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1.
Front Surg ; 9: 823219, 2022.
Article in English | MEDLINE | ID: mdl-35402479

ABSTRACT

Background and Objective: The cochlear implant (CI) electrode insertion process is a key step in CI surgery. One of the aims of advances in robotic-assisted CI surgery (RACIS) is to realize better cochlear structure preservation and to precisely control insertion. The aim of this literature review is to gain insight into electrode selection for RACIS by acquiring a thorough knowledge of electrode insertion and related complications from classic CI surgery involving a manual electrode insertion process. Methods: A systematic electronic search of the literature was carried out using PubMed, Scopus, Cochrane, and Web of Science to find relevant literature on electrode tip fold over (ETFO), electrode scalar deviation (ESD), and electrode migration (EM) from both pre-shaped and straight electrode types. Results: A total of 82 studies that include 8,603 ears implanted with a CI, i.e., pre-shaped (4,869) and straight electrodes (3,734), were evaluated. The rate of ETFO (25 studies, 2,335 ears), ESD (39 studies, 3,073 ears), and EM (18 studies, 3,195 ears) was determined. An incidence rate (±95% CI) of 5.38% (4.4-6.6%) of ETFO, 28.6% (26.6-30.6%) of ESD, and 0.53% (0.2-1.1%) of EM is associated with pre-shaped electrodes, whereas with straight electrodes it was 0.51% (0.1-1.3%), 11% (9.2-13.0%), and 3.2% (2.5-3.95%), respectively. The differences between the pre-shaped and straight electrode types are highly significant (p < 0.001). Laboratory experiments show evidence that robotic insertions of electrodes are less traumatic than manual insertions. The influence of round window (RW) vs. cochleostomy (Coch) was not assessed. Conclusion: Considering the current electrode designs available and the reported incidence of insertion complications, the use of straight electrodes in RACIS and conventional CI surgery (and manual insertion) appears to be less traumatic to intracochlear structures compared with pre-shaped electrodes. However, EM of straight electrodes should be anticipated. RACIS has the potential to reduce these complications.

2.
Cancers (Basel) ; 13(20)2021 Oct 11.
Article in English | MEDLINE | ID: mdl-34680232

ABSTRACT

INTRODUCTION: Temporal bone paragangliomas are rare tumors with high vascularization and usually benign entity. A variety of modalities, including gross total resection, subtotal resection, conventional or stereotactic radiotherapy including gamma-knife, embolization, and wait-and-scan strategy can be considered. The aim of this study was to compare long-term outcomes of different primary treatment modalities in temporal bone paragangliomas. MATERIALS AND METHODS: Patients with temporal bone paragangliomas treated between 1976 and 2018 at a tertiary referral center were retrospectively analyzed in this study. Collected patient data of 42 years were analyzed and long-term results including interdisciplinary management were assessed. Patient outcomes were compared within the different therapy modalities according to tumor control rate and complications. Clinical characteristics, radiological imaging, tumor extent and location (according to Fisch classification), symptoms, and follow-up were evaluated and a descriptive analysis for each treatment modality was performed. Tumor recurrence or growth progression and respective cranial nerve function before and after therapy were described. RESULTS: A total of 59 patients were treated with a single or combined treatment modality and clinical follow-up was 7 (13) years (median, interquartile range). Of the included patients 45 (76%) were female and 14 (24%) male (ratio 3:1) with a patient age range from 18 to 83 years. Total resection was performed on 31 patients, while 14 patients underwent subtotal resection. Eleven patients were treated with conventional primary radiotherapy or gamma-knife radiosurgery. Pulsatile tinnitus (n = 17, 29%) and hearing impairment (n = 16, 27%) were the most common symptoms in our patient group. Permanent lower cranial nerve deficits were observed only in patients with large tumors (Fisch C and D, n = 14, 24%). Among the 45 patients who were treated surgically, 88% of patients with Fisch A and B paragangliomas had no recurrent disease, while no tumor growth was perceived in 83% of patients with Fisch C and D paragangliomas. CONCLUSION: In conclusion, we propose surgery as a treatment option for patients with small tumors, due to a high control rate and less cranial nerve deficits compared to larger tumors. Although patients with Fisch C and D temporal bone paraganglioma can be treated surgically, only subtotal resections are possible in many cases. Additionally, frequent occurrence of cranial nerve deficits in those patients and tumor growth progression in long-term follow-up examinations make a combination of the therapy modalities or a primary radiotherapy more suitable in larger tumors.

