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Idiopathic sudden sensorineural hearing loss (ISSNHL) is one of the most common diseases in otolaryngology. Its etiology remains unknown. Furthermore, there is only a low level of evidence for the efficacy of established treatment modalities. In addition to systemic glucocorticoids, intratympanic corticosteroid treatment (ICT) has become increasingly important for treatment of ISSNHL. Different application strategies and treatment regimens have been described; however, uniform standards do not yet exist. ICT may be used for primary treatment as well as salvage therapy. Current data from meta-analyses show no benefit of intratympanic versus systemic primary therapy for sudden hearing loss (moderate evidence) but suggest a benefit of intratympanic secondary treatment over no treatment or placebo (high effect size, low evidence). Regarding combination of systemic and local glucocorticoid therapy in primary treatment of hearing loss, there may be a small benefit over systemic treatment alone (low effect size, low evidence)., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

A 41-year-old female patient presented due to acute onset of unilateral hearing loss 3 months previously and persistent since then. Systemic therapy with oral glucocorticoids in decreasing doses had been performed beforehand, but did not lead to any improvement. In the course of audiological diagnostics, based on subjective and objective methods, a retrocochlear hearing disorder was suspected. A meningioma was diagnosed by diagnostic imaging. Subsequent surgical removal achieved a significant hearing improvement., (© 2023. The Author(s).)

Cogan I syndrome is a rare disease consisting of vestibulocochlear symptoms and non-syphilitic interstitial keratitis. Although this disease was first described in 1945, its pathogenesis is still unknown. An autoimmune vasculitis etiology is currently discussed. Atypical manifestations are characterized by delayed ocular symptoms or variability of inflammatory eye symptoms. Physical examination often reveals bilateral sensorineural hearing loss. Intratympanic corticosteroid application can be successful., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Diagnostics of hearing loss comprises subjective and objective methods and is successfully applied since many decades. This article introduces into the methods of impedance audiometry, otoacoustic emissions, auditory evoked potentials and electrically evoked potentials and describes the respective methodologies. Using an exemplary case, in this article we guide through all stages of objective audiological diagnostics and introduce the scientific and technical background, the application and evaluation of the findings of the objective test procedures. An application pathway for objective audiometric diagnostic tools is also described, including newborn hearing screening, differential diagnostics of hearing loss, auditory neuropathy, sudden sensorineural hearing loss, vestibular schwannoma and pediatrics patients. Finally, the application to patients with active middle-ear implants and cochlear implants is described., Competing Interests: Erklärung zu finanziellen Interessen Forschungsförderung erhalten: ja, von einer anderen Institution (Pharma- oder Medizintechnikfirma usw.); Honorar/geldwerten Vorteil für Referententätigkeit erhalten: ja, von einer anderen Institution (Pharma- oder Medizintechnikfirma usw.); Bezahlter Berater/interner Schulungsreferent/Gehaltsempfänger: ja, von einer anderen Institution (Pharma- oder Medizintechnikfirma usw.); Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an im Bereich der Medizin aktiven Firma: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an zu Sponsoren dieser Fortbildung bzw. durch die Fortbildung in ihren Geschäftsinteressen berührten Firma: nein Erklärung zu nichtfinanziellen Interessen Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Patients with otosclerosis can suffer from different grades of combined hearing loss. In addition to surgery (stapedectomy), conventional hearing aids can be used in the treatment of otosclerosis. In cases of severe conductive components in addition to sensorineural hearing loss, treatment with normal hearing aids can be difficult or impossible. In these patients, implantable hearing systems such as cochlear implants represent a possible alternative. The totally implantable Carina middle ear system can be used in patients with even high-grade sensorineural hearing loss. Based on two exemplary cases, the option of using the Carina system in otosclerosis patients and post-implantation results are reported., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objectives: As a result of digitalization, the internet embodies the essential information medium. Especially, patients with sudden sensorineural hearing loss (SSNHL) require profound education due to unclear scientific evidence. Thus, our study investigated a German-language internet search about SSNHL., Design: The first 30 Google-search results with the term "Hörsturz" (SSNHL in German) were categorized, readability-statistic with different readability-scores (FRES: 0=complex, 100=easy; FKL; SMOG; GFI) calculated, and misinformation documented. A structured content-analysis was performed with the DISCERN-questionnaire (1=low, 5=high quality). Certification of the Health-On-The-Net-Foundation (HON) assessed the abidance of recommended standards., Results: 18 websites (60.0%) accounted for digital media, 7 (23.3%) manufacturers of medical devices, 2 (6.7%) government institutions, and respectively 1 (3.3%) healthcare provider, support-group, and scientific article. Mean word count was 1307.0±840.2, last update 17.1±32.5 months ago, and FRES 36.1±13.9, with the most difficult text by the scientific article (13.7). Mean of DISCERN was 2.2±0.7 with worst rating of manufacturers of medical devices (1.6±0.5). 2 websites (6.7%) were HON-certified, and 14 (46.7%) contained misinformation., Conclusion: Internet-based patient-information should be assessed cautiously due to poor readability, potential conflict of interests, low quality, or wrong information. Hence, healthcare providers and professional associations are urged to provide high-quality patient-information in the internet., Competing Interests: J. Spiegel erhielt eine Reisekostenerstattung von der Firma MED-EL, Innsbruck, Österreich, für einen Vortrag im Rahmen einer Konferenz im September 2019. Ansonsten bestehen keine weiteren Interessenkonflikte., (Thieme. All rights reserved.)

