Comparison of three different image enhancement systems for detection of laryngeal lesions.

OBJECTIVE: Image enhancement systems are important diagnostic tools in the detection of laryngeal pathologies. This study aimed to compare three different image enhancement systems: professional image enhancement technology, Image1 S and narrow-band imaging. METHOD: Using the three systems, 100 patients with laryngeal lesions were investigated using a flexible and a 30° rigid endoscope. The lesions were diagnosed by three experts and classified using the Ni classification. The findings were compared. RESULTS: Lesions classified as 'benign' were histopathologically confirmed in 50 per cent of patients, malignant lesions were confirmed in 41 per cent and recurrent respiratory papillomatosis were confirmed in 9 per cent. There was no significant difference between the experts' assessments of each image enhancement system. CONCLUSION: The three systems give comparable results in the detection of laryngeal lesions. With two additional systems, more users can perform image-enhanced endoscopy, resulting in a broadly available tool that can help to improve oncological assessment.

Melody of Vocants: Fixed Pattern or Shaped by Hearing?

INTRODUCTION: Vocants as infants' first vocalic utterances are produced laryngeally while the vocal tract is maintained in a neutral position. These "primitive" sounds have sometimes been described as largely innate and, therefore, as sounding alike in both healthy and hearing-impaired young infants. OBJECTIVE: The objective of this study is to compare melody features of vocants, recorded during face-to-face interaction, between infants (N = 8) with profound congenital sensorineural hearing loss (HI group) and age-matched (N = 18) controls (CO) group. The question was as follows: does a lack of auditory feedback have a noticeable effect on melodic features of vocants? METHODS: The cooing database totalled 6,998 vocalizations (HI: N = 2,847; CO: N = 4,151), all of which had been recorded during the observation period of 60-181 days of age. Identification of the vocants (N = 1,148) was based on broadband spectrograms (KAY-CSL) and auditory impressions. Fundamental frequency (F0) analyses were performed (PRAAT) and the pattern of the F0 contour (melody) analysed using specific in-lab software (CDAP, pw-project). Generalized mixed linear models were used to perform group comparisons. RESULTS: There was a clear predominance of a simple rising-falling pattern (single melody arcs) in vocants of both groups. Nonetheless, significantly more complex contours, particularly, double-arc structures, were found in vocants of the CO group. Moreover, vocants of the HI group were shorter than those uttered by the CO group, while the mean F0 did not significantly differ. CONCLUSION: Vocants are characterized by both, innate features, found in HI and CO groups, and features that additionally require a functioning auditory system. Even at an early pre-linguistic stage, somatosensory sensations cannot compensate for a lack of auditory feedback. Vocants might be relevant in the early diagnosis of hearing disorders and assessments of the effectiveness of, or adjustments required to, hearing aids.

Electrically evoked auditory responses: A classification for brainstem implant placement in Neurofibromatosis Type 2.

OBJECTIVE: In auditory brainstem implant (ABI) surgery, array placement may be optimized by electrophysiological information of adequate brainstem activation gained from electrically evoked auditory brainstem responses (EABR). This study aims 1) to characterize in detail the EABR from ABI implantation, 2) to introduce an EABR Classification Scheme, and 3) to analyze data for their correlation with individual patients' findings. METHODS: Out of a continuous series of 54 patients who received an ABI between 2005 and 2019, 23 Neurofibromatosis Type 2 patients with complete documentation of 154 recordings were selected for offline analysis and for development and evaluation of a new EABR Classification Scheme comprising Class A: three vertex positive peaks, Class B:two peaks, Class C: a combination of one peak and a second melted double peak, Class D: one sole vertex positive peak and Class E: no peaks. RESULTS: All 23 subjects showed EABR at final ABI position and experienced auditory sensations at first activation. The most frequent morphology consisted of two peaks, Classes B and C. Identified mean latencies were for P1 0.42 ms (±0.095), P2 1.42 ms (±0.244) and P3 2.41 ms (±0.329). Peak latencies correlated positively with tumor extensions (p < 0.005). CONCLUSIONS: This study provides clear instructions on optimal EABR performance and evaluation. SIGNIFICANCE: The new EABR Classification Scheme relies on a fast "online" identification of vertex positive peaks at the estimated post-artifact phase. The variability in EABR morphology provides an individual snapshot of the actual structural and functional status of the brainstem.