3.
Otol Neurotol ; 42(6): e648-e657, 2021 07 01.
Article in English | MEDLINE | ID: mdl-33710140

ABSTRACT

INTRODUCTION: Genetic hearing loss (HL) is often monogenic. Whereas more than half of autosomal recessive (AR) cases in Austria are caused by mutations in a single gene, no disproportionately frequent contributing genetic factor has been identified in cases of autosomal dominant (AD) HL. The genetic characterization of HL continues to improve diagnosis, genetic counseling, and lays a foundation for the development of personalized medicine approaches. METHODS: Diagnostic HL panel screening was performed in an Austrian multiplex family with AD HL, and segregation was tested with polymerase chain reaction and Sanger sequencing. In an independent approach, 18 unrelated patients with AD HL were screened for causative variants in all known HL genes to date and segregation was tested if additional family members were available. The pathogenicity of novel variants was assessed based on previous literature and bioinformatic tools such as prediction software and protein modeling. RESULTS: In six of the 19 families under study, candidate pathogenic variants were identified in MYO6, including three novel variants (p.Gln441Pro, p.Ser612Tyr, and p.Gln650ValfsTer7). Some patients carried more than one likely pathogenic variant in known deafness genes. CONCLUSION: These results suggest a potential high prevalence of MYO6 variants in Austrian cases of AD HL. The presence of multiple rare HL variants in some patients highlights the relevance of considering multiple-hit diagnoses for genetic counseling and targeted therapy design.


Subject(s)
Deafness , Hearing Loss , Austria/epidemiology , Humans , Mutation , Pedigree , Prevalence
4.
Laryngoscope ; 129(2): 477-481, 2019 02.
Article in English | MEDLINE | ID: mdl-30284273

ABSTRACT

OBJECTIVE: To assess the audiological and long-term medical and technical follow-up outcomes of an active middle ear implant. METHODS: This was a retrospective medical chart analysis of all patients provided with an active middle ear implant in a tertiary academic medical referral center between September 1, 1998, and July 31, 2015. Main outcome measures were medical and technical complications, revisions, reimplantations, explantations, coupling approaches, mean time of use, pre- and postoperative hearing thresholds, functional hearing gain across frequencies (250-4,000 Hz), and Freiburg monosyllablic word test at 65 dB. RESULTS: One hundred and three patients were identified. Fifteen were implanted bilaterally (n = 118 Vibrant Soundbridge devices [MED-EL, Innsbruck, Austria]). Seventy-seven devices were implanted for sensorineural and 41 for mixed and conductive hearing loss. Patients used the implant for 6.7 years (range 0.7 months-17.9 years) on average. Ninety-one patients (77.12%) were using the device at the end of the observation period. An overall complication rate of 16.1% was observed. The revision and explantation rates were higher for devices implanted between 2004 and 2006. The device failure rate was 3.4%. Audiological evaluation showed significant hearing gains for both hearing loss patient groups. CONCLUSION: This long-term follow-up reveals the reliability of the active middle ear implant in a single center. Overall complication rate and device failure rate are acceptable. The complication rate was higher during implementation of alternative coupling approaches. The audiological benefit was satisfactory in patients with all hearing loss types. The majority of implanted patients used the implant at the end of the observation period. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:477-481, 2019.


Subject(s)
Correction of Hearing Impairment/methods , Hearing Loss, Conductive/rehabilitation , Hearing Loss, Mixed Conductive-Sensorineural/rehabilitation , Hearing Loss, Sensorineural/rehabilitation , Ossicular Prosthesis/statistics & numerical data , Auditory Threshold/physiology , Female , Follow-Up Studies , Hearing/physiology , Hearing Loss, Conductive/physiopathology , Hearing Loss, Mixed Conductive-Sensorineural/physiopathology , Hearing Loss, Sensorineural/physiopathology , Humans , Male , Middle Aged , Postoperative Period , Retrospective Studies , Time Factors , Treatment Outcome
5.
Wien Klin Wochenschr ; 130(9-10): 299-306, 2018 May.
Article in English | MEDLINE | ID: mdl-28733840