Due to development of molecular techniques at hand, the number of genomic sequence variants detected in patient investigations is rising constantly. The number of potentially involved genes in hereditary hearing loss is rising simultaneously.In this overview, current methods for diagnostic workup on a molecular and functional level for variants of the SLC26A4 gene are described. Based on the description of the physiological function of the resulting protein Pendrin, molecular investigations for interpretation of the function are explained. Based on these investigations, the potential clinical consequences of a variant may be predicted more precisely and simplify routine reporting of a proven genotype and a phenotype, at hand. Finally, subsequent clinical investigations necessary, such as perchlorate discharge test, as well as therapeutic options are discussed., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Background: Differential diagnosis of dizziness with hearing loss requires standardized procedures for detection and classification of rare congenital and acquired malformations of the petrous part of the temporal bone., Objective: The aim of this study was to present the physiology and pathophysiology of endolymphatic and perilymphatic pressure regulation, diagnostic guidelines, and aspects of prognosis and treatment., Materials and Methods: Relevant publications and guidelines were evaluated and own cases are reported., Results: Enlarged vestibular aqueduct (EVA) is the most frequently observed malformation of the inner ear, which leads to increased internal hydrostatic pressure and cochleovestibular dysfunction. Non-syndromic and syndromic forms, e.g., Pendred syndrome, are known. Other pressure-relevant malformations are semicircular canal dehiscence syndrome (SCDS) and enlargement of the cochlear aqueduct. There are currently no treatment options for EVAS and enlarged cochlear aqueduct. Pendred syndrome generally requires treatment with cochlea implants (CI) in early childhood. Dizziness and autophony in patients with SCDS syndrome can be effectively treated by semicircular canal occlusion and coverage., Conclusion: Complaints in non-syndromic EVA and SCD syndrome are mainly caused by exposure of the inner ear to provoked and spontaneous pressure increases. Deafness and vestibular dysfunction in syndromic EVA (i.e., Pendred syndrome, branchiootorenal syndrome) are caused by malformation of the cochlea, genetic maldevelopment of the hair cells, and pressure effects.

Competing Interests: Interessenkonflikt: Die Autoren geben an, dass kein Interessenkonflikt besteht.

Introduction: The preterm birth is clearly associated with increased risk of developing congenital hearing impairment. Therefore, special attention must be paid to the postnatal control of auditory function in all preterm infants. The present work investigates if the latest scientific findings regarding prevalence, clinical diagnostics, therapy and risk factors of hearing impairment in premature infants are regularly implemented in daily practice. Methods: At the department of phoniatrics and pediatric audiology of the University Hospital of Magdeburg, the treatment data of 126 preterm children born between 2006 and 2011 were evaluated retrospectively. The additional analysis of all records available at the screening center (n=67 640) covering this period enables drawing conclusions on the total number and prevalence of hearing impairment in preterm infants in Saxony-Anhalt. Results: Almost all premature babies, like mature newborns, underwent postnatal hearing screening of both ears. The data analysis shows that the practical implementation often does not comply with the guideline of the G-BA (Gemeinsamer Bundesausschuss) in all details. For example, the recommended screening method for preterm infants (AABR) or the screening and treatment timing are not always applied in accordance with the guidelines of the G-BA. Discussion: Assessment of the practical implementation of universal newborn hearing screening was planned at the time of the introduction of the hearing screening program by the G-BA. As a part of this investigation, the practical care of vulnerable groups such as preterm infants must be given special attention. Based on the collected data, the diagnostics and therapy should be unified. Regardless of the maternity clinic where the infants were born, there should be the same opportunity for early diagnosis and thus for prognostically better treatment of congenital hearing impairment. Rapid postnatal fitting with hearing aid can stimulate the maturation of the central auditory system and potentially help to avoid problems of hearing and speech development., Competing Interests: Interessenkonflikt:: Die Autoren geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

