Sudden Deafness and Vestibulopathy in a Patient with Antibody Treatment for Metastatic Lung Cancer.

We describe the case of a patient who presented with sudden onset vertigo and bilateral deafness while under immune checkpoint therapy for metastatic bronchial carcinoma. Extensive audiologic assessment and vestibular function testing, as well as cranial magnetic resonance imaging (cMRI) and lumbar puncture was performed. The diagnostic workup confirmed bilateral sensorineural deafness and bilateral loss of vestibular function, while imaging revealed enhancement of the vestibulocochlear nerve. Initially, immunotherapy with PD-L1 antibody atezolizumab was the assumed cause of the described symptoms. However, further findings strengthened the suspicion of meningeosis neoplastica. The differential diagnoses of hearing loss and vestibulopathy in the context of platin-based chemotherapy, checkpoint inhibitor therapy and metastatic disease should be kept in mind for appropriate workup and therapy. Laryngoscope, 2024.

A Novel Diagnostic and Treatment Algorithm for Acute Mastoiditis in Children Based on 109 Cases.

BACKGROUND: Acute mastoiditis (AM) is a potentially life-threatening condition primarily affecting children. To date, there are no consistent criteria or valid guidelines for the diagnosis and treatment of pediatric AM. Therefore, this study evaluates the clinical course of AM in terms of clinical signs and treatment. In addition, a novel classification scheme for the disease and a treatment algorithm is being proposed. METHODS: Patient records over a 12-year period from a single center were reviewed to identify confirmed cases of AM in children. Data collected included clinical signs, body temperature, and infection parameters during the disease, as well as radiological imaging, antibiotics, and surgical as well as conservative treatment. In addition, a classification of the AM stages was established in accordance with the findings described and practical experience, consisting of four stages (1, mastoidal irritation; 2, mild AM; 3, advanced AM; 4, advanced AM and additional complications) with corresponding treatment recommendations. In the retrospective cohort, those AM cases that were treated alongside the classification were compared with the rest concerning clinical course and outcome. RESULTS: A total of 109 patients (mean age, 3.8 ± 3.8 years) were included. The main symptoms at hospital admission were auricular protrusion (n = 73; 67.0%), fever (n = 56; 51.4%) with a mean temperature of 38.3 ± 1.1°C, and otalgia (n = 28; 25.7%). The mean laboratory-tested levels of leukocytes and C-reactive protein at the time of hospital admission were 15.96 ± 8.7/nl and 59.6 ± 54.0 mg/L, respectively. During winter, there was a higher prevalence of AM, with peak hospital admissions in April (n = 22). The most common pathogen was Streptococcus pyogenes (32 cases). Treatment was purely conservative in four cases, whereas the remaining cases underwent surgery (41× grommet insertion, 64× plus mastoidectomy). The outcome was generally good, but in eight patients a second surgical procedure had to be performed as they showed signs of clinical deterioration. A total of 101 patients were treated according to the proposed algorithm, and all of which had a good outcome without the need for further interventions. CONCLUSION: Based on clinical experience in a large cohort of pediatric AM patients, a novel diagnostic and treatment algorithm has been developed and successfully tested in a retrospective cohort for AM in children to prevent further complications and to ease its management by pediatricians and otorhinolaryngologists in the emergency setting.

Sustained Cognitive Improvement in Patients over 65 Two Years after Cochlear Implantation.

This study aimed to evaluate the long-term benefits of cochlear implantation (CI) on cognitive performance, speech perception, and psychological status in post-lingually deafened patients older than 65 (n = 33). Patients were consecutively enrolled in this prospective study and assessed before, one year after, and two years after CI for speech perception, depressive symptoms, perceived stress, and working memory and processing speed. The Wechsler Adult Intelligence Scale (WAIS) was used for the latter. Thirty-three patients (fourteen men and nineteen women) were included. The scores indicating "hearing in quiet" and "hearing with background noise" improved significantly one year after CI and remained so two years after CI. The sound localization scores improved two years after CI. The depressive symptoms and perceived stress scores were low at the study's onset and remained unchanged. Working memory improved significantly two years after CI, while processing speed improved significantly one year after CI and was maintained after that. The improvement in working memory and processing speed two years after CI suggests there is a sustained positive effect of auditory rehabilitation with CI on cognitive abilities.

