ABSTRACT
BACKGROUND: Considering the different calibration and masking of the noise, the adaptive Freiburg monosyllabic speech test in noise (aFBE-S) and the Oldenburg sentence test in noise (OLSA-S) were shown to be comparable with respect to the accuracy of both tests in a previous study. However, the time requirement of the aFBE-S was significantly greater than that of the OLSA-S due to the adaptive measurement method. The purpose of this study is to theoretically determine whether the aFBE-S can be used with fewer test lists, given the low scatter of results, and to compare the results with those of the OLSA-S. METHODS: Using the results of 40 otologically healthy subjects who had already been tested in randomized order with the OLSA-S and aFBE-S, the mean difference of the 95 % confidence interval (95 % CI) of the signal-to-noise ratio for 50% speech understanding (S/N50) of the aFBE-S was calculated for three, four, and five test lists instead of 7.5. In addition, the time required for the reduced number of test lists was determined and the results were examined in comparison with those of the OLSA-S. RESULTS: In each case, no significant difference between the difference mean of the 95 %-CI of the S/N50 of the original aFBE-S, the aFBE-S shortened to 3, 4, or 5 test lists and the OLSA-S could be found. The time required for the aFBE-S with a reduced number of test lists was significantly less than for the OLSA-S in each case. CONCLUSION: The aFBE-S is not inferior with a reduced number of test lists in comparison to the OLSA-S. This would allow to use the shortened aFBE-S theoretically.
Subject(s)
Noise , Humans , Adult , Female , Male , Middle Aged , Perceptual Masking/physiology , Reference Values , Signal-To-Noise Ratio , Speech Discrimination Tests , Young Adult , Speech PerceptionABSTRACT
Many people in Germany and worldwide suffer from a reduced communication ability due to impaired hearing. Especially older people are affected. Hearing aids, which pick up ambient sounds, process them, and output airborne sound in the ear canal, can help in most cases. These so-called air-conduction hearing aids are commonly used to compensate for hearing loss and are described in more detail in this article. To this end, indications, differences to other hearing aids and implants, various designs, the importance of individual ear coupling, features of the signal processing, and the importance of the individual hearing aid fitting are explained in detail.
Subject(s)
Deafness , Hearing Aids , Hearing Loss , Aged , Hearing , Hearing Loss/diagnosis , Humans , Signal Processing, Computer-AssistedABSTRACT
BACKGROUND: Questionnaires, e.âg. the APHAB, and speech-audiometry are the most used elements in measuring the success of hearing aid fitting (HAF). This study investigates the correlations between the results of the Freiburg monosyllabic word test without and with noise (FBE, FBE-S) and the results of the APHAB before and after HAF. METHODS: Data of the FBE, the FBE-S, and the APHAB, generated within HAF of 156 subjects were analyzed. After exclusion of a normal distribution of the data, Spearman's correlation and Cohen's effect size were determined. RESULTS: 73 (46.8â%) of the 156 subjects were females, and 83 (53.2â%) males. No significant correlation could be found between the EC-, the RV-, and the cumulative ECBNRV-subscale and the benefit of HAF in the FBE and FBE-S (EC: ease of communication, BN: background noise, RV: reverberation). Most of the remaining coefficients of correlation had a weak positive effect size. A middle positive effect size could only be demonstrated for 6 combinations, for the EC-subscale mostly. CONCLUSION: The not strong effect sizes could assume that the results in speech-audiometry and the APHAB are not independent and complementing each other as parameters of hearing loss and benefit in HAF. The effect size could be explained by individually different possibilities for the compensation of hearing loss.