This study gives an estimation of the long term outcome of a screening procedure for cochlear implant candidacy in hearing aid users.In a recent retrospective study, hearing aid performance and audiometric measures in 185 subjects (318 ears) were analyzed. In the context of 2 years follow-up, the outcome of the ipsilateral screening procedure was analyzed. For patients who did receive a cochlear implant, their audiometric outcome measures were viewed into relation to their preoperative results.From the 96 ears identified as cochlear implant candidates, 34 were provided with a cochlear implant. 222 ears were identified as hearing aid users. Only 4 of these 222 ears were provided with a cochlear implant. Cochlear implant recipients with a preoperative speech recognition score with hearing aids above zero showed a mean improvement of 65% points in Freiburg monosyllabics.The audiometry-based screening procedure enables an effective management of the referral process of cochlear implant candidates., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: To date, the therapy of intralabyrinthine schwannoma consists mainly of a wait-and-see approach, completely ignoring auditory rehabilitation. Only a few single-case reports are as yet available on treatment with cochlear implants (CI)., Aim of the Study: This study aimed to assess the results of auditory rehabilitation after treatment with CI in a series of cases., Materials and Methods: The demographic findings, symptoms, and results of surgical therapy in 8 patients were evaluated in a retrospective analysis., Results: Prior to surgery, all patients presented with profound hearing loss and tinnitus. Episodic dizziness was reported by 3 patients. Among the patients, 4 had an intracochlear and 3 an intravestibular schwannoma, and a transmodiolar schwannoma was found in 1 patient. A total of 6 patients underwent treatment with CI. The results of auditory rehabilitation are favorable with open-set speech comprehension., Discussion: CI treatment following resection of an intralabyrinthine schwannoma is a promising option for auditory rehabilitation, even in single-sided deafness. This is a new treatment concept in contrast to the wait-and-scan policy. Expectant management appears justified only if the patient still has usable hearing.

Background: When setting the electrical stimulation level of cochlear implants during individual adjustment of the speech processor, especially in children, objective measures such as intracochlearly measured electrically evoked compound action potentials (eCAP) and intraoperative observation of electrically elicited stapedial reflexes (eSR) are indispensable. The benefit of these objective measures is based on the correlation between the derived response thresholds and psychometric data., Materials and Methods: The amplitude growth functions of eCAPs were measured intraoperatively for all electrodes in 30 ears of adult patients. The stimulus-dependent incidence of observable eSRs was recorded for all electrodes in 16 ears of adult patients. For evaluation of the data, new algorithms were applied which allowed the determination of thresholds without intervention of the investigator. Essential features were the conversion of observations into binary variables, and the consideration of logistic discrimination functions and their exceedance of a numeric threshold criterion., Results: Regarding the eCAP data, closer and significant correlations are observed between objective thresholds and psychometric measures in comparison to conventional procedures. Profiles are more efficient than pooled data. Significant correlations are also observed for eSR thresholds, albeit to a lesser extent and without an evident difference between profiles and pooled data., Conclusion: Considering the by no means consistent international literature, the results illustrate the need for a consistent definition of response thresholds and the consideration of electrode profiles.

Background: The aim of this study was to compare the clinical history and the findings in audiovestibular function tests in patients suffering from intralabyrinthine schwannoma or delayed endolymphatic hydrops (DEH)., Patients and Methods: Five patients diagnosed with intralabyrinthine schwannoma by magnetic resonance imaging (MRI) and five patients diagnosed with DEH by locally enhanced inner ear MRI (LEIM) were retrospectively studied., Results: All patients with intralabyrinthine schwannoma or DEH initially presented with hearing loss. Vertigo occurred in two patients with intralabyrinthine schwannoma and in all patients with DEH. While audiometry achieved poorer results for patients with intralabyrinthine schwannomas, vestibular function tests revealed normal results in about half of the patients in both groups., Conclusion: Patients with intralabyrinthine schwannomas may present with clinical symptoms similar to patients suffering from other inner ear disorders like delayed endolymphatic hydrops and may obtain similar findings in audiovestibular function tests. High-resolution MR imaging with locally applied contrast agent may provide evidence of both underlying pathologies.

Hearing loss is one of the most dominant handicaps in modern societies, which additionally very often is not realized or not admitted. About one quarter of the general population suffers from inner ear hearing loss and is therefore restricted in communicational skills. Demographic factors like increasing age play an important role as well as environmental influences and an increasing sound and noise exposure especially in leisure activities. Thus borders between a "classical" presbyacusis - if it ever existed - and envirionmentally induced hearing loss disappear. Today restrictions in hearing ability develop earlier in age but at the same time they are detected and diagnosed earlier. This paper can eventually enlighten the wide field of inner ear hearing loss only fragmentarily; therefore mainly new research, findings and developments are reviewed. The first part discusses new aspects of diagnostics of inner ear hearing loss and different etiologies., (© Georg Thieme Verlag KG Stuttgart · New York.)

Systemic steroids are widely used worldwide as a standard of care for primary therapy of idiopathic sudden sensorineural hearing loss (ISSHL). The German ISSHL guideline recommends high-dose steroids for primary therapy of ISSHL, without evidence from randomized controlled trials (RCTs). The rationale for the treatment of ISSHL using high dose steroids is only based on retrospective cohort studies.This article describes the planning and initiation of a multicenter, national, randomized, controlled clinical trial entitled Efficacy and safety of high dose glucocorticosteroid treatment for idiopathic sudden sensorineural hearing loss - a three-armed, randomized, triple-blind, multicenter trial (HODOKORT). This clinical trial aims to compare standard dose with two types of high-dose steroids for primary systemic therapy with respect to their efficacy in improving hearing, and thus communication ability, in patients with idiopathic sudden sensorineural hearing loss.This study is funded by the "Clinical Trials with High Patient Relevance" research program in the health research framework of the German Federal Ministry of Education and Research. It is one of two studies by the German Study Center of Clinical Trials of the German Society of Otorhinolaryngology, Head and Neck Surgery (DSZ-HNO). Planning and initiation was done in cooperation with the DSZ-HNO, the Coordination Center of Clinical Trials of the Martin-Luther-University Halle-Wittenberg, and the Study Center of the University Hospital Freiburg.

