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Objective: Investigation of the gustatory function in a large cohort of cochlear implanted patients using lateralized taste-strip tests., Patients and Methods: One hundred and seven unilaterally or bilaterally profoundly hearing impaired or deaf patients who received cochlear implants (n = 113) were included in this study. Data on gustometry, subjective gustatory dysfunction, and the detailed surgical procedure were acquired retrospectively. Gustatory function, assessed using lateralized taste-strip tests, was performed the day before, 3 days after cochlear implantation, and on the day of the initial CI adjustment (39 days ±7.3 SD)., Results: Averaged taste-strip scores of the cohort declined significantly from preoperatively 12.3 [11.8; 12.7] (mean [95% confidence intervals]) to 10.5 [9.7; 11.2] on the implanted side about 6 weeks after surgery. Patients with intraoperatively exposed and rerouted, or a severed, chorda tympani nerve (CTN) showed significantly reduced unilateral postoperative scores (10.1 [8.8; 11.4] and 9.3 [8.1; 10.5], respectively), when compared to not exposing or to leaving a bony layer over the CTN. Total taste-strip test scores showed a significant decline 6 weeks postoperatively in CI-patients expressing a subjective gustatory dysfunction (from 23.6 [21.4; 25.8] to 17.5 [14.2; 20.8]), as opposed to patients with a documented subjectively normal taste., Conclusion: We consider postoperative gustatory dysfunction as a relevant side effect post cochlear implantation, at least within the first month. Taste-strip based gustometry is a suitable diagnostic tool to assess taste function in CI patients and is recommended to be performed routinely., Level of Evidence: 3, retrospective, nonrandomized follow-up study., Competing Interests: The authors declare no conflicts of interest., (© 2021 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals LLC on behalf of The Triological Society.)

Background: Delayed complications after cochlear implant (CI) surgery can result in the need for revision surgery. Temporal bone computed tomography (tCT) is frequently used in the diagnostic work-up for these patients., Aims/objectives: We aimed to test for the value of tCT in delayed complications after CI surgery., Material and Methods: Retrospective chart review including all patients with CI who were consecutively enrolled in our academic tertiary care centre and received a tCT due to delayed complications between January 2008 and December 2017., Results: A total of 85 patients with a mean age of 28 years at CI surgery were identified. Mean time between CI and tCT was 4.3 years. Reasons for performance of tCT were device malfunction (20%), cephalgia (20%), scalp hematoma/seroma (15.3%), device failure (11.8%), vertigo (10.6%), local infection (7.1%), trauma (5.9%), meningism (3.5%), tinnitus (3.5%) and facial palsy (2.4%). No abnormalities in tCT were detected in 82.4%. However, an abnormal tCT was a predictor for surgical therapy ( p  < .001)., Conclusions and Significance: We recommend the performance of tCT in CI patients with delayed complications to identify or rule out pathology in order to choose the most ideal and relevant individual treatment.

Purpose The purpose of this letter is to compare results by Skuk et al. (2020) with Meister et al. (2016) and to point to a potential general influence of stimulus type. Conclusion Our conclusion is that presenting sentences may give cochlear implant recipients the opportunity to use timbre cues for voice perception. This might not be the case when presenting brief and sparse stimuli such as consonant-vowel-consonant or single words, which were applied in the majority of studies.

Purpose This study aims to investigate the effect of upgrading from the fine structure processing (FSP) coding strategy to the novel fine structure strategy "FS4" in adults in adults with cochlear implants manufactured by MED-EL GmbH (Innsbruck, Austria). Method A crossover, double-blinded study was conducted for 12 weeks. Twelve adult participants were randomly assigned to two groups. During the first 6-week test interval, one group continued to use their everyday FSP strategy, whereas the other group was upgraded to the FS4 strategy. In the second 6-week interval, the two groups switched coding strategies. Speech perception was measured at the end of each test interval with the Oldenburg Sentence Test and the Göttingen Sentence Test. Participants completed the Speech, Spatial and Qualities of Hearing Scale at the end of each test interval and a simple preference test at the end of the study. Results There was no significant difference in speech perception test results obtained with the Oldenburg Sentence Test and the Göttingen Sentence Test, neither in quiet nor in noise. Participants' Speech, Spatial and Qualities of Hearing Scale self-evaluation and preference test results showed that the two coding strategies had similar effects on their hearing perception. No clear preference for either of the strategies was found. Conclusions Speech perception test results and the participants' level of satisfaction were similar for the two FS coding strategies. Despite differences in the presentation of temporal fine structure between FSP and FS4, a clear benefit of the newer FS4 strategy could not be shown.

Cochlear implant (CI) recipients are limited in their perception of voice cues, such as the fundamental frequency (F0). This has important consequences for speech recognition when several talkers speak simultaneously. This examination considered the comparison of clear speech and noise-vocoded sentences as maskers. For the speech maskers it could be shown that good CI performers are able to benefit from F0 differences between target and masker. This was due to the fact that a F0 difference of 80 Hz significantly reduced target-masker confusions, an effect that was slightly more pronounced in bimodal than in bilateral users.

Aim: Hearing loss in infants is often diagnosed late, despite universal screening programmes. Risk factors of hearing impairment in high-risk neonates, identified from population-based studies, can inform policy around targeted screening. Our aim was to determine the prevalence and the risk factors of hearing loss in a high-risk neonatal population., Methods: This was a retrospective cohort study of neonates hospitalised at the University Hospital Cologne, Germany from January 2009 to December 2014 and were part of the newborn hearing screening programme. Multivariable regression analyses using the lasso approach was performed., Results: Data were available for 4512 (43% female) neonates with a mean gestational age at birth of 35.5 weeks. The prevalence of hearing loss was 1.6%, and 42 (0.9%) neonates had permanent hearing loss. Craniofacial anomalies, hyperbilirubinaemia requiring exchange transfusion, oxygen supplementation after 36 weeks of gestation and hydrops fetalis showed associations with permanent hearing loss., Conclusion: Our findings of risk factors for hearing loss were consistent with other studies. However, some commonly demonstrated risk factors such as perinatal infections, meningitis, sepsis and ototoxic drugs did not show significant associations in our cohort. Targeted screening based on risk factors may help early identification of hearing loss in neonates., (©2019 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Objective: To examine the possible correlation between the loss of short-term residual hearing and vestibular function after cochlear implantation (CI)., Study Design: Retrospective patient review., Setting: Academic tertiary referral center., Patients: One hundred twenty patients with normal vestibular function (VF) in the caloric testing and residual hearing (RH) at the frequencies 250, 500, and 1000 Hz on the surgery side between 2008 and 2016 were included in the study., Intervention: Primary CI on the first side via round window with a conventional full-length electrode., Main Outcome Measures: Changes of RH and VF 7 weeks after surgery were analyzed. Preservation of RH was defined as measurable postoperative thresholds at the frequencies 250, 500, and 1000 Hz in the pure-tone audiogram. Preservation of the VF after CI was assessed both by an absolute and relative threshold in the caloric testing., Results: Seven weeks after implantation, the preservation of RH was achieved in 52 (43.3 %) patients and the preservation of VF in 95 (79.2 %) patients on the operated side. There was no significant statistical correlation between these two parameters., Conclusion: The loss of RH was more than twice as frequent as the loss of VF. However, no statistical correlation between the failure of the two inner ear functions was found. The exact causes are still unknown, but our data could give an indication that there may be different underlying pathomechanisms.

