BACKGROUND: Hearing loss (HL) is one of the most common congenital conditions and exhibits substantial clinical and genetic heterogeneity. More than 150 genes are associated with non-syndromic hearing loss (NSHL), while over 600 genes are linked to syndromic hearing loss (SHL). Importantly, the absence of additional clinical symptoms at the time of diagnosis does not necessarily exclude SHL. An increasing number of functionally disruptive variants in a growing number of genes have been shown to initially present as isolated HL, only later revealing syndromic features. METHODS: We analyzed clinical data from 111 patients across 102 unrelated families, selected from over 600 individuals negative for GJB2 and STRC variants. Molecular inversion probe panel, exome, or genome sequencing was performed, and patients were retrospectively divided into three subgroups following variant interpretation. Molecular docking was performed on select non-synonymous substitutions. RESULTS: Subgroup 1 included 30 patients with variants in neurodevelopmental disorder (NDD)-associated genes. HL was the first clinical manifestation in 80% of patients, with it being the sole first symptom in half. Subgroup 2 was comprised of 52 patients with variants in SHL-associated genes unrelated to NDD, while subgroup 3 included 29 patients with variants in genes associated with both NSHL and SHL, such as SLC26A4 and USH1C. In subgroups 2 and 3, HL was the sole initial symptom for nearly all patients (92% and 100%, respectively). Across the cohort, 99 variants in 44 genes were identified, including 36 novel variants. CONCLUSION: The frequent absence of syndromic features at presentation may lead to genetic testing or analysis restricted to NSHL-associated genes. Our findings highlight the critical role of comprehensive genomic testing in the diagnostic workup of HL, enabling earlier identification of syndromic forms and facilitating timely medical management, genetic counseling, and anticipatory care.

OBJECTIVE:To evaluate the audiological efficacy, patient-reported outcomes, and safety of an active middle ear implant (AMEI) in children and adults with distinct coupling options. STUDY DESIGN:Prospective, longitudinal, multicenter, noninterventional observational study with repeated within-subject measures and a 12-month follow-up. SETTING:Nine tertiary referral centers in Germany, Austria, and Poland. PATIENTS:Adults and children with sensorineural, conductive, or mixed hearing loss. Adults were grouped by coupling method: incus long process (LP), incus short process (SP), round window (RW), or stapes head (SH); multiple couplers were used in children. INTERVENTION:AMEI with coupler. MAIN OUTCOME MEASURES:The primary outcome was the difference in speech recognition thresholds for 50% speech recognition (SRT50) in quiet between unaided preoperative and aided 12-month postactivation. Secondary outcomes included SRT50 in noise, sound-field thresholds, patient-reported outcomes, air- and bone-conduction thresholds, and adverse events. RESULTS:Audiological outcomes were evaluated in 67 patients. SRT50 in quiet significantly improved across all subgroups, with large effect sizes (Cohen dz>0.8): 17.9±9.3 dB (LP), 18.3±8.4 dB (SP), 25.3±6.3 dB (RW), 23.1±17.6 dB (SH), and 21.6±9.4 dB in children. SRT50 in noise improved by 4.3 to 9.2 dB, and functional gains ranged from 18.3 to 39.2 dB. Patients reported positive impacts on quality of life and perceived quality of hearing. Two device explantations and 2 revision surgeries occurred. Exploratory analyses showed no statistically detectable differences between coupling approaches. CONCLUSION:AMEI use was associated with significant audiological and patient‑reported improvements in adults and children. Within the limitations of this observational study, including limited power and baseline heterogeneity, no statistically detectable differences in outcomes were observed between coupling approaches. Coupler selection should therefore remain guided by anatomic and clinical considerations.

Pediatric dysphagia represents a complex and multifactorial clinical challenge with potentially profound implications for development, nutritional status, pulmonary health, and overall quality of life. In infants and children, the early identification of swallowing difficulties is critical due to their impact on growth trajectories, neurodevelopment, and the psychosocial well-being of both the child and caregivers. This comprehensive article examines the importance of a systematic, interdisciplinary diagnostic approach in evaluating pediatric dysphagia in a university hospital. Based on a ten-year retrospective cohort study involving 223 pediatric patients aged one month to sixteen years, we investigate how the integration of clinical history, physical examination, and state-of-the-art instrumental techniques - such as flexible endoscopic evaluation of swallowing (FEES) - within a collaborative, multi-specialist framework can enhance diagnostic precision. Findings underscore the necessity of individualized, developmentally sensitive diagnostic pathways involving phoniatricions, ENT specialists, pediatricians, speech-language pathologists, radiologists and nutrition experts.