[Prospective comparative analysis of cochlear implant patients with single-sided deafness and asymmetric hearing loss with regard to health-related quality of life, tinnitus distress and psychological comorbidities]. / Prospektive Vergleichsanalyse von CI-Patienten mit einseitiger Taubheit und asymmetrischem Hörverlust hinsichtlich der gesundheitsbezogenen Lebensqualität, Tinnitusbelastung und psychischen Komorbiditäten.

BACKGROUND: Patients with single-sided deafness (SSD) and asymmetric hearing loss (AHL) are increasingly being treated with cochlear implants (CI) due to the demonstrated improvements in auditory abilities and quality of life. To date, there are few published studies in which these two groups are comparatively studied. The aim of the current study was to examine which factors differ between those two patient groups, especially preoperatively. METHODS: A secondary analysis of the previously published raw data of 66 prospectively recruited CI patients (21 SSD/45 AHL) was performed. In addition to the hearing outcome, tinnitus distress (tinnitus questionnaire), health-related quality of life (Nijmegen Cochlear Implant Questionnaire, NCIQ), stress (Perceived Stress Questionnaire, PSQ), and psychological comorbidities (General Depression Scale, ADS­L and Generalized Anxiety Disorder scale, GAD-7) were assessed in SSD and AHL patients pre- and postoperatively. RESULTS: Preoperatively, SSD patients showed significantly higher scores in the NCIQ subdomains "elementary" and "advanced sound perception" than the AHL group. Stress (PSQ) and anxiety symptoms (GAD-7) were significantly higher preoperatively in SSD patients than in AHL patients. After CI, these differences were strongly reduced, with minimal differences being detectable between the groups in the investigated domains postoperatively. CONCLUSION: SSD and AHL patients differ significantly preoperatively in terms of their subjective hearing assessment and psychosocial parameters. In SSD patients, psychological stress factors may have a stronger impact on the quality of life than in AHL patients. These aspects should be taken into account in the preoperative counseling and postoperative rehabilitation.

Lateralization Pattern of the Weber Tuning Fork Test in Longstanding Unilateral Profound Hearing Loss: Implications for Cochlear Implantation.

The Weber tuning fork test is a standard otologic examination tool in patients with unilateral hearing loss. Sound should typically lateralize to the contralateral side in unilateral sensorineural hearing loss. The observation that the Weber test does not lateralize in some patients with longstanding unilateral deafness has been previously described but remains poorly understood. In the present study, we conducted a retrospective analysis of the medical records of patients with unilateral profound hearing loss (single-sided deafness or asymmetric hearing loss) for at least ten years. In this patient cohort, childhood-onset unilateral profound hearing loss was significantly associated with the lack of lateralization of the Weber tuning fork test (Fisher's exact test, p < 0.05) and the absence of tinnitus in the affected ear (Fisher's exact test, p < 0.001). The findings may imply a central adaptation process due to chronic unilateral auditory deprivation starting before the critical period of auditory maturation. This notion may partially explain the poor outcome of adult cochlear implantation in longstanding single-sided deafness. The findings may suggest a role for the Weber test as a simple, quick, and economical tool for screening poor cochlear implant candidates, thus potentially supporting the decision-making and counseling of patients with longstanding single-sided deafness.

Effects of auditory rehabilitation with cochlear implant on tinnitus prevalence and distress, health-related quality of life, subjective hearing and psychological comorbidities: Comparative analysis of patients with asymmetric hearing loss (AHL), double-sided (bilateral) deafness (DSD), and single-sided (unilateral) deafness (SSD).