Purpose: This study aimed to investigate whether adults with cochlear implants benefit from a change of fine structure (FS) coding strategies regarding the discrimination of prosodic speech cues, timbre cues, and the identification of natural instruments. The FS processing (FSP) coding strategy was compared to 2 settings of the FS4 strategy., Method: A longitudinal crossover, double-blinded study was conducted. This study consisted of 2 parts, with 14 participants in the first part and 12 participants in the second part. Each part lasted 3 months, in which participants were alternately fitted with either the established FSP strategy or 1 of the 2 newly developed FS4 settings. Participants had to complete an intonation identification test; a timbre discrimination test in which 1 of 2 isolated cues changed, either the spectral centroid or the spectral irregularity; and an instrument identification test., Results: A significant effect was seen in the discrimination of spectral irregularity with 1 of the 2 FS4 settings. The improvement was seen in the FS4 setting in which the upper envelope channels had a low stimulation rate. This improvement was not seen with the FS4 setting that had a higher stimulation rate on the envelope channels., Conclusions: In general, the FSP strategy and the 2 settings of the FS4 strategy provided similar levels in the perception of prosody and timbre cues, as well as in the identification of instruments.

Introduction: The opening of the round window and the insertion of the electrode array into the scala tympani during cochlear implant surgery can lead to a pressure shock of the delicate inner ear structures. By filling the tympanic cavity with Ringer Solution during these surgical steps (underwater technique), the hydrostatic pressure of the fluid acts as a smooth pressure stabilizer, avoiding a pressure shock of the inner ear structures. The aim of this retrospective study was to present long-term results of this new method of cochlear implantation in underwater technique., Methods: Altogether, 47 implantations in 43 patients with residual hearing at the frequencies 250, 500 and 1000 Hz in the unaided preoperative pure tone audiometry were included. A cochlear implantation via round window with a conventional full-length electrode was performed in underwater technique. Changes of residual hearing 7 weeks and 24 months after surgery were analyzed., Results: Overall postimplant hearing preservation 7 weeks after implantation was achieved in 22 ears (47%). Subsequent follow-up was performed on average 24 months after surgery (range 12 months-4.2 years) in all patients. At this late postoperative evaluation, preservation of hearing was recorded in 18 ears (38%). Neither the follow-up time nor the type of electrode had a significant impact on the postoperative hearing loss., Conclusion: The underwater technique is an atraumatic cochlear implantation technique with hearing preservation rates comparable to results in literature and a very small hearing preservation decline rate over time even when using full-length CI electrodes.

Congenital cranial dysinnervation disorders (CCDDs) comprise a heterogeneous spectrum of diseases characterized by congenital, non-progressive impairment of eye, eyelid and/or facial movements including Möbius syndrome, Duane retraction syndrome, congenital ptosis, and congenital fibrosis of the extraocular muscles. Over the last 20 years, several CCDDs have been identified as neurodevelopmental disorders that are caused by mutations of genes involved in brain and cranial nerve development, e.g. KIF21A and TUBB3 that each plays a pivotal role for microtubule function. In a five-generation pedigree, we identified a heterozygous mutation of TUBB6, a gene encoding a class V tubulin which has not been linked to a human hereditary disease so far. The missense mutation (p.Phe394Ser) affects an amino acid residue highly conserved in evolution, and co-segregates with a phenotype characterized by congenital non-progressive bilateral facial palsy and congenital velopharyngeal dysfunction presenting with varying degrees of hypomimia, rhinophonia, impaired gag reflex and bilateral ptosis. Expression of the mutated protein in yeast led to an impaired viability compared to wildtype cells when exposed to the microtubule-poison benomyl. Our findings enlarge the spectrum of tubulinopathies and emphasize that mutations of TUBB6 should be considered in patients with congenital non-progressive facial palsy. Further studies are needed to verify whether this phenotype is indeed part of the CCDD spectrum., (© The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

