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Sample records for nonsyndromic hearing loss

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

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

    2018-02-01

    Usher syndrome is rare genetic disorder impairing two human senses, hearing and vision, with the characteristic late onset of vision loss. This syndrome is divided into three types. In all cases, the vision loss is postlingual, while loss of hearing is usually prelingual. The vestibular functions may also be disturbed in Usher type 1 and sometimes in type 3. Vestibular areflexia is helpful in making a proper diagnosis of the syndrome, but, often, the syndrome is misdiagnosed as a nonsyndromic hearing loss. Here, we present a Polish family with hearing loss, which was clinically classified as nonsyndromic. After excluding mutations in the DFNB1 locus, we implemented the next-generation sequencing method and revealed that hearing loss was syndromic and mutations in the USH2A gene indicate Usher syndrome. This research highlights the importance of molecular analysis in establishing a clinical diagnosis of congenital hearing loss.

  2. Three novel GJB2 (connexin 26) variants associated with autosomal dominant syndromic and nonsyndromic hearing loss.

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    DeMille, Desiree; Carlston, Colleen M; Tam, Oliver H; Palumbos, Janice C; Stalker, Heather J; Mao, Rong; Zori, Roberto T; Viskochil, David H; Park, Albert H; Carey, John C

    2018-04-01

    Connexin 26 (Cx26), encoded by the GJB2 gene, is a key protein involved in the formation of gap junctions in epithelial organs including the inner ear and palmoplantar epidermis. Pathogenic variants in GJB2 are responsible for approximately 50% of inherited sensorineural deafness. The majority of these variants are associated with autosomal recessive inheritance; however, rare reports of dominantly co-segregating variants have been published. Since we began offering GJB2 testing in 2003, only about 2% of detected GJB2 variants from our laboratory have been classified as dominant. Here we report three novel dominant GJB2 variants (p.Thr55Ala, p.Gln57_Pro58delinsHisSer, and p.Trp44Gly); two associated with syndromic sensorineural hearing loss and one with nonsyndromic hearing loss. In the kindred with the p.Thr55Ala variant, the proband and his father present with only leukonychia as a cutaneous finding of their syndromic hearing loss. This phenotype has been previously documented in conjunction with palmoplantar hyperkeratosis, but isolated leukonychia is a novel finding likely associated with the unique threonine to alanine change at codon 55 (other variants at this codon have been reported in cases of nonsyndromic hearing loss). This report contributes to the short list of GJB2 variants associated with autosomal dominant hearing loss, highlights the variability of skin and nail findings associated with such cases, and illustrates the occurrence of both syndromic and nonsyndromic presentations with changes in the same gene. © 2018 Wiley Periodicals, Inc.

  3. Novel compound heterozygous MYO7A mutations in Moroccan families with autosomal recessive non-syndromic hearing loss.

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    Amina Bakhchane

    Full Text Available The MYO7A gene encodes a protein belonging to the unconventional myosin super family. Mutations within MYO7A can lead to either non syndromic hearing loss or to the Usher syndrome type 1B (USH1B. Here, we report the results of genetic analyses performed on Moroccan families with autosomal recessive non syndromic hearing loss that identified two families with compound heterozygous MYO7A mutations. Five mutations (c.6025delG, c.6229T>A, c.3500T>A, c.5617C>T and c.4487C>A were identified in these families, the latter presenting two differently affected branches. Multiple bioinformatics programs and molecular modelling predicted the pathogenic effect of these mutations. In conclusion, the absence of vestibular and retinal symptom in the affected patients suggests that these families have the isolated non-syndromic hearing loss DFNB2 (nonsyndromic autosomal recessive hearing loss presentation, instead of USH1B.

  4. Prevalence of GJB2 Mutations in Affected Individuals from United Arab Emirates with Autosomal Recessive Nonsyndromic Hearing Loss.

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    Tlili, Abdelaziz; Al Mutery, Abdullah; Kamal Eddine Ahmad Mohamed, Walaa; Mahfood, Mona; Hadj Kacem, Hassen

    2017-11-01

    Mutations in the gap junction protein beta 2 (GJB2) gene are responsible for more cases of nonsyndromic recessive hearing loss than any other gene. The purpose of our study was to evaluate the prevalence of GJB2 mutations among affected individuals from United Arab Emirates (UAE). There were 50 individuals diagnosed with hereditary hearing loss and 120 healthy individuals enrolled in the study. The Sanger sequencing method was used to screen the GJB2 coding region in all affected individuals. The c.-1G>A variant was determined by the polymerase chain reaction-restriction fragment length polymorphism method in normal individuals. Nine cases with bi-allelic mutations and three cases with mono-allelic mutations were detected in 12 out of 50 patients (24%). The homozygous mutation c.35delG was identified as the cause of hearing loss in six participants (12%). The mutation c.506G>A was identified in three affected individuals (6%). The allelic frequency (14%) and low percentage of individuals that were homozygous (2%) for the c.35delG mutation suggest that there are other genes responsible for nonsyndromic deafness in the UAE population. The results reported here are a preliminary step in collecting epidemiological data regarding autosomal recessive nonsyndromic hearing loss related to GJB2 gene mutations among the UAE population. The c.35delG mutation of the GJB2 gene is the most frequently seen causative mutation in the UAE and is followed by the p.Cys169Tyr mutation.

  5. Nonsyndromic Hearing Loss Caused by USH1G Mutations: Widening the USH1G Disease Spectrum

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    Oonk, A.M.M.; Huet, R.A.C. van; Leijendeckers, J.M.; Oostrik, J.; Venselaar, H.; WIjk, E. van; Beynon, A.J.; Kunst, H.P.M.; Hoyng, C.B.; Kremer, H.; Schraders, M.; Pennings, R.J.E.

    2015-01-01

    OBJECTIVE: Currently, six genes are known to be associated with Usher syndrome type I, and mutations in most of these genes can also cause nonsyndromic hearing loss. The one exception is USH1G, which is currently only known to be involved in Usher syndrome type I and atypical Usher syndrome. DESIGN:

  6. Mutation analysis of SLC26A4 for Pendred syndrome and nonsyndromic hearing loss by high-resolution melting

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    Chen, Neng; Tranebjærg, Lisbeth; Rendtorff, Nanna Dahl

    2011-01-01

    Pendred syndrome and DFNB4 (autosomal recessive nonsyndromic congenital deafness, locus 4) are associated with autosomal recessive congenital sensorineural hearing loss and mutations in the SLC26A4 gene. Extensive allelic heterogeneity, however, necessitates analysis of all exons and splice sites...

  7. Pendred syndrome (goitre and sensorineural hearing loss) maps to chromosome 7 in the region containing the nonsyndromic deafness gene DFNB4.

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    Coyle, B; Coffey, R; Armour, J A; Gausden, E; Hochberg, Z; Grossman, A; Britton, K; Pembrey, M; Reardon, W; Trembath, R

    1996-04-01

    Inherited causes account for about 50% of individuals presenting with childhood (prelingual) hearing loss, of which 70% are due to mutation in numerous single genes which impair auditory function alone (non-syndromic). The remainder are associated with other developmental anomalies termed syndromic deafness. Genes responsible for syndromic forms of hearing loss include the COL4A5 gene in Alport syndrome and the PAX3 and MITF genes in Waardenburg syndrome. Pendred syndrome is an autosomal recessive disorder associated with developmental abnormalities of the cochlea, sensorineural hearing loss and diffuse thyroid enlargement (goitre). Pendred syndrome is the most common syndromal form of deafness, yet the primary defect remains unknown. We have established a panel of 12 families with two or more affected individuals and used them to search for the location of the Pendred gene by linkage analysis. We excluded localization to four previously mapped nonsyndromic deafness loci but obtained conclusive evidence for linkage of the Pendred syndrome gene to microsatellite markers on chromosome 7q31 (D7S495 Zmax 7.32, Qmax = 0). This region contains a gene, DFNBL, for autosomal recessive non-syndromic sensorineural hearing loss. Multipoint analysis indicates that DFNB4 and Pendred syndrome co-localize to the same 5.5 centiMorgan (cM) interval flanked by D7S501 and D7S523. These data raise the possibility that Pendred syndrome is either allelic with DFNB4 or may represent an inherited contiguous gene disorder, not clinically manifest in the heterozygote.

  8. [Connexin gene 26 (GJB2) mutations in patients with hereditary non-syndromic sensorineural loss of hearing in the Republic of Sakha (Yakutia)].

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    Barashkov, N A; Dzhemileva, L U; Fedorova, S A; Maksimova, N R; Khusnutdinova, E K

    2008-01-01

    The aim of the study was to elucidate the causes of hereditary non-syndromic loss of hearing, a frequent monogene pathology in the Republic of Sakha (Yakutia). A search for mutations in the coding sequence of the connexin 26 gene gap-junction B2 (GJB2) was undertaken in 79 members of 65 unrelated families with the diagnosis of grade III-IV non-syndromic bilateral sensorineural loss of hearing. Five recessive mutations (35delG, V371, 312-326del14, 333-334delAA, R127H) and three polymorphic variants (V271, M34T, E114G) were identified in Yakut patients. Mutations 35delG (41.7%), 312-326dell4 (4.2%), and 333-334delAA (4.2%) were found in Caucasian patients (Russians, Ukrainians, Inguish). Yakuts were carriers of mutations 35delG (2.1%), V371 (2.1%), R127H (1.0%) and sequence variants V271 (6.3%), M34T (1.0%), E114G (1.0%). GJB2 mutations were identified in 50.1% of the Caucasian patients and in 7.2% of the Yakut patients. The low frequency of GJB2 mutations in Yakuts with non-syndromic sensorineural loss of hearing testifies to the presence of mutations of other genes controlling sound perception in this population.

  9. New polymorphic mtDNA restriction site in the 12S rRNA gene detected in Tunisian patients with non-syndromic hearing loss

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    Mkaouar-Rebai, Emna; Tlili, Abdelaziz; Masmoudi, Saber; Charfeddine, Ilhem; Fakhfakh, Faiza

    2008-01-01

    The 12S rRNA gene was shown to be a hot spot for aminoglycoside-induced and non-syndromic hearing loss since several deafness-associated mtDNA mutations were identified in this gene. Among them, we distinguished the A1555G, the C1494T and the T1095C mutations and C-insertion or deletion at position 961. One hundred Tunisian patients with non-syndromic hearing loss and 100 hearing individuals were analysed in this study. A PCR-RFLP analysis with HaeIII restriction enzyme showed the presence of the A1555G mutation in the 12S rRNA gene in only one out of the 100 patients. In addition, PCR-RFLP and radioactive PCR revealed the presence of a new HaeIII polymorphic restriction site in the same gene of 12S rRNA site in 4 patients with non-syndromic hearing loss. UVIDOC-008-XD analyses showed the presence of this new polymorphic restriction site with a variable heteroplasmic rates at position +1517 of the human mitochondrial genome. On the other hand, direct sequencing of the entire mitochondrial 12S rRNA gene in the 100 patients and in 100 hearing individuals revealed the presence of the A750G and A1438G polymorphisms and the absence of the C1494T, T1095C and 961insC mutations in all the tested individuals. Sequencing of the whole mitochondrial genome in the 4 patients showing the new HaeIII polymorphic restriction site revealed only the presence of the A8860G transition in the MT-ATP6 gene and the A4769G polymorphism in the ND2 gene

  10. Comprehensive molecular etiology analysis of nonsyndromic hearing impairment from typical areas in China

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    Kang Dongyang

    2009-09-01

    Full Text Available Abstract Background Every year, 30,000 babies are born with congenital hearing impairment in China. The molecular etiology of hearing impairment in the Chinese population has not been investigated thoroughly. To provide appropriate genetic testing and counseling to families, we performed a comprehensive investigation of the molecular etiology of nonsyndromic deafness in two typical areas from northern and southern China. Methods A total of 284 unrelated school children with hearing loss who attended special education schools in China were enrolled in this study, 134 from Chifeng City in Inner Mongolia and the remaining 150 from Nangtong City in JiangSu Province. Screening was performed for GJB2, GJB3, GJB6, SLC26A4, 12S rRNA, and tRNAser(UCN genes in this population. All patients with SLC26A4 mutations or variants were subjected to high-resolution temporal bone CT scan to verify the enlarged vestibular aqueduct. Results Mutations in the GJB2 gene accounted for 18.31% of the patients with nonsyndromic hearing loss, 1555A>G mutation in mitochondrial DNA accounted for 1.76%, and SLC26A4 mutations accounted for 13.73%. Almost 50% of the patients with nonsyndromic hearing loss in these typical Chinese areas carried GJB2 or SLC26A4 mutations. No significant differences in mutation spectrum or prevalence of GJB2 and SLC26A4 were found between the two areas. Conclusion In this Chinese population, 54.93% of cases with hearing loss were related to genetic factors. The GJB2 gene accounted for the etiology in about 18.31% of the patients with hearing loss, SLC26A4 accounted for about 13.73%, and mtDNA 1555A>G mutation accounted for 1.76%. Mutations in GJB3, GJB6, and mtDNA tRNAser(UCN were not common in this Chinese cohort. Conventionally, screening is performed for GJB2, SLC26A4, and mitochondrial 12S rRNA in the Chinese deaf population.

  11. The R245X mutation of PCDH15 in Ashkenazi Jewish children diagnosed with nonsyndromic hearing loss foreshadows retinitis pigmentosa.

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    Brownstein, Zippora; Ben-Yosef, Tamar; Dagan, Orit; Frydman, Moshe; Abeliovich, Dvorah; Sagi, Michal; Abraham, Fabian A; Taitelbaum-Swead, Riki; Shohat, Mordechai; Hildesheimer, Minka; Friedman, Thomas B; Avraham, Karen B

    2004-06-01

    Usher syndrome is a frequent cause of the combination of deafness and blindness due to retinitis pigmentosa (RP). Five genes are known to underlie different forms of Usher syndrome type I (USH1). In the Ashkenazi Jewish population, the R245X mutation of the PCDH15 gene may be the most common cause of USH1 (Ben-Yosef T, Ness SL, Madeo AC, Bar-Lev A, Wolfman JH, Ahmed ZM, Desnick RK, Willner JP, Avraham KB, Ostrer H, Oddoux C, Griffith AJ, Friedman TB N Engl J Med 348: 1664-1670, 2003). To estimate what percentage of Ashkenazi Jewish children born with profound hearing loss will develop RP due to R245X, we examined the prevalence of the R245X PCDH15 mutation and its carrier rate among Ashkenazi Jews in Israel. Among probands diagnosed with nonsyndromic hearing loss not due to mutations of connexin 26 (GJB2) and/or connexin 30 (GJB6), and below the age of 10, 2 of 20 (10%) were homozygous for the R245X mutation. Among older nonsyndromic deaf individuals, no homozygotes were detected, although one individual was heterozygous for R245X. The carrier rate of the R245X mutation among the normal hearing Ashkenazi population in Israel was estimated at 1%. Ashkenazi Jewish children with profound prelingual hearing loss should be evaluated for the R245X PCDH15 mutation and undergo ophthalmologic evaluation to determine whether they will develop RP. Rehabilitation can then begin before loss of vision. Early use of cochlear implants in such cases may rescue these individuals from a dual neurosensory deficit.

  12. Clinical and molecular analysis of a four-generation Chinese family with aminoglycoside-induced and nonsyndromic hearing loss associated with the mitochondrial 12S rRNA C1494T mutation

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    Wang Qiuju; Li Qingzhong; Han Dongyi; Zhao Yali; Zhao Lidong; Qian Yaping; Yuan Hu; Li Ronghua; Zhai Suoqiang; Young Wieyen; Guan Minxin

    2006-01-01

    We report here the clinical, genetic, and molecular characterization of a four-generation Chinese family with aminoglycoside-induced and nonsyndromic hearing loss. Five of nine matrilineal relatives had aminoglycoside-induced hearing loss. These matrilineal relatives exhibited variable severity and audiometric configuration of hearing impairment, despite sharing some common features: being bilateral and having sensorineural hearing impairment. Sequence analysis of mitochondrial DNA (mtDNA) in the pedigree identified 16 variants and the homoplasmic 12S rRNA C1494T mutation, which was associated with hearing loss in the other large Chinese family. In fact, the occurrence of the C1494T mutation in these genetically unrelated pedigrees affected by hearing impairment strongly indicated that this mutation is involved in the pathogenesis of aminoglycoside-induced and nonsyndromic hearing loss. However, incomplete penetrance of hearing loss indicated that the C1494T mutation itself is not sufficient to produce a clinical phenotype but requires the involvement of modifier factors for the phenotypic expression. Those mtDNA variants, showing no evolutional conservation, may not have a potential modifying role in the pathogenesis of the C1494T mutation. However, nuclear background seems to contribute to the phenotypic variability of matrilineal relatives in this family. Furthermore, aminoglycosides modulate the expressivity and penetrance of deafness associated with the C1494T mutation in this family

  13. An overview of hereditary hearing loss.

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    Bayazit, Yildirim A; Yilmaz, Metin

    2006-01-01

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

  14. [Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia].

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    Dzhemileva, L U; Posukh, O L; Tazetdinov, A M; Barashkov, N A; Zhuravskiĭ, S G; Ponidelko, S N; Markova, T G; Tadinova, V N; Fedorova, S A; Maksimova, N R; Khusnutdinova, E K

    2009-07-01

    Mitochondrial DNA (mtDNA) mutations play an important role in etiology of hereditary hearing loss. In various regions of the world, patients suffer from nonsyndromic sensorineural hearing loss initiated by aminoglycoside antibiotics. Mutations that had been shown as pathogenetically important for hearing function disturbance were identified in mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes while pathogenic role of several DNA sequences requires additional studies. This work presents the results of studying the spectrum of mutations and polymorphic variations in mtDNA genes 12S rRNA and tRNA(Ser(UGN)) in 410 patients with nonsyndromal sensoneural hearing impairment/loss from the Volga Ural region, St Petersburg, Yakutia, and Altai and in 520 individuals with normal hearing, which represent several ethnic groups (Russians, Tatars, Bashkirs, Yakuts, Altaians) residing in the Russian Federation. Pathogenetically significant mutation A1555G (12S rRNA) was found in two families (from Yakutia and St Peresburg) with hearing loss, probably caused by treatment with aminoglucosides, and in the population sample of Yakuts with a frequency of 0.83%. Further research is needed to confirm the role in hearing impairment of mutations 961insC, 961insC(n), 961delTinsC(n), T961G, T1095C (12S rRNA) and G7444A, A7445C (tRNA(Ser(UGN revealed in the patients. In addition, in the patients and the population groups, polymorphic mt DNA variants were detected, which are characteristic also of other Eurasian populations both in spectrum and frequency.

  15. [Research progress of mutational spectrum and pathophysiology of WFS1 gene in Wolfram syndrome and nonsyndromic low frequency sensorineural hearing loss].

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    Shi, S M; Han, Y H; Wang, H B

    2016-09-07

    Compound homozygous or heterozygous mutations in WFS 1 can lead to autosomal recessive Wolfram syndrome (WS), and heterozygous mutations in WFS 1 can lead to autosomal dominant non-syndromic low frequency sensorineural hearing loss (LFSNHL). In addition, mutations in the WFS region has relationship with diabetes and psychiatric diseases. In this paper, we provide an overview of genetic research with different phenotypes, including WS and LFSNHL.

  16. Clinical evaluation and mitochondrial DNA sequence analysis in two Chinese families with aminoglycoside-induced and non-syndromic hearing loss

    International Nuclear Information System (INIS)

    Zhao Lidong; Wang Qiuju; Qian Yaping; Li Ronghua; Cao Juayng; Hart, Laura Christine; Zhai Suoqiang; Han Dongyi; Young Wieyen; Guan Minxin

    2005-01-01

    We report here the clinical, genetic, and molecular characterization of two Chinese pedigrees with aminoglycoside-induced and non-syndromic hearing impairment. Clinical evaluation revealed the variable phenotype of hearing impairment including audiometric configuration in these subjects. Penetrances of hearing loss in BJ105 and BJ106 pedigrees are 67% and 33%, respectively. In particular, three of 10 affected matrilineal relatives of BJ105 pedigree had aminoglycoside-induced hearing loss, while seven affected matrilineal relatives in BJ105 pedigree and six affected matrilineal relatives in BJ106 pedigree did not have a history of exposure to aminoglycosides. Sequence analysis of the complete mitochondrial genomes in these pedigrees showed the identical homoplasmic A1555G mutation and distinct sets of mtDNA variants belonging to haplogroups F3 and M7b. These variants showed no evolutionary conservation, implying that mitochondrial haplotype may not play a significant role in the phenotypic expression of the A1555G mutation in these Chinese pedigrees. However, aminoglycosides and nuclear backgrounds appear to be major modifier factors for the phenotypic manifestation of the A1555G mutation in these Chinese families

  17. Single Nucleotide Polymorphisms of the GJB2 and GJB6 Genes Are Associated with Autosomal Recessive Nonsyndromic Hearing Loss

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    Ana Paula Grillo

    2015-01-01

    Full Text Available Single nucleotide polymorphisms (SNPs are important markers in many studies that link DNA sequence variations to phenotypic changes; such studies are expected to advance the understanding of human physiology and elucidate the molecular basis of diseases. The DFNB1 locus, which contains the GJB2 and GJB6 genes, plays a key role in nonsyndromic hearing loss. Previous studies have identified important mutations in this locus, but the contribution of SNPs in the genes has not yet been much investigated. The aim of this study was to investigate the association of nine polymorphisms located within the DFNB1 locus with the occurrence of autosomal recessive nonsyndromic hearing loss (ARNSHL. The SNPs rs3751385 (C/T, rs7994748 (C/T, rs7329857 (C/T, rs7987302 (G/A, rs7322538 (G/A, rs9315400 (C/T, rs877098 (C/T, rs945369 (A/C, and rs7333214 (T/G were genotyped in 122 deaf patients and 132 healthy controls using allele-specific PCR. There were statistically significant differences between patients and controls, in terms of allelic frequencies in the SNPs rs3751385, rs7994748, rs7329857, rs7987302, rs945369, and rs7333214 (P<0.05. No significant differences between the two groups were observed for rs7322538, rs9315400, and rs877098. Our results suggest that SNPs present in the GJB2 and GJB6 genes may have an influence on ARNSHL in humans.

  18. [Application of MALDI-TOF-MS in gene testing for non-syndromic hearing loss].

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    Zeng, Yun; Jiang, Dan; Feng, Da-fei; Jin, Dong-dong; Wu, Xiao-hui; Ding, Yan-li; Zou, Jing

    2013-12-01

    To investigate the feasibility of Matrix-Assisted Laser Desorption-Ionization Time of Flight Mass Spectrometry (MALDI-TOF-MS) , according to the genetic test of non-syndromic hearing loss (NSHL), and check using the direct sequencing. Peripheral blood was collected from 454 NSHL patients. DNA samples were extracted and 20 loci of the four common disease-causing genes were analysed by MALDI-TOF-MS, including GJB2 (35delG, 167delT, 176_191del16, 235delC, 299_300delAT ), GJB3 (538C→T, 547G→A), SLC26A4 (281C→T, 589G→A, IVS7-2A→G, 1174A→T, 1226G→A, 1229C→T, IVS15+5G→A, 1975G→C, 2027T→A, 2162C→T, 2168A→G), and mitochondrial 12S rRNA (1494C→T, 1555A→G). Direct sequencing was also used to analyse the aforementioned 20 loci in order to validate the accuracy of MALDI-TOF-MS. Among the 454 patients, 166 cases (36.56%) of disease-causing mutations were detected, which included 69 cases (21.15%) of GJB2 gene mutation, four cases (0.88%) of GJB3 gene mutation, 64 cases (14.10%) of SLC26A4 gene mutation, and three cases (0.66%) of mitochondrial 12S rRNA gene mutation. Moreover, the results obtained from direct sequencing and MALDI-TOF-MS were consistent, and the results showed that the two methods were consistent. The MALDI-TOF-MS detection method was designed based on the hearing loss-related mutation hotspots seen in the Chinese population, and it has a high detection rate for NSHL related mutations. In comparison to the conventional detection methods, MALDI-TOF-MS has the following advantages: more detection sites, greater coverage, accurate, high throughput and low cost. Therefore, this method is capable of satisfying the needs of clinical detection for hearing impairment and it is suitable for large-scale implementation.

  19. Non-syndromic hearing loss caused by the dominant cis mutation R75Q with the recessive mutation V37I of the GJB2 (Connexin 26) gene.

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    Kim, Juwon; Jung, Jinsei; Lee, Min Goo; Choi, Jae Young; Lee, Kyung-A

    2015-06-19

    GJB2 alleles containing two cis mutations have been rarely found in non-syndromic hearing loss. Herein, we present a Korean patient with non-syndromic hearing loss caused by the R75Q cis mutation with V37I, which arose de novo in the father and was inherited by the patient. Biochemical coupling and hemichannel permeability assays were performed after molecular cloning and transfection of HEK293T cells. Student's t-tests or analysis of variance followed by Tukey's multiple comparison test was used as statistical analysis. Biochemical coupling was significantly reduced in connexin 26 (Cx26)-R75Q- and Cx26-V37I-transfected cells, with greater extent in Cx26-R75Q and Cx26-R75Q+V37I cells. Interestingly, our patient and his father with the mutations had more residual hearing compared with patients with the dominant mutation alone. Although the difference in hemichannel activity between R75Q alone and R75Q in combination with V37I failed to reach significance, it is of note that there is a possibility that V37I located upstream of R75Q might have the ability to ameliorate R75Q expression. Our study emphasizes the importance of cis mutations with R75Q, as the gene effect of R75Q can be modulated depending on the type of additional mutation.

  20. Molecular Investigation of Pediatric Portuguese Patients with Sensorineural Hearing Loss

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    Célia Nogueira

    2011-01-01

    Full Text Available The understanding of the molecular genetics in sensorineural hearing loss (SNHL has advanced rapidly during the last decade, but the molecular etiology of hearing impairment in the Portuguese population has not been investigated thoroughly. To provide appropriate genetic testing and counseling to families, we analyzed the whole mitochondrial genome in 95 unrelated children with SNHL (53 nonsyndromic and 42 syndromic and searched for variations in two frequent genes, GJB2 and GJB6, in the non-syndromic patients. Mutations in mtDNA were detected in 4.2% of the cases, including a hitherto undescribed change in the mtDNA-tRNATrp gene (namely, m.5558A>G. We also identified mono- or biallelic GJB2 mutations in 20 of 53 non-syndromic cases and also detected two novel mutations (p.P70R and p.R127QfsX84. Our data further reinforce the notion that genetic heterogeneity is paramount in children with SNHL.

  1. Centre-level variation in outcomes and treatment for otitis media with effusion and hearing loss and the association of hearing loss with developmental outcomes at ages 5 and 7 years in children with non-syndromic unilateral cleft lip and palate: The Cleft Care UK study. Part 2.

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    Hall, A; Wills, A K; Mahmoud, O; Sell, D; Waylen, A; Grewal, S; Sandy, J R; Ness, A R

    2017-06-01

    To explore centre-level variation in otitis media with effusion (OME), hearing loss and treatments in children in Cleft Care UK (CCUK) and to examine the association between OME, hearing loss and developmental outcomes at 5 and 7 years. Two hundred and sixty-eight 5-year-old British children with non-syndromic unilateral cleft lip and palate (UCLP) recruited to CCUK. Children had air and bone conduction audiometry at age 5. Information on grommet and hearing aid treatment was obtained from parental questionnaire and medical notes. Hearing loss at age 5 was defined as >20 dB in the better ear and history of OME and hearing loss was determined from past treatment. Children with sensorineural hearing loss were excluded. Associations were examined with speech, behaviour and self-confidence at age 5 and educational attainment at age 7. Centre variation was examined using hierarchical models and associations between hearing variables and developmental outcomes were examined using logistic regression. There was centre-level variation in early grommet placement (variance partition coefficient (VPC) 18%, P=.001) and fitting of hearing aids (VPC 8%, P=.03). A history of OME and hearing loss was associated with poor intelligibility of speech (adjusted odds ratio=2.87, 95% CI 1.42-5.77) and aspects of educational attainment. Hearing loss is an important determinant of poor speech and treatment variation across centres suggest management of OME and hearing loss could be improved. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  2. AUDIOME: a tiered exome sequencing-based comprehensive gene panel for the diagnosis of heterogeneous nonsyndromic sensorineural hearing loss.

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    Guan, Qiaoning; Balciuniene, Jorune; Cao, Kajia; Fan, Zhiqian; Biswas, Sawona; Wilkens, Alisha; Gallo, Daniel J; Bedoukian, Emma; Tarpinian, Jennifer; Jayaraman, Pushkala; Sarmady, Mahdi; Dulik, Matthew; Santani, Avni; Spinner, Nancy; Abou Tayoun, Ahmad N; Krantz, Ian D; Conlin, Laura K; Luo, Minjie

    2018-03-29

    PurposeHereditary hearing loss is highly heterogeneous. To keep up with rapidly emerging disease-causing genes, we developed the AUDIOME test for nonsyndromic hearing loss (NSHL) using an exome sequencing (ES) platform and targeted analysis for the curated genes.MethodsA tiered strategy was implemented for this test. Tier 1 includes combined Sanger and targeted deletion analyses of the two most common NSHL genes and two mitochondrial genes. Nondiagnostic tier 1 cases are subjected to ES and array followed by targeted analysis of the remaining AUDIOME genes.ResultsES resulted in good coverage of the selected genes with 98.24% of targeted bases at >15 ×. A fill-in strategy was developed for the poorly covered regions, which generally fell within GC-rich or highly homologous regions. Prospective testing of 33 patients with NSHL revealed a diagnosis in 11 (33%) and a possible diagnosis in 8 cases (24.2%). Among those, 10 individuals had variants in tier 1 genes. The ES data in the remaining nondiagnostic cases are readily available for further analysis.ConclusionThe tiered and ES-based test provides an efficient and cost-effective diagnostic strategy for NSHL, with the potential to reflex to full exome to identify causal changes outside of the AUDIOME test.Genetics in Medicine advance online publication, 29 March 2018; doi:10.1038/gim.2018.48.

  3. A novel frameshift mutation of SMPX causes a rare form of X-linked nonsyndromic hearing loss in a Chinese family.

    Directory of Open Access Journals (Sweden)

    Zhijie Niu

    Full Text Available X-linked hearing impairment is the rarest form of genetic hearing loss (HL and represents only a minor fraction of all cases. The aim of this study was to investigate the cause of X-linked inherited sensorineural HL in a four-generation Chinese family. A novel duplication variant (c.217dupA, p.Ile73Asnfs*5 in SMPX was identified by whole-exome sequencing. The frameshift mutation predicted to result in the premature truncation of the SMPX protein was co-segregated with the HL phenotype and was absent in 295 normal controls. Subpopulation screening of the coding exons and flanking introns of SMPX was further performed for 338 Chinese patients with nonsydromic HL by Sanger sequencing, and another two potential causative substitutions (c.238C>A and c.55A>G in SMPX were identified in additional sporadic cases of congenital deafness. Collectively, this study is the first to report the role of SMPX in Chinese population and identify a novel frameshift mutation in SMPX that causes not only nonsyndromic late-onset progressive HL, but also congenital hearing impairment. Our findings extend the mutation and phenotypic spectrum of the SMPX gene.

  4. Genetic Linkage Analysis of DFNB2 Locus with Autosomal Recessive Hearing Loss in Families Negative for GJB2 Mutations in Khuzestan Province

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    Parisa Tahmasebi

    2016-09-01

    Full Text Available Abstract Background: Hearing loss is a common sensory impairment in humans which half of its causes are genetic reasons. Genetic hearing loss can be divided into the two types of syndromic and non-syndromic, which 80% of non-syndromic cases is Autosomal Recessive Non-Syndromic Hearing Loss. The aim of the present research is to determine the contribution of DFNB2 locus (MYO7A gene in causing an autosomal recessive hearing loss in the one group of the deaf families of Khuzestan province. Materials and Methods: This study was conducted on 26 families with autosomal recessive hearing loss (with 4 patients and negative for GJB2 mutations in Khuzestan province. 22 families suffered from ARNSHL and 4 families suffered from Usher syndrome. Linkage analysis was performed by using STR (Short Tandem Repeat markers related to DFNB2 locus. Each family’s genotype was determined by PCR-PAGE method. Furthermore, haplotypes drawing and LOD score calculations were performed. Results: From 26 families with hearing loss participating in this research, following genetic linkage analysis and haplotypes drawing, two families (7.7% of the families showed linkage to DFNB2 locus. One family (4.5% suffered from ARNSHL and another family suffered from Usher syndrome. Conclusion: The results of the present research show that the contribution of DFNB2 locus in causing hearing loss in the population of Khuzestan province was similar to other studies conducted in Iran and this locus with other important loci should be considered to check in the hearing loss panel.

  5. Mutations in the Wolfram syndrome 1 gene (WFS1) are a common cause of low frequency sensorineural hearing loss.

    NARCIS (Netherlands)

    Bespalova, I.N.; Camp, G. van; Bom, S.J.H.; Brown, D.J.; Cryns, K.; Wan, A.T. de; Erson, A.E.; Flothmann, K.; Kunst, H.P.M.; Kurnool, P.; Sivakumaran, T.A.; Cremers, C.W.R.J.; Leal, S.M.; Burmeister, M.; Lesperance, M.M.

    2001-01-01

    Non-syndromic low frequency sensorineural hearing loss (LFSNHL) affecting only 2000 Hz and below is an unusual type of hearing loss that worsens over time without progressing to profound deafness. This type of LFSNHL may be associated with mild tinnitus but is not associated with vertigo. We have

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

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    Masoud Motasaddi Zarandy

    2011-01-01

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

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

    International Nuclear Information System (INIS)

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

    2015-01-01

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

  8. A new nonsyndromic X-linked sensorineural hearing impairment linked to Xp21.2

    Energy Technology Data Exchange (ETDEWEB)

    Lalwani, A.K.; Brister, J.R.; Fex, J.; Grundfast, K.M.; Pikus, A.T.; Ploplis, B.; San Agustin, T.; Skarka, H.; Wilcox, E.R. [National Institutes of Health, Bethesda, MD (United States)

    1994-10-01

    X-linked deafness is a rare cause of hereditary hearing impairment. We have identified a family with X-linked dominant sensorineural hearing impairment, characterized by incomplete penetrance and variable expressivity in carrier females, that is linked to the Xp21.2, which contains the Duchenne muscular dystrophy (DMD) locus. The auditory impairment in affected males was congenital, bilateral, profound, sensorineural, affecting all frequencies, and without evidence of radiographic abnormality of the temporal bone. Adult carrier females manifested bilateral, mild-to-moderate high-frequency sensorineural hearing impairment of delayed onset during adulthood. Eighteen commercially available polymorphic markers from the X chromosome, generating a 10-15-cM map, were initially used for identification of a candidate region. DXS997, located within the DMD gene, generated a two-point LOD score of 2.91 at {theta} = 0, with every carrier mother heterozygous at this locus. Recombination events at DXS992 (located within the DMD locus, 3{prime} to exon 50 of the dystrophin gene) and at DXS1068 (5{prime} to the brain promoter of the dystrophin gene) were observed. No recombination events were noted with the following markers within the DMD locus: 5{prime}DYS II, intron 44, DXS997, and intron 50. There was no clinical evidence of Duchenne or Becker muscular dystrophy in any family member. It is likely that this family represents a new locus on the X chromosome, which when mutated results in nonsyndromic sensorineural hearing loss and is distinct from the heterogeneous group of X-linked hearing losses that have been previously described. 57 refs., 6 figs., 1 tab.

  9. Genetic Linkage Analysis of the DFNB21 Locus in Autosomal Recessive Hearing Loss in Large Families from Khuzestan Province

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    Mahtab Khosrofar

    2017-06-01

    Full Text Available Abstract Background: Hearing loss (HL is the most common congenital defect in humans. One or two in thousand newborn babies have prelingual hearing loss. Autosomal recessive non-syndromic hearing loss (ARNSHL is the most common form of hereditary deafness. Hearing loss is more common in the developing countries which is due to genetic and environmental (cultural -health factors reasons. HL has a wide range of clinical demonstrations including: congenital or late onset, conductive or sensory-neural, syndromic or non-syndromic hearing loss. The goal of this project is to determine the portion of the DFNB21 (TECTA in ARNSHL in families with negative GJB2 gene in Khuzestan province. Materials and Methods: We studied 21 families with ARNSHL with at least 4 patients and negative for GJB2 mutations from Khuzestan province. Genetic linkage analysis was performed using STR markers linked to DFNB21 locus. Results: Following genetic linkage analysis and haplotyping, out of 21 families with ARNSHL, one family showed linkage to the DFNB21 (TECTA locus. Conclusion: The results of this project confirm other studies in Iran and give insight into the most common loci causing ARNSHL in Iran which could be helpful in research and clinic.

  10. Finding new genes for non-syndromic hearing loss through an in silico prioritization study.

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    Matteo Accetturo

    Full Text Available At present, 51 genes are already known to be responsible for Non-Syndromic hereditary Hearing Loss (NSHL, but the knowledge of 121 NSHL-linked chromosomal regions brings to the hypothesis that a number of disease genes have still to be uncovered. To help scientists to find new NSHL genes, we built a gene-scoring system, integrating Gene Ontology, NCBI Gene and Map Viewer databases, which prioritizes the candidate genes according to their probability to cause NSHL. We defined a set of candidates and measured their functional similarity with respect to the disease gene set, computing a score ( S S M avg that relies on the assumption that functionally related genes might contribute to the same (disease phenotype. A Kolmogorov-Smirnov test, comparing the pair-wise distribution on the disease gene set with the distribution on the remaining human genes, provided a statistical assessment of this assumption. We found at a p-value 0.99. The twenty top-scored genes were finally examined to evaluate their possible involvement in NSHL. We found that half of them are known to be expressed in human inner ear or cochlea and are mainly involved in remodeling and organization of actin formation and maintenance of the cilia and the endocochlear potential. These findings strongly indicate that our metric was able to suggest excellent NSHL candidates to be screened in patients and controls for causative mutations.

  11. A novel KCNQ4 one-base deletion in a large pedigree with hearing loss: implication for the genotype-phenotype correlation.

    Science.gov (United States)

    Kamada, Fumiaki; Kure, Shigeo; Kudo, Takayuki; Suzuki, Yoichi; Oshima, Takeshi; Ichinohe, Akiko; Kojima, Kanako; Niihori, Tetsuya; Kanno, Junko; Narumi, Yoko; Narisawa, Ayumi; Kato, Kumi; Aoki, Yoko; Ikeda, Katsuhisa; Kobayashi, Toshimitsu; Matsubara, Yoichi

    2006-01-01

    Autosomal-dominant, nonsyndromic hearing impairment is clinically and genetically heterogeneous. We encountered a large Japanese pedigree in which nonsyndromic hearing loss was inherited in an autosomal-dominant fashion. A genome-wide linkage study indicated linkage to the DFNA2 locus on chromosome 1p34. Mutational analysis of KCNQ4 encoding a potassium channel revealed a novel one-base deletion in exon 1, c.211delC, which generated a profoundly truncated protein without transmembrane domains (p.Q71fsX138). Previously, six missense mutations and one 13-base deletion, c.211_223del, had been reported in KCNQ4. Patients with the KCNQ4 missense mutations had younger-onset and more profound hearing loss than patients with the 211_223del mutation. In our current study, 12 individuals with the c.211delC mutation manifested late-onset and pure high-frequency hearing loss. Our results support the genotype-phenotype correlation that the KCNQ4 deletions are associated with later-onset and milder hearing impairment than the missense mutations. The phenotypic difference may be caused by the difference in pathogenic mechanisms: haploinsufficiency in deletions and dominant-negative effect in missense mutations.

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

    Science.gov (United States)

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

    2011-01-01

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

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

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    Yanfei Wang

    2017-12-01

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

  14. A large scale hearing loss screen reveals an extensive unexplored genetic landscape for auditory dysfunction

    DEFF Research Database (Denmark)

    Bowl, Michael R.; Simon, Michelle M.; Ingham, Neil J.

    2017-01-01

    The developmental and physiological complexity of the auditory system is likely reflected in the underlying set of genes involved in auditory function. In humans, over 150 non-syndromic loci have been identified, and there are more than 400 human genetic syndromes with a hearing loss component. O...

  15. Identification of a novel homozygous mutation in MYO3A in a Chinese family with DFNB30 non-syndromic hearing impairment.

    Science.gov (United States)

    Qu, Ronggui; Sang, Qing; Xu, Yao; Feng, Ruizhi; Jin, Li; He, Lin; Wang, Lei

    2016-05-01

    Hearing loss is a common sensory impairment. Several genetic loci or genes responsible for non-syndrome hearing loss have been identified, including the well-known deafness genes GJB2, MT-RNR1 and SLC26A4. MYO3A belongs to the myosin superfamily. Previously only three mutations in this gene have been found in an Isreali family with DFNB30, in which patients demonstrated progressive hearing loss. In this study, we characterized a consanguineous Kazakh family with congenital hearing loss. By targeted sequence capture and next-generation sequencing, we identified a homozygous mutation and did bioinformatics analysis to this mutation. A homozygous mutation, MYO3A:c.1841C>T (p.S614F), was identified to be responsible for the disease. Ser614 is located in the motor domain of MYO3A that is highly conserved among different species. Molecular modeling predicts that the conserved Ser614 may play an important role in maintaining the stability of β-sheet and the interaction between neighboring β-strand. This is the second report on MYO3A mutations in deafness and the first report in China. The finding help facilitate establishing a better relationship between MYO3A mutation and hearing phenotypes. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  16. Aminoglycoside-induced and non-syndromic hearing loss is associated with the G7444A mutation in the mitochondrial COI/tRNASer(UCN) genes in two Chinese families

    International Nuclear Information System (INIS)

    Zhu Yi; Qian Yaping; Tang Xiaowen; Wang Jindan; Yang Li; Liao Zhisu; Li Ronghua; Ji Jinzhang; Li Zhiyuan; Chen Jianfu; Choo, Daniel I.; Lu Jianxin; Guan Minxin

    2006-01-01

    We report here the clinical, genetic, and molecular characterization of two Chinese families with aminoglycoside induced and non-syndromic hearing impairment. Clinical and genetic evaluations revealed the variable severity and age-of-onset in hearing impairment in these families. Strikingly, there were extremely low penetrances of hearing impairment in these Chinese families. Sequence analysis of the complete mitochondrial genomes in these pedigrees showed the distinct sets of mtDNA polymorphism, in addition to the identical G7444A mutation associated with hearing loss. Indeed, the G7444A mutation in the CO1 gene and the precursor of tRNA Ser(UCN) gene is present in homoplasmy only in the maternal lineage of those pedigrees but not other members of these families and 164 Chinese controls. Their mitochondrial genomes belong to the Eastern Asian haplogroups C5a and D4a, respectively. In fact, the occurrence of the G7444A mutation in these several genetically unrelated subjects affected by hearing impairment strongly indicates that this mutation is involved in the pathogenesis of hearing impairment. However, there was the absence of other functionally significant mtDNA mutations in two Chinese pedigrees carrying the G7444A mutation. Therefore, nuclear modifier gene(s) or aminoglycoside(s) may play a role in the phenotypic expression of the deafness-associated G7444A mutation in these Chinese pedigrees

  17. Advances in the Understanding of the Genetic Causes of Hearing Loss in Children Inform a Rational Approach to Evaluation.

    Science.gov (United States)

    Carey, John C; Palumbos, Janice C

    2016-10-01

    Hearing loss represents the most common sensory disability of children. Remarkable advances in the identification of genes underlying nonsyndromic and syndromic hearing loss in just the last 2 decades have led to the ability to determine the specific genetic cause of hearing loss in many children. Surprisingly one gene, GJB2, encoding the protein connexin-26, accounts for about 20 % of sensorineural hearing loss (including in India) and is considered the first tier test in evaluating an infant with unexplained congenital hearing loss. Using the knowledge of the etiology of hearing loss, the authors propose a diagnostic reasoning process for the assessment of a child in the pediatric setting. Second tier testing consists of the multiple gene panels using whole exome sequencing strategies, and is becoming available in some regions of the world including the US. Referral to medical genetics is always a consideration in a child with no explanation for the hearing loss and in families with questions about recurrence risk.

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

    Science.gov (United States)

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

    2009-04-01

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

  19. Genetics Home Reference: nonsyndromic hearing loss

    Science.gov (United States)

    ... Centre for Genetics Education (Australia) Disease InfoSearch: Deafness Harvard Medical School Center for Hereditary Deafness Hereditary Hearing ... Available from http://www.ncbi.nlm.nih.gov/books/NBK1434/ Citation on ... Bulletins Genetics Home Reference Celebrates Its 15th Anniversary ...

  20. Hearing loss

    Science.gov (United States)

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

  1. Concomitant imaging and genetic findings in children with unilateral sensorineural hearing loss.

    Science.gov (United States)

    Gruber, M; Brown, C; Mahadevan, M; Neeff, M

    2017-08-01

    To describe the concomitant imaging and genetic findings in children diagnosed with non-syndromic unilateral sensorineural hearing loss. A retrospective cohort study was conducted of 60 children diagnosed between January 2005 and December 2015 in a tertiary-level paediatric institution. Average age at diagnosis was 4.3 years. All children were considered non-syndromic. Hearing loss was categorised as mild (17 children), moderate (17 children), severe (7 children) or profound (19 children). Imaging was performed in 43 children (71.66 per cent). Nineteen patients (44.2 per cent) had positive computed tomography or magnetic resonance imaging findings. Genetic testing was performed in 51 children (85 per cent). Sixteen children (31 per cent) tested positive to connexin 26 (GJB2); 1 patient (2 per cent) had a homozygous mutation of GJB2 and 15 were heterozygous carriers. Amongst children who tested positive as heterozygous carriers of a GJB2 mutation, there was a high rate of positive imaging findings (47 per cent compared to 37.2 per cent in the total cohort). A genetic abnormality was confirmed in 50 per cent of children with positive imaging findings who underwent genetic testing. Rates of concomitant imaging and genetic findings suggest that both investigations are of value in the study of these patients.

  2. Localization of A Novel Autosomal Recessive Non-Syndromic Hearing Impairment Locus (DFNB38) to 6q26–q27 in a Consanguineous Kindred from Pakistan

    OpenAIRE

    Ansar, Muhammad; Ramzan, Mohammad; Pham, Thanh L.; Yan, Kai; Jamal, Syed Muhammad; Haque, Sayedul; Ahmad, Wasim; Leal, Suzanne M.

    2003-01-01

    For autosomal recessive nonsyndromic hearing impairment over 30 loci have been mapped and 19 genes have been identified. DFNB38, a novel locus for autosomal recessive nonsyndromic hearing impairment, was localized in a consanguineous Pakistani kindred to 6q26–q27. The affected family members present with profound prelingual sensorineural hearing impairment and use sign language for communications. Linkage was established to microsatellite markers located on chromosome 6q26–q27 (Multipoint lod...

  3. Allelic mutations of KITLG, encoding KIT ligand, cause asymmetric and unilateral hearing loss and Waardenburg syndrome type 2

    NARCIS (Netherlands)

    Zazo Seco, C. (Celia); Serrão De Castro, L. (Luciana); J.W.I. van Nierop; Morín, M. (Matías); S.N. Jhangiani (Shalini N.); E.J.J. Verver (Eva J. J.); M. Schraders (Margit); Maiwald, N. (Nadine); Wesdorp, M. (Mieke); H. Venselaar (Hanka); L. Spruijt (Liesbeth); Oostrik, J. (Jaap); J. Schoots (Jeroen); J. van Reeuwijk (Jeroen); Lelieveld, S.H. (Stefan H.); P.L.M. Huygen (Patrick); Insenser, M. (María); R.J. Admiraal (Ronald); R.J.E. Pennings (Ronald J.E.); E.H. Hoefsloot (Lies); A. Arias-Vásquez (Alejandro); J. de Ligt (Joep); H.G. Yntema; Jansen, J.H. (Joop H.); D. Muzny (Donna); G. Huls (Gerwin); M.M. van Rossum (Michelle); J.R. Lupski (James R.); Moreno-Pelayo, M.A. (Miguel Angel); H.P.M. Kunst (Henricus P.M.); H. Kremer (Hannie)

    2015-01-01

    textabstractLinkage analysis combined with whole-exome sequencing in a large family with congenital and stable non-syndromic unilateral and asymmetric hearing loss (NS-UHL/AHL) revealed a heterozygous truncating mutation, c.286-303delinsT (p.Ser96Ter), in KITLG. This mutation co-segregated with

  4. Genetic spectrum of autosomal recessive non-syndromic hearing loss in Pakistani families.

    Directory of Open Access Journals (Sweden)

    Sobia Shafique

    Full Text Available The frequency of inherited bilateral autosomal recessive non-syndromic hearing loss (ARNSHL in Pakistan is 1.6/1000 individuals. More than 50% of the families carry mutations in GJB2 while mutations in MYO15A account for about 5% of recessive deafness. In the present study a cohort of 30 ARNSHL families was initially screened for mutations in GJB2 and MYO15A. Homozygosity mapping was performed by employing whole genome single nucleotide polymorphism (SNP genotyping in the families that did not carry mutations in GJB2 or MYO15A. Mutation analysis was performed for the known ARNSHL genes present in the homozygous regions to determine the causative mutations. This allowed the identification of a causative mutation in all the 30 families including 9 novel mutations, which were identified in 9 different families (GJB2 (c.598G>A, p.Gly200Arg; MYO15A (c.9948G>A, p.Gln3316Gln; c.3866+1G>A; c.8767C>T, p.Arg2923* and c.8222T>C, p.Phe2741Ser, TMC1 (c.362+18A>G, BSND (c.97G>C, p.Val33Leu, TMPRSS3 (c.726C>G, p.Cys242Trp and MSRB3 (c.20T>G, p.Leu7Arg. Furthermore, 12 recurrent mutations were detected in 21 other families. The 21 identified mutations included 10 (48% missense changes, 4 (19% nonsense mutations, 3 (14% intronic mutations, 2 (9% splice site mutations and 2 (9% frameshift mutations. GJB2 accounted for 53% of the families, while mutations in MYO15A were the second most frequent (13% cause of ARNSHL in these 30 families. The identification of novel as well as recurrent mutations in the present study increases the spectrum of mutations in known deafness genes which could lead to the identification of novel founder mutations and population specific mutated deafness genes causative of ARNSHL. These results provide detailed genetic information that has potential diagnostic implication in the establishment of cost-efficient allele-specific analysis of frequently occurring variants in combination with other reported mutations in Pakistani populations.

  5. Elucidation of the Molecular Genetic Basis of Inherited Hearing Impairment.

    NARCIS (Netherlands)

    Luijendijk, M.W.J.

    2006-01-01

    Hearing loss is the most common sensory disorder in the human population. It affects 0.1% of all young children and by the age of 70, 30% of the population suffers from hearing loss greater than 40 dB. When early onset hearing loss is inherited, 70% is classified as nonsyndromic and 30% as

  6. Allelic Mutations of KITLG, Encoding KIT Ligand, Cause Asymmetric and Unilateral Hearing Loss and Waardenburg Syndrome Type 2

    NARCIS (Netherlands)

    Zazo Seco, C.; Castro, L.S. de; Nierop, J.W. van; Morin, M.; Jhangiani, S.; Verver, E.J.; Schraders, M.; Maiwald, N.; Wesdorp, F.M.; Venselaar, H.; Spruijt, L.; Oostrik, J.; Schoots, J.; Reeuwijk, J. van; Lelieveld, S.H.; Huygen, P.L.M.; Insenser, M.; Admiraal, R.J.C.; Pennings, R.J.E.; Hoefsloot, L.H.; Arias Vasquez, A.; Ligt, J. de; Yntema, H.G.; Jansen, J.H.; Muzny, D.M.; Huls, G.A.; Rossum, M.M. van; Lupski, J.R.; Moreno-Pelayo, M.A.; Kunst, H.P.M.; Kremer, H.

    2015-01-01

    Linkage analysis combined with whole-exome sequencing in a large family with congenital and stable non-syndromic unilateral and asymmetric hearing loss (NS-UHL/AHL) revealed a heterozygous truncating mutation, c.286_303delinsT (p.Ser96Ter), in KITLG. This mutation co-segregated with NS-UHL/AHL as a

  7. Non-syndromic hearing impairment in India: high allelic heterogeneity among mutations in TMPRSS3, TMC1, USHIC, CDH23 and TMIE.

    Directory of Open Access Journals (Sweden)

    Aparna Ganapathy

    Full Text Available Mutations in the autosomal genes TMPRSS3, TMC1, USHIC, CDH23 and TMIE are known to cause hereditary hearing loss. To study the contribution of these genes to autosomal recessive, non-syndromic hearing loss (ARNSHL in India, we examined 374 families with the disorder to identify potential mutations. We found four mutations in TMPRSS3, eight in TMC1, ten in USHIC, eight in CDH23 and three in TMIE. Of the 33 potentially pathogenic variants identified in these genes, 23 were new and the remaining have been previously reported. Collectively, mutations in these five genes contribute to about one-tenth of ARNSHL among the families examined. New mutations detected in this study extend the allelic heterogeneity of the genes and provide several additional variants for structure-function correlation studies. These findings have implications for early DNA-based detection of deafness and genetic counseling of affected families in the Indian subcontinent.

