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 Paper NumberTitle of Paper Author Information Year Started-Year Ended/ Ongoing Cohort InformationFundingStudy SpecificationsNumber of Participants DemographiccharacteristicsHearing Loss Data HearingdataOther InvestigationsGenetic Test TypeName of Specific gene(s) studiedMedical Condition(s) in Genotyped DataYear of genetic analysis (if different from date of publication)Key Study Findings Relevant to Hearing Loss Any Potential Targets For Therapy Identified in the Study? Yes:1 No:2Please specify the identified potential targetrs for therapy if yesHave Patients Within The Cohort Given Consent To Be Contacted? Yes:1 No:2 Unkown: 0 Which I.T. System/Software Is Used To Manage The Database? (if specified)
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Names of AuthorsCorresponding Author ContactName of Cohort used (if any)Name of the paper's target disease/hearing typeCountry/Region of CohortCohort type/targetStudy Design Prospective:1 Retrospective:2 Registry:3 Other:4Please specify if other Study Type Basic Science: 1 Clinical:2Number of participants in the studyNumber of participants in the Cohort (if different)AgeAge range Gender Both: 1 Female: 2 Male:3 EthnicityHearing Loss Laterality Unilateral:1 Bilateral:2 Both: 3 Unspecified:0Underlying diagnosis Glue ear: 1 Cholesteatoma: 2 ARHL: 3 SSNHL: 4 NIHL: 5 Other:6 Unspecified: 0Please specify underlying disease if otherPTA Yes:1 No:2Speech in Noise Yes:1 No:2 ABR Yes:1 No:2OAEs Yes:1 No:2Tympanometry Yes:1 No:2Other hearing tests / Other commentsCognitive Function Assessments Yes:1 No:2Please specify cognitive function assessments used, if yesImaging Studies Yes:1 No:2Please specify imaging studies used, if yesSerum Biomarkers e.g. Prestin Yes:1 No:2Please specify serum biomarkers used, if yesElectrophysiological tests Yes:1 No:2Please specify electrophysiological tests used, if yesCardiovascular measures Yes:1 No:2Please specify cardiovascular measures used, if yesOther Other investigations / Other comments GenomeWide Association Study Yes:1 No:2Whole Exome Sequencing Yes:1 No:2Gene Panel Sequencing Yes:2 No:2Single Gene Sequencing Yes:1 No:2Other Test (Please specify)
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43Recent Epidemiology of Pediatric Cochlear Implantation in the United States: Disparity Among Children of Different Ethnicity and Socioeconomic StatusR E Stern, B Yueh, C Lewis, S Norton, K C Y Sierestern@u.washington.edu1997 1. Washington 1997 Health Care and Utilization Project/Kids’ Inpatient Database 2. 2000 Galludet Research Institution (GRI) national demographic studySNHL - undifferentiatedNorth AmericaSNHL - UndifferentiatedRetrospective: 2Clinical : 2124(database updated annually)0-18Both: 1White, Asian, Hispanic, BlackSNHL: 1White and Asian pediatric patients received cochlear implants at proportionally higher rates than blacks and Hispanic pediatric patients, even after adjustment for the prevalence rates of severe to profound SNHL in each racial and ethnic group.No:2Unknown: 0
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221Factors Associated With Hearing Impairment in Patients With Congenital Hypothyroidism Treated Since the Neonatal Period: A National Population-Based StudyL Lichtenberger-Geslin, S Dos Santos, Y Hassani, E Ecosse, T Van Den Abbeele, J Légerjuliane.leger@rdb.aphp.fr1. 1978 - 1988, 2. 20031. Cohort of congenital hypothyroidism, 2. Decennial Health Survey SNHL - GeneticFranceOther disease registries/cohortsFrench Ministry of Health (Programme Hospitalier de Recherche Clinique AOM 05011), Pfizer Foundation for Children and AdolescentsProspective: 1Clinical : 21,162184224.518-23,5++Both: 1Both:3Other:6 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1RRM2B Ophthalmoparesis, Ptosis, Proximal muscle weakness, Bulbar dysfunction, Ataxia, Fatigue, Sensorineural hearing loss, Endocrinopathy, Gastrointestinal disturbance, Cognitive impairment/learning difficulties, Encephalopathy/stroke-like episodes, Renal disturbance, Depression, Neuropathy, Pigmented retinopathy, Cardiac dysfunction/arrhythmia, Cataracts, Migraine, Distal muscle weakness, Glaucoma, Short statureHearing loss was associated with the type of congenital hypothyroidism, with disease severity, and with other associated chronic diseasesNo:2Unknown: 0
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251Adults with RRM2B-related mitochondrial disease have distinct clinical and molecular characteristicsRDS Pitceathly, C Smith,C Fratter,CL Alston,L He, K Craig, EL Blakely, JC Evans, J Taylor,Z Shabbir,M Deschauer,U Pohl, ME Roberts, MC Jackson,CA Halfpenny, PD Turnpenny, PW Lunt, MG Hanna, AM Schaefer,R McFarland,R Horvath, PF Chinnery, DM Turnbull, J Poulton, RW Taylor and GS Gormangrainne.gorman@ncl.ac.ukDates unspecified1. Newcastle–Oxford–Halle, 2. Additional - systematic review of all previously published cases of RRM2B mutations (2012)SNHL - GeneticUKOther disease registries/cohortsMRC Centre for
Translational Research in Neuromuscular Disease Mitochondrial
Disease Patient Cohort (UK) (G0800674); Wellcome Trust
Centre for Mitochondrial Research (906919); MRC Centre for
Neuromuscular Diseases (G0601943); Newcastle University
Centre for Brain Ageing and Vitality supported by BBSRC,
EPSRC, ESRC and MRC (G0700718); Wellcome Trust
Programme Grant (074454/Z/04/Z); UK NIHR Biomedical
Research Centre for Ageing and Age-related disease award to
the Newcastle upon Tyne Foundation Hospitals NHS Trust.		Prospective: 1Basic Science: 126mean 40 yrs0-70Both: 1Unspecified:0SSNHL: 4 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1CIAS1Cryopyrin-associated periodic syndromesMutations in the nuclear-encoded mitochondrial maintenance gene RRM2B are associated with sensorineural hearing loss No:2Unknown: 0
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271Cryopyrin-Associated Periodic
Syndromes: Otolaryngologic and Audiologic Manifestations		Neda Ahmadi, Carmen C. Brewer, ,
Christopher Zalewski, Kelly A. King,
John A. Butman, Nicole Plass,
Cailin Henderson, Raphaela Goldbach-Mansky, H. Jeffrey Kim		H. Jeffrey Kim, MD, Department of Otolaryngology–HNS, Gorman 1, 3800
Reservoir Rd, NW, Washington, DC 20007, USA hk7@gunet.georgetown.edu		 2003 - 20091. NOMID/anakinra (03-AR0298), a natural history study (03-AR-0173), 2. FCAS/interleukin-1 Trap (05-AR-0014).Cryopyrin-associated periodic syndromes (CAPS)Bethesda,
Maryland, US		Other disease registries/cohortssponsored by the intramural research
programs of the National Institute of Arthritis and Musculoskeletal
and Skin Diseases and the National Institute on Deafness and Other
Communication Disorders at the NIH.		Prospective: 1Clinical : 2571-69Both: 1Unspecified:0Other:6 CHL, SNHL, MHLYes:1Yes:1Acoustic reflexYes:1 Inner Ear and Brain FLAIR-MRINo:2Lumbar puncture for patients with CNS symptomsHearing loss, especially sensorineural, is one of the most common phenotypic features in CAPS, and it can be progressive. Treatment of
CAPS with IL-1 antagonists such as anakinra has been promising, and further studies are needed to determine the effectivenessof IL-1 antagonists in preventing, stabilizing, or even reversing cochlear damage and HL		Yes:1IL-1 antagonists for patients wirt CAPS and hearing lossUnknown: 0
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183A Prospective Investigation of Dietary Intake and Functional Impairments Among the ElderlyJ Zhu, Y-B Xiang, H Cai, H Li, Y-T Gao, W Zheng and X-O Shuxiao-ou.shu@vanderbilt.edu1. 2012-2015 2. 1996-20151. Shanghai Men’s Health Study 2. Shanghai Women’s Health StudySNHL - ARHLChinaPopulation cohortThis work was supported by the US National Institutes of Health (grants UM1 CA182910 and UM1 CA173640).Prospective: 1Clinical : 274,94118,458>70Male: 3ChineseUnspecified:0No:2No:2No:2No:2No:2Outcomes for hearing/vision, memory, and decision-making ability were defined
as having no, minor, or serious impairments in functional status		Yes:1Self reported memory and decision-making ability outcomesNo:2No:2No:2No:2Dietary IntakeThis study indicates that dietary intake
plays an important role in the development of both physical and mental functional impairments among elderly Chinese. Hearing and vision were measured simultaneously in a single question, so that inferences cannot be made for each of them separately		Unknown: 0
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189Associations of obesity and weight change with physical and mental impairments in elderly Chinese peopleJ Zhu, Y-B Xiang, H Cai, H Li, Y-T Gao, W Zheng and X-O Shuxiao-ou.shu@vanderbilt.edu1. 2012-2015 2. 1996-20151. Shanghai Men’s Health Study 2. Shanghai Women’s Health StudySNHL - undifferentiatedChinaPopulation cohortThis work was supported by the National Institutes of Health [UM1 CA173640 and UM1 CA182910]. The study sponsors had no role in the design, methods, subject recruitment, data collection, analysis, or preparation of this paper.Prospective: 1Clinical : 274,94118,458>70Male: 3ChineseUnspecified:0No:2No:2No:2No:2No:2Outcomes for hearing/vision, memory, and decision-making ability were defined
as having no, minor, or serious impairments in functional status		Yes:1Self reported memory and decision-making ability outcomesNo:2No:2No:2No:2Dietary Intake, height, weight, and circumferences of waist and hipBeing underweight at middle age (OR = 0.86, 95% CI: 0.76, 0.97) was inversely associated with having minor problems in hearing/vision. Conversely, being underweight (BMI < 18.5) after age 70 (OR = 1.53,
95% CI: 1.29, 1.82) was positively associated with decreased function in hearing or vision. Weight gain from 20 years old to age at baseline was inversely associated with serious impairments in hearing or vision		No:2Unknown: 0
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246Targeted surveillance for postnatal hearing loss: A program evaluationR Beswick, C Driscoll, J Kei, S GlennonRachael_Beswick@health.qld.gov.auSeptember 2004 - December 20091. UNHS program, Healthy Hearing, 2. Queensland’s targeted surveillance programSNHL - undifferentiatedAustraliaALL - Undifferentiated1) School of Health and Rehabilitation Sciences, The University of Queensland, Brisbane, Queensland, Australia
2) Clinical and State-wide Services, Queensland Health, Queensland Government, Queensland, Australia		Prospective: 1Clinical : 27,32056around 34 weeksBoth: 1Both:3sensorineural, mixed, conductive, auditory neuropathyYes:1No:2Yes:1No:2No:2No:2No:2No:2No:2No:2Positive cases of postnatal hearing loss were detected through the targeted surveillance
program.		Yes:1For targeted surveillance to continue, time frames for assessment, assessments performed, and discharge
criteria need to be revisited.		Unknown: 0
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192Dichotic Digits Test Performance Across the Ages: Results From Two Large Epidemiologic Cohort StudiesME Fischer, KJ Cruickshanks, DM Nindahl, BEK Klein, R Klein, JS Pankow,TS Tweed, DS Dalton and AJ Paulsenfischer@episense.wisc.edu1. 1993 - 2010 2.2005-20081.Beaver Dam Epidemiology of Hearing Loss Study 2.Beaver Dam Offspring Study (BOSS)All - undifferentiatedNorth AmericaHearing loss populationThe project described was supported by Award Numbers R37AG011099 and R01AG021917 from the National
Institute on Aging (to K.J. Cruickshanks), Award Number EY06594 from the National Eye Institute (to R. Klein
and B.E.K. Klein), and by unrestricted funds from Research to Prevent Blindness (RPB).		Prospective: 1Clinical : 236551391Both: 1Unspecified:0ARHL: 3 Yes:1Dichotic Digits TestsYes:1The Mini-Mental State Examination (MMSE)Substantial variation in the total free recall DDT scores but very little variation in
the right ear directed recall DDT scores was observed. Age, sex, education, hearing loss severity,
and cognitive impairment were found to be significantly related to DDT scores but explained less
than 25% of the total variability in total free recall scores. The right ear directed recall DDT by
itself may not be of benefit in assessing central auditory processing in a general population 		No:2Unknown: 0
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281The Prevalence of Hearing Impairment
and Associated Risk Factors		SD Nash, KJ Cruickshanks, R Klein, BEK Klein, FJ Nieto, GH Huang, JS Pankow and TS Tweedsnash@wisc.edu2005 to 20081.Beaver Dam Epidemiology of Hearing Loss Study 2.Beaver Dam Offspring Study (BOSS)All - undifferentiatedUnited StatesHearing loss populationThis study was supported in part by R01AG021917 from the National Institute on Aging, the National Eye Institute, and the National Institute on Deafness and Other Communication Disorders.Prospective: 1Clinical : 2328521-84Both: 1Both:3Unspecified:0Yes:1Yes:1Yes:1Yes:1FundoscopyYes:1Cholesterol, HDL, WBC, HematocritYes:1Systolic and Diastolic Blood PressureHearing impairment is a common condition in middle-aged adults. Cardiovascular disease risk factors may be important correlates of age-related auditory dysfunctionNo:2Unknown: 0
