Das aktive Mittelohrimplantat in der Rehabilitation von sensorineuralen, kombinierten und Schallleitungs-Hörstörungen

Characterization of hearing loss in pediatric patients with osteogenesis imperfecta

The Bonebridge hearing aid (Med-EL, Innsbruck, Austria) is a semi-implantable hearing system with transcutaneous bone conduction that was implemented in Europe in 2012. It is indicated for patients with conductive and mixed hearing loss or unilateral profound sensorineural hearing loss, who do not obtain adequate benefits with conventional hearing aids and are not candidates for middle ear reconstructive surgery. The Bonebridge device, being an active transcutaneous bone conduction implant, can overcome the disadvantages of percutaneous bone conduction devices. The Vibrant Soundbridge hearing aid (Med-EL, Innsbruck, Austria) was implemented in Europe in 1996 as an implantable hearing device in the middle ear in patients with mild to severe sensorineural, conductive or mixed hearing loss with medical and audiological contraindications for the use of conventional hearing aids or with rapidly progressive sensorineural hearing loss. The Bonebridge and Vibrant Soundbridge devices, used in patients with the same indication criteria, had similar audiological results – adequate hearing rehabilitation with improvement of subjective hearing (perception of silent and noisy speech, spatial hearing, hearing quality, sound localization), a significant increase in perceived quality of life and patients’ satisfaction with the hearing aid (comfort, sound quality, cosmetic appearance). Because the Vibrant Soundbridge device requires the floating mass transducer to be coupled to the ossicles, round window, or oval window, this surgery carries a higher risk of inner ear injury than surgery with the Bonebridge device. The advantages of the Vibrant Soundbridge system, compared to the Bonebridge device, are the potential for greater stimulation power, especially at high frequencies, unilaterally stimulating the affected ear without influencing the contralateral ear through bone conduction.

On the basis of strong research, universal newborn screening should be performed before age 1 month with repeat or follow-up testing for those who do not pass performed before age 3 months and intervention started before age 6 months. On the basis of strong research and consensus statement, tympanostomy tubes should be considered for individuals with bilateral persistent middle ear effusion for 3 months or greater and a documented conductive hearing loss. On the basis of consensus statement, all children with suspected hearing loss should have an age appropriate hearing test. On the basis of strong research, the most common form of congenital hearing loss is genetic. Most of this is nonsyndromic hearing loss.

New Bone-Anchored Amplification Options for Children

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. Retrospective study. 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. 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. 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. 4.

Background and Objectives Middle ear implants (MEI) have been reported to be an effective and safe alternative for the treatment of sensorineural hearing loss (SNHL) and conductive hearing loss (CHL). This study aimed to compare the functional outcomes between SNHL and CHL in terms of audiological gains.Subjects and Method The medical records of 14 consecutive SNHL and CHL patients who underwent MEI surgeries from 2015 to 2019 by a single surgeon were retrospectively reviewed. Audiological changes using hearing aids (HA) and MEI were compared.Results In SNHL, the mean unaided air-conduction pure tone audiometry (PTA), (57.7 dB HL) decreased significantly using HA and MEI (44.7 and 41.4 dB HL), but with no significant difference from each other. Unaided word recognition score (WRS) at 65 dB HL (45.1%) was significantly improved using HA and MEI (72.6% and 76.6%), with no significant difference. In CHL, the mean unaided air-conduction and bone-conduction PTA were 77.1 (57.5-93.8) and 44.1 (26.3-57.5) dB HL. Three patients could not use conventional HAs due to otorrhea and deformity of the external auditory canal by previous surgeries and 4 patients had used conventional HAs before MEI. Though both HA and vibrant soundbridge (VSB) showed improvement of hearing thresholds (32.0 and 48.8 dB HL) and WRS (80.0% and 94.9%), it was significant only when using VSB. There was no significant difference between HA and VSB.Conclusion MEI can be an effective treatment option for both SNHL and CHL patients, especially for those who suffered from problems using conventional HA.

