Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review

10.3109/14992027.2015.1122237<br/>Wen Jiang

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Eficacia de las intervenciones para el tratamiento de la dislexia: una revisión

Publication date: Available online 14 January 2016
Source:Revista de Logopedia, Foniatría y Audiología
Author(s): Juan C. Ripoll Salceda, Gerardo Aguado Alonso
En este informe se revisa la investigación que respalda distintas intervenciones para el tratamiento de la dislexia, concretamente los trabajos de síntesis, en especial síntesis de las mejores evidencias, revisiones sistemáticas o metaanálisis publicados sobre cada tipo de intervención. En los casos en que no se ha publicado ningún trabajo de síntesis o estos trabajos son antiguos, se hace una revisión de la investigación primaria.Los trabajos localizados indican que los métodos fonológicos de intervención en la dislexia, es decir, aquellos en los que se combina el entrenamiento en habilidades fonológicas con el conocimiento de las letras y la práctica de la lectura, están ampliamente respaldados por la investigación. En cambio, la investigación sobre otros sistemas es sumamente escasa, ofrece resultados contradictorios o indica que se trata de sistemas ineficaces. Las intervenciones que están en estas situaciones son: la integración auditiva, el entrenamiento auditivo con soporte informático, la terapia visual, las lentes tintadas, el entrenamiento perceptivo-motor, las técnicas quiroprácticas, la integración sensorial, el método Davis, el neurofeedback, la musicoterapia y la educación musical o las dietas y suplementos alimenticios.A partir de esta revisión se realizan las siguientes recomendaciones: promocionar las intervenciones de tipo fonológico para el tratamiento de la dislexia, no utilizar o recomendar intervenciones que no estén respaldadas por la investigación científica, que los métodos de intervención que no hayan probado su eficacia no reciban el nombre de «terapia» o «tratamiento», y la creación y difusión de una guía sobre intervención en dislexia basada en evidencias científicas.In this report the research that supports various interventions for the treatment of dyslexia is revised, specifically the works of synthesis, especially synthesis of best evidence, systematic reviews or meta-analyses published about each type of intervention. A review of primary research is made in cases in which no synthesis work has been published or where these works are old.Localised works indicate that the phonological methods of intervention in dyslexia, i.e. those in which training in phonological skills is combined with knowledge of letters and the practice of reading, are widely supported by research. However, research on other methods is either extremely scarce or it offers conflicting results or it leads to the conclusion that they are inefficient methods. Interventions in this situation are: the auditory integration, the auditory training with computer support, visual therapy, the tinted lenses, motor-perceptual training, chiropractic techniques, sensory integration, the Davis method, neurofeedback, music therapy and music education or diets and dietary supplements.Promotion of phonological interventions is recommended; it is necessary to avoid recommendations of intervention methods for dyslexia which are not supported by scientific research; methods of intervention that have not proven their effectiveness may not be presented as therapies or treatments; and the creation and distribution of a guide on intervention in dyslexia based on scientific evidence is recommended.



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Vocal nodules in school age children

Publication date: Available online 15 January 2016
Source:Revista de Logopedia, Foniatría y Audiología
Author(s): Janete Coelho, Daniela Ramos, Isabel Monteiro, António Diogo Paiva
IntroductionVocal nodules are the most common organic and functional lesions among the paediatrics population, being the main cause of dysphonia during childhood. In Portugal, a few studies that describe the vocal parameters in school age children with this pathology were found. The objective of this paper is to analyse the perceptual and acoustic vocal parameters of school age children with vocal nodules and to compare them with a group of children without vocal nodules.MethodologyThe perceptual and acoustic parameters of 5 children, of both genders, aged from 7 years and 2 months to 12 years and 10 months, where clinical diagnosis of vocal nodules were analysed. The following assessment instruments were used: software Praat, GRBASI scale, maximum phonation time and s/z coefficient. The obtained data was compared to children without vocal nodules. The Mann–Whitney U test, with p<.05 significance level, in SPSS Statistics, version 22.0 was used for statistical analysis. The results are presented as mean and standard deviations.ResultsStatistically significant differences were registered between the group of vocal nodules vs. the group without vocal nodules, on the following parameters: fundamental frequency, shimmer, HNR, maximum phonation time for /a/ e /z/, s/z coefficient and GRBASI. On jitter and maximum phonation time for /s/ there were no statistically significant differences.Discussion and conclusionsThis study underlines the importance of assessing the perceptual and acoustic parameters in children with vocal nodules.



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Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review.

Related Articles

Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review.

Int J Audiol. 2016 Jan 15;:1-9

Authors: Jiang W, Zhao F, Guderley N, Manchaiah V

Abstract
OBJECTIVE: This systematic review aimed to explore the evidence on whether the preferred listening levels (PLLs) and durations of music listening through personal listening devices (PLDs) in adolescents and young adults exceed the current recommended 100% daily noise dose; together with the impact on hearing and possible influential factors of such listening behaviours.
DESIGN: A systematic search was conducted using multiple online bibliographic databases.
STUDY SAMPLE: The 26 studies were included on the basis of the inclusion and exclusion criteria.
RESULTS: The results showed that up to 58.2% of participants exceeded the 100% daily noise dose, particularly in the presence of background noise. Significantly positive correlations were found among background noise levels and mean PLLs, as well as the proportion of participants exceeding the 100% daily noise dose. Moreover, significantly worse hearing thresholds were found in PLD users using audiometry, and significantly poor results in otoacoustic emission (OAE), even in the participants with self-reported 'normal hearing'.
CONCLUSION: It is crucial to develop appropriate standards and safe recommendations for daily music exposure dose in future studies. Providing an essential guide and effective education to adolescents and young adults will help raise awareness, increase knowledge, and consequently change attitudes and listening habits.

PMID: 26768911 [PubMed - as supplied by publisher]

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Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review

10.3109/14992027.2015.1122237<br/>Wen Jiang

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Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review.

Related Articles

Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review.

