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1.
Eur Arch Otorhinolaryngol ; 281(4): 1781-1787, 2024 Apr.
Article in English | MEDLINE | ID: mdl-37943315

ABSTRACT

OBJECTIVES: To assess the prevalence of new hearing losses in patients with acute vestibular syndrome (AVS) and to start to evaluate its diagnostic value for the differentiation between peripheral and central causes. DESIGN: We performed a cross-sectional prospective study in AVS patients presenting to our Emergency Department (ED) from February 2015 to November 2020. All patients received an MRI, Head-impulse test, Nystagmus test and Test of skew ('HINTS'), caloric testing and a pure-tone audiometry. RESULTS: We assessed 71 AVS patients, 17 of whom had a central and 54 a peripheral cause of dizziness. 12.7% had an objective hearing loss. 'HINTS' had an accuracy of 78.9% to diagnose stroke, whereas 'HINTS' plus audiometry 73.2%. 'HINTS' sensitivity was 82.4% and specificity 77.8% compared to 'HINTS' plus audiometry showing a sensitivity of 82.4% and specificity of 70.4%. The four patients with stroke and minor stroke had all central 'HINTS'. 55% of the patients did not perceive their new unilateral hearing loss. CONCLUSIONS: We found that almost one-eighth of the AVS patients had a new onset of hearing loss and only half had self-reported it. 'HINTS' plus audiometry proved to be less accurate to diagnose a central cause than 'HINTS' alone. Audiometry offered little diagnostic accuracy to detect strokes in the ED but might be useful to objectify a new hearing loss that was underestimated in the acute phase. Complete hearing loss should be considered a red flag, as three in four patients suffered from a central cause.


Subject(s)
Deafness , Hearing Loss , Nystagmus, Pathologic , Stroke , Humans , Cross-Sectional Studies , Prevalence , Prospective Studies , Vertigo/etiology , Nausea/complications , Vomiting/complications , Hearing Loss/diagnosis , Hearing Loss/epidemiology , Acute Disease , Stroke/complications , Nystagmus, Pathologic/diagnosis
2.
Audiol Res ; 13(5): 730-740, 2023 Oct 08.
Article in English | MEDLINE | ID: mdl-37887846

ABSTRACT

OBJECTIVE: The transmastoid plugging of a superior semicircular canal is considered a safe and effective technique for the management of superior semicircular canal dehiscence (SSCD). The aim of this meta-analysis is to assess the postoperative hearing outcomes after the transmastoid plugging of the superior semicircular canal. Search method and data sources: A systematic database search was performed on the following databases until 30 January 2023: MEDLINE, Embase, Cochrane Library, Web of Science, CINAHL, ICTRP, and clinicaltrials.gov. A systematic literature review and meta-analysis of the pooled data were conducted. We also included a consecutive case series with SCDS for those who underwent transmastoid plugging treatment at our clinic. RESULTS: We identified 643 citations and examined 358 full abstracts and 88 full manuscripts. A total of 16 studies were eligible for the systematic review and 11 studies for the meta-analysis. Furthermore, 159 ears (152 patients) were included. The postoperative mean air conduction threshold remained unchanged (mean difference, 2.89 dB; 95% CI: -0.05, 5.84 dB, p = 0.58), while the mean bone conduction threshold was significantly worse (mean difference, -3.53 dB; 95% CI, -6.1, -0.95 dB, p = 0.9). CONCLUSION: The transmastoid plugging technique for superior semicircular canal dehiscence syndrome, although minimally worsening the inner ear threshold, is a safe procedure in terms of hearing preservation and satisfactory symptom relief.

