Middle Ear Implants Research Articles

Management of Congenital Aural Atresia and Microtia

Microtia is commonly associated with aural atresia, which will cause cosmetic problems as well as hearing impairment. The management of microtia and aural atresia should improve the aesthetic of the external ear and enhance functional hearing. The reconstruction of the auricle can be performed with autologous rib cartilage or a porous polyethylene framework. Surgeons ideally perform the autologous costal cartilage method at 9 years old or higher considering the development of costal cartilage. For porous polyethylene microtia repair, surgical reconstruction can be performed after 5 years of age. Atresiaplasty can be performed in patients who have normal inner ear function and a well-developed middle ear. The Jahrsdoerfer grading scale is used for assessment of middle ear anatomy and scoring 7 or higher expects good results. The other options for hearing rehabilitation include bone conduction hearing devices or middle ear implants. The surgery for bone conduction devices or middle ear implants can be proposed after 5 years of age. Atresiaplasty is recommended in combination with or after autologous rib graft microtia repair and before porous polyethylene microtia repair. The implantation of the hearing devices should not interfere with auricular reconstruction. It can be performed after autologous rib graft microtia repair or combined with ear elevation.

Application of an Intraoperative Auditory Brainstem Response Measurement System in Active Middle Ear Implant Revision Surgery.

To investigate the benefit of intraoperative auditory brainstem response (ABR) measurements in revision active middle ear implant surgery. Retrospective data analysis. Tertiary referral center with a large active middle ear implant program. Intraoperative ABR thresholds, audiogram, sound field thresholds, speech understanding in the Freiburger monosyllabic word test. Fourteen patients with active middle ear implant revision surgery. The application of the ABR measurement resulted in improved sound field thresholds and enhanced speech understanding. Analysis revealed a significant correlation of intraoperative gain in ABR threshold with the postoperative gain in sound field thresholds. ABR monitoring can be a useful tool to provide information intraoperatively about the coupling efficiency of the FMT. Especially in revision surgeries, this might help to improve postoperative hearing success.

Update on surgical and nonsurgical treatment options for age-related hearing loss.

Age-related hearing loss (ARHL) is one of the most common chronic conditions that impacts on everyday life far beyonds speech understanding. Chronic hearing loss has been associated with social isolation, depression, and cognitive decline. Early diagnosis and appropriate treatment are recommended. To give an overview of surgical and non-surgical treatment options for ARHL and the gap between the high prevalence of ARHL and its inadequate treatment to date. A selective literature search was carried out in PubMed. In case of mild to moderate hearing loss, provision of air conduction hearing aids is still the method of choice as it leads to alarge benefit in speech understanding and hearing-specific quality of life, and to a slight improvement in overall quality of life. Implantable middle ear systems are used for the treatment of special types of hearing impairment. In case of severe to profound hearing loss, cochlear implantation should be considered; however, only asmall number of older people with hearing loss are supplied with hearing aids or cochlear implants despite the well-known benefits of both. This also applies to high-income countries where the costs are covered by health insurance funds. Considering the low rate of properly treated people with hearing loss, large-scale screening programs, including better counselling of older people, should be developed.

Using Stapes Velocity to Estimate the Efficacy of Mechanical Stimulation of the Round Window With an Active Middle Ear Implant.