Introduction: Auditory rehabilitation with a cochlear implant (CI), in many cases, positively impacts tinnitus. However, it is unclear if the tinnitus-related benefit of CI is equal for patients with various indications for CI. Therefore, this study aimed to determine differences in tinnitus prevalence and distress, health-related quality of life, subjective hearing, perceived stress, and psychological comorbidities between patients diagnosed with asymmetric hearing loss (AHL), single-sided (unilateral) deafness (SSD), and double-sided (bilateral) deafness (DSD) before and six months after cochlear implantation. Methods: One hundred-one CI candidates were included in this prospective study (39 AHL patients, 23 DSD patients, and 39 SSD patients). The patients completed questionnaires measuring tinnitus distress, health-related quality of life, subjective hearing, perceived stress, and psychological comorbidities before and 6 months after CI. Results: The prevalence of tinnitus in the entire cohort (80.2% before CI) decreased 6 months after CI to 71.3%. The DSD group had the lowest tinnitus prevalence at both time points. The degree of tinnitus-induced distress decreased significantly in all three groups after CI. Differences in quality of life, subjective hearing, and psychological comorbidities between the groups at the study onset disappeared after CI. Significant correlations existed between anxiety, depression, and tinnitus distress in AHL and SSD but not in DSD patients before and after CI. Discussion: Our results demonstrate significant differences between the three groups of CI candidates, which might affect the implantation outcome. These differences suggest a need for personalized psychological counseling during the auditory rehabilitation process, focusing on anxiety and depressive symptoms for SSD and AHL patients.

Challenges of Cochlear Implantation in Intralabyrinthine Schwannoma Patients: Surgical Procedures and Auditory Outcome.

Intralabyrinthine schwannoma (ILS) is a rare benign tumor of the inner ear potentially causing unilateral sensorineural hearing loss and vertigo. This study evaluated the outcome of one surgical session comprising microsurgical ILS resection and cochlear implantation in terms of surgical feasibility, complications, and auditory outcome. Ten clinically and histologically confirmed ILS patients included in this study (three women and seven men; mean age 56.4 ± 8.6) underwent surgery between July 2015 and February 2020. Eight patients had intracochlear tumor location; the remaining two had vestibulocochlear and intravestibular ILS. One of the three following methods was used for tumor removal: an extended cochleostomy, subtotal cochleoectomy, or a translabyrinthine approach. Although negligible improvement was observed in two of the patients, two patients were lost to follow-up, and one opted out from using CI, the speech perception of the five remaining ILS patients improved as per the Freiburg Monosyllable Test (FMT) from 0% before surgery to 45- 50% after the implantation. Our study supports the presented surgical approach's feasibility and safety, enabling tumor removal and hearing restoration shortly after surgery.

Improvement of Working Memory and Processing Speed in Patients over 70 with Bilateral Hearing Impairment Following Unilateral Cochlear Implantation.

Several studies demonstrated the association of hearing disorders with neurocognitive deficits and dementia disorders, but little is known about the effects of auditory rehabilitation on the cognitive performance of the elderly. Therefore, the research question of the present study was whether cochlear implantation, performed in 21 patients over 70 with bilateral severe hearing impairment, could influence their cognitive skills. The measuring points were before implantation and 12 months after the first cochlear implant (CI) fitting. Evaluation of the working memory (WMI) and processing speed (PSI) was performed using the Wechsler Adult Intelligence Scale 4th edition (WAIS-IV). The audiological assessment included speech perception (SP) in quiet (Freiburg monosyllabic test; FMT), noise (Oldenburg sentence test; OLSA), and self-assessment inventory (Oldenburg Inventory; OI). Twelve months after the first CI fitting, not only the auditory parameters (SP and OI), but also the WMI and PSI, improved significantly (p < 0.05) in the cohort. The presented results imply that cochlear implantation of bilaterally hearing-impaired patients over 70 positively influences their cognitive skills.

White Matter Lesions as Possible Predictors of Audiological Performance in Adults after Cochlear Implantation.

The presented prospective study investigated whether structural brain damage, measured with the Fazekas score, could predict hearing rehabilitation outcomes with cochlear implantation (CI). With a follow-up period of 24 months, this study included 49 bilaterally, postlingually hearing impaired CI candidates for unilateral CI (67.3 ± 8.7 years; 20 men, 29 women) older than 50 at the time of implantation. The differences in the predictive value between two age groups, 50-70 year-olds (mid-age; n = 26) and over 70-year-olds (elderly; n = 23), were analyzed. The patients were evaluated using speech perception (SP) measured in quiet (Freiburg monosyllabic test; FMT) and noise (Oldenburg sentence test; OLSA). The subjective hearing ability was assessed using Oldenburg inventory (OI). The Fazekas PVWM score predicted postoperative speech perception two years after CI in the mid-age population. The periventricular white matter lesions (PVWM) could explain 27.4% of the speech perception (FMT) variance. Our findings support the hypothesis about the influence of pre-existing WMLs on CI outcome. We recommend the evaluation of Fazekas score as a predictive factor for post-implantation hearing ability.