3.
Acta Otolaryngol ; 143(9): 735-741, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37897347

ABSTRACT

BACKGROUND: Vestibular function (VF) in patients with cochlear implantation (CI) performed during childhood is underinvestigated. OBJECTIVE: To study VF in patients receiving CI during childhood. MATERIAL & METHODS: Sixty patients (22 females) from 7-34 years old, unilaterally (n = 21) and bilaterally (n = 39) implanted, were included. Deafness was congenital (n = 45), consequential to meningitis (n = 3), skull fracture (n = 1), perinatal CMV infection (n = 1), ototoxic drugs (n = 1), unknown etiology (n = 9). VF was measured between 1 to 22 years after implantation, including calorics, v-HIT, c-VEMPS. Dizziness handicap inventory (DHI), age at independent walking(IW), sport activities were also investigated. RESULTS: Nine CI-patients (15%) reported dizziness/vertigo either prior or months to years after surgery. Comparison between symptomatic (15%), asymptomatic (85%), uni-bilaterally CI-patients showed no significant difference on VF's impairment for calorics (p = .603) and v-HIT (p = 1). Symptoms were not related to vestibular impairment. Age at implantation (p = 0.956), uni- bilateral (p = .32), simultaneous versus sequential (p = .134) did not influence IW age. DHI showed a tendency for being symptomatic at higher implantation age. Interval between CI, IWage, current age between surgery and vestibular evaluation did not have a significant effect on symptomatology. CONCLUSION & SIGNIFICANCE: This first middle to long-term evaluation of the VF in CI-patients, implanted in childhood, pointed out that 85% of patients were asymptomatic, with a mean time of >10 years after surgery. Vestibular impairment and symptoms seem to be mainly due to the underlying inner ear's disease rather than surgery.


Subject(s)
Cochlear Implantation , Cochlear Implants , Female , Humans , Child , Adolescent , Young Adult , Adult , Cochlear Implantation/adverse effects , Cochlear Implants/adverse effects , Dizziness/diagnosis , Prospective Studies , Vertigo/etiology
4.
Trends Hear ; 27: 23312165231191382, 2023.
Article in English | MEDLINE | ID: mdl-37501653

ABSTRACT

Matrix sentence tests in noise can be challenging to the listener and time-consuming. A trade-off should be found between testing time, listener's comfort and the precision of the results. Here, a novel test procedure based on an updated maximum likelihood method was developed and implemented in a German matrix sentence test. It determines the parameters of the psychometric function (threshold, slope, and lapse-rate) without constantly challenging the listener at the intelligibility threshold. A so-called "credible interval" was used as a mid-run estimate of reliability and can be used as a termination criterion for the test. The procedure was evaluated and compared to a STAIRCASE procedure in a study with 20 cochlear implant patients and 20 normal hearing participants. The proposed procedure offers comparable accuracy and reliability to the reference method, but with a lower listening effort, as rated by the listeners (-1.8 points on a 10-point scale). Test duration can be reduced by 1.3 min on average when a credible interval of 2 dB is used as the termination criterion instead of testing 30 sentences. Particularly, normal hearing listeners and well performing, cochlear implant users can benefit from shorter test duration. Although the novel procedure was developed for a German test, it can easily be applied to tests in any other language.


Subject(s)
Speech Perception , Speech , Humans , Reproducibility of Results , Bayes Theorem , Signal-To-Noise Ratio , Patient-Centered Care , Speech Intelligibility
5.
Audiol Res ; 13(3): 459-465, 2023 Jun 07.
Article in English | MEDLINE | ID: mdl-37366686

ABSTRACT

BACKGROUND: With the advent of cochlear implants, tactile aids for the profoundly deaf became obsolete decades ago. Nevertheless, they might still be useful in rare cases. We report the case of a 25-year-old woman with Bosley-Salih-Alorainy Syndrome and bilateral cochlear aplasia. METHODS: After it was determined that cochlear or brainstem implants were not an option and tactile aids were not available anymore, a bone conduction device (BCD) on a softband was tried as a tactile aid. The usual retroauricular position and a second position close to the wrist, preferred by the patient, were compared. Sound detection thresholds were measured with and without the aid. Additionally, three bilaterally deaf adult cochlear implant users were tested under the same conditions. RESULTS: At 250-1000 Hz, sounds were perceived as vibrations above approximately 45-60 dB with the device at the wrist. Thresholds were approximately 10 dB poorer when placed retroauricularly. Differentiation between different sounds seemed difficult. Nevertheless, the patient uses the device and can perceive loud sounds. CONCLUSIONS: Cases where the use of tactile aids may make sense are probably very rare. The use of BCD, placed, e.g., at the wrist, may be useful, but sound perception is limited to low frequencies and relatively loud levels.

