Institute of Sensory Organs

Kajetany, Poland

Institute of Sensory Organs

Kajetany, Poland
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Skarzynski P.H.,Institute of Physiology and Pathology of Hearing | Skarzynski P.H.,Medical University of Warsaw | Skarzynski P.H.,Institute of Sensory Organs | Raj-Koziak D.,Institute of Physiology and Pathology of Hearing | And 2 more authors.
International Journal of Pediatric Otorhinolaryngology | Year: 2017

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

Aimoni C.,University of Ferrara | Ciorba A.,University of Ferrara | Cerritelli L.,University of Ferrara | Ceruti S.,University of Ferrara | And 5 more authors.
International Journal of Pediatric Otorhinolaryngology | Year: 2017

Background: Enlarged Vestibular Aqueduct (EVA) is one of the most common congenital malformations associated with sensorineural or mixed hearing loss. The association between hearing loss and EVA is described in syndromic (i.e. Pendred Syndrome, BOR, Waardenburg) and non-syndromic disorders, as isolate or familiar mutations of the SLC26A4 gene. The audiological phenotype of the EVA syndrome is heterogeneous, the type and entity of hearing loss may vary and vertigo episodes might also be present. Objective: The aim of this retrospective study was to describe the clinical and genetic features of a group of adolescent subjects presenting an EVA clinical profile, considering the presence of SLC26A4 gene mutations. Methods: 14 Caucasian patients were assessed (24 ears in total; 4 patients presented a monolateral EVA), 10 females and 4 males. Their age at the time of diagnosis was between 1 and 6 years (mean age 2.5 years). Subjects were assessed by an ENT microscopy evaluation with a complete audiometric assessment, CT & MRI scans and genetic tests for the evaluation of the pendrin gene mutations (SLC26A4). Results: Considering the presence of SLC26A4 mutations and thyroid function, we could identify three sub-groups of patients: group 1, non syndromic EVA (ns EVA, no SLC26A4 mutation and no thyroid dysfunction); group 2, EVA with DFNB4 (single SLC26A4 gene mutation and no thyroid dysfunction); group 3, EVA with Pendred Syndrome (two pathological mutation of SLC26A4 and thyromegaly with thyroid dysfunction).Patients of group 1 (ns-EVA) showed various degrees of hearing loss from mild (55%) to severe-profound (45%). In groups 2 (DFNB4) and 3 (PDS), the degree of hearing loss is severe to profound in 70-75% of the cases; middle and high frequencies are mainly involved. Conclusions: The phenotypic expressions associated with the EVA clinical profile are heterogeneous. From the available data, it was not possible to identify a representative audiological profile, in any of the three sub-groups. The data suggest that: (i) a later onset of hearing loss is usually related to EVA, in absence of SLC26A4 gene mutations; and (ii) hearing loss is more severe in patients with SLC26A4 gene mutations (groups 2 and 3 of this study). © 2017 Elsevier B.V.

Lazard D.S.,Bionics Institute | Vincent C.,Service dotologie et dotoneurologie | Venail F.,Montpellier University Hospital Center | van de Heyning P.,University of Antwerp | And 24 more authors.
PLoS ONE | Year: 2012

Objective: To test the influence of multiple factors on cochlear implant (CI) speech performance in quiet and in noise for postlinguistically deaf adults, and to design a model of predicted auditory performance with a CI as a function of the significant factors. Study Design: Retrospective multi-centre study. Methods: Data from 2251 patients implanted since 2003 in 15 international centres were collected. Speech scores in quiet and in noise were converted into percentile ranks to remove differences between centres. The influence of 15 pre-, per- and postoperative factors, such as the duration of moderate hearing loss (mHL), the surgical approach (cochleostomy or round window approach), the angle of insertion, the percentage of active electrodes, and the brand of device were tested. The usual factors, duration of profound HL (pHL), age, etiology, duration of CI experience, that are already known to have an influence, were included in the statistical analyses. Results: The significant factors were: the pure tone average threshold of the better ear, the brand of device, the percentage of active electrodes, the use of hearing aids (HAs) during the period of pHL, and the duration of mHL. Conclusions: A new model was designed showing a decrease of performance that started during the period of mHL, and became faster during the period of pHL. The use of bilateral HAs slowed down the related central reorganization that is the likely cause of the decreased performance. © 2012 Lazard et al.