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14Dietary Intake of Cholesterol Is Positively Associated and Use of Cholesterol-Lowering Medication Is Negatively Associated with Prevalent Age-Related Hearing LossB. Gopinath, V. Flood, E. Teber, C. McMahon, P. Mitchellpaul_mitchell@wmi. usyd.edu.au.1997 - 20041.The Blue Mountains Hearing Study (BMHS) 2. Blue Mountains Eye Study (BMES)SNHL - ARHLAustraliaHearing loss populationThe BMES was supported by the Australian National Health and Medical Research Council (grants no. 974159, 991407, 211069, and 262120).Registry: 3Clinical : 2295667Both: 1Unspecified:0SNHL: 1Yes:1Yes:1TG, Serum cholesterol, Dietary cholesterolExposure to noisy workplace, Educationa qualifications, History of diabetes, History of diagnosed strokeA diet high in cholesterol could have adverse influences on hearing, whereas treatment with statins and consumption of monounsaturated fats may have a beneficial influenceNo:2Unknown: 0
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18The Contribution of Family History to Hearing Loss in an Older PopulationC.McMahon, A. Kifley, E. Rochtchina, P. Newall, P.Mitchell paul_mitchell@wmi.usyd.edu.au1992 - 20001.The Blue Mountains Hearing Study (BMHS) 2. Blue Mountains Eye Study (BMES)SNHL - GeneticAustraliaHearing loss populationThe BMES was supported by the Australian National Health and Medical Research Council (grants no. 974159, 991407, 211069, and 262120).Registry: 3Clinical : 226693654 (BMHS 1)67.8 (without FH), 65.9 (with FH). Both: 1Unspecified:0ARHL: 3 Yes:1Questionnaire: family history of hearing loss, reported noise exposure at work, history of diabetes, history of stroke, socio-economic statusfamily history was most strongly associated with moderate to se- vere age-related hearing loss.Unknown: 0
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274Five-Year Incidence and Progression of Hearing
Impairment in an Older Population		Paul Mitchell, Bamini Gopinath, Jie Jin Wang, Catherine M. McMahon, Julie Schneider, Elena Rochtchina, and Stephen R. LeederPaul Mitchell, MD, PhD, Centre for Vision
Research, University of Sydney, Westmead Hospital, Hawkesbury Road, Westmead, NSW 2145, Australia. paul_mitchell@wmi.usyd.edu.au.		1997–19991.The Blue Mountains Hearing Study (BMHS) 2. Blue Mountains Eye Study (BMES)SNHL - ARHLAustraliaHearing loss populationThe BMES was supported by the Australian National Health and Medical Research Council (grants no. 974159, 991407, 211069, and 262120).Registry: 3Clinical : 21309BMES: 3654 BMHS:2956Male: 3Unspecified:0ARHL: 3 No:2No:2No:2No:2No:2Hearing Handicap Inventory
for the Elderly-Shortened version		No:2No:2Yes:1Blood glucose, HgNo:2Yes:1Blood pressureBilateral hearing impairment was an extremely
common disability in these older community-dwelling
persons. The high frequency of hearing loss in this older
population indicates the need to implement programs to reduce or eliminate preventable hearing loss and points to a major public health need		No:2Unknown: 0
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289The Association Between Reduced GFR and Hearing Loss: A Cross-sectional Population-Based StudyE Vilayur, B Gopinath, DC Harris, G Burlutsky, CM McMahon and P Mitchelpaul_mitchell@wmi.usyd.edu.au1992-20041.The Blue Mountains Hearing Study (BMHS) 2. Blue Mountains Eye Study (BMES)SNHL - undifferentiatedAustraliaHearing loss populationThe BMES was supported by the Australian National Health and Medical Research Council (grants no. 974159, 991407, 211069, and 262120).Prospective: 1Clinical : 22334>49Both: 1Bilateral:2Unspecified:0Yes:1Yes:1Yes:1Yes:1Serum creatinine, GlucoseYes:1Systolic and Diastolic Blood PressureBMI, Smoking status, socioeconomic characteristicsModerate CKD per se was associated independently with hearing loss. Recognizing this link could lead to earlier hearing assessment with appropriate interventions to preserve the hearing of patients with CKDNo:2Unknown: 0
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291Association Between Age-Related Hearing Loss and Stroke in an Older PopulationB Gopinath, J Schneider, E Rochtchina, SR Leeder and P Mitchelpaul_mitchell@wmi.usyd.edu.au1992 - 20041.The Blue Mountains Hearing Study (BMHS) 2. Blue Mountains Eye Study (BMES)SNHL - ARHLAustraliaHearing loss populationThe BMES was supported by the Australian National Health and Medical Research Council (grants no. 974159, 991407, 211069, and 262120).Prospective: 1Clinical : 22334>49Both: 1Unspecified:0ARHL: 3 Yes:1Yes:1CT, MRIBMI, Smoking status, socioeconomic characteristicsobserved a strong cross-sectional association between stroke and moderate to severe hearing loss. However, age-related hearing loss did not increase risk of incident stroke in our cohort. Insufficient study power or differing underlying pathologies of sudden sensorineural hearing loss and typical age-related hearing loss may account for the discrepant findings between these studies.No:2Unknown: 0
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297Dietary Glycemic Load Is a Predictor of Age-Related Hearing Loss in Older AdultsB Gopinath, VM Flood, CM McMahon, G Burlutsky, J Brand-Miller and P Mitchelpaul_mitchell@wmi.usyd.edu.au1992 - 20041.The Blue Mountains Hearing Study (BMHS) 2. Blue Mountains Eye Study (BMES)SNHL - ARHLAustraliaHearing loss populationThe BMES was supported by the Australian National Health and Medical Research Council (grants no. 974159, 991407, 211069, and 262120).Prospective: 1Clinical : 22956>49Both: 1Bilateral:2ARHL: 3 Yes:1Yes:1GlucoseDietary dataData suggest that high postprandial glycemia might be a potential underlying biological mechanism for age-related hearing loss.No:2Unknown: 0
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324Hearing Impairment and Health-Related Quality of Life: The Blue Mountains Hearing StudyEM Chia, JJ Wang, E Rochtchina, RR Cumming, P Newall, P Mitchellpaul_mitchell@wmi.usyd.edu.au1992-20041.The Blue Mountains Hearing Study (BMHS) 2. Blue Mountains Eye Study (BMES)SNHL - ARHLAustraliaHearing loss populationThe BMES was supported by the Australian National Health and Medical Research Council (grants no. 974159, 991407, 211069, and 262120).Prospective: 1Clinical : 2295636546750 to above 80Both: 1Both:3ARHL: 3 Yes:1No:2No:2No:2No:2Air and Bone conduction PTA + self-reported hearing lossNo:2No:2No:2No:2No:2SF-36 questionnairePersons with self-reported hearing loss had significantly poorer HRQOL (Health-Related Quality Of Life) than corresponding persons without, but persons with unilateral or high-frequency hearing loss did not have significantly different HRQOL scores than their corresponding counterparts.No:2Unknown: 0
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325Mitochondrial DNA Haplogroups and Age-Related Hearing LossN Manwaring, MM Jones, JJ Wang, E Rochtchina, C Howard, P Newall, P Mitchell, CM Suecsue@med.usyd.edu.au1992-20041.The Blue Mountains Hearing Study (BMHS) 2. Blue Mountains Eye Study (BMES)SNHL - GeneticAustraliaHearing loss populationThe BMES was supported by the Australian National Health and Medical Research Council (grants no. 974159, 991407, 211069, and 262120).Prospective: 1Clinical : 22765365450 to above 80Both: 1Bilateral:2ARHL: 3 Yes:1No:2No:2No:2No:2Air and Bone conduction PTA + self-reported hearing lossNo:2No:2No:2No:2No:2No:2No:2No:2No:2Mitocondrial DNA haplogroupsFindings from this older Australian population demonstrate an association between certain mtDNA haplogroups and ARHL, as well as a link to the susceptibility of other known risk factors for ARHLNo:2Unknown: 0
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179Neuropsychological Test Performance of Cognitively Healthy Centenarians: Normative Data From the Dutch 100-Plus StudyNina Beker, Sietske A.M. Sikkes, Marc Hulsman, Ben Schmand, Philip Scheltens, Henne HolstegeHenne Holstege, PhD, Alzheimer Center
Amsterdam, Department of Neurology, Amsterdam UMC, PO Box 7057,
1007 MB Amsterdam, The Netherlands. h.holstege@vumc.nl		Unspecified100-plus StudySNHL - undifferentiatedNetherlandsPopulation cohortStichting Alzheimer Nederland (WE09.2014-03), Stichting
Dioraphte (VSM 14 04 1402), and Stichting VUmc Fond		Other: 4case-controlClinical : 2235306>100Both: 1Unspecified:0Unspecified:0No:2No:2No:2No:2No:2No hearing test was done in this study. Hearing was categorized into “good,”
“moderate,” “poor,” and “very poor” based on the observations
of the study researcher and the self-reported rating of
hearing and vision abilities		Yes:1Mini-Mental State Examination (MMSE), Dutch Adult Reading Test(DART), The Digit Span, Trail Making Tests A and B (TMT A, TMT B) Controlled Oral Word Association Test, Category fluency, Behavioral Assessment of the Dysexecutive Syndrome Test Battery29 , Rivermead Behavioral Memory Test (RBMT), Visual Association Test (VAT), Number Location subtest of
the Visual Object and Space Perception Battery, Clock Drawing Test (CDT) 		No:2No:2No:2No:2Most of the centenarians retained
moderate-good vision (77%) and hearing capacities (88%). Difficulties with vision
(41%) and fatigue (22%) were the most common reasons
for not being able to complete a test, whereas hearing
impairment only rarely complicated test completion (4%). Vision
impairments and fatigue complicated test completion, while
hearing impairments or task incomprehension rarely did.		No:2Unknown: 0OpenClinica
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17A large cohort study of GJB2 mutations in Japanese hearing loss patientsK. Tsukada, S. Nishio, S. Usami, and the Deafness Gene Study Consortium.usami@shinshu-u.ac.jpUnspecified1000 Japanese hearing loss familiesSNHL - GeneticJapanSNHL - Genetic/HereditaryMinistry of Health and Welfare, Japan (S.U.), and a Grant-in-Aid for Scientific Research from the Ministry of Education, Science and Culture of Japan (S.U.).Prospective: 1Basic Science: 13056Bilateral:2SNHL: 1Yes:1Yes:1CT temporal bonesNo:2Yes:1No:2No:2GJB2Unknown: 0
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3341958 National Child Development StudyPrincipal Investigator: Alissa Goodmanarchive@essex.ac.uk1958-1958 National Child Development StudyEngland, Scotland and WalesPopulation cohort Economic and Social Research Council., Medical Research Council, the US National Institutes of Health and the Department for Work and PensionsOther: 4CohortClinical : 218558All agesBoth: 1Unspecified:0Unspecified:0Yes:1No:2No:2No:2No:2Self reported hearing assessmentYes:1Childhood cognitive development testsNo:2Yes:1glycosylated haemoglobin, total and high density lipoprotein (HDL)
cholesterol, triglycerides, fibrinogen, C-reactive protein, tissue plasminogen activator (t-PA), von
Willebrand factor, total immunoglobulin E (IgE)		No:2Yes:1BP, HRAntropometric measures, Spirometry, visual acuity   
Immortalised cell lines were created and DNA extracted and stored Birth CohortUnknown: 0
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313Receptive (aural) vocabulary development in children with permanent bilateral sensorineural hearing impairmentCKIESE-HIMMELProfessor Dr rer nat Dipl-Psych Christiane Kiese-Himmel, Clinical Psychologist, E-mail: ckiese@med.uni-goettingen.de1994 - (unspecified date)1994 German Goettinger Hearing Language RegisterSNHL - undifferentiatedSNHL - UndifferentiatedGrant from the Annelie-Frohn StiftungProspective: 1Clinical : 233Mean age at first testing point 56.2monthsBoth: 1Bilateral:2SNHL: 1Yes:1No:2Yes:1No:2Yes:1Yes:1Vocabulary assesmentNo:2No:2No:2No:2If permanent childhoodhearing impairment ismild and/orisdetected early, andif thechild grows up in a monolingual environment, the development of receptive vocabulary within the normal range is possible. No:2Unknown: 0
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300Executive Dysfunction and Presbycusis in Older Persons With and Without Memory Loss and DementiaGA. Gates, LE Gibbons, SM McCurry, PK Crane, MP Feeney and EB LarsonGeorge A. Gates, MD, Virginia Merrill Bloedel Hearing Research Center, University of Washington, Box 357923, CHDD Bldg. RM CD176, Seattle, WA 98195-7923 (e-mail: ggates@ u.washington.edu)1994 - (unspecified)Adult Changes in Thought surveillance cohortSNHL - ARHLUSAOther disease registries/cohortsProspective: 1Clinical : 2313Men 80 (+/- 5.6), women 79 (+/- 5.2)Both: 1Both:3ARHL: 3 Yes:1Yes:1Yes:1Yes:1No:2Dichotic Sentence Identification test (DSI), Dichotic Digits test (DDT), MLR, LLRYes:1Trail Making (parts A and B); Clock Drawing, Stroop Color, and Word test; and subtests from the CASI measuring mental concentration (digits backwards, serial 3s) and category fluency.No:2No:2No:2No:2Amplification of sound may be insufficient, because hearing aids do not resolve deficits in cognition or executive functioning. Our results suggest that elderly patients with substantial central auditory dysfunction should be referred for neurologic evaluation and neuropsychologic assessment.No:2Unknown: 0
