von Hippel-Lindau disease: deafness due to a non-MRI-visible endolymphatic sac tumor despite targeted screening.

Abstract:

OBJECTIVE:Endolymphatic sac tumours (ELSTs) of the inner ear occur in 16% of patients with the hereditary tumor syndrome von Hippel-Lindau disease (vHL). ELSTs of all sizes can cause irreversible hearing loss which can, however, be prevented through early diagnosis and treatment. We aim to emphasize the challenges of prophylactic ELST screening and to explore the role of audiometry in pre-symptomatic ELST screening. DESIGN:For a period of 17 years our patient was screened for ELSTs with inner-ear MRI (magnetic resonance imaging), audiometry, and clinical interviews. STUDY SAMPLE:A male vHL patient who became deaf in one ear due to a radiologically undetectable ELST. RESULTS:Despite annual MRIs, the ELST was not visible until four months after onset of deafness when it appeared as a 1.4 × 1.4 mm tumor mass. Although his hearing was objectively within normal limits for the first 14 years, a distinct pattern of low-frequency hearing loss could retrospectively be seen at all audiometries. CONCLUSIONS:Audiometry is a candidate screening tool for detection of non-symptomatic pre-MRI-visible ELSTs, and we have initiated an international collaborative study to further determine its application. At present, we suggest an ELST screening protocol of yearly audiological assessment and inner ear MRI.

journal_name

Int J Audiol

authors

Binderup ML,Gimsing S,Kosteljanetz M,Thomsen C,Bisgaard ML

doi

10.3109/14992027.2013.824117

subject

Has Abstract

pub_date

2013-11-01 00:00:00

pages

771-5

issue

11

eissn

1499-2027

issn

1708-8186

journal_volume

52

pub_type

杂志文章
  • Adaptation of the STARR test for adult Italian population: A speech test for a realistic estimate in real-life listening conditions.

    abstract:OBJECTIVES:To introduce the Italian adaptation of the STARR test based on a roving-level adaptive method to mimic challenging real-life listening conditions for use in people with auditory prostheses. DESIGN:Normative data were collected and interlist-variability, as well as learning effects, were investigated using a...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.3109/14992027.2015.1124296

    authors: Dincer D'Alessandro H,Ballantyne D,De Seta E,Musacchio A,Mancini P

    更新日期:2016-01-01 00:00:00

  • Total leisure noise exposure and its association with hearing loss among adolescents.

    abstract:OBJECTIVE:To investigate total leisure noise exposure among adolescents and to assess its association with hearing. DESIGN:Based on self-reported time spent on 19 leisure activities and associated mean sound pressure levels reported in the literature, total leisure noise exposure was evaluated and compared to noise at...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.3109/14992027.2015.1030510

    authors: Dehnert K,Raab U,Perez-Alvarez C,Steffens T,Bolte G,Fromme H,Twardella D

    更新日期:2015-01-01 00:00:00

  • The effects of training format on earplug performance.

    abstract::This experiment investigated the effect of small-group versus individual hearing loss prevention (HLP) training on the attenuation performance of passive insert-type hearing protection devices (HPDs). A subject-fit (SF) methodology, which gave naive listeners access only to the instructions printed on the HPD product ...

    journal_title:International journal of audiology

    pub_type: 杂志文章,随机对照试验

    doi:10.1080/14992020701438805

    authors: Joseph A,Punch J,Stephenson M,Paneth N,Wolfe E,Murphy W

    更新日期:2007-10-01 00:00:00

  • A comparison of intra- versus post-operatively acquired electrically evoked compound action potentials.

    abstract::The objective of this study was to compare the electrically evoked compound action potentials, intra- versus post-operatively, in cochlear implant patients. In a prospective study twenty-five consecutively implanted adult patients received a multichannel cochlear implant. In all patients, electrically evoked compound ...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020600833189

    authors: van Wermeskerken GK,van Olphen AF,van Zanten GA

    更新日期:2006-10-01 00:00:00

  • Dynamics of real time DPOAE contralateral suppression in chinchillas and humans.

    abstract::The dynamics of contralateral acoustic suppression were studied using real time (millisecond resolution) distortion product otoacoustic emissions (DPOAEs) in chinchillas and humans. Latency of DPOAE suppression onset is 26 ms in chinchillas and 45 ms in humans. After onset, suppression builds over time before tending ...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020400029996

    authors: James AL,Harrison RV,Pienkowski M,Dajani HR,Mount RJ

    更新日期:2005-02-01 00:00:00

  • The effects of 'supra-physiological' vitamin B12 administration on temporary threshold shift.

