Liberman, M Charles; Liberman, Leslie D; Maison, Stéphane F
Synapses between cochlear nerve terminals and hair cells are the most vulnerable elements in the inner ear in both noise-induced and age-related hearing loss, and this neuropathy is exacerbated in the absence of efferent feedback from the olivocochlear bundle. If age-related loss is dominated by a lifetime of exposure to environmental sounds, reduction of acoustic drive to the inner ear might improve cochlear preservation throughout life. To test this, we removed the tympanic membrane unilaterally in one group of young adult mice, removed the olivocochlear bundle in another group and compared their cochlear function and innervation to age-matched controls one year later. Results showed that tympanic membrane removal, and the associated threshold elevation, was counterproductive: cochlear efferent innervation was dramatically reduced, especially the lateral olivocochlear terminals to the inner hair cell area, and there was a corresponding reduction in the number of cochlear nerve synapses. This loss led to a decrease in the amplitude of the suprathreshold cochlear neural responses. Similar results were seen in two cases with conductive hearing loss due to chronic otitis media. Outer hair cell death was increased only in ears lacking medial olivocochlear innervation following olivocochlear bundle cuts. Results suggest the novel ideas that 1) the olivocochlear efferent pathway has a dramatic use-dependent plasticity even in the adult ear and 2) a component of the lingering auditory processing disorder seen in humans after persistent middle-ear infections is cochlear in origin.
Tanaka, Chiemi; Nguyen-Huynh, Anh; Loera, Katherine; Stark, Gemaine; Reiss, Lina
The Hybrid cochlear implant (CI), also known as Electro-Acoustic Stimulation (EAS), is a new type of CI that preserves residual acoustic hearing and enables combined cochlear implant and hearing aid use in the same ear. However, 30-55% of patients experience acoustic hearing loss within days to months after activation, suggesting that both surgical trauma and electrical stimulation may cause hearing loss. The goals of this study were to: 1) determine the contributions of both implantation surgery and EAS to hearing loss in a normal-hearing guinea pig model; 2) determine which cochlear structural changes are associated with hearing loss after surgery and EAS. Two groups of animals were implanted (n = 6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no direct acoustic or electric stimulation during this time frame. A third group (n = 6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem response thresholds were followed over time at 1, 2, 6, and 16 kHz. At the end of the study, the following cochlear measures were quantified: hair cells, spiral ganglion neuron density, fibrous tissue density, and stria vascularis blood vessel density; the presence or absence of ossification around the electrode entry was also noted. After surgery, implanted animals experienced a range of 0-55 dB of threshold shifts in the vicinity of the electrode at 6 and 16 kHz. The degree of hearing loss was significantly correlated with reduced stria vascularis vessel density and with the presence of ossification, but not with hair cell counts, spiral ganglion neuron density, or fibrosis area. After 10 weeks of stimulation, 67% of implanted, stimulated animals had more than 10 dB of additional threshold shift at 1 kHz, compared to 17% of implanted, non-stimulated animals and 0% of non-implanted animals. This 1-kHz hearing loss was not associated with changes in any of the cochlear measures
Skarzynski, Henryk; Lorens, Artur; Kruszynska, Marika; Obrycka, Anita; Pastuszak, Dorota; Skarzynski, Piotr Henryk
Cochlear implants improve the hearing abilities of individuals with unilateral hearing loss and no tinnitus. The benefit is no different from that seen in patients with unilateral hearing loss and incapacitating tinnitus. To evaluate hearing outcomes after cochlear implantation in individuals with unilateral hearing loss and no tinnitus and compare them to those obtained in a similar group who had incapacitating tinnitus. Six cases who did not experience tinnitus before operation and 15 subjects with pre-operative tinnitus were evaluated with a structured interview, a monosyllabic word test under difficult listening situations, a sound localization test, and an APHAB (abbreviated profile of hearing aid benefit) questionnaire. All subjects used their cochlear implant more than 8 hours a day, 7 days a week. In 'no tinnitus' patients, mean benefit of cochlear implantation was 19% for quiet speech, 15% for speech in noise (with the same signal-to-noise ratio in the implanted and non-implanted ear), and 16% for a more favourable signal-to-noise ratio at the implanted ear. Sound localization error improved by an average of 19°. The global score of APHAB improved by 16%. The benefits across all evaluations did not differ significantly between the 'no tinnitus' and 'tinnitus' groups.
Hua, Chiaho; Bass, Johnnie K.; Khan, Raja; Kun, Larry E.; Merchant, Thomas E.
Purpose: To determine the effect of cochlear dose on sensorineural hearing loss in pediatric patients with brain tumor treated by using conformal radiation therapy (CRT). Patients and Methods: We studied 78 pediatric patients (155 ears) with localized brain tumors treated in 1997-2001 who had not received platinum-based chemotherapy and were followed up for at least 48 months. They were evaluated prospectively by means of serial pure-tone audiograms (250 Hz-8 kHz) and/or auditory brainstem response before and every 6 months after CRT. Results: Hearing loss occurred in 14% (11 of 78) of patients and 11% (17 of 155) of cochleae, with onset most often at 3-5 years after CRT. The incidence of hearing loss was low for a cochlear mean dose of 30 Gy or less and increased at greater than 40-45 Gy. Risk was greater at high frequencies (6-8 kHz). In children who tested abnormal for hearing, average hearing thresholds increased from a less than 25 decibel (dB) hearing level (HL) at baseline to a mean of 46 ± 13 (SD) dB HL for high frequencies, 41 ± 7 dB HL for low frequencies, and 38 ± 6 dB HL for intermediate frequencies. Conclusions: Sensorineural hearing loss is a late effect of CRT. In the absence of other factors, including ototoxic chemotherapy, increase in cochlear dose correlates positively with hearing loss in pediatric patients with brain tumor. To minimize the risk of hearing loss for children treated with radiation therapy, a cumulative cochlear dose less than 35 Gy is recommended for patients planned to receive 54-59.4 Gy in 30-33 treatment fractions
Attias, Joseph; Hod, Roy; Raveh, Eyal; Mizrachi, Aviram; Avraham, Karen B; Lenz, Danielle R; Nageris, Ben I
The mechanism and the type of hearing loss induced by cochlear implants are mostly unknown. Therefore, this study evaluated the impact and type of hearing loss induced by each stage of cochlear implantation surgery in an animal model. Original basic research animal study. The study was conducted in a tertiary, university-affiliated medical center in accordance with the guidelines of the Institutional Animal Care and Use Committee. Cochlear implant electrode array was inserted via the round window membrane in 17 ears of 9 adult-size fat sand rats. In 7 ears of 5 additional animals round window incision only was performed, followed by patching with a small piece of periosteum (control). Hearing thresholds to air (AC) and bone conduction (BC), clicks, 1 kHz and 6 kHz tone bursts were measured by auditory brainstem evoked potential, before, during each stage of surgery and one week post-operatively. In addition, inner ear histology was performed. The degree of hearing loss increased significantly from baseline throughout the stages of cochlear implantation surgery and up to one week after (plosses were found for 1-kHz and 6-kHz frequencies. The hearing loss was not associated with significant changes in inner ear histology. Hearing loss following cochlear implantation in normal hearing animals is progressive and of mixed type, but mainly conductive. Changes in the inner-ear mechanism are most likely responsible for the conductive hearing loss. Copyright © 2016 Elsevier Inc. All rights reserved.
Fereczkowski, Michal; Jepsen, Morten Løve; Dau, Torsten
It has been suggested that the level corresponding to the knee-point of the basilar membrane (BM) input/output (I/O) function can be used to estimate the amount of inner- and outer hair-cell loss (IHL, OHL) in listeners with a moderate cochlear hearing impairment Plack et al. (2004). According...... to Jepsen and Dau (2011) IHL + OHL = HLT [dB], where HLT stands for total hearing loss. Hence having estimates of the total hearing loss and OHC loss, one can estimate the IHL. In the present study, results from forward masking experiments based on temporal masking curves (TMC; Nelson et al., 2001...... estimates of the knee-point level. Further, it is explored whether it is possible to estimate the compression ratio using only on-frequency TMCs. 10 normal-hearing and 10 hearing-impaired listeners (with mild-to-moderate sensorineural hearing loss) were tested at 1, 2 and 4 kHz. The results showed...
Brandt, CT; Caye-Thomsen, P; Lund, SP
of an augmented neutrophil response on the development of hearing loss and cochlear damage in a model of experimental pneumococcal meningitis in rats. Hearing loss and cochlear damage were assessed by distortion product oto-acoustic emissions (DPOAE), auditory brainstem response (ABR) and histopathology in rats...... infection. Pretreatment with G-CSF increased hearing loss 24 h after infection and on day 8 compared to untreated rats (Mann-Whitney, P = 0.012 and P = 0.013 respectively). The increased sensorineural hearing loss at day 8 was associated with significantly decreased spiral ganglion cell counts (P = 0...
Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya
Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.
Full Text Available Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months. Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.
Derinsu, Ufuk; Serin, Gediz Murat; Akdaş, Ferda; Batman, Çağlar
The goal of the cochlear implant surgery is to place the electrode array with minimal damage to preserve the residual hearing. Round-window insertion can be performed in a manner that is potentially less traumatic than the standard cochleostomy. The purpose of the study was to investigate audiological results of the round-window approach using standard electrode. A retrospective study was performed to evaluate our experience in patients with implanted through round window between January 2007 and March 2009. Sixty patients had undergone cochlear implant surgery through the round window with full insertion of a standard electrode array. Preoperative and postoperative pure-tone thresholds were measured for implanted ears in the range of 250 to 4000 Hz. Within these 60 cases, 31 patients had been evaluated. The population comprised 16 women and 15 men. The mean age was 15.96 years (range, 4-64 years). Follow-up times ranged from 6 to 26 months. Preservation of low-frequency hearing (250 and 500 Hz) was achieved in 27 (87%) of 31 patients. Complete hearing preservation (all frequencies) was accomplished in 11 patients (35.48%). No hearing could be determined postoperatively in 4 patients (12.9%), having preoperative thresholds of 120 dB at 250, 500, and 1000 Hz. Round-window approach has been widely used for preservation of residual hearing. In our patients with severe to profound hearing loss, we preserved residual hearing. Although the residual hearing cannot be sufficient for using additional acoustic stimulation, the preserved residual hearing means minimal damage and a more convenient cochlea, so this is promising for future development.
Myung, Nam-Suk; Lee, Il-Woo; Goh, Eui-Kyung; Kong, Soo-Keun
Lightning strike can produce an array of clinical symptoms and injuries. It may damage multiple organs and cause auditory injuries ranging from transient hearing loss and vertigo to complete disruption of the auditory system. Tympanic-membrane rupture is relatively common in patients with lightning injury. The exact pathogenetic mechanisms of auditory lesions in lightning survivors have not been fully elucidated. We report the case of a 45-year-old woman with bilateral profound sensorineural hearing loss caused by a lightning strike, who was successfully rehabilitated after a cochlear implantation. Copyright © 2012 Elsevier Inc. All rights reserved.
Scharp, Kristina M.; Barker, Brittan A.; Rucker, Sidney N.; Jones, Hannah D.
We aimed to determine the types of identities hearing parents construct when telling online stories about their children with hearing loss (HL) who use cochlear implants (CIs). To do so, we employed a qualitative design and sampled 20 different blogs United States origins and written by parents of children who use CIs. We then used thematic…
Teresa María Lizcano Tejado
Full Text Available Hearing loss is a significant public health problem. The incidence is difficult to establish because of the lack of data in people under age three, but is estimated about 1 per thousand for severe and profound hearing loss.A cochlear implant (CI is a device that converts sounds into electrical energy that triggers a sensation of hearing. The IC is indicated in patients with severe bilateral sensorineural hearing loss with null or poor benefit use of hearing aids.The general objective of this project is to understand the experiences of adult patients with severe-profound sensorineural hearing loss with IC postlingually throughout the implementation process.A personal vision of those implemented will allow us to learn how to face the possibility to hear and interact with their environment, applying this information to improve health care provided to them and identifying those areas where such assistance should be improved. Also allow us to compare the initial expectations and have been achieved, creating realistic expectations for future candidates.For its development we have designed a qualitative study, based on the principles and procedures of grounded theory, semistructured interviews, participant observation and discussion groups.The data will be analyzed using the software Nudist ViVo 9.
Reiss, Lina A J; Stark, Gemaine; Nguyen-Huynh, Anh T; Spear, Kayce A; Zhang, Hongzheng; Tanaka, Chiemi; Li, Hongzhe
Hybrid or electro-acoustic stimulation (EAS) cochlear implants (CIs) are designed to provide high-frequency electric hearing together with residual low-frequency acoustic hearing. However, 30-50% of EAS CI recipients lose residual hearing after implantation. The objective of this study was to determine the mechanisms of EAS-induced hearing loss in an animal model with high-frequency hearing loss. Guinea pigs were exposed to 24 h of noise (12-24 kHz at 116 dB) to induce a high-frequency hearing loss. After recovery, two groups of animals were implanted (n = 6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no stimulation during this time frame. A third group (n = 6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem responses were recorded biweekly to monitor changes in hearing. The organ of Corti was immunolabeled with phalloidin, anti-CtBP2, and anti-GluR2 to quantify hair cells, ribbons and post-synaptic receptors. The lateral wall was immunolabeled with phalloidin and lectin to quantify stria vascularis capillary diameters. Bimodal or trimodal diameter distributions were observed; the number and location of peaks were objectively determined using the Aikake Information Criterion and Expectation Maximization algorithm. Noise exposure led to immediate hearing loss at 16-32 kHz for all groups. Cochlear implantation led to additional hearing loss at 4-8 kHz; this hearing loss was negatively and positively correlated with minimum and maximum peaks of the bimodal or trimodal distributions of stria vascularis capillary diameters, respectively. After chronic stimulation, no significant group changes in thresholds were seen; however, elevated thresholds at 1 kHz in implanted, stimulated animals were significantly correlated with decreased presynaptic ribbon and postsynaptic receptor counts. Inner and outer hair cell counts did not differ between groups and
Shea, J J
In summary, fluctuant hearing loss is defined as a disorder of the inner ear characterized by fullness, roaring tinnitus, and fluctuations in hearing. It is believed to be caused by an inadequate absorption of endolymph from the endolymphatic sac, with or without one or more metabolic disorders, that interferes with the delicate balance between the production and absorption of endolymph and thus produces cochlear hydrops. This triad of fullness, roaring tinnitus, and fluctuant hearing loss resulting from cochlear hydrops is much more common than the quadrad of true turning vertigo, fullness, roaring tinnitus, and fluctuant hearing loss due to vestibular and cochlear hydrops known as Meniere's disease. Although patients with fluctuant hearing loss only may eventually develop vertigo as the chief complaint and then be said to have Meniere's disease, it is remarkable how many patients continue to suffer mainly from cochlear symptoms at all times. It would appear, because of the greater frequency of fluctuant hearing loss than in Meniere's disease, that the cochlear labyrinth is more susceptible to hydrops than the vestibular labyrinth. For the purposes of diagnosis and treatment it is very useful to separate patients into those with fluctuant hearing loss and those with Meniere's disease.
Chole, Richard A; Hullar, Timothy E; Potts, Lisa G
Changes in auditory thresholds following cochlear implantation are generally assumed to be due to damage to neural elements. Theoretical studies have suggested that placement of a cochlear implant can cause a conductive hearing loss. Identification of a conductive component following cochlear implantation could guide improvements in surgical techniques or device designs. The purpose of this study is to characterize new-onset conductive hearing losses after cochlear implantation. In a prospective study, air- and bone-conduction audiometric testing were completed on cochlear implant recipients. An air-bone gap equal to or greater than 15 dB HL at 2 frequencies determined the presence of a conductive component. Of the 32 patients with preoperative bone-conduction hearing, 4 patients had a new-onset conductive component resulting in a mixed hearing loss, with air-conduction thresholds ranging from moderate to profound and an average air-bone gap of 30 dB HL. One had been implanted through the round window, 2 had an extended round window, and 1 had a separate cochleostomy. Loss of residual hearing following cochlear implantation may be due in part to a conductive component. Identifying the mechanism for this conductive component may help minimize hearing loss. Postoperative hearing evaluation should measure both air- and bone-conduction thresholds.
Zachary, Stephen Paul; Fuchs, Paul Albert
Hearing loss among the elderly correlates with diminished social, mental, and physical health. Age-related cochlear cell death does occur, but growing anatomical evidence suggests that synaptic rearrangements on sensory hair cells also contribute to auditory functional decline. Here we present voltage-clamp recordings from inner hair cells of the C57BL/6J mouse model of age-related hearing loss, which reveal that cholinergic synaptic inputs re-emerge during aging. These efferents are functionally inhibitory, using the same ionic mechanisms as do efferent contacts present transiently before the developmental onset of hearing. The strength of efferent inhibition of inner hair cells increases with hearing threshold elevation. These data indicate that the aged cochlea regains features of the developing cochlea and that efferent inhibition of the primary receptors of the auditory system re-emerges with hearing impairment. Synaptic changes in the auditory periphery are increasingly recognized as important factors in hearing loss. To date, anatomical work has described the loss of afferent contacts from cochlear hair cells. However, relatively little is known about the efferent innervation of the cochlea during hearing loss. We performed intracellular recordings from mouse inner hair cells across the lifespan and show that efferent innervation of inner hair cells arises in parallel with the loss of afferent contacts and elevated hearing threshold during aging. These efferent neurons inhibit inner hair cells, raising the possibility that they play a role in the progression of age-related hearing loss. Copyright © 2015 the authors 0270-6474/15/359701-06$15.00/0.
Viana, Lucas M; O'Malley, Jennifer T; Burgess, Barbara J; Jones, Dianne D; Oliveira, Carlos A C P; Santos, Felipe; Merchant, Saumil N; Liberman, Leslie D; Liberman, M Charles
Recent animal work has suggested that cochlear synapses are more vulnerable than hair cells in both noise-induced and age-related hearing loss. This synaptopathy is invisible in conventional histopathological analysis, because cochlear nerve cell bodies in the spiral ganglion survive for years, and synaptic analysis requires special immunostaining or serial-section electron microscopy. Here, we show that the same quadruple-immunostaining protocols that allow synaptic counts, hair cell counts, neuronal counts and differentiation of afferent and efferent fibers in mouse can be applied to human temporal bones, when harvested within 9 h post-mortem and prepared as dissected whole mounts of the sensory epithelium and osseous spiral lamina. Quantitative analysis of five "normal" ears, aged 54-89 yrs, without any history of otologic disease, suggests that cochlear synaptopathy and the degeneration of cochlear nerve peripheral axons, despite a near-normal hair cell population, may be an important component of human presbycusis. Although primary cochlear nerve degeneration is not expected to affect audiometric thresholds, it may be key to problems with hearing in noise that are characteristic of declining hearing abilities in the aging ear. Copyright © 2015 Elsevier B.V. All rights reserved.