Background and Aim: Hearing loss is one of the most common disabilities in the elderly. The aim of this study was to investigate the relationship between pure-tone hearing loss and maximum monosyllabic perception and speech perception with hearing aids. The focus of the investigation was elderly patients., Materials and Methods: In this prospective study, 188 patients with sensorineural hearing loss were included. The pure-tone audiogram (4FPTA), the Freiburg speech intelligibility test with headphones and the word recognition score with hearing aids at 65 dB SPL were measured and evaluated., Results: An increasing age was associated with higher discrepancy between the maximum speech perception and speech understanding with hearing aids. The mean difference between maximum monosyllabic perception and speech perception with hearing aids is about 20% in the elderly population., Conclusion: The intended goal of hearing aid prescription, the match between maximum monosyllabic perception and word recognition score with hearing aids within 5 to 10%, is not achieved in the elderly population.

Introduction: Currently a sensitive early diagnosis of small vestibular schwannoma is only possible by using magnetic resonance imaging (MRI)., Objectives: The main objective was a differentiation of the cochlear and retrocochlear component of small vestibular schwannoma with the help of categorial loudness scaling (CLS) and the growth function of otoacoustic emissions (DPOAE I/O-functions)., Material and Methods: 34 patients (gr. 1: 17 patients with vestibular schwannoma ≤15 mm, gr. 2: 17 matched patients with an inner ear hearing disorder) were examined. Besides audiological standard procedures they also underwent CLS according to the Würzburger auditory field and a generation of DPOAE I/O-functions was conducted on the probands., Results: The gradients of the loudness growth function as part of the CLS and the DPOAE I/O-functions showed with few exceptions [500 Hz at 0-10 dB HL during CLS (p=0,040)] no significant differences between the groups (all p>0.05). A recruitment verification with the help of CLS was possible for tumors starting at a size of 5.45,mm at 3,000 and 4,000 Hz, respectively for tumors exceeding the size of 6.85 mm at 6,000 Hz with 100% sensitivity but only low specificity., Conclusions: A differentiation between a vestibular schwannoma and a mere cochlear hearing disorder with only the help of CLS and DPOAE I/O-functions is not possible. The results corroborate the thesis of an additional cochlear component even in small vestibular schwannoma. The implementation of CLS to determine cochlear deficits linked to vestibular schwannoma seems to be medically sensible if the tumor size exceeds 5 mm. According to the diagnostic method used to determine vestibular schwannoma MRI remains the first choice procedure., (© Georg Thieme Verlag KG Stuttgart · New York.)

Objective: Selecting subjects for clinical trials on hearing loss therapies relies on the patient meeting the audiological inclusion criteria. In studies on the treatment of idiopathic sudden sensorineural hearing loss, the patient's acute audiogram is usually compared with a previous audiogram, the audiogram of the non-affected ear, or a normal audiogram according to an ISO standard. Generally, many more patients are screened than actually fulfill the particular inclusion criteria. The inclusion criteria often require a calculation of pure-tone averages, selection of the most affected frequencies, and calculation of hearing loss differences., Materials and Methods: A software tool was developed to simplify and accelerate this inclusion procedure for investigators to estimate the possible recruitment rate during the planning phase of a clinical trial and during the actual study. This tool is Microsoft Excel-based and easy to modify to meet the particular inclusion criteria of a specific clinical trial. The tool was retrospectively evaluated on 100 patients with acute hearing loss comparing the times for classifying automatically and manually. The study sample comprised 100 patients with idiopathic sudden sensorineural hearing loss., Results and Conclusion: The age- and sex-related normative audiogram was calculated automatically by the tool and the hearing impairment was graded. The estimated recruitment rate of our sample was quickly calculated. Information about meeting the inclusion criteria was provided instantaneously. A significant reduction of 30% in the time required for classifying (30 s per patient) was observed.

In contrast to the fairly common exostoses in the external auditory canal, hyperostoses and osteomas of the internal auditory canal are extremely rare. In this case report we present a patient with sudden right-sided sensorineural hearing loss, in whom imaging revealed hyperostosis with bilateral stenosis of the internal auditory canal. Whether the connection of such radiological findings with dysfunction of cranial nerves VII and VIII be causal or coincidental is controversially discussed in the literature. Therefore, the indication for surgical intervention should be considered with extreme caution. Despite examination of our radiology database comprising almost 1000 MRI/CT temporal bone investigations, we could find no other cases of hyperostosis of the petrous bone. This case thus represents a rare disease, which should be considered a differential diagnosis.