  8. Coexistence of mitochondrial 12S rRNA C1494T and CO1/tRNASer(UCN) G7444A mutations in two Han Chinese pedigrees with aminoglycoside-induced and non-syndromic hearing loss

    International Nuclear Information System (INIS)

    Yuan Huijun; Chen Jing; Liu Xin; Cheng Jing; Wang Xinjian; Yang Li; Yang Shuzhi; Cao Juyang; Kang Dongyang; Dai Pu; Zha, Suoqiang; Han Dongyi; Young Wieyen; Guan Minxin

    2007-01-01

    Mutations in mitochondrial DNA are one of the important causes of hearing loss. We report here the clinical, genetic, and molecular characterization of two Han Chinese pedigrees with maternally transmitted aminoglycoside-induced and nonsyndromic bilateral hearing loss. Clinical evaluation revealed the wide range of severity, age-at-onset, and audiometric configuration of hearing impairment in matrilineal relatives in these families. The penetrances of hearing loss in these pedigrees were 20% and 18%, when aminoglycoside-induced deafness was included. When the effect of aminoglycosides was excluded, the penetrances of hearing loss in these seven pedigrees were 10% and 15%. Sequence analysis of the complete mitochondrial genomes in these pedigrees showed the presence of the deafness-associated 12S rRNA C1494T and CO1/tRNA Ser(UCN) G7444A mutations. Their distinct sets of mtDNA polymorphism belonged to Eastern Asian haplogroup C4a1, while other previously identified six Chinese mitochondrial genomes harboring the C1494T mutation belong to haplogroups D5a2, D, R, and F1, respectively. This suggested that the C1494T or G7444A mutation occurred sporadically and multiplied through evolution of the mitochondrial DNA (mtDNA). The absence of functionally significant mutations in tRNA and rRNAs or secondary LHON mutations in their mtDNA suggest that these mtDNA haplogroup-specific variants may not play an important role in the phenotypic expression of the 12S rRNA C1494T and CO1/tRNA Ser(UCN) G7444A mutations in those Chinese families. However, aminoglycosides and other nuclear modifier genes play a modifying role in the phenotypic manifestation of the C1494T mutation in these Chinese families

  9. Evaluation of family history of permanent hearing loss in childhood as a risk indicator in universal screening.

    Science.gov (United States)

    Valido Quintana, Mercedes; Oviedo Santos, Ángeles; Borkoski Barreiro, Silvia; Santana Rodríguez, Alfredo; Ramos Macías, Ángel

    Sixty percent of prelingual hearing loss is of genetic origin. A family history of permanent childhood hearing loss is a risk factor. The objective of the study is to determine the relationship between this risk factor and hearing loss. We have evaluated clinical and epidemiological characteristics and related nonsyndromic genetic variation. This was a retrospective, descriptive and observational study of newborns between January 2007 and December 2010 with family history as risk factor for hearing loss using transient evoked otoacoustic emissions and auditory brainstem response. A total of 26,717 children were born. Eight hundred and fifty-seven (3.2%) had family history. Fifty-seven(0.21%) failed to pass the second test. A percentage of 29.1 (n=16) had another risk factor, and 17.8% (n=9) had no classical risk factor. No risk factor was related to the hearing loss except heart disease. Seventy-six point four percent had normal hearing and 23.6% hearing loss. The mean of family members with hearing loss was 1.25. On genetic testing, 82.86% of homozygotes was normal, 11.43% heterozygosity in Connexin 26 gene (35delG), 2.86% R143W heterozygosity in the same gene and 2.86% mutant homozygotes (35delG). We found no relationship between hearing loss and mutated allele. The percentage of children with a family history and hearing loss is higher than expected in the general population. The genetic profile requires updating to clarify the relationship between hearing loss and heart disease, family history and the low prevalence in the mutations analyzed. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  10. A mutation in synaptojanin 2 causes progressive hearing loss in the ENU-mutagenised mouse strain Mozart.

    Science.gov (United States)

    Manji, Shehnaaz S M; Williams, Louise H; Miller, Kerry A; Ooms, Lisa M; Bahlo, Melanie; Mitchell, Christina A; Dahl, Hans-Henrik M

    2011-03-15

    Hearing impairment is the most common sensory impairment in humans, affecting 1:1,000 births. We have identified an ENU generated mouse mutant, Mozart, with recessively inherited, non-syndromic progressive hearing loss caused by a mutation in the synaptojanin 2 (Synj2), a central regulatory enzyme in the phosphoinositide-signaling cascade. The hearing loss in Mozart is caused by a p.Asn538Lys mutation in the catalytic domain of the inositol polyphosphate 5-phosphatase synaptojanin 2. Within the cochlea, Synj2 mRNA expression was detected in the inner and outer hair cells but not in the spiral ganglion. Synj2(N538K) mutant protein showed loss of lipid phosphatase activity, and was unable to degrade phosphoinositide signaling molecules. Mutant Mozart mice (Synj2(N538K/N538K)) exhibited progressive hearing loss and showed signs of hair cell degeneration as early as two weeks of age, with fusion of stereocilia followed by complete loss of hair bundles and ultimately loss of hair cells. No changes in vestibular or neurological function, or other clinical or behavioral manifestations were apparent. Phosphoinositides are membrane associated signaling molecules that regulate many cellular processes including cell death, proliferation, actin polymerization and ion channel activity. These results reveal Synj2 as a critical regulator of hair cell survival that is essential for hair cell maintenance and hearing function.

  11. CDH23 Mutation and Phenotype Heterogeneity: A Profile of 107 Diverse Families with Usher Syndrome and Nonsyndromic Deafness

    OpenAIRE

    Astuto, L. M.; Bork, J. M.; Weston, M. D.; Askew, J. W.; Fields, R. R.; Orten, D. J.; Ohliger, S. J.; Riazuddin, S.; Morell, R. J.; Khan, S.; Riazuddin, S.; Kremer, H.; van Hauwe, P.; Moller, C. G.; Cremers, C. W. R. J.

    2002-01-01

    Usher syndrome type I is characterized by congenital hearing loss, retinitis pigmentosa (RP), and variable vestibular areflexia. Usher syndrome type ID, one of seven Usher syndrome type I genetic localizations, have been mapped to a chromosomal interval that overlaps with a nonsyndromic-deafness localization, DFNB12. Mutations in CDH23, a gene that encodes a putative cell-adhesion protein with multiple cadherin-like domains, are responsible for both Usher syndrome and DFNB12 nonsyndromic deaf...

  12. Identification of Two Disease-causing Genes TJP2 and GJB2 in a Chinese Family with Unconditional Autosomal Dominant Nonsyndromic Hereditary Hearing Impairment

    Directory of Open Access Journals (Sweden)

    Hong-Yang Wang

    2015-01-01

    Full Text Available Background: There are more than 300 genetic loci that have been found to be related to hereditary hearing impairment (HHI, including 92 causative genes for nonsyndromic hearing loss, among which 34 genes are related to autosomal dominant nonsyndromic HHI (ADNSHHI. Traditional linkage analysis and candidate gene sequencing are not effective at detecting the ADNSHHI, especially for the unconditional families that may have more than one pathogenic cause. This study identified two disease-causing genes TJP2 and GJB2 in a Chinese family with unconditional ADNSHHI. Methods: To decipher the genetic code of a Chinese family (family 686 with ADNSHHI, different gene screening techniques have been performed, including linkage analysis, candidate genes screening, high-throughput sequencing and Sanger sequencing. These techniques were done on samples obtained from this family over a period of 10 years. Results: We identified a pathogenic missense mutation, c. 2081G>A (p.G694E, in TJP2, a gene that plays a crucial role in apoptosis and age-related hearing loss (ARHL. The mutation was co-segregated in this pedigree in all, but not in the two patients who presented with different phenotypes from the other affected family members. In one of the two patients, we confirmed that the compound heterozygosity for p.Y136FNx01 and p.G45E in the GJB2 gene may account for the phenotype shown in this patient. Conclusions: We identified the co-occurrence of two genetic causes in family 686. The possible disease-causing missense mutation of TJP2 in family 686 presents an opportunity for further investigation into ARHL. It is necessary to combine various genes screening methods, especially for some unconventional cases.

  13. GJB2 and mitochondrial A1555G gene mutations in nonsyndromic ...

    Indian Academy of Sciences (India)

    GJB2 mutations in 21.4% of the families in this country. (Bayazit et al. 2003). In this study, GJB2 gene mutations were responsible for 14.7% of genetic nonsyndromic hear- ing losses and 12.5% of the familial cases. These results are lower than in the previous reports where the patient selec- tion criteria may play a role.

  14. Localization of A Novel Autosomal Recessive Non-Syndromic Hearing Impairment Locus (DFNB38) to 6q26–q27 in a Consanguineous Kindred from Pakistan

    Science.gov (United States)

    Ansar, Muhammad; Ramzan, Mohammad; Pham, Thanh L.; Yan, Kai; Jamal, Syed Muhammad; Haque, Sayedul; Ahmad, Wasim; Leal, Suzanne M.

    2010-01-01

    For autosomal recessive nonsyndromic hearing impairment over 30 loci have been mapped and 19 genes have been identified. DFNB38, a novel locus for autosomal recessive nonsyndromic hearing impairment, was localized in a consanguineous Pakistani kindred to 6q26–q27. The affected family members present with profound prelingual sensorineural hearing impairment and use sign language for communications. Linkage was established to microsatellite markers located on chromosome 6q26–q27 (Multipoint lod score 3.6). The genetic region for DFNB38 spans 10.1 cM according to the Marshfield genetic map and is bounded by markers D6S980 and D6S1719. This genetic region corresponds to 3.4 MB on the sequence-based physical map. PMID:12890929

  15. CDH23 mutation and phenotype heterogeneity: a profile of 107 diverse families with Usher syndrome and nonsyndromic deafness.

    NARCIS (Netherlands)

    Astuto, L.M.; Bork, J.M.; Weston, M.D.; Askew, J.W.; Fields, R.R.; Orten, D.J.; Ohliger, S.J.; Riazuddin, S.; Morell, R.J.; Khan, S.; Kremer, J.M.J.; Hauwe, P. van; Moller, C.G.; Cremers, C.W.R.J.; Ayuso, C.; Heckenlively, J.R.; Rohrschneider, K.; Spandau, U.; Greenberg, J.; Ramesar, R.S.; Reardon, W.; Bitoun, P.; Millan, J.; Legge, R.; Friedman, T.B.; Kimberling, W.J.

    2002-01-01

    Usher syndrome type I is characterized by congenital hearing loss, retinitis pigmentosa (RP), and variable vestibular areflexia. Usher syndrome type ID, one of seven Usher syndrome type I genetic localizations, have been mapped to a chromosomal interval that overlaps with a nonsyndromic-deafness

  16. CDH23 mutation and phenotype heterogeneity: a profile of 107 diverse families with Usher syndrome and nonsyndromic deafness.

    Science.gov (United States)

    Astuto, L M; Bork, J M; Weston, M D; Askew, J W; Fields, R R; Orten, D J; Ohliger, S J; Riazuddin, S; Morell, R J; Khan, S; Riazuddin, S; Kremer, H; van Hauwe, P; Moller, C G; Cremers, C W R J; Ayuso, C; Heckenlively, J R; Rohrschneider, K; Spandau, U; Greenberg, J; Ramesar, R; Reardon, W; Bitoun, P; Millan, J; Legge, R; Friedman, T B; Kimberling, W J

    2002-08-01

    Usher syndrome type I is characterized by congenital hearing loss, retinitis pigmentosa (RP), and variable vestibular areflexia. Usher syndrome type ID, one of seven Usher syndrome type I genetic localizations, have been mapped to a chromosomal interval that overlaps with a nonsyndromic-deafness localization, DFNB12. Mutations in CDH23, a gene that encodes a putative cell-adhesion protein with multiple cadherin-like domains, are responsible for both Usher syndrome and DFNB12 nonsyndromic deafness. Specific CDH23 mutational defects have been identified that differentiate these two phenotypes. Only missense mutations of CDH23 have been observed in families with nonsyndromic deafness, whereas nonsense, frameshift, splice-site, and missense mutations have been identified in families with Usher syndrome. In the present study, a panel of 69 probands with Usher syndrome and 38 probands with recessive nonsyndromic deafness were screened for the presence of mutations in the entire coding region of CDH23, by heteroduplex, single-strand conformation polymorphism, and direct sequence analyses. A total of 36 different CDH23 mutations were detected in 45 families; 33 of these mutations were novel, including 18 missense, 3 nonsense, 5 splicing defects, 5 microdeletions, and 2 insertions. A total of seven mutations were common to more than one family. Numerous exonic and intronic polymorphisms also were detected. Results of ophthalmologic examinations of the patients with nonsyndromic deafness have found asymptomatic RP-like manifestations, indicating that missense mutations may have a subtle effect in the retina. Furthermore, patients with mutations in CDH23 display a wide range of hearing loss and RP phenotypes, differing in severity, age at onset, type, and the presence or absence of vestibular areflexia.

  17. CDH23 Mutation and Phenotype Heterogeneity: A Profile of 107 Diverse Families with Usher Syndrome and Nonsyndromic Deafness

    Science.gov (United States)

    Astuto, L. M.; Bork, J. M.; Weston, M. D.; Askew, J. W.; Fields, R. R.; Orten, D. J.; Ohliger, S. J.; Riazuddin, S.; Morell, R. J.; Khan, S.; Riazuddin, S.; Kremer, H.; van Hauwe, P.; Moller, C. G.; Cremers, C. W. R. J.; Ayuso, C.; Heckenlively, J. R.; Rohrschneider, K.; Spandau, U.; Greenberg, J.; Ramesar, R.; Reardon, W.; Bitoun, P.; Millan, J.; Legge, R.; Friedman, T. B.; Kimberling, W. J.

    2002-01-01

    Usher syndrome type I is characterized by congenital hearing loss, retinitis pigmentosa (RP), and variable vestibular areflexia. Usher syndrome type ID, one of seven Usher syndrome type I genetic localizations, have been mapped to a chromosomal interval that overlaps with a nonsyndromic-deafness localization, DFNB12. Mutations in CDH23, a gene that encodes a putative cell-adhesion protein with multiple cadherin-like domains, are responsible for both Usher syndrome and DFNB12 nonsyndromic deafness. Specific CDH23 mutational defects have been identified that differentiate these two phenotypes. Only missense mutations of CDH23 have been observed in families with nonsyndromic deafness, whereas nonsense, frameshift, splice-site, and missense mutations have been identified in families with Usher syndrome. In the present study, a panel of 69 probands with Usher syndrome and 38 probands with recessive nonsyndromic deafness were screened for the presence of mutations in the entire coding region of CDH23, by heteroduplex, single-strand conformation polymorphism, and direct sequence analyses. A total of 36 different CDH23 mutations were detected in 45 families; 33 of these mutations were novel, including 18 missense, 3 nonsense, 5 splicing defects, 5 microdeletions, and 2 insertions. A total of seven mutations were common to more than one family. Numerous exonic and intronic polymorphisms also were detected. Results of ophthalmologic examinations of the patients with nonsyndromic deafness have found asymptomatic RP–like manifestations, indicating that missense mutations may have a subtle effect in the retina. Furthermore, patients with mutations in CDH23 display a wide range of hearing loss and RP phenotypes, differing in severity, age at onset, type, and the presence or absence of vestibular areflexia. PMID:12075507

  18. Homoplasmy of the G7444A mtDNA and heterozygosity of the GJB2 c.35delG mutations in a family with hearing loss

    DEFF Research Database (Denmark)

    Kokotas, Haris; Grigoriadou, Maria; Yang, Li

    2011-01-01

    Mitochondrial mutations have been shown to be responsible for syndromic as well as non-syndromic hearing loss. The G7444A mitochondrial DNA mutation affects COI/the precursor of tRNA(Ser(UCN)), encoding the first subunit of cytochrome oxidase. Here we report on the first Greek family with the G74...

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

    Directory of Open Access Journals (Sweden)

    Marlene E Gubata

    2013-01-01

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

  20. Occupational hearing loss

    Science.gov (United States)

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

  1. [Presbycusis - Age Related Hearing Loss].

    Science.gov (United States)

    Fischer, N; Weber, B; Riechelmann, H

    2016-07-01

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

  2. Noise-Induced Hearing Loss

    Science.gov (United States)

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

  3. Missense mutation in the USH2A gene: association with recessive retinitis pigmentosa without hearing loss.

    Science.gov (United States)

    Rivolta, C; Sweklo, E A; Berson, E L; Dryja, T P

    2000-06-01

    Microdeletions Glu767(1-bp del), Thr967(1-bp del), and Leu1446(2-bp del) in the human USH2A gene have been reported to cause Usher syndrome type II, a disorder characterized by retinitis pigmentosa (RP) and mild-to-severe hearing loss. Each of these three frameshift mutations is predicted to lead to an unstable mRNA transcript that, if translated, would result in a truncated protein lacking the carboxy terminus. Here, we report Cys759Phe, a novel missense mutation in this gene that changes an amino-acid residue within the fifth laminin-epidermal growth factor-like domain of the USH2A gene and that is associated with recessive RP without hearing loss. This single mutation was found in 4.5% of 224 patients with recessive RP, suggesting that USH2A could cause more cases of nonsyndromic recessive RP than does any other gene identified to date.

  4. Sensorineural hearing loss in children.

    LENUS (Irish Health Repository)

    Wormald, R

    2010-02-01

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

  5. Digenic inheritance in autosomal recessive non-syndromic hearing loss cases carrying GJB2 heterozygote mutations: assessment of GJB4, GJA1, and GJC3.

    Science.gov (United States)

    Kooshavar, Daniz; Tabatabaiefar, Mohammad Amin; Farrokhi, Effat; Abolhasani, Marziye; Noori-Daloii, Mohammad-Reza; Hashemzadeh-Chaleshtori, Morteza

    2013-02-01

    Autosomal recessive non-syndromic hearing loss (ARNSHL) can be caused by many genes. However, mutations in the GJB2 gene, which encodes the gap-junction (GJ) protein connexin (Cx) 26, constitute a considerable proportion differing among population. Between 10 and 42 percent of patients with recessive GJB2 mutations carry only one mutant allele. Mutations in GJB4, GJA1, and GJC3 encoding Cx30.3, Cx43, and Cx29, respectively, can lead to HL. Combination of different connexins in heteromeric and heterotypic GJ assemblies is possible. This study aims to determine whether variations in any of the genes GJB4, GJA1 or GJC3 can be the second mutant allele causing the disease in the digenic mode of inheritance in the studied GJB2 heterozygous cases. We examined 34 unrelated GJB2 heterozygous ARNSHL subjects from different geographic and ethnic areas in Iran, using polymerase chain reaction (PCR) followed by direct DNA sequencing to identify any sequence variations in these genes. Restriction fragment length polymorphism (RFLP) assays were performed on 400 normal hearing individuals. Sequence analysis of GJB4 showed five heterozygous variations including c.451C>A, c.219C>T, c.507C>G, c.155_158delTCTG and c.542C>T, with only the latter variation not being detected in any of control samples. There were three heterozygous variations including c.758C>T, c.717G>A and c.3*dupA in GJA1 in four cases. We found no variations in GJC3 gene sequence. Our data suggest that GJB4 c.542C>T variant and less likely some variations of GJB4 and GJA1, but not possibly GJC3, can be assigned to ARNSHL in GJB2 heterozygous mutation carriers providing clues of the digenic pattern. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  6. Hearing: Noise-Induced Hearing Loss

    Science.gov (United States)

    ... 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 ...

  7. Individual Hearing Loss

    Directory of Open Access Journals (Sweden)

    Sébastien Santurette

    2016-06-01

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

  8. A novel mutation of the EYA4 gene associated with post-lingual hearing loss in a proband is co-segregating with a novel PAX3 mutation in two congenitally deaf family members.

    Science.gov (United States)

    Cesca, Federica; Bettella, Elisa; Polli, Roberta; Cama, Elona; Scimemi, Pietro; Santarelli, Rosamaria; Murgia, Alessandra

    2018-01-01

    This work was aimed at establishing the molecular etiology of hearing loss in a 9-year old girl with post-lingual non-syndromic mild sensorineural hearing loss with a complex family history of clinically heterogeneous deafness. The proband's DNA was subjected to NGS analysis of a 59-targeted gene panel, with the use of the Ion Torrent PGM platform. Conventional Sanger sequencing was used for segregation analysis in all the affected relatives. The proband and all the other hearing impaired members of the family underwent a thorough clinical and audiological evaluation. A new likely pathogenic mutation in the EYA4 gene (c.1154C > T; p.Ser385Leu) was identified in the proband and in her 42-year-old father with post-lingual non-syndromic profound sensorineural hearing loss. The EYA4 mutation was also found in the proband's grandfather and uncle, both showing clinical features of Waardenburg syndrome type 1. A novel pathogenic splice-site mutation (c.321+1G > A) of the PAX3 gene was found to co-segregate with the EYA4 mutation in these two subjects. The identified novel EYA4 mutation can be considered responsible of the hearing loss observed in the proband and her father, while a dual molecular diagnosis was reached in the relatives co-segregating the EYA4 and the PAX3 mutations. In these two subjects the DFNA10 phenotype was masked by Waardenburg syndrome. The use of NGS targeted gene-panel, in combination with an extensive clinical and audiological examination led us to identify the genetic cause of the hearing loss in members of a family in which different forms of autosomal dominant deafness segregate. These results provide precise and especially important prognostic and follow-up information for the future audiologic management in the youngest affected member. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. Hereditary Hearing Loss.

    Science.gov (United States)

    Tran, LenhAnh P.; Grundfast, Kenneth M.

    1997-01-01

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

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

    Science.gov (United States)

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

    2007-01-01

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

  11. Genes and Hearing Loss

    Science.gov (United States)

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

  12. OI Issues: Hearing Loss

    Science.gov (United States)

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

  13. Age-related hearing loss

    Science.gov (United States)

    ... 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 ...

  14. Definition of fluctuant hearing loss.

    Science.gov (United States)

    Shea, J J

    1975-06-01

    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.

  15. Hearing loss at work? Hearing loss from leisure activities?

    CERN Multimedia

    2006-01-01

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

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

    Science.gov (United States)

    Cohen, Brandon E.; Durstenfeld, Anne

    2014-01-01

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

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

    DEFF Research Database (Denmark)

    Mourtou, Eleni; Meis, Markus

    2012-01-01

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

  18. Acoustic Trauma - Hearing Loss in Teenagers

    Science.gov (United States)

    ... 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- ...

  19. Progressive hearing loss and degeneration of hair cell stereocilia in taperin gene knockout mice

    International Nuclear Information System (INIS)

    Chen, Mo; Wang, Qin; Zhu, Gang-Hua; Hu, Peng; Zhou, Yuan; Wang, Tian; Lai, Ruo-Sha; Xiao, Zi-An; Xie, Ding-Hua

    2016-01-01

    The TPRN gene encodes taperin, which is prominently present at the taper region of hair cell stereocilia. Mutations in TPRN have been reported to cause autosomal recessive nonsyndromic deafness 79(DFNB 79). To investigate the role of taperin in pathogenesis of hearing loss, we generated TPRN knockout mice using TALEN technique. Sanger sequencing confirmed an 11 bp deletion at nucleotide 177–187 in exon 1 of TPRN, which results in a truncated form of taperin protein. Heterozygous TPRN +/− mice showed apparently normal auditory phenotypes to their wide-type (WT) littermates. Homozygous TPRN −/− mice exhibited progressive sensorineural hearing loss as reflected by auditory brainstem response to both click and tone burst stimuli at postnatal days 15 (P15), 30 (P30), and 60 (P60). Alex Fluor-594 phalloidin labeling showed no obvious difference in hair cell numbers in the cochlea between TPRN −/− mice and WT mice under light microscope. However, scanning electronic microscopy revealed progressive degeneration of inner hair cell stereocilia, from apparently normal at postnatal days 3 (P3) to scattered absence at P15 and further to substantial loss at P30. The outer hair cell stereocilia also showed progressive degeneration, though much less severe, Collectively, we conclude that taperin plays an important role in maintenance of hair cell stereocilia. Establishment of TPRN knockout mice enables further investigation into the function of this gene. - Highlights: • TPRN −/− mice were generated using TALEN technique. • TPRN −/− mice presented progressive hearing loss. • WT and TPRN −/− mice showed no difference in hair cell numbers. • TPRN −/− mice showed progressive degeneration of hair cell stereocilia.

  20. Sudden bilateral hearing loss after organophosphate inhalation

    Directory of Open Access Journals (Sweden)

    Mehmet Akif Dundar

    2016-12-01

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

  1. Sensorineural hearing loss in hemorrhagic dengue?

    Directory of Open Access Journals (Sweden)

    Bruna Natália Freire Ribeiro

    2015-01-01

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

  2. Risk factors for hearing loss in neonates

    Directory of Open Access Journals (Sweden)

    Ni Luh Putu Maharani

    2016-11-01

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

  3. Delayed diagnosis of a patient with Usher syndrome 1C in a Louisiana Acadian family highlights the necessity of timely genetic testing for the diagnosis and management of congenital hearing loss.

    Science.gov (United States)

    Umrigar, Ayesha; Musso, Amanda; Mercer, Danielle; Hurley, Annette; Glausier, Cassondra; Bakeer, Mona; Marble, Michael; Hicks, Chindo; Tsien, Fern

    2017-01-01

    Advances in sequencing technologies and increased understanding of the contribution of genetics to congenital sensorineural hearing loss have led to vastly improved outcomes for patients and their families. Next-generation sequencing and diagnostic panels have become increasingly reliable and less expensive for clinical use. Despite these developments, the diagnosis of genetic sensorineural hearing loss still presents challenges for healthcare providers. Inherited sensorineural hearing loss has high levels of genetic heterogeneity and variable expressivity. Additionally, syndromic hearing loss (hearing loss and additional clinical abnormalities) should be distinguished from non-syndromic (hearing loss is the only clinical symptom). Although the diagnosis of genetic sensorineural hearing loss can be challenging, the patient's family history and ethnicity may provide critical information, as certain genetic mutations are more common in specific ethnic populations. The early identification of the cause of deafness can benefit patients and their families by estimating recurrence risks for future family planning and offering the proper interventions to improve their quality of life. Collaboration between pediatricians, audiologists, otolaryngologists, geneticists, and other specialists are essential in the diagnosis and management of patients with hearing disorders. An early diagnosis is vital for proper management and care, as some clinical manifestations of syndromic sensorineural hearing loss are not apparent at birth and have a delayed age of onset. We present a case of Usher syndrome (congenital deafness and childhood-onset blindness) illustrating the challenges encountered in the diagnosis and management of children presenting with congenital genetic sensorineural hearing loss, along with helpful resources for clinicians and families.

  4. Occupational hearing loss in Korea.

    Science.gov (United States)

    Kim, Kyoo Sang

    2010-12-01

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

  5. Genotype-phenotype correlation for DFNA22: characterization of non-syndromic, autosomal dominant, progressive sensorineural hearing loss due to MYO6 mutations

    DEFF Research Database (Denmark)

    Topsakal, Vedat; Hilgert, Nele; van Dinther, Joost

    2010-01-01

    Clinical and audiological examination was done in 2 Belgian families with autosomal dominant sensorineural hearing loss (SNHL) linked to DFNA22. Nineteen subjects in family 1 had mild to moderate SNHL starting in the third decade. The hearing loss was characterized by a flat audiogram affecting all......Hz. For all hitherto known DFNA22 families the audiological and clinical characteristics were correlated with the molecular data. This study describes the phenotype of 2 Belgian families with SNHL linked to DFNA22, both with a pathogenic change in the deafness gene MYO6. The phenotypes of all hitherto...

  6. Predictors of flourishing among children with hearing loss.

    Science.gov (United States)

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

    2016-12-01

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

  7. Etiology of hearing loss in children.

    Directory of Open Access Journals (Sweden)

    José Ignacio BENITO-OREJAS

    2017-06-01

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

  8. Hearing Loss: Symptoms, Diagnosis & Treatment

    Science.gov (United States)

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

  9. KCNJ10 may not be a contributor to nonsyndromic enlargement of vestibular aqueduct (NSEVA in Chinese subjects.

    Directory of Open Access Journals (Sweden)

    Jiandong Zhao

    Full Text Available BACKGROUND: Nonsyndromic enlargement of vestibular aqueduct (NSEVA is an autosomal recessive hearing loss disorder that is associated with mutations in SLC26A4. However, not all patients with NSEVA carry biallelic mutations in SLC26A4. A recent study proposed that single mutations in both SLC26A4 and KCNJ10 lead to digenic NSEVA. We examined whether KCNJ10 excert a role in the pathogenesis of NSEVA in Chinese patients. METHODS: SLC26A4 was sequenced in 1056 Chinese patients with NSEVA. KCNJ10 was screened in 131 patients who lacked mutations in either one or both alleles of SLC26A4. Additionally, KCNJ10 was screened in 840 controls, including 563 patients diagnosed with NSEVA who carried biallelic SLC26A4 mutations, 48 patients with nonsyndromic hearing loss due to inner ear malformations that did not involve enlargement of the vestibular aqueduct (EVA, 96 patients with conductive hearing loss due to various causes, and 133 normal-hearing individuals with no family history of hereditary hearing loss. RESULTS: 925 NSEVA patients were found carrying two-allele pathogenic SLC26A4 mutations. The most frequently detected KCNJ10 mutation was c.812G>A (p.R271H. Compared with the normal-hearing control subjects, the occurrence rate of c.812G>A in NSEVA patients with lacking mutations in one or both alleles of SLC26A4 had no significant difference(1.53% vs. 5.30%, χ(2 = 2.798, p = 0.172, which suggested that it is probably a nonpathogenic benign variant. KCNJ10 c.1042C>T (p.R348C, the reported EVA-related mutation, was not found in patients with NSEVA who lacked mutations in either one or both alleles of SLC26A4. Furthermore, the normal-hearing parents of patients with NSEVA having two SLC26A4 mutations carried the KCNJ10 c.1042C>T or c.812G>A mutation and a SLC26A4 pathogenic mutation. CONCLUSION: SLC26A4 is the major genetic cause in Chinese NSEVA patients, accounting for 87.59%. KCNJ10 may not be a contributor to NSEVA in Chinese population. Other

  10. Noise and Hearing Loss Prevention

    Science.gov (United States)

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

  11. Identification of a novel homozygous mutation, TMPRSS3: c.535G>A, in a Tibetan family with autosomal recessive non-syndromic hearing loss.

    Directory of Open Access Journals (Sweden)

    Dongyan Fan

    Full Text Available Different ethnic groups have distinct mutation spectrums associated with inheritable deafness. In order to identify the mutations responsible for congenital hearing loss in the Tibetan population, mutation screening for 98 deafness-related genes by microarray and massively parallel sequencing of captured target exons was conducted in one Tibetan family with familiar hearing loss. A homozygous mutation, TMPRSS3: c.535G>A, was identified in two affected brothers. Both parents are heterozygotes and an unaffected sister carries wild type alleles. The same mutation was not detected in 101 control Tibetan individuals. This missense mutation results in an amino acid change (p.Ala179Thr at a highly conserved site in the scavenger receptor cysteine rich (SRCR domain of the TMPRSS3 protein, which is essential for protein-protein interactions. Thus, this mutation likely affects the interactions of this transmembrane protein with extracellular molecules. According to our bioinformatic analyses, the TMPRSS3: c.535G>A mutation might damage protein function and lead to hearing loss. These data suggest that the homozygous mutation TMPRSS3: c.535G>A causes prelingual hearing loss in this Tibetan family. This is the first TMPRSS3 mutation found in the Chinese Tibetan population.

  12. Deafness and Hearing Loss.

    Science.gov (United States)

    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…

  13. Relationship between hearing complaint and hearing loss among older people

    Directory of Open Access Journals (Sweden)

    Teixeira, Adriane Ribeiro

    2009-03-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

  16. The circling mutant Pcdh15roda is a new mouse model for hearing loss.

    Science.gov (United States)

    Torres, Adriana Amorim; Rzadzinska, Agnieszka K; Ribeiro, Andrea Frozino; Silva, Daniel Almeida da Silva E; Guénet, Jean-Louis; Massironi, Sílvia Maria Gomes; Godard, Ana Lúcia Brunialti

    2013-01-01

    Mouse mutagenesis is a key tool for studying gene function and several mutant alleles have been described and constitute mouse models for human hereditary diseases. Genetic hearing loss represents over 50% of all hearing loss cases in children and, due to the heterogeneity of the disorder, there is still a demand for the isolation and characterization of new genes and alleles. Here we report phenotypic and molecular characterization of a new mouse model for hereditary hearing loss. The mutant rodador, isolated by Massironi and colleagues in 2006, presents an autosomal recessive disorder characterized by deafness and balance dysfunction associated with abnormal stereocilia in the inner ear. The mutation was mapped to mouse chromosome 10, and characterization of the gene Pcdh15 revealed an AT-to-GC transition in intron 23 of mutant animals. The alteration led to the switch of a dinucleotide ApA for ApG, creating a novel intronic acceptor splice site, which leads to incorporation of eight intronic bases into the processed mRNA and alteration of the downstream reading frame. In silico analysis indicated that the mutated protein is truncated and lacks two cadherin domains, and the transmembrane and cytoplasmic domains. Real Time PCR analyses revealed a significantly reduced Pcdh15 mRNA level in the brain of mutant mice, which might be due to the mechanism of non-sense mediated decay. In man, mutations in the orthologue PCDH15 cause non-syndromic deafness and Usher Syndrome Type 1F, a genetic disorder characterized by hearing loss and retinitis pigmentosa. Rodador mouse constitutes a new model for studying deafness in these conditions and may help in the comprehension of the pathogeneses of the disease, as well as of the mechanisms involved in the morphogenesis and function of inner ear stereocilia. This is a new ENU-induced allele and the first isolated in a BALB/c background. Copyright © 2013 Elsevier B.V. All rights reserved.

  17. Congenital sensorineural hearing loss

    International Nuclear Information System (INIS)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-01-01

    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

  18. Congenital sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    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.

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

    OpenAIRE

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

    2016-01-01

    Introduction Noise-induced hearing loss (NIHL) is a serious problem for workers and therefore for businesses. The hearing conservation program (HCP) is a set of coordinated measures to prevent the development or evolution of occupational hearing loss, which involves a continuous and dynamic process of implementation of hearing conservation routines through anticipation, recognition, evaluation, and subsequent control of the occurrence of existing environmental risks or of those thatmay exist...

  20. Hearing Screening and Diagnostic Evaluation of Children With Unilateral and Mild Bilateral Hearing Loss

    OpenAIRE

    Ross, Danielle S.; Holstrum, W. June; Gaffney, Marcus; Green, Denise; Oyler, Robert F.; Gravel, Judith S.

    2008-01-01

    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 ...

  1. Extremely low penetrance of hearing loss in four Chinese families with the mitochondrial 12S rRNA A1555G mutation

    International Nuclear Information System (INIS)

    Young Wieyen; Zhao Lidong; Qian Yaping; Wang Qiuju; Li Ning; Greinwald, John H.; Guan Minxin

    2005-01-01

    Mutations in mitochondrial DNA (mtDNA) have been found to be associated with sensorineural hearing loss. We report here the clinical, genetic, and molecular characterization of four Chinese pedigrees with aminoglycoside-induced and nonsyndromic hearing impairment. Clinical evaluation revealed the variable phenotype of hearing impairment including audiometric configuration in these subjects, although these subjects share some common features: bilateral and sensorineural hearing impairment. Strikingly, these Chinese pedigrees exhibited extremely low penetrance of hearing loss (5.2%, 4.8%, 4.2%, and 13.3%, respectively, and with an average 8% penetrance). In particular, four of all five affected matrilineal relatives of these pedigrees had aminoglycoside-induced hearing loss. Sequence analysis of the complete mitochondrial genomes in these pedigrees showed the distinct sets of mtDNA polymorphism, in addition to the identical homoplasmic A1555G mutation, associated with hearing impairment in many families from different genetic backgrounds. The fact that mtDNA of those pedigrees belonged to different haplogroups R9a, N9a, D4a, and D4 suggested that the A1555G mutation occurred sporadically and multiplied through evolution of the mtDNA in China. However, there was the absence of functionally significant mutations in tRNA and rRNAs or secondary LHON mutations in these Chinese families. These data imply that the nuclear background or/and mitochondrial haplotype may not play a significant role in the phenotypic expression of the A1555G mutation in these Chinese pedigrees. However, aminoglycoside appears to be a major modifier factor for the phenotypic manifestation of the A1555G mutation in these Chinese families

  2. Elderly With Different Types of Hearing Loss and Comorbidities: Satisfaction With Hearing Aids

    Directory of Open Access Journals (Sweden)

    Dashti

    2015-07-01

    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.

  3. A common SLC26A4-linked haplotype underlying non-syndromic hearing loss with enlargement of the vestibular aqueduct

    DEFF Research Database (Denmark)

    Chattaraj, Parna; Munjal, Tina; Honda, Keiji

    2017-01-01

    BACKGROUND: Enlargement of the vestibular aqueduct (EVA) is the most common radiological abnormality in children with sensorineural hearing loss. Mutations in coding regions and splice sites of the SLC26A4 gene are often detected in Caucasians with EVA. Approximately one-fourth of patients with E...

  4. Novel compound heterozygous mutations in MYO7A gene associated with autosomal recessive sensorineural hearing loss in a Chinese family.

    Science.gov (United States)

    Ma, Yalin; Xiao, Yun; Zhang, Fengguo; Han, Yuechen; Li, Jianfeng; Xu, Lei; Bai, Xiaohui; Wang, Haibo

    2016-04-01

    Mutations in MYO7A gene have been reported to be associated with Usher Syndrome type 1B (USH1B) and nonsyndromic hearing loss (DFNB2, DFNA11). Most mutations in MYO7A gene caused USH1B, whereas only a few reported mutations led to DFNB2 and DFNA11. The current study was designed to investigate the mutations among a Chinese family with autosomal recessive hearing loss. In this study, we present the clinical, genetic and molecular characteristics of a Chinese family. Targeted capture of 127 known deafness genes and next-generation sequencing were employed to study the genetic causes of two siblings in the Chinese family. Sanger sequencing was employed to examine those variant mutations in the members of this family and other ethnicity-matched controls. We identified the novel compound heterozygous mutant alleles of MYO7A gene: a novel missense mutation c.3671C>A (p.A1224D) and a reported insert mutation c.390_391insC (p.P131PfsX9). Variants were further confirmed by Sanger sequencing. These two compound heterozygous variants were co-segregated with autosomal recessive hearing loss phenotype. The gene mutation analysis and protein sequence alignment further supported that the novel compound heterozygous mutations were pathogenic. The novel compound heterozygous mutations (c.3671C>A and c.390_391insC) in MYO7A gene identified in this study were responsible for the autosomal recessive sensorineural hearing loss of this Chinese family. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  5. Hearing Loss due to Carbon Monoxide Poisoning

    DEFF Research Database (Denmark)

    Mehrparvar, Amir Houshang; Davari, Mohammad Hossein; Mollasadeghi, Abolfazl

    2013-01-01

    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....

  6. Imaging of post-traumatic hearing loss.

    Science.gov (United States)

    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.

  7. Hearing Loss: Screening Newborns

    Science.gov (United States)

    ... of this page please turn JavaScript on. Feature: Hearing Loss Screening Newborns Past Issues / Spring 2015 Table ... deafness, which account for most cases. Screening Newborns' Hearing Now Standard In 1993, children born in the ...

  8. Identifying hearing loss by means of iridology.

    Science.gov (United States)

    Stearn, Natalie; Swanepoel, De Wet

    2006-11-13

    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.

  9. Hearing Loss due to Carbon Monoxide Poisoning

    DEFF Research Database (Denmark)

    Mehrparvar, Amir Houshang; Davari, Mohammad Hossein; Mollasadeghi, Abolfazl

    2013-01-01

    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...

  10. [Hearing loss associated with smoking in male workers].

    Science.gov (United States)

    Takata, Yasumitsu

    2011-03-01

    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.

  11. Sudden Sensorineural Hearing Loss; Prognostic Factors

    OpenAIRE

    Arjun, Dass; Neha, Goel; Surinder K, Singhal; Ravi, Kapoor

    2015-01-01

    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...

  12. The Stigma of Hearing Loss

    Science.gov (United States)

    Wallhagen, Margaret I.

    2010-01-01

    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…

  13. Genes and Syndromic Hearing Loss.

    Science.gov (United States)

    Keats, Bronya J. B.

    2002-01-01

    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…

  14. Conductive hearing loss and bone conduction devices: restored binaural hearing?

    Science.gov (United States)

    Agterberg, Martijn J H; Hol, Myrthe K S; Cremers, Cor W R J; Mylanus, Emmanuel A M; van Opstal, John; Snik, Ad F M

    2011-01-01

    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.

  15. Hearing Loss in Cryptococcal Meningitis Survivors

    OpenAIRE

    Lofgren, Sarah; Montgomery, Martha; Yueh, Nathan; Namudde, Alice; Rhein, Joshua; Abassi, Mahsa; Musubire, Abdu; Meya, David; Boulware, David

    2017-01-01

    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...

  16. Does tinnitus, hearing asymmetry, or hearing loss predispose to occupational injury risk?

    Science.gov (United States)

    Cantley, Linda F; Galusha, Deron; Cullen, Mark R; Dixon-Ernst, Christine; Tessier-Sherman, Baylah; Slade, Martin D; Rabinowitz, Peter M; Neitzel, Richard L

    2015-02-01

    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.

  17. Hearing loss in children with growth hormone deficiency.

    Science.gov (United States)

    Muus, John S; Weir, Forest W; Kreicher, Kathryn L; Bowlby, Deborah A; Discolo, Christopher M; Meyer, Ted A

    2017-09-01

    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.

  18. Psychosocial Aspects of Hearing Loss in Children.

    Science.gov (United States)

    Sorkin, Donna L; Gates-Ulanet, Patricia; Mellon, Nancy K

    2015-12-01

    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.

  19. Hearing Loss in HIV-Infected Children in Lilongwe, Malawi.

    Science.gov (United States)

    Hrapcak, Susan; Kuper, Hannah; Bartlett, Peter; Devendra, Akash; Makawa, Atupele; Kim, Maria; Kazembe, Peter; Ahmed, Saeed

    2016-01-01

    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

  20. Hearing devices for children with unilateral hearing loss: Patient- and parent-reported perspectives.

    Science.gov (United States)

    Purcell, Patricia L; Jones-Goodrich, Rose; Wisneski, Meghan; Edwards, Todd C; Sie, Kathleen C Y

    2016-11-01

    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.

  1. Characteristics of children with unilateral hearing loss.

    Science.gov (United States)

    Fitzpatrick, Elizabeth M; Al-Essa, Rakan S; Whittingham, JoAnne; Fitzpatrick, Jessica

    2017-11-01

    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.

  2. Age-Related Hearing Loss

    Science.gov (United States)

    ... 29, 2017 Granicus - Health Topics Languages Español Download PDF version Order free publications Find organizations Syndicate content Related Topics Do You Need a Hearing Test? Hearing Loss and Older Adults News Spatial organization of cells in the inner ear enables the sense and ...

  3. Noise-induced hearing loss: a recreational noise perspective.

    Science.gov (United States)

    Ivory, Robert; Kane, Rebecca; Diaz, Rodney C

    2014-10-01

    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.

  4. Speech perception in noise in unilateral hearing loss

    OpenAIRE

    Mondelli, Maria Fernanda Capoani Garcia; dos Santos, Marina de Marchi; José, Maria Renata

    2016-01-01

    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...

  5. Hearing Loss in HIV-Infected Children in Lilongwe, Malawi

    Science.gov (United States)

    Hrapcak, Susan; Kuper, Hannah; Bartlett, Peter; Devendra, Akash; Makawa, Atupele; Kim, Maria; Kazembe, Peter; Ahmed, Saeed

    2016-01-01

    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

  6. Hearing aid fitting in older persons with hearing impairment: the influence of cognitive function, age, and hearing loss on hearing aid benefit.

    Science.gov (United States)

    Meister, Hartmut; Rählmann, Sebastian; Walger, Martin; Margolf-Hackl, Sabine; Kießling, Jürgen

    2015-01-01

    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

  7. Hearing aid-related satisfaction based on type and degree of hearing loss in elderly

    Directory of Open Access Journals (Sweden)

    Farzad FarajiKhiavi

    2015-02-01

    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.

  8. Self-Esteem in Children and Adolescents With Hearing Loss

    OpenAIRE

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

    2015-01-01

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

  9. Postural control assessment in students with normal hearing and sensorineural hearing loss.

    Science.gov (United States)

    Melo, Renato de Souza; Lemos, Andrea; Macky, Carla Fabiana da Silva Toscano; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

    2015-01-01

    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.

  10. Visual Prognosis in USH2A-Associated Retinitis Pigmentosa Is Worse for Patients with Usher Syndrome Type IIa Than for Those with Nonsyndromic Retinitis Pigmentosa.

    Science.gov (United States)

    Pierrache, Laurence H M; Hartel, Bas P; van Wijk, Erwin; Meester-Smoor, Magda A; Cremers, Frans P M; de Baere, Elfride; de Zaeytijd, Julie; van Schooneveld, Mary J; Cremers, Cor W R J; Dagnelie, Gislin; Hoyng, Carel B; Bergen, Arthur A; Leroy, Bart P; Pennings, Ronald J E; van den Born, L Ingeborgh; Klaver, Caroline C W

    2016-05-01

    USH2A mutations are an important cause of retinitis pigmentosa (RP) with or without congenital sensorineural hearing impairment. We studied genotype-phenotype correlations and compared visual prognosis in Usher syndrome type IIa and nonsyndromic RP. Clinic-based, longitudinal, multicenter study. Consecutive patients with Usher syndrome type IIa (n = 152) and nonsyndromic RP (n = 73) resulting from USH2A mutations from ophthalmogenetic clinics in the Netherlands and Belgium. Data on clinical characteristics, visual acuity, visual field measurements, retinal imaging, and electrophysiologic features were extracted from medical charts over a mean follow-up of 9 years. Cumulative lifetime risks of low vision and blindness were estimated using Kaplan-Meier survival analysis. Low vision and blindness. Participant groups had similar distributions of gender (48% vs. 45% males in Usher syndrome type IIa vs. nonsydromic RP; P = 0.8), ethnicity (97% vs. 99% European; P = 0.3), and median follow-up time (6.5 years vs. 3 years; P = 0.3). Usher syndrome type IIa patients demonstrated symptoms at a younger age (median age, 15 years vs. 25 years; P syndromic phenotype, whereas other combinations were present in both groups. We found novel variants in Usher syndrome type IIa (25%) and nonsyndromic RP (19%): 29 missense mutations, 10 indels, 14 nonsense mutations, 9 frameshift mutations, and 5 splice-site mutations. Most patients with USH2A-associated RP have severe visual impairment by age 50. However, those with Usher syndrome type IIa have an earlier decline of visual function and a higher cumulative risk of visual impairment than those without nonsyndromic RP. Complete loss of function of the USH2A protein predisposes to Usher syndrome type IIa, but remnant protein function can lead to RP with or without hearing loss. Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  11. Noise-induced hearing loss

    Directory of Open Access Journals (Sweden)

    Mariola Sliwinska-Kowalska

    2012-01-01

    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.

  12. Current amplification models of sensorineurall and conductive hearing loss

    OpenAIRE

    Ostojić, Sanja; Mikić, Branka; Mirić, Danica

    2012-01-01

    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 ...

  13. Hearing loss in children treated for multidrug-resistant tuberculosis.

    Science.gov (United States)

    Seddon, James A; Thee, Stephanie; Jacobs, Kayleen; Ebrahim, Adam; Hesseling, Anneke C; Schaaf, H Simon

    2013-04-01

    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.

  14. Auditory Memory deficit in Elderly People with Hearing Loss

    Directory of Open Access Journals (Sweden)

    Zahra Shahidipour

    2013-06-01

    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

  15. A canonical splice site mutation in GIPC3 causes sensorineural hearing loss in a large Pakistani family

    NARCIS (Netherlands)

    Siddiqi, S.; Ismail, M.; Oostrik, J.; Munawar, S.; Mansoor, A.; Kremer, H.; Qamar, R.; Schraders, M.

    2014-01-01

    With homozygosity mapping we have identified two large homozygous regions on chromosome 3q13.11-q13.31 and chromosome 19p13.3-q31.32 in a large Pakistani family suffering from autosomal recessive nonsyndromic hearing impairment (arNSHI). The region on chromosome 19 overlaps with the previously

  16. Sensorineural Hearing Loss following Carbon Monoxide Poisoning

    Directory of Open Access Journals (Sweden)

    Joseph P. Pillion

    2012-01-01

    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.

  17. The influence of mood on the perception of hearing-loss related quality of life in people with hearing loss and their significant others.

    Science.gov (United States)

    Preminger, Jill E; Meeks, Suzanne

    2010-04-01

    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.

  18. Current amplification models of sensorineurall and conductive hearing loss

    Directory of Open Access Journals (Sweden)

    Ostojić Sanja

    2012-01-01

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

  19. Hearing loss and social support in urban and rural communities.

    Science.gov (United States)

    Hay-McCutcheon, Marcia J; Hyams, Adriana; Yang, Xin; Parton, Jason

    2018-04-19

    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.

  20. Gd enhanced MRI in sensorineural hearing loss

    International Nuclear Information System (INIS)

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

    1996-01-01

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

  1. Print Knowledge of Preschool Children with Hearing Loss

    Science.gov (United States)

    Werfel, Krystal L.; Lund, Emily; Schuele, C. Melanie

    2015-01-01

    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.

  2. Mechanisms of hearing loss in neurofibromatosis type 2.

    Directory of Open Access Journals (Sweden)

    Ashok R Asthagiri

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

  3. Self-esteem in children and adolescents with hearing loss.

    Science.gov (United States)

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

    2015-03-09

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

  4. Self-Esteem in Children and Adolescents With Hearing Loss

    Science.gov (United States)

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

    2015-01-01

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

  5. Gambling among adolescents with and without hearing loss

    Directory of Open Access Journals (Sweden)

    Susanna Geidne

    2016-08-01

    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.

  6. Hearing loss in Usher syndrome type II is nonprogressive.

    Science.gov (United States)

    Reisser, Christoph F V; Kimberling, William J; Otterstedde, Christian R

    2002-12-01

    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.

  7. Sensorineural hearing loss after magnetic resonance imaging

    DEFF Research Database (Denmark)

    Mollasadeghi, Abolfazl; Mehrparvar, Amir Houshang; Atighechi, Saeid

    2013-01-01

    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....

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

    Directory of Open Access Journals (Sweden)

    Yu-Chen Hung

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

  9. Hearing loss - infants

    Science.gov (United States)

    ... 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 ...

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

    Directory of Open Access Journals (Sweden)

    Chao-Hui eYang

    2015-07-01

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

  11. Occupational hearing loss in farmers.

    OpenAIRE

    Plakke, B L; Dare, E

    1992-01-01

    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...

  12. Uncovering effective strategies for hearing loss prevention

    Science.gov (United States)

    Morata, Thais C.; Meinke, Deanna

    2016-01-01

    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

  13. Self-Esteem in Children and Adolescents With Hearing Loss

    Directory of Open Access Journals (Sweden)

    Andrea D. Warner-Czyz

    2015-03-01

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

  14. Sensorineural Hearing Loss after Magnetic Resonance Imaging

    Directory of Open Access Journals (Sweden)

    Abolfazl Mollasadeghi

    2013-01-01

    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.

  15. Hearing Loss: Issues in the Deaf and Hard of Hearing Communities.

    Science.gov (United States)

    Moreland, Christopher; Atcherson, Samuel R; Zazove, Philip; McKee, Michael M

    2015-07-01

    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.

  16. Sudden hearing loss after an explosion

    Directory of Open Access Journals (Sweden)

    Irfan Mohamad

    2013-12-01

    Full Text Available An 18-year-old man presented with a sudden onset of bilateral hearing loss after a home-made firework exploded near the right side of his scalp. The hearing loss was associated with tinnitus. Examination revealed an area of skin loss on the right pinna. There was mild bleeding from the right pinna and scalp at the mastoid region, which spontaneously resolved. An otoscopic examination is shown in Figure 1.

  17. Sensorineural hearing loss after magnetic resonance imaging

    DEFF Research Database (Denmark)

    Mollasadeghi, Abolfazl; Mehrparvar, Amir Houshang; Atighechi, Saeid

    2013-01-01

    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.......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...