Int J Audiol. 2016 Jan 15;:1-9

Authors: Jiang W, Zhao F, Guderley N, Manchaiah V

Abstract
OBJECTIVE: This systematic review aimed to explore the evidence on whether the preferred listening levels (PLLs) and durations of music listening through personal listening devices (PLDs) in adolescents and young adults exceed the current recommended 100% daily noise dose; together with the impact on hearing and possible influential factors of such listening behaviours.
DESIGN: A systematic search was conducted using multiple online bibliographic databases.
STUDY SAMPLE: The 26 studies were included on the basis of the inclusion and exclusion criteria.
RESULTS: The results showed that up to 58.2% of participants exceeded the 100% daily noise dose, particularly in the presence of background noise. Significantly positive correlations were found among background noise levels and mean PLLs, as well as the proportion of participants exceeding the 100% daily noise dose. Moreover, significantly worse hearing thresholds were found in PLD users using audiometry, and significantly poor results in otoacoustic emission (OAE), even in the participants with self-reported 'normal hearing'.
CONCLUSION: It is crucial to develop appropriate standards and safe recommendations for daily music exposure dose in future studies. Providing an essential guide and effective education to adolescents and young adults will help raise awareness, increase knowledge, and consequently change attitudes and listening habits.

PMID: 26768911 [PubMed - as supplied by publisher]

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Noise-Induced Neural Degeneration and Therapeutic Effect of Antioxidant Drugs.

Related Articles

Noise-Induced Neural Degeneration and Therapeutic Effect of Antioxidant Drugs.

J Audiol Otol. 2015 Dec;19(3):111-9

Authors: Choi SH, Choi CH

Abstract
The primary site of lesion induced by noise exposure is the hair cells in the organ of Corti and the primary neural degeneration occurs in synaptic terminals of cochlear nerve fibers and spiral ganglion cells. The cellular basis of noise-induced hearing loss is oxidative stress, which refers to a severe disruption in the balance between the production of free radicals and antioxidant defense system in the cochlea by excessive production of free radicals induced by noise exposure. Oxidative stress has been identified by a variety of biomarkers to label free radical activity which include four-hydroxy-2-nonenal, nitrotyrosine, and malondialdehyde, and inducible nitric oxide synthase, cytochrome-C, and cascade-3, 8, 9. Furthermore, oxidative stress is contributing to the necrotic and apoptotic cell deaths in the cochlea. To counteract the known mechanisms of pathogenesis and oxidative stress induced by noise exposure, a variety of antioxidant drugs including oxygen-based antioxidants such as N-acetyl-L-cystein and acetyl-L-carnitine and nitrone-based antioxidants such as phenyl-N-tert-butylnitrone (PBN), disufenton sodium, 4-hydroxy PBN, and 2, 4-disulfonyl PBN have been used in our laboratory. These antioxidant drugs were effective in preventing or treating noise-induced hearing loss. In combination with other antioxidants, antioxidant drugs showed a strong synergistic effect. Furthermore, successful use of antioxidant drugs depends on the optimal timing of treatment and the duration of treatment, which are highly related to the time window of free radical formation induced by noise exposure.

PMID: 26771008 [PubMed]

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Auditory nerve synapses persist in ventral cochlear nucleus long after loss of acoustic input in mice with early-onset progressive hearing loss.

Related Articles

Auditory nerve synapses persist in ventral cochlear nucleus long after loss of acoustic input in mice with early-onset progressive hearing loss.

Brain Res. 2015 Apr 24;1605:22-30

Authors: McGuire B, Fiorillo B, Ryugo DK, Lauer AM

Abstract
Perceptual performance in persons with hearing loss, especially those using devices to restore hearing, is not fully predicted by traditional audiometric measurements designed to evaluate the status of peripheral function. The integrity of auditory brainstem synapses may vary with different forms of hearing loss, and differential effects on the auditory nerve-brain interface may have particularly profound consequences for the transfer of sound from ear to brain. Loss of auditory nerve synapses in ventral cochlear nucleus (VCN) has been reported after acoustic trauma, ablation of the organ of Corti, and administration of ototoxic compounds. The effects of gradually acquired forms deafness on these synapses are less well understood. We investigated VCN gross morphology and auditory nerve synapse integrity in DBA/2J mice with early-onset progressive sensorineural hearing loss. Hearing status was confirmed using auditory brainstem response audiometry and acoustic startle responses. We found no change in VCN volume, number of macroneurons, or number of VGLUT1-positive auditory nerve terminals between young adult and older, deaf DBA/2J. Cell-type specific analysis revealed no difference in the number of VGLUT1 puncta contacting bushy and multipolar cell body profiles, but the terminals were smaller in deaf DBA/2J mice. Transmission electron microscopy confirmed the presence of numerous healthy, vesicle-filled auditory nerve synapses in older, deaf DBA/2J mice. The present results suggest that synapses can be preserved over a relatively long time-course in gradually acquired deafness. Elucidating the mechanisms supporting survival of central auditory nerve synapses in models of acquired deafness may reveal new opportunities for therapeutic intervention.

PMID: 25686750 [PubMed - indexed for MEDLINE]

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Eficacia de las intervenciones para el tratamiento de la dislexia: una revisión

Publication date: Available online 14 January 2016
Source:Revista de Logopedia, Foniatría y Audiología
Author(s): Juan C. Ripoll Salceda, Gerardo Aguado Alonso
En este informe se revisa la investigación que respalda distintas intervenciones para el tratamiento de la dislexia, concretamente los trabajos de síntesis, en especial síntesis de las mejores evidencias, revisiones sistemáticas o metaanálisis publicados sobre cada tipo de intervención. En los casos en que no se ha publicado ningún trabajo de síntesis o estos trabajos son antiguos, se hace una revisión de la investigación primaria.Los trabajos localizados indican que los métodos fonológicos de intervención en la dislexia, es decir, aquellos en los que se combina el entrenamiento en habilidades fonológicas con el conocimiento de las letras y la práctica de la lectura, están ampliamente respaldados por la investigación. En cambio, la investigación sobre otros sistemas es sumamente escasa, ofrece resultados contradictorios o indica que se trata de sistemas ineficaces. Las intervenciones que están en estas situaciones son: la integración auditiva, el entrenamiento auditivo con soporte informático, la terapia visual, las lentes tintadas, el entrenamiento perceptivo-motor, las técnicas quiroprácticas, la integración sensorial, el método Davis, el neurofeedback, la musicoterapia y la educación musical o las dietas y suplementos alimenticios.A partir de esta revisión se realizan las siguientes recomendaciones: promocionar las intervenciones de tipo fonológico para el tratamiento de la dislexia, no utilizar o recomendar intervenciones que no estén respaldadas por la investigación científica, que los métodos de intervención que no hayan probado su eficacia no reciban el nombre de «terapia» o «tratamiento», y la creación y difusión de una guía sobre intervención en dislexia basada en evidencias científicas.In this report the research that supports various interventions for the treatment of dyslexia is revised, specifically the works of synthesis, especially synthesis of best evidence, systematic reviews or meta-analyses published about each type of intervention. A review of primary research is made in cases in which no synthesis work has been published or where these works are old.Localised works indicate that the phonological methods of intervention in dyslexia, i.e. those in which training in phonological skills is combined with knowledge of letters and the practice of reading, are widely supported by research. However, research on other methods is either extremely scarce or it offers conflicting results or it leads to the conclusion that they are inefficient methods. Interventions in this situation are: the auditory integration, the auditory training with computer support, visual therapy, the tinted lenses, motor-perceptual training, chiropractic techniques, sensory integration, the Davis method, neurofeedback, music therapy and music education or diets and dietary supplements.Promotion of phonological interventions is recommended; it is necessary to avoid recommendations of intervention methods for dyslexia which are not supported by scientific research; methods of intervention that have not proven their effectiveness may not be presented as therapies or treatments; and the creation and distribution of a guide on intervention in dyslexia based on scientific evidence is recommended.