Priscila Carvalho Miranda
Full Text Available In the past, it was thought that hearing loss patients with residual low-frequency hearing would not be good candidates for cochlear implantation since insertion was expected to induce inner ear trauma. Recent advances in electrode design and surgical techniques have made the preservation of residual low-frequency hearing achievable and desirable. The importance of preserving residual low-frequency hearing cannot be underestimated in light of the added benefit of hearing in noisy atmospheres and in music quality. The concept of electrical and acoustic stimulation involves electrically stimulating the nonfunctional, high-frequency region of the cochlea with a cochlear implant and applying a hearing aid in the low-frequency range. The principle of preserving low-frequency hearing by a “soft surgery” cochlear implantation could also be useful to the population of children who might profit from regenerative hair cell therapy in the future. Main aspects of low-frequency hearing preservation surgery are discussed in this review: its brief history, electrode design, principles and advantages of electric-acoustic stimulation, surgical technique, and further implications of this new treatment possibility for hearing impaired patients.
Full Text Available Hearing impairment is common in patients with mitochondrial disorders, affecting over half of all cases at some time in the course of the disease. In some patients, deafness is only part of a multisystem disorder. By contrast, there are also a number of “pure” mitochondrial deafness disorders, the most common probably being maternally inherited. We retrospectively analyzed the last 60 genetically confirmed mitochondrial disorders diagnosed in our Department: 28 had bilateral sensorineural hearing loss, whereas 32 didn't present ear's abnormalities, without difference about sex and age of onset between each single group of diseases. We reported also a case of MELAS patient with sensorineural hearing loss, in which cochlear implantation greatly contributed to the patient's quality of life. Our study suggests that sensorineural hearing loss is an important feature in mitochondrial disorders and indicated that cochlear implantation can be recommended for patients with MELAS syndrome and others mitochondrial disorders.
Távora-Vieira, Dayse; Marino, Roberta; Acharya, Aanand; Rajan, Gunesh P
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.
Werfel, Krystal L.; Douglas, Michael; Ackal, Leigh
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…
Homøe, Preben; Andersen, Ture; Grøntved, Aksel
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...
Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya
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...
Hassan, Hatem Ezzeldin; Eldin, Sally Taher Kheir; Al Kasaby, Rasha Mohamed
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...
Jamesdaniel, Samson; Rosati, Rita; Westrick, Judy; Ruden, Douglas M
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.
Endo, Shiori; Mizuta, Kunihiro; Yamatodani, Takashi; Nakanishi, Hiroshi; Hosokawa, Kumiko; Misawa, Kiyoshi; Hosokawa, Seiji; Mineta, Hiroyuki
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.
Carraro, Mattia; Almishaal, Ali; Hillas, Elaine; Firpo, Matthew; Park, Albert; Harrison, Robert V
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.
Marnane, Vivienne; Ching, Teresa YC
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
Lachowska, Magdalena; Pastuszka, Agnieszka; Łukaszewicz-Moszyńska, Zuzanna; Mikołajewska, Lidia; Niemczyk, Kazimierz
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.
Purcell, Patricia L; Shinn, Justin R; Coggeshall, Scott S; Phillips, Grace; Paladin, Angelisa; Sie, Kathleen C Y; Horn, David L
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.
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,
Ramakers, Geerte G J; van Zon, Alice; Stegeman, Inge; Grolman, Wilko
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
Wong, Cara L; Ching, Teresa Y C; Cupples, Linda; Button, Laura; Leigh, Greg; Marnane, Vivienne; Whitfield, Jessica; Gunnourie, Miriam; Martin, Louise
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
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
Clarkson, Cheryl; Antunes, Flora M; Rubio, Maria E
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
Yoshinaga-Itano, Christine; Baca, Rosalinda L; Sedey, Allison L
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
Guilherme Machado de Carvalho
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.
Zong, Liang; Chen, Jin; Zhu, Yan; Zhao, Hong-Bo
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.
Warner-Czyz, Andrea D.; Loy, Betty A.; Evans, Christine; Wetsel, Ashton; Tobey, Emily A.
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...
Buus, Søren; Florentine, Mary; Poulsen, Torben
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...
Serin, Gediz Murat; Derinsu, Ufuk; Sari, Murat; Gergin, Ozgül; Ciprut, Ayça; Akdaş, Ferda; Batman, Cağlar
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.
Tian, Yanjing; Zhou, Huifang; Zhang, Jing; Yang, Dong; Xu, Yi; Guo, Yuxi
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.
Nordfalk, Karl Fredrik; Rasmussen, Kjell; Hopp, Einar; Greisiger, Ralf; Jablonski, Greg Eigner
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.
Nash-Kille, Amy; Sharma, Anu
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
Doherty, Joni K; Linthicum, Fred H
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.
Warner-Czyz, Andrea D; Loy, Betty A; Evans, Christine; Wetsel, Ashton; Tobey, Emily A
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.
Loy, Betty A.; Evans, Christine; Wetsel, Ashton; Tobey, Emily A.
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
Wang, Ye; Qiao, Li; Chen, Yang; Wen, Liting; Yue, Bo; Qiu, Jianhua; Wu, Shengxi
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.
Andrea D. Warner-Czyz
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.
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.
Fagan, Mary K; Pisoni, David B
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.
Segal, Osnat; Kishon-Rabin, Liat
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.
Huang, Y Y; Chen, J Y; Shen, M; Yang, J
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.
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.
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.
Worsøe, Lise Lotte; Brandt, C.T.; Lund, S.P.
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....
Jepsen, Morten Løve; Dau, Torsten
–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...
Masoud Motasaddi Zarandy
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.
Takenaka, Mika; Tono, Tetsuya; Toyama, Katsuhiro; Kano, Kiyo; Morimitsu, Tamotsu
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)
Worsøe, Lise Lotte; Brandt, C.T.; Lund, S.P.
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...
Laccourreye, L; Ettienne, V; Prang, I; Couloigner, V; Garabedian, E-N; Loundon, N
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.
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.
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.
Whittemore, Kenneth R; Dornan, Briana K; Lally, Tara; Dargie, Jenna M
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.
Santos, Saturnino; Domínguez, M Jesús; Cervera, Javier; Suárez, Alicia; Bueno, Antonio; Bartolomé, Margarita; López, Rafael
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.
Peters, Jeroen P M; Ramakers, Geerte G J; Smit, Adriana L; Grolman, Wilko
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.
Weiss, Bernhard G; Bertlich, Mattis; Bettag, Stephan A; Desinger, Hendrik; Ihler, Friedrich; Canis, Martin
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.
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
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.
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.
Beers, Alison N; McBoyle, Melanie; Kakande, Emily; Dar Santos, Rachelle C; Kozak, Frederick K
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
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
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.
So Young Park
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.
Oshima, Kazuo; Suchert, Steffen; Blevins, Nikolas H; Heller, Stefan
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.
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
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.
Chiossi, Julia Santos Costa; Hyppolito, Miguel Angelo
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.
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
Papsin, Blake C.; Paludetti, Gaetano; Gordon, Karen A.
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
Kretschmer, Richard R.; Kretschmer, Laura; Kuwahara, Katsura; Truax, Roberta
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.…
Mertens, Griet; Kleine Punte, Andrea; De Bodt, Marc; Van de Heyning, Paul
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.
Morrison, Helen Mccaffrey
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…
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
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.
Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun
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
Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun [College of Medicine, Yonsei Univ., Wonju (Korea, Republic of)
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.
Havenith, Sarah; Klis, Sjaak F L; Versnel, Huib; Grolman, Wilko
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.
Gfeller, Kate; Olszewski, Carol; Rychener, Marly; Sena, Kimberly; Knutson, John F; Witt, Shelley; Macpherson, Beth
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
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)
Jason A. Brant
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 20â25Â 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
A. V. Starokha
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
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.
Ertmer, David J.
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…
André L. L. Sampaio
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.
Bunta, Ferenc; Douglas, Michael; Dickson, Hanna; Cantu, Amy; Wickesberg, Jennifer; Gifford, René H.
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…
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
Money, M K; Pippin, G W; Weaver, K E; Kirsch, J P; Webster, D B
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.
Sanchez, Tanit Ganz; Rocha, Savya Cybelle Milhomem; Knobel, Keila Alessandra Baraldi; Kii, Márcia Akemi; Santos, Rosa Maria Rodrigues dos; Pereira, Cristiana Borges
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.
Santurette, Sébastien; Bianchi, Federica; Dau, Torsten
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....
Pillion, Joseph P.; Vernick, David; Shapiro, Jay
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
Blake S. Wilson
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
Hunter, Jacob B; Gifford, René H; Wanna, George B; Labadie, Robert F; Bennett, Marc L; Haynes, David S; Rivas, Alejandro
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.
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.
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...
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
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.
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.
Bishop, Charles E; Eby, Thomas L
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.
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.
Nittrouer, Susan; Lowenstein, Joanna H.
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,…
Kurabi, Arwa; Keithley, Elizabeth M; Housley, Gary D; Ryan, Allen F; Wong, Ann C-Y
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.
Seyede Faranak Emami
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.
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.
Stähr, Kerstin; Kuechler, Alma; Gencik, Martin; Arnolds, Judith; Dendy, Meaghan; Lang, Stephan; Arweiler-Harbeck, Diana
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.
Monshizadeh, Leila; Vameghi, Roshanak; Sajedi, Firoozeh; Yadegari, Fariba; Hashemi, Seyed Basir; Kirchem, Petra; Kasbi, Fatemeh
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.
Albera, Roberto; Dagna, Federico; Cassandro, Claudia; Canale, Andrea
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.
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.
Chen, Wei; Wang, Jianmin; Chen, Jing; Chen, Jichuan; Chen, Zhiqiang
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.
Nelson, Erik G; Hinojosa, Raul
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.
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.
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...
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.
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: email@example.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)
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.
Dalbert, Adrian; Pfiffner, Flurin; Hoesli, Marco; Koka, Kanthaiah; Veraguth, Dorothe; Roosli, Christof; Huber, Alexander
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...
Kidd, Ambrose R; Bao, Jianxin
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
Jennifer L. Hatton
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.
Hatton, Jennifer L.; Janssen, Renée M.; Stapells, David R.
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
Zahedi, F.D.; Rahman, R.A.; Abdullah, A.
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)
Richard R. Kretschmer
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.
Kalathottukaren, Rose Thomas; Purdy, Suzanne C; Ballard, Elaine
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
Derinsu, Ufuk; Ciprut, Ayca
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...
Huang, Xinghua; Chen, Mo; Ding, Yan; Wang, Qin
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.
Schuurbiers, Jasper; Dingemanse, Gertjan; Metselaar, Mick
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
Mauermann, M; Uppenkamp, S; van Hengel, P.W.J.; Kollmeier, B
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
Wiefferink, Carin H; Rieffe, Carolien; Ketelaar, Lizet; Frijns, Johan H M
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.
Oryadi-Zanjani, Mohammad Majid; Vahab, Maryam; Rahimi, Zahra; Mayahi, Anis
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
Bianchi, Federica; Fereczkowski, Michal; Zaar, Johannes
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...
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 ...
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
Rao, Deepti; Basura, Gregory J; Roche, Joseph; Daniels, Scott; Mancilla, Jaime G; Manis, Paul B
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
Härkönen, Kati; Kivekäs, Ilkka; Kotti, Voitto; Sivonen, Ville; Vasama, Juha-Pekka
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.
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
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
Percy-Smith, L.; Caye-Thomasen, P.; Gudman, M.
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...
Jill B Firszt
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
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.
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.
... 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 ...
Bento, Ricardo Ferreira
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.
Lenarz, Minoo; Sönmez, Hasibe; Joseph, Gert; Büchner, Andreas; Lenarz, Thomas
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.
Choi, June; Payne, Matthew R; Campbell, Luke J; Bester, Christo W; Newbold, Carrie; Eastwood, Hayden; O'Leary, Stephen J
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.
Whitlon, Donna S
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.
Gregan, Melanie J.; Nelson, Peggy B.; Oxenham, Andrew J.
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
Jacob, R; Stelzig, Y
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.
Postelmans, Job T. F.; van Spronsen, Erik; Grolman, Wilko; Stokroos, Robert J.; Tange, Rinze A.; Maré, Marcel J.; Dreschler, Wouter A.
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
Jakubíková, Janka; Kabátová, Zuzana; Pavlovcinová, Gabriela; Profant, Milan
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
Almeida-Branco, Mario S; Cabrera, Sonia; Lopez-Escamez, Jose A
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.
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].
Campos-Bañales, Eugenia María; López-Campos, Daniel; de Serdio-Arias, José Luis; Esteban-Rodriguez, J; García-Sáinz, Mar; Muñoz-Cortés, Álvaro; López-Aguado, Daniel
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.
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.
Phelan, E; Pal, R; Henderson, L; Green, K M J; Bruce, I A
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.
Nittrouer, Susan; Sansom, Emily; Low, Keri; Rice, Caitlin; Caldwell-Tarr, Amanda
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
Petersen, Bjørn; Gjedde, Albert; Wallentin, Mikkel
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...
Abdala, Carolina; Fitzgerald, Tracy S.
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.
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
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.
Hill, Kayla; Yuan, Hu; Wang, Xianren
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
Hill, Kayla; Yuan, Hu; Wang, Xianren; Sha, Su-Hua
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.
Peter T. Johannesen
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.
de Souza Melo, Renato
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.
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.
Aronoff, Justin M.; Freed, Daniel J.; Fisher, Laurel M.; Pal, Ivan; Soli, Sigfrid D.
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
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....
Harris, Suzan L; Kazmierczak, Marcin; Pangršič, Tina; Shah, Prahar; Chuchvara, Nadiya; Barrantes-Freer, Alonso; Moser, Tobias; Schwander, Martin
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.
Chung, Jae Ho; Shin, Myung Chul; Min, Hyun Jung; Park, Chul Won; Lee, Seung Hwan
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.
Cullen, Robert D; Zdanski, Carlton; Roush, Patricia; Brown, Carolyn; Teagle, Holly; Pillsbury, Harold C; Buchman, Craig
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.
Zheng, Yan; Liu, Bo; Dong, Ruijuan; Xu, Tianqiu; Chen, Jing; Chen, Xuejing; Zhong, Yan; Meng, Chao; Wang, Hong; Chen, Xueqing
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.
Ramakers, Geerte G.J.; Smulders, Yvette E.; Van Zon, Alice; van Zanten, Gijsbert A.; Grolman, Wilko; Stegeman, Inge
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
Goldblat, Ester; Most, Tova
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.
Dammeyer, Jesper Herup
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...
Most, Tova; Aviner, Chen
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.…
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.
Calton, Melissa A; Lee, Dasom; Sundaresan, Srividya; Mendus, Diana; Leu, Rose; Wangsawihardja, Felix; Johnson, Kenneth R; Mustapha, Mirna
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.
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
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.
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
Kerr, Rhorie; Kang, Elise; Hopkins, Brandon; Anne, Samantha
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.
Fukushima, Kunihiro; Kawasaki, Akihiro; Nagayasu, Rie; Kunisue, Kazuya; Maeda, Yukihide; Kariya, Shin; Kataoka, Yuko; Nishizaki, Kazunori
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.
Fitzpatrick, Elizabeth; Coyle, Douglas E; Durieux-Smith, Andrée; Graham, Ian D; Angus, Douglas E; Gaboury, Isabelle
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
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
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.
Hinojosa, Raul; Nelson, Erik G
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.
Martinez, Manuel; Bruno, Claudio; Martin, Eduardo; Canale, Nancy; De Luca, Laura; Spina, Juan C. h
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)
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.
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
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.
Amaral, Maria Stella Arantes do; Reis, Ana Cláudia Mirândola B; Massuda, Eduardo T; Hyppolito, Miguel Angelo
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.
Rivera, Diego; Saab, Said; Cordoba, Claudia; Montes, Guillermo; Barreto, Tatiana
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.
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
ANNA R. eFETONI
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.
Fetoni, Anna R; Eramo, Sara Letizia Maria; Di Pino, Antonella; Rolesi, Rolando; Paciello, Fabiola; Grassi, Claudio; Troiani, Diana; Paludetti, Gaetano
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.
... 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.
Claes, Annes J; Mertens, Griet; Gilles, Annick; Hofkens-Van den Brandt, Anouk; Fransen, Erik; Van Rompaey, Vincent; Van de Heyning, Paul
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
Snik, Ad; Agterberg, Martijn; Bosman, Arjan
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.
Waniusiow, Delphine; Campo, Pierre; Venet, Thomas; Cossec, Benoît; Cosnier, Frédéric; Beydon, Dominique; Rieger, Benoît; Burgart, Manuella; Ferrari, Luc; Parietti-Winkler, Cécile
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
Gabriela Ribeiro Ivo Rodrigues
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
R.N. Rainey (Robert N.); S.-Y. Ng (Sum-Yan); J. Llamas (Juan); G.T.J. van der Horst (Gijsbertus); N. Segil (Neil)
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
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
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
Vallés-Varela, Héctor; Royo-López, Juan; Carmen-Sampériz, Luis; Sebastián-Cortés, José M; Alfonso-Collado, Ignacio
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.
Valero, M D; Burton, J A; Hauser, S N; Hackett, T A; Ramachandran, R; Liberman, M C
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.
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
Sininger, Yvonne S; Grimes, Alison; Christensen, Elizabeth
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
Derinsu, Ufuk; Ciprut, Ayca
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...
Sułkowski, Wiesław; Owczarek, Kalina; Olszewski, Jurek
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
Zupan, Barbra; Sussman, Joan E
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.
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.
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.
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
Oryadi-Zanjani, Mohammad Majid; Vahab, Maryam; Bazrafkan, Mozhdeh; Haghjoo, Asghar
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.
M Charles Liberman
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.
Zwolan, T A; Shepard, N T; Niparko, J K
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.
Caye-Thomasen, Per; Dam, Mikkel Seidelin; Omland, Silje Haukali
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....
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 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
Gu, Lintao; Cui, Xinhua; Wei, Wei; Yang, Jia; Li, Xuezhong
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.
Yamasoba, Tatsuya; Lin, Frank R.; Someya, Shinichi; Kashio, Akinori; Sakamoto, Takashi; Kondo, Kenji
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
Kempfle, Judith S; Nguyen, Kim; Hamadani, Christine; Koen, Nicholas; Edge, Albert S; Kashemirov, Boris A; Jung, David H; McKenna, Charles E
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.
Rhee, Chung-Ku; He, Peijie; Jung, Jae Yun; Ahn, Jin-Chul; Chung, Phil-Sang; Lee, Min Young; Suh, Myung-Whan
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.
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.
Lin, Karen; Marrinan, Michelle S; Waltzman, Susan B; Roland, J Thomas
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.
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 . 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.
Marlene E Gubata
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.
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.
Sahli, Sanem; Belgin, Erol
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
de Oliveira, Adriana Kosma Pires; Hamerschmidt, Rogerio; Mocelin, Marcos; Rezende, Rodrigo K
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.
Oliveira, Adriana Kosma Pires de
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.
Choi, Ji Eun; Won, Jong Ho; Kim, Cheol Hee; Cho, Yang-Sun; Hong, Sung Hwa; Moon, Il Joon
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.
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.
Brand, Yves; Senn, Pascal; Kompis, Martin; Dillier, Norbert; Allum, John H. J.
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...
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.
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.
Grau, C.; Moller, K.; Overgaard, M.; Overgaard, J.; Elbrond, O.