Branchio-oto-renal syndrome (BOR) is an autosomal dominant mutation of the EYA1 and the more recently discovered the SIX1 gene.1 The phenotype and syndrome were comprehensively described by Melnick in 1975 to include hearing loss, auricular malformations, branchial arch remnants, and renal anomalies.2 The diagnosis of BOR is made using major and minor criteria as defined by Chang et al 2004.3 However, 60% of patients who meet phenotypic criteria do not have an identifiable mutation in the EYA1 gene, leading to recent interest in the EYA-SIX regulatory system.1 The most common manifestations include hearing loss (98.5%), preauricular pits (83.6%), branchial anomalies (68.5%), renal anomalies (38.2%), and external ear abnormalities (31.5%). In terms of the imaging characteristics, the most sensitive modality remains CT of the temporal bones. The most commonly reported anomalies on temporal bone imaging include but are not limited to 1)hypoplastic apical turn of the cochlea, 2) facial nerve deviated to the medial side of the cochlea, 3) funnel-shaped internal auditory canal, and 4) patulous eustachian tube. 4 The spectrum of hearing loss in BOR is variable but most commonly presents with mixed hearing loss (50%), pure sensorineural hearing loss (25%) and pure conductive hearing loss (25%) 5. The conductive component of the hearing loss is most often the result of ossicular chain abnormalities. A 42 year old male previously diagnosed with BOR using clinical criteria presented with a conductive hearing loss. His physical exam demonstrated small external auditory canals with a normal tympanic membrane. His audiogram demonstrated a mild left sensorineural hearing loss and a maximal conductive hearing loss in the right ear. Imaging with CT revealed several findings consisted with BOR: Bilateral enlarged air-filled eustachian tubes extending from the middle ear to the nasopharynx, a widened and flared internal acoustic meatus with the nervus intermedius extending into a funnel shaped labyrinthine segment of the temporal bone and hypo-plastic horizontal canal, hypo-plastic vestibular system/epitympanum, and lateral position of the facial nerve. The incus and malleus were malformed and fixed in the attic (Fig. 1). Figure 1 Computed tomography (CT) images. Panel A: Axial view of the head demonstrating enlarged eustachian tubes (arrow). Panel B: Axial view of right internal auditory (IAC) canal demonstrating hypoplastic horizontal semicircular canal(H), and funnel shaped ... Despite the findings on CT, the patient elected to pursue a middle ear exploration prior to pursuing other rehabilitative options. At surgery, middle ear exploration revealed a very small oval window niche with no clear oval window, or stapes footplate as shown on this view with a 30° endoscope (Fig. 2). The round window niche was visible. A dehiscent facial nerve was visible at the horizontal segment. Because no mobile footplate was found there was no attempt at ossiculoplasty. The patient recovered from surgery with no change in his hearing and later went on to a Baha which he found beneficial. Figure 2 Right middle ear as viewed through a 30° endoscope during surgery. There was no clear stapes footplate, but a narrow oval window (OW) niche. The round window (RW) niche was visible. The OW and RW are labeled to the right above the structures. ... The extreme ossicular abnormalities in this patient with BOR made his maximal conductive hearing loss not amenable to ossiculoplasty. We ultimately failed in our attempt to restore his conductive hearing loss due to agenesis of the oval window and lack of a mobile footplate. The intraoperative endoscopy revelaed the lack of suitable anatomy for an ossicular replacement prosthesis. The findings in our patient highlight the diagnostic findings in BOR on CT scan of the temporal bone and correlate well with his clinical findings. Although BOR patients may not be a homogeneous in terms of their middle ear anatomy, this patient suggests ossiculoplasty may not be a viable option in this population.

Middle ear active implants, such as the Vibrant Soundbridge (VSB), offer an alternative to reconstructive surgery and other implantable hearing aid systems for patients with conductive, mixed, or sensorineural hearing loss. The primary objective of this work is to describe the auditory results obtained with VSB in our patient cohort, measuring the auditory gain in terms of average tonal thresholds and spoken word discrimination at 65 dB. Secondly, auditory gain differences between different types of hearing loss, coupling to the ossicular chain compared to round and oval windows, and the impact of open versus more conservative surgical approaches, were analyzed. A cross-sectional observational study, with retrospective data collection, was conducted at a tertiary care center. Clinical and audiometric data pre- and post-implantation were included, from patients who underwent VSB device placement surgery between 2001 and 2024. 55 patients with an average age of 62.58 ± 17.83 years and a slight preference in terms of the female gender (52.72%) were included in the study. The average gain in the PTA for all types of hearing loss was 41.56 ± 22.63 dB, while for sensorineural hearing loss (SNHL) the gain was 31.04 ± 8.80 dB. For mixed-conductive hearing loss (C-MHL) a gain of 42.96 ± 17.70 was achieved, notably, in terms of absolute values, at frequencies of 4000 and 6000 Hz, with gains reaching 49.25 ± 20.26 dB at 4 K and 51.16 ± 17.48 dB at 6 K. In terms of spoken word discrimination, for all types of hearing loss, an improvement of 75.20 ± 10.11% was achieved. However, patients with C-MHL exhibited an approximately 13% higher gain compared to those with SNHL (69.32 ± 24.58% vs. 57.79 ± 15.28%). No significant differences in auditory gain were found between open and closed surgical techniques, nor in the proportion of adverse effects, when comparing one technique with the other. The VSB is effective in improving hearing in patients with mixed, conductive, and sensorineural hearing loss, with significant gains at high frequencies, especially through the round window membrane approach. The choice of surgical technique should consider the patient's anatomical characteristics and specific needs in order to optimize auditory outcomes and minimize postoperative complications.