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352African American Cardiovascular Study CohortPoint of contact: Cheryl Nelsonnelsonc@nhlbi.nih.gov2000-2012African American Cardiovascular Study CohortJackson (US)Population cohortNational Institutes of HealthOther: 4CohortClinical : 2530121-94Unspecified:0Unspecified:0Yes:1No:2No:2No:2Yes:1Audiometry, TympanometryNo:2Yes:1blood and urine samplesYes:1BP,HRDemographic and health information was collected during a series of home interviews and clinical examinations, medication use, cigarette smoking, and history of stroke)Unknown: 0
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108No Association Between Apolipoprotein E or N-Acetyltransferase 2 Gene Polymorphisms and Age-Related Hearing Loss
Piers Dawes, PhD; Hazel Platt, MSc; Michael Horan, MA, MB ChB, PhD; William Ollier, PhD, FRCPath; Kevin Munro, PhD; Neil Pendleton, MB ChB, MRCP, FRCP; Antony Payton, PhDAntony Payton, Centre for Integrated Genomic Medical Research, Stopford Building, University of Manchester, Oxford Road, Manchester, M13 9PT, UK. E-mail: tony.payton@manchester.ac.uk1997-2002age-related hearing loss in a cohort of 265 Caucasian elderly volunteers from Greater Manchester, United KingdomSNHL - GeneticGreater Manchester, UKSNHL - ARHLBiotechnology and Biological Sciences Research Council (BB/F022441/1)Prospective: 1Clinical : 226559-88Both: 1CaucasianBilateral:2ARHL: 3 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1No:2No:2No:2NAT2, APOE1998 and 2000no evidence that the NAT2 and APOE genes are involved in ARHL using a replication population of 265 elderly volunteersNo:2Unknown: 0
27
114Sex-specific predictors of hearing-aid use in older persons: The age, gene/environment susceptibility - Reykjavik studyDE Fisher, C Li, HJ Hoffman, MS Chiu, CL Themann, H Petersen, PV Jonsson, H Jonsson, F Jonasson, JE Sverrisdottir, LJ Launer, G Eiriksdottir, V Gudnason, MF Cotchdiana.fi sher@nih.gov2002-2006AGES-RSHearing aid useIcelandPopulation cohortIntramural Research Programs of the National Institute of Aging ZIAAG007380) and the National Eye Institute (ZIAEY000401), and the National Institute on Deafness and Other Communication Disorders (IAA Y2-C-1004-02), National Institutes of Health (N01-AG-12100), Bethesda, Maryland, USA; the Icelandic Heart Association, Kopavogur, Iceland; the Icelandic Parliament, Reykjavik, Iceland; the University of Iceland Research Fund, Reykjavik, Iceland; and the Helga Jonsdottir and Sigurlidi Kristjansson Research Fund, Reykjavik, Iceland.Prospective: 1Clinical : 25,764517267 years and olderBoth: 1Both:3ARHL: 3 Yes:1No:2No:2No:2No:2No:2Yes:1review of a series of cognitive examinations and classified as normal, mild cognitive impairment (MCI), or demented by a consensus panel using the Diagnostic and Statistical Manual of Mental Disorders , Fourth Edition (DSM-IV) guidelines No:2No:2No:2Yes:1Blood pressuredepressive symptomology, BMI, Visual acuity, Physical activity level, DiabetesHearing-aid use was comparable in Icelandic men and women with moderate or greater hearing loss. Self-recognition of hearing loss was the factor most predictive of hearing-aid useNo:2Unknown: 0
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355American Community Survey 2012 - 20162012-2016American Community Survey 2012 - 2016USPopulation cohortU.S. Census BureauOther: 4CohortClinical : 23.5 million households/yearall agesUnspecified:0Unspecified:0No:2No:2No:2No:2No:2Self reported hearing assessmentNo:2Social ( Education, Marital Status, Relationships, Fertility, Grandparents)
Economic ( Income, Employment, Occupation, Commuting to Work)
Housing(Occupancy and Structure, Housing Value and Costs, Utilities)
Demographic Characteristics ( Sex and Age, Race, Hispanic Origin, Housing Units)		Unknown: 0
29
4Genome-wide association analysis demonstrates the highly polygenic character of age-related hearing impairmentE. Fransen, S. Bonneux, J. Corneveaux, I. Schrauwen, F. Di Berardino, C. White, J.Ohmen, P. Van de Heyning, U. Ambrosetti, M. Huentelman, G. Van Camp, R. Friedmanguy.vancamp@uantwerpen.beUnspecifiedAntwerp University Hospital SNHL - ARHLBelgiumSNHL - ARHLBelgian Science Policy Office Interuniversity Attraction Poles (BELSPO-IAP) programme through the project IAP P7/43-BeMGI, through R01 grant DC010215, the Seaver Foundation, the Schwartz Foundation, and the State of Arizona.Prospective: 1Basic Science: 12161Unspecified:0ARHL: 3 Yes:1Yes:1No:2No:2No:2Age related hearing lossA study of the genetic architecture indicates for the first time that ARHI is highly polygenic in nature, with probably no major genes involved. No:2Unknown: 0
30
305Distribution of Hearing Loss Characteristics in a Clinical Population RH Margolis and GL SalyRobert H. Margolis, PhD, University of Minnesota, Department of Otolaryngology, MMC283, Minneapolis, MN 55455. E-mail: margo001@umn.edu. 1988 –1989archived records
of the University of Minnesota Hospital Audiology
Clinic		All - undifferentiatedMinnesota, USAHealthcare databaseRegistry: 3Clinical : 216,818all agesBoth: 1Both:3Unspecified:0Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2or lack of a validated classification of audiometric characteristics, it has not been possible to characterize the distributions of hearing loss types in various populations. AMCLASS provides
that capability. Although distributions of hearing loss characteristics are likely to differ in various clinical settings, this analysis provides prevalence rates of hearing loss configurations, severities, and sites of lesion from a large clinical database against which analyses of other databases can be compared.		No:2Unknown: 0
31
353Atherosclerosis Risk in communities Study (ARIC)PI: David CouperDavid_Couper@unc.edu1987Atherosclerosis Risk in communities Study (ARIC)USA (Washington County, Forsyth County, Jackson, Minneapolis)Population cohortNational Heart, Lung, and Blood Institute of the National Institutes of HealthOther: 4CohortClinical : 215,792 45-64Unspecified:0Unspecified:0Yes:1No:2No:2No:2No:2PTANo:2Yes:1Cholesterol
HDL Cholesterol
Triglycerides
Fibrinogen
Factor VIIc 		medical, social, and demographic dataDNA samples were stored for further multiple analysesAtherosclerosisUnknown: 0
32
73Risk Factors for Hearing Disorders: Epidemiologic Evidence of Change over Time in the UKA Davis, S Wodd, Ros Healy, H Webb and S RoweAdrian Davis, MRC Institute of
Hearing Research, Nottingham, UK NG7 2RD		1983-1988Audio case file from Nottingham, Oxford and SheffieldAll - undifferentiatedUnited KingdomALL - UndifferentiatedRetrospective: 2Clinical : 2200Both: 1Bilateral:2Other:6 SNHL: 1, MHL:3virologic tests(1) that the NICU graduates should be a major target for neonatal screening using ABR
or click evoked otoacoustic emissions methods, (2) that better understanding of the etiologies of the NICU hearing-impaired children should
eventually lead to a reduction in complication rates for premature babies and those ill at birth, and (3) that the family history group should be effectively targeted on maternity wards and offered neonatal screening as a priority. Finally, all children with meningitis should have a diagnostic assessment of hearing at a reasonable interval post-discharge.		No:2Unknown: 0
33
171Characteristics and Progression of Hearing Loss in Children with
Down Syndrome		Kathryn L. Kreicher, Forest W. Weir, Shaun A. Nguyen, Ted A. MeyerKathryn L. Kreicher. Department of Otolaryngology–Head and Neck
Surgery, Medical University of South Carolina 135 Rutledge
Ave, MSC550, Charleston, SC 29425. kreicher@musc.edu		2016 - unspecifiedAudiological and Genetic Database (AudGenDB)SNHL - GeneticUSAHearing loss populationThe AudGenDB is funded by the National Institutes on Deafness and Other Communication DisordersRegistry: 3Clinical : 21318105 000mean age 7.1Both: 1White, Black or African American, Hispanic, Asian, OtherBoth:3Glue ear: 1, Cholesteatoma: 2 , Other:6 Mixed HL , Pure Conductive HL and SNHLYes:1No:2No:2Yes:1Yes:1CT, MRIChildren with Down syndrome who have bilateral, mixed hearing loss or a history of seizures are at
risk for more severe hearing loss. SNHL and mixed hearing loss should not be overlooked in patients with CHL.
All patients with Down syndrome will benefit from serial audiograms, especially those children with SNHL or mixed
hearing loss, which is likely to worsen over time		No:2Unknown: 0
34
83Prevelance of 2.2 per mile of significant hearing loss at school age suggests rescreening after NHSDaniel Holzinger, Annette Weishaupt, Paul Fellinger, Christoph Beitel, Johannes FellingerD Holzinger: Hospital St. John of God, Institute for Senses and Language, Seilerst€atte 2, 4021, Linz, Austria. daniel.holzinger@bblinz.at Spring 2009 - 2011Austrian school-age children born between 1997 and 2001SNHL - undifferentiatedAustria, School age children living in federal state of Carinthia, AustriaALL - UndifferentiatedRetrospective: 2Clinical : 26110.57.5 - 13.6 (born between 1997 - 2001)Both: 1Not statedBilateral:2SNHL: 1Yes:1Yes:1No:2No:2No:2No:2Yes:1Kaufmann Assessment Battery for children (54) or the Vineland Screener No:2No:2No:2No:2High prevelence of school-age children with significant hearing loss compared the NHS emphasises the need for ongoing awareness of late-onset hearing lossNo:2Unknown: 0
35
33Ten-year outcome of newborn hearing screening in AustriaViktor Weichbold, Doris Nekahm-Heis, Kunigunde Welzl-MuellerV. Weichbold Tel.: +43 512 504 25438; fax: +43 512 504 23217. viktor.weichbold@uklibk.ac.at1990-2003Austrian universal neonatal hearing screeningAll - undifferentiatedAustriaALL - UndifferentiatedRetrospective: 2clinical : 23210-60 monthsboth: 1unspecified:0other:6 Congenital sensorineural hearing lossNo:2No:2No:2yes:1No:2No:2No:2No:2No:2No:2The success of neonatal hearing screening programs depends on factors subsequent to the detection of congenital hearing impairment through screening: tightly organized follow-up, good parental compliance, and sound training of all
involved health care professionals.		No:2Unknown: 0
36
112Does COPD have a clinically relevant impact on hearing loss? A retrospective matched cohort study with selection of patients diagnosed with COPDGustav Kamenski, Jana Bendova, Waltraud Fink, Andreas Sönnichsen, Wolfgang Spiegel, Sonja ZehetmayerDr Gustav Kamenski; kamenski@aon.at
Not specifiedAutrian 194 Adults with COPDAll - undifferentiatedLower AustriaOther disease registries/cohortsThis research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors. Retrospective: 2Clinical : 29735+Both: 1Not statedBilateral:2Unspecified: 0Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2spirometry does not support the hypothesisthat there is an association between COPD andhearing impairment No:2Unknown: 0
37
267Prevalence and risk factors for mild and highfrequency
bilateral sensorineural hearing loss at age 11 years old: A UK prospective cohort study		Amanda J. Hall, Elizabeth Midgley, Colin Steer, Rachel HumphrissAmanda J. Hall, Centre for Hearing and Balance Studies, 8 Woodland Road, Bristol, BS8 1TN, UK amanda.hall@bristol.ac.uk1991 - 2002Avon Longitudinal Study of Parents And Children (ALSPAC)SNHL - undifferentiatedAvon, UKBirth CohortThe UK Medical Research Council (Grant ref: 74882), the Wellcome
Trust (Grant ref: 076467), and the University of Bristol provide core
support for ALSPAC.		Prospective: 1Clinical : 2503214 0007, 9, and 11Both: 1White, non-whiteUnspecified:0Unspecified:0Yes:1No:2No:2No:2No:2No:2The prevalence of mild and high-frequency bilateral sensorineural hearing loss in the ALSPAC cohort study at age 11 years old was 0.5%. This is lower than prevalence estimates from population studies in the USA. There is some evidence that admission to hospital in the first two years of life may be associated with mild and high-frequency hearing loss, and that parents of these children are more likely to have suspected a hearing problem at age 3 years.No:2Unknown: 0
38
31Mitochondrial 12S rRNA gene mutations affect RNA secondary structure and lead to variable penetrance in hearing impairmentEster Ballana, Estela Morales, Raquel Rabionet, Ba`rbara Montserrat, Marina Ventayol, Olga Bravo, Paolo Gasparini, Xavier EstivillX. Estivill Fax: +34 932240899.