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
Most, Tova; Rothem, Hilla; Luntz, Michal
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…
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
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.
Fischer, N; Weber, B; Riechelmann, H
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.
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.
Fuller, Christina; Free, Rolien; Maat, Bert; Başkent, Deniz
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.
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Scarbel, Lucie; Beautemps, Denis; Schwartz, Jean-Luc; Sato, Marc
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.
Tomblin, J Bruce; Walker, Elizabeth A; McCreery, Ryan W; Arenas, Richard M; Harrison, Melody; Moeller, Mary Pat
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
Zhang, Dao-xing; Hu, Bao-hua; Xiao, Yu-li; Shi, Bo-ning
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.
Anacléia Melo da Silva Hilgenberg
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.
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.
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
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.
Asad, Areej Nimer; Purdy, Suzanne C; Ballard, Elaine; Fairgray, Liz; Bowen, Caroline
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.
Cejas, Ivette; Hoffman, Michael F; Quittner, Alexandra L
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.
... 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 ...
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.
Rohlfs, Anna-Katharina; Friedhoff, Johannes; Bohnert, Andrea; Breitfuss, Achim; Hess, Markus; Müller, Frank; Strauch, Anke; Röhrs, Marianne; Wiesner, Thomas
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
Tran, LenhAnh P.; Grundfast, Kenneth M.
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…
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
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.
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.
Daneshi, Ahmad; Hassanzadeh, Saeid; Abasalipour, Parvaneh; Emamdjomeh, Hessamaddin; Farhadi, Mohammad
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
Noble, Jack H.; Rutherford, Robert B.; Labadie, Robert F.; Majdani, Omid; Dawant, Benoit M.
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.
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...
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.
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
Magalhães, Ana Tereza de Matos
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.
Magalhães, Ana Tereza de Matos; Samuel, Paola Angélica; Goffi-Gomez, Maria Valeria Schimdt; Tsuji, Robinson Koji; Brito, Rubens; Bento, Ricardo Ferreira
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.
Tan Xiuzhong; Zhong Lansheng; Lan Bowen; Huang Yaosheng; Du Baowen; Zhu Jian
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)
Huo, Liang; Wang, Hua
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.
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.
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.
Segal, Nili; Shkolnik, Mark; Kochba, Anat; Segal, Avichai; Kraus, Mordechai
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.
... 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 ...
Rainey, Robert N.; Ng, Sum-yan; Llamas, Juan; van der Horst, Gijsbertus T. J.
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
Fitzpatrick, Elizabeth M; Hamel, Candyce; Stevens, Adrienne; Pratt, Misty; Moher, David; Doucet, Suzanne P; Neuss, Deirdre; Bernstein, Anita; Na, Eunjung
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.
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... 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 ...
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
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.
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
Barton, GR; Fortnum, HM; Stacey, PC; Summerfield, AQ
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...
... 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 ...
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.
Zuur, Charlotte L.; Simis, Yvonne J.; Lamers, Emmy A.; Hart, Augustinus A.; Dreschler, Wouter A.; Balm, Alfons J.; Rasch, Coen R.
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.
Fu, Qian-Jie; Chinchilla, Sherol; Galvin, John J
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.
Karino, Shotaro; Yamasoba, Tatsuya; Ito, Ken; Kaga, Kimitaka
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.
Brouwer, Kyle; Downing, Hannah; Westhoff, Sara; Wait, Ryann; Entwisle, Lavin K.; Messersmith, Jessica J.; Hanson, Elizabeth K.
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…
Schraer-Joiner, Lyn; Prause-Weber, Manuela
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…
Psarommatis, Ioannis; Voudouris, Charalampos; Kapetanakis, Ioannis; Athanasiadi, Faselida; Douros, Konstantinos
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.
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.
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
Worsøe, Lise Lotte; Brandt, C.T.; Lund, S.P.
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...
Ostojić, Sanja; Djoković, Sanja; Radić-šestić, Marina; Nikolić, Mina; Mikić, Branka; Mirić, Danica
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
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
Hammer, Annemiek; Coene, Martine
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
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
Nasralla, Heloisa Romeiro
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.
Nasralla, Heloisa Romeiro; Goffi Gomez, Maria Valéria Schimidt; Magalhaes, Ana Tereza; Bento, Ricardo Ferreira
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.
Watanabe, Nobuyuki; Ishii, Takuya; Fujitsu, Kazuhiko; Kaku, Shogo; Ichikawa, Teruo; Miyahara, Kosuke; Okada, Tomu; Tanino, Shin; Uriu, Yasuhiro; Murayama, Yuichi
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.
Wang, Shuo; Dong, Ruijuan; Zhou, Yun; Li, Jing; Qi, Beier; Liu, Bo
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.
Cohen, Brandon E.; Durstenfeld, Anne
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
Siem, Geir; Fagerheim, Toril; Jonsrud, Christoffer; Laurent, Claude; Teig, Erik; Harris, Sten; Leren, Trond P; Früh, Andreas; Heimdal, Ketil
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.
Gfeller, Kate; Christ, Aaron; Knutson, John; Witt, Shelley; Mehr, Maureen
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.
Mourtou, Eleni; Meis, Markus
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...
... 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- ...
Fitzpatrick, Elizabeth M; Crawford, Leah; Ni, Andy; Durieux-Smith, Andrée
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
Franceschi, Cacineli Marion de
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.
Walby, A P; Barrera, A; Schuknecht, H F
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
Rosanna Mariangela Giaffredo Angrisani
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
Mehmet Akif Dundar
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
Fuller, Christina Diechina
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
Sun, Changling; Wang, Xueling; Chen, Dongye; Lin, Xin; Yu, Dehong; Wu, Hao
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.
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…
Bruna Natália Freire Ribeiro
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?
Kh. M. Diab
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.
Ni Luh Putu Maharani
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.
Bayazit, Yildirim A; Yilmaz, Metin
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.
Nittrouer, Susan; Caldwell-Tarr, Amanda; Tarr, Eric; Lowenstein, Joanna H; Rice, Caitlin; Moberly, Aaron C
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.
Mancini, Patrizia; D'Elia, Chiara; Bosco, Ersilia; De Seta, Elio; Panebianco, Valeria; Vergari, Valeria; Filipo, Roberto
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.
Nakamura, T.; Naganawa, S.; Fukatsu, H.; Sakurai, Y.; Ishigaki, T.; Aoki, I.; Ninomiya, A.; Nakashima, T.
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.)
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)
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.)
Hidalgo, Céline; Falk, Simone; Schön, Daniele
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.
Peterson, Candida C
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.
Kim, Kyoo Sang
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.
Chang, Mun Young; Rah, Yoon Chan; Choi, Jun Jae; Woo, Shin Wook; Hwang, Yu-Jung; Eastwood, Hayden; O'Leary, Stephen J; Lee, Jun Ho
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.
Yamaguchi, Cintia Tizue
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.
Oxenham, Andrew J.; Dau, Torsten
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....
Pouyatos, BenoIt; Gearhart, Caroline A.; Fechter, Laurence D.
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
Orendorz-Fraczkowska, Krystyna; Jaworska, Marzena; Gawron, Wojciech; Badowski, Roman; Nadolska, Beata
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.
Nabors, Laura; Odar Stough, Cathleen; Merianos, Ashley; Peugh, James
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.
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.
José Ignacio BENITO-OREJAS
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.
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.
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Turğut, Nedim; Karlıdağ, Turgut; Başar, Figen; Yalçın, Şinasi; Kaygusuz, İrfan; Keleş, Erol; Birkent, Ömer Faruk
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.
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.
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…
Teixeira, Adriane Ribeiro
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.
Sundqvist, Annette; Lyxell, Björn; Jönsson, Radoslava; Heimann, Mikael
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.
Garcia, Marcelo de Mattos; Gonzaga, Juliana Gontijo
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)
Fonseca, Vinicius Ribas; Marques, Jair; Panegalli, Flavio; Gonçalves, Claudia Giglio de Oliveira; Souza, Wesley
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.
Fonseca, Vinicius Ribas; Marques, Jair; Panegalli, Flavio; Gonçalves, Claudia Giglio de Oliveira; Souza, Wesley
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
Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.
The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems
Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.
The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.
Ito, T; Nishio, A; Wangemann, P; Griffith, A J
Hearing loss of patients with enlargement of the vestibular aqueduct (EVA) can fluctuate or progress, with overall downward progression. The most common detectable cause of EVA is mutations of SLC26A4. We previously described a transgenic Slc26a4-insufficient mouse model of EVA in which Slc26a4 expression is controlled by doxycycline administration. Mice that received doxycycline from conception until embryonic day 17.5 (DE17.5; doxycycline discontinued at embryonic day 17.5) had fluctuating hearing loss between 1 and 6 months of age with an overall downward progression after 6 months of age. In this study, we characterized the cochlear functional and structural changes underlying irreversible hearing loss in DE17.5 mice at 12 months of age. The endocochlear potential was decreased and inversely correlated with auditory brainstem response thresholds. The stria vascularis was thickened and edematous in ears with less severe hearing loss, and thinned and atrophic in ears with more severe hearing loss. There were pathologic changes in marginal cell morphology and gene expression that were not observed at 3 months. We conclude that strial dysfunction and degeneration are the primary causes of irreversible progressive hearing loss in our Slc26a4-insufficient mouse model of EVA. This model of primary strial atrophy may be used to explore the mechanisms of progressive hearing loss due to strial dysfunction. Published by Elsevier Ltd.
candidates for cochlear implantation. Keywords: cochlear implantation, elderly, audiometry, speech perception, speech recognition, hearing loss, hearing aid
Fonseca, Vinicius Ribas; Marques, Jair; Panegalli, Flavio; Gonçalves, Claudia Giglio de Oliveira; Souza, Wesley
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 thatmay exist...
Ross, Danielle S.; Holstrum, W. June; Gaffney, Marcus; Green, Denise; Oyler, Robert F.; Gravel, Judith S.
More than 90% of newborns in the United States are now being screened for hearing loss. A large fraction of cases of unilateral hearing loss and mild bilateral hearing loss are not currently identified through newborn hearing screening. This is of concern because a preponderance of research has demonstrated that unilateral hearing loss and mild bilateral hearing loss can lead to developmental delays and educational problems for some children. To help address this probable underidentification ...
Morell, Maria; Brownlow, Andrew; McGovern, Barry; Raverty, Stephen A.; Shadwick, Robert E.; André, Michel
Assessment of the impact of noise over-exposure in stranded cetaceans is challenging, as the lesions that lead to hearing loss occur at the cellular level and inner ear cells are very sensitive to autolysis. Distinguishing ante-mortem pathology from post-mortem change has been a major constraint in diagnosing potential impact. Here, we outline a methodology applicable to the detection of noise-induced hearing loss in stranded cetaceans. Inner ears from two mass strandings of long-finned pilot whales in Scotland were processed for scanning electron microscopy observation. In one case, a juvenile animal, whose ears were fixed within 4 hours of death, revealed that many sensory cells at the apex of the cochlear spiral were missing. In this case, the absence of outer hair cells would be compatible with overexposure to underwater noise, affecting the region which transduces the lowest frequencies of the pilot whales hearing spectrum. Perfusion of cochlea with fixative greatly improved preservation and enabled diagnostic imaging of the organ of Corti, even 30 hours after death. This finding supports adopting a routine protocol to detect the pathological legacy of noise overexposure in mass stranded cetaceans as a key to understanding the complex processes and implications that lie behind such stranding events.
Schendzielorz, Philipp; Vollmer, Maike; Rak, Kristen; Wiegner, Armin; Nada, Nashwa; Radeloff, Katrin; Hagen, Rudolf; Radeloff, Andreas
A cochlear implant (CI) is an electronic prosthesis that can partially restore speech perception capabilities. Optimum information transfer from the cochlea to the central auditory system requires a proper functioning auditory nerve (AN) that is electrically stimulated by the device. In deafness, the lack of neurotrophic support, normally provided by the sensory cells of the inner ear, however, leads to gradual degeneration of auditory neurons with undesirable consequences for CI performance. We evaluated the potential of adipose-derived stromal cells (ASCs) that are known to produce neurotrophic factors to prevent neural degeneration in sensory hearing loss. For this, co-cultures of ASCs with auditory neurons have been studied, and autologous ASC transplantation has been performed in a guinea pig model of gentamicin-induced sensory hearing loss. In vitro ASCs were neuroprotective and considerably increased the neuritogenesis of auditory neurons. In vivo transplantation of ASCs into the scala tympani resulted in an enhanced survival of auditory neurons. Specifically, peripheral AN processes that are assumed to be the optimal activation site for CI stimulation and that are particularly vulnerable to hair cell loss showed a significantly higher survival rate in ASC-treated ears. ASC transplantation into the inner ear may restore neurotrophic support in sensory hearing loss and may help to improve CI performance by enhanced AN survival. Copyright © 2017 International Society for Cellular Therapy. Published by Elsevier Inc. All rights reserved.
Full Text Available Background Hearing loss is developing when age is rising. Initiation and progression rates of hearing loss vary among different individuals and groups. Objectives The current study aimed to determine satisfaction of the elderly with their hearing aids in different types of hearing loss and comorbidities. Patients and Methods The study was conducted on 40 elderly subjects suffering from hearing loss and using hearing aids. The data collection method included assessment of hearing loss in addition to using a questionnaire to estimate respondents' satisfaction with their hearing aids in daily life. The Persian version of the Satisfaction with Amplification in Daily Life (SADL questionnaire was administered. The data were analyzed using descriptive and inferential statistics by SPSS software version19. Results The mean satisfaction scores of the elderly were 4.83 ± 0.51 and 5.36 ± 0.30 in the sensorineural loss groups. There was no significant difference between different comorbidities. There was a significant difference between satisfaction level of cost and services subscales in the symmetrical styles of hearing loss (P value = 0.04. Conclusions The findings of the study indicated a high satisfaction of the elderly with their hearing aids, considering the type of hearing loss. Despite all the efforts to improve the audiologic services during verification process, the elderly should be consulted specifically in order to fit their hearing aid as well as their expectations from aid.
Stevens, Madelyn N; Baudhuin, Jacqueline E; Hullar, Timothy E
Cochlear implantation is a highly effective intervention for hearing loss, but insertion of an implant into the cochlea is often accompanied by loss of residual hearing function. Sometimes, postoperative testing also shows loss of function in the semicircular canals or otolith organs. The effect of this loss on equilibrium, particularly in the short term following surgery, and the risk of falling due to this loss is unknown. We prospectively measured balance in 16 consecutive adult cochlear implant patients before and 2 weeks after surgery. Subjects stood on a foam pad with eyes closed, feet together and arms at the side. The length of time over which this posture could be maintained was recorded up to a maximum value of 30 s indicating normal performance. Ten of 16 subjects reached a maximal time on preoperative testing. Nine of 16 subjects lost balance function after surgery. Four of the 10 subjects with normal preoperative balance function lost function. Subjects older than the age of 60 were more likely to lose balance function than younger subjects. We used previously published values relating balance performance on foam to risk of falling to calculate the fall risk among our subjects. The relative risk of falling increased after surgery by more than threefold in some patients. Imbalance after cochlear implantation may be much more common, particularly in the short term, than previously appreciated. This imbalance is accompanied by an increased risk of falling in many patients. Careful preoperative counseling before implantation and postoperative therapeutic intervention to improve comfort and reduce the chance of falling may be warranted, particularly in patients at a risk for injuries from falls (level of evidence: 2b). © 2014 S. Karger AG, Basel.
Kovačić, Damir; Balaban, Evan
The study was carried out to assess the role that five hearing history variables (chronological age, age at onset of deafness, age of first cochlear implant [CI] activation, duration of CI use, and duration of known deafness) play in the ability of CI users to identify speaker gender. Forty-one juvenile CI users participated in two voice gender identification tasks. In a fixed, single-interval task, subjects listened to a single speech item from one of 20 adult male or 20 adult female speakers and had to identify speaker gender. In an adaptive speech-based voice gender discrimination task with the fundamental frequency difference between the voices as the adaptive parameter, subjects listened to a pair of speech items presented in sequential order, one of which was always spoken by an adult female and the other by an adult male. Subjects had to identify the speech item spoken by the female voice. Correlation and regression analyses between perceptual scores in the two tasks and the hearing history variables were performed. Subjects fell into three performance groups: (1) those who could distinguish voice gender in both tasks, (2) those who could distinguish voice gender in the adaptive but not the fixed task, and (3) those who could not distinguish voice gender in either task. Gender identification performance for single voices in the fixed task was significantly and negatively related to the duration of deafness before cochlear implantation (shorter deafness yielded better performance), whereas performance in the adaptive task was weakly but significantly related to age at first activation of the CI device, with earlier activations yielding better scores. The existence of a group of subjects able to perform adaptive discrimination but unable to identify the gender of singly presented voices demonstrates the potential dissociability of the skills required for these two tasks, suggesting that duration of deafness and age of cochlear implantation could have
Mehrparvar, Amir Houshang; Davari, Mohammad Hossein; Mollasadeghi, Abolfazl
Carbon monoxide poisoning is one of the rare causes of hearing loss which may cause reversible or irreversible, unilateral or bilateral hearing loss after acute or chronic exposure. In this report, we present a case of bilateral sensorineural hearing loss in a secondary smelting workshop worker...... after an acute exposure to carbon monoxide. This complication was diagnosed by pure-tone audiometry and confirmed by transient evoked otoacoustic emissions. Hearing loss has not improved after 3 months of followup....
Mazón, M; Pont, E; Albertz, N; Carreres-Polo, J; Más-Estellés, F
Hearing loss is the most frequent complication of temporal bone trauma. The role of the radiologist is of great importance; the adequacy and selection of the imaging technique, as well as its correct interpretation, are crucial to establish the diagnosis, prognosis and enable the selection of appropriate treatment. With the aim of systematizing the most relevant concepts in the evaluation of image studies in this scenario, this review will be outlined according to the hearing loss type. The potential lesions of its components will be assessed; In each case the most appropriate imaging technique will be suggested and the findings will be described and depicted. In postraumatic hearing loss, computed tomography is the initial technique of choice and will allow the detection of alterations that cause conductive hearing loss; magnetic resonance imaging will be useful in the evaluation of sensorineural hearing loss. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.
Pfingst, Bryan E; Zhou, Ning; Colesa, Deborah J; Watts, Melissa M; Strahl, Stefan B; Garadat, Soha N; Schvartz-Leyzac, Kara C; Budenz, Cameron L; Raphael, Yehoash; Zwolan, Teresa A
Amazing progress has been made in providing useful hearing to hearing-impaired individuals using cochlear implants, but challenges remain. One such challenge is understanding the effects of partial degeneration of the auditory nerve, the target of cochlear implant stimulation. Here we review studies from our human and animal laboratories aimed at characterizing the health of the implanted cochlea and the auditory nerve. We use the data on cochlear and neural health to guide rehabilitation strategies. The data also motivate the development of tissue-engineering procedures to preserve or build a healthy cochlea and improve performance obtained by cochlear implant recipients or eventually replace the need for a cochlear implant. This article is part of a Special Issue entitled . Copyright © 2014 Elsevier B.V. All rights reserved.