Background: Most previous studies are separate dosimetric analyses of conductive or sensorineural hearing loss, and they are not conducive to a comprehensive assessment of auditory radiation damage.Aims/objectives: Our study aimed to evaluate the long-term incidence of sensorineural hearing loss (SNHL) or conductive hearing loss (CHL) in patients with nasopharyngeal carcinoma (NPC) after intensity-modulated radiation therapy (IMRT), and to investigate the relationship between SNHL or CHL and patient factors, treatment-related factors, and radiation dose parameters.Material and methods: Seventy patients (117 ears) with NPC, who were also treated with IMRT in our hospital from 2006 to 2014, were retrospectively analyzed. Radiation doses to the Eustachian tube (ET), middle ear (ME), cochlear (Co), and internal auditory canal (IAC) were assessed. Pure tone audiometry and impedance audiometry were performed before and during the follow-up period. The relationships between low-frequencies (0.5–2 kHz) or high-frequency (4 kHz) SNHL/CHL and radiotherapy dose parameters were analyzed.Results: Of the 117 ears studied, 7.69% had low-frequency SNHL, 35.9% had high-frequency SNHL, 23.93% had low-frequency CHL, and 18.80% had high-frequency CHL. The incidence of high-frequency CHL was higher in the T4 group than in the T (1–3) group (p < .05). When IAC Dmax > 42.13 Gy or IAC Dmean > 32.71 Gy, the risk of high-frequency SNHL increased in NPC patients. When ME Dmax > 44.27 Gy, ME Dmean > 29.28 Gy, or ET Dmax > 57.23 Gy, the risk of high-frequency CHL in NPC patients increased.Conclusions and significance: SNHL and CHL remain common ear complications after IMRT for NPC. IAC Dmax, IAC Dmean, ME Dmax, ME Dmean, and ET Dmax all need to be carefully considered during the IMRT treatment protocol.

Postoperative complications of myringotomy and tube placement often include otorrhea, tympanosclerosis, and tympanic membrane perforation. However, the incidence of sensorineural or conductive hearing loss has not been documented. Recent efforts to curb the use of preoperative audiometric testing requires documentation of this incidence. To define the incidence of conductive and sensorineural hearing loss associated with myringotomy and tube placement. A retrospective chart review of 550 patients undergoing myringotomy and tube placement was performed. A total of 520 patients undergoing 602 procedures (1204 ears), including myringotomy and tube placement, were assessed for preoperative and postoperative sensorineural and conductive hearing loss. No patient developed a postoperative sensorineural or conductive hearing loss. All patients resolved their conductive hearing loss after myringotomy and tube placement. There was a 1.3% incidence of preexisting sensorineural hearing loss. The incidence of sensorineural or conductive hearing loss after myringotomy and tube placement is negligible and the use of preoperative audiometric evaluation may be unnecessary in selected patients, but further studies need to be done to corroborate this small data set.

Confirm the safety and performance of an implantable active middle ear implant in patients who have been implanted for a minimum of 5 years before 2022. Otolaryngology departments of 4 German and Austrian hospitals. Retrospective, multicenter, longitudinal, open-label case series study. Six hundred forty-seven ears treated for conductive, mixed, or sensory neural hearing loss implanted for 5 years (622 aged 18y or older, 23 aged 5 to 17y, and 2 under 5y of age) at time of implantation. Implantation of the Vibrant Soundbridge (VSB) system, a partially implantable active middle ear implant (AMEI). Patients' audiometric pure tone averages (PTA4) (0.5, 1, 2, 4 kHz) thresholds (bone conduction, and sound field) and speech perception [word recognition scores (WRS)] were retrospectively collected up to 24 years postoperatively. Complications were recorded with focus on revision surgery and explantations. Subgroups were vibrant ossicular prothesis models 502 and 503 as well as conductive+mixed hearing loss (C/MHL), sensory neural hearing loss (SNHL), adults and children. Safety was established by stable bone conduction thresholds with a mean difference of -3.86 dB 5 years or later after implantation. Safety was further established by no technical failures reported for the VORP 503 and only 7 for the VORP 502 and high survival rates of 84.06% 10 years after implantation. For all subgroups (VORP 502, VORP 503, C/MHL, SNHL, adults and children) mean BC-PTA4 thresholds remained stable at 5 and 10 (only VORP 502) years postoperatively. Paired mean sound field PTA4 thresholds and word recognition scores are significantly improved as tested by post hoc analysis 5 years or later after implantation with functional gains of 29.87 dB (ALL), 28.64 dB (VORP 502), 36.22 dB (VORP 503), 30.93 dB (C/MHL), 26.95 dB (SNHL), 29.95 dB (adults), 28.63 dB (children) and word recognition scores of 59.81% (ALL), 59.93% (VORP 502), 58.33% (VORP 503), 63.35% (C/MHL), 54.52% (SNHL), 59.73% (adults), 61.25% (children). Furthermore, 5 years or later after implantation the absolute mean sound field PTA4 thresholds (37.45 dB HL) and word recognition scores (66.74%) remain significantly improved over time at as tested with linear mixed-effects model (LMM) fitted with restricted maximum likelihood estimation (REML). The findings of this study demonstrate that this AMEI remains safe and effective for up to 10 years with the vibrant ossicular prosthesis (VORP) 502 model and up to 5 years with the VORP 503 model.

