Congenital absence of the stapes and oval window with malpositioned facial nerve and anomalous eustachian tube.

Objective: To summarize the clinical features and postoperative efficacy of patients with oval window atresia accompanied by facial nerve aberration. Methods: The clinical data of patients with congenital middle ear malformation with facial nerve aberration admitted to our hospital from January 2015 to March 2023 were retrospectively analyzed. There were 97 cases (133 ears) in total. Among them, 39 patients (44 ears) had complete follow-up data, including 27 male patients and 12 females, aged 7-48 years old, with an average age of 17.8 years old. Of these, 14 cases (16 ears) were patients combined with facial nerve aberration, and 25 cases (28 ears) were without facial nerve aberration. The results of imaging examination, pure-tone audiometry, selection of surgical strategy, intraoperative findings and postoperative hearing improvement were summarized and analyzed. The malformations of malleus, incus, stapes, oval window and facial nerve were recorded. Prism 9 software was used to statistically analyze the mean bone conductance and air-bone gap of patients before and after surgery. Results: All the 14 patients (16 ears) with middle ear malformation accompanied by facial nerve aberration and oval window atresia showed poor hearing and no facial palsy since childhood. High resolution CT (HRCT) examination of temporal bone, pure tone audiometry and Gelle test were performed before surgery. The malformations of malleus, incus, stapes, oval window and facial nerve were recorded. Preoperative high-resolution CT (HRCT) examination of temporal bone found 12 ears with 4 or more deformities, accounting for 75.00%, in the group of patients with facial nerve malformation. The preoperative average bone conductive threshold was (15.3±10.4) dB and the average air-bone gap was (46.3±10.6) dB in pure-tone audiometry (0.5, 1, 2, 4kHz). According to the different degrees of facial nerve and ossicle malformation, we performed three different hearing reconstruction strategies for the 14 patients (16 ears) with facial nerve aberration and oval window atresia, including 7 ears of incus bypass artificial stape implantation, 7 ears of Malleostapedotomy (MS) and 2 ears of Malleus-cochlear-prothesis (MCP). After 3 months to 18 months of follow-up, all patients showed no facial paralysis. The postoperative mean bone conductive threshold was (15.7±7.9) dB and air-bone gap was (19.8±8.5) dB. There were significant differences in mean air-bone gap before and after operation (t=7.766, P<0.05), and there was no significant difference between the mean bone conductive threshold before and after surgery (t=0.225, P=0.824). There was no significant difference of mean reduction of air-bone gap between patients with and without facial nerve aberration (t=1.412, P=0.165). There was no significant difference between the three hearing reconstruction strategies. There was no significant displacement of the Piston examined by U-HRCT. Conclusion: For patients of middle ear malformation whose facial nerve cover the oval window partially, incus bypass artificial stape implantation or Malleostapedotomy (MS) can be selected according to the specific condition of auditory ossis malformation, and for patients whose facial nerve completely covers the oval window area, Malleus-cochlear-prothesis (MCP) can be selected. Three types of stapes surgery are safe and reliable for patients with oval window atresia accompanied by facial nerve aberration. There was no significant difference in efficacy between them. Preoperative HRCT assessment of middle ear malformation is effective. There is no significant difference of surgical effect with or without facial nerve aberration. The U-HRCT can be used to evaluate the middle ear malformation before surgery and the Piston implantation status after surgery. Due to the risks of surgery, those who do not want to undergo surgery can choose artificial hearing AIDS, such as hearing aid, vibrating soundbridge, bone bridge or bone-anchored hearing aid.

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.