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Vocal nodules in school age children

Publication date: Available online 15 January 2016
Source:Revista de Logopedia, Foniatría y Audiología
Author(s): Janete Coelho, Daniela Ramos, Isabel Monteiro, António Diogo Paiva
IntroductionVocal nodules are the most common organic and functional lesions among the paediatrics population, being the main cause of dysphonia during childhood. In Portugal, a few studies that describe the vocal parameters in school age children with this pathology were found. The objective of this paper is to analyse the perceptual and acoustic vocal parameters of school age children with vocal nodules and to compare them with a group of children without vocal nodules.MethodologyThe perceptual and acoustic parameters of 5 children, of both genders, aged from 7 years and 2 months to 12 years and 10 months, where clinical diagnosis of vocal nodules were analysed. The following assessment instruments were used: software Praat, GRBASI scale, maximum phonation time and s/z coefficient. The obtained data was compared to children without vocal nodules. The Mann–Whitney U test, with p<.05 significance level, in SPSS Statistics, version 22.0 was used for statistical analysis. The results are presented as mean and standard deviations.ResultsStatistically significant differences were registered between the group of vocal nodules vs. the group without vocal nodules, on the following parameters: fundamental frequency, shimmer, HNR, maximum phonation time for /a/ e /z/, s/z coefficient and GRBASI. On jitter and maximum phonation time for /s/ there were no statistically significant differences.Discussion and conclusionsThis study underlines the importance of assessing the perceptual and acoustic parameters in children with vocal nodules.



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Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review

10.3109/14992027.2015.1122237<br/>Wen Jiang

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Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review

10.3109/14992027.2015.1122237<br/>Wen Jiang

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Eficacia de las intervenciones para el tratamiento de la dislexia: una revisión

Publication date: Available online 14 January 2016
Source:Revista de Logopedia, Foniatría y Audiología
Author(s): Juan C. Ripoll Salceda, Gerardo Aguado Alonso
En este informe se revisa la investigación que respalda distintas intervenciones para el tratamiento de la dislexia, concretamente los trabajos de síntesis, en especial síntesis de las mejores evidencias, revisiones sistemáticas o metaanálisis publicados sobre cada tipo de intervención. En los casos en que no se ha publicado ningún trabajo de síntesis o estos trabajos son antiguos, se hace una revisión de la investigación primaria.Los trabajos localizados indican que los métodos fonológicos de intervención en la dislexia, es decir, aquellos en los que se combina el entrenamiento en habilidades fonológicas con el conocimiento de las letras y la práctica de la lectura, están ampliamente respaldados por la investigación. En cambio, la investigación sobre otros sistemas es sumamente escasa, ofrece resultados contradictorios o indica que se trata de sistemas ineficaces. Las intervenciones que están en estas situaciones son: la integración auditiva, el entrenamiento auditivo con soporte informático, la terapia visual, las lentes tintadas, el entrenamiento perceptivo-motor, las técnicas quiroprácticas, la integración sensorial, el método Davis, el neurofeedback, la musicoterapia y la educación musical o las dietas y suplementos alimenticios.A partir de esta revisión se realizan las siguientes recomendaciones: promocionar las intervenciones de tipo fonológico para el tratamiento de la dislexia, no utilizar o recomendar intervenciones que no estén respaldadas por la investigación científica, que los métodos de intervención que no hayan probado su eficacia no reciban el nombre de «terapia» o «tratamiento», y la creación y difusión de una guía sobre intervención en dislexia basada en evidencias científicas.In this report the research that supports various interventions for the treatment of dyslexia is revised, specifically the works of synthesis, especially synthesis of best evidence, systematic reviews or meta-analyses published about each type of intervention. A review of primary research is made in cases in which no synthesis work has been published or where these works are old.Localised works indicate that the phonological methods of intervention in dyslexia, i.e. those in which training in phonological skills is combined with knowledge of letters and the practice of reading, are widely supported by research. However, research on other methods is either extremely scarce or it offers conflicting results or it leads to the conclusion that they are inefficient methods. Interventions in this situation are: the auditory integration, the auditory training with computer support, visual therapy, the tinted lenses, motor-perceptual training, chiropractic techniques, sensory integration, the Davis method, neurofeedback, music therapy and music education or diets and dietary supplements.Promotion of phonological interventions is recommended; it is necessary to avoid recommendations of intervention methods for dyslexia which are not supported by scientific research; methods of intervention that have not proven their effectiveness may not be presented as therapies or treatments; and the creation and distribution of a guide on intervention in dyslexia based on scientific evidence is recommended.



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Vocal nodules in school age children

Publication date: Available online 15 January 2016
Source:Revista de Logopedia, Foniatría y Audiología
Author(s): Janete Coelho, Daniela Ramos, Isabel Monteiro, António Diogo Paiva
IntroductionVocal nodules are the most common organic and functional lesions among the paediatrics population, being the main cause of dysphonia during childhood. In Portugal, a few studies that describe the vocal parameters in school age children with this pathology were found. The objective of this paper is to analyse the perceptual and acoustic vocal parameters of school age children with vocal nodules and to compare them with a group of children without vocal nodules.MethodologyThe perceptual and acoustic parameters of 5 children, of both genders, aged from 7 years and 2 months to 12 years and 10 months, where clinical diagnosis of vocal nodules were analysed. The following assessment instruments were used: software Praat, GRBASI scale, maximum phonation time and s/z coefficient. The obtained data was compared to children without vocal nodules. The Mann–Whitney U test, with p<.05 significance level, in SPSS Statistics, version 22.0 was used for statistical analysis. The results are presented as mean and standard deviations.ResultsStatistically significant differences were registered between the group of vocal nodules vs. the group without vocal nodules, on the following parameters: fundamental frequency, shimmer, HNR, maximum phonation time for /a/ e /z/, s/z coefficient and GRBASI. On jitter and maximum phonation time for /s/ there were no statistically significant differences.Discussion and conclusionsThis study underlines the importance of assessing the perceptual and acoustic parameters in children with vocal nodules.