In about 20% of non-syndromic hearing loss (NSHL) cases, inheritance is autosomal dominant (ADNSHL). DIAPH1 mutations define the ADNSHL locus DFNA1. We identified two new families with heterozygous truncating DIAPH1 mutations (p.Ala1210Serfs*31 and p.Arg1213*). In contrast to the extensively studied original DFNA1 family, hearing loss was not confined to low frequencies, but congenital manifestation and rapid progression were confirmed. In line with a recent unrelated study, we identified an association with thrombocytopenia, reclassifying DFNA1 as a syndrome. Consequently, we suggest to include the blood count into the initial clinical workup of patients with autosomal dominant hearing loss to guide the genetic diagnosis. We provide the first data on DIAPH1 expression in the organ of Corti, where it localizes to the inner pillar cells, at the base of the outer hair cells. Homozygous truncating DIAPH1 mutations located N-terminally to the DFNA1 mutations have recently been identified in autosomal recessive microcephaly. It is therefore noteworthy that we found DIAPH1 expression also in spiral ganglion neurons and in the barrier between the myelinating glia of the peripheral nervous system and oligodendrocytes that form the myelinating glia of the central nervous system (CNS)., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: To date, the impact of conductive hearing loss on the auditory pathway at brainstem level has only been investigated in animal studies, which showed a species-specific delay of maturation. In this study, the functional maturation of auditory brainstem response (ABR) parameters in humans with unilateral atresia of the external auditory canal was investigated., Methods: 42 newborns and toddlers ranging in age from 13 days to 11 months were included. The click-evoked ABR interpeak latencies (IPL) of the atretic ears and the contralateral ears with normal hearing were evaluated. The children had no comorbidities and had never been fitted with any kind of hearing aid. The absolute latencies (AL) and IPL of a matched control group were compared to the contralateral normally hearing ears of the children with unilateral atresia., Results: The mean air-bone gap in the ears with atresia was 44 dB HL. Despite this partial acoustic deprivation, no significant difference between the IPLs of normal ears and ears with atresia could be detected. Both for AL and IPL, the differences between the normal ears and the control group were all within 1 standard deviation to the mean., Conclusion: The data showed that the monaural acoustic deprivation by a block of sound conduction does not produce any delay of functional maturation at brainstem level in this group of patients. With regard to the AL and IPL on brainstem level, no differences between the normal ears of children with unilateral atresia and children with bilateral normal hearing could be detected., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: Long term goal in early cochlea implantation in children without any additional disabilities is an age-appropriate speech development. Material and Methods: Speech development in deaf children with cochlear-implant(s) (n=60) was examined with the german language test battery SETK-2 ("Sprachentwicklungstest für 2-jährige Kinder") 2 years after first mapping of the speech processor. Results: More than 68% of the subjects show in all 4 subtests hearing-age equivalent results in receptive and expressive language. 12 children were additionally evaluated by chronological age. 4 of these children show age-appropriate results. There is no significant difference between the children implanted earlier in life (≤12 months) and later implanted children (≥13 months). But it must be kept in mind, that children who were implanted earlier show the same results at a younger age. The discrepancy between their chronological age and their speech development-age is smaller. Speech development in children who grow up bilingually was delayed in German. Conclusions: The results lead to the conclusion that the time of the cochlea implantation is crucial for further development of the children who were born deaf. Rehabilitation concepts have to put a special focus on children who grow up with more than one language., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Auditory synaptopathy/neuropathy (AS/AN) is a heterogeneous disorder, which may be caused by environmental factors like postnatal hyperbilirubinemia or by genetic factors. The genetic forms are subdivided into syndromic and non-syndromic types, and show different inheritance patterns with a strong preponderance of autosomal-recessive forms. To date, only a single locus for non-syndromic autosomal-dominant AS/AN (AUNA1) has been reported in a single family, in which a non-coding DIAPH3 mutation was subsequently described as causative., Materials and Methods: Here, we report detailed clinical data on a large German AS/AN family with slowly progressive postlingual hearing loss. Affected family members developed their first symptoms in their second decade. Moderate hearing loss in the fourth decade then progressed to profound hearing impairment in older family members. Comprehensive audiological and neurological tests were performed in the affected family members. Genetic testing comprised linkage analyses with polymorphic markers and a genome-wide linkage analysis using the Affymetrix GeneChip® Human Mapping 250K., Results and Conclusion: We identified a large family with autosomal-dominant AS/AN. By means of linkage analyses, the AUNA1 locus was excluded, and putatively linked regions on chromosomal bands 12q24 and 13q34 were identified as likely carrying the second locus for autosomal-dominant AS/AN (AUNA2). AUNA2 is associated with a slowly progressive postlingual hearing loss without any evidence for additional symptoms in other organ systems., (© 2017 S. Karger AG, Basel.)

Purpose: The focus of this study was to examine the influence of fundamental frequency (F0) and vocal tract length (VTL) modifications on speaker gender recognition in cochlear implant (CI) recipients for different stimulus types., Method: Single words and sentences were manipulated using isolated or combined F0 and VTL cues. Using an 11-point rating scale, CI recipients and listeners with normal hearing rated the maleness/femaleness of the corresponding voice., Results: Speaker gender ratings for combined F0 and VTL modifications were similar across all stimulus types in both CI recipients and listeners with normal hearing, although the CI recipients showed a somewhat larger ambiguity. In contrast to listeners with normal hearing, F0-VTL and F0-only modifications revealed similar ratings in the CI recipients when using words as stimuli. However, when sentences were used, a difference was found between F0-VTL-based and F0-based ratings. Modifying VTL cues alone did not affect ratings in the CI group., Conclusions: Whereas speaker gender ratings by listeners with normal hearing relied on combined VTL and F0 cues, CI recipients made only limited use of VTL cues, which might be one reason behind problems with identifying the speaker on the basis of voice. However, use of the voice cues depended on stimulus type, with the greater information in sentences allowing a more detailed analysis than single words in both listener groups.

Objectives/hypothesis: Bilateral vocal fold paralysis (BVFP) is a rare but life-threatening condition mostly caused by iatrogenic damage to the peripheral recurrent laryngeal nerve. Endoscopic enlargement techniques have been the standard treatment for decades. However, prospective studies using internationally accepted phoniatric and respiratory evaluation guidelines are rare., Study Design: Prospective observational multicenter study., Methods: Twelve clinical centers screened 61 patients, of whom 36 were eligible according to the study protocol. Subjects were assessed with specific phoniatric and respiratory tests preoperatively and at 1 and 6 months postoperatively., Results: Important respiratory parameters improved significantly 6 months postoperatively (peak expiratory and expiratory flow), confirming that a glottal enlargement effectively reduced the obstruction. Objective parameters dealing with voice quality worsened significantly (maximum phonation time, voice range profile, hoarseness), whereas subjective voice assessment (VHI-12) did not change significantly., Conclusion: Endoscopic glottal enlargement is an effective method for relieving symptoms of dyspnea due to BVFP. Postoperatively, voice quality objectively worsened; however, this was not perceived by the patients themselves. Laryngostroboscopic findings did not correlate strongly with voice and respiratory outcomes., Level of Evidence: 2b., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To compare the real-world verbal communication performance of children provided with cochlear implants (CIs) with their peers with hearing aids (HAs)., Study Design: Cross-sectional study in university tertiary referral centers and at hearing aid dispensers., Methods: Verbal communication performance was assessed by the Functioning after Pediatric Cochlear Implantation (FAPCI) instrument. The FAPCI was administered to 38 parents of children using CIs and 62 parents of children with HAs. According to the WHO classification, children with HAs were categorized into three groups (mild-moderate-severe hearing loss). Analysis of variance (ANOVA) was performed on the FAPCI scores, with study group, hearing age (i.e., device experience), and age at hearing intervention as sources of variation., Results: ANOVA showed that hearing age and study group significantly contribute to the FAPCI outcome. In all study groups except the children with mild hearing loss, FAPCI scores increased alongside growing experience with the devices. Children with mild hearing loss using HAs showed higher scores than those with severe hearing loss or implanted children. There were no significant differences between the children with CIs and the children with moderate or severe hearing loss using HAs., Conclusion: Real-world verbal communication abilities of children with CIs are similar to those of children with moderate-to-severe hearing loss using amplification. Because hearing age significantly influences performance, children with moderate-to-severe hearing loss using HAs and implanted children catch up with children with mild hearing loss at a hearing age of approximately 3 years.