Background: Classification of diseases on the molecular level is the basis for personalized medicine. Personalized medicine proposes to improve efficiency and quality of care, to reduce side effects and to increase long-term cost-effectiveness., Objectives: This paper is concerned with the role of genetic diagnostics in patients with a cochlear implant., Material and Methods: A selective literature search in PubMed was performed., Results: Genetic diagnosis allows ruling out syndromic hearing loss and thus prevents follow-up studies. It allows genetic counseling, prognosis and advice on family planning and targeted prevention. Due to its minimal invasiveness, it is suitable for evaluation of factors accounting for hearing loss in children., Conclusions: Molecular medicine plays a major role in the treatment of sensorineural hearings loss with cochlear implants.

The indications for local drug therapy of inner ear hearing loss include sudden sensorineural hearing loss, Menière's disease, autoimmune-associated hearing loss, ototoxicity as a side effect of other therapies, acute acoustic trauma and improvement of the safety and performance of cochlear implants. Various drugs are currently being used and tested for local treatment of inner ear hearing loss, including glucocorticoids, growth factors, apoptosis inhibitors, antioxidants, TNF-α inhibitors and antibodies. To further a better understanding of pharmacokinetics and the development of rational pharmacotherapy of the inner ear, the"liberation, absorption, distribution, metabolism, elimination" (LADME) principle can be applied to local therapy of the inner ear. Local application strategies can be differentiated into intratympanic applications to the middle ear cavity and direct intralabyrinthine or intracochlear applications.

Background: Alongside improvements in hearing and communication skills, the rehabilitation of children, adolescents and adults with a cochlear implant (CI) in recent years has increasingly taken into account mental health and quality of life issues. In the context of the programs offered, this study assesses the significance of dance for the mental health of adult clients with a CI., Methods: Eleven adult CI users participated in a dance project, which took place as a cooperation between the ENT University Hospital Heidelberg and the Baden State Theatre Karlsruhe. Participants were questioned at two different time points for assessment with the mental health scales (SPG). These scales measure seven different aspects of psychosocial well-being (including autonomy, willpower, affirmation of life and meaningfulness)., Results: Significant positive changes in the domains of affirmation of life, self-reflection and social integration were revealed by before and after comparisons; tendencies toward positive change were observed (p ≤ 0.10) in the domains of willpower, naturalness and meaningfulness. No changes were observed in the autonomy domain., Conclusion: The results indicate that the mental health of adult clients with a CI can be strengthened by dance as a complementary rehabilitation module. Concepts of CI rehabilitation should increasingly find anchor in the consideration of such arrangements for its range of offers.

Background and Aim: Speech perception is the most important social task of the auditory system. Consequently, speech audiometry is essential to evaluate hearing aid benefit. The aim of the study was to describe the correlation between pure-tone hearing loss and speech perception. In particular, pure-tone audiogram, speech audiogram, and speech perception with hearing aids were compared., Materials and Methods: In a retrospective study, 102 hearing aid users with bilateral sensorineural hearing loss were included. Pure-tone loss (PTA) was correlated to monosyllabic perception at 65 dB with hearing aid and with maximum monosyllabic perception with headphones., Results: Speech perception as a function of hearing loss can be represented by a sigmoid function. However, for higher degrees of hearing loss, substantial deviations are observed. Maximum monosyllabic perception with headphones is usually not achieved with hearing aids at standard speech levels of 65 dB., Conclusion: For larger groups, average pure-tone hearing loss and speech perception correlate significantly. However, prognosis for individuals is not possible. In particular for higher degrees of hearing loss substantial deviations could be observed. Speech performance with hearing aids cannot be predicted sufficiently from speech audiograms. Above the age of 80, speech perception is significantly worse.

Objective: Hearing impairment is the most common sensorineural disease in humans. About 1-3 per 1 000 neonates suffers at birth or in the first years from high-grade to severe hearing impairment. About half of the cases are due to genetic alterations. Most commonly, the GJB2 gene (connexin-26) is concerned with the mutation c.35delG. MATERIAL AND METHODES: All patients showed a severe to profound hearing impairment to the course. DNA isolation, amplification and sequencing was performed using standard techniques., Results: In the studied patient population we have 142 pa-tients with a homozygous deletion mutation in GJB2 gene (c.35delG) and 29 patients who are heterozygous for this mutation on one allele and heterozygous for another loss-of-function mutation in GJB2 gene. Of these 171 patients were 16 (9.3%) on an inconspicuous newborn hearing screening using Otoacoustic Emissions (OAE). Total was observed a progression of hearing impairment in 31 of these patients (18.1%)., Conclusions: This fact suggests that homozygous deletion mutation c.35delG does not always contribute to an congenital hearing impairment, but to a progressive hearing loss that might develop over the first months and years of life. Additionally, we have to re-evaluate the value of OAE for newborn hearing screening, keeping in mind that one positive result is no warranty for a normal development of hearing function, but a result that should be checked in the course. We recommend annual hearing tests to the paediatrician and with a known familial hearing loss and other risk factors pedaudiological controls., (© Georg Thieme Verlag KG Stuttgart · New York.)

The combination of sensorineural hearing loss and keratoderma on the hands and feet is rare. We report the case of a child that failed newborn hearing screening and also showed keratoderma on both hands and feet. The child's father exhibited the same constellation of symptoms, which is typical for mutilating keratoderma with deafness (Vohwinkel syndrome). This hereditary autosomal dominant disease is caused by mutation of the GJB2 gene that encodes the protein connexin 26. In our case it was highly likely that the GJB2 gene in the father carried a spontaneous mutation that was inherited by the daughter.

