Maria K Ho, Xin Li, Juemei Wang, Jeffrey D Ohmen, Rick A Friedman
{"title":"FVB/NJ mice demonstrate a youthful sensitivity to noise-induced hearing loss and provide a useful genetic model for the study of neural hearing loss.","authors":"Maria K Ho, Xin Li, Juemei Wang, Jeffrey D Ohmen, Rick A Friedman","doi":"10.1159/000357770","DOIUrl":"https://doi.org/10.1159/000357770","url":null,"abstract":"<p><p>The hybrid mouse diversity panel (HMDP), a panel of 100 strains, has been employed in genome wide association studies (GWAS) to study complex traits in mice. Hearing is a complex trait and the CBA/CaJ mouse strain is a widely used model for age-related hearing loss (ARHI) and noise induced hearing loss (NIHL). The CBA/CaJ strain's youthful sensitivity to noise and limited age-related loss led us to attempt to identify additional strains segregating a similar phenotype for our panel. FVB/NJ is part of the HMDP and has been previously described as having a similar ARHI phenotype to CBA/CaJ. For these reasons, we have studied the FVB/NJ mouse for ARHI and NIHL phenotypes in hopes of incorporating its phenotype into HMDP studies. We demonstrate that FVB/NJ exhibits ARHI at an earlier age than CBA/CaJ and young FVB/NJ mice are vulnerable to NIHL up until 10 to 12 weeks. This suggests that FVB/NJ may be used as an additional genetic model for neural forms of progressive hearing loss and for the study of youthful sensitivity to noise.</p>","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"4 1","pages":"1-11"},"PeriodicalIF":0.0,"publicationDate":"2014-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000357770","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32240715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C. Chiong, E. M. Cutiongco-de la Paz, M. R. Reyes-Quintos, C. A. Tobias, Karen Hernandez, R. Santos-Cortez
{"title":"GJB2 Variants and Auditory Outcomes among Filipino Cochlear Implantees","authors":"C. Chiong, E. M. Cutiongco-de la Paz, M. R. Reyes-Quintos, C. A. Tobias, Karen Hernandez, R. Santos-Cortez","doi":"10.1159/000346271","DOIUrl":"https://doi.org/10.1159/000346271","url":null,"abstract":"Objectives: To determine the prevalence of gap junction beta-2 (GJB2) or connexin 26 variants in Filipino cochlear implantees, and to describe radiologic findings, audiologic results and auditory performance after cochlear implantation (CI). Methods: Thirty cochlear implantees with unknown etiology of congenital severe-to-profound hearing loss and 30 controls provided venous blood samples for study. Radiologic evidence of temporal bone abnormalities, residual hearing status and post-CI auditory performance are described. Statistical analysis was performed for hearing thresholds before and after CI and for Parent Evaluation of Auditory/Oral Performance in Children (PEACH) scores based on the presence of cochleovestibular anomalies, age at CI and time since CI. Genomic DNA was extracted from venous blood, PCR-amplified and sequenced for GJB2 variants. Results: One patient was compound heterozygous (c.[35delG ];[235delC]) for GJB2 variants. The variants p.Gly4Asp, p.Val27Ile and p.Val37Ile were identified in both patients and controls, including 2 implantees who were homozygous for p.Val27Ile and p.Val37Ile. No significant association was found between post-CI improvement in threshold or PEACH scores and the following variables: age at CI, duration of hearing aid use prior to CI, presence of cochleovestibular anomalies and completeness of electrode insertion. Although no significant change in audiometric thresholds due to time since CI was detected, PEACH scores were significantly improved with longer implant use at all conditions (quiet, noise and overall; p < 0.05). Conclusions: The prevalence of causal GJB2 variants in Filipino cochlear implantees is low (3.3%). Within this population, the allele frequency of the p.Val37Ile variant in patients and controls is >10%, which supports a nonpathogenic role for this variant. The low prevalence of GJB2 variants precluded any association testing with CI outcome, although our results suggest better auditory outcome with longer CI use. Future genetic studies within the Filipino population should be able to control for population admixture.","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"3 1","pages":"1 - 8"},"PeriodicalIF":0.0,"publicationDate":"2013-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000346271","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"64634144","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C. Foster, Annand Ponnapan, Kathleen Zaccaro, Darcy Strong