6.
Article in English | MEDLINE | ID: mdl-36901552

ABSTRACT

BACKGROUND: The aim of this study was to investigate real-life speech levels of health professionals during communication with older inpatients in small group settings. METHODS: This is a prospective observational study assessing group interactions between geriatric inpatients and health professionals in a geriatric rehabilitation unit of a tertiary university hospital (Bern, Switzerland). We measured speech levels of health professionals during three typical group interactions (discharge planning meeting (n = 21), chair exercise group (n = 5), and memory training group (n = 5)) with older inpatients. Speech levels were measured using the CESVA LF010 (CESVA instruments s.l.u., Barcelona, Spain). A threshold of <60 dBA was defined as a potentially inadequate speech level. RESULTS: Overall, mean talk time of recorded sessions was 23.2 (standard deviation 8.3) minutes. The mean proportion of talk time with potentially inadequate speech levels was 61.6% (sd 32.0%). The mean proportion of talk time with potentially inadequate speech levels was significantly higher in chair exercise groups (95.1% (sd 4.6%)) compared to discharge planning meetings (54.8% (sd 32.5%), p = 0.01) and memory training groups (56.3% (sd 25.4%), p = 0.01). CONCLUSIONS: Our data show that real-life speech level differs between various types of group settings and suggest potentially inadequate speech levels by healthcare professionals requiring further study.


Subject(s)
Inpatients , Speech , Humans , Aged , Health Personnel , Communication , Delivery of Health Care
7.
Eur Arch Otorhinolaryngol ; 280(8): 3585-3591, 2023 Aug.
Article in English | MEDLINE | ID: mdl-36692617

ABSTRACT

PURPOSE: The SAMBA 2 BB audio processor for the BONEBRIDGE bone conduction implant features a new automatic listening environment detection to focus on target speech and to reduce interfering speech and background noises. The aim of this study was to evaluate the audiological benefit of the SAMBA 2 BB (AP2) and to compare it with its predecessor SAMBA BB (AP1). METHODS: Prospective within-subject comparison study. We compared the aided sound field hearing thresholds, speech understanding in quiet (Freiburg monosyllables), and speech understanding in noise (Oldenburg sentence test) with the AP1 and AP2. Each audio processor was worn for 2 weeks before assessment and seven users with single-sided sensorineural deafness (SSD) participated in the study. For speech understanding in noise, two complex noise scenarios with multiple noise sources including single talker interfering speech were used. The first scenario included speech presented from the front (S0NMIX), while in the second scenario speech was presented from the side of the implanted ear (SIPSINMIX). In addition, subjective evaluation using the SSQ12, APSQ, and the BBSS questionnaires was performed. RESULTS: We found improved speech understanding in quiet with the AP2 compared to the AP1 aided condition (on average + 17%, p = 0.007). In both noise scenarios, the AP2 lead to improved speech reception thresholds by 1.2 dB (S0NMIX, p = 0.032) and 2.1 dB (SIPSINMIX, p = 0.048) compared to the AP1. The questionnaires revealed no statistically significant differences, except an improved APSQ usability score with the AP2. CONCLUSION: Clinicians can expect that patients with SSD will benefit from the SAMBA 2 BB by improved speech understanding in both quiet and in complex noise scenarios, when compared to the older SAMBA BB.


Subject(s)
Cochlear Implants , Deafness , Hearing Aids , Hearing Loss, Sensorineural , Speech Perception , Humans , Bone Conduction , Prospective Studies , Hearing , Hearing Loss, Sensorineural/surgery , Deafness/surgery
8.
HNO ; 70(12): 891-902, 2022 Dec.
Article in German | MEDLINE | ID: mdl-36269381

ABSTRACT

BACKGROUND: Perfect hearing is crucial to the practice of various professions, such as instrument makers, musicians, sound engineers, and other professions not related to music, such as sonar technicians. For people of these occupational groups, we propose the term "professional ear user" (PEU) in analogy to "professional voice user". PEUs have special requirements for their hearing health, as they have well-known above-average auditory perceptual abilities on which they are professionally dependent. OBJECTIVE: The purpose of this narrative review is to summarize selected aspects of the prevention, diagnosis, and treatment of ear disorders in PEUs. RESULTS AND CONCLUSION: Prevention of hearing disorders and other ear diseases includes protection from excessive sound levels, avoidance of ototoxins and nicotine, and a safe manner of cleaning the outer auditory canal. Diagnosing hearing disorders in PEUs can be challenging, since subclinical but relevant changes in hearing cannot be reliably objectified by conventional audiometric methods. Moreover, the fact that a PEU is affected by an ear disease may influence treatment decisions. Further, physicians must be vigilant for non-organic ear diseases in PEUs. Lastly, measures to promote comprehensive ear health in PEUs as part of an educational program and to maintain ear health by means of a specialized otolaryngology service are discussed. In contrast to existing concepts, we lay the attention on the entirety of occupational groups that are specifically dependent on their ear health in a professional setting. In this context, we suggest avoiding a sole focus on hearing disorders and their prevention, but rather encourage the maintenance of a comprehensive ear health.