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

OBJECTIVE: To investigate the benefit of intraoperative auditory brainstem response (ABR) measurements in revision active middle ear implant surgery. STUDY DESIGN: Retrospective data analysis. SETTING: Tertiary referral center with a large active middle ear implant program. MAIN OUTCOME MEASURES: Intraoperative ABR thresholds, audiogram, sound field thresholds, speech understanding in the Freiburger monosyllabic word test. PATIENTS: Fourteen patients with active middle ear implant revision surgery. RESULTS: 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. CONCLUSION: 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.

Do cochlear microphonics evoked by narrow-band chirp stimuli affect the objective detection of auditory steady-state responses?

OBJECTIVE: It has recently been discussed whether hearing screening and hearing threshold assessment can accurately be completed using automated ASSR methods for children with auditory neuropathy spectrum disorder (ANSD). Possible causes for the claimed potential failures were investigated here. DESIGN: The study is based on the analysis of stored ASSR raw data. STUDY SAMPLE: This study reviewed raw ASSR data from 274 patients with a total of 5809 individual recordings. RESULTS: Cochlear microphonics (CM) were found in 18 of the 274 patient records. Four of these 18 were obtained from patients with ANSD. One patient with ANSD without click auditory brainstem responses up to 100 dBnHL demonstrated clear ASSR responses from 65 dBnHL upwards. Where click stimulation suggests an auditory nerve defect, narrow-band chirps were shown to evoke ASSR in certain patients. CMs are elicited by narrow-band chirps in the same way as by broadband stimuli. CM residuals as well as a presumed enlarged wave I with absent neural responses, always accompanied by CM, were found as possible causes of misinterpretation at high stimulus levels. A CM detector was created. CONCLUSIONS: The CM detector, indicating the presence of CM, will prevent misinterpretation of clinical ASSR results.

Longitudinal Development of Verbal and Nonverbal Intelligence After Cochlear Implantation According to Wechsler Tests in German-speaking Children: A Preliminary Study.

OBJECTIVES: Intelligence as a construct of cognitive abilities is the basis of knowledge and skill acquisition and the main predictor of academic achievement. As a broad construct, it is usually divided into subdomains, such as nonverbal and verbal intelligence. Verbal intelligence is one domain of intelligence but is not synonymous with specific linguistic abilities like grammar proficiency. We aim to address the general expectation that early cochlear implantation enables children who are hard of hearing to develop comprehensively, including with respect to verbal intelligence. The primary purpose of this study is to trace the longitudinal development of verbal and nonverbal intelligence in children with cochlear implants (CIs). DESIGN: Sixteen children with congenital hearing loss who received unilateral or bilateral implants and completed at least two intelligence assessments around the age of school entrance were included in the study. The first assessment was performed around 3 years after CI fitting (chronological age range: 3.93 to 7.03 years). The second assessment was performed approximately 2 years after the first assessment. To analyze verbal and nonverbal IQ in conjunction and across children at different ages, we used corresponding standardized and normalized tests from the same test family (Wechsler Preschool and Primary Scale of Intelligence and/or Wechsler Intelligence Scale for Children). RESULTS: Regarding longitudinal development, both verbal and nonverbal IQ increased, but verbal IQ increased more substantially over time. At the time of the second measurement, verbal and nonverbal IQ were on a comparable level. Nevertheless, we also observed strong inter-individual differences. The duration between both assessments was significantly associated with verbal IQ at the second measurement time point and thus with verbal IQ gain over time. Education mode (regular vs. special kindergarten/school) was significantly correlated with nonverbal IQ at the second assessment time point. CONCLUSIONS: The results, despite the small sample size, clearly suggest that children with CIs can achieve intellectual abilities comparable to those of their normal-hearing peers by around the third year after initial CI fitting, and they continue to improve over the following 2 years. We recommend further research focusing on verbal IQ assessed around the age of school entrance to be used as a predictor for further development and for the establishment of an individual educational program.