  18. Evaluation of Extended-Wear Hearing Technology for Children with Hearing Loss.

    Science.gov (United States)

    Wolfe, Jace; Schafer, Erin; Martella, Natalie; Morais, Mila; Mann, Misty

    2015-01-01

    Research shows that many older children and teenagers who have mild to moderately severe sensorineural hearing loss do not use their hearing instruments during all waking hours. A variety of reasons may contribute toward this problem, including concerns about cosmetics associated with hearing aid use and the inconvenience of daily maintenance associated with hearing instruments. Extended-wear hearing instruments are inserted into the wearer's ear canal by an audiologist and are essentially invisible to outside observers. The goal of this study was to evaluate the potential benefits and limitations associated with use of extended-wear hearing instruments in a group of children with hearing loss. A two-way repeated measures design was used to examine performance differences obtained with the participants' daily-wear hearing instruments versus that obtained with extended-wear hearing instruments. Sixteen children, ages 10-17 yr old, with sensorineural hearing loss ranging from mild to moderately severe. Probe microphone measures were completed to evaluate the aided output of device. Behavioral test measures included word recognition in quiet, sentence recognition in noise, aided warble-tone thresholds, and psychophysical loudness scaling. Questionnaires were also administered to evaluate subjective performance with each hearing technology. Data logging suggested that many participants were not using their daily-wear hearing instruments during all waking hours (mean use was less than 6 h/day). Real ear probe microphone measurements indicated that a closer fit to the Desired Sensation Level Version 5 prescriptive targets was achieved with the children's daily-wear instruments when compared to the extended-wear instruments. There was no statistically significant difference in monosyllabic word recognition at 50 or 60 dBA obtained with the two hearing technologies. Sentence recognition in noise obtained with use of the extended-wear devices was, however, significantly

  19. Clinical study on unilateral hearing loss in children

    International Nuclear Information System (INIS)

    Masuda, Sawako; Usui, Satoko

    2007-01-01

    A series of 60 children was studied (aged 0 to 10 years, 32 boys, 28 girls) with severe unilateral sensorineural hearing loss of unknown etiology. There were two peaks, at 0 and 6 years. In 19 children, hearing loss was identified during a conservative general health checkup for school or preschool children. In 21 children aged 0 years, 16 were suspected of hearing loss by newborn hearing screening. Temporal bone computed tomography scans were examined in 51 patients. Sixteen ears (31.4%) with hearing loss had inner ear and/or internal auditory canal abnormalities. In one patient, the anomaly was the presence of a bony wall dividing the internal auditory canal into two separate compartments associated with severe inner ear hypoplasia. Two patients had a common cavity. In one of these patients, the anomaly was revealed because of severe bacterial meningitis, and another was detected by newborn hearing screening. Six patients had a narrow internal auditory canal, 4 had a narrow internal auditory canal and hypoplastic cochlea, and 1 had a narrow internal auditory canal and cystic vestibule, and lateral semicircular canal dysplasia. Two patients had a cystic vestibule and lateral semicircular canal dysplasia. One case showed fluctuation of the hearing level in the contralateral ear with normal hearing during the observation period at an average of 20 months. The number of children whose unilateral hearing loss is detected early by newborn hearing screening has enormously increased. Strategies for follow-up, early intervention, and support for families are necessary for young children with unilateral hearing impairment. (author)

  20. An Auditory Model with Hearing Loss

    DEFF Research Database (Denmark)

    Nielsen, Lars Bramsløw

    An auditory model based on the psychophysics of hearing has been developed and tested. The model simulates the normal ear or an impaired ear with a given hearing loss. Based on reviews of the current literature, the frequency selectivity and loudness growth as functions of threshold and stimulus...... level have been found and implemented in the model. The auditory model was verified against selected results from the literature, and it was confirmed that the normal spread of masking and loudness growth could be simulated in the model. The effects of hearing loss on these parameters was also...... in qualitative agreement with recent findings. The temporal properties of the ear have currently not been included in the model. As an example of a real-world application of the model, loudness spectrograms for a speech utterance were presented. By introducing hearing loss, the speech sounds became less audible...

  1. Molecular diagnostics for congenital hearing loss including 15 deafness genes using a next generation sequencing platform

    Directory of Open Access Journals (Sweden)

    De Keulenaer Sarah

    2012-05-01

    Full Text Available Abstract Background Hereditary hearing loss (HL can originate from mutations in one of many genes involved in the complex process of hearing. Identification of the genetic defects in patients is currently labor intensive and expensive. While screening with Sanger sequencing for GJB2 mutations is common, this is not the case for the other known deafness genes (> 60. Next generation sequencing technology (NGS has the potential to be much more cost efficient. Published methods mainly use hybridization based target enrichment procedures that are time saving and efficient, but lead to loss in sensitivity. In this study we used a semi-automated PCR amplification and NGS in order to combine high sensitivity, speed and cost efficiency. Results In this proof of concept study, we screened 15 autosomal recessive deafness genes in 5 patients with congenital genetic deafness. 646 specific primer pairs for all exons and most of the UTR of the 15 selected genes were designed using primerXL. Using patient specific identifiers, all amplicons were pooled and analyzed using the Roche 454 NGS technology. Three of these patients are members of families in which a region of interest has previously been characterized by linkage studies. In these, we were able to identify two new mutations in CDH23 and OTOF. For another patient, the etiology of deafness was unclear, and no causal mutation was found. In a fifth patient, included as a positive control, we could confirm a known mutation in TMC1. Conclusions We have developed an assay that holds great promise as a tool for screening patients with familial autosomal recessive nonsyndromal hearing loss (ARNSHL. For the first time, an efficient, reliable and cost effective genetic test, based on PCR enrichment, for newborns with undiagnosed deafness is available.

  2. Behavioral Signs of (Central) Auditory Processing Disorder in Children With Nonsyndromic Cleft Lip and/or Palate: A Parental Questionnaire Approach.

    Science.gov (United States)

    Ma, Xiaoran; McPherson, Bradley; Ma, Lian

    2016-03-01

    Objective Children with nonsyndromic cleft lip and/or palate often have a high prevalence of middle ear dysfunction. However, there are also indications that they may have a higher prevalence of (central) auditory processing disorder. This study used Fisher's Auditory Problems Checklist for caregivers to determine whether children with nonsyndromic cleft lip and/or palate have potentially more auditory processing difficulties compared with craniofacially normal children. Methods Caregivers of 147 school-aged children with nonsyndromic cleft lip and/or palate were recruited for the study. This group was divided into three subgroups: cleft lip, cleft palate, and cleft lip and palate. Caregivers of 60 craniofacially normal children were recruited as a control group. Hearing health tests were conducted to evaluate peripheral hearing. Caregivers of children who passed this assessment battery completed Fisher's Auditory Problems Checklist, which contains 25 questions related to behaviors linked to (central) auditory processing disorder. Results Children with cleft palate showed the lowest scores on the Fisher's Auditory Problems Checklist questionnaire, consistent with a higher index of suspicion for (central) auditory processing disorder. There was a significant difference in the manifestation of (central) auditory processing disorder-linked behaviors between the cleft palate and the control groups. The most common behaviors reported in the nonsyndromic cleft lip and/or palate group were short attention span and reduced learning motivation, along with hearing difficulties in noise. Conclusion A higher occurrence of (central) auditory processing disorder-linked behaviors were found in children with nonsyndromic cleft lip and/or palate, particularly cleft palate. Auditory processing abilities should not be ignored in children with nonsyndromic cleft lip and/or palate, and it is necessary to consider assessment tests for (central) auditory processing disorder when an

  3. Hearing in young adults. Part I: The effects of attitudes and beliefs toward noise, hearing loss, and hearing protector devices

    Directory of Open Access Journals (Sweden)

    Hannah Keppler

    2015-01-01

    Full Text Available There is great concern regarding the development of noise-induced hearing loss (NIHL in youth caused by high sound levels during various leisure activities. Health-orientated behavior of young adults might be linked to the beliefs and attitudes toward noise, hearing loss, and hearing protector devices (HPDs. The objective of the current study was to evaluate the effects of attitudes and beliefs toward noise, hearing loss, and HPDs on young adults′ hearing status. A questionnaire and an audiological test battery were completed by 163 subjects (aged 18-30 years. The questionnaire contained the Youth Attitude to Noise Scale (YANS and Beliefs about Hearing Protection and Hearing Loss (BAHPHL. A more positive attitude or belief represented an attitude where noise or hearing loss is seen as unproblematic and attitudes and beliefs regarding HPDs is worse. Hearing was evaluated using (high frequency pure tone audiometry (PTA, transient evoked and distortion product otoacoustic emissions. First, mean differences in hearing between the groups with different attitudes and beliefs were evaluated using one-way analysis of variance (ANOVA. Second, a χ2 test was used to examine the usage of HPDs by the different groups with different attitudes and beliefs. Young adults with a positive attitude had significantly more deteriorated hearing and used HPDs less than the other subjects. Hearing conservation programs (HCPs for young adults should provide information and knowledge regarding noise, hearing loss, and HPDs. Barriers wearing HPDs should especially be discussed. Further, those campaigns should focus on self-experienced hearing related symptoms that might serve as triggers for attitudinal and behavioral changes.

  4. P300 in individuals with sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Ana Cláudia Mirandola Barbosa Reis

    2015-04-01

    Full Text Available INTRODUCTION: Behavioral and electrophysiological auditory evaluations contribute to the understanding of the auditory system and of the process of intervention.OBJECTIVE: To study P300 in subjects with severe or profound sensorineural hearing loss.METHODS: This was a descriptive cross-sectional prospective study. It included 29 individuals of both genders with severe or profound sensorineural hearing loss without other type of disorders, aged 11 to 42 years; all were assessed by behavioral audiological evaluation and auditory evoked potentials.RESULTS: A recording of the P3 wave was obtained in 17 individuals, with a mean latency of 326.97 ms and mean amplitude of 3.76 V. There were significant differences in latency in relation to age and in amplitude according to degree of hearing loss. There was a statistically significant association of the P300 results with the degrees of hearing loss (p = 0.04, with the predominant auditory communication channels (p < 0.0001, and with time of hearing loss.CONCLUSIONS: P300 can be recorded in individuals with severe and profound congenital sensorineural hearing loss; it may contribute to the understanding of cortical development and is a good predictor of the early intervention outcome.

  5. The relationship between nonverbal cognitive functions and hearing loss

    NARCIS (Netherlands)

    Zekveld, A.A.; Deijen, J.B.; Goverts, S.T.; Kramer, S.E.

    2007-01-01

    Purpose: This study investigated the relationship between hearing loss and memory and attention when nonverbal, visually presented cognitive tests are used. Method: Hearing loss (pure-tone audiometry) and IQ were measured in 30 participants with mild to severe hearing loss. Participants performed

  6. Applications of direct-to-consumer hearing devices for adults with hearing loss: a review

    Directory of Open Access Journals (Sweden)

    Manchaiah V

    2017-05-01

    Full Text Available Vinaya Manchaiah,1–4 Brian Taylor,5 Ashley L Dockens,1 Nicole R Tran,1 Kayla Lane,1 Mariana Castle,1 Vibhu Grover1 1Department of Speech and Hearing Sciences, Lamar University, Beaumont, TX, USA; 2The Swedish Institute for Disability Research, Department of Behavioural Sciences and Learning, Linköping University, Linköping, Sweden; 3Audiology India, Mysore, 4Department of Speech and Hearing, School of Allied Health Sciences, Manipal University, Manipal, India; 5Taylor Audio LLC, Minneapolis, MN, USA Background: This systematic literature review is aimed at investigating applications of direct-to-consumer hearing devices for adults with hearing loss. This review discusses three categories of direct-to-consumer hearing devices: 1 personal sound amplification products (PSAPs, 2 direct-mail hearing aids, and 3 over-the-counter (OTC hearing aids.Method: A literature review was conducted using EBSCOhost and included the databases CINAHL, MEDLINE, and PsycINFO. After applying prior agreed inclusion and exclusion criteria, 13 reports were included in the review.Results: Included studies fell into three domains: 1 electroacoustic characteristics, 2 consumer surveys, and 3 outcome evaluations. Electroacoustic characteristics of these devices vary significantly with some meeting the stringent acoustic criteria used for hearing aids, while others producing dangerous output levels (ie, over 120-dB sound pressure level. Low-end (or low-cost devices were typically poor in acoustic quality and did not meet gain levels necessary for most adult and elderly hearing loss patterns (eg, presbycusis, especially in high frequencies. Despite direct-mail hearing aids and PSAPs being associated with lower satisfaction when compared to hearing aids purchased through hearing health care professionals, consumer surveys suggest that 5%–19% of people with hearing loss purchase hearing aids through direct-mail or online. Studies on outcome evaluation suggest positive

  7. Murine CMV-induced hearing loss is associated with inner ear inflammation and loss of spiral ganglia neurons.

    Directory of Open Access Journals (Sweden)

    Russell D Bradford

    2015-04-01

    Full Text Available Congenital human cytomegalovirus (HCMV occurs in 0.5-1% of live births and approximately 10% of infected infants develop hearing loss. The mechanism(s of hearing loss remain unknown. We developed a murine model of CMV induced hearing loss in which murine cytomegalovirus (MCMV infection of newborn mice leads to hematogenous spread of virus to the inner ear, induction of inflammatory responses, and hearing loss. Characteristics of the hearing loss described in infants with congenital HCMV infection were observed including, delayed onset, progressive hearing loss, and unilateral hearing loss in this model and, these characteristics were viral inoculum dependent. Viral antigens were present in the inner ear as were CD(3+ mononuclear cells in the spiral ganglion and stria vascularis. Spiral ganglion neuron density was decreased after infection, thus providing a mechanism for hearing loss. The lack of significant inner ear histopathology and persistence of inflammation in cochlea of mice with hearing loss raised the possibility that inflammation was a major component of the mechanism(s of hearing loss in MCMV infected mice.

  8. Autism and peripheral hearing loss: a systematic review.

    Science.gov (United States)

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

    2014-01-01

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

  9. Novel mutations in the long isoform of the USH2A gene in patients with Usher syndrome type II or non-syndromic retinitis pigmentosa.

    Science.gov (United States)

    McGee, Terri L; Seyedahmadi, Babak Jian; Sweeney, Meredith O; Dryja, Thaddeus P; Berson, Eliot L

    2010-07-01

    Usher syndrome type II (USH2) is an autosomal recessive disorder characterised by retinitis pigmentosa (RP) and mild to moderate sensorineural hearing loss. Mutations in the USH2A gene are the most common cause of USH2 and are also a cause of some forms of RP without hearing loss (ie, non-syndromic RP). The USH2A gene was initially identified as a transcript comprised of 21 exons but subsequently a longer isoform containing 72 exons was identified. The 51 exons unique to the long isoform of USH2A were screened for mutations among a core set of 108 patients diagnosed with USH2 and 80 patients with non-syndromic RP who were all included in a previously reported screen of the short isoform of USH2A. For several exons, additional patients were screened. In total, 35 deleterious mutations were identified including 17 nonsense mutations, 9 frameshift mutations, 5 splice-site mutations, and 4 small in-frame deletions or insertions. Twenty-seven mutations were novel. In addition, 65 rare missense changes were identified. A method of classifying the deleterious effect of the missense changes was developed using the summed results of four different mutation assessment algorithms, SIFT, pMUT, PolyPhen, and AGVGD. This system classified 8 of the 65 changes as 'likely deleterious' and 9 as 'possibly deleterious'. At least one mutation was identified in 57-63% of USH2 cases and 19-23% of cases of non-syndromic recessive RP (calculated without and including probable/possible deleterious changes) thus supporting that USH2A is the most common known cause of RP in the USA.

  10. Risk factors for hearing loss in elderly

    Directory of Open Access Journals (Sweden)

    Kelly Vasconcelos Chaves Martins

    2012-06-01

    Full Text Available Objective: To identify risk factors related to sensorineural hearing loss in elderly. Methods: The sample consisted of 60 selected elderly, divided into two groups: the Case Group, composed by 30 individuals, 21 females and 9 males, aged at least 60 years, presenting sensorineural hearing loss, and the Control Group, composed by 30 individuals matched on gender and age, with normal hearing. The patients were submitted to audiological anamnesis and tonal audiometry. The hearing impairment was defined according to average threshold greater than 35dBNA, in the frequencies of 1,000; 2,000 and 4,000 Hz, in the best ear. Results: Statistically significant odds ratios were: a to audiological history: noise exposure and family history of deafness; b to situations involving hearing difficulty: television, church, telephone, silent environment, spatial location of sound, difficulty with voices and noisy environment; c to otologic history: tinnitus, otorrhea and nausea; and d to medical history: visual problems, smoke, alcohol, thyroid problems and kidney disease. Conclusion: The findings of this study highlighted, for sensorineural hearing loss, risk factors related to audiologic, otologic and medical history, and to situations involving hearing difficulty.

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

    DEFF Research Database (Denmark)

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

    2010-01-01

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

  12. Mutations in a Novel Isoform of TRIOBP That Encodes a Filamentous-Actin Binding Protein Are Responsible for DFNB28 Recessive Nonsyndromic Hearing Loss

    OpenAIRE

    Shahin, Hashem; Walsh, Tom; Sobe, Tama; Abu Sa’ed, Judeh; Abu Rayan, Amal; Lynch, Eric D.; Lee, Ming K.; Avraham, Karen B.; King, Mary-Claire; Kanaan, Moein

    2005-01-01

    In a large consanguineous Palestinian kindred, we previously mapped DFNB28—a locus associated with recessively inherited, prelingual, profound sensorineural hearing impairment—to chromosome 22q13.1. We report here that mutations in a novel 218-kDa isoform of TRIOBP (TRIO and filamentous actin [F-actin] binding protein) are associated with DFNB28 hearing loss in a total of nine Palestinian families. Two nonsense mutations (R347X and Q581X) truncate the protein, and a potentially deleterious mi...

  13. Vibrant Soundbridge rehabilitation of conductive and mixed hearing loss.

    Science.gov (United States)

    Lüers, Jan-Christoffer; Hüttenbrink, Karl-Bernd

    2014-12-01

    The Vibrant Soundbridge is the world's most often implanted active middle ear implant or hearing aid. During the last few years, the device indications have expanded from sensorineural hearing loss to conductive and mixed hearing loss. Titanium couplers have led to improved contact of the floating mass transducer with the middle ear structures. The resulting hearing gain is satisfying for most patients, but so far, there is no clear audiologic advantage over conventional hearing aids. Currently, the indications are mainly related to intolerance of conventional hearing aids (eg, chronic otitis externa), severe mixed hearing loss with a destructed middle ear and certain medical diagnosis (eg, congenital atresia). Copyright © 2014 Elsevier Inc. All rights reserved.

  14. P300 in individuals with sensorineural hearing loss.

    Science.gov (United States)

    Reis, Ana Cláudia Mirandola Barbosa; Frizzo, Ana Claudia Figueiredo; Isaac, Myriam de Lima; Garcia, Cristiane Fregonesi Dutra; Funayama, Carolina Araújo Rodrigues; Iório, Maria Cecília Martinelli

    2015-01-01

    Behavioral and electrophysiological auditory evaluations contribute to the understanding of the auditory system and of the process of intervention. To study P300 in subjects with severe or profound sensorineural hearing loss. This was a descriptive cross-sectional prospective study. It included 29 individuals of both genders with severe or profound sensorineural hearing loss without other type of disorders, aged 11 to 42 years; all were assessed by behavioral audiological evaluation and auditory evoked potentials. A recording of the P3 wave was obtained in 17 individuals, with a mean latency of 326.97ms and mean amplitude of 3.76V. There were significant differences in latency in relation to age and in amplitude according to degree of hearing loss. There was a statistically significant association of the P300 results with the degrees of hearing loss (p=0.04), with the predominant auditory communication channels (p<0.0001), and with time of hearing loss. P300 can be recorded in individuals with severe and profound congenital sensorineural hearing loss; it may contribute to the understanding of cortical development and is a good predictor of the early intervention outcome. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  15. Hearing loss in children with primary ciliary dyskinesia.

    Science.gov (United States)

    Kreicher, Kathryn L; Schopper, Heather K; Naik, Akash N; Hatch, Jonathan L; Meyer, Ted A

    2018-01-01

    To evaluate the type and severity of hearing impairment in pediatric patients with primary ciliary dyskinesia (PCD) and relate these measures to patient demographics, treatment options, and other otologic factors. A retrospective analysis of children with a diagnosis of PCD, Kartagener's syndrome, or situs inversus in the AudGen Database was conducted. Audiograms were analyzed for type of hearing loss (HL), severity, laterality, and progression. Medical charts were reviewed to identify factors that influence severity and progression of hearing loss. 56 patients met inclusion criteria and 42 patients had HL. 66.6% had bilateral and 33.3% had unilateral loss (70 total ears with HL). Conductive hearing loss (CHL) was the most common type of HL, though 30% of children had some sensorineural component to their hearing loss. 92.9% of children with HL received at least one diagnosis of otitis media, but HL did not improve in the majority (77.8%) of ears in our study regardless of ear tube placement. Slight to mild CHL and all types of otitis media are prevalent among patients with PCD, and some of these children have sensorineural hearing loss (SNHL). All patients diagnosed with situs inversus at birth should be evaluated by an otolaryngologist. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Ablation of whirlin long isoform disrupts the USH2 protein complex and causes vision and hearing loss.

    Directory of Open Access Journals (Sweden)

    Jun Yang

    2010-05-01

    Full Text Available Mutations in whirlin cause either Usher syndrome type II (USH2, a deafness-blindness disorder, or nonsyndromic deafness. The molecular basis for the variable disease expression is unknown. We show here that only the whirlin long isoform, distinct from a short isoform by virtue of having two N-terminal PDZ domains, is expressed in the retina. Both long and short isoforms are expressed in the inner ear. The N-terminal PDZ domains of the long whirlin isoform mediates the formation of a multi-protein complex that includes usherin and VLGR1, both of which are also implicated in USH2. We localized this USH2 protein complex to the periciliary membrane complex (PMC in mouse photoreceptors that appears analogous to the frog periciliary ridge complex. The latter is proposed to play a role in photoreceptor protein trafficking through the connecting cilium. Mice carrying a targeted disruption near the N-terminus of whirlin manifest retinal and inner ear defects, reproducing the clinical features of human USH2 disease. This is in contrast to mice with mutations affecting the C-terminal portion of whirlin in which the phenotype is restricted to the inner ear. In mice lacking any one of the USH2 proteins, the normal localization of all USH2 proteins is disrupted, and there is evidence of protein destabilization. Taken together, our findings provide new insights into the pathogenic mechanism of Usher syndrome. First, the three USH2 proteins exist as an obligatory functional complex in vivo, and loss of one USH2 protein is functionally close to loss of all three. Second, defects in the three USH2 proteins share a common pathogenic process, i.e., disruption of the PMC. Third, whirlin mutations that ablate the N-terminal PDZ domains lead to Usher syndrome, but non-syndromic hearing loss will result if they are spared.

  17. Sensorineural and conductive hearing loss in infants diagnosed in the program of universal newborn hearing screening.

    Science.gov (United States)

    Wroblewska-Seniuk, Katarzyna; Dabrowski, Piotr; Greczka, Grazyna; Szabatowska, Katarzyna; Glowacka, Agata; Szyfter, Witold; Mazela, Jan

    2018-02-01

    The aim of this study was to analyze infants diagnosed with sensorineural or conductive hearing deficit and to identify risk factors associated with these defects. A retrospective analysis of infants diagnosed with hearing deficit based on the database of the universal newborn hearing screening program and medical records of the patients. 27 935 infants were covered by the universal neonatal hearing screening program. 109 (0.39%) were diagnosed with hearing deficit and referred for treatment and rehabilitation. 56 (51.4%) children were diagnosed with conductive, 38 (34.9%) with sensorineural and 15 (13.8%) with mixed type of hearing deficit. Children with sensorineural hearing deficit more frequently suffered from hyperbilirubinemia (p conductive hearing loss were more frequently diagnosed with isolated craniofacial anomalies (p hearing deficit occurred almost 3 times more often bilaterally than unilaterally (p hearing deficit, the difference was not significant. In children with conductive and mixed type of hearing loss the impairment was mainly mild while among those with sensorineural hearing deficit in almost 45% it was severe and profound (p hearing screening test by means of otoacoustic emissions and the final diagnosis of hearing deficit we found that the highest agreement rate was observed in children with sensorineural hearing loss (p hearing deficit was similar in children with sensorineural, conductive and mixed type of hearing loss, only hyperbilirubinemia seemed to predispose to sensorineural hearing deficit and isolated craniofacial malformations seemed to be associated with conductive hearing loss. Sensorineural hearing deficit usually occurred bilaterally and was severe or profound, while conductive and mixed type of hearing deficit were most often of mild degree. Most children with the final diagnosis of sensorineural hearing deficit had positive result of hearing screening by means of otoacoustic emissions. Copyright © 2017 Elsevier B.V. All

  18. Prevalence of hearing loss among primary school children in Mbarara, Uganda.

    Science.gov (United States)

    Basañez, Irving; Nakku, Doreen; Stangl, Susan; Wanna, George B

    2015-12-01

    Hearing loss in children is a common entity worldwide. We examined the prevalence and etiology of hearing loss among primary school children in Mbarara, Uganda. Cross-sectional study in primary school children aged 5-14 was performed to determine the prevalence of hearing loss. Ugandan primary school children were screened for disabling hearing loss (threshold >30dB) and confirmatory audiometry was performed on those who failed the screening. There were 639 children screened. Thirty-five (5.5%) of children screened failed and were referred for further testing. Two children were lost to follow-up. The percentage of children with true hearing loss was 3.1%. The incidence of failed hearing screening and hearing loss in Mbarara, Uganda is similar to other populations. Hearing loss is a significant problem in Uganda and efforts should be made for primary, secondary, and tertiary prevention of hearing loss. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  19. Sudden Sensorineural Hearing Loss; Prognostic Factors

    Directory of Open Access Journals (Sweden)

    Arjun Dass

    2015-09-01

    Full Text Available 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, serial audiometric findings and course of disease were studied.   Results: Subjects presenting late (in older age, having associated vertigo, hypertension and diabetes had a significantly lower rate of recovery.   Conclusion:  Only 60–65% of patients experiencing SSNHL recover within a period of 1 month; this rate is further affected by presence of multiple prognostic indicators.

  20. Decreased postural control in people with moderate hearing loss

    Science.gov (United States)

    Thomas, Ewan; Martines, Francesco; Bianco, Antonino; Messina, Giuseppe; Giustino, Valerio; Zangla, Daniele; Iovane, Angelo; Palma, Antonio

    2018-01-01

    Abstract Balance is a complex process that involves multiple sensory integrations. The auditory, visual, and vestibular systems are the main contributors. Hearing loss or hearing impairment may induce inappropriate postural strategies that could affect balance and therefore increase the risk of falling. The aim of this study was to understand whether hearing loss could influence balance, cervical posture, and muscle activation in the cervical region. Thirteen patients (61 ± 13 years; 161.8 ± 11.0 cm; 70.5 ± 15.9 kg) with moderate hearing loss (Right ear −60 ± 21 dB; Left ear −61 ± 24 dB) underwent: an audiometric examination, a postural examination (with open and closed eyes) through a stabilometric platform, a cervical ROM examination through a head accelerometer, and a sternocleidomastoid electromyography (EMG) examination. A linear regression analysis has shown a regression coefficient (R2) 0.76 and 0.69 between hearing loss and the posturographic parameters, on the sagittal sway, with open and closed eyes, respectively. The combination of frontal and sagittal sway is able to explain up to 84% of the variance of the audiometric assessment. No differences were found between right and left hemibody between the audiometric, posturographic, cervical ROM parameters, and in EMG amplitude. ROM and EMG parameters have not shown any significant associations with hearing loss, for both right and left head rotation. Hearing loss is associated to increased posturographic measures, especially the sagittal sway, underlining a reduced postural control in people with hearing impairments. No association was found between the heads posture and neck activation with hearing loss. Hearing loss may be associated with an increased risk of falls. PMID:29620637

  1. Decreased postural control in people with moderate hearing loss.

    Science.gov (United States)

    Thomas, Ewan; Martines, Francesco; Bianco, Antonino; Messina, Giuseppe; Giustino, Valerio; Zangla, Daniele; Iovane, Angelo; Palma, Antonio

    2018-04-01

    Balance is a complex process that involves multiple sensory integrations. The auditory, visual, and vestibular systems are the main contributors. Hearing loss or hearing impairment may induce inappropriate postural strategies that could affect balance and therefore increase the risk of falling.The aim of this study was to understand whether hearing loss could influence balance, cervical posture, and muscle activation in the cervical region.Thirteen patients (61 ± 13 years; 161.8 ± 11.0 cm; 70.5 ± 15.9 kg) with moderate hearing loss (Right ear -60 ± 21 dB; Left ear -61 ± 24 dB) underwent: an audiometric examination, a postural examination (with open and closed eyes) through a stabilometric platform, a cervical ROM examination through a head accelerometer, and a sternocleidomastoid electromyography (EMG) examination.A linear regression analysis has shown a regression coefficient (R) 0.76 and 0.69 between hearing loss and the posturographic parameters, on the sagittal sway, with open and closed eyes, respectively. The combination of frontal and sagittal sway is able to explain up to 84% of the variance of the audiometric assessment. No differences were found between right and left hemibody between the audiometric, posturographic, cervical ROM parameters, and in EMG amplitude. ROM and EMG parameters have not shown any significant associations with hearing loss, for both right and left head rotation.Hearing loss is associated to increased posturographic measures, especially the sagittal sway, underlining a reduced postural control in people with hearing impairments. No association was found between the heads posture and neck activation with hearing loss. Hearing loss may be associated with an increased risk of falls.

  2. Hearing Loss Signals Need for Diagnosis

    Science.gov (United States)

    ... Products For Consumers Home For Consumers Consumer Updates Hearing Loss Signals Need for Diagnosis Share Tweet Linkedin ... you’re talking loudly? Thinking about ordering a hearing aid or sound amplifier from a magazine or ...

  3. Hearing loss among patients with Turner's syndrome: literature review

    Directory of Open Access Journals (Sweden)

    Cresio Alves

    2014-06-01

    Full Text Available INTRODUCTION: Turner's syndrome (TS is caused by a partial or total deletion of an X chromosome, occurring in 1:2,000 to 1:5,000 live born females. Hearing loss is one of its major clinical manifestations. However, there are few studies investigating this problem. OBJECTIVES: To review the current knowledge regarding the epidemiology, etiology, clinical manifestations and diagnosis of hearing impairment in patients with TS. METHODS: A bibliographic search was performed in the Medline and Lilacs databanks (1980-2012 to identify the main papers associating Turner's syndrome, hearing impairment and its clinical outcomes. CONCLUSIONS: Recurrent otitis media, dysfunction of the Eustachian tube, conductive hearing loss during infancy and sensorineural hearing loss in adolescence are the audiologic disorders more common in ST. The karyotype appears to be important in the hearing loss, with studies demonstrating an increased prevalence in patients with monosomy 45,X or isochromosome 46,i(Xq. Morphologic studies of the cochlea are necessary to help out in the clarifying the etiology of the sensorineural hearing loss.

  4. Bone-anchored hearing devices in children with unilateral conductive hearing loss: a patient-carer perspective.

    Science.gov (United States)

    Banga, Rupan; Doshi, Jayesh; Child, Anne; Pendleton, Elizabeth; Reid, Andrew; McDermott, Ann-Louise

    2013-09-01

    We sought to determine the outcome of implantation of a bone-anchored hearing device in children with unilateral conductive hearing loss. A retrospective case note analysis was used in a tertiary referral pediatric hospital to study 17 consecutive cases of pediatric patients with unilateral conductive hearing loss who were fitted with a bone-anchored hearing device between 2005 and 2010. The average age of the patients at the time of bone-anchored hearing device fitting was 10 years 6 months (range, 6 years 3 months to 16 years). Qualitative subjective outcome measures demonstrated benefit. The vast majority of patients reported improved social and physical functioning and improved quality of life. All 17 patients are currently using their bone-anchored hearing device on a daily basis after a follow-up of 6 months. This study has shown improved quality of life in children with unilateral hearing loss after implantation of their bone-anchored hearing device. There was a high degree of patient satisfaction and improvement in health status reported by children and/or carers. Bone-anchored hearing devices have an important role in the management of children with symptomatic unilateral hearing loss. Perhaps earlier consideration of a bone-anchored hearing device would be appropriate in selected cases.

  5. Comparison of Carina active middle-ear implant with conventional hearing aids for mixed hearing loss.

    Science.gov (United States)

    Savaş, V A; Gündüz, B; Karamert, R; Cevizci, R; Düzlü, M; Tutar, H; Bayazit, Y A

    2016-04-01

    To compare the auditory outcomes of Carina middle-ear implants with those of conventional hearing aids in patients with moderate-to-severe mixed hearing loss. The study comprised nine patients (six males, three females) who underwent middle-ear implantation with Carina fully implantable active middle-ear implants to treat bilateral moderate-to-severe mixed hearing loss. The patients initially used conventional hearing aids and subsequently received the Carina implants. The hearing thresholds with implants and hearing aids were compared. There were no significant differences between: the pre-operative and post-operative air and bone conduction thresholds (p > 0.05), the thresholds with hearing aids and Carina implants (p > 0.05), or the pre-operative (mean, 72.8 ± 19 per cent) and post-operative (mean, 69.9 ± 24 per cent) speech discrimination scores (p > 0.05). One of the patients suffered total sensorineural hearing loss three months following implantation despite an initial 38 dB functional gain. All except one patient showed clinical improvements after implantation according to quality of life questionnaire (Glasgow Benefit Inventory) scores. Acceptance of Carina implants is better than with conventional hearing aids in patients with mixed hearing loss, although both yield similar hearing amplification. Cosmetic reasons appear to be critical for patient acceptance.

  6. Relationship between conductive hearing loss and maxillary constriction.

    Science.gov (United States)

    Peyvandi, A A; Jamilian, A; Moradi, E

    2014-09-01

    To evaluate the relationship between conductive hearing loss and maxillary constriction. A total of 120 people, aged from 7 to 40 years, who were referred to an audiologist when taking out health insurance or for school pre-registration check-up, were selected for this study. A total of 60 participants who had hearing threshold levels greater than 15 dB in both ears were chosen as the conductive hearing loss group. The remaining 60, with normal hearing thresholds of less than 15 dB, were used as the control group. All participants were referred to an orthodontic clinic. Participants who had a posterior crossbite and high palatal vault were considered to suffer from maxillary constriction. There were no significant differences between the sex ratios and mean ages of the groups. However, participants with conductive hearing loss were 3.5 times more likely than controls to suffer from maxillary constriction. Patients who suffer from conductive hearing loss are likely to show a maxillary abnormality when examined by an orthodontist.

  7. Sudden (reversible) sensorineural hearing loss in pregnancy.

    LENUS (Irish Health Repository)

    Kenny, R

    2011-03-01

    Sudden hearing loss directly associated with pregnancy or birth is a little known and rare occurrence. The temporary, unilateral, low-frequency sensorineural hearing loss in this case was reported after the birth of the patient\\'s first child, and again during the third trimester of her second pregnancy.

  8. Sudden Hearing Loss after Rabies Vaccination

    OpenAIRE

    Güçlü, Oğuz; Dereköy, Fevzi Sefa

    2014-01-01

    Background: Sudden hearing loss developing after immunisation is a very rare situation. Rabies is a viral disease characterised by encephalitis and death. Treatment involves active and passive immunisation. Neurologic complications including Guillain-Barre syndrome or facial paralysis are reported in the literature as a side effect after rabies immunisation. Case Report: Sudden hearing loss was detected in an 11 year-old male patient who had taken the medication for rabies immunisatio...

  9. Sudden Hearing Loss after Rabies Vaccination

    OpenAIRE

    Güçlü, Oğuz; Dereköy, Fevzi Sefa

    2013-01-01

    Background: Sudden hearing loss developing after immunisation is a very rare situation. Rabies is a viral disease characterised by encephalitis and death. Treatment involves active and passive immunisation. Neurologic complications including Guillain-Barre syndrome or facial paralysis are reported in the literature as a side effect after rabies immunisation. Case Report: Sudden hearing loss was detected in an 11 year-old male patient who had taken the medication for rabies immunisat...

  10. Neural Alterations in Acquired Age-Related Hearing Loss

    Directory of Open Access Journals (Sweden)

    Raksha Anand Mudar

    2016-06-01

    Full Text Available Hearing loss is one of the most prevalent chronic health conditions in older adults. Growing evidence suggests that hearing loss is associated with reduced cognitive functioning and incident dementia. In this mini-review, we briefly examine literature on anatomical and functional alterations in the brains of adults with acquired age-associated hearing loss, which may underlie the cognitive consequences observed in this population, focusing on studies that have used structural and functional magnetic resonance imaging, diffusion tensor imaging, and event-related electroencephalography. We discuss structural and functional alterations observed in the temporal and frontal cortices and the limbic system. These neural alterations are discussed in the context of common cause, information-degradation, and sensory-deprivation hypotheses, and we suggest possible rehabilitation strategies. Although we are beginning to learn more about changes in neural architecture and functionality related to age-associated hearing loss, much work remains to be done. Understanding the neural alterations will provide objective markers for early identification of neural consequences of age-associated hearing loss and for evaluating benefits of intervention approaches.

  11. Quality of Life and Hearing Eight Years After Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Härkönen, Kati; Kivekäs, Ilkka; Rautiainen, Markus; Kotti, Voitto; Vasama, Juha-Pekka

    2017-04-01

    To explore long-term hearing results, quality of life (QoL), quality of hearing (QoH), work-related stress, tinnitus, and balance problems after idiopathic sudden sensorineural hearing loss (ISSNHL). Cross-sectional study. We reviewed the audiograms of 680 patients with unilateral ISSNHL on average 8 years after the hearing impairment, and then divided the patients into two study groups based on whether their ISSNHL had recovered to normal (pure tone average [PTA] ≤ 30 dB) or not (PTA > 30 dB). The inclusion criteria were a hearing threshold decrease of 30 dB or more in at least three contiguous frequencies occurring within 72 hours in the affected ear and normal hearing in the contralateral ear. Audiograms of 217 patients fulfilled the criteria. We reviewed their medical records; measured present QoL, QoH, and work-related stress with specific questionnaires; and updated the hearing status. Poor hearing outcome after ISSNHL was correlated with age, severity of hearing loss, and vertigo together with ISSNHL. Quality of life and QoH were statistically significantly better in patients with recovered hearing, and the patients had statistically significantly less tinnitus and balance problems. During the 8-year follow-up, the PTA of the affected ear deteriorated on average 7 dB, and healthy ear deteriorated 6 dB. Idiopathic sudden sensorineural hearing loss that failed to recover had a negative impact on long-term QoL and QoH. The hearing deteriorated as a function of age similarly both in the affected and the healthy ear, and there were no differences between the groups. The cumulative recurrence rate for ISSNHL was 3.5%. 4 Laryngoscope, 127:927-931, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  12. Alcohol Use among Students with and without Hearing Loss

    Science.gov (United States)

    Pinquart, Martin; Pfeiffer, Jens P.

    2015-01-01

    We compared alcohol use among adolescents with and without hearing loss. Adolescents with hearing loss reported consuming less alcohol, less binge drinking, fewer episodes of drunkenness, and a higher age at first drunkenness than their hearing peers. Alcohol use did not vary between students who were deaf or hard of hearing or between students…

  13. Menopause and postmenopausal hormone therapy and risk of hearing loss.

    Science.gov (United States)

    Curhan, Sharon G; Eliassen, A Heather; Eavey, Roland D; Wang, Molin; Lin, Brian M; Curhan, Gary C

    2017-09-01

    Menopause may be a risk factor for hearing loss, and postmenopausal hormone therapy (HT) has been proposed to slow hearing decline; however, there are no large prospective studies. We prospectively examined the independent relations between menopause and postmenopausal HT and risk of self-reported hearing loss. Prospective cohort study among 80,972 women in the Nurses' Health Study II, baseline age 27 to 44 years, followed from 1991 to 2013. Baseline and updated information was obtained from detailed validated biennial questionnaires. Cox proportional-hazards regression models were used to examine independent associations between menopausal status and postmenopausal HT and risk of hearing loss. After 1,410,928 person-years of follow-up, 18,558 cases of hearing loss were reported. There was no significant overall association between menopausal status, natural or surgical, and risk of hearing loss. Older age at natural menopause was associated with higher risk. The multivariable-adjusted relative risk of hearing loss among women who underwent natural menopause at age 50+ years compared with those aged less than 50 years was 1.10 (95% confidence interval [CI] 1.03, 1.17). Among postmenopausal women, oral HT (estrogen therapy or estrogen plus progestogen therapy) was associated with higher risk of hearing loss, and longer duration of use was associated with higher risk (P trend menopause and longer duration of postmenopausal HT are associated with higher risk of hearing loss.

  14. Factors associated with hearing loss in a normal-hearing guinea pig model of Hybrid cochlear implants.

    Science.gov (United States)

    Tanaka, Chiemi; Nguyen-Huynh, Anh; Loera, Katherine; Stark, Gemaine; Reiss, Lina

    2014-10-01

    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

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

    Science.gov (United States)

    Schuurbiers, Jasper; Dingemanse, Gertjan; Metselaar, Mick

    2017-12-01

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

  16. Predicting hearing thresholds and occupational hearing loss with multiple-frequency auditory steady-state responses.

    Science.gov (United States)

    Hsu, Ruey-Fen; Ho, Chi-Kung; Lu, Sheng-Nan; Chen, Shun-Sheng

    2010-10-01

    An objective investigation is needed to verify the existence and severity of hearing impairments resulting from work-related, noise-induced hearing loss in arbitration of medicolegal aspects. We investigated the accuracy of multiple-frequency auditory steady-state responses (Mf-ASSRs) between subjects with sensorineural hearing loss (SNHL) with and without occupational noise exposure. Cross-sectional study. Tertiary referral medical centre. Pure-tone audiometry and Mf-ASSRs were recorded in 88 subjects (34 patients had occupational noise-induced hearing loss [NIHL], 36 patients had SNHL without noise exposure, and 18 volunteers were normal controls). Inter- and intragroup comparisons were made. A predicting equation was derived using multiple linear regression analysis. ASSRs and pure-tone thresholds (PTTs) showed a strong correlation for all subjects (r = .77 ≈ .94). The relationship is demonstrated by the equationThe differences between the ASSR and PTT were significantly higher for the NIHL group than for the subjects with non-noise-induced SNHL (p tool for objectively evaluating hearing thresholds. Predictive value may be lower in subjects with occupational hearing loss. Regardless of carrier frequencies, the severity of hearing loss affects the steady-state response. Moreover, the ASSR may assist in detecting noise-induced injury of the auditory pathway. A multiple linear regression equation to accurately predict thresholds was shown that takes into consideration all effect factors.

  17. Hearing testing in the U.S. Department of Defense: Potential impact on Veterans Affairs hearing loss disability awards.

    Science.gov (United States)

    Nelson, J T; Swan, A A; Swiger, B; Packer, M; Pugh, M J

    2017-06-01

    Hearing loss is the second most common disability awarded by the U.S. Department of Veterans Affairs (VA) to former members of the U.S. uniformed services. Hearing readiness and conservation practices differ among the four largest uniformed military services (Air Force, Army, Marine Corps, and Navy). Utilizing a data set consisting of all hearing loss claims submitted to the VA from fiscal years 2003-2013, we examined characteristics of veterans submitting claims within one year of separation from military service. Our results indicate that having a hearing loss disability claim granted was significantly more likely for men, individuals over the age of 26 years at the time of the claim, individuals most recently serving in the U.S. Army, and those with at least one hearing loss diagnosis. Importantly, individuals with at least one test record in the Defense Occupational and Environmental Health Readiness System-Hearing Conservation (DOEHRS-HC) system were significantly less likely to have a hearing loss disability claim granted by the VA. Within the DOEHRS-HC cohort, those with at least one threshold shift or clinical hearing loss diagnosis while on active duty were more than two and three times more likely to have a hearing loss disability claim granted, respectively. These findings indicate that an established history of reduced hearing ability while on active duty was associated with a significantly increased likelihood of an approved hearing loss disability claim relative to VA claims without such a history. Further, our results show a persistent decreased rate of hearing loss disability awards overall. These findings support increased inclusion of personnel in DoD hearing readiness and conservation programs to reduce VA hearing loss disability awards. Copyright © 2016 Elsevier B.V. All rights reserved.

  18. Applications of direct-to-consumer hearing devices for adults with hearing loss: a review

    Science.gov (United States)

    Manchaiah, Vinaya; Taylor, Brian; Dockens, Ashley L; Tran, Nicole R; Lane, Kayla; Castle, Mariana; Grover, Vibhu

    2017-01-01

    Background This systematic literature review is aimed at investigating applications of direct-to-consumer hearing devices for adults with hearing loss. This review discusses three categories of direct-to-consumer hearing devices: 1) personal sound amplification products (PSAPs), 2) direct-mail hearing aids, and 3) over-the-counter (OTC) hearing aids. Method A literature review was conducted using EBSCOhost and included the databases CINAHL, MEDLINE, and PsycINFO. After applying prior agreed inclusion and exclusion criteria, 13 reports were included in the review. Results Included studies fell into three domains: 1) electroacoustic characteristics, 2) consumer surveys, and 3) outcome evaluations. Electroacoustic characteristics of these devices vary significantly with some meeting the stringent acoustic criteria used for hearing aids, while others producing dangerous output levels (ie, over 120-dB sound pressure level). Low-end (or low-cost) devices were typically poor in acoustic quality and did not meet gain levels necessary for most adult and elderly hearing loss patterns (eg, presbycusis), especially in high frequencies. Despite direct-mail hearing aids and PSAPs being associated with lower satisfaction when compared to hearing aids purchased through hearing health care professionals, consumer surveys suggest that 5%–19% of people with hearing loss purchase hearing aids through direct-mail or online. Studies on outcome evaluation suggest positive outcomes of OTC devices in the elderly population. Of note, OTC outcomes appear better when a hearing health care professional supports these users. Conclusion While some direct-to-consumer hearing devices have the capability to produce adverse effects due to production of dangerously high sound levels and internal noise, the existing literature suggests that there are potential benefits of these devices. Research of direct-to-consumer hearing devices is limited, and current published studies are of weak quality. Much

  19. The contribution of family history to hearing loss in an older population.

    Science.gov (United States)

    McMahon, Catherine M; Kifley, Annette; Rochtchina, Elena; Newall, Philip; Mitchell, Paul

    2008-08-01

    Although it has been well established that the prevalence of and severity of hearing loss increase with age, the contribution of familial factors to age-related hearing loss cannot be quantified. This is largely because hearing loss in older people has both genetic and environmental contributions. As environmental factors play an increasing role with age, it is difficult to delineate the separate contribution of genetic factors to age-related hearing loss. In a population-based survey of hearing loss in a representative older Australian community, we attempted to overcome this using logistic regression analysis, accounting for known factors associated with hearing loss including age, sex, noise exposure at work, diabetes, and current smoking. We tested hearing thresholds using pure tone audiometry and used a forced choice questionnaire to determine the nature of family history in a population of individuals aged 50 yrs or older in a defined region, west of Sydney, Australia (N = 2669). We compared the characteristics of participants with and without family history of hearing loss. Of those reporting a positive family history, we compared subgroups for age, gender and severity of hearing loss, and trends by the severity of hearing loss. Logistic regression was used to obtain odds ratios (ORs) with 95% confidence intervals (CIs) that compared the chances of having hearing loss in participants with and without family history, after adjusting for other factors known associated with hearing loss. Our findings indicate that family history was most strongly associated with moderate to severe age-related hearing loss. We found a strong association between maternal family history of hearing loss and moderate to severe hearing loss in women (adjusted OR 3.0; 95% CI 1.6-5.6 in women with without a maternal history). Paternal family history of hearing loss was also significantly, though less strongly, associated with moderate-severe hearing loss in men (adjusted OR 2.0; CI 1

  20. Analysis of risk factors associated with unilateral hearing loss in children who initially passed newborn hearing screening.

    Science.gov (United States)

    Appelbaum, Eric N; Howell, Jessica B; Chapman, Derek; Pandya, Arti; Dodson, Kelley M

    2018-03-01

    To analyze 2007 Joint Committee on Infant Hearing (JCIH) risk factors in children with confirmed unilateral hearing loss (UHL) who initially passed newborn hearing screening. Retrospective record review of 16,108 infants who passed newborn hearing screening but had one or more JCIH risk factors prompting subsequent follow-up through the universal newborn hearing screening (UNHS) program in Virginia from 2010 to 2012. The study was reviewed and qualified as exempt by the Virginia Commonwealth University Institutional Review Board (IRB) and the Virginia Department of Health. Over the 2-year study period, 14896 (4.9% of total births) children passed UNHS but had the presence of one or more JCIH risk factor. Ultimately, we identified 121 babies from this group with confirmed hearing loss (0.7%), with 48 babies (0.2%) showing UHL. The most common risk factors associated with the development of confirmed UHL after passing the initial screen were neonatal indicators, craniofacial anomalies, family history, and stigmata of syndrome associated with hearing loss. Neonatal indicators and craniofacial anomalies were the categories most often found in children with confirmed unilateral hearing loss who initially passed their newborn hearing screen. While neonatal indicators were also the most common associated risk factor in all hearing loss, craniofacial abnormalities are relatively more common in children with UHL who initially passed newborn hearing screening. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted. Copyright © 2018 Elsevier B.V. All rights reserved.

  1. Noise-Induced Hearing Loss - A Preventable Disease?

    DEFF Research Database (Denmark)

    Frederiksen, Thomas W.; Ramlau-Hansen, Cecilia H.; Stokholm, Zara A.

    2017-01-01

    AIMS: To survey current, Danish industrial noise levels and the use of hearing protection devices (HPD) over a 10-year period and to characterise the association between occupational noise and hearing threshold shift in the same period. Furthermore, the risk of hearing loss among the baseline and...

  2. Binaural integration: a challenge to overcome for children with hearing loss.

    Science.gov (United States)

    Gordon, Karen A; Cushing, Sharon L; Easwar, Vijayalakshmi; Polonenko, Melissa J; Papsin, Blake C

    2017-12-01

    Access to bilateral hearing can be provided to children with hearing loss by fitting appropriate hearing devices to each affected ear. It is not clear, however, that bilateral input is properly integrated through hearing devices to promote binaural hearing. In the present review, we examine evidence indicating that abnormal binaural hearing continues to be a challenge for children with hearing loss despite early access to bilateral input. Behavioral responses and electrophysiological data in children, combined with data from developing animal models, reveal that deafness in early life disrupts binaural hearing and that present hearing devices are unable to reverse these changes and/or promote expected development. Possible limitations of hearing devices include mismatches in binaural place, level, and timing of stimulation. Such mismatches could be common in children with hearing loss. One potential solution is to modify present device fitting beyond providing audibility to each ear by implementing binaural fitting targets. Efforts to better integrate bilateral input could improve spatial hearing in children with hearing loss.

  3. Outcomes for conservative management of traumatic conductive hearing loss.

    Science.gov (United States)

    Grant, Jonathan R; Arganbright, Jill; Friedland, David R

    2008-04-01

    To evaluate the natural history of traumatic conductive hearing loss. Retrospective chart review. Otologic and audiometric evaluations of patients in the early posttraumatic phase were compared with evaluations at follow-up. Assessment included etiologies of trauma, classification of hearing loss, factors causing conductive loss, and analyses of changes in air-bone gaps, pure-tone averages and hearing loss class. There were 45 patients, representing 47 ears, with sufficient initial and follow-up documentation to analyze the natural history of traumatic conductive hearing loss. Overall, 77% of ears showed an improvement in pure-tone averages without surgical intervention. Air-bone gaps closed from an average of 24.8 +/- 12.1 to 13.2 +/- 11.1 dB. Only 11% of ears demonstrated a decrease in pure-tone averages, and 12% showed no change in thresholds. All forms of injury contributing to the conductive hearing loss had good outcomes. Specifically, tympanic membrane perforations showed final air-bone gaps of 14.9 +/- 11.2 dB; cases of hemotympanum had final air-bone gaps of 10.0 +/- 8.1 dB; and suspected ossicular chain disruptions had final air-bone gaps of 13.9 +/- 12.3 dB. Only 5 of 47 ears ultimately required surgical intervention for persistent pathology. Patients with all forms of traumatic conductive hearing loss can be initially managed conservatively. Even suspected ossicular chain disruptions have a high rate of spontaneous reparation. Surgical intervention for perforation or conductive hearing loss should be undertaken in the rare cases when these conditions persist greater than 6 months.

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

    Science.gov (United States)

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

    2015-12-30

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

  5. Professionals with hearing loss: maintaining that competitive edge.

    Science.gov (United States)

    Tye-Murray, Nancy; Spry, Jacqueline L; Mauzé, Elizabeth

    2009-08-01

    The goals of this investigation were to gauge how hearing loss affects the self-perceived job performance and psycho-emotional status of professionals in the workforce and to develop a profile of their aural rehabilitation needs. Forty-eight participants who had at least a high school education and who hold salaried positions participated in one of seven focus groups. Participants first answered questions about a hypothetical executive who had hearing loss and considered how she might react to various communication issues. They then addressed questions about their own work-related predicaments. The sessions were audiovideo recorded and later transcribed for analysis. Unlike workers who have occupational hearing loss, the professionals in this investigation seem not to experience an inordinate degree of stigmatization in their workplaces, although most believe that hearing loss has negatively affected their job performance. Some of the participants believe that they have lost their "competitive edge," and some believe that they have been denied promotions because of hearing loss. However, most report that they have overcome their hearing-related difficulties by various means, and many have developed a determination and stamina to remain active in the workforce. The majority of the participants seemed to be unfamiliar with the Americans with Disability Act, Public Law 101-336. The overriding theme to emerge is that professionals desire to maintain their competency to perform their jobs and will do what they have to do to "get the job done." The situations of professionals who have hearing loss can be modeled, with a central theme of maintaining job competency or a competitive edge. It is hypothesized that five factors affect professionals' abilities to continue their optimal work performance in the face of hearing loss: (a) self-concept and sense of internal locus of control, (b) use of hearing assistive technology, (c) supervisor's and co-workers' perceptions and

  6. Prevalence of noise-induced hearing loss in drivers

    Directory of Open Access Journals (Sweden)

    Andréa Cintra Lopes1, , , ,

    2012-01-01

    Full Text Available Introduction: Work-related hearing loss is one of the most common occupational illness progresses over the years of noise exposure associated with the work environment, may cause damage to undertake physical activity, the individual's physiological and mental besides causing hearing damage irreversible interfering with communication and quality of life. With high prevalence of male evaluates which is the second leading cause of hearing loss. Since there is no medical treatment for this type of hearing loss, it is evident the importance of preventive and conferences aimed at preserving hearing and health as a whole. Objective: To assess the prevalence of hearing loss in audiometry admission of drivers. Methods: Retrospective study. By 76 charts of professional drivers in leased transport companies. We analyzed data from specific interview and pure tone audiometry. Results: The prevalence of abnormal tests was 22.36% with the lowest thresholds for tritonal average of 3,000, 4,000 and 6,000 Hz. The higher the age, the higher thresholds. Conclusion: This study has highlighted the occurrence of hearing in the absence of complaints. Considering that PAIR is preventable, justifies the importance of coordinated and multidisciplinary involving not only health teams and safety, but also the institutions involved in preserving the health of workers, as the team SESMET, unions or prosecutors.

