Multicenter Results With an Active Transcutaneous Bone Conduction Implant in Patients With Single-sided Deafness.

OBJECTIVE: To evaluate the audiological and subjective benefit from hearing rehabilitation with an active bone conduction implant in subjects with single-sided sensorineural deafness (SSD). STUDY DESIGN: Prospective, multicenter, single-subject repeated measures. SETTING: Tertiary referral center, five clinics in Germany and Switzerland. PATIENTS: Seventeen subjects aged 18 years and older with severe to profound unilateral sensorineural hearing loss and contralateral normal hearing were followed up for 24 months. INTERVENTION: Active bone conduction implant. MAIN OUTCOME MEASURES: Speech understanding in noise was assessed in three situations: with signal from front, deaf, or normal hearing side (with noise from front in all set-ups). Subjective benefit was evaluated using the Speech, Spatial, and Qualities of Hearing (SSQ-B) and Bern Benefit in Single-Sided Deafness (BBSS) questionnaire. RESULTS: When the signal was coming from the deaf side the mean improvement of the speech reception threshold in noise ranged from 1.5 up to 2.2 dB with the device and was statistically and clinically significant at all tested timepoints. No significant difference between the aided and unaided situation was found when signal and noise were coming from the front. With the signal from the normal hearing side no clinically significant difference, that is, greater than 1 dB between the aided and unaided situation was found. The SSQ-B and BBSS questionnaire showed an overall improvement with no significant difference between time points. CONCLUSIONS: The study demonstrates long-term efficacy and benefit of the device in adults with SSD. Patients reported substantial and persistent subjective benefit from the active bone conduction implant.

The vibrating ossicular prosthesis in children and adolescents: a retrospective study.

PURPOSE: The primary objective of the retrospective study was to collect speech intelligibility data on children and adolescents implanted with the vibrating ossicular prosthesis (VORP) 503. METHODS: This was a retrospective, multicentre study on 55 children and adolescents from 6 German clinics aged between 5 and 17 years suffering from mixed or conductive hearing loss implanted with a VORP 503. Pre- and postoperative bone-conduction pure tone thresholds were measured at 0.5, 1, 2 and 4 kHz, and word recognition scores in the unaided and VORP 503-aided conditions using monosyllabic speech intelligibility tests measured at 65-dB sound pressure level (SPL) were determined. RESULTS: Mean pre- and postoperative bone-conduction thresholds remained unchanged, showing the preservation of inner ear hearing. Speech intelligibility assessed in quiet at 65-dB SPL improved on average from 24.5% (SD ± 25.4) unaided to 86.4% (SD ± 13.4) aided. The average improvement of 61.9% (SD ± 25.3) was clinically and statistically significant. A total of three complications were found in the medical records of 55 subjects. The responsible investigators judged these events as procedure related. CONCLUSION: The treatment of children suffering from conductive or mixed hearing loss with the VORP 503 implant demonstrates excellent aided benefit in terms of speech understanding and only minor complications.

International Consensus Recommendations on Microtia, Aural Atresia and Functional Ear Reconstruction.

The aim of this report is to provide international recommendations for functional ear reconstruction in patients with microtia and aural atresia. All patients with microtia and external auditory atresia should be seen in the setting of a multidisciplinary team and agreed treatment outcomes should be measured, so that techniques, approaches, and results can be compared. The methods are expert opinion from the members of the International Microtia and Atresia Workgroup (IMAW). The consensus recommendations reported herein take into account the variability in practice patterns present among experts in the field; the degree of consensus was quantified by presenting the percentage of above authors who agree or partially agree with each statement. Recommendations include the definition and classification of microtia/atresia, treatment of microtia, treatment of congenital aural atresia, flowchart of functional ear reconstruction, and future research directions. Patients with microtia and aural atresia can be guided by the consensus recommendations provided herein.

A Transcutaneous Active Middle Ear Implant (AMEI) in Children and Adolescents: Long-term, Multicenter Results.