Vestibular Aqueduct Morphology and Meniere's Disease-Development of the "Vestibular Aqueduct Score" by 3D Analysis.

Improved radiological examinations with newly developed 3D models may increase understanding of Meniere's disease (MD). The morphology and course of the vestibular aqueduct (VA) in the temporal bone might be related to the severity of MD. The presented study explored, if the VA of MD and non-MD patients can be grouped relative to its angle to the semicircular canals (SCC) and length using a 3D model. Scans of temporal bone specimens (TBS) were performed using micro-CT and micro flat panel volume computed tomography (mfpVCT). Furthermore, scans were carried out in patients and TBS by computed tomography (CT). The angle between the VA and the three SCC, as well as the length of the VA were measured. From these data, a 3D model was constructed to develop the vestibular aqueduct score (VAS). Using different imaging modalities it was demonstrated that angle measurements of the VA are reliable and can be effectively used for detailed diagnostic investigation. To test the clinical relevance, the VAS was applied on MD and on non-MD patients. Length and angle values from MD patients differed from non-MD patients. In MD patients, significantly higher numbers of VAs could be assigned to a distinct group of the VAS. In addition, it was tested, whether the outcome of a treatment option for MD can be correlated to the VAS.

High-sensitivity FEES® with the professional image enhancement technology "PIET".

PURPOSE: Flexible endoscopic evaluation of swallowing (FEES®) is a standard diagnostic tool in dysphagia. The combination of FEES® and narrow band light (narrow band imaging; NBI) provides a more precise and detailed investigation method. So far, this technique could only be performed with the NBI illumination. The new version of the "professional image enhancement technique" (PIET) provides another image enhancing system. This study investigates the eligibility of PIET in the FEES® procedure. METHODS: Both techniques, NBI and PIET, were compared using a target system. Furthermore, the image enhancement during FEES® was performed and recorded with the two systems during daily routine. RESULTS: Performing an image enhancement during FEES® is possible with both systems PIET and NBI. On the target system, the contrast of the PIET showed a brighter and a more detailed picture. In dysphagia patients, no difference between PIET and NBI was detected. CONCLUSIONS: PIET proved to be non-inferior to NBI during image enhancement FEES®. So far, image enhancement FEES® was exclusively connected to NBI. With the PIET system, an alternative endoscopy technology is available for certain indications.

Cry features of healthy neonates who passed their newborn hearing screening vs. those who did not.

OBJECTIVES: Temporal and fundamental frequency (fo) variations in infant cries provide critical insights into the maturity of vocal control and hearing performances. Earlier research has examined the use of vocalisation properties (in addition to hearing tests) to identify infants at risk of hearing impairment. The aim of this study was to determine whether such an approach could be suitable for neonates. METHODS: To investigate this, we recruited 74 healthy neonates within their first week of life as our participants, assigning them to either a group that passed the ABR-based NHS (PG, N = 36) or a group that did not, but were diagnosed as normally hearing in follow-up check at 3 months of life, a so-called false-positive group (NPG, N = 36). Spontaneously uttered cries (N = 2330) were recorded and analysed quantitatively. The duration, minimum, maximum and mean fo, as well as two variability measures (fo range, fo sigma), were calculated for each cry utterance, averaged for individual neonates, and compared between the groups. RESULTS: A multiple analysis of variance (MANOVA) revealed no significant effects. This confirms that cry features reflecting vocal control do not differ between healthy neonates with normal hearing, irrespective of the outcome of their initial NHS. CONCLUSIONS: Healthy neonates who do not pass the NHS but are normal hearing in the follow-up (false positive cases) have the same cry properties as those with normal hearing who do. This is an essential prerequisite to justify the research strategy of incorporating vocal analysis into NHS to complement ABR measures in identifying hearing-impaired newborns.