Subject(s)
Ear Diseases , Hearing Loss, Noise-Induced , Music , Occupational Diseases , Humans , Hearing Loss, Noise-Induced/diagnosis , Occupational Diseases/diagnosis , Occupational Diseases/prevention & control , Audiometry
9.
Pediatr Blood Cancer ; 69(9): e29755, 2022 09.
Article in English | MEDLINE | ID: mdl-35723448

ABSTRACT

BACKGROUND: Hearing loss is a potential side effect from childhood cancer treatment. We described the severity of hearing loss assessed by audiometry in a representative national cohort of childhood cancer survivors (CCS) and identified clinical risk factors. PROCEDURE: We included all CCS from the Swiss Childhood Cancer Registry who were diagnosed ≤18 age and treated with platinum-based chemotherapy between 1990 and 2014. We extracted audiograms, treatment-related information, and demographic data from medical records. Two reviewers independently assessed the severity of hearing loss at latest follow-up using the Münster Ototoxicity Scale. We used ordered logistic regression to identify clinical risk factors for severity of hearing loss. RESULTS: We analyzed data from 270 CCS. Median time from cancer diagnosis to last audiogram was 5 years (interquartile range 2.5-8.1 years). We found 53 (20%) CCS with mild, 78 (29%) with moderate, and 75 (28%) with severe hearing loss. Higher severity grades were associated with (a) younger age at cancer diagnosis (odds ratio [OR] 5.4, 95% confidence interval [CI]: 2.5-12.0 for <5 years); (b) treatment in earlier years (OR 4.8, 95% CI: 2.1-11.0 for 1990-1995); (c) higher cumulative cisplatin doses (OR 13.5, 95% CI: 4.7-38.8 for >450 mg/m2 ); (d) concomitant cranial radiation therapy (CRT) (OR 4.4, 95% CI: 2.5-7.8); and (e) hematopoietic stem cell transplantation (HSCT) (OR 2.7, 95% CI: 1.0-7.2). CONCLUSION: Three of four CCS treated with platinum-based chemotherapy experienced some degree of hearing loss. We recommend closely monitoring patient's hearing function if treated at a young age with high cumulative cisplatin doses, and concomitant CRT as part of long-term care.


Subject(s)
Antineoplastic Agents , Cancer Survivors , Hearing Loss , Neoplasms , Antineoplastic Agents/adverse effects , Carboplatin , Child , Cisplatin , Hearing Loss/chemically induced , Hearing Loss/epidemiology , Humans , Neoplasms/therapy , Platinum/therapeutic use
10.
Eur Arch Otorhinolaryngol ; 279(2): 645-652, 2022 Feb.
Article in English | MEDLINE | ID: mdl-33616750

ABSTRACT

PURPOSE: In unilateral cochlear implant (CI) recipients, a contralateral routing of signals (CROS) device enables to receive auditory information from the unaided side. This study investigates the feasibility as well as subjective and objective benefits of using a CI processor as a CROS device in unilateral CI recipients. METHODS: This is a single-center, prospective cohort study. First, we tested the directionality of the CROS processor in an acoustic chamber. Second, we examined the difference of speech perception in quiet and in noise in ten unilateral CI recipients with and without the CROS processor. Third, subjective ratings with the CROS processor were evaluated according to the Client Oriented Scale of Improvement Questionnaire. RESULTS: There was a time delay between the two devices of 3 ms. Connection of the CROS processor led to a summation effect of 3 dB as well as a more constant amplification along all azimuths. Speech perception in quiet showed an increased word recognition score at 50 dB (mean improvement 7%). In noise, the head shadow effect could be mitigated with significant gain in speech perception (mean improvement 8.4 dB). This advantage was reversed in unfavorable listening situations, where the CROS device considerably amplified the noise (mean: - 4.8 dB). Subjectively, patients who did not normally wear a hearing aid on the non-CI side were satisfied with the CROS device. CONCLUSIONS: The connection and synchronization of a CI processor as a CROS device is technically feasible and the signal processing strategies of the device can be exploited. In contra-laterally unaided patients, a subjective benefit can be achieved when wearing the CROS processor.