Gieseler, Anja; Tahden, Maike A S; Thiel, Christiane M; Colonius, Hans
There is converging evidence for altered audiovisual integration abilities in hearing-impaired individuals and those with profound hearing loss who are provided with cochlear implants, compared to normal-hearing adults. Still, little is known on the effects of hearing aid use on audiovisual integration in mild hearing loss, although this constitutes one of the most prevalent conditions in the elderly and, yet, often remains untreated in its early stages. This study investigated differences in the strength of audiovisual integration between elderly hearing aid users and those with the same degree of mild hearing loss who were not using hearing aids, the non-users, by measuring their susceptibility to the sound-induced flash illusion. We also explored the corresponding window of integration by varying the stimulus onset asynchronies. To examine general group differences that are not attributable to specific hearing aid settings but rather reflect overall changes associated with habitual hearing aid use, the group of hearing aid users was tested unaided while individually controlling for audibility. We found greater audiovisual integration together with a wider window of integration in hearing aid users compared to their age-matched untreated peers. Signal detection analyses indicate that a change in perceptual sensitivity as well as in bias may underlie the observed effects. Our results and comparisons with other studies in normal-hearing older adults suggest that both mild hearing impairment and hearing aid use seem to affect audiovisual integration, possibly in the sense that hearing aid use may reverse the effects of hearing loss on audiovisual integration. We suggest that these findings may be particularly important for auditory rehabilitation and call for a longitudinal study.
Wan, Liangcai; Guo, Menghe; Chen, Shuaijun; Liu, Shuangriu; Chen, Hao; Gong, Jian
To describe the multi-channel cochlear implantation in patients with Waardenburg syndrome including surgeries, pre and postoperative hearing assessments as well as outcomes of speech recognition. Multi-channel cochlear implantation surgeries have been performed in 12 cases with Waardenburg syndrome type II in our department from 2000 to 2008. All the patients received multi-channel cochlear implantation through transmastoid facial recess approach. The postoperative outcomes of 12 cases were compared with 12 cases with no inner ear malformation as a control group. The electrodes were totally inserted into the cochlear successfully, there was no facial paralysis and cerebrospinal fluid leakage occurred after operation. The hearing threshold in this series were similar to that of the normal cochlear implantation. After more than half a year of speech rehabilitation, the abilities of speech discrimination and spoken language of all the patients were improved compared with that of preoperation. Multi-channel cochlear implantation could be performed in the cases with Waardenburg syndrome, preoperative hearing and images assessments should be done.
Stearn, Natalie; Swanepoel, De Wet
Isolated reports of hearing loss presenting as markings on the iris exist, but to date the effectiveness of iridology to identify hearing loss has not been investigated. This study therefore aimed to determine the efficacy of iridological analysis in the identification of moderate to profound sensorineural hearing loss in adolescents. A controlled trial was conducted with an iridologist, blind to the actual hearing status of participants, analyzing the irises of participants with and without hearing loss. Fifty hearing impaired and fifty normal hearing subjects, between the ages of 15 and 19 years, controlled for gender, participated in the study. An experienced iridologist analyzed the randomised set of participants' irises. A 70% correct identification of hearing status was obtained by iridological analyses with a false negative rate of 41% compared to a 19% false positive rate. The respective sensitivity and specificity rates therefore came to 59% and 81%. Iridological analysis of hearing status indicated a statistically significant relationship to actual hearing status (P iridology were not comparable to those of traditional audiological screening procedures.
Dorman, Michael F; Cook, Sarah; Spahr, Anthony; Zhang, Ting; Loiselle, Louise; Schramm, David; Whittingham, JoAnne; Gifford, Rene
Many studies have documented the benefits to speech understanding when cochlear implant (CI) patients can access low-frequency acoustic information from the ear opposite the implant. In this study we assessed the role of three factors in determining the magnitude of bimodal benefit - (i) the level of CI-only performance, (ii) the magnitude of the hearing loss in the ear with low-frequency acoustic hearing and (iii) the type of test material. The patients had low-frequency PTAs (average of 125, 250 and 500 Hz) varying over a large range (70 dB HL) in the ear contralateral to the implant. The patients were tested with (i) CNC words presented in quiet (n = 105) (ii) AzBio sentences presented in quiet (n = 102), (iii) AzBio sentences in noise at +10 dB signal-to-noise ratio (SNR) (n = 69), and (iv) AzBio sentences at +5 dB SNR (n = 64). We find maximum bimodal benefit when (i) CI scores are less than 60 percent correct, (ii) hearing loss is less than 60 dB HL in low-frequencies and (iii) the test material is sentences presented against a noise background. When these criteria are met, some bimodal patients can gain 40-60 percentage points in performance relative to performance with a CI. This article is part of a Special Issue entitled . Copyright © 2014 Elsevier B.V. All rights reserved.
Mehrparvar, Amir Houshang; Davari, Mohammad Hossein; Mollasadeghi, Abolfazl
Carbon monoxide poisoning is one of the rare causes of hearing loss which may cause reversible or irreversible, unilateral or bilateral hearing loss after acute or chronic exposure. In this report, we present a case of bilateral sensorineural hearing loss in a secondary smelting workshop worker a...
This retrospective study compared the cochlear implantation outcomes of first- and second-generation deaf children. The study group consisted of seven deaf, cochlear-implanted children with deaf parents. An equal number of deaf children with normal-hearing parents were selected by matched sampling as a reference group. Participants were matched based on onset and severity of deafness, duration of deafness, age at cochlear implantation, duration of cochlear implantation, gender, and cochlear implant model. We used the Persian Auditory Perception Test for the Hearing Impaired, the Speech Intelligibility Rating scale, and the Sentence Imitation Test, in order to measure participants' speech perception, speech production and language development, respectively. Both groups of children showed auditory and speech development. However, the second-generation deaf children (i.e. deaf children of deaf parents) exceeded the cochlear implantation performance of the deaf children with hearing parents. This study confirms that second-generation deaf children exceed deaf children of hearing parents in terms of cochlear implantation performance. Encouraging deaf children to communicate in sign language from a very early age, before cochlear implantation, appears to improve their ability to learn spoken language after cochlear implantation.
A cross-sectional study was designed to examine the effect of smoking habit on hearing loss at 1000 and 4000 Hz in the workplace. Among 1,875 male workers, including 287 subjects with occupational noise exposure, the ratio of the number with hearing loss at 1000 or 4000 Hz increased with smoking habits and that relation at 4000 Hz was statistically significant. These hearing losses showed a significant relation with age but not with working- duration under occupational noise exposure by multiple regression analysis. The amount of smoking showed a weak but significant association with hearing loss at 4000 Hz. However, among the 287 male subjects with occupational noise exposure, there was no significant relation between smoking habits and hearing loss. Therefore, both hearing loss induced by occupational noise exposure and that related with smoking habit were well controlled in this workplace. These results indicate that hearing check-ups and education to prevent noise-induced hearing impairment in the workplace might be useful to prevent the hearing loss associated with smoking habit among male workers.
Arjun, Dass; Neha, Goel; Surinder K, Singhal; Ravi, Kapoor
Introduction: Sudden sensorineural hearing loss (SSNHL) is a frightening and frustrating symptom for the patient as well as the physician. Prognosis is affected by multiple factors including duration of hearing loss, presence of associated vertigo and tinnitus, and co-morbidities such as hypertension and diabetes. Materials and Methods: Forty subjects presenting to our department with features of sudden hearing loss were included in the study. Detailed otological history and examination, se...
Wallhagen, Margaret I.
Purpose: To explore dimensions of stigma experienced by older adults with hearing loss and those with whom they frequently communicate to target interventions promoting engagement and positive aging. Design and Methods: This longitudinal qualitative study conducted interviews over 1 year with dyads where one partner had hearing loss. Participants…
Keats, Bronya J. B.
This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…
Zhao, Fei; Manchaiah, Vinaya K C; French, David; Price, Sharon M
It has been generally accepted that excessive exposure to loud music causes various hearing symptoms (e.g. tinnitus) and consequently leads to a risk of permanent hearing damage, known as noise-induced hearing loss (NIHL). Such potential risk of NIHL due to loud music exposure has been widely investigated in musicians and people working in music venues. With advancements in sound technology and rapid developments in the music industry, increasing numbers of people, particularly adolescents and young adults, are exposing themselves to music on a voluntary basis at potentially harmful levels, and over a substantial period of time, which can also cause NIHL. However, because of insufficient audiometric evidence of hearing loss caused purely by music exposure, there is still disagreement and speculation about the risk of hearing loss from music exposure alone. Many studies have suggested using advanced audiological measurements as more sensitive and efficient tools to monitor hearing status as early indicators of cochlear dysfunction. The purpose of this review is to provide further insight into the potential risk of hearing loss caused by exposure to loud music, and thus contribute to further raising awareness of music induced hearing loss.
Agterberg, Martijn J H; Hol, Myrthe K S; Cremers, Cor W R J; Mylanus, Emmanuel A M; van Opstal, John; Snik, Ad F M
An important aspect of binaural hearing is the proper detection of interaural sound level differences and interaural timing differences. Assessments of binaural hearing were made in patients with acquired unilateral conductive hearing loss (UCHL, n = 11) or congenital UCHL (n = 10) after unilateral application of a bone conduction device (BCD), and in patients with bilateral conductive or mixed hearing loss after bilateral BCD application. Benefit (bilateral versus unilateral listening) was assessed by measuring directional hearing, compensation of the acoustic head shadow, binaural summation and binaural squelch. Measurements were performed after an acclimatization time of at least 10 weeks. Unilateral BCD application was beneficial, but there was less benefit in the patients with congenital UCHL as compared to patients with acquired UCHL. In adults with bilateral hearing loss, bilateral BCD application was clearly beneficial as compared to unilateral BCD application. Binaural summation was present, but binaural squelch could not be proven. To explain the poor results in the patients with congenital UCHL, two factors seemed to be important. First, a critical period in the development of binaural hearing might affect the binaural hearing abilities. Second, crossover stimulation, referring to additional stimulation of the cochlea contralateral to the BCD side, might deteriorate binaural hearing in patients with UCHL. Copyright © 2011 S. Karger AG, Basel.
Westerberg, Brian D; Pijl, Sipke; McDonald, Michael
To review processes of resource allocation and the ethical considerations relevant to the fair allocation of a limited number of cochlear implants to increasing numbers of potential recipients. Review of relevant considerations. Tertiary referral hospital. Editorial discussion of the ethical issues of resource allocation. Heterogeneity of audiometric thresholds, self-reported disability of hearing loss, age of the potential cochlear implant recipient, cost-effectiveness, access to resources, compliance with follow-up, social support available to the recipient, social consequences of hearing impairment, and other recipient-related factors. In a publicly funded health care system, there will always be a need for decision-making processes for allocation of finite fiscal resources. All candidates for cochlear implantation deserve fair consideration. However, they are a heterogeneous group in terms of needs and expected outcomes consisting of traditional and marginal candidates, with a wide range of benefit from acoustic amplification. We argue that implant programs should thoughtfully prioritize treatment on the basis of need and potential benefit. We reject queuing on the basis of "first-come, first-served" or on the basis of perceived social worth.
Full Text Available BACKGROUND: Auditory training involves active listening to auditory stimuli and aims to improve performance in auditory tasks. As such, auditory training is a potential intervention for the management of people with hearing loss. OBJECTIVE: This systematic review (PROSPERO 2011: CRD42011001406 evaluated the published evidence-base for the efficacy of individual computer-based auditory training to improve speech intelligibility, cognition and communication abilities in adults with hearing loss, with or without hearing aids or cochlear implants. METHODS: A systematic search of eight databases and key journals identified 229 articles published since 1996, 13 of which met the inclusion criteria. Data were independently extracted and reviewed by the two authors. Study quality was assessed using ten pre-defined scientific and intervention-specific measures. RESULTS: Auditory training resulted in improved performance for trained tasks in 9/10 articles that reported on-task outcomes. Although significant generalisation of learning was shown to untrained measures of speech intelligibility (11/13 articles, cognition (1/1 articles and self-reported hearing abilities (1/2 articles, improvements were small and not robust. Where reported, compliance with computer-based auditory training was high, and retention of learning was shown at post-training follow-ups. Published evidence was of very-low to moderate study quality. CONCLUSIONS: Our findings demonstrate that published evidence for the efficacy of individual computer-based auditory training for adults with hearing loss is not robust and therefore cannot be reliably used to guide intervention at this time. We identify a need for high-quality evidence to further examine the efficacy of computer-based auditory training for people with hearing loss.
Wallis, Kate E; Blum, Nathan J; Waryasz, Stephanie A; Augustyn, Marilyn
Sonia is a 4 years 1 month-year-old girl with Waardenburg syndrome and bilateral sensorineural hearing loss who had bilateral cochlear implants at 2 years 7 months years of age. She is referred to Developmental-Behavioral Pediatrics by her speech/language pathologist because of concerns that her language skills are not progressing as expected after the cochlear implant. At the time of the implant, she communicated using approximately 20 signs and 1 spoken word (mama). At the time of the evaluation (18 months after the implant) she had approximately 70 spoken words (English and Spanish) and innumerable signs that she used to communicate. She could follow 1-step directions in English but had more difficulty after 2-step directions.Sonia was born in Puerto Rico at 40 weeks gestation after an uncomplicated pregnancy. She failed her newborn hearing test and was given hearing aids that did not seem to help.At age 2 years, Sonia, her mother, and younger sister moved to the United States where she was diagnosed with bilateral severe-to-profound hearing loss. Genetic testing led to a diagnosis of Waardenburg syndrome (group of genetic conditions that can cause hearing loss and changes in coloring [pigmentation] of the hair, skin, and eyes). She received bilateral cochlear implants 6 months later.Sonia's mother is primarily Spanish-speaking and mostly communicates with her in Spanish or with gestures but has recently begun to learn American Sign Language (ASL). In a preschool program at a specialized school for the deaf, Sonia is learning both English and ASL. Sonia seems to prefer to use ASL to communicate.Sonia receives speech and language therapy (SLT) 3 times per week (90 minutes total) individually in school and once per week within a group. She is also receiving outpatient SLT once per week. Therapy sessions are completed in English, with the aid of an ASL interpreter. Sonia's language scores remain low, with her receptive skills in the first percentile, and her
Lofgren, Sarah; Montgomery, Martha; Yueh, Nathan; Namudde, Alice; Rhein, Joshua; Abassi, Mahsa; Musubire, Abdu; Meya, David; Boulware, David
Abstract Background Hearing loss is a known complication cryptococcal meningitis (CM); however, there is a paucity of data. We aimed to describe hearing loss in CM survivors. Methods We assessed hearing via audiometry 8 and 18 weeks after diagnosis of CM in Kampala, Uganda from 2015-2016. We measured at 0.5, 1, 2, 4 Hz. Normal hearing was defined as minimum hearing level at 25 cm H2O 113 24 (71%) 28 (45%) 0.017 Average Opening Pressure >20 cm H20 96 34 (81%) 43 (61%) 0.025 Quantitative Cultur...
Cantley, Linda F; Galusha, Deron; Cullen, Mark R; Dixon-Ernst, Christine; Tessier-Sherman, Baylah; Slade, Martin D; Rabinowitz, Peter M; Neitzel, Richard L
To determine the relative contributions of tinnitus, asymmetrical hearing loss, low frequency hearing loss (pure tone average of 0.5, 1, 2, 3 kHz; PTA.5123), or high frequency hearing loss (pure tone average of 4, 6 kHz; PTA46), to acute injury risk among a cohort of production and maintenance workers at six aluminum manufacturing plants, adjusting for ambient noise exposure and other recognized predictors of injury risk. Retrospective analysis. The study considered 9920 workers employed during 2003 to 2008. The cohort consisted of 8818 workers (89%) whose complete records were available. Adjusting for noise exposure and other recognized injury predictors, a 25% increased acute injury risk was observed among workers with a history of tinnitus in conjunction with high-frequency hearing loss (PTA46). Low frequency hearing loss may be associated with minor, yet less serious, injury risk. We did not find evidence that asymmetry contributes to injury risk. These results provide evidence that tinnitus, combined with high-frequency hearing loss, may pose an important safety threat to workers, especially those who work in high-noise exposed environments. These at risk workers may require careful examination of their communication and hearing protection needs.
Muus, John S; Weir, Forest W; Kreicher, Kathryn L; Bowlby, Deborah A; Discolo, Christopher M; Meyer, Ted A
Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database. Data for each patient were selected based on the first encounter with available complete audiometric data or the first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and otologic issues were documented. A separate cohort comprised of children with GHD without hearing loss was compared as a control. 209 children with GHD met inclusion criteria. 173 (83%) of these patients had hearing loss. 79% of losses were bilateral and 21% were unilateral (309 total ears with hearing loss). 293 of the 309 ears with hearing loss had audiograms with ear-specific thresholds; 47 had conductive, 24 had sensorineural, 65 had mixed and 157 had undefined hearing loss with incomplete audiograms. Pure-tone averages (PTA) were higher among patients with mixed hearing loss compared to patients with all other loss types. Hearing loss is prevalent in children with GHD with a predisposition to be bilateral. These findings suggest the need for increased awareness and routine hearing screening for patients with GHD. Further studies may elucidate the etiology of the hearing impairment in children with GHD to better aid pediatricians, endocrinologists, otolaryngologists and audiologists when assessing and managing these children. Copyright © 2017 Elsevier B.V. All rights reserved.
Sorkin, Donna L; Gates-Ulanet, Patricia; Mellon, Nancy K
Pediatric hearing loss changed more in the past two decades than it had in the prior 100 years with children now identified in the first weeks of life and fit early with amplification. Dramatic improvements in hearing technology allow children the opportunity to listen, speak and read on par with typically hearing peers. National laws mandate that public and private schools, workplaces, and anywhere people go must be accessible to individuals with disabilities. In 2015, most children with hearing loss attended mainstream schools with typically hearing peers. Psychosocial skills still present challenges for some children with hearing loss. Copyright © 2015 Elsevier Inc. All rights reserved.
Adachi, Nodoka; Ito, Ken; Sakata, Hideaki; Yamasoba, Tatsuya
To evaluate the incidence of newborn hearing loss in a Japanese population and to elucidate etiological factors and one-year prognosis. Screening of newborn hearing. Children's tertiary referral center. Between 1999 and 2008, 101,912 newborn infants were screened, with 693 infants (0.68%) referred. Etiology investigation included CT, detection of cytomegalovirus (CMV) DNA, and connexin 26 mutation. Abnormal results (auditory brainstem response [ABR] threshold > or = 35 normal hearing level [dB nHL] in either side) were observed in 312 infants (0.31%), and 133 subjects (0.13%) with ABR thresholds > or = 50 dB nHL on both sides were classified into the habilitation group. In this group, inner ear/internal auditory meatus anomalies were detected in 20 of 121 subjects (17%) tested, middle/external ear anomalies in 14 of 121 subjects (12%), CMV DNA in 13 of 77 subjects (17%), and connexin 26 mutation in 28 of 89 subjects (31%). In 68 subjects undergoing all three investigations (CT, CMV, and connexin 26), 41 (60%) had positive results in at least one test. With inclusion of otitis media with effusion and perinatal problems, this rate amounted to 78% (53 subjects). Of the 97 infants in the habilitation group successfully followed up to one year, 36 (37%) showed a threshold change of 20 dB or more in either ear: 11 (11%) progression and 25 (26%) improvement, and 15 infants (15%) were reclassified into a less severe classification. Considering that 26 percent of infants with bilateral moderate to severe hearing loss showed improvement in one year, habilitation protocols, especially very early cochlear implantation within one year of birth, should be reconsidered. 2010 American Academy of Otolaryngology-Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.