Subject(s)
Hearing Aids , Hearing Loss , Speech Perception , Audiometry, Speech , Female , Hearing Loss/diagnosis , Humans , Male , Noise , Surveys and QuestionnairesABSTRACT
BACKGROUND: Since 2012, the APHAB is part of the quality agreement for statutory insured patients in hearing aid fitting (HAF). So far, individual results could be interpreted by using percentile curves only, but not for the improvement quotient and the cumulated benefit. The presented study should close this gap.âMoreover, it should be clarified if an individual constancy within percentile exists. METHODS: Using the data of 6861 hearing aid fitted patients from a database, we calculated the benefit by improvement quotient and cumulated benefit for different age-classes and percentile-groups and presented by a heatmap.âIndividual constancy of percentile would be calculated using Spearman's rank correlation. RESULTS: The average benefit was 21.41. The average of the improvement quotient was 41.01. It was significantly higher (44.36â%) in subjects younger than the average (27.26 years ± 11.86) than in the elderly (37.66â%). It decreased in cases of lower APHAB-scores before HAF concerning the percentile-group, ranging from 23.22â% to 52.07â%. Spearman's rank coefficient for the APHAB benefit was 0.285, Cohen's effect size was small. The correlation between the APHAB-score before HAF and the cumulated benefit was 0.582 and the improvement quotient was 0.270. CONCLUSIONS: An individual constancy within percentile before and after HAF was not detectable. Nevertheless, some relationships of the improvement quotient and the age resp.âpercentile-groups could be demonstrated. The benefit of HAF was less in older subjects with lower APHAB-scores and best in young subjects with higher APHAB-scores before HAF.
Subject(s)
Hearing Aids , Aged , Hearing Tests , Humans , Surveys and QuestionnairesABSTRACT
COVID-19 is a global health crisis that has been affecting our daily lives throughout the past year. The symptomatology of COVID-19 is heterogeneous with a severity continuum. Many symptoms are related to pathological changes in the vocal system, leading to the assumption that COVID-19 may also affect voice production. For the first time, the present study investigates voice acoustic correlates of a COVID-19 infection based on a comprehensive acoustic parameter set. We compare 88 acoustic features extracted from recordings of the vowels /i:/, /e:/, /u:/, /o:/, and /a:/ produced by 11 symptomatic COVID-19 positive and 11 COVID-19 negative German-speaking participants. We employ the Mann-Whitney U test and calculate effect sizes to identify features with prominent group differences. The mean voiced segment length and the number of voiced segments per second yield the most important differences across all vowels indicating discontinuities in the pulmonic airstream during phonation in COVID-19 positive participants. Group differences in front vowels are additionally reflected in fundamental frequency variation and the harmonics-to-noise ratio, group differences in back vowels in statistics of the Mel-frequency cepstral coefficients and the spectral slope. Our findings represent an important proof-of-concept contribution for a potential voice-based identification of individuals infected with COVID-19.
Subject(s)
COVID-19 , Voice , Acoustics , Humans , Phonation , SARS-CoV-2 , Speech Acoustics , Voice QualityABSTRACT
BACKGROUND: In the second half of life, an untreated hearing impairment seems to be associated to increase the risk of dementia, depression, fall, cognitive decline, or general helpless. Most of the affected persons are not treated adequately, e.âg. with hearing aids. The Mini-Audio-Test (MAT) is an evaluated inventory for detecting an unknown hearing loss. This study should investigate whether the MAT could be used in a representative sample of adults. METHODS: In spring 2019, in total 123 subjects have been investigated on hearing loss using the MAT. Subjects with positive results were ask to consult an ENT-physician for further diagnostics. All data have been collected anonymously and analyzed by descriptive statistics. RESULTS: In general, 79 of all questioned subjects, aged from 18 to 93 years, agreed to be investigated. At all, 63 of them answered the MAT, 22 of them intended to consult an ENT-physician; only on subject really did. CONCLUSION: Today, an unspecific use of the MAT for screening on hearing loss seems not to be successful. Thus, a structured approach, e.âg. during a regular consultation of a physician at the age of 50 years and more, seems to be more sensible. Moreover, the public should be better informed in the risks of untreated hearing loss.