  7. The relationship between neonatal hyperbilirubinemia and sensorineural hearing loss.

    Science.gov (United States)

    Corujo-Santana, Cándido; Falcón-González, Juan Carlos; Borkoski-Barreiro, Silvia Andrea; Pérez-Plasencia, Daniel; Ramos-Macías, Ángel

    2015-01-01

    Severe jaundice that requires exchange transfusion has become a relatively rare situation today. About 60% of full term neonates and 80% of premature ones will suffer from jaundice within the first week of life. Hyperbilirubinemia at birth is a risk factor associated with hearing loss that is usually further linked to other factors that might have an effect on hearing synergistically. This study aimed to identify the relationship between hyperbilirubinemia at birth as a risk factor for sensorineural hearing loss in children born at Complejo Hospitalario Universitario Insular Materno-Infantil de Gran Canaria, in the 2007-2011 period. This was a retrospective study of 796 newborns that had hyperbilirubinemia at birth, using transient evoked otoacoustic emissions and evoked auditory brainstem response. Hundred eighty-five newborns (23.24%) were referred for evoked auditory brainstem response. Hearing loss was diagnosed for 35 (4.39%): 18 neonates (51.43%) with conductive hearing loss and 17 (48.57%) with sensorineural hearing loss, 3 of which were diagnosed as bilateral profound hearing loss. Half of the children had other risk factors associated, the most frequent being exposure to ototoxic medications. The percentage of children diagnosed with sensorineural hearing loss that suffered hyperbilirubinemia at birth is higher than for the general population. Of those diagnosed, none had levels of indirect bilirubin≥20mg/dl, only 47% had hyperbilirubinemia at birth as a risk factor and 53% had another auditory risk factor associated. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

  8. Statistical model for prediction of hearing loss in patients receiving cisplatin chemotherapy.

    Science.gov (United States)

    Johnson, Andrew; Tarima, Sergey; Wong, Stuart; Friedland, David R; Runge, Christina L

    2013-03-01

    This statistical model might be used to predict cisplatin-induced hearing loss, particularly in patients undergoing concomitant radiotherapy. To create a statistical model based on pretreatment hearing thresholds to provide an individual probability for hearing loss from cisplatin therapy and, secondarily, to investigate the use of hearing classification schemes as predictive tools for hearing loss. Retrospective case-control study. Tertiary care medical center. A total of 112 subjects receiving chemotherapy and audiometric evaluation were evaluated for the study. Of these subjects, 31 met inclusion criteria for analysis. The primary outcome measurement was a statistical model providing the probability of hearing loss following the use of cisplatin chemotherapy. Fifteen of the 31 subjects had significant hearing loss following cisplatin chemotherapy. American Academy of Otolaryngology-Head and Neck Society and Gardner-Robertson hearing classification schemes revealed little change in hearing grades between pretreatment and posttreatment evaluations for subjects with or without hearing loss. The Chang hearing classification scheme could effectively be used as a predictive tool in determining hearing loss with a sensitivity of 73.33%. Pretreatment hearing thresholds were used to generate a statistical model, based on quadratic approximation, to predict hearing loss (C statistic = 0.842, cross-validated = 0.835). The validity of the model improved when only subjects who received concurrent head and neck irradiation were included in the analysis (C statistic = 0.91). A calculated cutoff of 0.45 for predicted probability has a cross-validated sensitivity and specificity of 80%. Pretreatment hearing thresholds can be used as a predictive tool for cisplatin-induced hearing loss, particularly with concomitant radiotherapy.

  9. Interventions to prevent occupational noise-induced hearing loss.

    Science.gov (United States)

    Tikka, Christina; Verbeek, Jos H; Kateman, Erik; Morata, Thais C; Dreschler, Wouter A; Ferrite, Silvia

    2017-07-07

    This is the second update of a Cochrane Review originally published in 2009. Millions of workers worldwide are exposed to noise levels that increase their risk of hearing disorders. There is uncertainty about the effectiveness of hearing loss prevention interventions. To assess the effectiveness of non-pharmaceutical interventions for preventing occupational noise exposure or occupational hearing loss compared to no intervention or alternative interventions. We searched the CENTRAL; PubMed; Embase; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; and OSH UPDATE to 3 October 2016. We included randomised controlled trials (RCT), controlled before-after studies (CBA) and interrupted time-series (ITS) of non-clinical interventions under field conditions among workers to prevent or reduce noise exposure and hearing loss. We also collected uncontrolled case studies of engineering controls about the effect on noise exposure. Two authors independently assessed study eligibility and risk of bias and extracted data. We categorised interventions as engineering controls, administrative controls, personal hearing protection devices, and hearing surveillance. We included 29 studies. One study evaluated legislation to reduce noise exposure in a 12-year time-series analysis but there were no controlled studies on engineering controls for noise exposure. Eleven studies with 3725 participants evaluated effects of personal hearing protection devices and 17 studies with 84,028 participants evaluated effects of hearing loss prevention programmes (HLPPs). Effects on noise exposure Engineering interventions following legislationOne ITS study found that new legislation in the mining industry reduced the median personal noise exposure dose in underground coal mining by 27.7 percentage points (95% confidence interval (CI) -36.1 to -19.3 percentage points) immediately after the implementation of stricter legislation. This roughly translates to a 4.5 dB(A) decrease in

  10. Double Jeopardy: Hearing Loss and Tinnitus Among Noise-Exposed Workers.

    Science.gov (United States)

    Hong, OiSaeng; Chin, Dal Lae; Phelps, Stephanie; Joo, Yoonmee

    2016-06-01

    The purpose of this study was to determine the prevalence and characteristics of tinnitus and assess the relationship between tinnitus and hearing loss among firefighters and operating engineers, who are exposed to noise on-the-job. The study analyzed existing data from two different populations (154 firefighters and 769 operating engineers) who completed a survey and audiometric tests as part of a hearing loss prevention intervention study. Approximately 40% of both groups reported tinnitus; 34% of firefighters and 59% of operating engineers showed hearing loss at noise-sensitive frequencies (4 kHz and 6 kHz). Firefighters with high frequency hearing loss (odds ratio [OR] = 2.31; 95% confidence interval [CI] = [1.05, 5.11]) and those with perceived impaired hearing status (OR = 3.53; 95% CI = [1.27, 9.80]) were significantly more likely to report tinnitus. Similarly, operating engineers who had hearing loss at both low (OR = 2.10; 95% CI = [1.40, 3.15]) and high frequencies (OR = 2.00; 95% CI = [1.37, 2.90]), and perceived impaired hearing status (OR = 2.17; 95% CI = [1.55, 3.05]) were twice as likely to report tinnitus. This study demonstrated that tinnitus is a considerable problem for noise-exposed workers. Workers with hearing loss demonstrated significantly higher rates of tinnitus. Comprehensive workplace hearing conservation programs should include tinnitus management for noise-exposed workers, along with other key elements such as noise control and hearing protection. © 2016 The Author(s).

  11. Disabling Hearing Loss In Two Industries In Lagos, Nigeria ...

    African Journals Online (AJOL)

    Hearing loss was significantly associated with age older than 35 years (p= 0.009) and duration of employment greater than 5 years ( p = 0.02 ). Conclusion: Disabling hearing loss was significantly higher in the noise – exposed subjects and indicates the need for a hearing conservation programme amongst these workers.

  12. Age of diagnosis for congenital hearing loss at Universitas Hospital ...

    African Journals Online (AJOL)

    Background. Congenital hearing loss affects 3 - 6/1 000 children worldwide. The benefits of early identification of hearing loss and early intervention have been clearly established. There are no previous studies reporting on the age of diagnosis of congenital hearing loss in the Free State province. Objectives. To determine ...

  13. [Acute hearing loss and tinnitus caused by amplified recreational music].

    Science.gov (United States)

    Metternich, F U; Brusis, T

    1999-11-01

    Hearing loss resulting from exposure to permanent or repeated amplified music in professional musicians and music consumers is described in literature. The risk of hearing loss does not exist only after prolonged exposure to music. Short-term exposure to very high sound levels, for example in concerts, can also cause hearing loss and tinnitus. The retrospective study includes 24 patients who required rheologic therapy between 1994 and 1997 due to a music related acoustic trauma. The type, intensity, and length of music exposure as well as the distance and the position to the source of noise were examined. The type of hearing damage and its development during rheological treatment was studied by pure-tone audiometry. In the majority of examined patients (67%) the hearing loss developed on the basis of one-time exposure at a rock concert or pop concert, followed by hearing loss from attending discotheques (17%) or parties (12%), and music exposure from personal cassette players (4%). The majority of patients showed a maximum hearing loss of 40-60 dB (A) in a frequency between 3 kHz and 4 kHz. Pure-tone audiometry in 58% of the patients exhibited a unilateral threshold in a frequency between 3 kHz and 4 kHz combined with ipsilateral tinnitus of the same frequency. Twenty-one percent of the patients showed a symmetric bilateral threshold and tinnitus between 3 kHz and 4 kHz. In 8% there was a unilateral tinnitus, and in 13% a bilateral tinnitus without any hearing loss. All patients improved their hearing loss during rheologic treatment. Improvement in the tinnitus was only achieved in 33% of the examined cases. The risk of permanent hearing loss resulting from short-term exposure to amplified music is low compared to the risk of continuous tinnitus. Given the lack of acceptance of personal ear protectors, the risk of acute hearing damage due to amplified music could be reduced by avoiding the immediate proximity to the speakers.

  14. An Introduction to the Outcomes of Children with Hearing Loss Study.

    Science.gov (United States)

    Moeller, Mary Pat; Tomblin, J Bruce

    2015-01-01

    The landscape of service provision for young children with hearing loss has shifted in recent years as a result of newborn hearing screening and the early provision of interventions, including hearing technologies. It is expected that early service provision will minimize or prevent linguistic delays that typically accompany untreated permanent childhood hearing loss. The post-newborn hearing screening era has seen a resurgence of interest in empirically examining the outcomes of children with hearing loss to determine if service innovations have resulted in expected improvements in children's functioning. The Outcomes of Children with Hearing Loss (OCHL) project was among these recent research efforts, and this introductory article provides background in the form of literature review and theoretical discussion to support the goals of the study. The Outcomes of Children with Hearing Loss project was designed to examine the language and auditory outcomes of infants and preschool-age children with permanent, bilateral, mild-to-severe hearing loss, and to identify factors that moderate the relationship between hearing loss and longitudinal outcomes. The authors propose that children who are hard of hearing experience limitations in access to linguistic input, which lead to a decrease in uptake of language exposure and an overall reduction in linguistic experience. The authors explore this hypothesis in relation to three primary factors that are proposed to influence children's access to linguistic input: aided audibility, duration and consistency of hearing aid use, and characteristics of caregiver input.

  15. Newborn hearing screening program: association between hearing loss and risk factors

    OpenAIRE

    Pereira, Priscila Karla Santana; Martins, Adriana de Souza; Vieira, Márcia Ribeiro; Azevedo, Marisa Frasson de

    2007-01-01

    BACKGROUND: hearing loss in newborns. Aim: to verify the prevalence of auditory alterations in newborns of Hospital São Paulo (hospital), observing if there are any correlations with the following variables: birth weight, gestational age, relation weight/gestational age and risk factors for hearing loss. METHOD: A retrospective analysis of the hospital records of 1696 newborns; 648 records of preterm infants and 1048 records of infants born at term. All of the infants had been submitted to an...

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

    Science.gov (United States)

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

    2012-10-01

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

  17. POU4F3 mutation screening in Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis identified novel variants associated with autosomal dominant hearing loss.

    Directory of Open Access Journals (Sweden)

    Tomohiro Kitano

    Full Text Available A variant in a transcription factor gene, POU4F3, is responsible for autosomal dominant nonsyndromic hereditary hearing loss, DFNA15. To date, 14 variants, including a whole deletion of POU4F3, have been reported to cause HL in various ethnic groups. In the present study, genetic screening for POU4F3 variants was carried out for a large series of Japanese hearing loss (HL patients to clarify the prevalence and clinical characteristics of DFNA15 in the Japanese population. Massively parallel DNA sequencing of 68 target candidate genes was utilized in 2,549 unrelated Japanese HL patients (probands to identify genomic variations responsible for HL. The detailed clinical features in patients with POU4F3 variants were collected from medical charts and analyzed. Novel 12 POU4F3 likely pathogenic variants (six missense variants, three frameshift variants, and three nonsense variants were successfully identified in 15 probands (2.5% among 602 families exhibiting autosomal dominant HL, whereas no variants were detected in the other 1,947 probands with autosomal recessive or inheritance pattern unknown HL. To obtain the audiovestibular configuration of the patients harboring POU4F3 variants, we collected audiograms and vestibular symptoms of the probands and their affected family members. Audiovestibular phenotypes in a total of 24 individuals from the 15 families possessing variants were characterized by progressive HL, with a large variation in the onset age and severity with or without vestibular symptoms observed. Pure-tone audiograms indicated the most prevalent configuration as mid-frequency HL type followed by high-frequency HL type, with asymmetry observed in approximately 20% of affected individuals. Analysis of the relationship between age and pure-tone average suggested that individuals with truncating variants showed earlier onset and slower progression of HL than did those with non-truncating variants. The present study showed that variants

  18. Hearing loss and enlarged internal auditory canal in children.

    Science.gov (United States)

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

    2014-01-01

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

  19. Targeted surveillance for postnatal hearing loss: a program evaluation.

    Science.gov (United States)

    Beswick, Rachael; Driscoll, Carlie; Kei, Joseph; Glennon, Shirley

    2012-07-01

    The importance of monitoring hearing throughout early childhood cannot be understated. However, there is a lack of evidence available regarding the most effective method of monitoring hearing following the newborn screen. The goal of this study was to describe a targeted surveillance program using a risk factor registry to identify children with a postnatal hearing loss. All children who were born in Queensland, Australia between September 2004 and December 2009, received a bilateral 'pass' on newborn hearing screening, and had at least one risk factor, were referred for targeted surveillance and were included in this study. The cohort was assessed throughout early childhood in accordance with Queensland's diagnostic assessment protocols. During the study period, 7320 (2.8% of 261,328) children were referred for targeted surveillance, of which 56 were identified with a postnatal hearing loss (0.77%). Of these, half (50.0%) were identified with a mild hearing loss, and 64.3% were identified with a sensorineural hearing loss. In regards to risk factors, syndrome, craniofacial anomalies, and severe asphyxia had the highest yield of positive cases of postnatal hearing loss for children referred for targeted surveillance, whereas, low birth weight, bacterial meningitis, and professional concern had a particularly low yield. Limitations of the targeted surveillance program were noted and include: (1) a lost contact rate of 32.4%; (2) delays in first surveillance assessment; (3) a large number of children who required on-going monitoring; and (4) extensive diagnostic assessments were completed on children with normal hearing. Examination of the lost contact rate revealed indigenous children were more likely to be documented as lost contact. In addition, children with one risk factor only were significantly more likely to not attend a surveillance appointment. Positive cases of postnatal hearing loss were detected through the targeted surveillance program. However, the

  20. CORRELATION BETWEEN TYMPANIC MEMBRANE PERFORATION AND HEARING LOSS

    Directory of Open Access Journals (Sweden)

    Lidija RISTOVSKA

    2016-04-01

    Full Text Available Introduction: Perforation of the tympanic membrane primarily results from middle ear infections, trauma or iatrogenic causes. The perforation causes conductive hearing loss by reducing the surface area available for sound transmission to the ossicular chain. Objective: The objective was to analyze the characteristics of tympanic membrane perforations in relation to hearing loss and to determine the type and degree of hearing loss. Materials and methods: We analyzed audiometric, otoscopic findings and medical reports of 218 patients, 114 males (52.3% and 104 females (47.7%, aged 9 to 75 years (mean age of 47.9 years, examined during the period of November 2012 to October 2015. For statistical data analysis we used Chi-square test with level of significance p<0.05. Results: Most of the patients had unilateral perforations (89% with right ear predominance and involvement of two quadrants of pars tensa (37.2%. Mean air-bone gap was 23.9 dB. The largest air-bone gap was at frequency of 250 Hz. Most of the patients (73.1% had mixed hearing loss (p=0.032, and average hearing thresholds from 21 to 40 dB. Conclusion: Mean air-bone gap is largest at the lower frequencies, and decreases as frequency increases. Size of the perforation has effect on hearing loss. Mean air-bone gap increases with increasing size of the perforation. There is no big difference between the mean air-bone gap in posterior versus anterior perforations.

  1. Conductive hearing loss and middle ear pathology in young infants referred through a newborn universal hearing screening program in Australia.

    Science.gov (United States)

    Aithal, Sreedevi; Aithal, Venkatesh; Kei, Joseph; Driscoll, Carlie

    2012-10-01

    Although newborn hearing screening programs have been introduced in most states in Australia, the prevalence of conductive hearing loss and middle ear pathology in the infants referred through these programs is not known. This study was designed to (1) evaluate the prevalence of conductive hearing loss and middle ear pathology in infants referred by a newborn hearing screening program in north Queensland, (2) compare prevalence rates of conductive hearing loss and middle ear pathology in indigenous and nonindigenous infants, and (3) review the outcomes of those infants diagnosed with conductive hearing loss and middle ear pathology. Retrospective chart review of infants referred to the Audiology Department of The Townsville Hospital was conducted. Chart review of 234 infants referred for one or both ears from a newborn hearing screening program in north Queensland was conducted. A total of 211 infants attended the diagnostic appointment. Review appointments to monitor hearing status were completed for 46 infants with middle ear pathology or conductive hearing loss. Diagnosis of hearing impairment was made using an age-appropriate battery of audiological tests. Results were analyzed for both initial and review appointments. Mean age at initial diagnostic assessment was 47.5 days (SD = 31.3). Of the 69 infants with middle ear pathology during initial diagnostic assessment, 18 had middle ear pathology with normal hearing, 47 had conductive hearing loss, and 4 had mixed hearing loss. Prevalence of conductive hearing loss in the newborns was 2.97 per 1,000 while prevalence of middle ear pathology (with or without conductive hearing loss) was 4.36 per 1,000. Indigenous Australians or Aboriginal and Torres Strait Islander (ATSI) infants had a significantly higher prevalence of conductive hearing loss and middle ear pathology than non-ATSI infants (35.19 and 44.45% vs 17.83 and 28.66%, respectively). ATSI infants also showed poor resolution of conductive hearing loss

  2. Prevalence of hearing loss and accuracy of self-report among factory workers.

    Science.gov (United States)

    McCullagh, Marjorie C; Raymond, Delbert; Kerr, Madeleine J; Lusk, Sally L

    2011-01-01

    Noise represents one of the most common occupational health hazards. A Healthy People 2020 objective aims to reduce hearing loss in the noise-exposed public. The purpose of this study was to describe and compare perceived and measured hearing, and to determine the prevalence of hearing loss among a group of factory workers. Data collected as part of an intervention study promoting hearing protector use among workers at an automotive factory in the Midwest were used. Plant employees (n=2691) provided information regarding their perceived hearing ability, work role, and other demographics. The relationships among audiograms, a single-item measure of perceived hearing ability, and demographic data were explored using chi-square, McNemar's test, Mann-Whitney U-test, sensitivity, and specificity. The prevalence of hearing loss among noise-exposed factory workers was 42% (where hearing loss was defined as >25 dB loss at the OSHA-recommended frequencies of 2, 3, and 4 kHz in either ear). However, 76% of workers reported their hearing ability as excellent or good. The difference in perceived hearing ability was significant at each tested frequency between those with and without measured hearing loss. Self-reported hearing ability was poorly related to results of audiometry. Although this group of workers was employed in a regulated environment and served by a hearing conservation program, hearing loss was highly prevalent. These findings, together with national prevalence estimates, support the need for evaluation of hearing conservation programs and increased attention to the national goal of reducing adult hearing loss.

  3. Prevalence of hearing loss and accuracy of self-report among factory workers

    Directory of Open Access Journals (Sweden)

    Marjorie C McCullagh

    2011-01-01

    Full Text Available Noise represents one of the most common occupational health hazards. A Healthy People 2020 objective aims to reduce hearing loss in the noise-exposed public. The purpose of this study was to describe and compare perceived and measured hearing, and to determine the prevalence of hearing loss among a group of factory workers. Data collected as part of an intervention study promoting hearing protector use among workers at an automotive factory in the Midwest were used. Plant employees (n=2691 provided information regarding their perceived hearing ability, work role, and other demographics. The relationships among audiograms, a single-item measure of perceived hearing ability, and demographic data were explored using chi-square, McNemar′s test, Mann-Whitney U-test, sensitivity, and specificity. The prevalence of hearing loss among noise-exposed factory workers was 42% (where hearing loss was defined as >25 dB loss at the OSHA-recommended frequencies of 2, 3, and 4 kHz in either ear. However, 76% of workers reported their hearing ability as excellent or good. The difference in perceived hearing ability was significant at each tested frequency between those with and without measured hearing loss. Self-reported hearing ability was poorly related to results of audiometry. Although this group of workers was employed in a regulated environment and served by a hearing conservation program, hearing loss was highly prevalent. These findings, together with national prevalence estimates, support the need for evaluation of hearing conservation programs and increased attention to the national goal of reducing adult hearing loss.

  4. Efficient estimates of cochlear hearing loss parameters in individual listeners

    DEFF Research Database (Denmark)

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

    2013-01-01

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

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

    DEFF Research Database (Denmark)

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

    2010-01-01

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

  6. Teaching Vocabulary to Preschool Children with Hearing Loss

    Science.gov (United States)

    Lund, Emily; Douglas, W. Michael

    2016-01-01

    Despite poor vocabulary outcomes for children with hearing loss, few studies have evaluated the effectiveness of specific vocabulary teaching methods on vocabulary learning for this group. The authors compared three vocabulary instruction conditions with preschool children with hearing loss: (a) explicit, direct instruction; (b) follow-in…

  7. Hearing loss and work participation: a cross-sectional study in Norway.

    Science.gov (United States)

    Svinndal, Elisabeth Vigrestad; Solheim, Jorunn; Rise, Marit By; Jensen, Chris

    2018-04-27

    To study work participation of persons with hearing loss, and associations with hearing disabilities, self-reported workability, fatigue and work accommodation. Cross-sectional internet-based survey. A total of 10,679 persons with hearing loss within working-age were invited to answer the survey, where 3330 answered (35.6%). Degree of hearing loss was associated with low workability, fatigue and work place accommodation, while sick leave was associated with fatigue. Degree of hearing loss was positively associated with being unemployed (p part-time work (p < .01) (often combined with disability benefits) for women. Work place accommodation was more frequently provided among respondents working with sedentary postures, high seniority, long-term sick leave or low workability. Additional unfavourable sensory conditions were associated with decreased employment (p < .001) and workability, and an increase in sick leave (p < .01) and fatigue (p < .001). Hearing loss seemed to influence work participation factors negatively; particularly, for moderate hearing loss and for women, even though the degree of employment was high. A lack of work place accommodation when there was a need for such was found. This implies increased attentiveness towards individual needs concerning the experienced disability a hearing loss may produce. A more frequent use of hearing disability assessment is suggested.

  8. Hearing loss among older construction workers: Updated analyses.

    Science.gov (United States)

    Dement, John; Welch, Laura S; Ringen, Knut; Cranford, Kim; Quinn, Patricia

    2018-04-01

    A prior study of this construction worker population found significant noise-associated hearing loss. This follow-up study included a much larger study population and consideration of additional risk factors. Data included audiometry, clinical chemistry, personal history, and work history. Qualitative exposure metrics for noise and solvents were developed. Analyses compared construction workers to an internal reference group with lower exposures and an external worker population with low noise exposure. Among participants (n = 19 127) an overall prevalence of hearing loss of 58% was observed, with significantly increased prevalence across all construction trades. Construction workers had significantly increased risk of hearing loss compared to reference populations, with increasing risk by work duration. Noise exposure, solvent exposure, hypertension, and smoking were significant risk factors in multivariate models. Results support a causal relationship between construction trades work and hearing loss. Prevention should focus on reducing exposure to noise, solvents, and cigarette smoke. © 2018 Wiley Periodicals, Inc.

  9. Prevalence of occupational noise induced hearing loss amongst traffic police personnel

    OpenAIRE

    Singh, V K; Mehta, A K

    1999-01-01

    Traffic branch personnel of Pune traffic police were screened for presence of noise induced hearing loss. A very significant number (81.2%) showed sensorineural hearing loss. The various factors responsible for noise induced hearing loss are discussed.

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

    Directory of Open Access Journals (Sweden)

    Jill B Firszt

    2013-12-01

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

  11. Salivary Cortisol Profiles of Children with Hearing Loss

    Science.gov (United States)

    Bess, Fred H.; Gustafson, Samantha J.; Corbett, Blythe A.; Lambert, E. Warren; Camarata, Stephen M.; Hornsby, Benjamin W. Y.

    2016-01-01

    Objectives: It has long been speculated that effortful listening places children with hearing loss at risk for fatigue. School-age children with hearing loss experiencing cumulative stress and listening fatigue on a daily basis might undergo dysregulation of hypothalamic-pituitary-adrenal (HPA) axis activity resulting in elevated or flattened…

  12. Radiation Therapy and Hearing Loss

    International Nuclear Information System (INIS)

    Bhandare, Niranjan; Jackson, Andrew; Eisbruch, Avraham; Pan, Charlie C.; Flickinger, John C.; Antonelli, Patrick; Mendenhall, William M.

    2010-01-01

    A review of literature on the development of sensorineural hearing loss after high-dose radiation therapy for head-and-neck tumors and stereotactic radiosurgery or fractionated stereotactic radiotherapy for the treatment of vestibular schwannoma is presented. Because of the small volume of the cochlea a dose-volume analysis is not feasible. Instead, the current literature on the effect of the mean dose received by the cochlea and other treatment- and patient-related factors on outcome are evaluated. Based on the data, a specific threshold dose to cochlea for sensorineural hearing loss cannot be determined; therefore, dose-prescription limits are suggested. A standard for evaluating radiation therapy-associated ototoxicity as well as a detailed approach for scoring toxicity is presented.

  13. Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

    Science.gov (United States)

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

    2011-01-01

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

  14. Speech-evoked auditory brainstem responses in children with hearing loss.

    Science.gov (United States)

    Koravand, Amineh; Al Osman, Rida; Rivest, Véronique; Poulin, Catherine

    2017-08-01

    The main objective of the present study was to investigate subcortical auditory processing in children with sensorineural hearing loss. Auditory Brainstem Responses (ABRs) were recorded using click and speech/da/stimuli. Twenty-five children, aged 6-14 years old, participated in the study: 13 with normal hearing acuity and 12 with sensorineural hearing loss. No significant differences were observed for the click-evoked ABRs between normal hearing and hearing-impaired groups. For the speech-evoked ABRs, no significant differences were found for the latencies of the following responses between the two groups: onset (V and A), transition (C), one of the steady-state wave (F), and offset (O). However, the latency of the steady-state waves (D and E) was significantly longer for the hearing-impaired compared to the normal hearing group. Furthermore, the amplitude of the offset wave O and of the envelope frequency response (EFR) of the speech-evoked ABRs was significantly larger for the hearing-impaired compared to the normal hearing group. Results obtained from the speech-evoked ABRs suggest that children with a mild to moderately-severe sensorineural hearing loss have a specific pattern of subcortical auditory processing. Our results show differences for the speech-evoked ABRs in normal hearing children compared to hearing-impaired children. These results add to the body of the literature on how children with hearing loss process speech at the brainstem level. Copyright © 2017 Elsevier B.V. All rights reserved.

  15. Age-related hearing loss in sea lions and their scientists

    Science.gov (United States)

    Schusterman, Ronald J.; Southall, Brandon; Kastak, David; Reichmuth Kastak, Colleen

    2002-05-01

    Interest in the hearing capabilities of California sea lions (Zalophus californianus) was first stimulated by the echolocation hypothesis and more recently by rising concern about coastal noise pollution. During a series of audiometric tests, we measured the absolute hearing sensitivity of two sea lions and two of their human investigators. Aerial hearing curves for each subject were obtained with a go/no-go procedure and standard psychophysics. Additionally, underwater hearing curves were obtained for the sea lions using the same procedures. Underwater, the older sea lion (22-25 years of age) showed hearing losses relative to the younger sea lion (13-16 years) that ranged from 10 dB at lower frequencies to 50 dB near the upper frequency limit. The older sea lions' hearing losses in air were consistent with those measured underwater. The older human (69 years) tested also showed losses relative to the younger human (22 years). These differences ranged from 15 dB at lower frequencies up to 35 dB at the highest frequency tested. The results obtained in this study document age-related hearing losses in sea lions and humans. The findings are consistent with data on presbycusis in other mammalian species, showing that maximum hearing loss occurs at the highest frequencies.

  16. The prevalence of noise-induced occupational hearing loss in dentistry personnel.

    Science.gov (United States)

    Khaimook, Wandee; Suksamae, Puwanai; Choosong, Thitiworn; Chayarpham, Satit; Tantisarasart, Ratchada

    2014-09-01

    Occupational hearing loss is the second most common health problem in the industrialized world. Dental personnel exposed to occupational noise may experience hearing loss. This article compares the prevalence of hearing loss in the general population to that of dental personnel exposed to noise during work hours and identifies risk factors for hearing loss among workers at a dental school. This prospective study included 76 dental personnel on the faculty of dentistry at a major university in Asia who were exposed to noise and 76 individuals in a control group. Nearly 16% of the study group and 21% of the control group had lost hearing, a nonsignificant difference (p = .09). Hearing loss was significantly related to work tenure longer than 15 years and age older than 40 years (p < .001 ).

  17. Minocycline attenuates noise-induced hearing loss in rats.

    Science.gov (United States)

    Zhang, Jing; Song, Yong-Li; Tian, Ke-Yong; Qiu, Jian-Hua

    2017-02-03

    Noise-induced hearing loss (NIHL) is a serious health concern and prevention of hair cell death or therapeutic intervention at the early stage of NIHL is critical to preserve hearing. Minocycline is a semi-synthetic derivative of tetracycline and has been shown to have otoprotective effects in ototoxic drug-induced hearing impairment, however, whether minocycline can protect against NIHL has not been investigated. The present study demonstrated elevated ABR (auditory brainstem response) thresholds and outer hair cell loss following traumatic noise exposure, which was mitigated by intraperitoneal administration of minocycline (45mg/kg/d) for 5 consecutive days. In conclusion, the present study demonstrated that minocycline, a clinically approved drug with a good safety profile, can attenuate NIHL in rats and may potentially be used for treatment of hearing loss in clinic. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  18. Disease: H00604 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00604 Deafness, autosomal dominant Hereditary deafness is divided into syndromic forms (in which hearing... with 2-3% of the cases of non-syndromic hearing loss. Autosomal-dominant forms of deafness are usually post...-lingual and progressive. Dominant mutations may be consistent with initial function and subsequent hearin...Griffin A, Fernandez B, Lee MK, King MC ... TITLE ... Non-syndromic progressive hearing loss DFNA38 is caused ...2 deficiency impairs otic development and hearing ability in a zebrafish model of

  19. Hearing loss is associated with decreased nonverbal intelligence in rural Nepal.

    Science.gov (United States)

    Emmett, Susan D; Schmitz, Jane; Pillion, Joseph; Wu, Lee; Khatry, Subarna K; Karna, Sureshwar L; LeClerq, Steven C; West, Keith P

    2015-01-01

    To evaluate the association between adolescent and young-adult hearing loss and nonverbal intelligence in rural Nepal. Cross-sectional assessment of hearing loss among a population cohort of adolescents and young adults. Sarlahi District, southern Nepal. Seven hundred sixty-four individuals aged 14 to 23 years. Evaluation of hearing loss, defined by World Health Organization criteria of pure-tone average greater than 25 decibels (0.5, 1, 2, 4 kHz), unilaterally and bilaterally. Nonverbal intelligence, as measured by the Test of Nonverbal Intelligence, 3rd Edition standardized score (mean, 100; standard deviation, 15). Nonverbal intelligence scores differed between participants with normal hearing and those with bilateral (p = 0.04) but not unilateral (p = 0.74) hearing loss. Demographic and socioeconomic factors including male sex; higher caste; literacy; education level; occupation reported as student; and ownership of a bicycle, watch, and latrine were strongly associated with higher nonverbal intelligence scores (all p intelligence score based on unilateral hearing loss (0.97; 95% confidence interval, -1.67 to 3.61; p = 0.47). Nonverbal intelligence is adversely affected by bilateral hearing loss even at mild hearing loss levels. Socio economic well-being appears compromised in individuals with lower nonverbal intelligence test scores.

  20. Noise Induced Hearing Loss in Iran: (1997–2012): Systematic Review Article

    Science.gov (United States)

    SOLTANZADEH, Ahmad; EBRAHIMI, Hossein; FALLAHI, Majid; KAMALINIA, Mojtaba; GHASSEMI, Shadi; GOLMOHAMMADI, Rostam

    2014-01-01

    Noise-induced hearing loss, which is one of the 10 leading occupational diseases, is a debilitating and irreversible disease. During the recent 15-years period (1997–2012), several studies have investigated the association between noise, hearing damage and other side effects of noise in Iran. The aim of this study was to review systematically the relevant literature related to noise-induced hearing loss, lead to developing noise exposure limits. In this systematic review, two researchers independently extracted the data from 31 past studies that had considered noise-induced hearing loss (including hearing loss, temporary and permanent hearing threshold shift and auditory trauma). The data were then recorded in a modified form and Statistical analyses were performed using SPSS, version 16.0. In analyzed studies the weighted average equivalent sound pressure level [L Aeq ] was 90.29 dB(A) and average hearing loss was 26.44 dB(A). The Highest degree of hearing loss in the right ear was associated at 4000 Hz, and the highest degree of hearing loss in the left ear was associated to 1000 and 4000 Hz. The majority of the reviewed studies have confirmed that exposure to a noise level above 85 dB (A) can lead to an increased chance of hearing loss. Furthermore, the results of the present review indicated that as L Aeq increased up to 85 dB(A), so did the severity of the hearing loss. PMID:26171352

  1. Management of Children with Mild, Moderate, and Moderately Severe Sensorineural Hearing Loss.

    Science.gov (United States)

    Tharpe, Anne Marie; Gustafson, Samantha

    2015-12-01

    Any degree of hearing loss can have a negative impact on child development. The amount of impact is largely determined by the type, quality, and timeliness of intervention. Early identification and management of hearing loss is essential for minimizing the impact of hearing loss and ensuring that children can reach their cognitive, linguistic, educational, and social potential. Advances in hearing technology and broadening of candidacy for same, have resulted in improved outcomes for many children with hearing loss. Through ongoing hearing monitoring throughout childhood, children with congenital, late-onset, or progressive losses can receive timely management from interprofessional, collaborative teams. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. Disease: H01209 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H01209 Deafness, X-linked Hereditary deafness is divided into syndromic forms (in which hearing... of the cases of non-syndromic hearing loss. X-linked deafness is clinically and genetically heterogeneous d...isorder. PRPS1 and POU3F4 have been identified as the genes to be implicated in X-linked non-syndromic hea...ring loss. Nervous system disease; Nervous system disease PRPS1 [HSA:5631] [KO:K009

  3. Hearing loss and employment in the United States.

    Science.gov (United States)

    Kooser, Cathy

    2013-01-01

    This Sounding Board article will briefly review the biopsychosocial impact of hearing loss. It will consider the individual and employment; the laws supporting employment and the current vocational rehabilitation system assisting people with hearing loss remain in the workplace. It concludes with the author's suggestion of three systematic changes to enhance the employee's workplace success.

  4. The Sound of Silence: Mouse Models for Hearing Loss

    Directory of Open Access Journals (Sweden)

    Sumantra Chatterjee

    2011-01-01

    Full Text Available Sensorineural hearing loss is one of the most common disabilities in humans. It is estimated that about 278 million people worldwide have slight to extreme hearing loss in both ears, which results in an economic loss for the country and personal loss for the individual. It is thus critical to have a deeper understanding of the causes for hearing loss to better manage and treat the affected individuals. The mouse serves as an excellent model to study and recapitulate some of these phenotypes, identify new genes which cause deafness, and to study their roles in vivo and in detail. Mutant mice have been instrumental in elucidating the function and mechanisms of the inner ear. The development and morphogenesis of the inner ear from an ectodermal layer into distinct auditory and vestibular components depends on well-coordinated gene expression and well-orchestrated signaling cascades within the otic vesicle and interactions with surrounding layers of tissues. Any disruption in these pathways can lead to hearing impairment. This review takes a look at some of the genes and their corresponding mice mutants that have shed light on the mechanism governing hearing impairment (HI in humans.

  5. Hearing outcomes in patients with cleft lip/palate.

    Science.gov (United States)

    Skuladottir, Hildur; Sivertsen, Ase; Assmus, Jorg; Remme, Asa Rommetveit; Dahlen, Marianne; Vindenes, Hallvard

    2015-03-01

    Objective : Children with cleft lip and palate or cleft palate only have a high incidence of conductive hearing loss from otitis media with effusion. Studies demonstrating longitudinal results are lacking. This study was undertaken to investigate long-term longitudinal hearing outcomes of children with cleft lip and/or cleft palate and cleft palate only. Design : Retrospective chart review. Setting : Clinical charts of patients born with cleft lip and palate or cleft palate only in 1985 to 1994 who were referred to the cleft team in Bergen, Norway. Study findings include 15 years of follow-up. Participants : The study population consisted of 317 children of whom 159 had nonsyndromic cleft lip and palate and 158 had nonsyndromic cleft palate. Main Outcome Measures : Pure tone average calculated from pure tone audiometry at ages 4, 6, and 15 years. Results : The median pure tone average significantly improved with increasing age. For the cleft lip and palate group, the median pure tone average at ages 4, 6, and 15 years was 16 dB hearing level (HL), 13 dB HL, and 9 dB HL, respectively (P ≤ .001). In the cleft palate group the median pure tone average at ages 4, 6, and 15 years was 15 dB HL, 12 dB HL, and 9 dB HL, respectively (P ≤ .001). There was no significant difference in the hearing levels between the two groups. Patients who had surgical closure of the palate at age 18 months had a significantly better pure tone average outcome at age 15 compared with patients who had surgery at 12 months. Conclusions : Hearing improves significantly from childhood to adolescence in patients with cleft lip and palate and cleft palate only.

  6. Management of non-organic hearing loss in children - A case study.

    Science.gov (United States)

    Skarzynski, Piotr Henryk; Raj-Koziak, Danuta; Rajchel, Joanna Jadwiga; Skarzynski, Henryk

    2017-06-01

    A 10 year-old girl was admitted due to the claim of progressively developing hearing loss. The impedance audiometry showed no abnormalities but it was impossible to obtain reliable outcomes during pure tone audiometry assessment. The girl was additionally sent for speech audiometry, indicating a bilateral hearing loss and objective evaluations such as distortion product otoacoustic emissions and auditory brainstem responses, which results indicated a normal hearing. On the second day, repeated subjective audiometric tests showed also normal hearing, despite constantly reported hearing loss. After the psychological consultation and exclusion of neurologic pathology, the diagnosis of non-organic hearing loss was stated and the girl was scheduled for regular appointments with psychologist. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Application of a New Genetic Deafness Microarray for Detecting Mutations in the Deaf in China.

    Directory of Open Access Journals (Sweden)

    Hong Wu

    Full Text Available The aim of this study was to evaluate the GoldenGate microarray as a diagnostic tool and to elucidate the contribution of the genes on this array to the development of both nonsyndromic and syndromic sensorineural hearing loss in China.We developed a microarray to detect 240 mutations underlying syndromic and nonsyndromic sensorineural hearing loss. The microarray was then used for analysis of 382 patients with nonsyndromic sensorineural hearing loss (including 15 patients with enlarged vestibular aqueduct syndrome, 21 patients with Waardenburg syndrome, and 60 unrelated controls. Subsequently, we analyzed the sensitivity, specificity, and reproducibility of this new approach after Sanger sequencing-based verification, and also determined the contribution of the genes on this array to the development of distinct hearing disorders.The sensitivity and specificity of the microarray chip were 98.73% and 98.34%, respectively. Genetic defects were identified in 61.26% of the patients with nonsyndromic sensorineural hearing loss, and 9 causative genes were identified. The molecular etiology was confirmed in 19.05% and 46.67% of the patients with Waardenburg syndrome and enlarged vestibular aqueduct syndrome, respectively.Our new mutation-based microarray comprises an accurate and comprehensive genetic tool for the detection of sensorineural hearing loss. This microarray-based detection method could serve as a first-pass screening (before next-generation-sequencing screening for deafness-causing mutations in China.

  8. Hearing loss and tinnitus in rock musicians: A Norwegian survey

    Directory of Open Access Journals (Sweden)

    Carl Christian Lein Størmer

    2015-01-01

    Full Text Available Our focus in this study was to assess hearing thresholds and the prevalence and characteristics of tinnitus in a large group of rock musicians based in Norway. A further objective was to assess related factors such as exposure, instrument category, and the preventive effect of hearing protection. The study was a cross-sectional survey of rock musicians selected at random from a defined cohort of musicians. A random control group was included for comparison. We recruited 111 active musicians from the Oslo region, and a control group of 40 nonmusicians from the student population at the University of TromsØ. The subjects were investigated using clinical examination, pure tone audiometry, tympanometry, and a questionnaire. We observed a hearing loss in 37.8% of the rock musicians. Significantly poorer hearing thresholds were seen at most pure-tone frequencies in musicians than controls, with the most pronounced threshold shift at 6 kHz. The use of hearing protection, in particular custom-fitted earplugs, has a preventive effect but a minority of rock musicians apply them consistently. The degree of musical performance exposure was inversely related to the degree of hearing loss in our sample. Bass and guitar players had higher hearing thresholds than vocalists. We observed a 20% prevalence of chronic tinnitus but none of the affected musicians had severe tinnitus symptomatology. There was no statistical association between permanent tinnitus and hearing loss in our sample. We observed an increased prevalence of hearing loss and tinnitus in our sample of Norwegian rock musicians but the causal relationship between musical exposure and hearing loss or tinnitus is ambiguous. We recommend the use of hearing protection in rock musicians.

  9. [A temporal bone CT study of the infants with hearing loss referred from universal newborn hearing screening].

    Science.gov (United States)

    Tao, Zheng; Li, Yun; Hou, Zheng; Cheng, Lan

    2007-02-01

    To explore the high resolution CT image of temporal bone in infants with hearing loss, and its value in evaluating the cause of hearing loss. In 2005, 0.12 million newborns have been included in the hearing screening system in Shanghai, and 1077 infants have failed to pass the hearing screening. One hundred and eight four infants were diagnosed as congenital hearing loss from mild to profound. A temporal bone HRCT scanning was performed to these infants. Among the 184 patients with congenital hearing loss, HRCT showed that 26 cases (14.1%) were associated with external ear malformation, and 21 cases (11.4%) were associated with middle ear malformation, 31 cases (16.8%) associated with inner ear malformation. The patients with inner ear malformation included 12 cases with Mondini malformation, 1 case with common cavity malformation, 6 cases with large vestibule malformation, 5 cases with internal auditory canal abnormalities, and 10 cases with vestibule, semicircular canals abnormalities. In addition, there were 20 cases (10.8%) with fluid in middle ear. HRCT image play an important role in the differential diagnosis and treatment of infants with congenital hearing loss.

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

    Science.gov (United States)

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

    2010-01-01

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

  11. Development of conductive hearing loss due to posterior semicircular canal dehiscence.

    Science.gov (United States)

    Kubota, Marie; Kubo, Kazuhiko; Yasui, Tetsuro; Matsumoto, Nozomu; Komune, Shizuo

    2015-06-01

    We herein report a case of posterior semicircular canal dehiscence (SCD) syndrome who had been audiologically followed up for eight years. The patient originally had sensorineural hearing loss. The audiogram had gradually transformed to pure conductive hearing loss. The posterior SCD was identified in CT scan. The reported case showed the possibility to distinguish the mechanism at play underlying the typical conductive hearing loss in SCD patients by tracing the transition of the hearing loss pattern. This information is of much help to predict the hearing outcomes if surgical intervention were chosen for the treatment. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  12. Pitch and Loudness from Tinnitus in Individuals with Noise-induced Hearing Loss

    Directory of Open Access Journals (Sweden)

    Flores, Leticia Sousa

    2015-08-01

    Full Text Available Introduction Tinnitus is one of the symptoms that affects individuals suffering from noise induced hearing loss. This condition can be disabling, leading the affected individual to turn away from work. Objective This literature review aims to analyze the possible association between gender and tinnitus pitch and loudness, the degree of hearing loss and the frequencies affected in subjects with noise-induced hearing loss. Methods This contemporary cohort study was conducted through a cross-sectional analysis. The study sample consisted of adults with unilateral or bilateral tinnitus, who had been diagnosed with noise-induced hearing loss. The patients under analysis underwent an otorhinolaryngological evaluation, pure tone audiometry, and acuphenometry. Results The study included 33 subjects with noise-induced hearing loss diagnoses, of which 22 (66.7% were men. Authors observed no statistical difference between gender and loudness/pitch tinnitus and loudness/pitch in subjects with bilateral tinnitus. Authors found an inverse relation between tinnitus loudness with intensity greater hearing threshold and the average of the thresholds and the grade of hearing loss. The tinnitus pitch showed no association with higher frequency of hearing threshold. Conclusion Data analysis shows that, among the individuals evaluated, the greater the hearing loss, the lower the loudness of tinnitus. We did not observe an association between hearing loss and tinnitus pitch.

  13. Consequences of Early Conductive Hearing Loss on Long-Term Binaural Processing.

    Science.gov (United States)

    Graydon, Kelley; Rance, Gary; Dowell, Richard; Van Dun, Bram

    The aim of the study was to investigate the long-term effects of early conductive hearing loss on binaural processing in school-age children. One hundred and eighteen children participated in the study, 82 children with a documented history of conductive hearing loss associated with otitis media and 36 controls who had documented histories showing no evidence of otitis media or conductive hearing loss. All children were demonstrated to have normal-hearing acuity and middle ear function at the time of assessment. The Listening in Spatialized Noise Sentence (LiSN-S) task and the masking level difference (MLD) task were used as the two different measures of binaural interaction ability. Children with a history of conductive hearing loss performed significantly poorer than controls on all LiSN-S conditions relying on binaural cues (DV90, p = binaural cues. Fifteen children with a conductive hearing loss history (18%) showed results consistent with a spatial processing disorder. No significant difference was observed between the conductive hearing loss group and the controls on the MLD task. Furthermore, no correlations were found between LiSN-S and MLD. Results show a relationship between early conductive hearing loss and listening deficits that persist once hearing has returned to normal. Results also suggest that the two binaural interaction tasks (LiSN-S and MLD) may be measuring binaural processing at different levels. Findings highlight the need for a screening measure of functional listening ability in children with a history of early otitis media.

  14. Mobile phone usage does not affect sudden sensorineural hearing loss.

    Science.gov (United States)

    Sagiv, D; Migirov, L; Madgar, O; Nakache, G; Wolf, M; Shapira, Y

    2018-01-01

    Recent studies found that mobile phone users had a significantly greater risk of having elevated thresholds in speech frequencies. This study investigated the correlation between the laterality of sudden sensorineural hearing loss, handedness and the preferred ear for mobile phone use. The study included all patients who presented with sudden sensorineural hearing loss to the Department of Otolaryngology - Head and Neck Surgery in our tertiary referral medical centre between 2014 and 2016. Patients were asked to indicate their dominant hand and preferred ear for mobile phone use. The study comprised 160 patients. No correlation was found between the dominant hand or preferred ear for mobile phone use and the side of sudden sensorineural hearing loss. There was no correlation between the side of the sudden sensorineural hearing loss (preferable or non-preferable for mobile phone use) and audiometric characteristics. No correlation was found between the laterality of ears used for mobile phone and sudden sensorineural hearing loss.

  15. Hearing loss in the elderly: History of occupational noise exposure.

    Science.gov (United States)

    Meneses-Barriviera, Caroline Luiz; Melo, Juliana Jandre; Marchiori, Luciana Lozza de Moraes

    2013-04-01

     Noise exposure is one of the most common health risk factors, and workers are exposed to sound pressure levels capable of producing hearing loss.  To assess the prevalence of hearing loss in the elderly and its possible association with a history of occupational noise exposure and with sex.  A prospective study in subjects aged over 60 years. The subjects underwent anamnesis and audiological assessment. The Mann-Whitney test and multiple logistic regression, with 95% confidence interval and p hearing I (500, 1000, and 2000 Hz p = 0.8318) and the mean hearing II (3000, 4000, and 6000 Hz; p occupational noise exposure, we obtained the medium hearing I (p = 0.9542) and the mean hearing II (p = 0.0007).  There was a statistically significant association between hearing loss at high frequencies and the risk factors being male and occupational noise exposure.

  16. The dose-response relationship between in-ear occupational noise exposure and hearing loss.

    Science.gov (United States)

    Rabinowitz, Peter M; Galusha, Deron; Dixon-Ernst, Christine; Clougherty, Jane E; Neitzel, Richard L

    2013-10-01

    Current understanding of the dose-response relationship between occupational noise and hearing loss is based on cross-sectional studies prior to the widespread use of hearing protection, and with limited data regarding noise exposures below 85 dBA. We report on the hearing loss experience of a unique cohort of industrial workers, with daily monitoring of noise inside of hearing protection devices. At an industrial facility, workers exhibiting accelerated hearing loss were enrolled in a mandatory programme to monitor daily noise exposures inside of hearing protection. We compared these noise measurements (as time-weighted LAVG) to interval rates of high-frequency hearing loss over a 6-year period using a mixed-effects model, adjusting for potential confounders. Workers' high-frequency hearing levels at study inception averaged more than 40 dB Hearing threshold level (HTL). Most noise exposures were less than 85 dBA (mean LAVG 76 dBA, IQR 74-80 dBA). We found no statistical relationship between LAvg and high-frequency hearing loss (p=0.53). Using a metric for monthly maximum noise exposure did not improve model fit. At-ear noise exposures below 85 dBA did not show an association with risk of high-frequency hearing loss among workers with substantial past noise exposure and hearing loss at baseline. Therefore, effective noise control to below 85 dBA may lead to significant reduction in occupational hearing loss risk in such individuals. Further research is needed on the dose-response relationship of noise and hearing loss in individuals with normal hearing and little prior noise exposure.

  17. STUDY OF HEARING OUTCOMES IN SUDDEN SENSORINEURAL HEARING LOSS TREATED WITH TISSUE PLASMINOGEN ACTIVATOR (TPA

    Directory of Open Access Journals (Sweden)

    Rama Krishna

    2015-09-01

    Full Text Available Sudden Sensorineural Hearing Loss (SSHNL is a clinical condition that requires immediate management. There are many treatment options, which may not always revert the hearing to normal. Not only recording the degree of hearing loss, but also establishing the concurrent dysfunction of saccule by VEMP has facilitated a new approach to treatment strategy. Recombinant tissue Plasminogen Activator ((rtPA proved its efficacy in stroke and subsequently considered an option in the management of ISSNHL. The curren t study, conducted at different centres, on 15 patients utilized rtPA. The results showed a promising trend when saccular pathology is also evident by VEMP in association with Hearing loss. We recommend use of rtPA as primary modality in cases of ISSNHL wi th Saccular involvement.

  18. Morphological correlates of hearing loss after cochlear implantation and electro-acoustic stimulation in a hearing-impaired Guinea pig model.

    Science.gov (United States)

    Reiss, Lina A J; Stark, Gemaine; Nguyen-Huynh, Anh T; Spear, Kayce A; Zhang, Hongzheng; Tanaka, Chiemi; Li, Hongzhe

    2015-09-01

    Hybrid or electro-acoustic stimulation (EAS) cochlear implants (CIs) are designed to provide high-frequency electric hearing together with residual low-frequency acoustic hearing. However, 30-50% of EAS CI recipients lose residual hearing after implantation. The objective of this study was to determine the mechanisms of EAS-induced hearing loss in an animal model with high-frequency hearing loss. Guinea pigs were exposed to 24 h of noise (12-24 kHz at 116 dB) to induce a high-frequency hearing loss. After recovery, two groups of animals were implanted (n = 6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no stimulation during this time frame. A third group (n = 6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem responses were recorded biweekly to monitor changes in hearing. The organ of Corti was immunolabeled with phalloidin, anti-CtBP2, and anti-GluR2 to quantify hair cells, ribbons and post-synaptic receptors. The lateral wall was immunolabeled with phalloidin and lectin to quantify stria vascularis capillary diameters. Bimodal or trimodal diameter distributions were observed; the number and location of peaks were objectively determined using the Aikake Information Criterion and Expectation Maximization algorithm. Noise exposure led to immediate hearing loss at 16-32 kHz for all groups. Cochlear implantation led to additional hearing loss at 4-8 kHz; this hearing loss was negatively and positively correlated with minimum and maximum peaks of the bimodal or trimodal distributions of stria vascularis capillary diameters, respectively. After chronic stimulation, no significant group changes in thresholds were seen; however, elevated thresholds at 1 kHz in implanted, stimulated animals were significantly correlated with decreased presynaptic ribbon and postsynaptic receptor counts. Inner and outer hair cell counts did not differ between groups and

  19. [Sensorineural hearing loss due to neonatal hyperbilirubinemia].

    Science.gov (United States)

    Clarós, P; Turcanu, D; Caballero, M; Costa, C; Clavería, M A; Clarós, A; Clarós, A

    2003-01-01

    In this article, the sensorineural hearing loss is presented as a possible sequelae of neonatal hyperbilirubinemia. In our program of early hipoacusia detection, 241 babies were examined from January 1996 until November 1999; 7 cases had a history of hyperbilirubinemia in the neonatal period and 2 of them were diagnosed of sensorineural hearing loss. We discuss how the bilirubin or any other associated factor might have been the cause and this could explain the selective affectation of some children.