Single-centre experience and practical considerations of the benefit of a second cochlear implant in bilaterally deaf adults.

PURPOSE: Bilateral cochlear implant (CI) implantation is increasingly used in the auditory rehabilitation of bilaterally deafened adults. However, after successful unilateral implantation, objective patient counselling is essential. METHODS: We investigated the extra benefit of a second CI in adults in terms of health-related quality of life, tinnitus, stress, anxiety, depression, quality of hearing, and speech recognition. Hearing ability was assessed by using the Freiburg monosyllable speech discrimination test (FB MS) and the Oldenburg sentence test with azimuth variations. In a prospective patient cohort, we administered validated questionnaires before a CI, after a first CI and after a second CI implantation. RESULTS: The study included 29 patients, made up of nine women and 20 men. The median time between the first and the second implantation was 23 months. The mean total NCIQ score and TQ before a CI improved significantly after both implantations. Stress, anxiety, and depression were stable over time and were not significantly affected by CI implantations. Speech recognition with noise significantly improved after the first and again after the second CI. Correlation analysis showed a strong connection between auditory performance and HRQoL. CONCLUSION: We demonstrated that a unilateral CI benefitted many fields and that the second sequential CI leads again to additional improvement. Bilateral CI implantation should, therefore, be the standard form of auditory rehabilitation in deafened adults.

Binaural Hearing Rehabilitation Improves Speech Perception, Quality of Life, Tinnitus Distress, and Psychological Comorbidities.

OBJECTIVES: To determine and compare the benefit of binaural hearing rehabilitation via cochlear implantation (CI) on speech perception, assessment of auditory abilities, tinnitus distress, health-related quality of life (HRQoL) and psychological comorbidities in patients suffering from asymmetric hearing loss (AHL) as well as bilaterally-deafened and sequentially bilaterally-implanted patients. METHODS: 53 patients were implanted between 2011 and 2016. 24 AHL patients were implanted unilaterally, using a hearing aid on the other side. 29 bilaterally-deafened patients were sequentially implanted bilaterally. Speech perception, subjective hearing quality, HRQoL, tinnitus distress, anxiety, depressiveness, perceived stress level and coping abilities were evaluated before implantation, as well as 6 and 24 months postoperatively. RESULTS: Before CI, AHL and bilaterally-deaf patients showed significant differences regarding assessment of auditory abilities, speech discrimination, tinnitus distress and HRQoL. 24 months after CI both groups significantly improved in those scales. We could not find a significant difference between the groups after 2 years. Tinnitus distress significantly decreased 6 and 24 months postoperatively in both groups. CONCLUSIONS: This study demonstrates the long-term benefit of binaural hearing rehabilitation in AHL and bilaterally-deaf patients not only regarding speech perception but also HRQoL, tinnitus distress and subjective hearing quality. Bilaterally-deafened patients present lower scores preoperatively, but they did not differ from AHL patients 2 years after CI. Up to now, this is the first study evaluating the outcome of CI in AHL patients compared to bilaterally-implanted patients and demonstrating the benefit of binaural hearing rehabilitation in these specific groups.

Hearing Implants in the Era of Digitization. / Hörimplantate im Zeitalter der Digitalisierung.