Bilateral Cochlear Implantation in Children: Long-Term Outcome in the Adult Population With Special Emphasis on the Bilateral Benefit.

OBJECTIVE: To investigate the benefit of bilateral cochlear implantation in adults, who had been implanted being a child under the age of 10 years. STUDY DESIGN: Retrospective data analysis. SETTING: Tertiary referral center with a large cochlear implant program. MAIN OUTCOME MEASURES: Speech understanding in the Freiburg monosyllabic words in quiet and the HSM sentence test in quiet and in background noise. PATIENTS: Seventy-seven bilaterally cochlear implantation implanted adults. RESULTS: Bilateral cochlear implantation in children under the age of 10 years results in a significant benefit in speech comprehension in adulthood. In addition, a dependency regarding the time between the implantations and speech intelligibility was found. CONCLUSION: The results emphasize the benefit of bilateral cochlear implantation with a short interval between the operations in young children not only during formative years but also in adulthood.

Using ASSR with narrow-band chirps to evaluate hearing in children and adults.

PURPOSE: All studies concerning the reliability and threshold prediction of auditory steady-state responses (ASSR) focused on a particular group of patients. The present article evaluates the use of narrow-band, chirp-evoked ASSR for testing hearing in adults and children of all ages and with different types of hearing loss, as well as normal hearing. The aims are: to determine whether there are possible influencing factors, mainly the degree of hearing loss; and to validate the clinical value of using ASSR with chirp-stimuli. METHODS: This is a retrospective study of 667 patients who had been diagnosed with and treated for hearing loss at our tertiary referral center. The following results were compared: ASSR to pure tone audiometry (PTA); click-ABRs to PTA; and click-ABRs to ASSR. We then calculated mean, median and standard deviation. A regression analysis was used to examine the correlation between: ASSR and click-ABRs; "estimated" audiogram and PTA; click-ABRs and PTA; and ASSR and PTA. RESULTS: We found significant correlations at all frequencies when comparing ASSR to click-ABRs, click-ABRs to PTA, and ASSR to PTA. Concerning the degree of hearing loss, there were significant differences between the patients with normal hearing and those with moderate-to-profound hearing loss. CONCLUSION: ASSR with narrow-band chirps are a reliable tool for estimating hearing thresholds in children and adults with all kinds of hearing loss. We have demonstrated that threshold differences between PTA and ASSR are negligible in the clinical routine. The "estimated" ASSR audiogram is a good approach for communicating ASSR results to the average user.

The Vocalist in the Crib: the Flexibility of Respiratory Behaviour During Crying in Healthy Neonates.

OBJECTIVE: To evaluate the flexibility of respiratory behavior during spontaneous crying using an objective analysis of temporal measures in healthy neonates. PARTICIPANTS: A total of 1,375 time intervals, comprising breath cycles related to the spontaneous crying of 72 healthy, full-term neonates (35 females) aged between two and four days, were analyzed quantitatively. METHODS: Digital recordings (44 kHz, 16 bit) of cries emitted in a spontaneous, pain-free context were obtained at the University Children's Hospital Wurzburg. The amplitude-by-time representation of PRAAT: doing phonetics by computer (38) was used for the manual segmentation of single breath-cycles involving phonation. Cursors were set in these time intervals to mark the duration of inspiratory (IPh) and expiratory phases (EPh), and double-checks were carried out using auditory analyses. A PRAAT script was used to extract temporal features automatically. The only intervals analyzed were those that contained an expiratory cry utterance embedded within preceding and subsequent inspiratory phonation (IP). Beyond the reliable identification of IPh and EPh, this approach also guaranteed inter-individual and inter-utterance homogenization with respect to inspiratory strength and an unconstructed vocal tract. RESULTS: Despite the physiological constraints of the neonatal respiratory system, a high degree of flexibility in the ratio of IPh/EPh was observed. This ratio changed hyperbolically (r = 0.71) with breath-cycle duration. Descriptive statistics for all the temporal measures are reported as reference values for future studies. CONCLUSION: The existence of respiratory exploration during the spontaneous crying of healthy neonates is supported by quantitative data. From a clinical perspective, the data demonstrate the presence of a high degree of flexibility in the respiratory behavior, particularly neonates' control capability with respect to variable cry durations. These data are discussed in relation to future clinical applications.