{"title":"A Comparison of Two Home Exercises for Benign Positional Vertigo: Half Somersault versus Epley Maneuver","authors":"C. Foster, Annand Ponnapan, Kathleen Zaccaro, Darcy Strong","doi":"10.1159/000337947","DOIUrl":"https://doi.org/10.1159/000337947","url":null,"abstract":"Benign paroxysmal positional vertigo (BPPV) frequently recurs after treatment, so a home exercise would be desirable. We designed a self-administered exercise, the half somersault, for home use. In this randomized single-blind study, we compare the efficacy of our exercise to self-administered Epley maneuvers in patients with BPPV. Subjects performed exercises twice while observed, were re-tested with the Dix Hallpike, and then reported on exercise use for 6 months. Outcome measures were the reduction of nystagmus intensity, tolerability of induced dizziness, and long-term efficacy. Both exercises resulted in a significant reduction in nystagmus after two self-applications. The Epley maneuver was significantly more efficacious in reducing nystagmus initially, but caused significantly more dizziness during application than the half somersault. During the 6-month follow-up, the Epley group had significantly more treatment failures than the half somersault group. We believe that both exercises can be self-applied to control symptoms, but the half somersault is tolerated better and has fewer side effects as a home exercise.","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"2 1","pages":"16 - 23"},"PeriodicalIF":0.0,"publicationDate":"2012-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000337947","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"64586360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Mapping Sites on Bone and Soft Tissue of the Head, Neck and Thorax at Which a Bone Vibrator Elicits Auditory Sensation","authors":"Michael Kaufmann, C. Adelman, H. Sohmer","doi":"10.1159/000336159","DOIUrl":"https://doi.org/10.1159/000336159","url":null,"abstract":"This study was designed to map the sites on the skin of the head, neck and thorax at which a clinical bone vibrator elicits auditory sensation. In 10 subjects with normal hearing, a bone vibrator delivering a 2000-Hz warble tone, at an intensity which was at least 5 dB below the intensity which elicited a sensation by air conduction over each site, was applied to 25 sites with an application force of 500 g. Auditory sensations were elicited at many soft tissue (underlying bone >1 cm below the skin) and osseous (bone <0.5 cm below the skin) sites on the head, neck and thorax in all subjects, down to the sternum and thoracic vertebrae. Therefore, auditory vibrations induced at many sites on the head, neck and thorax can reach the cochlea and elicit auditory sensation.","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"2 1","pages":"9 - 15"},"PeriodicalIF":0.0,"publicationDate":"2012-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000336159","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"64572871","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. S. Durante, Stefanie Mariano, Mariana de Souza Pires, P. Pachi
{"title":"School-Age Outcomes in Preterm Children Born with Risk Factors for Hearing Loss: Contralateral Suppression of Transient Evoked Otoacoustic Emissions","authors":"A. S. Durante, Stefanie Mariano, Mariana de Souza Pires, P. Pachi","doi":"10.1159/000335462","DOIUrl":"https://doi.org/10.1159/000335462","url":null,"abstract":"We investigated the effect of contralateral suppression of linear transient evoked otoacoustic emissions (TEOAEs) in 16 preterm children born with risk factors for hearing loss. We evaluated those children, together with a control group of term children, at 8–10 years of age. The magnitude of the TEOAE suppression effect in both ears was significantly smaller in the preterm children than in the term children (p < 0.05). There is increasing evidence of adverse school-age outcomes (including hearing loss) in preterm children. Such outcomes can interfere with the school performance of those infants. The TEOAE suppression effect seems to be a promising early marker of hearing outcomes in preterm infants.","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"2 1","pages":"1 - 8"},"PeriodicalIF":0.0,"publicationDate":"2012-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000335462","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"64567343","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Performance of Toddlers, Children and Young Adults Using Unilateral or Bilateral Cochlear Implants on a Left versus Right Loudspeaker Identification Task","authors":"K. Galvin, K. Hughes, J. Holland, Mansze Mok","doi":"10.1159/000335170","DOIUrl":"https://doi.org/10.1159/000335170","url":null,"abstract":"Thirty-six participants with bilateral cochlear implants aged 1–19 years completed a left versus right loudspeaker identification task. The majority performed at chance in the unilateral condition (n = 24) and significantly above chance in the bilateral condition (n = 28). Cluster analysis identified three groups; one group performed above chance in both conditions and was older at second implant and older at testing, with longer delay between implants. There were no such differences between the group performing at chance in both conditions and the group which scored highly in the bilateral condition only, thus demonstrating a bilateral benefit. Unilateral, but not bilateral, scores were correlated with age at second and at first implant, time between implants, and age at testing. Bilateral benefit was negatively correlated with age at second implant, time between implants and age at testing. Co-linearity made it difficult to isolate the relationships with demographic factors, though age at testing may have had the most influence on unilateral scores. Spatial hearing skills with bilateral implants cannot be predicted for individuals.","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"1 1","pages":"30 - 44"},"PeriodicalIF":0.0,"publicationDate":"2011-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000335170","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"64565157","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S. Marlin, L. Jonard, N. Loundon, C. Bonnet, N. Leboulanger, L. Van Maldergem, S. Gherbi, M. Louha, P. Deltenre, J. Collette, R. Couderc, E. Garabédian, F. Denoyelle