ABSTRACT

BACKGROUND: Non-syndromic autosomal dominant hearing impairment is characteristically postlingual in onset. Genetic diagnostics are essential for genetic counselling, disease prognosis and understanding of the molecular mechanisms of disease. To date, 36 causative genes have been identified, many in only individual families. Gene selection for genetic screening by traditional methods and genetic diagnosis in autosomal dominant patients has therefore been fraught with difficulty. Whole-exome sequencing provides a powerful tool to analyze all protein-coding genomic regions in parallel, thus allowing the comprehensive screening of all known genes and associated alterations. METHODS: In this study, a previously undiagnosed late-onset progressive autosomal dominant hearing loss in an Austrian family was investigated by means of whole-exome sequencing. Results were confirmed by Sanger sequencing. RESULTS: A previously described c.151C>T missense (p.Pro51Ser) mutation in the LCCL (limulus factor C, cochlin, late gestation lung protein Lgl1) domain of the cochlin gene (COCH) was identified as causative and segregated with disease in five members of the family. Molecular diagnostics led to the decision to perform cochlear implantation in an index patient who subsequently showed excellent postoperative auditory performance. The c.151C>T mutation was not found in 18 screened Austrian families with autosomal dominant hearing loss but was represented alongside other known pathogenic mutant COCH alleles in the Genome Aggregation Database (gnomAD) in European populations. A combined allele frequency of 0.000128 implies an orphan disease frequency for COCH-induced hearing loss of 1:3900 in Europe. CONCLUSIONS: Exome sequencing successfully resolved the genetic diagnosis in a family suffering from autosomal dominant hearing impairment and allowed prediction of purported auditory outcome after cochlear implantation in an index patient. Personalized treatment approaches based on the molecular mechanisms of disease may become increasingly important in the future.


Subject(s)
Exome Sequencing , Hearing Loss, Sensorineural , Austria , Europe , Exome , Extracellular Matrix Proteins , Female , Hearing Loss, Sensorineural/genetics , Hearing Loss, Sensorineural/rehabilitation , Humans , Male , Mutation , Pedigree , Exome Sequencing/methods
6.
Hear Res ; 350: 226-234, 2017 07.
Article in English | MEDLINE | ID: mdl-28527538

ABSTRACT

It has been shown that patients with electric acoustic stimulation (EAS) perform better in noisy environments than patients with a cochlear implant (CI). One reason for this could be the preserved access to acoustic low-frequency cues including the fundamental frequency (F0). Therefore, our primary aim was to investigate whether users of EAS experience a release from masking with increasing F0 difference between target talker and masking talker. The study comprised 29 patients and consisted of three groups of subjects: EAS users, CI users and normal-hearing listeners (NH). All CI and EAS users were implanted with a MED-EL cochlear implant and had at least 12 months of experience with the implant. Speech perception was assessed with the Oldenburg sentence test (OlSa) using one sentence from the test corpus as speech masker. The F0 in this masking sentence was shifted upwards by 4, 8, or 12 semitones. For each of these masker conditions the speech reception threshold (SRT) was assessed by adaptively varying the masker level while presenting the target sentences at a fixed level. A statistically significant improvement in speech perception was found for increasing difference in F0 between target sentence and masker sentence in EAS users (p = 0.038) and in NH listeners (p = 0.003). In CI users (classic CI or EAS users with electrical stimulation only) speech perception was independent from differences in F0 between target and masker. A release from masking with increasing difference in F0 between target and masking speech was only observed in listeners and configurations in which the low-frequency region was presented acoustically. Thus, the speech information contained in the low frequencies seems to be crucial for allowing listeners to separate multiple sources. By combining acoustic and electric information, EAS users even manage tasks as complicated as segregating the audio streams from multiple talkers. Preserving the natural code, like fine-structure cues in the low-frequency region, seems to be crucial to provide CI users with the best benefit.


Subject(s)
Cochlear Implantation/instrumentation , Cochlear Implants , Hearing Loss/rehabilitation , Hearing , Perceptual Masking , Persons With Hearing Impairments/rehabilitation , Speech Perception , Acoustic Stimulation , Adult , Aged , Aged, 80 and over , Audiometry, Pure-Tone , Auditory Threshold , Case-Control Studies , Cues , Electric Stimulation , Female , Hearing Loss/diagnosis , Hearing Loss/physiopathology , Hearing Loss/psychology , Humans , Male , Middle Aged , Noise/adverse effects , Persons With Hearing Impairments/psychology , Speech Intelligibility , Speech Reception Threshold Test , Young Adult
7.
Acta Otolaryngol ; 137(4): 356-360, 2017 Apr.
Article in English | MEDLINE | ID: mdl-27827000