Background:Understanding the phenotypic spectrum of disease-associated genes is essential for accurate diagnosis and targeted therapy. FRMPD4 (FERM and PDZ Domain Containing 4) has previously been associated with intellectual disability and epilepsy. However, its potential role in non-syndromic hearing loss has not been explored. Methods:We performed genetic analysis in two unrelated families presenting with non-syndromic sensorineural hearing loss, identifying maternally inherited missense variants in FRMPD4. Clinical phenotyping included audiological assessment and evaluation for neurodevelopmental involvement. Cross-species expression analyses were conducted in Drosophila, zebrafish, and mouse. Functional characterization included quantitative evaluation of sound-evoked responses in Drosophila nicht gut hörend (ngh) mutants, assessment of neuronal development and acoustic startle responses in zebrafish loss of function models, and morphological cochlear analyses with auditory brainstem response measurements in knockout mice. Results:Three affected males from two unrelated families presented with prelingual, bilaterally symmetrical sensorineural hearing loss, with confirmed congenital onset in one individual and no evidence of neurodevelopmental abnormalities. Cross-species analyses demonstrated evolutionarily conserved expression of FRMPD4 in auditory structures. In Drosophila, quantitative analysis of sound-evoked responses in ngh mutants revealed impaired auditory function. Zebrafish loss of function models exhibited reduced neuronal populations in the otic vesicle and posterior lateral line, abnormal neuromast development, and diminished acoustic startle responses. In mice, Frmpd4 knockout resulted in high-frequency hearing loss and cochlear abnormalities consistent with the human phenotype. Conclusions:Our findings expand the phenotypic spectrum of FRMPD4 to include non-syndromic sensorineural hearing loss and establish its evolutionarily conserved role in auditory function. These results have direct implications for genetic diagnosis and variant interpretation in patients with hearing loss.

Universal newborn hearing screening (UNHS) has become widely adopted worldwide as a standard of care for the early detection of congenital hearing loss. The Egyptian UNHS program started as a presidential initiative by the Ministry of Health in November 2019. The program was initiated in 1346 primary health care units (PHCUs) located throughout the 26 governorates. A retrospective study was conducted to assess the performance of the Egyptian Program during the period from November 2019 to July 2023. Quality measures recommended by the Joint Committee on Infant Hearing including coverage rate, rate of referral to a second screening, follow up rate of attendance of second screening, referral for diagnosis rate, and follow up rate of attendance of diagnostic assessment, were analyzed. Over a period of 3 years and 9 months, more than five and half million infants underwent a first screening. The coverage rate was initially 39% and increased to reach 82% in 2023. The rate of referral to a second screen was 7.2% in 2019 and reached 5.2% in 2023. The follow-up rate of attendance of a second screening improved throughout the study period, from 75.5% to 92.1% but did not reach the benchmark of 95%. The rate of referrals for diagnosis was less than 1.7% and rate of attendance of a diagnostic assessment was initially 20% and improved to more than 65% in 2023. The very low rate of attendance of diagnostic assessment in 2020 and 2021 was attributed to the effects of the COVID pandemic.

There is a paucity of knowledge about the early vocal development of infants with profound sensorineural hearing loss. In a search for potential differences in cooing vocalisations between hearing impaired (n = 12) and healthy infants (n = 12) aged 2–4 months, more than 2300 sounds were quantitatively analysed. Using objective criteria, laryngeal activity was analysed. Vocalisation melodies (time function of fundamental frequency) were recorded and grouped into simple (single arc) or complex (multiple arc) melody pattern. Occurrence of supra-laryngeal (articulatory) activity was also analysed using frequency spectrograms. Statistical analysis employed multilevel mixed-effects modified Poisson regression models, with cooing vocalisations nested within infants. Infant age, but not sex, was significantly associated with increasing complexity in both vocal activities over the observation period. Constrained auditory feedback affects both laryngeal and articulatory activity. The group of hearing impaired infants produced significantly less melodic complexity and their cooing showed fewer articulatory activity. Recognition of a delay in these early developmental processes will considerably improve our understanding of deviations in early preparatory processes for language acquisition in hearing impaired infants. Most importantly, it highlights the need to address the lack of objective, detailed studies of pre-lingual sound production in the context of increasingly earlier hearing aid provision.