Härkönen, Kati; Kivekäs, Ilkka; Rautiainen, Markus; Kotti, Voitto; Sivonen, Ville; Vasama, Juha-Pekka
This prospective study shows that working performance, quality of life (QoL), and quality of hearing (QoH) are better with two compared with a single cochlear implant (CI). The impact of the second CI on the patient's QoL is as significant as the impact of the first CI. To evaluate the benefits of sequential bilateral cochlear implantation in working, QoL, and QoH. We studied working performance, work-related stress, QoL, and QoH with specific questionnaires in 15 patients with unilateral CI scheduled for sequential CI of another ear. Sound localization performance and speech perception in noise were measured with specific tests. All questionnaires and tests were performed before the second CI surgery and 6 and 12 months after its activation. Bilateral CIs increased patients' working performance and their work-related stress and fatigue decreased. Communication with co-workers was easier and patients were more active in their working environment. Sequential bilateral cochlear implantation improved QoL, QoH, sound localization, and speech perception in noise statistically significantly.
Hitchcock, Ying J.; Tward, Jonathan D.; Szabo, Aniko; Bentz, Brandon G.; Shrieve, Dennis C.
Purpose: To investigate the risk of sensorineural hearing loss (SNHL) in patients with head-and-neck cancer and treated with radiation therapy (RT) or concomitant cisplatin-based chemoradiation, the relationship among SNHL and radiation dose to the cochlea, the use of two common cisplatin dose regimens. Methods and Materials: A total of 62 head-and-neck cancer patients treated with curative intent were included in this prospective study. Of the patients, 21 received RT alone, 27 received 40 mg/m 2 weekly cisplatin, 13 received 100 mg/m 2 every 3 weeks during RT, and 1 received RT with weekly epidermal growth factor receptor inhibitor antibody. The effect of chemotherapy and RT dose on hearing was determined using a model that accounted for the age and variability between each ear for each patient. Results: We constructed a model to predict dose-dependent hearing loss for RT or cisplatin-based chemotherapy either alone or in combination. For patients only receiving RT, no significant hearing loss was found at doses to the cochlea of less than 40 Gy. Patients receiving 100 mg/m 2 or 40 mg/m 2 of cisplatin chemotherapy had an estimated +21.5 dB and +9.5 dB hearing loss at 8,000 Hz with low radiation doses (10 Gy), which rose to +38.4 dB and +18.9 dB for high radiation doses (40 Gy). Conclusions: Use of RT alone with doses of less than 40 Gy did not result in clinically significant hearing loss. High-frequency SNHL was profoundly damaged in patients who received concomitant cisplatin when doses of 100 mg/m 2 were used. The threshold cochlear dose for hearing loss with cisplatin-based chemotherapy and RT was predicted to be 10 Gy. The inner ear radiation dose constraints and cisplatin dose intensity should be considered in the treatment of advanced head-and-neck cancer
Mantokoudis, Georgios; Dubach, Patrick; Pfiffner, Flurin; Kompis, Martin; Caversaccio, Marco; Senn, Pascal
Telephone communication is a challenge for many hearing-impaired individuals. One important technical reason for this difficulty is the restricted frequency range (0.3-3.4 kHz) of conventional landline telephones. Internet telephony (voice over Internet protocol [VoIP]) is transmitted with a larger frequency range (0.1-8 kHz) and therefore includes more frequencies relevant to speech perception. According to a recently published, laboratory-based study, the theoretical advantage of ideal VoIP conditions over conventional telephone quality has translated into improved speech perception by hearing-impaired individuals. However, the speech perception benefits of nonideal VoIP network conditions, which may occur in daily life, have not been explored. VoIP use cannot be recommended to hearing-impaired individuals before its potential under more realistic conditions has been examined. To compare realistic VoIP network conditions, under which digital data packets may be lost, with ideal conventional telephone quality with respect to their impact on speech perception by hearing-impaired individuals. We assessed speech perception using standardized test material presented under simulated VoIP conditions with increasing digital data packet loss (from 0% to 20%) and compared with simulated ideal conventional telephone quality. We monaurally tested 10 adult users of cochlear implants, 10 adult users of hearing aids, and 10 normal-hearing adults in the free sound field, both in quiet and with background noise. Across all participant groups, mean speech perception scores using VoIP with 0%, 5%, and 10% packet loss were 15.2% (range 0%-53%), 10.6% (4%-46%), and 8.8% (7%-33%) higher, respectively, than with ideal conventional telephone quality. Speech perception did not differ between VoIP with 20% packet loss and conventional telephone quality. The maximum benefits were observed under ideal VoIP conditions without packet loss and were 36% (P = .001) for cochlear implant users, 18
Hrapcak, Susan; Kuper, Hannah; Bartlett, Peter; Devendra, Akash; Makawa, Atupele; Kim, Maria; Kazembe, Peter; Ahmed, Saeed
With improved access to antiretroviral therapy (ART), HIV infection is becoming a chronic illness. Preliminary data suggest that HIV-infected children have a higher risk of disabilities, including hearing impairment, although data are sparse. This study aimed to estimate the prevalence and types of hearing loss in HIV-infected children in Lilongwe, Malawi. This was a cross-sectional survey of 380 HIV-infected children aged 4-14 years attending ART clinic in Lilongwe between December 2013-March 2014. Data was collected through pediatric quality of life and sociodemographic questionnaires, electronic medical record review, and detailed audiologic testing. Hearing loss was defined as >20 decibels hearing level (dBHL) in either ear. Predictors of hearing loss were explored by regression analysis generating age- and sex-adjusted odds ratios. Children with significant hearing loss were fitted with hearing aids. Of 380 patients, 24% had hearing loss: 82% conductive, 14% sensorineural, and 4% mixed. Twenty-one patients (23% of those with hearing loss) were referred for hearing aid fitting. There was a higher prevalence of hearing loss in children with history of frequent ear infections (OR 7.4, 4.2-13.0) and ear drainage (OR 6.4, 3.6-11.6). Hearing loss was linked to history of WHO Stage 3 (OR 2.4, 1.2-4.5) or Stage 4 (OR 6.4, 2.7-15.2) and history of malnutrition (OR 2.1, 1.3-3.5), but not to duration of ART or CD4. Only 40% of caregivers accurately perceived their child's hearing loss. Children with hearing impairment were less likely to attend school and had poorer emotional (p = 0.02) and school functioning (p = 0.04). There is an urgent need for improved screening tools, identification and treatment of hearing problems in HIV-infected children, as hearing loss was common in this group and affected school functioning and quality of life. Clear strategies were identified for prevention and treatment, since most hearing loss was conductive in nature, likely due to
Purcell, Patricia L; Jones-Goodrich, Rose; Wisneski, Meghan; Edwards, Todd C; Sie, Kathleen C Y
Management of children with unilateral hearing loss is not standardized. The primary goal of this study was to elicit patient- and parent-reported perspectives regarding usage of hearing devices in pediatric UHL and to suggest a basic algorithmic approach to management. Our tertiary care center recruited families of youth ages 5-19 years with unilateral hearing loss from January 2014 through October 2015. Parents of all youths completed a 36-item survey, and some youth ages 11-19 years participated in hour-long interviews. We assessed patterns of hearing device usage among participants, and performed qualitative data analysis to understand factors considered by youths when deciding whether or not to use a hearing device. Survey information was collected for 50 patients. Distribution of hearing loss severity in affected ear was mild 14%, moderate 26%, severe 22%, and profound 38%. The majority of children had sensorineural hearing loss (57%), followed by mixed (32%), and then conductive (11%). 34 children (68%) had tried a hearing device; 20 continued to use the device. Retention rates were similar among children with different degrees of hearing loss: mild 66%, moderate 50%, severe 60%, profound 64%. Sixteen children tried a wireless contralateral routing of signal (CROS) device, and 15 tried a behind-the-ear (BTE) hearing aid. Retention rates for CROS and BTE devices were 69% and 47%, respectively. The most common reason for cessation of use was discomfort, followed by lack of benefit. A majority of children with unilateral hearing loss who tried a hearing device continued to use it, and retention rates were similar across all degrees of hearing loss. These findings suggest that personal hearing devices should be included in management protocols. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.
Full Text Available Christiane Völter,1 Lisa Götze,1 Stefan Dazert,1 Michael Falkenstein,2,3 Jan Peter Thomas1 1Department of Otorhinolaryngology, Head and Neck Surgery, Ruhr University Bochum, St. Elisabeth-Hospital, Bochum, Germany; 2Institute for Work, Learning and Ageing (ALA, Bochum, Germany; 3Leibniz Research Centre for Working Environment and Human Factors, Dortmund, Germany Introduction: The relationship between cognition and the ability to hear is well known. Due to changes in demographics, the number of people with sensorineural hearing loss and cognitive impairment is increasing. The aim of this study was to identify the impact of hearing rehabilitation via cochlear implantation on cognitive decline among the aging population. Patients and methods: This prospective study included 60 subjects aged between 50 and 84 years (mean 65.8 years, SD=8.9 with a severe to profound bilateral hearing impairment. A computer-based evaluation of short- and long-term memory, processing speed, attention, working memory and inhibition was performed prior to surgery as well as 6 and 12 months after cochlear implantation. Additionally, speech perception at 65 and 80 dB (Freiburger monosyllabic speech test as well as disease-related (Nijmegen Cochlear Implant Questionnaire and general (WHOQOL-OLD quality of life were assessed. Results: Six months postimplantation, speech perception, quality of life and also neurocognitive abilities significantly increased. The most remarkable improvement after 6 months was detected in executive functions such as attention (p<0.001, inhibition (p=0.025 and working memory (n-back: p=0.002; operation span task: p=0.008, followed by delayed recall (p=0.03. In contrast, long-term memory showed a significant change of performance only after 12 months (p=0.021. After 6 months, most cognitive domains remained stable, except working memory assessed by the operation span task, which significantly improved between 6 and 12 months (p<0.001. No
Most, Tova; Michaelis, Hilit
This study aimed to investigate the effect of hearing loss (HL) on emotion-perception ability among young children with and without HL. A total of 26 children 4.0-6.6 years of age with prelingual sensory-neural HL ranging from moderate to profound and 14 children with normal hearing (NH) participated. They were asked to identify happiness, anger, sadness, and fear expressed by an actress when uttering the same neutral nonsense sentence. Their auditory, visual, and auditory-visual perceptions of the emotional content were assessed. The accuracy of emotion perception among children with HL was lower than that of the NH children in all 3 conditions: auditory, visual, and auditory-visual. Perception through the combined auditory-visual mode significantly surpassed the auditory or visual modes alone in both groups, indicating that children with HL utilized the auditory information for emotion perception. No significant differences in perception emerged according to degree of HL. In addition, children with profound HL and cochlear implants did not perform differently from children with less severe HL who used hearing aids. The relatively high accuracy of emotion perception by children with HL may be explained by their intensive rehabilitation, which emphasizes suprasegmental and paralinguistic aspects of verbal communication.
Zhang, Li; Qiu, Jianxin; Qin, Feifei; Zhong, Mei; Shah, Gyanendra
A common cavity deformity (CCD) is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity that results in profound hearing loss. There are few studies paying attention to common cavity. Our group is engrossed in observing the improvement of auditory and verbal abilities in children who have received cochlear implantation (CI), and comparing these targets between children with common cavity and normal inner ear structure. A retrospective study was conducted in 12 patients with profound hearing loss that were divided into a common cavity group and a control group, six in each group matched in sex, age and time of implantation, based on inner ear structure. Categories of Auditory Performance (CAP) and speech intelligibility rating (SIR) scores and aided hearing thresholds were collected and compared between the two groups. All patients wore CI for more than 1 year at the Cochlear Center of Anhui Medical University from 2011 to 2015. Postoperative CAP and SIR scores were higher than before operation in both groups (p good as in those without CCD, CI provides benefits in auditory perception and communication skills in these children.
Nordfalk, Karl Fredrik; Rasmussen, Kjell; Bunne, Marie; Jablonski, Greg Eigner
The aim of this study was to compare the outcomes of vestibular tests and the residual hearing of patients who have undergone full insertion cochlear implant surgery using the round window approach with a hearing preservation protocol (RW-HP) or the standard cochleostomy approach (SCA) without hearing preservation. A prospective study of 34 adults who underwent unilateral cochlear implantation was carried out. One group was operated using the RW-HP (n = 17) approach with Med-El +Flex(SOFT) electrode array with full insertion, while the control group underwent a more conventional SCA surgery (n = 17) with shorter perimodiolar electrodes. Assessments of residual hearing, cervical vestibular-evoked myogenic potentials (cVEMP), videonystagmography, subjective visual vertical/horizontal (SVH/SVV) were performed before and after surgery. There was a significantly (p < 0.05) greater number of subjects who exhibited complete or partial hearing preservation in the deep insertion RW-HP group (9/17) compared to the SCA group (2/15). A higher degree of vestibular loss but a lower degree of vertigo symptoms could be seen in the RW-HP group, but the differences were not statistically significant. It is possible to preserve residual hearing to a certain extent also with deep insertion. Full insertion with hearing preservation was less harmful to residual hearing particularly at 125 Hz (p < 0.05), than was the standard cochleostomy approach.
Nittrouer, Susan; Caldwell-Tarr, Amanda; Low, Keri E.; Lowenstein, Joanna H.
Purpose: Verbal working memory in children with cochlear implants and children with normal hearing was examined. Participants: Ninety-three fourth graders (47 with normal hearing, 46 with cochlear implants) participated, all of whom were in a longitudinal study and had working memory assessed 2 years earlier. Method: A dual-component model of…
Escorihuela García, Vicente; Pitarch Ribas, María Ignacia; Llópez Carratalá, Ignacio; Latorre Monteagudo, Emilia; Morant Ventura, Antonio; Marco Algarra, Jaime
The studies that have evaluated the effectiveness of bilateral cochlear implantation in children suggest an improvement in hearing about sound localization and speech discrimination. In this paper we show the differences in audio-linguistic achievements with early bilateral cochlear implantation versus unilateral, and differences between simultaneous and sequential bilateral implantation. We present 88 children with bilateral profound sensorineural hearing loss, treated with bilateral cochlear implantation in 32 cases and unilateral in 56 cases, during the first 12 months (27 children) of life and between 12 and 24 months (61 children). We conducted a statistical comparison of both groups in the audiometry, IT-Mais, Nottingham, LittlEars scales and verbal tests. No significant differences in hearing thresholds and questionnaires between unilateral and bilateral implantation were detected in either the first or second year. Verbal tests do show statistically significant differences: children with bilateral cochlear implant obtain 100% recognition of disyllabic and phrases within 2-3 years after implantation whilst children with one implant do not obtain those results at 5 years after surgery. No differences between simultaneous and sequential bilateral implantation were detected. We emphasize the importance of ensuring good early audiological screening, to carry out an early and bilateral cochlear implantation with the consequent development of audio-language skills similar to normal hearing children. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.
Fitzpatrick, Elizabeth M; Al-Essa, Rakan S; Whittingham, JoAnne; Fitzpatrick, Jessica
The purpose of this study was to describe the clinical characteristics of children with unilateral hearing loss (UHL), examine deterioration in hearing, and explore amplification decisions. Population-based data were collected prospectively from time of diagnosis. Serial audiograms and amplification details were retrospectively extracted from clinical charts to document the trajectory and management of hearing loss. The study included all children identified with UHL in one region of Canada over a 13-year period (2003-2015) after implementation of universal newborn hearing screening. Of 537 children with permanent hearing loss, 20.1% (108) presented with UHL at diagnosis. They were identified at a median age of 13.9 months (IQR: 2.8, 49.0). Children with congenital loss were identified at 2.8 months (IQR: 2.0, 3.6) and made up 47.2% (n = 51), reflecting that a substantial portion had late-onset, acquired or late-identified loss. A total of 42.4% (n = 39) showed deterioration in hearing, including 16 (17.4%) who developed bilateral loss. By study end, 73.1% (79/108) of children had received amplification recommendations. Up to 20% of children with permanent HL are first diagnosed with UHL. About 40% are at risk for deterioration in hearing either in the impaired ear and/or in the normal hearing ear.
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Ivory, Robert; Kane, Rebecca; Diaz, Rodney C
This review will discuss the real-world risk factors involved in noise-induced hearing loss as a result of common and popular recreational activities prone to mid and high levels of noise exposure. Although there are currently no interventional measures available to reverse or mitigate preexisting hearing loss from noise, we discuss the vital importance of hearing loss prevention from noise exposure avoidance and reduction. Despite a seeming understanding of the effects of noise exposure from various recreational activities and devices, a large percentage of the general public who is at risk of such noise-induced hearing loss still chooses to refrain from using hearing protection instruments. While occupational exposures pose the greatest traditional risk to hearing conservation in selected workers, recreational risk factors for noise-induced hearing loss may be more insidious in overall effect given the indifferent attitude of much of the general public and particularly our youths toward hearing protection during recreational activities. Active counseling regarding the consequences of excessive noise exposure and the potential benefits to hearing from usage of hearing protection instruments is critical to providing best possible care in the hearing health professions.
Mondelli, Maria Fernanda Capoani Garcia; dos Santos, Marina de Marchi; José, Maria Renata
ABSTRACT INTRODUCTION: Unilateral hearing loss is characterized by a decrease of hearing in one ear only. In the presence of ambient noise, individuals with unilateral hearing loss are faced with greater difficulties understanding speech than normal listeners. OBJECTIVE: To evaluate the speech perception of individuals with unilateral hearing loss in speech perception with and without competitive noise, before and after the hearing aid fitting process. METHODS: The study included 30 adu...
