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1.
Background
Fabry disease is an X-linked lysosomal storage disease involving deficient activity of α-galactosidase A, which leads initially to pain, and later to renal insufficiency, cardiomyopathy and stroke. Until now few details are available on hearing impairment in patients with Fabry disease, and especially few relating to female patients.Patients and methods
We examined 43 female and 29 male patients. In this study we looked into the question of whether and to what extent patients of both genders are affected by hearing impairment.Results
Hearing loss is characteristic being more severe at high frequencies frequencies. Overall, 22 female and 15 male patients were found to have suffered a hearing loss. Patients with severe symptoms of Fabry disease usually demonstrate more prominent hearing losses.Conclusions
Both men and women with Fabry disease are affected by hearing impairment. It seems that the hearing loss is less marked in female than in male patients. Children with Fabry disease complain of tinnitus more frequently than other children and quite early in the course of the disease. 相似文献2.
Background
The present study analyzes the best combination of frequencies for the calculation of mean hearing loss in pure tone threshold audiometry for correlation with hearing loss for numbers in speech audiometry, since the literature describes different calculation variations for plausibility checking in expertise. Three calculation variations, A (250, 500 and 1000?Hz), B (500 and 1000?Hz) and C (500, 1000 and 2000?Hz), were compared.Methods
Audiograms in 80 patients with normal hearing, 106 patients with hearing loss and 135 expertise patients were analyzed in a retrospective manner. Differences between mean pure tone audiometry thresholds and hearing loss for numbers were calculated and statistically compared separately for the right and the left ear in the three patient collectives.Results
We found the calculation variation A to be the best combination of frequencies, since it yielded the smallest standard deviations while being statistically different to calculation variations B and C. The 1- and 2.58-fold standard deviation (representing 68.3% and 99.0% of all values) was ±4.6 and ±11.8?dB for calculation variation A in patients with hearing loss, respectively.Conclusions
For plausibility checking in expertise, the mean threshold from the frequencies 250, 500 and 1000?Hz should be compared to the hearing loss for numbers. The common recommendation reported by the literature to doubt plausibility when the difference of these values exceeds ±5?dB is too strict as shown by this study. 相似文献3.
Background
In spite of modern technology conventional hearing aids are only helpful in a limited number of patients with sensorineural hearing loss. In particular, patients presenting with moderate to severe high frequency hearing loss but only mild hearing loss in the low frequencies suffer from problems associated with conventional hearing aids such as occlusion of the ear canal and feedback.The aim of the study was the evaluation of rehabilitation of patients with high frequency hearing loss with the active middle ear implant Vibrant Soundbridge®.Patients and methods
The Vibrant Soundbridge® was surgically implanted into 30 patients, and the floating mass transducer was clipped onto the long process of the incus. Out of the 30 patients 9 presented with a ski slope audiogram of high frequency hearing loss of 25 dB within 1 octave in the frequency range 1000–8000 Hz. Main outcome measures were pure tone audiometry, speech audiometry in quiet and in noise.Results
Residual hearing was preserved in all cases. Functional hearing gain was in proportion to the individual hearing losses and was remarkably high in the high frequencies up to 8000 Hz. Mean functional gain was 34 dB in the frequencies between 2000–8000 Hz. Speech recognition scores in quiet and in noise were significantly higher with the implant compared to the unaided situation.Conclusion
The middle ear implant Vibrant Soundbridge® has been shown to be perform extremely well especially in the high frequencies. It offers a new solution for rehabilitation of high frequency hearing loss. 相似文献4.
Preliminary remarks
The acceptance of hearing-aids by elderly people is generally poor. Hearing loss on the other hand increases with age, and the ability to communicate, especially in random noise, diminishes.Patients and methods
Audiometric data on 331 individuals older than 60 years were collected. Sixty people between 15 and 30 and 86 people between 31 and 60 years acted as controls. Tympanograms, hearing thresholds, speech audiometry and dichotic hearing as well as binaural masking level differences and otoacustic emissions for eight frequencies were measured. Hearing aid status was also determined.Results
Our data show more hearing deficits at low frequencies as indicated in the literature. Presbycusis is present solely in the internal ear in 11.5–27.7%, in central hearing structures in 3.8–21.4%, but mainly in all parts of the auditory pathway 50.9–84.6% of cases. Only 15.3% of patients needing hearing aids were actually provided with them.Conclusion
Hearing decreases with age but some older people have completely normal hearing. Therefore, a physiological presbycusis does not exist. There are deficits in the inner ear as well as in the central auditory structures which should be determined by audiometric diagnosis. Hearing aid supply should be carefully regulated and audiotherapy carried out. 相似文献5.