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Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review.

Related Articles

Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review.

Int J Audiol. 2016 Jan 15;:1-9

Authors: Jiang W, Zhao F, Guderley N, Manchaiah V

Abstract
OBJECTIVE: This systematic review aimed to explore the evidence on whether the preferred listening levels (PLLs) and durations of music listening through personal listening devices (PLDs) in adolescents and young adults exceed the current recommended 100% daily noise dose; together with the impact on hearing and possible influential factors of such listening behaviours.
DESIGN: A systematic search was conducted using multiple online bibliographic databases.
STUDY SAMPLE: The 26 studies were included on the basis of the inclusion and exclusion criteria.
RESULTS: The results showed that up to 58.2% of participants exceeded the 100% daily noise dose, particularly in the presence of background noise. Significantly positive correlations were found among background noise levels and mean PLLs, as well as the proportion of participants exceeding the 100% daily noise dose. Moreover, significantly worse hearing thresholds were found in PLD users using audiometry, and significantly poor results in otoacoustic emission (OAE), even in the participants with self-reported 'normal hearing'.
CONCLUSION: It is crucial to develop appropriate standards and safe recommendations for daily music exposure dose in future studies. Providing an essential guide and effective education to adolescents and young adults will help raise awareness, increase knowledge, and consequently change attitudes and listening habits.

PMID: 26768911 [PubMed - as supplied by publisher]

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Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review

10.3109/14992027.2015.1122237<br/>Wen Jiang

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[Frequency-specific analysis of the hVOR Prior of Cochlear Implant Operation].

Related Articles

[Frequency-specific analysis of the hVOR Prior of Cochlear Implant Operation].

Laryngorhinootologie. 2015 Mar;94(3):173-8

Authors: Hülse R, Hülse M, Wenzel A, Hörmann K, Servais JJ

Abstract
Dizziness is one of the most common postoperative complications after a cochlear-implant (CI) surgery. With our prospective, matched-paired controlled study, we could demonstrate that patients with distinctive sensorineural hearing loss--even without any complaints of dizziness--already have a reduced horizontal vestibular-ocular-reflex (hVOR). Compared to controls, CI patients presented with a significantly reduced gain. 9 out of 17 CI patients showed physiological results in rotatory testing and video head thrust testing. One patient presented with pathological results in both tests. Remarkably, there were 2 patients who presented with pathological head impulse testing but normal values in rotatory testing and 5 patients who showed normal gains in video head impulse testing but abnormal rotatory tests. These findings clearly show the importance of a differentiated, frequency-dependent pre-operative vestibular assessment including rotatory testing and video-head impulse testing. Additionally, only an accurate pre-operative vestibular testing allows evaluating possible post-operative dizziness related complications and should be documented precisely, also for forensic reasons. This is the key to differentiate post-operative dizziness from an pre-operatively existing vestibular disorder that possibly might not be clinically apparent by the time of testing.

PMID: 25265227 [PubMed - indexed for MEDLINE]

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Auditory intensity processing: Effect of MRI background noise

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Nicole Angenstein, Jörg Stadler, André Brechmann
Studies on active auditory intensity discrimination in humans showed equivocal results regarding the lateralization of processing. Whereas experiments with a moderate background found evidence for right lateralized processing of intensity, functional magnetic resonance imaging (fMRI) studies with background scanner noise suggest more left lateralized processing. With the present fMRI study, we compared the task dependent lateralization of intensity processing between a conventional continuous echo planar imaging (EPI) sequence with a loud background scanner noise and a fast low-angle shot (FLASH) sequence with a soft background scanner noise. To determine the lateralization of the processing, we employed the contralateral noise procedure. Linearly frequency modulated (FM) tones were presented monaurally with and without contralateral noise. During both the EPI and the FLASH measurement, the left auditory cortex was more strongly involved than the right auditory cortex while participants categorized the intensity of FM tones. This was shown by a strong effect of the additional contralateral noise on the activity in the left auditory cortex. This means a massive reduction in background scanner noise still leads to a significant left lateralized effect. This suggests that the reversed lateralization in fMRI studies with loud background noise in contrast to studies with softer background cannot be fully explained by the MRI background noise.



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Temperature Sensitive Auditory Neuropathy

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Qiujing Zhang, Lan Lan, Wei Shi, Lan Yu, Lin-yi Xie, Fen Xiong, Cui Zhao, Na Li, Zifang Yin, Liang Zong, Jing Guan, Dayong Wang, Wei Sun, Qiuju Wang
Temperature sensitive auditory neuropathy is a very rare and puzzling disorder. In the present study, we reported three unrelated 2 to 6 year-old children who were diagnosed as auditory neuropathy patients who complained of severe hearing loss when they had fever. Their hearing thresholds varied from the morning to the afternoon. Two of these patients’ hearing improved with age, and one patient received positive results from cochlear implant. Genetic analysis revealed that these three patients had otoferlin (OTOF) homozygous or compound heterozygous mutations with the genotypes c.2975_2978delAG/c.4819C>T, c.4819C>T/c.4819C>T, or c.2382_2383delC/c.1621G>A, respectively. Our study suggests that these gene mutations may be the cause of temperature sensitive auditory neuropathy. The long term follow up results suggest that the hearing loss in this type of auditory neuropathy may recover with age.



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

Related Articles

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

Int J Pediatr Otorhinolaryngol. 2015 Jul;79(7):1049-54

Authors: Alkhamra RA

Abstract
OBJECTIVE: Exploring the perspective of parents on the cochlear implant process in Jordan.
METHODS: Sixty parents of deaf children were surveyed on the information gathering process prior to cochlear implant surgery, and their implant outcome expectations post-surgery. Whether child or parent characteristics may impact parents' post-surgical expectations was explored.
RESULTS: Although parents used a variety of information sources when considering a cochlear implant, the ear, nose and throat doctor comprised their major source of information (60%). Parents received a range of information prior to cochlear implant but agreed (93.3%) on the need for a multidisciplinary team approach. Post-surgically, parents' expected major developments in the areas of spoken language (97%), and auditory skills (100%). Receiving education in mainstream schools (92%) was expected too. Parents perceived the cochlear implant decision as the best decision they can make for their child (98.3%). A significant correlation was found between parents contentment with the cochlear implant decision and expecting developments in the area of reading and writing (r=0.7). Child's age at implantation and age at hearing loss diagnosis significantly affected parents' post-implant outcome expectations (p<0.05).
CONCLUSION: Despite the general satisfaction from the information quantity and quality prior to cochlear implant, parents agree on the need for a comprehensive multidisciplinary team approach during the different stages of the cochlear implant process. Parents' education about cochlear implants prior to the surgery can affect their post-surgical outcome expectations. The parental perspective presented in this study can help professionals develop better understanding of parents' needs and expectations and henceforth improve their services and support during the different stages of the cochlear implant process.