E-mail address: xavier.estivill@crg.es 		UnspecifiedBarcelona 443 families with hearing impairment,SNHL - GeneticSpainSNHL - Genetic/Hereditary‘‘Fundacio´ La
Marato´ de TV3’’ (993610) and ‘‘Instituto de Salud Carlos
III,’’ FIS-ISCIII (G03/203). E.B. is the recipient of a FI fellowship
from ‘‘Departament d’Universitats i Societat de la
Informacio´ ,’’ Generalitat de Catalunya (2003FI00066)		Other: 4case-controlclinical : 2443 familiesUnspecified:0Other:6 Non syndromic SNHLYes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1No:212S rRNA gene, GJB2, GJB6hearing lossThe reported data confirm the high prevalence of mutation A1555G in deafness cases and the major role of
the 12S rRNA gene in hearing. The two novel changes reported here might have different contributions as deafness-related variants.
T1291C fulfills the criteria of a disease-causing change. As in the case of mutation A1555G, the underlying phenotype of T1291C is not homogeneous for all family members, providing evidence for the implication of environmental and/or additional genetic factors		No:2Unknown: 0
39
70Is Age-Related Maculopathy Related to Hearing Loss?R Klein, K J Cruickshanks, B E K Klein, D M Nondahl and T Wileykleinr@epi.ophth.wisc.edu1993-1995Beaver Dam Epidemiology of Hearing Loss StudySNHL - ARHLNorth AmericaHearing loss populationSupported by grants EYO6594 (Drs R and B Klein) and AG11099 (Dr Cruickshanks) from the National Institute of Health, Bethesda, Md, and a Senior Scientific Investigator Award, from Research to Prevent Blindness, New York, NY (Dr R Klein)Prospective: 1Clinical : 2339748-92Both: 1North AmericaBoth:3Unspecified: 0, Unspecified: 0Yes:1Yes:1Yes:1Colour fundus photographsNo:2CholesterolYes:1Systolic and diastolic BPBone conduction audiometry, medical and family history, history of noise exposure, cardiovascular history, smoking status, visual acuityThese population-based estimates document the frequent coexistence of signs of ARM and hearing loss. As late ARM is an important cause of loss of vision, and hearing loss is associated with diffuculty in communicating, the high frequencies of sensory comorbidity may affect maintenance of independent functioning as people age.No:2Unknown: 0
40
137Long-term Assessment of Systemic Inflammation and the Cumulative Incidence of Age-related Hearing Impairment in the Epidemiology of Hearing Loss StudySD Nash, KJ Cruickshanks, W Zhan, MY Tsai, R Klein, R Chappell, FJ Nieto, BEK Klein, CR Schubert, DS Dalton, TS Tweedsnash@wisc.edu1993-1995 and evaluation every 5 yearsBeaver Dam Epidemiology of Hearing Loss StudySNHL - ARHLBeaver Dam, Wisconsin, USAHearing loss populationNational Institutes on Aging, National Eye Institute, National Institute of Diabetes and Digestive and Kidney Diseases, Research to Prevent BlindnessProspective: 1Clinical : 249261.07363.853.2–88.0Both: 1Unspecified:0ARHL: 3 Yes:1No:2No:2No:2Yes:1Bone conduction testingNo:2No:2Yes:1CRP, IL-6, TNF-αNo:2No:2Blood pressureAssociations between long-term serum C-reactive protein levels and incident hearing impairment were observed in the cohort as a whole, but differed significantly by age group, with statistically significant associations observed in adults less than 60 years, participants moving through the peak risk period for hearing impairment over the course of the studyYes:1CRPUnknown: 0
41
6Comparative study of mutation spectrums of MT-RNR1 m.1555A>G, GJB2, and SLC26A4 between familial and sporadic patients with nonsyndromic sensorineural hearing loss in Chinese HanL. Qian, J. Yubin, H. Bing, Z. Liang, L. Lan, Z. Yali, W. Hongyang, W.Dayong, W Qiujuwangdy301@126.com 2003 - 2009Beijing 301 familial probands and 703 sporadic patients with NSHLSNHL - GeneticChinaSNHL - Genetic/HereditaryNational Key Basic Research Program of China (No. 2014CB943001), the National Natural Science Foundation of China, Major Project (No. 81120108009), the Key Medical Technology Research Program of China People’s Liberation Army (No. BWS11J026), and the Nursery Foundation of China People’s Liberation Army (No. 14KMZ04).Prospective: 1Basic Science: 110040.4 - 51Both: 1Chinese HanUnspecified:0Yes:1Yes:1No:2No:2Yes:1No:2MT-RNR1 m.1555A>G, GJB2, and SLC26A4non-syndromic sensorineural hearing loss2003-2009Mutations of GJB2, SLC26A4, and MT- RNR1 m.1555A>G are the most important etiological factors in Chinese Han patients, among which SLC26A4 might be the most frequent.No:2Unknown: 0
42
37Surgical Findings and Long-Term Hearing Results in 3,050 Stapedotomies for Primary Otosclerosis: A Prospective Study with the Otology-Neurotology DatabaseR Vincent, N M Sperling, J Oates, and M JindalRobvinc@aol.com1991-2004Beziers 3,050 stapedotomiesPrimary OtosclerosisNSCHL - OtosclerosisNSProspective: 1Clinical : 2252547.38-91Both: 1NSUnspecified:0SNHL: 1Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2stapedotomy with vein graft interposition for otosclerotic stapes fixation is a
safe and successful treatment with sustained long-term hearing improvement		No:2No:2Unknown: 0The Otology-Neurotology Database (ONDB) (AS Multimedia Inc, Cassagne, France)
43
356Born in BradfordPI: John Wrightjohn.wright@bthft.nhs.uk2007-Born in BradfordBradford, West Yorkshire in the United KingdomPopulation cohortMedical Research Council and Economic and Social Research Council (Populations and Systems Medicine / General Population Science BoardsOther: 4CohortClinical : 212500 pregnant woman-13,500 childrenall agesUnspecified:0Unspecified:0No:2No:2No:2No:2No:2Medical recordsNo:2Yes:1Rubella status, Urinalysis , FBC, Group Antibody,
FBC ,Syphilis,HIV,Hepatitis B ,Electrophoresis,MSU
TT,Hep C
Medical & Obstetric History,Social Details, medical examinationYes:1Yes:1DNA samples were stored for further multiple analysesUnknown: 0
44
357Breakthrough Generations StudyPI: Anthony Swerdlow2004Breakthrough Generations Study?Population cohortBreakthrough Breast Cancer and The Institute of Cancer Research (ICR)Other: 4CohortClinical : 2113000Unspecified:0Unspecified:0No:2Yes:1Blood samplesQuestionnaires (how many children a woman has had, and when her pregnancies happened, whether and when she has reached menopause, and whether she has had breast disease. Questions are also asked, for instance, about work, diet, smoking and drinking)Yes:1DNA samples were stored for further multiple analysesBreast cancerUnknown: 0
45
253Hearing in 44e45 year olds with m.1555A>G, a genetic mutation predisposing to aminoglycoside-induced deafness: a population based cohort studyS Rahman, R Ecob, H Costello, MG Sweeney, AJ Duncan, K Pearce, D Strachan, A Forge, A Davis, M Bitner-Glindziczshamima.rahman@ucl.ac.uk1958 (1 week duration) - 2004 (follow up)British 1958 birth
cohort 		SNHL - GeneticUKBirth CohortSparks, the Children’s Medical Research Charity and by a Summer Studentship from the Royal
National Institute for Deaf People (now Action on Hearing Loss). Research at
the University College London Institute of Child Health and Great Ormond
Street Hospital for Children National Health Service Trust benefits from R&D
funding received from the National Health Service Executive. MB-G and SR are
supported by Great Ormond Street Hospital Children’s Charity		Prospective: 1Basic Science: 173501944-45Both: 1Unspecified:0SSNHL: 4 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2hearing in those with
m.1555A>G is not significantly different from the
general population and appears to be preserved at
least until 44e45 years of age.		No:2Unknown: 0
46
335British 1970 Cohort StudyPrincipal Investigator: Alice Sullivanalice.sullivan@ucl.ac.uk1970-British 1970 Cohort Study England, Scotland and Wales Population cohort Economic and Social Research Council,Medical Research Council, British Heart Foundation.Other: 4CohortClinical : 217198All agesBoth: 1Unspecified:0Unspecified:0Yes:1No:2No:2No:2No:2Self reported hearing assessmentYes:1Childhood cognitive development tests,
British Ability Scales		No:2Yes:1cholesterol and hba1c, storage for future analyses and DNA extractionNo:2Yes:1BP, HRAntropometric measurementsYes:1DNA samples were stored for further multiple analysesUnknown: 0
47
369British Birth Cohort 1946(1946BC)PI: Diana Kuhmrclha.enquiries@ucl.ac.ukBritish Birth Cohort 1946(1946BC)Population cohortOther: 4CohortClinical : 2current estimated sample size: 3116Unspecified:0Unspecified:0No:2Unknown: 0
48
341British Household Panel Survey Institute for Social and Economic Research of the University of Essexiser@essex.ac.uk1991-British Household Panel SurveyUKPopulation cohortHealth Education AuthorityOther: 4CohortClinical : 28000Unspecified:0Unspecified:0No:2No:2No:2No:2No:2Self reported hearing assessmentNo:2Unknown: 0
49
358British Regional Heart Study (BRHS)Study Manager: Lucy Lennonl.lennon@ucl.ac.uk1974-British Regional Heart Study (BRHS) England, Scotland and WalesPopulation cohortBritish Heart FoundationOther: 4CohortClinical : 2773540 and olderUnspecified:0Unspecified:0No:2No:2No:2No:2No:2self reported hearing assessmentYes:1Carotid artery ultrasound
Pulse wave velocity
Ankle brachial pressure index 		Yes:1blood/urine samplesAnthropometry
Weight, Mid-arm circumference, Physical function, Grip strength
Walking test, Chair stand test
Dental assessments, Physical activity assessmen
Actigraph GT3X physical activity monitor worn for 1 week for objective physical activity measures along with a log diary Hearing, eyesight, sleep patterns, activities of daily living, dental health, memory, depression, local environment, medications, personal circumstances (marital status, accommodation) and diet. Test your memory self-completed questionnaire		Unknown: 0
50
359British Women’s Heart and Health Study (BWHHS)PI: JP Casas Romerojulie.a.taylor@ucl.ac.uk1999-British Women’s Heart and Health Study (BWHHS)England, Scotland and WalesPopulation cohortThe study is currently funded by the British Heart Foundation and has been since 2009. The Department of Health funded the study from when it began until 2012.Other: 4CohortClinical : 2428660 and olderUnspecified:0Unspecified:0No:2No:2No:2No:2No:2self reported hearing assessment, medical recordsNo:2Yes:1haematology, biochemistry and storage for future analysisclinical measurements height, weight, blood pressure, lung function and a resting electrocardiogram, Actigraph Hearing, eyesight, sleep patterns, activities of daily living, dental health, memory, depression, local environment, medications, personal circumstances (marital status, accommodation) and diet. Test your memory self-completed questionnaireDNA samples were stored for further multiple analysesUnknown: 0
51
167Extensive cardiopulmonary resuscitation of preterm
neonates at birth and mortality and developmental
outcomes		Nicole Fischer, Amuchou Soraisham, Prakesh S. Shah, Anne Synnes, Yacov Rabi, Nalini Singhal, Joseph Y. Ting, Dianne Creighton, Deborah Deweyb, Marilyn Ballantyne, Abhay Lodha on behalf of the Canadian Neonatal NetworkTM (CNN) and the Canadian Neonatal Follow-up Network (CNFUN) Canadian Neonatal Network (CNN) Site InvestigatorsN. Fischer Department of Pediatrics & Department of Community Health Sciences, Foothills Medical Centre, Room C211, 29 Street NW,
Calgary, Alberta, T2N 2T9, Canada.