Objective: The individual outcome after cochlear implantation in children with auditory synaptopathy/neuropathy (AS/AN) is difficult to predict. A tool for preoperative assessment would be helpful for counseling parents. This study evaluates the outcome after CI in children with AS/AN and with sensorineural hearing loss (SNHL), and correlates it with the preoperative ECochG results in order to find specific parameters of prognostic value., Design: The improvement of auditory behavior after CI was retrospectively assessed using the LittlEARS questionnaire and quantified in a score (LS). This score was correlated with the CAP/SP ratio in the preoperative ECochG. The score was further correlated with the patient's age six months following CI., Study Sample: Nine children with AS/AN were compared to nine children with SNHL., Results: Both groups showed a significant improvement in LS following CI. There was a significant positive correlation between the CAP/SP ratio and the improvement in LS in all children. The correlation between age and LS was significantly negative in the SNHL group and positive in the AS/AN group., Conclusion: All children with AS/AN and SNHL benefit to a similar extent from CI. The preoperatively assessed CAP/SP ratio has a prognostic value for the development of auditory behavior following CI.

Objectives/hypothesis: To describe postoperative adverse events (AEs) and outcomes after transoral surgery for bilateral vocal fold paralysis (BVFP)., Study Design: Prospective observational multicenter study., Methods: Thirty-six patients with BVFP underwent transoral surgery using standard surgical procedures to unilaterally widen the glottic area. Postoperative adverse events (AEs) including severe adverse events (SAEs) were registered continuously. Pre- and 6-month postoperative evaluations included the 6-Minute Walk Test, the 36-Item Short Form Health Survey (SF-36), the Glasgow Benefit Inventory, the 12-Item Voice Handicap Index (VHI-12), and a Fiberoptic Endoscopic Evaluation of Swallowing graded according to the Penetration-Aspiration-Scale., Results: The patients underwent posterior cordotomy, partial arytenoidectomy, or permanent laterofixation as single procedures or in combination. Forty-seven percent of the patients had postoperative AEs. Dyspnea was the most frequent AE (45%). In 40% of AEs, the events were severe (SAEs), and 72.5% were related to the study intervention. Revision surgery leading to prolonged hospitalization or rehospitalization was necessary in nine cases (25%). Laterofixation was correlated to a decreased risk of AEs (P = 0.042). Six months after surgery, a significant improvement was seen in the SF-domains: Physical component score (P = 0.008), physical functioning (P = 0.001), physical role (P = 0.031), and vitality (P = 0.032). Concerning the voice handicap, only the VHI-12 physical subscore showed a decrease (P = 0.005). The total score and other VHI-12 subscores did not change significantly (all P > 0.05)., Conclusion: BCVP patients profit from modern transoral surgery for unilateral glottic widening; quality of life is improved; and the voice is preserved. Nevertheless, postoperative complications are frequent., Level of Evidence: 2b., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: This study's aim is the assessment of language development of children with Cochlea Implant (CI). It focusses on receptive and expressive language development as well as auditory memory skills., Material and Methods: Grimm's language development test (SETK 3-5) evaluates receptive, expressive language development and auditory memory. Data of 49 children who received their implant within their first 3 years of life were compared to the norms of hearing children at the age of 3.0-3.5 years. According to the age at implantation the cohort was subdivided in 3 groups: cochlear implantation within the first 12 months of life (group 1), during the 13th and 24th months of life (group 2) and after 25 or more months of life (group 3)., Results: It was possible to collect complete data of all SETK 3-5 subtests in 63% of the participants. A homogeneous profile of all subtests indicates a balanced receptive and expressive language development. Thus reduces the gap between hearing/language age and chronological age. Receptive and expressive language and auditory memory milestones in children implanted within their first year of life are achieved earlier in comparison to later implanted children., Conclusion: The Language Test for Children (SETK 3-5) is an appropriate test procedure to be used for language assessment of children who received a CI. It can be used from age 3 on to administer data on receptive and expressive language development and auditory memory., (© Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: The early diagnosis of AS/AN in children remains challenging because it exclusively relies on the detection of OAE and/or CM, while ABR are pathologically changed or missing. The aim of our study was to ensure the diagnosis of AS/AN, demarcate it to an outer hair cell damage and possibly differentiate between pre- and postsynaptic pathologies., Methods: We retrospectively evaluated the transtympanic ECochG results of ten children with AS/AN and compared them to a matched group with SNHL and without any signs of AS/AN. We analyzed the thresholds, latencies and - as a new parameter - the amplitude ratio between CAP and SP., Results: CM and SP thresholds were significantly lower than CAP thresholds in AS/AN patients and significantly lower than SP and CM thresholds in SNHL patients with comparable CAP thresholds. The CAP/SP ratio of amplitudes in SNHL children was more than three times (significantly) higher than in AS/AN children. The cutoff value was set at 1.0 in order to differentiate between both groups with a 80-90% sensitivity and specificity. It was not possible to differentiate between a pre- and postsynaptic type of AS/AN in our collective., Summary and Conclusion: The ECochG can add valuable information for a precise differential diagnosis of AS/AN, especially in babyhood. We identified the CAP/SP ratio as a new parameter for differentiation between AS/AN and SNHL. When the CAP/SP ratio falls below 1.0, patients can be diagnosed AS/AN with high specificity and sensitivity. Significantly smaller SPL are needed to evoke SP and CM in the AS/AN group, thus showing the preserved hair cell function., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Objective: To investigate the prognostic significance of various factors in hearing preservation after traditional cochlear implantation (CI)., Study Design: Retrospective case review., Setting: Academic tertiary referral center., Patients: A total of 153 implantations with mean patient age at implantation of 36 years (from 10 mo to 83 yr) and residual hearing at the frequencies 250, 500, and 1,000 Hz on the unaided preoperative pure-tone audiometry were included., Intervention(s): CI with a conventional full-length electrode., Main Outcome Measure(s): The changes on the residual hearing 3 months after implantation were analyzed regarding patients' demographic factors, shape of the preoperative threshold curve, type of the electrode carrier, and approach of electrode insertion in the cochlea. Preservation of residual hearing was defined as measurable postoperative threshold at the frequencies 250, 500, and 1,000 Hz., Results: Preservation of residual hearing was observed in almost half of the cases (47%). In more than half of these patients (54%), a maximal to complete hearing preservation (0-10 dB loss) was achieved. About one-third of these implantations (29%) showed a moderate preservation of residual hearing (11-20 dB loss). In the remaining 17%, the preservation of hearing was marginal (>21 dB loss). Hearing preservation and its extent were significantly better in children and adolescents compared with those in adults., Conclusion: The preservation of residual hearing after conventional CI is possible. Young age seems to have a positive impact on hearing preservation.

Purpose: The objective of the study was to investigate the influence of noise (energetic) and speech (energetic plus informational) maskers on the head shadow (HS), squelch (SQ), and binaural summation (SU) effect in bilateral and bimodal cochlear implant (CI) users., Method: Speech recognition was measured in the presence of either a competing talker or modulated speech-shaped noise in 10 bimodal and 10 bilateral adult CI users. HS, SQ, and SU effects were calculated. The interfering signals were manipulated with respect to F0 to consider the influence of different speaker voices., Results: The effects HS, SQ, and SU differed depending on the type of masker. A detailed analysis of errors was used to dissociate energetic and informational masking effects. The analysis showed a release from energetic than from informational masking., Conclusion: Noise interferers are not sufficient to reflect difficulties experienced with speech understanding in noise for bilateral and bimodal CI users.