To test a method to measure the efficacy of active middle ear implants when coupled to the round window. Data previously published in Koka et al. ( Hear Res 2010;263:128-137) were used in this study. Simultaneous measurements of cochlear microphonics (CM) and stapes velocity in response to both acoustic stimulation (forward direction) and round window (RW) stimulation (reverse direction) with an active middle ear implant (AMEI) were made in seven ears in five chinchillas. For each stimulus frequency, the amplitude of the CM was measured separately as a function of intensity (dB SPL or dB mV). Equivalent vibrational input to the cochlea was determined by equating the acoustic and AMEI-generated CM amplitudes for a given intensity. In the condition of equivalent CM amplitude between acoustic and RW stimulation-generated output, we assume that the same vibrational input to the cochlea was present regardless of the route of stimulation. The measured stapes velocities for equivalent CM output from the two types of input were not significantly different for low and medium frequencies (0.25-4 kHz); however, the velocities for AMEI-RW drive were significantly lower for higher frequencies (4-14 kHz). Thus, for RM stimulation with an AMEI, stapes velocities can underestimate the mechanical input to the cochlea by ~20 dB for frequencies greater than ~4 kHz. This study confirms that stapes velocity (with the assumption of equivalent stapes velocity for forward and reverse stimulation) cannot be used as a proxy for effective input to the cochlea when it is stimulated in the reverse direction. Future research on application of intraoperative electrophysiological measurements during surgery (CM, compound action potential, or auditory brainstem response) for estimating efficacy and optimizing device coupling and performance is warranted.

Hearing rehabilitation and microbial shift after middle ear surgery with Vibrant Soundbridge in patients with chronic otitis media

IntroductionPatients with otitis media (OM) encounter significant functional hearing impairment with conductive, or a combined hearing loss and long-term sequelae involving impaired speech/language development in children, reduced academic achievement and irreversible disorders of middle and inner ear requiring a long time therapy and/or multiple surgeries. In its persistent chronic form, Otitis media (COM) can often only be treated by undergoing ear surgery for hearing restoration. The persistent inflammatory reaction plays a major role, often caused by multi-resistant pathogens in the ear. Herein, we present outcomes of patients implanted with currently the only FDA approved active Middle Ear Implant Vibrant Soundbridge (VSB), suffering from persistent COM.MethodsThe study enrolled 42 patients, treated by performing middle ear (ME) surgery to different extents and implanted with the VSB to various structures in the ME. Included were 17 children and 25 adults that had recurrent and/or persisting OM and significant hearing loss. Preoperative and postoperative patients' audiometric data were evaluated and the benefit with VSB assessed using the Glasgow Benefit Inventory for adults and pediatric cohorts. The microbial spectrum of pathogens was assessed before and after surgery, exploring the colonization of the otopathogens, as well as the intestinal microbiome from individually burdened patients.ResultsThe mean functional gain is 29.7 dB HL (range from 10 to 56.2 dB HL) with a significant improvement in speech intelligibility in quiet. Following VSB implantation, no significant differences in coupling were observed at low complication rates. Postoperatively patients showed significantly increased benefit with VSB compared to the untreated situation, including less otorrhea, pain, medical visits, and medication intake, with no recurrent OM and significant bacterial shift in otopathogens. The analysis of the intestinal microbiome displayed a high abundance of bacterial strains that might be linked to chronic and persistent inflammation.ConclusionsFunctional ear surgery including rehabilitation with a VSB in patients suffering from COM present to be safe and effective. The successful acceptance accompanied by the improved audiological performance resulted in significant benefit with VSB, with a shift in the ear pathogens and altered microbiome and thus is a great opportunity to be treated.

Bone Anchored Hearing Aid: A Narrative Review

Bone Anchored Hearing Aid (BAHA) is a small vibrator that can be reversibly attached to a Titanium (Ti) screw and implanted behind the ear. It uses the bone conduction channel to activate the cochleae by converting sound into the vibration of the screw. The two main indications are conductive hearing loss and unilateral deafness when using traditional hearing aids is not possible, as a rehabilitative or mixed hearing loss with a moderate perceptual component. For patients with canal atresia, Single-sided Deafness (SSD), and chronically discharged ears despite treatment, the BAHA implant is an option. Combination hearing loss is a crucial indicator for implanted hearing implants. Various options are accessible based on the bone conduction threshold. Patients with modest sensorineural impairment usually benefit from transcutaneous Bone Conduction Implants (BCI), while those with intermediate hearing loss may also benefit from percutaneous BCI devices. For combined, active middle ear implants are advised for hearing deficits with moderate and severe cochlear hearing loss. For individuals who need middle ear surgery or who are incompatible with other options for therapy, implants are a helpful and successful addition. Skin-drive Bone Conduction Devices (BCDs) are BCDs that vibrate the bone via the skin can also be separated into passive subcutaneous devices and traditional devices that are coupled, for instance, with soft bands with implanted magnets. BCDs that directly stimulate the bone, percutaneous devices, and dynamic transcutaneous devices are examples of direct-drive devices. The latter kind of apparatus uses embedded transducers to stimulate bone effectively via healthy skin. The BAHA, also known as the percutaneous direct-drive device (BCD), now rules the market. More direct-drive and skin-drive transcutaneous solutions are now being studied, partly due to problems with the transdermal implant and partly for aesthetic reasons.