Hrapcak, Susan; Kuper, Hannah; Bartlett, Peter; Devendra, Akash; Makawa, Atupele; Kim, Maria; Kazembe, Peter; Ahmed, Saeed
Introduction With improved access to antiretroviral therapy (ART), HIV infection is becoming a chronic illness. Preliminary data suggest that HIV-infected children have a higher risk of disabilities, including hearing impairment, although data are sparse. This study aimed to estimate the prevalence and types of hearing loss in HIV-infected children in Lilongwe, Malawi. Methods This was a cross-sectional survey of 380 HIV-infected children aged 4–14 years attending ART clinic in Lilongwe between December 2013-March 2014. Data was collected through pediatric quality of life and sociodemographic questionnaires, electronic medical record review, and detailed audiologic testing. Hearing loss was defined as >20 decibels hearing level (dBHL) in either ear. Predictors of hearing loss were explored by regression analysis generating age- and sex-adjusted odds ratios. Children with significant hearing loss were fitted with hearing aids. Results Of 380 patients, 24% had hearing loss: 82% conductive, 14% sensorineural, and 4% mixed. Twenty-one patients (23% of those with hearing loss) were referred for hearing aid fitting. There was a higher prevalence of hearing loss in children with history of frequent ear infections (OR 7.4, 4.2–13.0) and ear drainage (OR 6.4, 3.6–11.6). Hearing loss was linked to history of WHO Stage 3 (OR 2.4, 1.2–4.5) or Stage 4 (OR 6.4, 2.7–15.2) and history of malnutrition (OR 2.1, 1.3–3.5), but not to duration of ART or CD4. Only 40% of caregivers accurately perceived their child’s hearing loss. Children with hearing impairment were less likely to attend school and had poorer emotional (p = 0.02) and school functioning (p = 0.04). Conclusions There is an urgent need for improved screening tools, identification and treatment of hearing problems in HIV-infected children, as hearing loss was common in this group and affected school functioning and quality of life. Clear strategies were identified for prevention and treatment, since most
Gerdes, Timo; Salcher, Rolf Benedikt; Schwab, Burkard; Lenarz, Thomas; Maier, Hannes
In conductive, mixed hearing losses and single-sided-deafness bone-anchored hearing aids are a well-established treatment. The transcutaneous transmission across the intact skin avoids the percutaneous abutment of a bone-anchored device with the usual risk of infections and requires less care.In this study, the audiological results of the Bonebridge transcutaneous bone conduction implant (MED-EL) are compared to the generally used percutaneous device BP100 (Cochlear Ltd., Sydney, Australia). Ten patients implanted with the transcutaneous hearing implant were compared to 10 matched patients implanted with a percutaneous device. Tests included pure-tone AC and BC thresholds and unaided and aided sound field thresholds. Speech intelligibility was determined in quiet using the Freiburg monosyllable test and in noise with the Oldenburg sentence test (OLSA) in sound field with speech from the front (S0). The subjective benefit was assessed with the Abbreviated Profile of Hearing Aid Benefit. In comparison with the unaided condition there was a significant improvement in aided thresholds, word recognition scores (WRS), and speech reception thresholds (SRT) in noise, measured in sound field, for both devices. The comparison of the two devices revealed a minor but not significant difference in functional gain (Bonebridge: PTA = 27.5 dB [mean]; BAHA: PTA = 26.3 dB [mean]). No significant difference between the two devices was found when comparing the improvement in WRSs and SRTs (Bonebridge: improvement WRS = 80% [median], improvement SRT = 6.5 dB SNR [median]; BAHA: improvement WRS = 77.5% [median], BAHA: improvement SRT = 6.9 dB SNR [median]). Our data show that the transcutaneous bone conduction hearing implant is an audiologically equivalent alternative to percutaneous bone-anchored devices in conductive hearing loss with a minor sensorineural hearing loss component.
Liu Chinghsiung; Lin Shinnkuang E-mail: firstname.lastname@example.org; Chang Yeujhy
The authors describe a patient with isolated involvement of vestibulocochlear nerve by a huge vascular loop from vertebral dolichoectasia. No other neurological deficit was found except for unilateral hearing loss. Abnormal brainstem auditory evoked potential study indicated a retrocochlear lesion. The brain computed tomography (CT) and magnetic resonance imaging (MRI) studies demonstrated an abnormally enhanced vascular lesion impinged on the left porus acusticus with a displacement of the brainstem to the right. There was no infarction in the brainstem. A cerebral angiography demonstrated a megadolichoectatic horizontal loop at the intracranial portion of the left vertebral artery. There was no thrombus or atherosclerosis in the vertebrobasilar system. A mechanical compression by a vascular loop is the only possible pathogenesis for hearing loss. The authors diagnose this condition as cochlear vertebral entrapment syndrome.
Meister, Hartmut; Rählmann, Sebastian; Walger, Martin; Margolf-Hackl, Sabine; Kießling, Jürgen
To examine the association of cognitive function, age, and hearing loss with clinically assessed hearing aid benefit in older hearing-impaired persons. Hearing aid benefit was assessed using objective measures regarding speech recognition in quiet and noisy environments as well as a subjective measure reflecting everyday situations captured using a standardized questionnaire. A broad range of general cognitive functions such as attention, memory, and intelligence were determined using different neuropsychological tests. Linear regression analyses were conducted with the outcome of the neuropsychological tests as well as age and hearing loss as independent variables and the benefit measures as dependent variables. Thirty experienced older hearing aid users with typical age-related hearing impairment participated. Most of the benefit measures revealed that the participants obtained significant improvement with their hearing aids. Regression models showed a significant relationship between a fluid intelligence measure and objective hearing aid benefit. When individual hearing thresholds were considered as an additional independent variable, hearing loss was the only significant contributor to the benefit models. Lower cognitive capacity - as determined by the fluid intelligence measure - was significantly associated with greater hearing loss. Subjective benefit could not be predicted by any of the variables considered. The present study does not give evidence that hearing aid benefit is critically associated with cognitive function in experienced hearing aid users. However, it was found that lower fluid intelligence scores were related to higher hearing thresholds. Since greater hearing loss was associated with a greater objective benefit, these results strongly support the advice of using hearing aids regardless of age and cognitive function to counter hearing loss and the adverse effects of age-related hearing impairment. Still, individual cognitive capacity might
Full Text Available Background and Aim: Hearing loss is one of the most prevalent chronic diseases in the elderly; using a hearing aid to alleviate auditory impairment can positively affect their quality of life. This research aimed to determine the level of satisfaction concerning hearing aids in elderly people with hearing impairment based on the type and degree of hearing loss.Methods: An analytic cross-sectional research design was used ; the sample included 40 elderly people who used hearing aids. According to the World Health Organization (WHO age classification, participants were divided into two age groups: 65-74 years (n=20 and 75-90 years (n=20. Satisfaction levels were assessed using a standard satisfaction with amplification in daily life (SADL questionnaire.Results: Satisfaction levels in the 65-74 age group were significantly higher than that in the 75-90 age group (p=0.02. Participants with mixed hearing loss revealed higher satisfaction levels than participants with sensorineural hearing loss (p=0.02. On the negative effects dimension, participants with severe hearing loss exhibited significantly higher satisfaction levels than participants with moderate or moderate to severe hearing loss (p=0.01.Conclusion: Total satisfaction mean scores were relatively high in the elderly participants . Negative features could be reduced via careful consultation regarding the aids’ amplifying capabilities and limitations in groups with moderate or moderate to severe hearing loss.
Heidenreich, Katherine D; Basura, Gregory J; Zwolan, Teresa A; El-Kashlan, Hussam K; Telian, Steven A
Document the use of transcanal labyrinthectomy to treat disabling attacks of vertigo after unilateral cochlear implantation. A 46-year-old woman with severe-profound bilateral sensorineural hearing loss secondary to enlarged vestibular aqueducts underwent cochlear implantation for her right ear with a Nucleus Freedom device. The surgery was uneventful, and postoperative imaging confirmed that the electrode was positioned properly. She developed episodic vertigo 10 to 14 days after the implant surgery, which failed to improve with aggressive vestibular rehabilitation therapy. Plugging of the round window for possible perilymphatic fistula did not relieve her symptoms. Right transcanal labyrinthectomy supplemented by filling the vestibule with gentamicin-soaked Gelfoam and then a customized vestibular rehabilitation program. Comparison of vestibular symptoms and cochlear implant performance before and after transcanal labyrinthectomy. The patient had immediate relief of symptoms, and the function of the cochlear implant was not adversely affected. Transcanal labyrinthectomy may be an effective method to ablate the vestibular end organ after unilateral cochlear implantation. It can offer relief of disabling vertigo without adversely affecting the performance of the implant.
Pereira Fred A
Full Text Available Abstract Background Cochlear outer hair cells change their length in response to variations in membrane potential. This capability, called electromotility, is believed to enable the sensitivity and frequency selectivity of the mammalian cochlea. Prestin is a transmembrane protein required for electromotility. Homozygous prestin knockout mice are profoundly hearing impaired. In humans, a single nucleotide change in SLC26A5, encoding prestin, has been reported in association with hearing loss. This DNA sequence variation, IVS2-2A>G, occurs in the exon 3 splice acceptor site and is expected to abolish splicing of exon 3. Methods To further explore the relationship between hearing loss and the IVS2-2A>G transition, and assess allele frequency, genomic DNA from hearing impaired and control subjects was analyzed by DNA sequencing. SLC26A5 genomic DNA sequences from human, chimp, rat, mouse, zebrafish and fruit fly were aligned and compared for evolutionary conservation of the exon 3 splice acceptor site. Alternative splice acceptor sites within intron 2 of human SLC26A5 were sought using a splice site prediction program from the Berkeley Drosophila Genome Project. Results The IVS2-2A>G variant was found in a heterozygous state in 4 of 74 hearing impaired subjects of Hispanic, Caucasian or uncertain ethnicity and 4 of 150 Hispanic or Caucasian controls (p = 0.45. The IVS2-2A>G variant was not found in 106 subjects of Asian or African American descent. No homozygous subjects were identified (n = 330. Sequence alignment of SLC26A5 orthologs demonstrated that the A nucleotide at position IVS2-2 is invariant among several eukaryotic species. Sequence analysis also revealed five potential alternative splice acceptor sites in intron 2 of human SLC26A5. Conclusion These data suggest that the IVS2-2A>G variant may not occur more frequently in hearing impaired subjects than in controls. The identification of five potential alternative splice acceptor sites in
Huber, Alexander M; Mlynski, Robert; Müller, Joachim; Dillier, Norbert; Holzmann, David; Wolframm, Mario D; Hagen, Rudolf
The primary objective was to report on experiences regarding the safety and efficacy of the Vibrant Soundbridge (VSB) using a floating mass transducer (FMT)-partial/total ossicular replacement prosthesis (PORP/TORP) assembly as a treatment for conductive and mixed hearing losses of mild-to-moderate/severe degree. The secondary objective was to gather information regarding device fitting, as well as to refine surgical procedures. Five German-speaking adults from 2 European study sites were implanted with a VSB using an FMT-PORP/TORP assembly and evaluated before and after surgery for air-and bone-conduction thresholds and speech recognition performance. Evaluating the safety and efficacy of the VSB in combination with a PORP or TORP to treat conductive and mixed hearing loss. Residual cochlear hearing was unaffected by implantation with the device. Functional gain (measured as the difference between preoperative unaided and postoperative VSB-aided thresholds) could be calculated in 2 of 5 subjects, demonstrating that the VSB is effective in treating bone-conduction hearing losses of moderate/severe degree. Word recognition tests in quiet and noise showed good improvement in 3 of the cases. One patient experienced several other medical problems, making her audiological outcomes limited. One patient was excluded from the study owing to insufficient benefit and subsequently underwent revision surgery with FMT placement at the round window. The use of the VSB, implanted using the FMT-PORP/TORP assembly, was safe in all and efficacious in 3 of the 5 cases in this study. These are patients who may have few, if any, other options to manage their hearing loss.
Young, J.P.; O'Sullivan, B.T.; Gibson, W.P.; Sefton, A.E.; Mitchell, T.E.; Sanli, H.; Cervantes, R.; Withall, A.; Royal Prince Alfred Hospital, Sydney,
Full text: Cochlear implantation has been an extraordinarily successful method of restoring hearing and the potential for full language development in pre-lingually and post-lingually deaf individuals (Gibson 1996). Post-lingually deaf patients, who develop their hearing loss later in life, respond best to cochlear implantation within the first few years of their deafness, but are less responsive to implantation after several years of deafness (Gibson 1996). In pre-lingually deaf children, cochlear implantation is most effect in allowing the full development language skills when performed within a critical period, in the first 8 years of life. These clinical observations suggest considerable neural plasticity of the human auditory cortex in acquiring and retaining language skills (Gibson 1996, Buchwald 1990). Currently, electrocochleography is used to determine the integrity of the auditory pathways to the auditory cortex. However, the functional integrity of the auditory cortex cannot be determined by this method. We have defined the extent of activation of the auditory cortex and auditory association cortex in 6 normal controls and 6 cochlear implant patients using 15 O PET functional brain imaging methods. Preliminary results have indicated the potential clinical utility of 15 O PET cortical mapping in the pre-surgical assessment and post-surgical follow up of cochlear implant patients. Copyright (1998) Australian Neuroscience Society
Full Text Available Cochlear implantation has become established worldwide as a safe and effective method of auditory rehabilitation of selected severely and profound deaf children and adults. Over 100,000 patients have received cochlear implants worldwide with the paediatric population proving to be the main beneficiaries. The Libyan cochlear implant programme was set up in 2004. Data relating to the patients who received cochlear implantation at Tripoli Medical Centre between October 2007 and February 2010 were analysed. Implant operations were performed on 37 patients. All patients received Med-El SONATATI 100 devices. Thirty-four (91.9% of these patients were children, whilst three (8.1% were adults. Combined, congenital hearing loss (56.8% and perinatal/neonatal (29.7% were the two main aetiological factors in children. Seventeen patients (45.9% had a positive family history of deafness. Sixteen patients (43.2% were born to blood-related parents. The overall rate of minor and major complications was 16.2%, which is comparable to previous studies.
Salamat, Ali; Esriti, Anwer; Ehtuish, Asia; El-Ogbi, Samya
Cochlear implantation has become established worldwide as a safe and effective method of auditory rehabilitation of selected severely and profound deaf children and adults. Over 100,000 patients have received cochlear implants worldwide with the paediatric population proving to be the main beneficiaries. The Libyan cochlear implant programme was set up in 2004. Data relating to the patients who received cochlear implantation at Tripoli Medical Centre between October 2007 and February 2010 were analysed. Implant operations were performed on 37 patients. All patients received Med-El SONATA(TI) (100) devices. Thirty-four (91.9%) of these patients were children, whilst three (8.1%) were adults. Combined, congenital hearing loss (56.8%) and perinatal/neonatal (29.7%) were the two main aetiological factors in children. Seventeen patients (45.9%) had a positive family history of deafness. Sixteen patients (43.2%) were born to blood-related parents. The overall rate of minor and major complications was 16.2%, which is comparable to previous studies.
A cochlear implant is an electronic device designed to provide sound information for adults and children who have bilateral profound hearing loss. The task of representing speech signals as electrical stimuli is central to the design and performance of cochlear implants. Studies have shown that the current speech- processing strategies provide significant benefits to cochlear implant users. However, the evaluation and development of speech-processing strategies have been complicated by hardware limitations and large variability in user performance. To alleviate these problems, an acoustic model of a cochlear implant with the SPEAK strategy is implemented in this study, in which a set of acoustic stimuli whose psychophysical characteristics are as close as possible to those produced by a cochlear implant are presented on normal-hearing subjects. To test the effectiveness and feasibility of this acoustic model, a psychophysical experiment was conducted to match the performance of a normal-hearing listener using model- processed signals to that of a cochlear implant user. Good agreement was found between an implanted patient and an age-matched normal-hearing subject in a dynamic signal discrimination experiment, indicating that this acoustic model is a reasonably good approximation of a cochlear implant with the SPEAK strategy. The acoustic model was then used to examine the potential of the SPEAK strategy in terms of its temporal and frequency encoding of speech. It was hypothesized that better temporal and frequency encoding of speech can be accomplished by higher stimulation rates and a larger number of activated channels. Vowel and consonant recognition tests were conducted on normal-hearing subjects using speech tokens processed by the acoustic model, with different combinations of stimulation rate and number of activated channels. The results showed that vowel recognition was best at 600 pps and 8 activated channels, but further increases in stimulation rate and
Zou, Jing; Pyykkö, Ilmari; Sutinen, Päivi; Toppila, Esko
Transcranial vibration was applied for seven animals at a frequency of 250 Hz for 15 min, and five animals were used as normal controls to investigate cellular and molecular mechanism linked to vibration-induced hearing loss in animal model. Compound action potential (CAP) thresholds were measured by round window niche electrode. The expression of tumour necrosis factor alpha (TNF-alpha) and its receptors (TNF R1, TNF R2), vascular endothelium growth factor (VEGF) and its receptors (VEGF R1, VEGF R2) were analysed by immunohistochemistry. Transcranial vibration caused expression of TNF-alpha, TNF R1 and TNF R2 in the cochlea and the expression of TNF R2 was stronger than that of TNF R1. Vibration also induced VEGF and VEGF R2 expression in the cochlea. The average immediate hearing loss was 62 dB and after three days still 48 dB. It is concluded that transcranial vibration as during temporal bone drilling produces cochlear shear stress that is connected with up-regulation of TNF-alpha and its receptors. Also VEGF and VEGF R2 are up-regulated. These responses may be linked to both the damage and repair process of the cochlea.
Melo, Renato de Souza; Lemos, Andrea; Macky, Carla Fabiana da Silva Toscano; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica
Children with sensorineural hearing loss can present with instabilities in postural control, possibly as a consequence of hypoactivity of their vestibular system due to internal ear injury. To assess postural control stability in students with normal hearing (i.e., listeners) and with sensorineural hearing loss, and to compare data between groups, considering gender and age. This cross-sectional study evaluated the postural control of 96 students, 48 listeners and 48 with sensorineural hearing loss, aged between 7 and 18 years, of both genders, through the Balance Error Scoring Systems scale. This tool assesses postural control in two sensory conditions: stable surface and unstable surface. For statistical data analysis between groups, the Wilcoxon test for paired samples was used. Students with hearing loss showed more instability in postural control than those with normal hearing, with significant differences between groups (stable surface, unstable surface) (ppostural control compared to normal hearing students of the same gender and age. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.
Full Text Available Recent evidence has shown that noise-induced damage to the synapse between inner hair cells (IHCs and type I afferent auditory nerve fibers (ANFs may occur in the absence of permanent threshold shift (PTS, and that synapses connecting IHCs with low spontaneous rate (SR ANFs are disproportionately affected. Due to the functional importance of low-SR ANF units for temporal processing and signal coding in noisy backgrounds, deficits in cochlear coding associated with noise-induced damage may result in significant difficulties with temporal processing and hearing in noise (i.e., hidden hearing loss. However, significant noise-induced coding deficits have not been reported at the single unit level following the loss of low-SR units. We have found evidence to suggest that some aspects of neural coding are not significantly changed with the initial loss of low-SR ANFs, and that further coding deficits arise in association with the subsequent reestablishment of the synapses. This suggests that synaptopathy in hidden hearing loss may be the result of insufficient repair of disrupted synapses, and not simply due to the loss of low-SR units. These coding deficits include decreases in driven spike rate for intensity coding as well as several aspects of temporal coding: spike latency, peak-to-sustained spike ratio and the recovery of spike rate as a function of click-interval.
Full Text Available Noise-induced hearing loss (NIHL still remains a problem in developed countries, despite reduced occupational noise exposure, strict standards for hearing protection and extensive public health awareness campaigns. Therefore NIHL continues to be the focus of noise research activities. This paper summarizes progress achieved recently in our knowledge of NIHL. It includes papers published between the years 2008-2011 (in English, which were identified by a literature search of accessible medical and other relevant databases. A substantial part of this research has been concerned with the risk of NIHL in the entertainment sector, particularly in professional, orchestral musicians. There are also constant concerns regarding noise exposure and hearing risk in "hard to control" occupations, such as farming and construction work. Although occupational noise has decreased since the early 1980s, the number of young people subject to social noise exposure has tripled. If the exposure limits from the Noise at Work Regulations are applied, discotheque music, rock concerts, as well as music from personal music players are associated with the risk of hearing loss in teenagers and young adults. Several recent research studies have increased the understanding of the pathomechanisms of acoustic trauma, the genetics of NIHL, as well as possible dietary and pharmacologic otoprotection in acoustic trauma. The results of these studies are very promising and offer grounds to expect that targeted therapies might help prevent the loss of sensory hair cells and protect the hearing of noise-exposed individuals. These studies emphasize the need to launch an improved noise exposure policy for hearing protection along with developing more efficient norms of NIHL risk assessment.