nfischer@hollandbloorview.ca 		2010 - 2012Canadian Neonatal Follow-up Network (CNFUN) SNHL - undifferentiatedCanadaOther disease registries/cohortsAlthough no specific funding has been received for this study, organizational support for the Canadian Neonatal Network was
provided by the Maternal-Infant Care Research Centre (MiCare) at Mount Sinai Hospital in Toronto, Ontario, Canada. MiCare and the Canadian Neonatal Follow-Up Network are supported by a Canadian
Institutes of Health Research (CIHR) Team Grant (CTP 87518), the
Ontario Ministry of Health, and in-kind support from Mount Sinai
Hospital.		Retrospective: 2clinical : 22760neonatesBoth: 1Unspecified:0Other:6 Mixed HL and SNHLYes:1No:2Unknown: 0
52
232Age-Dependent Cost-Utility of Pediatric Cochlear ImplantationYR Semenov, ST Yeh, M Seshamani, NY Wang, EA Tobey, LS Eisenberg, AL Quittner, KD Frick, JK Niparko, the CDaCI Investigative Teamjnipark@jhmi.eduNovember 2002 to December 2011CDaCI - Childhood Development after Cochlear Implantation (6 CI centres Johns Hopkins)SNHL - undifferentiatedUSASNHL - UndifferentiatedNational Institute on Deafness and Other Communication Disorders, the CityBridge Foundation, and the Sidgmore Family FoundationProspective: 1Clinical : 2175Both: 1Both:3SNHL: 1Yes:1No:2No:2No:2No:2Health Utility Index Mark HUI2 and HUI3 surveysYes:1Bayley Psychomotor Development Index & Leiter-R Brief IQNo:2No:2No:2No:2Early (<18 months) intervention with CI was associated with greater and longer quality of-life improvements, similar direct costs of implantation, and economically valuable improved classroom placement, without a greater incidence of medical and surgical complications when compared to CI at older agesNo:2Unknown: 0
53
322Childhood Development after Cochlear Implantation (CDaCI) study: Design and baseline characteristicsNE Fink, NY Wang, J Visaya, JK Niparko, A Quittner, LS Eisenberg, EA Tobeynfink@jhsph.eduUnspecified dates (two-year period)CDaCI - Childhood Development after Cochlear Implantation (6 CI centres Johns Hopkins)SNHL - undifferentiatedUSASNHL - UndifferentiatedNIDCDProspective: 1Clinical : 21884252.21-7Both: 1Both:3SNHL: 1No:2No:2No:2No:2No:2No detailsYes:1Bayley Scales of Infant Development Mental Scale or Motor Scale (BSID II) and Leiter International Performance Scale-RevisedNo:2No:2No:2No:2Reynell Development Language Scales (RDLS) This longitudinal cohort study addresses questions related to high variability in language outcomes. Identifying sources of that variance requires research designs that: characterise potential predictors with accuracy, use samples that adequately power a study, and employ controls and approaches to analysis that limit bias and error. The CDaCI study was designed to generate a more complete picture of the interactive processes of language learning after implantationNo:2Unknown: 0
54
71Progressive and fluctuating sensorineural hearing loss in children with asymptomatic congenital cytomegalovirus infectionK B Fowler, F P McCollister, A J Dale, S Boppana, W J Britt and R F PassKaren B. Fowler, DrPH, Department of Pediatrics, University of Alabama at Birmingham, 1600 7th Ave. South, Suite 752, Birmingham, AL 35233.1980-1995Chiildren born between 1980-1995 who were
identified by newborn screening for congenital CMV infection at two hospitals in Birmingham, Ala		SNHL - InfectiousNorth AmericaOther disease registries/cohortsSupported in part by a research grant (5 P01 HD 10699) from the National Institute of Child Health and Human Development, a re- search grant (5 R01 DC 02139) from the National Institute on Deafness and Other Communication Disorders, and a research grant (5 M01 RR 00032) from the General Clinical Research Center, Na- tional Institutes of Health, the Deafness Research Foundation, and the Civitan International Research Center. Prospective: 1Clinical : 2307424Both: 1Both:3SNHL: 1Yes:1Yes:1Yes:1Yes:1CMV Serologic tests, otoscopyOtoscopyAsymptomatic congenital CMV infection is likely a leading cause of SNHL in young children. The continued deterioration of hearing and delayed onset of SNHL in these children emphasizes the need for continued monitoring of their hearing statusNo:2Unknown: 0
55
30Hearing Genes and Cisplatin Deafness: A Pilot StudyChristine Knoll, Richard J. H. Smith, Carol Shores, Julie BlattJulie Blatt, University of North Carolina School of Medicine, CB 7220 Chapel Hill, NC, 27599-7220, U.S.A. jblat@med.unc.eduUnspecifiedChildhood Cancer Survivor Study databaseSNHL - GeneticUnited StatesOther disease registries/cohortsRetrospective: 2Clinical : 2111250younger thanboth: 1Unspecified:0Other:6 Ototoxicity Drug induced hearing lossYes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1No:2GJB2 , SLC26A4, mtDNA genesChildhood Cancers (Osteosarcoma, Soft tissue sarcoma, CNS tumor2003It is unlikely that mutations in GJB2 or SLC26A4 or the 3 mtDNA mutations A1555G, A3243G, or A7445G contribute to cisplatin ototoxicity. Nonetheless, the current study should be considered only a pilot and continued study of this population should remain a priority.No:2Unknown: 0
56
350China Ageing REespiratory infections Study (CARES)Cowling BJ, Xu C, Tang F, Zhang J, Shen J, Havers F, Wendladt R, Leung NH, Greene C, Iuliano A, Shifflett P, Song Y, Zhang R, Kim L, Chen Y, Chu DK Zhu H, Shu Y, Yu H, Thompson MG; CARES investigatorsCorrespondence to Dr Benjamin J Cowling: kh.ukh@gnilwocb2015-2016China Ageing REespiratory infections Study (CARES)Eastern ChinaPopulation cohortInfluenza Division of the US Centers for Disease Control and Prevention Other: 4CohortClinical : 2153260-89Unspecified:0Unspecified:0No:2No:2No:2No:2No:2Self reported hearing impairment (Groningen Frailty Index)No:2Yes:1Blood samples,mid-turbinate nasal and oropharyngeal swabs baseline data on demographics, general health, chronic diseases, functional status and cognitive function through face-to-face interviews using a standardised questionnaireUnknown: 0
57
296Mitochondrial 12S rRNA variants in 1642 Han Chinese pediatric subjects with aminoglycoside-induced and nonsyndromic hearing lossJ Lu, Z Li, A Yang, R Li, J Zeng, Q Cai, G Peng, W Tang, B Chen, J Chen, Z Liao, L Yang, Y Li, J You, Y Ding, H Yu, J Wang, D Sun, J Zhao, L Xue and M-X Guanmin-xin.guan@cchmc.orgUnspecified datesChinese 1642 unrelated hearing-impaired subjectsSNHL - GeneticChinaSNHL - nonsyndromic - undifferentiatedThis work was supported by Public Health Service grants RO1DC05230 and RO1DC07696 from the National Institute on Deafness and Other Communication Disorders, and grants from National Basic Research Priorities Program of China 2004CCA02200, Ministry of Public Heath of Zhejiang Province 2006A100, Ministry of Science and Technology of Zhejiang Province 2007G50G2090026 and Zhejiang Provincial Program for the Cultivation of High level Innovative Health talents to M.X.G. and Ministry of Science and Technology of Wenzhou City Y20060089 to
Z.L. and Natural Science Foundation of Zhejiang Province Y207307 to Y.Z.		Prospective: 13284PaediatricBoth: 1Han ChineseUnspecified:0Other:6 Deafness, aminoglycoside-induced and nonsyndromic hearing impairmenYes:1Yes:1Yes:1No:2No:2Yes:1No:212S rRNA gene, GJB2, TRMUMutational analysis of 12S rRNA gene in these subjects identified 68 (54 known and 14 novel) variants. The frequencies of known 1555A>G and 1494C>T mutations were 3.96% and 0.18%, respectively, in this cohort with nonsyndromic and aminoglycoside-induced hearing loss.Yes:168 (54 known and 14 novel) variantsUnknown: 0
58
84The European GWAS-identified risk SNP rs457717 within IQGAP2 is not associated with age-related hearing impairment in Han male Chinese population
Huajie Luo, Hao Wu, Hailian Shen, Haifeng Chen, Tao Yang, Zhiwu Huang, Xiaojie Jin, Xiuhong Pang, Lei Li, Xianting Hu, Xuemei Jiang, Zhuping Fan, Jiping LI.
Jiping LI drlijiping@163.com
Huajie Luo luohuajie2000@qq.com
Participants recruited between 07/2011 - 07/2014Chinese Han 2420 patients with ARHISNHL - GeneticXinhua and RenJi Hospitals, ShanghaiSNHL - ARHLGrants from Medical Engineering (Technology) Cross Research Fund, Shanghai Jiao Tong University (No. YG2014MS47), and grants from the Medical Research Fund of Shanghai Municipal Health and Family Planning Commission (No. 201440295).
Retrospective:2Basic Science: 1242050-100Male: 3Southern Chinese HAN population (Asian)Unspecified:0ARHL: 3 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1No:2No:2No:2IQGAP2IQGAP2 and ARHI in an Asian ethnic group, IQGAP2 risk SNP locus (rs457717: A/G) in intron 13 identified by a European GWAS was not associated with ARHI in Han male Chinese individuals.No:2Unknown: 0 QUANTO software version 1.2 4. All calculations were performed using the statistical software package 19.0.0 (IBM SPSS Statistics, Inc., Chicago, IL).
59
229Cochlear implantation in patients deafened by ototoxic drugsJ Nichani, IA Bruce, D Mawman, S Khwaja, R Ramsden, K Greenjaya.nichani@cmft.nhs.uk1997 to 2011CI program - Manchester Auditory Implant Centre databaseSNHL - IatrogenicManchester, UKSNHL - UndifferentiatedProspective: 1Clinical : 214Both: 1Unspecified:0Other:6 No:2No:2No:2No:2No:2Bamford–Kowal–Bench (BKB) word testNo:2No:2No:2No:2No:2Patients with profound hearing loss secondary to ototoxic agents can be rehabilitated successfully with CI. The outcomes may be variable and may be dependent on the underlying pathology for which the ototoxic agents were prescribedNo:2Unknown: 0
60
286Prevalence of Connexin 26 (GJB2) and Pendred (SLC26A4) Mutations in a Population of Adult ochlear Implant CandidatesJB Hochman, TL Stockley. D Shipp, VYW Lin, JM Chen and JM Nedzelskijordanhochman@
hotmail.com		November 2007 - April 2009CI program (Sunnybrook
Health Sciences Centre-Quaternary Healthcare Facility)		SNHL - GeneticCanadaSNHL - UndifferentiatedProspective: 1Bilateral:2Other:6 severe sensorineural hearing lossYes:1Yes:1Yes:1 computer tomography of the petrous bone, otomicroscopyYes:1 electronystagmographyNo:2No:2Yes:1exon sequencingGJB2, GJB6, SLC26A4The prevalence of GJB2- and SLC26A4-related hearing impairment in an adult population with early-onset severe sensorineural hearing loss is significant, suggesting the need for routine assessment for genetic etiologies in this group.No:2Unknown: 0
61
268A Descriptive Analysis of Language and Speech Skills in 4- to 5-Yr-Old Children With Hearing LossElizabeth M. Fitzpatrick, Leah Crawford, Andy Ni, Andre´e Durieux-SmithElizabeth M. Fitzpatrick, Faculty of Health
Sciences, Audiologie-Speech-Language Pathology, 451 Smyth Road (3071),
Ottawa, Ontario K1H 8M5, Canada elizabeth.fitzpatrick@uottawa.ca.		1999 to 2004CI programmes (x3) in Ontario, CanadaSNHL - undifferentiatedOttawa and Toronto, CanadaSNHL - UndifferentiatedCanadian Language and Literacy Research
Network and the Masonic Foundation of Ontario.