Background and objective
For patients suffering from recurrent sudden hearing loss (SHL) that is refractory to infusion therapy, new therapeutic options must be established.Patients and methods
Patients suffering from recurrent and progressive SHL refractory to infusion therapy according to German guidelines were analysed retrospectively. After unsuccessful infusion therapy following the last onset of SHL, patients were treated with Rheopheresis twice. Hearing gain and recovery of speech discrimination were analysed.Results
Twenty-five patients with a mean of 2.1±0.4 events of SHL within 30.0±21.6 months were examined. The patients’ mean hearing loss before the first onset of SHL was 34 dB and was reduced by infusion therapy to 20 dB. With the second onset of SHL, hearing loss remained almost unchanged after infusion therapy. Patients showed a mean improvement of 20 dB after two consecutive Rheopheresis treatments. Forty percent showed complete remission of SHL, and a further 28% showed partial remission.Conclusion
Rheopheresis can efficiently improve the hearing of patients with recurrent SHL refractory to infusion therapy. 相似文献6.
Background
Bone-anchored hearing aids provide a therapy option for patients with conductive and mixed hearing loss, with the possibility of audiological rehabilitation. Test rod and tension headband offer the patient a preoperative impression of their postoperative hearing. The aim of this study was to evaluate the tools available for preoperative assessment of candidates for bone-anchored hearing aids and to ascertain predictive audiometric values.Methods
Following implantation of a BAHA Compact system, sound and speech audiograms were performed in each of 16 patients with the bone-anchored BAHA device and with test rod and tension headband.Results
Both with the BAHA and with the test devices, closure of the air-bone gap occurred and resulted in a clear improvement in hearing threshold and speech perception. The threshold increase with the BAHA was 9 dB greater on average (mean) than that measured with the test rod and the tension headband. Examinaation of speech perception indicated a mean advantage of 14–20% for the anchored BAHA system over the tension headband in monosyllabic testing.Conclusion
Preoperative testing before providing a BAHA system is a good way of predicting what level of hearing and speech perception can be expected after implantation. 相似文献7.
Background and aim
Speech perception is the most important social task of the auditory system. Consequently, speech audiometry is essential to evaluate hearing aid benefit. The aim of the study was to describe the correlation between pure-tone hearing loss and speech perception. In particular, pure-tone audiogram, speech audiogram, and speech perception with hearing aids were compared.Materials and methods
In a retrospective study, 102 hearing aid users with bilateral sensorineural hearing loss were included. Pure-tone loss (PTA) was correlated to monosyllabic perception at 65 dB with hearing aid and with maximum monosyllabic perception with headphones.Results
Speech perception as a function of hearing loss can be represented by a sigmoid function. However, for higher degrees of hearing loss, substantial deviations are observed. Maximum monosyllabic perception with headphones is usually not achieved with hearing aids at standard speech levels of 65 dB.Conclusion
For larger groups, average pure-tone hearing loss and speech perception correlate significantly. However, prognosis for individuals is not possible. In particular for higher degrees of hearing loss substantial deviations could be observed. Speech performance with hearing aids cannot be predicted sufficiently from speech audiograms. Above the age of 80, speech perception is significantly worse. 相似文献8.