Subject(s)
Deafness , Hearing Aids , Hearing Loss , Adolescent , Adult , Aged , Aged, 80 and over , Hearing Loss/diagnosis , Hearing Loss/epidemiology , Humans , Mass Screening , Middle Aged , Pilot Projects , Young AdultABSTRACT
BACKGROUND: An untreated hearing impairment could have a severe influence on the morbidity. The Mini-Audio-Test (MAT) has been developed for early detecting of a relevant hearing loss. This follow-up investigation should determine the sensitivity, specificity, and the predictive values for a minimum-level of detected hearing loss of 25, 30, 35, and 40âdB, both, in one octave-frequency between 0.5 and 4âkHz and the average of hearing loss for these four frequencies. METHODS: This survey uses data which were collected in 2016 and 2017. 943 patients parted into two groups (aged 50 to 59 years and aged 60 years and more), were investigated using the MAT. Statistical analysis on the sensitivity, specificity, and predictive values in respect were done as proportions together with the 95â%-confidence interval by a logistic regression. RESULTS: The sensitivity of the MAT was increasing in both groups of age by increasing the minimum of to be detected hearing loss. The specificity was decreasing as well, but less. The positive predictive values were higher for the older age-group and are decreasing by increasing of the minimum of to be detected hearing loss. In general, the values using the averaged hearing loss are noticeably lower than for the single frequency detecting. The values for the negative predictive values were contrary. CONCLUSION: The results for persons aged 60 years and more are worse than for the younger. Therefore, a first screening on hearing loss starting at the age of 50 years seems to be recommended urgently. Only by this, a sufficient rehabilitation of the hearing loss could be done in time so that the negative consequences of an untreated hearing impairment could be influenced positively.
Subject(s)
Deafness , Hearing Loss , Aged , Audiometry, Pure-Tone , Hearing Loss/diagnosis , Humans , Mass Screening , Middle Aged , Sensitivity and Specificity , Surveys and QuestionnairesABSTRACT
BACKGROUND: Questionnaires measuring the subjective outcome of hearing aid benefit are used in diagnostics of hearing loss additionally. Since 2012, the APHAB is part of the quality agreement for statutory insured patients in hearing aid fitting. Actually, calculating the benefit by the given formula (improvement quotient A) causes distortion by patients with negative results. In general, these dues to misinterpretation of the results. In this study, we proposed two alternative ways of calculation and compared the results together. METHODS: Using the data of 6861 hearing aid fitted patients from a database, we calculated the benefit by A and two new formulas (improvement quotient B and cumulated benefit) and compared the results together. RESULTS: The average for A was -29.02â% (SD ±â47.94) and a minimum of -637.93â%. The average for B was -13.42â% (SD ±â13.14) and a minimum of -78,63. No relevant differences by calculating the improvement quotients between A and B occurred. The average APHAB-score (EC-, BN-, and RV-subscale) was 50.70 before and 29.29 after hearing aid fitting, the average of the cumulated benefit was 21.41. CONCLUSIONS: Quotient B avoids the intrinsic potential of distortion of A in favor of patients with negative results in hearing aid benefit. It would be useful for the further developing of the quality agreement if B in exchange for A and, in addition, the cumulated benefit should be used.
Subject(s)
Deafness , Hearing Aids , Hearing Loss , Humans , Prosthesis Fitting , Surveys and QuestionnairesABSTRACT
OBJECTIVE: The APHAB questionnaire measures the subjective hearing impairment in different daily-life situations. Recently, a survey investigated the daily life relevance of APHAB-questions. This study should evaluate the influence of the daily-life relevance of each APHAB question on the answers of the APHAB questions itself. METHODS: Between July 2016 and November 2017, the amount of 517 hearing impaired out-clinic patients from 11 different ENT-offices in the state of Schleswig-Holstein, Germany, have been investigated by using the APHAB. Additionally, every APHAB question ought to be evaluated with respect to its daily life relevance using a four-step scale (often - sometimes - rarely - never) as well. The answers have been listed in a cross-table and statistically analyzed using the χ2-test and Cramérs V. RESULTS: The average age of all subjects was 67.4â years (±â 14.0â years); the gender of 45.3â % of all participants was female and 54.7â % was male. Using the χ2-test and Cramérs V, 4 questions of the EC-, 3 of the BN-, and 2 of the RV-subscale had a middle-grade level of coherence. Regarding a weak level of coherence, the results were exactly the opposite. However, most of the inverted APHAB-questions demonstrated such a weak coherence using Cramérs V (pâ <â 0.001). CONCLUSIONS: Indeed, a correlation between daily-life relevance and ranking of APHAB-questions could be demonstrated. This coherence is in a half of the APHAB-questions weak and in a half middle-grade. The reasons are unclear and need further investigation.