BackgroundIn cases of congenital sensorineural hearing loss, testing for genetic etiologies and congenital cytomegalovirus (cCMV) infection have become common practice.Aims/ObjectivesThe purpose of this study is to determine which specific testing methodologies should be used and when.Material and methodsWe surveyed 20 practicing otolaryngologists across eighteen institutions in thirteen countries about their approach to cCMV, GJB2, and wider genetic testing.ResultsWe found 90% of respondents employ all three tests, either in routine or special cases. cCMV testing is widely used, with 95% of respondents incorporating it into their clinical practice. GJB2 testing was employed by 90%. In cases with negative GJB2 test results, a majority of respondents proceeded to wider genetic screening. Test reimbursement was also examined for each test. For cCMV testing, 63.1% reported reimbursement, 68.4% reported reimbursement for GJB2 variant testing and 52.6% reported reimbursement for wider genetic screening.Conclusions and significanceA common approach is to perform cCMV and GJB2 testing as the first tests, followed by wider genetic testing. This study offers insight into the prevalence, methodologies, and reimbursement status of these testing methodologies across multiple hearing centers and countries. Current consensus and future directions are described based on the current survey. (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic), (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic) (cCMV) (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic) 13 (sic)(sic)(sic)/(sic)(sic)18(sic)(sic)(sic)(sic)20(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic), (sic)(sic)(sic)(sic)(sic)cCMV,GJB2(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).(sic)(sic)(sic)(sic)(sic)(sic) 90% (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).cCMV(sic)(sic)(sic)(sic)(sic)(sic)(sic), 95% (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).90% (sic)(sic)(sic)(sic)(sic) GJB2 (sic)(sic).(sic) GJB2 (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic), (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).(sic)(sic) cCMV (sic)(sic), 63.1% (sic)(sic)(sic)(sic)(sic)(sic)(sic), 68.4% (sic)(sic)(sic)(sic)(sic) GJB2 (sic)(sic)(sic)(sic)(sic)(sic)(sic), 52.6% (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic) cCMV (sic) GJB2 (sic)(sic), (sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)/(sic)(sic)(sic)(sic)(sic)(sic)(sic),(sic)(sic)(sic)(sic)(sic)(sic)(sic).(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic)(sic).

Hearing loss (HL) is one of the most common sensory disorders. Despite of environmental causes, genetics plays a major role for HL, especially for children. Due to the enormous clinical and genetic heterogeneity of HL, a complex diagnostic approach is necessary for understanding the underlying pathogenesis in a given individual or family. Even though there is no gene therapy for HL yet, the diagnostic results have an influence on further treatment. Herein, we present as an example two cases which explore the importance of genetic diagnostic. The resulting insight ended in development of the already existing genetic consultation at our hearing center into a B-Center for rare diseases, the ZGI (Zentrum für genetische Innenohrschwerhörigkeit). Familial and case history of the HL were recorded in our hearing center and the Institute of Human Genetics, followed by the DNA analysis using state-of-the-art NGS technologies. The results were interdisciplinary discussed and communicated to the patients. This procedure was optimized in the ZGI. Our first case was initially supposed to have a CMV associated HL without familiar HL, but we discovered a pathogenic variant in TMC1. The second case was a familial HL with brachio-otic symptoms. The detected pathogenic variant in EYA1 in this family is known to be associated with brachio-oto-renal syndrome, which requires further medical care for these patients. On general, as well as in the ZGI, the estimated solve rate of genetic HL is around 50%, which could be increased with technical progress and knowledge. These cases demonstrate the importance of a good collaboration between clinician and geneticists. The aim of the ZGI is to improve this interaction in order to support mental, physical, and clinical patient care.

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.

