than their age peers with autism. Hearing preschoolers scored significantly higher than all other groups. For the deaf and the autistic children, as well as the preschoolers, rate of language development and verbal maturity significantly predicted variability in ToM, over and above chronological age. The finding that deaf children with cochlear implants are as delayed in ToM development as children with autism and their deaf peers with hearing aids or late sign language highlights the likely significance of peer interaction and early fluent communication with peers and family, whether in sign or in speech, in order to optimally facilitate the growth of social cognition and language.

Occupational hearing loss in Korea.

Science.gov (United States)

Kim, Kyoo Sang

2010-12-01

In this article, current status of noise exposure in workplaces, trend of workers with noise-induced hearing loss (NIHL), and prevalence of NIHL in workers by industry and job category in Korea were reviewed. In addition, trends of research on the audiological effects such as hearing loss from noise and occupational hearing loss from non-noise in Korea were addressed through reports in industrial audiology. Though noise exposure level has improved, noise still shows the highest rate of cases exceeding exposure limit among workplace hazards. NIHL is the most common occupational disease except work-related disease such as musculoskeletal disorders and cerebrovascular diseases, and NIHL prevalence is thought to be much higher than reported in official publications. Noise affecting hearing comes from various sources such as workplaces, military settings, areas with exposure to high noise, and specific noise sources. There is also occupational hearing loss by non-noise including chemicals such as organic solvents and heavy metals, barotrauma, and trauma due to welding spark. Noise affects daily life through audiological effects such as hearing loss and tinnitus, non-audiological physical effects (e.g., cardiovascular), and psychosocial and behavioral effects. Development of systematic and comprehensive hearing conservation programs for lowering the noise level in workplaces and preventing the NIHL, and preparation of technological, administrative system for its settlement at workplace are urgently needed.

The Effect of Systemic Steroid on Hearing Preservation After Cochlear Implantation via Round Window Approach: A Guinea Pig Model.

Science.gov (United States)

Chang, Mun Young; Rah, Yoon Chan; Choi, Jun Jae; Woo, Shin Wook; Hwang, Yu-Jung; Eastwood, Hayden; O'Leary, Stephen J; Lee, Jun Ho

2017-08-01

When administered perioperatively, systemic dexamethasone will reduce the hearing loss associated with cochlear implantation (CI) performed via the round window approach. The benefits of electroacoustic stimulation have led to interest in pharmacological interventions to preserve hearing after CI. Thirty guinea pigs were randomly divided into three experimental groups: a control group; a 3-day infusion group; and a 7-day infusion group. Dexamethasone was delivered via a mini-osmotic pump for either 3 or 7 days after CI via the round window. Pure tone-evoked auditory brainstem response (ABR) thresholds were monitored for a period of 12 weeks after CI. The cochleae were then collected for histology. At 4 and 12 weeks after CI, ABR threshold shifts were significantly reduced in both 7-day and 3-day infusion groups compared with the control group. Furthermore, the 7-day infusion group has significantly reduced ABR threshold shifts compared with the 3-day infusion group. The total tissue response, including fibrosis and ossification, was significantly reduced in the 7-day infusion group compared with the control group. On multiple regression the extent of fibrosis predicted hearing loss across most frequencies, while hair cell counts predicted ABR thresholds at 32 kHz. Hearing protection after systemic administration of steroids is more effective when continued for at least a week after CI. Similarly, this treatment approach was more effective in reducing the fibrosis that encapsulates the CI electrode. Reduced fibrosis seemed to be the most likely explanation for the hearing protection.