Blamey P.,Bionics Institute | Blamey P.,University of Melbourne | Artieres F.,Institute Saint Pierre | Artieres F.,Montpellier University Hospital Center | And 29 more authors.
Audiology and Neurotology | Year: 2013

Objective: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome. Study Design: Retrospective multicenter study. Methods: Data from 2251 adult patients implanted since 2003 in 15 international centers were collected and speech scores in quiet were converted to percentile ranks to remove differences between centers. Results: The negative effect of long duration of severe to profound hearing loss was less important in the new data than in 1996; the effects of age at CI and age at onset of severe to profound hearing loss were delayed until older ages; etiology had a smaller effect, and the effect of CI experience was greater with a steeper learning curve. Patients with longer durations of severe to profound hearing loss were less likely to improve with CI experience than patients with shorter duration of severe to profound hearing loss. Conclusions: The factors that were relevant in 1996 were still relevant in 2011, although their relative importance had changed. Relaxed patient selection criteria, improved clinical management of hearing loss, modifications of surgical practice, and improved devices may explain the differences. © 2012 S. Karger AG, Basel.

Jedrzejczak W.W.,Institute of Physiology and Pathology of Hearing | Jedrzejczak W.W.,World Hearing Center | Kochanek K.,Institute of Physiology and Pathology of Hearing | Kochanek K.,World Hearing Center | And 8 more authors.
Ear and Hearing | Year: 2012

Objective: The aim of this study was to assess the diagnostic value of click-evoked otoacoustic emissions (CEOAEs) and tone-burst evoked otoacoustic emissions (TBOAEs) in identifying the residual hearing of subjects with partial deafness (PD)-a condition in which subjects have normal thresholds at low frequencies and severe-to-profound sensorineural hearing loss at high frequencies. Design: Otoacoustic emissions (OAEs) were measured in 23 subjects with severe-to-profound sensorineural hearing loss, 46 with PD, and 15 with normal hearing (NH). The PD subjects were divided into three subgroups according to the frequency at which hearing loss started: PD250 (NH up to 250 Hz)-20 ears; PD500 (NH to 500 Hz)-18 ears; and PD1000 (NH up to 1000 Hz)-20 ears. Standard-click stimuli, and 0.5- and 1-kHz tone bursts (average amplitude 80 ± 3 peak dB SPL, nonlinear averaging protocol), were used. The tone bursts were four cycles long with equal rise/fall times and no plateau. Recordings were performed in two acquisition windows: a standard one 20-msec wide for clicks and 1-kHz tone bursts, and one 30-msec wide for 0.5-kHz tone bursts. OAE response levels, signal-to-noise ratios, and reproducibility were examined in terms of wide-band responses and in terms of half-octave bands centered at 0.5 and 1 kHz. Receiver operator characteristic analysis was used to determine which type of stimuli best differentiates partially deaf subjects from subjects with severe-to-profound sensorineural hearing loss through the range 125 to 8000 Hz. Results: Nearly all recordings from groups PD500 and PD1000 showed 0.5-kHz TBOAEs. By contrast, 1-kHz TBOAEs and CEOAEs were generally found only in the PD1000 group. It was also possible to detect 0.5-kHz TBOAE responses in approximately 50% of ears from the PD250 group. Receiver operator characteristic analysis demonstrated that click and 1-kHz tone bursts provide a good diagnostic measure of residual hearing even when hearing loss starts as low as 1 kHz; moreover, the 0.5-kHz TBOAE could identify residual hearing when hearing loss started just > 0.5 kHz, a situation in which clicks failed to elicit a response. In the case of partially deaf subjects, diagnosis was more accurate when OAEs were analyzed by 1/2 octave bands. Furthermore, the use of a 0.5-kHz tone burst gave responses in the highest number of subjects, even when there were hearing losses in neighboring bands. Conclusions: The results of this study indicate that a 0.5-kHz TBOAE is a more powerful test than the standard CEOAE when cochlear function at low frequencies is of interest. The 0.5-kHz TBOAE may be used to identify partial deafness in patients who generally fail to show a response to the commonly used clicks. In addition, use of 1/2 octave-band filtering can increase the reproducibility and power of the test. Copyright © 2012 by Lippincott Williams & Wilkins • Printed in the U.S.A.

Skarzynski H.,Institute of Physiology and Pathology of Hearing | Skarzynski H.,World Hearing Center | Olszewski L.,Institute of Physiology and Pathology of Hearing | Olszewski L.,World Hearing Center | And 13 more authors.
European Archives of Oto-Rhino-Laryngology | Year: 2014

The objective of this study was to present 5 years of surgical experience, and the extended results of hearing preservation (based on 3-year follow-up), with the Med-El Vibrant Soundbridge (VSB) in which the floating mass transducer (FMT) is placed directly against the round window membrane, and the fascia is used only as covering tissue to keep it in position. A retrospective survey of surgical and audiological data was conducted to evaluate the performance and stability of patient hearing, with audiometric measurements performed over fixed time intervals up to 36 months. 21 patients, aged 19-62 years (mean 48.4), with mixed or conductive, bilateral or unilateral hearing loss were included in this study. Surgical intervention involved monaural implantation of the Med-El VSB between 2006 and 2009. The results were assessed using pure tone audiometry. In 5 years of experience with the technique, no significant complications or device extrusion were observed except for two revision surgeries requiring FMT repositioning. In the 3-year follow-up, we observed stable hearing in the implanted ear. It is concluded that direct round window stimulation without interposed fascia is an alternative for patients with hearing impairment caused by chronic otitis media and/or lack of ossicles, especially after modified radical mastoidectomy. It allows good results in a selected group of patients, although further observation on a larger population is needed to confirm long-term validity and effectiveness. © 2013 The Author(s).