Cochlear implantation in children without preoperative computed tomography diagnostics. Analysis of procedure and rate of complications.

INTRODUCTION: To evaluate the safety in cochlear implantation without preoperative computed tomography diagnostics, which was implemented into the protocol of cochlear implantation in 2013, since in the year before, new evidence concerning the risks of ionizing radiation especially in children arose. METHODS: In this retrospective data analysis 89 children under 36 months, which were cochlear implanted from 2008 until 2018 at a tertiary referral centre with a large cochlear implant program were analysed. Fortyfour of the children were implanted before the date of change in 2013 and 45 in the following years up to now. The data about the operative procedures, the postoperative care and the complication rate before and after implementation of the new protocol were compared. RESULTS: Before the date of change in 2013, 100% of patients received preoperative CT diagnostics, in the following years 13.3%. No difference in the duration of surgery, the procedure related and the late complications between the two groups was identified. CONCLUSION: Cochlear implantation in very young children under the age of 36 months without preoperative radiological diagnostics by CT scan of the temporal bone is a safe procedure without additional risks for the patients.

Long-term treatment outcomes in children with auditory neuropathy spectrum disorder (ANSD).

INTRODUCTION: The present article shows long-term results in the hearing and speech development of children with auditory neuropathy spectrum disorder (ANSD). Some children were followed up for nearly 20 years, monitoring their progress through childhood into adulthood. METHODS: This retrospective study examined data from 10 children who were diagnosed and treated at our tertiary referral center. All children were provided with hearing aids and/or cochlear implants. The children's hearing and speech development and their social and educational development were evaluated. RESULTS: Seven children were provided with cochlear implants on both sides; three children, one of which had single-sided deafness, received hearing aids. All children with cochlear implants on both sides used their devices full-time. Speech perception ranged between 100% and 0% on one side. Five children attended a school for the hearing impaired; four children attended a regular school. Four children attended vocational training. CONCLUSIONS: At present, there is a lack of literature on the long-term outcomes of treatment in children with ANSD. The data presented show that the hearing and speech development in children with ANSD are significantly heterogeneous. Regular school education and social integration of children with ANSD can be achieved with intensive and supportive rehabilitative methods.

Usefulness of cochlear implantation in children with single sided deafness.

OBJECTIVES: Children with single sided deafness (SSD) show a poorer performance at school, which is attributable to reduced speech discrimination in noise, to reduced localization ability, and to a decreased power of concentration due to faster hearing exhaustion. Therefore, it is important to provide children with SSD with adequate hearing amplification to restore binaural hearing. This can only be achieved by provision with a cochlear implant (CI). But these treatment option in children with SSD is still under discussion. The aim of the present study is to evaluate audiological and clinical results in children with SSD following cochlear implantation. A special focus was placed on the duration of deafness before implantation and on the frequency of CI-use in everyday life. METHODS: Seven children with SSD of different etiologies who were provided with a CI between 3 and 16 years of age were evaluated. Every child underwent multiple audiological tests before and after cochlear implantation. After cochlear implantation speech recognition tests in noise using the HSM (Hochmair, Schulz and Moser 1997) test and localization tests were performed. Furthermore, the frequency of implant use was evaluated. RESULTS: Speech recognition in noise with CI compared to the unaided condition significantly improved in all children in different settings. Improvement of the localization ability measured by the root mean square error (RMSE) was shown in all children. All children are very satisfied with the decision to have undergone cochlear implantation and are all full-time users. CONCLUSIONS: Cochlear implantation benefits speech recognition in noise and sound localization ability in children with SSD at different ages. All implanted children are full-time users regardless of age or duration of deafness before implantation.