A bone-anchored hearing aid (BAHA) consists of a permanent titanium fixture, which is surgically implanted into the skull bone behind the ear, and a small detachable sound processor that clips onto the fixture. BAHAs are suitable for people with conductive or mixed hearing loss who cannot benefit fully from conventional hearing aids. To assess the clinical effectiveness and cost-effectiveness of BAHAs for people who are bilaterally deaf. Nineteen electronic resources, including MEDLINE, EMBASE and The Cochrane Library (inception to November 2009). Additional studies were sought from reference lists and clinical experts. Inclusion criteria were applied by two reviewers independently. Data extraction and quality assessment were undertaken by one reviewer and checked by a second. Prospective studies of adults or children with bilateral hearing loss were eligible. Comparisons were BAHAs versus conventional hearing aids [air conduction hearing aid (ACHA) or bone conduction hearing aid (BCHA)], unaided hearing and ear surgery; and unilateral versus bilateral BAHAs. Outcomes included hearing measures, validated measures of quality of life (QoL), adverse events and measures of cost-effectiveness. For the review of cost-effectiveness, full economic evaluations were eligible. Twelve studies were included (seven cohort pre-post studies and five cross-sectional 'audiological comparison' studies). No prospective studies comparing BAHAs with ear surgery were identified. Overall quality was rated as weak for all included studies and meta-analysis was not possible due to differences in outcome measures and patient populations. There appeared to be some audiological benefits of BAHAs compared with BCHAs and improvements in speech understanding in noise compared with ACHAs; however, ACHAs may produce better audiological results for other outcomes. The limited evidence reduces certainty. Hearing is improved with BAHAs compared with unaided hearing. Improvements in QoL with BAHAs were identified by a hearing-specific instrument but not generic QoL measures. Studies comparing unilateral with bilateral BAHAs suggested benefits of bilateral BAHAs in many, but not all, situations. Prospective case series reported between 6.1% and 19.4% loss of implants. Most participants experienced no or minor skin reactions. A decision analytic model was developed. Costs and benefits of unilateral BAHAs were estimated over a 10-year time horizon, applying discount rates of 3.5%. The incremental cost per user receiving BAHA, compared with BCHA, was £ 16,409 for children and £ 13,449 for adults. In an exploratory analysis the incremental cost per quality-adjusted life-year (QALY) gained was between £ 55,642 and £ 119,367 for children and between £ 46,628 and £ 100,029 for adults for BAHAs compared with BCHA, depending on the assumed QoL gain and proportion of each modelled cohort using their hearing aid for ≥ 8 or more hours per day. Deterministic sensitivity analysis suggested that the results were highly sensitive to the assumed proportion of people using BCHA for ≥ 8 hours per day, with very high incremental cost-effectiveness ratio values (£ 500,000-1,200,000 per QALY gained) associated with a high proportion of people using BCHA. More acceptable values (£ 15,000-37,000 per QALY gained) were associated with a low proportion of people using BCHA for ≥ 8 hours per day (compared with BAHA). The economic evaluation presented in this report is severely limited by a lack of robust evidence on the outcome of hearing aid provision. This has lead to a more restricted analysis than was originally anticipated (limited to a comparison of BAHA and BCHA). In the absence of useable QoL data, the cost-effectiveness analysis is based on potential utility gains from hearing, that been inferred using a QoL instrument rather than measures reported by hearing aid users themselves. As a result the analysis is regarded as exploratory and the reported results should be interpreted with caution. Exploratory cost-effectiveness analysis suggests that BAHAs are unlikely to be a cost-effective option where the benefits (in terms of hearing gain and probability of using of alternative aids) are similar for BAHAs and their comparators. The greater the benefit from aided hearing and the greater the difference in the proportion of people using the hearing aid for ≥ 8 hours per day, the more likely BAHAs are to be a cost-effective option. The inclusion of other dimensions of QoL may also increase the likelihood of BAHAs being a cost-effective option. A national audit of BAHAs is needed to provide clarity on the many areas of uncertainty surrounding BAHAs. Further research into the non-audiological benefits of BAHAs, including QoL, is required.

The patient’s imaging features, especially the honeycomb pattern of ossific changes in the geniculate fossa, were virtually pathognomonic for ossifying hemangioma of the facial nerve.

New Bone-Anchored Amplification Options for Children

McRackan, Theodore R.; Carlson, Matthew L.; Reda, Fitsum A.; Noble, Jack H.; Rivas, Alejandro Author Information