Impact on Daily Life of an Active Middle Ear Implant

Impact on Daily Life of an Active Middle Ear Implant

EE157 Cost-Utility of Active Middle Ear Implants for Sensorineural Hearing Loss

Active middle ear implants (aMEI) are a safe and effective treatment for mild to severe sensorineural hearing loss (SNHL) when acoustic hearing aids (HA) are contraindicated for medical reasons. The objective of the presented study was to evaluate the cost-effectiveness of aMEIs in the Italian health care setting.

Efficient Bone Conduction Hearing Device With a Novel Piezoelectric Transducer Using Skin as an Electrode.

Bone conduction hearing aids are the only non-surgical devices used for conductive hearing loss. However, they are impractical for lifelong use since they require close contact of the transducer with the head skin, causing skin erosion and discomfort. Bone conduction hearing implants and active middle ear implants do not present these issues; however, they require surgery and can sometimes cause issues in the skin surrounding the devices. This study aimed to develop a new bone conduction hearing device that does not exert pressure on the skin or require surgery. Our device modified a piezoelectric element by using the skin of a pinna as one of the two electrodes of a conventional piezoelectric device. We compared the sound transmission of a speaker, a conventional piezoelectric device, or the new device to the Guinea pig cochlea, a physiological sound transducer to the auditory nerve, in normal and air-conductive hearing loss conditions. The novel device transmitted sound to the cochlea even after causing air-conductive hearing loss. Its bone conduction was more efficient than the speaker and the conventional piezoelectric device. We developed a novel type of bone conduction device that efficiently transmits sound to the cochlea by skipping the external auditory canal, tympanic membrane, and middle ear ossicles. This device does not exert pressure on the skin that can result in skin damage, an adverse effect of a conventional bone conduction hearing aid. Our novel hearing device can be used as a substitute for current bone-conduction hearing devices.

Methods and reference data for middle ear transfer functions

Human temporal bone specimens are used in experiments measuring the sound transfer of the middle ear, which is the standard method used in the development of active and passive middle ear implants. Statistical analyses of these experiments usually require that the TB samples are representative of the population of non-pathological middle ears. Specifically, this means that the specimens must be mechanically well-characterized. We present an in-depth statistical analysis of 478 data sets of middle ear transfer functions (METFs) from different laboratories. The data sets are preprocessed and various contributions to the variance of the data are evaluated. We then derive a statistical range as a reference against which individual METF measurements may be validated. The range is calculated as the two-sided 95% tolerance interval at audiological frequencies. In addition, the mean and 95% confidence interval of the mean are given as references for assessing the validity of a sample group. Finally, we provide a suggested procedure for measuring METFs using the methods described herein.

Comparison of an Implantable Middle Ear Microphone and Conventional External Microphone for Cochlear Implants: A Clinical Feasibility Study.