E. M. Fitzpatrick received funding through a Canadian Institutes of Health
Research New Investigator Award.		Other: 4ObservationalClinical : 2884-5Both: 1Unspecified:0Other:6 Children with SNHL who received a HA or a CIPeabody Picture Vocabulary
Test, Third Edition (PPVT-III), the Preschool Language
Scale, Fourth Edition (PLS-4), and the Goldman-Fristoe Test
of Articulation, Second Edition (GFTA-2), 		No:2No:2No:2No:2The Child Development Inventory
(CDI)		Results indicated that overall, children with all degrees of
hearing loss who were fit with hearing technology and who received auditory-based rehabilitation services during the preschool years demonstrated the potential to develop spoken language communication skills. As a group, children with CIs and children with HAs did not differ significantly on language abilities although there were differences in articulation skills. Their performance at age 4 to 5 yrs was delayed compared with a group of hearing peers.		No:2Unknown: 0
62
270Etiology profile of the patients implanted in the cochlear implant
program		Clara Maria Dias Ferreira Calháu, Luiz Rodolpho Penna Lima Júnior, Ana Maria da Costa dos Santos Reis,
Ana Karla Bigois Capistrano, Danielle do Vale Silva Penna Lima, Ana Carolina Dias Ferreira Calháu, Fábio
de Alencar Rodrigues Júnior		Rua Dr. José Gonçalves 1963 - Lagoa Nova 59056-570August 2000 - May 2008CI progrram- BrazilSNHL - undifferentiatedBrazilSNHL - UndifferentiatedRetrospective: 2Clinical : 22001-73Both: 1Unspecified:0Unspecified:0unknown etiology continues prevailing, which points
to the need of carrying out genetic studies, in cases of
congenital sensorineural hearing loss without an apparent
cause, with the goal of reaching a real etiologic profile.
Rubella was the second most commonly found
cause, and for this etiology there already are preventive
measures, similarly to what we have for meningitis. Even
then, the incidences of these diseases are still high		No:2Unknown: 0
63
283Functional Null Mutations of MSRB3 Encoding Methionine Sulfoxide Reductase Are Associated with Human Deafness DFNB74ZM Ahmed, R Yousaf, BC Lee, SN Khan, S Lee, K Lee, T Husnain, AU Rehman, S Bonneux, M Ansar, W Ahmad, SM Leal, VN Gladyshev, IA Belyantseva, GV Camp, S Riazuddin, TB Friedman and S Riazuddinsaima.riazuddin@cchmc.orgUnspecified datesCincinnati 5 consanguinous families with NSHLSNHL - GeneticPakistanSNHL - Genetic/HereditaryThis work was supported by Cincinnati Children’s Hospital Research Foundation (CCHMC) intramural research funds to SR and ZA, the National Institute on Deafness and Other Communication Disorders, National Institutes of Health (NIDCD/NIH) research grant R00-DC009287-03, a Career Development Award from Research to Prevent Blindness (to Z.A.), and National Institute on Aging, NIH grant AG021518 (to V.N.G.) . Work in Pakistan was supported by the Higher Education Commission to S.R. (Lahore); funding from World Health Organization Regional Office for the Eastern Mediterranean (EMRO) and COMSTECH (Organization of the Islamic Conference [OIC] Standing Committee on Scientific and Technological Cooperation) and from the Ministry of Science and Technology (MoST) to S.R. (Lahore); the International Center for Genetic Engineering and Biotechnology, Trieste, Italy under project CRP/PAK08-01 contract no. 08/009 to S.R. (Islamabad). Part of the work was funded by the Higher Education Commission (HEC), Government of Pakistan, and the NIH National Institute of Deafness and other Communication Disorders grant DC03594 to S.M.L. Genotyping services were partially provided by the Center for Inherited Disease Research (CIDR). CIDR is fully funded through a federal contract from the NIH to The Johns Hopkins University, contract number N01-HG-65403. Work at NIDCD/ NIH was supported by intramural funds DC00039-14 to T.B.FProspective: 1104053-67Both: 1Unspecified:0Unspecified:0Yes:1tandem-gait and Romberg testing, fundoscopyYes:1Genome Wide Linkage AnalysisDFNB74, MSRB3In summary, an in vitro assay revealed that p.Cys89Gly completely abolished MSRB3 enzymatic activity and that p.Arg19X is a null allele for MSRB3 mitochondrial isoforms, indicating that DFNB74 deafness might be a mitochondrial disease limited to the inner ear.No:2Unknown: 0
64
41Audiometric, clinical and educational outcomes in a pediatric symptomatic congenital cytomegalovirus (CMV) population with sensorineural hearing lossC Madden, S Wiley, M Schleiss, C Benton, J Meinzen-Derr, J Greinwald, D Choodaniel.choo@cchmc.orgUnspecifiedCincinnatii Paediatric hearing impaired databaseSNHL - InfectiousNorth AmericaALL - UndifferentiatedRetrospective: 2Clinical : 2211500neonatesBoth: 1Unspecified:0SNHL: 1Yes:1Yes:1Yes:1Non-contrast cranial computed tomography and magnetic resonance imaging (MRI) scansNo:2No:2No:2Statistical associations between
clinical findings such as hepatic dysfunction, CP and hearing level were identified		No:2No:2Unknown: 0
65
342CLOSER The Cohort and Longitudinal Studies Enhancement Resources CLOSER The Cohort and Longitudinal Studies Enhancement Resources Population cohortOther: 4CohortClinical : 2Unspecified:0Unspecified:0No:2Unknown: 0
66
166Racial and Ethnic Differences in the Prevalence of Congenital Cytomegalovirus InfectionKaren B. Fowler, Shannon A. Ross, Masako Shimamura, Amina Ahmed, April L. Palmer, Marian G. Michaels, David I. Bernstein, Pablo J. SánchezKaren B. Fowler, DrPH, Department of Pediatrics, University
of Alabama at Birmingham, 1600 7th Avenue South CHB 304, Birmingham, AL 35233. kfowler@uab.edu		UnspecifiedCMV and Hearing Multicenter
Screening (CHIMES) Study		SNHL - InfectiousUSOther disease registries/cohortsSupported by the National Institute on Deafness. Other
Communication Disorders (NIDCD) provided grant
support for the CHIMES Study (N01 DC50008, HHS-N-
263-2012-00010-C)		Registry: 3Clinical : 2100.332infantsBoth: 1American Indian, Asian, Black, White, Hispanic, White, non-Hispanic, MultiracialUnspecified:0SNHL: 1No:2Yes:1CMV, BilirubinNo significant
differences in symptomatic cCMV and sensorineural hearing loss were observed between the
race/ethnic groups.		No:2Unknown: 0
67
360Cognitive Function and Ageing Studies I (CFAS I)CI:Carol Brayne cb105@medschl.cam.ac.uk1989-Cognitive Function and Ageing Studies I (CFAS I)UKPopulation cohortMedical Research CouncilOther: 4CohortClinical : 21800065 and olderUnspecified:0Unspecified:0Yes:1No:2No:2No:2No:2Hearing test, self reported hearing impairmentYes:1No:2Yes:1Blood, saliva samplesOrientation, demographics, cognitive functions,medical history,DNA samples were stored for further multiple analysesUnknown: 0
68
361Cognitive Function and Ageing Studies II (CFAS II)CI:Carol Brayne cb105@medschl.cam.ac.uk2008Cognitive Function and Ageing Studies II (CFAS II)Cambridgeshire, Newcastle and NottinghamPopulation cohortUK Medical Research Council, Alzheimer’s Society UK and utilised resources of the UK National Institute of Health Research collaboration for leadership in applied health research and care for Cambridgeshire and Peterborough and the Cambridge Biomedical Research Centre infrastructures, Nottingham city and Nottinghamshire county NHS primary care trusts, and UK NIHR biomedical research centre for ageing and age-related disease award to Newcastle Upon Tyne hospital foundation trustOther: 4CohortClinical : 2>500065 and overUnspecified:0Unspecified:0Yes:1No:2No:2No:2No:2Hearing testNo:2Yes:1blood, saliva, postmortem brain donationsDNA samples were stored for further multiple analysesUnknown: 0
69
22Prevalence of auditory neuropathy/synaptopathy in a population of children with profound hearing lossAstrid Foerst , Dirk Beutner, Ruth Lang-Roth, Karl-Bernd Huttenbrink, Hasso von Wedel, Martin WalgerAstrid Foerst :  Tel.: +49 221 478 4769/3711; fax: +49 221 478 3581  astrid.foerst@uni-koeln.de (A. Foerst).1997 -2004Cologne 5190 children, with profound hearing loss or risk or risk of HLSNHL- ANSDGermanySNHL - ANSDscholarship of the ‘‘Novartis-Stiftung fu¨r therapeutische Forschung’’ to Dirk BeutnerRetrospective: 2Clinical : 251901-15Both: 1Unspecified:0Unspecified: 0Yes:1Yes:1Yes:1No:2Yes:1No:2No:2No:2No:2No:2More research aiming to investigate infectious backgrounds of auditory dysfunction is needed. Results of the present study indicate - but do not prove -that two common epidemic diseases, influenza and possibly pertussis, are hitherto unrecognized pre or early postnatal risk factors for mild hearing loss in young adulthood.No:2Unknown: 0
70
24The incidence of hearing impairment after successful treatment of neuroblastomaT. Simon , B. Hero , W. Dupuis- , B. Selle , F. BertholdThorsten Simon Zentrum fur Kindreonkologie und Hämatologie Joseph-Stelzmann-Strabe 9. 50924 Köln Thorsten.simon@medizin.uni-koeln.deUnspecifiedCooperative German Neuroblastoma Trials NB90 and NB97SNHL - IatrogenicGermany Other disease registries/cohortsRetrospective: 2Clinical : 21170childrenBoth: 1Unspecified:0Unspecified: 0Unspecified hearing testsOne fourth of of high risk Neuroblastoma survivors suffer from treatment induced hearing impairment. The substitution of cisplatin with carboplatin was not associated with increased rate of tumor recurrences. (However they were not able to detect the ototoxicity of Carboplatin as it was administered after the hearing loss was documented.No:2Unknown: 0
71
68Prevalence of hereditary hearing impairment in adultsY Sakihara, B Christensen and A Parvingbbhaudap@pip.dknet.dk1987-1991Danish Birth CohortSNHL - GeneticDenmarkBirth CohortRetrospective: 2Clinical : 21265276927022-98Both: 1Both:3SNHL: 1Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2the established database may be of
importance in the aggregation of very rare diseases, and for
providing the inspiration for future prospective population studies,
resulting in knowledge on the epidemiology of hereditary hearing
impairment in adults.		No:2Unknown: 0
72
139Atherogenic Risk Factors and Hearing ThresholdsTW Frederiksen, CH Ramlau-Hansen, ZA Stokholm, MB Grynderup, AM Hansen, SP Lund, JM Vestergaard, J Kristiansen, JP Bonde, HA Kolstadthofre@rm.dk2009-2010Danish workers surveySNHL - NIHLAarhus, DenmarkOccupationalDanish Work Environment Research FundProspective: 1Clinical : 257666539.8-53.4Both: 1Unspecified:0Unspecified:0Yes:1No:2No:2No:2No:2No:2No:2Yes:1LDL, HDL, TGNo:2Yes:1Blood pressureAtherogenic risk factors seemed associated with increased low-frequency hearing thresholds, but only at a borderline level of statistical significance. Associations were generally strongest with hearing levels of the worst hearing ear. We found no statistically significant associations between atherogenic risk factors and high-frequency hearing thresholds.No:2Unknown: 0
73
233Sensorineural Hearing Loss Incidence Among U.S. Military Aviators Between 1997 and 2011CA Orsello, JE Moore, C Reeseorselloc@yahoo.com 1997 and 2011Defense Medical Epidemiological Database DMEDSNHL - NIHLUSAOccupationalRetrospective: 2Clinical : 246706420 to above 40Both: 1Both:3SNHL: 1Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2The average annual incidence rate of SNHL between 1997-2011 was higher for fi xed than for rotary-wing aviators, and higher for U.S. Army and Air Force than Navy and Marine aviators.No:2Unknown: 0
74
165Whole-genome sequencing reveals new insights into age-related hearing loss: cumulative effects, pleiotropy and the role of selectionDragana Vuckovic, Massimo Mezzavilla, Massimiliano Cocca, Anna Morgan, Marco Brumat, Eulalia Catamo, Maria Pina Concas, Ginevra Biino, Annamaria Franzè, Umberto Ambrosetti,
Mario Pirastu, Paolo Gasparini, Giorgia Girotto		dragana.vuckovic@burlo.trieste.itUnspecifiedDiscovery CohortSNHL - GeneticItalySNHL - ARHLRBSI14AG8P-SIR2014 to
GG.		Prospective: 1Basic Science: 1212>50Both: 1Unspecified:0ARHL: 3 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1No:2No:2No:2In conclusion, we show that this
multistep strategy (WGS, selection, expression, pathway analysis and targeted re-sequencing) can provide major insights
into the molecular characterization of complex diseases such as ARHL.		Yes:1additional functional studies of
CSMD1 and PTPRD genes/variants 		Unknown: 0
75
362Dunedin Multidisciplinary Health and Develoment StudyDirector: Richie Poultonrichie.poulton@otago.ac.nz1972-Dunedin Multidisciplinary Health and Develoment StudyDunedin, New ZealandPopulation cohortNew Zealand Ministry of Business, Innovation and Employment in 2015.Major funders include the Health Research Council of New Zealand, US National Institutes of Health (various branches) and the UK Medical Research CouncilOther: 4CohortClinical : 21037All agesUnspecified:0Unspecified:0Yes:1No:2No:2No:2Yes:1Audiometry, tympanometry, self reported surveyNo:2Perinatal, demographic and anthropometric informationUnknown: 0
76
32Outcome at 2 years for very low birthweight infants in a geographical population: Risk factors, cost, and impact of congenital anomaliesA. Salt, A. D’Amore, J. Ahluwalia, A. Seward, S. Kaptoge, S. Halliday, J. DorlingJ. Dorling Tel.: +44 116 252 5879; fax: +44 116 252 3282.