    abstract::The aim of this study was to evaluate, in 20 young volunteer subjects, the effects of supra-physiological vitamin B12 administration on noise-induced temporary threshold shift (TTS). All subjects had hearing thresholds within 15 dBHL and type A tympanograms. The subjects were randomly assigned to two different groups....

    journal_title:International journal of audiology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1080/14992020400050022

    authors: Quaranta A,Scaringi A,Bartoli R,Margarito MA,Quaranta N

    更新日期:2004-03-01 00:00:00

  • Paediatric hearing aid management: a demonstration project for using virtual visits to enhance parent support.

    abstract:OBJECTIVE:The purpose of this study was to explore the use of virtual visits to monitor hearing aid use with data logging measurements and provide parent support for hearing aid management. DESIGN:A 6-month longitudinal case study design was used. STUDY SAMPLE:Four families and two providers participated. RESULTS:Av...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2016.1226521

    authors: Muñoz K,Kibbe K,Preston E,Caballero A,Nelson L,White K,Twohig M

    更新日期:2017-02-01 00:00:00

  • Distortion product otoacoustic emissions: an objective technique for the screening of hearing loss in children treated with platin derivatives.

    abstract::In order to develop a sensitive audiometric protocol for identifying ototoxicity in children, a retrospective study of 16 children treated with cisplatin and/or carboplatin was performed. Audiometric testing was done by means of pure-tone threshold audiometry (PTA), high-frequency audiometry (HFA), and distortion prod...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020600582117

    authors: Dhooge I,Dhooge C,Geukens S,De Clerck B,De Vel E,Vinck BM

    更新日期:2006-06-01 00:00:00

  • The Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor: multicentric evaluation of outcomes in adults and children.

    abstract::Objective: This study evaluated the outcomes of the Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor.Design: Neuro One users were upgraded to Neuro 2. Monosyllabic word identification was evaluated in adults with Neuro One after ≥5 months, with Neuro 2 at upgrade, and with Neuro 2 after 3 mont...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2019.1671616

    authors: Franco-Vidal V,Parietti-Winkler C,Guevara N,Truy E,Loundon N,Bailleux S,Ardoint M,Saaï S,Hoen M,Laplante-Lévesque A,Mosnier I,Bordure P,Vincent C

    更新日期:2020-02-01 00:00:00

  • A qualitative review of parents' perspectives on the value of CAEP recording in influencing their acceptance of hearing devices for their child.

    abstract::Objective: To obtain a parental perspective on how audiological tests, including recording cortical auditory-evoked potentials (CAEP) to speech sounds, influenced their uptake of hearing devices for their infant. Design: A focus group was established by parents of hearing-impaired children. A facilitator explored how ...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2019.1592250

    authors: Mehta K,Mahon M,Watkin P,Marriage J,Vickers D

    更新日期:2019-07-01 00:00:00

  • Quality of life in persons with hearing loss: a study of patients referred to an audiological service.

    abstract::Objective: To investigate the relationship between hearing loss (HL) and general quality of life (QoL) in adults seeking hearing aids (HAs). Design: The patients completed the European Organisation for Research and Treatment of Cancer (EORTC) Quality of Life Questionnaire's general part and a questionnaire measuring s...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2019.1627010

    authors: Nordvik Ø,Heggdal POL,Brännström JK,Hjermstad MJ,Aarstad AK,Aarstad HJ

    更新日期:2019-11-01 00:00:00

  • Patient-centred audiological rehabilitation: perspectives of older adults who own hearing aids.

    abstract:OBJECTIVE:Patient-centred care is a term frequently associated with quality health care. Despite extensive literature from a range of health-care professions that provide description and measurement of patient-centred care, a definition of patient-centredness in audiological rehabilitation is lacking. The current study...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.3109/14992027.2013.866280

    authors: Grenness C,Hickson L,Laplante-Lévesque A,Davidson B

    更新日期:2014-02-01 00:00:00

  • Robust relationship between reading span and speech recognition in noise.

    abstract:OBJECTIVE:Working memory refers to a cognitive system that manages information processing and temporary storage. Recent work has demonstrated that individual differences in working memory capacity measured using a reading span task are related to ability to recognize speech in noise. In this project, we investigated wh...

    journal_title:International journal of audiology

    pub_type: 杂志文章,多中心研究

    doi:10.3109/14992027.2015.1043062

    authors: Souza P,Arehart K

    更新日期:2015-01-01 00:00:00

  • The protective role of tiopronin in cisplatin ototoxicity in Wistar rats.