Background: Normal-hearing children show signs of various phonological processes during language development. These processes represent simplifications of articulation, which are overcome at different time points. For the German language, there are currently no reliable data regarding whether these developmental stages also apply to deaf children with cochlear implants (CI)., Materials and Methods: Phonological development in deaf children with CI was examined and evaluated with the PLAKSS ("Psycholinguistischen Analyse kindlicher Sprechstörungen"). The results of this analysis (time of test 1 = T1, n = 33) were compared to those of a PLAKSS diagnostic evaluation performed 1 year previously (time of test 0 = T0, n = 31)., Results: At T1, 76 % of the whole group showed a phonological development that did not correspond to their hearing age (as measured from the time of the first CI implantation). The most frequently observed phonological processes were the reduction of consonant clusters and fronting. However, 83 % of the group had fewer phonological processes inappropriate to their hearing age at T1 than they did at T0., Conclusion: The phonological development of children with CI is not equivalent to their hearing age and is structured differently to that of normal-hearing children.

Objectives: To investigate the discrimination of two isolated spectral timbre cues, spectral centroid (Fc) and spectral irregularity (spIrr), in cochlear implant (CI) listeners. To examine whether the perception of Fc and spIrr changes is related to the perception of loudness and pitch and the identification of musical instruments., Methods: Stimuli were based on French horn recordings which were artificially manipulated with respect to isolated changes in Fc and spIrr. Difference limens for Fc and spIrr were determined and changes in loudness and pitch perception based on these modifications were examined. Identification of musical instruments was additionally assessed., Results: Mean difference limens were 161 Hz for Fc and 0.63 dB for spIrr. Modifications in spectral timbre cues caused changes in loudness and pitch perception. None of the timbre cues examined showed a significant correlation with musical instrument identification. In contrast, instrument identification was significantly related to the frequency of listening to music prior to onset of deafness., Conclusions: CI recipients are able to detect small modifications in spectral timbre cues which are in turn associated with changes in loudness and pitch. Variations of spectral centroid have a larger impact on loudness and pitch perception than variations of spectral irregularity. Music listening behaviour prior to onset of deafness is significantly associated with musical instrument identification.

Background: Bearing in mind the impending evaluation of newborn hearing screening in Germany, this study investigated whether multicenter analysis of the screening results from four German states is possible and to what extent the results meet national quality and outcome criteria., Materials and Methods: The screening data from 170 hospitals and a total of 533,150 newborns (21 % of all German newborns) from 2009 to 2012 were evaluated according to definite rules and analyzed in terms of averages, as well as over time., Results: During the investigated period and averaged over the hospitals, the quality criteria "percentage of screened newborns" (91.4 %) and "percentage requiring further follow-up" (5.0 %), the "day of screening" (day 4), as well as the target parameter "age at diagnosis" (4.8 months) were not met. Steady improvements were observed over time: in the last year of the evaluation, 95.3 % of children were examined; only 4.8 % required follow-up and the age at diagnosis decreased to 4.2 months. On average, 83 % of the babies were screened before day 4. The steady reduction in variance of most of the variables from the participating hospitals indicates continual improvement., Conclusion: A multicenter analysis of screening data is possible and valid in the case of good quality data.

Background: Since 2009, all newborns in Germany have been entitled to universal neonatal hearing screening (UNHS). UNHS with tracking of test results leads to earlier detection of hearing disorders. The Association of German Hearing Screening Centers (Verband Deutscher Hörscreening-Zentralen, VDHZ) was founded to promote nationwide tracking, validity and quality control of UNHS results., Objectives: A comparable data structure in the different screening centers, with uniform definitions of primary parameters is essential for the nationwide evaluation of UNHS results. To address the question of whether a data structure with comparable definitions already exists or still has to be created, the existing structures and primary parameter definitions in the hearing screening centers should be investigated and compared., Methods: A survey was conducted in all hearing screening centers to assess how data on the primary UNHS parameters defined in pediatric guidelines was gathered. In the case of discrepancies, uniform definitions were created. Finally, the practicability of these definitions was evaluated., Results: Due to differing definitions of primary parameters, some of the data were not comparable between the individual centers. Therefore, uniform definitions were created in a consensus process. In the centers, the screening method, the two-step first screening and the result of the first screening now correspond to these uniform definitions. Other parameters, e.g. the total number of newborns, still vary widely, rendering the comparison of screening rates almost impossible., Conclusion: Valid evaluation of UNHS not only requires nationwide establishment of hearing screening centers, but also unified data structures and parameter definitions.

Background: Very immature preterm infants are at risk of developing symptomatic or severe infection if cytomegalovirus is transmitted via breast milk. It is still a matter of debate whether human cytomegalovirus (HCMV) infection may lead to long-term sequelae., Objectives: We hypothesized that symptomatic and severe HCMV infection transmitted via breast milk affects extremely immature infants at a very high rate., Methods: In 2012, untreated breast milk was fed to extremely low birth weight infants after parental informed consent was obtained. We retrospectively analyzed data on HCMV infection of infants born in 2012 between 22 and 24 weeks of gestation., Results: 17 infants were born to HCMV IgG-seropositive mothers. 11 (65%) of these were diagnosed with symptomatic infection. In all cases, thrombocytopenia was the reason to analyze the infant's urine. HCMV infection was diagnosed at a median time of 12 weeks after birth. In 5 (45%) infants, thrombocytopenia was the only symptom and resolved without antiviral therapy or platelet transfusion. 6 (55%) infants developed sepsis-like disease with mildly elevated CRP values and showed signs of respiratory failure. 3 (27%) were able to be stabilized on CPAP, 3 (27%) had to be intubated and mechanically ventilated. 4 children were treated with ganciclovir and/or valganciclovir. 55% failed otoacoustic emissions and/or automated auditory brainstem response testing at discharge., Conclusions: In very immature infants born at the border of viability and suffering from multiple preexisting problems, HCMV infection may trigger a severe deterioration of the clinical course., (© 2013 S. Karger AG, Basel.)