ABSTRACT

CONCLUSION: Alterations within a novel putative Exon 1a within the gap junction beta 2 (GJB2) gene may play a role in the development of genetic hearing impairment in Austria. OBJECTIVES: Mutations in the GJB2 gene are the most common cause of hereditary sensorineural deafness. Genome-wide screening for alternative transcriptional start sites in the human genome has revealed the presence of an additional GJB2 exon (E1a). This study tested the hypothesis of whether alternative GJB2 transcription involving E1a may play a role in the development of congenital sensorineural deafness in Austria. METHODS: GJB2 E1a and flanking regions were sequenced in randomized normal hearing control subjects and three different patient groups with non-syndromic hearing impairment (NSHI), and bioinformatic analysis was performed. Statistical analysis of disease association was carried out using the Cochran-Armitage test for trend. RESULTS: A single change 2410 bp proximal to the translational start site (c.-2410T > C, rs7994748, NM_004004.5:c.-23 + 792T > C) was found to be significantly associated with the common c.35delG GJB2 mutation (p = .009). c.35delG in combination with c.-2410CC occurred at a 6.9-fold increased frequency compared to the control group. Additionally, one patient with idiopathic congenital hearing loss was found to be homozygous c.-2410CC.


Subject(s)
Connexins/genetics , Hearing Loss, Sensorineural/genetics , Alternative Splicing , Austria , Base Sequence , Case-Control Studies , Connexin 26 , Exons , Gene Frequency , Genetic Testing , Hearing Loss, Sensorineural/congenital , Hearing Loss, Sensorineural/diagnosis , Heterozygote , Humans , Mass Screening , Polymorphism, Genetic
8.
J Negat Results Biomed ; 15: 10, 2016 May 11.
Article in English | MEDLINE | ID: mdl-27164957

ABSTRACT

BACKGROUND: Selective glucocorticoid receptor modulators (SEGRMs) comprise a novel class of drugs promising both reduced side effects and similar pharmacological potency relative to glucocorticoids, which presently serve as the only clinical treatment for many otologic disorders. In the first otologic SEGRM experiment in an animal model of noise trauma, we compare the effects of Compound A (a SEGRM) and dexamethasone (potent glucocorticoid). METHODS: Forty adult guinea pigs received experimental treatment once daily for ten days. The animals were divided into four cohorts based on the treatment received: Compound A (1 mg/kg or 3 mg/kg), dexamethasone (1 mg/kg) as gold standard, or water as negative control. After five applications, animals were exposed to broadband noise (8-16 kHz) at 115 dB for three hours. Hearing thresholds were determined by recording auditory brainstem responses to clicks and noise bursts (1-32 kHz) and were assessed a week prior to and immediately after exposure, as well as on days 1, 3, 7, 14, 21, and 28. Cochleae were prepared as whole-mounts or embedded and sectioned for histological analysis. RESULTS: Relative to the control treatments, Compound A failed to preserve auditory thresholds post-noise exposure with statistical significance. Histological analyses confirm the physiological result. CONCLUSION: The present findings suggest that Compound A does not have substantial otoprotective capacities in a noise trauma model.


Subject(s)
Dexamethasone/administration & dosage , Noise/adverse effects , Receptors, Glucocorticoid/drug effects , Animals , Guinea Pigs
9.
Acta Otolaryngol ; 135(12): 1277-85, 2015.
Article in English | MEDLINE | ID: mdl-26223816

ABSTRACT

CONCLUSION: Bone conduction implants are useful in patients with conductive and mixed hearing loss for whom conventional surgery or hearing aids are no longer an option. They may also be used in patients affected by single-sided deafness. OBJECTIVES: To establish a consensus on the quality standards required for centers willing to create a bone conduction implant program. METHOD: To ensure a consistently high level of service and to provide patients with the best possible solution the members of the HEARRING network have established a set of quality standards for bone conduction implants. These standards constitute a realistic minimum attainable by all implant clinics and should be employed alongside current best practice guidelines. RESULTS: Fifteen items are thoroughly analyzed. They include team structure, accommodation and clinical facilities, selection criteria, evaluation process, complete preoperative and surgical information, postoperative fitting and assessment, follow-up, device failure, clinical management, transfer of care and patient complaints.


Subject(s)
Bone Conduction/physiology , Consensus , Hearing Loss, Mixed Conductive-Sensorineural/surgery , Prostheses and Implants/standards , Prosthesis Fitting/methods , Speech Perception/physiology , Follow-Up Studies , Hearing Loss, Mixed Conductive-Sensorineural/physiopathology , Humans , Prosthesis Design , Time Factors
10.
Acta Otolaryngol ; 135(4): 313-9, 2015 Apr.
Article in English | MEDLINE | ID: mdl-25720453