    abstract::The purpose of this study was to evaluate cisplatin-induced ototoxicity and the protective effects of tiopronin. Twenty-four adult Wistar rats served as subjects and were divided into three groups. Eight rats receiving only saline (group A) were used as controls. Eight rats received cisplatin (2 mg/kg) injections (gro...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020400050059

    authors: Fetoni AR,Quaranta N,Marchese R,Cadoni G,Paludetti G,Sergi B

    更新日期:2004-09-01 00:00:00

  • Prevalence of labyrinthine ossification in CT and MR imaging of patients with acute deafness to severe sensorineural hearing loss.

    abstract:OBJECTIVE:To evaluate the prevalence of labyrinthine ossification, and especially cochlear ossification, in a cohort of patients with unilateral sudden deafness or severe sensorineural hearing loss. DESIGN:Retrospective data collection. STUDY SAMPLE:Sixty-four consecutive patients with unilateral sudden deafness or s...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.3109/14992027.2013.786191

    authors: Braun T,Dirr F,Berghaus A,Hempel JM,Krause E,Müller J,Ertl-Wagner B

    更新日期:2013-07-01 00:00:00

  • Spoken language and everyday functioning in 5-year-old children using hearing aids or cochlear implants.

    abstract:OBJECTIVE:This study investigated the factors influencing 5-year language, speech and everyday functioning of children with congenital hearing loss. DESIGN:Standardised tests including PLS-4, PPVT-4 and DEAP were directly administered to children. Parent reports on language (CDI) and everyday functioning (PEACH) were ...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2017.1370140

    authors: Cupples L,Ching TY,Button L,Seeto M,Zhang V,Whitfield J,Gunnourie M,Martin L,Marnane V

    更新日期:2018-05-01 00:00:00

  • Measurement of pitch perception as a function of cochlear implant electrode and its effect on speech perception with different frequency allocations.

    abstract:OBJECTIVE:An experiment was conducted to investigate the possibility that speech perception could be improved for some cochlear implant (CI) users by adjustment of the frequency allocation to the electrodes, following assessment of pitch perception along the electrode array. STUDY SAMPLE:Thirteen adult CI users with M...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2018.1516048

    authors: Grasmeder ML,Verschuur CA,van Besouw RM,Wheatley AMH,Newman TA

    更新日期:2019-03-01 00:00:00

  • Decision-making and outcomes of hearing help-seekers: A self-determination theory perspective.

    abstract:OBJECTIVE:To explore the explanatory power of a self-determination theory (SDT) model of health behaviour change for hearing aid adoption decisions and fitting outcomes. DESIGN:A quantitative approach was taken for this longitudinal cohort study. Participants completed questionnaires adapted from SDT that measured aut...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.3109/14992027.2015.1120893

    authors: Ridgway J,Hickson L,Lind C

    更新日期:2016-07-01 00:00:00

  • Hearing among 75-year-old people in three Nordic localities: a comparative study.

    abstract::The aim of this study was to compare auditory functions and to analyse the prevalence of hearing impairment and the relationship of self-reported hearing disability with audiometric test results among 75-year-old people in three Nordic localities. The representative samples came from Glostrup, Denmark (n = 571), Göteb...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020500189112

    authors: Hietanen A,Era P,Henrichsen J,Rosenhall U,Sorri M,Heikkinen E

    更新日期:2005-09-01 00:00:00

  • Global and regional needs, unmet needs and access to hearing aids.

    abstract::Objectives: This article aims to estimate the global and regional needs, unmet needs and access to hearing aids, as well as the morbidity that can be averted by their use.Design: The study was based on Global Burden of Disease data. Study sample: Hearing impaired individuals "in need" of a hearing aid were defined as ...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2020.1721577

    authors: Orji A,Kamenov K,Dirac M,Davis A,Chadha S,Vos T

    更新日期:2020-03-01 00:00:00

  • The viability of speech-in-noise audiometric screening using domestic audio equipment.

    abstract::Speech-in-noise audiometry has potential application as a low-cost, self-screening test for sensorineural hearing loss. To realize this potential, the influence of variations in audio equipment and listening environment need assessment. The present study assessed: 1) the frequency response and distortion produced by a...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020500267017

    authors: Culling JF,Zhao F,Stephens D

    更新日期:2005-12-01 00:00:00

  • Impacts of face coverings on communication: an indirect impact of COVID-19.

