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Purpose: To investigate sound localization in patients bilaterally fitted with bone conduction devices (BCDs). Additionally, clinically applicable methods to improve localization accuracy were explored., Methods: Fifteen adults with bilaterally fitted percutaneous BCDs were included. At baseline, sound localization, (un)aided pure-tone thresholds, device use, speech, spatial and qualities of hearing scale (SSQ) and York hearing-related quality of life (YHRQL) questionnaire were measured. Settings to optimize sound localizing were added to the BCDs. At 1 month, sound localization was assessed again and localization was practiced with a series of sounds with visual feedback. At 3 months¸ localization performance, device use and questionnaire scores were determined again., Results: At baseline, one patient with congenital hearing loss demonstrated near excellent localization performance and four other patients (three with congenital hearing loss) localized sounds (quite) accurately. Seven patients with acquired hearing loss were able to lateralize sounds, i.e. identify whether sounds were coming from the left or right side, but could not localize sounds accurately. Three patients (one with congenital hearing loss) could not even lateralize sounds correctly. SSQ scores were significantly higher at 3 months. Localization performance, device use and YHRQL scores were not significantly different between visits., Conclusion: In this study, the majority of experienced bilateral BCD users could lateralize sounds and one third was able to localize sounds (quite) accurately. The localization performance was robust and stable over time. Although SSQ scores were increased at the last visit, optimizing device settings and a short practice session did not improve sound localization., (© 2021. The Author(s).)

Background: Hearing loss is one of the most prevalent disabilities worldwide, and has a significant impact on quality of life. The adult-onset type of the condition is highly heritable but the genetic causes are largely unknown, which is in contrast to childhood-onset hearing loss., Methods: Family and cohort studies included exome sequencing and characterisation of the hearing phenotype. Ex vivo protein expression addressed the functional effect of a DNA variant., Results: An in-frame deletion of 12 nucleotides in RIPOR2 was identified as a highly penetrant cause of adult-onset progressive hearing loss that segregated as an autosomal dominant trait in 12 families from the Netherlands. Hearing loss associated with the deletion in 63 subjects displayed variable audiometric characteristics and an average (SD) age of onset of 30.6 (14.9) years (range 0-70 years). A functional effect of the RIPOR2 variant was demonstrated by aberrant localisation of the mutant RIPOR2 in the stereocilia of cochlear hair cells and failure to rescue morphological defects in RIPOR2-deficient hair cells, in contrast to the wild-type protein. Strikingly, the RIPOR2 variant is present in 18 of 22 952 individuals not selected for hearing loss in the Southeast Netherlands., Conclusion: Collectively, the presented data demonstrate that an inherited form of adult-onset hearing loss is relatively common, with potentially thousands of individuals at risk in the Netherlands and beyond, which makes it an attractive target for developing a (genetic) therapy., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To evaluate short- and long-term hearing results of surgery for acquired atresia of the external auditory canal (EAC) in a large patient cohort and to define preoperative audiometric conditions useful for patient counseling., Study Design: Retrospective cohort study., Setting: Academic tertiary referral center., Patients: Seventy-eight ears from 72 patients with postinflammatory acquired atresia of the EAC who underwent canal- and meatoplasty were included. Patients with involvement of the ossicular chain, (syndromic) external ear malformations, or congenital aural atresia were excluded., Intervention: Canal- and meatoplasty., Main Outcome Measures: Mean pure-tone averages of thresholds at 0.5, 1, 2, and 3 kHz (PTA0.5,1,2,3) for air conduction (AC), bone conduction, and air-bone gap (ABG) were calculated preoperatively and at short-term (≤0.55 yr) and long-term follow-up (>0.55 yr). Additionally, the numbers of ears with a closed ABG ≤10 dB and ≤20 dB, and with Social hearing (defined as: AC PTA0.5,1,2,3 ≤35 dB) were assessed., Results: At short-term follow-up AC PTA0.5,1,2,3 improved by 18 dB. Social hearing was obtained in 81% of the ears. Postoperatively, 35% of the ears had a closed ABG ≤10 dB, 83% was closed ≤20 dB. During follow-up, significant deterioration of 5 to 7 dB occurred for AC thresholds at 0.25, 0.5, and 1 kHz., Conclusions: Surgery for acquired atresia of the EAC is often beneficial. This study suggests overall advantageous surgery when the preoperative indication criteria ABG PTA0.5,1,2,3 >20 dB and AC PTA0.5,1,2,3 >35 dB are applied.