Subject(s)
Cochlear Implantation , Cochlear Implants , Hearing Aids , Speech Perception , Humans , Prospective Studies
11.
Biomed Res Int ; 2021: 1518385, 2021.
Article in English | MEDLINE | ID: mdl-34722757

ABSTRACT

Bone-anchored hearing systems (BAHS) transmit sound via osseointegrated implants behind the ear. They are used to treat patients with conductive or mixed hearing loss, but speech understanding may be limited especially in users with substantial additional cochlear hearing losses. In recent years, BAHS with higher maximum power output (MPO) and more advanced digital processing including loudness compression have become available. These features may be useful to increase speech understanding in users with mixed hearing loss. We have tested the effect of 4 combinations of two different MPO levels (highest level available and level reduced by 12 dB) and two different compression thresholds (CT) levels (50 dB and 65 dB sound pressure level) in 12 adult BAHS users on speech understanding in quiet and in noise. We have found that speech understanding in quiet was not influenced significantly by any of the changes in these two fitting parameters. In contrast, in users with average bone-conduction (BC) threshold of 25 dB or more, speech understanding in noise was improved by +0.8 dB to +1.1 dB (p < 0.03) when using the higher MPO level. In this user group, there may be an additional, but very small benefit of +0.1 dB to +0.4 dB when using the lower rather than the higher CT value, but the difference was not statistically significant (p > 0.27). In users with better average BC thresholds than 25 dB, none of the improvement was statistically significant. Higher MPOs and possibly, to a lesser degree, lower CTs seem to be able to improve speech understanding in noise in users with higher BC thresholds, but even their combined effect seems to be limited.


Subject(s)
Bone Conduction/physiology , Hearing Aids/trends , Speech Perception/physiology , Adult , Aged , Auditory Threshold , Bone-Anchored Prosthesis , Cochlear Implants , Data Compression/methods , Electric Power Supplies , Female , Hearing/physiology , Hearing Loss/therapy , Hearing Loss, Sensorineural/therapy , Hearing Tests , Humans , Male , Middle Aged , Noise , Speech
12.
Rev Med Suisse ; 17(753): 1706-1709, 2021 Oct 06.
Article in French | MEDLINE | ID: mdl-34614312

ABSTRACT

Single sided deafness diminishes speech understanding in noise and sound localization and thereby globally auditory performance. Most patients also suffer from tinnitus and indicate reduced quality of life. Patients have the choice to adapt to the new situation without treatment, to restore pseudostereophonic hearing by contralateral routing of signal (CROS) hearing aids or to restore binaural hearing using a cochlear implant in the deaf ear. This article summarizes the physiological base of binaural hearing and treatment options for single sided deafness with a special emphasis on the cochlear implant.


La surdité unilatérale diminue la compréhension dans le bruit et la capacité de localiser les sources sonores affectant ainsi globalement la performance auditive. De plus, la qualité de vie est souvent impactée par la présence d'un acouphène dérangeant. Les patients ont le choix de s'adapter à la situation sans traitement ou de reconstituer une pseudo-stéréophonie à l'aide d'un appareillage qui transmet l'information auditive arrivant sur l'oreille sourde à l'oreille saine (Contralateral Routing of Signals (CROS)). L'implant cochléaire est une alternative récente qui permet de « réactiver ¼ l'oreille atteinte et de redonner ainsi une audition binaurale. Les différentes options de réhabilitation auditive en cas de surdité unilatérale en mettant l'accent sur l'implant cochléaire sont discutées à l'aide d'un cas clinique.


Subject(s)
Cochlear Implantation , Deafness , Humans , Quality of Life
13.
Environ Sci Pollut Res Int ; 28(37): 52093-52105, 2021 Oct.
Article in English | MEDLINE | ID: mdl-34002308

ABSTRACT

The Spisská Magura mountain range, located in the Middle Spis, is one of the regions in Slovakia most contaminated by heavy metals resulting from mining and smelting activities. Heavy metals and other potentially toxic elements have accumulated in mountain areas via atmospheric transport. The influence of the daily range size of the European hare on its contamination by heavy metals was investigated in three habitat types (forest, woodland edge, meadow) in the Spisská Magura mountain range in the West Carpathians. Individual hares (n = 21) were traced and located by GPS following snowfall. Pair samples of their faeces (n = 64) and food (n = 64) were collected from feeding sites. The maps created were used for determination of the size of the daily range as being small or large. All hares that have a small daily range avoid meadows and open spaces due to the higher predation risk. However, individuals with a large daily range feed in all habitats, including meadows. Hares with a small daily range in a forest habitat ingested higher amounts of bio-elements Ca, Cr, S, and Mn as well as higher amounts of heavy metals Ba and Pb than hares with a large daily range. Moreover, dominant hares with a small daily range, with access to abundant food sources in a forest habitat, may gradually take on higher levels of bio-elements including heavy metals that are present in their food source. In contrast, in the woodland edge, hares with a small daily range had a smaller concentration of Ca, Cr, Mn, S, Ba, and Pb compared to hares with a large daily range. Caecotrophy plays a very significant role as far as the intake of nutrients and other elements is concerned. We found significant dependence between concentrations of the elements Cr, S, Ba, Pb, and Cd in the food of European hares and in their faeces.