All commercially available cochlear implant (CI) systems use an external microphone and sound processor; however, external equipment carries lifestyle limitations. Although totally implantable devices using subcutaneous microphones have been developed, these are compromised by problems with soft tissue sound attenuation, feedback, and intrusive body noise. This in vivo pilot study evaluates a middle ear microphone (MEM) that aims to overcome these issues and compares hearing performance with that of an external CI microphone. Six adult participants with an existing CI were implanted with a temporary MEM in the contralateral ear. Signals from the MEM were routed via a percutaneous plug and cable to the CI sound processor. Testing was performed in the CI microphone and MEM conditions using a range of audiometric assessments, which were repeated across four visits. Performance of the MEM did not differ significantly from that of the CI on the assessments of Auditory Speech Sounds Evaluation loudness scaling at either 250 or 1000 Hz, or in the accuracy of repeating keywords presented at 70 dB. However, the MEM had significantly poorer aided sound-field thresholds, particularly at higher frequencies (≥4000 Hz), and significantly poorer performance on Arthur Boothroyd words presented at 55 dB, compared with the CI. In this pilot study, the MEM showed comparable performance to that of an external CI microphone across some audiometric assessments. However, performance with the MEM was poorer than the CI in soft-level speech (55 dB) and at higher frequencies. As such, the benefits of MEM need to be considered against the compromises in hearing performance. However, with future development, MEM is a potentially promising technology.

Congenital aural atresia: hearing rehabilitation using Active Middle Ear Implants.

To assess hearing rehabilitation in patients with congenital aural atresia using an active middle-ear implant (Vibrant Soundbridge). Of a cohort of 70 microtia and atresia patients, 10 underwent Vibrant Soundbridge implantation between 2008 and 2021. Two of the 10 patients had binaural implantation, resulting in 12 ears for analysis. Pre- and post-operative audiometry data were analysed, and patient satisfaction was evaluated. Surgical issues regarding coupling sites and outcomes were analysed. Pure tone average (0.5, 1, 2 and 4 kHz) improved from a pre-operative mean (standard deviation) of 65.3 (8.7) dB HL to a post-operative mean of 26.8 (4.9) dB HL. This resulted in a mean pure tone average gain of 38.5 dB HL. The results indicate no obvious difference between stapes (n = 8) and incus (n = 4) coupling. The mean effective gain for 0.5, 1, 2 and 4 kHz was -17.8 dB HL (standard deviation = 4.3). Concerning effective gain, Vibrant Soundbridge performed best at 2 kHz. Patients reported high overall satisfaction, good sound quality and strongly improved directional hearing. An active middle-ear implant (Vibrant Soundbridge) allows hearing rehabilitation in selected atretic ears, and provides long-term hearing stability in children and adults.

Long-term outcomes of retrospective case series of middle ear implantation with Vibrant Soundbridge in children with congenital aural atresia.

to assess audiological performance in quiet and noise, quality of life and side effects of Vibrant Soundbridge (VSB) in children with congenital aural atresia (CAA). A retrospective study including consecutive patients with unilateral or bilateral CAA implanted with VSB from 2009 to 2020 in a tertiary referral centre. 18 patients with CAA and a present stapes were included (3 simultaneous bilateral VSB implants) and 21 ears: 17 VSB were attached to the short incus process, and four to the stapes. Age at implantation ranged from 4.7 to 15.8years. Average follow-up was 6.5years (± 3.7years). In unilateral VSB, air conduction pure tone average (ACPTA) thresholds increased from 75.3 ± 15.2 to 32.6 ± 8.3dB post-operatively (VSB activated) (n = 15; p < 0.01). The speech reception threshold (SRT) and the word recognition score (WRS) were significantly improved from 81.5 ± 10.4 to 43.9 ± 7.6dB and 0% to 84.8 ± 8.5% postoperatively (n = 15; p < 0.01). The signal to noise ratio (SNR) was significantly improved from 2.1 ± 2.9dB VSB inactivated to 0.3 ± 2.7dB VSB activated (n = 15; p < 0.01). There was no significant difference in performance according to floating mass transducer (FMT) placement. 5/15 children were non-users at last follow-up in unilateral VSB and 0/3 in bilateral. CAA ears with VSB activated had a significant improvement of ACPTA, WRS, SRT and SNR. A third of patients with unilateral CAA became non-users at last follow-up. The main challenge is to target the indications for the implantation of the VSB to avoid its discontinuation.