Laryngeal Constriction Phenomena in Infant Vocalizations.

Purpose Instances of laryngeal constriction have been noted as a feature of infant vocal development. The purpose of this study was to directly evaluate the developmental occurrence of laryngeal constriction phenomena in infant crying, cooing, and babbling vocalizations. Method The cry and noncry vocalizations of 20 healthy term-born infants between the ages of 1 and 7 months were examined for instances of laryngeal constriction. Approximately 20,000 vocalization samples were acoustically evaluated, applying a combined visual (frequency spectra and melody curves) and auditory analysis; the occurrence of instances of different constriction phenomena was analyzed. Results Laryngeal constrictions were found during the production of cry and noncry vocalizations. The developmental pattern of constrictions for both vocalizations was charac-terized by an increase in constrictions followed by a decrease. During the age period of 3-5 months, when cry and noncry vocalizations were co-occurring, laryngeal constrictions were observed in 14%-22% of both types of vocalizations. An equal percentage of constrictions was found for both vocalizations at 5 months of age. Conclusions The findings confirm that the production of laryngeal constriction is a regularly occurring phenomenon in healthy, normally developing infants' spontaneous crying, cooing, and marginal babbling. The occurrence of constriction in both cry and noncry vocalizations suggests that an infant is exploiting physiological constraints of the sound-generating system for articulatory development during vocal exploration. These results lend support to the notion that the laryngeal articulator is the principal articulator that infants 1st start to control as they test and practice their phonetic production skills from birth through the 1st several months of life.

Evaluation and therapy outcome in children with auditory neuropathy spectrum disorder (ANSD).

OBJECTIVES: The aims of the present study are to: describe diagnostic findings in patients with auditory neuropathy spectrum disorder (ANSD); and demonstrate the outcomes of different therapies like hearing aids (HAs) or cochlear implantation. METHODS: 32 children were diagnosed and treated at our tertiary referral center and provided with HAs or cochlear implants (CIs). All of them underwent free-field or pure-tone audiometry. Additionally, otoacoustic emissions (OAEs), impedance measurements, auditory brainstem responses (ABRs), auditory steady-state responses (ASSR), electrocochleography, and cranial magnetic resonance imaging (cMRI) were all performed. Some patients also underwent genetic evaluation. Following suitable provision pediatric audiological tests, psychological developmental diagnostic and speech and language assessments were carried out at regular intervals in all the children. RESULTS: OAEs could initially be recorded in most of the children; 17 had no ABRs. The other eight children had a poor ABR morphology. Most of the children had typical, long-oscillating cochlear microphonics (CMs) in their ABRs, which was also observed in all of those who underwent electrocochleography. Eight children were provided with a HA and 17 received a CI. The functional gain was between 32 and 65 decibel (dB) with HAs and between 32 and 50 dB with CI. A speech discrimination level between 35 and 100% was achieved during open-set monosyllabic word tests in quiet with HA or CI. With the Hochmair-Schulz-Moser (HSM) sentence test at 65 dB SPL (sound pressure level), 75% of the children with a CI achieved a speech discrimination in noise score of at least 60% at a signal to noise ratio (SNR) of 5, and four scored 80% or higher. Most of the children (72%) were full-time users of their devices. All the children with a CI used it on a regular basis. CONCLUSION: Only a few case reports are available in the literature regarding the long-term outcomes of ANSD therapy. The present study reveals satisfactory outcomes with respect to hearing and speech discrimination in children with CIs or HAs. The nearly permanent use of the devices reflects a subjective benefit for the children. Provision with a suitable hearing device depends on audiological results, the speech and language development of an individual child, and any accompanying disorders. Repeated audiological evaluations, interdisciplinary diagnostics, and intensive hearing and speech therapy are essential for adequate rehabilitation of this group of children.

Hearing Impairment in Old Age.