Subject(s)
Hares , Metals, Heavy , Animals , Ecosystem , Forests , Humans , Metals, Heavy/analysis , Mining
14.
Ear Hear ; 42(1): 214-222, 2021.
Article in English | MEDLINE | ID: mdl-32701730

ABSTRACT

OBJECTIVES: To compare the sound-source localization, discrimination, and tracking performance of bilateral cochlear implant users with omnidirectional (OMNI) and pinna-imitating (PI) microphone directionality modes. DESIGN: Twelve experienced bilateral cochlear implant users participated in the study. Their audio processors were fitted with two different programs featuring either the OMNI or PI mode. Each subject performed static and dynamic sound field spatial hearing tests in the horizontal plane. The static tests consisted of an absolute sound localization test and a minimum audible angle test, which was measured at eight azimuth directions. Dynamic sound tracking ability was evaluated by the subject correctly indicating the direction of a moving stimulus along two circular paths around the subject. RESULTS: PI mode led to statistically significant sound localization and discrimination improvements. For static sound localization, the greatest benefit was a reduction in the number of front-back confusions. The front-back confusion rate was reduced from 47% with OMNI mode to 35% with PI mode (p = 0.03). The ability to discriminate sound sources straight to the sides (90° and 270° angle) was only possible with PI mode. The averaged minimum audible angle value for the 90° and 270° angle positions decreased from a 75.5° to a 37.7° angle when PI mode was used (p < 0.001). Furthermore, a non-significant trend towards an improvement in the ability to track moving sound sources was observed for both trajectories tested (p = 0.34 and p = 0.27). CONCLUSIONS: Our results demonstrate that PI mode can lead to improved spatial hearing performance in bilateral cochlear implant users, mainly as a consequence of improved front-back discrimination with PI mode.


Subject(s)
Cochlear Implantation , Cochlear Implants , Hearing Aids , Sound Localization , Speech Perception , Humans
15.
Eur J Cancer ; 138: 212-224, 2020 10.
Article in English | MEDLINE | ID: mdl-32905960

ABSTRACT

BACKGROUND: Irreversible sensorineural hearing loss is a common side effect of platinum treatment with the potential to significantly impair the neurocognitive, social and educational development of childhood cancer survivors. Genetic association studies suggest a genetic predisposition for cisplatin-induced ototoxicity. Among other candidate genes, thiopurine methyltransferase (TPMT) is considered a critical gene for susceptibility to cisplatin-induced hearing loss in a pharmacogenetic guideline. The aim of this cross-sectional cohort study was to confirm the genetic associations in a large pan-European population and to evaluate the diagnostic accuracy of the genetic markers. METHODS: Eligibility criteria required patients to be aged less than 19 years at the start of chemotherapy, which had to include cisplatin and/or carboplatin. Patients were assigned to three phenotype categories: no, minor and clinically relevant hearing loss. Fourteen variants in eleven candidate genes (ABCC3, OTOS, TPMT, SLC22A2, NFE2L2, SLC16A5, LRP2, GSTP1, SOD2, WFS1 and ACYP2) were investigated. Multinomial logistic regression was performed to model the relationship between genetic predictors and platinum ototoxicity, adjusting for clinical risk factors. Additionally, measures of the diagnostic accuracy of the genetic markers were determined. RESULTS: 900 patients were included in this study. In the multinomial logistic regression, significant unique contributions were found from SLC22A2 rs316019, the age at the start of platinum treatment, cranial radiation and the interaction term [platinum compound]∗[cumulative dose of cisplatin]. The predictive performance of the genetic markers was poor compared with the clinical risk factors. CONCLUSIONS: PanCareLIFE is the largest study of cisplatin-induced ototoxicity to date and confirmed a role for the polyspecific organic cation transporter SLC22A2. However, the predictive value of the current genetic candidate markers for clinical use is negligible, which puts the value of clinical factors for risk assessment of cisplatin-induced ototoxicity back into the foreground.