OBJECTIVE: Evaluation of the long-term safety and performance of an active middle ear implant (AMEI) in the treatment of hearing loss in children and adolescents with a primary focus on improvement in speech discrimination. STUDY DESIGN: Prospective, multicentric, single-subject repeated-measures design in which each subject serves as his or her own control. SUBJECTS: Thirty-one pediatric subjects aged 5 to 17 years. INTERVENTION: Implantation of an active middle ear implant. METHODS: Improvement in word recognition scores, speech reception thresholds (SRT) in quiet and noise, in addition to air conduction, bone conduction, and sound field thresholds were evaluated in two age groups. RESULTS: Residual hearing did not change over time and speech intelligibility significantly improved and remained stable after 36 months. Children aged 5 to 9 improved in WRS from 21.92 to 95.38% and in SRT in quiet and in noise respectively from 62.45 dB SPL (sound pressure level) and +1.14 dB SNR to 42.07 dB SPL and -4.45 dB SNR. Adolescents aged 10 to 17 improved in WRS from 12.78 to 84.71% and in SRT in quiet and in noise respectively from 63.96 dB SPL and +3.32 dB SNR to 35.31 dB SPL and -4.55 dB SNR. CONCLUSIONS: The AMEI, under investigation, is a safe treatment for children and adolescents, and significantly improved audiological performance that remains stable on the long-term scale (up to 36 mo postimplantation). In general, all adult-related issues and questions regarding safety and performance can also be applied to the pediatric population, as no apparent specific issues developed.

Hearing Rehabilitation in Congenital Middle Ear Malformation.

Microtia and atresia cause significant conductive hearing loss of up to 60 dB HL. The bilateral cases suffer from severely restricted communication abilities and require immediate acoustic stimulation. There is also growing evidence that unilateral cases benefit from an early and selective stimulation of the affected side. Hearing restoration can be performed in selected cases of minor malformation by classic middle ear reconstruction. However, the majority of patients presumably benefit better from a hearing aid. There are 3 main types: active middle ear implants, active bone conduction implants and passive bone conduction implants. All implants improve speech perception, speech recognition, the signal-to-noise ratio and directional hearing. The extent varies among implants and requires further studies. Decision making on the implant type depends on the extent of malformation and hence the preoperative imaging. New scoring systems provide reliable risk stratification. Second it depends on the age of the patient. The active middle ear implants provide a selective stimulation of the affected side and are beneficial if implanted in the first years of life during the maturation period of the auditory system. In conclusion, hearing rehabilitation of congenital atresia should be performed as early as possible. This includes not only the bilateral but also the unilateral affected patients.

Mammary analogue secretory carcinoma of salivary glands: diagnostic pitfall with distinct immunohistochemical profile and molecular features.

Mammary analogue secretory carcinoma (MASC) is a newly defined entity among salivary gland malignancies which has just been established in the 4th edition of the WHO classification of head and neck tumors. MASC (synonym: secretory carcinoma) are characterized by a specific rearangement of the ETV6 gene locus. Here, we present a series of 3 MASC cases including clinical data with follow-up for up to 26 months. All tumours immunhistochemically displayed strong positivity for cytokeratin 7, and mammaglobin, focal positivity for S100, cytokeratin 5/6 and muc-4. In contrast, immunhistochemical stainings against cytokeratin 14, hormon receptors, Her2/neu, androgen receptor and prostate-specific antigen were consistently negative. FISH analysis showed translocation of the ETV6 gene locus in the majority of tumour cell nuclei. During clinical follow-up, no local relapse or metastasis was detected. As these carcinomas are clinically and radiologically indistinguishable from other salivary gland tumours and as therapeutic approaches and prognosis might differ, we need to be able to diagnose MASC correctly.

Systematic review to evaluate the safety, efficacy and economical outcomes of the Vibrant Soundbridge for the treatment of sensorineural hearing loss.

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.

Measurement of Vibration Detection Threshold and Tactile Spatial Acuity in Human Subjects.

Tests that allow the precise determination of psychophysical thresholds for vibration and grating orientation provide valuable information about mechanosensory function that are relevant for clinical diagnosis as well as for basic research. Here, we describe two psychophysical tests designed to determine the vibration detection threshold (automated system) and tactile spatial acuity (handheld device). Both procedures implement a two-interval forced-choice and a transformed-rule up and down experimental paradigm. These tests have been used to obtain mechanosensory profiles for individuals from distinct human cohorts such as twins or people with sensorineural deafness.

The Rib Cartilage Concept in Microtia.