Subject(s)
Hearing Aids , Hearing Loss , Aged , Aged, 80 and over , Ambulatory Care Facilities , Female , Germany , Humans , Male , Middle Aged , Surveys and QuestionnairesABSTRACT
Hypersalivation describes a relatively excessive salivary flow, which wets the patient himself and his surroundings. It may result because of insufficient oro-motor function, dysphagia, decreased central control and coordination. This update presents recent changes and innovation in the treatment of hypersalivation.Multidisciplinary diagnostic and treatment evaluation is recommended already at early stage and focus on dysphagia, saliva aspiration, and oro-motor deficiencies. Clinical screening tools and diagnostics such as fiberoptic endoscopic evaluation of swallowing generate important data on therapy selection and control. Many cases profit from swallowing therapy programmes in order to activate compensation mechanisms as long compliances is given. In children with hypotonic oral muscles, oral stimulation plates can induce a relevant symptom release because of the improved lip closure. The pharmacologic treatment improved for pediatric cases as glycopyrrolate fluid solution (Sialanar®) is now indicated for hypersalivation within the E. U. The injection of botulinum toxin into the salivary glands has shown safe and effective results with long lasting saliva reduction. Here, a phase III trial is completed for Incobotulinum toxin A and, in the U. S., is indicated for the treatment of adult patients with chronic hypersalivation. Surgical treatment should be reserved for isolated cases. External radiation is judged as a safe and effective therapy when using modern 3 D techniques to minimize tissue damage. Therapy effects and symptom severity has to be followed, especially in cases with underlying neurodegenerative disease.
Subject(s)
Sialorrhea , Botulinum Toxins, Type A , Deglutition Disorders , Glycopyrrolate , Humans , Neurodegenerative Diseases , Salivary GlandsABSTRACT
Hypersalivation describes a relatively excessive salivary flow, which wets the patient himself and his surroundings. It may result because of insufficient oro-motor function, dysphagia, decreased central control and coordination. This update presents recent changes and innovation in the treatment of hypersalivation. Multidisciplinary diagnostic and treatment evaluation is recommended already at early stage and focus on dysphagia, saliva aspiration, and oro-motor deficiencies. Clinical screening tools and diagnostics such as fiberoptic endoscopic evaluation of swallowing generate important data on therapy selection and control. Many cases profit from swallowing therapy programmes to activate compensation mechanisms as long compliances are given. In children with hypotonic oral muscles, oral stimulation plates can induce a relevant symptom release because of the improved lip closure. The pharmacologic treatment improved for pediatric cases as glycopyrrolate fluid solution (Sialanar®) is now indicated for hypersalivation within the EU. The injection of botulinum toxin into the salivary glands has shown safe and effective results with long-lasting saliva reduction. Here, a phase III trial is completed for incobotulinum toxin A and, in the US, is indicated for the treatment of adult patients with chronic hypersalivation. Surgical treatment should be reserved for isolated cases. External radiation is judged as a safe and effective therapy when using modern 3D techniques to minimize tissue damage. Therapy effects and symptom severity have to be followed, especially in cases with underlying neurodegenerative disease.