Han, Yu; Zhong, Cuiping [Department of Otolaryngology, Xijing Hospital, Fourth Military Medical University, Xi' an, 710032 Shaanxi Province (China); Hong, Liu [First Division of Digestive Diseases, Xijing Hospital, Fourth Military Medical University, Xi' an, 710032 Shaanxi Province (China); Wang, Ye; Qiao, Li [Department of Otolaryngology, Xijing Hospital, Fourth Military Medical University, Xi' an, 710032 Shaanxi Province (China); Qiu, Jianhua, E-mail: email@example.com [Department of Otolaryngology, Xijing Hospital, Fourth Military Medical University, Xi' an, 710032 Shaanxi Province (China)
Noise over-stimulation may induce hair cells loss and hearing deficit. The c-myc oncogene is a major regulator for cell proliferation, growth, and apoptosis. However, the role of this gene in the mammalian cochlea is still unclear. The study was designed to firstly investigate its function under noise condition, from the aspect of cochlear ultrastructural changes. We had established the adenoviral vector of c-myc gene and delivered the adenovirus suspension into the scala tympani of guinea pigs 4 days before noise exposure. The empty adenoviral vectors were injected as control. Then, all subjects were exposed to 4-kHz octave-band noise at 110 dB SPL for 8 h/day, 3 days consecutively. Auditory thresholds were assessed by auditory brainstem response, prior to and 7 days following noise exposure. On the seventh days after noise exposure, the cochlear sensory epithelia surface was observed microscopically and the cochleae were taken to study the ultrastructural changes. The results indicated that auditory threshold shift after noise exposure was higher in the ears treated with Ad.EGFP than that treated with Ad.c-myc-EGFP. Stereocilia loss and the disarrangement of outer hair cells were observed, with greater changes found in the Ad.EGFP group. Also, the ultrastructure changes were severe in the Ad.EGFP group, but not obvious in the Ad.c-myc-EGFP group. Therefore, c-myc gene might play an unexpected role in hearing functional and morphological protection from acoustic trauma.
Han, Yu; Zhong, Cuiping; Hong, Liu; Wang, Ye; Qiao, Li; Qiu, Jianhua
Noise over-stimulation may induce hair cells loss and hearing deficit. The c-myc oncogene is a major regulator for cell proliferation, growth, and apoptosis. However, the role of this gene in the mammalian cochlea is still unclear. The study was designed to firstly investigate its function under noise condition, from the aspect of cochlear ultrastructural changes. We had established the adenoviral vector of c-myc gene and delivered the adenovirus suspension into the scala tympani of guinea pigs 4 days before noise exposure. The empty adenoviral vectors were injected as control. Then, all subjects were exposed to 4-kHz octave-band noise at 110 dB SPL for 8 h/day, 3 days consecutively. Auditory thresholds were assessed by auditory brainstem response, prior to and 7 days following noise exposure. On the seventh days after noise exposure, the cochlear sensory epithelia surface was observed microscopically and the cochleae were taken to study the ultrastructural changes. The results indicated that auditory threshold shift after noise exposure was higher in the ears treated with Ad.EGFP than that treated with Ad.c-myc-EGFP. Stereocilia loss and the disarrangement of outer hair cells were observed, with greater changes found in the Ad.EGFP group. Also, the ultrastructure changes were severe in the Ad.EGFP group, but not obvious in the Ad.c-myc-EGFP group. Therefore, c-myc gene might play an unexpected role in hearing functional and morphological protection from acoustic trauma.
Pogson, Jacob M; Taylor, Rachael L; Young, Allison S; McGarvie, Leigh A; Flanagan, Sean; Halmagyi, G Michael; Welgampola, Miriam S
Acute vertigo with sudden sensorineural hearing loss (SSNHL) is a rare clinical emergency. Here, we report the audio-vestibular test profiles of 27 subjects who presented with these symptoms. The vestibular test battery consisted of a three-dimensional video head impulse test (vHIT) of semicircular canal function and recording ocular and cervical vestibular-evoked myogenic potentials (oVEMP, cVEMP) to test otolith dysfunction. Unlike vestibular neuritis, where the horizontal and anterior canals with utricular function are more frequently impaired, 74 % of subjects with vertigo and SSNHL demonstrated impairment of the posterior canal gain (0.45 ± 0.20). Only 41 % showed impairment of the horizontal canal gains (0.78 ± 0.27) and 30 % of the anterior canal gains (0.79 ± 0.26), while 38 % of oVEMPs [asymmetry ratio (AR) = 41.0 ± 41.3 %] and 33 % of cVEMPs (AR = 47.3 ± 41.2 %) were significantly asymmetrical. Twenty-three subjects were diagnosed with labyrinthitis/labyrinthine infarction in the absence of evidence for an underlying pathology. Four subjects had a definitive diagnosis [Ramsay Hunt Syndrome, vestibular schwannoma, anterior inferior cerebellar artery (AICA) infarction, and traction injury]. Ischemia involving the common-cochlear or vestibulo-cochlear branches of the labyrinthine artery could be the simplest explanation for vertigo with SSNHL. Audio-vestibular tests did not provide easy separation between ischaemic and non-ischaemic causes of vertigo with SSNHL.
Full Text Available Christiane Völter,1 Lisa Götze,1 Michael Falkenstein,2 Stefan Dazert,1 Jan Peter Thomas1 1Department of Otorhinolaryngology, Head and Neck Surgery, Ruhr University Bochum, St Elisabeth-Hospital, Bochum, 2Institute for Work, Learning and Ageing (ALA, Leibniz Research Centre for Working Environment and Human Factors, Dortmund, Germany Introduction: Due to demographic changes, the number of people suffering not only from dementia illness but also from hearing impairment with the need for hearing rehabilitation have increased noticeably. Even with the association between hearing, age, and cognitive decline being well known, this issue has so far not played an important role in daily clinical Ear Nose Throat settings. The aim of the present study was to evaluate the use of a computer-based battery of tests of neurocognitive abilities in older patients with and without hearing loss.Patients and methods: A total of 120 patients aged 50 years and older were enrolled in this prospective clinical study: 40 patients suffered from severe bilateral hearing loss and were tested before cochlear implantation and 80 patients showed normal hearing thresholds between 500 and 4,000 Hz bilaterally. The test battery covered a wide range of cognitive abilities such as long- and short-term memory, working memory (WM, attention, inhibition, and other executive functions. Individuals with severe depression or cognitive impairment were excluded.Results: Hearing status was a significant predictor of performance on delayed recall (P=0.0082 and verbal fluency after adjusting for age (P=0.0016. Age predominantly impacted on inhibition (P=0.0039 and processing speed (P<0.0001, whereas WM measured by the Operation Span task (OSPAN and the attention were influenced by both age and hearing. The battery of tests was feasible and practical for testing older patients without prior computer skills.Conclusion: A computerized neurocognitive assessment battery may be a suitable tool
Fortin, Dominique; Ng, Angela; Tsang, Derek; Sharpe, Michael; Laperriere, Norm; Hodgson, David
Purpose: The increased sparing of normal tissues in intensity modulated proton therapy (IMPT) in pediatric brain tumor treatments should translate into improved neurocognitive outcomes. Models were used to estimate the intelligence quotient (IQ) and the risk of hearing loss 5 years post radiotherapy and to compare outcomes of proton against photon in pediatric brain tumors. Methods: Patients who had received intensity modulated radiotherapy (IMRT) were randomly selected from our retrospective database. The existing planning CT and contours were used to generate IMPT plans. The RBE-corrected dose was calculated for both IMPT and IMRT. For each patient, the IQ was estimated via a Monte Carlo technique, whereas the reported incidence of hearing loss as a function of cochlear dose was used to estimate the probability of occurrence. Results: The integrated brain dose was reduced in all IMPT plans, translating into a gain of 2 IQ points on average for protons for the whole cohort at 5 years post-treatment. In terms of specific diseases, the gains in IQ ranged from 0.8 points for medulloblastoma, to 2.7 points for craniopharyngioma. Hearing loss probability was evaluated on a per-ear-basis and was found to be systematically less for proton versus photon: overall 2.9% versus 7.2%. Conclusions: A method was developed to predict IQ and hearing outcomes in pediatric brain tumor patients on a case-by-case basis. A modest gain was systematically observed for proton in all patients. Given the uncertainties within the model used and our reinterpretation, these gains may be underestimated.
Fortin, Dominique; Ng, Angela; Tsang, Derek; Sharpe, Michael; Laperriere, Norm; Hodgson, David [Princess Margaret Cancer Centre, University of Toronto (Canada)
Purpose: The increased sparing of normal tissues in intensity modulated proton therapy (IMPT) in pediatric brain tumor treatments should translate into improved neurocognitive outcomes. Models were used to estimate the intelligence quotient (IQ) and the risk of hearing loss 5 years post radiotherapy and to compare outcomes of proton against photon in pediatric brain tumors. Methods: Patients who had received intensity modulated radiotherapy (IMRT) were randomly selected from our retrospective database. The existing planning CT and contours were used to generate IMPT plans. The RBE-corrected dose was calculated for both IMPT and IMRT. For each patient, the IQ was estimated via a Monte Carlo technique, whereas the reported incidence of hearing loss as a function of cochlear dose was used to estimate the probability of occurrence. Results: The integrated brain dose was reduced in all IMPT plans, translating into a gain of 2 IQ points on average for protons for the whole cohort at 5 years post-treatment. In terms of specific diseases, the gains in IQ ranged from 0.8 points for medulloblastoma, to 2.7 points for craniopharyngioma. Hearing loss probability was evaluated on a per-ear-basis and was found to be systematically less for proton versus photon: overall 2.9% versus 7.2%. Conclusions: A method was developed to predict IQ and hearing outcomes in pediatric brain tumor patients on a case-by-case basis. A modest gain was systematically observed for proton in all patients. Given the uncertainties within the model used and our reinterpretation, these gains may be underestimated.
Zupan, Barbra; Sussman, Joan E.
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…
Ostojić, Sanja; Mikić, Branka; Mirić, Danica
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 ...
Full Text Available The aim of the following study is to evaluate immediate protective effect of ear plug from noise morphologically and functionally. An 1-month aged 29 male C57BL/6 mice. Subjects were divided into four groups as normal control(G1, bilaterally plugged group (G2, unilaterally plugged group (G3 and noise control group (G4 and later 3 groups were exposed to 110 sound pressure level white noise for 60 min. Immediately after noise exposure, audiologic tests were performed and cochlear morphology and expression levels of a-synuclein in the cochlea were investigated. There were no functional changes in G2 and plugged ears of G3 after noise exposure, whereas unplugged ears of G3 and G4 showed significant hearing loss. In morphological study, there were a significant degeneration of the organ of Corti and mean number and diameter of efferent buttons, in unplugged ears of G3 and G4. Plugged ears of G3 also showed mild changes in morphological study. Reduction of a-synuclein was observed at the efferent terminals or cochlear extracts after noise exposure. The protective effect of ear plug on noise exposure was proven morphologically and functionally in the animal model of noise-induced hearing loss. Further study on cellular or ultrastructural level with ear plug will be needed to reveal more precise mechanism.
Öztürk, Erkan; Doruk, Can; Orhan, Kadir Serkan; Çelik, Mehmet; Polat, Beldan; Güldiken, Yahya
Cochlear implants are mechanical devices used for patients with severe sensory-neural hearing loss, which has an inner magnet. It is proven that 1.5 Tesla magnetic resonance imaging (MRI) scanners are safe to use in patients with cochlear implant. In our patient, the authors aim to introduce a rare complication caused after a 1.5 Tesla MRI scanning and the management of this situation; the reversion of the magnet of the implant without displacement and significance of surgery in management.
Seddon, James A; Thee, Stephanie; Jacobs, Kayleen; Ebrahim, Adam; Hesseling, Anneke C; Schaaf, H Simon
The aminoglycosides and polypeptides are vital drugs for the management of multidrug-resistant (MDR) tuberculosis (TB). Both classes of drug cause hearing loss. We aimed to determine the extent of hearing loss in children treated for MDR-TB. In this retrospective study, children (Hearing was assessed and classified using audiometry and otoacoustic emissions. Ninety-four children were included (median age: 43 months). Of 93 tested, 28 (30%) were HIV-infected. Twenty-three (24%) children had hearing loss. Culture-confirmed, as opposed to presumed, diagnosis of TB was a risk factor for hearing loss (OR: 4.12; 95% CI: 1.13-15.0; p = 0.02). Seven of 11 (64%) children classified as having hearing loss using audiometry had progression of hearing loss after finishing the injectable drug. Hearing loss is common in children treated for MDR-TB. Alternative drugs are required for the treatment of paediatric MDR-TB. Copyright © 2012 The British Infection Association. Published by Elsevier Ltd. All rights reserved.
Full Text Available Introduction: Hearing loss is one of the most common problems in elderly people. Functional side effects of hearing loss are various. Due to the fact that hearing loss is the common impairment in elderly people; the importance of its possible effects on auditory memory is undeniable. This study aims to focus on the hearing loss effects on auditory memory. Materials and Methods: Dichotic Auditory Memory Test (DVMT was performed on 47 elderly people, aged 60 to 80; that were divided in two groups, the first group consisted of elderly people with hearing range of 24 normal and the second one consisted of 23 elderly people with bilateral symmetrical ranged from mild to moderate Sensorineural hearing loss in the high frequency due to aging in both genders. Results: Significant difference was observed in DVMT between elderly people with normal hearing and those with hearing loss (P
Liliane Aparecida Fagundes Silva
Full Text Available The purpose of this study was to longitudinally assess the behavioral and electrophysiological hearing changes of a girl inserted in a CI program, who had bilateral profound sensorineural hearing loss and underwent surgery of cochlear implantation with electrode activation at 21 months of age. She was evaluated using the P1 component of Long Latency Auditory Evoked Potential (LLAEP; speech perception tests of the Glendonald Auditory Screening Procedure (GASP; Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS; and Meaningful Use of Speech Scales (MUSS. The study was conducted prior to activation and after three, nine, and 18 months of cochlear implant activation. The results of the LLAEP were compared with data from a hearing child matched by gender and chronological age. The results of the LLAEP of the child with cochlear implant showed gradual decrease in latency of the P1 component after auditory stimulation (172 ms–134 ms. In the GASP, IT-MAIS, and MUSS, gradual development of listening skills and oral language was observed. The values of the LLAEP of the hearing child were expected for chronological age (132 ms–128 ms. The use of different clinical instruments allow a better understanding of the auditory habilitation and rehabilitation process via CI.
Silva, Liliane Aparecida Fagundes; Couto, Maria Inês Vieira; Tsuji, Robinson Koji; Bento, Ricardo Ferreira; de Carvalho, Ana Claudia Martinho; Matas, Carla Gentile
The purpose of this study was to longitudinally assess the behavioral and electrophysiological hearing changes of a girl inserted in a CI program, who had bilateral profound sensorineural hearing loss and underwent surgery of cochlear implantation with electrode activation at 21 months of age. She was evaluated using the P1 component of Long Latency Auditory Evoked Potential (LLAEP); speech perception tests of the Glendonald Auditory Screening Procedure (GASP); Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS); and Meaningful Use of Speech Scales (MUSS). The study was conducted prior to activation and after three, nine, and 18 months of cochlear implant activation. The results of the LLAEP were compared with data from a hearing child matched by gender and chronological age. The results of the LLAEP of the child with cochlear implant showed gradual decrease in latency of the P1 component after auditory stimulation (172 ms–134 ms). In the GASP, IT-MAIS, and MUSS, gradual development of listening skills and oral language was observed. The values of the LLAEP of the hearing child were expected for chronological age (132 ms–128 ms). The use of different clinical instruments allow a better understanding of the auditory habilitation and rehabilitation process via CI. PMID:26881163
Joseph P. Pillion
Full Text Available A case study is presented of a 17-year-old male who sustained an anoxic brain injury and sensorineural hearing loss secondary to carbon monoxide poisoning. Audiological data is presented showing a slightly asymmetrical hearing loss of sensorineural origin and mild-to-severe degree for both ears. Word recognition performance was fair to poor bilaterally for speech presented at normal conversational levels in quiet. Management considerations of the hearing loss are discussed.
Paul, Brandon T; Waheed, Sajal; Bruce, Ian C; Roberts, Larry E
Noise exposure and aging can damage cochlear synapses required for suprathreshold listening, even when cochlear structures needed for hearing at threshold remain unaffected. To control for effects of aging, behavioral amplitude modulation (AM) detection and subcortical envelope following responses (EFRs) to AM tones in 25 age-restricted (18-19 years) participants with normal thresholds, but different self-reported noise exposure histories were studied. Participants with more noise exposure had smaller EFRs and tended to have poorer AM detection than less-exposed individuals. Simulations of the EFR using a well-established cochlear model were consistent with more synaptopathy in participants reporting greater noise exposure.
Preminger, Jill E; Meeks, Suzanne
The purpose of this research was to investigate the congruent/incongruent perceptions of hearing-loss related quality of life between members of couples and to determine how incongruence was affected by individual psychosocial characteristics, specifically measures of mood (negative affect and positive affect), stress, and communication in the marriage. An exploratory correlational analysis was performed on data for 52 couples in which only one member had a hearing loss. In the regression analyses the independent variables were hearing-loss related quality of life scores measured in people with hearing loss, measured in significant others, and differences in hearing-loss related quality of life among members of a couple. The results demonstrate that both in people with hearing loss and their significant others, perceptions of hearing-loss related quality of life is highly correlated with negative mood scores. Incongruence in hearing-loss related quality of life scores reported by members of a couple were highly correlated with negative affect measured within each individual. Future research evaluating the effectiveness of audiologic rehabilitation can use measures of mood as an outcome variable.
Litovsky, Ruth Y; Moua, Keng; Godar, Shelly; Kan, Alan; Misurelli, Sara M; Lee, Daniel J
In recent years, cochlear implants (CIs) have been provided in growing numbers to people with not only bilateral deafness but also to people with unilateral hearing loss, at times in order to alleviate tinnitus. This study presents audiological data from 15 adult participants (ages 48 ± 12 years) with single sided deafness. Results are presented from 9/15 adults, who received a CI (SSD-CI) in the deaf ear and were tested in Acoustic or Acoustic + CI hearing modes, and 6/15 adults who are planning to receive a CI, and were tested in the unilateral condition only. Testing included (1) audiometric measures of threshold, (2) speech understanding for CNC words and AzBIO sentences, (3) tinnitus handicap inventory, (4) sound localization with stationary sound sources, and (5) perceived auditory motion. Results showed that when listening to sentences in quiet, performance was excellent in the Acoustic and Acoustic + CI conditions. In noise, performance was similar between Acoustic and Acoustic + CI conditions in 4/6 participants tested, and slightly worse in the Acoustic + CI in 2/6 participants. In some cases, the CI provided reduced tinnitus handicap scores. When testing sound localization ability, the Acoustic + CI condition resulted in improved sound localization RMS error of 29.2° (SD: ±6.7°) compared to 56.6° (SD: ±16.5°) in the Acoustic-only condition. Preliminary results suggest that the perception of motion direction, whereby subjects are required to process and compare directional cues across multiple locations, is impaired when compared with that of normal hearing subjects. Copyright © 2018 Elsevier B.V. All rights reserved.