Prevalence of Contralateral Hearing Aid Use in Adults with Cochlear Implants

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Yamaguchi, Cintia Tizue

2013-09-01

Full Text Available Introduction: The exclusive use of a cochlear implant (CI in one ear allows patients to effectively hear speech in a quiet environment. However, in environments with competing noise, the processing of multiple sounds becomes complex. In an attempt to promote binaural hearing in a noninvasive manner, the use of a hearing aid in the nonimplanted ear is suggested for patients with a unilateral CI. Aims: To identify the prevalence of hearing aid use in the contralateral ear in adults who already have a CI; to determine the reasons why some patients do not use contralateral hearing aids (CHAs; and to analyze the effects of residual hearing in CHA users. Materials and Methods: This is a clinical study in 82 adult patients with CI implants who responded to a questionnaire designed to determine current use of CHA. Results: In our patient sample, 70 CHA nonusers were identified. The prevalence of CHA users was determined to be 12% with a 95% confidence interval of 11 to 13%. About 58.2% of the CHA nonusers reported a lack of noticeable benefit even after wearing hearing aids, and 23.6% reported not having received the option to use a CHA. CHA users had a pure tone average of 107-dB hearing level, whereas CHA nonusers had a pure tone average of 117-dB hearing level. Conclusion: The prevalence of the use of a CHA is low in our study. We attribute the low use of a CHA to either a lack of residual hearing or to a lack of benefit from the amplification.

Masker phase effects in normal-hearing and hearing-impaired listeners: evidence for peripheral compression at low signal frequencies

DEFF Research Database (Denmark)

Oxenham, Andrew J.; Dau, Torsten

2004-01-01

curvature. Results from 12 listeners with sensorineural hearing loss showed reduced masker phase effects, when compared with data from normal-hearing listeners, at both 250- and 1000-Hz signal frequencies. The effects of hearing impairment on phase-related masking differences were not well simulated...... are affected by a common underlying mechanism, presumably related to cochlear outer hair cell function. The results also suggest that normal peripheral compression remains strong even at 250 Hz....

Acrylonitrile potentiates hearing loss and cochlear damage induced by moderate noise exposure in rats

International Nuclear Information System (INIS)

Pouyatos, BenoIt; Gearhart, Caroline A.; Fechter, Laurence D.

2005-01-01

The diversity of chemical and drugs that can potentiate noise-induced hearing loss (NIHL) has impeded efforts to predict such interactions. We have hypothesized that chemical contaminants that disrupt intrinsic antioxidant defenses hold significant risk for potentiating NIHL. If this is true, then acrylonitrile (ACN) would be expected to potentiate NIHL. ACN, one of the 50 most commonly used chemicals in the United States, is metabolized via two pathways that are likely to disrupt intrinsic reactive oxygen species (ROS) buffering systems: (1) it conjugates glutathione, depleting this important antioxidant rapidly; (2) a second pathway involves the formation of cyanide, which can inhibit superoxide dismutase. We hypothesized that moderate noise exposure, that does not produce permanent hearing loss by itself, could initiate oxidative stress and that ACN could render the inner ear more sensitive to noise by disrupting intrinsic antioxidant defenses. Temporary and persistent effects of ACN alone (50 mg/kg, sc 5 days), noise alone (95 or 97 dB octave band noise, 4 h/day for 5 days), or ACN in combination with noise were determined using distortion product otoacoustic emissions (DPOAEs) and compound action potential (CAP) amplitudes. Histopathological damage to hair cells resulting from these treatments was also investigated using surface preparations of the organ of Corti. Individually, neither ACN nor noise exposures caused any permanent hearing or hair cell loss; only a reversible temporary threshold shift was measured in noise-exposed animals. However, when given in combination, ACN and noise induced permanent threshold shifts (13-16 dB between 7 and 40 kHz) and a decrease in DPOAE amplitudes (up to 25 dB at 19 kHz), as well as significant outer hair cell (OHC) loss (up to 20% in the first row between 13 and 47 kHz). This investigation demonstrates that ACN can potentiate NIHL at noise levels that are realistic in terms of human exposure, and that the OHCs are the

[Vestibulo-cochlear disturbances in the course of vaso-neural conflict of vestibule-cochlear nerve--diagnostic and therapeutic problems].