Skarzynski H.,Institute of Physiology and Pathology of Hearing | Lorens A.,Institute of Physiology and Pathology of Hearing | Dziendziel B.,Institute of Physiology and Pathology of Hearing | Skarzynski P.H.,Institute of Physiology and Pathology of Hearing | And 2 more authors.
International Journal of Pediatric Otorhinolaryngology | Year: 2015

Background: Some adolescents have hearing impairments characterized by normal or slightly elevated thresholds in the low and mid-frequency bands (below 1500. Hz) and nearly total deafness in the high frequency range. These patients often remain beyond the scope of effective hearing aid treatment. Case report: This study presents the case of a 16-year-old adolescent with good hearing in the range 125-1500. Hz and deafness at other frequencies. An implant was used to restore hearing at high frequencies, while preserving low and mid frequency acoustic hearing in the implanted ear. This is described as electro-natural stimulation (ENS) of the inner ear. Conclusions: The results demonstrate that low and mid frequency hearing (up to 1500. Hz) can be preserved using the round window surgical technique. A substantial improvement in speech discrimination was also observed when electrical stimulation on one side was combined with acoustic stimulation on both sides. There is scope to extend qualifying criteria for cochlear implantation to include adolescents who are suited to ENS. © 2015 Elsevier Ireland Ltd.

PubMed | Institute of Sensory Organs, Institute of Physiology and Pathology of Hearing, Medical University of Warsaw, University of Warsaw and Medical University of Lublin
Type: Evaluation Studies | Journal: Annals of agricultural and environmental medicine : AAEM | Year: 2015

Hearing disorders among school-age children are a current concern. Continuing studies have been performed in Poland since 2008, and on 2 December 2011 the EU Council adopted Conclusions on the Early Detection and Treatment of Communication Disorders in Children, Including the Use of e-Health Tools and innovative Solutions. The discussion now focuses not only on the efficacy of hearing screening programmes in schoolchildren, but what should be its general aim and what tests it should include? This paper makes the case that it is important to include central auditory processing disorder (CAPD) tests. One such test is the dichotic digits test (DDT). The aim of the presented study was to evaluate the usefulness of the DDT in detecting central hearing disorders in school-age children.During hearing screening programmes conducted in Poland in 2008-2010, exactly 235,664 children (7-12-years-old) were screened in 9,325 schools. Of this number, 7,642 were examined using the DDT test for CAPD. Screening programmes were conducted using the Sense Examination Platform.With the cut-off criterion set at the 5th percentile, results for the DDT applied in a divided attention mode were 11.4% positive for 7-year-olds and 11.3% for 12-year-olds. In the focused attention mode, the comparable result for 12-year-olds was 9.7%. There was a clear right ear advantage. In children with positive DDT results, a higher incidence of other disorders, such as dyslexia, was observed.A test for CAPD should be included in the hearing screening of school-age children. The results of this study form the basis for developing Polish standards in this area.

Skarzynski H.,Speech of the Institute of Physiology | Lorens A.,Speech of the Institute of Physiology | Piotrowska A.,Speech of the Institute of Physiology | Skarzynski P.H.,Speech of the Institute of Physiology | Skarzynski P.H.,Institute of Sensory Organs
Medical Science Monitor | Year: 2010

Background: Partial deafness (PD) is a condition in which hearing loss occurs in at least 1 frequency critical to speech understanding. Current options for partial deafness treatment (PDT) rely on preoperative hearing preservation, which, along with the use of different means of acoustic and electric stimulation, enable extending the indications for various assistive hearing devices. Possible solutions include acoustic methods only, the use of hearing aids or middle ear implants, electric complementation, and a combination of electric and acoustic stimulation. Material/Methods: A total of 95 patients (63 adults, 32 children) with different types of PD were treated using selected types of electrodes and the optimal "round window" approach to the inner ear, with at least 36 months of observation. Results: The extension of PDT indications created an opportunity for patients with different hearing impairments who obtained no benefit from a hearing aid and did not qualify for standard cochlear implant application. The authors' observations are based on the findings that preservation of preoperative hearing had been achieved in 97.1% of adult patients (8 years' observation) and in 100% of children (6 years' observation). Those results send the important message that PDT is feasible and effective. Conclusions: To allow comparison of PDT results from different studies, the authors developed the Skarzynski PDT classification system, which permits the comparison of postoperative results, including the degree of hearing preservation and, more importantly, the patient's understanding of speech after treatment. © Med Sci Monit 2010.

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