PMID: 25990943 [PubMed - indexed for MEDLINE]

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Early auditory preverbal skills development in Mandarin speaking children with cochlear implants.

Related Articles

Early auditory preverbal skills development in Mandarin speaking children with cochlear implants.

Int J Pediatr Otorhinolaryngol. 2015 Jan;79(1):71-5

Authors: Liu H, Jin X, Li J, Liu L, Zhou Y, Zhang J, Ge W, Ni X

Abstract
OBJECTIVE: The purpose of this study was to investigate the development of auditory preverbal skills in Mandarin speaking infants/toddlers with cochlear implants (CIs).
METHODS: Participants were recruited from the Pediatric Audiology Center of Beijing Children's Hospital, Capital Medical University. A total of 33 children with severe-to-profound hearing loss who received CIs participated in the study. The evaluation tools were LittlEARS(®) Auditory Questionnaire (LEAQ) and self-designed demographic information questionnaire. Evaluations were administrated immediately after the CI was switched on (0-month), and at 1, 3, 6, 9, 12, 18, and 24-month intervals of CI use.
RESULTS: The mean total scores of the LEAQ in 0, 1, 3, 6, 9, 12, 18, and 24-month were 1, 5, 10, 15, 21, 24, 30, and 33 points, respectively. The developmental trajectory of early auditory preverbal skills in the CI children was consistent with the published norm data of the LEAQ, and the expected value even slightly higher than the norms. Analysis showed that the parents' level of education and age of implantation influenced the final LEAQ score significantly (ANOVA, p<0.0001).
CONCLUSIONS: Auditory preverbal skills improved dramatically after cochlear implantation in the first 2 years of implant use. Early implanted children exhibited a steeper and faster improvement in auditory preverbal developmental compared to the later implanted peers. This study described the developmental trajectories of preverbal auditory skills and confirmed the effectiveness of early implantation on the development of auditory preverbal skills. The results could provide guidance for auditory/speech rehabilitation in Mandarin speaking infants/toddlers who received CIs in their early age.

PMID: 25434480 [PubMed - indexed for MEDLINE]

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Auditory intensity processing: Effect of MRI background noise

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Nicole Angenstein, Jörg Stadler, André Brechmann
Studies on active auditory intensity discrimination in humans showed equivocal results regarding the lateralization of processing. Whereas experiments with a moderate background found evidence for right lateralized processing of intensity, functional magnetic resonance imaging (fMRI) studies with background scanner noise suggest more left lateralized processing. With the present fMRI study, we compared the task dependent lateralization of intensity processing between a conventional continuous echo planar imaging (EPI) sequence with a loud background scanner noise and a fast low-angle shot (FLASH) sequence with a soft background scanner noise. To determine the lateralization of the processing, we employed the contralateral noise procedure. Linearly frequency modulated (FM) tones were presented monaurally with and without contralateral noise. During both the EPI and the FLASH measurement, the left auditory cortex was more strongly involved than the right auditory cortex while participants categorized the intensity of FM tones. This was shown by a strong effect of the additional contralateral noise on the activity in the left auditory cortex. This means a massive reduction in background scanner noise still leads to a significant left lateralized effect. This suggests that the reversed lateralization in fMRI studies with loud background noise in contrast to studies with softer background cannot be fully explained by the MRI background noise.



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Temperature Sensitive Auditory Neuropathy

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Qiujing Zhang, Lan Lan, Wei Shi, Lan Yu, Lin-yi Xie, Fen Xiong, Cui Zhao, Na Li, Zifang Yin, Liang Zong, Jing Guan, Dayong Wang, Wei Sun, Qiuju Wang
Temperature sensitive auditory neuropathy is a very rare and puzzling disorder. In the present study, we reported three unrelated 2 to 6 year-old children who were diagnosed as auditory neuropathy patients who complained of severe hearing loss when they had fever. Their hearing thresholds varied from the morning to the afternoon. Two of these patients’ hearing improved with age, and one patient received positive results from cochlear implant. Genetic analysis revealed that these three patients had otoferlin (OTOF) homozygous or compound heterozygous mutations with the genotypes c.2975_2978delAG/c.4819C>T, c.4819C>T/c.4819C>T, or c.2382_2383delC/c.1621G>A, respectively. Our study suggests that these gene mutations may be the cause of temperature sensitive auditory neuropathy. The long term follow up results suggest that the hearing loss in this type of auditory neuropathy may recover with age.



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Prevalence and Audiological Profiles of GJB2 Mutations in a Large Collective of Hearing Impaired Patients

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): W.F. Burke, A. Warnecke, A. Schöner-Heinisch, A. Lesinski-Schiedat, H. Maier, T. Lenarz
Mutations in the GJB2 gene are known to represent the commonest cause of hereditary and congenital hearing loss. In this study, a complete sequencing of the GJB2 gene in a cohort of 506 patients from a single, large cochlear implant program in Europe was performed. Audiological testing for those patients who could actively participate was performed using pure tone audiometry (PTA). Those unable to undergo PTA were measured using click-auditory brainstem response (ABR). Data analysis was performed to determine genotype-phenotype correlations of the mutational status vs. audiological profiles and vs. age at the time of presentation. An overall prevalence of biallelic mutations of 13.4 % was found for the total collective. When subsets of younger patients were examined, the prevalence increased to 27 % of those up to age 18 and 35 % of those up to age 5 at the time of testing, respectively. This increase was found to be highly significant (p < 0.001). Analysis of the mean PTA thresholds revealed a strong correlation between allele combination status and mean PTA (p = 0.021). The prevalence of simple heterozygotes was found to be approximately 10.1 %, which is around 3.3 times the value expected in the general population. As GJB2 follows a recessive pattern of inheritance, the question arises as to why such a large fraction of simple heterozygotes was observed among the hearing impaired patients included in this study.