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Orendorz-Fraczkowska, Krystyna; Jaworska, Marzena; Gawron, Wojciech; Badowski, Roman; Nadolska, Beata

2007-01-01

Symptoms encompassing sensorineural hearing loss, tinnitus and vertigo occur in many diseases of various origin. The diagnostics in such cases is especially difficult and often requires interdisciplinary cooperation. Despite of that many cases remain unexplained. The two cases with above mentioned symptoms (52 year-old woman and 46 year-old man) with differentiated clinical course were presented. The woman for one year experienced left sided, extreme tinnitus with paroxysmal vertigo and dizziness. The man with sudden monolateral hearing loss and tinnitus that disappeared after corticosteroid therapy, complained about recurrence of fluctuating hearing loss and tinnitus accompanied by chronic instability. The diagnostics of hearing and balance organs was performed (pure tone audiometry, impedance audiometry, DPOAE, ABR, ENG) complemented with computed tomography and Nuclear Resonance. Female patient presented bilateral mild sensorineural hearing loss, more intensive on the left side, male patient right sided sensorineural hearing loss in the frequencies from 250 to 1500 Hz. The elongation of some peak values in ABR test (with bilaterally proper otoacoustic emissions) as well as partial canal paresis on the hearing loss side suggested primary diagnosis of neoplasmatic process. It was an indication to perform radiological diagnostics. The radiologic findings revealed the contact of vestibulo-cochlear nerve with the loop of cerebellar inferior anterior artery within the internal acoustic canal. The course of the disease and diagnostic tests do not provide characteristic data that let diagnose neuro-vascular conflict. One has to be aware of such possibility in patients with intensive vertigo and dizziness that do not improve after treatment when other causes do not justify the symptoms.

Predictors of flourishing among children with hearing loss.

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Nabors, Laura; Odar Stough, Cathleen; Merianos, Ashley; Peugh, James

2016-12-01

To examine parent report of flourishing in children with hearing impairments compared to children without hearing impairments, and to explore whether school engagement and bullying related to child flourishing. Participants were 655 children with hearing impairments and 44, 618 children without hearing impairments who were 10-17 years of age. Caregivers completed telephone interviews about their child's functioning for the National Survey of Children's Health. Children without hearing loss had higher parent-reported flourishing compared to children with hearing loss when controlling for child demographics (i.e., race, age, sex). School engagement was positively related to flourishing of children with hearing loss. Bullying behaviors were not related to flourishing of children with hearing loss. Improving school engagement may increase flourishing of children with hearing loss, which is critical given that children with hearing loss experience lower flourishing than children without hearing loss. Examining the relationships among other risk and resilience factors and flourishing for children with hearing loss will provide information for interventions to enhance the adaptation of these children. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Recovery of Abnormal ABR in Neonates and Infants at Risk of Hearing Loss

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Ioannis Psarommatis

2017-01-01

Full Text Available The purpose of this retrospective study is to present the clinical experience of a single institution on the recovery of ABR thresholds in a large population of neonates and infants at risk of hearing loss. Potential prognostic factors associated with this phenomenon were also investigated. Out of 2248 high risk infants, 384 had abnormal ABR at initial hearing evaluation and 168 of them had absent ABR or a threshold ≥80 dBnHL. From this subgroup, a significant percentage showed complete or partial recovery on reexamination (32.7% and 9.3%, resp., performed 4–6 months later. The presence of normal otoacoustic emissions was associated with the ABR restoration on reexamination. Moreover, the very young age at the initial hearing screening seems to be related to higher probabilities of false positive ABR. The potential recovery of hearing in HR infants raises concerns about the very early cochlear implantation in HR infants less than one year. Such a treatment modality should be decided cautiously and only after obtaining valid and stable objective and subjective hearing thresholds. This holds especially true for infants showing an auditory neuropathy profile, as they presented a much greater probability of ABR recovery.

Etiology of hearing loss in children.