Subject(s)
Sialorrhea , Germany , HumansABSTRACT
BACKGROUND: Worldwide approximately 360 million people suffer from hearing impairment, 328 million of whom are adults. Up to now there has been no systematic evaluation of any representative epidemiological data on the prevalence of hearing loss among adults in Germany. The present paper is intended to investigate this within the framework of a systematic review. METHODS: A systematic literature search was carried out in electronic databases as well as by means of hand-searching. Studies published after 1975 and indicating the prevalence or incidence of hearing impairment among German adults were included. Study selection, data extraction and additional quality assessments were made by two independent reviewers. RESULTS: By means of a systematic literature search it was possible to identify 6 sources, which provided solely cross-sectional data, whereby the reported data are based on a study population of between some hundred and 10 million people living in Germany. The prevalences ascertained showed a broad range of between 16% and 25% and varied according to age, study setting, definition of hearing loss and method of data capture. At present there are no utilizable data on the extent of the use of hearing aids. DISCUSSION: The present review demonstrates clearly that evidence-based information relating to Germany can only be made on the basis of a clear definition of hearing loss within the framework of an up-to-date and representative epidemiological study carried out with appropriate methodology. In view of the high prevalence of illnesses causing hearing impairment and of the risks to health associated with untreated hearing impairment as well as of socio-economic costs, such an epidemiological study is of great social significance.
Subject(s)
Hearing Aids/statistics & numerical data , Hearing Loss/epidemiology , Adult , Aged , Aged, 80 and over , Deafness/epidemiology , Female , Germany/epidemiology , Hearing Loss/rehabilitation , Humans , Male , Middle Aged , PrevalenceABSTRACT
BACKGROUND: In Germany, about 15 million people are suffering from hearing loss (HL), whereas only 16 % are using hearing aids. Untreated hearing loss may lead to severe complications (e. g. social isolation, depression, progress of dementia). An early and widespread screening, beginning at the age of 50, is meant to improve this shortage in medical care. By this study, the Mini-Audio-Test (MAT), a six-question and three-step answers containing questionnaire on subjective HL, should be verified on a normal collective of subjects (sensitivity (Se), specificity (Sp), positive predictive value (Ppv)). METHODS: 943 subjects (older 50 years) without any history of ear disease answered the MAT and received pure-tone audiometry. The Se, Sp, and Pv to detect a relevant HL with the MAT for the age-group < 60 years (AG1) and ≥ 60 years (AG2) were determined. RESULTS: The Se for AG1 was 0.66, the Sp 0.61, the Ppv 0.60, for AG2 the Se was 0.47, the Sp 0.80, the Ppv 0,89. CONCLUSION: Following our results, the MAT is recommended as a general screening-tool for HL in patients over 50 years of age for general practitioners. Hereby, severe secondary diseases (loss of cognitive power, risk of fall, depression, dementia) could be influenced positively.
Subject(s)
Hearing Loss/diagnosis , Hearing Tests/methods , Audiometry, Pure-Tone , General Practitioners , Humans , Mass Screening , Middle Aged , Predictive Value of Tests , Sensitivity and Specificity , Surveys and QuestionnairesABSTRACT
Introduced in the late 90s, the active middle ear implant Vibrant Soundbridge (VSB) is nowadays used for hearing rehabilitation in patients with mild to severe sensorineural hearing loss (SNHL) unable to tolerate conventional hearing aids. In experienced hands, the surgical implantation is fast done, safe and highly standardized. Here, we present a systematic review, after more than 15 years of application, to determine the efficacy/effectiveness and cost-effectiveness, as well as patient satisfaction with the VSB active middle ear implant in the treatment of mild to severe SNHL. A systematic search of electronic databases, investigating the safety and effectiveness of the VSB in SNHL plus medical condition resulted in a total of 1640 papers. After removing duplicates, unrelated articles, screening against inclusion criteria and after in-depth screening, the number decreased to 37 articles. 13 articles were further excluded due to insufficient outcome data. 24 studies remained to be systematically reviewed. Data was searched on safety, efficacy and economical outcomes with the VSB. Safety-oriented outcomes included complication/adverse event rates, damage to the middle/inner ear, revision surgery/explant rate/device failure and mortality. Efficacy outcomes were divided into audiological outcomes, including hearing thresholds, functional gain, speech perception in quiet and noise, speech recognition thresholds, real ear insertion gain and subjective outcomes determined by questionnaires and patient-oriented scales. Data related to quality of life (QALY, ICER) were considered under economical outcomes. The VSB turns out to be a highly reliable and a safe device which significantly improves perception of speech in noisy situations with a high sound quality. In addition, the subjective benefit of the VSB was found to be mostly significant in all studies. Finally, implantation with the VSB proved to be a cost-effective and justified health care intervention.