Objectives: This study investigates differences between the self-assessment and external rating of a person's voice with regard to sex characteristics, age, and attractiveness of the voice and mean fundamental frequency (F0)., Study Design: Cross-sectional study., Methods: A group of 47 participants with a balanced sex distribution was recruited and the following data were collected: videostroboscopy, voice range profile, F0, self-assessment questionnaire (attractiveness, masculinity or femininity of voice, and appearance), Voice Handicap Index, and questionnaires to determine levels of depression and quality of life. External rating was performed by four experts and four laymen., Results: In both sexes, fair to moderate significant correlations between the self-assessment of masculinity (men)/femininity (women) of voice and masculinity/femininity of appearance could be found, but not between the self-assessment of attractiveness of voice and appearance. In men, a statistically significant correlation was found between external ratings and self-assessment of attractiveness and, with the exception of the female rating group, of masculinity. In women, self-assessment of femininity and attractiveness of voice did not correlate to a statistically significant extent with the evaluation of the external rater. Additionally, the statistical correlation between estimated and real ages was high., Conclusions: Although the objective parameters of age and gender identification could be rated with a high degree of accuracy, subjective parameters showed significant differences between self-assessment and external rating, in particular in rating women's voices. Taking these findings into account in treatments for modifying voice could impede successful interventions. As one consequence, we recommend summarizing target agreements in detail before the treatment., (Copyright © 2014 The Voice Foundation. Published by Mosby, Inc. All rights reserved.)

Background: After cochlear implantation, most parents expect a normal speech and general development of their child. However, it remains unclear how quickly after early cochlear implantation these children can compensate for their deficits compared to normal-hearing children., Methods: This study retrospectively analyzed ELFRA-1 questionnaire data from 40 children with borderline deafness or high-grade hearing loss (without other known impairments) who had undergone cochlear implantation at a university medical center before reaching 2 years of age. ELFRA-1 questionnaires were filled out parents assisted by specialists 12 months after implantation. Questions assessed the children's speech production and comprehension, as well as their use of gestures and fine motoric skills., Results: At an average hearing-age of 12 months, the children achieved normal values in all of the subgroups that were comparable to those of 12-month-old children without hearing impairments. A significant correlation (p = 0.01) between the individual subgroups of the ELFRA-1 (speech production, speech comprehension, gestures and fine motor skills) was observed. Unilingual educated children performed significantly better overall., Conclusion: Within 12 months of receiving a cochlear implant, all children passed the four categories of the ELFRA-1. This demonstrates a rapid compensation of deficits in speech, motor skills and gesture development by children undergoing early cochlear implantation.

The objective of this study was to evaluate retrospectively the risk of cochlear implant migration and complications related to the here presented alternative surgical fixation technique of the receiver/stimulator without any foreign body materials. Fixation of the implant was achieved by an "L-shaped" muscle-periosteal flap and an exactly shaped bony well. Between January 2006 and December 2009, 247 consecutive primary cochlear implantations have been performed with the described technique in the Department of Otorhinolaryngology, Head and Neck surgery of the University of Cologne (tertiary referral center). Devices from different manufacturers have been implanted. Implantation age ranged from 6 months to 78 years (mean age: 24.50 years). Follow-up time ranged from 12 to 60 months. Neither implant dislocations nor migrations were observed in our patients. In four very young children (1.6 %), additional suture fixation of the implant was performed. Postoperative complications, i.e. seroma or hematoma were observed in a total of six cases (2.4 %). The here presented surgical fixation technique is a sufficient and reliable way for fixation of cochlear implants independent of the device type.

Mutations in MYO6 encoding an atypical myosin motor protein important for inner ear hair cell function have been associated with autosomal recessive (DFNB37) and autosomal dominant (DFNA22) types of hearing loss in a few families worldwide. After genome-wide linkage analysis, we identified a novel MYO6 mutation at the splice acceptor site of exon 7 (c.554-1G>A) in an extended German family with autosomal dominant postlingual non-syndromic hearing impairment. Analysis of blood-derived cDNA revealed different aberrantly spliced mRNAs caused by the mutation, which are predicted to severely interfere with protein function. Two of the family members underwent cochlear implantation at ages 53 and 65. Here, we present detailed clinical data of this family which suggest a favourable outcome of cochlear implantation in hearing-impaired individuals with a MYO6 mutation., (Copyright © 2013 S. Karger AG, Basel.)

Although recognized as a valuable diagnostic tool for more than 60 years, many laryngologists do not routinely use laryngeal electromyography (LEMG). This may be due to a persisting lack of agreement on methodology, interpretation, validity, and clinical application of LEMG. To achieve consensus in these fields, a laryngeal electromyography working group of European neurolaryngologic experts was formed in order to (1) evaluate guidelines for LEMG performance and (2) identify issues requiring further clarification. To obtain an overview of existing knowledge and research, English-language literature about LEMG was identified using Medline. Additionally, cited works not detected in the initial search were screened. Evidence-based recommendations for the performance and interpretation of LEMG and also for electrostimulation for functional evaluation were considered, as well as published reports based on expert opinion and single-institution retrospective case series. To assess the data obtained by this literature evaluation, the working group met five times and performed LEMG together on more than 20 patients. Subsequently, the results were presented and discussed at the 8th Congress of the European Laryngological Society in Vienna, Austria, September 1-4, 2010, and consensus was achieved in the following areas: (1) minimum requirements for the technical equipment required to perform and record LEMG; (2) best practical implementation of LEMG; (3) criteria for interpreting LEMG. Based on this consensus, prospective trials are planned to improve the quality of evidence guiding the proceedings of practitioners.

Auditory synaptopathy/neuropathy (AS/AN) is a special subtype of sensorineural hearing disorders with heterogeneous phenotypes and underestimated incidence. AS/AN generally develops in infancy, occasionally in adulthood. Symptoms include fluctuating, mostly bilateral hearing loss and abnormally reduced speech comprehension, especially in noisy environments. Within audiological assessments, patients with AS/AN present otoacoustic emissions (TEOAE; DPOAE) and cochlear microphonics (CM), absence of stapedius reflexes (SR) as well as absent or pathologically altered auditory evoked brainstem potentials (ABR). Children with AS/AN cannot be identified within OAE-based newborn hearing screening programs. Clinical findings, transtympanic electrocochleography (ECoG) and further diagnostic tools permit further identification of individual characteristics. In individual cases conventional amplification and the use of FM systems may improve hearing and communication skills. If these interventions, accompanied by intensive hearing, speech and language therapy are unsuccessful, cochlear implants (CI) or alternative forms of communication may be useful options for rehabilitation.