Dear Editor,&#x0D; &#x0D; The tympanic membrane and the ossicular chain contribute roughly 28 dB in hearing gain. In chronic suppurative otitis media, loss of tympanic membrane and lysis of the ossicular chain are significant causes of hearing loss.1 Through the years, hearing impairment has been augmented using various devices such as ear trumpets, carbon hearing aids, vacuum tube and transistor hearing aids, bone anchored hearing aids, and cochlear implants.2 This case report describes how a cotton wick was used to amplify sound.&#x0D; &#x0D; Case Report&#x0D; A 65-year-old man consulted for hearing loss. He had a childhood history of recurrent ear discharge and hearing loss and was diagnosed with chronic suppurative otitis media. At age 55, he underwent tympanomastoidectomy of the left ear. While surgery stopped the left ear discharge, there was complete hearing loss in this ear. For this reason, he opted not to have surgery on the right ear. There was subsequent recurrent ear disease of the right ear. He would clean his ear with a cotton wick and apply antibiotic drops during bouts of ear discharge. He observed that leaving the ear wick with a few drops of topical otic preparations (polymyxin-neomycin-steroid or ofloxacin) would lessen the frequency of ear discharge and improve his hearing. He found that morning application and positioning of the cotton wick in his right ear using tweezers and a toothpick allowed him to hear adequately to conduct his daily activities as an architect. (Figure 1, 2) The fear of hearing loss from another surgery, cost of a commercial hearing aid, and great utility of a simple cotton wick made him continue his practice for these ten years.&#x0D; Examination of the right middle ear without the cotton wick showed thickened mucosa, absent malleus and incus structures, a patent Eustachian tube and a near – total tympanic membrane perforation. There was no keratinous material or foul smelling discharge. (Figure 3) Pure tone audiometry confirmed that with the cotton wick, the right air-bone gap decreased at 500 hz, 1kHz, 2Kh and 4KHz by 30db, 40dB, 35dB and 25dB respectively. (Table 1)&#x0D; DISCUSSION&#x0D; At different anatomic levels, mechanical sound energy is amplified and transmitted to the functional parts of the ear. The tympanic membrane and oval window ratio of 21:1 and malleus-incus lever mechanism ratio of 1.3:1 provide a 28 dB amplification of conductive hearing.1 This gain is reflected by frequency specific air-bone gaps, which can range between 25-40 dB. With the contribution from the external ear, the overall conductive gain is 60 dB.1,3 Damage to the auditory system often results in a loss of hearing sensitivity that is frequency – specific. The presence of a frequency – specific wide air-bone gap suggests ossicular chain discontinuity among patients with chronic otitis media.4 Narrowing of the air-bone gap, which in this case was provided by insertion of the cotton wick, may lead to at least partial restoration of ossicular coupling.&#x0D; The ability of the cotton wick to improve hearing may be attributed to its possession of characteristics for sound conduction and acoustic impedance, such as stiffness, resistance and mass. The effectiveness of the cotton wick was reported to be dependent on its positioning in the ear; the patient would have to insert the wick down to the level of the promontory or oval window, occasionally blow his nose, or reposition the cotton wick to achieve an acceptable hearing level. However, for a patient with completely deaf contralateral ear, a 32.5 dB gain in hearing is very pronounced and significant. &#x0D; The hearing gain produced by the cotton wick only amplified the air conductive component of hearing but not bone conduction. While it afforded amplification of sound and a route of medicine administration, it may also have contributed to sensorineural hearing loss brought about by ototoxicity of medications and thickening of the oval and round window from chronic irritation. For this reason, utmost caution must be advised before considering use of a “cotton wick” to amplify hearing in this manner-- a practice we do not endorse.&#x0D; The cotton wick may have served as a vibrating piston on top of the oval window which amplified hearing. Such a mechanism may conceivably prognosticate potential gain from a contemplated tympanoplasty in the same way that the “paper patch test”5 predicts simple myringoplasty outcomes. Having said that, the diagnostic utility of such a cotton wick requires further investigation before potential clinical applications such as prognostication of tympanoplasty are theorized. Could future studies show that a preoperative cotton wick (or equivalent device) may approximate potential gains from a good tympanoplasty with ossiculoplasty in a patient with total tympanic perforation and ossicular chain loss?&#x0D; &#x0D; Sincerely,&#x0D; Ryner Jose D. Carrillo, MD, MSc&#x0D; Precious Eunice R. Grullo, MD, MPH&#x0D; Maria Luz M. San Agustin, RN, MClinAudio&#x0D; &#x0D;

Implantable hearing aids comprise two distinct subgroups: bone anchored hearing aids (BAHA) and middle ear implants (MEI). Both require surgeries and both require that some portion, or an entire device, be implanted either behind the ear in the mastoid area (BAHA) or in the middle ear cavity (MEI). Depending on the manufacturer, some MEIs also require that some components be implanted in the mastoid area. Other than these two common points, these implantable aids differ in use and function. The BAHA is used for those with conductive or mixed hearing loss, while the MEI is used most often for those with sensorineural hearing loss. Historically, some MEIs were designed for use with conductive hearing losses, but the vast majority of MEIs are to be used in patients with sensorineural hearing losses.