Dr. J. Löhler B. Akcicek M. Pilnik K. Saager-Post S. Dazert S. Biedron J. Oeken D. Mürbe J. Löbert R. Laszig T. Wesarg C. Langer S. Plontke T. Rahne U. Machate R. Noppeney K. Schultz P. Plinkert S. Hoth M. Praetorius P. Schlattmann H.W. Pau K. Ehrt R. Hagen W. Shehata-Dieler M. Cebulla L.E. Walther A. Ernst 《HNO》2013,61(7):586-591
Background
The Freiburg speech test has been the gold standard in speech audiometry in Germany for many years. Previously, however, this test had not been evaluated in assessing the effectiveness of a hearing aid in background noise. Furthermore, the validity of particular word lists used in the test has been questioned repeatedly in the past, due to a suspected higher variation within these lists as compared to the other word list used.Patients and methods
In this prospective study, two groups of subjects [normal hearing control subjects and patients with SNHL (sensorineural hearing loss) that had been fitted with hearing aid] were examined. In a first group, 113 control subjects with normal age- and gender-related pure tone thresholds were assessed by means of the Freiburg monosyllabic test under free-field conditions at 65 dB. The second group comprised 104 patients that had been fitted with hearing aids at least 3 months previously to treat their SNHL. Members of the SNHL group were assessed by means of the Freiburg monosyllabic test both with and without hearing aids, and in the presence or absence of background noise (CCITT-noise; 65/60 dB signal-noise ratio, in accordance with the Comité Consultatif International Téléphonique et Télégraphique), under free-field conditions at 65 dB.Results
The first (control) group exhibited no gender-related differences in the Freiburg test results. In a few instances, inter-individual variability of responses was observed, although the reasons for this remain to be clarified. Within the second (patient) group, the Freiburg test results under the four different measurement conditions differed significantly from each other (p>0.05). This group exhibited a high degree of inter-individual variability between responses. In light of this, no significant differences in outcome could be assigned to the different word lists employed in the Freiburg speech test.Conclusion
The Freiburg monosyllabic test is able to assess the extent of hearing loss, as well as the effectiveness of a fitted hearing aid, in the presence or absence of background-noise (CCITT-noise). The present study could not evidence statistically significant differences in outcome when using the different word lists in this test battery. 相似文献9.
Objectives
Data on the prevalence of otorhinolaryngological (ORL) symptoms in patients with cystic fibrosis (CF) are limited. A standard annual examination would be useful for definition of risk factors and/or management alternatives for these symptoms.Study design
Prospective observational study of an unselected group of CF patients.Patients and methods
Between 1997 and 2004, 123 CF patients were routinely examined. The examinations included nasal endoscopy and audiological evaluation.Results
All patients who were approached to take part in the study gave their consent and were extremely cooperative. In 40% of the patients nasal polyps were found endoscopically. In 22% rhinological surgery had already been performed. Of the 123 patients, 13 (12%) showed different grades of sensorineural hearing loss, and all of these patients had received aminoglycoside antibiotics.Conclusions
CF patients demonstrate a high prevalence of ORL symptoms. Therefore, an annual standard examination would provide data for identifying potential risk factors and for optimizing the management of these symptoms in CF patients. 相似文献10.
Background
Following surgical repair of cleft lip and palate, hearing and speech and language development are important issues for the continued care of affected childhood and adolescent patients. Therefore, PEAKS (Program for Evaluation and Analysis of all Kinds of Speech Disorders) was developed in order to rate speech intelligibility automatically and reduce the time required for diagnostics. PEAKS is based on a speech recognition system and was extended to incorporate a speaker model. This investigation validated PEAKS for isolated cleft palate.Methods
From each of the 39 children with isolated cleft palate (3.1–14.5years), 99 word productions were recorded digitally and analyzed—once “subjectively” by five experts and five nonexperts; once “objectively” using PEAKS.Results
The automatic speech recognition system and the experts arrive at similar results with regard to speech intelligibility. The expert and nonexpert ratings differ significantly from each other. Within the group of nonexperts, a weak interrater reliability demonstrates the uncertainty associated with their ratings.Conclusion
PEAKS delivers reliable and representative results with regard to speech intelligibility among children and adolescents with isolated cleft palate. The automatic measurement of speech quality in children and adolescents with isolated cleft palate is possible. 相似文献11.
Meister H Schreitmüller S Grugel L Landwehr M von Wedel H Walger M Meister I 《HNO》2011,59(7):689-695
Background
Elderly persons frequently complain about problems with speech understanding especially in complex acoustic situations. Besides hearing impairment the decline of cognitive functions might explain these problems.Methods
In 12 normal hearing young subjects and 14 elderly listeners with extraordinarily good hearing speech perception was measured in a broad range of different acoustic situations. Cognitive functioning was evaluated with different neuropsychological tests.Results
Despite comparable pure tone thresholds the elderly listeners revealed worse speech discrimination than the young subjects in almost all test situations. Largest differences were found in situations with fluctuating maskers and competing talkers. Most of the speech perception results revealed significant correlations with the outcome from a neuropsychological test addressing declarative verbal memory.Conclusions
In complex listening situations elderly persons reveal worse speech understanding than younger subjects. Differences in speech perception can partly be attributed to cognitive abilities. In particular, working memory seems to be an important factor. 相似文献12.