  20. Individual Hearing Loss: Characterization, Modelling, Compensation Strategies

    DEFF Research Database (Denmark)

    Santurette, Sébastien; Dau, Torsten; Christensen-Dalsgaard, Jakob

    2016-01-01

    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...

  1. Ear malformations, hearing loss and hearing rehabilitation in children with Treacher Collins syndrome.

    Science.gov (United States)

    Rosa, Francisco; Coutinho, Miguel Bebiano; Ferreira, João Pinto; Sousa, Cecilia Almeida

    2016-01-01

    The aim of this study was to assess the main ear malformations, hearing loss and auditory rehabilitation in children with Treacher Collins syndrome. We performed a retrospective study of 9 children with Treacher Collins syndrome treated in a central hospital between January 2003 and January 2013. This study showed a high incidence of malformations of the outer and middle ear, such as microtia, atresia or stenosis of the external auditory canal, hypoplastic middle ear cavity, dysmorphic or missing ossicular chain. Most patients had bilateral hearing loss of moderate or high degree. In the individuals studied, there was functional improvement in patients with bone-anchored hearing aids in relation to conventional hearing aids by bone conduction. Treacher Collins syndrome is characterized by bilateral malformations of the outer and middle ear. Hearing rehabilitation in these children is of utmost importance, and bone-anchored hearing aids is the method of choice. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  2. Auditory and language outcomes in children with unilateral hearing loss.

    Science.gov (United States)

    Fitzpatrick, Elizabeth M; Gaboury, Isabelle; Durieux-Smith, Andrée; Coyle, Doug; Whittingham, JoAnne; Nassrallah, Flora

    2018-03-13

    Children with unilateral hearing loss (UHL) are being diagnosed at younger ages because of newborn hearing screening. Historically, they have been considered at risk for difficulties in listening and language development. Little information is available on contemporary cohorts of children identified in the early months of life. We examined auditory and language acquisition outcomes in a contemporary cohort of early-identified children with UHL and compared their outcomes at preschool age with peers with mild bilateral loss and with normal hearing. As part of the Mild and Unilateral Hearing Loss in Children Study, we collected auditory and spoken language outcomes on children with unilateral, bilateral hearing loss and with normal hearing over a four-year period. This report provides a cross-sectional analysis of results at age 48 months. A total of 120 children (38 unilateral and 31 bilateral mild, 51 normal hearing) were enrolled in the study from 2010 to 2015. Children started the study at varying ages between 12 and 36 months of age and were followed until age 36-48 months. The median age of identification of hearing loss was 3.4 months (IQR: 2.0, 5.5) for unilateral and 3.6 months (IQR: 2.7, 5.9) for the mild bilateral group. Families completed an intake form at enrolment to provide baseline child and family-related characteristics. Data on amplification fitting and use were collected via parent questionnaires at each annual assessment interval. This study involved a range of auditory development and language measures. For this report, we focus on the end of follow-up results from two auditory development questionnaires and three standardized speech-language assessments. Assessments included in this report were completed at a median age of 47.8 months (IQR: 38.8, 48.5). Using ANOVA, we examined auditory and language outcomes in children with UHL and compared their scores to children with mild bilateral hearing loss and those with normal hearing. On most

  3. The Dose Response Relationship between In Ear Occupational Noise Exposure and Hearing Loss

    Science.gov (United States)

    Rabinowitz, Peter M.; Galusha, Deron; Dixon-Ernst, Christine; Clougherty, Jane E.; Neitzel, Richard L.

    2014-01-01

    Objectives Current understanding of the dose-response relationship between occupational noise and hearing loss is based on cross-sectional studies prior to the widespread use hearing protection and with limited data regarding noise exposures below 85dBA. We report on the hearing loss experience of a unique cohort of industrial workers with daily monitoring of noise inside of hearing protection devices. Methods At an industrial facility, workers exhibiting accelerated hearing loss were enrolled in a mandatory program to monitor daily noise exposures inside of hearing protection. We compared these noise measurements (as time-weighted LAVG) to interval rates of high frequency hearing loss over a six year period using a mixed effects model, adjusting for potential confounders. Results Workers’ high frequency hearing levels at study inception averaged more than 40 dB hearing threshold level (HTL). Most noise exposures were less than 85dBA (mean LAVG 76 dBA, interquartile range 74 to 80 dBA). We found no statistical relationship between LAvg and high frequency hearing loss (p = 0.53). Using a metric for monthly maximum noise exposure did not improve model fit. Conclusion At-ear noise exposures below 85dBA did not show an association with risk of high frequency hearing loss among workers with substantial past noise exposure and hearing loss at baseline. Therefore, effective noise control to below 85dBA may lead to significant reduction in occupational hearing loss risk in such individuals. Further research is needed on the dose response relationship of noise and hearing loss in individuals with normal hearing and little prior noise exposure. PMID:23825197

  4. Hearing loss in the royal Norwegian Navy: a cross-sectional study.

    Science.gov (United States)

    Irgens-Hansen, Kaja; Sunde, Erlend; Bråtveit, Magne; Baste, Valborg; Oftedal, Gunnhild; Koefoed, Vilhelm; Lind, Ola; Moen, Bente Elisabeth

    2015-07-01

    Prior studies have indicated a high prevalence of noise-induced hearing loss (NIHL) among Navy personnel; however, it is not clear whether this is caused by work on board. The present study aimed to assess the prevalence of hearing loss among Navy personnel in the Royal Norwegian Navy (RNoN), and to investigate whether there is an association between work on board RNoN vessels and occurrence of hearing loss. Navy personnel currently working on board RNoN vessels were recruited to complete a questionnaire on noise exposure and health followed by pure tone audiometry. Hearing loss was defined as hearing threshold levels ≥25 dB in either ear at the frequencies 3,000, 4,000 or 6,000 Hz. Hearing thresholds were adjusted for age and gender using ISO 7029. The prevalence of hearing loss among Navy personnel was 31.4 %. The work exposure variables: years of work in the Navy, years on vessel(s) in the Navy and years of sailing in the Navy were associated with reduced hearing after adjusting for age, gender and otitis as an adult. Among the work exposure variables, years of sailing in the Navy was the strongest predictor of reduced hearing, and significantly reduced hearing was found at the frequencies 1,000, 3,000 and 4,000 Hz. Our results indicate that time spent on board vessels in the RNoN is a predictor of reduced hearing.

  5. Management of Conductive Hearing Loss in Children.

    Science.gov (United States)

    Dougherty, William; Kesser, Bradley W

    2015-12-01

    Conductive hearing loss (CHL), far more common than sensorineural hearing loss in children, can be acquired or congenital, can range from mild to moderately severe, and can be caused by a simple cerumen impaction, middle ear fluid, or complex middle ear abnormalities with or without the absence of the ear canal (congenital aural atresia). This article presents evidence-based recommendations for the evaluation and management of the child with both acquired and congenital CHL. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Identification and molecular modelling of a mutation in the motor head domain of myosin VIIA in a family with autosomal dominant hearing impairment (DFNA11)

    NARCIS (Netherlands)

    Luijendijk, M.W.J.; Wijk, E. van; Bischoff, A.M.L.C.; Krieger, E.; Huygen, P.L.M.; Pennings, R.J.E.; Brunner, H.G.; Cremers, C.W.R.J.; Cremers, F.P.M.; Kremer, J.M.J.

    2004-01-01

    Myosin VIIA is an unconventional myosin that has been implicated in Usher syndrome type 1B, atypical Usher syndrome, non-syndromic autosomal recessive hearing impairment (DFNB2) and autosomal dominant hearing impairment (DFNA11). Here, we present a family with non-syndromic autosomal dominant

  7. Identification and molecular modelling of a mutation in the motor head domain of myosin VIIA in a family with autosomal dominant hearing impairment (DFNA11).

    NARCIS (Netherlands)

    Luijendijk, M.W.J.; Wijk, E. van; Bischoff, A.M.L.C.; Krieger, E.; Huygen, P.L.M.; Pennings, R.J.E.; Brunner, H.G.; Cremers, C.W.R.J.; Cremers, F.P.M.; Kremer, J.M.J.

    2004-01-01

    Myosin VIIA is an unconventional myosin that has been implicated in Usher syndrome type 1B, atypical Usher syndrome, non-syndromic autosomal recessive hearing impairment (DFNB2) and autosomal dominant hearing impairment (DFNA11). Here, we present a family with non-syndromic autosomal dominant

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

    Science.gov (United States)

    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

  9. Educational Implications of Conductive Hearing Loss in School Children.

    Science.gov (United States)

    Lyon, David J.; And Others

    1986-01-01

    The study investigated specific linguistic abilities/disabilities of 15 children with conductive hearing loss and a history of middle ear dysfunction. Results found significant deficits in verbal intelligence, word recognition, and receptive syntactic skills substantiating the finding that conductive hearing loss due to otitis media is deleterious…

  10. Round window stimulation for conductive and mixed hearing loss.

    Science.gov (United States)

    Dillon, Margaret T; Tubbs, Rhonda S; Adunka, Marcia C; King, English R; Hillman, Todd A; Adunka, Oliver F; Chen, Douglas A; Buchman, Craig A

    2014-10-01

    Assess surgical complications, postoperative residual hearing, and speech perception outcomes of placement of a middle ear implant on the round window in conductive and mixed hearing loss cases. Single-subject, repeated-measures design where each subject served as his or her own control. Tertiary referral medical systems. Eighteen subjects with either conductive or mixed hearing loss who could not benefit from conventional amplification were enrolled in a clinical trial investigating vibratory stimulation of the round window. The floating mass transducer (FMT) was positioned in the round window niche. Unaided residual hearing, and aided sound field thresholds and speech perception abilities were evaluated preoperatively, and at 1, 3, 6, and 10 months post-activation of the external speech processor. Six subjects experienced complications that either required further medical management or resolved on their own. There was no difference in residual bone conduction thresholds or unaided word discrimination over time. All subjects experienced a significant improvement in aided speech perception abilities as compared to preoperative performance. Subjects with conductive and mixed hearing loss with placement of the FMT in the round window niche experienced improved sound field thresholds and speech perception, without compromising residual hearing thresholds. Vibratory stimulation of the round window via a middle ear implant may be an appropriate treatment option for patients with conductive and mixed hearing loss. Additional research is needed on the preferred placement of the FMT, improvement of functional gain, and methods to limit postoperative complications and need for revision surgery.

  11. Vibroplasty for mixed and conductive hearing loss.

    Science.gov (United States)

    Luers, Jan Christoffer; Hüttenbrink, Karl-Bernd; Zahnert, Thomas; Bornitz, Matthias; Beutner, Dirk

    2013-08-01

    To summarize new application methods of an active middle ear implant (Vibrant Soundbridge) in patients with conductive or mixed hearing loss. Publications listed in the Medline/PubMed database. All publications published in English language; search term Vibrant Soundbridge AND floating mass transducer in all fields. Structured analysis of all publications. Extraction of significant findings and conclusions and audiometric data. Modern application methods of an active middle ear implant (VSB) open new therapeutic options for patients with various outer and middle ear diseases resulting in conductive or mixed hearing loss. Titanium couplers can help to couple the active middle ear implant in a standardized way to remnants of the ossicular chain or to the round window. Thus, the active middle ear implant has been established as an alternative treatment option for patients with mixed and conductive hearing. However, the heterogeneity of the studies published so far complicates the analysis of the audiometric results, and thus, the functional hearing gain after VSB implantation varies a lot.

  12. Characterization of a new full length TMPRSS3 isoform and identification of mutant alleles responsible for nonsyndromic recessive deafness in Newfoundland and Pakistan

    Directory of Open Access Journals (Sweden)

    Shotland Lawrence I

    2004-09-01

    Full Text Available Abstract Background Mutant alleles of TMPRSS3 are associated with nonsyndromic recessive deafness (DFNB8/B10. TMPRSS3 encodes a predicted secreted serine protease, although the deduced amino acid sequence has no signal peptide. In this study, we searched for mutant alleles of TMPRSS3 in families from Pakistan and Newfoundland with recessive deafness co-segregating with DFNB8/B10 linked haplotypes and also more thoroughly characterized the genomic structure of TMPRSS3. Methods We enrolled families segregating recessive hearing loss from Pakistan and Newfoundland. Microsatellite markers flanking the TMPRSS3 locus were used for linkage analysis. DNA samples from participating individuals were sequenced for TMPRSS3. The structure of TMPRSS3 was characterized bioinformatically and experimentally by sequencing novel cDNA clones of TMPRSS3. Results We identified mutations in TMPRSS3 in four Pakistani families with recessive, nonsyndromic congenital deafness. We also identified two recessive mutations, one of which is novel, of TMPRSS3 segregating in a six-generation extended family from Newfoundland. The spectrum of TMPRSS3 mutations is reviewed in the context of a genotype-phenotype correlation. Our study also revealed a longer isoform of TMPRSS3 with a hitherto unidentified exon encoding a signal peptide, which is expressed in several tissues. Conclusion Mutations of TMPRSS3 contribute to hearing loss in many communities worldwide and account for 1.8% (8 of 449 of Pakistani families segregating congenital deafness as an autosomal recessive trait. The newly identified TMPRSS3 isoform e will be helpful in the functional characterization of the full length protein.

  13. Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss.

    Science.gov (United States)

    Reitsma, Sietze; Stokroos, Robert; Weber, Jacobiene W; van Tongeren, Joost

    2015-12-01

    To present the rare case of a young boy with idiopathic intracranial hypertension presenting with bilateral sensorineural hearing loss developing over several months. This was accompanied by headaches, otalgia, tinnitus, and vertigo. Furthermore, we aim to provide a concise review on this matter, as this report represents the second case in literature of pediatric idiopathic intracranial hypertension presenting with hearing loss. Workup of a 9-year-old boy with bilateral sensorineural hearing loss, including (among others) physical examination, audiometry, diagnostic imaging, and lumbar puncture. Physical examination including fundoscopy as well as imaging showed no abnormalities. At presentation, pure tone audiometry revealed bone conduction thresholds of about 30 dB HL in both ears. Two months later, this declined to about 35 dB HL in both ears. Lumbar puncture revealed an increased intracranial pressure. The boy was thus diagnosed with idiopathic intracranial hypertension. After the lumbar puncture, the otological complaints gradually resolved, and the hearing normalized (bone conduction thresholds of 0-5 dB HL). Although rare, sensorineural hearing loss in the pediatric population together with otalgia, tinnitus, and vertigo can be due to idiopathic intracranial hypertension and as such can be reversible. © The Author(s) 2015.

  14. Audiological results with Baha in conductive and mixed hearing loss.

    Science.gov (United States)

    Pfiffner, Flurin; Caversaccio, Marco-Domenico; Kompis, Martin

    2011-01-01

    The level of improvement in the audiological results of Baha(®) users mainly depends on the patient's preoperative hearing thresholds and the type of Baha sound processor used. This investigation shows correlations between the preoperative hearing threshold and postoperative aided thresholds and audiological results in speech understanding in quiet of 84 Baha users with unilateral conductive hearing loss, bilateral conductive hearing loss and bilateral mixed hearing loss. Secondly, speech understanding in noise of 26 Baha users with different Baha sound processors (Compact, Divino, and BP100) is investigated. Linear regression between aided sound field thresholds and bone conduction (BC) thresholds of the better ear shows highest correlation coefficients and the steepest slope. Differences between better BC thresholds and aided sound field thresholds are smallest for mid-frequencies (1 and 2 kHz) and become larger at 0.5 and 4 kHz. For Baha users, the gain in speech recognition in quiet can be expected to lie in the order of magnitude of the gain in their hearing threshold. Compared to its predecessor sound processors Baha(®) Compact and Baha(®) Divino, Baha(®) BP100 improves speech understanding in noise significantly by +0.9 to +4.6 dB signal-to-noise ratio, depending on the setting and the use of directional microphone. For Baha users with unilateral and bilateral conductive hearing loss and bilateral mixed hearing loss, audiological results in aided sound field thresholds can be estimated with the better BC hearing threshold. The benefit in speech understanding in quiet can be expected to be similar to the gain in their sound field hearing threshold. The most recent technology of Baha sound processor improves speech understanding in noise by an order of magnitude that is well perceived by users and which can be very useful in everyday life. Copyright © 2011 S. Karger AG, Basel.

  15. Static and dynamic balance of children and adolescents with sensorineural hearing loss

    OpenAIRE

    Melo, Renato de Souza; Marinho, Sônia Elvira dos Santos; Freire, Maryelly Evelly Araújo; Souza, Robson Arruda; Damasceno, Hélio Anderson Melo; Raposo, Maria Cristina Falcão

    2017-01-01

    ABSTRACT Objective To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. Methods A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Results Hearing loss students showed more changes in static ...

  16. Static and dynamic balance of children and adolescents with sensorineural hearing loss

    OpenAIRE

    Melo, Renato de Souza; Marinho, Sônia Elvira dos Santos; Freire, Maryelly Evelly Araújo; Souza, Robson Arruda; Damasceno, Hélio Anderson Melo; Raposo, Maria Cristina Falcão

    2017-01-01

    ABSTRACT Objective To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. Methods A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Results Hearing loss students showed more changes in s...

  17. Hearing loss impacts neural alpha oscillations under adverse listening conditions

    Directory of Open Access Journals (Sweden)

    Eline Borch Petersen

    2015-02-01

    Full Text Available Degradations in external, acoustic stimulation have long been suspected to increase the load on working memory. One neural signature of working memory load is enhanced power of alpha oscillations (6 ‒ 12 Hz. However, it is unknown to what extent common internal, auditory degradation, that is, hearing impairment, affects the neural mechanisms of working memory when audibility has been ensured via amplification. Using an adapted auditory Sternberg paradigm, we varied the orthogonal factors memory load and background noise level, while the electroencephalogram (EEG was recorded. In each trial, participants were presented with 2, 4, or 6 spoken digits embedded in one of three different levels of background noise. After a stimulus-free delay interval, participants indicated whether a probe digit had appeared in the sequence of digits. Participants were healthy older adults (62 – 86 years, with normal to moderately impaired hearing. Importantly, the background noise levels were individually adjusted and participants were wearing hearing aids to equalize audibility across participants. Irrespective of hearing loss, behavioral performance improved with lower memory load and also with lower levels of background noise. Interestingly, the alpha power in the stimulus-free delay interval was dependent on the interplay between task demands (memory load and noise level and hearing loss; while alpha power increased with hearing loss during low and intermediate levels of memory load and background noise, it dropped for participants with the relatively most severe hearing loss under the highest memory load and background noise level. These findings suggest that adaptive neural mechanisms for coping with adverse listening conditions break down for higher degrees of hearing loss, even when adequate hearing aid amplification is in place.

  18. Comparison between hearing screening-detected cases and sporadic cases of delayed-onset hearing loss in preschool-age children.

    Science.gov (United States)

    Lü, Jingrong; Huang, Zhiwu; Ma, Yan; Li, Yun; Mei, Ling; Yao, Guoyin; Wang, Yu; Shen, Xiaoming; Wu, Hao

    2014-04-01

    This study aimed to compare the diagnosis and ages of intervention for cases of delayed-onset hearing loss identified sporadically or via a preschool hearing screening program. Retrospective study with the comparative analysis of two groups of children. Cases identified from screening were selected from 34 321 preschool children who underwent screening for delayed-onset hearing loss between October 2009 and May 2011. Sporadic cases of delayed-onset hearing loss were selected from pediatric clinical records. Cases from the first group were excluded from the latter to avoid duplication. Two groups were given the same questionnaire to record risk indicators, diagnosis, and age at intervention. The average age of 26 children at the time of diagnosis in the screening group (52.81 ± 13.23 months) was significantly earlier than in the 33 cases identified in the sporadic group (62.03 ± 12.86 months; p children with bilateral moderate to severe hearing loss in the screening group (50.40 ± 10.76 months) was also earlier than in the sporadic group (62.73 ± 13.77 months; p hearing screening for preschool children with no significant symptoms of delayed-onset hearing loss.

  19. Shortened stapedius tendon: a rare cause of conductive hearing loss.

    Science.gov (United States)

    Zawawi, F; Varshney, R; Schloss, M D

    2014-01-01

    Anomalies of the stapedius tendon have been reported to cause conductive hearing loss; in theory, such anomalies limit the movement of the stapes. To demonstrate a rare cause of conductive hearing loss resulting from anomaly of the stapedius tendon and to compare the clinical findings of this patient to other stapedius tendon anomalies reported in the literature. Case report of a single case of shortened stapedius tendon and a review of the English literature on stapedius tendon anomalies. This is a case report of a 15-year-old boy with shortened stapedius tendon causing unilateral hearing loss, accompanied by a review of the literature. Contrary to other reported cases, this patient did not have an ossified tendon, but rather an extremely short tendon. The boy regained normal hearing following excision of the stapedius tendon. A shortened stapedius tendon is a very rare diagnosis, yet it should be considered as a possible cause of conductive hearing loss.

  20. Loss-of-Function CNKSR2 Mutation Is a Likely Cause of Non-Syndromic X-Linked Intellectual Disability

    OpenAIRE

    Houge, G.; Rasmussen, I.H.; Hovland, R.

    2011-01-01

    In a non-dysmorphic 5-year-old boy with developmental delay, well-controlled epilepsy, and microcephaly, a 234-kb deletion of Xp22.12 was detected by copy number analysis. The maternally inherited deletion removed the initial 15 of the 21 exons of the connector enhancer of KSR-2 gene called CNKSR2 or CNK2. Our finding suggests that loss of CNKSR2 is a novel cause of non-syndromic X-linked mental retardation, an assumption supported by high gene expression in the brain, localization to the pos...

  1. Screening of delayed-onset hearing loss in preschool children in the mid-south of China.

    Science.gov (United States)

    Chen, Guanming; Fu, Siqing; Luo, Shaojun; Zhang, Wei; Yang, Guoqiang

    2013-08-01

    Newborn hearing screening has been successfully implemented worldwide to improve the detection of hearing loss. However, delayed-onset hearing loss subsequent to newborn hearing screening remains a concern. This study aimed to investigate the prevalence of delayed-onset hearing loss in preschool children who previously passed newborn hearing screening in Hubei Province in mid-south China. Preschool children were screened by transient evoked otoacoustic emission (TEOAE) for delayed-onset hearing loss. Children referred after the TEOAE screening were assessed audiologically. Between March 2010 and September 2011, 28 546 preschool children (4.86 ± 1.67 years old), who had passed newborn hearing screening were targeted for screening from four cities in Hubei Province, China. During the study period, 540 children (1.89%) were referred for audiologic assessment and 22 (0.77/1000) of them had permanent delayed-onset hearing loss, including 8 (0.28/1000) with bilateral moderate hearing loss, 10 (0.35/1000) with mild bilateral hearing loss, 2 (0.07/1000) with unilateral moderate hearing loss, and 2 (0.07/1000) with unilateral mild hearing loss. Despite the success of newborn hearing screening, the provision of hearing screening in preschool remains essential for identifying delayed-onset hearing loss.

  2. Hearing loss in children with Fabry disease

    NARCIS (Netherlands)

    Suntjens, E.; Dreschler, W. A.; Hess-Erga, J.; Skrunes, R.; Wijburg, F. A.; Linthorst, G. E.; Tøndel, C.; Biegstraaten, M.

    2017-01-01

    Background Hearing loss (HL) is a well-known feature of Fabry disease (FD). Its presence and characteristics have mainly been studied in adult patients, while only limited data are available on the presence and degree of HL in children with FD. This prompted us to study hearing sensitivity in

  3. Effects of musical training and hearing loss on pitch discrimination

    DEFF Research Database (Denmark)

    Santurette, Sébastien; Bianchi, Federica; Dau, Torsten

    2018-01-01

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

  4. Occupational hearing loss of market mill workers in the city of Accra, Ghana.

    Science.gov (United States)

    Kitcher, Emmanuel D; Ocansey, Grace; Abaidoo, Benjamin; Atule, Alidu

    2014-01-01

    Noise induced hearing loss (NIHL) is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within markets in the city of Accra, Ghana, were evaluated using a structured questionnaire and pure tone audiometry. The questionnaire assessed factors including self-reported hearing loss, tinnitus, knowledge on the effects of noise on hearing health and the use of hearing protective devices. Pure tone audiometric testing was conducted for both mill workers and controls. Noise levels at the work premises of the mill workers and controls were measured. Symptoms of hearing loss were reported by 24 (23.76%) and 8 (7.7%) mill workers and controls respectively. Fifty-five (54.5%) and fifty-four (52.37%) mill workers and controls exhibited knowledge of the effects of noise on hearing health. Five (5.0%) mill workers used hearing protective devices. There was significant sensorineural hearing loss and the presence of a 4 kHz audiometric notch among mill workers when compared with controls for the mean thresholds of 2 kHz, 3 kHz and 4 kHz (P = 0. 001). The prevalence of hearing loss in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively (P hearing loss, which may be characteristic of NIHL in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively. The majority of mill workers did not use hearing protection.

  5. Occupational hearing loss of market mill workers in the city of Accra, Ghana

    Directory of Open Access Journals (Sweden)

    Emmanuel D Kitcher

    2014-01-01

    Full Text Available Noise induced hearing loss (NIHL is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within markets in the city of Accra, Ghana, were evaluated using a structured questionnaire and pure tone audiometry. The questionnaire assessed factors including self-reported hearing loss, tinnitus, knowledge on the effects of noise on hearing health and the use of hearing protective devices. Pure tone audiometric testing was conducted for both mill workers and controls. Noise levels at the work premises of the mill workers and controls were measured. Symptoms of hearing loss were reported by 24 (23.76% and 8 (7.7% mill workers and controls respectively. Fifty-five (54.5% and fifty-four (52.37% mill workers and controls exhibited knowledge of the effects of noise on hearing health. Five (5.0% mill workers used hearing protective devices. There was significant sensorineural hearing loss and the presence of a 4 kHz audiometric notch among mill workers when compared with controls for the mean thresholds of 2 kHz, 3 kHz and 4 kHz (P = 0. 001. The prevalence of hearing loss in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively (P < 0.5. The prevalence of hearing loss, which may be characteristic of NIHL in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively. The majority of mill workers did not use hearing protection.

  6. Hearing loss research from NIH | NIH MedlinePlus the Magazine

    Science.gov (United States)

    ... loss affects millions Follow us By the Numbers: Hearing Loss Affects Millions Approximately 15 percent of American ... million) aged 18 and over report some trouble hearing. That makes it the third most chronic health ...

  7. Determination of Hearing Loss Prevalence in Preschool Children of Ahwaz

    Directory of Open Access Journals (Sweden)

    Mozafar Sarafraz

    2011-03-01

    Full Text Available Introduction: Children learn to communicate by hearing sounds. If there is hearing loss, the cognitive and speaking abilities and language learning will deteriorate. Early detection and intervention are important factors in the successful treatment of hearing loss in children. Hearing loss (HL is divided into two main groups: conductive hearing loss (CHL and sensorineural hearing loss (SNHL, the prevalence of the former being higher in children, many whose causes are easy to detect and treat. Material and Methods: In this descriptive, cross-sectional study, 785 children, aged 6-7 years, entering elementary school Grade 1 in the school year 2010/2011, were randomly selected from 10% of Ahwaz Hearing Loss Screening Centers, and their audiograms were studied. The collected data were analyzed using SPSS and descriptive statistics. Results: Out of the 785 preschool children examined in this study, 77 children (9.8% suffered from HL (42.9% female and 57.1% male, 59.7% from CHL, and 40.3% from SNHL. Twenty-six percent suffered from bilateral HL and 74% from unilateral HL. Thirty-eight point ninety-six percent had abnormal tympanometry, 61% of whom were Type B. Most of the children (53% had mild HL. Thirty-one point two percent of parents were aware of their children's HL. Conclusion: Considering the high prevalence of HL, especially SNHL, in this study, which is usually permanent but detectable at the neonatal ages, raising public awareness and early screening of ear diseases, which can lead to the detection and treatment in most cases, seem to be vital.

  8. Language Outcomes in Young Children with Mild to Severe Hearing Loss.

    Science.gov (United States)

    Tomblin, J Bruce; Harrison, Melody; Ambrose, Sophie E; Walker, Elizabeth A; Oleson, Jacob J; Moeller, Mary Pat

    2015-01-01

    This study examined the language outcomes of children with mild to severe hearing loss during the preschool years. The longitudinal design was leveraged to test whether language growth trajectories were associated with degree of hearing loss and whether aided hearing influenced language growth in a systematic manner. The study also explored the influence of the timing of hearing aid fitting and extent of use on children's language growth. Finally, the study tested the hypothesis that morphosyntax may be at particular risk due to the demands it places on the processing of fine details in the linguistic input. The full cohort of children in this study comprised 290 children who were hard of hearing (CHH) and 112 children with normal hearing who participated in the Outcomes of Children with Hearing Loss (OCHL) study between the ages of 2 and 6 years. CHH had a mean better-ear pure-tone average of 47.66 dB HL (SD = 13.35). All children received a comprehensive battery of language measures at annual intervals, including standardized tests, parent-report measures, and spontaneous and elicited language samples. Principal components analysis supported the use of a single composite language score for each of the age levels (2, 3, 4, 5, and 6 years). Measures of unaided (better-ear pure-tone average, speech intelligibility index) and aided (residualized speech intelligibility index) hearing were collected, along with parent-report measures of daily hearing aid use time. Mixed modeling procedures were applied to examine the rate of change (227 CHH; 94 children with normal hearing) in language ability over time in relation to (1) degree of hearing loss, (2) aided hearing, (3) age of hearing aid fit and duration of use, and (4) daily hearing aid use. Principal components analysis was also employed to examine factor loadings from spontaneous language samples and to test their correspondence with standardized measures. Multiple regression analysis was used to test for

  9. The Potential in Preparing Community Health Workers to Address Hearing Loss.

    Science.gov (United States)

    Sánchez, Daisey; Adamovich, Stephanie; Ingram, Maia; Harris, Frances P; de Zapien, Jill; Sánchez, Adriana; Colina, Sonia; Marrone, Nicole

    2017-06-01

    In underserved areas, it is crucial to investigate ways of increasing access to hearing health care. The community health worker (CHW) is a model that has been applied to increase access in various health arenas. This article proposes further investigation into the application of this model to audiology. To assess the feasibility of training CHWs about hearing loss as a possible approach to increase accessibility of hearing health support services in an underserved area. A specialized three-phase training process for CHWs was developed, implemented, and evaluated by audiologists and public health researchers. The training process included (1) focus groups with CHWs and residents from the community to raise awareness of hearing loss among CHWs and the community; (2) a 3-hr workshop training to introduce basic topics to prepare CHWs to identify signs of hearing loss among community members and use effective communication strategies; and (3) a 24-hr multisession, interactive training >6 weeks for CHWs who would become facilitators of educational and peer-support groups for individuals with hearing loss and family members. Twelve Spanish-speaking local CHWs employed by a federally qualified health center participated in a focus group, twelve received the general training, and four individuals with prior experience as health educators received further in-person training as facilitators of peer-education groups on hearing loss and communication. Data was collected from each step of the three-phase training process. Thematic analysis was completed for the focus group data. Pre- and posttraining assessments and case study discussions were used to analyze results for the general workshop and the in-depth training sessions. CHWs increased their knowledge base and confidence in effective communication strategies and developed skills in facilitating hearing education and peer-support groups. Through case study practice, CHWs demonstrated competencies and applied their learning

  10. Experiences of adult patients hearing loss postlingually with Cochlear Implant

    Directory of Open Access Journals (Sweden)

    Teresa María Lizcano Tejado

    2013-09-01

    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.

  11. Speech and Language Consequences of Unilateral Hearing Loss: A Systematic Review.

    Science.gov (United States)

    Anne, Samantha; Lieu, Judith E C; Cohen, Michael S

    2017-10-01

    Objective Unilateral hearing loss has been shown to have negative consequences for speech and language development in children. The objective of this study was to systematically review the current literature to quantify the impact of unilateral hearing loss on children, with the use of objective measures of speech and language. Data Sources PubMed, EMBASE, Medline, CINAHL, and Cochrane Library were searched from inception to March 2015. Manual searches of references were also completed. Review Methods All studies that described speech and language outcomes for children with unilateral hearing loss were included. Outcome measures included results from any test of speech and language that evaluated or had age-standardized norms. Due to heterogeneity of the data, quantitative analysis could not be completed. Qualitative analysis was performed on the included studies. Two independent evaluators reviewed each abstract and article. Results A total of 429 studies were identified; 13 met inclusion criteria and were reviewed. Overall, 7 studies showed poorer scores on various speech and language tests, with effects more pronounced for children with severe to profound hearing loss. Four studies did not demonstrate any difference in testing results between patients with unilateral hearing loss and those with normal hearing. Two studies that evaluated effects on speech and language longitudinally showed initial speech problems, with improvement in scores over time. Conclusions There are inconsistent data regarding effects of unilateral hearing loss on speech and language outcomes for children. The majority of recent studies suggest poorer speech and language testing results, especially for patients with severe to profound unilateral hearing loss.

  12. Towards a comprehensive approach for managing transitions of older workers with hearing loss.

    Science.gov (United States)

    Fok, Daniel; Shaw, Lynn; Jennings, Mary Beth; Cheesman, Margaret

    2009-01-01

    Demographic and legislative trends suggest that many older workers may remain at work past the traditional retirement age. This extended work trajectory poses new challenges and opportunities for workers with acquired hearing loss as they age. Workplaces require a new approach to enable transitions of older workers with hearing loss to remain safe and productive. A review of the literature on older workers, those with hearing loss, and strategies used to accommodate them suggests that individualized and piecemeal approaches are predominant. While universal design represents a fresh ideology that may help create more accessible and usable products and environments, its application to improve workplaces for older workers with hearing loss is limited. This paper proposes that occupational science be integrated with knowledge in hearing sciences, accessibility, and usability to assist with the transitions faced by older workers with hearing loss. A more comprehensive approach including the following three key components will be posited to examine the nexus of aging, hearing loss and work: (1) the use of an occupational perspective, along with concepts in hearing sciences to examine hearing demands and improve hearing access; (2) the use of contextual processes to promote physical and social change, and (3) the inclusion of Universal Design for Hearing (UDH) considerations as stakeholders develop more hearing friendly workplaces.

  13. Quantification of hearing loss in patients with posterior semicircular canal dehiscence.

    Science.gov (United States)

    Bear, Zachary W; McEvoy, Timothy P; Mikulec, Anthony A

    2015-01-01

    Patients with posterior semicircular canal dehiscence (PSCD) have low frequency conductive hearing loss similar to patients with superior semicircular canal dehiscence (SSCD) secondary to a pathologic third window. PSCD can result in conductive hearing loss, but the magnitude of this hearing loss remains to be quantified. Patients with SSCD have been shown to have low frequency conductive hearing loss. The underlying pathophysiology of hearing loss from PSCD and SSCD is similar and related to a pathologic third window. A PubMed search was completed for a meta-analysis of patients with PSCD. Articles with quality audiograms were obtained. Air conduction thresholds for ears with posterior semicircular canal dehiscence were compared to the opposite ear as well as normal control data. Eight articles with 21 patients with PSCD and quality audiograms were included. Two patients had bilateral PSCD and one of those was excluded because hearing thresholds were at the limit of the audiometer. Patients with posterior semicircular canal dehiscence have statistically significant lower air conduction thresholds in frequencies at and below 2000 Hz.

  14. Hearing loss and speech perception in noise difficulties in Fanconi anemia.

    Science.gov (United States)

    Verheij, Emmy; Oomen, Karin P Q; Smetsers, Stephanie E; van Zanten, Gijsbert A; Speleman, Lucienne

    2017-10-01

    Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties). Our study aimed to describe the prevalence of hearing loss and speech perception in noise difficulties in Dutch Fanconi anemia patients. Retrospective chart review. A retrospective chart review was conducted at a Dutch tertiary care center. All patients with Fanconi anemia at clinical follow-up in our hospital were included. Medical files were reviewed to collect data on hearing loss and speech perception in noise difficulties. In total, 49 Fanconi anemia patients were included. Audiograms were available in 29 patients and showed hearing loss in 16 patients (55%). Conductive hearing loss was present in 24.1%, sensorineural in 20.7%, and mixed in 10.3%. A speech in noise test was performed in 17 patients; speech perception in noise was subnormal in nine patients (52.9%) and abnormal in two patients (11.7%). Hearing loss and speech perception in noise abnormalities are common in Fanconi anemia. Therefore, pure tone audiograms and speech in noise tests should be performed, preferably already at a young age, because hearing aids or assistive listening devices could be very valuable in developing language and communication skills. 4. Laryngoscope, 127:2358-2361, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  15. Mutation analysis of GJB2 gene and prenatal diagnosis in a non-syndromic deafness family

    Directory of Open Access Journals (Sweden)

    Xiao-hua CHEN

    2014-08-01

    Full Text Available Objective To identify the pathogenic gene in a non-syndromic deafness family, provide an accurate genetic consultation and early intervention for deaf family to reduce the incidence of congenital deafness. Methods Mutation analysis was carried out by polymerase chain reaction followed by DNA sequencing of coding region of GJB2 gene. The fetal DNA was extracted from the amniotic fluid cells by amniocentesis at 20 weeks during pregnancy. The genotype of the fetus was characterized for predicting the status of hearing. Results Complex heterozygous mutations 235delC and 176-191del16bp were detected in the proband of the family, heterozygous mutation 176-191del16bp was detected in the father, and 235delC was detected in the mother. Fetus carried 235delC heterozygous mutation inherited from his mother. Conclusions The proband's hearing loss is resulted from the complex heterozygous mutations 235delC and 176-191del16bp in GJB2 gene. Fetus is a heterozygous mutation 235delC carrier. Prenatal diagnosis for deafness assisted by genetic test can provide efficient guidance about offspring's hearing condition, and prevent another deaf-mute member from birth. DOI: 10.11855/j.issn.0577-7402.2014.07.09

  16. Nonorganic hearing loss in children: audiometry, clinical characteristics, biographical history and recovery of hearing thresholds.

    Science.gov (United States)

    Schmidt, Claus-Michael; am Zehnhoff-Dinnesen, Antoinette; Matulat, Peter; Knief, Arne; Rosslau, Ken; Deuster, Dirk

    2013-07-01

    The term "nonorganic hearing loss" (NOHL) (pseudohypacusis, functional or psychogenic hearing loss) describes a hearing loss without a detectable corresponding pathology in the auditory system. It is characterized by a discrepancy between elevated pure tone audiometry thresholds and normal speech discrimination. The recommended audiological management of NOHL in children comprises history taking, diagnosis, and counseling. According to the literature, prognosis depends on the severity of the patient's school and/or personal problems. Routine referral to a child psychiatrist is discussed as being controversial. The clinical history of 34 children with NOHL was retrospectively evaluated. In 15 children, follow up audiometry was performed. Results of biographical history, subjective and objective audiometry, additional speech and language assessment, psychological investigations and follow up audiometry are presented and discussed. The prevalence of NOHL was 1.8% in children with suspected hearing loss. Mean age at diagnosis was 10.8 years. Girls were twice as often affected as boys. Patient history showed a high prevalence of emotional and school problems. Pre-existing organic hearing loss can be worsened by nonorganic causes. Children with a fast recovery of hearing thresholds (n=6) showed a high rate (4/6) of family, social and emotional problems. In children with continuous threshold elevation (n=9), biographical history showed no recognizable or obvious family, social or emotional problems; learning disability (4/9) was the most frequently presented characteristic. Due to advances in objective audiometry, the diagnosis of NOHL is less challenging than management and counseling. Considering the high frequency of personal and school problems, a multidisciplinary setting is helpful. On the basis of our results, drawing conclusions from hearing threshold recovery on the severity of underlying psychic problems seems inappropriate. As a consequence, a referral to a

  17. Osteogenesis imperfecta and hearing loss--description of three case reports.

    Science.gov (United States)

    Pereira da Silva, Ana; Feliciano, Telma; Figueirinhas, Rosário; Almeida E Sousa, Cecília

    2013-01-01

    Osteogenesis imperfecta is the commonest connective tissue hereditary disease. Its clinical presentation has a wide spectrum of characteristics, which includes skeletal deformities and hearing loss. We describe three case reports of individuals carriers of this disease presenting with different patterns of hearing loss. Hearing loss prevalence and patterns are variable and have no clear relation with genotype. Its assessment at initial evaluation and posterior monitoring is essential to provide the best therapeutic alternatives. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  18. Noise-induced hearing loss: an occupational medicine perspective.

    Science.gov (United States)

    Stucken, Emily Z; Hong, Robert S

    2014-10-01

    Up to 30 million workers in the United States are exposed to potentially detrimental levels of noise. Although reliable medications for minimizing or reversing noise-induced hearing loss (NIHL) are not currently available, NIHL is entirely preventable. The purpose of this article is to review the epidemiology and pathophysiology of occupational NIHL. We will focus on at-risk populations and discuss prevention programs. Current prevention programs focus on reducing inner ear damage by minimizing environmental noise production and through the use of personal hearing protective devices. NIHL is the result of a complex interaction between environmental factors and patient factors, both genetic and acquired. The effects of noise exposure are specific to an individual. Trials are currently underway evaluating the role of antioxidants in protection from, and even reversal of, NIHL. Occupational NIHL is the most prevalent occupational disease in the United States. Occupational noise exposures may contribute to temporary or permanent threshold shifts, although even temporary threshold shifts may predispose an individual to eventual permanent hearing loss. Noise prevention programs are paramount in reducing hearing loss as a result of occupational exposures.

  19. WFS1 and non-syndromic low-frequency sensorineural hearing loss: a novel mutation in a Portuguese case.

    Science.gov (United States)

    Gonçalves, A C; Matos, T D; Simões-Teixeira, H R; Pimenta Machado, M; Simão, M; Dias, O P; Andrea, M; Fialho, G; Caria, H

    2014-04-01

    Low-frequency sensorineural hearing loss (LFSNHL) is an unusual type of HL in which frequencies at 2,000 Hz and below are predominantly affected. Most of the families with LFSNHL carry missense mutations in WFS1 gene, coding for wolframin. A Portuguese patient aged 49, reporting HL since her third decade of life, and also referring tinnitus, was shown to display bilateral moderate LFSNHL after audiological evaluation. Molecular analysis led to the identification of a novel mutation, c.511G>A (p.Asp171Asn), found in heterozygosity in the exon 5 of the WFS1 gene, and changing the aspartic acid at position 171 to an asparagine, in the extracellular N-terminus domain of the wolframin protein. This novel mutation wasn't present either in 200 control chromosomes analyzed or in the hearing proband's half-brother, and it had not been reported in 1000 Genomes, Exome Variant Server, HGMD or dbSNP databases. No mutations were found in GJB2 and GJB6 genes. Multi-alignment of 27 wolframin sequences from mammalian species, against the human wolframin sequence in ConSurf, indicated a conservation score corresponding to 7 in a 1-9 color scale where 9 is conserved and 1 is variable. In addition, the mutation p.Asp171Asn was predicted to be damaging and possibly damaging by SIFT and Polyphen-2, respectively. The auditory phenotype of this patient could thus be due to the novel mutation p.Asp171Asn. Further functional characterization might enable to elucidate in which way the change in the residue 171, as other changes introduced by LFSNHL-associated mutations previously described, leads to this type of HL. Copyright © 2014 Elsevier B.V. All rights reserved.

  20. Risk of hearing loss among workers with vibration-induced white fingers.

    Science.gov (United States)

    Pettersson, Hans; Burström, Lage; Hagberg, Mats; Lundström, Ronnie; Nilsson, Tohr

    2014-12-01

    We examined the risk of hearing loss for workers who use hand-held vibrating tools with vibration-induced white fingers (VWF) compared to workers without VWF. Data on 184 participants from a 21-year cohort were gathered with questionnaires and measurements. The effects on hearing status of VWF, hand-arm vibration exposure, smoking habits, age and two-way interactions of these independent variables were examined with binary logistic regression. Analyses were made for the right hand and ear as well as for the hand with VWF and the ear with worse categorized hearing status. Workers with VWF in their right hand had an increased risk of hearing loss (odds ratio 2.2-2.3) in the right ear. Workers with VWF in any hand did not have any increased risk of hearing loss in the ear with worse hearing status. This study supports the hypothesis that VWF increases the risk of hearing loss among workers who use hand-held vibrating tools in a noisy environment. © 2014 Wiley Periodicals, Inc.

  1. The relationships between cognitive function and hearing loss among the elderly.

    Science.gov (United States)

    Huh, MyungJin

    2018-01-01

    [Purpose] Research related to dementia has gained momentum in South Korea and studies have found that the auditory sense affects dementia. This study aims to examine the relationship between the decline in hearing function and the overall cognitive function among the elderly. [Subjects and Methods] Eighty-two older adults aged 65-90 years (mean age: 79.3, SD: 5.2) participated. The Korean Mini-Mental State Examination was used to assess cognitive function. Further, to assess the hearing function, pure-tone audiometry was performed prior to the cognitive function test. We used a paired t-test and Pearson's correlation test for the analysis. [Results] Generally, the higher the frequency band, the more hearing loss was identified among the elderly. In addition, the difference in hearing between both ears was significant; particularly, hearing loss in the right ear was significantly higher than that in the left. Cognitive function was not related to age, however, the correlation between cognitive function and hearing loss in the right ear was statistically significant. [Conclusion] Hearing loss influences cognitive function among the elderly.

  2. Hearing Parents of Children with Hearing Loss: Perceptions of the IEP Process

    Science.gov (United States)

    Stegman, Robin Fern

    2016-01-01

    Under federal guidelines, parents of school-aged children with hearing loss are required to attend an individualized education program (IEP) meeting on behalf of their child. However, it remains unclear how prepared hearing parents are to oversee development of IEPs that guarantee their children the best educational outcomes, as well as how much…

  3. Association of hearing loss with decreased employment and income among adults in the United States.

    Science.gov (United States)

    Jung, David; Bhattacharyya, Neil

    2012-12-01

    We evaluated the association of hearing loss with employment and income in adults. Patients with a coded diagnosis of hearing loss were identified from the 2006 and 2008 Medical Expenditure Panel Survey linked household and medical conditions files and compared to patients without hearing loss. Differences in employment, wage income, and Supplemental Security Income were evaluated with multivariate regression models after adjustment for several demographic and Charlson comorbidity variables. An estimated 933,921 +/- 88,474 adults were identified with hearing loss (54.7% of whom were male; mean age for all, 51.0 years). Patients with hearing loss were more likely to be unemployed or partly unemployed than those without hearing loss (adjusted odds ratio, 2.2; p hearing loss were less likely to have any wage income than those without hearing loss (adjusted odds ratio, 2.5; p hearing loss earned a mean wage of $23,481 +/- $3,366, versus $31,272 +/- $517 for the population without hearing loss (difference in wages, $7,791; p hearing loss and receiving Supplemental Security Income was not significant (p = 0.109). Adults with hearing loss are more likely to be unemployed and on average earn significantly less wage income than adults without hearing loss. Further work is needed to determine the potential impact of treatment on these differences.

  4. Exploring reasons for late identification of children with early-onset hearing loss.

    Science.gov (United States)

    Fitzpatrick, Elizabeth M; Dos Santos, Johnny Cesconetto; Grandpierre, Viviane; Whittingham, JoAnne

    2017-09-01

    Several studies have shown that early identification of childhood hearing loss leads to better language outcomes. However, delays in the confirmation of hearing loss persist even in the presence of well-established universal newborn hearing screening programs (UNHS). The objective of this population-based study was to document the proportion of children who experienced delayed confirmation of congenital and early onset hearing loss in a UNHS program in one region of Canada. The study also sought to determine the reasons for delayed confirmation of hearing loss in children. Population level data related to age of first assessment, age of identification and clinical characteristics were collected prospectively for all children identified through the UNHS program. We documented the number of children who experienced delay (defined as more than 3 months) from initial audiologic assessment to confirmation of hearing loss. A detailed chart review was subsequently performed to examine the reasons for delay to confirmation. Of 418 children identified from 2003 to 2013, 182 (43.5%) presented with congenital or early onset hearing loss, of whom 30 (16.5%) experienced more than 3 months delay from initial audiologic assessment to confirmation of their hearing disorder. The median age of first assessment and confirmation of hearing loss for these 30 children was 3.7 months (IQR: 2.0, 7.6) and 13.8 months (IQR: 9.7, 26.1) respectively. Close examination of the factors related to delay to confirmation revealed that for the overwhelming majority of children, a constellation of factors contributed to late diagnosis. Several children (n = 22; 73.3%) presented with developmental/medical issues, 15 of whom also had middle ear dysfunction at assessment, and 9 of whom had documented family follow-up concerns. For the remaining eight children, additional reasons included ongoing middle ear dysfunction for five children, complicated by family follow-up concerns (n = 3) and mild

  5. Is otolithic vertigo accompanied by hearing loss caused by sacculocochlear endolymphatic hydrops?

    Science.gov (United States)

    Murofushi, Toshihisa; Komiyama, Sakurako; Hayashi, Yushi; Yoshimura, Eriko

    2016-01-01

    Otolithic vertigo is sometimes accompanied by hearing loss. Otolithic vertigo accompanied by hearing loss seems to be caused by sacculocochlear endolymphatic hydrops. To clarify the lesion site and pathophysiology of otolithic vertigo (OV) accompanied by hearing loss. The clinical records of four patients (two men and two women) that had been diagnosed with OV accompanied by hearing loss according to pre-determined diagnostic criteria were reviewed. The patients' main symptoms involved a sensation of movement in the pitch plane. All of the patients had low frequency-dominant hearing loss and either exhibited decreased cervical vestibular evoked myogenic potentials (cVEMP) or did not produce cVEMP. Two patients produced normal ocular VEMP (oVEMP). Caloric tests obtained normal results in all patients.

  6. A Socio-Ecological Approach in Addressing Hearing Loss and Disparities in Access to Hearing Health Care Among Older Adults

    Directory of Open Access Journals (Sweden)

    Maia Ingram

    2016-08-01

    Full Text Available Hearing loss is associated with cognitive decline and impairment in daily living activities. Access to hearing health care has broad implications for healthy aging of the U.S. population. This qualitative study investigated factors related to the socio-ecological domains of hearing health in a US-Mexico border community experiencing disparities in access to care. A multidisciplinary research team partnered with Community Health Workers (CHWs from a Federally Qualified Health Center in designing the study. CHWs conducted interviews with people with hearing loss (n=20 and focus groups with their family/friends (n=27 and with members of the community-at-large (n=47. The research team conducted interviews with FQHC providers and staff (n=12. Individuals experienced depression, sadness and social isolation, as well as frustration and even anger regarding communication. Family members experienced negative impacts of deteriorating communication, but expressed few coping strategies. There was general agreement across data sources that hearing loss was not routinely addressed within primary care and assistive hearing technology was generally unaffordable. Community members described stigma related to hearing loss and a need for greater access to hearing health care and broader community education. Findings confirm the causal sequence of hearing impairment on quality of life aggravated by socio-economic conditions and lack of access to hearing health care. Hearing loss requires a comprehensive and innovative public health response across the socio-ecological framework that includes both individual communication intervention and greater access to hearing health resources. Community health workers can be effective in tailoring intervention strategies to community characteristics.

  7. Hearing loss in former prisoners of war of the Japanese.

    Science.gov (United States)

    Grossman, T W; Kerr, H D; Byrd, J C

    1996-09-01

    To describe the prevalence, degree, and types of hearing loss present in a group of older American veterans who had been prisoners of war of the Japanese. A descriptive study. A Veterans Affairs university hospital. Seventy-five male veterans, mean age 68 (+/- 3.6) years. Hearing aids were prescribed for eight veterans. Subjects were examined, and pure tone air and bone conduction, speech reception threshold, and speech discrimination were determined. Results were compared with age- and sex-matched controls from the largest recent American population study of hearing loss. 95% of subjects had been imprisoned longer than 33 months. Starvation conditions (100%), head trauma (85%), and trauma-related loss of consciousness (23%) were commonly reported. A total of 73% complained of hearing loss, and 29% (22/75) dated its onset to captivity. Most of those with the worst losses in hearing and speech discrimination were found in this subgroup. When the entire group was compared with published age- and sex-matched controls from the Framingham Study, no significant differences were found. We advocate screening examinations and long-term follow-up of populations with similar histories of starvation, head trauma, and torture.

  8. Fractionated Stereotactic Radiotherapy of Vestibular Schwannomas Accelerates Hearing Loss

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    Rasmussen, Rune, E-mail: rune333@gmail.com [Department of Neurosurgery, Rigshospitalet, Copenhagen (Denmark); Claesson, Magnus [Department of Neurosurgery, Rigshospitalet, Copenhagen (Denmark); Stangerup, Sven-Eric [Ear, Nose, and Throat Department, Rigshospitalet, Copenhagen (Denmark); Roed, Henrik [Department of Radiation Oncology, Rigshospitalet, Copenhagen (Denmark); Christensen, Ib Jarle [Finsen Laboratory, Rigshospitalet, Copenhagen (Denmark); Caye-Thomasen, Per [Ear, Nose, and Throat Department, Rigshospitalet, Copenhagen (Denmark); Juhler, Marianne [Department of Neurosurgery, Rigshospitalet, Copenhagen (Denmark)

    2012-08-01

    Objective: To evaluate long-term tumor control and hearing preservation rates in patients with vestibular schwannoma treated with fractionated stereotactic radiotherapy (FSRT), comparing hearing preservation rates to an untreated control group. The relationship between radiation dose to the cochlea and hearing preservation was also investigated. Methods and Materials: Forty-two patients receiving FSRT between 1997 and 2008 with a minimum follow-up of 2 years were included. All patients received 54 Gy in 27-30 fractions during 5.5-6.0 weeks. Clinical and audiometry data were collected prospectively. From a 'wait-and-scan' group, 409 patients were selected as control subjects, matched by initial audiometric parameters. Radiation dose to the cochlea was measured using the original treatment plan and then related to changes in acoustic parameters. Results: Actuarial 2-, 4-, and 10-year tumor control rates were 100%, 91.5%, and 85.0%, respectively. Twenty-one patients had serviceable hearing before FSRT, 8 of whom (38%) retained serviceable hearing at 2 years after FSRT. No patients retained serviceable hearing after 10 years. At 2 years, hearing preservation rates in the control group were 1.8 times higher compared with the group receiving FSRT (P=.007). Radiation dose to the cochlea was significantly correlated to deterioration of the speech reception threshold (P=.03) but not to discrimination loss. Conclusion: FSRT accelerates the naturally occurring hearing loss in patients with vestibular schwannoma. Our findings, using fractionation of radiotherapy, parallel results using single-dose radiation. The radiation dose to the cochlea is correlated to hearing loss measured as the speech reception threshold.