Objective: The German version of the Functioning after Paediatric Cochlear Implantation (FAPCI) inventory was designed to capture the communicative performance of cochlear implanted children. In order to be able to compare cochlear-implanted children to their normal hearing peers, normative growth curves were designed. Furthermore it was of interest how the communicative performance develops over time and whether it is influenced by age at implantation., Method: A polynomial regression curve was fit to the data of 133 normal hearing children. This normative curve was compared to individual growth curves of 90 cochlear implanted children. The cochlear-implanted study sample was split up into four groups depending on the age at implantation., Results: The normative growth curve increases from 1 year of age until saturation is reached with 3 years of age. The individual FAPCI trajectories of cochlear implanted children are heterogeneous, but in general they are delayed in comparison to the normative growth curve. "Early implanted children" follow the development of their normal hearing peers more closely than "later-implanted children"., Conclusion: The German version of the FAPCI parental questionnaire constitutes an additional instrument in monitoring the communicative performance development of cochlear implanted children and allows for comparison to normal hearing peers., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Conclusion: The approach to the cochlea did not influence postural control after cochlear implantation (CI) surgery. Most patients already have impaired vestibular function before surgery. These balance deficits did not change after CI surgery in the majority of patients but static balance might be improved by CI surgery., Objectives: To determine whether the technique used to insert the CI electrode has an influence on vestibular function., Methods: Static and dynamic postural control and caloric vestibular function were measured prospectively before and after CI surgery in 36 CI patients with standard cochleostomy (SC) insertion and 16 patients with insertion across the round window (RW). The test battery consisted of the modified clinical test of sensory interaction on balance (mCTSIB), the Rhythmic Weight Shift (RWS), the Walk Across (WA), and the Tandem Walk (TW) test., Results: Caloric testing was normal in the majority of CI candidates and remained normal after surgery. mCTSIB results were not significantly different before and after CI surgery between the SC and RW groups. Within both subgroups, the mCTSIB composite value improved within 6 weeks after surgery compared with the values obtained before surgery. Dynamic standing balance showed no postoperative change in RWS.

On a Balance Master computerized force plate static and dynamic postural control was measured in 24 severe hearing-impaired patients before and after CI surgery, and 19 healthy adults in a prospective study. The test battery consisted of the modified clinical test of sensory interaction on balance, the Rhythmic Weight Shift, the Walk Across, and the Tandem Walk test. Postural control was significantly worse in CI candidates than in healthy adults. In contrast, caloric testing of the vestibular organ was normal in the majority of CI candidates and remained normal after CI surgery. Static and dynamic standing balance parameters remained unchanged after CI surgery for the vast majority of parameters. Male gender and age >51 years were negative risk factors in some balance tasks. The static and dynamic standing balance performance seemed to improve to some degree with longer time interval before the CI surgery. Postural control was impaired but not complained of in many CI candidates before CI surgery and remained impaired after CI surgery although caloric testing is normal in most patients.

The management of patients characterised by the presence of otoacoustic emissions and/or cochlear microphonics suggesting normal outer hair cell function in conjunction with absent or grossly abnormal auditory brainstem responses is often associated with particularly poor response to amplification. Cochlear implantation has been shown to be an option in affected patients. Here, we report a case of successful cochlear implantation and preserved otoacoustic emissions in a child suffering from this hearing disorder.

Background: The Functioning After Pediatric Cochlear Implantation (FAPCI) instrument was recently developed to determine the communicative performance of 2-5-year-old prelingually deafened, cochlear-implanted children. Because of its high reliability and validity, as well as possible additional information compared with existing questionnaires, the 23-item parent-proxy questionnaire was translated from U.S. English to German prior to validation., Material and Methods: Initially, the German inventory was qualitatively developed by experts in audiology and speech pathology in collaboration with a professional American translator. Based on a sample of parents' responses, the outcome was quantitatively validated using psychometric methods (Cronbach's alpha, principal components analysis). Finally, the nomological validity was verified by correlating the overall FAPCI value with an external criterion (i.e., hearing age)., Results: For almost all age groups, Cronbach's alpha exceeded the minimum value of the original study (0.86). Principal components analysis revealed a two-factor solution (speech perception/production). The fitting of a nonparametric regression line to the data points showed that the total FAPCI score was positively associated with the time of implant use., Conclusion: The results show concordance between the German and the English versions of the FAPCI. The two instruments agree in reliability as well as in validity. The suitability of the German version in the clinical and therapeutic routine needs to be confirmed in further investigations.

We have identified a consanguineous family from Morocco segregating autosomal recessive congenital progressive hearing loss (ARNSHL) and retinal degeneration. Detailed clinical investigation of the six siblings revealed combined severe cone-rod dystrophy (CORD) and severe/profound hearing impairment in two of them, while there is isolated CORD in three and nonsyndromic profound hearing loss in one. We therefore assumed a partial overlap of two nonsyndromic autosomal recessive conditions instead of a monogenic syndrome and performed genomewide linkage analysis. The disease loci were mapped to chromosome 2q31.1-2q32.1 for ARNSHL and to 2q13-2q14.1 for CORD, respectively. The retinal phenotype was shown to be due to homozygosity for a novel splice site mutation, c.2189+1G>T, in the retinitis pigmentosa gene MERTK. The ARNSHL interval comprised the DFNB59 locus. The DFNB59 gene has been identified recently, and two missense mutations (p.R183W and p.T54I) have been shown to cause auditory neuropathy in both humans and transgenic mice. Mutation screening in the DFNB59 gene in our family revealed homozygosity for a 1-bp insertion in exon 2 (c.113&#95;114insT), predicting a truncated protein of 47 amino acids, in all three hearing impaired subjects. This is the first description of biallelic putative loss-of-function of the DFNB59 gene. Detailed audiological investigation clearly indicated hair cell dysfunction and, in contrast to cases reported previously, excluded auditory neuropathy. We show that besides otoferlin (OTOF), DFNB59 is the second known gene in which mutations can result in these two distinct forms of hearing impairment. Moreover, all patients in our family with homozygosity for the DFNB59 mutation display central vestibular dysfunction., ((c) 2007 Wiley-Liss, Inc.)

Background and Objective: Prosody has a myriad of linguistic functions and involves specific aspects of speech, such as stress, intonation and pauses. The underlying acoustic quantities (amplitude envelope, pitch frequency, and temporal structure) can be processed and transmitted by cochlear implants (CI) only to a limited extent. At present, no adequate tests are available in the German-speaking world for evaluation of the perception of prosodic elements. Different experiments have been conducted to address several prosodic cues, and the results are to be used as a basis for appropriate tests., Methods: Various prosodic materials were used for the experiments. Discrimination was measured for minimal pairs differing in frequency and/or duration, accents in words and phrases, questions versus statements and phrasing. Measurements were performed in ten normal-hearing subjects and five with cochlear implants., Results and Conclusions: In all test modules, the subjects with normal hearing proved to have high discrimination rates of 96-100%. The test of word stresses was problematic because the results were influenced by different confounders. The other measurements did prove to be basically suitable for use in the subjects with implants. Early results revealed that the subjects with CI had few problems with prosodic cues based on the temporal structure, the outcome being similar to that of the subjects with normal hearing in these tests. In contrast, the performance of subjects with CI in perceiving prosodic cues based on amplitude variations and, especially, on alterations in pitch frequency was worse, even though some of them achieved very good results in these tests too. These preliminary tests can form the basis for development of a German-language prosody test battery with a limited number of subtests addressing different prosodic cues.