{"title":"Genetic Update on Auditory Neuropathy","authors":"S. Marlin, L. Jonard, N. Loundon, C. Bonnet, N. Leboulanger, L. Van Maldergem, S. Gherbi, M. Louha, P. Deltenre, J. Collette, R. Couderc, E. Garabédian, F. Denoyelle","doi":"10.1159/000329545","DOIUrl":"https://doi.org/10.1159/000329545","url":null,"abstract":"Auditory neuropathy (AN) is defined as a sensorineural hearing impairment (HI) characterized by preserved cochlear outer hair cell function with normal otoacoustic emissions but abnormal or absent brainstem evoked potentials. The prevalence of AN in HIs remains to be established and its pathophysiology has not been determined. Several environmental and genetic causes have been identified, but the majority of cases remain unexplained. In the last 10 years, many genes have been identified in isolated and/or syndromic HI. Some of them (OTOF, PJVK, DIAPH3, OPA1, FXN, PMP22, ERCC6, ERCC8, SLC19A2 and TIMM8A, for example) are responsible for phenotypes corresponding to the AN definition. This review will focus on isolated and syndromic forms of AN for which the causative genes have been identified.","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"1 1","pages":"20 - 29"},"PeriodicalIF":0.0,"publicationDate":"2011-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000329545","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"64538966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Comparison of Clinical Characteristics and Somatic Modulation between Somatic Tinnitus and Otic Tinnitus","authors":"Yong-Hwi An, A. Choi, S. Yoon, H. Shim","doi":"10.1159/000332048","DOIUrl":"https://doi.org/10.1159/000332048","url":null,"abstract":"Aims: To compare the clinical characteristics of tinnitus among patients with somatic tinnitus (ST), otic tinnitus with somatic modulation (OT+) and otic tinnitus without somatic modulation (OT–), and to examine differences in somatic modulation between patients with ST and OT+. Methods: We retrospectively reviewed the medical records of 65 patients with unilateral tinnitus and classified the patients into three groups: ST (n = 24), OT+ (n = 21) and OT– (n = 20). Results: Only one difference in clinical characteristics was found between the OT+ and OT– groups. Maneuvers related to the temporomandibular joint showed the highest frequency of somatic modulation in both the ST and OT+ groups. There was no significant difference between the ST and OT+ groups with regard to the rate of somatic modulation or the rate of increase or decrease of tinnitus among all aspects of somatic testing. Conclusions: The presence or absence of somatic modulation does not influence the clinical characteristics of otic tinnitus, and the somatosensory-auditory interaction is a general auditory physiologic phenomenon.","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"1 1","pages":"9 - 19"},"PeriodicalIF":0.0,"publicationDate":"2011-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000332048","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"64551238","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Effect of Transient Ischemic Attack on Hearing Thresholds of Older Subjects","authors":"J.H. Hwang, H. C. Ho, M. Hsu, J. Chen","doi":"10.1159/000329795","DOIUrl":"https://doi.org/10.1159/000329795","url":null,"abstract":"The effects of transient ischemic attack (TIA) on pure tone average of low frequencies (PTA-low), of middle frequencies (PTA-mid), and of high frequencies (PTA-high), and audiometric configurations, which were classified by cluster analysis, were analyzed in older subjects. TIA showed significant positive association with PTA-high [β coefficient (β) ± standard error (SE) = 8.63 ± 2.81, p = 0.002] after adjusting for age, sex, hypertension, and coronary artery disease. Besides, multinomial logistic regression for audiogram patterns showed that the moderate 8-kHz dip pattern (β ± SE = 1.70 ± 0.78, p = 0.029) was significantly more prevalent in the TIA group after adjusting for age, sex, and mean hearing level at all frequencies. We concluded that TIA without an obvious infarct lesion could contribute to hearing degeneration in older subjects, especially in high frequencies.","PeriodicalId":90109,"journal":{"name":"Audiology and neurotology. Extra","volume":"1 1","pages":"1 - 8"},"PeriodicalIF":0.0,"publicationDate":"2011-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000329795","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"64540456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}