To evaluate whether the bone-anchored hearing aid (BAHA) can be applied successfully to patients with conductive hearing loss and moderate mental retardation. Retrospective clinical evaluation. Tertiary referral center. Twenty-two patients with congenital moderate mental retardation and conductive or mixed hearing loss were selected to receive a BAHA at the University Medical Centre Nijmegen, the Netherlands. Four of them were fitted despite a limited air-bone gap. Rehabilitative BAHA application. Implantation results, skin reactions, and audiological data were evaluated during a mean follow-up of 36 months. All the patients were still using the BAHA 7 days a week and for more than 8 hours a day after a follow-up period between 5 and 96 months. Two implants (9%) were lost due to insufficient integration but were reimplanted successfully. With the BAHA, mean free-field thresholds showed a clear mean improvement of 9 dB compared with the previous hearing aid. Considerable improvements in daily activities were seen in at least five patients. Moderate mental retardation should no longer be considered as a contraindication for BAHA application. Although implant loss was low, extra attention may be required from the personal care providers to maintain the percutaneous implant. The BAHA was well-accepted by the patients with moderate mental retardation and was being used for most of the day. Implementation of the BAHA as hearing aid treatment in patients with moderate mental retardation proved to be sufficiently effective and may have strongly positive effects on activities at school or at work.

Active middle ear implants, such as the Vibrant Soundbridge, are used as an important part in the rehabilitation of sensorineural, conductive hearing, or mixed hearing loss. The attachment of the Vibrant Soundbridge at the round window and the usage of the Vibroplasty couplers strongly expanded the application of the Vibrant Soundbridge.The Vibrant Soundbridge is developed for patients who have an intolerance to hearing aids and a moderate to profound sensorineural hearing loss. The VSB also provides an optimal solution for patients with failed middle ear reconstructions or patients with atresia. To capture the improvement with the VSB Implant with different hearing losses a literature analysis was conducted. The functional gain was analyzed for 107 patients with conductive hearing loss and for 214 patients with sensorineural hearing loss out of 14 studies.Patients with conductive and mixed hearing loss resulted in a functional gain from 30 to 58 dB with the VSB. Patients with a pure sensorineural hearing loss showed a functional gain of 23-30 dB. The VSB bone conduction threshold shift was analyzed for all studies conducted in the years between 2000 and 2009. In 11 of the 16 studies there was no significant (p=0.05) change found. In 5 studies, the pre- to post-surgical bone conduction threshold shift was less than 10 dB. None of these studies measured a threshold shift of more than 10 dB.The flexible attachment at either the long process of the incus with sensorineural hearing loss, with an conductive hearing loss at the round window or the use of Vibroplasty couplers at the oval window, head of the stapes or round window makes the VSB an extremely versatile instrument. If patients can't wear conventional hearing aids, had failed middle ear reconstructions or atresia the VSB presents, due to the significant hearing improvement in any type of hearing loss, an ideal solution.