Background: The pathogenesis of idiopathic sudden sensorineural hearing loss (ISSHL) still remains unclear. This retrospective study was performed to evaluate the effectiveness of tympanotomy and sealing of the round window membrane after unilateral idiopathic sudden sensorineural hearing loss., Methods: A total of 74 patients with idiopathic sudden sensorineural hearing loss were treated with antiphlogistic-rheologic infusion therapy according to Stennert (steroids and pentoxyphylline). In addition, all patients underwent exploratory tympanotomy and sealing of the round window membrane. Pure tone audiometry was performed pre- and postinterventionally., Results: The average hearing loss (four pure tone average) of all patients was 58.9 dB pre-, and 46 dB postinterventionally, which is an average improvement of 12.9 dB. Patients with hearing loss of more than 60% improved significantly compared to patients with hearing loss less than 60% (33.9% vs. +3.3%). Sealing of the round window membrane was found to be more effective when performed within 8 days after ISSHL. A membrane rupture did not lead to better therapy results. No significant correlation was found between therapy outcome and other clinical symptoms., Conclusion: Sealing of the window membrane shows equal results to conservative methods. If patients with hearing loss of more than 60% have more benefit in tympanotomy with sealing of the window membrane than patients with less hearing loss--as shown as in this study--has to be proved in randomized examinations. Intraoperatively found ruptures of the round window membrane had no significant effect on the therapy outcome.

We report on a rare case of an exon 16 mutation of the MYH9 gene in a 23-year-old woman. This gene encodes for non-muscular myosin IIA, which acts as a cytoskeletal contractile protein in diverse cell types. This disorder led to sensorineural hearing loss, macrothrombocytopenia, and proteinuria. MYH9 gene mutation can lead to diverse organ manifestation like pre-senile cataract or renal failure which are progressive in course. Due to the current lack of causal treatment, diagnostic steps, advice for follow-up examinations and symptomatic therapy approaches are presented.

Background: Therapy for idiopathic sudden sensorineural hearing loss is still controversial. Although there are no evidenced-based studies, therapy with systemic steroids is widely accepted as the gold standard. Intratympanic administration of steroids appears to be an alternative or additional method of management without the disadvantage of systemic side effects and, therefore, makes therapy accessible for patients with contraindication for systemic steroids., Material and Methods: This retrospective analysis compares the audiometric results of 25 patients who were treated with standard therapy (prednisolone, hydroxyethyl starch, pentoxyfylline) with 23 patients who additionally received intratympanic steroids (IT group). A total of 4 injections were administered within 10 days. The solution used consisted of 0.3 ml dexamethasone (8 mg/ml) and 0.2 ml hyaluronic acid 0.2%. The pure-tone average (PTA) was evaluated prior to and 3 months after treatment., Results: The PTA 3 months after treatment showed an improvement of 48 dB in the IT group and 38 dB in the standard treatment group. The IT group achieved better recovery with an average PTA improvement of 68% compared to the standard treatment group with an average improvement of 59%. Neither result reached significance., Conclusion: Combination therapy with intratympanic steroids showed a tendency for better hearing results without serious side effects. However, because current evidence is not adequate, randomized placebo-controlled multicenter studies are needed.

Background: A hearing screening among 14- to 15-year-old pupils was performed to estimate the number of hearing-impaired individuals in the adolescent population., Materials and Methods: A total of 1,298 pupils from 30 schools in Tyrol (Austria) participated in the screening. Hearing tests were performed in a silent room at the school. Sinus tones at frequencies 0.5/1/2/4/6 kHz and at levels of 25/20/20/20/20 dB HL, respectively, were delivered via headphones to either ear. Failure of screening was defined as not hearing one or more frequencies in one or both ears., Results: The screening was failed by 16.3% of the pupils. There was a small but not significant difference between males and females (17.0 vs. 15.2%). Most of the pupils failed at only one frequency (9.6%). Failing at two or more frequencies in the same ear occurred in 3.9% of the pupils, thereof in 1.1% bilaterally., Conclusion: As the specificity of our screening is limited, false-positive results may result. Thus, the rate of hearing deficits in our sample is probably a bit lower than indicated by the figures above. Most of the adolescents who failed the screen failed at only one frequency. These subjects have a small elevation of their hearing threshold, not a hearing loss in the sense of a raised averaged threshold. A hearing loss in the latter sense is supposed to be present in only very few percent of adolescents, a bilateral hearing loss in perhaps less than 1%.