    abstract:OBJECTIVE:To understand the impact of face coverings on hearing and communication. DESIGN:An online survey consisting of closed-set and open-ended questions distributed within the UK to gain insights into experiences of interactions involving face coverings, and of the impact of face coverings on communication. SAMPL...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2020.1851401

    authors: Saunders GH,Jackson IR,Visram AS

    更新日期:2020-11-27 00:00:00

  • The relationship between pre-fitting expectations and willingness to use hearing aids.

    abstract::Hearing-aid use in the elderly population is problematic since not all people who would benefit from hearing aids actually obtain one. In this study, the relationship between pre-fitting expectations and willingness to use hearing aids is addressed. One hundred adult hearing aid candidates (mean age 68.6 years) were r...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020701843111

    authors: Meister H,Walger M,Brehmer D,von Wedel UC,von Wedel H

    更新日期:2008-04-01 00:00:00

  • The effect of hearing loss configuration on cochlear implantation uptake rates: an Australian experience.

    abstract::Objective: Recent changes to cochlear implant (CI) candidacy criteria have led to the inclusion of candidates with greater levels of hearing in the contralateral and/or implanted ear. This study assessed the impact of various hearing loss configurations on CI uptake rates (those assessed as eligible for CI, who procee...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992027.2020.1768445

    authors: Sucher CM,Eikelboom RH,Stegeman I,Jayakody DMP,Atlas MD

    更新日期:2020-11-01 00:00:00

  • A comparative study of hearing aids and round window application of the vibrant sound bridge (VSB) for patients with mixed or conductive hearing loss.

    abstract:OBJECTIVE:This study was undertaken to determine the efficacy of the round window (RW) application of the vibrant soundbridge (VSB) in patients with mixed or conductive hearing loss. DESIGN:Speech in quiet and in noise were compared to preoperative data attained with conventional hearing aids so that each subject serv...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.3109/14992027.2012.750431

    authors: Marino R,Linton N,Eikelboom RH,Statham E,Rajan GP

    更新日期:2013-04-01 00:00:00

  • Effects of FM-receiver gain on speech-recognition performance of adults with cochlear implants.

    abstract::The primary aim of this study was to examine the effects of frequency-modulated (FM) settings on speech-recognition performance in noise of listeners using two different cochlear implant speech processors. Participants included eight users of ESPrit 3G speech processors and nine users of Auria speech processors. A mod...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020802572635

    authors: Schafer EC,Wolfe J,Lawless T,Stout B

    更新日期:2009-04-01 00:00:00

  • Protocol for the provision of amplification within the Ontario infant hearing program.

    abstract::This document describes the protocol for the provision of amplification to infants and preschool children registered with the Ontario infant hearing program (OIHP) in Canada. The provision of amplification includes the prescription and dispensing of hearing instruments to infants and preschool children identified with...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.3109/14992020903080751

    authors: Bagatto M,Scollie SD,Hyde M,Seewald R

    更新日期:2010-01-01 00:00:00

  • A questionnaire study of the quality of life and quality of family life of individuals complaining of tinnitus pre- and post-attendance at a tinnitus clinic.

    abstract::Tinnitus is a condition that is difficult to treat, and treatment outcomes are difficult to measure The majority of people who experience tinnitus are not troubled by it; however, the troubled minority are referred by an otorhinolaryngologist for specialist clinic help. The aim of this study was to investigate how the...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020400050052

    authors: El Refaie A,Davis A,Kayan A,Baskill J,Lovell E,Owen V

    更新日期:2004-07-01 00:00:00

  • Effect of inverting electrode on mismatch negativity presence for perceptible/imperceptible tonal frequency contrasts.

    abstract::The effect of inverting electrode location and stimulus perceptibility on the presence of the mismatch negativity (MMN) evoked response was investigated in 20 normal-hearing adult females. Perceptible and imperceptible frequency contrasts were employed in an oddball paradigm. The perceptible contrast consisted of an 1...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.1080/14992020802283241

    authors: Walker-Black L,Stuart A

    更新日期:2008-11-01 00:00:00

  • On the relationship between functional hearing and depression.

    abstract:OBJECTIVE:To establish the effect of self-rated and measured functional hearing on depression, taking age and gender into account. Additionally, the study investigates if hearing-aid usage mitigates the effect, and if other physical health problems and social engagement confound it. DESIGN:Cross-sectional data from th...

    journal_title:International journal of audiology

    pub_type: 杂志文章

    doi:10.3109/14992027.2015.1046503

    authors: Keidser G,Seeto M,Rudner M,Hygge S,Rönnberg J

    更新日期:2015-01-01 00:00:00