  9. Fractionated Stereotactic Radiotherapy of Vestibular Schwannomas Accelerates Hearing Loss

    International Nuclear Information System (INIS)

    Rasmussen, Rune; Claesson, Magnus; Stangerup, Sven-Eric; Roed, Henrik; Christensen, Ib Jarle; Cayé-Thomasen, Per; Juhler, Marianne

    2012-01-01

    Objective: To evaluate long-term tumor control and hearing preservation rates in patients with vestibular schwannoma treated with fractionated stereotactic radiotherapy (FSRT), comparing hearing preservation rates to an untreated control group. The relationship between radiation dose to the cochlea and hearing preservation was also investigated. Methods and Materials: Forty-two patients receiving FSRT between 1997 and 2008 with a minimum follow-up of 2 years were included. All patients received 54 Gy in 27-30 fractions during 5.5-6.0 weeks. Clinical and audiometry data were collected prospectively. From a “wait-and-scan” group, 409 patients were selected as control subjects, matched by initial audiometric parameters. Radiation dose to the cochlea was measured using the original treatment plan and then related to changes in acoustic parameters. Results: Actuarial 2-, 4-, and 10-year tumor control rates were 100%, 91.5%, and 85.0%, respectively. Twenty-one patients had serviceable hearing before FSRT, 8 of whom (38%) retained serviceable hearing at 2 years after FSRT. No patients retained serviceable hearing after 10 years. At 2 years, hearing preservation rates in the control group were 1.8 times higher compared with the group receiving FSRT (P=.007). Radiation dose to the cochlea was significantly correlated to deterioration of the speech reception threshold (P=.03) but not to discrimination loss. Conclusion: FSRT accelerates the naturally occurring hearing loss in patients with vestibular schwannoma. Our findings, using fractionation of radiotherapy, parallel results using single-dose radiation. The radiation dose to the cochlea is correlated to hearing loss measured as the speech reception threshold.

  10. Masking Release in Children and Adults with Hearing Loss When Using Amplification

    Science.gov (United States)

    Brennan, Marc; McCreery, Ryan; Kopun, Judy; Lewis, Dawna; Alexander, Joshua; Stelmachowicz, Patricia

    2016-01-01

    Purpose: This study compared masking release for adults and children with normal hearing and hearing loss. For the participants with hearing loss, masking release using simulated hearing aid amplification with 2 different compression speeds (slow, fast) was compared. Method: Sentence recognition in unmodulated noise was compared with recognition…

  11. Hearing Loss in otitis media with effusion- Types and management- A study of hundred cases

    Directory of Open Access Journals (Sweden)

    Sriram Nathan

    2012-01-01

    Full Text Available Introduction- Serous Ottis media or otitis media with effusion is a common cause of hearing loss of acute onset. The common presentation will be a block or reduced hearing possible after a travel or flight. Most of the cases present with conductive hearing loss while some may present with mixed or even pure sensory hearing loss. Background- we study hundred patients diagnosed with serous otitis media for the type of hearing loss and characterize the management strategy. Conclusion- Serous otitis media is a common cause of hearing loss which is mostly conductive and is amenable to treatment while some rare cases which may persist may require assistive hearing with amplification. Although sensorineral hearing loss is rare in otitis media, it is seen in practice along with mixed hearing loss. The pathophysiology of the neural affection of hearing loss remains a mystery although many theories exist.

  12. Hearing Loss Associated with US Military Combat Deployment

    Science.gov (United States)

    2015-02-01

    Jason M. Jones Tomoko I. Hooper Isabel G. Jacobson Edward J. Boyko Report No. 13-59 The views expressed in this article are those of the...limited. A study of US Army soldiers who visited audiology clinics noted that hearing loss was identifi ed in 68.6% of post-deployment diagnoses and...disorders, hearing loss, military personnel Access this article online Quick Response Code: Website: www.noiseandhealth.org DOI: 10.4103/1463

  13. Representations of workers with hearing loss in Canadian newspapers: a thematic analysis.

    Science.gov (United States)

    Koerber, Raphaelle; Jennings, Mary Beth; Shaw, Lynn; Cheesman, Margaret

    2017-04-01

    Participation in the labour force with a hearing impairment presents a number of challenges. This study describes how Canadian newspapers represent workers with hearing loss. Taking a critical framing theory approach, thematic analysis was performed through coding relevant articles, abstracting and hierarchically categorising themes. Seven English-language Canadian newspapers were searched for publications between 1995 and 2016. Twenty-six articles met our criteria: discussing paid workers with hearing loss who used English rather than sign language on the job and making reference to workers' competence. We identified a global theme, Focussing on a good worklife or focussing on a limited worklife, composed of three organising themes (1) Prominent individuals struggle, take action, and continue despite hearing loss, (2) Workers with hearing loss in the community create their best day themselves, and (3) Workers with hearing loss, as a generalised whole, are portrayed as either competent or limited. The dominant framing portrays individual workers as ingenious, determined, and successful. Negative framings were predominantly generalisations to these workers as a group. To generate more positive framings, professionals can build relationships with consumer groups and, when contacted by the media, direct journalists to interview workers with hearing loss.

  14. Is hearing loss a feature of Joubert syndrome, a ciliopathy?

    NARCIS (Netherlands)

    Kroes, H.Y.; Van Zanten, B.G.A.; De Ru, S.A.; Boon, M.; Mancini, G.M.S.; van der Knaap, M.S.; Poll-The, B.; Lindhout, D.

    2010-01-01

    Objective: To assess if hearing loss is a feature of Joubert syndrome (JBS), one of the ciliopathies and therefore possibly associated with hearing loss. Design: Retrospective case series. Setting: University Children's Hospital. Patients: Dutch patients with JBS. Main outcome measures: Audiological

  15. Interventions to prevent occupational noise induced hearing loss

    NARCIS (Netherlands)

    Verbeek, Jos H.; Kateman, Erik; Morata, Thais C.; Dreschler, Wout; Sorgdrager, Bas

    2009-01-01

    BACKGROUND: Millions of workers worldwide are exposed to noise levels that increase their risk of hearing impairment. Little is known about the effectiveness of hearing loss prevention interventions. OBJECTIVES: To assess the effectiveness of non-pharmaceutical interventions for preventing

  16. Interventions to prevent occupational noise-induced hearing loss

    NARCIS (Netherlands)

    Verbeek, Jos H.; Kateman, Erik; Morata, Thais C.; Dreschler, Wouter A.; Mischke, Christina

    2012-01-01

    Background Millions of workers worldwide are exposed to noise levels that increase their risk of hearing impairment. Little is known about the effectiveness of hearing loss prevention interventions. Objectives To assess the effectiveness of non-pharmaceutical interventions for preventing

  17. Bilateral sudden sensorineural hearing loss as a first symptom of infective endocarditis: two case reports.

    Science.gov (United States)

    Chroni, M; Prappa, E; Kokkevi, I

    2018-04-01

    Septic emboli are an unusual cause of sudden sensorineural hearing loss, for which few reports exist in the literature. This paper presents two cases of sudden sensorineural hearing loss, initially considered as idiopathic, but which were caused by septic emboli. Hearing loss in these cases was bilateral, sequential and total. The first patient had mild fever one week prior to their presentation with sudden sensorineural hearing loss; the other patient had no additional symptoms at presentation. These patients were later diagnosed with infective endocarditis, at two and seven months following the sudden sensorineural hearing loss respectively, showing that septic emboli had been the cause of sudden sensorineural hearing loss. Septic emboli should be considered as a possible cause of sudden sensorineural hearing loss in cases of total hearing loss. This form of hearing loss should prompt the otolaryngologist to further investigate for infective endocarditis.

  18. Conductive and Mixed Hearing Losses: A Comparison between Summer and Autumn.

    Science.gov (United States)

    Nickbakht, Mansoureh; Borzoo, Samira

    2014-04-01

    Conductive hearing loss is common among children and adults. This study aims at comparing the results of conductive hearing loss in summer and autumn. Puretone audiometry and tympanometry tests were done for all patients who referred to the Iranian-based audiology center of Imam Khomeini Hospital in Ahvaz. Data on the patients with conductive or mixed hearing loss were analyzed. The impacts of season, age, and etiology of the disease were analyzed on the patients who visited the audiology clinic due to the conductive hearing loss in summer and autumn. One hundred and fifty nine patients in summer and 123 patients in autumn had conductive or mixed hearing loss. Their age ranged from four to 82 years, with the average age of 35. The percentage of the patients, with acute otitis media and chronic otitis media (COM), who visited this clinic, was significantly higher than those with middle ear problems. COM and mastoid surgeries rate was higher in summer than autumn among adults. There is no relationship between season and middle ear diseases between children and juveniles, but COM and mastoid problems are more common in summer among adults visiting this clinic. Most of the patients had mild conductive hearing loss and bilateral middle ear impairments.

  19. Sensorineural hearing loss following irradiation to the malignant tumor of the head and neck

    International Nuclear Information System (INIS)

    Murakami, Masafumi; Kobari, Hitomi; Kanno, Hidetaka; Aikawa, Tohru; Anzai, Tomohiro; Okamura, Hiro-oki; Ohtani, Iwao; Hoshino, Toshiaki

    1989-01-01

    We observed sensorineural hearing loss following X-ray irradiation to the malignant tumor of head and neck. There were 24 patients whose auditory organs lied within the irradiation field. Ten of these patients were affected by sensorineural hearing loss. Hearing loss occurred at a high frequency in elderly patients, epipharynx tumor and high dose of irradiation. Many cases revealed high tone hearing loss. Most cases showed about a 20∼30 dB hearing loss, so their impediment seemed not severe in daily life. In some of these cases, we could have temporal bone findings, but there were no particular findings relevant to sensorineural hearing loss. (author)

  20. The hearing benefit of cochlear implantation for individuals with unilateral hearing loss, but no tinnitus.

    Science.gov (United States)

    Skarzynski, Henryk; Lorens, Artur; Kruszynska, Marika; Obrycka, Anita; Pastuszak, Dorota; Skarzynski, Piotr Henryk

    2017-07-01

    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.

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

    Science.gov (United States)

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

    2017-07-22

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

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

    Science.gov (United States)

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

    2015-01-01

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

  3. Social Skills in Preschool Children with Unilateral and Mild Bilateral Hearing Loss

    Science.gov (United States)

    Laugen, Nina J.; Jacobsen, Karl H.; Rieffe, Carolien; Wichstrøm, Lars

    2017-01-01

    Hearing loss may represent a risk for developing social skills difficulties; however, little is known about the potential risk resulting from unilateral or mild bilateral hearing loss (UMHL). We compared the social skills of 14 children with UMHL and 21 children with moderate to severe hearing loss (MSHL) with those of 123 children with typical…

  4. Is hearing loss a feature of Joubert syndrome, a ciliopathy?

    NARCIS (Netherlands)

    Kroes, Hester Y.; Van Zanten, Bert G. A.; De Ru, Sander A.; Boon, Maartje; Mancini, Grazia M. S.; Van der Knaap, Marjo S.; Poll-The, Bwee Tien; Lindhout, Dick

    Objective To assess if hearing loss is a feature of Joubert syndrome (JBS). one of the ciliopathies and therefore possibly associated with hearing loss Design: Retrospective case series. Setting University Children's Hospital Patients Dutch patients with JBS. Main outcome measures Audiological data.

  5. Is hearing loss a feature of Joubert syndrome, a ciliopathy?

    NARCIS (Netherlands)

    Kroes, Hester Y.; van Zanten, Bert G. A.; de Ru, Sander A.; Boon, Maartje; Mancini, Grazia M. S.; van der Knaap, Marjo S.; Poll-The, Bwee Tien; Lindhout, Dick

    2010-01-01

    Objective To assess if hearing loss is a feature of Joubert syndrome (JBS). one of the ciliopathies and therefore possibly associated with hearing loss Design: Retrospective case series. Setting University Children's Hospital Patients Dutch patients with JBS. Main outcome measures Audiological data.

  6. Age-related changes in auditory and cognitive abilities in elderly persons with hearing aids fitted at the initial stages of hearing loss

    Directory of Open Access Journals (Sweden)

    C. Obuchi

    2011-03-01

    Full Text Available In this study, we investigated the relation between the use of hearing aids at the initial stages of hearing loss and age-related changes in the auditory and cognitive abilities of elderly persons. 12 healthy elderly persons participated in an annual auditory and cognitive longitudinal examination for three years. According to their hearing level, they were divided into 3 subgroups - the normal hearing group, the hearing loss without hearing aids group, and the hearing loss with hearing aids group. All the subjects underwent 4 tests: pure-tone audiometry, syllable intelligibility test, dichotic listening test (DLT, and Wechsler Adult Intelligence Scale-Revised (WAIS-R Short Forms. Comparison between the 3 groups revealed that the hearing loss without hearing aids group showed the lowest scores for the performance tasks, in contrast to the hearing level and intelligibility results. The other groups showed no significant difference in the WAIS-R subtests. This result indicates that prescription of a hearing aid during the early stages of hearing loss is related to the retention of cognitive abilities in such elderly people. However, there were no statistical significant correlations between the auditory and cognitive tasks.

  7. Trends in worker hearing loss by industry sector, 1981-2010.

    Science.gov (United States)

    Masterson, Elizabeth A; Deddens, James A; Themann, Christa L; Bertke, Stephen; Calvert, Geoffrey M

    2015-04-01

    The purpose of this study was to estimate the incidence and prevalence of hearing loss for noise-exposed U.S. workers by industry sector and 5-year time period, covering 30 years. Audiograms for 1.8 million workers from 1981-2010 were examined. Incidence and prevalence were estimated by industry sector and time period. The adjusted risk of incident hearing loss within each time period and industry sector as compared with a reference time period was also estimated. The adjusted risk for incident hearing loss decreased over time when all industry sectors were combined. However, the risk remained high for workers in Healthcare and Social Assistance, and the prevalence was consistently high for Mining and Construction workers. While progress has been made in reducing the risk of incident hearing loss within most industry sectors, additional efforts are needed within Mining, Construction and Healthcare and Social Assistance. © 2015 Wiley Periodicals, Inc.

  8. Children With Mild Bilateral and Unilateral Hearing Loss: Parents’ Reflections on Experiences and Outcomes

    Science.gov (United States)

    Fitzpatrick, Elizabeth; Grandpierre, Viviane; Durieux-Smith, Andrée; Gaboury, Isabelle; Coyle, Doug; Na, Eunjung; Sallam, Nusaiba

    2016-01-01

    Abstract Children with mild bilateral and unilateral hearing loss are now commonly identified early through newborn hearing screening initiatives. There remains considerable uncertainty about how to support parents and about which services to provide for children with mild bilateral and unilateral hearing loss. The goal of this study was to learn about parents’ experiences and understand, from their perspectives, the impact of hearing loss in the mild range on the child’s functioning. Parents of 20 children in Ontario, Canada, participated in the study. The median age of identification of hearing loss was 4.6 months (interquartile range: 3.6, 10.8). Parents appreciated learning early about hearing loss, but their experiences with the early process were mixed. Parents felt that professionals minimized the importance of milder hearing loss. There was substantial uncertainty about the need for hearing aids and the findings suggest that parents need specific guidance. Parents expressed concerns about the potential impact of hearing loss on their child’s development, particularly at later ages. PMID:26433195

  9. [Current aspects of harmonization of classification of occupational hearing loss].

    Science.gov (United States)

    Pankova, V B; Sinëva, E L; Tavartkiladze, G A; Fedina, I N; Preobrazhenskaia, E A; Mukhamedova, G R

    2013-01-01

    The objective of the present work was to develop unified criteria for the evaluation of the severity of noise-induced hearing loss. Current approaches to taking expert decisions based on the results of medical examination of the patients with impaired hearing are substantially different due to the considerable difference between the criteria for the estimation of hearing envisaged by the international classification, occupational pathology classification, and the national system of medico-social expertise. We undertook an attempt to correct and harmonize the existing criteria for the estimation of severity of occupational hearing loss based on the integration of all the three classification in order to "reduce them to a common denominator" and thereby to ensure the basis for the unified diagnostic and expert decisions for the cases of hearing impairment of any etiology. The project proposed in this paper makes it possible to use unified criteria for the assessment of the degree of hearing loss caused by occupational noises for diagnostic purposes and expertise compatible with the internationally accepted approaches.

  10. Static and dynamic balance of children and adolescents with sensorineural hearing loss.

    Science.gov (United States)

    Melo, Renato de Souza; Marinho, Sônia Elvira Dos Santos; Freire, Maryelly Evelly Araújo; Souza, Robson Arruda; Damasceno, Hélio Anderson Melo; Raposo, Maria Cristina Falcão

    2017-01-01

    To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Hearing loss students showed more changes in static and dynamic balance as compared to normal hearing, in all tests used (pRomberg, Romberg-Barré, Fournier and Unterberger test p values were, respectively, p=0.004, pRomberg, Romberg-Barré and Fournier tests were, respectively, p=0.007, pRomberg, Romberg-Barré, Fournier and Unterberger tests were p=0.002, pRomberg-Barré, Fournier and Unterberger tests were, respectively, p=0.037, p<0.001 and p=0.037. Hearing-loss students showed more changes in static and dynamic balance comparing to normal hearing of same sex and age groups.

  11. Genetics Home Reference: age-related hearing loss

    Science.gov (United States)

    ... quality of life. Because affected individuals have trouble understanding speech, the condition affects their ability to communicate. It can contribute to social isolation, depression, and loss of self-esteem. Age-related hearing loss also causes safety issues if individuals become ...

  12. Clinical Study on 136 Children with Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Li, Feng-Jiao; Wang, Da-Yong; Wang, Hong-Yang; Wang, Li; Yang, Feng-Bo; Lan, Lan; Guan, Jing; Yin, Zi-Fang; Rosenhall, Ulf; Yu, Lan; Hellstrom, Sten; Xue, Xi-Jun; Duan, Mao-Li; Wang, Qiu-Ju

    2016-04-20

    The prevalence of sudden sensorineural hearing loss in children (CSSNHL) is consistently increasing. However, the pathology and prognosis of CSSNHL are still poorly understood. This retrospective study evaluated clinical characteristics and possible associated factors of CSSNHL. One hundred and thirty-six CSSNHL patients treated in Department of Otolaryngology-Head and Neck Surgery and Institute of Otolaryngology at Chinese PLA General Hospital between July 2008 and August 2015 were included in this study. These patients were analyzed for clinical characteristics, audiological characteristics, laboratory examinations, and prognostic factors. Among the 136 patients (151 ears), 121 patients (121 ears, 80.1%) were diagnosed with unilaterally CSSNHL, and 15 patients (30 ears, 19.9%) with bilateral CSSNHL. The complete recovery rate of CSSNHL was 9.3%, and the overall recovery rate was 37.7%. We found that initial degree of hearing loss, onset of treatment, tinnitus, the ascending type audiogram, gender, side of hearing loss, the recorded auditory brainstem response (ABR), and distortion product otoacoustic emissions (DPOAEs) had prognostic significance. Age, ear fullness, and vertigo had no significant correlation with recovery. Furthermore, the relevant blood tests showed 30.8% of the children had abnormal white blood cell (WBC) counts, 22.1% had elevated homocysteine levels, 65.8% had high alkaline phosphatase (ALP), 33.8% had high IgE antibody levels, and 86.1% had positive cytomegalovirus (CMV) IgG antibodies. CSSNHL commonly occurs unilaterally and results in severe hearing loss. Initial severe hearing loss and bilateral hearing loss are negative prognostic factors for hearing recovery, while positive prognostic factors include tinnitus, gender, the ascending type audiogram, early treatment, identifiable ABR waves, and DPOAEs. Age, vertigo, and ear fullness are not correlated with the recovery. Some serologic indicators, including the level of WBC, platelet

  13. Loss-of-Function CNKSR2 Mutation Is a Likely Cause of Non-Syndromic X-Linked Intellectual Disability.

    Science.gov (United States)

    Houge, G; Rasmussen, I H; Hovland, R

    2012-01-01

    In a non-dysmorphic 5-year-old boy with developmental delay, well-controlled epilepsy, and microcephaly, a 234-kb deletion of Xp22.12 was detected by copy number analysis. The maternally inherited deletion removed the initial 15 of the 21 exons of the connector enhancer of KSR-2 gene called CNKSR2 or CNK2. Our finding suggests that loss of CNKSR2 is a novel cause of non-syndromic X-linked mental retardation, an assumption supported by high gene expression in the brain, localization to the post-synaptic density, and a role in RAS/MAPK-dependent signal transduction.

  14. Conductive and Mixed Hearing Losses: A Comparison between Summer and Autumn

    OpenAIRE

    Nickbakht, Mansoureh; Borzoo, Samira

    2014-01-01

    Background and Objectives Conductive hearing loss is common among children and adults. This study aims at comparing the results of conductive hearing loss in summer and autumn. Subjects and Methods Puretone audiometry and tympanometry tests were done for all patients who referred to the Iranian-based audiology center of Imam Khomeini Hospital in Ahvaz. Data on the patients with conductive or mixed hearing loss were analyzed. The impacts of season, age, and etiology of the disease were analyze...

  15. Pathophysiology of Age-Related Hearing Loss (Peripheral and Central)

    OpenAIRE

    Lee, Kyu-Yup

    2013-01-01

    Age-related hearing loss (presbycusis) refers to bilaterally symmetrical hearing loss resulting from aging process. Presbycusis is a complex phenomenon characterized by audiometric threshold shift, deterioration in speech-understanding and speech-perception difficulties in noisy environments. Factors contributing to presbycusis include mitochondria DNA mutation, genetic disorders including Ahl, hypertension, diabetes, metabolic disease and other systemic diseases in the intrinsic aspects. Ext...

  16. Tracking occupational hearing loss across global industries: a comparative analysis of metrics.

    Science.gov (United States)

    Rabinowitz, Peter M; Galusha, Deron; McTague, Michael F; Slade, Martin D; Wesdock, James C; Dixon-Ernst, Christine

    2012-01-01

    Occupational hearing loss is one of the most prevalent occupational conditions; yet, there is no acknowledged international metric to allow comparisons of risk between different industries and regions. In order to make recommendations for an international standard of occupational hearing loss, members of an international industry group (the International Aluminium Association) submitted details of different hearing loss metrics currently in use by members. We compared the performance of these metrics using an audiometric data set for over 6000 individuals working in 10 locations of one member company. We calculated rates for each metric at each location from 2002 to 2006. For comparison, we calculated the difference of observed-expected (for age) binaural high-frequency hearing loss (in dB/year) for each location over the same time period. We performed linear regression to determine the correlation between each metric and the observed-expected rate of hearing loss. The different metrics produced discrepant results, with annual rates ranging from 0.0% for a less-sensitive metric to more than 10% for a highly sensitive metric. At least two metrics, a 10dB age-corrected threshold shift from baseline and a 15dB nonage-corrected shift metric, correlated well with the difference of observed-expected high-frequency hearing loss. This study suggests that it is feasible to develop an international standard for tracking occupational hearing loss in industrial working populations.

  17. Interventions to prevent occupational noise-induced hearing loss: A Cochrane systematic review

    Science.gov (United States)

    Verbeek, Jos H.; Kateman, Erik; Morata, Thais C.; Dreschler, Wouter A.; Mischke, Christina

    2015-01-01

    Objective To assess the effectiveness of interventions for preventing occupational noise exposure or hearing loss compared to no intervention or alternative interventions. Design We searched biomedical databases up to 25 January 2012 for randomized controlled trials (RCT), controlled before-after studies and interrupted time-series of hearing loss prevention among workers exposed to noise. Study sample We included 19 studies with 82 794 participants evaluating effects of hearing loss prevention programs (HLPP). The overall quality of studies was low to very low, as rated using the GRADE approach. Results One study of stricter legislation showed a favorable effect on noise levels. Three studies, of which two RCTs, did not find an effect of a HLPP. Four studies showed that better use of hearing protection devices in HLPPs decreased the risk of hearing loss. In four other studies, workers in a HLPP still had a 0.5 dB greater hearing loss at 4 kHz (95% CI – 0.5 to 1.7) than non-exposed workers. In two similar studies there was a substantial risk of hearing loss in spite of a HLPP. Conclusions Stricter enforcement of legislation and better implementation of HLPPs can reduce noise levels in workplaces. Better evaluations of technical interventions and long-term effects are needed. PMID:24564697

  18. Interventions to prevent occupational noise-induced hearing loss: a Cochrane systematic review.

    Science.gov (United States)

    Verbeek, Jos H; Kateman, Erik; Morata, Thais C; Dreschler, Wouter A; Mischke, Christina

    2014-03-01

    To assess the effectiveness of interventions for preventing occupational noise exposure or hearing loss compared to no intervention or alternative interventions. We searched biomedical databases up to 25 January 2012 for randomized controlled trials (RCT), controlled before-after studies and interrupted time-series of hearing loss prevention among workers exposed to noise. We included 19 studies with 82 794 participants evaluating effects of hearing loss prevention programs (HLPP). The overall quality of studies was low to very low, as rated using the GRADE approach. One study of stricter legislation showed a favorable effect on noise levels. Three studies, of which two RCTs, did not find an effect of a HLPP. Four studies showed that better use of hearing protection devices in HLPPs decreased the risk of hearing loss. In four other studies, workers in a HLPP still had a 0.5 dB greater hearing loss at 4 kHz (95% CI - 0.5 to 1.7) than non-exposed workers. In two similar studies there was a substantial risk of hearing loss in spite of a HLPP. Stricter enforcement of legislation and better implementation of HLPPs can reduce noise levels in workplaces. Better evaluations of technical interventions and long-term effects are needed.

  19. Epidemiological association of olfactory dysfunction with hearing loss and dysphonia in the Korean population

    Science.gov (United States)

    Park, Jae Hong; Byeon, Hyung Kwon; Park, Ki Nam; Kim, Jae Wook; Lee, Seung Won; Han, Kyung-do; Chang, Jae Won; Kim, Won Shik; Koh, Yoon Woo; Ban, Myung Jin

    2017-01-01

    Abstract The aim of the study is to investigate the association between olfactory dysfunction (OD), hearing loss, and dysphonia. The cross-sectional data for 17,984 adults who completed the Korea National Health and Nutrition Examination Surveys (2010−12) were analyzed. OD, hearing loss, and dysphonia were assessed using self-reporting questionnaires. The association of OD with hearing loss and dysphonia was evaluated. Hearing loss and dysphonia were significantly more prevalent in patients with OD than in those without OD (hearing loss, 28.1% vs 11.3%; dysphonia, 11.1% vs 5.9%; both P dysphonia, and was greater in those with combined hearing loss and dysphonia than in both patients without these dysfunctions and in those with a single dysfunction (odds ratio 3.115, 95% confidence interval 1.973–4.917). OD was significantly associated with hearing loss and dysphonia. PMID:29382018

  20. Binaural Interference and the Effects of Age and Hearing Loss.

    Science.gov (United States)

    Mussoi, Bruna S S; Bentler, Ruth A

    2017-01-01

    The existence of binaural interference, defined here as poorer speech recognition with both ears than with the better ear alone, is well documented. Studies have suggested that its prevalence may be higher in the elderly population. However, no study to date has explored binaural interference in groups of younger and older adults in conditions that favor binaural processing (i.e., in spatially separated noise). Also, the effects of hearing loss have not been studied. To examine binaural interference through speech perception tests, in groups of younger adults with normal hearing, older adults with normal hearing for their age, and older adults with hearing loss. A cross-sectional study. Thirty-three participants with symmetric thresholds were recruited from the University of Iowa community. Participants were grouped as follows: younger with normal hearing (18-28 yr, n = 12), older with normal hearing for their age (73-87 yr, n = 9), and older with hearing loss (78-94 yr, n = 12). Prior noise exposure was ruled out. The Connected Speech Test (CST) and Hearing in Noise Test (HINT) were administered to all participants bilaterally, and to each ear separately. Test materials were presented in the sound field with speech at 0° azimuth and the noise at 180°. The Dichotic Digits Test (DDT) was administered to all participants through earphones. Hearing aids were not used during testing. Group results were compared with repeated measures and one-way analysis of variances, as appropriate. Within-subject analyses using pre-established critical differences for each test were also performed. The HINT revealed no effect of condition (individual ear versus bilateral presentation) using group analysis, although within-subject analysis showed that 27% of the participants had binaural interference (18% had binaural advantage). On the CST, there was significant binaural advantage across all groups with group data analysis, as well as for 12% of the participants at each of the two

  1. Triple Difficulties in Japanese Women with Hearing Loss: Marriage, Smoking, and Mental Health Issues

    Science.gov (United States)

    Kobayashi, Yoko; Tamiya, Nanako; Moriyama, Yoko; Nishi, Akihiro

    2015-01-01

    Objective To examine the consequences of early-onset hearing loss on several social and health measures and any related gender differences in Japanese populations. Methods Data from a 2007 nationally representative cross-sectional household survey of 136,849 men and women aged 20 to 39 years were obtained (prevalence of self-reported hearing loss: 0.74%). We focused particularly on four social and health measures: employment status (employed/unemployed), marital status (married/unmarried), smoking behavior (yes/no), and psychological distress (K6 instrument: ≥ 5 or not). We examined the association of hearing loss for each measure using generalized estimating equations to account for correlated individuals within households. Findings There was no significant association with employment status (p = 0.447). Men with hearing loss were more likely to be married, whereas women with hearing loss were less likely to be married (p hearing loss was not associated with a current smoking status in men, women with hearing loss were more likely to be current smokers (p hearing loss was associated with psychological distress in men and women (both p hearing loss is related to social and health issues in daily life, including a lower likelihood of marriage, more frequent smoking, and poorer mental health, especially in women. These issues may reflect a gap between the actual needs of women with hearing loss and the formal support received as a result of existing public health policies in Japan. PMID:25651532

  2. The standpoint of persons with hearing loss on work disparities and workplace accommodations.

    Science.gov (United States)

    Shaw, Lynn; Tetlaff, Britta; Jennings, Mary Beth; Southall, Kenneth E

    2013-01-01

    The perspectives of persons who live and work with hearing loss were sought to examine workplace accommodation challenges and strategies. A convenience sample of seven older adults with hearing loss participated in in-depth interviews. A systematic grounded theory approach informed the study design and analysis. Categories of facilitators and challenges in the data were identified through axial coding and clustering. Core categories of social processes emerged through constant comparison and theoretical sampling of the data to reveal the actions and interactions used to negotiate or implement adaptations or workplace accommodations. Persons with hearing loss use a realm of strategies to live and work with a hearing loss. Social processes used to navigate the challenges to working with hearing loss and to manage optimal work performance included: self-accommodation, self-advocacy, self-management of hearing loss, and lobbying. Success in overcoming work disparities for persons with hearing loss requires individuals to take control of identifying their needs within the workplace and at home, and to negotiate for specific accommodations. These strategies and processes draw attention to the need for a repository on contextualized workplace accommodation strategies for improving communication and hearing in the workplace. Further to this a best practice guide for use by workers, employers, and work rehabilitation and health care workers is indicated.

  3. Interventions to prevent occupational noise-induced hearing loss

    NARCIS (Netherlands)

    Tikka, Christina; Verbeek, Jos H.; Kateman, Erik; Morata, Thais C.; Dreschler, Wouter A.; Ferrite, Silvia

    2017-01-01

    This is the second update of a Cochrane Review originally published in 2009. Millions of workers worldwide are exposed to noise levels that increase their risk of hearing disorders. There is uncertainty about the effectiveness of hearing loss prevention interventions. To assess the effectiveness of

  4. Investigation And Comparison of Fifth Grade Elementary Student’s Reading Skills with Severe Hearing Loss and Hearing in Tehran

    Directory of Open Access Journals (Sweden)

    Mohammad Razaei

    2013-04-01

    Full Text Available Objective: Since the written language is based on spoken language, hearing impairments may cause delays and defects in reading skills. This study is aimed to investigate reading problems in children with hearing loss and comparison of reading skills of fifth-grade elementary students’ reading skills suffering severe hearing loss. Materials & Methods: In this cross-sectional comparative study, 16 children with hearing loss were selected based on inclusion criteria from the whole fifth-grade elementary students with severe hearing loss in the Baghcheban schools and compared with 16 normal children matched upon the grade with sample group. To gather the data, Reading Test in elementary students was used as well as SPSS for data analysis. Results: Results showed children with hearing loss performed similarly as the control group on some skills, including naming speed skills (P=0.385, auditory-verbal sounds (reverse memory (P=0.345, visual-verbal pictures memory (P=1, phonological deletion (P=0.817 and nonword reading accuracy (P=0.633, however, they had poorer functions in the other domains. Conclusion: According to the result, it is concluded that auditory processing plays the key role in all prerequisite reading skills and children with hearing loss performed poorly on tasks based on auditory and language processing, whereas, the same perform on visual-processing-base tasks to normal children.

  5. The protective effect of melanocortins on cisplatin-induced hearing loss

    NARCIS (Netherlands)

    Wolters, Francisca Louisa Carolina

    2003-01-01

    Cisplatin is widely used for the treatment of a variety of tumors. Unfortunately, the therapeutic effect of cisplatin is limited because patients can develop a high frequency hearing loss in both ears. Recovery of this hearing loss is observed sporadically. Animal studies have shown that chronic

  6. Informational Masking and Spatial Hearing in Listeners with and without Unilateral Hearing Loss

    Science.gov (United States)

    Rothpletz, Ann M.; Wightman, Frederic L.; Kistler, Doris J.

    2012-01-01

    Purpose: This study assessed selective listening for speech in individuals with and without unilateral hearing loss (UHL) and the potential relationship between spatial release from informational masking and localization ability in listeners with UHL. Method: Twelve adults with UHL and 12 normal-hearing controls completed a series of monaural and…

  7. Factors that influence disclosure of hearing loss in the workplace.

    Science.gov (United States)

    Southall, Kenneth; Jennings, Mary Beth; Gagné, Jean-Pierre

    2011-10-01

    The objective of the study was to identify factors that lead individuals to conceal or disclose their hearing loss in the workplace. A qualitative research paradigm called qualitative description was selected to address this issue. Twelve people who had an adult onset hearing loss, and were gainfully employed, participated in audio-recorded semi-structured interviews designed to probe issues related to disclosure of hearing loss. A photo elicitation interview technique was employed during the interviews. Content analyses were used to extract pertinent information from verbatim transcripts. Five recurring themes emerged as important considerations in relation to this topic: (1) perceived importance of the situation; (2) perceived sense of control; (3) community affiliation; (4) burden of communication; and (5) coexisting issues related to hearing loss. The findings are discussed in relation to other concealable stigmatizing traits, stigma-theory, and social-cognitive theory. The clinical implications of these findings are discussed, with particular emphasis placed on worker self-efficacy.

  8. The Need for Improved Detection and Management of Adult-Onset Hearing Loss in Australia

    Directory of Open Access Journals (Sweden)

    Catherine M. McMahon

    2013-01-01

    Full Text Available Adult-onset hearing loss is insidious and typically diagnosed and managed several years after onset. Often, this is after the loss having led to multiple negative consequences including effects on employment, depressive symptoms, and increased risk of mortality. In contrast, the use of hearing aids is associated with reduced depression, longer life expectancy, and retention in the workplace. Despite this, several studies indicate high levels of unmet need for hearing health services in older adults and poor use of prescribed hearing aids, often leading to their abandonment. In Australia, the largest component of financial cost of hearing loss (excluding the loss of well-being is due to lost workplace productivity. Nonetheless, the Australian public health system does not have an effective and sustainable hearing screening strategy to tackle the problem of poor detection of adult-onset hearing loss. Given the increasing prevalence and disease burden of hearing impairment in adults, two key areas are not adequately met in the Australian healthcare system: (1 early identification of persons with chronic hearing impairment; (2 appropriate and targeted referral of these patients to hearing health service providers. This paper reviews the current literature, including population-based data from the Blue Mountains Hearing Study, and suggests different models for early detection of adult-onset hearing loss.

  9. Hearing loss in children in Østfold county 2000-09.

    Science.gov (United States)

    Nelson, Siri; Andersen, Ronny; Anderssen, Sven-Harald

    2015-01-27

    The Directorate of Health recommends hearing screening of all neonates with the aid of otoacoustic emissions (OAE). The goal is to achieve initiation of treatment before the child is six months old. Congenital hearing loss occurs in 1-3 per 1,000 newborns, and the purpose of this study was to identify the prevalence in a large Norwegian dataset. During the period 1 January 2000-31 December 2009, all children who were born in Østfold county, 29,485 neonates in total, were offered hearing screening with the aid of otoacoustic emissions. Those children in whom no emissions were detected were referred to the hearing centre. The dataset also includes children born during the same period who had normal emissions during their neonatal period, but later were diagnosed with hearing loss. The follow-up period of these children lasted until 31 December 2013. Altogether 568 children were referred to the hearing centre after screening. Of these, 73 children underwent brainstem response audiometry under anaesthesia, and 31 (0.11%) were diagnosed with hearing loss through absence of emissions at birth. Eleven (35%) of these received their diagnosis before the age of six months. The median age of diagnosis decreased from 13 (7-74) to 6 (3-23) months, while their number increased from nine to 22 from 2000-04 to 2005-09. Hearing loss after normal otoacoustic emissions at the neonatal stage was diagnosed in ten children (0.03%) and the median age of diagnosis among these was 36 (18-86) months. After the introduction of automated auditory brainstem response audiometry (AABR) during sleep, the use of auditory brainstem response audiometry (ABR) under narcosis fell by half. The prevalence of hearing loss is equal to other Norwegian and international figures. The age of diagnosis decreased during the period. Otoacoustic emissions appear to be an effective screening method in paediatric wards. The use of automated auditory brainstem response audiometry reduces the need for testing under

  10. Annual rate of hearing loss falls after fractionated stereotactic irradiation for vestibular schwannoma

    International Nuclear Information System (INIS)

    Sakamoto, Touru; Shirato, Hiroki; Takeichi, Norihito; Aoyama, Hidefumi; Fukuda, Satoshi; Miyasaka, Kazuo

    2001-01-01

    Purpose: The rate of hearing loss in a population before and after irradiation was investigated to determine the effect of irradiation on hearing impairment. Methods and materials: In 72 patients with vestibular schwannoma who received fractionated stereotactic irradiation from 1992 to 1999, 21 had had their hearing levels examined 3 months or more before the treatment. The mean time between the initial examination and treatment was 18.6 months (range: 3-89 months), and the mean time between treatment and the last follow-up was 24.2 months (12-69 months). Thirty-six to 50 Gy in 20-25 fractions over 5 to 6 weeks was given using an X-ray beam from a linear accelerator. Pure tone average (PTA) was measured using the mean hearing level at five frequencies, and the annual rate of hearing loss was defined as [(hearing loss in PTA(dB))/(follow-up period (months)x12)]. Results: The actual cumulative curve of decrease in tumor size of 2 mm or more was 38.3% at 2 years and 80.0% at 3 years. The mean of hearing loss in PTA was 11.6±10.3 dB (-1 to 35 dB) from the initial examination to the start of irradiation and 11.9±14.4 dB (-14 to 37 dB) from the start of irradiation to the last follow-up. The mean annual rates of hearing loss before irradiation and in the 1st, 2nd, 3rd and 4th years after irradiation were, respectively, 18.6, 11.2, 6.2, 5.1, and 5.0 dB/year. The annual rates of hearing loss in the 2nd year (P=0.025) and 3rd year (P=0.018) were significantly slower than the rate before irradiation. Conclusions: The mean annual rate of hearing loss was higher before irradiation than after irradiation, and hearing loss slowed rather than accelerated after irradiation. Although hearing loss after the treatment was usually permanent, fractionated stereotactic irradiation was suggested to be effective to lower the rate of hearing loss

  11. Factors influencing pursuit of hearing evaluation: Enhancing the health belief model with perceived burden from hearing loss on communication partners.

    Science.gov (United States)

    Schulz, Kristine A; Modeste, Naomi; Lee, Jerry; Roberts, Rhonda; Saunders, Gabrielle H; Witsell, David L

    2016-07-01

    There is limited application of health behavior-based theoretical models in hearing healthcare, yet other fields utilizing these models have shown their value in affecting behavior change. The health belief model (HBM) has demonstrated appropriateness for hearing research. This study assessed factors that influence an individual with suspected hearing loss to pursue clinical evaluation, with a focus on perceived burden of hearing loss on communication partners, using the HBM as a framework. Cross-sectional design collecting demographics along with three validated hearing-loss related questionnaires. Patients from Duke University Medical Center Otolaryngology Clinic aged 55-75 years who indicated a communication partner had expressed concern about their hearing. A final sample of 413 completed questionnaire sets was achieved. The HBM model construct 'cues to action' was a significant (p loss on communication partners was a significant (p model fit when added to the HBM: 72.0% correct prediction when burden is added versus 66.6% when not (p models in hearing healthcare is warranted.

  12. Effects of sensorineural hearing loss on visually guided attention in a multitalker environment.

    Science.gov (United States)

    Best, Virginia; Marrone, Nicole; Mason, Christine R; Kidd, Gerald; Shinn-Cunningham, Barbara G

    2009-03-01

    This study asked whether or not listeners with sensorineural hearing loss have an impaired ability to use top-down attention to enhance speech intelligibility in the presence of interfering talkers. Listeners were presented with a target string of spoken digits embedded in a mixture of five spatially separated speech streams. The benefit of providing simple visual cues indicating when and/or where the target would occur was measured in listeners with hearing loss, listeners with normal hearing, and a control group of listeners with normal hearing who were tested at a lower target-to-masker ratio to equate their baseline (no cue) performance with the hearing-loss group. All groups received robust benefits from the visual cues. The magnitude of the spatial-cue benefit, however, was significantly smaller in listeners with hearing loss. Results suggest that reduced utility of selective attention for resolving competition between simultaneous sounds contributes to the communication difficulties experienced by listeners with hearing loss in everyday listening situations.

  13. Congenital non-syndromal autosomal recessive deafness in Bengkala, an isolated Balinese village.

    Science.gov (United States)

    Winata, S; Arhya, I N; Moeljopawiro, S; Hinnant, J T; Liang, Y; Friedman, T B; Asher, J H

    1995-01-01

    Bengkala is an Indonesian village located on the north shore of Bali that has existed for over 700 years. Currently, 2.2% of the 2185 people in this village have profound congenital deafness. In response to the high incidence of deafness, the people of Bengkala have developed a village specific sign language which is used by many of the hearing and deaf people. Deafness in Bengkala is congenital, sensorineural, non-syndromal, and caused by a fully penetrant autosomal recessive mutation at the DFNB3 locus. The frequency of the DFNB3 mutation is estimated to be 9.4% among hearing people who have a 17.2% chance of being heterozygous for DFNB3. PMID:7616538

  14. Radiotherapy-induced hearing loss in patients with laryngeal and hypopharyngeal carcinomas.

    Science.gov (United States)

    Gudelj, Goran; Trotić, Robert; Herceg, Tonko; Parazajder, Domagoj; Vagić, Davor; Geber, Goran

    2014-09-01

    The purpose of this study was to investigate a hypothesized correlation of development of a sensorineural hearing loss and radiotherapy in patients with laryngeal and hypopharyngeal carcinoma. This prospective study included a total of 50 patients, which after strict exclusion critera (audiologic problems before RT primary tumors of the auditory system, spread of the primary tumor to any part of the auditory system) resulted in 23 analyzed patients, ranging between 50 and 76 years of age, with a mean age of 60. Audiometry measuring frequency-specific thresholds was performed in three time points: one month before radiotherapy, one and six months after radiotherapy. A significant statistical difference in hearing tresholds after radiotherapy was found in 6 out of 23 patients. An obvious tendency towards hearing loss without statistical significance at 250 and 4000 Hz was found for a whole tested population (p < or = 0.3 with Bonferroni correction). Observed tendency towards hearing loss after radiotherapy of laryngeal carcinoma was related to side of the tumor and less severe when chemotherapy was not added as adjuvant therapy. These results should help to decrease a rate of hearing loss by careful planing of ear protection, by using observed frequencies as relevant markers of hearing loss and by reconsidering adjuvant chemoterapy during radiotherapy of laryngeal carcinoma.

  15. High-frequency audiometry: A means for early diagnosis of noise-induced hearing loss

    Directory of Open Access Journals (Sweden)

    Amir H Mehrparvar

    2011-01-01

    Full Text Available Noise-induced hearing loss (NIHL, an irreversible disorder, is a common problem in industrial settings. Early diagnosis of NIHL can help prevent the progression of hearing loss, especially in speech frequencies. For early diagnosis of NIHL, audiometry is performed routinely in conventional frequencies. We designed this study to compare the effect of noise on high-frequency audiometry (HFA and conventional audiometry. In a historical cohort study, we compared hearing threshold and prevalence of hearing loss in conventional and high frequencies of audiometry among textile workers divided into two groups: With and without exposure to noise more than 85 dB. The highest hearing threshold was observed at 4000 Hz, 6000 Hz and 16000 Hz in conventional right ear audiometry, conventional left ear audiometry and HFA in each ear, respectively. The hearing threshold was significantly higher at 16000 Hz compared to 4000. Hearing loss was more common in HFA than conventional audiometry. HFA is more sensitive to detect NIHL than conventional audiometry. It can be useful for early diagnosis of hearing sensitivity to noise, and thus preventing hearing loss in lower frequencies especially speech frequencies.

  16. Commentary: Listening Can Be Exhausting--Fatigue in Children and Adults with Hearing Loss

    Science.gov (United States)

    Bess, Fred H.; Hornsby, Benjamin W.Y.

    2014-01-01

    Anecdotal reports of fatigue after sustained speech-processing demands are common among adults with hearing loss; however, systematic research examining hearing loss-related fatigue is limited, particularly with regard to fatigue among children with hearing loss (CHL). Many audiologists, educators, and parents have long suspected that CHL…

  17. Late-Onset Hearing Loss: Strategies for Effective Counseling.

    Science.gov (United States)

    Smith, S. Mae; Kampfe, Charlene M.

    1999-01-01

    Late-onset hearing loss is one of the major chronic conditions experienced by older individuals. The term "presbycusis" is typically used when describing this condition. Presbycusis refers to many degenerative changes that affect older people's hearing. This article provides practical suggestions for working with persons with this…

  18. Comparison of Different Levels of Reading Comprehension between Hearing-Impaired Loss and Normal-Hearing Students

    Directory of Open Access Journals (Sweden)

    Azam Sharifi

    2011-12-01

    Full Text Available Background and Aim: Reading skill is one of the most important necessities of students' learning in everyday life. This skill is referred to the ability of comprehension, comment and conclusion from texts and receiving the meaning of the massage which is composed. Educational development in any student has a direct relation with the ability of the comprehension. This study is designed to investigate the effects of hearing loss on reading comprehension in hearing-impaired students compared to normal-hearing ones.Methods: Seventeen hearing-impaired students in 4th year of primary exceptional schools in Karaj, Robatkarim and Shahriyar, Iran, were enrolled in this cross-sectional study. Seventeen normal-hearing students were randomly selected from ordinary schools next to exceptional ones as control group. They were compared for different levels of reading comprehension using the international standard booklet (PIRLS 2001. Results: There was a significant difference in performance between hearing-impaired and normal- hearing students in different levels of reading comprehension (p<0.05.Conclusion: Hearing loss has negative effects on different levels of reading comprehension, so in exceptional centers, reconsideration in educational planning in order to direct education from memorizing to comprehension and deeper layers of learning seems necessary.

  19. Bone-anchored hearing aids in conductive and mixed hearing losses: why do patients reject them?

    Science.gov (United States)

    Siau, Richard T K; Dhillon, Baljeet; Siau, Derrick; Green, Kevin M J

    2016-10-01

    This study aimed to report the bone-anchored hearing aid uptake rate and the reasons for their rejection by patients with conductive and mixed hearing losses. A retrospective review was performed of 113 consecutive patients with unilateral or bilateral conductive or mixed hearing loss referred to the Greater Manchester bone-anchored hearing aid (BAHA) programme between September 2008 and August 2011. 98 (86.7 %) patients were deemed audiologically suitable for BAHA implantation. Of these, 38 (38.8 %) had BAHA implanted; 60 (61.2 %) patients declined. Of those who declined, 27 (45 %) cited anxiety over surgery, 18 (30 %) cited cosmetic reasons, 16 (26.7 %) perceived limited benefit from the device and six (10 %) preferred conventional hearing aids. Our study highlights a 38.8 % BAHA uptake rate in audiologically suitable patients. The main reasons cited for rejection of BAHA were anxiety over surgery and cosmetic concerns. It is important that clinicians address these early during consultation with prospective BAHA recipients and avoid rushing to implant these patients with a bone-anchored hearing aid.

  20. Examination of characteristics and management of children with hearing loss and autism spectrum disorders.

    Science.gov (United States)

    Fitzpatrick, Elizabeth M; Lambert, Linda; Whittingham, JoAnne; Leblanc, Emma

    2014-09-01

    Up to 40% of children with hearing loss present with other developmental disabilities. The purpose of this study was to document the prevalence of autism spectrum disorders (ASD) in children with permanent hearing loss, to describe the audiologic characteristics, and to examine clinical management. Prospective data related to clinical characteristics of children identified with hearing loss and ASD were examined. A retrospective chart review was also conducted to explore clinical management and uptake of amplification. The study included all children in one Canadian region identified with permanent hearing loss and followed from 2002-2010. Of a total of 785 children with permanent hearing loss, 2.2% (n = 17) also received a diagnosis of ASD. The 13 boys and 4 girls presented with a range of audiologic profiles from unilateral to profound bilateral hearing loss. Four of five children with unilateral hearing loss experienced progression to bilateral loss. Amplification was recommended for all but one child and 9 of 16 children continued to use their hearing devices. The higher prevalence rate of ASD in this clinical population is consistent with previous reports. Our findings suggest that some children with autism can derive benefits from the use of amplification.

  1. Treatment of Noise-Induced Hearing Loss

    National Research Council Canada - National Science Library

    d'Aldin, Gervais

    1999-01-01

    .... Guinea pigs are subjected to an acoustic trauma. The recovery of the noise-induced hearing loss is followed up to 14 days post exposure by electrocochleography and morphologic examination of the cochlea is performed...

  2. Gene structure and mutant alleles of PCDH15: nonsyndromic deafness DFNB23 and type 1 Usher syndrome.

    Science.gov (United States)

    Ahmed, Zubair M; Riazuddin, Saima; Aye, Sandar; Ali, Rana A; Venselaar, Hanka; Anwar, Saima; Belyantseva, Polina P; Qasim, Muhammad; Riazuddin, Sheikh; Friedman, Thomas B

    2008-10-01

    Mutations of PCDH15, encoding protocadherin 15, can cause either combined hearing and vision impairment (type 1 Usher syndrome; USH1F) or nonsyndromic deafness (DFNB23). Human PCDH15 is reported to be composed of 35 exons and encodes a variety of isoforms with 3-11 ectodomains (ECs), a transmembrane domain and a carboxy-terminal cytoplasmic domain (CD). Building on these observations, we describe an updated gene structure that has four additional exons of PCDH15 and isoforms that can be subdivided into four classes. Human PCDH15 encodes three alternative, evolutionarily conserved unique cytoplasmic domains (CD1, CD2 or CD3). Families ascertained on the basis of prelingual hearing loss were screened for linkage of this phenotype to markers for PCDH15 on chromosome 10q21.1. In seven of twelve families segregating USH1, we identified homozygous mutant alleles (one missense, one splice site, three nonsense and two deletion mutations) of which six are novel. One family was segregating nonsyndromic deafness DFNB23 due to a homozygous missense mutation. To date, in our cohort of 557 Pakistani families, we have found 11 different PCDH15 mutations that account for deafness in 13 families. Molecular modeling provided mechanistic insight into the phenotypic variation in severity of the PCDH15 missense mutations. We did not find pathogenic mutations in five of the twelve USH1 families linked to markers for USH1F, which suggest either the presence of mutations of yet additional undiscovered exons of PCDH15, mutations in the introns or regulatory elements of PCDH15, or an additional locus for type I USH at chromosome 10q21.1.

  3. Triple difficulties in Japanese women with hearing loss: marriage, smoking, and mental health issues.

    Directory of Open Access Journals (Sweden)

    Yoko Kobayashi

    Full Text Available To examine the consequences of early-onset hearing loss on several social and health measures and any related gender differences in Japanese populations.Data from a 2007 nationally representative cross-sectional household survey of 136,849 men and women aged 20 to 39 years were obtained (prevalence of self-reported hearing loss: 0.74%. We focused particularly on four social and health measures: employment status (employed/unemployed, marital status (married/unmarried, smoking behavior (yes/no, and psychological distress (K6 instrument: ≥ 5 or not. We examined the association of hearing loss for each measure using generalized estimating equations to account for correlated individuals within households.There was no significant association with employment status (p = 0.447. Men with hearing loss were more likely to be married, whereas women with hearing loss were less likely to be married (p < 0.001 for interaction. Although hearing loss was not associated with a current smoking status in men, women with hearing loss were more likely to be current smokers (p < 0.001 for interaction. Moreover, hearing loss was associated with psychological distress in men and women (both p < 0.001.These findings suggest that hearing loss is related to social and health issues in daily life, including a lower likelihood of marriage, more frequent smoking, and poorer mental health, especially in women. These issues may reflect a gap between the actual needs of women with hearing loss and the formal support received as a result of existing public health policies in Japan.