Objective: To analyze the clinical characteristics and appropriate surgical procedures, and discuss the classification of congenital middle ear malformation. Methods: All cases were from the Center of Otorhinolaryngology, the Sixth Medical Center of Department of PLA General Hospital. All of these cases, including 26 male patients (ears) , 10 female patients (11 ears) , aged from 7 to 57 years old, had normal external auditory canal, tympanic membrane, conductive hearing loss, type A tympanogram and negative Gelle's test. Tympanoplasty was performed in all cases. The deformity was classified to three types,i.e., Type I (stapes foot plate mobility): Ⅰa, ossicular chain deformity with normal stapes suprastructure; Ⅰb, ossicular chain deformity with abnormal stapes suprastructure; Type Ⅱ (stapes foot plate fixation): Ⅱ a,normal ossicular chain, Ⅱ b, ossicular chain malformation; and Type Ⅲ: vestibular window osseous atresia or undeveloped, or with round window atresia. The malformation of type Ⅱ and Ⅲ may be accompanied with abnormal facial nerve. In addition, the papers on middle ear malformation published from 1982 to 2017 were analyzed retrospectively. The clinical data of 451 ears malformation were summarized. Results: According to the revisional classification criteria in 37 ear samples from our hospital, 20 ears were type I. 6 type Ⅰa cases were used PORP (partial ossicular replacement prosthesis) to reconstruct the ossicular chain; 14 type Ⅰb cases were used TORP (total ossicular replacement prosthesis) to reconstruct the ossicular chain. For the 5 ears of type Ⅱ, 2 of which were type Ⅱ a and 3 were type Ⅱ b. 4 ear samples of type Ⅱ were implanted with Piston ossicular prosthesis, 1 was implanted with TORP in which the ossificated foot plate was removed with periosteum preserved. 12 ear samples were type Ⅲ, with vestibular window osseous atresia, facial nerve malformation, and stapes suprastructure malformation. The pistons ossicular prosthesis were implanted in vestibular window in 3 ears with facial nerve covering vestibular window partially. The surgery had to be given up in 5 ears, and TORP was implanted in 4 ears at the opening with preserved periosteum at the beginning of the tympanic scala because of facial nerve covering vestibular window totally. 30 ears with complete follow-up data had no sensorineural hearing loss and the average air-bone conduction decreased 23.3±10.7 dB (P<0.05).There were 234 ears of type Ⅰ in 451 ears of congenital middle ear malformation reported in the literature. 113 of which were type Ⅰa, the basic surgery was ossicular chain shaking and artificial or autogenous PORP implantation. Type Ⅰb was 121 ears, with autogenous or artificial TORP and PORP. Type Ⅱ was125 ears, including type Ⅱa 22 ears, Ⅱb 60 ears, and no subclassification for 43 ears. The surgery of type Ⅱ was the same as otosclerosis. The vestibular window atresia of type Ⅲ was 92 ears, the surgery of 17 ears had to be abandoned, the other ears underwent vestibular window, promontory or semicircular canal opening to reconstruct hearing with Piston, autogenous or artificial TORP. Conclusion: Referring to the classification of congenital middle ear malformation combining with appropriate surgical materials and methods, otologists can better understand and choose appropriate surgical method to the middle ear malformation.

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.

The implementation of universal newborn hearing screening programs has increased the number of infants diagnosed with hearing loss and referred for amplification in the first few weeks of life. There is compelling evidence that children born with hearing loss who have been identified and begun intervention by 6 months of age will have significantly better language development than their later-identified peers. 1-3 The challenges of fitting appropriate amplification on this population include limited behavioral response to sound, possible complicating middle ear fluid, parental uncertainties, possible existence of additional handicaps, and limited resources to support and pay for hearing aids. Despite these issues, there is consensus that the diagnosis of congenital hearing loss should be completed in the first 3 months of life and be followed immediately with the consideration of hearing aid use. 4 As access to follow-up from newborn hearing screening improves, pediatric audiologists are faced with recommending technology for infants during their early weeks of life. The options available to them are expanding rapidly as amplification technology grows and diversifies. Outcome studies of children fitted with advanced amplification features are not common and cannot keep up with the features being introduced to the market. Most infants are fitted with the goal of providing audibility for environmental speech to enhance speech and language development and with the expectation that patients will use their hearing aids daily. The pediatric audiologist’s role is to share information on hearing loss and available amplification technology with the parents and their medical and support team. Parents depend on various professionals to acquire the information and tools they need to become empowered, informed consumers. Reaching decisions about the choice of technology for an infant includes examining available features and selecting what might be the best fit for the infant given all the available information and circumstances.

P131: Bone-Anchored Hearing Aid Abutment Skin Overgrowth Reduction

To study the benefit of the application of a bone-anchored hearing aid in patients with a unilateral air-bone gap. Prospective evaluation in eight patients. Binaural hearing was assessed in the sound field by comparing aided and unaided scores obtained with a sound localization test and a speech recognition in noise test with spatially separated sound and noise sources. Tertiary referral center. The patients had subnormal hearing and unilateral conductive hearing loss. Sound localization improved significantly in the six patients with acquired hearing loss. The binaural advantage, studied with speech-in-noise tests with spatially separated speech and noise sources, proved to be comparable with that in a control group of subjects with normal hearing when they were listening monaurally versus binaurally. For one of the two patients with unilateral congenital conductive hearing loss, the results were ambiguous. This patient's age at the time of surgery was high: 40 years (the other patient was 19 years old at the time of surgery). This might have played a role. If reconstructive surgery is not possible (e.g., in a patient with a chronically draining ear or a severe congenital malformation), a bone-anchored hearing aid is an option to reestablish binaural hearing. The results reported herein suggest that, at least for patients with acquired hearing loss, the bone-anchored hearing aid is an effective treatment of unilateral conductive hearing loss.