Subject(s)
Antineoplastic Agents/adverse effects , Cancer Survivors , Carboplatin/adverse effects , Cisplatin/adverse effects , Hearing Loss, Sensorineural/genetics , Hearing/drug effects , Neoplasms/drug therapy , Organic Cation Transporter 2/genetics , Pharmacogenomic Variants , Polymorphism, Single Nucleotide , Adolescent , Age of Onset , Child , Child, Preschool , Cross-Sectional Studies , Europe , Female , Genetic Association Studies , Genetic Predisposition to Disease , Hearing Loss, Sensorineural/chemically induced , Hearing Loss, Sensorineural/physiopathology , Humans , Infant , Infant, Newborn , Male , Ototoxicity , Pharmacogenomic Testing , Prospective Studies , Retrospective Studies , Risk Assessment , Risk Factors
16.
Data Brief ; 32: 106227, 2020 Oct.
Article in English | MEDLINE | ID: mdl-32939381

ABSTRACT

Genetic association studies suggest a genetic predisposition for cisplatin-induced ototoxicity. Among other candidate genes, thiopurine methyltransferase (TPMT) is considered a critical gene for susceptibility to cisplatin-induced hearing loss in a pharmacogenetic guideline. The PanCareLIFE cross-sectional cohort study evaluated the genetic associations in a large pan-European population and assessed the diagnostic accuracy of the genetic markers. 1,112 pediatric cancer survivors who had provided biomaterial for genotyping were screened for participation in the pharmacogenetic association study. 900 participants qualified for inclusion. Based on the assessment of original audiograms, patients were assigned to three phenotype categories: no, minor, and clinically relevant hearing loss. Fourteen variants in eleven candidate genes (ABCC3, OTOS, TPMT, SLC22A2, NFE2L2, SLC16A5, LRP2, GSTP1, SOD2, WFS1, and ACYP2) were genotyped. The genotype and phenotype data represent a resource for conducting meta-analyses to derive a more precise pooled estimate of the effects of genes on the risk of hearing loss due to platinum treatment.

17.
Otol Neurotol ; 41(5): e580-e587, 2020 06.
Article in English | MEDLINE | ID: mdl-32221106

ABSTRACT

OBJECTIVES: Evaluation of foreign language acquisition at school in cochlear implant patients. STUDY DESIGN: Multicenter cohort study. SETTING: CI centers. PATIENTS: One hundred twenty-five CI users (10-18 yr) in the German-speaking part of Switzerland were enrolled. Demographic data were obtained by means of written questionnaires. German-speaking children with mainstream foreign language tuition (English and/or French) were enrolled for further testing. The control group of normal-hearing individuals was matched on age, class, and number of foreign language lessons attended. RESULTS: Overall, 100 questionnaires were returned. The 12 CI users without foreign language learning attended special schools. CI users who attended foreign language classes had better German speech comprehension compared with those without foreign language tuition (89 versus 51%; p < 0.05). Thirty-one CI users of different grades were further tested. All (10/10) CI 6th graders attained the school objectives for both English reading and listening skills. French performance at 6th grade for reading was 3/7 and for listening only 1/7. There were 13 matched normal-hearing pairs for English and 10 for French. The total scores were on average 7% higher, with a statistical significance for English reading (p < 0.05). CONCLUSIONS: Almost 90% of CI children in Switzerland learn foreign language(s) at school. All the tested patients reached the current school objectives for English reading. The success rate for French was lower, especially regarding listening tasks. The 13 matched pairs with normal-hearing did not score substantially better.


Subject(s)
Cochlear Implantation , Cochlear Implants , Deafness , Speech Perception , Adolescent , Child , Cohort Studies , Deafness/surgery , Humans , Language , Language Development , Schools , Switzerland , Young Adult
18.
Acta Otolaryngol ; 140(3): 225-229, 2020 Mar.
Article in English | MEDLINE | ID: mdl-31825702

ABSTRACT

Background: Maximum power output (MPO) levels of bone anchored hearing systems (BAHS) vary between different devices, but are significantly lower than those of conventional hearing aids, potentially limiting speech understanding.Aims/objectives: To investigate, how MPO influences speech understanding in quiet and in noise in BAHS users.Materials and methods: 12 adult BAHS users with a bilateral conductive hearing loss and additional sensorineural hearing components between 4 and 45 dB (500-4000 Hz) participated in the study. Speech understanding was measured at 65 dB in quiet and in noise with 4 different MPO level settings, covering the approximate range of MPOs found in currently available BAHS devices.Results: Speech understanding in quiet and in noise decreased with increasing sensorineural hearing loss. MPOs levels did not influence speech understanding in quiet significantly. In contrast, speech understanding in noise was better with higher MPOs (average improvement in signal-to-noise ratio +3.2 dB, p < .001), but only if the sensorineural hearing loss component was above approximately 35 dB.Conclusions and significance: At normal conversational levels, higher MPOs have little or no influence on speech understanding in quiet with BAHS. However, they can improve speech understanding in noise for users with additional sensorineural hearing loss.