There is a long history of rib cartilage use in reconstruction for microtia. This article aims to describe the modern techniques of autologous auricular reconstruction in detail, to elucidate the modifications for certain anatomical or disease-related conditions, and to contrast the different strategies used by other surgeons. The two mainstays of successful reconstruction with excellent results are the fabrication of the framework and soft tissue management. A three-dimensional framework mimics the curves, indentations, and peaks of a normal ear with maximal stability. Recently, the creation of a conchal bowl and an additional tragal strut has become a standard procedure. Soft tissue management is highly specific to each patient and equally contributes to the final result of the procedure. The correct position of the incision line and the trimming of the local flaps require thorough planning. Most of the individual modifications must be made in soft tissue management, particularly in patients with a low hairline and facial asymmetry. Autologous auricular reconstruction is a standard procedure that needs to be individualized to each patient. However, it is challenging and requires intensive training. In experienced hands, patients experience excellent results.

Patient selection for upper airway stimulation: is concentric collapse in sleep endoscopy predictable?

PURPOSE: Upper airway stimulation is an effective treatment option for obstructive sleep apnea after failure of positive airway pressure (PAP) therapy. To ensure a therapeutic effect, closed-loop hypoglossal nerve implants require the absence of palatal complete concentric collapse during sleep endoscopy. The frequency and potential predictors of this exclusion criterion are unknown. METHODS: Over a 2-year period, 74 consecutive patients with sleep apnea who sought PAP alternatives were evaluated with sleep endoscopy using propofol. The influence of sleep apnea severity and anthropometric characteristics as predictors of sleep endoscopy results was investigated. RESULTS: One-fifth of all patients showed a concentric collapse. Gender and age did not predict the presence of concentric collapse, but higher body mass and apnea hypopnea index values were predictive (p = 0.011; e.g., 0.026). The most commonly used body mass index values for upper airway stimulation indications demonstrated acceptable specificity (BMI 32 kg/m(2) 0.71, 95 % confidence interval = 0.57, 0.82; e.g., 35 kg/m(2) 0.81, 95 % confidence interval = 0.69, 0.90). Despite the association with overweight, a significant number of severely overweight patients had no concentric palatal collapse. CONCLUSIONS: Concentric collapse is a somewhat common condition encountered in sleep endoscopy evaluations of PAP alternatives, and cannot be sustainably predicted with anthropometric or sleep assessments. Sleep surgeons should be aware of the possibility of concentric collapse, especially in candidates who are more overweight and have severe sleep apnea. Sleep endoscopy can be useful for providing information about continuous positive airway pressure (CPAP) alternatives or to motivate patient adherence to treatment.

Trauma Management of the Auricle.

Smaller injuries of the auricle, such as lacerations without tissue loss, have more or less standardized treatment protocols that require thorough wound closure of each affected layer. Even extended lacerations of larger parts of the ear quite often heal with only minor irregularities. New in vivo diagnostic tools have aided the understanding of this outstanding "skin flap behavior." At the other end of the trauma severity spectrum are partial or complete amputations of the ear. Here, the debate has become more intense over the last decade. There were numerous reports of successful microvascular reattachments in the 1990s. Consequently, pocket methods and their variations have received increasing attention because the results seem to be convincing. Nevertheless, the pressure damage due to banking larger parts of the elastic cartilage in the mastoid region is tremendous, and the tissue for secondary reconstruction is severely injured. Particularly in cases of acute trauma with relevant concomitant injuries to the patient and in cases in which the amputated area is in a critical state, direct wound closure is a straightforward and safe option. Subsequent thoughtfully planned secondary reconstruction using ear or rib cartilage, or even allogenous material as an ear framework, can achieve excellent aesthetic results.

The Vibrant Soundbridge in Children and Adolescents: Preliminary European Multicenter Results.