jsd10@le.ac.uk		1993—1997East Anglian Very Low Birthweight ProjectSNHL - undifferentiatedEast AngliaOther disease registries/cohortsFunding was initially received from regional audit funds,
funding from regional research and development and from
the Anglia Clinical Audit and Effectiveness Team in Cambridge		prospective: 1clinical : 29472Both: 1unspecified:0Unspecified:0Unspecified hearing testsYes:1No:2No:2No:2No:2No:2Unknown: 0
77
363East London Genes and HealthCI: David van Heeld.vanheel@qmul.ac.uk2015-East London Genes and HealthEast LondonPopulation cohortWellcome TrustOther: 4CohortClinical : 2target: 100000All agesUnspecified:0Unspecified:0No:2No:2No:2No:2No:2Medical recordsNo:2Yes:1Blood, salivaMedical records, questionnaires: Anthropometric: Height, Weight, Blood pressure
Physical: Cardiovascular, Respiratory, Musculoskeletal, Hearing and vision, Reproductive
Psychological: Mental health, Cognitive function
Lifestyle: Smoking, Physical activity, Dietary habits, Alcohol
Socio-economic: Ethnic group		Yes:1No:2No:22018, 2019, 2020Unknown: 0
78
185Association of Cognition and Age-Related Hearing Impairment in the English Longitudinal Study of AgeingJ Ray, G Popli and G Fellg.popli@shef.ac.uk2002 - unspecified, objective hearing data 2014 - 15English Longitudinal Study of Ageing (ELSA)SNHL - ARHLUnited KingdomPopulation cohortRetrospective: 2cross-sectional analysisClinical : 273859666>50Both: 1Unspecified:0ARHL: 3 No:2No:2No:2No:2No:2HearCheck screener device (Siemans)Yes:1simple tests of memory and executive function (outlined in methods)No:2No:2No:2No:2Although hearing loss and cognition are linked, untreated hearing loss drives the association. Social isolation is a mediating factor only for those who have untreated hearing loss. Authors found that cognitive decline associated with ARHI is probably preventable by early rehabilitation and increased opportunistic screening for the elderlyNo:2Unknown: 0
79
25Epidemiology of Permanent Childhood Hearing Impairment in Estonia, 1985-1990Kai Uus & Adrian C. Davis1985-1990Estonian birth cohort 1985-90SNHL - undifferentiatedEstoniaBirth CohortEstonian Science Foundation Grant No:2376Retrospective: 2Clinical : 22488-13Both: 1Unspecified:0Unspecified: 0Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Unknown: 0
80
364European Prospective Investigation of Cancer Norfolk (EPIC – Norfolk)PI: Kay-Tee Khawepic@srl.cam.ac.uk1993-European Prospective Investigation of Cancer Norfolk (EPIC – Norfolk)Norfolk (UK)Population cohortMRC Programme GranOther: 4CohortClinical : 23000040 and olderUnspecified:0Unspecified:0No:2No:2No:2No:2No:2self reported hearing assessmentYes:1ultrasound measurement of heel bone Yes:1Blood and urine samples
Demographics,height, weight, waist, hip and chest measurements,blood pressure and lung capacity, density and impedance assessment of total body fat cognition, visual function and physical performance, grip strength, balance test, gait speed and chair stand, body composition, including lean muscle mass by DEXA, and physical activity and sedentary behaviour, by accelerometry (Actigraph),diet		Yes:1Unknown: 0
81
15Functional Variants in NOS1 and NOS2A Are Not Associated with Progressive Hearing Loss in Me ́nie`re’s Disease
in a European Caucasian Population		I.Gazquez,1,2 Jose A. Lopez-Escamez,1,3 Antonia Moreno,2 Colleen A. Campbell,4 Nicole C. Meyer,4 John P. Carey,5 Lloyd B. Minor,5 Bruce J. Gantz,4 Marlan R. Hansen,4 Charles C. Della Santina,5 Ismael Aran,6 Andres Soto-Varela,7 Sofia Santos,7 Angel Batuecas,8 Herminio Perez-Garrigues,9
Alicia Lopez-Nevot,10 Richard J.H. Smith,4 and Miguel A. Lopez-Nevot2		antonio.lopezescamez@genyo.es2005 - 2010European two independent MD sets(273 patients in total) and 550 controls.Meniere's DiseaseSpainOther disease registries/cohortsIS PI10/0920 Research Project from ISCIII. J.A.L.-E. was partially supported by ISCIII research grant INT09/229. The 3130 XL Genetics Analyzer was funded by grant IF06/37291 from Ministry of Science. This work was partially supported by the University of Iowa,
Department of Otolaryngology and the Research Fund of the American Otological Society (to R.J.H.S.)		Prospective: 1Basic Science: 1823273Both: 1Unspecified:0SNHL: 1Yes:1Caloric testingNo:2No:2Yes:1No:2NOS1, NOS2AMeniere's DiseaseUnknown: 0
82
36Hearing loss in Fabry disease: data from the Fabry Outcome SurveyS. Hegemann, D. Hajioff, G. Conti, M. Beck, G. Sunder-Plassmann, U. Widmer, A. Mehta, and A. KeilmannDr D. Hajioff, Department of Otolaryngology,
Southmead Hospital, Bristol, BS10 5NB, UK. Tel.: +44 117950 5050; fax: +44 117959 5850; ejci@hajioff.fmail.co.uk		2004Fabry Outcome
Survey		SNHL - GeneticGermany, UK, Czech Republic, Switzerland, Italy, Spain, France, Belgium, Norway, Sweden, AustriaOther disease registries/cohortsShire Human Genetic Therapies, Cambridge, MA, USARegistry: 3Clinical : 263814-66Both: 1unspecified:0Unspecified:0Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2In general, hearing in Fabry patients
resembles the age-related hearing loss in a normal population
but starts earlier and progresses faster. Male patients
had more severe hearing loss than female patients		No:2Unknown: 0
83
222Addition of host genetic variants in a prediction rule for post meningitis hearing loss in childhood: a model updating studyMS Sanders, RCJ de Jonge, CB Terwee, MW Heymans, I Koomen, S Ouburg, L Spanjaard, SA Morré, AM van Furthr.c.j.dejonge@erasmusmc.nl1986-2001Files of the Netherlands
Reference Laboratory for BM (NRLBM)		SNHL - Geneticthe NetherlandsSNHL - InfectiousProspective: 1Clinical : 266916052.60 – 9Both: 1Dutch-CaucasianUnspecified:0Other:6 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1No:2TLR2, TLR4, TLR9, NOD1, NOD2, CASP1, TRAIL-692T Bacterial meningitisaddition of genetic risk factors did not significantly improve the clinical prediction model for post-meningitis hearing lossNo:2Unknown: 0
84
227Independent Validation of an Existing Model Enables Prediction of Hearing Loss after Childhood Bacterial MeningitisRCJ de Jonge, MS Sanders, CB Terwee, MW Heymans, RJBJ Gemke, I Koomen, L Spanjaard, AM van Furthr.c.j.dejonge@erasmusmc.nl1986-2001Files of the Netherlands
Reference Laboratory for BM (NRLBM) 		SNHL - Infectiousthe NetherlandsSNHL - InfectiousProspective: 1Clinical : 23581605Unspecified:0Other:6 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2This prediction model for identifying children at risk for hearing loss after bacterial meningitis might be used as a screening tool and can help to identify those children that need special attention and a long follow-up period or more frequent auditory testingNo:2Unknown: 0
85
26Prevalance of hearing loss at the age of 15 in a birth cohort of 12000 children from northern FinlandMartti Sprri, Paula Rantakallio1966Finland Birth Cohort from 1966SNHL - undifferentiatedFinland, Finland, FinlandBirth CohortOther: 4Cross-sectionalClinical : 24251178014Both: 1Unspecified:0Unspecified: 0Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Unknown: 0
86
148Sensorineural Hearing Loss Associated
with Neomycin Eardrops and Nonintact
Tympanic Membranes		Almut G. Winterstein, Wei Liu, Dandan Xu and
Patrick J. Antonelli		pa@ufl.edu1999 - 2006Forida's Administrative claims dataSNHL - IatrogenicUSAHealthcare database1)Department of Pharmaceutical Outcome and Policy, College of Pharmacy,
University of Florida, Gainesville, Florida, USA
2) Department of Epidemiology, College of Public Health and Health
Professions and College of Medicine, University of Florida, Gainesville,
Florida, USA
3) Department of Otolaryngology, College of Medicine, University of Florida,
Gainesville, Florida, USA		Retrospective: 2Clinical : 2134,598<18 yearsBoth: 1Unspecified:0Other:6 non-intact tympanic membraneYes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2Short-term use of neomycin eardrops in patients
with NITMs is not associated with an increased risk of SNHL;
however, repeated doses (ie, �2 prescriptions) showed a significant
association with an increased risk of SNHL.		No:2Unknown: 0
87
351Framingham Heart StudyFHS@bu.edu1948Framingham Heart StudyFramingham in Massachusetts (US)Population cohortNational Heart Lung and Blood Institute (NHLBI) of the National Institutes of Health and Boston University School of MedicineOther: 4CohortClinical : 2520930-62Unspecified:0Unspecified:0Yes:1No:2No:2No:2No:2Hearing testNo:2Yes:1Blood samplesYes:1,cardiac vascular function (tonometry), echocardiogram,ECG,blood pressure,physical examinations; height, weight, and waist, grip strength, medical historyUnknown: 0
88
97Self-Reported Hearing Loss Predicts 5-Year Decline in Higher-Level Functional Capacity in High-Functioning Elderly Adults: The Fujiwara-Kyo Study Kimiko Tomioka, MD, PhD,* Nozomi Okamoto, DDS, PhD,† Masayuki Morikawa, MD, PhD,‡ and Norio Kurumatani, MD, PhD*†
Kimiko Tomioka, Nara Prefectural Health Research Center, Nara Medical University, Shijo-cho 840, Kashihara City, Nara 6348521, Japan. E-mail: tkimiko@naramed-u.ac.jp
2007–08 follow-up examinations in 2012-2013Fujiwara-Kyo StudyDementiaPopulation cohortThis work was supported by Grant-in-Aid for Scientific Research 24249043 from the Ministry of Education, Culture, Sports, Science, and Technology, Japan. The authors wish to express their gratitude to all the participants for their cooperation in this study. 3,93665-93Both: 1Not statedUnspecified:0Unspecified: 0No:2No:2No:2No:2No:2No:2No:2No:2Yes:1triglycerides, LDL, HbA1cNo:2Yes:1Previous cardiac events (e.g. Mis)Self reported HL, questionaire Self-reported HL was found to be a significant predictor of decline in IA and SR. Preventive intervention against age-related HL may contribute to maintaining high-level functional capacity in independent elderly adults. No:2Unknown: 0
89
203PrevalenceofHearingLossAmongChildren9to11YearsOld TheGenerationRStudyCMP leClercq, GijsvanIngen, L Ruytjens, A Goedegebure, HA Moll, H Raat, VWV Jaddoe, RJB Jong, MP vanderSchroeffCarlijnM.P. leClercq c.leclercq @erasmusmc.nl2012 - 2015Generation RAll - undifferentiatedRotterdam, NetherlandsBirth CohortTheGenerationRStudyandDr JaddoereceivefinancialsupportfromErasmusMC Rotterdam,ErasmusUniversityRotterdam,andThe NetherlandsOrganisationforHealthResearchand Development(ZonMw). Retrospective: 2Clinical : 253684189-11Both: 1Majority white, grouped as white and non-whiteBoth:3SSNHL: 4 Yes:1No:2No:2No:2Yes:1No:2No:2No:2No:2No:2Maternal education, hx AOMIn thiscross-sectionalassessment of a population-based prospective birth cohort study, 7.8% of children were estimated to have low-orhigh-frequency SNHL in either ear, with a prevalence of 0.9% of mild or more severe SNHL (≥26dBHL). A history of re
current acute otitis media and lower maternal educational status were associated with the presence of presumed SNHL.		Unknown: 0
90
348Genetic Epidemiology Research on Adult Health and Aging  (GERA)info@tasciences.com