Subject(s)
Hearing Loss, Sensorineural/surgery , Ossicular Prosthesis , Speech Perception , Audiometry , Cost-Benefit Analysis , Hearing Aids , Humans , Ossicular Prosthesis/adverse effects , Ossicular Prosthesis/economics , Patient Satisfaction , Quality of Life , Reoperation , Surveys and Questionnaires , Treatment OutcomeABSTRACT
Subharmonics are often observed in running speech spectrograms that are difficult to quantify. This study investigates the relationship between rough voice quality and the presence-and amount-of subharmonics in connected speech material in a group of 35 male and 35 female speakers with voice pathology. Spectrum analysis was undertaken in 145 pathologic voices, of which 77 had subharmonics in connected speech. Only 34% of 70 subjects under investigation developed subharmonics in both connected speech and sustained phonations. All voices were judged as perceptually rough. The results of this study indicate that male and female voices with subharmonics do not differ in examined acoustic characteristics except for the modal fundamental frequency (F(0)). A small but significant difference in roughness scores between genders seems to be caused by the confounding perception of factors not related to acoustic measurements of F(0) and number of subharmonics. The degree of roughness has a significant relationship with the irregularity index, percentage of low F(0) estimates, and, to a lesser extent, power of subharmonics.
Subject(s)
Speech Acoustics , Voice Disorders , Voice Quality , Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Humans , Male , Middle Aged , Phonation , Regression Analysis , Sex Characteristics , Young AdultABSTRACT
BACKGROUND: The treatment of refractory epilepsy by vagus nerve stimulation (VNS) is a well-established therapy option for patients not suitable for epilepsy surgery and therapy refractory depressions. OBJECTIVE: To analyze surgical and technical complications after implantation of left-sided VNS in patients with therapy-refractory epilepsy and depression. METHODS: One hundred five patients receiving a VNS or VNS-related operations (n = 118) from 1999 to 2008 were investigated retrospectively. RESULTS: At the time of operation, 84 patients were younger than 18 years, with a mean age of 10.5 years. Twenty (19%) patients had technical problems or complications. In 6 (5.7%) patients these problems were caused by the operation. The device was removed in 8 cases. The range of surgically and technically induced complications included electrode fractures, early and late onset of deep wound infections, transient vocal cord palsy, cardiac arrhythmia under test stimulation, electrode malfunction, and posttraumatic dysfunction of the stimulator. CONCLUSION: VNS therapy is combined with a wide spread of possible complications. Technical problems are to be expected, including electrode fracture, dislocation, and generator malfunction. The major complication in younger patients is the electrode fracture, which might be induced by growth during adolescence. Surgically induced complications of VNS implantation are comparably low. Cardiac symptoms and recurrent nerve palsy need to be taken into consideration.