BACKGROUND: Hearing impairment associated with old age (presbycusis) is becoming more common because the population is aging. METHODS: This review is based on publications retrieved by a selective search in Medline and Google Scholar, including individual studies, meta-analyses, guidelines, Cochrane reviews, and other reviews. RESULTS: The cardinal symptom of presbycusis is impaired communication due to bilateral hearing impairment. Patients may be unaware of the problem for a long time because of its insidious progression. Evidence suggests that untreated hearing impair- ment in old age can have extensive adverse effects on the patient's mental, physical, and social well-being. Early detection is possible with the aid of simple diagnostic tests or suitable questionnaires. In most cases, bilateral hearing aids are an effective treatment. Surgery is rarely indicated. For patients with uni- or bilateral deafness, a cochlear implant is the treatment of choice. These treatments can improve many patients' quality of life. CONCLUSION: The small amount of evidence that is currently available suggests that presbycusis is underdiagnosed and under- treated in Germany. Early detection by physicians of all specialties, followed in each case by a specialized differential diagnostic evaluation, is a desirable goal.

Bilateral cochlear implantation is regarded as very beneficial: results from a worldwide survey by online questionnaire.

PURPOSE: Bilateral cochlear implant (CI) provision is now widely regarded as the most beneficial hearing intervention for acceptable candidates. This study sought to determine if a number of well-regarded hearing professionals at highly reputable clinics shared similar practices and beliefs regarding bilateral CI provision, use, and rehabilitation in children and adults. METHODS: An 11-question online questionnaire was created and distributed to all 27 clinics in the HEARRING group. Questions 1-5 asked for facts; questions 6-11 asked for opinions. RESULTS: 20 completed questionnaires were returned. All 20 respondents reported that their clinics perform bilateral cochlear implantation in children; 18 do so in adults. Regarding the fact-based questions, bilateral CI provision is more commonly performed and more likely to be reimbursed in children than in adults. Children are also much more likely to be implanted simultaneously than are adults. Regarding the opinion-based questions, respondents gave broadly similar answers. Communication between the CIs and speech coding strategies specifically developed for bilateral CI users were regarded as the two future technologies that would most enhance the benefit of bilateral CI use. CONCLUSIONS: Most clinics in the HEARRING group are very familiar with bilateral CI provision and hold similar opinions on its results and benefits. Hopefully the results described herein will lead to a greater acceptance and regular reimbursement of bilateral CI provision, especially in adults.

Phenotypic Characterization of DFNB16-associated Hearing Loss.

HYPOTHESIS: We hypothesized that patients with DFNB16 caused hearing loss show characteristical audiological findings depending on genetic results. BACKGROUND: Hearing loss belongs to the most frequent congenital diseases. In 50-70% of individuals, hearing loss is caused by genetic defects. DFNB1 (deafness, neurosensory, autosomal-recessive) is the most frequently affected locus. Despite its great genetic heterogeneity, comprehensive analysis of genes like STRC, encoding stereocilin (DFNB16) is possible. The genetic architecture of the DFNB16 locus is challenging and requires a unique molecular genetic testing assay. The aim of the study is a systematic characterization of the audiological phenotype in DFNB16-positive patients. METHODS: Since 2011, 290 patients with suspicion of inherited hearing loss received a human genetic exploration. Eighty two DFNB1-negative patients advanced to further testing in the DFNB16 locus. STRC-positive patients obtained complete audiological diagnostic workup. Additionally, epidemiological data was collected. RESULTS: Nine of 82 (11%) of the examined patients (mean age 5 yr) showed mutations in the STRC (3 homozygous, 6 compound heterozygous). Aside from a moderate hearing loss in the pure tone audiogram, auditory brainstem response thresholds were 40-50 dB nHL. Otoacoustic emissions were detectable in only one patient. CONCLUSIONS: Examination of the DFNB16-locus should be a standard diagnostic test after negative DFNB1-gene screening result. Notably, DFNB16-associated hearing loss can be audiologically characterized as moderate sensorineural hearing loss in the main speech field with absent otoacoustic emissions. Our study is the first to correlate audiological findings with genetic results in patients with hearing loss due to STRC.