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José Ignacio BENITO-OREJAS

2017-06-01

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

Transient Abnormalities in Masking Tuning Curve in Early Progressive Hearing Loss Mouse Model

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Marion Souchal

2018-01-01

Full Text Available Damage to cochlear outer hair cells (OHCs usually affects frequency selectivity in proportion to hearing threshold increase. However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or “hidden” synaptopathy overlooks possible underlying OHC impairment. Here, we document the part played by OHCs in influencing suprathreshold auditory performance in the presence of noise in a mouse model of progressive hair cell degeneration, the CD1 strain, at postnatal day 18–30 stages when high-frequency auditory thresholds remained near-normal. Nonetheless, total loss of high-frequency distortion product otoacoustic emissions pointed to nonfunctioning basal OHCs. This “discordant profile” came with a huge low-frequency shift of masking tuning curves that plot the level of interfering sound necessary to mask the response to a probe tone, against interfering frequency. Histology revealed intense OHC hair bundle abnormalities in the basal cochlea uncharacteristically associated with OHC survival and preserved coupling with the tectorial membrane. This pattern dismisses the superficial diagnosis of “hidden” neuropathy while underpinning a disorganization of cochlear frequency mapping with optimistic high-frequency auditory thresholds perhaps because responses to high frequencies are apically shifted. The audiometric advantage of frequency transposition is offset by enhanced masking by low-frequency sounds, a finding essential for guiding rehabilitation.

Hearing Loss: Symptoms, Diagnosis & Treatment

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... of this page please turn JavaScript on. Feature: Hearing Loss Symptoms, Devices, Prevention & Research Past Issues / Spring ... a disease. It can accompany any type of hearing loss. It can be a side effect of ...

Noise and Hearing Loss Prevention

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... message, please visit this page: About CDC.gov . NOISE AND HEARING LOSS PREVENTION Language: English (US) Español ( ... when hazardous noise levels cannot be adequately reduced. Noise and Hearing Loss on the NIOSH Science Blog ...

[Examination of relationship between level of hearing and written language skills in 10-14-year-old hearing impaired children].

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Turğut, Nedim; Karlıdağ, Turgut; Başar, Figen; Yalçın, Şinasi; Kaygusuz, İrfan; Keleş, Erol; Birkent, Ömer Faruk

2015-01-01

This study aims to review the relationship between written language skills and factors which are thought to affect this skill such as mean hearing loss, duration of auditory deprivation, speech discrimination score, and pre-school education attendance and socioeconomic status of hearing impaired children who attend 4th-7th grades in primary school in inclusive environment. The study included 25 hearing impaired children (14 males, 11 females; mean age 11.4±1.4 years; range 10 to 14 years) (study group) and 20 children (9 males, 11 females; mean age 11.5±1.3 years; range 10 to 14 years) (control group) with normal hearing in the same age group and studying in the same class. Study group was separated into two subgroups as group 1a and group 1b since some of the children with hearing disability used hearing aid while some used cochlear implant. Intragroup comparisons and relational screening were performed for those who use hearing aids and cochlear implants. Intergroup comparisons were performed to evaluate the effect of the parameters on written language skills. Written expression skill level of children with hearing disability was significantly lower than their normal hearing peers (p=0.001). A significant relationship was detected between written language skills and mean hearing loss (p=0.048), duration of auditory deprivation (p=0.021), speech discrimination score (p=0.014), and preschool attendance (p=0.005), when it comes to socioeconomic status we were not able to find any significant relationship (p=0.636). It can be said that hearing loss affects written language skills negatively and hearing impaired individuals develop low-level written language skills compared to their normal hearing peers.

A Comparison of Linguistic Skills between Persian Cochlear Implant and Normal Hearing Children

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Mohammad Rahimi

2013-04-01

Full Text Available Objectives: A large number of congenitally deaf children are born annually. If not treated, this will have destructive effects on their language and speech development, educational achievements and future occupation. In this study it has been tried to determine the level of language skills in children with Cochlear Implants (CI in comparison with Normal Hearing (NH age-mates. Methods: Test of Language Development was administered to 30 pre-lingual, severe-to-profound CI children between the ages of 5 to 8. The obtained scores were compared to a Persian database from scores of normally hearing children with the same age range. Results: Results indicated that in spite of great advancements in different areas of language after hearing gain, CI children still lag behind their hearing age-mates in almost all aspects of language skills. Discussion: Based on the results, it is suggested that children with average or above average cognitive skills who use CI have the potential to produce and understand language comparable to their normally hearing peers.