Objective: To present the hearing results of a 5-year longitudinal study in a pediatric population undergoing surgery for extensive cholesteatoma using a canal wall up (CWU) approach with bony obliteration of the mastoid and epitympanic space, with a standard residual rate of 5.8%, a recurrence rate of 2.9%, and all ears waterproof, free of otorrhea and all external ear canals patent and self-cleaning., Study Design: Retrospective consecutive study., Patients: Thirty-three children (≤18 yr) undergoing surgery for cholesteatoma (34 ears) between 1997 and 2009., Interventions: Therapeutic., Setting: Tertiary referral center., Main Outcome Measures: Hearing and gain in hearing at 1- and 5-year postsurgery: (1) pure-tone average (PTA), (2) pure-tone average high frequency, (3) pure-tone average including 3 kHz, (4) bone conduction at corresponding frequency averages, (5) gain at corresponding air conduction and bone conduction (gain at corresponding air conduction) frequency averages. (6) The Amsterdam Hearing Evaluation Plots were used to study the individual cases., Results: The Amsterdam Hearing Evaluation Plots at 5-year showed in 58.8% of patients a positive gain air conduction. In 23.5% a successful functional result was achieved, defined as an air-bone gap closure to 20 dBHL or less. In 6 patients (17.6%) a limited bone conduction deterioration was shown all limited to maximum 20 dBHL., Conclusion: The CWU bony obliteration tympanoplasty in a consecutive series of pediatric extensive cholesteatoma shows a similar to slightly improved hearing outcome as compared with CWU surgery without BOT. Although the series shows a clearly reduced reoperation rate and a significantly improved safety and hygienic outcome as compared with CWU without BOT, better hearing outcomes remain desirable in this group of children.

An important aspect of binaural hearing is the proper detection of interaural sound level differences and interaural timing differences. Assessments of binaural hearing were made in patients with acquired unilateral conductive hearing loss (UCHL, n = 11) or congenital UCHL (n = 10) after unilateral application of a bone conduction device (BCD), and in patients with bilateral conductive or mixed hearing loss after bilateral BCD application. Benefit (bilateral versus unilateral listening) was assessed by measuring directional hearing, compensation of the acoustic head shadow, binaural summation and binaural squelch. Measurements were performed after an acclimatization time of at least 10 weeks. Unilateral BCD application was beneficial, but there was less benefit in the patients with congenital UCHL as compared to patients with acquired UCHL. In adults with bilateral hearing loss, bilateral BCD application was clearly beneficial as compared to unilateral BCD application. Binaural summation was present, but binaural squelch could not be proven. To explain the poor results in the patients with congenital UCHL, two factors seemed to be important. First, a critical period in the development of binaural hearing might affect the binaural hearing abilities. Second, crossover stimulation, referring to additional stimulation of the cochlea contralateral to the BCD side, might deteriorate binaural hearing in patients with UCHL., (Copyright © 2011 S. Karger AG, Basel.)

In the past 30 years, a large amount of clinical and audiological research on bone conduction hearing devices has been performed. In this review, we give a brief history of the developments in indications, surgical techniques and sound processors with respect to implantable bone conduction devices like the bone-anchored hearing aid or Baha. Starting with the use of Baha in patients with bilateral conductive or mixed hearing loss (HL), the indications for such devices have been extended to patients with unilateral HL, children and moderate mentally retarded patients. Bilateral fitting has been shown to be beneficial in restoring binaural hearing in patients with bilateral acquired or congenital conductive HL. In addition, the surgical techniques used to implant the titanium fixture for Baha application have been modified and further developed to reach two main goals: (a) optimal osseointegration, and (b) preparation of the implant site to minimize the occurrence of soft tissue reactions. Currently, the most used techniques are the pedicled skin flap, dermatome and linear incision techniques. Several generations of the Baha(®) sound processor have been developed by Cochlear(TM) to provide sufficient amplification in different hearing situations. Improvements in sound quality, aesthetics and handling have been major points of interest. The Baha sound processors most often used today are the Baha(®) Divino, the Baha(®) Intenso and the Baha(®) Cordelle. Recently, the more flexible Baha(®) BP100 sound processor was launched., (Copyright © 2011 S. Karger AG, Basel.)

A stapes gusher is the result of a congenital inner ear anomaly showing at tone audiometry a conductive or mixed hearing loss. The conductive part of the hearing loss could lead to the thought to explore the middle ear. The congenital origin should lead to a high resolution. CT-scanning to evaluate a widening of the internal acoustic canal. Repeated audiometry could show especially a large conductive impairment in the lowest frequencies with a closure of the airbone gap at 2 khz and a high sensorineural high frequency loss at 4 and 8 khz. Contralateral stapedial reflexes may be present. Since the x-recessive mixed deafness syndrome (DFN3) frequently involves males with an early childhood hearing impairment, clinical suspicion should be high. When stapes surgery is considered a precise medical history is essential regarding on the start of the hearing impairment. A continuous suspicion will guide to the audiological, radiological and molecular genetic clues to trace the correct diagnosis before embarking on stapes surgery.