  4. Ethylbenzene-induced hearing loss, neurobehavioral function, and neurotransmitter alterations in petrochemical workers.

    Science.gov (United States)

    Zhang, Ming; Wang, Yanrang; Wang, Qian; Yang, Deyi; Zhang, Jingshu; Wang, Fengshan; Gu, Qing

    2013-09-01

    To estimate hearing loss, neurobehavioral function, and neurotransmitter alteration induced by ethylbenzene in petrochemical workers. From two petrochemical plants, 246 and 307 workers exposed to both ethylbenzene and noise were recruited-290 workers exposed to noise only from a power station plant and 327 office personnel as control group, respectively. Hearing and neurobehavioral functions were evaluated. Serum neurotransmitters were also determined. The prevalence of hearing loss was much higher in petrochemical groups than that in power station and control groups (P workers (P hearing loss, neurobehavioral function impairment, and imbalance of neurotransmitters.

  5. [Management of sudden neurosensory hearing loss in a Primary Care Centre].

    Science.gov (United States)

    Muñoz-Proto, F; Carnevale, C; Bejarano-Panadés, N; Ferrán-de la Cierva, L; Mas-Mercant, S; Sarría-Echegaray, P

    2014-04-01

    Sudden hearing loss is a rapid loss of neurosensory hearing that may occur within hours or days in an apparently healthy patient. Its origins are variable and multifactorial. Most patients do not recover hearing if not treated, and some even develop cophosis (deafness) in the affected ear. It is an otological emergency, as early therapeutic management offers a better hearing prognosis. As there is limited knowledge on this condition, it may be underdiagnosed in Primary Health Care Centers. It should be suspected in patients with abrupt hearing loss or tinnitus. Sophisticated instruments are not required for its diagnosis, just a detailed history, basic otoscopy, and proper interpretation of the hearing test. In this way, an accurate diagnosis is achieved in most cases, which is confirmed by audiometry. Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.

  6. Hearing Loss and Disability Exit: Measurement Issues and Coping Strategies

    DEFF Research Database (Denmark)

    T. Christensen, Vibeke; V. Rasmussen, Martin; Datta Gupta, Nabanita

    Using unique representative data containing self-reported functional and clinically measured hearing ability for the Danish population aged 50-64, we estimate the effect of hearing loss on receipt of disability benefits accounting for potential endogeneity of functional hearing. Our identification...

  7. Smoking, Smoking Cessation, and the Risk of Hearing Loss: Japan Epidemiology Collaboration on Occupational Health Study.

    Science.gov (United States)

    Hu, Huanhuan; Sasaki, Naoko; Ogasawara, Takayuki; Nagahama, Satsue; Akter, Shamima; Kuwahara, Keisuke; Kochi, Takeshi; Eguchi, Masafumi; Kashino, Ikuko; Murakami, Taizo; Shimizu, Makiko; Uehara, Akihiko; Yamamoto, Makoto; Nakagawa, Tohru; Honda, Toru; Yamamoto, Shuichiro; Hori, Ai; Nishiura, Chihiro; Okazaki, Hiroko; Imai, Teppei; Nishihara, Akiko; Miyamoto, Toshiaki; Tomita, Kentaro; Kabe, Isamu; Mizoue, Tetsuya; Kunugita, Naoki; Dohi, Seitaro

    2018-03-14

    We aimed to determine the prospective association of smoking status, smoking intensity, and smoking cessation with the risk of hearing loss in a large Japanese cohort. The cohort study included 50195 employees, who were aged 20-64 years and free of hearing loss at baseline. Participants were followed up for a maximum of 8 years. Pure-tone audiometric testing was performed annually to identify hearing loss at 1 and 4 kHz. Cox proportional hazards regression models were used to investigate the association between smoking and hearing loss. During follow-up, 3532 individuals developed high-frequency hearing loss, and 1575 developed low-frequency hearing loss. The hazard ratio (HR) associated with current smokers was 1.6 (95% confidence interval [CI] = 1.5 to 1.7) and 1.2 (95% CI = 1.1 to 1.4) for high- and low-frequency hearing loss, respectively, as compared with never smokers. The risk of high- and low-frequency hearing loss increased with the number of cigarettes smoked per day (both p for trend hearing loss, respectively. The analysis by quitting years showed a decline in risk of hearing loss after quitting smoking, even among those who quitted less than 5 years before baseline. Smoking is associated with increased risk of hearing loss, especially at the high frequency, in a dose-response manner. The excess risk of hearing loss associated with smoking disappears in a relatively short period after quitting. The prospective association between smoking and hearing loss has not been well studied. To the best of our knowledge, our study is the largest to date investigating the association between smoking and incident hearing loss. Our results indicate that smoking is associated with increased risk of hearing loss in a dose-response manner. Quitting smoking virtually eliminates the excess risk of hearing loss, even among quitters with short duration of cessation. These results suggest that smoking may be a causal factor for hearing loss, although further research would

  8. Non-suicidal self-injury among children with hearing loss and intellectual disability.

    Science.gov (United States)

    Akram, Bushra; Tariq, Amina; Rafi, Zeeshan

    2017-10-01

    To find the prevalence and to identify the predictors of non-suicidal self-injury among school-going children.. This cross-sectional study was conducted at the University of Gujrat, Gujrat Pakistan, from September 2015 to October 2016, and comprised children with intellectual disability and hearing loss. Participants were recruited from schools for special children located in Gujranwala, Jhelum and Gujrat. Multistage stratified sampling technique was used. Of the 325 children, 178(50.4%) had intellectual disability and 175(49.6%) had hearing loss. Findings indicated that the prevalence of self-injurious behaviour was higher in children with intellectual disability 48(27%) compared to their counterparts with hearing loss 3(2%). Neural network, when administered on whole data set, indicated type of disability 0.474(100%), education/training 0.99(20.9%) and access of counselling 0.114(24%) as important predictors of non-suicidal self-injury in both groups. On the other hand, the degree of disability (hearing loss 0.42[100%]; intellectual disability 0.32[100%]), education/ training (hearing loss 0.18[43%]; intellectual disability 0.27[84.5%]) and access of counselling (hearing loss 0.175[41.8%]; intellectual disability 0.256[78.7%]) were important predictors of non-suicidal self-injury among the participants, when neural network was run on the split files on the basis of disability. The prevalence of non-suicidal self-injury among children with intellectual disability was higher as compared to those with hearing loss.

  9. Tracking occupational hearing loss across global industries: A comparative analysis of metrics

    Directory of Open Access Journals (Sweden)

    Peter M Rabinowitz

    2012-01-01

    Full Text Available Occupational hearing loss is one of the most prevalent occupational conditions; yet, there is no acknowledged international metric to allow comparisons of risk between different industries and regions. In order to make recommendations for an international standard of occupational hearing loss, members of an international industry group (the International Aluminium Association submitted details of different hearing loss metrics currently in use by members. We compared the performance of these metrics using an audiometric data set for over 6000 individuals working in 10 locations of one member company. We calculated rates for each metric at each location from 2002 to 2006. For comparison, we calculated the difference of observed-expected (for age binaural high-frequency hearing loss (in dB/year for each location over the same time period. We performed linear regression to determine the correlation between each metric and the observed-expected rate of hearing loss. The different metrics produced discrepant results, with annual rates ranging from 0.0% for a less-sensitive metric to more than 10% for a highly sensitive metric. At least two metrics, a 10dB age-corrected threshold shift from baseline and a 15dB nonage-corrected shift metric, correlated well with the difference of observed-expected high-frequency hearing loss. This study suggests that it is feasible to develop an international standard for tracking occupational hearing loss in industrial working populations.

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

    Science.gov (United States)

    de Souza Melo, Renato

    2017-02-01

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

  11. Challenges in management of childhood sensorineural hearing loss in sub-Saharan Africa, Nigeria.

    Science.gov (United States)

    Lasisi, O A; Ayodele, J K; Ijaduola, G T A

    2006-04-01

    The evaluation of a hearing-impaired child attempts to determine the aetiology, the degree of hearing loss and intervention to aid speech and language. This remains a challenge to practising otolaryngologists, especially in the developing countries as 85-90% of causes of hearing losses were never discovered leading to delayed intervention and irreversible effects. In a review of children presenting at the otolaryngology outpatient in the University College Hospital, Ibadan, Nigeria, sensorineural hearing loss was found in 103, giving a hospital prevalence rate of 14%, we still perceived this figure to be unrepresentatively and low, probably due to poor access to medicare, poverty and other factors. Genetic factor accounted for 25%, followed by measles infections 13% and meningitis 8%. About 60% of them had educationally significant hearing loss at presentation. Access to hearing aid was poor as only 12.5% of the patients could afford it and the rest were managed by deaf training. We conclude by suggesting an audiologic programme which has the comprehensive function of neonatal and infant hearing screening, subsidized hearing aid services and hearing rehabilitation surgery.

  12. [Transcultural adaptation of an instrument to evaluate hearing handicap in workers with noise-induced hearing loss].

    Science.gov (United States)

    Holanda, Wanessa Tenório Gonçalves; de Lima, Maria Luiza Carvalho; Figueiroa, José Natal

    2011-01-01

    The noise-induced hearing loss (NIHL) is a chronic and irreversible disease resulting of the exposure to noise in high levels at work. Even in the beginning, this hearing loss can damage in many degrees the worker's quality of life. Before this study, there wasn't an instrument, in Portuguese, to evaluate the psychosocial disadvantages of workers with NIHL. The aim of this research was to make a transcultural adaptation of an especific instrument to evaluate the hearing handicap from the original language to Portuguese, and check the reliability and legitimacy. The selected instrument passed by a process of semantic equivalence that was conducted in five stages: translation, back translation, critical appraisal of the versions, pre-test and a final review by a multi-professional group to develop a consensual version of the instrument for current use in Brazil. The instrument called "Inabilities Scale and Hearing Handicap" had, in general, acceptable psychometric measures, considering the little size of the sample and the fact that workers' hearing loss weren't too significant. Therefore, the Portuguese version of this instrument needs to be further tested in a representative sample of Brazilian workers with NIHL to ratify its utility in order to evaluate hearing handicap in this population.

  13. A Novel Locus Harbouring a Functional CD164 Nonsense Mutation Identified in a Large Danish Family with Nonsyndromic Hearing Impairment

    DEFF Research Database (Denmark)

    Nyegaard, Mette; Rendtorff, Nanna D; Nielsen, Morten S

    2015-01-01

    Nonsyndromic hearing impairment (NSHI) is a highly heterogeneous condition with more than eighty known causative genes. However, in the clinical setting, a large number of NSHI families have unexplained etiology, suggesting that there are many more genes to be identified. In this study we used SNP......-based linkage analysis and follow up microsatellite markers to identify a novel locus (DFNA66) on chromosome 6q15-21 (LOD 5.1) in a large Danish family with dominantly inherited NSHI. By locus specific capture and next-generation sequencing, we identified a c.574C>T heterozygous nonsense mutation (p.R192......-genome and exome sequence data. The predicted effect of the mutation was a truncation of the last six C-terminal residues of the cytoplasmic tail of CD164, including a highly conserved canonical sorting motif (YXX phi). In whole blood from an affected individual, we found by RT-PCR both the wild...

  14. Perda auditiva genética Genetic hearing loss

    Directory of Open Access Journals (Sweden)

    Ricardo Godinho

    2003-01-01

    Full Text Available O progresso das pesquisas relacionadas à perda auditiva genética tem provocado um importante avanço do entendimento dos mecanismos moleculares que governam o desenvolvimento, a função, a resposta ao trauma e o envelhecimento do ouvido interno. Em países desenvolvidos, mais de 50% dos casos de surdez na infância é causada por alterações genéticas e as perdas auditivas relacionadas à idade têm sido associadas com mecanismos genéticos. OBJETIVO: O objetivo desta revisão é relatar as informações mais recentes relacionadas às perdas audtivas de origem genética. FORAMA DE ESTUDO: Revisão sistemática. MATERIAL E MÉTODO: A revisão da literatura inclui artigos indexados à MEDLINE (Biblioteca Nacional de Saúde, NIH-USA e publicados nos últimos 3 anos, além das informações disponíveis na Hereditary Hearing Loss Home Page. CONCLUSÃO: Os recentes avanços no entendimento das perdas auditivas de origem genética têm favorecido a nossa compreensão da função auditiva e tornado o diagnóstico mais apurado. Possivelmente, no futuro, este conhecimento também proporcionará o desenvolvimento de novas terapias para o tratamento das causas genéticas das perdas auditivas.The progress in the research of genetic hearing loss has advanced our understanding of the molecular mechanisms that govern inner ear development, function and response to injury and aging. In the developed world, over 50% of childhood deafness is attributable to genetic causes and even age-related hearing loss has been associated with genetic mechanisms. AIM: The objective of this review is to summarize recent knowledge in genetic hearing loss. STUDY DESIGN: Sistematic review. MATERIAL AND METHODS: The literature review included articles indexed at MEDLINE (The National Library of Medicine, The National Institute of Health - USA focusing on publications from the past 3 years plus the information available at the Hereditary Hearing Loss Home Page. CONCLUSION

  15. Congenital hearing loss. Is CT enough?

    African Journals Online (AJOL)

    Mahmoud Agha

    2014-01-24

    Jan 24, 2014 ... Congenital hearing loss is one of the developmental disorders that may be not clearly .... Alport syndrome, Klippel-Feil, Norrie disease and Waarden- .... Bismuth eye shield was routinely used for all patients (AttenuRad;.

  16. Discovery of CDH23 as a Significant Contributor to Progressive Postlingual Sensorineural Hearing Loss in Koreans.

    Directory of Open Access Journals (Sweden)

    Bong Jik Kim

    Full Text Available CDH23 mutations have mostly been associated with prelingual severe-to-profound sensorineural hearing loss (SNHL in either syndromic or nonsyndromic SNHL (DFNB12. Herein, we demonstrate the contribution of CDH23 mutations to postlingual nonsyndromic SNHL (NS-SNHL. We screened 32 Korean adult probands with postlingual NS-SNHL sporadically or in autosomal recessive fashion using targeted panel or whole exome sequencing. We identified four (12.5%, 4/32 potential postlingual DFNB12 families that segregated the recessive CDH23 variants, qualifying for our criteria along with rapidly progressive SNHL. Three of the four families carried one definite pathogenic CDH23 variant previously known as the prelingual DFNB12 variant in a trans configuration with rare CDH23 variants. To determine the contribution of rare CDH23 variants to the postlingual NS-SNHL, we checked the minor allele frequency (MAF of CDH23 variants detected from our postlingual NS-SNHL cohort and prelingual NS-SNHL cohort, among the 2040 normal control chromosomes. The allele frequency of these CDH23 variants in our postlingual cohort was 12.5%, which was significantly higher than that of the 2040 control chromosomes (5.53%, confirming the contribution of these rare CDH23 variants to postlingual NS-SNHL. Furthermore, MAF of rare CDH23 variants from the postlingual NS-SNHL group was significantly higher than that from the prelingual NS-SNHL group. This study demonstrates an important contribution of CDH23 mutations to poslingual NS-SNHL and shows that the phenotypic spectrum of DFNB12 can be broadened even into the presbycusis, depending on the pathogenic potential of variants. We also propose that pathogenic potential of CDH23 variants and the clinical fate of DFNB12 may be predicted by MAF.

  17. Acoustic Reflex Screening of Conductive Hearing Loss for Third Window Disorders.

    Science.gov (United States)

    Hong, Robert S; Metz, Christopher M; Bojrab, Dennis I; Babu, Seilesh C; Zappia, John; Sargent, Eric W; Chan, Eleanor Y; Naumann, Ilka C; LaRouere, Michael J

    2016-02-01

    This study examines the effectiveness of acoustic reflexes in screening for third window disorders (eg, superior semicircular canal dehiscence) prior to middle ear exploration for conductive hearing loss. Case series with chart review. Outpatient tertiary otology center. A review was performed of 212 ears with acoustic reflexes, performed as part of the evaluation of conductive hearing loss in patients without evidence of chronic otitis media. The etiology of hearing loss was determined from intraoperative findings and computed tomography imaging. The relationship between acoustic reflexes and conductive hearing loss etiology was assessed. Eighty-eight percent of ears (166 of 189) demonstrating absence of all acoustic reflexes had an ossicular etiology of conductive hearing loss. Fifty-two percent of ears (12 of 23) with at least 1 detectable acoustic reflex had a nonossicular etiology. The positive and negative predictive values for an ossicular etiology were 89% and 57% when acoustic reflexes were used alone for screening, 89% and 39% when third window symptoms were used alone, and 94% and 71% when reflexes and symptoms were used together, respectively. Acoustic reflex testing is an effective means of screening for third window disorders in patients with a conductive hearing loss. Questioning for third window symptoms should complement screening. The detection of even 1 acoustic reflex or third window symptom (regardless of reflex status) should prompt further workup prior to middle ear exploration. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

  18. [Subclinical sensorineural hearing loss in female patients with rheumatoid arthritis].

    Science.gov (United States)

    Treviño-González, José Luis; Villegas-González, Mario Jesús; Muñoz-Maldonado, Gerardo Enrique; Montero-Cantu, Carlos Alberto; Nava-Zavala, Arnulfo Hernán; Garza-Elizondo, Mario Alberto

    2015-01-01

    The rheumatoid arthritis is a clinical entity capable to cause hearing impairment that can be diagnosed promptly with high frequencies audiometry. To detect subclinical sensorineural hearing loss in patients with rheumatoid arthritis. Cross-sectional study on patients with rheumatoid arthritis performing high frequency audiometry 125Hz to 16,000Hz and tympanometry. The results were correlated with markers of disease activity and response to therapy. High frequency audiometry was performed in 117 female patients aged from 19 to 65 years. Sensorineural hearing loss was observed at a sensitivity of pure tones from 125 to 8,000 Hz in 43.59%, a tone threshold of 10,000 to 16,000Hz in 94.02% patients in the right ear and in 95.73% in the left ear. Hearing was normal in 8 (6.84%) patients. Hearing loss was observed in 109 (93.16%), and was asymmetric in 36 (30.77%), symmetric in 73 (62.37%), bilateral in 107 (91.45%), unilateral in 2 (1.71%), and no conduction and/or mixed hearing loss was encountered. Eight (6.83%) patients presented vertigo, 24 (20.51%) tinnitus. Tympanogram type A presented in 88.90% in the right ear and 91.46% in the left ear, with 5.98 to 10.25% type As. Stapedius reflex was present in 75.3 to 85.2%. Speech discrimination in the left ear was significantly different (p = 0.02)in the group older than 50 years. No association was found regarding markers of disease activity, but there was an association with the onset of rheumatoid arthritis disease. Patients with rheumatoid arthritis had a high prevalence of sensorineural hearing loss for high and very high frequencies. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

  19. Occupational Hearing Loss among Chinese Municipal Solid Waste Landfill Workers: A Cross-Sectional Study.

    Science.gov (United States)

    Liu, Yuewei; Wang, Haijiao; Weng, Shaofan; Su, Wenjin; Wang, Xin; Guo, Yanfei; Yu, Dan; Du, Lili; Zhou, Ting; Chen, Weihong; Shi, Tingming

    2015-01-01

    Occupational hearing loss is an increasingly prevalent occupational condition worldwide, and has been reported to occur in a wide range of workplaces; however, its prevalence among workers from municipal solid waste landfills (MSWLs) remains less clear. This study aimed to investigate the occupational hearing loss among Chinese MSWL workers. A cross-sectional study of 247 workers from 4 Chinese MSWLs was conducted. Noise and total volatile organic compounds (TVOCs) levels at worksites were determined. We conducted hearing examinations to determine hearing thresholds. A worker was identified as having hearing loss if the mean threshold at 2000, 3000 and 4000 Hz in either ear was equal to or greater than 25 dB. Prevalence of occupational hearing loss was then evaluated. Using unconditional Logistic regression models, we estimated the odds ratios (ORs) of MSWL work associated with hearing loss. According to the job title for each worker, the study subjects were divided into 3 groups, including group 1 of 63 workers without MSWL occupational hazards exposure (control group), group 2 of 84 workers with a few or short-period MSWL occupational hazards exposure, and group 3 of 100 workers with continuous MSWL occupational hazards exposure. Both noise and TVOCs levels were significantly higher at worksites for group 3. Significantly poorer hearing thresholds at frequencies of 2000, 3000 and 4000 Hz were found in group 3, compared with that in group 1 and group 2. The overall prevalence rate of hearing loss was 23.5%, with the highest in group 3 (36.0%). The OR of MSWL work associated with hearing loss was 3.39 (95% confidence interval [CI]: 1.28-8.96). The results of this study suggest significantly higher prevalence of hearing loss among MSWL workers. Further studies are needed to explore possible exposure-response relationship between MSWL occupational hazards exposure and hearing loss.

  20. Hearing Loss After Radiotherapy for Pediatric Brain Tumors: Effect of Cochlear Dose

    International Nuclear Information System (INIS)

    Hua, Chiaho; Bass, Johnnie K.; Khan, Raja; Kun, Larry E.; Merchant, Thomas E.

    2008-01-01

    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

  1. Smartphone-based audiometric test for screening hearing loss in the elderly.

    Science.gov (United States)

    Abu-Ghanem, Sara; Handzel, Ophir; Ness, Lior; Ben-Artzi-Blima, Miri; Fait-Ghelbendorf, Karin; Himmelfarb, Mordechai

    2016-02-01

    Hearing loss is widespread among the elderly. One of the main obstacles to rehabilitation is identifying individuals with potentially correctable hearing loss. Smartphone-based hearing tests can be administered at home, thus greatly facilitating access to screening. This study evaluates the use of a smartphone application as a screening tool for hearing loss in individuals aged ≥ 65 years. Twenty-six subjects aged 84.4 ± 6.73 years (mean ± SD) were recruited. Pure-tone audiometry was administered by both a smartphone application (uHear for iPhone, v1.0 Unitron, Canada) and a standard portable audiometer by trained personnel. Participants also completed a questionnaire on their hearing. Pure-tone thresholds were compared between the two testing modalities and correlated with the questionnaire results. The cutoff point for failing screening tests was a pure tone average of 40 dB for the frequencies 250-6,000 Hz. The smartphone application's pure tone thresholds were higher (poorer hearing) than the audiometric thresholds, with a significant difference in all frequencies but 2,000 Hz. The application and the audiometric values were in agreement for 24 subjects (92 %). The application had a sensitivity of 100 % and specificity of 60 % for screening compared with the audiometer. The questionnaire was significantly less accurate, having assigned a passing score to three participants who failed both the application and audiometric tests. While a smartphone application may not be able to accurately determine the level of hearing impairment, it is useful as a highly accessible portable audiometer substitute for screening for hearing loss in elderly populations.

  2. Initial assessment of hearing loss using a mobile application for audiological evaluation.

    Science.gov (United States)

    Derin, S; Cam, O H; Beydilli, H; Acar, E; Elicora, S S; Sahan, M

    2016-03-01

    This study aimed to compare an Apple iOS mobile operating system application for audiological evaluation with conventional audiometry, and to determine its accuracy and reliability in the initial evaluation of hearing loss. The study comprised 32 patients (16 females) diagnosed with hearing loss. The patients were first evaluated with conventional audiometry and the degree of hearing loss was recorded. Then they underwent a smartphone-based hearing test and the data were compared using Cohen's kappa analysis. Patients' mean age was 53.59 ± 18.01 years (range, 19-85 years). The mobile phone audiometry results for 39 of the 64 ears were fully compatible with the conventional audiometry results. There was a statistically significant concordant relationship between the two sets of audiometry results (p Apple iPhone 5 that can measure hearing loss with reliable results.

  3. Case report: Unilateral conduction hearing loss due to central venous occlusion.

    Science.gov (United States)

    Ribeiro, Phillip; Patel, Swetal; Qazi, Rizwan A

    2016-05-07

    Central venous stenosis is a well-known complication in patients with vascular access for hemodialysis. We report two cases involving patients on hemodialysis with arteriovenous fistulas who developed reversible unilateral conductive hearing loss secondary to critical stenosis of central veins draining the arteriovenous dialysis access. A proposed mechanism for the patients' reversible unilateral hearing loss is pterygoid venous plexus congestion leading to decreased Eustachian tube patency. Endovascular therapy was conducted to treat the stenosis and the hearing loss of both patients was returned to near normal after successful central venous angioplasty.

  4. Hearing loss on social media: Who is winning hearts and minds?

    Science.gov (United States)

    Crowson, Matthew G; Tucci, Debara L; Kaylie, David

    2018-06-01

    To analyze specific patterns of Twitter usage using common references to hearing loss, and characterize the virtual public that comprises the hearing loss community to inform hearing loss stakeholders for opportunities for engagement and outreach. Social media network analysis. Twitter tweets were sampled from July 2016 to September 2016 using #hearing, #hearingloss, #deaf, #hearingimpairment, #hardofhearing, #deafness, #hearingmatters, #hearinghealth, and #hearingimpaired tags. User and Twitter social community metrics were examined including temporal trends, tweet content, user activity, tweet reach, and an analysis of the tweets' social network. We identified and analyzed 49,208 tweets from July 2016 to September 2016 with tags relevant to hearing loss. Of the 100 most active Twitter accounts, organizations owned 67% compared to 33% owned by individuals. Commercial/for profit and informational organizations were the most common organization account owners (26% and 16%, respectively). Five unique tweets were identified as each having a reach of over 100,000 Twitter users, with the greatest reach exceeding 250,000 users. Temporal analysis identified marked retweet outliers (>300 retweets per hour) that corresponded with a widely publicized event involving the dismissal of a deaf employee from a fast-food chain store. Twitter accounts owned by organizations outnumbered individual accounts, and commercial/for profit user accounts were the most frequently active organization account type. Tweets pertaining to hearing loss may have a broad reach to a large community base. Analyses of social media use can be helpful in discovering issues of interest to the hearing loss community, as well as determining which users and organizations are dominating social network conversations. NA. Laryngoscope, 128:1453-1461, 2018. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

  5. [FEDERAL CLINICAL RECOMMENDATIONS IN DIAGNOSIS, TREATMENT AND PREVENTION OF HEARING LOSS DUE TO NOISE].

    Science.gov (United States)

    Adeninskaya, E E; Bukhtiarov, I V; Bushmanov, A Iu; Dayhes, N A; Denisov, E I; Izmerov, N F; Mazitova, N N; Pankova, V B; Preobrazhenskaya, E A; Prokopenko, L V; Simonova, N I; Tavartkiladze, G A; Fedina, I N

    2016-01-01

    Noise induced hearing loss is a slowly developing hearing impairment, caused by occupational exposure to excessive noise levels, constitutes a lesion of the auditory analyzer and clinically manifested as chronic bilateral sensorineural hearing loss. Currently, there is not a treatment that provide a cure of sensorineural hearing loss. Regular, individually tailored treatment should be directed to the pathogenic mechanisms and specific clinical symptoms of hearing loss, as well as the prevention of complications. We recommend using non-drug therapies that can improve blood flow in labyrinth, tissue and cellular metabolism.

  6. Hearing loss and contributing factors among airport workers in Malaysia.

    Science.gov (United States)

    Nasir, H M; Rampal, K G

    2012-02-01

    Sensorineural hearing loss is a common and important source of disability among the workers and often caused by occupational noise exposure. Aims of the study were to determine the prevalence and contributing factors of hearing loss among airport workers. A cross-sectional study was carried out at an airport in Malaysia. This study used stratified sampling method that involved 358 workers who were working in 3 different units between November 2008 and March 2009. Data for this study were collected by using questionnaires eliciting sociodemographic, occupational exposure history (previous and present), life-style including smoking habits and health-related data. Otoscopic and pure-tone audiometric tests were conducted for hearing assessment. Noise exposure status was categorize by using a noise logging dosimeter to obtain 8-hour Time-Weighted Average (TWA). Data was analyzed by using SPSS version 12.0.1 and EpiInfo 6.04. The prevalence of hearing loss was 33.5%. Age >40 years old (aOR 4.3, 95%CI 2.2-8.3) is the main risk factors for hearing loss followed by duration of noise exposure >5 years (aOR 2.5, 95%CI 1.4-4.7), smoking (aOR 2.1, 95%CI 1.2-3.4), duration of service >5 years (aOR 2.1, 95%CI 1.1-3.9), exposure to explosion (aOR 6.1, 95%CI 1.3-29.8), exposure to vibration (aOR 2.2, 95%CI 1.1-4.3) and working in engineering unit (aOR 5.9, 95%CI 1.1-30.9). The prevalence rate ratio of hearing loss for nonsmokers aged 40 years old and younger, smokers aged 40 years old and younger, non-smokers older than 40 years old and smokers older than 40 years old was 1.0, 1.7, 2.8 and 4.6 respectively. This result contributes towards better understanding of risk factors for hearing loss, which is relatively common among Malaysian workers.

  7. Auditory steady-state response thresholds in adults with conductive and mild to moderate sensorineural hearing loss.

    Science.gov (United States)

    Hosseinabadi, Reza; Jafarzadeh, Sadegh

    2015-01-01

    The Auditory steady state response (ASSR) provides a frequency-specific and automatic assessment of hearing sensitivity and is used in infants and difficult-to-test adults. The aim of this study was to compare the ASSR thresholds among various types (normal, conductive, and sensorineural), degree (normal, mild, and moderate), and configuration (flat and sloping) of hearing sensitivity, and measuring the cutoff point between normal condition and hearing loss for different frequencies. This clinical trial was performed in Iran and included patients who were referred from Ear, Nose, and Throat Department. A total of 54 adults (27 with sensorineural hearing loss, 17 with conductive hearing losses, and 10 with normal hearing) were randomly chosen to participate in our study. The type and degree of hearing loss were determined through testing by otoscopy, tympanometry, acoustic reflex, and pure tone audiometry. Then the ASSR was tested at carrier frequencies of 500, 1000, 2000, and 4000 Hz. The ASSR accurately estimates the behavioral thresholds as well as flat and sloping configurations. There was no correlation between types of hearing loss and difference of behavioral and ASSR thresholds (P = 0.69). The difference between ASSR and behavioral thresholds decreased as severity of hearing loss increased. The 40, 35, 30, and 35 dB could be considered as cutoffs between normal hearing and hearing loss for 500, 1000, 2000, and 4000 Hz, respectively. The ASSR can accurately predict the degree and configuration of hearing loss and discriminate the normal hearing from mild or moderate hearing loss and mild from moderate hearing loss, except for 500 Hz. The Air-conducted ASSR could not define the type of hearing loss.

  8. A mismatch negativity study in Mandarin-speaking children with sensorineural hearing loss.

    Science.gov (United States)

    Fu, Mingfu; Wang, Liyan; Zhang, Mengchao; Yang, Ying; Sun, Xibin

    2016-12-01

    a) To examine the effects of sensorineural hearing loss on the discriminability of linguistic and non-linguistic stimuli at the cortical level, and b) to examine whether the cortical responses differ based on the chronological age at intervention, the degree of hearing loss, or the acoustic stimulation mode in children with severe and profound hearing loss. Mismatch negativity (MMN) responses were collected from 43 children with severe and profound bilateral sensorineural hearing loss, and 20 children with normal hearing (age: 3-6 years). In the non-verbal stimulation condition, pure tones with frequencies of 1 kHz and 1.1 kHz were used as the standard and the deviant respectively. In the verbal stimulation condition, the Chinese mandarin tokens/ba2/and/ba4/were used as the standard and the deviant respectively. Latency and amplitude of the MMN responses were collected and analyzed. Overall, children with hearing loss showed longer latencies and lower amplitudes of the MMN responses to both non-verbal and verbal stimulations. The latency of the verbal/ba2/-/ba4/pair was longer than that of the nonverbal 1 kHz-1.1 kHz pair in both groups of children. Children with hearing loss, especially those who received intervention after 2 years of age, showed substantial weakness in the neural responses to lexical tones and pure tones. Thus, the chronological age when the children receive hearing intervention may have an impact on the effectiveness of discriminating between verbal and non-verbal signals. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  9. Improvements in Speech Understanding With Wireless Binaural Broadband Digital Hearing Instruments in Adults With Sensorineural Hearing Loss

    OpenAIRE

    Kreisman, Brian M.; Mazevski, Annette G.; Schum, Donald J.; Sockalingam, Ravichandran

    2010-01-01

    This investigation examined whether speech intelligibility in noise can be improved using a new, binaural broadband hearing instrument system. Participants were 36 adults with symmetrical, sensorineural hearing loss (18 experienced hearing instrument users and 18 without prior experience). Participants were fit binaurally in a planned comparison, randomized crossover design study with binaural broadband hearing instruments and advanced digital hearing instruments. Following an adjustment peri...

  10. Prevention of noise-induced hearing loss in the Canadian military.

    Science.gov (United States)

    Pelausa, E O; Abel, S M; Simard, J; Dempsey, I

    1995-10-01

    A prospective study was undertaken to investigate the development of noise-induced hearing loss in Canadian military recruits, and to assess the effectiveness of the hearing conservation program currently in place. The participants were 134 men and women, 20 to 30 years of age, employed in four trades, three of these (infantry, artillery, and armour) associated with high noise levels. The data comprised audiometric measurements made at the time of entry and after 3 years of employment, and responses to a questionnaire mainly relating to noise exposure in the workplace and during leisure activities, and the utilization of personal hearing protective devices. The findings showed that group audiograms at entry and at the 3-year recall were characterized by a 6-kHz notch that was indicative of noise-induced hearing loss, although mean threshold values were within normal limits. By the 3-year recall, 11% of the infantry had sustained a mild-to-moderate hearing loss in the left ear, greater than 25-dB HL, that was consistent with the use of small-calibre weapons. Responses to the questionnaire indicated that, while subjects appreciated the potential benefit of wearing hearing protectors, instructions in their proper use and education on the hazards of noise exposure were poor. The results suggested methods to strengthen the existing scheme for hearing conservation to further minimize risk.

  11. Revalence of Hearing Loss and the Related Factors in BuAli Hospital

    Directory of Open Access Journals (Sweden)

    Shohreh Jalaei

    2004-06-01

    Full Text Available Objective:Determining the prevalence of hearing loss and correlated factors in clients referring to audiology clinic of Buali hospital. Method and Material: This cross-sectional analytic-descriptive survery was carried out on six thousand and twenty ears of 3010 clients (1651 ,a;e amd 1359 female in Audiology clinic of Buali hospital , during Sept 2000 to Sept 2001. Results: Hearing loss is the most common reason for referring the clients (20.5%. 1319 ears (21.9% Showed sensorineural hearing impairment and conductive and mixed hearing loss are observed in 1059 (17.6% and 234 (3.9% ears, respectedly. Hearing loss degree most cases is mild (14% in both ears. There is no significant difference between male and female hearing threshold means (p>0.05 ‘ but a significant difference between hearing thresholds is observed in terms of age (p<0.05. Audiogram configuration in most cases is flat (55.1%. Otoscopic examination reveals abnormal condition in 2333 ears (38.8% . 37.2% of the studied cases have abnormal tympanogram mostly type B (15.9%. 2.2% of the clients wear hearing aid that mostly have B.T.E ones (1.5%. 6.2% of the clients , depends on their hearing impairment type and degree need rehabilitation services. Conclusion: The results are Valid only in the context of this study and it’s generaliztion needs further researches.

  12. The impact of hyperacusis and hearing loss on tinnitus perception in German teachers

    Directory of Open Access Journals (Sweden)

    Sandra P Meuer

    2015-01-01

    Full Text Available Recent research indicates that a notable number of teachers are concerned with conditions of auditory impairment such as tinnitus, hyperacusis, and hearing loss. Studies focussing on characteristics and interdependencies of single hearing disorders (HD are rare. This explorative study examines tinnitus, hyperacusis, hearing loss, and all possible combinations (tinnitus + hyperacusis; tinnitus + hearing loss; hyperacusis + hearing loss; tinnitus, hyperacusis + hearing loss in German teachers. The impact of single HD on perceived distress, depending on the number and kind of comorbid HD, was of special interest. Information was collected via online survey and includes self-reported data as well as data from the Mini-Tinnitus Questionnaire (Mini-TQ. Results show that most of the 1468 participants (45% suffered from two HD in different combinations, and the fewest (25% were afflicted with only one HD. Considering the seven HD groups, most teachers (30% suffered from all three HD. Across all groups, tinnitus was present in 1096, hyperacusis in 988, and hearing loss in 937 teachers. Multiple intergroup comparisons revealed that self-rated tinnitus-related distress rose significantly with the increasing number of HD. No significant differences were found for distress ratings of hyperacusis between the four groups including hyperacusis and between the four groups with hearing loss. In the Mini-TQ, groups including hyperacusis scored considerably higher than those excluding hyperacusis. The frequent prevalence of HD in German teachers points to a need of better noise prevention in German schools as one priority of occupational safety.

  13. Hear here: children with hearing loss learn words by listening.

    Science.gov (United States)

    Lew, Joyce; Purcell, Alison A; Doble, Maree; Lim, Lynne H

    2014-10-01

    Early use of hearing devices and family participation in auditory-verbal therapy has been associated with age-appropriate verbal communication outcomes for children with hearing loss. However, there continues to be great variability in outcomes across different oral intervention programmes and little consensus on how therapists should prioritise goals at each therapy session for positive clinical outcomes. This pilot intervention study aimed to determine whether therapy goals that concentrate on teaching preschool children with hearing loss how to distinguish between words in a structured listening programme is effective, and whether gains in speech perception skills impact on vocabulary and speech development without them having to be worked on directly in therapy. A multiple baseline across subjects design was used in this within-subject controlled study. 3 children aged between 2:6 and 3:1 with moderate-severe to severe-profound hearing loss were recruited for a 6-week intervention programme. Each participant commenced at different stages of the 10-staged listening programme depending on their individual listening skills at recruitment. Speech development and vocabulary assessments were conducted before and after the training programme in addition to speech perception assessments and probes conducted throughout the intervention programme. All participants made gains in speech perception skills as well as vocabulary and speech development. Speech perception skills acquired were noted to be maintained a week after intervention. In addition, all participants were able to generalise speech perception skills learnt to words that had not been used in the intervention programme. This pilot study found that therapy directed at listening alone is promising and that it may have positive impact on speech and vocabulary development without these goals having to be incorporated into a therapy programme. Although a larger study is necessary for more conclusive findings, the

  14. Epileptiform electroencephalogram abnormality in children with congenital sensorineural hearing loss.

    Science.gov (United States)

    El-Badry, Mohamed Mohamed; Hamdy, Nermin Aly; Sobhy, Sayed; Gamal, Reham

    2014-04-01

    This work was designed to study electroencephalogram findings in children with congenital sensorineural hearing loss and correlate these findings with the SNHL parameters as duration, etiology, severity, and type. Ninety children with bilateral congenital sensorineural hearing loss served as the study group. They were free from any neurological disorders or symptoms that are commonly associated with abnormal electroencephalogram as convulsions or loss of consciousness. Twenty children having normal hearing with no history of otological or neurological disorders served as the control group. All children participating in the study were subjected to full medical and audiological history, otological examination, neurological examination, audiological evaluation and electroencephalogram recording. Mean age of the children in the control group was 3.56 ± 2.1 years and mean age of the children in the study group was 3.8 ± 2.2 years. While none of the control children had abnormal electroencephalogram, 38 (42.2%) of children with congenital SNHL had epileptiform electroencephalogram abnormality. The epileptiform abnormality was generalized in 14 children (36.8%), focal temporal in 17 children (44.7%) and focal other than temporal in 7 children (18.4%). According to the hemispheric side affected, the abnormality was right in 14 children (36.8%), left in 10 children (26.3%) and bilateral in 14 children (36.8%). No statistically significant predominance of specific site or side of the epileptiform abnormality was found. Similarly, no statistical significant prevalent of the epileptiform abnormality was found in relation to the age or sex of children, duration of hearing loss or etiology of hearing loss (i.e., genetic vs. neonatal insults). On the other hand, the epileptiform abnormality was statistically prevalent in children with moderate degree of hearing loss, and in children with auditory neuropathy spectrum disorder. The epileptiform electroencephalogram abnormality is

  15. Study the Age of Hearing Loss Diagnosis and the Related Factors in a Group of School-Age Children in Baghch-e-Ban School of Hard of Hearing

    Directory of Open Access Journals (Sweden)

    Mahnaz Ahmadi

    1996-04-01

    Full Text Available Background: Estimation of the age of hearing loss diagnosis in every society is recommended to be compared with standard criteria to establish common dirth in management and rehabilitation. This study was conducted in order to determine the age of hearing loss diagnosis with respect to common criteria in Baghche-Ban school of hard of hearing children in Tehran.Methods: In this retrospective cross-sectional study, 445 children in stages 2, 6 and 10 of Baghche-Ban school of hard of hearing participated. the research was performed by means of the three-stages questionaires that children filled with help of their parents.Results: The mean age of detection of hearing loss in both acquired and congenital hearing loss in children was 3.5 year-old. while the age in which hearing loss was doubted was 1.8 year-old. In another word the mean age of hearing loss detection decreased as the hearing loss increases(in moderate- profound hearing loss(P<0.01. Conclusion: among the refrence sources for refering children with hearing loss the least refering was related to physicians. Adversly the mean age of doubting and detection of hearing loss increased in children with family history of hearing loss.(P<0.01. Meanwhile no relationship was detected between the age of diagnosis of hearing loss and the number of deaf members in the family(P<0.02.

  16. Hearing Loss in Patients with Nasopharyngeal Carcinoma after Chemotherapy and Radiation

    Directory of Open Access Journals (Sweden)

    Ling-Feng Wang

    2003-04-01

    Full Text Available In light of the possible adverse effects of radiation on hearing, we conducted a study to evaluate the long-term sensorineural hearing status following radiotherapy (RT in patients suffering from nasopharyngeal carcinoma. Audiologic examinations were performed at regular intervals before and after RT. We also analyzed the effects of age, chemotherapy, pre-RT hearing status, and post-RT otitis media with effusion (OME on post-RT hearing change. A total of 150 patients (261 ears were enrolled in this study and followed up for a mean of 43.8 months. After RT, 8.9-28.8% of ears had at least a 10 dB loss in bone conduction threshold at speech frequency, which was defined as an average of hearing threshold at 0.5 kHz, 1 kHz, and 2 kHz, while the percentage was 18-34.2% at 4 kHz. Patient age was related to these changes at speech frequency, and the presence of post-RT OME was related to significant loss at both speech frequency and 4 kHz. Pre-RT hearing status and chemotherapy did not influence hearing change. To sum up, sensorineural hearing loss began as early as after completion of RT. Early changes may be transient, but the effect of radiation on hearing tended to be chronic and progressive.

  17. Contemporary noise-induced hearing loss (NIHL) prevention.

    Science.gov (United States)

    Sułkowski, Wiesław; Owczarek, Kalina; Olszewski, Jurek

    2017-08-31

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

  18. A Preliminary Study of a Spanish Graphic Novella Targeting Hearing Loss Prevention.

    Science.gov (United States)

    Guiberson, Mark; Wakefield, Emily

    2017-09-18

    This preliminary study developed a digital graphic novella targeting hearing protection beliefs of Spanish-speaking agricultural workers. Researchers used pretest-posttest interview surveys to establish if the novella had an immediate influence on the participants' beliefs about noise-induced hearing loss and usage of hearing protection devices. Researchers developed a digital graphic novella directed to increase knowledge about noise-induced hearing loss and increase the proper use of hearing protection devices. The novella was tailored to meet the specific linguistic and literacy needs of Spanish-speaking agricultural workers. Thirty-one Spanish-speaking farmworkers of Mexican nationality participated. This study included an interview survey with specific questions on noise-induced hearing loss, myths, and hearing protection device usage. A pretest-posttest design was applied to measure the graphic novella's immediate influence on workers. The posttest scores on Hearing Protection Beliefs statements were significantly better than pretest scores, with a large effect size observed. Digital media may be an effective way to overcome language and literacy barriers with Spanish-speaking workers when providing health education and prevention efforts.

  19. Auditory Temporal-Organization Abilities in School-Age Children with Peripheral Hearing Loss

    Science.gov (United States)

    Koravand, Amineh; Jutras, Benoit

    2013-01-01

    Purpose: The objective was to assess auditory sequential organization (ASO) ability in children with and without hearing loss. Method: Forty children 9 to 12 years old participated in the study: 12 with sensory hearing loss (HL), 12 with central auditory processing disorder (CAPD), and 16 with normal hearing. They performed an ASO task in which…

  20. Gait performance of children and adolescents with sensorineural hearing loss.

    Science.gov (United States)

    Melo, Renato de Souza

    2017-09-01

    Several studies have demonstrated that children with sensorineural hearing loss (SNHL) may exhibit balance disorders, which can compromise the gait performance of this population. Compare the gait performance of normal hearing (NH) children and those with SNHL, considering the sex and age range of the sample, and analyze gait performance according to degrees of hearing loss and etiological factors in the latter group. This is a cross-sectional study that assessed 96 students, 48 NH and 48 with SNHL, aged between 7 and 18 years. The Brazilian version of the Dynamic Gait Index (DGI) was used to analyze gait and the Mann-Whitney test for statistical analysis. The group with SNHL obtained lower average gait performance compared to NH subjects (p=0.000). This was also observed when the children were grouped by sex female and male (p=0.000). The same difference occurred when the children were stratified by age group: 7-18 years (p=0.000). The group with severe and profound hearing loss exhibited worse gait performance than those with mild and moderate loss (p=0.048) and children with prematurity as an etiological factor demonstrated the worst gait performance. The children with SNHL showed worse gait performance compared to NH of the same sex and age group. Those with severe and profound hearing loss and prematurity as an etiological factor demonstrated the worst gait performances. Copyright © 2017 Elsevier B.V. All rights reserved.

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

    Science.gov (United States)

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

    2017-01-01

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

  2. Hearing outcomes of the active bone conduction system Bonebridge® in conductive or mixed hearing loss.

    Science.gov (United States)

    Carnevale, Claudio; Til-Pérez, Guillermo; Arancibia-Tagle, Diego J; Tomás-Barberán, Manuel D; Sarría-Echegaray, Pedro L

    2018-05-18

    The active transcutaneous bone conduction implant Bonebridge ® , is indicated for patients affected by bilateral conductive/mixed hearing loss or unilateral sensorineural hearing loss, showing hearing outcomes similar to other percutaneous bone conduction implants, but with a lower rate of complications. The aim of this study was to analyze the hearing outcomes in a series of 26 patients affected by conductive or mixed hearing loss and treated with Bonebridge ® . 26 of 30 patients implanted with Bonebridge ® between October 2012 and May 2017, were included in the study. We compared the air conduction thresholds at the frequencies 500, 1000, 2000, 3000, 4000Hz, the SRT50% and the percentage of correct answers at an intensity of 50dB with and without the implant. "Pure tone average" with the implant was 34.91dB showing an average gain of 33.46dB. Average SRT 50% with the implant was 34.33dB, whereas before the surgery no patient achieved 50% of correct answers at a sound intensity of 50dB. The percentage of correct answers at 50dB changed from 11% without the implant to 85% with it. We only observed one complication consisting of an extrusion of the implant in a patient with a history of 2 previous rhytidectomies. The hearing outcomes obtained in our study are similar to those published in the literature. Bonebridge ® represents an excellent alternative in the treatment of conductive or mixed hearing loss, and with a lower rate of complications. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

  3. Social identity management strategies used by workers with acquired hearing loss.

    Science.gov (United States)

    Jennings, Mary Beth; Southall, Kenneth; Gagné, Jean-Pierre

    2013-01-01

    There is a paucity of knowledge about social identity-management by persons with hearing loss. The objective of the study was to gain an understanding from the perspective of the participants, the ways in which workers with acquired hearing loss manage their identity in the workplace. Twelve persons with acquired hearing loss, who were gainfully employed in a variety of settings and occupations in three Canadian cities, participated in audio-recorded semi-structured interviews. A secondary qualitative analysis was conducted on transcripts of interviews collected in a previous study on factors that influence disclosure of hearing loss in the workplace. A qualitative descriptive research paradigm was adopted and content analyses were used to extract pertinent information from verbatim transcripts. Participants described a range of identity-management strategies enacted in the workplace. Five recurrent themes emerged as important considerations in the Art of Identity Management in the workplace: 1. Managing the situation, 2. Having a buddy system, 3. Feeling comfortable, 4. Using personal resources, 5. It gets easier with time. Social identity-management is a complex process. Although persons with acquired hearing loss experience different challenges from other persons with invisible stigmas, similarities in the range of social identity-management strategies employed were evident in our findings. In addition, the social cognitive learning model of disclosure appears to be relevant to the experiences of our participants. The implications of the findings emphasize the importance of all stakeholders working collaboratively to address the issues of the growing population of workers with hearing loss.

  4. Occupational hearing loss of market mill workers in the city of Accra, Ghana

    OpenAIRE

    Emmanuel D Kitcher; Grace Ocansey; Benjamin Abaidoo; Alidu Atule

    2014-01-01

    Noise induced hearing loss (NIHL) is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within m...

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

    Science.gov (United States)

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

    2017-06-01

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

  6. Psychological Assessment of Children with Multiple Handicaps Who Have Hearing Loss

    Science.gov (United States)

    Schum, Robert

    2004-01-01

    This article discusses issues involved in psychological assessment of children with hearing loss who have additional disabilities or special needs. It provides recommendations for appropriate methods of assessment that accommodate the communication difficulties associated with hearing loss. This article includes assessment procedures for children…

  7. The Effects of Simulated Hearing Loss on Simultaneous Speech Recognition and Walking Navigation Tasks

    Science.gov (United States)

    2013-02-01

    Spectral equalizations as mentioned above are commonly used in audiology to simulate hearing loss for patient education, research, and development of...www.hearingreview.com/issues/ articles /2012-01_04.asp. Saunders, G. H.; Griest, S. Hearing Loss in Veterans and the Need for Hearing Loss

  8. Noise exposure and hearing conservation practices in an industry with high incidence of workers' compensation claims for hearing loss.

    Science.gov (United States)

    Daniell, William E; Swan, Susan S; McDaniel, Mary M; Stebbins, John G; Seixas, Noah S; Morgan, Michael S

    2002-10-01

    Washington State has experienced a striking increase in workers' compensation claims for hearing loss. This cross-sectional study examined noise exposures and hearing conservation practices in one industry with a high rate of hearing loss claims. We evaluated 10 representative foundries with personal noise dosimetry, management interviews, employee interviews, and existing audiometry. Noise levels routinely exceeded 85 dBA. All companies were out of compliance with hearing conservation regulations. Most employees with important findings on audiograms were not aware of their findings. There was a significant positive correlation between management-interview scores and worksite-average employee-interview scores (r = 0.70, P = 0.02). Companies where more effort is put into hearing conservation program activities can achieve a greater positive impact on employee awareness. However, there were broad deficiencies even in the better programs in this sample, suggesting that workers in this industry probably face a continuing substantial risk of occupational hearing loss. Copyright 2002 Wiley-Liss, Inc.

  9. Hearing Loss in Children With Otitis Media With Effusion: Actual and Simulated Effects on Speech Perception.

    Science.gov (United States)

    Cai, Ting; McPherson, Bradley; Li, Caiwei; Yang, Feng

    2017-11-14

    Conductive hearing loss simulations have attempted to estimate the speech-understanding difficulties of children with otitis media with effusion (OME). However, the validity of this approach has not been evaluated. The research aim of the present study was to investigate whether a simple, frequency-specific, attenuation-based simulation of OME-related hearing loss was able to reflect the actual effects of conductive hearing loss on speech perception. Forty-one school-age children with OME-related hearing loss were recruited. Each child with OME was matched with a same sex and age counterpart with normal hearing to make a participant pair. Pure-tone threshold differences at octave frequencies from 125 to 8000 Hz for every participant pair were used as the simulation attenuation levels for the normal-hearing children. Another group of 41 school-age otologically normal children were recruited as a control group without actual or simulated hearing loss. The Mandarin Hearing in Noise Test was utilized, and sentence recall accuracy at four signal to noise ratios (SNR) considered representative of classroom-listening conditions were derived, as well as reception thresholds for sentences (RTS) in quiet and in noise using adaptive protocols. The speech perception in quiet and in noise of children with simulated OME-related hearing loss was significantly poorer than that of otologically normal children. Analysis showed that RTS in quiet of children with OME-related hearing loss and of children with simulated OME-related hearing loss was significantly correlated and comparable. A repeated-measures analysis suggested that sentence recall accuracy obtained at 5-dB SNR, 0-dB SNR, and -5-dB SNR was similar between children with actual and simulated OME-related hearing loss. However, RTS in noise in children with OME was significantly better than that for children with simulated OME-related hearing loss. The present frequency-specific, attenuation-based simulation method reflected

  10. Occupational Noise Exposure, Bilateral High-Frequency Hearing Loss, and Blood Pressure.

    Science.gov (United States)

    Gan, Wen Qi; Mannino, David M

    2017-11-13

    The aim of this study was to investigate the relationships between occupational noise exposure and blood pressure using self-reported occupational exposure and bilateral high-frequency hearing loss. This study included 4548 participants aged 20 to 69 years from the National Health and Nutrition Examination Survey 1999 to 2004. On the basis of self-reported exposure status, participants were divided into the current, former, or never exposed groups. Bilateral high-frequency hearing loss was defined as the average high-frequency hearing threshold at least 25 dB in both ears. The currently exposed participants had slightly increased diastolic blood pressure compared with those never exposed. Among previously exposed participants, those with bilateral high-frequency hearing loss had increased systolic blood pressure, heart rate, and the prevalence of hypertension compared with those with normal high-frequency hearing. Although there were some significant results, the evidence was not consistent to support the associations between occupational noise exposure and blood pressure.