HS ist eine der häufigsten sensorischen Störungen. Neben umweltbedingten Ursachen spielt die genetische Veranlagung eine große Rolle bei HS, speziell bei Kindern. Aufgrund der verschiedenen Arten von genetisch bedingter HS ist ein diagnostischer Ansatz zur Klärung nötig. Trotz fehlender Gentherapie haben die diagnostischen Ergebnisse Einfluss auf die weitere Behandlung. Wir stellen hier exemplarisch zwei Fälle vor, die die Bedeutung der genetischen Diagnostik aufzeigen. Die daraus resultierenden Erkenntnisse mündeten in den Ausbau der bereits bestehenden genetischen Sprechstunde an unserem Hörzentrum zu einem B-Zentrum für seltene Erkrankungen, dem ZGI (Zentrum für genetische Innenohrschwerhörigkeit). Familien- und Krankengeschichte der HS wurden im Hörzentrum und dem Institut für Humangenetik erfasst, gefolgt von der DNA-Analyse mit neuen NGS-Technologien. Die Ergebnisse wurden interdisziplinär diskutiert und den Patienten mitgeteilt. Diese Kooperation wurde im ZGI optimiert. Beim ersten Fall wurde zunächst eine CMV-assoziierte HS ohne familiäre HS vermutet, aber wir entdeckten eine pathogene Variante in TMC1. Beim zweiten Fall handelte es sich um einen familiären HS mit brachio-otischen Symptomen. Die in dieser Familie entdeckte pathogene Variante in EYA1 ist bekanntlich mit dem brachio-oto-renalen Syndrom assoziiert, was eine weitere medizinische Betreuung dieser Patienten erfordert. Allgemein sowie im ZGI beträgt die geschätzte Aufklärungsrate der genetischen HS etwa 50%, die mit technischem Fortschritt und Wissen gesteigert werden könnte. Diese Fälle zeigen, wie wichtig eine gute Zusammenarbeit zwischen Klinikern und Genetikern ist. Das Ziel des ZGI ist die Interaktion zur Unterstützung der psychischen, physischen und klinischen Patientenversorgung zu verbessern.

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: 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: Does a lack of auditory feedback have a noticeable effect on melodic features of vocants?Methods: The cooing database totalled 6998 vocalizations (HI: N= 2847; CO: N= 4151), all of which had been recorded during the observation period of 60-181 days of age. Identification of the vocants (N=1148) 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.

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.

ObjectiveTo investigate the benefit of intraoperative auditory brainstem response (ABR) measurements in revision active middle ear implant surgery.Study designRetrospective data analysis.SettingTertiary referral center with a large active middle ear implant program.Main outcome measuresIntraoperative ABR thresholds, audiogram, sound field thresholds, speech understanding in the Freiburger monosyllabic word test.PatientsFourteen patients with active middle ear implant revision surgery.ResultsThe 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.ConclusionABR 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.

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.

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.

CAEP dienen nicht nur der Erfassung der objektiven Hörschwelle für komplexe Stimuli, sondern auch der Untersuchung der thalamo-kortikalen Hörbahn. Bei einer Hörminderung im Alter kommt es etwa ab dem 50. Lebensjahr durch Veränderungen der Cochlea, des Hörnervs aber auch der zentralen Hörbahn zu einer progredienten Schwerhörigkeit. In dieser Arbeit wurden die CAEP mit unterschiedlichen Silben- und Rauschstimuli bei normalhörenden jungen Erwachsenen und über 50-jährigen Erwachsenen untersucht.

CAEP are not only a useful tool for objective measurement of hearing thresholds for complex stimuli, but they also allow gaining information on the integrity of the thalamo-cortical auditory pathway. As a part of the aging process, morphological and functional changes of the cochlea, the auditory nerve and the central auditory pathway will lead to the development of a slowly progressive hearing loss. To evaluate such changes, the latencies and morphology of the CAEP after different syllable and noise stimuli were investigated in normal-hearing young adults and adults older than 50 years.

CAEP can be evoked with a variety of stimuli with and without hearing aids. They offer a possibility to objectively obtain aided responses to complex stimuli. P1 can be used as a measurement tool for the evaluation of the benefit from amplification, as well as for the evaluation of auditory pathway maturation in children. The aim of this study was to investigate CAEP wave identifiability and latencies after different syllable and noise stimuli in different age groups.

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.