ABSTRACT

CONCLUSION: The intraoperative application of glucocorticoid-loaded hydrogels seems to cause a reduction in neutrophil infiltration. No beneficial effect on hearing thresholds was detected. OBJECTIVES: To evaluate the application of dexamethasone- and triamcinolone acetonide-loaded hydrogels for effects on hearing preservation and foreign body reaction in a guinea pig model for cochlear implantation (CI). METHODS: A total of 48 guinea pigs (n = 12 per group) were implanted with a single channel electrode and intraoperatively treated with 50 µl of a 20% w/v poloxamer 407 hydrogel loaded with 6% dexamethasone or 30% triamcinolone acetonide, a control hydrogel, or physiological saline. Click- and tone burst-evoked compound action potential thresholds were determined preoperatively and directly postoperatively as well as on days 3, 7, 14, 21, and 28. At the end of the experiment, temporal bones were prepared for histological evaluation by a grinding/polishing technique with the electrode in situ. Three ears per treatment group were serially sectioned and evaluated for histological alterations. RESULTS: The intratympanic application of glucocorticoid-loaded hydrogels did not improve the preservation of residual hearing in this cochlear implant model. The foreign body reaction to the electrode appeared reduced in the glucocorticoid-treated animals. No correlation was found between the histologically described trauma to the inner ear and the resulting hearing threshold shifts.


Subject(s)
Cochlear Implantation , Foreign-Body Reaction/prevention & control , Glucocorticoids/administration & dosage , Hearing/drug effects , Hydrogels , Intraoperative Care , Action Potentials , Animals , Cochlea/drug effects , Cochlea/pathology , Cochlea/surgery , Cochlear Implants/adverse effects , Dexamethasone/administration & dosage , Disease Models, Animal , Evoked Potentials, Auditory, Brain Stem , Female , Foreign-Body Reaction/etiology , Foreign-Body Reaction/pathology , Guinea Pigs , Male , Neutrophil Infiltration , Triamcinolone Acetonide/administration & dosage
11.
Laryngoscope ; 125(4): E149-55, 2015 Apr.
Article in English | MEDLINE | ID: mdl-25382757

ABSTRACT

OBJECTIVES/HYPOTHESIS: To evaluate the selective glucocorticoid receptor agonist (SEGRA) compound A, a potential novel therapeutic for inner ear disorders, for ototoxic effects. STUDY DESIGN: Laboratory animal study. METHODS: Experimental guinea pigs were grouped as follows: Systemic application of compound A (1.5 mg/kg and 4.5 mg/kg; n = 6/group) and intratympanic application of compound A (1 mM and 10 mM; n = 6/group). Contralateral ears in topically treated animals served as controls. Hearing thresholds were determined by auditory brainstem response before and directly after the application of compound A, as well as on days 3, 7, 14, 21, and 28. At the end of the experiments, temporal bones were harvested for histological evaluation. RESULTS: Systemic administration of compound A (1.5 mg/kg and 4.5 mg/kg) did not cause hearing threshold shifts, whereas the intratympanic injection (1 mM and 10 mM) resulted in a hearing loss. Histological analysis of the middle and inner ears after topical compound A application showed alterations in the tympanic membranes, the auditory ossicles, and the round window membranes, whereas spiral ganglion cells and hair cells were not affected. CONCLUSION: SEGRAs such as compound A could provide novel therapeutic options for the treatment of inner ear disorders and reduce metabolic side effects. Whereas the intratympanic application of compound A resulted in a hearing loss, the systemic application of compound A merits evaluation for otoprotective effects in trauma models.


Subject(s)
Evoked Potentials, Auditory, Brain Stem/drug effects , Receptors, Glucocorticoid/antagonists & inhibitors , Tympanic Membrane/drug effects , Tympanic Membrane/pathology , Adenine/pharmacology , Administration, Topical , Animals , Auditory Threshold/physiology , Biopsy, Needle , Citrates/pharmacology , Disease Models, Animal , Evoked Potentials, Auditory, Brain Stem/physiology , Female , Glucose/pharmacology , Guinea Pigs , Immunohistochemistry , Injections, Intraperitoneal , Male , Phosphates/pharmacology , Random Allocation , Reference Values
12.
Laryngoscope ; 124(12): 2802-6, 2014 Dec.
Article in English | MEDLINE | ID: mdl-25142577

ABSTRACT

OBJECTIVES/HYPOTHESIS: The aim of this study was to evaluate functional hearing gain, speech understanding, and preoperative bone-conduction thresholds with the bone-conduction implant Bonebridge. STUDY DESIGN: Retrospective study at a tertiary referral center. METHODS: Twenty-four consecutive Bonebridge patients were identified. Nine patients suffered from combined hearing loss (HL), 12 from atresia of the external auditory canal and three from single-sided deafness. One patient was lost to follow-up. Twenty-three patients were therefore analyzed. RESULTS: The overall average functional hearing gain of all patients (n = 23) was 28.8 dB (±16.1 standard deviation [SD]). Monosyllabic word scores at 65 dB sound pressure level in quiet increased statistically significantly from 4.6 (±7.4 SD) percentage points to 53.7 (±23.0 SD) percentage points. Evaluation of preoperative bone-conduction thresholds revealed three patients with thresholds higher than 45 dB HL in the high frequencies starting at 2 kHz. These three patients had a very limited benefit of their bone-conduction implants. CONCLUSIONS: The Bonebridge bone-conduction implant provides satisfactory results concerning functional gain and speech perception if preoperative bone conduction lies within 45 dB HL. LEVEL OF EVIDENCE: 4.