Optoacoustic tones generated by nanosecond laser pulses can cover the entire human hearing range.

The aim of this work is to generate defined tones that cover the human hearing range in aqueous media for a later application in middle or inner ear implants. In our experiments, we investigated the characteristics of single laser pulses and pulse trains with different laser repetition rates of nanosecond laser pulses that were focused into aqueous media in a small volume. The frequency of the generated tones was limited by the spectral properties of the single acoustic pulses, which depended on the medium. Tones with fundamental frequencies above 8kHz were generated using laser pulses focused into water. By replacing water with gel, tones between 500 Hz and 20 kHz could be produced. The generation of tones in the low-frequency range was only possible when laser pulse trains with pulse density modulated pulse patterns were applied in gel. This enabled the generation of tones between 20 Hz and 2kHz. Consequently, the combination of different pulse patterns for the different frequency ranges allows generating optoacoustic tones between 20 Hz and 20 kHz in gel. Thus, we can cover the complete range of human hearing through optoacoustically generated tones.

Influence of the Coupling on the Hearing Outcome After Implantation of an Active Middle Ear Implant: Comparison of the Transmission Behavior in Temporal Bone Experiments With Clinical Data.

The active middle ear implant, Vibrant Soundbridge (VSB), can be implanted with a variety of couplers. Hearing outcome after implantation has been investigated in both temporal bone (TB) experiments and patient studies, but the relationship between experimental and clinical data is still weak in the literature. Therefore, experimental data from TB experiments should be compared with patient data in a retrospective study, in which the floating mass transducer is used with couplers of the third generation. Actuator coupling structures included the long (LP coupler) and short (SP coupler) incus process, the stapes head (Clip coupler), and the round window membrane (RW soft coupler). In the TB experiments, the sound transmission after vibroplasty on the above-mentioned actuator coupling structures was determined in 32 specimens by means of laser Doppler vibrometry on the stapes footplate. Data of 69 patients were analyzed. The main target audiometric parameters were the postoperative aided word recognition score (WRS) in the free field at 65 dB SPL (WRS 65 dB in %), the preoperative and postoperative pure-tone average (PTA4, including the frequencies 0.5, 1, 2, and 4 kHz) of the bone conduction hearing threshold (PTA4BC), the aided postoperative air conduction hearing threshold in the free field (PTA4FF) and the direct threshold (Vibrogram) at least 6 months postoperatively. The coupling efficiency of the actuator (Vibrogram-PTA4BC) as well as the effective hearing gain (PTA4FF-PTA4BC) was compared between the couplers. The analysis in the main speech range (0.5-4 kHz) indicated that in the TB experiments, the LP coupler tends to have the best coupling quality at low frequencies (500-1000 Hz). This was up to 15 dB above the worst actuator (RW soft coupler). However, the results missed the significance level ( p > 0.05). In the high frequencies (2000-4000 Hz), the Clip coupler showed the best coupling quality. This was 15 dB above the worst actuator (SP coupler). However, the results missed the significance level ( p > 0.05), too. The postoperative WRS at 65 dB SPL and the postoperative PTA4FF were independent of the actuator coupling structure. The PTA4BC was stable at 6 months postoperatively. For the PTA4 of the coupling efficiency, there were no significant differences between the actuator coupling structures (LP 8.9 dB ± 12.9; SP 9.5 ± 6.5 dB; Clip 5.2 ± 10.5 dB; RW 12.7 ± 11.0 dB). However, the tendential inferiority of the RW soft coupler with regard to transmission in the low-frequency range and the tendential superiority of the Clip coupler in the high-frequency range that have already been displayed experimentally could be confirmed in the clinical results. However, the clinical results missed the significance level, too ( p > 0.05). In vivo, there are no significant differences in the postoperative outcome stratified according to coupling the target structure. The differences known from the experimental setting were repressed by individual biasing factors. However, to ensure sufficient postoperative speech intelligibility, the frequency-specific transmission behavior of the couplers should be taken into account when setting the indication for VSB implantation.