2007Genetic Epidemiology Research on Adult Health and Aging  (GERA)Northern California (US)Population cohortResource for Genetic Epidemiology Research in Adult Health and Aging (RC2 AG033067; Schaefer and Risch, PIs) awarded to the Kaiser Permanente Research Program on Genes, Environment, and Health (RPGEH) and the UCSF Institute for Human Genetics. The RPGEH was supported by grants from the Robert Wood Johnson Foundation, the Wayne and Gladys Valley Foundation, the Ellison Medical Foundation, Kaiser Permanente Northern California, and the Kaiser Permanente National and Northern California Community Benefit Programs.Other: 4CohortClinical : 2140,00018 and olderUnspecified:0Unspecified:0No:2No:2No:2No:2No:2clinical records, self reported surveyNo:2clinical data from electronic health records(ICD-9 coded diagnoses), survey data on demographic ( gender, race/ethnicity, marital status, and education),body mass index, behavioral factors( smoking, alcohol consumption), and environmental data from various sources, Yes:1Multiple, see individual studyUnknown: 0
91
90Age-related hearing decline in individuals with and without occupational noise exposureChristina Hederstierna, Ulf RosenhallDr. Christina Hederstierna, Department of Audiology and Neurotology, Karolinska University Hospital, SE-171 76 Stockholm, Sweden. E-mail: christina.forshell-hederstierna@karolinska.se
1971 - Gerontological and Geriatric Population StudyARHL and NIHL Gothenburg Population cohortThis study was part of the Gothenburg Gerontological and Geriatric Investigation, and was supported by The Tysta Skolan Foundation and Hörselforskningsfonden.Prospective: 1Clinical : 2101370-75yrsBoth: 1Not statedBilateral:2NIHL: 5 , ARHL: 3 Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Unknown: 0
92
347GESA project - Multi Cohort study for psychiatric diseasesJuliane Burghardt, Ana Nanette Tibubos, Danielle Otten, Elmar Brähler, Harald Binder, Hans Grabe, Johannes Kruse, Karl Heinz Ladwig, Georg Schomerus, Philipp S Wild, Manfred E Beutel
Juliane.Burghardt@uni-hamburg.deGESA project - Multi Cohort study for psychiatric diseasesGERMANYPopulation cohort German Federal Ministry of Education and Research Other: 4CohortClinical : 240,00020-79Unspecified:0Unspecified:0No:2No:2No:2No:2No:2Self-reported hearing assessmentNo:2Unknown: 0
93
272Otological diagnoses and probable age-related auditory neuropathy in “younger” and “older” elderly personsUlf Rosenhall, Christina Hederstierna & Esma IdrizbegovicUlf Rosenhall, Department of Hearing and Balance, Karolinska University Hospital Solna, SE 17176 Stockholm, Sweden. ulf.rosenhall@karolinska.se1901-1922Gothenburg Gerontological and Geriatric investigationSNHL- ANSDSwedenPopulation cohortThe study is part of the Gothenburg Gerontological and Geriatric
investigation, and it was supported by the foundation Tysta Skolan		Retrospective: 2clinical : 272670, 75, 85Both: 1Both:3Cholesteatoma: 2 , ARHL: 3 , NIHL: 5 , Other:6 Conductive HL and Mixed HLYes:1Yes:1No:2No:2No:2SiN test was monosyllabicNo:2No:2No:2No:2No:2The study, based on identifi able otological and audiological diagnoses in old age, showed marked differences between ‘ younger ’ (70 – 75 years) and ‘ older ’ (85 years) elderly persons. The incidence of probable age-related auditory neuropathy increased markedly from 70 – 75 year to 85 years of age. Bilateral deafness was not seen in the 70 – 75-year cohorts, only in the 85-year cohort. Conductive hearing impairments were more common in the older subgroup than in the younger one.No:2Unknown: 0
94
69Auditory Function in 70- and 75-Year-Olds of Four Age CohortsR Jönsson, U Rosenhall, I Gause-Nilsson and B SteenRadi Jo¨nsson, Department of Audiology, Sahlgrenska University Hospital, S-413 45 Go¨teborg, Sweden. (Fax. +46 31 829811)1922-1992Gottenburgh 473 elderly personsSNHL - ARHLSwedenSNHL - ARHLThe studies was funded by grants from: Hjalmar
Svensson’s Foundation; Ho¨rselskadades Riksfo¨rbund; the
Swedish Ministry of Health and Social Affairs, Commission
for Social Research; the Swedish Medical Research Council; the
Go¨teborg Medical Services Administration; the Go¨teborg
Administration of Social Services and Wilhelm and Martina
Lundgren’s Foundation. 		Prospective: 1Clinical : 284070/75Both: 1SwedishUnspecified:0ARHL: 3 Yes:1Yes:1This cross-sectional and time-lag study of 70- and 75- year-old men and women, representative of an urban population, demonstrates a lack of consistent changes in auditory function over the past two decades. This implies that presbyacusis in elderly people living in industrialized countries is a relatively age-stable functional deficit. Hearing thresholds in the high frequency range in men, a probable sign of exposure to occupational and leisure noise, did not show any definite improvement between 1971–72 and 1992.No:2Unknown: 0
95
275Detection of deafness-causing mutations in the Greek mitochondrial genomeHaris Kokotasa, Maria Grigoriadoua, George S. Korresa, Elisabeth Ferekidoua, Dimitrios Kandilorosb,
Stavros Korresc and Michael B. Petersena		Haris Kokotas, Department of Genetics,
Institute of Child Health, ‘Aghia Sophia’ Children’s Hospital, Athens
11527, Greece. Tel.: +30 213 2037 333; Fax: +30 210 7700 111; hkokotas@yahoo.gr.		1999 - 2010Greek 513 patients suffering from childhood onset prelingual or postlingual, bilateral, sensorineural, syndromic or non-syndromic hearing loss of any degreeSNHL - GeneticGreeceSNHL - UndifferentiatedThis study was
supported in part by a grant from Oticon Fonden, Denmark
(MBP)		Other: 4case-controlBasic Science: 1513Both: 1Bilateral:2SNHL: 1Yes:1No:2No:2No:2No:2No:2No:2No:2No:2No:2No:2Yes:1MTRNR1, MTRNR1, MTTL1, MTTS1Childhood onset hearing impairment1999 - 2010mtDNA mutations are not a major risk factor for sensorineural deafness in the Greek
population.		No:2Unknown: 0
96
343Growing Up in ScotlandPI: Paul Bradshawgus@scotcen.org.uk.2005-Growing Up in ScotlandScotlandPopulation cohortScottish Government Other: 4CohortClinical : 214,202all agesUnspecified:0Unspecified:0No:2No:2No:2No:2No:2parent/self reported hearingYes:1Mental health, Cognitive functionNo:2Anthropometric measurements,
Lifestyle: Smoking, Physical activity, Dietary habits, Alcohol
Socio-economic: Occupation, Finances, Family circumstances, Housing, Education, Ethnic group, Marital status, Social support		Unknown: 0
97
339Gutenberg Health StudyPrincipal Investigator: Philipp Wildinfo@ghs-mainz.de2007-Gutenberg Health StudyMainz (Germany)Population cohortGovernment of Rheinland-Pfalz , the research programs “Wissen schafft Zukunft” and “Center for Translational Vascular Biology (CTVB)” of the Johannes Gutenberg-University of Mainz, and its contract with Boehringer Ingelheim, PHILIPS Medical Systems and Novartis Pharma, including an unrestricted grant for the Gutenberg Health StudyOther: 4CohortClinical : 21500035-74Unspecified:0Unspecified:0No:2No:2No:2No:2No:2Self reported hearing assessmentYes:1
electrocardiogram, carotid US, echocardiography
Yes:1Blood , urine testsYes:1blood pressure and heart rate, Sociodemography, medical history, cancer screening, Gender issues, Family history, children health behavior, Hobbies and leisure behavior, Smoking, secondhand smoke, alcohol consumption, Professional history, Fine dust and noise pollution, Life satisfaction and environmental factors, Home environment
Medical examinations: Spirometry, Measurement of carbon monoxide in the alveolar air
Tooth pocket swab
- Simultaneous determination of flow-mediated vasodilation and arterial stiffness by measuring the reactivity of the brachial artery using ultrasound, volume plethysmography of the digital artery using Endo-PAT and
digital photoplethysmographic pulse curve analysis
- Neurocardial regulation
ankle brachial index
- Acquisition of the current weather data
- anthropometry

Ophthalmic examination
- Visual quality of life
- NutritionPatient Health Questionnaire (PHQ-9), Generalized Anxiety Disorder [GAD]-7 Scale		Yes:1Unknown: 0
98
190Improved hearing in Swedish 70-year olds—a cohort comparison over more than four decades (1971–2014)M Hoff, T Tengstrand, A Sadeghi, I Skoog and U Rosenhallmaria.hoff@gu.se1970 - unspecifiedH70 frameworkSNHL - ARHLSwedenPopulation cohortThis project was supported by grants from the Foundation
Agneta Prytz-Folke and Gösta Folke [2013-0613]; the
Foundation Tysta Skolan [2015-0601]; Göteborgs Läkaresällskap [2014-1101]; Hörselforskningsfonden [2015-484]; Region Västra Götaland [2014-0601]; Swedish Research Council [2012-5041, 2015-02830 and 2013-8717]; Swedish Research Council for Health, Working Life and Welfare [2013-1202]; AGECAP [2013-2300, 2013-2496 and 2013- 0475]; University of Gothenburg UGOT Challenge; Sahlgrenska University Hospital (ALF); Stena Foundation;
Konung Gustaf V:s och Drottning Victorias Frimurarestiftelse; Swedish Alzheimer’s Foundation; Hjärnfonden; Eivind och Elsa K:son Sylvans stiftelse; Stiftelsen Söderström-Königska Sjukhemmet; Magnus Bergvalls stiftelse; Stiftelsen för Gamla Tjänarinnor; and Handlanden Hjalmar Svenssons Forskningsfond		Prospective: 1Clinical : 21,135>70Both: 1SwedishUnspecified:0Unspecified:0Yes:1Yes:1Unspecified: cognitive examinations and
interviews		No:2No:2Hearing acuity and prevalence of hearing loss in 70-year-old Swedes have improved over the last half-century. The most distinct improvements were seen in men and in the high frequencies. Although the reasons for the improvement were not investigated in this study, the nature of the findings suggests that noise-induced hearing impairment could be an important factor. The findings highlight the importance of modifiable risk factors in the prevention of ARHLNo:2Unknown: 0
99
277Genetic Characteristics in Children with Cochlear Implants and the Corresponding Auditory PerformanceChen-Chi Wu, Tien-Chen Liu, Shih-Hao Wang, Chuan-Jen Hsu, Che-Ming WuChuan-Jen Hsu, MD, Department of Otolaryngology, National Taiwan University Hospital, 7 Chung-Shan S. Road, Taipei, 100, Taiwan cjhsu@ntu.edu.tw2002 to 2010Han 743 unrelated chinese children with idiopathic sensorineural hearing impairmentSNHL - GeneticTaiwan SNHL - UndifferentiatedThis study was supported by research grants from the
National Science Council of the Executive Yuan of the
Republic of China (NSC- 97-2314-B-002-092-MY3) and
National Taiwan University Hospital (NTUH-098-
001156).		Other: 4case-controlclinical : 27437.1 6 4.8Both: 1Han ChineseUnspecified:0SNHL: 1yes:1Categories of Auditory Performance Scores (CAP)No:2Yes:1High Resolution Temporal Bone CT +/- MRINo:2No:2No:2No:2Yes:1GJB2, SLC26A4, 12S rRNA, OTOFidiopathic SNHL2002 - 2010A significant prevalence of genetic mutations was identified in children with CIs, suggesting the need for
routine genetic assessments. The frequencies of common deafness-associated mutations were different between children with and without CIs. The presence of genetic mutations was associated with an excellent long-term auditory performance outcome after implantation		No:2Unknown: 0
100
7Prevalence and range of GJB2 and SLC26A4 mutations in patients with autosomal recessive non‐syndromic hearing lossH. Jiang, J. Chen, X. Shan, Y. Li, J. He, B. Yangyangbb1959@sina.com UnspecifiedHangzhou 176 unrelated pediatric patients with ARNHSNHL - GeneticChinaSNHL - Genetic/HereditaryProspective: 1Basic Science: 117659y - 18Both: 1ChineseBilateral:2SNHL: 1Yes:1Yes:1Yes:1Yes:1Yes:1CT temporal bonesYes:1GJB2, SLC26A4, GJB3ARNHLNo:2Unknown: 0