Background
The aim of the present paper is to evaluate the clinical parameters in patients implanted for combined, ipsilateral electric-acoustic stimulation of the auditory system.Methods
A total of 18 patients with residual deep frequency hearing were implanted with a Combi 40+cochlear implant (MED-EL, Austria). Insertion depths ranged from 18 to 22 mm (360°). A modified surgical technique should contribute to hearing preservation in low frequency regions of the cochlea. Pure-tone audiometric thresholds were measured pre- and postoperatively. A speech audiometric evaluation was performed on two subjects.Results
Utilizing adapted surgical procedures, the preservation of low frequency hearing was accomplished in 16 of 18 subjects (88.9%). Seven (38.9%) patients had complete and nine (50.0%) partial preservation of residual hearing. The speech discrimination scores of two patients documented an increase in sentence intelligibility when compared with only the cochlear implant.Conclusions
Hearing preservation in cochlear implant surgery is possible. Insertions of 360° provide a full functioning cochlear implant to stimulate sufficient neural structures for above average discrimination scores with the implant alone. A synergistic effect of the electric and the acoustic stimulation modes leads to high discrimination scores in background noise. 相似文献13.
Background
The term Mondegreens describes misperceptions as defined by ??slips of the ear??, denoting that an utterance is perceived differently to what has actually been said. The analysis of these misperceptions permits a deeper insight into speech processing in the human brain.Method
A selective literature research in PubMed has been carried out for this systematic review.Results
Altogether, 24 publications were found addressing the issue of auditory misperceptions.Discussion
The analysis of Mondegreens enables a characterization of the different levels of speech processing as well as the processing steps involved. The hard of hearing in particular suffer from misperceptions. Therefore, it should be considered whether listening programs could be helpful in combination with hearing aids. 相似文献14.
Objective
For the determination of speech intelligibility in the expertise of hearing loss, the Freiburg speech test (number test and monosyllable test) is recommended in the Federal Republic of Germany. In the former German Democratic Republic, Sauer’s binaural number test with 70?dB background noise (“beidohriger Zahlentest”, BZT) was a standard element in expert opinions and was used in the calculation of bodily injury (“K?rperschaden”). In the current practice, a hearing test in noise is still lacking. The present study analyzes whether and to what degree the impairment (“Grad der Sch?digungsfolgen”, GdS) changes when also considering Sauer’s test.Material and methods
In a collective of 78 patients with hearing loss (66 patients with high-frequency hearing loss and 12 patients with pancochlear hearing loss) and 22 normal hearing controls, the following audiometric measurements were conducted: pure tone audiometry, speech audiometry (Freiburg speech test), free field audiometry with and without noise, and Sauer’s test. Subsequently, the hearing loss for both sides was calculated taking into consideration the values obtained with and without Sauer’s test, and the respective GdS was determined.Results
Patients with high-frequency hearing loss and pancochlear hearing loss had a trend for higher GdS (approximately 2 and 5%, respectively), compared to the established algorithm without the use of hearing tests in noise. However, neither the Mann–Whitney U-test nor the Bland–Altman analysis yielded relevant differences between the two methods to calculate the GdS.Conclusion
The routine implementation of Sauer’s test in the expertise of hearing loss cannot be recommended, since no relevant change in the GdS can be expected. This is especially true for high-frequency hearing loss. In pancochlear hearing loss, use of Sauer’s test can be considered if problems concerning hearing in situations with background noise are present—at least until more advanced hearing tests optimized for use in noise have been integrated into the tables for GdS calculation. 相似文献15.
Introduction
Single-sided deafness has a strong impact on quality of life. Besides the difficulties in sound localization, patients also require increased effort to hear. The latter makes it difficult to follow conversations for an extended period, leading to social isolation. Cochlear implantation (CI) represents a possible treatment option for deafness in one ear.Methods
In the present study, 13 patients with unilateral deafness where treated with CI. All patients were examined by pure tone audiometry and speech tests (Freiburger; HSM; OlSa), as well as sound localisations tests. The single-sided deaf situation is compared to CI-aided binaural hearing.Results
At 100%, the acceptance rate was very high. The two different auditory inputs were well integrated. Patients reported satisfactory restoration of acoustic orientation and sound localization as well as great ease of listening in noisy surroundings. There was no negative impact on the normal hearing side.Conclusion
These prelimary results confirm the effectiveness of CI treatment in unilateral deafness. 相似文献16.