Design and optimization of auditory prostheses using the finite element method: a narrative review.

Background and ObjectiveAn auditory prosthesis refers to a device designed to restore hearing. Some parameters of the auditory prosthesis, such as mass, implanted position, and degree, need to be repeatedly designed and optimized based on the realistic geometry of the ear. Numerous auditory prostheses designs were based on animal or specimen experiments involving many complex instruments, and the experimental specimens had low repeatability. The finite element method (FEM) can overcome these disadvantages and be carried out on the computer with substantial flexibility in modifying the prosthetic parameters to optimize them. This narrative review aims to analyze the recent advances in the design and optimization of auditory prostheses using the FEM and provides suggestions for future development.MethodsThe literature on the design of auditory prostheses using the FEM has been extensively studied using the PubMed and Web of Science databases, including different ear models and relevant parameters of different auditory prostheses that need to be designed and optimized.Key Content and FindingsThe process of designing and optimizing a prosthesis using the FEM includes building an ear model and a prosthesis model to simulate the implantation process. The related parameters of the prosthesis can be designed and modified conveniently. The post-implantation response could be used as an indicator to evaluate the prosthesis’s performance.ConclusionsThe review concluded that the FEM had been widely studied in designing and optimizing middle ear implants and cochlear implants and obtained good results. FEM can be utilized to explore more effective directions for auditory prosthesis design and optimization in the future.

Influence of Hearing Rehabilitation With Active Middle Ear and Bone Conduction Implants on Postural Control.

BackgroundAs audition also seems to contribute to balance control, additionally to visual, proprioceptive, and vestibular information, we hypothesize that hearing rehabilitation with active middle ear and bone conduction implants can influence postural control.MethodsIn a prospective explorative study, the impact of hearing rehabilitation with active middle ear [Vibrant Soundbrige (VSB), MED-EL, Innsbruck, Austria] and bone conduction implants [Bonebridge (BB), MED-EL, Innsbruck, Austria] on postural control in adults was examined in three experiments. Vestibulospinal control was measured by cranio-corpography (CCG), trunk sway velocity (°/s) by the Standard Balance Deficit Test (SBDT), and postural stability with a force plate system, each time in best aided (BA) and unaided (UA) condition with frontal-noise presentation (Fastl noise, 65 dB SPL), followed by subjective evaluation, respectively.ResultsIn 26 subjects [age 55.0 ± 12.8 years; unilateral VSB/BB: n = 15; bilateral VSB/BB: n = 3, bimodal (VSB/BB + hearing aid): n = 8], CCG-analysis showed no difference between BA and UA conditions for the means of distance, angle of displacement, and angle of rotation, respectively. Trunk sway measurements revealed a relevant increase of sway in standing on foam (p = 0.01, r = 0.51) and a relevant sway reduction in walking (p = 0.026, r = 0.44, roll plane) in BA condition. Selective postural subsystem analysis revealed a relevant increase of the vestibular component in BA condition (p = 0.017, r = 0.47). As measured with the Interactive Balance System (IBS), 42% of the subjects improved stability (ST) in BA condition, 31% showed no difference, and 27% deteriorated, while no difference was seen in comparison of means. Subjectively, 4–7% of participants felt that noise improved their balance, 73–85% felt no difference, and 7–23% reported deterioration by noise. Furthermore, 46–50% reported a better task performance in BA condition; 35–46% felt no difference and 4–15% found the UA situation more helpful.ConclusionsSubjectively, approximately half of the participants reported a benefit in task performance in BA condition. Objectively, this could only be shown in one mobile SBDT-task. Subsystem analysis of trunk sway provided insights in multisensory reweighting mechanisms.