Subject(s)
Bone Conduction/physiology , Hearing Aids , Hearing Loss, Conductive/surgery , Prosthesis Implantation/methods , Speech Perception/physiology , Adolescent , Adult , Aged , Aged, 80 and over , Audiometry, Pure-Tone , Auditory Threshold , Child , Female , Follow-Up Studies , Hearing Loss, Conductive/physiopathology , Humans , Male , Middle Aged , Prosthesis Design , Retrospective Studies , Suture Anchors , Suture Techniques/instrumentation , Treatment Outcome , Young Adult
13.
Ear Hear ; 35(6): e272-81, 2014.
Article in English | MEDLINE | ID: mdl-25127325

ABSTRACT

OBJECTIVES: The aim of the present study was to compare two novel fine structure strategies "FS4" and "FS4-p" with the established fine structure processing (FSP) strategy. FS4 provides fine structure information on the apical four-electrode channels. With FS4-p, these electrodes may be stimulated in a parallel manner. The authors evaluated speech perception, sound quality, and subjective preference. DESIGN: A longitudinal crossover study was done on postlingually deafened adults (N = 33) who were using FSP as their default strategy. Each participant was fitted with FS4, FS4-p, and FSP, for 4 months in a randomized and blinded order. After each run, an Adaptive Sentence test in noise (Oldenburger Sentence Test [OLSA]) and a Monosyllable test in quiet (Freiburger Monosyllables) were performed, and subjective sound quality was determined with a Visual Analogue Scale. At the end of the study the preferred strategy was noted. RESULTS: Scores of the OLSA did not reveal any significant differences among the three strategies, but the Freiburger test showed a statistically significant effect (p = 0.03) with slightly worse scores for FS4 (49.7%) compared with FSP (54.3%). Performance of FS4-p (51.8%) was comparable with the other strategies. Both audiometric tests depicted a high variability among subjects. The number of best-performing strategies for each participant individually was as follows: (a) for the OLSA: FSP, N = 10.5; FS4, N = 10.5; and FS4-p, N = 12; and (b) for the Freiburger test: FSP, N = 14; FS4, N = 9; and FS4-p, N = 10. A moderate agreement was found in the best-performing strategies of the Speech tests within the participants. For sound quality, speech in quiet, classical, and pop music were assessed. No significant effects of strategy were found for speech in quiet and classical music, but auditory impression of pop music was rated as more natural in FSP compared with FS4 (p = 0.04). It is interesting that at the end of the study, a majority of the participants favored the new coding strategies over their previous default FSP (FSP, N = 13; FS4, N = 13; FS4-p, N = 7). CONCLUSIONS: In summary, FS4 and FS4-p offer new and further options in audio processor fitting, with similar levels of speech understanding in noise as FSP. This is an interesting result, given that the strategies' presentation of temporal fine structure differs from FSP. At the end of the study, 20 of 33 subjects chose either FS4 or FS4-p over their previous default strategy FSP.


Subject(s)
Cochlear Implants , Deafness/rehabilitation , Signal Processing, Computer-Assisted , Speech Perception , Adult , Aged , Cross-Over Studies , Double-Blind Method , Female , Humans , Longitudinal Studies , Male , Middle Aged , Speech Reception Threshold Test , Young Adult
14.
Audiol Neurootol ; 19(3): 193-202, 2014.
Article in English | MEDLINE | ID: mdl-24714604

ABSTRACT

The pharmacokinetic properties and tolerability of a triamcinolone acetonide poloxamer 407 hydrogel for intratympanic application were investigated in a guinea pig model. Evaluation of in vivo release kinetics showed very high initial perilymph drug levels, with clinically relevant levels present for a minimum of 10 days. Assessment of auditory brainstem response thresholds showed a minimal, delayed and transient threshold shift, which was apparent on day 3 and resolved by day 10. No relevant histological changes of the middle and inner ear structures were noted, and hair cell counts showed no significant differences between treated and untreated ears. Thus, the triamcinolone-acetonide-loaded poloxamer 407 hydrogel is an effective vehicle for sustained high-dose inner ear glucocorticoid delivery.