Background: Previous studies comparing the Freiburg speech tests (FST) currently used in Germany to assess the severity of hearing loss with two modern speech intelligibility tests [the Göttingen sentence test (GöSa) and the monosyllabic rhyme test devised by von Wallenberg and Kollmeier (WaKo)] have indicated that a replacement of the old procedure would be possible. The current study investigates the consequences of the modern test procedures for the estimation of reduction in earning capacity resulting from hearing loss, and considers the optimal presentation levels for the WaKo test., Methods: The FST, GöSa, and WaKo speech intelligibility tests were performed on 29 volunteers with a hearing impairment. All tests were conducted in silence. The presentation levels for the Freiburg monosyllabic test were 60, 80, and for some participants also 100 dB SPL. The WaKo test was carried out once at 45, 65, and 85 dB SPL (a reduction of 15 dB relative to the FST) and again at 40, 60 and 80 dB SPL (reduction of 20 dB relative to the FST)., Results: A consideration across all presentation levels revealed that the best correlation match between the two monosyllabic tests was achieved at a 20-dB reduction in presentation level for the WaKo test relative to the FST. On average, the application of modern procedures and the different options for level reduction had only a minor effect on the quantitative assessment of reduction in earning capacity.

Background: In addition to the standardized Freiburg sentence test in silence validated speech tests in noise are available. They are mainly used to test the benefit of hearing systems in everyday-life situations. In diagnostics tests in noise can also give additional information of the patients' hearing impairment. The determined speech reception threshold (SRT) depends on the degree of hearing loss and the used speech and noise test material. Aim of this study was to analyze the results of the Göttingen sentence test in noise in relation to different audiogram classes., Material and Methods: The Göttingen sentence test in stationary noise (65 dB) was performed for 135 patients with different degrees of hearing losses. Based on the air-conducted threshold all ears were categorized to an audiogram class by an automated procedure., Results: For the mild hearing losses the results of the Göttingen sentence test increased with increasing pure-tone-average (PTA) and the values are smaller than 0 dB SNR with a small standard deviation (± 1-2 dB SNR). For the moderate hearing losses values of 5 dB SNR with a standard deviation of 6 dB SNR were achieved on average. Patients with a high hearing loss achieved values higher than 20 dB SNR., Conclusion: For the mild hearing losses the results were located in a small range. However, they can give important information about the patient's hearing impairment mainly appears in a noisy environment. With increasing hearing loss also the reduced hearing in silence affects the results achieved with the Göttingen sentence test in noise. Therefore the hearing in silence has to be taken into account at the interpretation of the results., (© Georg Thieme Verlag KG Stuttgart · New York.)

A 68-year-old woman presented with unilateral herpetiform skin lesions of the face. She also showed reduced eye lid and mimic function as well as hearing loss. In addition grouped vesicles were seen in the outer ear canal. We diagnosed Ramsay Hunt syndrome and administered intravenous acyclovir, prednisolone and pentoxifylline. Within a few weeks, there was complete remission including the neurological symptoms.

Background: Brainstem electric response audiometries (BERA) are in clinical use for a number of years. The aim of our study was to evaluate data regarding the long-term reliability of BERA-determined frequency specific thresholds in hearing disabled children., Material and Methods: In a group of 97 hearing disabled children we sought to compare Notched-Noise- (NN) BERA threshold as well as Click-BERA thresholds taken shortly after birth with behavioral audiometry thresholds determined after 3.2 years (mean)., Results: We found a significant correlation between both BERA methods and the behavioral tests. However, the correlation coefficients for NN-BERA were higher than for Click-BERA thresholds., Conclusion: Our results provide evidence for a high reliability of the NN-BERA for characterization of early onset hearing disabilities in children. Our data suggest that pathologic findings in the Click-BERA should always be followed by a frequency specific analysis with NN-BERA., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: In order to obtain a low-frequency specific auditory brainstem response (ABR) there are less stimuli that plege a satisfying accordance with the ABR threshold and the behavioural threshold. This study investigates the so called low-chirp-ABR as to predict a low-frequency-amblyacousia and compares it to the notched-noise-500 Hz-ABR., Material and Methods: We evaluated behavioural and ABR thresholds to a low-chirp respectively a 500 Hz tone presented in notched noise masking from 32 patients with a severe hearing loss at low-frequencies and 28 patients with normal hearing., Results: The average difference ± single standard deviation between low-chirp-ABR and behavioural threshold is 1.53 dB ± 7.68 dB in the group of patient with low-frequency hearing loss and 2.55 dB ± 5.46 dB in the group of normal hearing. On the contrary the difference between notched-noise-500 Hz- and behavioural thereshold averages 2.67 dB ± 9.29 dB (low-frequency hearing loss) respectively 8.82 dB ± 7.90 dB (normal hearing). In order to verify the equivalence between the several methods we did a equivalence test that shows that low-chip-ABR and behavioural threshold can be considered as equal to obtain a low-frequency threshold. In contrast the comparison of notched-noise-500 Hz-ABR and behavioural threshold proves no equivalence., Conclusion: We detected a significant equivalence between low-chirp-ABR and pure-tone audiometric. The ABR to a low-chirp stimulus seems to be rather precise in contrast to the notched-noise-500 Hz-evoked-ABR in order to diagnose a low-frequency-hearing loss., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Intraoperative monitoring (IOM) of the cochlear nerve function during acoustic neuroma surgery is employed to assist in preserving hearing. So far, Auditory Brain-stem Response (ABR) is considered to be an optimal method for intraoperative monitoring in transtemporal approach. The aim of this study was to perform direct recording of the cochlear nerve action potential after resection of the tumor by using a ball-electrode and to evaluate the use of this method in predicting the postoperative hearing. The obtained data were compared to the simultaneous ABR results and to the postoperative hearing., Material and Methods: In 2009, 38 patients have undergone acoustic Neuroma Surgery at the ENT University hospital, Wuerzburg. In 33 patients an intraoperative ABR as well as a direct measurement from the cochlear nerve using a ball electrode were performed. In 5 patients the postoperative hearing was predicted using the direct measurement at the cochlear nerve only., Results: The direct recording from the cochlear nerve gave very robust responses. Even in cases where ABR recording was not possible, the identification of clear cochlear nerve action potential could still be reached. Using the direct recordings from the cochlear nerve to predict the post operative hearing turned out to have a sensitivity of 100% and a specificity of 70%., Conclusions: These results show that intraoperative monitoring with direct recording from the cochlear nerve via a ball-electrode in transtemporal approaches offers a valuable method to predict the postoperative hearing. Further investigation will be made to provide additional information., (© Georg Thieme Verlag KG Stuttgart · New York.)