The last years' developments could show that the rehabilitation with hearing implants is a field with the highest potential for development and innovation in otorhinolaryngology. New or extended indications were seen with developments of implants, new surgical techniques, and respective rehabilitation strategies.With the background of limited resources, the increasing number of subjects suffering from hearing disorders, the extended indications and thus the increasing number of CI carriers as well as the need of life-long CI follow-up are one of the major challenges of the future. In order to cope with this situation, completely new strategies are required beside a close interdisciplinary cooperation and continuous development of the therapy. In this context, digitization of all these processes plays a key role.This manuscript will describe and discuss the current developments from the perspective of a cochlea implant (CI) providing hospital. The contribution will elucidate manifold digital applications that may be implemented in all phases of CI provision, starting with patient information about the possibilities of hearing screening and preoperative evaluation up to life-long follow-up and clinical research.The focus is mainly placed on specific applications that play a particular role in the development of digital progresses and structures in the context of cochlea implantation and that are crucial for understanding the further development.The options of simplified fitting result for example from automated MAP creation (artificial intelligence); remote care networks (telemedicine, apps) foster the active contribution of the patients themselves and allow completely new types of location-independent healthcare (automated technical implant control, individual settings, upgrades). Central databases may create backups of the current MAP (for example in cases of repair), and document technical data and the hearing performance. Some applications described here, are already implemented in the routine, others are currently being developed.Understanding the possibilities of digitization and their implementation in the context of hearing rehabilitation with hearing implants as well as the recognition of the enormous potential for effective, time-efficient structures is essential in order to use this potential. We as ENT specialists are important protagonists in the healthcare system and beside our high specific expertise we have to meet the requirements of our qualification with regard to digital applications so that we might actively contribute to the success of this process.

Clinical Significance and Indication for Surgical Treatment of Occult Cervical and Intraglandular Nodal Involvement in Parotid Malignancy.

PURPOSE: Surgical management, including the extent of surgery, for patients with parotid malignant tumors has been challenging. The aims of the present study were to identify the factors associated with survival, estimate the incidence of occult nodal and intraglandular metastases, and determine the predictive influence on overall and disease-free survival, with consideration of the surgical extent. PATIENTS AND METHODS: We implemented a retrospective cohort study and identified incident cases with parotid malignant tumors from 2003 to 2016 at a single quaternary medical care and cancer center. A medical record analysis was performed retrospectively of the patient and histopathologic data. The predictor variables were age; tumor grade; T stage; N stage; facial nerve palsy; perineural, vascular, and lymphovascular invasion; completion parotidectomy; elective neck dissection; and pN0 versus pN+. Overall and disease-free survival were evaluated as primary and secondary outcome variables. Data analysis was performed as a time-to-event analysis (Kaplan-Meier method). RESULTS: A total of 102 patients with parotid carcinoma had undergone surgery from 2003 to 2016 at the University Hospital Freiburg, Germany; 69 patients had undergone completion parotidectomy (total, 36; radical, 33). A total of 13 patients had occult intraparotideal lymph node metastases after elective completion parotidectomy. All the patients who had undergone elective neck dissection and clinically had had no nodal involvement (cN0) had had no pathologic lymph node metastases found (pN0). However, the predictive factors for cervical nodal involvement were high-grade tumors, locally advanced tumors (cT3-cT4), and the presence of intraparotideal nodal metastases. CONCLUSIONS: The rate of occult cervical metastases in the present study was surprisingly low (0%). However, owing to the various predictive factors, elective neck dissection should be performed for high-grade or locally advanced (stage T3-T4) parotid malignoma. Moreover, we highly recommend performing completion parotidectomy for all high-grade tumors and also for locally advanced (stage T3-T4) low-grade tumors, owing to the >12% of occult intraparotideal metastases found in the present study.

Asymmetric hearing loss and the benefit of cochlear implantation regarding speech perception, tinnitus burden and psychological comorbidities: a prospective follow-up study.

OBJECTIVES: We determined the audiological outcome, the subjective and objective hearing quality in patients suffering from asymmetric hearing loss (AHL). Furthermore, we evaluated psychological comorbidities and tinnitus burden before and after cochlear implantation. STUDY DESIGN: Prospective cohort study. METHODS: 44 AHL patients were unilaterally implanted with a multichannel cochlear implant between 2011 and 2016. Speech discrimination (Freiburg Monosyllable Word Test, Oldenburg Sentence Test) was measured before, 6 and 12 months after implantation. Subjective hearing quality, health-related quality of life (HRQoL), tinnitus burden, anxiety, depressiveness, perceived stress level and coping abilities were evaluated before implantation, 6 and 12 months postoperative using specific validated questionnaires (Oldenburg Inventory, Nijmegen Cochlear Implantation Questionnaire, Tinnitus Questionnaire, General Anxiety Disorder-7, Depression Scale, Perceived Stress Questionnaire and Cope Inventory). RESULTS: Subjective and objective hearing quality, speech discrimination and health-related quality of life were significantly increased in AHL patients. Tinnitus burden significantly decreased over the 12 postoperative months. No significant alteration was observed for anxiety, depressiveness, coping abilities and stress level. CONCLUSIONS: This study demonstrates that cochlear implantation achieves hearing rehabilitation, increases HRQoL and decreases tinnitus burden in patients suffering from AHL. Subjective hearing quality increased, while tinnitus burden significantly decreased 6 and 12 months after implantation. HRQoL in AHL patients is an important factor to focus on and is significantly increased postoperatively. In contrast, general anxiety, depressiveness, coping abilities and perceived stress level remained unaffected.