OBJECTIVE: Evaluation of safety and efficacy of the Vibrant Soundbridge in the treatment of hearing loss in children and adolescents with primary focus on improvement in speech discrimination. STUDY DESIGN: Prospective, single-subject repeated-measures design in which each subject serves as his/her own control. SETTING: Tertiary referral center. PATIENTS: Nineteen patients aged 5 to 17 years. INTERVENTION: Implantation of an active middle ear implant. MAIN OUTCOME MEASURE: Improvement in word recognition scores, speech reception thresholds, and signal-to-noise ratios (SNRs) were evaluated, in addition to air and bone conduction. Oldenburger Kids Satztest/Oldenburger Satztest sentences and Göttinger/Freiburger monosyllables at 65-dB hearing level were tested in two age groups. RESULTS: Significant speech discrimination improvement was seen in all patients after 6 months. In children 5 to 9 years old, mean monosyllable recognition improved from 28.9% (unaided) to 95.5% (Soundbridge-aided). Aided 50% sentence discrimination at 44.1 dB and SNR of -4.9 dB were measured. In patients 10 to 17 years old, mean word recognition improved from 18.5% to 89.0%, sentence reception threshold improved to 40.2 dB, and SNR to -3.6 dB. Comparison between age groups indicated a slight trend toward quicker adaptation by older subjects. However, after initial adjustment, a higher level of overall benefit was seen at 6 months in younger children. CONCLUSIONS: Currently, the only middle ear implant approved for pediatric patients, the Vibrant Soundbridge, provides an option in cases of congenital aural atresia or disease-induced defects, when surgical intervention and reconstruction is indicated. The 6-month results in this comparatively large study population validated conclusions found in previous trials.

Water-induced finger wrinkles do not affect touch acuity or dexterity in handling wet objects.

Human non-hairy (glabrous) skin of the fingers, palms and soles wrinkles after prolonged exposure to water. Wrinkling is a sympathetic nervous system-dependent process but little is known about the physiology and potential functions of water-induced skin wrinkling. Here we investigated the idea that wrinkling might improve handling of wet objects by measuring the performance of a large cohort of human subjects (n = 40) in a manual dexterity task. We also tested the idea that skin wrinkling has an impact on tactile acuity or vibrotactile sensation using two independent sensory tasks. We found that skin wrinkling did not improve dexterity in handling wet objects nor did it affect any aspect of touch sensitivity measured. Thus water-induced wrinkling appears to have no significant impact on tactile driven performance or dexterity in handling wet or dry objects.

Amplification options in unilateral aural atresia: an active middle ear implant or a bone conduction device?

BACKGROUND: There is no consensus on treatment of patients with congenital unilateral aural atresia. Currently, 3 intervention options are available, namely, surgical reconstruction, application of a bone-conduction device (BCD), or application of a middle ear implant. OBJECTIVE: The present study aims to compare the BCD with the application of a middle ear implant. We hypothesized that cross-hearing (stimulating the cochlea by means of bone conduction contralateral to the implanted side) would cause BCD users to have difficulty performing localization tasks. METHODS: Audiologic data of 4 adult patients with a middle ear implant coupled directly to the cochlea were compared with data of 4 adult patients fitted with an osseointegrated BCD. All patients were fitted during adulthood. The emphasis of this study is on directional hearing. RESULTS: The middle ear implant and the BCD improved sound localization of patients with congenital unilateral aural atresia. Unaided scores demonstrate a large variation. CONCLUSION: Our results demonstrate that there was no advantage of the middle ear implant over the BCD for directional hearing in patients who had no amplification in childhood. The BCD users had the best bandwidth.

Grading system for the selection of patients with congenital aural atresia for active middle ear implants.

INTRODUCTION: Active middle ear implants (aMEI) are being increasingly used for hearing restoration in congenital aural atresia. The existing gradings used for CT findings do not meet the requirements for these implants. Some items are expendable, whereas other important imaging factors are missing. We aimed to create a new grading system that could describe the extent of the malformation and predict the viability and challenges of implanting an aMEI. METHODS: One hundred three malformed ears were evaluated using HRCT of the temporal bone. The qualitative items middle ear and mastoid pneumatization, oval window, stapes, round window, tegmen mastoideum displacement and facial nerve displacement were included. An anterior- and posterior round window corridor, oval window and stapes corridor were quantified and novelly included. They describe the size of the surgical field and the sight towards the windows. RESULTS: The ears were graded on a 16-point scale (16-13 easy, 12-9 moderate, 8-5 difficult, 4-0 high risk). The strength of agreement between the calculated score and the performed implantations was good. The comparison of the new 16-point scale with the Jahrsdoerfer score showed that both were able to conclusively detect the high-risk group; however, the new 16-point scale was able to further determine which malformed ears were favorable for aMEI, which the Jahrsdoerfer score could not do. CONCLUSION: The Active Middle Ear Implant Score for aural atresia (aMEI score) allows more precise risk stratification and decision making regarding the implantation. The use of operative corridors seems to have significantly better prognostic accuracy than the Jahrsdoerfer score.