The current guidelines for hearing aid supply in Germany employ the often criticised Freiburg monosyllabic speech test (FBE) in quiet. This test can be replaced with the monosyllabic rhyme test by von Wallenberg and Kollmeier (WaKo) in quiet and by the measurement of a speech reception threshold in an interfering noise at a moderate level (45 dB SPL) using either the Göttingen or the Oldenburg sentence test (criterion: 2 dB improvement in SNR, "signal-to-noise ratio"). This procedure was investigated in a group of 38 participants with a sensorineural hearing impairment (mild, moderate or severe hearing loss) and 11 volunteers with normal hearing. On average, comparable indications were achieved. Participants with a mild hearing loss and a selective problem with listening in interfering noise were assessed more fairly.

Susac syndrome, named after John Susac, the first to describe this condition, is characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Although certainly a rare disease, Susac syndrome needs to be considered in the differential diagnosis of a broad variety of diseases. The pathogenesis is not yet clear. Autoimmune processes leading to damage and inflammation-related occlusion of the microvessels in brain, retina, and inner ear are thought to play a causal role. The diagnosis is based primarily on the clinical presentation, the documentation of branch retinal artery occlusion by fluorescence angiography, and characteristic findings on cerebral MRI. Usually, immunosuppressive therapy is required, though controlled therapy trials are missing so far. The intention of this review article is to raise awareness of this disease among neurologists, psychiatrists, ophthalmologists, and ENT specialists as a high number of unreported cases probably exists. Accordingly, the focus is on the clinical presentation and the diagnostic approach.

Background: The present study analyzes the best combination of frequencies for the calculation of mean hearing loss in pure tone threshold audiometry for correlation with hearing loss for numbers in speech audiometry, since the literature describes different calculation variations for plausibility checking in expertise. Three calculation variations, A (250, 500 and 1000 Hz), B (500 and 1000 Hz) and C (500, 1000 and 2000 Hz), were compared., Methods: Audiograms in 80 patients with normal hearing, 106 patients with hearing loss and 135 expertise patients were analyzed in a retrospective manner. Differences between mean pure tone audiometry thresholds and hearing loss for numbers were calculated and statistically compared separately for the right and the left ear in the three patient collectives., Results: We found the calculation variation A to be the best combination of frequencies, since it yielded the smallest standard deviations while being statistically different to calculation variations B and C. The 1- and 2.58-fold standard deviation (representing 68.3% and 99.0% of all values) was ±4.6 and ±11.8 dB for calculation variation A in patients with hearing loss, respectively., Conclusions: For plausibility checking in expertise, the mean threshold from the frequencies 250, 500 and 1000 Hz should be compared to the hearing loss for numbers. The common recommendation reported by the literature to doubt plausibility when the difference of these values exceeds ±5 dB is too strict as shown by this study.

We present the case of a 12-year-old boy with right-sided pantonal sensorineural hearing loss. With the help of imaging diagnostics a tumour of the right temporal bone was detected. It was resected using a transmastoid approach. Histopathological study showed a low-grade adenocarcinoma of the endolymphatic sac, known as Heffner tumour. An association with the von-Hippel-Lindau complex - as often reported in the medical literature - could not be proven.

A dull head injury can lead to isolated damage of the inner ear (cochlear labyrinthine concussion) or damage of the otolithe organ (vestibular labyrinthine concussion) due to a bone conduction pressure . A typical sign is a high frequency SNHL in form of a c5-dip. The c5-dip can be bilateral or unilateral or different on each side - dependant on the side of injury. In case of a unilateral skull base fracture a contralateral labyrinthine concussion is also possible. Moreover a lot of cases also show an accompanying tinnitus. This knowledge is based on animal and human experiments, as well as data from clinical and medical report examinations over decades. It is important to differentiate between a SNHL caused by accident or uni- or bilateral endogenic degenerative hearing loss., (© Georg Thieme Verlag KG Stuttgart · New York.)