Graphical abstract

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Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review.

Related Articles

Daily music exposure dose and hearing problems using personal listening devices in adolescents and young adults: A systematic review.

Int J Audiol. 2016 Jan 15;:1-9

Authors: Jiang W, Zhao F, Guderley N, Manchaiah V

Abstract
OBJECTIVE: This systematic review aimed to explore the evidence on whether the preferred listening levels (PLLs) and durations of music listening through personal listening devices (PLDs) in adolescents and young adults exceed the current recommended 100% daily noise dose; together with the impact on hearing and possible influential factors of such listening behaviours.
DESIGN: A systematic search was conducted using multiple online bibliographic databases.
STUDY SAMPLE: The 26 studies were included on the basis of the inclusion and exclusion criteria.
RESULTS: The results showed that up to 58.2% of participants exceeded the 100% daily noise dose, particularly in the presence of background noise. Significantly positive correlations were found among background noise levels and mean PLLs, as well as the proportion of participants exceeding the 100% daily noise dose. Moreover, significantly worse hearing thresholds were found in PLD users using audiometry, and significantly poor results in otoacoustic emission (OAE), even in the participants with self-reported 'normal hearing'.
CONCLUSION: It is crucial to develop appropriate standards and safe recommendations for daily music exposure dose in future studies. Providing an essential guide and effective education to adolescents and young adults will help raise awareness, increase knowledge, and consequently change attitudes and listening habits.

PMID: 26768911 [PubMed - as supplied by publisher]

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Noise-Induced Neural Degeneration and Therapeutic Effect of Antioxidant Drugs.

Related Articles

Noise-Induced Neural Degeneration and Therapeutic Effect of Antioxidant Drugs.

J Audiol Otol. 2015 Dec;19(3):111-9

Authors: Choi SH, Choi CH

Abstract
The primary site of lesion induced by noise exposure is the hair cells in the organ of Corti and the primary neural degeneration occurs in synaptic terminals of cochlear nerve fibers and spiral ganglion cells. The cellular basis of noise-induced hearing loss is oxidative stress, which refers to a severe disruption in the balance between the production of free radicals and antioxidant defense system in the cochlea by excessive production of free radicals induced by noise exposure. Oxidative stress has been identified by a variety of biomarkers to label free radical activity which include four-hydroxy-2-nonenal, nitrotyrosine, and malondialdehyde, and inducible nitric oxide synthase, cytochrome-C, and cascade-3, 8, 9. Furthermore, oxidative stress is contributing to the necrotic and apoptotic cell deaths in the cochlea. To counteract the known mechanisms of pathogenesis and oxidative stress induced by noise exposure, a variety of antioxidant drugs including oxygen-based antioxidants such as N-acetyl-L-cystein and acetyl-L-carnitine and nitrone-based antioxidants such as phenyl-N-tert-butylnitrone (PBN), disufenton sodium, 4-hydroxy PBN, and 2, 4-disulfonyl PBN have been used in our laboratory. These antioxidant drugs were effective in preventing or treating noise-induced hearing loss. In combination with other antioxidants, antioxidant drugs showed a strong synergistic effect. Furthermore, successful use of antioxidant drugs depends on the optimal timing of treatment and the duration of treatment, which are highly related to the time window of free radical formation induced by noise exposure.

PMID: 26771008 [PubMed]

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Auditory nerve synapses persist in ventral cochlear nucleus long after loss of acoustic input in mice with early-onset progressive hearing loss.

Related Articles

Auditory nerve synapses persist in ventral cochlear nucleus long after loss of acoustic input in mice with early-onset progressive hearing loss.

Brain Res. 2015 Apr 24;1605:22-30

Authors: McGuire B, Fiorillo B, Ryugo DK, Lauer AM

Abstract
Perceptual performance in persons with hearing loss, especially those using devices to restore hearing, is not fully predicted by traditional audiometric measurements designed to evaluate the status of peripheral function. The integrity of auditory brainstem synapses may vary with different forms of hearing loss, and differential effects on the auditory nerve-brain interface may have particularly profound consequences for the transfer of sound from ear to brain. Loss of auditory nerve synapses in ventral cochlear nucleus (VCN) has been reported after acoustic trauma, ablation of the organ of Corti, and administration of ototoxic compounds. The effects of gradually acquired forms deafness on these synapses are less well understood. We investigated VCN gross morphology and auditory nerve synapse integrity in DBA/2J mice with early-onset progressive sensorineural hearing loss. Hearing status was confirmed using auditory brainstem response audiometry and acoustic startle responses. We found no change in VCN volume, number of macroneurons, or number of VGLUT1-positive auditory nerve terminals between young adult and older, deaf DBA/2J. Cell-type specific analysis revealed no difference in the number of VGLUT1 puncta contacting bushy and multipolar cell body profiles, but the terminals were smaller in deaf DBA/2J mice. Transmission electron microscopy confirmed the presence of numerous healthy, vesicle-filled auditory nerve synapses in older, deaf DBA/2J mice. The present results suggest that synapses can be preserved over a relatively long time-course in gradually acquired deafness. Elucidating the mechanisms supporting survival of central auditory nerve synapses in models of acquired deafness may reveal new opportunities for therapeutic intervention.

PMID: 25686750 [PubMed - indexed for MEDLINE]

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[Frequency-specific analysis of the hVOR Prior of Cochlear Implant Operation].

Related Articles

[Frequency-specific analysis of the hVOR Prior of Cochlear Implant Operation].

Laryngorhinootologie. 2015 Mar;94(3):173-8

Authors: Hülse R, Hülse M, Wenzel A, Hörmann K, Servais JJ

Abstract
Dizziness is one of the most common postoperative complications after a cochlear-implant (CI) surgery. With our prospective, matched-paired controlled study, we could demonstrate that patients with distinctive sensorineural hearing loss--even without any complaints of dizziness--already have a reduced horizontal vestibular-ocular-reflex (hVOR). Compared to controls, CI patients presented with a significantly reduced gain. 9 out of 17 CI patients showed physiological results in rotatory testing and video head thrust testing. One patient presented with pathological results in both tests. Remarkably, there were 2 patients who presented with pathological head impulse testing but normal values in rotatory testing and 5 patients who showed normal gains in video head impulse testing but abnormal rotatory tests. These findings clearly show the importance of a differentiated, frequency-dependent pre-operative vestibular assessment including rotatory testing and video-head impulse testing. Additionally, only an accurate pre-operative vestibular testing allows evaluating possible post-operative dizziness related complications and should be documented precisely, also for forensic reasons. This is the key to differentiate post-operative dizziness from an pre-operatively existing vestibular disorder that possibly might not be clinically apparent by the time of testing.