Influence of silicone gel on standardized postoperative scars.

BACKGROUND: Silicone gel is one therapeutic approach in the treatment and prevention of excessive scarring. The likely mechanism of action is the hydration of the tissue. This should lead to reduced angiogenesis and capillary blood flow. The efficacy is still controversial and the evidence base, insufficient. The aim of this prospective and standardized study is to investigate silicone gel in the preventive treatment of scars. PATIENTS AND METHODS: Included in the study were 20 patients with costal cartilage harvest. Half of a standard chest scar was treated for three months with a silicone gel. The other half served as an internal control. After three months both scar sides were compared subjectively by visual analog scale and objectively by elasticity, moisture and color measurements. RESULTS: Of 19 patients 8 had a better subjective result in the treated half. In one subject, no difference was seen. A worse subjective result in the treated half was seen in 10 out of 19. The objective measurements showed no significant difference. A correlation between the different results was not seen. CONCLUSIONS: The use of silicone gel caused subjective differences within the same scar (worsening as well as improvement of the appearance). Positive effects were not detectable in the investigated parameters.

The Lübeck flowchart for functional and aesthetic rehabilitation of aural atresia and microtia.

OBJECTIVE: Current strategies for functional rehabilitation of microtia-atresia patients with bone-anchored implants or surgical atresia repair have been extended by the feasibility of active middle ear implants. The aim of the present research is to evaluate a new flowchart of the treatment of these patients that considers active middle ear implants. PATIENTS: Congenital aural atresia and microtia. INTERVENTION(S): Bilateral cases are provided with a conductive hearing aid after birth and implanted with an active middle ear implant within the second year. Unilateral cases are provided with a conductive hearing aid and implanted with a middle ear or bone-conduction device in early childhood. Unilateral cases without amplification in the vulnerable time after birth are carefully selected for late implantation. At age 8 to 10, the auricular reconstruction is completed. MAIN OUTCOME MEASURE(S): Feasibility of implantation irrespective of age, functional gain in audiometry. RESULTS: The results of early implantation are as good as those previously published for adolescents. Mean reaction threshold with the Vibrant Soundbridge was 21 dB. Mean functional gain was 48 dB. The local tissues are unaltered and ready for auricular reconstruction. CONCLUSION: Active middle ear implants allow early and selective stimulation of the auditory pathway in children with congenital conductive hearing loss and are expected to lead to the normal development of the binaural hearing functions. To date, it is the only option if the stimulation is to be started at the age of 12 to 18 months. This was implemented into a new flowchart for aural atresia-microtia.

A genetic basis for mechanosensory traits in humans.

In all vertebrates hearing and touch represent two distinct sensory systems that both rely on the transformation of mechanical force into electrical signals. There is an extensive literature describing single gene mutations in humans that cause hearing impairment, but there are essentially none for touch. Here we first asked if touch sensitivity is a heritable trait and second whether there are common genes that influence different mechanosensory senses like hearing and touch in humans. Using a classical twin study design we demonstrate that touch sensitivity and touch acuity are highly heritable traits. Quantitative phenotypic measures of different mechanosensory systems revealed significant correlations between touch and hearing acuity in a healthy human population. Thus mutations in genes causing deafness genes could conceivably negatively influence touch sensitivity. In agreement with this hypothesis we found that a proportion of a cohort of congenitally deaf young adults display significantly impaired measures of touch sensitivity compared to controls. In contrast, blind individuals showed enhanced, not diminished touch acuity. Finally, by examining a cohort of patients with Usher syndrome, a genetically well-characterized deaf-blindness syndrome, we could show that recessive pathogenic mutations in the USH2A gene influence touch acuity. Control Usher syndrome cohorts lacking demonstrable pathogenic USH2A mutations showed no impairment in touch acuity. Our study thus provides comprehensive evidence that there are common genetic elements that contribute to touch and hearing and has identified one of these genes as USH2A.