Results and Quality of Life after Implantation of Active Middle Ear Implants.

The provision of implantable hearing aids represents an area with high development and innovation potential. On the one hand, this review article provides an overview of current indication criteria for the treatment with active middle ear implants. On the other hand, outcome parameters as well as functional results after implantation of active middle ear implants are demonstrated and discussed. The focus is mainly placed on audiological results as well as the subjective health status. "Patient Reported Outcome Measures" (PROMs) have become an integral part of the evaluation of hearing implant treatment. Due to low evidence level criteria, the study situation regarding audiological as well as subjective outcome parameters is not satisfactory. The lack of an international consensus on accepted outcome parameters makes a meta-analytical analysis of results immensely difficult. In the studies published to date, patients with sensorineural hearing loss and patients with conductive or mixed hearing loss offered better speech recognition after implantation of an active middle ear implant compared to conventional hearing aids. Current analyses show a significant improvement in general as well as hearing-specific quality of life after implantation of an active middle ear implant. To date, no validated, hearing-specific quality-of-life measurement instruments exist for assessing the success of fitting in children. Especially in children with complex malformations of the outer ear and the middle ear, excellent audiological results were shown. However, these results need to be substantiated by quality-of-life measurements in future.

Consensus Statement on Bone Conduction Devices and Active Middle Ear Implants in Conductive and Mixed Hearing Loss.

Nowadays, several options are available to treat patients with conductive or mixed hearing loss. Whenever surgical intervention is not possible or contra-indicated, and amplification by a conventional hearing device (e.g., behind-the-ear device) is not feasible, then implantable hearing devices are an indispensable next option. Implantable bone-conduction devices and middle-ear implants have advantages but also limitations concerning complexity/invasiveness of the surgery, medical complications, and effectiveness. To counsel the patient, the clinician should have a good overview of the options with regard to safety and reliability as well as unequivocal technical performance data. The present consensus document is the outcome of an extensive iterative process including ENT specialists, audiologists, health-policy scientists, and representatives/technicians of the main companies in this field. This document should provide a first framework for procedures and technical characterization to enhance effective communication between these stakeholders, improving health care.

Otological complications in inversa type recessive dystrophic epidermolysis bullosa.

The rare inversa subtype of recessive dystrophic epidermolysis bullosa (RDEB-I) is characterized by predominant intertriginous skin blistering and marked mucosal involvement. Specific recessive missense mutations in the collagen VII triple helix are implicated in the disease. To date, otological complications have been reported infrequently in this patient group. We conducted an observational, retrospective, double institution case record review of patients with RDEB-I who presented with otological complications between January 2000 and June 2020. Diagnosis was established on the basis of clinical features, family history and mutation analysis of the COL7A1 gene. In total, 11 (44%) of 25 patients with RDEB-I in our database (2 paediatric, 9 adult; mean age 40.9 years, range 8-72 years) experienced otological complications. Of these 11 patients, 10 (90.9%) had recurrent otitis externa, 7 (63.6%) had meatal stenosis and 7 (63.6%) had recurrent blistering of the external auditory canals. All 11 patients reported hearing difficulties, with conductive hearing loss confirmed by audiology testing in 6 (54.5%) of these. Of the 11 patients, 3 (27.3%) went on to have implantable hearing aids [2 bone-anchored hearing aids (BAHA) and 1 middle ear implant (MEI)] fitted with favourable outcome, while a fourth paediatric patient presented with a cholesteatoma that was surgically managed. We observed a higher prevalence of otological morbidity in RDEB-I than previously reported, and present the first case of cholesteatoma in epidermolysis bullosa (EB). Our data indicate that BAHA and MEI are safe and effective treatment options for hearing loss in EB. Clinicians should be vigilant in screening for ear symptoms in RDEB-I and consider early referral to an Ear, Nose and Throat specialist.