  11. A Review of Hearing Loss in Cleft Palate Patients

    Directory of Open Access Journals (Sweden)

    Bilal Gani

    2012-01-01

    Full Text Available Background. Cleft palate is associated with recurrent otitis media with effusion and hearing loss. This study analysed the way these patients’ hearing is managed in Alder Hey Children’s Hospital. Method. A retrospective audit was carried out on cleft palate patients in Alder Hey Children’s Hospital. Audiology assessment and treatment options were reviewed. Comparisons were made between the use of ventilation tubes (VTs and hearing aids (HAs. The types of cleft, types of hearing loss, and the management output of the audiology regions were also reviewed. Results. The audiology assessments of 254 patients were examined. The incidence of VT insertion in this group of patients was 18.9%. The hearing aid incidence rate was 10.1%. The VT-related complication rate was 25.5% and the HA related complication rate was 9.1%. Conclusion. The data demonstrates that both treatments are viable, and a new protocol which combines the short term benefit of VT insertion with the lower complication rate of HA is required.

  12. Classification of Hearing Loss Disorders Using Teoae-Based Descriptors

    Science.gov (United States)

    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.

  13. Depression, Anxiety and Stress Scale in patients with tinnitus and hearing loss.

    Science.gov (United States)

    Gomaa, Mohammed Abdel Motaal; Elmagd, Manal Hassan Abo; Elbadry, Mohammed Mohammed; Kader, Rafeek Mohammed Abdel

    2014-08-01

    The study was proposed to evaluate co-morbid depression, anxiety and stress associated with tinnitus patients. The study was done on 196 subjects: 100 patients suffering from subjective tinnitus associated with hearing loss (tinnitus group), 45 patients suffering from hearing loss only (hearing loss group) and 50 healthy subjects not suffering from tinnitus or hearing loss (control group); the age ranges from 20 to 60 years old. The studied sample was subjected to full ear, nose and throat examinations and audiological evaluation. Depression, Anxiety and Stress Scale (DASS) was developed by Levibond H and Levibond F to assess three self-report scales designed to measure the negative emotional status of depression, anxiety and stress. All patients and control group were evaluated by DASS. (1) Depression: males were affected more than females. All patients over 60 years were affected by depression. The duration of tinnitus seems correlating with the severity of depression. Only 2 patients (4.3 %) of the hearing loss group suffer from depression. (2) Anxiety: 90 % of males suffer from anxiety as compared to 83.3 % females. The age group 20-29 years old suffers more than other age groups. Only 4 patients (8.7 %) of hearing loss group suffer from anxiety. (3) Stress: females seem to be affected by the stress (76.7 %) more than males (67.5). Patients in age group 30-39 suffer the most from the disease. There is a direct correlation between duration of tinnitus and severity of stress. No one of the hearing loss group suffers from stress. In conclusion, depression, anxiety and stress should be taken into consideration in the treatment of patients suffering from tinnitus.

  14. Profound, prelingual nonsyndromic deafness maps to chromosome 10q21 and is caused by a novel missense mutation in the Usher syndrome type IF gene PCDH15.

    Science.gov (United States)

    Doucette, Lance; Merner, Nancy D; Cooke, Sandra; Ives, Elizabeth; Galutira, Dante; Walsh, Vanessa; Walsh, Tom; MacLaren, Linda; Cater, Tracey; Fernandez, Bridget; Green, Jane S; Wilcox, Edward R; Shotland, Lawrence I; Shotland, Larry; Li, Xiaoyan Cindy; Li, X C; Lee, Ming; King, Mary-Claire; Young, Terry-Lynn

    2009-05-01

    We studied a consanguineous family (Family A) from the island of Newfoundland with an autosomal recessive form of prelingual, profound, nonsyndromic sensorineural hearing loss. A genome-wide scan mapped the deafness trait to 10q21-22 (max LOD score of 4.0; D10S196) and fine mapping revealed a 16 Mb ancestral haplotype in deaf relatives. The PCDH15 gene was mapped within the critical region and was an interesting candidate because truncating mutations cause Usher syndrome type IF (USH1F) and two missense mutations have been previously associated with isolated deafness (DFNB23). Sequencing of the PCDH15 gene revealed 33 sequencing variants. Three of these variants were homozygous exclusively in deaf siblings but only one of them was not seen in ethnically matched controls. This novel c.1583 T>A transversion predicts an amino-acid substitution of a valine with an aspartic acid at codon 528 (V528D). Like the two DFNB23 mutations, the V528D mutation in Family A occurs in a highly conserved extracellular cadherin (EC) domain of PCDH15 and is predicted to be more deleterious than the previously identified DFNB23 missense mutations (R134G and G262D). Physical assessment, vestibular and visual function testing in deaf adults ruled out syndromic deafness because of Usher syndrome. This study validates the DFNB23 designation and supports the hypothesis that missense mutations in conserved motifs of PCDH15 cause nonsyndromic hearing loss. This emerging genotype-phenotype correlation in USH1F is similar to that in several other USH1 genes and cautions against a prognosis of a dual sensory loss in deaf children found to be homozygous for hypomorphic mutations at the USH1F locus.

  15. Spatial Release From Masking in Children: Effects of Simulated Unilateral Hearing Loss.

    Science.gov (United States)

    Corbin, Nicole E; Buss, Emily; Leibold, Lori J

    The purpose of this study was twofold: (1) to determine the effect of an acute simulated unilateral hearing loss on children's spatial release from masking in two-talker speech and speech-shaped noise, and (2) to develop a procedure to be used in future studies that will assess spatial release from masking in children who have permanent unilateral hearing loss. There were three main predictions. First, spatial release from masking was expected to be larger in two-talker speech than in speech-shaped noise. Second, simulated unilateral hearing loss was expected to worsen performance in all listening conditions, but particularly in the spatially separated two-talker speech masker. Third, spatial release from masking was expected to be smaller for children than for adults in the two-talker masker. Participants were 12 children (8.7 to 10.9 years) and 11 adults (18.5 to 30.4 years) with normal bilateral hearing. Thresholds for 50%-correct recognition of Bamford-Kowal-Bench sentences were measured adaptively in continuous two-talker speech or speech-shaped noise. Target sentences were always presented from a loudspeaker at 0° azimuth. The masker stimulus was either co-located with the target or spatially separated to +90° or -90° azimuth. Spatial release from masking was quantified as the difference between thresholds obtained when the target and masker were co-located and thresholds obtained when the masker was presented from +90° or -90° azimuth. Testing was completed both with and without a moderate simulated unilateral hearing loss, created with a foam earplug and supra-aural earmuff. A repeated-measures design was used to compare performance between children and adults, and performance in the no-plug and simulated-unilateral-hearing-loss conditions. All listeners benefited from spatial separation of target and masker stimuli on the azimuth plane in the no-plug listening conditions; this benefit was larger in two-talker speech than in speech-shaped noise. In the

  16. Hearing loss and disability exit: Measurement issues and coping strategies.

    Science.gov (United States)

    Christensen, Vibeke Tornhøj; Datta Gupta, Nabanita

    2017-02-01

    Hearing loss is one of the most common conditions related to aging, and previous descriptive evidence links it to early exit from the labor market. These studies are usually based on self-reported hearing difficulties, which are potentially endogenous to labor supply. We use unique representative data collected in the spring of 2005 through in-home interviews. The data contains self-reported functional and clinically-measured hearing ability for a representative sample of the Danish population aged 50-64. We estimate the causal effect of hearing loss on early retirement via disability benefits, taking into account the endogeneity of functional hearing. Our identification strategy involves the simultaneous estimation of labor supply, functional hearing, and coping strategies (i.e. accessing assistive devices at work or informing one's employer about the problem). We use hearing aids as an instrument for functional hearing. Our main empirical findings are that endogeneity bias is more severe for men than women and that functional hearing problems significantly increase the likelihood of receiving disability benefits for both men and women. However, relative to the baseline the effect is larger for men (47% vs. 20%, respectively). Availability of assistive devices in the workplace decreases the likelihood of receiving disability benefits, whereas informing an employer about hearing problems increases this likelihood. Copyright © 2016 Elsevier B.V. All rights reserved.

  17. Acute otitis media associated bilateral sudden hearing loss: case report and literature review.

    Science.gov (United States)

    Smith, A; Gutteridge, I; Elliott, D; Cronin, M

    2017-07-01

    Sudden sensorineural hearing loss is a rare otological condition with potential for dire outcomes including permanent hearing loss. Although the majority of cases are deemed idiopathic, bilateral sudden sensorineural hearing loss represents a rare subset typically related to systemic conditions, with higher morbidity and mortality. A controversial association with acute otitis media has been reported, with few bilateral cases published in the literature. A very rare case of bilateral sudden sensorineural hearing loss associated with acute otitis media is described, with a review of the literature. The limited evidence available suggests that acute otitis media with tinnitus and/or bacterial pathology may have an increased risk of sudden sensorineural hearing loss, which is consistent with the case described. Although there is no sufficiently powered published evidence to provide definitive treatment guidelines, the literature reviewed suggests that early myringotomy and antibiotics may greatly improve treatment outcomes.

  18. Noise-Induced Hearing Loss (NIHL): literature review with a focus on occupational medicine.

    Science.gov (United States)

    Metidieri, Mirella Melo; Rodrigues, Hugo Fernandes Santos; Filho, Francisco José Motta Barros de Oliveira; Ferraz, Daniela Pereira; Neto, Antonio Fausto de Almeida; Torres, Sandro

    2013-04-01

    According to the Ministry of Health (2006), Noise-Induced Hearing Loss (NIHL) is hearing loss caused by prolonged exposure to noise. It is characterized as sensorineural hearing loss and is usually bilateral, irreversible, and progressive while the exposure to noise continues. A NIHL is a predictable and preventable disease with an epidemiologically relevant prevalence in urban communities. The hearing loss begins and predominates in the frequencies of 3, 4, and 6 kHz and eventually progresses to 8, 2, 1, 0.5, and 0.25 kHz. In Brazil, regulatory standard 15 limits the exposure to continuous noise to no more than 4 hours' exposure to 90 dBA and a maximum level of 85 dB for a full 8-hour working period. As NIHL is a preventable and predictable disease, preventive action by professionals may be able to change the prevalence of hearing loss in noisy environments.

  19. Congenital stapes malformation: Rare conductive hearing loss in a patient with Waardenburg syndrome.

    Science.gov (United States)

    Melzer, Jonathan M; Eliason, Michael; Conley, George S

    2016-04-01

    Waardenburg syndrome is a known autosomal dominant cause of congenital hearing loss. It is characterized by a distinctive phenotypic appearance and often involves sensorineural hearing loss. Temporal bone abnormalities and inner ear dysmorphisms have been described in association with the disease. However, middle ear abnormalities as causes of conductive hearing loss are not typically seen in Waardenburg syndrome. We discuss a case of an 8-year-old female who meets diagnostic criteria for Waardenburg syndrome type 3 and who presented with a bilateral conductive hearing loss associated with congenital stapes fixation. We discuss management strategy in this previously unreported phenotype. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

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

    Science.gov (United States)

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

    2017-12-01

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

  1. Phonological Awareness and Vocabulary Performance of Monolingual and Bilingual Preschool Children with Hearing Loss

    Science.gov (United States)

    Lund, Emily; Werfel, Krystal L.; Schuele, C. Melanie

    2015-01-01

    This pilot study compared the phonological awareness skills and vocabulary performance of English monolingual and Spanish-English bilingual children with and without hearing loss. Preschool children with varying degrees of hearing loss (n = 18) and preschool children without hearing loss (n = 19) completed measures of phonological awareness and…

  2. Noise Induced Hearing Loss Among Cotton Textile and Carpet Mill Workers

    OpenAIRE

    ERTEM, Melikşah

    1998-01-01

    In industry increased mechanisation results in increased noise levels. Operation of textile machines carries a high risk of hearing loss. In this study the evaluation of textile worker's noise induced hearing loss was reviewed cross sectionally. The hearing of 260 textile workers exposed to noise levels between 85-95 dB(A) in carpet and cotton textile factories was assessed by means of air and bone conductance audiograms obtained. The subjects were grouped into five hearin...

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

    NARCIS (Netherlands)

    Beelen, E. van; Oonk, A.M.M.; Leijendeckers, J.M.; Hoefsloot, E.H.; Pennings, R.J.E.; Feenstra, I.; Dieker, H.J.; Huygen, P.L.M.; Snik, A.F.M.; Kremer, H.; Kunst, H.P.M.

    2016-01-01

    OBJECTIVES: Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the

  4. Prevalence and Risk Factors of Hearing Loss Using the Korean Working Conditions Survey

    Science.gov (United States)

    Kwon, Oh Jun

    2012-01-01

    Background and Objectives The hearing loss of workers can occur when they are affected by age, otologic disease, and work-related risks such as noise and chemicals. Based on the Korean Working Conditions Survey (KWCS) in 2010, this research aimed to estimate the prevalence rate of hearing loss and to identify the risk factors affecting its occurrence. Subjects and Methods The subjects were 10019 employees who completed an interview conducted as part of KWCS in 2010. The prevalence rate of hearing loss according to sex, age, education, income, smoking, drinking, hypertension, industrial type, occupations, employment status, working period, and hazards at the workplace were assessed. The factors that could affect the occurrence of hearing loss were investigated based on a logistic regression analysis. Results The prevalence rate of hearing loss was 2.7%. In a logistic multivariate analysis, sex, age, occupations, working period, noise, and exposure to chemicals showed statistically significant correlations to the occurrence of hearing loss. The adjusted odd ratios were as follows: 1.74 [95% confidence interval (CI), 1.03-2.96] for males, 2.11 (95% CI, 1.14-3.89) for those in their 40s, 2.24 (95% CI, 1.19-4.20) for those in their 50s, 2.21 (95% CI, 1.18-4.15) for manage/professional works, 2.73 (95% CI, 1.69-4.41) for manufacturing, 2.07 (95% CI, 1.36-3.15) for those who have worked for more than 20 years, 1.72 (95% CI, 1.14-2.58) for noise exposure, 1.53 (95% CI, 1.02-2.30) for vibration exposure and 1.58 (95% CI, 1.11-2.24) for chemical exposure. Conclusions The overall occupational and non-occupational risk factors related to employees' hearing loss were reviewed. In addition to the exposure to noise, occupational risks of hearing loss, such as isolated exposure to vibration and chemicals, and combined exposure to noise and these hazards, were identified. Multiple exposure to hazards, along with prolonged noise exposure increased the risk of hearing loss. PMID

  5. Prevalence of hearing loss and accuracy of self-report among factory workers

    OpenAIRE

    Marjorie C McCullagh; Delbert Raymond; Madeleine J Kerr; Sally L Lusk

    2011-01-01

    Noise represents one of the most common occupational health hazards. A Healthy People 2020 objective aims to reduce hearing loss in the noise-exposed public. The purpose of this study was to describe and compare perceived and measured hearing, and to determine the prevalence of hearing loss among a group of factory workers. Data collected as part of an intervention study promoting hearing protector use among workers at an automotive factory in the Midwest were used. Plant employees (n=2691) p...

  6. Editorial: Equal opportunities for children with hearing loss by ...

    African Journals Online (AJOL)

    ... the complications of otitis media. Although the South African governmental policy guidelines favour the philosophy of screening for hearing loss in infants the implementation is not realised. Widespread newborn and infant hearing screening programmes must be established to ensure equal opportunities for children with ...

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

    Science.gov (United States)

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

    2017-04-01

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

  8. Effects of long-term non-traumatic noise exposure on the adult central auditory system. Hearing problems without hearing loss.

    Science.gov (United States)

    Eggermont, Jos J

    2017-09-01

    It is known that hearing loss induces plastic changes in the brain, causing loudness recruitment and hyperacusis, increased spontaneous firing rates and neural synchrony, reorganizations of the cortical tonotopic maps, and tinnitus. Much less in known about the central effects of exposure to sounds that cause a temporary hearing loss, affect the ribbon synapses in the inner hair cells, and cause a loss of high-threshold auditory nerve fibers. In contrast there is a wealth of information about central effects of long-duration sound exposures at levels ≤80 dB SPL that do not even cause a temporary hearing loss. The central effects for these moderate level exposures described in this review include changes in central gain, increased spontaneous firing rates and neural synchrony, and reorganization of the cortical tonotopic map. A putative mechanism is outlined, and the effect of the acoustic environment during the recovery process is illustrated. Parallels are drawn with hearing problems in humans with long-duration exposures to occupational noise but with clinical normal hearing. Copyright © 2016 Elsevier B.V. All rights reserved.

  9. Mobile phone induced sensorineural hearing loss

    International Nuclear Information System (INIS)

    Al-Dousary, Surayie H.

    2007-01-01

    The increased use of mobile phones worldwide has focused interest on the biological effects and possible health outcomes of exposure to radiofrequency fields from mobile phones, and their base stations. Various reports suggest that mobile phone use can cause health problems like fatigue, headache, dizziness, tension and sleep disturbances, however, only limited research data is available in medical literature regarding interaction between electromagnetic fields emitted by mobile phones and auditory function and the possible impact on hearing. We report a case of sensorineural hearing loss due to Global System for Mobile Communication mobile phone use in a 42-year-old male. (author)

  10. Historical review of efforts to reduce noise-induced hearing loss in the United States.

    Science.gov (United States)

    Kerr, Madeleine J; Neitzel, Richard L; Hong, OiSaeng; Sataloff, Robert T

    2017-06-01

    Noise-induced hearing loss is a centuries-old problem that is still prevalent in the United States and worldwide. To describe highlights in the development of hearing loss prevention in the U.S. from World War II to the present. Literature review. Approaches to occupational noise-induced hearing loss prevention in the United States over the past seven decades are described using a hierarchy of controls framework and an interdisciplinary perspective. Historical timelines and developmental milestones related to occupational noise-induced hearing loss prevention are summarized as a life course. Lessons are drawn for other countries in their hearing conservation efforts. Future developments building on the hearing loss prevention work of the past 70 years can prevent the problem of occupational NIHL in the 21st century. Am. J. Ind. Med. 60:569-577, 2017. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  11. Exposure to loud noise, bilateral high-frequency hearing loss and coronary heart disease.

    Science.gov (United States)

    Gan, Wen Qi; Moline, Jacqueline; Kim, Hyun; Mannino, David M

    2016-01-01

    Bilateral high-frequency hearing loss is an indicator for chronic exposure to loud noise. This study aimed to examine the association between bilateral high-frequency hearing loss and the presence of coronary heart disease (CHD). This study included 5223 participants aged 20-69 years who participated in the audiometry examination of the National Health and Nutrition Examination Survey 1999-2004. Bilateral high-frequency hearing loss was defined as the average high-frequency (3, 4 and 6 kHz) hearing threshold ≥25 dB in both ears. CHD was defined as self-reported diagnoses by doctors or other health professionals. Compared with those with normal high-frequency hearing, participants with bilateral high-frequency hearing loss were more likely to have CHD (OR 1.91; 95% CI 1.28 to 2.85) after adjustment for various covariates. This association was particularly strong for currently employed workers who were exposed to loud occupational noise (OR 4.23; 95% CI 1.32 to 13.55). For this subgroup, there was no significant association of CHD with unilateral high-frequency hearing loss, and unilateral or bilateral low-frequency hearing loss. Furthermore, there was no significant association of CHD with any types of hearing loss for participants who were not exposed to loud noise. Stratified analyses for participants exposed to loud noise showed that the observed association was particularly strong for those who were less than 50 years of age, less educated and current smokers. On the basis of an objective indicator for personal chronic exposure to loud noise, this study confirmed that exposure to loud occupational noise is associated with the presence of CHD. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  12. Congenital conductive hearing loss in dyschondrosteosis.

    NARCIS (Netherlands)

    Leenheer, E. de; Oudesluijs, G.G.; Kuijpers-Jagtman, A.M.; Rappold, G.A.; Sengers, R.C.A.; Cremers, C.W.R.J.

    2003-01-01

    Conductive hearing loss was detected in a boy with a previous diagnosis of dyschondrosteosis. Dyschondrosteosis is a rare inherited condition characterized by mesomelic dwarfism and Madelung's deformity. The syndrome can be caused by mutations in the SHOX gene, and in that case, the pattern of

  13. Experimental Research Into High Barometric Oxygen Prevention of Guinea Pig Hearing Loss,

    Science.gov (United States)

    1992-08-28

    PREVENTION OF GUINEA PIG HEARING LOSS by Yin Jiacai, Sun Fang ren, et al. DTIC MLECTE •<• EP 2 9 1992 Approved for public release, Distribution unlimited...PREVENTION OF GUINEA PIG HEARING LOSS By: Yin Jiacai, Sun Fang ren, et al. English pages: 9 Source: Chung-Hua I Shueh Tsa Chih, Vol. 65, Nr. 11, Nov.eember...Distributionf._DL~~~t .•b • / or __ Dlist szeccat .lef ’ ~1 EXPERIMENTAL RESEARCH INTO HIGH BAROMETRIC OXYGEN PREVENTION OF GUINEA PIG HEARING LOSS BY: Yin

  14. Clinical and experimental studies on the sensorineural hearing loss caused by irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, M [Kobe Univ. (Japan). School of Medicine

    1979-08-01

    In our study, 43 patients whose ears were presumably irradiated, were examined periodically before and after the irradiation; 10 were found to be affected by the sensorineural hearing loss, and these 10 were classified into two types. Type I; Patients of this group had a hearing impairment during or soon after irradiation and usually revealed marked deterioration of bone conduction in high frequencies accompanied by worsening of air conduction in low frequencies. The impaired hearing of this group soon recovered to the pre-treatment level. Type II; Patients of this group developed a slow progressive sensorineural hearing loss. They noticed the deafness with tinnitus several months after irradiation. To clarify the mechanism of the sensorineural hearing loss, histopathological investigations were done using nembutal anesthetized guinea pigs which were irradiated in the confined ear region unilaterally. We also examined histopathologically one human temporal bone belonging to a patient who had been irradiated for middle ear carcinoma. The histopathology of the guinea pigs and human case revealed the following conclusions: Type I hearing loss may be caused by toxic labyrinthitis secondary to the radiation otitis media or by the aseptic labyrinthitis as the result of hyperemia and increased permeability of the irradiated blood vessels in the cochlea. Type II hearing loss may be caused by the late rediation response of the cochleal blood vessels i.e. by the vasculitis which gives rise to obliteration of the vascular lumen and affects the blood supply of the hair cells.

  15. Clinical and experimental studies on the sensorineural hearing loss caused by irradiation

    International Nuclear Information System (INIS)

    Yamamoto, Matsunori

    1979-01-01

    In our study, 43 patients whose ears were presumably irradiated, were examined periodically before and after the irradiation; 10 were found to be affected by the sensorineural hearing loss, and these 10 were classified into two types. Type I; Patients of this group had a hearing impairment during or soon after irradiation and usually revealed marked deterioration of bone conduction in high frequencies accompanied by worsening of air conduction in low frequencies. The impaired hearing of this group soon recovered to the pre-treatment level. Type II; Patients of this group developed a slow progressive sensorineural hearing loss. They noticed the deafness with tinnitus several months after irradiation. To clarify the mechanism of the sensorineural hearing loss, histopathological investigations were done using nembutal anesthetized guinea pigs which were irradiated in the confined ear region unilaterally. We also examined histopathologically one human temporal bone belonging to a patient who had been irradiated for middle ear carcinoma. The histopathology of the guinea pigs and human case revealed the following conclusions: Type I hearing loss may be caused by toxic labyrinthitis secondary to the radiation otitis media or by the aseptic labyrinthitis as the result of hyperemia and increased permeability of the irradiated blood vessels in the cochlea. Type II hearing loss may be caused by the late rediation response of the cochleal blood vessels i.e. by the vasculitis which gives rise to obliteration of the vascular lumen and affects the blood supply of the hair cells. (author)

  16. Acute sensorineural hearing loss and severe otalgia due to scrub typhus

    Directory of Open Access Journals (Sweden)

    Kim Dong-Min

    2009-10-01

    Full Text Available Abstract Background Scrub typhus is an acute febrile illness caused by Orientia tsutsugamushi. Case presentations We encountered a patient with sensorineural hearing loss complicating scrub typhus, and three patients with scrub typhus who complained of otalgia, which was sudden onset, severe, paroxysmal, intermittent yet persistent pain lasting for several seconds, appeared within 1 week after the onset of fever and rash. The acute sensorineural hearing loss and otalgia were resolved after antibiotic administration. Conclusion When patients in endemic areas present with fever and rash and have sensorineural hearing loss or otalgia without otoscopic abnormalities, clinicians should suspect scrub typhus and consider empirical antibiotic therapy.

  17. The risk ratio for development of hereditary sensorineural hearing loss in consanguineous marriage offspring.

    Science.gov (United States)

    Sanyelbhaa, Hossam; Kabel, Abdelmagied; Abo El-Naga, Heba Abd El-Rehem; Sanyelbhaa, Ahmed; Salem, Hatem

    2017-10-01

    This study aims to define the relative risk of development of hearing loss in offspring of consanguineous marriages. This is a retrospective case-control study conducted in a tertiary referral center in Jeddah, KSA. The study group included 1600 probands (848 males, 752 females), with age range 0.5-12 years (6.6 ± 3.6). The study group comprised of two equal, age and sex matched subgroups; Hearing Loss (HL) group and Normal Hearing (NH) group. The children included in the HL group should have idiopathic or non syndromic genetic sensorineural hearing loss. The HL Group comprised 800 children with variable degrees of sensorineural hearing loss. Profound and severe degrees of hearing loss were the most prevalent degrees (P marriage offspring in the NH group was 42.5%, while in the HL group it was 68.9% (P  0.05). The relative risk and 95% confidence interval (RR, 95% CI) for development of hearing loss in offspring of consanguineous marriage was 1.76 (95% CI 1.57-1.97, P marriage progeny to develop SNHL when compared to non consanguineous progeny. Copyright © 2017. Published by Elsevier B.V.

  18. Noise-Induced Hearing Loss (NIHL: literature review with a focus on occupational medicine

    Directory of Open Access Journals (Sweden)

    Metidieri, Mirella Melo

    2013-04-01

    Full Text Available According to the Ministry of Health (2006, Noise-Induced Hearing Loss (NIHL is hearing loss caused by prolonged exposure to noise. It is characterized as sensorineural hearing loss and is usually bilateral, irreversible, and progressive while the exposure to noise continues. A NIHL is a predictable and preventable disease with an epidemiologically relevant prevalence in urban communities. The hearing loss begins and predominates in the frequencies of 3, 4, and 6 kHz and eventually progresses to 8, 2, 1, 0.5, and 0.25 kHz. In Brazil, regulatory standard 15 limits the exposure to continuous noise to no more than 4 hours' exposure to 90 dBA and a maximum level of 85 dB for a full 8-hour working period. As NIHL is a preventable and predictable disease, preventive action by professionals may be able to change the prevalence of hearing loss in noisy environments.

  19. The relation of hearing loss degrees and oral stereognosis in 5-year-old children

    Directory of Open Access Journals (Sweden)

    Seyede Zohre Mousavi

    2012-12-01

    Full Text Available Background and Aim: Oral stereognosis is the ability to recognize the objects placed in the mouth; this plays a significant role in speech sounds production. Since the children with hearing loss have articulation disorders, this study aimed to clear the relation of hearing loss degrees and oral stereognosis in 5-year-old children.Methods: In this cross-sectional non-invasive study, 40 children of 5-year-old (30 children with different degrees of hearing loss and 10 normal children were involved. Oral steriognostic test was done for all of them and the Mann-Whitney U was used for statistical analysis.Results: There were significant differences between the mean of oral stereognostic ability between the normal children and the children with severe (p<0.01 or profound hearing loss (p=0.05. There was no significant difference between the mean of oral stereognostic ability among the children with moderate, severe and profound hearing loss compared with together. Besides, there was no significant difference between the mean of the time of diagnosis among all of hearing loss and normal children.Conclusion: The study shows that the children with moderate, severe and profound hearing loss have inefficient oral stereognosis in comparison with the normal children.

  20. Communication strategies and accommodations utilized by health care providers with hearing loss: a pilot study.

    Science.gov (United States)

    Trotter, Alanna R; Matt, Susan B; Wojnara, Danuta

    2014-03-01

    Poor communication between health care providers and patients may negatively impact patient outcomes, and enhancing communication is one way to improve outcomes. Effective communication is particularly important for health care providers who have hearing loss. The authors found that a systematic survey of the communication strategies and experiences of health care providers with hearing loss had not yet been conducted. In this pilot study, 32 health care professionals with hearing loss were recruited via the Association of Medical Professionals With Hearing Losses and were asked to complete a 28-question survey. Health care providers with hearing loss already employ strategies that all health care providers are encouraged to use in order to enhance patient–provider communication, and survey participants have found the strategies to be effective. The communication techniques and assistive technologies used by individuals with hearing loss seem to be effective: All participants reported feeling able to communicate effectively with patients at least most of the time. More research is needed to determine if use of these communication techniques has similar results for health care providers without hearing loss.

  1. Noise-induced hearing loss in small-scale metal industry in Nepal.

    Science.gov (United States)

    Whittaker, J D; Robinson, T; Acharya, A; Singh, D; Smith, M

    2014-10-01

    There has been no previous research to demonstrate the risk of noise-induced hearing loss in industry in Nepal. Limited research on occupational noise-induced hearing loss has been conducted within small-scale industry worldwide, despite it being a substantial and growing cause of deafness in the developing world. The study involved a cross-sectional audiometric assessment, with questionnaire-based examinations of noise and occupational history, and workplace noise level assessment. A total of 115 metal workers and 123 hotel workers (control subjects) were recruited. Noise-induced hearing loss prevalence was 30.4 per cent in metal workers and 4.1 per cent in hotel workers, with a significant odds ratio of 10.3. Except for age and time in occupation, none of the demographic factors were significant in predicting outcomes in regression analyses. When adjusted for this finding, and previous noise-exposed occupations, the odds ratio was 13.8. Workplace noise was significantly different between the groups, ranging from 65.3 to 84.7 dBA in metal worker sites, and from 51.4 to 68.6 dBA in the control sites. Metal workers appear to have a greater risk of noise-induced hearing loss than controls. Additional research on occupational noise-induced hearing loss in Nepal and small-scale industry globally is needed.

  2. Noise Exposure and Hearing Loss Among Sand and Gravel Miners

    OpenAIRE

    Landen, Deborah; Wilkins, Steve; Stephenson, Mark; McWilliams, Linda

    2004-01-01

    The objectives of this study were to describe workplace noise exposures, risk factors for hearing loss, and hearing levels among sand and gravel miners, and to determine whether full shift noise exposures resulted in changes in hearing thresholds from baseline values. Sand and gravel miners (n = 317) were interviewed regarding medical history, leisure-time and occupational noise exposure, other occupational exposures, and use of hearing protection. Audiometric tests were performed both before...

  3. The Importance of Hearing: A Review of the Literature on Hearing Loss for Older People with Learning Disabilities

    Science.gov (United States)

    Bent, Sarah; McShea, Lynzee; Brennan, Siobhan

    2015-01-01

    Background: Hearing loss has a significant impact on living well and on communication in all adults, with the numbers affected increasing with age, and adults with learning disabilities being at particular risk. Methods: A review of the literature on hearing loss in older adults with learning disabilities was completed. Results: A significant…

  4. Novel syndrome with conductive hearing loss and congenital glaucoma in three generations.

    Science.gov (United States)

    Takeuchi, Kazuhiko; Kitano, Masako; Sakaida, Hiroshi; Masuda, Sawako

    2017-08-01

    The objective of this paper was to describe the clinical and otological findings in multiple members of a family with congenital glaucoma, cardiac anomaly, and conductive hearing loss due to ossicular chain anomalies. We performed a retrospective review of the medical charts and otological materials of multiple members of the same family. Congenital glaucoma and hearing loss were inherited by the proband and her daughter, son, and mother, suggesting autosomal dominant inheritance. The son and daughter also showed atrial septal defects. Exploratory tympanotomies revealed anomalies of the long process of the incus in the proband and her daughter, and tympanoplasty improved hearing loss in both patients. This represents the first description of coexisting congenital glaucoma and conductive hearing loss due to ossicular chain anomalies in multiple members of a single family. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  5. Noise-induced hearing loss and combined noise and vibration exposure.

    Science.gov (United States)

    Turcot, A; Girard, S A; Courteau, M; Baril, J; Larocque, R

    2015-04-01

    While there is a wide body of literature addressing noise-induced hearing loss (NIHL) and hand-arm vibration syndrome (HAVS) independently, relatively few studies have considered the combined effects of noise and vibration. These studies have suggested an increased risk of NIHL in workers with vibration white finger (VWF), though the relationship remains poorly understood. To determine whether hearing impairment is worse in noise-exposed workers with VWF than in workers with similar noise exposures but without VWF. The Quebec National Institute of Public Health audiometric database was used in conjunction with work-related accident and occupational diseases data from the Quebec workers' compensation board to analyse differences in audiometry results between vibration-exposed workers in the mining and forestry industries and the overall source population, and between mining and forestry workers with documented VWF and those without VWF. The International Organization for Standardization (ISO) 7029 standards were used to calculate hearing loss not attributable to age. 15751 vibration-exposed workers were identified in an overall source population of 59339. Workers with VWF (n = 96) had significantly worse hearing at every frequency studied (500, 1000, 2000 4000 Hz) compared with other mining and forestry workers without VWF. This study confirms previous findings of greater hearing loss at higher frequencies in workers with VWF, but also found a significant difference in hearing loss at low frequencies. It therefore supports the association between combined noise and hand-arm vibration (HAV) exposure and NIHL. © The Author 2015. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  6. Transient Hearing Loss in Adults Associated With Zika Virus Infection.

    Science.gov (United States)

    Vinhaes, Eriko S; Santos, Luciane A; Dias, Lislane; Andrade, Nilvano A; Bezerra, Victor H; de Carvalho, Anderson T; de Moraes, Laise; Henriques, Daniele F; Azar, Sasha R; Vasilakis, Nikos; Ko, Albert I; Andrade, Bruno B; Siqueira, Isadora C; Khouri, Ricardo; Boaventura, Viviane S

    2017-03-01

    In 2015, during the outbreak of Zika virus (ZIKV) in Brazil, we identified 3 cases of acute hearing loss after exanthematous illness. Serology yielded finding compatible with ZIKV as the cause of a confirmed (n = 1) and a probable (n = 2) flavivirus infection, indicating an association between ZIKV infection and transient hearing loss. © The Author 2016. Published by Oxford University Press for the Infectious Diseases Society of America.

  7. Deterioration of noise-induced hearing loss among bottling factory workers.

    Science.gov (United States)

    Ologe, F E; Olajide, T G; Nwawolo, C C; Oyejola, B A

    2008-08-01

    Repeated exposure to excessive noise will eventually lead to an irreversible increase in hearing thresholds. In theory, the damage reflects both the intensity of the noise and the duration of exposure. This is not linear with respect to duration of exposure; rather, the worker may experience a disproportionate loss in the early years of exposure. A prospective study surveying workers of the production section (i.e. most noise-exposed area) of a bottling factory was carried out in December 2003 and in December 2005. A self-administered questionnaire was used to extract information about worker's demographic characteristics, drug intake, and medical and occupational history, as well as information on the use of hearing protection devices. Noise mapping of the various departments of the factory was carried out. Otological examination, tympanometry and audiometry were also carried out on selected subjects. Eighty-four workers, 76 (90.5 per cent) men and eight (9.5 per cent) women, were studied. Their mean age was 33.0 +/- 7.6 years in 2003 and 35.0 +/- 7.6 years in 2005. The recorded noise levels in the factory production section ranged between 91.5 and 98.7 dBA. The prevalence of sensorineural hearing loss among workers was noted to be 64.9 and 86.9 per cent for test one (2003) and test two (2005), respectively. The degree of hearing deterioration within the two years of this study was 1.0-3.2 dB for the right ear and 1.6-3.4 dB for the left ear. This deterioration was at discrete frequencies. More than half (53.6 per cent) of the workers did not have a hearing protection device. Of the 46.4 per cent who did, only 38.5 per cent claimed to have used it regularly. These findings showed that there was a high prevalence of mild sensorineural hearing loss and significant hearing deterioration among workers, due to exposure to excessive noise over a two-year period. The study demonstrates the practical importance of serial audiometry for noise-exposed workers as a means of

  8. Acquisition of who-question comprehension in German children with hearing loss.

    Science.gov (United States)

    Wimmer, Eva; Rothweiler, Monika; Penke, Martina

    2017-05-01

    For children with sensorineural hearing loss the ability to understand wh-questions might be particularly challenging because they often have only restricted access to spoken language input during optimal periods of language acquisition. In previous research it has been suggested that this restricted input during critical stages in language acquisition might lead to syntactic deficits that persist into adolescence. In this study we want to pursue this issue by investigating the comprehension of wh-questions in German children with bilateral sensorineural hearing loss. We report results of a who-question comprehension task in a group of 21 3- to 4-year-old German hard-of-hearing children compared to a group of age-matched children with normal hearing. The group data and individual performance patterns suggest that the syntactic comprehension difficulties observed in some, but not all, of the children with hearing loss reflect a delay in the acquisition of who-question comprehension rather than a persistent syntactic deficit. Follow-up data elicited from a subgroup of children confirm this supposition. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. [Progressive noise induced hearing loss caused by hearing AIDS, a dilemma for the worker and the expert alike].

    Science.gov (United States)

    Feldmann, H

    2001-12-01

    Investigating cases of noise induced hearing loss the expert is often confronted with the situation that the hearing loss is progressive although the noise exposure has been reduced to almost non-damaging levels. Other causes such as age, hereditary deafness, head injuries, blasts, internal diseases can be excluded. Hearing aids as sources of damaging noise? By consulting the protocol of the hearing-aid acoustician and by own examinations the expert should obtain the following data: loudness level that yields best discrimination score of speech; level of discomfort for tones and speech, discrimination score that is achieved under free field condition with a speech level of 65 dB, using the hearing aids. Furthermore he should explore the circumstances under which the hearing aids are used: how many hours per day, at what occasions etc.? It is likely that in using the hearing aids they are adjusted to emit an intensity level identical to the one yielding the optimal discrimination score. If this e. g. is 100 dB and the hearing aids are used for 2 hours per day this would be equivalent to an exposure to industrial noise of 94 dB (A) for 8 hours daily without ear protection. Among all individuals working under industrial noise exposure today only about 1 - 2 % having unusually vulnerable inner ears will suffer a noise induced hearing loss. On the other hand workers in industrial noise are accustomed to loud noise levels, usually have a raised threshold of discomfort and therefore are likely to adjust their hearing aids to such high intensities. The expert will have to decide whether in an individual case the industrial noise exposure or the use of the hearing aids is the dominant risk for further damage. The consequences in respect to the regulations of the workers' health insurance are discussed.

  10. Gipc3 mutations associated with audiogenic seizures and sensorineural hearing loss in mouse and human

    NARCIS (Netherlands)

    Charizopoulou, N.; Lelli, A.; Schraders, M.; Ray, K.; Hildebrand, M.S.; Ramesh, A.; Srisailapathy, C.R.; Oostrik, J.; Admiraal, R.J.C.; Neely, H.R.; Latoche, J.R.; Smith, R.J.; Northup, J.K.; Kremer, J.M.J.; Holt, J.R.; Noben-Trauth, K.

    2011-01-01

    Sensorineural hearing loss affects the quality of life and communication of millions of people, but the underlying molecular mechanisms remain elusive. Here, we identify mutations in Gipc3 underlying progressive sensorineural hearing loss (age-related hearing loss 5, ahl5) and audiogenic seizures

  11. Consumption of omega-3 fatty acids and fish and risk of age-related hearing loss.

    Science.gov (United States)

    Gopinath, Bamini; Flood, Victoria M; Rochtchina, Elena; McMahon, Catherine M; Mitchell, Paul

    2010-08-01

    Identification of modifiable risk factors that could prevent or slow the development of age-related hearing loss (presbycusis) would be valuable. Dietary polyunsaturated fatty acid (PUFA) intake may be related to age-related hearing loss. We aimed to determine the association between dietary intakes of omega-3 (n-3) PUFAs and fish and the risk of presbycusis. The Blue Mountains Hearing Study is a population-based survey of age-related hearing loss (1997-1999 to 2002-2004). We collected dietary data by using a semiquantitative food-frequency questionnaire and calculated PUFA and fish intakes. In 2956 participants (aged > or =50 y), we measured presbycusis, which we defined as the pure-tone average of frequencies 0.5, 1.0, 2.0, and 4.0 kHz >25 decibels of hearing loss. There was an inverse association between total n-3 PUFA intake and prevalent hearing loss [odds ratio (OR) per SD increase in energy-adjusted n-3 PUFAs: 0.89; 95% CI: 0.81, 0.99]. There was an inverse association between long-chain n-3 PUFAs and incident hearing loss (OR per SD increase in long-chain n-3 PUFAs: 0.76; 95% CI: 0.60, 0.97). Participants who had > or =2 servings of fish/wk compared with participants who had consumption of > or =1 to consumption of fish and hearing loss. Dietary intervention with n-3 PUFAs could prevent or delay the development of age-related hearing loss.

  12. Improvements in speech understanding with wireless binaural broadband digital hearing instruments in adults with sensorineural hearing loss.

    Science.gov (United States)

    Kreisman, Brian M; Mazevski, Annette G; Schum, Donald J; Sockalingam, Ravichandran

    2010-03-01

    This investigation examined whether speech intelligibility in noise can be improved using a new, binaural broadband hearing instrument system. Participants were 36 adults with symmetrical, sensorineural hearing loss (18 experienced hearing instrument users and 18 without prior experience). Participants were fit binaurally in a planned comparison, randomized crossover design study with binaural broadband hearing instruments and advanced digital hearing instruments. Following an adjustment period with each device, participants underwent two speech-in-noise tests: the QuickSIN and the Hearing in Noise Test (HINT). Results suggested significantly better performance on the QuickSIN and the HINT measures with the binaural broadband hearing instruments, when compared with the advanced digital hearing instruments and unaided, across and within all noise conditions.

  13. Auditory Outcomes with Hearing Rehabilitation in Children with Unilateral Hearing Loss: A Systematic Review.

    Science.gov (United States)

    Appachi, Swathi; Specht, Jessica L; Raol, Nikhila; Lieu, Judith E C; Cohen, Michael S; Dedhia, Kavita; Anne, Samantha

    2017-10-01

    Objective Options for management of unilateral hearing loss (UHL) in children include conventional hearing aids, bone-conduction hearing devices, contralateral routing of signal (CROS) aids, and frequency-modulating (FM) systems. The objective of this study was to systematically review the current literature to characterize auditory outcomes of hearing rehabilitation options in UHL. Data Sources PubMed, EMBASE, Medline, CINAHL, and Cochrane Library were searched from inception to January 2016. Manual searches of bibliographies were also performed. Review Methods Studies analyzing auditory outcomes of hearing amplification in children with UHL were included. Outcome measures included functional and objective auditory results. Two independent reviewers evaluated each abstract and article. Results Of the 249 articles identified, 12 met inclusion criteria. Seven articles solely focused on outcomes with bone-conduction hearing devices. Outcomes favored improved pure-tone averages, speech recognition thresholds, and sound localization in implanted patients. Five studies focused on FM systems, conventional hearing aids, or CROS hearing aids. Limited data are available but suggest a trend toward improvement in speech perception with hearing aids. FM systems were shown to have the most benefit for speech recognition in noise. Studies evaluating CROS hearing aids demonstrated variable outcomes. Conclusions Data evaluating functional and objective auditory measures following hearing amplification in children with UHL are limited. Most studies do suggest improvement in speech perception, speech recognition in noise, and sound localization with a hearing rehabilitation device.

  14. Cochlear implantation: is hearing preservation necessary in severe to profound hearing loss?

    Science.gov (United States)

    Derinsu, Ufuk; Serin, Gediz Murat; Akdaş, Ferda; Batman, Çağlar

    2011-03-01

    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.

  15. Occupational noise-induced hearing loss in Indian steel industry workers: an exploratory study.

    Science.gov (United States)

    Singh, Lakhwinder Pal; Bhardwaj, Arvind; Deepak, Kishore Kumar

    2013-04-01

    The present study focused on exploring the current level of hearing protection and subsequently determined the prevalence of occupational noise-induced hearing loss among casting and forging industry workers. The casting and forging industry provides employment to a significant portion of the population. The level of hearing protection was assessed through questionnaire survey of 572 workers. Out of these workers, 165 and another control group of 57 participants were assessed by formal audiometry. Audiometric tests were conducted at frequencies of 1.0 KHz to 8.0 KHz.The occurrence of hearing loss was determined on the basis of a hearing threshold level with a low fence of 25 dB. Student's test and ANOVA were used to compare the various groups; a p value steel industry are highly exposed to occupational noise. The majority of workers are not protected from noise-induced hearing loss. There is a need to provide special ear protectors for workers engaged in forging. A complete hearing protection program, including training, audiometry, job rotation, and the use of hearing protection devices, needs to be introduced.

  16. Conductive Hearing Loss during Infancy: Effects on Later Auditory Brain Stem Electrophysiology.

    Science.gov (United States)

    Gunnarson, Adele D.; Finitzo, Terese

    1991-01-01

    Long-term effects on auditory electrophysiology from early fluctuating hearing loss were studied in 27 children, aged 5 to 7 years, who had been evaluated originally in infancy. Findings suggested that early fluctuating hearing loss disrupts later auditory brain stem electrophysiology. (Author/DB)

  17. Medical surveillance of occupational Noise Induced Hearing Loss (NIHL) at RAPS hospital

    International Nuclear Information System (INIS)

    Manuwal, A.K.; Ganesan, S.

    2009-01-01

    Full text: One of the most challenging roles of a Certifying Surgeon is to act as catalyst for change. This paper is a presentation of the impact of such an effort. Noise is the insidious of all industrial pollutants, involving every industry and causing severe hearing loss. Exposure to excessive noise is the major avoidable cause of permanent hearing impairment. Noise - induced hearing loss (NIHL) is bilateral and symmetrical, usually affecting the higher frequencies 3 k, 4 k or 6 kHz ) and then spreading to the lower frequencies (0.5 k,1 k or 2 kHz). The major health effects are lack of concentration, irritation, fatigue, headache, sleep disturbances etc. Hearing protectors should be used when engineering controls and work practices are not feasible for reducing noise exposure to safe levels. Ear muffs, ear plugs and ear canal caps are the main types of hearing protectors. Awareness should be created among workers about the harmful effects of noise on hearing and other body systems by implementing compulsory education noise conservation programmes. The practice followed at RAPS Hospital for medical surveillance of Occupational Noise Induced Hearing Loss is being briefed

  18. The Relationship between Language Development and Behaviour Problems in Children with Hearing Loss

    Science.gov (United States)

    Stevenson, Jim; McCann, Donna; Watkin, Peter; Worsfold, Sarah; Kennedy, Colin

    2010-01-01

    Background: There are well-replicated findings that link poor development on a range of communication skills with increased behavioural problems. This paper examines this relationship in children with hearing loss. Method: One hundred and twenty children with hearing loss (67 boys, 53 girls) and 63 hearing children (37 boys, 26 girls) with a mean…

  19. Musical hallucination associated with hearing loss.

    Science.gov (United States)

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

    2011-01-01

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

  20. [Analysis on occupational noise-induced hearing loss of different type workers in underground mining].

    Science.gov (United States)

    Liu, Q C; Duo, C H; Wang, Z; Yan, K; Zhang, J; Xiong, W; Zhu, M

    2017-11-20

    Objective: To investigate hearing loss status of blasters, drillers mechanics and so on in underground mining, and put forward suggestion diagnosis of occupational explosive deafness and occupational deafness. Methods: Underground excavation workers in a metal mine were recruited in this study, those with a history of ear disease and non-occupational deafness were all excluded. Finally, the features of pure tone audiometry of 459 noise-exposed workers were analyzed. Results: High-frequency hearing loss occurred on 351workers and the positive detection rate was 74.29%, workers who had both high-frequency and linguistic frequency hearing loss were 51 and the positive detection rate was 11.11%. The positive detection of high-frequency hearing loss in right ear (χ(2)=9.427 and P = 0.024) and in left ear (χ(2)=14.375, P =0.002) was significantly different between different exposure age groups. The positive detection of high-frequency hearing loss of driving group was the highest, followed by blasting group, mining group and machine repair group. The characteristics of the hearing loss caused by drilling noise of the blasting workers with no accident occurred were in line with that of noise-induced hearing loss. Conclusion: The diagnosis grading should be carried out according to the diagnostic criteria of occupational noise-induced deafness for the employees who engaged in the blasting operation with no record of blast accident.

  1. An unusual cause of conductive hearing loss: bilateral complete meatal obstruction following otoplasty.

    Science.gov (United States)

    Toplu, Yuksel; Toplu, Sibel Altunisik; Sapmaz, Emrah; Deliktas, Hacim

    2014-01-01

    There are many causes of conductive hearing loss. Otoplasty is a commonly performed cosmetic surgery to correction for prominent ear. We discussed an unusual cause of conductive hearing loss, bilateral complete meatal obstruction following otoplasty, in this article. This complication was probably due to unsuitable placement of the Furnas sutures. In the literature, as a cause of conductive hearing loss, unilateral complete meatal obstruction has been described rarely, but bilateral complete obstruction has not been defined. Hearing loss recovered completely after revision surgery. Correct diagnosis, accurate preoperative or perioperative examination, choice of the proper technique and surgeon experience are essential for avoiding inadequate results or complications of otoplasty. The surgeon should be aware of the cartilage elasticity and suture techniques to avoid this complication.

  2. Children with unilateral hearing loss may have lower intelligence quotient scores: A meta-analysis.

    Science.gov (United States)

    Purcell, Patricia L; Shinn, Justin R; Davis, Greg E; Sie, Kathleen C Y

    2016-03-01

    In this meta-analysis, we reviewed observational studies investigating differences in intelligence quotient (IQ) scores of children with unilateral hearing loss compared to children with normal hearing. PubMed Medline, Cumulative Index to Nursing and Allied Health Literature, Embase, PsycINFO. A query identified all English-language studies related to pediatric unilateral hearing loss published between January 1980 and December 2014. Titles, abstracts, and articles were reviewed to identify observational studies reporting IQ scores. There were 261 unique titles, with 29 articles undergoing full review. Four articles were identified, which included 173 children with unilateral hearing loss and 202 children with normal hearing. Ages ranged from 6 to 18 years. Three studies were conducted in the United States and one in Mexico. All were of high quality. All studies reported full-scale IQ results; three reported verbal IQ results; and two reported performance IQ results. Children with unilateral hearing loss scored 6.3 points lower on full-scale IQ, 95% confidence interval (CI) [-9.1, -3.5], P value analysis suggests children with unilateral hearing loss have lower full-scale and performance IQ scores than children with normal hearing. There also may be disparity in verbal IQ scores. Laryngoscope, 126:746-754, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  3. Effects of sound amplification in self-perception of tinnitus and hearing loss in the elderly

    Directory of Open Access Journals (Sweden)

    Tiago de Melo Araujo

    Full Text Available ABSTRACT OBJECTIVE: To determine the effect of the use of hearing aids in self-perception of tinnitus and hearing loss in the elderly. METHODS: A total of 24 elderly patients between 60 and 70 years of age with moderate-grade sensorineural hearing loss were evaluated and divided into two groups according to the presence or absence of tinnitus. All volunteers were fitted with binaural micro-channel hearing aids of the same brand and model and submitted to tests, scales, and questionnaires relevant to this topic. The evaluations were performed before and after one and three months of effective use of these hearing aids. RESULTS: Acoustic stimulation through the effective use of hearing aids caused a reduction in the perception of tinnitus sound intensity (especially in evaluations with the prosthesis on and in nuisance associated with this symptom and with hearing loss. In addition, all participants were satisfied with the use of hearing aids. CONCLUSION: The continuous use of hearing aids is beneficial for the treatment of tinnitus and hearing loss, bringing satisfaction to users.

  4. Hearing loss in children with e-waste lead and cadmium exposure.

    Science.gov (United States)

    Liu, Yu; Huo, Xia; Xu, Long; Wei, Xiaoqin; Wu, Wengli; Wu, Xianguang; Xu, Xijin

    2018-05-15

    Environmental chemical exposure can cause neurotoxicity and has been recently linked to hearing loss in general population, but data are limited in early life exposure to lead (Pb) and cadmium (Cd) especially for children. We aimed to evaluate the association of their exposure with pediatric hearing ability. Blood Pb and urinary Cd were collected form 234 preschool children in 3-7years of age from an electronic waste (e-waste) recycling area and a reference area matched in Shantou of southern China. Pure-tone air conduction (PTA) was used to test child hearing thresholds at frequencies of 0.25, 0.5, 1, 2, 4 and 8kHz. A PTA≥25dB was defined as hearing loss. A higher median blood Pb level was found in the exposed group (4.94±0.20 vs 3.85±1.81μg/dL, phearing loss (28.8% vs 13.6%, phearing thresholds at average low and high frequency, and single frequency of 0.5, 1 and 2kHz were all increased in the exposed group. Positive correlations of child age and nail biting habit with Pb, and negative correlations of parent education level and child washing hands before dinner with Pb and Cd exposure were observed. Logistic regression analyses showed the adjusted OR of hearing loss for Pb exposure was 1.24 (95% CI: 1.029, 1.486). Our data suggest that early childhood exposure to Pb may be an important r