Gantz, Bruce J; Dunn, Camille C; Walker, Elizabeth A; Kenworthy, Maura; Van Voorst, Tanya; Tomblin, Bruce; Turner, Chris
The purpose of this feasibility study was to evaluate whether the use of a shorter-length cochlear implant (10 mm) on one ear and a standard electrode (24 mm) on the contralateral ear is a viable bilateral option for children with profound bilateral sensorineural hearing loss. A secondary purpose of this study was to determine whether the ear with the shorter-length electrode performs similarly to the standard-length electrode. Our goal was to provide an option of electrical stimulation that theoretically might preserve the structures of the scala media and organ of Corti. The study is being conducted as a repeated-measure, single-subject experiment. University of Iowa-Department of Otolaryngology. Eight pediatric patients with profound bilateral sensorineural hearing loss between the ages of 12 and 24 months. Nucleus Hybrid S12 10-mm electrode and a Nucleus Freedom implant in the contralateral ear. The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) parent questionnaire, Early Speech Perception, Glendonald Auditory Screening Procedure word test, and Children's Vowel tests will be used to evaluate speech perception and the Minnesota Child Development Inventory and Preschool Language Scales 3 test will be used to evaluate language growth. Preliminary results for 8 children have been collected before and after the operation using the IT-MAIS. All 3 children showed incremental improvements in their IT-MAIS scores overtime. Early Speech Perception, Glendonald Auditory Screening Procedure word test, and Children's Vowel word perception results indicated no difference between the individual ears for the 2 children tested. Performance compared with age-matched children implanted with standard bilateral cochlear implants showed similar results to the children implanted with Nucleus Hybrid S12 10-mm electrode and a Nucleus Freedom implant in contralateral ears. The use of a shorter-length cochlear implant on one ear and a standard-length electrode on the
Cayé-Thomasen, Per; Worsøe, Lise; Brandt, Christian Thomas
OBJECTIVES/HYPOTHESIS: To examine the routes, dynamics and correlates of cochlear inflammation in meningitis to provide information on the pathogenesis of the associated hearing loss and indications for rational pharmacotherapeutical intervention. STUDY DESIGN: A well-established rat model...... with inflammatory cells via cochlear aqueduct, whereas the endolymphatic space was infiltrated from the spiral ligament. Rosenthal's canal was infiltrated through osseous spiral lamina canaliculi. In the untreated group, the degree of inflammation correlated with time of death, whereas antibiotic treatment reversed...... this development. Perilymphatic inflammation correlated significantly with the CSF leukocyte count, whereas endolymphatic inflammation correlated with spiral ligament inflammation. CONCLUSIONS: Meningogenic inflammation of the rat cochlea occurs via the cochlear aqueduct and the spiral ligament capillary bed...
Deshpande, Aniruddha K; Tan, Lirong; Lu, Long J; Altaye, Mekibib; Holland, Scott K
Despite the positive effects of cochlear implantation, postimplant variability in speech perception and oral language outcomes is still difficult to predict. The aim of this study was to identify neuroimaging biomarkers of postimplant speech perception and oral language performance in children with hearing loss who receive a cochlear implant. The authors hypothesized positive correlations between blood oxygen level-dependent functional magnetic resonance imaging (fMRI) activation in brain regions related to auditory language processing and attention and scores on the Clinical Evaluation of Language Fundamentals-Preschool, Second Edition (CELF-P2) and the Early Speech Perception Test for Profoundly Hearing-Impaired Children (ESP), in children with congenital hearing loss. Eleven children with congenital hearing loss were recruited for the present study based on referral for clinical MRI and other inclusion criteria. All participants were stimulation. A voxel-based analysis technique was utilized to generate z maps showing significant contrast in brain activation between auditory stimulation conditions (spoken narratives and narrow band noise). CELF-P2 and ESP were administered 2 years after implantation. Because most participants reached a ceiling on ESP, a voxel-wise regression analysis was performed between preimplant fMRI activation and postimplant CELF-P2 scores alone. Age at implantation and preimplant hearing thresholds were controlled in this regression analysis. Four brain regions were found to be significantly correlated with CELF-P2 scores. These clusters of positive correlation encompassed the temporo-parieto-occipital junction, areas in the prefrontal cortex and the cingulate gyrus. For the story versus silence contrast, CELF-P2 core language score demonstrated significant positive correlation with activation in the right angular gyrus (r = 0.95), left medial frontal gyrus (r = 0.94), and left cingulate gyrus (r = 0.96). For the narrow band noise versus
Dubach, Patrick; Pfiffner, Flurin; Kompis, Martin; Caversaccio, Marco
Background Telephone communication is a challenge for many hearing-impaired individuals. One important technical reason for this difficulty is the restricted frequency range (0.3–3.4 kHz) of conventional landline telephones. Internet telephony (voice over Internet protocol [VoIP]) is transmitted with a larger frequency range (0.1–8 kHz) and therefore includes more frequencies relevant to speech perception. According to a recently published, laboratory-based study, the theoretical advantage of ideal VoIP conditions over conventional telephone quality has translated into improved speech perception by hearing-impaired individuals. However, the speech perception benefits of nonideal VoIP network conditions, which may occur in daily life, have not been explored. VoIP use cannot be recommended to hearing-impaired individuals before its potential under more realistic conditions has been examined. Objective To compare realistic VoIP network conditions, under which digital data packets may be lost, with ideal conventional telephone quality with respect to their impact on speech perception by hearing-impaired individuals. Methods We assessed speech perception using standardized test material presented under simulated VoIP conditions with increasing digital data packet loss (from 0% to 20%) and compared with simulated ideal conventional telephone quality. We monaurally tested 10 adult users of cochlear implants, 10 adult users of hearing aids, and 10 normal-hearing adults in the free sound field, both in quiet and with background noise. Results Across all participant groups, mean speech perception scores using VoIP with 0%, 5%, and 10% packet loss were 15.2% (range 0%–53%), 10.6% (4%–46%), and 8.8% (7%–33%) higher, respectively, than with ideal conventional telephone quality. Speech perception did not differ between VoIP with 20% packet loss and conventional telephone quality. The maximum benefits were observed under ideal VoIP conditions without packet loss and
Hay-McCutcheon, Marcia J; Hyams, Adriana; Yang, Xin; Parton, Jason
Perceived social support and hearing handicap were assessed in adults with and without hearing loss who lived in different geographical regions of Alabama. The Hearing Handicap Inventory for Adults (HHIA) assessed emotional and social consequences of hearing loss. The Medical Outcomes Study (MOS) Social Support Survey and the Social Functioning, Role Emotional and Mental Health scales of the SF-36 were administered. Data were collected from 71 study participants with hearing loss and from 45 adults without hearing loss. Degree of hearing loss and outcomes from the HHIA did not differ between adults who lived in rural or urban settings. Tangible support was poorer for adults with hearing loss who lived in rural settings compared to those who lived in urban settings. For adults without hearing loss, residency was not associated with tangible support. For these adults, income was associated with other types of social support (i.e. informational support, affection, positive social interaction). Adults with hearing loss living in rural areas had poor perceived tangible support. The provision of support to address a hearing loss could be worse for these adults compared to adults who lived in urban settings.
Werfel, Krystal L.; Lund, Emily; Schuele, C. Melanie
Measures of print knowledge were compared across preschoolers with hearing loss and normal hearing. Alphabet knowledge did not differ between groups, but preschoolers with hearing loss performed lower on measures of print concepts and concepts of written words than preschoolers with normal hearing. Further study is needed in this area.
Full Text Available Abstract Objectives This exploratory study investigates the prevalence of gambling, preferred types of gambling, and problem gambling in Swedish young people aged 15–18 years with and without hearing loss. Methods A cross-sectional health survey was conducted in Örebro County, Sweden in 2014. A standardized questionnaire was distributed to 4888 students, and 4329 filled it. There were 318 (8 % students with hearing loss. The response rate was 82 %. The 2-item Lie/Bet questionnaire (Johnson et al. in Psychol Rep 80:83–88, 1997 was used for measuring problem gambling. Results More students with hearing loss had gambled during their lifetime (35 % and in the past year (25 % than their hearing counterparts (lifetime: 24 %; past-year: 19 %. More students with hearing loss compared to normal hearing students were identified as problem gamblers (7.7 % compared to 4.3 %. Conclusion More research is needed on gambling among people with hearing loss as well as other disabilities.
Reisser, Christoph F V; Kimberling, William J; Otterstedde, Christian R
Usher syndrome is an autosomal recessive disorder characterized by sensorineural hearing loss and progressive visual loss secondary to retinitis pigmentosa. In the literature, a possible progression of the moderate to severe hearing loss in Usher syndrome type II (Usher II) is controversial. We studied the development of the hearing loss of 125 patients with a clinical diagnosis of Usher syndrome type II intraindividually and interindividually by repeatedly performing complete audiological and neuro-otologic examinations. Our data show a very characteristic slope of the hearing curve in all Usher II patients and no clinically relevant progression of the hearing loss over up to 17 years. The subjective impression of a deterioration of the communicative abilities of Usher II patients must therefore be attributed to the progressive visual loss. The patients should be reassured that changes in their hearing abilities are unlikely and should be provided with optimally fitted modern hearing aids.
Mollasadeghi, Abolfazl; Mehrparvar, Amir Houshang; Atighechi, Saeid
Magnetic resonance imaging (MRI) devices produce noise, which may affect patient's or operators' hearing. Some cases of hearing impairment after MRI procedure have been reported with different patterns (temporary or permanent, unilateral or bilateral, with or without other symptoms like tinnitus......). In this report, a case of bilateral sensorineural hearing loss in an otherwise healthy patient underwent brain MRI was described. The patient's hearing loss was accompanied with tinnitus and was not improved after 3 months of followup....
... can allow many infants to develop normal language skills without delay. In infants born with hearing loss, ... therapy allow many children to develop normal language skills at the same age as their peers with ...
Plakke, B L; Dare, E
Studies have shown that there is a great deal of high-frequency sensorineural hearing loss among farmers. The studies have failed, however, to differentiate farmers who have occupational noise exposure only from other potential hearing loss etiologies. This study, through extensive case history information, has isolated a farm noise-exposure group and matched its members by age with persons with no significant noise exposure. Results indicate that farmers exposed only to noise from farming ha...
Full Text Available Abstract Background Tinnitus is an auditory sensation frequently following hearing loss. After cochlear injury, deafferented neurons become sensitive to neighbouring intact edge-frequencies, guiding an enhanced central representation of these frequencies. As psychoacoustical data 123 indicate enhanced frequency discrimination ability for edge-frequencies that may be related to a reorganization within the auditory cortex, the aim of the present study was twofold: 1 to search for abnormal auditory mismatch responses in tinnitus sufferers and 2 relate these to subjective indicators of tinnitus. Results Using EEG-mismatch negativity, we demonstrate abnormalities (N = 15 in tinnitus sufferers that are specific to frequencies located at the audiometrically normal lesion-edge as compared to normal hearing controls (N = 15. Groups also differed with respect to the cortical locations of mismatch responsiveness. Sources in the 90–135 ms latency window were generated in more anterior brain regions in the tinnitus group. Both measures of abnormality correlated with emotional-cognitive distress related to tinnitus (r ~ .76. While these two physiological variables were uncorrelated in the control group, they were correlated in the tinnitus group (r = .72. Concerning relationships with parameters of hearing loss (depth and slope, slope turned out to be an important variable. Generally, the steeper the hearing loss is the less distress related to tinnitus was reported. The associations between slope and the relevant neurophysiological variables are in agreement with this finding. Conclusions The present study is the first to show near-to-complete separation of tinnitus sufferers from a normal hearing control group based on neurophysiological variables. The finding of lesion-edge specific effects and associations with slope of hearing loss corroborates the assumption that hearing loss is the basis for tinnitus development. It is likely that some central
Qian, Haifeng; Loizou, Philipos C; Dorman, Michael F
Hearing-impaired people, and particularly hearing-aid and cochlear-implant users, often have difficulty communicating over the telephone. The intelligibility of telephone speech is considerably lower than the intelligibility of face-to-face speech. This is partly because of lack of visual cues, limited telephone bandwidth, and background noise. In addition, cellphones may cause interference with the hearing aid or cochlear implant. To address these problems that hearing-impaired people experience with telephones, this paper proposes a wireless phone adapter that can be used to route the audio signal directly to the hearing aid or cochlear implant processor. This adapter is based on Bluetooth technology. The favorable features of this new wireless technology make the adapter superior to traditional assistive listening devices. A hardware prototype was built and software programs were written to implement the headset profile in the Bluetooth specification. Three cochlear implant users were tested with the proposed phone-adapter and reported good speech quality.
Morata, Thais C.; Meinke, Deanna
Occupational health agencies, researchers and policy makers have recognized the need for evidence on the effectiveness of interventions designed to reduce or prevent workplace injuries and illnesses. While many workplaces comply with legal or obligatory requirements and implement recommended interventions, few publications exist documenting the effectiveness of these actions. Additionally, some workplaces have discovered through their own processes, novel ways to reduce the risk of injury. Peer-reviewed information on the effectiveness of the many strategies and approaches currently in use could help correct weaknesses, or further encourage their adoption and expansion. The evaluation of intervention effectiveness would certainly contribute to improved worker health and safety. This need is particularly relevant regarding noise exposure in the workplace and hearing loss prevention interventions. In a 2006 review of the U.S. National Institute for Occupational Safety and Health (NIOSH) Hearing Loss Research Program, the independent National Academies of Sciences recommended that NIOSH place greater emphasis on identifying the effectiveness of hearing loss prevention measures on the basis of outcomes that are as closely related as possible to reducing noise exposure and work related hearing loss (http://www.nap.edu/openbook.php?record_id=11721). NIOSH used two different approaches to address that recommendation: the first one was to conduct research, including broad systematic reviews on the effectiveness of interventions to prevent occupational noise-induced hearing loss. The second was to create an award program, the Safe-In-Sound Excellence in Hearing Loss Prevention Award™, to identify and honor excellent real-world examples of noise control and other hearing loss prevention practices and innovations. PMID:27397968
Eom, Tae-Ho; Lee, Sungsu; Cho, Hyong-Ho; Cho, Yong-Beom
Despite the well-established nature of bromate-induced ototoxicity, cochlear implantation after bromate intoxication has been rarely documented. We hereby present a case of a 51-year-old female deafened completely after bromate ingestion. Her hearing was not restored by systemic steroid treatment and hearing aids were of no use. A cochlear implantation was performed on her right ear 3 months after the bromate ingestion. In bromate intoxication cases, early monitoring of hearing level is necessary and other drugs with potential ototoxicity should be avoided. The outcome of cochlear implantation was excellent in this case of bromate-induced deafness.
David B. Pisoni
Full Text Available Cochlear implants (CIs often work very well for many children and adults with profound sensorineural (SNHL hearing loss. Unfortunately, while many CI patients display substantial benefits in recognizing speech and understanding spoken language following cochlear implantation, a large number of patients achieve poor outcomes. Understanding and explaining the reasons for poor outcomes following implantation is a very challenging research problem that has received little attention despite the pressing clinical significance. In this paper, we discuss three challenges for future research on CIs. First, we consider the issue of individual differences and variability in outcomes following implantation. At the present time, we still do not have a complete and satisfactory account of the causal underlying factors that are responsible for the enormous individual differences and variability in outcomes. Second, we discuss issues related to the lack of preimplant predictors of outcomes. Very little prospective research has been carried out on the development of preimplant predictors that can be used to reliably identify CI candidates who may be at high risk for a poor outcome following implantation. Other than conventional demographics and hearing history, there are no prognostic tools available to predict speech recognition outcomes after implantation. Finally, we discuss the third challenge — what to do with a CI-user who has a poor outcome. We suggest that new research efforts need to be devoted to studying this neglected clinical population in greater depth to find out why they are doing poorly with their CI and what novel interventions and treatments can be developed to improve their speech recognition outcomes. Using these three challenges as objectives for future research on CIs, we suggest that the field needs to adopt a new narrative grounded in theory and methods from Cognitive Hearing Science and information processing theory. Without knowing
Full Text Available Magnetic resonance imaging (MRI devices produce noise, which may affect patient’s or operators’ hearing. Some cases of hearing impairment after MRI procedure have been reported with different patterns (temporary or permanent, unilateral or bilateral, with or without other symptoms like tinnitus. In this report, a case of bilateral sensorineural hearing loss in an otherwise healthy patient underwent brain MRI was described. The patient’s hearing loss was accompanied with tinnitus and was not improved after 3 months of followup.
Moreland, Christopher; Atcherson, Samuel R; Zazove, Philip; McKee, Michael M
Hearing loss can lead to impairments in language and speech acquisition, educational attainment, social development, and reading achievement. More than 90% of deaf and hard of hearing (DHH) children are born to hearing parents who may lack the knowledge or experience to effectively care for a child with hearing loss. Family involvement is crucial for teaching self-advocacy and global communication skills, optimizing social development, and helping DHH individuals understand and manage external attitudes about deafness and hearing loss. American Sign Language is a naturally developed language with an always-expanding lexicon and grammatical structures different from those of English. Teaching spoken English and American Sign Language equally, often called bilingual bimodal education, can enhance academic and reading achievement as well as language and psychosocial development. Formal schooling options for a DHH child include enrollment in a public or private school system (often called inclusion, integration, or mainstreaming), a school for the deaf, or a bilingual school. Individuals with hearing loss experience stereotypes and biases that create disparities in health insurance coverage, health care access, and outcomes of mental and physical conditions. Family physicians should recognize and minimize biases to improve health care in the DHH community. Written permission from the American Academy of Family Physicians is required for reproduction of this material in whole or in part in any form or medium.
Brand, Yves; Senn, Pascal; Kompis, Martin; Dillier, Norbert; Allum, John H J
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 been made since then. Modern, multichannel implant systems feature complex speech processing strategies, high stimulation rates and multiple sites of stimulation in the cochlea. Equipped with such a state-of-the-art system, the majority of recipients today can communicate orally without visual cues and can even use the telephone. The impact of CIs on deaf individuals and on the deaf community has thus been exceptional. To date, more than 300,000 patients worldwide have received CIs. In Switzerland, the first implantation was performed in 1977 and, as of 2012, over 2,000 systems have been implanted with a current rate of around 150 CIs per year. The primary purpose of this article is to provide a contemporary overview of cochlear implantation, emphasising the situation in Switzerland.
Full Text Available An 18-year-old man presented with a sudden onset of bilateral hearing