Head and neck rhabdomyosarcoma in children: a 20-year retrospective study at a tertiary referral center.

OBJECTIVE: The purpose of this study was to assess rhabdomyosarcomas (RMS) of the head and neck in pediatric patients with regard to clinical presentation, treatment, and survival. METHODS: Data were retrospectively obtained from patient charts with regard to RMS of the head and neck diagnosed between 1996 and 2016 at a tertiary referral center. Clinical course, treatment modalities, and side effects were analyzed. Survival analysis was carried out using the Kaplan-Meier method. RESULTS: Twenty-eight patients (17 male, 11 female) with a mean age at diagnosis of 6.8 ± 5.0 years have been included. Fourteen patients (50%) presented with painless swelling in the head and neck region and nine patients (32.1%) presented with cranial nerve deficit at initial diagnosis. The location of the majority of rhabdomyosarcomas was orbital (N = 9; 32.1%), followed by parapharyngeal (n = 4; 14.2%) and sinonasal (n = 3; 10.7%). All patients (n = 28; 100%) received polychemotherapy, additional adjuvant radiation therapy was carried out in 24 patients (85.7%), and surgery was performed in 12 cases (42.9%). The 5-year overall survival was 91.3%, and median progression-free survival was 46 ± 67.4 months. Common side effects resulting from chemotherapy and radiation therapy included neutropenia, mucositis, nausea and vomiting, and radiodermatitis, long-term side effects included microsomia and facial mutilation. CONCLUSION: Multimodality treatment of rhabdomyosarcomas of the head and neck in pediatric patients may lead to a 5-year overall survival of up to 91.3%.

Cochlear Implantation of Bilaterally Deafened Patients with Tinnitus Induces Sustained Decrease of Tinnitus-Related Distress.

OBJECTIVE: Tinnitus is a common symptom of hearing impairment. Patients who are bilaterally hard of hearing are often affected by tinnitus. However, they cannot undergo any of the standard tinnitus therapies, since they rely on hearing. Cochlear implantation (CI) used to treat severe hearing disabilities, such as bilateral hearing loss, was also shown to reduce tinnitus. Our goal was to determine if CI induces sustained reduction of tinnitus. We performed prospective, longitudinal analyses of tinnitus-related distress in a uniform group of bilaterally deafened patients after CI. PATIENTS AND METHODS: The homogenous sample consisted of 41 patients who met the inclusion criteria and were consecutively included in this study. The impact of unilateral CI on tinnitus-related distress, health-related quality of life (HRQoL), and hearing abilities was studied with validated instruments. The follow-up appointments were scheduled at 6, 12, and 24 months after CI surgery. During the appointments, hearing abilities were estimated with monosyllabic Freiburg test, whereas the tinnitus-related distress, the HRQoL, and the subjective hearing were measured with standard questionnaires [Tinnitus Questionnaire (TQ), Nijmegen Cochlear Implantation Questionnaire, and Oldenburg Inventory, respectively]. RESULTS: Tinnitus-related distress decreased significantly from the mean TQ score of 35.0 (SD = 19.6) prior to surgery to the mean TQ = 27.54 (SD = 20.0) 6 months after surgery and remained sustained low until the end of follow-up period. In addition, CI significantly improved the hearing abilities and the HRQoL of all patients. CONCLUSION: The results from our prospective study suggest that in a homogenous sample of bilaterally deafened, implanted patients who report having tinnitus prior to surgery, CI alone not only improves the hearing abilities but also significantly reduces the tinnitus-related distress and improves the HRQoL in a sustained way.