Aims: It was the aim of this study to describe risk factors in auditory neuropathy/auditory synaptopathy (AN/AS)., Methods: Between 1997 and 2005, we diagnosed 37 children with AN/AS. They underwent a critical chart review for risk factors and etiological coincidences in this idiosyncratic disorder., Results: Eighteen neonates had a history of prematurity and low birth weight. Hyperbilirubinaemia was present in 13 children. Three patients had evidence of infection during pregnancy, and AN/AS was associated with complex syndromal diseases in 2 cases. A congenital, familial pattern was seen in 2 siblings. Seven patients had idiopathic AN/AS., Conclusion: Rather than being a single etiological entity, AN/AS comprises a spectrum of risk factors and associated problems affecting the cochlea and the auditory pathway. This study shows that the majority of AN/AS in children is the result of perinatal problems and is not genetic in origin. Hyperbilirubinaemia is a common and etiologically significant finding in infants suffering from AN/AS. Thus, early hearing screening for AN/AS including transient evoked otoacoustic emissions and auditory brainstem response assessment among neonates with risk factors for AN/AS is crucial in order to better manage patients suffering from this disorder., (Copyright (c) 2007 S. Karger AG, Basel.)

Pathological auditory brainstem responses (lack of responses, elevated thresholds and perturbed waveforms) in combination with present otoacoustic emissions are typical audiometric findings in patients with a hearing impairment that particularly affects speech comprehension or complete deafness. This heterogenous group of disorders first described as "auditory neuropathy" includes dysfunction of peripheral synaptic coding of sound by inner hair cells (synaptopathy) and/or of the generation and propagation of action potentials in the auditory nerve (neuropathy). This joint statement provides prevailing background information as well as recommendations on diagnosis and treatment. The statement focuses on the handling in the german language area but also refers to current international statements.

Objective: To examine the prevalence of auditory neuropathy/synaptopathy (AN/AS) in a cohort of children with profound hearing loss., Methods: From 1997 until 2004, 5190 children, aged 1-15 years, whose hearing ability was uncertain or who had risk factors for hearing impairment were investigated with subjective and objective hearing tests. Three thousand four hundred and fifteen from these children were screened for AN/AS using pure-tone audiometry, impedance measurement, transient evoked otoacoustic emissions (TEOAE) and click-evoked auditory brainstem responses (ABR)., Results: From 3415 patients who participated in an ABR and TEOAE assessment, 379 children showed absent or elevated (> or = 80 dB nHL) ABR thresholds. Within this group we found 32 cases with evidence of AN/AS via visible TEOAE and/or cochlear microphonics (CM) coupled with absent ABR. In the remaining 3036 children, AN/AS, could be ruled out by means of detectable ABR-thresholds and coherent findings in pure-tone audiometry and TEOAE assessment. This results in a prevalence of AN/AS of 0.94% within the group at risk for hearing loss, compared to 8.44% among profoundly hearing impaired children., Conclusion: This study shows that AN/AS is a common finding in the population of hearing impaired infants. In the majority of our AN/AS children (50%, n=16), an early audiological diagnosis was made under the age of 12 months. Therefore, clinicians and other health care professionals should generally be sensitised for AN/AS in infants, so that an appropriate treatment can promptly be initiated. Further research on clinical and pathophysiological aspects is necessary to better identify and manage patients suffering from AN/AS.

Nasal continuous positive airway pressure (nCPAP) in preterm infants is closely linked to improvements in the primary management of respiratory failure. We report on a severe complication involving the external ear, which is usually covered by the fixing straps of the nCPAP application system. The very low birthweight infant (233/7 weeks' gestation) was treated with nCPAP for more than 2 months. At the age of 51 days, the child developed a fluctuating seroma of the right external ear. Applied surgical treatments including punctation and compression of the ear resulted in full recovery after 3 months. Due to shearing forces associated with straps used for attaching the nasal application system, the infant developed a severe auricular trauma. Ear trauma can be minimised by careful padding of these straps. Continuous monitoring of the nCPAP-system, including the straps, is required.

Investigating a large German pedigree with non-syndromic hearing impairment of early onset and autosomal dominant mode of inheritance, linkage to known DFNA loci was excluded and in a subsequent genomic scan the phenotype was mapped to a 10-cM interval on chromosome 3q22; a maximum two-point lod score of 3.77 was obtained for the marker D3S1292. The new locus, DFNA18, is excluded from neighbouring deafness loci, DFNB15 and USH3, and it overlaps with the recently described DM2/PROMM locus. As hearing loss has been described as one feature of the PROMM phenotype, the DFNA18 gene might also be responsible for hearing loss in DM2/PROMM.

Thyroglossal duct cysts are common primary neck tumors, resulting from remnants of the ductus thyroglossus. They can occur at any point along the migratory path of the thyroid gland anlage until the 2nd or 3rd decade of life. The usual symptoms leading to the diagnosis are painless midline neck masses orfistulas. Despite their close proximity, the cysts normally do not affect the larynx. We report on the eighth case mentioned in the world literature of a thyroglossal duct cyst invading the larynx in a 62-year-old patient. Preoperatively, the voice of the patient sounded metallic, and the frequency of the voice field was reduced. Postoperatively, the voice onset was physiological, and the voice increased by an octave in the higher frequencies. The intonation of the voice was steady. The analysis of formants of the vowels "a" and "i" showed that the fourth formant of the vowel "i" was lower in frequency postoperatively.

Actinomycosis is currently an uncommonly diagnosed human disease. However, it can still complicate trauma to the respiratory and digestive tracts, including operative procedures. A patient with cervicofacial actinomycosis commonly gives a history of recent dental manipulation which usually, involves extraction of a mandibular molar. The common initial symptoms of infection--such as, sudden onset of cervicofacial pain, swelling, erythema, edema and suppuration--can be absent. Infection due to actinomyces is a well-known mimic of malignancy is clinical, radiological and pathological findings. In the case described a 65-year-old man had a mass in his right parotid area. Because of its painless rapid growth with infiltration of the mandible as demonstrated by clinical findings and CT and MRI scans, we established an initial diagnosis of salivary gland malignancy. Salivary gland biopsy then revealed a histological picture of infection due to actinomyces. Therapy was initiated with intravenous amoxicillin and sulbactam. After 20 days of treatment diseased soft tissues and bone were resected. To prevent a relapse the patient received oral clindamycin for 6 weeks. After a followup of two years, the patient has remained free of disease.