Prof. Dr. J. Oeken 《HNO》2013,61(6):504-509
Introduction
It is recommended that a stapedotomy be performed under local anaesthesia to enable intraoperative monitoring of hearing and vestibular function. In contrast, we prefer to carry out stapedotomies under general anesthesia. The aim of this study was to investigate whether this practice has an adverse effect on hearing results.Patients and methods
All stapedotomies performed in our department between May 2003 and February 2012 were included in the analysis. Revision surgery was excluded. All interventions were performed under general anesthesia with an endotracheal tube by the same surgeon using the same technique. Pure tone and speech audiometry, acoustic reflex testing and Schüller radiology were performed preoperatively. Follow-up examinations (pure tone and speech audiometry) took place 4–6 weeks following surgery.Results
A total of 262 stapedotomies were carried out on 228 patients. Follow-up examinations could not be performed on six patients. Of the remaining 256 cases, closure of the air–bone gap to less than 10 dB was achieved in 220 patients (86%) and in 29 patients (11%) it was closed to less than 20 dB. Conductive hearing loss persisted in seven cases (3%). A mild sensorineural hearing loss with complete closure of the air–bone gap was experienced by two patients (0.8%). There was no instance of postoperative deafness.Conclusions
Performing stapedotomies under general rather than local anesthesia has no adverse effects on audiological results. 相似文献17.
Background
After cochlear implantation, most parents expect a normal speech and general development of their child. However, it remains unclear how quickly after early cochlear implantation these children can compensate for their deficits compared to normal-hearing children.Methods
This study retrospectively analyzed ELFRA-1 questionnaire data from 40 children with borderline deafness or high-grade hearing loss (without other known impairments) who had undergone cochlear implantation at a university medical center before reaching 2 years of age. ELFRA-1 questionnaires were filled out parents assisted by specialists 12 months after implantation. Questions assessed the children’s speech production and comprehension, as well as their use of gestures and fine motoric skills.Results
At an average hearing-age of 12 months, the children achieved normal values in all of the subgroups that were comparable to those of 12-month-old children without hearing impairments. A significant correlation (p?=?0.01) between the individual subgroups of the ELFRA-1 (speech production, speech comprehension, gestures and fine motor skills) was observed. Unilingual educated children performed significantly better overall.Conclusion
Within 12 months of receiving a cochlear implant, all children passed the four categories of the ELFRA-1. This demonstrates a rapid compensation of deficits in speech, motor skills and gesture development by children undergoing early cochlear implantation. 相似文献18.
Background
Numerous people with cochlear implants (CI) report difficulties in listening to music even though they understand speech quite well. One reason for this is a limited perception of pitch and timbre. In this study ability of adult CI subjects to discriminate musical pitch is investigated.Patients and methods
In two psychoacoustic experiments, each conducted in 10 adult CI subjects provided with MED-EL Combi 40+ cochlear implant devices and a control group of subjects with normal hearing, individual discrimination abilities for musical pitch perception were determined. To investigate the influence of the group of instruments on discrimination ability, stimuli representing four different groups of instruments were used: woodwind (clarinet), brass (trumpet), strings (violin) and keyboard instruments (piano).Results
The discrimination thresholds determined varied between individual CI subjects, and on average they were significantly higher for the piano than for the other three instruments.Conclusions
The results show that in subjects with CI pitch perception differs from instrument to instrument and is in general worse than in persons with normal hearing. 相似文献19.
B. Bartke T. Haderlein M. Döllinger E. Nöth S. Graf U. Eysholdt Dr. A. Ziethe 《HNO》2013,61(8):672-677
Background
Patients with chronic laryngitis and T1 vocal cord cancer were compared using perceptual and text-based objective voice and speech analyses in order to determine which group is more affected in its ability to communicate and whether a distinction between the two pathologies is possible.Patients and methods
In all, 13 patients with histologically proven chronic laryngitis and 13 patients with T1 vocal cord cancer were compared perceptually by five speech therapists on the basis of seven criteria and objectively by a speech recognition system and prosodic analysis.Results
Both, the data of the five speech therapists and the results of the automatic analysis revealed no significant differences between the two patient groups.Conclusion
A distinction between chronic laryngitis and T1 vocal cord carcinoma by mere voice and speech analysis is not possible, because the patient groups do not show significant differences in their voice quality. 相似文献20.