PMID: 25265227 [PubMed - indexed for MEDLINE]

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Prevalence and Audiological Profiles of GJB2 Mutations in a Large Collective of Hearing Impaired Patients

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): W.F. Burke, A. Warnecke, A. Schöner-Heinisch, A. Lesinski-Schiedat, H. Maier, T. Lenarz
Mutations in the GJB2 gene are known to represent the commonest cause of hereditary and congenital hearing loss. In this study, a complete sequencing of the GJB2 gene in a cohort of 506 patients from a single, large cochlear implant program in Europe was performed. Audiological testing for those patients who could actively participate was performed using pure tone audiometry (PTA). Those unable to undergo PTA were measured using click-auditory brainstem response (ABR). Data analysis was performed to determine genotype-phenotype correlations of the mutational status vs. audiological profiles and vs. age at the time of presentation. An overall prevalence of biallelic mutations of 13.4 % was found for the total collective. When subsets of younger patients were examined, the prevalence increased to 27 % of those up to age 18 and 35 % of those up to age 5 at the time of testing, respectively. This increase was found to be highly significant (p < 0.001). Analysis of the mean PTA thresholds revealed a strong correlation between allele combination status and mean PTA (p = 0.021). The prevalence of simple heterozygotes was found to be approximately 10.1 %, which is around 3.3 times the value expected in the general population. As GJB2 follows a recessive pattern of inheritance, the question arises as to why such a large fraction of simple heterozygotes was observed among the hearing impaired patients included in this study.

Graphical abstract

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Auditory intensity processing: Effect of MRI background noise

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Nicole Angenstein, Jörg Stadler, André Brechmann
Studies on active auditory intensity discrimination in humans showed equivocal results regarding the lateralization of processing. Whereas experiments with a moderate background found evidence for right lateralized processing of intensity, functional magnetic resonance imaging (fMRI) studies with background scanner noise suggest more left lateralized processing. With the present fMRI study, we compared the task dependent lateralization of intensity processing between a conventional continuous echo planar imaging (EPI) sequence with a loud background scanner noise and a fast low-angle shot (FLASH) sequence with a soft background scanner noise. To determine the lateralization of the processing, we employed the contralateral noise procedure. Linearly frequency modulated (FM) tones were presented monaurally with and without contralateral noise. During both the EPI and the FLASH measurement, the left auditory cortex was more strongly involved than the right auditory cortex while participants categorized the intensity of FM tones. This was shown by a strong effect of the additional contralateral noise on the activity in the left auditory cortex. This means a massive reduction in background scanner noise still leads to a significant left lateralized effect. This suggests that the reversed lateralization in fMRI studies with loud background noise in contrast to studies with softer background cannot be fully explained by the MRI background noise.



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Auditory intensity processing: Effect of MRI background noise

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Nicole Angenstein, Jörg Stadler, André Brechmann
Studies on active auditory intensity discrimination in humans showed equivocal results regarding the lateralization of processing. Whereas experiments with a moderate background found evidence for right lateralized processing of intensity, functional magnetic resonance imaging (fMRI) studies with background scanner noise suggest more left lateralized processing. With the present fMRI study, we compared the task dependent lateralization of intensity processing between a conventional continuous echo planar imaging (EPI) sequence with a loud background scanner noise and a fast low-angle shot (FLASH) sequence with a soft background scanner noise. To determine the lateralization of the processing, we employed the contralateral noise procedure. Linearly frequency modulated (FM) tones were presented monaurally with and without contralateral noise. During both the EPI and the FLASH measurement, the left auditory cortex was more strongly involved than the right auditory cortex while participants categorized the intensity of FM tones. This was shown by a strong effect of the additional contralateral noise on the activity in the left auditory cortex. This means a massive reduction in background scanner noise still leads to a significant left lateralized effect. This suggests that the reversed lateralization in fMRI studies with loud background noise in contrast to studies with softer background cannot be fully explained by the MRI background noise.



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Temperature Sensitive Auditory Neuropathy

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Qiujing Zhang, Lan Lan, Wei Shi, Lan Yu, Lin-yi Xie, Fen Xiong, Cui Zhao, Na Li, Zifang Yin, Liang Zong, Jing Guan, Dayong Wang, Wei Sun, Qiuju Wang
Temperature sensitive auditory neuropathy is a very rare and puzzling disorder. In the present study, we reported three unrelated 2 to 6 year-old children who were diagnosed as auditory neuropathy patients who complained of severe hearing loss when they had fever. Their hearing thresholds varied from the morning to the afternoon. Two of these patients’ hearing improved with age, and one patient received positive results from cochlear implant. Genetic analysis revealed that these three patients had otoferlin (OTOF) homozygous or compound heterozygous mutations with the genotypes c.2975_2978delAG/c.4819C>T, c.4819C>T/c.4819C>T, or c.2382_2383delC/c.1621G>A, respectively. Our study suggests that these gene mutations may be the cause of temperature sensitive auditory neuropathy. The long term follow up results suggest that the hearing loss in this type of auditory neuropathy may recover with age.



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Temperature Sensitive Auditory Neuropathy

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Qiujing Zhang, Lan Lan, Wei Shi, Lan Yu, Lin-yi Xie, Fen Xiong, Cui Zhao, Na Li, Zifang Yin, Liang Zong, Jing Guan, Dayong Wang, Wei Sun, Qiuju Wang
Temperature sensitive auditory neuropathy is a very rare and puzzling disorder. In the present study, we reported three unrelated 2 to 6 year-old children who were diagnosed as auditory neuropathy patients who complained of severe hearing loss when they had fever. Their hearing thresholds varied from the morning to the afternoon. Two of these patients’ hearing improved with age, and one patient received positive results from cochlear implant. Genetic analysis revealed that these three patients had otoferlin (OTOF) homozygous or compound heterozygous mutations with the genotypes c.2975_2978delAG/c.4819C>T, c.4819C>T/c.4819C>T, or c.2382_2383delC/c.1621G>A, respectively. Our study suggests that these gene mutations may be the cause of temperature sensitive auditory neuropathy. The long term follow up results suggest that the hearing loss in this type of auditory neuropathy may recover with age.