Subject(s)
Delayed-Action Preparations/pharmacokinetics , Glucocorticoids/pharmacokinetics , Hydrogels/administration & dosage , Triamcinolone Acetonide/pharmacokinetics , Tympanic Membrane/drug effects , Animals , Delayed-Action Preparations/administration & dosage , Glucocorticoids/administration & dosage , Guinea Pigs , Hydrogels/pharmacokinetics , Triamcinolone Acetonide/administration & dosage , Tympanic Membrane/metabolism
15.
Laryngoscope ; 124(6): 1436-43, 2014 Jun.
Article in English | MEDLINE | ID: mdl-24338550

ABSTRACT

OBJECTIVES/HYPOTHESIS: To evaluate modified coupling techniques of the Vibrant Soundbridge system in patients with mixed and conductive hearing loss and to compare it with conventional vibroplasty. STUDY DESIGN: Retrospective study. METHODS: Two different groups were evaluated: 1) nine cases of conventional incus vibroplasty in comparison with 2) nine patients with modified coupling of the floating mass transducer. In the modified coupling approach, the vibrant floating mass transducer was attached to 1) the stapes/oval window, 2) the round window, or 3) the drilled promontory bone (promontory fenestration window). In three patients, an additional ossiculoplasty was performed. Preoperative and postoperative aided and unaided pure-tone and free-field audiometry and Freiburg monosyllabic word test were used to assess hearing outcome. RESULTS: Functional hearing gain obtained in patients with mixed and conductive hearing loss who underwent modified coupling was 39 dB. Patients with pure sensorineural hearing loss who received conventional incus coupling showed a functional hearing gain of 25 dB. Average functional gain was 41 dB in the oval window group, 45 dB in the round window group, and 30 dB in the promontory fenestration window group. Word recognition test revealed an average improvement of 51% and 21% in the modified and in the conventional approach, respectively. CONCLUSIONS: Modified vibroplasty is a safe and effective treatment for patients with conductive and mixed hearing loss. Coupling the floating mass transducer to the promontory bone (promontory fenestration window) is a viable option in chronically disabled ears if oval and round window coupling is not possible. LEVEL OF EVIDENCE: 4.


Subject(s)
Hearing Loss, Conductive/surgery , Hearing Loss, Mixed Conductive-Sensorineural/surgery , Ossicular Replacement/methods , Round Window, Ear/surgery , Adolescent , Aged , Audiometry/methods , Audiometry, Pure-Tone/methods , Child , Child, Preschool , Cohort Studies , Electrodes, Implanted , Female , Follow-Up Studies , Hearing Loss, Conductive/diagnosis , Hearing Loss, Mixed Conductive-Sensorineural/diagnosis , Humans , Male , Middle Aged , Ossicular Prosthesis , Otologic Surgical Procedures/methods , Retrospective Studies , Risk Assessment , Round Window, Ear/physiopathology , Severity of Illness Index , Treatment Outcome
17.
Laryngoscope ; 122(10): 2300-3, 2012 Oct.
Article in English | MEDLINE | ID: mdl-22777797

ABSTRACT

In the present article we report an endolymphatic sac tumor in a 15-year-old male who had additional angiomatous lesions in the nasal and pharyngeal mucosa and was diagnosed with von Hippel-Lindau disease postoperatively. Preoperative imaging excluded cholesteatoma, but did not provide sufficient information to distinguish between jugular paraganglioma and endolymphatic sac tumor. To the authors' knowledge this is the first description of angiomatous lesions in the mucosa of the upper respiratory tract in a von Hippel-Lindau disease patient, a potentially useful finding for future radiological differential diagnosis in cases presenting with endolymphatic sac tumor.


Subject(s)
Angiomatosis/diagnosis , Ear Neoplasms/diagnosis , Nasal Mucosa/pathology , Nose Diseases/diagnosis , Pharyngeal Diseases/diagnosis , von Hippel-Lindau Disease/diagnosis , Adolescent , Diagnosis, Differential , Endolymphatic Sac/diagnostic imaging , Endolymphatic Sac/pathology , Humans , Magnetic Resonance Imaging , Male , Tomography, X-Ray Computed
18.
Article in English | MEDLINE | ID: mdl-21997337