Deafness and Hearing Loss.

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National Information Center for Children and Youth with Disabilities, Washington, DC.

This brief overview provides information on the definition, incidence, and characteristics of children with hearing impairments and deafness. The federal definitions of hearing impairment and deafness are provided. The different types of hearing loss are noted, including: (1) conductive (caused by diseases or obstructions in the outer or middle…

Relationship between hearing complaint and hearing loss among older people

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Teixeira, Adriane Ribeiro

2009-03-01

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

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.

Imaging of acquired non-traumatic cochlear lesions: iconographic essay

International Nuclear Information System (INIS)

Garcia, Marcelo de Mattos; Gonzaga, Juliana Gontijo

2006-01-01

Different non-traumatic acquired cochlear lesions are shown in this article with imaging methods. They may be responsible for neuro sensorial hearing loss or vertigo. The method of choice is computed tomography when evaluating the osseous labyrinth whereas magnetic resonance imaging has superior resolution in the studies of the membranaceous labyrinth. (author)

Prevention of the Evolution of Workers' Hearing Loss from Noise-Induced Hearing Loss in Noisy Environments through a Hearing Conservation Program.

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Fonseca, Vinicius Ribas; Marques, Jair; Panegalli, Flavio; Gonçalves, Claudia Giglio de Oliveira; Souza, Wesley

2016-01-01

Introduction Noise-induced hearing loss (NIHL) is a serious problem for workers and therefore for businesses. The hearing conservation program (HCP) is a set of coordinated measures to prevent the development or evolution of occupational hearing loss, which involves a continuous and dynamic process of implementation of hearing conservation routines through anticipation, recognition, evaluation, and subsequent control of the occurrence of existing environmental risks or of those that may exist in the workplace and lead to workers' hearing damage. Objective The aim of this study was to evaluate the effectiveness of the HCP in preventing further hearing loss in workers with audiograms suggestive of NIHL. The audiometric tests and medical records of 28 furniture company workers exposed to noise were reviewed and monitored for 2 years. Methods This retrospective, cross-sectional study examined five audiometric tests in the medical records (on admission and every semester) of 28 workers in a furniture company (totaling 140 audiometric exams) following the introduction of the HCP. Results Data analysis showed no differences between the audiometric tests conducted on admission and those performed every semester. Conclusions The HCP implemented was effective in preventing the worsening of hearing loss in workers already with NIHL when exposed to occupational noise. Therefore, such a measure could be useful for the employment of workers with hearing loss in job sectors that have noise exposure.

Prevention of the Evolution of Workers' Hearing Loss from Noise-Induced Hearing Loss in Noisy Environments through a Hearing Conservation Program

Science.gov (United States)

Fonseca, Vinicius Ribas; Marques, Jair; Panegalli, Flavio; Gonçalves, Claudia Giglio de Oliveira; Souza, Wesley

2015-01-01

Introduction Noise-induced hearing loss (NIHL) is a serious problem for workers and therefore for businesses. The hearing conservation program (HCP) is a set of coordinated measures to prevent the development or evolution of occupational hearing loss, which involves a continuous and dynamic process of implementation of hearing conservation routines through anticipation, recognition, evaluation, and subsequent control of the occurrence of existing environmental risks or of those that may exist in the workplace and lead to workers' hearing damage. Objective The aim of this study was to evaluate the effectiveness of the HCP in preventing further hearing loss in workers with audiograms suggestive of NIHL. The audiometric tests and medical records of 28 furniture company workers exposed to noise were reviewed and monitored for 2 years. Methods This retrospective, cross-sectional study examined five audiometric tests in the medical records (on admission and every semester) of 28 workers in a furniture company (totaling 140 audiometric exams) following the introduction of the HCP. Results Data analysis showed no differences between the audiometric tests conducted on admission and those performed every semester. Conclusions The HCP implemented was effective in preventing the worsening of hearing loss in workers already with NIHL when exposed to occupational noise. Therefore, such a measure could be useful for the employment of workers with hearing loss in job sectors that have noise exposure. PMID:26722345

Congenital sensorineural hearing loss

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