Evaluation of hearing function in patients with lichen planus

Objective: Our purpose was to evaluate the results of Vibrant Soundbridge (VSB) in conductive or mixed hearing loss. Materials and Methods: Twenty-five adult patients (29 ears) with a mixed or conductive hearing loss and various etiologies were included in this retrospective study. The preoperative ipsilateral pure tone average was 71 ± 3.0 dB, and the average bone conduction threshold was 42 ± 2.8 dB (n = 29). The transducer was placed on the long apophysis of the incus (n = 16), in the round window (n = 10) or on the stapes (n = 3). Results: No complications were noted. The bone conduction threshold remained unchanged. VSB was activated in all cases. The postoperative pure tone average without VSB was 63 ± 3.9 dB (n = 24) and with VSB in free-field condition 24 ± 2.1 dB (n = 22). Conclusions: VSB is safe and efficacious for auditory rehabilitation in conductive and mixed hearing losses.

Middle ear implants (MEIs) such as the Vibrant Soundbridge (VSB) are attractive and alternative treatments for patients with conductive, sensorineural, and mixed hearing loss who do not benefit from, or who choose not to wear, conventional hearing aids (HAs). Recent studies suggest that MEIs can provide better improvements in functional gain, speech perception, and quality of life than HAs, although there are certain risks associated with the surgery which should be taken into consideration, including facial nerve or chorda tympanic nerve damage, dysfunctions of the middle and inner ears, and future device failure/explantation. In Japan, a multi-center clinical trial of VSB was conducted between 2011-2014. A round window vibroplasty via the transmastoid approach was adopted in the protocol. The bony lip overhanging the round window membrane (RWM) was extensively but very carefully drilled to introduce the Floating Mass Transducer (FMT). Perichondrium sheets were used to stabilize the FMT onto the RWM. According to the audiological criteria, the upper limit of bone conduction should be 45 dB, 50 dB, and 65 dB from 500 Hz to 4, 000 Hz. Twenty-five patients underwent the surgery so far at 13 different medical centers. The age at the surgery was between 26-79 years old, and there were 15 males and 10 females. The cause of conductive or mixed hearing loss was middle ear diseases in 23 cases and congenital aural atresia in two cases. The data concerning on the effectiveness and safety of VSB was collected before the surgery and 20 weeks after the surgery. Significant improvements of free-field Pure Tone Audiogram (PTA) from 250 Hz to 8, 000 Hz were confirmed (p < 0.001). Hearing gain up to 40 dB was achieved in the 1, 000 Hz to 4, 000 Hz range. No deterioration in either air conduction or bone conduction at PTA was noted at 20 weeks after the surgery. Monosyllable speech perception in both quiet and noisy conditions improved significantly (p < 0.001). The speech discrimination score in both quiet and noisy conditions improved significantly too (p < 0.001). In the future, it is likely that there will be an increasing population even in Japan that will meet the criteria for MEIs such as VSB. However, the long-term efficacy and safety of these devices should be established.

The number of potential options for rehabilitation of patients with conductive or mixed hearing loss is continually expanding. To be able to inform patients and other stakeholders, there is a need to identify and develop patient-centred outcomes for treatment of hearing loss. To identify outcome measures in the physical core area used when reporting the outcome after treatment of conductive and mixed hearing loss in adult patients. Systematic review. Systematic review of the literature related to reported physical outcome measures after treatment of mixed or conductive hearing loss without restrictions regarding type of intervention, treatment or device. Any measure reporting the physical outcome after treatment or intervention of mixed or conductive hearing loss was sought and categorised. The physical outcome measures that had been extracted were then grouped into domains. The literature search resulted in the identification of 1434 studies, of which 153 were selected for inclusion in the review. The majority (57%) of papers reported results from middle ear surgery, with the remainder reporting results from either bone conduction hearing devices or middle ear implants. Outcomes related to complications were categorised into 17 domains, whereas outcomes related to treatment success was categorised into 22 domains. The importance of these domains to patients and other stakeholders needs to be further explored in order to establish which of these domains are most relevant to interventions for conductive or mixed hearing loss. This will allow us to then assess which outcome measures are most suitable for inclusion in the core set.