ABSTRACT

CONCLUSION: This study demonstrates that electric-acoustic stimulation (EAS) significantly decreases the subjective impairment in speech perception. OBJECTIVES: To assess the subjective benefit of EAS over the first 12 months after EAS fitting using the Abbreviated Profile of Hearing Aid Benefit (APHAB). METHOD: Twenty-three EAS users, implanted with either the PULSAR(CI)(100) FLEX(EAS) provided with the DUET EAS processor or the COMBI40+ Medium provided with the TEMPO+ speech processor, were included. Electric stimulation was activated about 1 month postoperatively; ipsilateral acoustic stimulation was added 2 months thereafter. EAS benefit was measured preoperatively with only a hearing aid and postoperatively at EAS fitting and then 3, 6 and 12 months after EAS fitting using the APHAB. RESULTS: Subjects reported significant improvements in the global score with a mean decrease in impairment from 74% preoperatively to 45% after 3 months of EAS use. Furthermore, clinical relevance was demonstrated in multiple subscales between preoperative and first fitting reflecting a true benefit of EAS with a probability of 95%.


Subject(s)
Acoustic Stimulation/methods , Cochlear Implantation/rehabilitation , Electric Stimulation/methods , Hearing Aids , Hearing Loss, Bilateral/rehabilitation , Speech Perception , Adult , Aged , Auditory Threshold , Cochlear Implantation/psychology , Combined Modality Therapy , Female , Hearing , Hearing Loss, Bilateral/psychology , Humans , Male , Middle Aged , Patient Satisfaction , Pitch Perception , Psychoacoustics , Surveys and Questionnaires , Young Adult
19.
Otol Neurotol ; 32(7): 1094-101, 2011 Sep.
Article in English | MEDLINE | ID: mdl-21817932

ABSTRACT

OBJECTIVE: The transmission of fine structure information to cochlear implant users is an expanding area of research. Previous studies comparing the fine structure processing (FSP) speech coding strategy to the envelope-based continuous interleaved sampling (CIS) strategy indicated improved speech perception when using the fine structure strategy. Those investigations were performed with an extended frequency spectrum in the low frequencies together with the fine structure strategy. The current study addresses the question whether these improvements are due to the presentation of fine structure per se or rather the extended frequency spectrum. Hence, this cross over study compares the two strategies using an identical frequency spectrum. STUDY DESIGN: Randomized crossover study. PATIENTS: 31 patients were randomly assigned to two groups. INTERVENTIONS: One group was fitted with a CIS map for 4 weeks, tested and subsequently fitted with a FSP map for 4 weeks. The other group followed the same pattern in reverse. MAIN OUTCOME MEASURES: Test material consisted of sentence tests in noise, monosyllables in quiet and melody recognition. RESULTS: No statistical significance was noted between the different speech coding strategies at an identical frequency spectrum. CONCLUSION: This study shows that there is no difference in speech perception with FSP compared to CIS at an extended frequency spectrum. Therefore, the extended frequency spectrum in the low frequencies might explain a benefit of FSP observed in previous studies.


Subject(s)
Cochlear Implants , Hearing Loss/physiopathology , Speech Perception/physiology , Adult , Aged , Cochlear Implantation , Cross-Over Studies , Female , Hearing Loss/surgery , Humans , Male , Middle Aged , Speech Discrimination Tests
20.
Wien Klin Wochenschr ; 123(19-20): 599-602, 2011 Oct.
Article in English | MEDLINE | ID: mdl-21858425

ABSTRACT

OBJECTIVE: Cochlear implantation of patients with high-frequency hearing loss and residual low-frequency hearing has become a new treatment standard within the last years. The objective of this study was to evaluate the rate of hearing preservation in cochlear implantation for electric-acoustic stimulation using the suprameatal approach. METHODS: Five patients (mean age 48.2 years) who were supplied with Med El (Combi 40+, Pulsar) cochlear implants and various different electrodes (Custom made, Flex soft, Flex EAS) were evaluated for residual hearing preservation after a mean follow-up time of 35.6 months (range 24 to 77 months). RESULTS: Three patients showed partial hearing preservation, whereas 2 patients experienced a complete loss of residual hearing. CONCLUSION: Although hearing preservation rates in this series of patients operated on using the suprameatal approach were inferior as compared with a series using the standard mastoidectomy approach, this study shows that it is in fact possible to preserve residual hearing using a non-mastoidectomy surgical technique for cochlear implantation. Nevertheless, more experience is necessary to answer the question whether this technique can be thoroughly recommended in cochlear implantation for electric-acoustic stimulation.


Subject(s)
Cochlear Implantation/instrumentation , Cochlear Implantation/methods , Cochlear Implants , Hearing Loss/diagnosis , Hearing Loss/rehabilitation , Adult , Cranial Fossa, Middle/surgery , Female , Humans , Male , Middle Aged , Treatment Outcome
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