Subject(s)
Neurosurgical Procedures/adverse effects , Postoperative Complications/epidemiology , Prosthesis Implantation/adverse effects , Vagus Nerve Stimulation/adverse effects , Vagus Nerve/surgery , Adolescent , Adult , Aged, 80 and over , Arrhythmias, Cardiac/epidemiology , Arrhythmias, Cardiac/physiopathology , Arrhythmias, Cardiac/prevention & control , Child , Child, Preschool , Depressive Disorder/physiopathology , Depressive Disorder/surgery , Electrodes, Implanted/adverse effects , Electrodes, Implanted/standards , Epilepsy/physiopathology , Epilepsy/surgery , Equipment Failure/statistics & numerical data , Female , Humans , Infant , Male , Neurosurgical Procedures/methods , Postoperative Complications/physiopathology , Postoperative Complications/prevention & control , Prosthesis Implantation/methods , Retrospective Studies , Surgical Wound Infection/epidemiology , Surgical Wound Infection/physiopathology , Vagus Nerve/anatomy & histology , Vagus Nerve/physiology , Vagus Nerve Stimulation/methods , Vocal Cord Paralysis/epidemiology , Vocal Cord Paralysis/physiopathology , Vocal Cord Paralysis/prevention & controlABSTRACT
The purpose of the study was to delineate clinical and electrophysiological characteristics as well as laryngoscopical and transcranial ultrasound (TCS) findings in THAP1 mutation carriers (MutC). According to recent genetic studies, DYT6 (THAP1) gene mutations are an important cause of primary early-onset dystonia. In contrast to DYT1 mutations, THAP1 mutations are associated with primary early-onset segmental or generalised dystonia frequently involving the craniocervical region and the larynx. Blood samples from twelve individuals of three German families with DYT6 positive index cases were obtained to test for THAP1 mutations. Eight THAP1 MutC were identified. Of these, six (three symptomatic and three asymptomatic) THAP1 MutC could be clinically evaluated. Laryngoscopy was performed to evaluate laryngeal dysfunction in patients. Brainstem echogenicity was investigated in all MutC using TCS. Two of the patients had undergone bilateral pallidal DBS. In all three symptomatic MutC, early-onset laryngeal dystonia was a prominent feature. Laryngeal assessment demonstrated adductor-type dystonia in all of them. On clinical examination, the three asymptomatic MutC also showed subtle signs of focal or segmental dystonia. TCS revealed increased substantia nigra (SN) hyperechogenicity in all MutC. Intraoperative microelectrode recordings under general anesthesia in two of the patients showed no difference between THAP1 and previously operated DYT1 MutC. The presence of spasmodic dysphonia in patients with young-onset segmental or generalised dystonia is a hallmark of DYT6 dystonia. SN hyperechogenicity on TCS may represent an endophenotype in these patients. Pallidal DBS in two patients was unsatisfactory.
Subject(s)
Apoptosis Regulatory Proteins/genetics , DNA-Binding Proteins/genetics , Dystonic Disorders/diagnosis , Dystonic Disorders/physiopathology , Electrophysiology , Nuclear Proteins/genetics , Adult , Aged , DNA Mutational Analysis , Dystonic Disorders/genetics , Electroencephalography/methods , Female , Globus Pallidus/pathology , Globus Pallidus/physiopathology , Humans , Laryngeal Diseases/diagnosis , Laryngeal Diseases/etiology , Male , Microelectrodes , Middle Aged , Mutation/genetics , Phenotype , Ultrasonography, Doppler, Transcranial/methods , Voice Disorders/diagnosis , Voice Disorders/etiologyABSTRACT
OBJECTIVES/HYPOTHESIS: Patients with high-grade atresia-microtia suffer from a combined malformation of the outer and middle ears, typically leading to a severe hearing impairment. Long-term results of middle ear reconstruction with tympanoplasty are often insufficient due to persistent air-bone gaps, and new techniques in hearing rehabilitation are required. The objective of this research is to evaluate the active middle ear implant, the Vibrant Soundbridge (VSB), for hearing rehabilitation of patients with unilateral osseous aural atresia. STUDY DESIGN: Prospective analysis of a consecutive cohort of seven atresia patients (mean age = 15 years). METHODS: During plastic auricular reconstruction of unilateral atresia-microtia cases, an access through the bony atresia plate was drilled. The floating mass transducer was coupled to the stapes (or remaining stapes suprastructure), ossicular chain, or round window, depending on the anatomic needs of the patient. Audiometric testing, including pure-tone thresholds, and speech testing in quiet and noise were performed. RESULTS: : The mean threshold with the VSB activated in the free field warble tone audiometry was 23.8 dB hearing level (HL). Mean functional gain was 45.5 dB HL. Mean aided free field speech discrimination in quiet was 64% at 50 dB, 99% at 65 dB, and 100% at 80 dB. CONCLUSIONS: By circumventing the malformed middle ear and directly stimulating the cochlea, the VSB provides a new rehabilitation option for atresia patients. We conclude that the procedure is safe and effective and can be implemented in combination with outer ear reconstruction.