Subject(s)
Bone-Anchored Prosthesis , Hearing Aids , Hearing Loss, Mixed Conductive-Sensorineural/rehabilitation , Speech Perception , Adult , Aged , Auditory Threshold , Bone Conduction , Female , Hearing Tests , Humans , Linear Models , Male , Middle Aged , Noise , Prospective Studies
19.
Swiss Med Wkly ; 149: w20171, 2019 Dec 16.
Article in English | MEDLINE | ID: mdl-31880806

ABSTRACT

AIM OF THE STUDY: The aim of this multicentre, prospective, open, nonrandomised clinical trial was to demonstrate the clinical efficiency and outcomes of cochlear implants (CIs) in adult patients with post-lingual single-sided deafness (SSD). METHODS: A group of five left and five right SSD participants were investigated with various clinical tests and questionnaires before and 12 months after CI activation. Changes in hearing thresholds, speech understanding in noise, sound localisation, tinnitus (Tinnitus Handicap Inventory; THI), subjective hearing ability (Speech, Spatial and Qualities of Hearing Scale; SSQ), and quality of life (WHOQOL-BREF) were assessed. In addition, the pre- and postoperative results of the SSD patients were compared with an age- and gender-matched normal hearing control group. RESULTS: Surgery was uncomplicated in all patients. Two years after implantation, 9 of the 10 patients used their CI regularly for an average of more than 11 hours a day. A significant improvement in speech understanding in noise measured in the sound field using the Oldenburg sentence test could be demonstrated in the two situations in which patients with SSD experience the greatest difficulty: speech from the front and noise at the healthy ear, and speech to the implanted ear and noise from the front. The sound localisation test showed significant improvement of the mean localisation error and the root mean square error after CI activation. Furthermore, a significant reduction of the THI was measured, and the SSQ showed a significant improvement in the subscale speech comprehension and in the subscale spatial hearing. Also, quality of life measured with the WHOQOL-BREF showed a general improvement, which was significant in the global subscale. For this questionnaire, there was no significant difference between the normal-hearing control group and the patients after 12 months of CI use. CONCLUSION: This study confirmed the clinical benefit of cochlear implantation in patients with SSD. The significant improvement of speech understanding in noise, sound localisation, tinnitus perception, subjective hearing ability, and in particular the improved quality of life support the recommendation that patients with recently acquired SSD should be offered a CI. (Clinical trial registration number on clinicaltrial.gov: NCT01749592).


Subject(s)
Cochlear Implants/psychology , Hearing Loss, Bilateral/psychology , Hearing Loss, Bilateral/surgery , Quality of Life/psychology , Adult , Cochlear Implantation/methods , Female , Hearing , Humans , Male , Middle Aged , Prospective Studies , Speech , Surveys and Questionnaires
20.
Otol Neurotol ; 40(10): e962-e965, 2019 12.
Article in English | MEDLINE | ID: mdl-31577637

ABSTRACT

OBJECTIVE: To report an unusual case of musical ear syndrome, and to present a theoretical framework for this condition, merging information from the presented case and from former case reports. PATIENT: A 67-year-old semi-professional musician, who underwent bilateral cochlear implantation and experienced musical ear syndrome, i.e., hearing music, where none was present. INTERVENTIONS: Cochlear implantation with a bilateral cochlear implant, and cochlear explantation 17 months later. MAIN OUTCOME MEASURE: Report of presence or absence of musical ear syndrome by the patient. RESULTS: Musical ear syndrome started 1 day after implantation. It ceased immediately after cochlear explantation, but reappeared 3 months later. CONCLUSIONS: Several types of factors seem to determine whether a musical ear syndrome is present or not. We propose to differentiate between triggers, modifiers, and conditions, which determine a base vulnerability. Vulnerability seems to be increased by auditory deprivation and by habitual retrieval of music from memory. Cochlear implantation or explantation seems to act as triggers. The proposed framework may help to stimulate reporting of more potentially relevant factors in future case reports on musical ear syndrome, and ultimately to help to understand this condition better.


Subject(s)
Cochlear Implants/adverse effects , Illusions , Music , Postoperative Complications/etiology , Aged , Cochlear Implantation , Female , Hearing , Humans , Syndrome
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