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Prevalence and Audiological Profiles of GJB2 Mutations in a Large Collective of Hearing Impaired Patients

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): W.F. Burke, A. Warnecke, A. Schöner-Heinisch, A. Lesinski-Schiedat, H. Maier, T. Lenarz
Mutations in the GJB2 gene are known to represent the commonest cause of hereditary and congenital hearing loss. In this study, a complete sequencing of the GJB2 gene in a cohort of 506 patients from a single, large cochlear implant program in Europe was performed. Audiological testing for those patients who could actively participate was performed using pure tone audiometry (PTA). Those unable to undergo PTA were measured using click-auditory brainstem response (ABR). Data analysis was performed to determine genotype-phenotype correlations of the mutational status vs. audiological profiles and vs. age at the time of presentation. An overall prevalence of biallelic mutations of 13.4 % was found for the total collective. When subsets of younger patients were examined, the prevalence increased to 27 % of those up to age 18 and 35 % of those up to age 5 at the time of testing, respectively. This increase was found to be highly significant (p < 0.001). Analysis of the mean PTA thresholds revealed a strong correlation between allele combination status and mean PTA (p = 0.021). The prevalence of simple heterozygotes was found to be approximately 10.1 %, which is around 3.3 times the value expected in the general population. As GJB2 follows a recessive pattern of inheritance, the question arises as to why such a large fraction of simple heterozygotes was observed among the hearing impaired patients included in this study.

Graphical abstract

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Auditory intensity processing: Effect of MRI background noise

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Nicole Angenstein, Jörg Stadler, André Brechmann
Studies on active auditory intensity discrimination in humans showed equivocal results regarding the lateralization of processing. Whereas experiments with a moderate background found evidence for right lateralized processing of intensity, functional magnetic resonance imaging (fMRI) studies with background scanner noise suggest more left lateralized processing. With the present fMRI study, we compared the task dependent lateralization of intensity processing between a conventional continuous echo planar imaging (EPI) sequence with a loud background scanner noise and a fast low-angle shot (FLASH) sequence with a soft background scanner noise. To determine the lateralization of the processing, we employed the contralateral noise procedure. Linearly frequency modulated (FM) tones were presented monaurally with and without contralateral noise. During both the EPI and the FLASH measurement, the left auditory cortex was more strongly involved than the right auditory cortex while participants categorized the intensity of FM tones. This was shown by a strong effect of the additional contralateral noise on the activity in the left auditory cortex. This means a massive reduction in background scanner noise still leads to a significant left lateralized effect. This suggests that the reversed lateralization in fMRI studies with loud background noise in contrast to studies with softer background cannot be fully explained by the MRI background noise.



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Temperature Sensitive Auditory Neuropathy

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): Qiujing Zhang, Lan Lan, Wei Shi, Lan Yu, Lin-yi Xie, Fen Xiong, Cui Zhao, Na Li, Zifang Yin, Liang Zong, Jing Guan, Dayong Wang, Wei Sun, Qiuju Wang
Temperature sensitive auditory neuropathy is a very rare and puzzling disorder. In the present study, we reported three unrelated 2 to 6 year-old children who were diagnosed as auditory neuropathy patients who complained of severe hearing loss when they had fever. Their hearing thresholds varied from the morning to the afternoon. Two of these patients’ hearing improved with age, and one patient received positive results from cochlear implant. Genetic analysis revealed that these three patients had otoferlin (OTOF) homozygous or compound heterozygous mutations with the genotypes c.2975_2978delAG/c.4819C>T, c.4819C>T/c.4819C>T, or c.2382_2383delC/c.1621G>A, respectively. Our study suggests that these gene mutations may be the cause of temperature sensitive auditory neuropathy. The long term follow up results suggest that the hearing loss in this type of auditory neuropathy may recover with age.



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Prevalence and Audiological Profiles of GJB2 Mutations in a Large Collective of Hearing Impaired Patients

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): W.F. Burke, A. Warnecke, A. Schöner-Heinisch, A. Lesinski-Schiedat, H. Maier, T. Lenarz
Mutations in the GJB2 gene are known to represent the commonest cause of hereditary and congenital hearing loss. In this study, a complete sequencing of the GJB2 gene in a cohort of 506 patients from a single, large cochlear implant program in Europe was performed. Audiological testing for those patients who could actively participate was performed using pure tone audiometry (PTA). Those unable to undergo PTA were measured using click-auditory brainstem response (ABR). Data analysis was performed to determine genotype-phenotype correlations of the mutational status vs. audiological profiles and vs. age at the time of presentation. An overall prevalence of biallelic mutations of 13.4 % was found for the total collective. When subsets of younger patients were examined, the prevalence increased to 27 % of those up to age 18 and 35 % of those up to age 5 at the time of testing, respectively. This increase was found to be highly significant (p < 0.001). Analysis of the mean PTA thresholds revealed a strong correlation between allele combination status and mean PTA (p = 0.021). The prevalence of simple heterozygotes was found to be approximately 10.1 %, which is around 3.3 times the value expected in the general population. As GJB2 follows a recessive pattern of inheritance, the question arises as to why such a large fraction of simple heterozygotes was observed among the hearing impaired patients included in this study.

Graphical abstract

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Prevalence and Audiological Profiles of GJB2 Mutations in a Large Collective of Hearing Impaired Patients

Publication date: Available online 15 January 2016
Source:Hearing Research
Author(s): W.F. Burke, A. Warnecke, A. Schöner-Heinisch, A. Lesinski-Schiedat, H. Maier, T. Lenarz
Mutations in the GJB2 gene are known to represent the commonest cause of hereditary and congenital hearing loss. In this study, a complete sequencing of the GJB2 gene in a cohort of 506 patients from a single, large cochlear implant program in Europe was performed. Audiological testing for those patients who could actively participate was performed using pure tone audiometry (PTA). Those unable to undergo PTA were measured using click-auditory brainstem response (ABR). Data analysis was performed to determine genotype-phenotype correlations of the mutational status vs. audiological profiles and vs. age at the time of presentation. An overall prevalence of biallelic mutations of 13.4 % was found for the total collective. When subsets of younger patients were examined, the prevalence increased to 27 % of those up to age 18 and 35 % of those up to age 5 at the time of testing, respectively. This increase was found to be highly significant (p < 0.001). Analysis of the mean PTA thresholds revealed a strong correlation between allele combination status and mean PTA (p = 0.021). The prevalence of simple heterozygotes was found to be approximately 10.1 %, which is around 3.3 times the value expected in the general population. As GJB2 follows a recessive pattern of inheritance, the question arises as to why such a large fraction of simple heterozygotes was observed among the hearing impaired patients included in this study.

Graphical abstract

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